Intro to Neo/Peds

Cardiac Anomalies and ECMO

Guided Homework

Due to the submission box by 10/14/24 by 11:59

• Review the course material

• Fill in The Guided Notes over Congenital Cardiac Defects and ECMO power points & complete the Homework questions at the end.

• Read & study Walsh Ch. 11 and 38

Cardiac Anomalies

1. Categories

   1. Right-to-Left shunts result in profound cyanosis at birth from blood shunting from the right side of the heart to the left side of the heart_______________________________

   2. Left-to-Right shunts result in blood shunting from the left side of the heart to the right side of the heart_______________________________

1. Right-to-Left Shunts (cyanotic)

1. Tetralogy of Fallot (TOF)

   1. What are the four specific defects present with TOF?

      1. Ventricular septal defect

      2. Overriding aorta (over the septum)

      3. Pulmonary artery stenosis

      4. Right ventricular hypertrophy

1. Blood is shunted from the right ventricle through the large VSD________________________ into the overriding aorta

2. Clinical Presentation/Diagnosis

   1. What causes the profound cyanosis? Profound cyanosis presents when the ductus arteriosus constricts and begins to close

1. What is the appearance of the heart shadow on a chest x-ray? Like a boot

2. What causes “Tet” spells? Hyperpnea (exaggerated deep breathing), irritability and prolonged crying

1. What procedure is used to confirm the diagnosis? ECHO

1. What are the components of treatment for a newborn with TOF?

   1. Supportive care—oxygen as needed

   2. Administer prostaglandin E1 to keep ductus open and improve pulmonary circulation

   3. Surgical correction:

      1. Temporary shunt created between aorta and PA to improve blood flow

      2. Complete repair by patching the hold between the ventricles and pulmonary valve is repaired or replaced. May remove thickened muscle below the pulmonary valve or widen the pulmonary artery 

2. Transposition of the Great Vessels (TGV)

   1. Describe the anatomic changes that occur in a newborn with TGV reversal of the origin of the aorta and the pulmonary artery so that the RV leads to the aorta and the Left ventricle leads to the pulmonary artery. Often accompanied by a PDA and ASD. Venous and arterial blood must find some way to mix

   2. Venous and arterial blood mixes through a 

      1. ASD

1. VSD

1. PDA 

1. Clinical Presentation/Diagnosis

   1. When does cyanosis and hypoxia occur?

Profound cyanosis and hypoxia occur during the first week of life as the PDA closes. 

1. What is the appearance of the heart shadow on the chest x-ray? Appears like an egg on a string

1. What procedure is used to confirm the diagnosis?

ECHO

1. What are the components of treatment for a newborn with TGV?

   1. Give prostaglandin E1 to keep ductus open

   2. Supportive care (oxygen if needed)

   3. Balloon atrial septostomy to create shunt for mixing blood

   4. Surgical correction (arterial switch)

1. Coarctation of the Aorta 

   1. Define Coarctation of the Aorta

Severe narrowing of the aortic lumen resulting in decreased blood flow through the aorta

1. What type of shunt results from a preductal coarctation? Right to left shunt occurs if the shunt is from a preductal coarctation

1. What type of shunt results from a postductal coarctation? Left to right shunt occurs if the shunt is from a post ductal coarctation.

1. Why is it important to measure blood pressure in all four extremities when the presence of Coarctation of the Aorta is suspected? Blood pressure may be higher in the upper extremities than the lower extremeties

1. What are the components of treatment for a newborn with Coarctation of the Aorta?

   1. Supportive care (digitalis for heart failure)

   2. Administer prostaglandin E1 to keep ductus open

   3. Surgical correction to resect the coarctation is usually necessary

1. Truncus Arteriosus

   1. Describe the anatomic changes that occur in truncus arteriosus Single common vessel for both the aorta and the pulmonary artery over a vsd. The pulmonary artery and the aorta exit the heart together

   2. Blood flow for the systemic and pulmonary circulations are delivered through ___the single ventricle_________________________________.

   3. What are the components of treatment for a newborn with truncus arteriosus?

      1. Be conservative with oxygen to maintain high PVR (rule of 40’s—keep PaCO2 around 40 mmHg and PaO2 around 40 mmHg) to balance pulmonary and systemic blood flow. May consider sub ambient oxygen therapy if too much pulmonary blood flow is present. Acidosis and hypercarbia causes increased PVR due to pulmonary vasoconstriction 

      2. Pulmonary artery banding to control blood flow

      3. serVSD closure

      4. Surgical repair—complete biventricular repair in the newborn period

2. Hypoplastic Left Heart Syndrome

   1. Describe the anatomic changes that occur in hypoplastic left heart syndrome. Seriously underdeveloped left ventricle with stenosis or atresia of the mitral valve. Dependent upon right ventricle for blood supply to both the pulmonary and systemic circulations

   2. What techniques are employed to balance pulmonary and systemic vascular resistance?

      1. Oxygen therapy is withheld

      2. Often deliver sub ambient oxygen (17-20%)

      3. Target SpO2 before repair 70-80%

      4. Rule of 40’s: PaCO2 and PaO2 of 40 mmHg

   3. What are the components of treatment for a newborn with hypoplastic left heart syndrome?

      1. Prostaglandin E1 to keep ductus open

      2. Avoid oxygen (consider sub ambient)

      3. Hypercarbic gas mixtures: acidosis and hypercarbia causes pulmonary vasoconstriction, must hav ETCO2 inline, only works if intubated

      4. Complex surgical repair requiring several stages

      5. Chronic fluid management and inotropic support 

1. Total Anomalous Pulmonary Venous Return (TAPVR)

   1. Describe the anatomic changes in a newborn with TAPVR pulmonary veins fail to connect to the LA, may connect to the superior and inferior vena cava resulting in unilateral pulmonary circulation only. ASD is vital for survival in addition to a PDA

1. Pulmonary veins may connect superior_______________ and ___inferior vena cava_______________________ resulting in unilateral pulmonary circulation only.

2. ASD_____________________ is vital for survival in addition ot a patent ductus arteriosus.

3. Typical chest x-ray finding is a Snowman shaped heart______________________.

4. Treatment/management

   1. Prostaglandin E1 to keep ductus open

   2. Balloon septostomy to create or maintain ASD 

   3. Surgical repair

1. Left-to-Right Shunts (Acyanotic)

   1. Atrial septal Defects (ASD)

      1. Describe the anatomic change that occurs in a newborn with ASD Failure of the foramen ovale to close or failure of the septum to develop correctly. Blood moves from the LA to the RA through the opening

1. Clinicalive presentation/diagnosis

   1. What is the cause of the right heart failure? May occur due to increased workload on RV

1. Identify the presentation of a newborn with an ASD

   1. Initially healthy, term infant with failure to thr

1. Difficulty feeding 

1. Turns blue occasionall with crying, feeding, burping, playing or sleeping on side due to increased intrathoracic pressure 

1. Right heart failure may occur 

1. What are the components of treatment for a newborn with ASD?

   1. Oxygen as needed

1. Surgical correction if severe symptoms

1. Ventricular Septal Defect (VSD)

   1. Describe the anatomic change that occurs in a newborn with a VSD opening between the ventricles, majority of the CO is pushed through the opening into the RV and then through the lungs resulting in increased pulmonary blood flow (increased CVP and PAP)

1. Treatment/Management

   1. How are small defects treated? Spontaneous closure and supportive therapy to include oxygen, diuretics and digoxin

1. How are large defects treated? Surgery to patch the septal wall and pulmonary artery banding to restrict flow

1. Patent Ductus Arteriosus (PDA)

   1. Why does blood shunt from the aorta into the pulmonary circulation? Blood may shunt from the aorta into the pulmonary circulation if left heart pressures are higher than right heart pressures 

   2. How is the patient evaluated for the presence of a PDA? Right to left shunts diagnosed with pre/post ductal PaO2 differene of >/= 15 mmHg or pre/post SpO2 >/=15%. Confirmed with an ECHO

1. How is a newborn with a PDA treated?

   1. Prostaglandin inhibitor

   2. Oxygen

   3. Fluid restrictions and diuretics

   4. Surgical clamp of duct (ligation) 

1. Atrioventricular Septal Defect (AVSD)

   1. What other terms are used to refer to an atrioventricular septal defect? Complete atrioventricular canal

1. Explain the components of the defect absence of atrioventricular septum resulting in a single common atrial ventricular chamber

1. This is the most common congenital heart defect in patients with ___Trisomy 21 (Down Syndrome)___________________.

2. List the components of treatment of a newborn with atrioventricular septal defect

   1.   Children without CHF may be managed with diuretics and digoxin

   2. SpO2 75-90% may be tolerated well (pulmonary vasoconstriction to control pulmonary blood flow).  Oxygen should be delivered cautiously since higher SpO2 will reduce PVR and increase Pulmonary blood flow

   3. Surgical correction and valve reconstruction

3. Dextrocardia

   1. Explain dextrocardia Situs Inversus the heart as well as abdominal and thoracic organs are mirror images of their normal positions

1. Explain dextrocardia without Situs Inversus The heart is on the right side but the other organs remain in their usual positions

1. How is dextrocardia diagnosis? Dextrocardia is often detected through routine imaging, such as chest x-rays, ECHOs which will show the heart’s unusual position

1. What is the clinical significance? While some individuals may lead normal lives with no symptoms, others, especially those with associated heart defects, may require medical intervention or surgery to correct related conditions

1. Monitoring of Patients with Cardiac Anomalies

   1. What is pulse pressure? Pulse pressure is the difference between systolic and diastolic blood pressure

1. What does a narrow pulse pressure suggest? Suggestive of a low stroke volume

1. What are potential causes of decreased ETCO2 readings?

   1. ETT dislodgement or obstruction

   2. Air trapping

   3. Decreased pulmonary blood flow

   4. Low cardiac output 

   5. hyperventilation

2. What are potential causes of increased ETCO2 readings?

   1. Hypoventilation

   2. Fever

   3. Malignant hyperthermia

3. What are potential causes of increased gradient between PaCO2 and ETCO2?

   1. Increased dead space

   2. Lower pulmonary blood flow

   3. High airway pressures leading to alveolar over distention

   4. Pulmonary embolism

1. What can be determined by the magnitude or size of the ETCO2 tracing and the reading? Efficacy of CPR

1. A precipitous drop in the ETCO2 may indicate what? May indicate a loss of pulmonary blood flow as a result of shunt thrombosis

1. What is a common cause of persistent bradycardia and desaturation in congenital heart defects? Inadequate ventilation

Knowledge Check

1. Acyanotic heart diseases are diseases in which blood shunts from left to right. The congenital heart disease that is NOT considered acyanotic is:

1. Atrial septal defect

2. ventricular septal defect

3. transposition of the great arteries

4. patent ductus arteriosus

1. Which of the following vessels return blood to the right ventricle via the right atrium?

I. Pulmonary vein

II. Inferior vena cava

III. Superior vena cava

IV. Coronary sinus

A. I and IV only

B. II and III only

C. I, II, and III only

D. II, III, and IV only

1. For which of the following congenital cardiac defects may spontaneous closure of the ductus arteriosus be catastrophic?

I. Tetralogy of Fallot with pulmonary atresia

II. Atrial septal defect

III. Severe coarctation of the aorta

IV. Hypoplastic left heart syndrome

A. I and III only

B. III and IV only

C. I, II, and IV only

D. I, III, and IV only

Cardiac Anomalies and ECMO

Guided Homework

Due to the submission box by 10/14/24 by 11:59

• Review the course material

• Fill in The Guided Notes over Congenital Cardiac Defects and ECMO power points & complete the Homework questions at the end.

• Read & study Walsh Ch. 11 and 38

Cardiac Anomalies

1. Categories

   1. Right-to-Left shunts result in profound cyanosis at birth from blood shunting from the right side of the heart to the left side of the heart_______________________________

   2. Left-to-Right shunts result in blood shunting from the left side of the heart to the right side of the heart_______________________________

1. Right-to-Left Shunts (cyanotic)

1. Tetralogy of Fallot (TOF)

   1. What are the four specific defects present with TOF?

      1. Ventricular septal defect

      2. Overriding aorta (over the septum)

      3. Pulmonary artery stenosis

      4. Right ventricular hypertrophy

1. Blood is shunted from the right ventricle through the large VSD________________________ into the overriding aorta

2. Clinical Presentation/Diagnosis

   1. What causes the profound cyanosis? Profound cyanosis presents when the ductus arteriosus constricts and begins to close

1. What is the appearance of the heart shadow on a chest x-ray? Like a boot

2. What causes “Tet” spells? Hyperpnea (exaggerated deep breathing), irritability and prolonged crying

1. What procedure is used to confirm the diagnosis? ECHO

1. What are the components of treatment for a newborn with TOF?

   1. Supportive care—oxygen as needed

   2. Administer prostaglandin E1 to keep ductus open and improve pulmonary circulation

   3. Surgical correction:

      1. Temporary shunt created between aorta and PA to improve blood flow

      2. Complete repair by patching the hold between the ventricles and pulmonary valve is repaired or replaced. May remove thickened muscle below the pulmonary valve or widen the pulmonary artery 

2. Transposition of the Great Vessels (TGV)

   1. Describe the anatomic changes that occur in a newborn with TGV reversal of the origin of the aorta and the pulmonary artery so that the RV leads to the aorta and the Left ventricle leads to the pulmonary artery. Often accompanied by a PDA and ASD. Venous and arterial blood must find some way to mix

   2. Venous and arterial blood mixes through a 

      1. ASD

1. VSD

1. PDA 

1. Clinical Presentation/Diagnosis

   1. When does cyanosis and hypoxia occur?

Profound cyanosis and hypoxia occur during the first week of life as the PDA closes. 

1. What is the appearance of the heart shadow on the chest x-ray? Appears like an egg on a string

1. What procedure is used to confirm the diagnosis?

ECHO

1. What are the components of treatment for a newborn with TGV?

   1. Give prostaglandin E1 to keep ductus open

   2. Supportive care (oxygen if needed)

   3. Balloon atrial septostomy to create shunt for mixing blood

   4. Surgical correction (arterial switch)

1. Coarctation of the Aorta 

   1. Define Coarctation of the Aorta

Severe narrowing of the aortic lumen resulting in decreased blood flow through the aorta

1. What type of shunt results from a preductal coarctation? Right to left shunt occurs if the shunt is from a preductal coarctation

1. What type of shunt results from a postductal coarctation? Left to right shunt occurs if the shunt is from a post ductal coarctation.

1. Why is it important to measure blood pressure in all four extremities when the presence of Coarctation of the Aorta is suspected? Blood pressure may be higher in the upper extremities than the lower extremeties

1. What are the components of treatment for a newborn with Coarctation of the Aorta?

   1. Supportive care (digitalis for heart failure)

   2. Administer prostaglandin E1 to keep ductus open

   3. Surgical correction to resect the coarctation is usually necessary

1. Truncus Arteriosus

   1. Describe the anatomic changes that occur in truncus arteriosus Single common vessel for both the aorta and the pulmonary artery over a vsd. The pulmonary artery and the aorta exit the heart together

   2. Blood flow for the systemic and pulmonary circulations are delivered through ___the single ventricle_________________________________.

   3. What are the components of treatment for a newborn with truncus arteriosus?

      1. Be conservative with oxygen to maintain high PVR (rule of 40’s—keep PaCO2 around 40 mmHg and PaO2 around 40 mmHg) to balance pulmonary and systemic blood flow. May consider sub ambient oxygen therapy if too much pulmonary blood flow is present. Acidosis and hypercarbia causes increased PVR due to pulmonary vasoconstriction 

      2. Pulmonary artery banding to control blood flow

      3. serVSD closure

      4. Surgical repair—complete biventricular repair in the newborn period

2. Hypoplastic Left Heart Syndrome

   1. Describe the anatomic changes that occur in hypoplastic left heart syndrome. Seriously underdeveloped left ventricle with stenosis or atresia of the mitral valve. Dependent upon right ventricle for blood supply to both the pulmonary and systemic circulations

   2. What techniques are employed to balance pulmonary and systemic vascular resistance?

      1. Oxygen therapy is withheld

      2. Often deliver sub ambient oxygen (17-20%)

      3. Target SpO2 before repair 70-80%

      4. Rule of 40’s: PaCO2 and PaO2 of 40 mmHg

   3. What are the components of treatment for a newborn with hypoplastic left heart syndrome?

      1. Prostaglandin E1 to keep ductus open

      2. Avoid oxygen (consider sub ambient)

      3. Hypercarbic gas mixtures: acidosis and hypercarbia causes pulmonary vasoconstriction, must hav ETCO2 inline, only works if intubated

      4. Complex surgical repair requiring several stages

      5. Chronic fluid management and inotropic support 

1. Total Anomalous Pulmonary Venous Return (TAPVR)

   1. Describe the anatomic changes in a newborn with TAPVR pulmonary veins fail to connect to the LA, may connect to the superior and inferior vena cava resulting in unilateral pulmonary circulation only. ASD is vital for survival in addition to a PDA

1. Pulmonary veins may connect superior_______________ and ___inferior vena cava_______________________ resulting in unilateral pulmonary circulation only.

2. ASD_____________________ is vital for survival in addition ot a patent ductus arteriosus.

3. Typical chest x-ray finding is a Snowman shaped heart______________________.

4. Treatment/management

   1. Prostaglandin E1 to keep ductus open

   2. Balloon septostomy to create or maintain ASD 

   3. Surgical repair

1. Left-to-Right Shunts (Acyanotic)

   1. Atrial septal Defects (ASD)

      1. Describe the anatomic change that occurs in a newborn with ASD Failure of the foramen ovale to close or failure of the septum to develop correctly. Blood moves from the LA to the RA through the opening

1. Clinicalive presentation/diagnosis

   1. What is the cause of the right heart failure? May occur due to increased workload on RV

1. Identify the presentation of a newborn with an ASD

   1. Initially healthy, term infant with failure to thr

1. Difficulty feeding 

1. Turns blue occasionall with crying, feeding, burping, playing or sleeping on side due to increased intrathoracic pressure 

1. Right heart failure may occur 

1. What are the components of treatment for a newborn with ASD?

   1. Oxygen as needed

1. Surgical correction if severe symptoms

1. Ventricular Septal Defect (VSD)

   1. Describe the anatomic change that occurs in a newborn with a VSD opening between the ventricles, majority of the CO is pushed through the opening into the RV and then through the lungs resulting in increased pulmonary blood flow (increased CVP and PAP)

1. Treatment/Management

   1. How are small defects treated? Spontaneous closure and supportive therapy to include oxygen, diuretics and digoxin

1. How are large defects treated? Surgery to patch the septal wall and pulmonary artery banding to restrict flow

1. Patent Ductus Arteriosus (PDA)

   1. Why does blood shunt from the aorta into the pulmonary circulation? Blood may shunt from the aorta into the pulmonary circulation if left heart pressures are higher than right heart pressures 

   2. How is the patient evaluated for the presence of a PDA? Right to left shunts diagnosed with pre/post ductal PaO2 differene of >/= 15 mmHg or pre/post SpO2 >/=15%. Confirmed with an ECHO

1. How is a newborn with a PDA treated?

   1. Prostaglandin inhibitor

   2. Oxygen

   3. Fluid restrictions and diuretics

   4. Surgical clamp of duct (ligation) 

1. Atrioventricular Septal Defect (AVSD)

   1. What other terms are used to refer to an atrioventricular septal defect? Complete atrioventricular canal

1. Explain the components of the defect absence of atrioventricular septum resulting in a single common atrial ventricular chamber

1. This is the most common congenital heart defect in patients with ___Trisomy 21 (Down Syndrome)___________________.

2. List the components of treatment of a newborn with atrioventricular septal defect

   1.   Children without CHF may be managed with diuretics and digoxin

   2. SpO2 75-90% may be tolerated well (pulmonary vasoconstriction to control pulmonary blood flow).  Oxygen should be delivered cautiously since higher SpO2 will reduce PVR and increase Pulmonary blood flow

   3. Surgical correction and valve reconstruction

3. Dextrocardia

   1. Explain dextrocardia Situs Inversus the heart as well as abdominal and thoracic organs are mirror images of their normal positions

1. Explain dextrocardia without Situs Inversus The heart is on the right side but the other organs remain in their usual positions

1. How is dextrocardia diagnosis? Dextrocardia is often detected through routine imaging, such as chest x-rays, ECHOs which will show the heart’s unusual position

1. What is the clinical significance? While some individuals may lead normal lives with no symptoms, others, especially those with associated heart defects, may require medical intervention or surgery to correct related conditions

1. Monitoring of Patients with Cardiac Anomalies

   1. What is pulse pressure? Pulse pressure is the difference between systolic and diastolic blood pressure

1. What does a narrow pulse pressure suggest? Suggestive of a low stroke volume

1. What are potential causes of decreased ETCO2 readings?

   1. ETT dislodgement or obstruction

   2. Air trapping

   3. Decreased pulmonary blood flow

   4. Low cardiac output 

   5. hyperventilation

2. What are potential causes of increased ETCO2 readings?

   1. Hypoventilation

   2. Fever

   3. Malignant hyperthermia

3. What are potential causes of increased gradient between PaCO2 and ETCO2?

   1. Increased dead space

   2. Lower pulmonary blood flow

   3. High airway pressures leading to alveolar over distention

   4. Pulmonary embolism

1. What can be determined by the magnitude or size of the ETCO2 tracing and the reading? Efficacy of CPR

1. A precipitous drop in the ETCO2 may indicate what? May indicate a loss of pulmonary blood flow as a result of shunt thrombosis

1. What is a common cause of persistent bradycardia and desaturation in congenital heart defects? Inadequate ventilation

Knowledge Check

1. Acyanotic heart diseases are diseases in which blood shunts from left to right. The congenital heart disease that is NOT considered acyanotic is:

1. Atrial septal defect

2. ventricular septal defect

3. transposition of the great arteries

4. patent ductus arteriosus

1. Which of the following vessels return blood to the right ventricle via the right atrium?

I. Pulmonary vein

II. Inferior vena cava

III. Superior vena cava

IV. Coronary sinus

A. I and IV only

B. II and III only

C. I, II, and III only

D. II, III, and IV only

1. For which of the following congenital cardiac defects may spontaneous closure of the ductus arteriosus be catastrophic?

I. Tetralogy of Fallot with pulmonary atresia

II. Atrial septal defect

III. Severe coarctation of the aorta

IV. Hypoplastic left heart syndrome

A. I and III only

B. III and IV only

C. I, II, and IV only

D. I, III, and IV only

EXTRACORPOREAL MEMBRANE OXYGENATION

Is ECLS and ECMO the same therapy (explain)?

ECLS is extracorporeal life support and is a broad term that covers any type of life support that occurs outside the body.  ECMO is extracorporeal membrane oxygenator which is an invasive technique in which blood is drained from the venous system, mechanically pumped through an artificial lung and reinfused to the patient through either the pulmonary or systemic systems.

• What is a VAD (explain in detail)? A VAD is a mechanical pump that supports the heart’s ventricles in moving blood through the body when they are too weak to do so on their own.

• Define LVAD Left ventricular assist device, 

• Define RVAD Right ventricular assist device

• Define BiVAD Both right and left ventricular assist device

• What are the indications for a VAD?

  1. Severe heart failure

  2. Biventricular heart failure

  3. Bridge to transplant

  4. Bridge to recovery

  5. Destination therapy

• What is ECCO₂R (explain in detail)? Extracorporeal carbon dioxide remover. Carbon dioxide is removed and reinfused back into the patient’s venous circulation.  The native heart is used to propel the blood forward

• Indications

• Acute exacerbation of COPD with severe hypercarbia that cannot be managed by non invasive or mechanical ventilation alone 

• Acute respiratory distress symdrome: allows for ventilator settings to be reduced to prevent further lung injury.

• Bridge to transplant: give ability to control CO2 in a patient population that is typically difficult to control

• Weaning from mechanical ventilation: those wih chronic respiratory conditions may struggle with hypercapnia during weaning.  ECCO2R assists in management of CO2 facilitating weaning

• Hypercapnic respiratory failure due to neuromuscular disorder: conditions like ALS, Muscular dystrophy or spinal cord injuries lead to chronic CO2 retention. ECCO2R assists with controlling CO2 when non invasive or invasive ventilation is insufficient or not well tolerated 

• Metabolic acidosis with hypercapnia: Certain metabolic conditions like diabetic ketoacidosis or renal failure result in both acidosis and CO2 retention.  ECCO2R can assist in removal of CO2

• Explain the limitations of ECCO₂R:  Does not oxygenate the blood

• What is ECMO (explain in detail)? An invasive technique in which blood is drained grom the venous system, mechanical pumped through an artificial lung, and reinfused to the patient either through the pulmonary or the systemic systems  

• The primary aims 

  • To support _____organ function_____________________ in patients with respiratory_______ or cardiorespiratory______________________ failure in order:

• To provide time for the disease process to reverse or further evaluate the underlying condition

• To determine if medical or surgical treatment options are available

• A drug delivery device for ____oxygen___________________ 

• Used with ___neonatal______________, ___pediatric_____________, and adult___________ populations. 

• Can provide complete cardiopulmonary bypass.

• Artificial ___lung___________ and Artificial heart_______________ 

• Is the ____pinnacle___________________ of life support

  • But not ____everyone is a candidate_____________________________________________

Define the following Terms:

• Persistent pulmonary hypertension of the newborn: a clinical syndrome that occurs as a result of disruption in the normal perinatal fetal-neonatal circulatory transition characterized by inappropriately sustained elevated PVR and alterations in pulmonary vasoreactivity, resulting in right to left shunting of blood across the foramen ovale or the PDA

• Meconium aspiration syndrome Aspiration of meconium or amniotic fluid containing meconium either in utero or during birth.  Meconium causes a release of cytokines, serious airway obstruction, air trapping and enhanced growth of bacteria in the lung.  It also can compete with surfactant components for adsorption to the alveolar surface, and enzymes in meconium can break down certain surfactant components, causing secondary surfactant deficiency.

• Sepsis Life threatening organ dysfunction caused by a dysregulated host response to infection. Septic shock is a subset of sepsis in which underlying circulatory, cellular, and metabolic abnormalities are associated with a greater risk of mortality than sepsis alone

• Congenital diaphragmatic hernia Occurs when there is a failure of fusion of the pleura and peritoneum that would normally form the diaphragm resulting in a herniation of the abdominal contents into the thoracic cavity 

• Congenital cardiac defects A malformation of the heart, aorta or other large blood vessels that is the most common of major birth defect in newborns.  Can result in right to left shunting or left to right shunting of blood.  There are many different classifications of CHD but the most common is to separate into cyanotic (right to left shunt) and acyanotic (left to right shunt) defects. Cyanotic defects include: hypoplastic left heart syndrome, total anomalous pulmonary venous return, tetralogy of fallot, truncus arteriosus, transposition of great arteries (vessels), and pulmonary atresia with an intact ventricular septum, coarctation of the aorta. Acyanotic defects include: patent ductus arteriosus, atrial septal defect, ventricular septal defect, atrioventricular septal defect (also known as complete atrioventricular canal.

The terms you just defined are a few pathologies that ECMO may be used as a treatment to manage the pathology.

Explain in detail the indications for:

• Neonatal Population: 

  • Decision guiding aspect is the reversible nature of the underlying condition and the time of the decision. 

• Oxygen index (OI) >40 if on CMV or >60 if on HFOV

• Length of time on mechanical ventilation. >7-10 days may represent irreversible lung condition

• Gestation age > 32 weeks (most will consider >34 weeks as cut off)

• Pediatric Population:

  • A reversible pathology is known or suspected, and in whom providing ECMO poses less risks than not providing extracorporeal support

• Offered in all patients with acute severe respiratory failure who demonstrate progressive persistent failure despite optimized conventional therapies and maneuvers. 

• Conditions

  1. ARDS

  2. Viral or bacterial pneumonia

  3. Aspiration pneumonia

  4. Status asthmaticus

  5. Mediastinal masses

  6. Pulmonary hemorrhage

  7. Severe air leak

  8. Bridge to transplant

  9. Perioperative support to airway surgery

  10. Temporary lung non-function (example: extensive bronchial alveolar lavage)

• Cardiac Failure

  • Cardiogenic shock unresponsive to standard medical therapies

  • Preprocedural stabilization for inadequate systemic cardiac output

• Persistent systemic systolic pressure < 50 mmHg with lactic acidosis

• Central venous oxygen saturation <60% or arteriovenous oxygen saturation difference >30% in cyanotic CHD

• Altered mental status due to low CHD

• Failure to wean from cardiopulmonary bypass or low CO in the postoperative period

• Postoperative arrhythmia

• Circulatory failure due to other etiologies like

• Pulmonary hypertension

• Pulmonary emboli

• Sepsis

• Anaphylaxis

Explain in detail the contraindications (both absolute and relative) for neonatal patients: Absolute: lethal chromosomal disorder (includes T-13 and T-18), severe brain damage or neuro deficit (IVH >Grade 2), uncontrollable bleeding and vessel size too small for cannulation.

Relative: Irreversible organ damage (unless considered for organ transplant), <2 kg (consider vessel size via ultrasound) and <34 weeks (may get approval to 32 weeks if vessel size is big enough)

Explain in detail the contraindications (both absolute and relative) for pediatric patients: ECMO should not be employed when the patient has an overall poor prognosis or when there is high likelihood of survival with unacceptable disability.

Relative: Large IVH with need for surgical intervention, hypoxic cardiac arrest without adequate CPR, irreversible underlying cardiac or lung pathology (not a transplant candidate), pulmonary hypertension with chronic lung disease, chronic multi organ dysfunction, incurable malignancy, and allogenic bone marrow recipients with pulmonary infiltrates. Conditions with worse prognosis on ECMO support like hepatic failure, pertussis infection in infants, fungal pneumonia and immunodeficiency; vessel anomalies or having previously been clipped or ligated for prior ECMO; localized site infection.

Explain in detail the contraindications for cardiac failure patients: Patient level factors—ECMO support would be unlikely to facilitate survival without likelihood of major morbidity (prolonged state of cardiogenic shock >6 hours will unlikely benefit from initiation of ECMO. Procedural factors—inability to achieve vascular or central access for cannulation.

Explain the following parts of an ECMO circuit:

• Oxygenator serves as the gas exchange organ (artificial lung); adds oxygen to the blood and removes carbon dioxide

• Pump provides mechanical pumping action for the circuit and cardiovascular system (cardiac output). Roller and centrifugal pump designs

• Heat exchanger rewards blood to body temperature before returning it to the patient, also serves as a bubble filter

• Sweep gas bled in oxygen that removes CO2.  If ECMO circuit is too efficient, can exchange oxygen for CO2

Two ways to cannulate a patient: 

• Veno-Arterial Cannulation (explain) supports both the cardiac and the lung functions

• Veno-Venous Cannulation (explain) used for patients who only need pulmonary support and have intact cardiac function

Clinical management: 

• Hemodynamics—flows are maintained to control cardiac output (CO)/Cardiac index (CI) and mean arterial pressure (MAP) are used to assess response to flow.  More flow=more support. Minimum circuit flow >60 mL/kg/min. Initial post cannulation support is usually 100-150 mL/kg/min. Peds and adults need less overall flow, infants and cardiac patients may require >80 mL/kg/min or more. Normal CI >2.5 to 4.0; MAPs >40 to maintain renal function.

• Renal function—maintain fluid balance with CVVHD (continuous veno-venous) hemodialysis or CRRT (continuous renal replacement therapy)

• Temperature regulation 

• Organ perfusion—SvO2 normal 75-80%

• Lab tests—coagulation studies, electrolyte values, ABGs, renal values, circuit health 

• Neurological assessment—CT studies (when necessary), daily head ultrasounds (infants)

• Respiratory support—lung protective settings or “rest” settings (PCV-SIMV, 12-15 breaths, PIP 20-25 cmH2O, PEEP 6-10 cmH2O and FiO2 < 0.4)

• Fluid balance—CVVHD, CRRT or SCUF (slow continuous ultra filtration)

• Skin management/pressure ulcer prevention 

Complications:

• Thrombus

• Air embolism

• Bleeding/coagulopathy

• Infection

• Kidney injury

• Non-pulsatile flow (VA is non-pulsatile, VV is pulsatile because the patient’s heart is what generates blood flow/stroke volume

• Immobilization

• Decreased perfusion

Explain in detail the process of liberating the patient from ECMO Consider liberation from ECMO when ECMO flow = 20-30 mL/kg/min, lung compliance is in acceptable range, chest x-ray appearance has improved and there is a need to increase CO2 bled into circuit (signifies lungs are working).  Increase ventilator settings to recruit lungs that have been rested then perform a trial separation by unclamping the bridge between the venous and arterial sides of the circuit and clamping the circuit after the bridge so that the blood circulates from the patient through the bridge and back to the patient. If blood pressure, heart rate and SpO2 remain acceptable and ABG results are within acceptable range after 30 min of clamping, the patient is removed from ECMO.

What is the main goal of ECMO therapy? The main goal is discharge without any disability

• Question: What does the abbreviation mPaw mean? Mean airway pressure

                  What does the abbreviation Paw mean? Airway pressure

• What is the equation to calculate OI? OI=mPaw x (FiO2/PaO2) x 100

• Calculate the OI:

Patient 1: is on an FiO2 of 0.7, has a mPaw of 30 cmH20, and a PaO2 of 110 torr.

Patient 2: is on an FiO2 of 0.5, has a mPaw of 27 cmH20, and a PaO2 of 60 torr.

Patient 3: is on an FiO2 of 0.6, has a mPaw of 15 cmH20, and a PaO2 of 80 torr.

Patient 4: is on an FiO2 of 1.0, has a mPaw of 35 cmH20, and a PaO2 of 90 torr.

•                     Normal OI is _________, OI of ___________ indicates lung disease, OI of __________ is increased mortality, and an OI of ____________ indicates ECMO

How does the following impact patient selection?

•               Length of time on mechanical ventilation

• Gestation age

•                   Intracranial (intraventricular) hemorrhage

•                                   Pulmonary disease

Knowledge check!

1.   Which of the following statements describe veno-arterial ECLS?

I. A cannula is inserted into the subclavian vein for the removal of blood.

II. Blood is removed from the venous circulation through the right internal jugular vein.

III. Blood returns to the heart through the subclavian artery.

IV. A cannula is inserted into the right common carotid artery for arterial return.

A. I and III only

B. I and IV only

C. II and III only

 D. II and IV only

2.   What effect can a mediastinal shift have on the function of a venous cannula?

 A. Decreased venous return

B. Increased venous pressure

C. Increased laminar flow in the system

D. Decreased circuit transit time

3.   In the gas membrane exchanger, what limits the transfer rate of oxygen across the membrane?

A. The flow of blood

 B. The concentration gradient of the gas across the membrane

C. The thickness of the blood film between the membrane layers

D. The flow of gas in relationship to the flow of blood

                                                                                                 RESB 344 Introduction to Neonatal/Pediatric Respiratory Care

Therapeutic Procedures for Neonatal & Pediatric Disorders Homework

Fall Semester

Due to the submission box by 9/30/2024 by 11:59

• Review the course material

• Fill in The Guided Note over the Aerosol administration and Pharm power point & complete the Homework questions at the end.

• Read & study Walsh Ch. 34 and 39

• DO NOT USE GOOGLE TO ANSWER YOUR QUESTIONS.  YOU ARE NOT TESTED ON GOOGLE INFORMATION

Therapeutics Guided Notes

AEROSOL

• Compared to adults:

  • Smaller______ airway diameters

  • Higher_____ and less________ regular breathing rate

  • Nose___ breathing

  • Mouthpiece administration difficult________

  • Techniques depend on_______ age and developmental ability

Factors that reduce the rate and depth of aerosol particle deposition

1. Gravitational sedimentation—aerosol particles fall out due to gravity

2. Inertial impaction—primary mechanism for deposition of particles with diameters of 5µm or greater and an important mechanism for particles as small as µm in diameter

3. Diffusion—the primary mechanism for deposition of particles less than 3 μm in diameter in the airway

4.Translocation—aerosol medication first must efficiently deposit in the airway and then must translocate across the mucous barrier; affected by particle size, charge, solubility and the biophysical properties of secretions

5. Delivery flow rate and density of gas used—Heliox will not carry medication as far as oxygen 

6. Heat and Humidity of inhaled gas

7. Solubility of medication

8. Location of device in the mechanical ventilator circuit—MDI: Adapter placed at patient wye; Neb: placement approximately 30 cm from the ETT is more efficient than placement between the patient wye and ETT

9. Breathing pattern of patient—fast, shallow respirations cause medications to be deposited higher in the airway. For patients who are on a ventilator, delivery of the MDI synchronized with inspiration results in approximately 30% greater efficiency of aerosol delivery compared with actuation during exhalation.

Explain: 

• Clarks Rule: An adjustment made to medication dose for patients 2-17 years old.  Defined as the weight of the patient in pounds divided by the average weight of 150 pounds (68 kg) multiplied by the adult dose of a drug equals the pediatric medication dose.  

  • Patient weight in pounds = then multiplied by the adult dose

150

• Youngs Rule: An adjustment made to medication dose for patients 2-17 years old. Defined as an equation to calculate pediatric medication dosage based on the patient's age and the known recommended adult dose.  Age of patient divided by the age + 12; then multiplied by the recommended adult dose

  •     Age     = then multiplied by the adult dose

Age + 12

Device Selection & Compliance

• Newborns to 4 years of age

  • ____ Nebulizer or pMDI with VHC

• Children 4 to 5 years of age

  • _____ Nebulizer or pMDI with VHC

• Children 6 to 12 years of age

  • pMDI with or without VHC

  • DPI or breath-actuated pMDI

Emergency Bronchodilator Resuscitation

Read this section in your textbook and summarize the important points (pages 567-569)

Consider the following “high-points” in your summary Patient/Family Interview, home drugs, and dosages, intermittent vs. continuous aerosol therapy, undiluted bronchodilator administration benefit.

Onset of exacerbation often times will start 12-36 hours prior to the ED visit. This means that the child has often used rescue inhalers/meds at home for a substantial amount of time without relief.  Medications used at the hospital need to be of increased doses or different medications since the normal medications aren’t working. Along with the increased WOB and SOB, the child and parents/care givers are going to be anxious, exhausted and uncomfortable.

Interview questions should include the following in addition to normal patient interview questions

• When did symptoms start?

• Any known trigger exposures?

• What medication, dose and frequency has been used at home

• Has this happened before?

• Has the child ever needed to be intubated due to an asthma exacerbation? (increases likelihood of happening again)

Medications:

• Usually will administer Atrovent as first step

• Albuterol doses increased to 2.5 mg (if rescue dose is less) to 5 mg or 4-8 puffs via MDI with a VHC every 20 min for the first hour

• If relief isn’t achieved after the first hour with the above treatment, continuous delivery of Albuterol of doses between 2.5 mg to 15 mg per hour can be delivered.  This can be achieved by premixing the concentration and using an IV pump to deliver the medication into the nebulizer at a rate of 6-8 mL/min or use of a mini Heart neb running at 10 L/min oxygen to deliver 30 mL/hour.  

• An alternative to continuous is to use undiluted bronchodilator where 1-2 mL straight Albuterol (5-10 mg) is nebulized.  This requires close monitoring due to the increased chance of  adverse effects from the increased dose.

• Keep in mind, poor relief from bronchodilators may indicate a non-bronchoconstriction cause of the wheezing and increased WOB (tracheitis, bronchiolitis or even foreign body aspiration)

Other Medications for Aerosol Delivery

Home Care & Monitoring Compliance

Patient education requires adherence to written medicine________ and action plans__________ since these are critical in adhering to the prescribed therapy.

Administered along ADLs makes this regiment easier to remember.  Place MDI next to a toothbrush so there is a reminder to use this after the teeth have been brushed.

Keep diary of medication use helps to form habits.

RESB 344 Introduction to Neonatal/Pediatric Respiratory Care

Fetal Development & Birth Homework

Fall Semester 2024

FRIDAY CLASS ASSIGNMENT:

Due to the submission box by 8/30/24 by 11:59 pm (It is due for everyone on this day)

• Review the course material

• Read & study Walsh Ch. 1-3

• Watch the following videos

Fetal Heart Rate Monitoring- Part 1: https://www.youtube.com/watch?v=1PwGRDnXwow  

Heart Format: https://www.youtube.com/watch?v=uwswhoKfkmM 

Fetal Circulation & Baby’s First Breath: https://www.youtube.com/watch?v=OV8wtPYGE-I 

HOMEWORK:

All questions must be answered in complete sentences:

1. At what time are you considered a fetus, neonate, infant, and child? 

Fetus: from approximately 8 weeks gestation until birth

Neonate: From birth to 1 month of age

Infant: from 2 month of age to 1 year of age

Child:  >1 year

1. In what order and timeframe do the major organs develop?

Heart: begins to form around day 18 gestation and starts beating around week 4 of gestation

Neural tube begins developing into the central nervous system around week 3-4 of gestation

Limb buds begin to develop around week 5 of gestation

Brain becomes identifiable around week 5 of gestation

Liver starts to form around week 6 of gestation

Respiratory system begins to form around weeks 3-6 beginning with the trachea then major bronchi then pleura.  Weeks 5-16 is the development of the remaining conducting airways.  Weeks 17-26 development of airway framework and respiratory acini.  Weeks 26-36 increase complexity of saccules.  Weeks 36 and on development of alveoli

Kidneys begin development around week 6 of gestation

Pancreas and intestines begin around week 7

Eyes and ears begin to develop around week 8

1. Describe the development of the lungs of the fetus.

5 Stages of lung development: Embryonic, pseudoglandular, canalicular, saccular and alveolar.

• Embryonic: first 2 months of gestation, primitive lung development as lung buds emerge from the pharynx 26 days post conception. Left and right pul veins start to develop about 5 weeks gestation, respiratory epihelium develops from the endoderm (foregut bud), the diaphragm also develops and is completed appox 7 weeks gestation

• Pseudoglandular: 8 weeks to 16 weeks gestation, extensive subdividing of conducting airways, acini may appear, lymphatics appear in the hilar region, cilia appear in tracheal epithelium, goblet cells, submucosal glands and airway cartilage develop; airways, arteries and veins have developed in the pattern corresponding to that found in an adult

• Canalicular: 17 to 26 weeks; growth of vascular bed, capillaries develop at 20 weeks of gestation and by 22 weeks have increased in number; appearance of surfactant; pulmonary acinar units formed distal to terminal bronchioles; exrauterine viability 22-24 weeks due to ability for gas exchange to take place

• Saccular: 26-35/36 weeks gestation; at the beginning  of this phase the terminal structures are referred to as saccules (smooth walled cylindrical structures); development of mature alveoli can be seen about 32 weeks; increased potential for gas exchange

• Alveolar: about 36 weeks to 18 months post gestation; not easily distinguishable from saccular phase; alveolar maturation and proliferation, important point is alveolarization is rapidly progressing during the period of development from late fetal to early neonatal life

• Lung development is progressing—meaning it will not be completed until about 8 years of age

1. What is the placenta?

The placenta is the vascular interface between the fetus and the mom that allows for gas and nutrient exchange between the chorionic villi and the umbilical cord.  Its three main functions are to transport nutrients, antibodies and hormones to the fetus; responsible for all gas exchange between the fetus and the environment (organ of respiration) and removes waste.  Gas exchange is by passive diffusion (no contact between blood supply of mom and blood supply of fetus); oxygenated blood returns to the fetus via fetal venules; maintenance of pressure gradients depends on normal maternal blood flows and blood gases.

1. What is the umbilicus and what are the components?

The umbilicus supplies oxygenated blood to the fetus from the placenta through 1 umbilical vein and returns deoxygenated blood from the fetus to the placenta via 2 umbilical arteries.  The arteries and vein are surrounded by Wharon’s Jelly which prevents kinking that would result in occluding blood flow

1. Describe the function and abnormalities of the amniotic fluid.

Amniotic fluid is fluid that surrounds the fetus inside of the amnion sac.  Its purpose is to absorb shock and prevent trauma to the fetus; stabilize temperature; permit fetal movement and prevent pressure points during labor. Abnormalities of amniotic fluid include Oligohydramnios (inadequate amount of fluid)—Potter’s syndrome, fetal renal agenesis, fetal urethral obstruction and pulmonary hypoplasia; and Polyhydramnios (>2000 mL of amniotic fluid)—severe brain malformation like hydrocephalus, anencephaly or spina bifida; fetal GI malfunction like esophageal atresia; down’s syndrome, congenital heart defect or prematurity

1. What is the Lecithin / Sphingomyelin ratio?

Ratio traditionally used to predict lung maturity (2 types of surfactant).  Normal L/S ratio is 2:1 to 2.5:1.  <2:1 ratio is significant for immature fetal lung development.  Test results are given as a ratio of lecithin to sphingomyelin. The range of results are:

• A ratio of less than 1.5:1. This means that your baby's lungs are immature. If born now, your baby may have breathing problems.

• A ratio between 1.5:1 and 1.9:1. This means that your baby may be at risk for immature lungs and breathing problems.

• A ratio of more than 2:1. This means that your baby has mature lungs and is ready for life outside the uterus.

1. What are lung complications associated near/or around birth?

Clearance of fetal lung fluid is essential for normal neonatal respiratory adaptation.  Retention of fetal lung fluid can result in TTN (transient tachypnea of the newborn—very fast RR); Type II RDS (oxygenation issues); impaired gas exchange; occurs more frequently after C-sections; may require positive pressure ventilation or CPAP to enhance absorption

1. Describe fetal circulation.

Oxygenated blood from the mom travels to the fetus through the umbilical vein. There are 3 normal shunts (Foramen Ovale between the atria, Ductus Arteriosus between the aorta and pulmonary artery, Ductus Venous bypasses the liver) to shunt oxygenated blood away from the fetal lungs and liver since the mother’s lungs and liver perform most of the metabolic functions required by the same fetal organs.  Fetal circulation allows the shunted blood to go through the fetal heart and pump to the placenta where gas exchange occurs.  The deoxygenated blood returns to the mother through the 2 umbilical arteries.  

1. What areas of fetal circulation are high or low pressure?

The area of high pressure is the pulmonary vasculature to impede blood flow through the fetal lungs.  The area of low pressure is the systemic vasculature to enable blood flow through the heart into the systemic circulatory pathway bypassing the lungs and liver.

1. What is a shunt?

A specialized blood vessel (Ductus Arteriosus and Ductus Venosus) or an anatomical structure/opening (Foramen Ovale) that allows blood to bypass certain areas of the heart or lungs that are not fully functional or needed because the mother’s organs fulfill these roles

1. What is amniocentesis?

An amniocentesis is a needle aspiration of amniotic fluid and cellular composition for analysis of Rh compatibility; lung maturity; certain genetic disorders; fetal age/sex; inborn errors of metabolism and meconium.

1. What are Decels?

Decels are decelerations of the fetal heartrate.  Early decel:  fetal HR decelerates at the same time as a contraction occurs then returns to normal between contractions. This can be due to stimulation of the Vagus nerve following head compression, increased ICP activated impulses from medulla through Vagus nerve to the heart.  Usually no clinical significance.  Late deceleration: Fetal HR decreases after onset of contractions and returns to normal after contraction has ended (lag in response time) due to impaired maternal blood flow to placenta from compression of placental vessels.  Usually associate with fetal asphyxia (hypoxia) and low APGAR scores.  Treat by giving mom O2 and monitor mom’s hemodynamic status.  Variable decelerations: no relationship between contractions and decelerations of fetal HR.  Can present as an abrupt increase in fetal HR and return to preexisting levels.  This is the most common decel and is usually associated with compression of the umbilical cord by fetal parts.  Long periods may result in hypoxia, acidosis then asphyxia. Treat by repositioning mom to left side

1. What is APGAR?

Standardized assessment tool of a newborn based on HR, respiratory effort, muscle tone, reflex irritability and skin color.  Normal apgar score is 7-10.  Newborns with a score of 4-6 have moderate depression of their vital signs and newborns with a score of 0-3 have severely depressed vital signs and are at great risk of dying unless actively resuscitated.  Assessed at 1 and 5 minutes post delivery.  If the newborn has not reached a score of 7 by 5 minutes, they must be reassessed every 5 minutes until they do.

1. Describe the sequence of events that occur in the normal development of the pulmonary system and stages of lung development. 

Embryonic (3-6 weeks gestation) development of trachea, major bronchi and pleura; Pseudoglandular (5-16 weeks gestation) development of remaining conducting airways and preacinar arteries; Canalicular (17-26 weeks gestation) development of vascular bed and airway framework for respiratory acini; Saccular (26-36 weeks gestation) increased complexiy of saccules and microcirculation; Alveolar (36-40 weeks gestation) development of alveoli and alveolar capillary network.

1. Explain the importance of pulmonary surfactant, its components, how and when it is developed, the relationship to gestational age, and identify the gestational age for premature infants developing greater risks for RDS.

The primary purpose of surfactant is to lower the surface tension in the alveolus. This allows the lung to easily open to allow in air. Without surfactant the alveoli will not open. It is made up of phospholipids, neutral lipids, and proteins. Surfactant first appears around 22 weeks of gestation during the canalicular phase. The type II pneumocytes retain their cytoplasmic shape of their precursors and contain concentric layers of lipid and protein important for the production of surfactant called lamellar bodies. Between 22 and 24 weeks gestation, due to the development and expanding number of capillaries around the alveoli, gas exchange begins.  At this point viability becomes possible however, newborns born this prematurely are at greater risk of developing RDS.

1. Explain what an L/S ratio is, and what is normal and abnormal

Ratio traditionally used to predict lung maturity (2 types of surfactant).  Normal L/S ratio is 2:1 to 2.5:1.  <2:1 ratio is significant for immature fetal lung development.  Test results are given as a ratio of lecithin to sphingomyelin. The range of results are:

• A ratio of less than 1.5:1. This means that your baby's lungs are immature. If born now, your baby may have breathing problems.

• A ratio between 1.5:1 and 1.9:1. This means that your baby may be at risk for immature lungs and breathing problems.

• A ratio of more than 2:1. This means that your baby has mature lungs and is ready for life outside the uterus.

1. Discuss fetal lung fluid and describe mechanisms responsible for removal of fetal lung fluid

Fetal lungs secrete about 250-300 mL of fluid/day.  This fluid prevents alveolar collapse in utero.  It is swallowed or expelled into amniotic fluid and is essential for normal lung development as it prevents the developing lung from collapsing.  Fetal lungs make breathing like movements even though they are not participating in gas exchange.  Clearance of fetal lung fluid is essential for normal neonatal respiratory adaptation.  Removal of lung liquid begins with the passage through the birth canal as the thorax is compressed.  It continues after birth.  When breathing begins, air inflation shifts residual liquid from the lumen into distensible perivascular spaces around large pulmonary blood vessels and bronchi.  Accumulation of liquid in these connective tissue spaces, which are distant from the sites of respiratory gas exchange, allows time for small blood vessels and lymphatics to remove the displaced liquid with little or no impairment of neonatal lung function at this crucial juncture.

1. Describe the function of the placenta and the umbilical cord

The placenta is responsible for transporting nutrients, antibodies and hormones.  It is the organ of respiration for the fetus meaning it is responsible for all gas exchange between the fetus and the environment.  The placenta also removes waste.  

The umbilical cord is comprised of 2 umbilical arteries and 1 umbilical vein which are surrounded by Wharton’s jelly.  The umbilical vein transports oxygenated blood from the mother to the fetus and the 2 umbilical arteries transport deoxygenated blood from the fetus to the mom

1. Identify changes that occur with the transition to extrauterine life, discuss the factors that stimulate respiration at birth.  Discuss what intrapleural pressures are required for the first breaths and why it is high.

The first breath is triggered by touch, temperature change and chemical changes that occur when the umbilical cord is clamped/cut.  The initial pressure needed to open the lung is between -40 to-100 cmH2O.  The pressure is so high because the lungs have no FRC and must be stretched open.  

1. Identify the PaO2 of fetal arterial blood and the effects of fetal hemoglobin.

The PaO2 of fetal arterial blood is between 16-20 mmHg. The PvO2 of fetal venous blood is around 29 mmHg.  Despite the low venous PO2, sufficient blood oxygen content is possible due to a larger fetal hemoglobin concentration and a shift in the oxygen dissociation curve to the left creating a greater affinity for oxygen so less oxygen is released at the tissue level.  Fetal hemoglobin carries more oxygen at a given oxygen tension than maternal blood does.

1. Identify the factor causing a decrease in pulmonary vascular resistance and an increase in systemic vascular resistance after birth. Know the factors causing closure of each of the fetal shunts and the differences in the circulation of the neonate after birth.

Removal of the placenta creates an increase in the SVR.  Aeration of the lungs during the first few breaths after deliver decreased the PVR.  The shift in pressures causes blood to flow through the lungs instead of through the fetal shunts.  The lack of use/need causes a functional closure of the foramen ovale.  The Ductus Arteriosus remains open for about the first 15 hours after delivery to allow for a transition in circulation.  It typically closes completely by day 4 of age. As the shunts close, circulation begins to mimic that of an adult.

1. Identify various high-risk conditions, such as preeclampsia, multiple births, gestational diabetes, and infectious diseases that may adversely affect pregnancy outcome.

Preeclampsia is when pregnant women have high blood pressure, protein in their urine and swelling in their legs, feet and hands.  It can range from mild to sever and usually happens late in pregnancy, however it can happen earlier or just after delivery.

Preterm premature rupture of membranes is if the water breaks before the 37^th week of pregnancy. Leakage or complete emptying of amniotic fluid makes the mom susceptible to infections and puts stress on the baby.

Placenta previa is a problem during pregnancy when the placenta completely or partially covers the cervix

Other risk factors that place a pregnancy at increased risk for an adverse pregnancy outcome include:

• Diabetes mellitus

  • Pregestational (diagnosed before onset of pregnancy)—increased risk of developing diabetic ketoacidosis, proliferative retinopathy and preeclampsia/eclampsia, can lead to maternal cerebrovascular complications and HELLP syndrome (hemolysis, elevated liver enzymes and low platelets), DIC; adverse fetal outcomes—stillbirth; major fetal structural malformations (most common is congenital heart defects) and macrosomia (birth weight >4000 g)

  • Gestational (diagnosded during pregnancy)—can result in macrosomia, shoulder dystocia, stillbirth or neonatal metabolic disorders

• Infectious diseases

  • Group B strep—infants at risk for death or severe morbidity due to perinatal infection

  • Genital herpes—increased risk for vertical transmission to the infant

  • HIV—frequency of HIV is about 1 per 1000 pregnancies; rates have been decreased due to advent of cART and prophylactic c-sections

  • Hep B—high risk of becoming infected at birth; treated with anti-hepetitis B immunoglobulin

  • The following can significantly affect the mother, fetus and the fetoplacental unit

    • CMV

    • Rubella

    • Toxoplasma gondii

    • Listeria monocytogenes

    • Mycobacterial species

    • Syphilis

• Substance abuse by the mother

  • Alcohol—usually seen in women who comsume 4-6 drinks daily during pregnancy; results in Fetal Alcohol Syndrome (mental retardation, prenatal and postnatal growth restriction, and abnormalities of the brain, heart, spine and craniofacial structures)

  • Smoking—higher incidence of low birth weight; premature rupture of membranes; placental abruption and placenta previa; SIDS

  • Cocaine—results in placental abruption; preterm delivery and growth restriction

  • Opiates—can be either prescription or illicitly obtained; infants at risk for neonatal abstinence syndrome (go through withdrawal)

  • Other substances—infants born to women abusing drugs during pregnancy can have significant withdrawal symptoms after birth and tend to be small for gestational age

1. Describe the stages of labor.

Typically divided into 3 stages:

• Stage 1 has 3 phases

  • Phase 1:  called the latent phase; contractions are becoming more frequent (usually 5-20 minutes apart) however discomfort is minimal.  The cervix dilates (opens approx. 3-4 cm) and effaces (thins out).  Some women may not recognize that they are in labor if the contractions are mild and irregular.  Usually is the longest and least intense phase.  Usually admitted to the hospital during this phase

  • Phase 2:  called the active phase; is signaled by the dilation of the cervix from 4-7 cm.  Contractions become more severe and more frequent (usually 3-4 min apart)

  • Phase 3:  called the transition phase.  The cervix dilates from 8-10 cm; contractions are usually very bad and last 60-90 sec occurring every few minutes.  Most women feel the urge to push during this phase.

• Stage 2:  Begins when the cervix is completely opened and ends with the delivery of the baby.  Often referred to as the “pushing” stage.  The woman becomes actively involved by pushing the baby through the birth canal to the outside world.  When the baby’s head is visible at the opening of the vagina, it is called crowning.  The second stage is shorter than the first stage and may take between 30 min to 2 hours for a woman’s first pregnancy

• Stage 3:  After the baby is delivered the new mother enters the third and final stage of labor—delivery of the placenta.  Usually lasts just a few minutes and involves passage of the placenta out of the uterus and through the vagina.  Each labor experience is different and the amount of time in each stage will vary.  However labor in a first pregnancy usually last about 12 to 14 hours and is usually shorter for subsequent pregnancies

1. Explain what preterm labor is.

Labor that begins before 37 weeks gestation

1. Identify a medication used to stop labor and briefly describe how the action of this drug stops labor.

Tocolytics are the class of drugs given to stop preterm labor.  This class includes Magnesium sulfate, Beta-mimetic agents (terbutaline and ritodrine), indomethacin (prostaglandin inhibitor) or nifedipine (calcium channel blocker)

1. NEUTRAL THERMAL ENVIRONMENT

   1. What does the term neutral thermal environment mean?

An environment where the temperature and humidity are such that the neonate does not have to expend energy to maintain its core temperature. Heat production and heat loss are balanced

1. How does this help stabilize the neonate?

Hypothermia can cause vasoconstriction and a shift in the oxygen dissociation curve.  By maintaining an environment that prevents hypothermia from happening, this prevents the outcomes mentioned from happening

1. Describe how a neonate losses heat by the four illustrated mechanisms (Conduction, Convection, Evaporation, Radiation).

Conduction: transfer of heat through direct contact between materials or surfaces. Use an overhead heater to make sure the surface the neonate is placed on is warm to prevent heat loss to the surface it is laid on. Can place baby on mom’s chest to prevent while cord is being cut

Convection: heat transfer through the movement of air or fluids around the body.  Prevent cold air from blowing on the baby

Evaporation: heat loss through the transformation of liquid into vapor.  Dry and wrap baby in warm blankets to prevent heat loss through evaporation of amniotic fluid on skin

Radiation:  heat loss through electromagnetic waves without direct contact. Delivery rooms should be kept warm to prevent heat loss of the baby to the room temp

RESB 344 Introduction to Neonatal/Pediatric Respiratory Care

Examination & Assessment of the Neonatal/Pediatric Patient Homework

Fall Semester 2024

Homework Assignment due to the submission box by 9-9-24 by 11:59 pm

• Review the course material

• Read & study Walsh Ch. 4, 5, 6, 15, 17 and 18

HOMEWORK:

ALL QUESTIONS MUST BE ANSWERED IN COMPLETE SENTENCES:

1. What is the Ballard scale?

The Ballard Scale is an objective tool used to assess gestational age of a patient based on physical and neuromuscular maturity

1. What criteria are evaluated on the Ballard scale?

There are 2 pieces of the Ballard scale: Neuromuscular maturity and Physical maturity.  Neuromuscular maturity is assessed through posture, the “square window” created from bending the wrist, arm recoil, popliteal angle, the “scarf sign” created from bending the arm across the chest and the flexion of the heel to ear with lower extremities.  Physical maturity is assessed through skin, lanugo (fine hair covering the fetus that usually falls off shortly after birth, the purpose to provide warmth in utero), plantar surface (assessing presence of creases on the sole of the foot), breast bud development, eye/ear development (assessing fusion of eyelids and cartilage development and strength in the ear), genitals (males assessing for descended testicles; females assessing development of external clitoris and labia)

1. What is the ideal score for the Ballard scale?

A score of 35 and greater is ideal to demonstrate “term” gestation

1. What test/scale did the Ballard scale replace?

Dubowitz

1. What is the Silverman scale?

An objective scale used to assess respiratory distress in neonates

1. What is the ideal score for the Silverman scale?

The ideal score is 0, the lower the score the better

1. What are the initial steps of neonatal resuscitation?

Warm, dry, stimulate, position in a neutral (sniffing) position, suction mouth than nose

1. What are normal neonatal vital signs?

RR 30-60; HR 120-160; >600 g BP 45/20 with MAP 26, >100 g BP 48/25 with MAP 35, >2000 g 50/30 with MAP 40, >3000 g 50/35 with MAP 45, >4000 g 65/40 with MAP 50, newborn >12 hours old 75/50 with MAP 60; temp 97.5-99.3 degrees F

1. Describe the process of transillumination.

Transillumination of the chest is done to help diagnose a pneumothorax.  You place a light source against the chest wall.  If the chest lights up like a jack-o-lantern a pneumothorax should be high on the differential diagnosis list.  Compare side to side

1. Explain pre-ductal vs post-ductal oxygen saturations.

A pulse ox probe is placed on the right upper extremity (usually hand or wrist) and left lower (can use either)(usually around the arch of the foot) to assess the SpO2.  If persistent fetal circulation exists, there will be >2% difference between the 2 sites or both are low (<90%) despite the neonate looking healthy.

1. What are the areas/categories of patient inspection?

Respiratory function (inspect for nasal flaring, grunting, retractions, paradoxical respirations, thoracic and spine deformities); abdominal (assess shape—scaphoid, flat, distended, prune belly appearance or other abnormalities); head and neck (bruising, molding, assess fontanels, scalp edema), eyes/ears development and positioning, oral opening, development of jaw; inspect extremities for deformities; skin for color

1. What is Congenital Diaphragmatic Hernia (CDH)?

CDH is a hole in the diaphragm (usually the left) that allows the abdominal contents to float up into the thoracic cavity that should be occupied by the lung.  This results in an underdeveloped lung on the affected side.

1. What is myelomeningocele?

Myelomeningocele occurs when the neuro tube does not completely close during development of the spinal column resulting in a sac formation that protrudes though the patient’s back containing fluid, the spinal cord and nerves.

1. What is the proper procedure to implement for an infant know to have experienced meconium aspiration at birth?

Suction nose then mouth if the baby is vigorous (HR 100+, strong respirations and good muscle tone).  If baby not vigorous, NRP guidelines are to treat all newborns the same (suction nose then mouth) over intubation. 

1. What is periodic breathing in the newborn?

A common finding among premature newborns. Intermittent breathing pattern with periods of apnea of at least 5 seconds but no more than 20 seconds

1. What does the term “position” mean when referring to the x-ray beam?

Position refers to the position the patient is in for the x-ray.  The patient could be positioned supine (on back) or prone (on stomach).  If supine, the x-ray obtained will be an AP film as the patient will be laying with their back on the plate.  If the patient is prone, the x-ray obtained will be a PA film as the patient will be laying with their front side against the plate. 

1. Where should the endotracheal tube be positioned on a chest x-ray?

Mid thoracic trachea between the inferior clavicular border and the carina.

1. Referring specifically to x-rays, describe the type of x-ray recommended to diagnosis epiglottitis and the specific x-ray findings associated with this illness.

The recommended x-ray to diagnose epiglottitis is a lateral neck (airway) film.  You would expect to see a “thumb” in the area the epiglottitis is on the x-ray at the glottic opening of the trachea.

1. Explain how a patient would be positioned for the x-ray and what type of x-ray would be performed to assess a pleural effusion and to assess a pneumothorax

The patient would be positioned with the affected side down for a lateral decubitus film to diagnose a pleural effusion (look for fluid line in the dependent lung cavity).  The patient would be laying with the affected side up for a lateral decubitus film to diagnose a pneumothorax (air rises so the good lung is down to allow for air accumulation in the top lung)

1. Explain the recommended systematic approach for assessing a chest x-ray in a neo/peds patient.  Why is it important to use this approach?

You should have a systematic approach that is used every time an x-ray is assessed.  One example of this approach is to assess the Airway (is trachea midline without deviation, is the trachea patent?) Mediastinum (is the mediastinum normal size, is the mediastinum shift left/right, is the heart size normal, does the thymus appear normal?) Diaphragm borders (are the costophrenic angles sharp or blunted) Lungs (are the borders of the diaphragm shart, are there any areas of hyperlucency indicating a pneumothorax, areas of hyper opaque indicating consolidation or foreign body aspiration?) Bones (count the vertebrae—should see 9-10 vertebrae/ribs to indicated adequately inflated lungs, is there symmetry side to side, does the vertebral processes appear symmetrical?) Supportive devices (what is present, is it in the right location) Everything else (is there anything else that stands out, looks out of place, catches your eye?)

1. Describe what a “sail sign” is on a chest x-ray.  What does this finding indicate?

The sail sign is present on a chest x-ray in the right upper lung area.  It is an opaque area in the shape of a triangle or a sailboat sail.  This finding is indicative of a prominent thymus gland and is considered a normal finding.

1. Explain 3 barriers that the respiratory therapist is likely to encounter doing a physical exam and assessment on a pediatric patient that they likely would not encounter in an adult patient.

Stranger danger—patient hesitant to interact with RT due to them being a stranger.  The patient crying and being uncooperative during the assessment because they don’t feel good. The patient being too young to talk therefor any questions will be answered by a care provider.  If child is old enough to talk, may be afraid to answer questions honestly in fear of implicating a care provider for unsafe/unclean living conditions or other instances of child endangerment.  Care providers aren’t always honest either fearing losing custody of the child.

1. Why is it important to include living conditions and caregiver social history when performing a pediatric patient history?

Unclean living conditions are well documented sources of respiratory illnesses in pediatric populations.  It is also important to note who is responsible for the care and wellbeing of the child so they receive the education needed to keep the child healthy and out of the hospital.

1. Explain the 4 parts of a pediatric pulmonary exam and how you would perform each of them.

Auscultation: listening to the heart, lungs and GI system using a stethoscope

Inspection: Often needs to be done from the doorway before proceeding too far into the room.  Include vital signs, looking for signs of respiratory distress (increased WOB, tachypnea, tachycardia, retractions, head bobbing, cyanosis, nasal flaring), and chest wall symmetry (pectus excavatum, pectus carinatum, kyphosis, scoliosis, kyphoscoliosis, bell shaped thoracic cage or other abnormal chest wall shape)

Palpation: Rhoncal or bronchial fremitus, tactile fremitus, palpation of anterior neck (assessing tracheal position—midline or shifted to one side or the other?)

Percussion: performed by tapping one finger on one hand with a finger from the other.  Done as a way to assess for the presence of air (hyper resonant sound) or consolidation (dull percussion sound)

1. List 4 examples each of noninvasive and invasive lab assessment

Noninvasive: chest x-ray, ETCO2, chest CT, chest MRI, PFT, exercise tolerance (desat) test, sweat chloride test, skin allergy testing, sputum culture, overnight PSG

Invasive: ABG, CBG, VBG, bronchoscopy, pH probe, lung biopsy, CBC, serum immunoglobulin studies, TB skin test, barium swallow

1. Explain the difference between a diagnostic and a therapeutic bronchoscopy

A diagnostic bronchoscopy is performed in order to diagnose a lung condition of unknown etiology, may include a BAL

A therapeutic bronchoscopy is performed in order to remove secretions or a foreign body, done to improve lung conditions, could be done to aid in a difficult intubation or direct instillation of medication, whole lung lavage, lung biopsy, examination of the nasopharynx, aerodigestive evaluation. 

1. List 8 indications for a pediatric bronchoscopy

Symptoms—Persistent or recurrent respiratory symptoms are the most common indication for flexible bronchoscopy

• Diagnose causes of Stridor (Inspiratory or expiratory, Laryngomalacia, Intermittent laryngeal obstruction, Tracheomalacia, Bronchomalacia), investigate wheezes that don’t respond to conventional treatment (albuterol), Cough of unknown etiology, Investigate Radiographic abnormalities, Recurrent or persistent atelectasis or infiltrates, Bronchiectasis, Localized pulmonary consolidation or hyperinflation, Recurrent pneumonia, Hyperlucencies (Air trapping related to either intrinsic obstruction or extrinsic compression from lymphadenopathies, aberrant blood vessels, or mediastinal masses), Inhalation injury, Immunocompromised patient

1. List the supplies needed to perform a bronchoscopy

Light source, video recorder, and monitor

Lidocaine spray/jelly

Lukens trap

10-mL normal saline aliquots for lavage 

Monitoring equipment

Cardiac monitor, pulse oximeter, noninvasive blood pressure monitor, and emergency resuscitation cart 

Oxygen and suction equipment

Swivel adapter for an endotracheal tube, 

PEEP valves

Tracheostomy tubes, wire brushes for cytology, transbronchial needle catheters

PPE—Bronchs are aerosolizing procedures

1. What are 3 absolute contraindications and 4 relative contraindications for performing a bronchoscopy on a pediatric patient?

Absolute: absence of informed consent, inadequate facilities and personnel, risks outweigh benefits

Relative: Cardiovascular instability, Inability to oxygenate the patient adequately, Uncontrolled coagulopathy or bleeding, Severe bleeding diatheses or coagulopathy, Hypercapnia with acidosis, Bronchospasm, Hypoxemia, Severe pulmonary hypertension 

1. How would you prepare the pediatric patient for a bronchoscopy?

Obtain History and physical, Review of radiographic studies, review Current health status, note any Drug allergies, Obtain Informed consent, Nothing by mouth (NPO) for3 to 4 hours for infants < 6 months, 4 to 6 hours for older infants and toddlers or 8 hours for older children

RESB 344 Introduction to Neonatal/Pediatric Respiratory Care

Surfactant Replacement Therapy & Specialty Gases Guided Notes and Homework

Fall Semester

Due to the submission box by 10-3-2024 at 11:59 pm

• Review the course material

• Fill in The Guided Note over Surfactant Replacement Therapy & Specialty Gases. Complete the Homework questions at the end of this packet.

• Read & study Walsh Ch. 12 & 37

Surfactant Replacement Therapy Guided Notes

Discovery of Surfactant

• In the 1940’s a mucoprotein_______ was discovered in the lining of the lung that had surface tension lowering properties.

• In the 1950’s the Harvard School of Public Health discovered that surface forces___________ at the lung’s air-liquid interface contributes to the elastic recoil_________ of the lung.

• Also, in the 1950’s, it was discovered that the role of the alveolar lining layer mediates surface tension which stabilizes__________ the alveoli.

• Science linked lack of surfactant____________ to respiratory distress in pre-term infants by performing autopsies where the pre-term infant lacked “foam” in the airway.  The lack of “foam” was due to the lack of surfactant.

• Explain the Law of LaPlace as it pertains to Surfactant. (You may need to refer to your textbook Ch 12.) The Law of LaPlace states that the distending pressure is equal to double the surface tension divided by the radius of a sphere.  This means that the pressure to keep an alveoli open (distended) requires double the pressure created when the surface tension is divided by the radius.  It also demonstrates that at end exhalation when the alveoli are the smallest, the pressure required to keep the alveoli open would be higher than one at end inhalation.  However, with surfactant, that pressure is decreased by decreasing the surface tension or the pull inward that creates the “want” to collapse.  Surfactant also increases lung compliance.

Surfactant

Function

• This is any molecule that localizes on what type of surface? Aqueous surface

• In the lung, alveolar surfaces are lined with a layer of fluid which is the pulmonary surfactant.

• This fluid reduces_________ the surface tension in the lung in proportion____________ to the size of the alveoli.

• What does surfactant do to lung compliance? Increases it

• Surfactant maintains the Functional Reserve Capacity (FRC)_____________________________.

Without surfactant the lungs become ___stiff __________ and ____atelectasis______________________ forms.

With the lack of surfactant, the body will display increase_____ WOB, _____hypoxia___________, and respiratory failure______.

Composition

What are the functions of surfactant (Box 12-1 page 189 in textbook)

• Prevents collapse of lung during deflation (expiration)

• Lessens work of breathing (decreases oxygen consumption

• Optimizes surface are for gas exchange and ventilation perfusion matching

• Optimizes lung compliance (high at low lung volume and low at high lung volumes

• Protects the lung epithelium and facilitates clearance of foreign material

• Prevents capillary leakage of fluid into the alveoli

• Defends against microorganisms (infection)

Pulmonary surfactant is a complex mixture of specific pneumocytes, lipids, proteins and carbohydrates, which is produced in the lungs by type II alveolar epithelial cells.

• What type of pneumocytes? Type II pneumocyte

• What percentage of lipids? 90% lipids

• What percentage of proteins? 10% proteins (SP-A, B, C, D)

The mixture is surface active and acts to decrease surface tension at the air–liquid interface of the alveoli.

The surfactant mixture is an essential group of molecules to support air breathing. Thus, preterm infants, who are born with immature lungs and are surfactant deficient, develop respiratory distress syndrome after being born.

What is one of the most significant advances in neonatology that improved the viability of pre-term infants? Replacement of natural surfactant therapy with purified surfactant from lungs of non-human species is one of the most significant advances in neonatology and has resulted in improved limits of viability of preterm infants

Hormonal Effect

What does the term antenatal mean?

• Antenatal steroids have been shown to decrease respiratory distress syndrome (RDS) in fetuses between 24________ and ____34______ weeks gestation.

• A single course of corticosteroids is the recommended course of treatment for woman who are at risk of preterm labor within 7__________days. 

• The course may consist of betamethasone______________ (2 doses  ____24__________ hours apart or ___Dexamethasone________________________ (4 doses 12___________ hours apart).

• The full effect on surfactant production is present by 48_____ hours after the first dose of the corticosteroid.

Respiratory Distress

• RDS affects infants born before what gestational age? Infants born before 35 weeks gestation

• Weight of the pre-term infant is a predictor of RDS.

- Infants weighing 501 to 750_______ grams have a 86________ % chance of developing RDS.

- Infants weighing 751-1000 grams__________ have a 79 % chance of developing RDS.

- Infants weighing 1001_- 1250 grams have a 48________ % chance of developing RDS.

- Infants weighing 1251-1500 grams________ have a 27 % chance of developing RDS.

• Sustained improvement in oxygenation could only be demonstrated with natural surfactant complexes.

• Prophylactic surfactant is administered within the first 15________________ minutes after birth.

• Rescue surfactant is considered when administering between 1.5 to 7.4__________ hours after birth.

Clinical Presentation of Surfactant Deficiency RDS (refer to Table 12.3 on pg 195 in textbook)

Pathophysiology

1. atelectasis

2. decreased FRC

3. ventilation perfusion mismatch

Laboratory Changes

1. decreased PO2

2. increased PCO2

3. metabolic acidosis

Physical Examination

1. Abnormal lung exam

2. apnea

3. increased WOB (nasal flaring, retractions, grunting)

4. decreased breath sounds

5. poor air entry

6. tachypnea

7. Abnormal cardiovascular exam

8. cyanosis

9. pallor

10. poor perfusion

Radiographic Changes

1.diffuse reticular granular pattern

2.air bronchograms

3.atelectasis

Pulmonary Hemorrhage

• Blood is a strong inactivator of surfactant.  Several of the components of the blood contribute to the inactivation.  These components are: hemoglobin____________, ____fibrin____________, fivrinogen_______________, RBC_______________, ___lipids_________________,  immunoglobulins and _____plasma proteins______________.

• Occurs in 3___ to 5%__ of infants with RDS.

• The presence of a PDA_____ is a risk_________ factor.

Meconium Aspiration Syndrome (MAS)

• MAS affects 5 – 10 percent of all infants born through meconium-stained fluid.

• Surfactant inactivation can result in MAS due to:

  • Mechanical airway obstruction

  • Chemical pneumonitis

  • Secondary infection

  • Disruption of the surfactant monolayer by fatty acids present in the meconium

  • Production of holes in Type II cells by phospholipids, causing asymmetry of the surfactant monolayer

  • Bile acid-induced calcium influx in type II cells

  • Influx of neutrophisl producing proteases that degrade surfactant 1 to 2 hours after aspiration

  • Decreased levels SP-A and SP-B

Surfactant is given early_____ in patients with MAS

Pneumonia and Sepsis

• Infection and inflammation are associated with inflammatory mediators that lead to ____surfactant ______________ alteration_________.  

• This will also lead to capillary leak__________________ and pulmonary edema.

• The combination of edema________ and leak______ of plasma proteins into the alveoli will lead to surfactant dysfunction.

Congenital Diaphragmatic Hernia

• The lungs are immature which is equal to a deficiency in surfactant. 

Extracorporeal Membrane Oxygenation

• ECMO is associated with capillary leak syndrome, which as stated above “blood is a strong surfactant inactivator”.

ARDS

• Surfactant alterations in ARDS include decreased production_________, ____increased degradation__________, and decreased levels of surfactant associated ___proteins_________.

• These changes result in a decreased surface tension_________, resulting in atelectasis and a decreased_________ lung compliance.

Viral Bronchiolitis, Asthma, & Cystic Fibrosis

• Impairment in the surfactant function in all three of the pathologies is due to reduced levels of surfactant proteins and phospholipids. 

With all of the causes of inactivation, the addition of surfactant replacement therapy has proven to be beneficial.

ADMINISTRATION OF GAS MIXTURES GUIDED NOTES

• Nitric Oxide

  • NO is composed of ___nitrogen___________________ and oxygen___________________

  • NO is odorless____________________, colorless, sweet tasting_______________________ and a nonflammable gas.

  • Physiologic basis of action

    • ___pulmonary smooth muscle relaxation________________________________________________________

    • ____endogenous NO production decreases with iNO__________________________________________________________

    • iNO will ___relax___________ only pulmonary smooth muscle________________ ______________ adjacent to _____functioning__________________ _____alveoli____________.

• Newborn Hypoxic Respiratory Failure

  • Is used to treat persistent ___pulmonary hypertension of term________________ and  ____near term________________ neonates with hypoxic respiratory failure associated pulmonary hypertension_______________________.

  • Dosing strategies

    • Starting does is 20 ppm (parts per million)

    • Often reduced to 5 ppm at the end of 4 hours of initial treatment

    • Increasing PEEP (FRC) has shown to improve response

NO can be used with other forms of therapy including high frequency oscillatory ventilation and surfactant therapy

• Pediatric Acute Respiratory Distress Syndrome (PARDS)

  • Current therapy is _primarily supportive 

  • Suggested to improve oxygenation and ventilation perfusion (V/Q) mismatch_______

  • However, studies have failed_________ to demonstrate improved outcomes

• Toxicity & adverse effects

NO Quickly reacts to O₂ and forms poisonous ___nitrogen dioxide_________________________ _and ___the patient and health care providers can be adversely affected________________________________

Rebound ___hypoxemia______________________-stopping NO abruptly

• Methemoglobinemia

Decreased ability of hemoglobin to carry/deliver oxygen.  Nitroprusside, Nitroglycerin may exacerbate

Withdrawing and warnings

• Discontinuing iNO abruptly may result in a decrease_____________________ of the PaO2

  • Treatment should continue until oxygen desaturation problem has been resolved

• Reduce inhaled nitric oxide dose by 50% (20, 10, 5, 1 ppm). Wean off patient slowly.  

• Direct pulmonary injury

  • Excessive levels of NO2 caused by free-radical NO binding to oxygen

• Ambient air contamination

Indications for Heliox

• Helium is a ____low-density gas_______________________-useful in overcoming ____airway resistance____________________ and ____obstruction___________________

• Used in patients with increased airway resistance, edema_____________, ___asthma_______________, ____upper airway obstruction__________________________, or partial vocal cord paralysis

• Helium will ___not___________________ sustain LIFE

• Must be combined with Oxygen

  • ___80% He/20% ___________________ O2 is most common 

     ___70% He/30i%___________________ O2 mixture

• This mixture oxygenates about the same as room air but with dramatically lower _______WOB________________

• May distort voice!

Heliox

• Delivery 

  • Tight fitting NRB, ET tube or trach

    • Flows set to meet or exceed patient demand

    • Ventilator requirement: must be approved for He delivery_________________________________________

      • Can lead to volume induced lung injury

      • Conversion factors may be needed to adjust settings

      • Close patient monitoring

Heliox

• Hazards & Troubleshooting

  • Poor particle transport

    • Especially for large heavy bland aerosol

  • Cough ____impairment___________________________- due to low density

  • Voice _____distortion_________________________-due to low density

  • Cylinder ____layering_________________________-due to low density

    • Can result in 100% delivery of Helium

      • ___must analyze oxygen____________________________________

Who Could Benefit from Heliox?

• COPD

• Asthma

• Upper airway obstruction

• Post op stridor

• Croup

• SHORT TERM TREATMENT ONLY

• Carbon Dioxide (CO2)

• Delivery in specific scenarios

• Must be controlled delivery

  • To control ventilation and ABGs

  • Measured with end tidal monitoring

• Alveolar reperfusion injury

  • ECMO

• Sub-atmospheric delivery of oxygen to avoid over-circulation

  • Right to Left cardiac lesions

REVIEW QUESTION

A MD orders He/O2 therapy for a toddler with a tracheal obstruction.  The treatment will be administered with a non-rebreathing mask 80:20 heliox mixture. An O2 calibrated flowmeter is used to control gas flow and is set at 10L/min. What is the actual gas flow to the NRB?

A-5.6L

B-10L

C-16L

D-18L

HOMEWORK:

All questions must be answered in complete sentences:

1. Recreate in your own handwriting the table 12-4 on page 198 in the textbook Surfactant Delivery (no photos of the page!!)

1. What is the most common route of delivery? Surfactant is most commonly administered in 2 aliquots via bolus dosing using an ETT

2. Define the term pneumothoraces? the occurrence of more than one pneumothorax

3. Describe Flolan, Epoprostenol and Veletri. Other medications that treat pulmonary hypertension. Can be given IV or aerosolized. Very short half life so either route must be given continuously

4. How are these medicines related to NO? they treat pulmonary hypertension