Cardiac - Outline

🩺 Fetal Circulation (In Utero)

Key Concept: Blood bypasses the lungs because gas exchange occurs in the placenta.

• Oxygen source: Mom’s oxygen → via placenta.

• Umbilical vein: Brings oxygenated blood from placenta to fetus.

• Umbilical arteries: Return deoxygenated blood from fetus to placenta.

• Highest oxygen concentration blood goes to heart and brain.

🔄 Fetal Shunts (Bypass Mechanisms)

1. Foramen Ovale

   • One-way opening from right atrium → left atrium.

   • Diverts blood away from lungs.

2. Ductus Arteriosus

   • Connects pulmonary artery → aorta.

   • Diverts blood away from the lungs directly to systemic circulation.

3. Ductus Venosus

   • Connects umbilical vein → inferior vena cava.

   • Bypasses the liver and portal system.

   • Closes after birth; becomes ligament by 2 months.

🫁 Fetal Lungs

• Collapsed in utero.

• Minimal blood flow.

• Mom provides oxygen via placenta.

🔄 Circulatory Changes After Birth

🫁 Transition From Fetal to Pulmonary Circulation

• Starts: At birth, when the placenta is removed.

• Ends: Around days 10–21, with permanent closure of ductus arteriosus.

• Triggered by: First breath → lungs expand → increased oxygen.

💨 Hemodynamic Shifts

• Pulmonary vascular resistance drops.

• Systemic vascular resistance increases.

• Blood now flows into lungs for oxygen exchange.

⬆ Pressure Shift

• Left atrium pressure ↑ from increased pulmonary return.

• Right atrium pressure ↓ due to decreased pulmonary resistance.

• Result:

  • Foramen ovale closes (pressure on left > right).

  • Ductus arteriosus closes (oxygen triggers vasoconstriction).

👶 Neonatal Circulation

⚠ Risks

• Immature myocardium = high risk for heart failure.

• Sensitive to volume/pressure overload.

• Lower LV strength → low systolic BP.

🧠 Key Facts

• Heart is size of baby’s fist.

• Positioned centrally, slightly to the left.

• Neonates rely on heart rate to increase cardiac output.

🩺 Clinical Assessment (No Monitors Needed)

• Cardiac Output Check:

  • Pulse strength

  • Heart rate (HR)

  • Capillary refill

  • Skin color

• Kidney perfusion:

  • Urine output

• Neurologic perfusion:

  • Alertness

  • Orientation

💡 Normal Chamber Oxygen Saturation

• Right Atrium (RA): ~72–80%

• Left Atrium (LA): ~95%

❤Pediatric Cardiac Dysfunction: Assessment

🧸Physical Indicators

These are often the earliest clues in infants and young children.

• Poor Feeding

  • Classic sign in infants

  • Infants struggle with suck-swallow-breathe coordination

  • Feeding exhausts them

  • Results in reduced intake and weight gain

• Tachypnea (Rapid breathing)

  • Baby is working hard to breathe

  • Especially noticeable during/after feeding or crying

• Tachycardia (Rapid heart rate)

  • Heart is working overtime to compensate for poor perfusion

• Failure to Thrive (FTT)

  • Due to poor feeding and high metabolic demand

  • In infants: inadequate weight gain

  • In older children: food intolerance, can’t keep up nutritionally

🧠Developmental Indicators

• Developmental Delays

  • Delayed milestones (rolling, crawling, walking, speech, etc.)

  • Often due to poor oxygenation or energy exhaustion

• Important History Questions:

  • Any delays in gross/fine motor, language, or cognitive development?

  • Are activity levels appropriate for age?

📝Prenatal History (Must Ask)

Important to gather maternal and fetal history that may indicate risk.

🤰 Maternal Risk Factors

• Rubella infection during pregnancy

• Alcohol use (Fetal Alcohol Syndrome)

• Dilantin (antiepileptic drug)

• Diabetes (especially poorly controlled)

• Lupus

• SIDS in siblings

• Frequent fetal losses/stillbirths

• Family history of congenital heart disease

👶 Fetal/Neonatal Indicators

• Congenital anomalies (especially multiple system defects)

• High birth weight is associated with increased cardiac disease risk

🫀Assessment: Heart Murmurs in Children

📌Definition

• A murmur is an extra heart sound caused by the turbulent flow of blood within the heart or great vessels.

• Can result from:

  • High blood flow through normal or abnormal valves

  • Structural abnormalities in heart or vessels

🩺 Assessment of Murmurs

🔊Auscultation (Listening with Stethoscope)

• Listen for:
  

  • High-frequency sounds – sharper, more intense

  • Low-frequency sounds – softer, rumbling

  • Loud and distinct – easily heard over normal heart sounds

✋Palpation (Feeling the Chest)

• Thrill: A vibration felt on the chest wall, suggests a loud or pathological murmur

❗Causes of Murmurs

• Forward blood flow through a narrowed or irregular valve

• Valvular stenosis or abnormal shape

• Dilated chamber or vessel (increased turbulence)

• Regurgitant flow – blood leaks backward (incompetent valve)

• Septal defects or structural heart defects

✅Innocent (Functional) Murmurs

Not caused by heart disease — common in healthy children

Characteristics:

• Grade 1 to 3 (on a 1–6 scale)

• Low-pitched

• Systolic in timing (occurs between S1 and S2)

• Normal S1 and S2 heart sounds

• Heard best in supine position, disappears when sitting or standing

• Occurs in up to 50% of all children at some point

🧠 “Innocent until proven pathological!”

🧪Tests to Evaluate Cardiac Function

Used when murmur is abnormal, loud, or persistent:

• ECG (Electrocardiogram)

  • Evaluates electrical activity and rhythm

• Chest X-ray

  • Checks heart size and pulmonary circulation

• Echocardiogram (Echo)

  • Ultrasound of the heart – views structures and flow

• Cardiac Catheterization

  • Invasive test; provides precise structural and pressure data

🫀Cardiovascular Dysfunction / Disorders in Children

❤Congenital Heart Disease (CHD)

• Definition: A structural defect of the heart present at birth.

• CHD is a major cause of death in the first year of life after prematurity.

• The most common type: Ventricular Septal Defect (VSD)

📊Incidence

• Occurs in 5–8 per 1,000 live births

• 2–3 per 1,000 are symptomatic within the first year of life

• 28% of CHD cases are associated with other anomalies (e.g., Trisomy 21, 13, 18)

⏳Fetal Heart Development

• Heart develops between 4th–7th week of gestation

• Most critical time; fetus is highly vulnerable to teratogens

• Many mothers don’t yet know they’re pregnant at this stage

🧬Causes of CHD

🗂Classification of CHD

🧵Older System

• Acyanotic → May develop cyanosis later

• Cyanotic → May initially appear pink

• CHF may develop in both types

🔄Newer (Based on Hemodynamics)

⚠Consequences of CHD

1.Congestive Heart Failure (CHF)

• Common in left → right shunt defects (↑ pulmonary blood flow)

• Heart can’t pump effectively; lungs become congested

2.Hypoxemia

• Occurs in right → left shunt defects (↓ pulmonary blood flow)

• Leads to cyanosis, clubbing, polycythemia

💡Remember:

The type and severity of the defect + hemodynamic pattern determines the clinical signs (e.g., CHF, cyanosis, murmur).

🫀Congestive Heart Failure (CHF) – Pediatrics

🧾Definition

• CHF = The heart is unable to pump enough blood to meet the body’s metabolic needs.

• In children, CHF is usually secondary to:

  • Structural heart defects (e.g., CHD)

  • Increased volume/pressure in the heart

  • Conditions increasing metabolic demand (sepsis, anemia, hypothyroidism)

🧩Types of Heart Failure

• Right-Sided Failure
  → Right ventricle cannot pump blood effectively into the pulmonary artery

• Left-Sided Failure
  → Left ventricle cannot pump blood into systemic circulation

• High Output Failure
  → Heart structure is normal, but demand is high (e.g., sepsis, anemia, hypothyroidism)

💡 In children, it’s hard to separate R vs. L failure—signs often overlap.

⚠Causes of CHF in Children

• Volume overload → e.g., Left → Right shunt

• Pressure overload → Obstructions (e.g., Coarctation of the Aorta)

• Decreased contractility → Ischemia, electrolyte imbalance

• High metabolic demand → Sepsis, fever, anemia

• Cor Pulmonale → Heart failure caused by lung disease

👶Clinical Presentation of CHF in Children

1. 🫀Impaired Myocardial Function

• Tachycardia

• Fatigue, weakness

• Pallor, cool extremities

• Decreased BP and urine output

• Restlessness, diminished contractility

2. 🫁Pulmonary Congestion (Mostly LHF)

• Tachypnea, dyspnea

• Respiratory distress

• Cyanosis

• Exercise intolerance

• Cough, crackles

3. 💦Systemic Venous Congestion (Mostly RHF)

• Generalized edema

• Periorbital swelling

• Weight gain

• Ascites

• Hepatomegaly

• Jugular venous distention (JVD)

🔍CHF Assessment Tips

• Caregiver input is critical – they know the child best.

• Early sign: Respiratory changes

  • Breath sounds, congestion, coughing

• Check: Pulse strength, feeding tolerance, tachycardia, tachypnea

🎯Therapeutic Management Goals

Stabilize medically before surgical repair

• ✅ Improve Cardiac Function

  • ↑ Cardiac output

  • ↑ Myocardial contractility

  • ↓ HR

• 💨 Improve Oxygenation

  • Decrease oxygen demand

• 💧 Remove Excess Fluid & Sodium

  • Diuretics

• ⬇ Reduce Cardiac Workload

  • Limit activity

  • Provide rest

💊Medications & Nursing Considerations

1.Cardiac Glycosides: Digoxin

• Improves contractility

• Very narrow therapeutic range!

  • Apical pulse x 1 full minute

    • Hold if HR < 90–110 bpm

  • Usual range: 0.8–2.0 mcg/L

• Toxicity Signs:

  • Vomiting (early)

  • Bradycardia

  • DO NOT REGIVE → call provider

✅ Always double-checked by 2 RNs

💚 Green liquid – usually <1 mL, use 1 mL syringe

2.ACE Inhibitors (e.g., Captopril, Enalapril)

• Causes vasodilation → ↓ pulmonary and systemic vascular resistance

• Monitor BP before giving

• Used to decrease afterload

3.Diuretics

• Teach parents potassium-rich foods:

  • Bananas, oranges, leafy greens

🍽Nutritional Support

• High metabolic demand = high caloric needs

• Small, frequent feeds to avoid fatigue

• Orogastric feeding preferred (nasal breathers)

• High-calorie formulas (more kcal/oz)

🛏Additional Nursing Interventions

• Elevate HOB → reduce respiratory distress

• Treat fever/infection promptly

• Bundle care to allow for rest

• Emotional support to child and family

🩸Hypoxemia vs. Hypoxia

Hypoxemia can lead to hypoxia if not corrected.

🫁Hypoxemia in Children

🧾Definition

• Decreased oxygen saturation (SpO₂) in the circulating blood

• Common in congenital heart defects with decreased pulmonary blood flow

🧠Clinical Presentation of Hypoxemia

• Cyanosis

  • Bluish discoloration (especially lips, fingertips)

  • Chronic hypoxemia seen when SpO₂ = 80–85%

• Polycythemia

  • Body compensates by producing more RBCs to carry oxygen

  • Results in:

    • ↑ Hematocrit (normal: ~33–40%)

    • >50% = concern; >55–60% = dangerous

    • Thick, viscous blood → ↑ risk for clots and stroke

• Clubbing

  • Rounding/enlargement of fingers or toes

  • Sign of long-term hypoxemia

• TET Spells (Hypercyanotic Spells)

  • Sudden, severe drop in oxygen

  • Seen in children with Tetralogy of Fallot

🩺Nursing Considerations for Hypoxemia

🔴Managing TET Spells

Classic emergency management for hypercyanotic episodes

• STOP activity/stress

• SQUAT (older children) or Knee-to-Chest (infants)

  • Increases systemic vascular resistance

  • Redirects blood to lungs and vital organs

  • Improves oxygenation

• Calm the child – stress worsens the episode

💧Managing Polycythemia

• Ensure adequate hydration

  • Prevents blood from becoming too thick

  • Reduces risk of thromboembolism (stroke)

👨‍👩‍👧Support for Child and Family

• Bundle care to minimize energy use

• Educate family on recognizing signs of:

  • TET spells

  • Cyanosis

  • Signs of dehydration or stroke

❤Congenital Heart Disease (CHD)

Structural defects in the heart present at birth that lead to altered blood flow.

🔄Newer Classification of CHD – Based on Hemodynamics

🔼Increased Pulmonary Blood Flow Defects

🧠Pathophysiology

• Abnormal connection allows oxygenated blood from the left side of the heart to flow back into the right side

  • Could be at the septum (ASD/VSD) or great vessels (PDA)

• Results in:

  • Increased blood volume on the right side

  • Increased pulmonary blood flow

  • Decreased systemic blood flow

• Left-to-right shunting

🧠 Over time, the pulmonary system becomes overwhelmed, leading to:

• Increased pulmonary vascular resistance (PVR)

• Pulmonary hypertension

• If untreated: RV hypertrophy and CHF

📋Clinical Consequences

• High Basal Metabolic Rate (BMR)

  • Infants burn calories quickly → poor weight gain

• Congestive Heart Failure

  • Tachycardia

  • Tachypnea

  • Hypotension

  • Edema

  • Delayed cap refill

• Increased susceptibility to respiratory infections

  • Lungs overloaded with fluid → infection risk ↑

🧾Common Defects with Increased Pulmonary Blood Flow

1.Atrial Septal Defect (ASD)

• Hole between right and left atria

• Blood flows left → right due to higher LA pressure

• Usually asymptomatic in infancy

• S&S (if moderate to large):

  • Murmur

  • Mild CHF

  • Risk for atrial arrhythmias in adolescence/adulthood

2.Ventricular Septal Defect (VSD)

• Hole between right and left ventricles

• Most common CHD

• Blood flows left → right

• S&S:

  • Loud, harsh murmur at left sternal border

  • CHF (moderate to large)

  • Poor feeding, fatigue

  • Frequent respiratory infections

3.Patent Ductus Arteriosus (PDA)

• Failure of fetal ductus arteriosus (between aorta & pulmonary artery) to close

• Aortic blood flows into pulmonary artery

• S&S:

  • Continuous “machine-like” murmur

  • Bounding pulses

  • Widened pulse pressure

  • Tachypnea

  • Frequent URIs

⚠Complications if Left Untreated

• Pulmonary hypertension

• Right ventricular hypertrophy

• Irreversible vascular changes

• Heart failure

• Risk for Eisenmenger syndrome (shunt reversal → cyanosis)

🫀 Increased Pulmonary Blood Flow Defects

Left-to-right shunting defects that send extra oxygenated blood to the lungs, overwhelming the pulmonary system and leading to congestion, CHF, and respiratory infections.

❤ Atrial Septal Defect (ASD)

🧠 Definition:

• A hole in the septum that separates the left and right atria.

• Causes left-to-right shunting → oxygen-rich blood from LA flows into RA.

• This results in increased blood flow to the lungs.

🔍 Pathophysiology:

• Left atrium has higher pressure than the right → blood moves left → right.

• Right atrium becomes enlarged from volume overload.

• Over time, this leads to stretching of conduction fibers and pulmonary changes.

🧬 Causes/Risk Factors:

• Often congenital and may be asymptomatic at birth.

• Sometimes associated with genetic syndromes (e.g., Down syndrome).

⚠ Complications (if untreated):

• Atrial arrhythmias (irregular heart rhythms) – from stretching of the atrium

• Pulmonary hypertension – due to constant high blood flow to lungs

• Emboli or stroke risk – from stagnant blood flow in a stretched right atrium

🩺 Clinical Signs & Symptoms:

• Often asymptomatic in infants

• If large:

  • Fatigue

  • Recurrent respiratory infections

  • Mild CHF symptoms (tachypnea, poor feeding, delayed growth)

  • Soft systolic murmur

💊 Treatment:

💓 Ventricular Septal Defect (VSD)

🧠 Definition:

• A hole in the septum between the left and right ventricles.

• Causes left-to-right shunting of blood → more blood flows to lungs.

🔍 Pathophysiology:

• Left ventricle has higher pressure → blood moves to right ventricle.

• Right ventricle & lungs receive more volume = pulmonary congestion.

• Over time: Right ventricular hypertrophy, increased pulmonary vascular resistance.

🧬 Causes/Risk Factors:

• Most common congenital heart defect

• Frequently seen with genetic syndromes, especially Down syndrome

• Can be isolated or part of complex CHD

⚠ Complications (if untreated):

• Pulmonary hypertension

• CHF – from overload on the right heart and lungs

• Delayed growth, poor weight gain

• Eisenmenger syndrome (reversal of shunt to R→L → cyanosis)

🩺 Clinical Signs & Symptoms:

• Loud, harsh holosystolic murmur

• Tachypnea, tachycardia

• Poor feeding, fatigue

• Diaphoresis with feeding

• Frequent respiratory infections

• Signs of CHF (hepatomegaly, edema, crackles)

💊 Treatment:

🌬 Patent Ductus Arteriosus (PDA)

🧠 Definition:

• Ductus arteriosus is a normal fetal connection between the aorta and pulmonary artery.

• Normally closes after birth due to increased oxygen and drop in prostaglandins.

• PDA = fails to close, causing left-to-right shunting.

🔍 Pathophysiology:

• Oxygenated blood from aorta flows back into pulmonary artery.

• This increases blood flow to lungs → pulmonary overload.

🧬 Causes/Risk Factors:

• Most common in preterm infants

• Failure to close may be linked to:

  • Prematurity

  • Hypoxia

  • Genetic disorders

  • Other heart defects

⚠ Complications (if untreated):

• CHF

• Pulmonary hypertension

• Frequent respiratory infections

• Long-term lung damage and RV strain

🩺 Clinical Signs & Symptoms:

• “Machine-like” continuous murmur

• Bounding pulses

• Widened pulse pressure

• Tachypnea

• Crackles in lungs

• Difficulty feeding, poor weight gain

• CHF signs if large

💊 Treatments

• Medication:

  • Indomethacin or Ibuprofen (↓ prostaglandin levels to close PDA)

• Interventional:

  • Cardiac catheterization with clip or coil

  • Surgery if meds/cath fail |

🔁 Summary Table – Key Comparisons

🚫 Obstructive Defects (CHD – Hemodynamic Category)

🧠 Definition:

• These defects involve narrowing (stenosis) of a blood vessel or valve.

• Blood trying to exit the heart meets resistance, leading to:

  • Increased pressure in the ventricle

  • Decreased cardiac output

  • Potential for heart failure or cyanosis, depending on side affected

🔄 General Effects:

🩺 1. Coarctation of the Aorta (CoA)

💡 What is it?

• Localized narrowing of the aorta, usually near the ductus arteriosus.

• Blood flow is obstructed after it leaves the left ventricle.

🧠 Pathophysiology:

• Causes increased pressure above the narrowing (head/upper extremities)

• Causes decreased pressure below the narrowing (lower extremities)

• Leads to left-sided CHF from backflow pressure into lungs

🧬 Clinical Presentation:

• Increased BP and bounding pulses in upper extremities

• Weak/absent femoral pulses & low BP in lower extremities

• Signs of CHF in infants:

  • Tachypnea, poor feeding, irritability, sweating with feeds

• Older children may have:

  • Headaches, leg cramps, cool lower limbs

💊 Treatment:

• NOTE: CoA is outside of the heart, so open-heart surgery is NOT needed

🫀 2. Aortic Stenosis (AS)

💡 What is it?

• Narrowing of the aortic valve → obstruction to blood flow from left ventricle to aorta

• Increases workload of left ventricle

🧠 Pathophysiology:

• ↑ Resistance to ejection of blood from LV

• Leads to LV hypertrophy, ↓ cardiac output

• Can reduce coronary perfusion, ↑ risk of MI

🧬 Clinical Presentation:

• Weak pulses

• Hypotension

• Poor feeding

• Tachycardia

• Signs of CHF |
  | Older Children |

• Chest pain

• Dizziness

• Exercise intolerance

• Murmur over aortic area |

💊 Treatment Options:

🌬 3. Pulmonic Stenosis (PS)

💡 What is it?

• Narrowing at the pulmonary valve or entrance to pulmonary artery

• Affects the right side of the heart

🧠 Pathophysiology:

• Resistance to blood leaving RV → RV hypertrophy

• ↑ Right atrial pressure → may reopen the foramen ovale

• Right-to-left shunting can occur → cyanosis

Severe form: Pulmonary atresia (valve is completely fused → no blood flow to lungs!)

🧬 Clinical Presentation:

• May hear a loud systolic ejection murmur at upper left sternal border

💊 Treatment:

• Brock procedure: surgical valvotomy

• Valve replacement if severely malformed |

✅ Quick Comparison Table:

🔽Decreased Pulmonary Blood Flow Defects

🧠 Definition:

• Blood flow to the lungs is obstructed + a defect (ASD, VSD, PDA) allows mixing between right and left sides of the heart.

• Results in less oxygenated blood reaching the lungs, and unoxygenated blood enters systemic circulation → cyanosis.

⚠ Clinical Manifestations

💔 1. Tetralogy of Fallot (TOF)

🔍 Four Defects (TETRA = 4):

1. Pulmonary Stenosis → ↓ blood flow to lungs

2. Ventricular Septal Defect (VSD) → mixing of oxygenated & deoxygenated blood

3. Overriding Aorta → aorta receives blood from both ventricles

4. Right Ventricular Hypertrophy → thickened RV muscle due to pumping against resistance

🧠Hemodynamics:

• Pressures may be equal in both ventricles due to large VSD

• Shunt direction depends on resistance:

  • ↑ Pulmonary resistance → Right-to-Left shunt (cyanosis)

  • ↑ Systemic resistance → Left-to-Right shunt

🩺 Clinical Presentation:

• Cyanosis

• Tet spells → triggered by crying, feeding, or agitation

• Lethargy, decreased activity

• Hypoxemia

• Polycythemia

• Systolic murmur

✋ Avoid crying — can trigger Tet spells

Squat or knee-to-chest position improves systemic vascular resistance → redirects blood to lungs

💊 Treatment:

• Surgical repair in first year of life:
  

  • Patch VSD

  • Relieve pulmonary stenosis

  • Reposition/repair overriding aorta

🚫 2. Tricuspid Atresia

🧠 Definition:

• Tricuspid valve is absent or didn’t form

• No connection between the right atrium and right ventricle

• Blood must bypass the right side to reach the lungs

🔁 Compensatory Pathways:

• ASD or Foramen Ovale: Allows right → left shunt → unoxygenated blood goes to left atrium and out to body

• VSD: Some blood flows to lungs for oxygenation, but often insufficient

• Often associated with pulmonary stenosis

🩺 Clinical Presentation:

• Severe cyanosis

• Dyspnea, tachypnea

• Poor feeding, poor weight gain

• Tet spells

• Polycythemia → ↑ stroke risk

• Murmurs if VSD present

💊 Treatment:

• Keep PDA open using Prostaglandin E1 (Indomethacin)

• Ensures blood can flow from aorta → pulmonary artery |
  | Palliative surgery |

• Blalock-Taussig shunt → increases pulmonary blood flow |
  | Final repair |

• Fontan Procedure:

  • Connects venous blood (SVC & IVC) directly to pulmonary arteries

  • Bypasses right heart entirely

  • Used in single ventricle defects

✅ Summary Table: Decreased Pulmonary Blood Flow Defects

🔄 Mixed Blood Flow Defects

Congenital heart defects in which oxygenated and deoxygenated blood mix, leading to variable oxygen delivery to the body.

🧠Overview & Pathophysiology

• Very complex lesions, often involving multiple structural abnormalities.

• Survival depends on blood mixing between the pulmonary and systemic circulations via:

  • ASD (Atrial Septal Defect)

  • VSD (Ventricular Septal Defect)

  • PDA (Patent Ductus Arteriosus)

• Without these communications, survival is not possible.

⚠General Clinical Manifestations

• Pulmonary congestion from left-sided overload or high pulmonary pressures

• Decreased cardiac output

• Congestive heart failure (CHF) signs: tachypnea, feeding difficulty, poor weight gain

• Volume overload

• Variable cyanosis: Depending on degree of blood mixing

• Relative systemic desaturation: Even if SpO₂ appears “normal,” systemic blood may still be poorly oxygenated

• Tachycardia, hypotension

• Low Apgar scores, poor perfusion

🫀1. Transposition of the Great Vessels (TGV)

🧬 What Is It?

• Aorta arises from right ventricle, and pulmonary artery arises from left ventricle (reversed positions).

• Two parallel circulations:

  • Unoxygenated blood cycles through body

  • Oxygenated blood cycles through lungs

• No direct connection between oxygenated and systemic flow unless defects exist

🔄 Required Compensatory Defects:

• ASD or PFO (Patent Foramen Ovale)

• VSD

• PDA

➤ Without one of these, death occurs quickly after birth

🩺 Clinical Manifestations:

• Cyanosis at birth (doesn’t improve with oxygen)

• Tachypnea, grunting, nasal flaring

• Signs of CHF if pulmonary overcirculation occurs

• Decreased systemic perfusion

• Low or absent pulses, cold extremities

• Low Apgar scores, poor responsiveness

💊 Treatment:

• Prostaglandin E1 (PGE1) → Keeps PDA open for temporary blood mixing

• Balloon Atrial Septostomy (Rashkind procedure):

  • Done via cardiac cath

  • Enlarges ASD to allow more effective mixing

• Surgical Repair:

  • Arterial Switch Operation (Jatene Procedure)

    • Done in first few days of life

    • Repositions great arteries correctly

    • Coronary arteries are also reimplanted

🎀2. Total Anomalous Pulmonary Venous Connection (TAPVC)

🧬 What Is It?

• Pulmonary veins connect abnormally to the right atrium or systemic veins (e.g., SVC, IVC) instead of left atrium.

• Oxygenated blood goes back to the right heart

• Must have an ASD/PFO to allow blood to reach the left side of the heart

➤ Known as the “Easter Basket Defect” due to the tangled appearance of pulmonary venous drainage

🧠 Types of TAPVC:

Infracardiac TAPVC is the most severe — prone to obstruction and acidosis

🩺 Clinical Manifestations:

• Cyanosis (mild to severe, depending on drainage and mixing)

• Tachypnea, dyspnea, nasal flaring

• Poor feeding

• Failure to thrive

• Signs of CHF: hepatomegaly, crackles, tachycardia

• Right ventricular hypertrophy (volume overload)

• Small left atrium

💊 Treatment:

• Emergency surgery required

• Keep PDA open with PGE1 until surgery

• Create/enlarge ASD if needed via balloon septostomy

• Surgical repair: Reconnect pulmonary veins to left atrium, ligate abnormal connections

💔3. Hypoplastic Left Heart Syndrome (HLHS)

🧬 What Is It?

• Underdevelopment of the entire left side of the heart, including:

  • Mitral valve atresia

  • Aortic valve atresia or stenosis

  • Small or absent left ventricle

  • Ascending aorta is hypoplastic (tiny)

➤ No ability to pump blood to systemic circulation

🔄 Pathophysiology:

• Blood enters left atrium, but can’t exit due to blocked/absent mitral valve

• Blood flows through ASD/PFO into right atrium → right ventricle → pulmonary artery

• Descending aorta is perfused via PDA from pulmonary artery

➤ If PDA closes, baby goes into cardiogenic shock and dies rapidly

🩺 Clinical Manifestations:

• Mild cyanosis at birth

• Rapid decompensation as PDA closes

• Severe CHF

• Cool extremities, delayed cap refill

• Absent pulses, low/absent urine output

• Metabolic acidosis, lactic acidosis

• Organ failure (brain, kidney, gut) → cardiovascular collapse

• Hypothermia, unresponsiveness

💊 Treatment:

• Keep PDA open with Prostaglandin E1

• Mechanical ventilation, inotropes |
  | Surgical (3-stage palliation) |

• Stage 1: Norwood → Replaces aorta with pulmonary artery + BT shunt

• Stage 2: Glenn → SVC connected directly to pulmonary artery (6–9 months)

• Stage 3: Fontan → IVC connected to pulmonary artery (2–4 years) |
  | Alternative | Heart transplant (rare and requires donor availability) |

✅ Summary Table – Mixed Defects Comparison

🫀 Cardiovascular Dysfunction

📍 Post-Procedural Treatment & Care for CHD

💉 Interventional Cardiac Catheterization Procedures

Minimally invasive procedures done via catheter to diagnose or treat CHDs.

🔧Types of Interventions:

🩺 Cardiac Catheterization – Nursing Care

🕑Pre-Procedural:

• Explain the procedure to child and family (age-appropriate)

• Ensure NPO status (typically 4–6 hrs before)

• Sedation prep (conscious sedation or general)

• Baseline VS and labs

• Check for allergies (e.g., iodine, shellfish)

⏳Post-Procedural:

• Cardiac & Pulse Oximetry Monitoring

• Vital Signs: q15min initially

• Neurovascular checks:

  • Check distal pulses, warmth, color, sensation

  • Compare both legs

• Dressing: Check for bleeding at insertion site (usually femoral)

• I&O Monitoring:

  • Urine output should be ≥1 mL/kg/hr

• Blood Glucose (esp. in infants)

• Flat time: Child may need to lie flat for 4–6 hrs post-cath

🛠 Surgical Interventions for CHD

🧠Purpose:

To correct or palliate structural heart defects not managed via catheterization.

Types:

• Cardiac Shunt: Temporary path to redirect blood flow

• Open-Heart Surgery: Requires cardiopulmonary bypass

• Closed-Heart Surgery: Done without bypass (e.g., CoA repair)

• Staged Procedures: Multi-step repairs (e.g., Norwood, Glenn, Fontan)

👨‍👩‍👧 Family & Child Preparation:

• Take to PICU post-op

• Honest but age-appropriate explanations

• Prepare for ECMO if needed (life support system)

• Anticipate:

  • Possible cyanosis

  • CHF symptoms

  • Need for ionotropic meds (e.g., milrinone, dopamine)

  • AIRWAY is priority

  • Monitor I&O, electrolytes, and vitals

👨‍👩‍👦 Family Support in CHD

• Help family understand and adjust to diagnosis

• Educate about condition, procedures, and medications

• Support emotional needs; refer to counseling or support groups

• Normalize life as much as possible (return to school, play, etc.)

🏥 Postoperative Care – Key Focus Areas

🔴Hemodynamic Monitoring:

• Arterial line: For BP

• CVP line: Venous pressure monitoring

• Frequent VS (q15min to q1hr)

• Monitor for dysrhythmias, hypotension

🫁Respiratory Needs:

• Ventilator support PRN

• Oxygen, suctioning, ABG monitoring

😌Pain & Comfort:

• Morphine, acetaminophen, or PCA pump

• Provide rest & cluster care

💧Fluid Management:

• Monitor:

  • All intake: IV fluids, meds, flushes

  • All output: Urine, chest tube, NG, surgical drains

• Watch for:

  • Urine output <1 mL/kg/hr → sign of renal failure

  • Related to ↓ cardiac output

💉 Chest Tube Monitoring

• Check hourly for drainage color:

  • Bright red initially, should turn serous

• Notify surgeon if:

  • 3 mL/kg/hr for 3 hours

  • OR >5–10 mL/kg in 1 hour

• Watch for cardiac tamponade:

  • Rapid, life-threatening pericardial fluid accumulation

⚠Cardiac Tamponade Signs:

• Narrow pulse pressure

• ↑ Heart rate

• Dyspnea

• Muffled heart sounds

• Decreased cardiac output

❗ Common Postoperative Complications

📌 Postpericardiotomy Syndrome

🧠 What Is It?

• Inflammatory reaction seen post cardiac surgery

• May occur immediately or 7–21 days after

🤒 Symptoms:

• High fever (38–40°C)

• Pericardial friction rub

• Pleural or pericardial effusion

• Chest pain, malaise

💊 Treatment:

• Anti-inflammatories (NSAIDs or steroids)

• Pericardiocentesis or pleurocentesis if fluid buildup is severe

❤ Cardiovascular Dysfunction – Acquired Heart Disease (Pediatrics)

Can occur in a normal heart or be superimposed on congenital heart disease

Influenced by infections, autoimmune responses, environmental factors, or genetics

🦠Infective Endocarditis (IE)

📌 Also Known As:

• Bacterial Endocarditis (BE)

• Infective Endocarditis (IE)

• Subacute Bacterial Endocarditis (SBE)

🧠 Pathophysiology:

• Turbulent blood flow damages the heart endothelium (esp. valves)

• Microorganisms (commonly Strep) adhere to damaged areas → trigger platelet + fibrin deposits

• Vegetations form → may break off and embolize

• Can invade valves, myocardium, or cause systemic emboli (e.g., brain, lungs, kidneys)

🧬 Risk Factors:

• Congenital heart defects (especially with prosthetic valves)

• Recent dental, GI, or respiratory procedures

• Invasive devices (e.g., central lines)

🩺 Clinical Manifestations:

• Low-grade fever, malaise, anorexia

• New murmur or change in existing murmur

• Signs of CHF

• Respiratory distress, tachycardia

• Splenomegaly

• Osler Nodes: Painful red nodules on finger/toe pads (immune complexes)

• Janeway Lesions: Painless red or purple macules on palms/soles (microemboli)

💊 Therapeutic Management:

• High-dose IV antibiotics for 2–8 weeks

• Repeat blood cultures to assess effectiveness

• If ineffective: Surgical removal of vegetation, valve replacement

• Fungal IE: Treat with Amphotericin B

✅ Prevention:

• Prophylactic antibiotics 1 hour before:

  • Dental procedures

  • Invasive respiratory procedures

  • Soft tissue procedures (e.g., biopsies)

• NOT needed for GI/GU procedures

🧼 Parent Teaching:

• Dental hygiene is critical to prevent IE

🔁Rheumatic Fever (RF) & Rheumatic Heart Disease (RHD)

🧠 What Is It?

• Autoimmune inflammatory disease following untreated Group A Strep (GABHS) pharyngitis

• Rheumatic Heart Disease = permanent valve damage due to RF

🧬 Pathophysiology:

• Molecular mimicry: Immune response against strep also attacks heart, joints, skin, brain

🩺 Clinical Manifestations:

🔺 Major Criteria (Jones Criteria):

• Carditis: Most often affects mitral valve

• Polyarthritis: Migratory, affects large joints

• Chorea (Sydenham’s chorea): Involuntary jerking, emotional lability

• Erythema marginatum: Pink rash with clear centers, trunk/extremities

• Subcutaneous nodules: Painless, over bony areas (hands, elbows, scapula)

🔻 Minor Criteria:

• Fever

• Arthralgia (joint pain)

• ↑ CRP, ESR (inflammatory markers)

• Prolonged PR interval on ECG

🧪 Histologic Finding:

• Aschoff bodies: Inflammatory lesions in heart tissue (seen under microscope)

💊 Management:

• Treat strep:

  • Penicillin G IM x1

  • Penicillin V PO x10 days

  • Erythromycin if PCN-allergic

• Anti-inflammatory therapy (aspirin, steroids)

• Bedrest, monitor cardiac status

• Long-term prophylaxis:

  • Monthly Penicillin G IM for years to prevent recurrence

🔥Kawasaki Disease

🧠 What Is It?

• Acute systemic vasculitis (unknown cause)

• Self-limited, but can cause coronary artery aneurysms (20% if untreated)

• Most common in children <5 years

• #1 cause of acquired heart disease in U.S. children

📆 3 Phases:

🩺 Key Clinical Signs:

• Prolonged fever >5 days

• Bilateral conjunctival injection

• Strawberry tongue, cracked lips

• Swelling/erythema of hands/feet, skin peeling

• Rash (esp. perineum)

• Cervical lymphadenopathy

🧬 Complications:

• Coronary artery aneurysms

• Myocarditis, pericarditis, MI

💊 Treatment:

• IVIG (within first 7–10 days):

  • ↓ risk of coronary aneurysms

• Aspirin (ASA):

  • Anti-inflammatory and anticoagulant

  • Continue until platelets normalize

  • Switch to warfarin if aneurysms develop

• ⚠ Monitor for Reye’s Syndrome (esp. with viral illness)

🧪 Follow-up:

• Serial echocardiograms

• Monitor platelets, ESR, CRP

💢Systemic Hypertension (HTN)

🧠 Types:

• Primary HTN: No known cause (rare in children)

• Secondary HTN: Caused by:

  • Renal disease (most common)

  • Cardiac defects

  • Endocrine or neurologic disorders

📋 Screening:

• Begin at age 3

• Measure BP in all four limbs in cases of suspected CoA

🧬Hyperlipidemia (Pediatric)

📌 Overview:

• Atherosclerosis begins in childhood → early identification is key

• Children with family history or risk factors should be screened

🩺 Screening:

• Total cholesterol, LDL, HDL, triglycerides

• Target LDL < 110 mg/dL

💊 Management:

• 1st line: Dietary modifications

  • ↓ Fat, ↓ cholesterol, ↑ fiber, exercise

• Medications (if diet fails):

  • Cholestyramine (Questran)

  • Colestipol (Colestid)