Congenital Heart Disease — MCQs

10 questions

A 2-month-old infant is brought to the clinic because of poor feeding, sweating, and difficulty breathing. The parents state that she was doing very well, and has actually been a "very easy going baby", until about a week ago. They assumed that she was developing a "cold", but it has not passed, and the symptoms have been worsening. Cardiac examination reveals a loud, harsh systolic murmur with a thrill that is heard best at the left sternal border. The most likely underlying abnormality is

Commonest type of congenital cyanotic heart disease is -

A 2-week-old girl is found to have a harsh murmur along the left sternal border. The parents report that the baby gets "bluish" when she cries or drinks from her bottle. Echocardiogram reveals a congenital heart defect associated with pulmonary stenosis, ventricular septal defect, dextroposition of the aorta, and right ventricular hypertrophy. What is the appropriate diagnosis?

In a child with coarctation of aorta, all the following are seen in plain chest radiograph except:

A child presented at 10 weeks with recurrent episode of pneumonia and failure to thrive. X-ray shows cardiomegaly & pulmonary plethora. What is the diagnosis?

Which of the following is the most common congenital cardiac malformation?

A 45-year-old woman presents with clubbing and cyanosis. Chest X-ray shows a prominent pulmonary artery. Which congenital heart disease should be considered?

Flask shaped heart is seen in –

Boot shape of heart in TOF is due to:

Q10

In the condition shown below, rib notching is present in which of the following ribs? (AIIMS Nov 2015)

Congenital Heart Disease Indian Medical PG Practice Questions and MCQs

Question 1: A 2-month-old infant is brought to the clinic because of poor feeding, sweating, and difficulty breathing. The parents state that she was doing very well, and has actually been a "very easy going baby", until about a week ago. They assumed that she was developing a "cold", but it has not passed, and the symptoms have been worsening. Cardiac examination reveals a loud, harsh systolic murmur with a thrill that is heard best at the left sternal border. The most likely underlying abnormality is

A. aortic stenosis
B. atrial septal defect
C. patent ductus arteriosus
D. ventricular septal defect (Correct Answer)

Explanation: ***ventricular septal defect*** - A **ventricular septal defect (VSD)** causes a **harsh, holosystolic (pansystolic) murmur** best heard at the **left sternal border**, often with a palpable thrill, due to turbulent blood flow through the defect from the high-pressure left ventricle to the lower-pressure right ventricle. - Symptoms like **poor feeding, sweating, and difficulty breathing** in an infant, especially with recent onset and worsening, are consistent with **congestive heart failure** secondary to a large VSD causing significant left-to-right shunting. - The timing of symptom onset (around 2 months) is typical, as pulmonary vascular resistance drops after the first 4-6 weeks of life, increasing the left-to-right shunt and precipitating heart failure. *aortic stenosis* - **Aortic stenosis** typically presents with a **systolic ejection murmur** loudest at the **right upper sternal border** with radiation to the neck, not a harsh holosystolic murmur at the left sternal border. - While severe aortic stenosis can lead to heart failure symptoms, the murmur's location and quality are inconsistent with this diagnosis. *atrial septal defect* - An **atrial septal defect (ASD)** typically causes a **systolic ejection murmur** at the **pulmonary area** (left upper sternal border) due to increased flow across the pulmonic valve, often accompanied by a **fixed, wide splitting of S2**. - Significant heart failure in infancy is rare with an isolated ASD, as the pressure gradient across the atria is usually low, leading to asymptomatic presentation until later childhood or adulthood. *patent ductus arteriosus* - A **patent ductus arteriosus (PDA)** is characterized by a **continuous, machine-like murmur** loudest below the left clavicle (infraclavicular area), not a harsh systolic murmur with a thrill at the left sternal border. - While a large PDA can cause heart failure symptoms in infancy, the distinctive continuous murmur differentiates it from a VSD.

Question 2: Commonest type of congenital cyanotic heart disease is -

A. ASD
B. TOF (Correct Answer)
C. PDA
D. Single ventricle defects

Explanation: ***TOF (Correct Answer)*** - **Tetralogy of Fallot (TOF)** is the **most common cyanotic congenital heart disease**, accounting for approximately 10% of all congenital heart defects. - Characterized by **four main defects**: ventricular septal defect (VSD), pulmonary stenosis, overriding aorta, and right ventricular hypertrophy. - The degree of **pulmonary stenosis** determines the severity of cyanosis and clinical presentation. - Classic presentation includes **cyanotic spells** (Tet spells) and **squatting** behavior in children. *ASD (Incorrect)* - **Atrial Septal Defect (ASD)** is an **acyanotic** congenital heart defect with left-to-right shunting. - Does not cause cyanosis under normal circumstances because oxygenated blood from the left atrium shunts to the right atrium. - Symptoms are usually mild in childhood and may include fatigue or shortness of breath. *PDA (Incorrect)* - **Patent Ductus Arteriosus (PDA)** is generally an **acyanotic** heart defect with left-to-right shunting. - The ductus arteriosus remains open after birth, connecting the aorta to the pulmonary artery. - While it can cause **differential cyanosis** in rare instances of severe pulmonary hypertension with shunt reversal (Eisenmenger syndrome), it is not primarily classified as a cyanotic heart disease. *Single ventricle defects (Incorrect)* - **Single ventricle defects** (e.g., hypoplastic left heart syndrome, tricuspid atresia) are complex **cyanotic** heart defects, but they are **less common than Tetralogy of Fallot**. - These defects involve only one functional ventricle, leading to mixing of oxygenated and deoxygenated blood. - Require staged surgical palliation (e.g., Norwood, Glenn, Fontan procedures).

Question 3: A 2-week-old girl is found to have a harsh murmur along the left sternal border. The parents report that the baby gets "bluish" when she cries or drinks from her bottle. Echocardiogram reveals a congenital heart defect associated with pulmonary stenosis, ventricular septal defect, dextroposition of the aorta, and right ventricular hypertrophy. What is the appropriate diagnosis?

A. Atrial septal defect
B. Tetralogy of Fallot (Correct Answer)
C. Coarctation of aorta, postductal
D. Coarctation of aorta, preductal

Explanation: ***Tetralogy of Fallot*** - The combination of **pulmonary stenosis**, **ventricular septal defect**, **dextroposition of the aorta** (overriding aorta), and **right ventricular hypertrophy** is the classic definition of Tetralogy of Fallot. - The "bluish" episodes (cyanosis) when crying or feeding are characteristic of **tet spells**, indicating right-to-left shunting and reduced pulmonary blood flow, exacerbated by activity. *Atrial septal defect* - An ASD primarily involves a **left-to-right shunt** and typically presents with a **fixed, split S2** and a **pulmonic flow murmur**, usually without cyanosis in infancy. - It does not involve the characteristic four defects seen in this patient, particularly the significant pulmonary stenosis and cyanosis. *Coarctation of aorta, postductal* - **Postductal coarctation** typically presents in older children or adults with **hypertension in the upper extremities** and **diminished or absent femoral pulses**, often without cyanosis. - This condition is a narrowing of the aorta **distal to the ductus arteriosus** and does not involve the four specific intracardiac defects described. *Coarctation of aorta, preductal* - **Preductal coarctation** can present in neonates with **heart failure** and **differential cyanosis** (upper body pink, lower body blue), or signs of shock if the ductus arteriosus closes. - This condition involves a narrowing of the aorta **proximal to the ductus arteriosus** and is not characterized by the four specific tetralogy defects.

Question 4: In a child with coarctation of aorta, all the following are seen in plain chest radiograph except:

A. Three sign
B. 'E' Sign or 'Reverse Three sign' (Correct Answer)
C. Prominent ascending aorta
D. Rib notching

Explanation: ***'E' Sign or 'Reverse Three sign' (Reverse ε sign)*** - The **'E' sign** or **'reverse three sign'** is seen on **barium esophagram** (lateral view), NOT on a plain chest X-ray - On barium swallow, the esophagus shows indentation creating a reverse '3' or 'ε' shape due to impression from the dilated pre-stenotic aorta, the coarctation site, and the dilated post-stenotic aorta - **This is the correct answer** as the question asks specifically about plain chest radiograph findings - This sign requires contrast study and cannot be visualized on plain radiography *Three sign ('3' sign)* - The **'three sign'** is a **classic finding** in coarctation on plain chest X-ray (PA view) - Seen on the **left heart border** representing: (1) dilated left subclavian artery, (2) indentation at coarctation site, (3) post-stenotic dilation of descending aorta - Creates the shape of the numeral '3' along the aortic knuckle region - This is directly visible on plain radiograph *Prominent ascending aorta* - **Commonly seen** in coarctation due to increased afterload on the left ventricle - Results in **left ventricular hypertrophy** and dilation of the ascending aorta - Part of the cardiovascular remodeling in response to chronic pressure overload - Visible as widening of the superior mediastinum on plain chest X-ray *Rib notching* - **Classic finding** in long-standing coarctation of the aorta (usually after 5-6 years of age) - Due to **collateral circulation** through dilated intercostal arteries that erode the inferior rib margins - Typically affects **ribs 3-9** bilaterally - Represents chronic compensatory mechanism to bypass the obstruction

Question 5: A child presented at 10 weeks with recurrent episode of pneumonia and failure to thrive. X-ray shows cardiomegaly & pulmonary plethora. What is the diagnosis?

A. VSD (Correct Answer)
B. TOF
C. Patent foramen ovale
D. ASD

Explanation: ***VSD*** - **Ventricular septal defect (VSD)** is the most common cause of this presentation in early infancy (symptoms typically appear at **6-10 weeks** of age). - Large VSDs cause significant **left-to-right shunt** leading to pulmonary overcirculation, resulting in **recurrent pneumonia** and **failure to thrive**. - **Cardiomegaly** (due to volume overload of left atrium and ventricle) and **pulmonary plethora** (increased pulmonary vascular markings) on X-ray are classic findings. - The infant may also present with tachypnea, feeding difficulties, and poor weight gain. *TOF* - **Tetralogy of Fallot (TOF)** is a **cyanotic heart defect** with right-to-left shunt, presenting with cyanosis and hypoxic spells, not recurrent pneumonia. - X-ray shows **boot-shaped heart** and **pulmonary oligemia** (decreased pulmonary vascular markings), not pulmonary plethora. - Does not typically cause failure to thrive in the same manner as acyanotic left-to-right shunt lesions. *Patent foramen ovale* - A **patent foramen ovale (PFO)** is a normal variant in infants and typically remains **asymptomatic**. - Does not cause significant hemodynamic shunting in the absence of elevated right atrial pressure. - Does not cause **cardiomegaly**, **pulmonary plethora**, recurrent pneumonia, or failure to thrive. *ASD* - An **atrial septal defect (ASD)** also causes left-to-right shunt with pulmonary plethora, but the shunt develops **gradually** over time. - ASD typically presents **later in childhood or adulthood** with milder symptoms (fatigue, exercise intolerance) due to lower pressure gradient across atria. - **Recurrent pneumonia and failure to thrive at 10 weeks** are uncommon with isolated ASD, as the hemodynamic changes are less pronounced in early infancy compared to VSD. - When symptomatic in infancy, large ASDs present later (around 6 months to 1 year) rather than at 10 weeks.

Question 6: Which of the following is the most common congenital cardiac malformation?

A. Persistent truncus arteriosus (PTA)
B. Common ventricle (CV)
C. Ventricular septal defect (VSD) (Correct Answer)
D. Atrial septal defect (ASD)

Explanation: ***Ventricular septal defect (VSD)*** - VSDs are the **most common congenital heart defect**, accounting for approximately 25-30% of all congenital cardiac malformations. - They involve a **hole in the septum** separating the left and right ventricles, leading to a left-to-right shunt. *Persistent truncus arteriosus (PTA)* - PTA is a rare congenital heart defect where a **single arterial trunk** arises from the heart, supplying both systemic and pulmonary circulation. - Its incidence is much **lower than VSD**, representing less than 1% of congenital heart defects. *Common ventricle (CV)* - A common ventricle, also known as **single ventricle**, is a complex and rare congenital defect where only one functional ventricle is present. - It is a **severe malformation** and much less common than VSD. *Atrial septal defect (ASD)* - ASDs are congenital heart defects involving a **hole in the wall between the atria** of the heart. - While relatively common, ASDs are **less frequent than VSDs**, accounting for about 5-10% of congenital heart defects.

Question 7: A 45-year-old woman presents with clubbing and cyanosis. Chest X-ray shows a prominent pulmonary artery. Which congenital heart disease should be considered?

A. Patent ductus arteriosus (PDA) with Eisenmenger syndrome.
B. Atrial septal defect (ASD) with Eisenmenger syndrome. (Correct Answer)
C. Tetralogy of Fallot
D. Pulmonary stenosis (PS)

Explanation: Atrial septal defect (ASD) with Eisenmenger syndrome. - Clubbing, cyanosis, and a prominent pulmonary artery in a 45-year-old suggest long-standing pulmonary hypertension with shunt reversal, characteristic of Eisenmenger syndrome [1]. - An ASD is a common congenital heart defect that can lead to significant left-to-right shunting, eventually causing pulmonary hypertension [1]. and reversed shunt flow (Eisenmenger syndrome) over decades [2]. *Patent ductus arteriosus (PDA) with Eisenmenger syndrome.* - While PDA can also lead to Eisenmenger syndrome, it typically presents with a continuous murmur and often causes symptoms earlier in life if the shunt is large. - The patient's age and the specific features presented are more classic for an ASD progressing to Eisenmenger syndrome. *Tetralogy of Fallot* - This is a cyanotic congenital heart disease from birth or early childhood, commonly presenting with cyanosis and "tet spells" [3]. - While it causes cyanosis, a prominent pulmonary artery is not a typical feature; instead, there is often reduced pulmonary blood flow and a small pulmonary artery due to pulmonary outflow obstruction [3]. *Pulmonary stenosis (PS)* - Pulmonary stenosis primarily causes obstruction to blood flow out of the right ventricle, leading to right ventricular hypertrophy and potentially a systolic murmur. - While severe PS can cause cyanosis due to a right-to-left shunt through an ASD (if present), isolated PS is typically acyanotic and does not usually present with a prominent pulmonary artery or clubbing unless it's very severe and associated with other defects.

Question 8: Flask shaped heart is seen in –

A. Pericardial effusion (Correct Answer)
B. TOF
C. Ebstein anomaly
D. TAPVC

Explanation: ***Pericardial effusion*** - A **"flask-shaped"** or **"water bottle-shaped" heart** on chest X-ray is a classic finding in significant pericardial effusion. - This appearance results from the accumulation of a large amount of fluid in the **pericardial sac**, which causes the cardiac silhouette to enlarge symmetrically and assume a globular shape. *TOF (Tetralogy of Fallot)* - TOF typically presents with a **"boot-shaped" heart** (coeur en sabot) on chest X-ray due to right ventricular hypertrophy and a concave pulmonary artery segment. - This morphology is distinctly different from the flask-shaped appearance of pericardial effusion. *Ebstein anomaly* - Ebstein anomaly is characterized by apical displacement of the tricuspid valve, leading to **massive right atrial enlargement**. - On chest X-ray, this often results in a **markedly enlarged oval-shaped heart**, which can be quite massive but does not typically have the distinct flask/water bottle shape. *TAPVC (Total Anomalous Pulmonary Venous Connection)* - TAPVC can present with different X-ray findings depending on the type, but a classic finding for the supracardiac type is a **"snowman" or "figure-of-8" heart** in children. - This appearance is due to the dilated superior vena cava and anomalous veins draining to it, not a flask shape.

Question 9: Boot shape of heart in TOF is due to:

A. Right ventricular hypertrophy (Correct Answer)
B. Enlargement of the left atrium
C. Enlargement of the right atrium
D. Hypertrophy of both ventricles

Explanation: ***Right ventricular hypertrophy*** - The characteristic **boot-shaped heart (coeur en sabot)** seen in Tetralogy of Fallot (TOF) on a chest X-ray is primarily due to **right ventricular hypertrophy** and the small pulmonary artery. - The hypertrophied right ventricle lifts the cardiac apex, while the concavity in the area of the pulmonary artery (due to **pulmonary stenosis**) gives the heart its distinctive shape. *Enlargement of the left atrium* - Left atrial enlargement is not a feature of **Tetralogy of Fallot**; in fact, chronic pulmonary outflow obstruction often leads to a relatively normal or small left atrium. - This condition involves right-sided heart abnormalities, and left atrial enlargement would suggest increased left-sided pressures, which are not typical for TOF. *Enlargement of the right atrium* - While right atrial enlargement can occur in severe cases of TOF due to increased resistance to blood flow, it is **right ventricular hypertrophy** that is the primary determinant of the classic boot-shaped cardiac silhouette. - Right atrial enlargement alone does not create the specific "boot" appearance which is largely due to the ventricular contour. *Hypertrophy of both ventricles* - In Tetralogy of Fallot, the primary ventricular abnormality is **right ventricular hypertrophy**, driven by the need to pump blood through a stenosed pulmonary artery. - The left ventricle typically maintains a normal size and function, as it primarily pumps into the systemic circulation and is not directly affected by the primary defects in the same way as the right ventricle.

Question 10: In the condition shown below, rib notching is present in which of the following ribs? (AIIMS Nov 2015)

A. 3rd to 9th ribs (Correct Answer)
B. 1st to 9th ribs
C. 11th and 12th ribs
D. All ribs

Explanation: **3rd to 9th ribs** - The image provided depicts **coarctation of the aorta**, characterized by a narrowing of the aorta, typically distal to the origin of the left subclavian artery. - In coarctation of the aorta, collateral circulation develops through the **intercostal arteries** to bypass the constriction, leading to their enlargement and subsequent erosion of the inferior margins of the **3rd to 9th ribs**, a finding known as "rib notching." *1st to 9th ribs* - While rib notching affects upper ribs, it typically **spares the 1st and 2nd ribs** because the superior intercostal arteries (which supply these ribs) originate directly from the subclavian artery, often proximal to the coarctation, so they do not participate in collateral circulation as significantly. - The pattern of notching is usually more concentrated in the mid-thoracic region. *11th and 12th ribs* - Rib notching from coarctation of the aorta is rarely observed in the **floating ribs** (11th and 12th ribs). - These ribs have a different anatomical relationship with the pleura and typically do not bear the brunt of increased collateral flow from the intercostal arteries in the same way as the higher ribs. *All ribs* - Rib notching is a localized phenomenon reflecting increased blood flow through specific intercostal arteries involved in collateral circulation due to aortic coarctation. - Therefore, it does **not affect all ribs**, and its absence in certain ribs (like the 1st, 2nd, 11th, and 12th) helps differentiate this condition radiologically.

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