ABP Pediatric Cardiology key terms

A hole in the ventricular septum allowing blood to shunt between the left and right ventricles. Small defects may close spontaneously; large ones cause heart failure, pulmonary overcirculation, and eventual pulmonary vascular disease.

A congenital lesion with four features: ventricular septal defect, right ventricular outflow obstruction, an overriding aorta, and right ventricular hypertrophy. Cyanosis varies with the degree of obstruction, and surgical repair is usually performed in infancy.

A cyanotic defect in which the aorta arises from the right ventricle and the pulmonary artery from the left, creating parallel circulations. Survival before repair depends on mixing through a septal defect, a patent ductus, or balloon septostomy.

Narrowing of the aortic isthmus that raises upper-body blood pressure and reduces lower-body perfusion. It may present in neonates with shock when the ductus closes, or later with hypertension; treatment is surgical or by catheter stenting.

Persistence after birth of the fetal vessel connecting the pulmonary artery and descending aorta. It causes left-to-right shunting and pulmonary overcirculation, while in duct-dependent lesions keeping it open with prostaglandin can be life-saving.

A staged surgical pathway routing systemic venous blood directly to the pulmonary arteries without a ventricular pump, used for single-ventricle hearts. Patients live with elevated central venous pressure and risks of arrhythmia, protein-losing enteropathy, and liver disease.

Irreversible pulmonary vascular remodeling caused by long-standing left-to-right shunting, which reverses the shunt and produces cyanosis. Defect closure is then contraindicated; care focuses on pulmonary vasodilators, oxygen, and avoiding factors that further raise vascular resistance.

An acute systemic vasculitis of childhood, most common under age five, causing prolonged fever, conjunctivitis, rash, mucosal changes, and swollen extremities. Coronary involvement makes timely intravenous immunoglobulin and aspirin essential to prevent aneurysms and thrombosis.

Abnormal dilation of a coronary artery, most often a complication of Kawasaki disease, quantified by echocardiographic Z-scores. Giant aneurysms carry risks of thrombosis and myocardial infarction, so antiplatelet or anticoagulant therapy and serial imaging are required.

An autoimmune inflammatory response to untreated group A streptococcal pharyngitis, producing carditis, arthritis, chorea, rash, and nodules. Valve damage, especially mitral regurgitation, may be permanent; penicillin prophylaxis prevents recurrence and further injury.

Inflammation of heart muscle, usually triggered by viral infection or immune activation, causing arrhythmia, chest pain, or heart failure. Diagnosis rests on clinical findings, troponin, imaging, and occasionally biopsy, while treatment is largely supportive.

Pericardial fluid under pressure compressing the heart and impairing filling. Findings include tachycardia, hypotension, pulsus paradoxus, and distended neck veins; urgent pericardiocentesis or surgical drainage is required.

A rapid narrow-complex rhythm arising above the ventricles, most often from a reentrant circuit in infancy. Infants may present with poor feeding and heart failure; vagal maneuvers and adenosine are first-line therapies.

A congenital accessory pathway between atria and ventricles that bypasses the atrioventricular node, producing a short PR interval and delta wave. It predisposes to reentrant tachycardia and, rarely, rapid conduction of atrial fibrillation causing sudden death.

Complete failure of atrial impulses to conduct to the ventricles, present at birth and often related to maternal anti-Ro or anti-La antibodies. It causes fetal bradycardia, and symptomatic infants usually require permanent pacing.

An inherited or acquired repolarization abnormality with a prolonged QT interval on ECG, predisposing to torsades de pointes, syncope, and sudden death. Management includes beta-blockers, avoiding triggering drugs, and sometimes an implantable defibrillator.

A percutaneous procedure that destroys abnormal myocardial tissue causing arrhythmia, usually with radiofrequency energy or cryotherapy. It is curative for many accessory pathways and focal tachycardias, with small risks of heart block and perforation.

An implanted device that monitors rhythm and delivers shocks or pacing to terminate dangerous ventricular arrhythmias. In children it is considered after cardiac arrest or in high-risk inherited arrhythmia syndromes and cardiomyopathies.

A cardiomyopathy with a dilated, poorly contracting left ventricle and reduced ejection fraction, leading to heart failure and arrhythmia. Causes include myocarditis, genetic mutations, and neuromuscular disease, and treatment follows evidence-based heart failure pathways.

Cardiomyopathy with otherwise unexplained left ventricular hypertrophy, often from sarcomere gene mutations. It can obstruct outflow, impair diastolic filling, and cause arrhythmia or sudden death; beta-blockers and formal risk assessment guide care.

Immune-mediated injury to a transplanted heart, classified as acute cellular or antibody-mediated. It presents with graft dysfunction or arrhythmia and is detected by biopsy or donor-specific antibodies; treatment intensifies immunosuppression.

Elevated pressure in the pulmonary arteries from increased vascular resistance, which strains the right ventricle. In children it may be idiopathic, heritable, or associated with congenital shunts, and therapy includes vasodilators, oxygen, and avoiding hypoxia.

The resistance opposing blood flow through the pulmonary vascular bed, calculated from the pressure gradient and cardiac output. It determines operability in shunt lesions and guides the treatment of pulmonary hypertension.

Noninvasive ultrasound imaging of the heart with a probe on the chest, showing chamber size, valve function, shunts, and ventricular performance. It is the first-line tool for diagnosing congenital and acquired lesions and for serial follow-up.

An invasive procedure passing catheters through blood vessels into the heart to measure pressures and oxygen saturations and to perform interventions. It assesses operability, shunt size, and vascular resistance before surgery or in follow-up.

A method of calculating cardiac output as oxygen consumption divided by the arteriovenous oxygen difference. In the catheterization laboratory it underpins derived measures such as shunt fraction and pulmonary vascular resistance.

Ultrasound examination of the fetal heart and great vessels, usually between 18 and 24 weeks, to detect structural and rhythm abnormalities. It guides prenatal counseling, delivery planning, and selected fetal interventions.

The normal sequence after birth in which lung expansion and ductal closure redirect blood flow from the placenta to the lungs. Failure of these changes causes persistent fetal circulation or duct-dependent cyanosis.

A continuous intravenous infusion that keeps the ductus arteriosus open in newborns with duct-dependent congenital lesions. It may cause apnea, so airway readiness and monitoring are needed when it is started.

Extracorporeal life support that oxygenates and pumps blood outside the body, sustaining the heart or lungs during reversible failure. In children it is used for refractory shock, fulminant myocarditis, or post-cardiotomy collapse.

A circuit that temporarily takes over heart and lung function during open heart surgery by diverting venous blood to an oxygenator and returning it to the aorta. It permits a still, bloodless operative field.

Evaluation of young athletes before sports to detect conditions that could cause sudden cardiac death, using history, examination, and sometimes ECG. Findings such as long QT syndrome or hypertrophic cardiomyopathy prompt restriction and specialist referral.

A heritable connective tissue disorder, usually from FBN1 mutations, causing tall stature, lens dislocation, and progressive aortic root dilation. Beta-blockade and elective aortic root replacement reduce the risk of dissection.

A microdeletion syndrome with conotruncal heart defects, hypocalcemia, immune deficiency, and palatal anomalies. It is suspected in newborns with tetralogy of Fallot, interrupted aortic arch, or truncus arteriosus and confirmed by genetic testing.

A collaborative process in which clinicians present options, benefits, and harms while patients and families share values and preferences, arriving at a joint plan. It is central to informed consent in pediatric cardiology.

A structured, data-driven effort to improve care processes and outcomes through repeated cycles of measurement, intervention, and reassessment. In pediatric cardiology it targets safety, timeliness, and consistency of complex team-based care.