ABIM ACHD flashcards

32 cards. Read the term and the definition here. The deck at the top of this page is optional.

CHD birth prevalence

Congenital heart disease occurs in approximately 8 to 9 per 1,000 live births; including bicuspid aortic valve, prevalence approaches 1% to 2% of the population.

Most common congenital heart lesion

Ventricular septal defect is the most common congenital cardiac malformation at birth, accounting for roughly 30% to 40% of cases; bicuspid aortic valve is the most common overall anomaly.

22q11.2 deletion syndrome

A microdeletion at 22q11.2 causing conotruncal defects such as tetralogy of Fallot, truncus arteriosus, and interrupted aortic arch, plus hypocalcemia and immunodeficiency.

Down syndrome cardiac lesions

Approximately 40% to 50% of infants with trisomy 21 have congenital heart disease; atrioventricular septal defect is most characteristic, followed by ventricular septal defect.

Tetralogy of Fallot components

Four lesions: ventricular septal defect, overriding aorta, right ventricular outflow tract obstruction, and right ventricular hypertrophy; cyanosis severity depends on the degree of outflow obstruction.

Eisenmenger syndrome

Pulmonary vascular disease from a long-standing left-to-right shunt causes pulmonary hypertension and shunt reversal, producing cyanosis and a poor prognosis.

Transposition of the great arteries

The aorta arises from the right ventricle and the pulmonary artery from the left ventricle, creating parallel circulations that require mixing through an atrial septal defect, ventricular septal defect, or patent ductus arteriosus.

Fontan circulation physiology

Systemic venous blood flows passively to the lungs without a ventricular pump; cardiac output depends on low pulmonary vascular resistance and adequate ventricular filling.

Qp:Qs shunt calculation

Ratio of pulmonary to systemic blood flow computed from oxygen saturations using the Fick principle; a Qp:Qs greater than 1.5 indicates a hemodynamically significant shunt.

Echocardiographic RVSP estimate

Right ventricular systolic pressure is estimated from the tricuspid regurgitation jet velocity using the modified Bernoulli equation plus an estimate of right atrial pressure.

Cardiac MRI in ACHD

Provides accurate quantification of ventricular volumes, function, and valve regurgitation and is the reference standard for serial surveillance after tetralogy of Fallot repair.

ECG in secundum ASD

Shows an incomplete right bundle branch block pattern and right axis deviation from right ventricular volume overload; findings may be absent in young children.

Prostaglandin E1 indication

Infusion maintains ductal patency in ductal-dependent lesions such as transposition of the great arteries, pulmonary atresia, interrupted aortic arch, and hypoplastic left heart syndrome.

Endocarditis prophylaxis indication

Recommended only for prosthetic valves or material, prior endocarditis, unrepaired cyanotic congenital heart disease, repaired lesions with residual defects, and within six months of repair.

Rashkind procedure

Balloon atrial septostomy enlarges the interatrial communication to improve mixing in transposition of the great arteries and in obstructive lesions with a restrictive atrial septum.

Tetralogy of Fallot repair

Surgical repair closes the ventricular septal defect and relieves right ventricular outflow obstruction, usually performed electively between three and six months of age.

Turner syndrome cardiovascular risk

Bicuspid aortic valve and coarctation are common; aortic root dilation requires serial imaging, and pregnancy carries an increased risk of aortic dissection.

Marfan syndrome aortic surgery threshold

Aortic root replacement is generally recommended at a diameter of 5.0 cm, or 4.5 cm when risk factors such as family history of dissection are present.

Williams syndrome cardiac lesion

Supravalvular aortic stenosis and peripheral pulmonary stenosis result from elastin gene deletion at 7q11.23; coronary ostial stenosis increases anesthetic risk.

Noonan syndrome cardiac findings

Pulmonary valve stenosis with dysplastic leaflets and hypertrophic cardiomyopathy are characteristic; PTPN11 mutations are the most common genetic cause.

Pregnancy hemodynamic changes

Plasma volume rises 40% to 50%, cardiac output increases 30% to 50%, and systemic vascular resistance falls, peaking in the second trimester and at delivery.

Eisenmenger pregnancy mortality

Maternal mortality is approximately 30% to 50%; pregnancy is contraindicated, and women should receive preconception counseling and effective contraception.

Warfarin in pregnancy

Warfarin crosses the placenta and causes embryopathy; in mechanical valve recipients, low-molecular-weight heparin is often used in the first trimester with careful monitoring.

mWHO class IV lesions

Includes pulmonary arterial hypertension, severe systemic ventricular dysfunction, severe aortic stenosis, and Marfan syndrome with aortic root above 4.5 cm; pregnancy is not recommended.

Tetralogy hypercyanotic spell

Knee-chest position, supplemental oxygen, morphine, intravenous fluids, beta-blockade, and phenylephrine increase systemic vascular resistance and reduce right-to-left shunting.

Aortic dissection in ACHD

Sudden chest or back pain in Marfan, Turner, or coarctation patients requires urgent CT angiography and blood pressure control before surgical consultation.

Fontan-associated plastic bronchitis

Airway casts cause severe hypoxemia in Fontan patients; treatment includes inhaled fibrinolytics, steroids, and optimization of Fontan hemodynamics.

Endocarditis in ACHD

Fever with a new regurgitant murmur or embolic phenomena warrants blood cultures and echocardiography; empiric antibiotics should follow collection of cultures.

Tetralogy MRI surveillance interval

Cardiac MRI is typically repeated every three to five years after repair to assess right ventricular dilation and pulmonary regurgitation and to time pulmonary valve replacement.

Fontan liver surveillance

Annual assessment includes liver enzymes, ultrasound, and elastography because Fontan-associated liver disease and hepatocellular carcinoma risk increase with time.

ACHD transition of care

Structured transition from pediatric to adult congenital heart centers during adolescence improves adherence, disease education, and continuity of lifelong surveillance.

Bicuspid aortic valve surveillance

Serial echocardiography monitors valve function and aortic root dimensions; aortic dilation increases dissection risk and may warrant surgical referral.