Acquired and Systemic Heart Diseases
M. Yasir Qureshi
Emily R. Levy
1. A 3-month-old female presented with 5 days of fever, bilateral conjunctivitis, maculopapular rash, cracked lips, and cervical lymphadenopathy. A diagnosis of Kawasaki disease (KD) was established. The initial echocardiogram showed diffuse coronary artery ectasia. The proximal right coronary artery dimension Z-score was 3.4, left main coronary artery Z-score was 3.0, and the proximal left anterior descending artery Z-score was 3.8. One dose of intravenous immunoglobulins was administered resulting in complete resolution of the clinical features of KD within 12 hours of treatment. High-dose aspirin was continued for 48 hours after the alleviation of clinical features, and low-dose aspirin was started after that. Which of the following is the next best step of management?
A. Repeat echocardiogram twice a week until the coronary dimensions have stabilized or normalized
B. Repeat IVIG due to significant coronary artery ectasia
C. Start oral steroids to prevent coronary aneurysms
D. Repeat echocardiogram in 1 to 2 weeks
E. Repeat echocardiogram in 4 to 6 weeks
1. (A) KD guidelines of 2017 recommend repeating echocardiograms twice a week in patients with important or evolving coronary artery abnormalities (Z-score >2.5) noted during the acute phase of KD, until the coronary dimensions have stopped progressing. As the clinical features of inflammation have resolved with the 1st dose of IVIG, a repeat dose of IVIG or oral steroids is not needed.
2. A 16-year-old female is recently diagnosed with systemic lupus erythematosus. Her initial echocardiogram as part of her workup was completely normal. While counseling the patient about cardiac involvement in systemic lupus erythematosus, pericarditis and valvulitis were discussed. Which of the following valves is most likely to be involved in valvulitis associated with systemic lupus erythematosus?
A. Mitral valve
B. Aortic valve
C. Tricuspid valve
D. Pulmonary valve
E. Eustachian valve
2. (A) The mitral valve is the most commonly involved cardiac valve in systemic lupus erythematosus. The aortic valve is the 2nd most common, followed by the tricuspid valve and finally the pulmonary valve.
3. A 32-year-old gravida 1 para 0 female presented for a fetal echocardiogram at 19 weeks of gestation. She has a history of Sjogren syndrome with positive SSA/SSB antibodies. The fetal echocardiogram showed normal sinus rhythm, normal biventricular function and no pericardial effusion. What is the risk of congenital complete atrioventricular block in this fetus?
A. No risk as the fetal echocardiogram is normal
B. 1% to 5% risk
C. 10% to 20% risk
D. 50% risk
E. 80% risk
3. (B) The risk in the 1st child in mothers with positive SSA/SSB antibodies is 1% to 5%. If the 1st child has congenital complete heart block, the risk in 2nd child goes up to 11% to 19%. Complete heart block can manifest any time between 16 and 28 weeks of gestation; therefore, a normal fetal echocardiogram at 19 weeks of gestation does not alleviate the risk.
4. A 17-year-old female presents with a 1-year history of intermittent fevers, loss of appetite, and weight loss. On examination, she was noted to be hypertensive in her arms and had diminished femoral pulses with significant radiofemoral delay. An echocardiogram showed left ventricular hypertrophy with normal aortic valve function and a normal aortic arch. A magnetic resonance (MR) angiogram was obtained and is shown in Figure 12.1.
The most likely diagnosis in this patient is:
A. Systemic hypertension
B. Coarctation of aorta
C. Systemic lupus erythematosus
D. Takayasu arteritis
E. Infective endocarditis
5. A 4-month-old full-term male neonate was noted to be hypotonic on examination. Pulse oximetry showed an oxygen saturation of 99% in both the arms and legs. A chest x-ray showed significant cardiomegaly. A 12-lead electrocardiogram showed a short PR interval and biventricular hypertrophy. An echocardiogram was ordered and showed biventricular hypertrophy without any outflow tract obstruction. Which of the following therapies is most likely to benefit this infant?
B. Beta blocker
C. Implantable cardioverter-defibrillator
D. Surgical sympathectomy
E. Enzyme replacement therapy
5. (E) The clinical picture is consistent with Pompe disease, which is a lysosomal storage disease due to acid alphaglucosidase deficiency. Enzyme replacement therapy with alglucosidase alfa given intravenously every 2 weeks is the most effective treatment for cardiomyopathy and developmental delays.
6. A 15 year old with sickle cell anemia and a history of multiple blood transfusions is being evaluated for possible hemosiderosis. Which of the following is the best test to assess for hemosiderosis?
A. Transthoracic echocardiogram with tissue Doppler imaging
B. Cardiac catheterization and endomyocardial biopsy
C. Cardiac and liver MRI with T2* quantification
D. Magnetic resonance elastography of the liver
E. Liver biopsy
6. (C) Magnetic resonance imaging with T2* quantification is the best test to quantitate iron load in myocardium and the liver. Echocardiography and tissue Doppler may show restrictive pattern in hemosiderosis. Cardiac catheterization, liver magnetic resonance elastography, and liver biopsy are not needed for this purpose.
7. A 10 year old was diagnosed with Lyme disease 4 months ago. Which of the following is a clinical feature of late Lyme disease?
A. Erythema migrans
B. Large joint arthritis
C. Facial palsy
D. First-degree heart block
7. (B) Large joint arthritis is a feature of late Lyme disease. All other options are features of early localized and early disseminated Lyme disease.
8. A 7-year-old male presents with 4 days of fever, 3 weeks after being treated for acute pharyngitis with oral amoxicillin. He also reports right knee and right elbow swelling, erythema, and pain. On examination, tachypnea and increased work of breathing is noted. Cardiac examination revealed a 3/6 holosystolic murmur at the cardiac apex radiating to the left axilla. Chest x-ray showed mild cardiomegaly with increased pulmonary vascular markings and hazy lung fields. Laboratory evaluation showed elevated C-reactive protein and serum streptococcal antibody titers. The transthoracic echocardiogram is shown in Figure 12.2.
Which of the following options is the best treatment strategy for this patient?
A. Intramuscular penicillin G benzathine, naproxen, and furosemide
B. Intravenous immunoglobulin (IVIG) and high-dose aspirin
C. IVIG and low-dose aspirin
D. IVIG, prednisone, and high-dose aspirin
E. IVIG, amoxicillin, and aspirin
8. (A) The patient has acute rheumatic fever with carditis. The echocardiogram shows severe mitral valve regurgitation, which is the most common manifestation of acute rheumatic carditis. Left heart enlargement and a small amount of pericardial effusion along the right atrium and right ventricle can also be noted. The treatment includes (1) antibiotics against group A streptococcal infection, (2) anti-inflammatory therapy with NSAIDs or high-dose aspirin, and (3) treatment of congestive heart failure. Penicillin G benzathine is a long-acting intramuscular injection that serves as prevention of group A streptococcal infection. Naproxen has favorable side effect profile compared to high-dose aspirin and is a preferred choice as an anti-inflammatory agent. Furosemide is needed in this child as there is evidence of respiratory distress and pulmonary edema from mitral regurgitation. IVIG and high-dose aspirin are first-line treatment for Kawasaki disease. IVIG is typically not used in acute rheumatic fever unless there is severe Sydenham chorea which is not responding to other therapy.
9. Which of the following is the most common presentation of chronic rheumatic heart disease?
A. Mitral valve regurgitation
B. Mitral valve stenosis
C. Aortic valve regurgitation
D. Aortic valve stenosis
E. Tricuspid valve disease
9. (B) Mitral valve stenosis in isolation and in combination with aortic valve disease is the most common manifestation of chronic rheumatic heart disease.
10. A 9-month-old infant is being seen in pediatric cardiology clinic for routine follow-up. He had a history of a large membranous ventricular septal defect for which he underwent surgical repair at 4 months of age. The postoperative course was complicated by junctional tachycardia which required treatment with amiodarone. One month after discontinuation of amiodarone, he had another episode of junctional tachycardia for which oral amiodarone was restarted. The infant has gaining weight but has severe constipation. The mother also reports some facial puffiness and dry skin. Some initial laboratory tests were ordered by the referring pediatrician prior to this visit. Which of the following laboratory findings can be expected in this infant?
A. Low thyroid-stimulating hormone (TSH) and low free T4
B. Normal TSH and low free T4
C. Low TSH and high free T4
D. High TSH and low free T4
E. High TSH and high free T4
11. A 17-year-old male is referred to pediatric cardiology for evaluation of claudication. His physical examination revealed decreased femoral pulses. A transthoracic echocardiogram showed a diffusely narrowed descending thoracic and abdominal aorta. Which of the following is associated with vasculopathy leading to stenosis of large vessels?
A. Late syphilis
B. Human immunodeficiency virus (HIV) disease
C. Kawasaki disease
D. Henoch-Schönlein purpura
E. Rheumatic fever
11. (B) HIV disease can cause vasculitis of the aorta and pulmonary arteries that can lead to diffuse stenosis. Late syphilis causes occlusion of vasa vasorum of large vessels resulting in aneurysmal dilation, not stenosis, of aorta. Other options are not associated with large vessel vasculopathy.
12. An asymptomatic 22-year-old cancer survivor of thoracic lymphoma was previously treated with chemotherapy and radiation therapy. A surveillance echocardiogram showed calcification and thickening of the mitral valve annulus. This pattern of valvular involvement is typical of which of the following?
A. Anthracycline exposure
B. Radiation exposure
C. Healed infective endocarditis
D. Nonbacterial thrombotic endocarditis
E. Rheumatic carditis
12. (B) Thoracic radiation exposure can lead to a degenerative process of the mitral valve which typically starts from the annulus and progresses toward the leaflet tips. Anthracycline exposure is more commonly associated with cardiomyopathy. Infective and nonbacterial thrombotic endocarditis typically do not involve the valve annulus primarily.
13. A 15-year-old female was diagnosed with systemic lupus erythematosus 2 years ago. She is completely asymptomatic from a cardiovascular standpoint. Her lupus is well controlled. Her echocardiogram shows vegetations on the mitral valve leaflets. Which of the following is true about these vegetations?
A. Benign vegetations, no treatment needed
B. Can get infected, prophylactic antibiotics are needed
C. Need treatment with intravenous antibiotics
D. Can embolize
E. Need surgical resection
13. (D) Nonbacterial thrombotic endocarditis in systemic lupus erythematosus is not infective and does not need antibiotic prophylaxis. However, these vegetations can embolize and are not completely inconsequential. Rarely, surgical resection is needed for large vegetations.
14. An 8-year-old is admitted to the hospital with abdominal pain, vomiting, fevers >102°F for 7 days, cracked red lips, and swollen hands and feet. The emergency room has obtained a CBC with differential and ESR. The absolute lymphocyte count (ALC) is noted to be 550 cells/uL (0.55). CRP is elevated at 102 mg/L. The family mentions that they all were mildly ill with cough, congestion, and pharyngitis approximately 1 month prior. The patient’s mother had a “COVID19” test at the time which was a positive SARS-CoV-2 PCR. You suspect this child may have multisystem inflammatory syndrome in children (MIS-C). You plan to perform an echocardiogram. If there are no coronary artery aneurysms, the appropriate first-line therapy could include:
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