Congenital heart disease nursing: a complete reference for students

Congenital heart disease (CHD) is the most common group of birth defects, affecting roughly 1 in 100 live births. It encompasses a broad spectrum of structural anomalies – from small holes that close on their own, to complex single-ventricle physiology that requires staged surgical reconstruction over the first few years of life. For nurses in the NICU, pediatric cardiac unit, or any unit that receives infants and children, CHD demands fluency in hemodynamic classification, recognition of decompensation, and the clinical judgment to act on subtle early signs.

This reference organizes CHD into two major categories – acyanotic (left-to-right shunt, increased pulmonary blood flow) and cyanotic (right-to-left shunt or mixing, decreased pulmonary blood flow or parallel circulation) – and covers the eight defects most likely to appear on NCLEX. It also covers prostaglandin E1 (PGE1) management, infant feeding in cardiac disease, surgical care in the cardiac intensive care unit (CICU), and more than 15 NCLEX-specific tips. For the broader neonatal context, see the neonatal nursing reference. For pulmonary hypertension that may complicate CHD, see PPHN nursing.

At a glance: congenital heart defects

Acyanotic defects: left-to-right shunts

In acyanotic CHD, blood shunts from the high-pressure left side to the low-pressure right side of the heart. This increases pulmonary blood flow. The infant is not cyanotic at baseline – oxygenated blood still reaches the systemic circulation – but the extra volume load on the right heart and pulmonary vasculature causes its own complications: pulmonary overcirculation, right heart enlargement, pulmonary hypertension, and, over time, the risk of Eisenmenger syndrome.

VSD – ventricular septal defect

VSD is the most common congenital heart defect, accounting for approximately 25–30% of all CHD. It is a hole in the interventricular septum, most commonly located in the perimembranous (membranous) portion near the aortic valve.

Hemodynamics. Oxygenated blood shunts from the left ventricle to the right ventricle with each systole. The size of the shunt (Qp:Qs ratio) determines clinical severity. Small VSDs may produce loud murmurs with minimal hemodynamic impact; large VSDs cause significant volume overload of the right ventricle and pulmonary vasculature.

Clinical features.

• Harsh, holosystolic murmur at the left lower sternal border (LLSB), 3rd–4th intercostal space – the murmur radiates across the precordium
• Infants with large VSDs: tachypnea, diaphoresis with feeds, poor weight gain, recurrent respiratory infections (all signs of congestive heart failure from volume overload)
• Systolic thrill may be palpable at LLSB

Management. Small VSDs (restrictive) often close spontaneously in the first two years of life. Large or symptomatic VSDs require closure: surgical patch repair or, for muscular VSDs, catheter-based device closure. Medical management of CHF (diuretics, ACE inhibitors, calorie-dense feeds) bridges the infant to surgery.

Key NCLEX fact: VSD is the most common CHD. The murmur is harsh and holosystolic at the LLSB – not diastolic, not continuous.

Eisenmenger risk. Untreated large VSDs allow sustained high-pressure, high-flow pulmonary blood flow. Over time (months to years) this causes irreversible pulmonary vascular remodeling. Pulmonary vascular resistance (PVR) rises until it exceeds systemic vascular resistance (SVR), at which point the shunt reverses direction – blood now flows right-to-left, producing cyanosis. This is Eisenmenger syndrome, and once established, surgical repair of the VSD is contraindicated because closing the hole would leave the right ventricle with no outlet against the now-fixed high PVR.

ASD – atrial septal defect

ASD is an opening in the interatrial septum. The most common type is the ostium secundum defect (70% of ASDs), located in the central fossa ovalis region. Ostium primum defects are located lower in the septum near the atrioventricular valves and are associated with Down syndrome.

Hemodynamics. Blood shunts from the left atrium to the right atrium. The right ventricle and pulmonary vasculature handle increased volume. Because this is an atrial-level shunt (low pressure differential), the clinical impact in childhood is usually modest.

Clinical features.

• Fixed, widely split S2 – this is the pathognomonic finding. Normally, the split of S2 widens with inspiration and narrows with expiration. In ASD, the right ventricle is perpetually volume-loaded, so its prolonged ejection time fixes the split regardless of the respiratory cycle.
• A systolic ejection murmur at the upper left sternal border (pulmonic area) from increased flow across the pulmonary valve – this is a flow murmur, not from the ASD itself
• Most children are asymptomatic; the defect is often found incidentally on a murmur workup

Late complications. Left unrepaired into adulthood:

• Paradoxical embolism: a venous clot crosses the ASD and enters the arterial circulation, causing stroke
• Atrial fibrillation from right atrial enlargement
• Pulmonary hypertension (less common than with VSD/PDA, but possible)

Management. Catheter-based closure with a septal occluder device (for secundum ASDs) or open surgical repair (for primum and large defects). Closure is recommended before school age if the defect is hemodynamically significant.

Key NCLEX fact: Fixed split S2 = ASD. This is the single most tested ASD finding on NCLEX. Do not confuse it with VSD or PDA.

PDA – patent ductus arteriosus

The ductus arteriosus is a fetal shunt connecting the pulmonary artery to the aorta, designed to bypass the uninflated fetal lungs. It normally closes within 24–72 hours of birth in response to rising arterial oxygen tension and falling prostaglandin E2 (PGE2). A PDA persists when this closure does not occur.

Risk factors. Prematurity (the ductus is more prostaglandin-sensitive and less responsive to oxygen in premature infants), maternal rubella infection in the first trimester, hypoxia at birth, and altitude.

Hemodynamics. Blood shunts from the high-pressure aorta into the lower-pressure pulmonary artery throughout the cardiac cycle – both systole and diastole – producing a continuous left-to-right shunt. This floods the pulmonary vasculature with additional blood.

Clinical features.

• Continuous “machinery” murmur heard best at the left infraclavicular region – the murmur runs through systole and diastole without interruption, peaking at S2
• Bounding peripheral pulses (wide pulse pressure from diastolic runoff into the pulmonary circuit)
• Tachycardia, tachypnea, and widened pulse pressure
• In premature infants: apnea, worsening respiratory distress, and need for increased ventilatory support

Management.

• Premature infants: indomethacin (or ibuprofen) IV – COX inhibitor that reduces prostaglandin synthesis, causing the ductus to constrict. Indomethacin is most effective in the first days of life. Contraindicated if the infant has renal failure, thrombocytopenia, or necrotizing enterocolitis.
• Term infants and older children: surgical ligation via left thoracotomy, or catheter-based coil/device occlusion.
• Conservative: fluid restriction and diuretics in infants who are not surgical candidates.

Key NCLEX fact: Continuous “machinery” murmur = PDA. The murmur is continuous through systole and diastole. Indomethacin closes the PDA by reducing prostaglandins – it does not open it.

Coarctation of the aorta

Coarctation is a discrete narrowing of the aorta, almost always at or just distal to the ductus arteriosus (juxtaductal coarctation). The narrowing obstructs systemic blood flow, causing a pressure differential between the upper body (proximal to the coarctation) and the lower body (distal to it).

Associated conditions. Turner syndrome (45,X) is strongly associated – coarctation occurs in 10–35% of females with Turner syndrome. Bicuspid aortic valve co-occurs in 50–85% of patients with coarctation.

Clinical features.

• Hypertension in the upper extremities and hypotension in the lower extremities – this pressure gradient is the defining hemodynamic finding
• Femoral pulse deficit (weak or absent femoral pulses compared to brachial) or brachial-femoral pulse lag (femoral pulse palpated after the brachial pulse)
• Blood pressure differential: measure in right arm (pre-ductal) and a lower extremity; a gradient >20 mmHg is significant
• Rib notching on chest X-ray in older children and adults – intercostal arteries enlarge as collateral vessels, eroding the undersurface of the posterior ribs (seen from the 3rd–9th ribs)
• Infants with critical coarctation: shock and metabolic acidosis when the PDA closes, because ductal flow was providing systemic perfusion distal to the obstruction

Management. Balloon angioplasty with stent placement or surgical resection with end-to-end anastomosis. Critical neonatal coarctation with ductal dependency requires PGE1 infusion to maintain patency of the ductus and preserve distal perfusion until repair.

Key NCLEX fact: Always check four-extremity blood pressures in suspected coarctation. Upper extremity HTN + lower extremity hypotension + femoral pulse deficit = coarctation until proven otherwise.

Cyanotic defects: right-to-left shunts and mixing lesions

In cyanotic CHD, deoxygenated blood bypasses the lungs and enters the systemic circulation (right-to-left shunt), or oxygenated and deoxygenated blood mix at a cardiac level, producing systemic oxygen desaturation. The infant is blue – hence the clinical term “blue baby.”

A useful mnemonic for the cyanotic defects is the 5 Ts: Tetralogy of Fallot, Transposition of the great arteries, Truncus arteriosus, Total anomalous pulmonary venous return (TAPVR), and Tricuspid atresia.

Tetralogy of Fallot (TOF)

TOF is the most common cyanotic congenital heart defect and one of the highest-yield NCLEX topics in all of pediatric cardiac nursing.

The four components:

1. Ventricular septal defect (VSD) – large, non-restrictive, perimembranous
2. Right ventricular outflow tract obstruction (RVOTO) – usually subvalvular (infundibular) pulmonary stenosis
3. Overriding aorta – the aortic root straddles the VSD, receiving blood from both ventricles
4. Right ventricular hypertrophy (RVH) – a consequence of chronic right ventricular pressure overload from the RVOTO

Hemodynamics. Pulmonary stenosis increases right ventricular pressure. Blood takes the path of least resistance through the VSD into the overriding aorta, bypassing the lungs. The degree of pulmonary stenosis determines the degree of cyanosis: mild stenosis may allow near-normal oxygen saturation at rest (“pink tet”), while severe stenosis causes profound cyanosis.

Clinical features.

• Cyanosis – present from birth or emerging over the first months of life as the right ventricular infundibulum hypertrophies
• Clubbing of fingers and toes (chronic hypoxia → periosteal proliferation)
• Boot-shaped heart on chest X-ray – upturned apex from right ventricular hypertrophy, concave main pulmonary artery segment (pulmonary stenosis), and a small pulmonary vascular shadow
• Systolic ejection murmur at the upper left sternal border (from pulmonary stenosis – not from the VSD, which is non-restrictive and generates no significant gradient)
• Polycythemia on CBC – compensatory increase in red cell mass from chronic hypoxia

Tet spells (hypercyanotic episodes). Tet spells are acute episodes of severe cyanosis and hypoxia triggered by infundibular spasm, crying, feeding, defecation, or fever. The infundibulum goes into spasm, pulmonary blood flow drops acutely, and right-to-left shunting through the VSD suddenly increases. The infant becomes profoundly cyanotic and may lose consciousness.

Tet spell management (priority order):

1. Knee-chest position (or squatting in older children) – increases SVR by kinking the femoral arteries, which raises aortic pressure above right ventricular pressure, reducing right-to-left shunting and forcing more blood through the pulmonary circuit
2. Supplemental oxygen – pulmonary vasodilator, but less important than mechanical SVR increase
3. Morphine IV or IM – reduces infundibular spasm, decreases respiratory drive and agitation
4. IV fluids – volume augments preload and improves cardiac output
5. Phenylephrine (alpha agonist) – raises SVR pharmacologically when position alone is insufficient
6. Propranolol – beta-blocker that reduces infundibular spasm; also used as oral prophylaxis between spells

Key NCLEX fact: During a tet spell, knee-chest position is the priority nursing intervention – not oxygen alone. Squatting increases SVR, which counteracts right-to-left shunting.

Surgical management. Total intracardiac repair: patch closure of the VSD and widening of the right ventricular outflow tract (infundibuloplasty ± pulmonary valvotomy or transannular patch). Typically performed between 3–6 months of age.

Transposition of the great arteries (TGA)

TGA is a cyanotic defect in which the aorta arises from the anatomic right ventricle and the pulmonary artery arises from the anatomic left ventricle – the opposite of normal. This creates two parallel, non-communicating circulatory circuits: deoxygenated blood from the body returns to the right heart and is pumped back out to the body via the aorta; oxygenated blood from the lungs returns to the left heart and is pumped back to the lungs via the pulmonary artery.

This arrangement is not compatible with life unless there is some communication between the two circuits. Most newborns with TGA survive the first hours because a PDA and/or a patent foramen ovale (PFO) allows mixing. Mixing is the only source of systemic oxygen delivery.

Clinical features.

• Profound cyanosis from birth that does not respond to supplemental oxygen – the lungs are receiving oxygenated blood; the systemic circulation is not
• “Egg on a string” chest X-ray appearance – narrow mediastinum (because the aorta and pulmonary artery are stacked front-to-back rather than side-by-side) and a rounded cardiac silhouette; the “string” is the narrow vascular pedicle
• Tachypnea without respiratory distress (no lung pathology – the lungs are fully oxygenated)
• Metabolic acidosis from systemic hypoxia

Immediate management.

1. Prostaglandin E1 (PGE1/alprostadil) IV continuous infusion – keeps the PDA patent to maintain mixing and systemic oxygen delivery. This is a life-saving bridge to definitive repair.
2. Balloon atrial septostomy (Rashkind procedure) – a balloon catheter is passed through the foramen ovale and pulled back forcefully, tearing a larger interatrial communication to improve mixing at the atrial level. Performed at the bedside in the NICU.
3. Arterial switch operation (Jatene procedure) – definitive surgical correction performed within the first 2 weeks of life, while the left ventricle is still conditioned against pulmonary pressures. The aorta and pulmonary artery are transected and switched to their correct ventricular origins; the coronary arteries are reimplanted into the new aorta.

Key NCLEX fact: In TGA, the two circulations are parallel – not in series. The only therapy that keeps the baby alive until surgery is PGE1 (to maintain ductal mixing) and potentially balloon atrial septostomy. Oxygen alone does nothing because the lungs are already receiving fully oxygenated blood.

Hypoplastic left heart syndrome (HLHS)

HLHS describes a spectrum of defects in which the left-sided structures of the heart are severely underdeveloped: the left ventricle is tiny and non-functional, the mitral valve is hypoplastic or atretic, and the ascending aorta is severely hypoplastic. The left heart cannot support systemic circulation.

Ductal dependency. The entire systemic circulation depends on the right ventricle pumping blood through the PDA (from the pulmonary artery, retrograde into the aorta) to reach the body. If the PDA closes, systemic perfusion collapses and the infant dies. PGE1 is life-saving and mandatory from birth until surgical palliation.

Oxygen management in HLHS. This is one of the most counterintuitive concepts in neonatal cardiac nursing: do not give 100% oxygen to an HLHS infant before stage 1 palliation. The right ventricle is simultaneously perfusing the lungs and (through the PDA) the body. High inspired oxygen causes pulmonary vasodilation, which steals blood flow from the systemic circuit by dramatically increasing flow to the low-resistance lungs. The target SpO2 for stage 1 HLHS physiology is 75–85% – an intentionally low target that reflects the mixing of oxygenated pulmonary venous and deoxygenated systemic venous blood in the single right ventricle.

Staged surgical palliation. There is no corrective surgery for HLHS – the left ventricle cannot be rehabilitated. Palliation redirects the right ventricle to serve as the sole pump:

1. Norwood procedure (neonate) – creates a reconstructed aorta from the main pulmonary artery and the native hypoplastic aorta; a shunt (Blalock-Thomas-Taussig or right ventricle-to-pulmonary artery Sano shunt) provides controlled pulmonary blood flow.
2. Glenn procedure (3–6 months) – the superior vena cava is connected directly to the pulmonary artery, so upper body venous return bypasses the heart and flows passively into the lungs.
3. Fontan procedure (2–4 years) – the inferior vena cava is also connected to the pulmonary artery, completing the total cavopulmonary connection. All systemic venous return now flows passively into the lungs; the right ventricle pumps only systemic arterial blood.

Key NCLEX fact: HLHS requires PGE1 immediately after birth. Target SpO2 is 75–85%, not >95%. Supplemental oxygen can be harmful before stage 1 palliation.

Truncus arteriosus

Truncus arteriosus is a rare defect in which a single great vessel (the truncus) exits both ventricles instead of the normal two separate outlets (aorta from the left ventricle, pulmonary artery from the right). The truncus then gives rise to both the aortic arch and the pulmonary arteries. A large VSD is always present, because the truncal valve overrides both ventricles.

Hemodynamics. Oxygenated and deoxygenated blood mix completely in the single great vessel, resulting in a fixed oxygen saturation that is lower than normal but higher than fully cyanotic defects. Pulmonary blood flow is typically increased (the pulmonary circuit receives the same systemic pressure), leading to early pulmonary vascular disease.

Clinical features. Mild to moderate cyanosis from mixing; signs of pulmonary overcirculation and heart failure (tachypnea, poor feeding, diaphoresis); a single semilunar valve that may be stenotic or regurgitant.

Management. Early surgical repair (within the first weeks to months of life) – the Rastelli procedure separates the pulmonary artery from the truncus, closes the VSD, and connects the right ventricle to the pulmonary artery via a conduit.

Hemodynamic classification table

Eisenmenger syndrome. When any uncorrected left-to-right shunt (VSD, ASD, PDA) allows prolonged high-pressure, high-flow pulmonary blood flow, the pulmonary vasculature undergoes irreversible remodeling. PVR rises progressively. When PVR exceeds SVR, the shunt reverses direction (now right-to-left), producing cyanosis, clubbing, and erythrocytosis. Surgical repair of the original defect is contraindicated at this stage – removing the shunt eliminates the right ventricle’s only pressure relief valve, causing acute right heart failure.

Prostaglandin E1 (PGE1/alprostadil): the bridge to surgery

PGE1 is one of the most critical medications in neonatal cardiac nursing. It maintains or restores patency of the ductus arteriosus in infants with ductal-dependent CHD – lesions in which survival depends on ductal blood flow.

Ductal-dependent lesions requiring PGE1:

• TGA – ductal mixing is the only source of systemic oxygenation
• HLHS – ductal flow from the pulmonary artery retrograde into the aorta provides systemic perfusion
• Critical coarctation of the aorta – ductal flow supplies the descending aorta distal to the coarctation
• Pulmonary atresia – ductal flow is the only source of pulmonary blood flow
• Tricuspid atresia – depending on associated anatomy, pulmonary blood flow may be ductal-dependent

Mechanism. PGE1 acts on prostaglandin receptors on the smooth muscle of the ductus arteriosus, causing vasodilation and maintaining patency. It counteracts the oxygen-mediated and prostaglandin-deficiency-driven constriction that normally closes the ductus after birth.

Administration. Continuous IV infusion via a dedicated central or umbilical venous catheter. Starting doses typically range from 0.01–0.05 mcg/kg/min, titrated to clinical response (SpO2, femoral pulse quality, perfusion).

Critical side effects – NCLEX priority:

Key NCLEX fact: The most dangerous side effect of PGE1 is apnea. Before starting a PGE1 infusion, confirm that resuscitation equipment (bag-valve-mask, intubation supplies) is immediately available. Never initiate PGE1 in a setting where you cannot manage the airway.

Nursing priorities across all CHD

Pre- and post-ductal pulse oximetry

Pre-ductal SpO2 is measured on the right hand (right radial artery is pre-ductal; it branches from the aorta before the ductus arteriosus insertion). Post-ductal SpO2 is measured on either foot. A gradient greater than 3% (pre-ductal higher than post-ductal) suggests a significant right-to-left ductal shunt – ductal-dependent systemic lesions or persistent pulmonary hypertension. Newborn critical CHD screening uses this principle: all newborns are screened with both pre- and post-ductal saturations before discharge.

Infant feeding and CHF management

Heart failure in infants manifests very differently from adult heart failure. Watch for:

• Diaphoresis with feeds – the effort of feeding is equivalent to exercise in a cardiac-compromised infant
• Tachypnea during or after feeds – respiratory rate >60 breaths per minute during feeding suggests pulmonary overcirculation or edema
• Fatigue and falling asleep before completing feeds – inadequate caloric intake leads to failure to thrive
• Poor weight gain – the hallmark of uncompensated CHD in infancy

Caloric strategies. Infants with CHD need 120–150 kcal/kg/day – 40–50% more than a healthy infant – because of increased metabolic demand and inefficient feeding. Interventions:

• High-calorie formula: 24–27 kcal/oz (standard formula is 20 kcal/oz)
• Fortified breast milk: human milk fortifier or calorie-dense supplement added to expressed breast milk
• Small, frequent feeds: 30–45 minute feeds every 2–3 hours to minimize fatigue
• Nasogastric (NG) or orogastric (OG) tube feeding overnight to ensure caloric goal is met
• Consultation with a pediatric dietitian

Oxygen saturation targets by lesion type

Oxygen targets are NOT the same for all CHD. In lesions where high oxygen causes pulmonary vasodilation that steals systemic flow (HLHS stage 1, truncus arteriosus), targeting SpO2 >95% is harmful. Know the targets:

• Most CHD (postoperative): SpO2 >95%
• HLHS before Norwood: SpO2 75–85% (intentionally low – balanced circulation)
• Single-ventricle palliation (Glenn/Fontan): SpO2 80–85% is expected and acceptable

Infection prevention and SBE prophylaxis

Certain CHD lesions carry a lifelong risk of infective endocarditis. Antibiotic prophylaxis before dental procedures (specifically procedures that cause gingival bleeding or perforate oral mucosa) is recommended by the American Heart Association for:

• Unrepaired cyanotic CHD (including palliated lesions with residual defects)
• Prosthetic cardiac valves (including surgical and transcatheter)
• Prior episode of infective endocarditis
• Repaired CHD with residual defects adjacent to a prosthetic patch or device (turbulent flow near the prosthetic material sustains endothelial damage)

Prophylaxis is NOT required for:

• Repaired ASD, VSD, or PDA with no residual defect if more than 6 months have elapsed since the repair (endothelialization of the repair site is complete)
• Bicuspid aortic valve without stenosis or regurgitation (historically recommended; current AHA guidelines removed this indication)

Standard prophylaxis: amoxicillin 2 g PO 30–60 minutes before the procedure (or ampicillin IM/IV if unable to take oral). For penicillin allergy: clindamycin, azithromycin, or clarithromycin.

Developmental monitoring and family support

Infants and children with CHD are at elevated risk for neurodevelopmental delay from chronic hypoxia, cardiopulmonary bypass exposure, and prolonged hospitalization. Nursing responsibilities include:

• Developmental screening using validated tools (ASQ-3, Bayley Scales) at each follow-up visit
• Referral to early intervention services when delays are identified
• Family education: activity restrictions by lesion (many children with repaired CHD have no restrictions; those with residual disease or single-ventricle physiology need individualized guidance), signs of decompensation (increased respiratory rate, new or worsening cyanosis, decreased activity tolerance, feeding difficulties), when to call the provider
• Emotional support for families navigating a diagnosis that requires staged surgeries and lifelong cardiology follow-up

Surgical care

Pre-operative management

• PGE1 infusion – continued until the moment of surgical repair in ductal-dependent lesions; confirm infusion is running on arrival to the operating suite
• Hemodynamic monitoring – continuous SpO2 (both pre- and post-ductal for ductal-dependent lesions), arterial line for continuous blood pressure, central venous access
• Baseline labs – CBC (hematocrit, platelet count), metabolic panel (glucose, electrolytes, lactate), coagulation studies (PT, PTT, fibrinogen)
• NPO – standard surgical NPO guidelines; clear fluids up to 2 hours before induction in most centers
• Temperature management – neonates are at high risk for hypothermia; maintain normothermia until the surgical team intentionally induces cooling for bypass

Post-operative care in the CICU

Post-cardiac surgery nursing is among the most complex in all of pediatric nursing. Key monitoring:

Hemodynamic monitoring:

• Arterial line – continuous beat-to-beat blood pressure; also used for frequent ABG sampling
• Central venous pressure (CVP) line – reflects right heart filling; guides fluid management
• Pulmonary artery catheter (selected cases) – measures pulmonary artery pressure and pulmonary capillary wedge pressure; monitors Qp:Qs (pulmonary-to-systemic flow ratio) in mixing lesions
• Near-infrared spectroscopy (NIRS) – cerebral and somatic oximetry; trends more important than absolute values; a sudden drop >20% from baseline triggers urgent assessment

Chest tubes:

• Mediastinal chest tube – drains blood and fluid from the pericardial space; watch for cardiac tamponade (Beck’s triad: hypotension, muffled heart sounds, jugular venous distension – or in infants, sudden rise in CVP + fall in cardiac output)
• Pleural chest tube – drains the pleural space if opened during surgery
• Measure output hourly – bleeding >3 mL/kg/hr sustained for 3+ hours warrants surgical re-exploration

Temporary epicardial pacing wires:

• Placed on the right atrium and/or right ventricle during surgery; exit through the skin below the sternum
• Enable pacing if the patient develops bradycardia or complete heart block post-bypass
• Handle with care: protect wire ends, use insulated gloves – bare wires are direct conduits to the myocardium

Junctional ectopic tachycardia (JET):

• The most common arrhythmia after pediatric cardiac surgery, especially after TOF repair and VSD closure
• Mechanism: thermal or mechanical trauma to the His bundle during surgery
• Heart rate typically 160–220 bpm with narrow QRS and AV dissociation (atrial rate slower than ventricular rate)
• Treatment: mild hypothermia (cooling to 35–36°C), amiodarone infusion, and atrial overdrive pacing to re-establish AV synchrony

Fluid management:

• Capillary leak after bypass causes significant fluid third-spacing in the first 24–48 hours; anticipate fluid bolus requirements
• Diuresis with furosemide begins as hemodynamics stabilize (usually post-operative day 1–2) to mobilize extravascular fluid
• Monitor urine output: >1 mL/kg/hr is acceptable; <0.5 mL/kg/hr for >2 hours warrants assessment

Discharge and home going

• Wound care: sternal incision instructions, signs of wound infection
• Medications: diuretics, digoxin (in some centers), aspirin (post-device implantation or Fontan), enalapril
• Activity: individualized by surgeon – most children with repaired simple lesions return to full activity; single-ventricle children have specific restrictions
• Dietary: continuation of high-calorie feeds until adequate weight gain is established post-operatively
• Follow-up: cardiology clinic within 2–4 weeks of discharge; outpatient echocardiography schedule
• Signs to return immediately: SpO2 below the established baseline, increased work of breathing, sternal instability or clicking, fever >38°C, significantly decreased feeding or wet diapers

NCLEX tips

1. VSD is the most common CHD. When a question asks for the most common congenital heart defect – the answer is VSD, not TOF, not ASD.

2. Fixed split S2 = ASD – not VSD, not PDA, not TOF. No other common defect produces this finding. If you see “fixed split S2” on NCLEX, the answer is ASD.

3. Continuous “machinery” murmur = PDA. The murmur is heard throughout the cardiac cycle (systole and diastole), loudest at the left infraclavicular area.

4. Holosystolic murmur at the LLSB = VSD. Distinguish from the systolic ejection murmur of TOF (upper left sternal border, from pulmonary stenosis) and the ejection murmur of ASD (flow murmur, pulmonic area, not from the ASD itself).

5. Boot-shaped heart on CXR = TOF. Right ventricular hypertrophy turns the apex upward; the concave pulmonary artery segment creates the “boot toe.”

6. “Egg on a string” CXR = TGA. The narrow mediastinum (aorta and pulmonary artery stacked rather than side-by-side) creates the string; the round cardiac silhouette is the egg.

7. Tet spell priority intervention: knee-chest position (not oxygen alone). Squatting/knee-chest position kinks the femoral arteries, raising SVR, which pushes more blood into the pulmonary circuit. Giving oxygen alone without increasing SVR is insufficient.

8. PGE1 most dangerous side effect: apnea. Before starting any PGE1 infusion, confirm that resuscitation equipment is at the bedside. This is the most common NCLEX answer when asked about PGE1 safety.

9. Indomethacin closes the PDA – it is a COX inhibitor that reduces prostaglandin synthesis, causing the ductus to constrict. It does NOT open the ductus. Do not confuse with PGE1, which keeps the ductus open.

10. Coarctation: always check four-extremity blood pressures. Upper extremity hypertension + lower extremity hypotension + absent or delayed femoral pulses = coarctation until proven otherwise.

11. Turner syndrome associations: coarctation + bicuspid aortic valve. Any question mentioning Turner syndrome (45,X) and a cardiac finding is almost certainly pointing toward coarctation or bicuspid aortic valve.

12. TGA: two parallel, non-communicating circuits. Oxygenated blood recirculates to the lungs; deoxygenated blood recirculates to the body. The infant needs mixing (PDA, ASD) to survive. PGE1 is the first intervention.

13. HLHS: target SpO2 75–85%, not >95%. High oxygen delivery before stage 1 palliation causes pulmonary vasodilation, which steals blood from the systemic circuit. This is counterintuitive but critical.

14. Eisenmenger syndrome: once established, surgery is contraindicated. Closing the original defect at this stage removes the right ventricle’s only pressure relief, causing fatal right heart failure. Signs: cyanosis, clubbing, polycythemia in a patient with a known left-to-right shunt.

15. Rubella in first trimester → PDA + pulmonary artery stenosis + cataracts (part of the TORCH sequence – Toxoplasmosis, Other, Rubella, CMV, Herpes). Rubella-associated CHD is specifically PDA, not VSD or ASD.

16. SBE prophylaxis before dental procedures: required for unrepaired/palliated cyanotic CHD, prosthetic valves, and prior endocarditis. NOT required for fully repaired ASD/VSD/PDA with no residual defect more than 6 months post-repair. For more detail, see infective endocarditis nursing.

17. Infant CHF feeding signs: diaphoresis + fatigue + poor weight gain. Sweating during feeds is the hallmark – it reflects the sympathetic response to increased cardiac work. Use high-calorie formula (24–27 kcal/oz) and small, frequent feeds.

NCLEX differentiation table

NANDA-I nursing care plans for congenital heart disease

The following five nursing diagnoses address the highest-priority problems across CHD presentations – from the cyanotic neonate requiring PGE1 to the post-operative infant recovering from VSD repair to the adult with palliated single-ventricle physiology. Interventions are anchored to AHA/ACC guideline thresholds and reflect physiology specific to named defects where applicable.

Decreased cardiac output (Priority 1)

Related to: Intracardiac shunting and structural obstruction – including volume overload secondary to large ventricular septal defect (VSD) or patent ductus arteriosus (PDA), pressure overload secondary to right ventricular outflow tract obstruction in tetralogy of Fallot (TOF), or parallel non-communicating circulation in transposition of the great arteries (TGA)

As evidenced by: Tachycardia, tachypnea, SpO2 below defect-specific target, hypotension or narrow pulse pressure, capillary refill >3 seconds, cool and mottled extremities, decreased urine output (<1 mL/kg/hr in infants), metabolic acidosis on ABG, diaphoresis with feeds in infants with volume-overloaded defects

Expected outcomes:

• Patient maintains heart rate, blood pressure, and SpO2 within defect-specific parameters (e.g., SpO2 75–85% for pre-Norwood HLHS, ≥92% for post-operative VSD repair) within 1 hour of intervention
• Urine output remains ≥1 mL/kg/hr, capillary refill ≤2 seconds, and extremities warm within 4 hours of hemodynamic stabilization

Nursing interventions and rationales:

1. Monitor SpO2 continuously using pre-ductal (right hand) and post-ductal (foot) probes and compare readings every 1–4 hours – A pre-to-post-ductal SpO2 gradient >3% indicates significant right-to-left ductal shunting, as occurs in ductal-dependent lesions (TGA, HLHS, critical coarctation). For HLHS before stage 1 palliation, target SpO2 is 75–85%; targeting >95% with supplemental oxygen causes pulmonary vasodilation that steals systemic blood flow through the PDA, worsening systemic perfusion.

2. Administer PGE1 (alprostadil) by continuous IV infusion at 0.01–0.05 mcg/kg/min via a dedicated central or umbilical venous line in ductal-dependent lesions, titrating to clinical response – PGE1 maintains ductal patency by binding prostaglandin receptors on ductal smooth muscle, counteracting oxygen-mediated and prostaglandin-deficiency-driven closure. Without ductal flow, HLHS, TGA, and critical coarctation of the aorta result in cardiovascular collapse. Confirm resuscitation equipment is at the bedside before initiation due to a 10–12% apnea risk.

3. Assess hemodynamic parameters every 1–2 hours: heart rate, blood pressure (four-extremity BPs in coarctation), mean arterial pressure, CVP, and peripheral perfusion – Upper-extremity hypertension combined with lower-extremity hypotension and absent or delayed femoral pulses identifies coarctation physiology; a >20 mmHg upper-to-lower limb BP gradient is clinically significant. CVP monitoring post-operatively guides fluid resuscitation and identifies tamponade (rising CVP + falling cardiac output).

4. Administer prescribed diuretics (furosemide 1–2 mg/kg IV) and vasoactive agents (dopamine, dobutamine, milrinone) per physician order; monitor urine output hourly and weigh daily – In volume-overloaded states (large VSD, PDA), diuretics reduce right ventricular preload and pulmonary edema. Milrinone, a phosphodiesterase inhibitor, improves myocardial contractility and reduces afterload, particularly useful post-cardiopulmonary bypass. Target urine output ≥1 mL/kg/hr; output <0.5 mL/kg/hr for >2 hours warrants urgent reassessment.

5. Position the infant with the head elevated 30 degrees and facilitate rest by clustering care activities and minimizing noxious stimuli – Agitation and crying increase oxygen consumption by up to 50% and can precipitate tet spells in TOF infants by intensifying right ventricular infundibular spasm. Clustering care reduces sympathetic activation; maintaining a semi-upright position improves pulmonary mechanics and reduces the work of breathing in infants with pulmonary overcirculation.

Activity intolerance (Priority 2)

Related to: Reduced pulmonary blood flow and chronic arterial oxygen desaturation in right-to-left shunting lesions (TOF, tricuspid atresia), or increased myocardial workload from volume or pressure overload in acyanotic defects (VSD, PDA), resulting in an imbalance between oxygen supply and demand during exertion

As evidenced by: SpO2 drop ≥5% below baseline with feeding or activity, tachycardia disproportionate to level of exertion, tachypnea with respiratory rate >60 breaths per minute during feeds, extreme fatigue (falling asleep before completing feeds), squatting behavior in ambulatory children with TOF, cyanotic episodes (tet spells) triggered by crying or feeding

Expected outcomes:

• Infant or child completes feedings without oxygen desaturation below baseline or respiratory rate exceeding 60 breaths per minute within the established care plan
• School-age child participates in structured low-intensity activity for 10–20 minutes without SpO2 drop >4% from resting baseline or heart rate exceeding age-appropriate limits

Nursing interventions and rationales:

1. Monitor SpO2 and heart rate continuously during and after feedings and planned activities; record baseline and peak values – Feeding is the primary exertional activity for infants and metabolically equivalent to moderate exercise in a healthy adult. Documenting the SpO2 nadir and recovery time establishes the infant’s functional reserve and guides feed volume and frequency adjustments.

2. Implement a structured feeding protocol: offer small volumes (30–60 mL per feed) every 2–3 hours; limit feed duration to 20–30 minutes; supplement with nasogastric tube feeds overnight if caloric goals are not met – Fatigue during prolonged oral feeds reduces caloric intake, exacerbating the underlying energy deficit. Infants with CHD require 120–150 kcal/kg/day – up to 50% more than healthy infants – due to increased metabolic demand; this target cannot be met if feeds are repeatedly abandoned due to fatigue.

3. Teach caregivers to recognize and respond to tet spells in TOF: place the child in the knee-chest position immediately, administer supplemental oxygen, and call for emergency assistance – The knee-chest position kinks the femoral arteries, increasing systemic vascular resistance (SVR); as SVR rises above right ventricular pressure, right-to-left shunting through the VSD decreases and pulmonary blood flow increases. This is the first-line intervention and is more effective at reversing the spell than oxygen supplementation alone.

4. Cluster nursing care activities to allow uninterrupted rest periods of at least 60–90 minutes between interventions in infants – In infants with compromised cardiac reserve, repeated disturbance prevents recovery from the metabolic cost of prior activity. Longer rest periods allow heart rate and SpO2 to return to baseline, reducing cumulative oxygen debt.

5. Collaborate with cardiology, physical therapy, and occupational therapy to establish individualized activity guidelines for older children and adults with CHD – Activity restrictions vary significantly by defect and repair status. Many children with repaired simple lesions (ASD, VSD, PDA) have no restrictions. Those with palliated single-ventricle physiology (Glenn or Fontan), residual stenotic lesions, or moderate-to-severe ventricular dysfunction require individualized intensity limits and should not engage in isometric or competitive sports without cardiology clearance per AHA/ACC 2018 adult CHD guidelines.

Imbalanced nutrition: less than body requirements (Priority 3)

Related to: Increased caloric demand from elevated myocardial work in volume-overloaded defects (VSD, PDA, truncus arteriosus), combined with reduced intake secondary to dyspnea and fatigue during feeding and, in some infants, tachypnea sufficient to interfere with oral coordination

As evidenced by: Weight gain less than 15–30 g/day (expected for age), weight-for-length below the 3rd percentile, diaphoresis during feeds, tachypnea with respiratory rate >60 breaths per minute during feeding, feeding times exceeding 30 minutes with volumes less than prescribed, serum albumin <3.5 g/dL or prealbumin trending downward in older patients

Expected outcomes:

• Infant achieves a minimum weight gain of 20–30 g/day over a rolling 5-day period, measured on the same scale at the same time daily
• Total daily caloric intake reaches ≥120 kcal/kg/day within 72 hours of initiating the nutritional care plan

Nursing interventions and rationales:

1. Obtain daily weights on the same scale at the same time each day (before the first morning feed, after voiding) and plot on a WHO or CDC growth chart; report a failure to gain >15 g/day for 3 consecutive days to the medical team – Daily weight is the most reliable objective measure of nutritional adequacy and fluid balance in infants with CHD. A plateau or downtrend in weight despite adequate caloric provision may signal worsening heart failure, malabsorption, or increased energy expenditure from increased work of breathing.

2. Advance formula or expressed breast milk to 24–27 kcal/oz under dietitian guidance; fortify breast milk with human milk fortifier (HMF) at 1–2 packets per 100 mL when the mother is expressing – Standard formula is 20 kcal/oz. Infants with CHD cannot reliably consume sufficient volume to meet caloric needs at standard concentration because fatigue limits intake. Caloric fortification delivers more energy per milliliter, reducing the volume needed per feed and therefore the metabolic cost of feeding.

3. Insert a nasogastric (NG) tube and supplement oral feeds with continuous or bolus NG feeds overnight when oral intake consistently falls short of the prescribed caloric goal – Overnight NG supplementation allows uninterrupted gastric delivery of remaining calories without imposing the respiratory and cardiovascular stress of waking oral feeding. This strategy preserves the infant’s opportunity to develop oral feeding skills while ensuring caloric adequacy.

4. Position the infant at a 45-degree angle during feeds and for 30 minutes afterward; use a slow-flow (level 1 or premature) nipple if the infant is bottle-feeding – A semi-upright position reduces the work of breathing by improving diaphragmatic excursion, decreasing the respiratory effort component of total caloric expenditure during feeding. Slow-flow nipples reduce the bolus rate, giving the infant time to coordinate suck-swallow-breathe without desaturation.

5. Refer to a pediatric dietitian at admission or at the first sign of inadequate weight gain; reassess caloric goals weekly or after any change in cardiac status, diuretic regimen, or surgical intervention – Diuretics (furosemide, spironolactone) increase insensible losses and can unmask electrolyte deficits (hypokalemia, hyponatremia) that affect appetite and feeding behavior. Post-operative metabolic demand peaks in the first 48–72 hours; caloric targets must be recalculated after any surgery involving cardiopulmonary bypass.

Risk for infection (Priority 4)

Related to: Structural cardiac defects that create turbulent blood flow and endothelial damage (unrepaired VSD, PDA, aortic stenosis), presence of prosthetic cardiac material (patches, valves, conduits), post-surgical incisions and invasive monitoring lines, immunologic immaturity in neonates, and altered mucosal barriers from nasogastric tubes

Risk factors present: Unrepaired or palliated cyanotic CHD, prosthetic cardiac valve or conduit, prior infective endocarditis, repaired CHD with residual defect adjacent to prosthetic material, central venous or umbilical catheters, presence of sternal or thoracotomy incision, prolonged ICU admission

Expected outcomes:

• Patient remains afebrile (temperature <38°C/100.4°F) and shows no signs of surgical site infection (erythema, purulent drainage, wound dehiscence) through the 30-day post-operative period
• Patient and caregiver correctly state indications for subacute bacterial endocarditis (SBE) prophylaxis and the need to notify all dental and surgical providers of CHD diagnosis before procedures

Nursing interventions and rationales:

1. Adhere to strict aseptic technique for all invasive line access, dressing changes, and catheter site care; follow institutional CLABSI prevention bundle including daily necessity assessment for all central lines – Children with CHD are at elevated risk for infective endocarditis (IE) once bacteria reach the bloodstream. Turbulent flow across defects or prosthetic material damages the endothelium, creating a nidus for bacterial colonization. Central venous catheter-associated bacteremia is the most common preventable portal of entry for IE in the CICU.

2. Administer prescribed pre-procedural antibiotic prophylaxis (amoxicillin 2 g PO or ampicillin 2 g IV 30–60 minutes before dental or surgical procedures that breach mucosal integrity) in patients who meet AHA indications – AHA/ACC guidelines require SBE prophylaxis for: (1) unrepaired cyanotic CHD including palliated lesions with residual defects, (2) prosthetic cardiac valves (surgical or transcatheter), (3) prior IE, and (4) repaired CHD with residual defect adjacent to a prosthetic patch or device within 6 months of repair. Prophylaxis is NOT required for repaired ASD, VSD, or PDA with no residual defect beyond 6 months post-repair, as endothelialization of the repair site is complete by that time.

3. Monitor temperature every 4 hours in CICU patients and at each clinical encounter in outpatients; assess central and peripheral line sites daily for signs of infection; obtain blood cultures before initiating antibiotics if fever develops – New fever (≥38°C) in a patient with CHD, prosthetic cardiac material, or indwelling lines should be evaluated for bacteremia and IE until proven otherwise. Paired blood cultures (peripheral plus central) improve the sensitivity for detecting line-related bacteremia; always draw cultures before antibiotic administration.

4. Educate family on wound care for sternal and thoracotomy incisions: keep the site dry for 48–72 hours post-discharge, report any erythema extending >1 cm from the incision margin, purulent drainage, sternal clicking or instability, or fever >38°C – Mediastinitis and sternal wound infection are rare but potentially fatal complications of cardiac surgery. Early recognition allows prompt surgical debridement or antibiotic therapy before mediastinal involvement deepens. Sternal instability (clicking with movement) specifically signals potential sternal dehiscence requiring urgent surgical evaluation.

5. Ensure age-appropriate immunizations are current and consult with cardiology before administering live vaccines in patients with functionally single-ventricle physiology or chronic cyanosis; administer influenza vaccine annually and RSV prophylaxis (palivizumab) in eligible infants – Respiratory infections are a leading cause of CHD decompensation. Influenza and RSV can precipitate pulmonary hypertensive crises in infants with increased pulmonary blood flow (VSD, ASD, AVSD). Palivizumab is recommended by the American Academy of Pediatrics for infants with hemodynamically significant CHD in the first RSV season.

Deficient knowledge: disease management and home care (Priority 5)

Related to: Complexity of CHD diagnosis and staged management, unfamiliarity with medication regimens and monitoring parameters, lack of prior exposure to cardiac nursing concepts, anxiety surrounding surgical interventions, and the ongoing nature of lifelong cardiology follow-up

As evidenced by: Caregiver verbalization of incorrect or incomplete understanding of diagnosis, inability to state medication names, doses, or indications, inability to identify warning signs requiring emergency evaluation, failure to schedule follow-up appointments, expressed fear about activity and feeding at home

Expected outcomes:

• Caregiver correctly demonstrates medication administration technique, states the dose, frequency, and two key side effects of each prescribed medication before discharge
• Caregiver correctly lists three warning signs requiring immediate emergency evaluation (SpO2 below established baseline, increased work of breathing, poor feeding or decreased wet diapers) before discharge

Nursing interventions and rationales:

1. Conduct structured discharge education sessions using the teach-back method: ask the caregiver to explain in their own words what the diagnosis means, what each medication does, and what changes should prompt a call to cardiology or a trip to the emergency department – Teach-back identifies misunderstanding before it becomes a missed medication dose or delayed emergency presentation. CHD caregivers manage a higher medication burden (diuretics, digoxin, ACE inhibitors, aspirin, anticoagulants in Fontan patients) than most pediatric home settings; confirming mastery prevents medication errors that can precipitate decompensation.

2. Provide written action plans specifying the patient’s individual SpO2 baseline and the threshold below which the caregiver should call 911, the signs of heart failure decompensation (increased respiratory rate, new or worsening cyanosis, difficulty feeding, decreased wet diapers or urine output), and contact information for the cardiac team – Cognitive overload is high in caregivers of newly diagnosed CHD infants. A written, individualized reference card reduces reliance on memory in high-stress situations and improves the likelihood of timely escalation. SpO2 thresholds must be individualized: a baseline of 82% in a Glenn patient is normal; 82% in a post-operative VSD repair patient is an emergency.

3. Teach medication administration technique for each discharge medication with return demonstration before discharge; clarify common misconceptions (e.g., digoxin toxicity signs: bradycardia, vomiting, visual changes; furosemide monitoring: daily weights, signs of dehydration and electrolyte depletion) – Digoxin has a narrow therapeutic index; toxicity (bradycardia, AV block, GI symptoms) can occur within the pediatric dosing range. Caregivers must know to hold the dose and call cardiology if the infant’s heart rate drops below 80–90 bpm (infant threshold). Furosemide causes hypokalemia and dehydration, which can worsen arrhythmia risk.

4. Discuss activity guidelines specific to the child’s defect and repair status; clarify which activities are safe and reinforce that most children with repaired simple defects can participate in normal childhood activities – Caregiver over-restriction of activity is common after CHD surgery and can impair neurodevelopmental and social outcomes. Providing clear, defect-specific guidance reduces anxiety and prevents unnecessary restriction. Children with single-ventricle physiology or significant residual disease require individualized cardiology-directed activity guidelines, while those with repaired VSD, ASD, or PDA and no residual defect typically need no activity restriction.

5. Arrange follow-up appointments with pediatric cardiology within 2–4 weeks of discharge and ensure the caregiver has confirmed the appointment date and the cardiology team’s after-hours contact number before leaving the hospital – Non-compliance with cardiology follow-up is a major cause of preventable decompensation and missed surveillance for post-operative complications (residual shunts, arrhythmias, pulmonary hypertension, ventricular dysfunction). Confirming the appointment date at discharge, rather than instructing caregivers to call for scheduling, has been shown to improve follow-up compliance rates.

Frequently asked questions

What are the nursing priorities for a patient with congenital heart disease?

The top nursing priorities for CHD are maintaining adequate cardiac output, monitoring oxygen saturation within the defect-specific target range, supporting nutritional intake in infants, and preventing infection – particularly infective endocarditis. Priority order shifts by defect type: for a neonate with TGA or HLHS, maintaining PGE1 infusion patency and pre- and post-ductal SpO2 monitoring are the most urgent tasks; for a stable infant with a VSD, feeding support and caloric adequacy often dominate the care plan. Every CHD patient requires a written plan that specifies individualized SpO2 targets, medication thresholds (e.g., hold digoxin if heart rate <90 bpm), and warning signs for immediate escalation.

What is the most common congenital heart defect?

Ventricular septal defect (VSD) is the most common congenital heart defect, accounting for approximately 25–30% of all CHD cases. It is a hole in the interventricular septum and produces a harsh, holosystolic murmur at the left lower sternal border. Small VSDs often close spontaneously in the first two years of life; large VSDs require surgical or catheter-based closure. This is the most frequently tested CHD fact on NCLEX – if asked for the most common congenital heart defect, the answer is VSD.

What are the signs and symptoms of congenital heart disease in adults?

Adults with undiagnosed or palliated CHD present differently from infants. Common presentations include exertional dyspnea and exercise intolerance (from reduced cardiac reserve), palpitations or arrhythmias (atrial fibrillation is particularly common in adults with repaired or unrepaired ASD due to chronic right atrial dilation), peripheral edema, and – in those with longstanding uncorrected left-to-right shunts – progressive cyanosis and clubbing from Eisenmenger syndrome. Some adults present with paradoxical embolism or stroke from an undetected ASD. A systematic cardiovascular history, including any childhood murmur or cardiac workup, is essential in adults presenting with these findings.

How do you monitor oxygen saturation in patients with congenital heart disease?

In CHD, pulse oximetry must be interpreted against the patient’s defect-specific target range rather than normal adult values. Pre-ductal SpO2 (right hand) and post-ductal SpO2 (either foot) should both be measured in neonates; a gradient >3% (pre-ductal higher) indicates significant right-to-left ductal shunting. For pre-Norwood HLHS infants, the target is 75–85% and supplemental oxygen should not be used to push SpO2 above this range, as pulmonary vasodilation from oxygen will reduce systemic perfusion. For Glenn and Fontan patients, SpO2 of 80–85% is expected due to the passive cavopulmonary connection mixing. Most repaired CHD patients target SpO2 ≥92–95%.

What is the nursing care for a child with tetralogy of Fallot?

The most critical nursing skill for TOF is recognizing and managing tet spells (hypercyanotic episodes). When a tet spell occurs: place the child in the knee-chest position immediately (this increases systemic vascular resistance by kinking the femoral arteries, which pushes more blood through the pulmonary circuit), administer supplemental oxygen, prepare morphine for IV or IM administration to reduce infundibular spasm, and notify the medical team. Ongoing care includes continuous SpO2 monitoring, protecting the child from agitation and crying (triggers of infundibular spasm), supporting caloric intake given feeding difficulties, and educating caregivers on tet spell recognition and response. Propranolol may be prescribed as oral prophylaxis between spells.

What medications are used in congenital heart disease nursing?

Key medications in CHD nursing include: prostaglandin E1 (PGE1/alprostadil), which maintains ductal patency in ductal-dependent lesions and has a 10–12% apnea risk requiring airway-ready bedside preparation; indomethacin (or ibuprofen), a COX inhibitor that closes the PDA in premature infants by reducing prostaglandin synthesis; furosemide, a loop diuretic used to manage fluid overload and pulmonary edema in volume-loaded defects; digoxin, which increases contractility and slows AV conduction in infants with heart failure from large shunts; ACE inhibitors (enalapril, captopril), which reduce systemic afterload; and aspirin or anticoagulation in Fontan patients to reduce thromboembolic risk. Each has pediatric-specific dosing thresholds and side effect profiles that nurses must monitor closely.

When should a nurse call the physician for a patient with congenital heart disease?

Escalate immediately for any of the following: SpO2 falling more than 5% below the patient’s established baseline or dropping below the defect-specific minimum target; new or worsening cyanosis; heart rate outside age-appropriate limits (bradycardia below 80–90 bpm in infants, tachycardia above 180–200 bpm); systolic BP drop >20% from baseline or femoral pulse quality change (relevant in coarctation post-repair); urine output <0.5 mL/kg/hr for >2 hours; apnea in a patient on PGE1; chest tube output >3 mL/kg/hr sustained over 3 hours post-surgery; new fever ≥38°C in a patient with prosthetic cardiac material; or sternal clicking or wound dehiscence after surgery.

What is the nurse’s role in congenital heart disease education?

The nurse is the primary educator for families navigating a CHD diagnosis. Core educational responsibilities include: explaining the defect in plain language using diagrams or models; teaching medication administration with return demonstration and written instructions; providing individualized SpO2 action plans with clear thresholds for calling cardiology versus calling 911; clarifying activity guidelines specific to the child’s defect and repair status; teaching recognition of decompensation signs (increased respiratory rate, poor feeding, decreased wet diapers, new cyanosis); explaining SBE prophylaxis requirements and the need to notify all future dental and surgical providers; and confirming follow-up appointment dates before discharge. Using teach-back at each session ensures retention before the family leaves the hospital.

Key sources

Clinical content in this reference is drawn from the following authoritative sources:

• Allen HD, Shaddy RE, Penny DJ, et al. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents. 9th ed. Wolters Kluwer, 2016.
• American Heart Association / American College of Cardiology. 2018 AHA/ACC Guideline for the Management of Adults with Congenital Heart Disease. JACC 2019;73(12):e81–e192.
• Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart. 2016;102(14):1081–1086.
• Mahle WT, Newburger JW, Matherne GP, et al. AHA scientific statement: role of pulse oximetry in examining newborns for congenital heart disease. Circulation. 2009;120(5):447–458.
• National Heart, Lung, and Blood Institute (NHLBI). Congenital heart defects – overview and clinical summaries. Available at: https://www.nhlbi.nih.gov/
• Park MK. Park’s Pediatric Cardiology for Practitioners. 6th ed. Elsevier, 2014.
• Wilson W, Taubert KA, Gewitz M, et al. AHA guideline: prevention of infective endocarditis. Circulation. 2007;116(15):1736–1754 (updated 2021).
• NCSBN NCLEX-RN Detailed Test Plan (current edition) – cardiac content domains including hemodynamic monitoring, pediatric cardiac care.

For the broader neonatal context in which many of these defects present, see the neonatal nursing reference. For pulmonary hypertension as a complication of CHD, see PPHN nursing. For endocarditis risk in CHD patients, see infective endocarditis nursing. For heart failure management relevant to CHD adults and post-operative patients, see heart failure nursing.