Cardiology Practice Questions

Q: A 3-year-old boy is brought by his mother because he has had a high fever for the past week. Physical examination reveals bilateral injected conjunctivae, palmar erythema, oral mucositis, cervical lymphadenopathy, and solar erythema. What is the most likely diagnosis?

Kawasaki disease. ***Kawasaki disease*** - The constellation of **prolonged fever**, **bilateral conjunctival injection**, **oral mucositis** (strawberry tongue, cracked lips), **palmar erythema**, **cervical lymphadenopathy**, and a polymorphous rash (including solar erythema) are classic diagnostic criteria for **Kawasaki disease**. - This condition is an acute **vasculitis** of childhood that can lead to serious complications like **coronary artery aneurysms** if untreated. *Erysipelas* - This is a **superficial cellulitis** characterized by a well-demarcated, raised, red, and warm rash, typically on the face or lower extremities. - It does not present with bilateral conjunctival injection, oral mucositis, or systemic features seen in the patient, beyond fever. *Scarlet fever* - Caused by **Streptococcus pyogenes**, it presents with a characteristic **sandpaper-like rash**, **strawberry tongue**, and **pharyngitis**. - While it can cause fever and a strawberry tongue, it typically does not cause conjunctivitis, striking palmar erythema, or diffuse lymphadenopathy in the pattern observed. *Meningococcemia* - This is a severe systemic infection caused by **Neisseria meningitidis**, often presenting with features of **sepsis** and a **petechial or purpuric rash**. - While it causes high fever and can be rapidly progressive, it lacks the specific mucocutaneous findings like conjunctivitis, oral mucositis, and acral erythema that are characteristic of Kawasaki disease.

Q: In which condition is PGE most commonly used to maintain patent ductus arteriosus in an infant?

Hypoplastic left heart syndrome. ***Hypoplastic left heart syndrome*** - **HLHS** is the most common indication for PGE1 infusion to maintain a patent ductus arteriosus in neonates - In HLHS, the left ventricle and aorta are severely underdeveloped, making the **entire systemic circulation ductus-dependent** - The PDA allows right ventricular output to supply systemic blood flow by shunting blood from the pulmonary artery to the descending aorta - Without PGE1 to maintain ductal patency, these infants develop **severe circulatory shock and acidosis** as the ductus closes naturally - This is a **universally accepted life-saving indication** for PGE1 until surgical intervention (Norwood procedure or transplant) *TGA with intact ventricular septum* - While TGA with intact septum does require mixing of circulations and PGE1 is used, these infants typically have a patent foramen ovale (PFO) that provides some mixing at the atrial level - PGE1 helps maintain the PDA for additional mixing, but the primary issue is **circulatory separation**, not complete dependence on the ductus for systemic flow - Many centers perform urgent balloon atrial septostomy rather than relying solely on PGE1 *Pulmonary stenosis* - **Critical pulmonary stenosis** represents ductus-dependent pulmonary circulation - PGE1 maintains the PDA to provide pulmonary blood flow when right ventricular outflow is severely obstructed - While important, it is less commonly encountered than HLHS as the primary indication for PGE1 therapy *Tricuspid atresia* - In tricuspid atresia, there is no communication between the right atrium and right ventricle - Pulmonary blood flow is ductus-dependent if there is no VSD or if the VSD is restrictive - PGE1 may be required, but this is less common than HLHS overall

Q: Treatment of choice for Kawasaki disease is:

IV Immunoglobulins. ***IV Immunoglobulins*** - **Intravenous immunoglobulins (IVIG)** at a dose of **2 g/kg as a single infusion** are the cornerstone of therapy for Kawasaki disease, significantly reducing the risk of **coronary artery aneurysms** from ~25% to ~5%. - IVIG should be administered along with **high-dose aspirin (80-100 mg/kg/day)** within the first 10 days of fever onset for optimal effectiveness. - This combination therapy is the **gold standard treatment** for acute Kawasaki disease. *Corticosteroids* - While corticosteroids have anti-inflammatory properties, they are generally **not the primary treatment of choice** for acute Kawasaki disease. - They may be considered as **adjunctive therapy in IVIG-resistant cases** (persistent or recurrent fever ≥36 hours after initial IVIG) or in severe disease, but their routine use is debated. *Azathioprine* - **Azathioprine is an immunosuppressant** typically used in autoimmune diseases or organ transplantation. - It is **not indicated** for the acute management of Kawasaki disease. *Methotrexate* - **Methotrexate is an immunosuppressant and anti-inflammatory drug** used in conditions like rheumatoid arthritis and certain cancers. - It is **not used** for the acute treatment of Kawasaki disease.

Q: What will be the likely cause of death in a 4-year-old boy who tires easily, exhibits weakness in the pelvic and shoulder girdles, and calf muscle enlargement, with elevated serum creatine kinase levels, and a biopsy showing marked variation in muscle fiber size and shape, muscle fiber necrosis, myophagocytosis, regenerating fibers, and fibrosis?

Respiratory failure/complications. ***Cardiomyopathy*** - In boys with **Duchenne muscular dystrophy (DMD)**, cardiomyopathy is a significant complication leading to **heart failure** and death. - The muscle biopsy findings support **muscular dystrophy** [1], and the patient's symptoms indicate weakening of the heart muscle over time. *End-stage renal disease* - Typically results from **chronic kidney conditions**, which are not indicated by the case presented. - The symptoms described, including **muscle weakness** and **fibrosis**, are not directly related to renal dysfunction. *Cerebrovascular disease* - Generally manifests as a sudden neurological deficit and is uncommon in **pediatric muscular conditions**. - There is no indication of **neurological symptoms** in this patient's presentation. *Pulmonary saddle embolism* - This condition typically presents with sudden **shortness of breath** or **chest pain**, neither of which is mentioned here. - The findings focus more on **muscle degeneration** rather than any acute pulmonary events. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Peripheral Nerves and Skeletal Muscles, pp. 1244-1245.

Q: Infants of diabetic mothers are likely to have the following cardiac anomaly:

Transposition of great arteries. ***Transposition of great arteries*** - **Transposition of the great arteries (TGA)** is one of the most common cardiac anomalies in infants of diabetic mothers. - In TGA, the **aorta arises from the right ventricle** and the **pulmonary artery arises from the left ventricle**, resulting in two parallel circulations. - Infants of diabetic mothers have a **3-5 fold increased risk** of congenital heart defects, with TGA being among the most frequently observed. - Other common cardiac abnormalities in IDMs include **ventricular septal defect (VSD)** and **hypertrophic cardiomyopathy**. *Coarctation of aorta* - While coarctation can occur in infants of diabetic mothers, it is **less specifically associated** with maternal diabetes compared to TGA. - Coarctation involves **narrowing of the aorta**, typically near the ductus arteriosus. - More commonly associated with **Turner syndrome** and **bicuspid aortic valve**. *Fallot's tetralogy* - **Tetralogy of Fallot** is not specifically increased in incidence in infants of diabetic mothers. - It is characterized by four components: **pulmonary stenosis**, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. - Not a characteristic anomaly of maternal diabetes. *Ebstein's anomaly* - This anomaly involves the **tricuspid valve** being displaced into the right ventricle. - **Not associated with maternal diabetes** - instead, it is associated with maternal **lithium use** during pregnancy. - Presents with tricuspid regurgitation and right heart dysfunction.

Q: The newborn heart rate is about what?

120 - 160 /min. ***120 - 160 /min*** - A healthy newborn's heart rate typically ranges between **120 and 160 beats per minute** while awake and calm. - This elevated rate is necessary to meet the **metabolic demands** of rapid growth and development. *160 - 180 /min* - While a newborn's heart rate can temporarily increase into this range during periods of **crying, agitation, or fever**, it is not the typical resting rate. - A persistent heart rate in this range without apparent cause could indicate **tachycardia** or other underlying issues. *180 - 200 /min* - This range is generally considered **tachycardic** for a newborn and warrants further investigation for potential medical conditions such as **supraventricular tachycardia (SVT)**, fever, or infection. - It is not a normal physiological heart rate for a healthy, resting newborn. *200 - 220 /min* - A heart rate consistently within this very high range is indicative of **severe tachycardia** and is a medical emergency in a newborn. - It suggests significant stress, illness, or a cardiac arrhythmia requiring immediate medical attention.

Question 1

Which of the following is a characteristic feature of Barth syndrome?

Accepted Answer

Dilated cardiomyopathy + skeletal myopathy + congenital neutropenia

Answer

Cardiac tamponade + skeletal myopathy + neutropenia

Answer

Restrictive cardiomyopathy + skeletal myopathy + neutropenia

Answer

Restrictive cardiomyopathy + skeletal myopathy + lymphopenia

Question 2

A 3-year-old boy is brought by his mother because he has had a high fever for the past week. Physical examination reveals bilateral injected conjunctivae, palmar erythema, oral mucositis, cervical lymphadenopathy, and solar erythema. What is the most likely diagnosis?

Accepted Answer

Kawasaki disease

Answer

Erysipelas

Answer

Scarlet fever

Answer

Meningococcemia

Question 3

In which condition is PGE most commonly used to maintain patent ductus arteriosus in an infant?

Accepted Answer

Hypoplastic left heart syndrome

Answer

TGA with intact ventricular septum

Answer

Pulmonary stenosis

Answer

Tricuspid atresia

Question 4

Treatment of choice for Kawasaki disease is:

Accepted Answer

IV Immunoglobulins

Answer

Methotrexate

Answer

Corticosteroids

Answer

Azathioprine

Question 5

What will be the likely cause of death in a 4-year-old boy who tires easily, exhibits weakness in the pelvic and shoulder girdles, and calf muscle enlargement, with elevated serum creatine kinase levels, and a biopsy showing marked variation in muscle fiber size and shape, muscle fiber necrosis, myophagocytosis, regenerating fibers, and fibrosis?

Accepted Answer

Respiratory failure/complications

Answer

Cerebrovascular complications

Answer

Chronic kidney disease

Answer

Pulmonary embolism

Question 6

Infants of diabetic mothers are likely to have the following cardiac anomaly:

Accepted Answer

Transposition of great arteries

Answer

Coarctation of aorta

Answer

Fallot's tetralogy

Answer

Ebstein's anomaly

Question 7

The newborn heart rate is about what?

Accepted Answer

120 - 160 /min

Answer

160 - 180 /min

Answer

180 - 200 /min

Answer

200 - 220 /min

Cardiology — MCQs

Cardiology — MCQs

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