ABP Pediatric Cardiology cheat sheet

A restrictive ventricular septal defect produces a loud holosystolic murmur at the left lower sternal border; a large unrestrictive defect may be quiet and present with heart failure.

Tetralogy of Fallot consists of right ventricular outflow obstruction, ventricular septal defect, overriding aorta, and right ventricular hypertrophy; the chest radiograph classically shows a boot-shaped heart.

Hypercyanotic tetralogy spell: knee-chest position, oxygen, morphine, intravenous fluids, phenylephrine, and beta-blockade; avoid isoproterenol, which can worsen dynamic outflow obstruction.

d-Transposition of the great arteries creates parallel systemic and pulmonary circulations; prostaglandin E1 maintains mixing, and the arterial switch operation is preferred within the first 2 weeks of life.

Coarctation of the aorta causes upper-extremity hypertension and a blood pressure gradient to the legs; bicuspid aortic valve coexists in up to 50%, and neonatal collapse occurs when the ductus arteriosus closes.

Atrial septal defect produces a fixed split second heart sound; closure is indicated for right ventricular dilation or a pulmonary-to-systemic flow ratio of at least 1.5.

Ebstein anomaly shows apical displacement of the septal tricuspid leaflet by at least 8 mm/m² body surface area, causing right atrialization of the ventricle and predisposition to Wolff-Parkinson-White syndrome.

Hypoplastic left heart syndrome is ductal-dependent; prostaglandin E1 is started at diagnosis, and staged palliation proceeds through the Norwood, bidirectional Glenn, and Fontan operations.

Truncus arteriosus is a single arterial trunk supplying systemic, pulmonary, and coronary circulation over a ventricular septal defect; 22q11.2 deletion is common, and complete repair is performed in early infancy.

Kawasaki disease: give intravenous immunoglobulin 2 g/kg over 10–12 hours with aspirin; obtain echocardiography at diagnosis, at 2 weeks, and at 6–8 weeks.

Kawasaki coronary risk factors include age under 1 year, male sex, fever lasting more than 10 days, and failure to respond to initial intravenous immunoglobulin; giant aneurysms are at least 8 mm.

Acute rheumatic fever diagnosis uses Jones criteria: evidence of preceding group A streptococcal infection plus two major or one major and two minor manifestations; echocardiographic carditis supports the diagnosis.

Rheumatic fever prophylaxis: without carditis, penicillin for 5 years or until age 21; with carditis and residual valve disease, prophylaxis for 10 years or until age 40, sometimes lifelong.

Acute pericarditis causes positional chest pain, a friction rub, diffuse ST elevation with PR depression, and pulsus paradoxus when tamponade develops; colchicine reduces recurrence.

Cardiac tamponade shows pulsus paradoxus, electrical alternans, and echocardiographic diastolic chamber collapse; urgent pericardiocentesis is indicated for hemodynamic compromise.

Myocarditis is supported by troponin, B-type natriuretic peptide, echocardiography, and cardiac magnetic resonance imaging; management is supportive, with inotropes or mechanical support for refractory shock.

Small Kawasaki coronary aneurysms often regress, whereas giant aneurysms persist and require anticoagulation, antiplatelet therapy, and serial imaging because of thrombosis and stenosis risk.

Infant supraventricular tachycardia typically has a narrow QRS with a rate of 220–300 beats/min; vagal maneuvers are followed by adenosine 0.1 mg/kg rapid intravenous push, then 0.2 mg/kg if needed.

Adenosine and other atrioventricular nodal blockers should be avoided in pre-excited atrial fibrillation because they can accelerate conduction over the accessory pathway and precipitate ventricular fibrillation.

Wolff-Parkinson-White syndrome shows a short PR interval and delta wave; accessory pathway ablation is considered for symptomatic tachycardia or high-risk pathway characteristics.

Long QT syndrome: corrected QT interval equals QT divided by the square root of the RR interval; a QTc above 480 ms increases risk, and beta-blockers plus avoidance of QT-prolonging drugs are first-line measures.

Congenital complete atrioventricular block is associated with maternal anti-Ro/SSA or anti-La/SSB antibodies; pacing is indicated for symptoms, low ventricular rate, or ventricular dysfunction.

Unstable ventricular tachycardia requires synchronized cardioversion; stable monomorphic ventricular tachycardia may be treated with amiodarone or lidocaine while reversible causes are corrected.

An implantable cardioverter-defibrillator is considered after cardiac arrest, sustained ventricular tachycardia, or high-risk inherited arrhythmia syndromes such as long QT syndrome with syncope.

Postoperative junctional ectopic tachycardia is managed with cooling, sedation, magnesium, and amiodarone; atrioventricular nodal blocking drugs are generally ineffective and may worsen hemodynamics.

Infant heart failure presents with tachypnea, feeding difficulty, poor weight gain, diaphoresis, hepatomegaly, and a gallop rhythm; diuretics and afterload reduction are common initial therapies.

Chronic pediatric heart failure therapy includes diuretics for congestion, angiotensin-converting enzyme inhibitors for afterload reduction, beta-blockers, and aldosterone antagonists; milrinone is used for acute decompensation.

Dilated cardiomyopathy is the most common pediatric cardiomyopathy; causes include myocarditis, genetic mutations, metabolic disease, and anthracycline toxicity.

Hypertrophic cardiomyopathy: avoid dehydration, intense vasodilation, and tachycardia; beta-blockers or calcium channel blockers are used, and sudden death risk prompts consideration of an implantable defibrillator.

Cardiac transplantation rejection surveillance uses echocardiography and endomyocardial biopsy; coronary vasculopathy is detected by angiography or intravascular ultrasound and limits long-term survival.

Fontan circulatory failure may present with protein-losing enteropathy, plastic bronchitis, arrhythmia, or exercise intolerance; evaluation addresses obstruction, fenestration, ventricular function, and pulmonary vascular resistance.

Ventricular assist devices can bridge children with refractory heart failure to transplantation or recovery; anticoagulation, infection, and stroke are major management concerns.

B-type natriuretic peptide supports the diagnosis and monitoring of heart failure but does not establish a specific cause; trends are interpreted with clinical status and echocardiography.

Pulmonary hypertension is defined hemodynamically by a mean pulmonary artery pressure above 20 mm Hg; precapillary disease includes a pulmonary vascular resistance above 3 Wood units.

Indexed pulmonary vascular resistance equals mean pulmonary artery pressure minus pulmonary capillary wedge pressure, divided by cardiac index; a value above 3 Wood units·m² is abnormal.

Acute vasoreactivity testing uses inhaled nitric oxide, oxygen, or epoprostenol; a positive response predicts potential benefit from high-dose calcium channel blockers in idiopathic pulmonary arterial hypertension.

Eisenmenger syndrome is pulmonary vascular disease with reversed or bidirectional shunt; avoid pregnancy and systemic vasodilators, and use pulmonary vasodilators with careful monitoring.

Pulmonary arterial hypertension therapy includes phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and prostacyclin analogues; oxygen and diuretics address hypoxemia and congestion.

Heritable pulmonary arterial hypertension is associated with BMPR2 mutations; genetic counseling is indicated, and family screening may identify affected relatives.

Pregnancy is poorly tolerated in pulmonary hypertension because increased blood volume and reduced pulmonary vascular reserve precipitate right heart failure; contraception and preconception counseling are essential.

In congenital heart disease with pulmonary arterial hypertension, closure decisions depend on pulmonary vascular resistance, shunt direction, and vasoreactivity; irreversible pulmonary vascular disease contraindicates shunt closure.

Qp:Qs equals systemic arterial oxygen saturation minus superior vena cava saturation, divided by pulmonary venous saturation minus pulmonary arterial saturation, measured during cardiac catheterization.

The Fick principle calculates cardiac output as oxygen consumption divided by the arteriovenous oxygen content difference; measured and assumed oxygen consumption values must be interpreted consistently.

Echocardiographic z-scores express a cardiac structure's size relative to body surface area; a z-score beyond plus or minus two indicates abnormal size and guides congenital heart disease decisions.

Cardiac magnetic resonance is the reference method for right ventricular volumes and function in tetralogy of Fallot; progressive dilation or dysfunction supports pulmonary valve replacement.

Computed tomography angiography is useful for coronary artery anatomy, pulmonary veins, and vascular rings when echocardiography is insufficient; radiation exposure requires justification.

Cardiac catheterization provides hemodynamic data, shunt calculation, and angiography; complications include bleeding, arrhythmia, vessel injury, contrast reaction, and air embolism.

Sedation for imaging or catheterization requires cardiorespiratory monitoring because children with congenital heart disease have limited reserve and may develop hypoxemia or hypotension.

Interventional catheterization can perform balloon valvuloplasty, angioplasty, stent placement, and device closure of septal defects or patent ductus arteriosus when anatomy is suitable.

Fetal circulation includes right-to-left shunts through the foramen ovale and ductus arteriosus; the ductus venosus bypasses the liver, and pulmonary blood flow is low because of high pulmonary vascular resistance.

After birth, lung expansion and increased oxygen reduce pulmonary vascular resistance, pulmonary blood flow rises, and the foramen ovale and ductus arteriosus functionally close.

The ductus arteriosus functionally closes within 24–72 hours after birth and anatomically over weeks; prostaglandin E1 maintains patency in ductal-dependent lesions.

Ductal-dependent lesions include transposition of the great arteries, hypoplastic left heart syndrome, critical aortic stenosis, critical coarctation, and pulmonary atresia with intact ventricular septum.

Fetal echocardiography is usually performed between 18 and 22 weeks gestation; earlier or later studies are used for high-risk families, abnormal screening, or fetal arrhythmia.

Newborn critical congenital heart disease screening uses preductal and postductal oxygen saturation; a difference above 3% or saturation below 90% requires prompt echocardiography.

Fetal arrhythmia evaluation includes M-mode and Doppler echocardiography to assess atrial and ventricular rates, atrioventricular relationship, and signs of hydrops.

Neonatal presentation of ductal-dependent lesions may include cyanosis, shock, acidosis, or absent femoral pulses when the ductus closes; prostaglandin E1 is started urgently.

Postoperative low cardiac output syndrome after congenital heart surgery presents with tachycardia, oliguria, poor perfusion, metabolic acidosis, and widened arteriovenous oxygen difference.

Milrinone is a phosphodiesterase-3 inhibitor that increases contractility and causes vasodilation; it is used for low cardiac output after congenital heart surgery but can cause hypotension.

Delayed sternal closure is used when myocardial edema, bleeding, or arrhythmia makes chest closure unsafe after congenital heart surgery; staged closure follows in the intensive care unit.

Extracorporeal membrane oxygenation provides temporary circulatory support for refractory low cardiac output, cardiac arrest, or hypoxemia; bleeding, infection, and neurologic injury are major complications.

Ventricular assist devices provide longer-term support than extracorporeal membrane oxygenation and can bridge children to transplantation or recovery; anticoagulation and infection prevention are critical.

The vasoactive-inotropic score quantifies cardiovascular support intensity by weighting dopamine, dobutamine, epinephrine, norepinephrine, milrinone, and vasopressin doses.

Perioperative management of pulmonary hypertension includes avoiding hypoxemia, acidosis, pain, and agitation; inhaled nitric oxide and pulmonary vasodilators reduce pulmonary vascular resistance.

Nutrition and feeding after pediatric cardiac surgery require attention to vocal cord dysfunction, aspiration risk, calorie needs, and fluid balance; early enteral feeding is used when hemodynamically stable.

The 22q11.2 deletion causes conotruncal anomalies including tetralogy of Fallot, truncus arteriosus, and interrupted aortic arch, plus hypocalcemia and immunodeficiency.

Williams syndrome, caused by a 7q11.23 deletion, commonly causes supravalvar aortic stenosis and peripheral pulmonary stenosis; it also features elfin facies and hypercalcemia.

Noonan syndrome is associated with PTPN11 and related mutations; cardiovascular features include pulmonary valve stenosis, hypertrophic cardiomyopathy, and atrial septal defect.

Marfan syndrome, from FBN1 mutations, causes aortic root dilation; beta-blockers or angiotensin receptor blockers and serial imaging aim to prevent dissection, with surgery for severe dilation.

Loeys-Dietz syndrome causes aggressive arterial tortuosity and aneurysm formation; TGFBR1, TGFBR2, and SMAD3 mutations warrant early imaging and low thresholds for surgical repair.

Familial hypercholesterolemia is suggested by markedly elevated low-density lipoprotein cholesterol, family history of premature cardiovascular disease, or tendon xanthomas; statin therapy is indicated in children when criteria are met.

Preparticipation sports screening includes personal and family history of sudden death, syncope, chest pain, murmur, and hypertension; abnormal findings prompt cardiology evaluation.

Genetic testing in congenital heart disease can identify syndromic causes, inform family screening, and guide reproductive counseling; results are interpreted with genetic counseling.

The I-PASS handoff includes illness severity, patient summary, action list, situation awareness, and contingency planning, plus synthesis by the receiver; it reduces communication errors.

Closed-loop communication requires the sender to confirm that the receiver heard and understood the message, and the receiver to verify completion of the requested action.

Root cause analysis examines systems factors behind a serious safety event, identifies contributing causes, and produces action plans; it is nonpunitive and distinct from individual performance review.

The Plan-Do-Study-Act cycle tests a small change, studies the result, and adjusts before broader implementation; repeated cycles support measurable improvement in clinical processes.

Shared decision making integrates clinician evidence, family values, and patient preferences; it is essential for complex congenital heart disease treatment choices and informed consent.

Quality metrics in pediatric cardiology include surgical mortality, length of stay, readmission, infection rates, catheterization complications, and appropriate echocardiography use.

Systems-based practice includes care coordination, transitions between inpatient and outpatient settings, resource stewardship, and recognizing how insurance and social factors affect outcomes.

Scholarship includes formulating a research question, ethical review, accurate data collection, critical appraisal, and dissemination; quality improvement projects can be scholarly when rigorously designed and reported.