1. Which of the following would best describe a mitral arcade?
A. Thickened mitral valve leaflets
B. Absent/abnormal chordal insertions
C. Absent papillary muscles
D. Fused papillary muscles
E. Decreased interpapillary muscle distance
2. A neonate is diagnosed with truncus arteriosus. Which of the following are you most likely to find regarding the morphology of the truncal valve?
A. Unicuspid
B. Bicuspid
C. Tricuspid
D. Quadricuspid
E. Pentacuspid
3. A 12-year-old male is diagnosed with mild aortic stenosis. You suspect that he has an abnormal aortic valve. Which of the following is the most likely aortic leaflet pattern present in this patient?
A. Bicuspid valve with fusion of the right and noncoronary cusps
B. Bicuspid valve with fusion of the right and left cusps
C. Unicuspid valve with fusion of more than one cusp
D. Quadricuspid valve with a cleft of the left coronary cusp
E. Bicuspid valve with fusion of the left and noncoronary cusps
4. Which of the following is true regarding the atrioventricular (AV) node?
A. AV nodal artery is most commonly a branch of the left coronary artery.
B. AV node is positioned within triangle of Koch.
C. AV node is posterior to coronary sinus.
D. AV node is anterior to membranous septum.
E. AV node is a subepicardial structure.
5. Which coronary artery typically supplies the posteromedial papillary muscle of the mitral valve?
A. Left circumflex
B. Left anterior descending
C. Obtuse marginal
D. Right coronary
E. Conal branch
6. Which of the following is true about a straddling cardiac valve?
A. Cannot coexist with overriding
B. Is not associated with malalignment type of ventricular septal defect (VSD)
C. Is most commonly involving the pulmonary valve
D. Is a common component of tetralogy of Fallot (TOF)
E. Involves anomalous insertion of chordae tendineae
7. A 28-year-old woman develops severe rubella infection. The fetus is at increased risk for which congenital heart disease (CHD)?
A. Complex heterotaxy
B. Ebstein’s anomaly
C. Coarctation of the aorta
D. d-Transposition of the great arteries (d-TGA)
E. Valvar and supravalvular pulmonic stenosis
8. You are asked to evaluate a 2-year-old boy for a cardiac murmur. You note that the child, for a 2-year old, is quite friendly, has stellate irises, a long philtrum, depressed nasal bridge, prominent lower lip, and enamel hypoplasia. There is a 3/6 systolic ejection murmur and no click. Which of the following deletions is most likely in this patient?
A. 18q
B. 22p
C. 8p23
D. 7q11.23
E. 22q11
9. Which of the following papillary muscle arrangements are seen most commonly with complete AV canal defect?
A. The papillary muscles are closer together, the anterior muscle is closer to the septum than normal, and the posterior muscle is farther from the septum than normal.
B. The papillary muscles are closer together, the anterior muscle is farther from the septum than normal, and the posterior muscle is closer to the septum than normal.
C. The papillary muscles are closer together and positioned clockwise from their normal location.
D. The papillary muscles are farther apart, the anterior muscle is closer to the septum than normal, and the posterior muscle is farther from the septum than normal.
E. The papillary muscles are farther apart, the anterior muscle is farther from the septum than normal, and the posterior muscle is closer to the septum than normal.
10. The postoperative hypertension after coarctation repair in the first few hours is best characterized by which of the following?
A. Increased diastolic pressure
B. Mesenteric arteritis
C. Elevation in norepinephrine
D. Elevation in renin
E. Response to therapy with angiotensin-converting enzyme (ACE)an inhibition
11. A 3-year-old patient with single ventricle returns from the operating room after a Fontan operation. The pulmonary artery (PA) pressure is 18 mm Hg and the left atrial (LA) pressure is 13 mm Hg. Which of the following is the least likely etiology for this hemodynamic profile?
A. Ventricular dysfunction
B. AV valve regurgitation
C. Pleural effusion
D. Pericardial effusion
E. Unfavorable mass/volume change
12. What is the most appropriate antimicrobial regimen to treat a 5-year-old patient with rheumatic fever if the patient has a penicillin allergy (rash)?
A. Amoxicillin for 10 days
B. Cephalexin for 10 days
C. Azithromycin for 10 days
D. Amoxicillin for 5 days
E. Clindamycin for 5 days
13 In a patient with a reported syncopal episode, which of the following features would prompt hospitalization or intense outpatient workup?
A. Syncope with exertion
B. Syncope after rising from laying to standing
C. Family history of bicuspid aortic valve
D. Previous near syncopal episode
E. Loss of bladder control during syncopal episode
14. Which statement is true regarding blood pressure (BP) measurement techniques?
A. The oscillometric method is more susceptible to external noise, but less susceptible to motion artifact.
B. Systolic blood pressure (SBP) and diastolic blood pressure (DBP) corresponds to the first and third Korotkoff sounds.
C. The current “gold standard” for clinical measurement of BP is an appropriately calibrated aneroid sphygmomanometer.
D. When using the oscillometric method, the point of maximal oscillation corresponds to the mean pressure.
15. In which of the following scenarios is it most appropriate to use the simplified Bernoulli equation to estimate a change in pressure?
A. Severe pulmonary valve stenosis
B. Aortic coarctation with bicuspid aortic valve
C. Patent ductus arteriosus (PDA)
D. Subaortic stenosis and aortic stenosis
E. Blalock–Taussig shunt
16. A single sinoatrial (SA) node in a normal position is typically found in which of the following?
A. Left juxtaposition of the atrial appendages
B. Right juxtaposition of the atrial appendages
C. Right atrial isomerism
D. LA isomerism
E. Situs inversus of the atria
17. Which of the following events is responsible for early, functional closure of the PDA?
A. Hemorrhage and necrosis in the subintimal region
B. Medial smooth muscle cell migration into the wall of the ductus
C. Equalization of pulmonary and systemic vascular resistance
D. Infolding of the endothelium
E. Thinning of the intimal layer
18. A 7-year-old well child with a recent history of palpitations. Electrocardiogram (ECG) shows an irregularly irregular wide complex tachycardia with variable ventricular rates, No P waves are discernible on the ECG. Vagal maneuvers have failed. His BP is 100/60 mm Hg. The patient has been cardioverted three times, with transient return to sinus rhythm, but the tachycardia recurs. Which of the following medications would be most likely to treat this patient’s arrhythmia?
A. IV (intravenous) adenosine
B. IV digitalis
C. IV amiodarone
D. IV β-blocker
E. IV diltiazem
19 A 13-year-old boy with a history of catecholaminergic polymorphic ventricular tachycardia (CPVT) is admitted to the intensive care unit (ICU) after an episode of syncope with exertion. In the ICU, he is noted to have frequent episodes of polymorphic ventricular tachycardia associated with hypotension. Which of the following is the best antiarrhythmic therapy for this child?
A. Amiodarone
B. Lidocaine
C.β-blocker
D. Calcium channel blocker (CCB)
E. Digoxin
20 A newborn baby is noted to be bradycardic with a heart rate (HR) of 40 bpm. An ECG shows complete AV block. Which of the following medications would be most useful to increase the HR in this scenario?
A. Milrinone infusion
B. Atropine infusion
C. Digoxin infusion
D. Isoproterenol infusion
E. Dobutamine infusion
ANSWERS
1. B. The mitral arcade, or hammock mitral valve, is characterized by absent or abnormal chordal insertions, and the leaflet edges may connect directly to the papillary muscles. The papillary muscles themselves are often small and abnormal. The leaflet edges are often thickened and rolled. Owing to this direct insertion of the leaflets to the papillary muscles, the leaflets are relatively tethered and display poor coaptation. Mitral regurgitation is most common, although a functional mitral stenosis can also occur.
2. C. The truncal valve in truncus arteriosus is most commonly tricuspid (~70%), but can also be quadricuspid (~20%), bicuspid (~10%), pentacuspid (<1%), or unicommissural (<1%). The valve is in fibrous continuity with the mitral valve in all patients, but can also rarely be in fibrous continuity with the tricuspid valve.
3. B. Of patients with a bicuspid aortic valve, by far the most common form is fusion of the right and left cusps (75%). The next most common pattern are patients with fusion of the right and noncoronary cusps, followed by those with left and noncoronary cusp fusion.
4. B. The AV node is located in the subendocardium within the triangle of Koch. It is adjacent to the central fibrous trigone (the central fibrous body). The borders of the triangle of Koch include the septal tricuspid annulus, the coronary sinus ostium, and tendon of Todaro. The AV node gives rise to the bundle of His, which then travels through the central fibrous body.
5. D. While the anterolateral papillary muscle typically has a dual blood supply from the left anterior descending and circumflex coronary arteries, the posteromedial papillary muscle is typically solely supplied by the right coronary artery (RCA).
6. E. The definition of a straddling AV valve is one that involves anomalous insertion of the chordae tendineae. It is important to identify preoperatively as it may prevent the surgeon from attempting certain repairs. There has to be a VSD, but it may or may not be a malalignment-type VSD.
7. E. Heart defects in congenital rubella syndrome include pulmonic stenosis (valvar, supravalvular, or peripheral) and PDA. TOF has also been reported.
8. D. This vignette describes Williams syndrome, which is characterized in part by CHDs, hypercalcemia in infancy, skeletal and renal anomalies, cognitive deficits, social personality, and so-called “elfin facies.” Approximately 90% of patients with the clinical diagnosis of Williams syndrome have a deletion at chromosome 7q11.23, which is not generally apparent on a routine karyotype but can be detected by fluorescence in situ hybridization (FISH). Approximately 55–80% of patients with Williams syndrome have CHDs, which typically include supravalvular aortic stenosis and/or supravalvular pulmonary stenosis.
9. A. The common AV valve has five leaflets. Beneath the five commissures are five papillary muscles. The two left-sided papillary muscles are oriented closer together than in a normal heart, and the lateral leaflet is smaller than usual. In addition, the two papillary muscles are often rotated counterclockwise, thus positioning the posterior muscle farther from the septum than normal and the anterior muscle closer to the septum. This papillary muscle arrangement, along with a large anterolateral muscle bundle, can contribute to progressive LVOT obstruction
10. C. Post-coarctectomy hypertension has a biphasic pattern. The first phase is most likely secondary to surgical stimulation of the sympathetic nerve fibers in the tissue of the aortic isthmus. This process leads to release of norepinephrine (as well as epinephrine) and can be effectively neutralized with preoperative treatment with propranolol. The second phase of post-coarctectomy hypertension occurs after the initial 24 hours and is characterized by an increase in renin and an elevated diastolic pressure. This phase is also associated with mesenteric arteritis.
11. C. Elevated PA pressure after the Fontan operation can be categorized into (a) abnormal transpulmonary gradient (>5 mm Hg): etiologies include elevated pulmonary vascular resistance, pulmonary artery distortion, pulmonary issues (e.g., pleural effusion, pneumothorax, atelectasis), and pulmonary venous obstruction; and (b) normal transpulmonary gradient (but abnormal LA pressure): etiologies include ventricular dysfunction, unfavorable mass/volume changes (diastolic dysfunction), AV valve regurgitation, subaortic stenosis, AV dyssynchrony, and pericardial effusion.
12. B. In a patient with penicillin allergy, amoxicillin should also be avoided. A narrow-spectrum cephalosporin, such as cephalexin, for 10 days would be appropriate. In patients with a severe hypersensitivity reaction to penicillin, other choices include azithromycin, but a 5-day course is recommended, and clindamycin for a 10-day course.
13. A. Refer to guidelines for the diagnosis and management of syncope.
14. E. The point of maximal oscillation correlates with the mean intraarterial BP. This technique has the advantage of being less susceptible to external noise, but more susceptible to movement and low frequency vibration. This method is often used in ambulatory BP monitoring devices and many home BP monitors.
15. A. The simplified Bernoulli equation ignores the components of flow acceleration and viscous friction. Doppler velocities across a PDA or Blalock–Taussig shunt will likely be underestimated due to viscous friction in these tortuous connections and difficulties with proper ultrasound beam alignment. Multiple obstructions in series, such as multiple sites of LVOT obstruction (subvalvular, valvar, coarctation), will need to account for flow acceleration proximal to the distal site(s) of obstruction. Isolated valvar stenoses would be an appropriate use of the simplified Bernoulli equation.
16. B. In patients with right atrial isomerism, bilateral sinus nodes can be encountered. In LA isomerism, the sinus node can be absent or malpositioned. In left-sided juxtaposition of the atrial appendages, the sinus node is often displaced anteriorly or inferiorly. Left-juxtaposition is associated more with abnormal ventriculo-arterial connections, while right-sided juxtaposition is more commonly associated with simpler lesions, like atrial septal defects (ASDs).
17. B. Immediately after birth, in the first stage of closure, contraction and migration of medial smooth muscle cells into the wall of the PDA produce increased wall thickness and luminal protrusion of the thickened intima. This results in functional closure of the PDA and occurs within 12 hours of birth in normal full-term infants. The second stage of closure includes infolding of the endothelium, disruption of the elastic lamina, and necrosis of the subintima. There is eventual replacement of muscle and endothelium with fibrotic tissue, producing the ligamentum arteriosum.
18. C. Atrial fibrillation with pre-excitation is the diagnosis. This is the most likely rhythm with an irregularly irregular wide complex tachycardia in an otherwise healthy patient. In atrial fibrillation with pre-excitation, the patient would be conducting antegrade to the ventricle through both AV node and accessory pathway, and some beats are likely to be fusion beats. Any AV nodal blocking agent (adenosine, digitalis, diltiazem, β-blocker) is likely to result in unopposed ventricular activation through accessory pathway and can result in ventricular fibrillation. Thus, AV nodal blocking agents are best avoided.
19. C. In CPVT patients with ventricular tachycardia (VT)/ventricular fibrillation (VT/VF) storm, IV β-blocker therapy is considered to be the first line of treatment. General anesthesia can be used as a last resort if β-blocker therapy is ineffective.
20. D. Isoproterenol stimulates myocardial β-1 receptors resulting in positive chronotropy and inotropy. It can result in the generation of a stable junctional/ventricular escape rhythm that is helpful in this setting allowing for additional time to pursue interventions like temporary pacemaker if necessary. Atropine is anticholinergic/vagolytic agent and only works in reversing AV block to excessive vagal effect. It would not be helpful in this situation. Milrinone and dobutamine do not have the same effect as isoproterenol and therefore are not indicated. Digoxin may slow the junctional rate and therefore is not indicated.
