Cyanotic Congenital Heart Defects Study Guide | Lecture 7 | Quizlet

Right-to-left

EXCEPT for Hypoplastic left heart, all other cyanotic heart defects are assoc with what kind of shunt

• Tetralogy of Fallot

• Transposition

• Tricuspid Atresia

• Truncus Arteriosus

• Total Anomalous Pulmonary Venous Return

what are the 5 T's of cyanotic heart defects (most common)?

Persistent Pulmonary Hypertension (PPH)

• Failed transition from fetal state of undilated, high resistance in the pulmonary blood vessels

• Due to high pulmonary pressure most blood goes from the pulmonary artery through the ductus (or PFO) to the Aorta

• A right→left shunt

Persistent Pulmonary Hypertension (PPH)

• There are no cardiac defects, but abnormal smooth muscle development and hypertrophy is seen in the pulmonary blood vessels

• More common in term infants with a stressor

Persistent Pulmonary Hypertension (PPH)

-Respiratory distress, Tachypnea,

Tachycardia, Hypotension, Pallor, Cyanosis

unresponsive to O2, Right sided heart dilitation

and subsequent heart failure, Shock

-symptoms develop immediately after birth

Persistent Pulmonary Hypertension (PPH)

Physical Exam:

• Preductal O2 saturation typically higher than postductal

• Single loud S2

• Harsh Systolic Murmur (If tricuspid Regurgitation is present)

Persistent Pulmonary Hypertension (PPH)

CXR

• Often normal

• May have decreased lung markings (pneumonia or changes consistent with meconium aspiration syndrome)

ECHO

• Elevated pressure in the pulmonary artery

• Blood flow across the PDA (or occasionally PFO)

• Electrolyte and nutrition support

• Mechanical ventilation support– conventional and high frequency

• Circulatory support (Ionotropes and Pressors)

• (If not responding) Inhaled nitric oxide and ECMO

treatment for Persistent Pulmonary Hypertension (PPH)

Truncus Arteriosus

• The truncus never completely divides into the aorta and pulmonary arteries = one great vessel with a single valve

• Large VSD

• Pulmonary Hypertension

Truncus Arteriosus

• Cyanosis is minimal

• A continuous systolic ejection murmur that radiates to the back with a single loud S2.

-Sometimes an early diastolic murmur.

• Bounding pulses

• In the first couple weeks these infants develop CHF, tachypnea and cough

Truncus Arteriosus

EKG

•May show bilateral ventricular hypertrophy and cardiomegaly

CXR

•Large heart with increased pulmonary vascular markings

ECHO

•Single overriding great vessel arising from the heart

•Abnormal Valves

•VSD

-Diuretics

-Surgical treatment (in first few weeks of life)

-Conduit revisions

Truncus Arteriosus treatment

one year

Truncas arteriosus is fatal by _______________ if left untreated

Transposition of the Great Arteries (TGA)

• Aorta connects to the RV

• Pulmonary artery to the LV

• Survival depends on having a mixing lesion (PFO, PDA, ASD, VSD)

• Ductal dependent for both pulmonic and systemic circulation

Transposition of the Great Arteries (TGA)

• Infant is cyanotic from birth (as soon as the ductus starts to close)

• Unresponsive to O2

• Typically, no murmur

• Single S2

• Tachypnea, clubbing

Transposition of the Great Arteries (TGA)

EKG

• RVH and R Axis deviation

CXR

• Heart with an “egg on a string” appearance and increased pulmonary markings

ECHO

• Aorta connects to the RV

• Pulmonary artery to the LV

-PgE1

-Surgery (within first week)

TGA treatment

Arterial Switch

what is the surgery of choice for TGA?

Alprostadil

-a derivative of an endogenous compound that keeps the ductus arteriosus open in utero

Prostaglandin E1 (PGE1)

-administration prevents ductus closure

-side effects: Apnea, hypotension, fever, flushing, seizure

Intubate

if you give your pt PgE1, you must be prepared to ________________________

Tetralogy of Fallot (TOF)

-most common cyanotic CHD

• Pulmonic Stenosis

• RV Hypertrophy

• Large VSD

• Overriding Aorta

Tetralogy of Fallot (TOF)

• High pitched systolic ejection murmur

• Cyanosis within the 1st year of life and hypoxic "Tet" spells (5mo)

Tet Spells

• Decreased systemic vascular resistance or increased right ventricular outflow tract obstruction increase right to left shunting

• Resulting in agitation, sudden increased cyanosis and subsequent syncope

• Severe spell may lead to prolonged unconsciousness, seizure or death

put in knee to chest position or older children will squat

how do you get someone out of a tet spell

Tetralogy of Fallot (TOF)

EKG

• RVH

CXR

• Upturned Cardiac Apex (Boot Shaped Heart) and decreased pulmonary vasculature

ECHO

• 4 defects

-PgE1

-beta blockers (while waiting for surgery)

-surgery

-artificial pulmonic valve

TOF treatment

dilated cardiomyopathy and right heart failure

what can happen if TOF goes untreated?

Tricuspid Atresia

• Endocardial cushions fail to form valve

• Complete absence of the tricuspid valve

• Hypoplastic R Ventricle

• An ASD or PFO and VSD or PDA are required for mixing and survival

Tricuspid Atresia

• Cyanosis unresponsive to O2

• Single S2 with a Holosystolic murmur

• Sometimes No murmur

• Tachypnea

Tricuspid Atresia

EKG

• LVH, RAH and Left axis deviation

CXR

• Variable heart size with decreased pulmonary vascular markings

ECHO

• Complete absence of the tricuspid valve

• Hypoplastic R Ventricle

• Mixing lesion

Surgery (pallative, not curative)

Tricuspid Atresia treatment

Total Anomalous Pulmonary Venous Return (TAPVR)

• The pulmonary veins are malpositioned

• They return blood to the right sided circulation not left

• A PFO or ASD must be present for survival

Total Anomalous Pulmonary Venous Return (TAPVR)

• Cyanosis severity is dependent on amount of obstruction of drainage from the pulmonary veins

• Poor growth, tachypnea and dyspnea

• Fixed split S2, systolic ejection murmur and sometimes a mid diastolic murmur

Total Anomalous Pulmonary Venous Return (TAPVR)

EKG

• Right axis deviation and right ventricular

hypertrophy

X-Ray

• Cardiomegaly

• Snowman’s sign

Echo shows defect

-PgE1

-Surgical Correction (first month of life)

TAPVR correction

Double Outlet Right Ventricle

• Both great arteries connect to the right ventricle

• A VSD is always present

Double Outlet Right Ventricle

• Cyanosis can present early (severe disease) or late

• Patients may have hyper cyanotic spells like "tet spells"

• Holosystolic murmur and a diastolic rumble

• Loud pulmonic component of S2

Double Outlet Right Ventricle

EKG

• Left axis deviation

• RVH

• Atrial enlargement

• Sometimes LVH

CXR

• May have cardiomegaly. Nonspecific.

ECHO

• Both great vessels arising from RV

• VSD

-Diuretics

-Ace Inhibitors

-Surgical Correction

Double Outlet Right Ventricle Treatment

Hypoplastic Left Heart (HLHS)

• Most common cardiac defect to cause death in the 1st month of life

• Hypoplastic left ventricle

• Stenotic or no mitral valve

• Small aorta and small or stenotic aortic valve

• ASD

Hypoplastic Left Heart (HLHS)

• Cyanosis develops as the ductus closes

• 1/3 of infants present in shock

• Soft Systolic ejection murmur

• Single Loud S2

Hypoplastic Left Heart (HLHS)

EKG

• Sinus Tachycardia

• RA enlargement

• RVH

• Right axis deviation

CXR

• Cardiomegaly and increased pulmonary vascular markings

Echo for diagnosis

-PgE1

-Palliative Surgical Treatment

treatment for HLHS

1-2 weeks

if HLHS goes untreated, death will occur within ________________ of life

Ebstein's Anomaly

• Maternal Lithium use

• Presents at any age

• Opening of the tricuspid valve is displaced into the RV and the RA dilates

• Often comes with a PFO or ASD or PS

Ebstein's Anomaly

• Gallop, Split S2, Pansystolic murmur of tricuspid insufficiency

• CXR- Very, very large heart and decreased pulmonary vascular markings

• ECHO is diagnostic

Tetralogy of Fallot (TOF)

"boot-shaped"

Transposition of the Great Arteries (TGA)

"Egg on a string" appearance

Transposition of the Great Arteries (TGA)

small heart with increased pulmonary vascular markings

Tricupsid Atresia

small heart with decreased pulmonary vascular markings

Patent Ductus Arteriosus (PDA)

normal to large heart with increased pulmonary vascular markings

Large VSD

large heart with increased pulmonary vascular markings

Truncas Arteriosus

enlarged heart with increaed pulmonary vascular markings and right aortic arch