Cardiology Practice Questions

Q: A 5-year-old child presents with exertional dyspnea and a history of cyanotic spells. On examination, there is clubbing and a harsh systolic murmur heard at the left upper sternal border. Chest X-ray is shown below. What is the most likely diagnosis?

Tetralogy of Fallot. ***Tetralogy of Fallot*** - The clinical triad of **exertional dyspnea**, **cyanotic spells** (tet spells), and a **harsh systolic murmur** at the left upper sternal border (due to pulmonary stenosis) is classic for this condition. - The chest X-ray shows a characteristic **"boot-shaped heart"** (coeur en sabot), which results from **right ventricular hypertrophy** (forming the toe) and a concave main pulmonary artery segment. *Total Anomalous Pulmonary Venous Connection* - The classic chest X-ray finding for TAPVC is a **"snowman sign"** or **"figure-of-eight"** appearance, which is not seen in the provided image. - While it is a cyanotic condition, it results from the mixing of oxygenated and deoxygenated blood and is associated with signs of right heart failure and pulmonary edema. *Transposition of the Great Arteries* - This condition typically presents with severe **cyanosis at birth** and requires immediate intervention for survival; presentation at age 5 is highly unlikely without prior surgery. - The characteristic radiographic sign is an **"egg-on-a-string"** appearance with a narrow superior mediastinum, differing from the boot shape seen here. *Atrial Septal Defect* - An ASD is an **acyanotic** heart defect (left-to-right shunt), so it does not cause cyanotic spells or clubbing in a 5-year-old. - The hallmark physical finding is a **wide, fixed splitting of S2**, not a harsh systolic murmur.

Q: A child presents with kawasaki disease with multiple small coronary artery aneurysms. Treatment is?

Aspirin for 6 weeks. ***Aspirin for 6 weeks*** - After the acute phase is treated with **IVIG** and high-dose aspirin, the regimen is switched to low-dose aspirin for its **antiplatelet** effects to prevent thrombosis in the affected coronary arteries. - This low-dose therapy is typically continued for 6-8 weeks, at which point a follow-up **echocardiogram** is performed and inflammatory markers (like **ESR** and **CRP**) should have normalized. *Aspirin lifelong* - Lifelong antiplatelet therapy is generally reserved for patients with **large** or **giant** coronary artery aneurysms due to a high risk of **thrombosis** and stenosis. - For small aneurysms, which often resolve, therapy is guided by serial echocardiograms and is not typically lifelong from the outset. *Aspirin for 4 weeks* - A 4-week duration is insufficient, as it may not cover the entire period of coronary wall inflammation and thrombocytosis, which usually peaks in the subacute phase. - The standard follow-up interval for re-evaluation with an **echocardiogram** is at 6-8 weeks, making it logical to continue therapy at least until that point. *Aspirin and clopidogrel for 6 weeks* - **Dual antiplatelet therapy** with aspirin and clopidogrel is recommended for patients with **medium-sized** or **giant** coronary artery aneurysms, not for small aneurysms. - The flowchart provided indicates that for small aneurysms (Z score ≥2.5 to <5), **single antiplatelet therapy** with low-dose aspirin is the appropriate treatment.

Q: What could be the possible diagnosis for a newborn exhibiting weak lower limb pulses and strong upper limb pulses?

COA. ***COA*** (Coarctation of the Aorta) - The classic presentation of **Coarctation of the Aorta** involves a narrowing of the aorta, usually distal to the left subclavian artery, leading to increased pressure proximal to the coarctation (strong upper limb pulses) and decreased pressure distal to it (weak lower limb pulses). - This severe pressure gradient is manifested as **differential pulses** and potential **blood pressure discrepancy** between the arms and legs, making this the most likely diagnosis. *TGA* - **Transposition of the Great Arteries** is characterized by severe **cyanosis** presenting shortly after birth due to two parallel circulations (aorta from RV, pulmonary artery from LV). - It does not typically cause a significant differential in pulse strength between the upper and lower limbs unless complicated by underlying aortic arch anomalies. *TOF* - **Tetralogy of Fallot** typically presents with cyanosis and **hypercyanotic spells** (**tet spells**) due to severe right ventricular outflow tract obstruction and a large VSD. - While it is a common cyanotic heart disease, it primarily affects flow to the pulmonary circulation and does not cause obstruction in the systemic aorta leading to differential pulses. *Ebstein anomaly* - This condition involves the apical displacement of the **tricuspid valve** leaflets into the **right ventricle**, leading to tricuspid regurgitation and large right atrium. - Clinical features usually include varying degrees of cyanosis and signs of right heart failure but do not typically involve a differential in systemic arterial pulses.

Q: A 9-month-old infant presents with severe respiratory distress, central cyanosis not improving with oxygen, and a harsh systolic murmur. Chest X-ray shows boot-shaped heart with decreased pulmonary vascular markings. Echocardiography confirms the diagnosis. What is the most likely underlying cardiac defect?

Tetralogy of Fallot. ***Tetralogy of Fallot*** - The combination of **severe respiratory distress**, **central cyanosis** unresponsive to oxygen (a **cyanotic spell**), **harsh systolic murmur** (due to **pulmonary stenosis**), and a **boot-shaped heart** (coeur en sabot) on chest X-ray with **decreased pulmonary vascular markings** (due to reduced blood flow) is the classic presentation of **Tetralogy of Fallot** (TOF). - TOF is the most common cyanotic congenital heart disease and consists of four defects: large **VSD**, **pulmonary stenosis**, **overriding aorta**, and **right ventricular hypertrophy**. *Transposition of Great Arteries (TGA)* - TGA presents with severe cyanosis, but the chest X-ray typically shows a **'egg-on-a-string'** appearance due to a narrow mediastinum and cardiomegaly, not a boot shape. - Pulmonary vascular markings are often **increased** or normal, depending on whether there is associated pulmonary stenosis, and the murmur is typically non-specific or absent. *Total Anomalous Pulmonary Venous Return (TAPVR)* - This condition presents with cyanosis and can show a **'snowman'** or **'figure-of-eight'** sign on the chest X-ray if the supra-cardiac type is present, due to enlarged vertical vein and SVC. - Pulmonary vascular markings are usually **increased** due to pulmonary venous obstruction. *Truncus Arteriosus* - Although cyanotic, truncus arteriosus is characterized by a single great artery overriding the ventricular septum, leading to **increased pulmonary blood flow** and consequently **increased pulmonary vascular markings** on the chest X-ray. - The heart shape is typically **cardiomegaly** with a common trunk arising from the heart, often resulting in a late-onset or subtle cyanosis compared to TOF or TGA.

Q: A 4-year-old child presents with high-grade fever for 5 days, bilateral non-purulent conjunctival injection, strawberry tongue, cervical lymphadenopathy (>1.5 cm), and polymorphous rash. On day 6, desquamation of fingertips begins. Echocardiography shows coronary artery dilation. What is the most appropriate immediate treatment?

Intravenous immunoglobulin (IVIG) with aspirin. ***Intravenous immunoglobulin (IVIG) with aspirin*** - This is the **gold standard, immediate treatment** for acute Kawasaki disease to reduce systemic inflammation and prevent coronary artery complications - **IVIG** (2 g/kg as a single infusion over 10-12 hours) works by modulating the immune response and reducing inflammation - **High-dose aspirin** (80-100 mg/kg/day divided into 4 doses) provides anti-inflammatory effects during the acute febrile phase - This combination must be started **within 10 days of fever onset** (ideally within 7 days) to significantly reduce the risk of **coronary artery aneurysms** - Given that this child already has coronary artery dilation on echo, urgent IVIG + aspirin therapy is critical to prevent progression *High-dose aspirin alone* - Aspirin alone is **insufficient** to address the underlying immune-mediated vasculitis in Kawasaki disease - While aspirin has anti-inflammatory and antiplatelet effects, it does **not provide the immunomodulatory benefits** of IVIG - Monotherapy with aspirin has been shown to be **significantly less effective** at preventing coronary complications compared to combination therapy *Supportive care only* - Supportive care (hydration, antipyretics) addresses symptoms but **does not treat the underlying vasculitis** - Without specific immunomodulatory therapy, up to **25% of untreated KD patients** develop coronary artery aneurysms - This child already has coronary involvement, making specific therapy even more urgent *Oral corticosteroids* - Corticosteroids are generally reserved for **IVIG-refractory cases** (patients who remain febrile 36-48 hours after initial IVIG) or high-risk patients - They are **not recommended as first-line therapy** according to current AHA guidelines - IVIG remains superior for initial treatment in preventing coronary complications

Q: A 3-year-old child is brought to the clinic with a history of cyanosis since infancy. Which of the following is a component of Tetralogy of Fallot (TOF)?

Infundibular pulmonary stenosis. ***Infundibular pulmonary stenosis*** - This is the most common anatomic type of **pulmonary stenosis** seen in TOF, caused by hypertrophy of the muscle below the pulmonary valve (infundibulum). - The degree of this stenosis dictates the direction of flow across the VSD and, consequently, the severity of **cyanosis**. *Inter atrial septal defect* - An ASD is not a primary component of TOF. When TOF is associated with an ASD, the condition is termed **Pentalogy of Fallot**. - ASD typically causes a **left-to-right shunt** and is usually an acyanotic or late-onset cyanotic condition, unlike classic TOF. *Left ventricular hypertrophy (LVH)* - The pressure overload due to **pulmonary stenosis** and the large **VSD** leads to **Right Ventricular Hypertrophy (RVH)**. - LVH suggests conditions like severe **aortic stenosis** or systemic overloading, not the typical hemodynamics of TOF. *Transposition of the great arteries (TGA)* - **TGA** is a separate, distinct cyanotic congenital heart disease where the great arteries are transposed (aorta from RV, pulmonary artery from LV). - The components of TOF involve a single great artery relationship but with a large **Ventricular Septal Defect (VSD)** and overriding aorta.

Q: A neonate presents with a congenital heart disease as shown below. Which drug should be started immediately? (Recent NEET Pattern 2016-17)

Alprostadil. ***Alprostadil*** - The image shows a **transposition of the great arteries (TGA)** with the aorta originating from the right ventricle and the pulmonary artery from the left ventricle. In this condition, a patent ductus arteriosus (PDA) is crucial to maintain mixing of oxygenated and deoxygenated blood to ensure systemic circulation. - **Alprostadil (prostaglandin E1 analog)** is used to keep the **ductus arteriosus patent**, allowing for mixing of blood and improving systemic oxygenation in neonates with ductal-dependent congenital heart defects like severe TGA. *Digoxin* - **Digoxin** is a cardiac glycoside used to improve **cardiac contractility** and control heart rate in certain heart conditions, but it is not the immediate drug of choice for maintaining patency of the ductus arteriosus in TGA. - Its primary role is in managing heart failure symptoms or arrhythmias, not directly addressing the immediate ductal dependency of TGA. *Ivabradine* - **Ivabradine** is a hyperpolarization-activated cyclic nucleotide-gated (HCN) channel blocker that selectively reduces heart rate by acting on the **sinoatrial node**. - It is used in adults with chronic heart failure or stable angina, and is not indicated for the acute management of congenital heart defects like TGA in neonates. *Propranolol* - **Propranolol** is a non-selective beta-blocker used to reduce heart rate and blood pressure, or to manage conditions like **tetralogy of Fallot spells**. - It does not have any role in maintaining the patency of the ductus arteriosus, which is critical for survival in ductal-dependent congenital heart lesions like TGA.

Q: Which congenital heart disease is associated with the defect shown?

Endocardial cushion defect. ***Endocardial cushion defect*** - The image displays a **single palmar crease** (also known as a **simian crease**), which is a common dermatological feature observed in patients with **Down syndrome (Trisomy 21)**. - Approximately 40-50% of individuals with Down syndrome have **congenital heart disease**, with **endocardial cushion defects** (also known as atrioventricular septal defects) being the most common type. *Tricuspid atresia* - This is a complex cyanotic congenital heart defect characterized by the **absence of the tricuspid valve**, preventing blood flow from the right atrium to the right ventricle. - While it is a congenital heart defect, it is **not specifically associated with a single palmar crease** or Down syndrome as commonly as endocardial cushion defects. *Double outlet right ventricle* - This is a rare cyanotic congenital heart defect where **both the aorta and pulmonary artery arise primarily from the right ventricle**. - It is not typically associated with specific dermatological signs like a single palmar crease or specifically linked to Down syndrome. *Aortic regurgitation* - This is a condition where the **aortic valve does not close tightly**, causing blood to leak back into the left ventricle during diastole. - Aortic regurgitation is an **acquired or congenital valve defect**, but it is not directly linked to genetic syndromes like Down syndrome or the presence of a single palmar crease.

Question 1

A 5-year-old child presents with exertional dyspnea and a history of cyanotic spells. On examination, there is clubbing and a harsh systolic murmur heard at the left upper sternal border. Chest X-ray is shown below. What is the most likely diagnosis?

Accepted Answer

Tetralogy of Fallot

Answer

Total Anomalous Pulmonary Venous Connection

Answer

Atrial Septal Defect

Answer

Transposition of the Great Arteries

Question 2

A child presents with kawasaki disease with multiple small coronary artery aneurysms. Treatment is?

Accepted Answer

Aspirin for 6 weeks

Answer

Aspirin for 4 weeks

Answer

Aspirin lifelong

Answer

Aspirin and clopidogrel for 6 weeks

Question 3

What could be the possible diagnosis for a newborn exhibiting weak lower limb pulses and strong upper limb pulses?

Accepted Answer

COA

Answer

TOF

Answer

Ebstein anomaly

Answer

TGA

Question 4

A 9-month-old infant presents with severe respiratory distress, central cyanosis not improving with oxygen, and a harsh systolic murmur. Chest X-ray shows boot-shaped heart with decreased pulmonary vascular markings. Echocardiography confirms the diagnosis. What is the most likely underlying cardiac defect?

Accepted Answer

Tetralogy of Fallot

Answer

Total anomalous pulmonary venous return

Answer

Transposition of great arteries

Answer

Truncus arteriosus

Question 5

A 4-year-old child presents with high-grade fever for 5 days, bilateral non-purulent conjunctival injection, strawberry tongue, cervical lymphadenopathy (>1.5 cm), and polymorphous rash. On day 6, desquamation of fingertips begins. Echocardiography shows coronary artery dilation. What is the most appropriate immediate treatment?

Accepted Answer

Intravenous immunoglobulin (IVIG) with aspirin

Answer

High-dose aspirin alone

Answer

Supportive care only

Answer

Oral corticosteroids

Question 6

A 3-year-old child is brought to the clinic with a history of cyanosis since infancy. Which of the following is a component of Tetralogy of Fallot (TOF)?

Accepted Answer

Infundibular pulmonary stenosis

Answer

Inter atrial septal defect

Answer

Left ventricular hypertrophy (LVH)

Answer

Transposition of the great arteries (TGA)

Question 7

A 6-week-old term baby presents with poor feeding. All are true about the condition shown except:

Accepted Answer

Ductal-dependent systemic circulation

Answer

Ibuprofen inhibits Prostaglandin production leading to closure

Answer

Left ventricular failure

Answer

Anatomical closure occurs by 2-3 weeks

Question 8

A neonate presents with a congenital heart disease as shown below. Which drug should be started immediately? (Recent NEET Pattern 2016-17)

Accepted Answer

Alprostadil

Answer

Digoxin

Answer

Ivabradine

Answer

Propranolol

Question 9

Which congenital heart disease is associated with the defect shown?

Accepted Answer

Endocardial cushion defect

Answer

Tricuspid atresia

Answer

Double outlet right ventricle

Answer

Aortic regurgitation

Question 10

A 1 day old neonate presents with central cyanosis. CXR and ECG are performed (shown in the image). Which diagnosis is most consistent with these findings?

Accepted Answer

Cardiomegaly with normal ECG suggestive of hypoplastic left heart syndrome

Answer

Cardiomegaly and Tall T waves in ECG suggestive of tetralogy of Fallot

Answer

Cardiomegaly with delta waves in ECG suggestive of Wolff-Parkinson-White syndrome

Answer

Cardiomegaly, Himalayan P waves in ECG suggestive of tricuspid atresia

Cardiology — MCQs

Cardiology — MCQs

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