Facts read from nccwebsite.org · last checked Nov 7, 2026
The heart accounts for 10 of the scored questions. Most start from a cyanotic or shocked newborn, a murmur or a screening result, and ask which lesion explains it or what keeps the baby alive until surgery.
Practice questions
60 questions written to the NNP-BC outline; each choice is explained once you pick.
Cyanotic lesions: Ebstein anomaly, tetralogy of Fallot, truncus arteriosus, transposition, total anomalous pulmonary venous return, tricuspid atresia.
Acyanotic and obstructive lesions: VSD, ASD, coarctation and interrupted arch, hypoplastic left heart, atrioventricular canal, aortic stenosis.
The ductus: patent ductus arteriosus; prostaglandins.
Physiology: heart failure, hypertension and hypotension, shock, dysrhythmias.
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15 neonatal cardiac questions
0 / 15 answered15 questions
Tap an option: why each one is right or wrong appears under it.
Neonatal cardiac care · 10 items
A 25-week infant on day 3 has a hemodynamically significant PDA. Platelets are 38,000/mm³, urine output is 1.5 mL/kg/h, and there is no NEC. If pharmacologic closure is chosen, the preferred agent is
With platelets below 50,000/mm³, NSAIDs are contraindicated because they impair platelet function; acetaminophen acts on the peroxidase site of prostaglandin synthesis without that bleeding risk.
Indomethacin is effective for ductal closure, but its antiplatelet effect makes it a poor choice with a platelet count of 38,000/mm³.
Furosemide eases pulmonary edema from a PDA but does not close the duct, and its prostaglandin effect can even favor patency.
A term newborn has high-output heart failure, bounding carotid pulses, and a continuous bruit over the anterior fontanelle. Echocardiography shows a dilated superior vena cava and a structurally normal heart. The most likely diagnosis is
Hepatic hemangiomas also cause high-output failure, but the bruit is over the liver and the inferior, not superior, vena cava is dilated.
A cranial bruit with high-output failure and a dilated superior vena cava, which carries the shunted return from the head, points to a vein of Galen arteriovenous malformation.
A large PDA can cause bounding pulses and failure, but the echo shows a structurally normal heart and the bruit is over the skull.
A newborn has a broad forehead, periorbital fullness, a long philtrum, a serum calcium of 12.8 mg/dL, and supravalvular aortic stenosis on echocardiogram. The most likely diagnosis is
Alagille syndrome pairs peripheral pulmonary stenosis with cholestasis and butterfly vertebrae, not aortic narrowing and high calcium.
22q11.2 deletion is the near-miss genetic heart syndrome, but it causes low calcium with conotruncal defects, not hypercalcemia with supravalvular stenosis.
Williams syndrome, a 7q11.23 deletion that includes the elastin gene, combines supravalvular aortic stenosis, a distinctive facies and infantile hypercalcemia.
A term infant has a loud holosystolic murmur at the left lower sternal border on day 3. Echocardiography shows a 2-mm muscular VSD, and the infant is feeding well. The most appropriate plan is
Diuretics and digoxin treat the failure of a large VSD; a loud murmur in a well-feeding infant reflects a small, restrictive defect.
Current endocarditis guidance does not recommend prophylaxis for an unrepaired small VSD, a carry-over from older rules.
A small muscular VSD is loud because of the high pressure gradient, causes no heart failure and often closes on its own, so follow-up is all that is needed.
A newborn with esophageal atresia also has a sacral hemivertebra, and the echocardiogram is normal. Which study is most important to complete the VACTERL screen?
VACTERL covers vertebral, anal, cardiac, tracheoesophageal, renal and limb anomalies; with the spine and heart already assessed, a renal ultrasound finds the often silent kidney defects.
VACTERL is a clinical association without a typical karyotype abnormality, so a karyotype does not complete its screen.
An eye exam for coloboma belongs to the CHARGE workup, which overlaps with esophageal atresia but is a different association.
An infant with trisomy 21 has an atrial septal defect in the lower atrial septum with a cleft mitral valve. This defect is
The secundum defect is the most common ASD, which tempts the choice, but it sits in the mid-septum and does not involve the mitral valve.
Failed fusion of the septum primum with the endocardial cushions leaves a low defect with a cleft mitral valve, the partial AV canal typical of trisomy 21.
Sinus venosus defects lie high near the superior vena cava and come with anomalous pulmonary venous return, not a cleft mitral valve.
A term infant passes the CCHD pulse-oximetry screen at 26 hours. Which critical lesion is this result LEAST able to exclude?
Tricuspid atresia forces complete mixing with obligatory desaturation, a pattern pulse oximetry detects well.
Left-sided obstructive lesions such as coarctation often keep normal saturations in hand and foot, so they are the critical lesions most often missed by pulse-oximetry screening.
Pulmonary atresia depends on the ductus for lung blood flow and usually lowers saturations, so the screen tends to catch it.
A neonate with complex cyanotic heart disease has a midline liver, and Howell–Jolly bodies are seen on the blood smear. The most likely diagnosis is
22q11.2 deletion brings conotruncal lesions, hypocalcemia and thymic hypoplasia, but not a midline liver or absent splenic function.
Situs inversus totalis is a complete mirror image with a normal spleen and usually a normal heart, so Howell–Jolly bodies do not fit.
Howell–Jolly bodies mean no functioning spleen; with a midline liver and complex cyanotic disease this is asplenia (right isomerism), which also needs penicillin prophylaxis.
A 2-day-old presents in shock with weak pulses in all extremities, hepatomegaly and a lactate of 8 mmol/L. A ductal-dependent systemic lesion is suspected, but echocardiography is not yet available. The first medication should be
When a duct-dependent systemic lesion presents in shock as the ductus closes, alprostadil must start at once without waiting for echo, with readiness to support the airway for apnea.
Furosemide treats pulmonary congestion but cannot restore systemic blood flow that depends on reopening the ductus.
Adenosine terminates supraventricular tachycardia; it has no role when the problem is loss of ductal flow to the body.
A newborn has a secundum atrial septal defect and bilateral hypoplastic, triphalangeal thumbs. His mother has a repaired ASD and a short thumb. The most likely diagnosis is
Fanconi anemia also causes thumb anomalies, but it is recessive and leads to marrow failure later, not an inherited ASD from a parent.
Holt-Oram is an autosomal dominant TBX5 disorder pairing thumb and radial-ray defects with a secundum ASD or VSD, and an affected parent fits the dominant pattern.
VACTERL can include limb and heart defects, but it is sporadic, so a mother with the same ASD-plus-thumb pattern points elsewhere.
After perinatal asphyxia, a 1-day-old has a new holosystolic murmur at the lower left sternal border, hepatomegaly and an elevated troponin. Echo shows a structurally normal heart with tricuspid regurgitation and poor RV function. The MOST likely diagnosis is
Asphyxia causes transient myocardial ischemia of the subendocardium and papillary muscles, giving tricuspid regurgitation, poor ventricular function and a raised troponin that usually recover over days.
Myocarditis can also raise troponin, but it typically follows a viral illness later in the first weeks and lacks the asphyxia history.
Hypertrophic cardiomyopathy, as in infants of diabetic mothers, would be visible as thickened walls on an echo that is otherwise structurally normal here.
A term neonate with d-TGA and intact ventricular septum remains at SpO2 55% on PGE1; echo shows a small, restrictive foramen ovale. The BEST next step is:
In d-TGA with an intact septum the two circulations run in parallel, and a restrictive atrial septum limits mixing that PGE1 cannot create, so urgent balloon septostomy is needed.
Volume treats hypotension or hypovolemia; it does nothing for the mixing defect that is causing this infant's profound hypoxemia.
iNO lowers pulmonary resistance, which helps PPHN, but it does not open a route for oxygenated blood to cross into the systemic circuit in d-TGA.
Which finding MOST suggests a hemodynamically significant PDA in a 26-week infant on day 4?
Weak pulses with a narrow pulse pressure point to low cardiac output or left-sided obstruction, the opposite of ductal steal.
A large left-to-right ductal shunt runs off blood in diastole, lowering diastolic pressure and giving bounding pulses, a wide pulse pressure and an active precordium.
An apical holosystolic murmur suggests mitral regurgitation; a significant PDA in a preterm infant may even be silent or systolic at the left upper sternal border.
A 26-week infant with a UVC tip over the right atrium suddenly develops hypotension, bradycardia and muffled heart sounds. The radiograph shows a newly enlarged, globular cardiac silhouette. The most appropriate immediate intervention is
A UVC tip in the heart can erode the wall and leak infusate into the pericardium; hypotension, muffled heart sounds and a globular silhouette mean tamponade, relieved only by drainage.
Epinephrine adds contractility, but no inotrope can help a ventricle that is unable to fill, so tamponade persists while time is lost.
A saline bolus can briefly support filling, but it cannot overcome a heart compressed by pericardial fluid and delays the definitive step.
A running total sits here; it fills in after your last pick.
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Heart questions that catch candidates
The CCHD screen by the 2011 rule. AAP's 2025 clinical report on pulse oximetry screening tests the right hand and one foot at 24 hours or later. A pass is 95% or more in both with a gap of 3 points or less; under 90% anywhere fails; anything else gets one repeat, and a result that still does not pass is a failed screen that needs an echocardiogram. The older algorithm accepted 95% in either limb and allowed two repeats.
Waiting for the echo. In a newborn in shock or deep cyanosis with a suspected duct-dependent lesion, prostaglandin starts before the anatomy is confirmed; apnea is a known effect, so be ready to support the airway.
Expecting prostaglandin to fix everything. Obstructed total anomalous pulmonary venous return does not improve with it and needs surgery. The film, with a small heart and congested lungs, is easily read as lung disease.
Treating every PDA in the most preterm. In the BeNeDuctus trial (NEJM, 2023), expectant management in infants born before 28 weeks was noninferior to early ibuprofen, so drug closure is no longer the reflex answer.
The hyperoxia test misread. A PaO2 that climbs well in 100% oxygen argues for a lung cause; a PaO2 that stays low suggests intracardiac mixing. Sample from the right radial artery, which is pre-ductal.