Cardiology Practice Questions

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: Which one of the following conditions is associated with Kawasaki's syndrome?

Coronary artery aneurysm. ***Coronary artery aneurysm*** - **Coronary artery aneurysms** are a major and potentially life-threatening complication, occurring in 15-25% of untreated children with Kawasaki disease [1]. - This is due to the disease's predilection for causing **vasculitis** in medium-sized arteries, particularly the coronary arteries [1]. *Acute rheumatic fever* - Acute rheumatic fever is an **inflammatory disease** that can develop after an infection with Group A Streptococcus (strep throat or scarlet fever) [2]. - It primarily affects the **heart, joints, brain, and skin**, but its pathophysiology and clinical presentation are distinct from Kawasaki disease [2]. *Pulmonary embolus* - A **pulmonary embolus** is a block in one of the pulmonary arteries in your lungs, usually caused by blood clots that travel to the lungs from the legs or, rarely, other parts of the body. - It is not a characteristic feature or complication of **Kawasaki disease**. *Systemic lupus erythematosus* - **Systemic lupus erythematosus (SLE)** is a chronic autoimmune disease that can affect almost any part of the body, leading to a wide range of symptoms. - While it can cause vasculitis, its epidemiology, clinical presentation, and specific organ involvement are distinctly different from **Kawasaki disease**.

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: 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: All of the following are true regarding Tetralogy of Fallot except which of the following?

Predominantly left to right shunt. ***Predominantly left to right shunt*** - Tetralogy of Fallot is characterized by a **right-to-left shunt** due to subpulmonary stenosis, leading to cyanosis [1]. - A persistent **left-to-right shunt** is inconsistent with the typical presentation of Tetralogy of Fallot [1]. *Ejection systolic murmur in second intercostal space* - The **ejection systolic murmur** in the second intercostal space is due to the **pulmonic stenosis**, which is a key component of Tetralogy of Fallot [1]. - The murmur's intensity is inversely proportional to the severity of the obstruction. *Single second heart sound* - The single second heart sound is a common finding because of the **reduced pulmonary blood flow** and thus the soft or absent pulmonary component of S2. - The **aortic component** is typically heard clearly, but the pulmonary component is diminished. *Normal jugular venous pressure* - **Normal jugular venous pressure** is expected in Tetralogy of Fallot unless there is associated right-sided heart failure, which is not a primary feature of uncomplicated TOF. - The primary hemodynamic derangement involves shunting at the ventricular level, not elevated right atrial pressure.

Q: What is the most important prognostic marker of tetralogy of Fallot?

Degree of pulmonary stenosis. **Degree of pulmonary stenosis** - The severity of **pulmonary stenosis** dictates the degree of right ventricular outflow obstruction and thus the amount of **right-to-left shunting** through the VSD, which directly impacts the patient's cyanosis and overall prognosis [1]. - More severe stenosis leads to greater cyanosis, earlier presentation of symptoms, and a higher risk of complications and mortality without intervention. *Ventricular septal defect (VSD)* - A **VSD** is a required component of tetralogy of Fallot, allowing for communication between the ventricles [1]. - While essential for the pathophysiology, its presence alone does not determine the severity of the clinical presentation or prognosis as much as the degree of pulmonary outflow obstruction. *Overriding aorta* - An **overriding aorta** is another structural component of tetralogy of Fallot, meaning the aorta originates over both ventricles [1]. - This anatomical feature is a defining characteristic but does not directly dictate the clinical severity or prognosis as much as the blood flow dynamics determined by pulmonary stenosis. *Right ventricular hypertrophy* - **Right ventricular hypertrophy** is a compensatory response to the increased pressure load from pulmonary stenosis [1]. - It is a consequence of the obstruction, not the primary determinant of prognosis, although severe hypertrophy can lead to complications such as arrhythmias and decreased ventricular function.

Question 1

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 2

Which one of the following conditions is associated with Kawasaki's syndrome?

Accepted Answer

Coronary artery aneurysm

Answer

Acute rheumatic fever

Answer

Pulmonary embolus

Answer

Systemic lupus erythematosus

Question 3

Treatment of choice for Kawasaki disease is:

Accepted Answer

IV Immunoglobulins

Answer

Methotrexate

Answer

Corticosteroids

Answer

Azathioprine

Question 4

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 5

All of the following are true regarding Tetralogy of Fallot except which of the following?

Accepted Answer

Predominantly left to right shunt

Answer

Single second heart sound

Answer

Ejection systolic murmur in second intercostal space

Answer

Normal jugular venous pressure

Question 6

What is the most important prognostic marker of tetralogy of Fallot?

Accepted Answer

Degree of pulmonary stenosis

Answer

Overriding aorta

Answer

Ventricular septal defect (VSD)

Answer

Right ventricular hypertrophy

Cardiology — MCQs

Cardiology — MCQs

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