Cardiology Practice Questions

Q: Which of the following is NOT a ductus dependent congenital heart disease?

Truncus arteriosus. ***Truncus arteriosus*** - In **truncus arteriosus**, a single great artery arises from both ventricles, supplying both systemic and pulmonary circulation. The **pulmonary blood flow is typically unrestricted**, meaning the ductus arteriosus is not required for survival. - While a VSD is usually present, allowing mixing of blood, the pulmonary blood flow does not depend on the **ductus arteriosus** for patency. - This is the only condition among the options that is **NOT ductus-dependent**. *Tricuspid atresia* - **Tricuspid atresia** is a ductus-dependent lesion because without a patent **ductus arteriosus**, there is inadequate mixing or blood flow to reach either the pulmonary or systemic circulation depending on the anatomic variant. - The **ductus arteriosus** is crucial for survival in the neonatal period, often necessitating prostaglandin infusion to maintain ductal patency. *Tetralogy of Fallot with pulmonary atresia* - In **TOF with pulmonary atresia**, there is complete obstruction of the right ventricular outflow tract, making the **ductus arteriosus** the only source of pulmonary blood flow. - If the **ductus arteriosus closes**, the infant will suffer from severe **cyanosis** due to complete absence of pulmonary blood flow. - Note: Standard TOF without pulmonary atresia is NOT ductus-dependent as some blood reaches the lungs through the stenotic pulmonary valve. *Interrupted Aortic Arch* - **Interrupted Aortic Arch** is a ductus-dependent lesion because postnatal survival depends on a patent **ductus arteriosus** to supply blood flow to the descending aorta and lower body. - Without the **ductus arteriosus**, the lower body lacks systemic blood supply, leading to **shock** and **organ damage**.

Q: Which one of the following does not produce cyanosis in the first year of life -

Atrial septal defect. ***Atrial septal defect*** - An **atrial septal defect (ASD)** typically causes a **left-to-right shunt**, leading to increased blood flow to the lungs rather than systemic hypoxemia, thus not producing cyanosis. - Cyanosis in an ASD usually only occurs if there is significant **pulmonary hypertension** leading to shunt reversal (Eisenmenger syndrome), which is rare in the first year of life. *Truncus arteriosus* - **Truncus arteriosus** involves a single great artery arising from both ventricles, causing a mixing of systemic and pulmonary venous blood. - This mixing results in a significant amount of **deoxygenated blood** entering the systemic circulation, leading to severe cyanosis. *Double outlet right ventricle* - In **double outlet right ventricle (DORV)**, both great arteries (aorta and pulmonary artery) arise mainly from the right ventricle. - Depending on the relationship of the great arteries and the presence of a **ventricular septal defect (VSD)** location, significant deoxygenated blood can enter the systemic circulation, causing cyanosis. *Hypoplastic left heart syndrome* - **Hypoplastic left heart syndrome (HLHS)** is characterized by a severely underdeveloped left ventricle, aorta, and mitral valve. - This condition necessitates a right-to-left shunt through a patent foramen ovale or ductus arteriosus to supply systemic circulation with mixed blood, leading to severe and progressive cyanosis shortly after birth.

Q: All of the following about PDA are true except

More common in males. ***More common in males*** - **Patent Ductus Arteriosus (PDA)** is **more common in females** (female to male ratio of 2:1), thus this statement is incorrect. - The increased prevalence in females is thought to be due to hormonal influences during fetal development. *Common heart lesion in rubella* - PDA is indeed a **common cardiac anomaly associated with congenital rubella syndrome**, especially when infection occurs during the first trimester. - The rubella virus can interfere with the normal closure mechanisms of the ductus arteriosus. *Treatment is closure of defect by ligation and division of ductus* - **Surgical ligation and division of the ductus arteriosus** is a definitive treatment for PDA, particularly in older children or adults. - In neonates, especially premature infants, **indomethacin** (a prostaglandin inhibitor) is often the first-line treatment to induce closure. *Hypoxia and immaturity are important in maintaining the patency* - **Prematurity** is a major risk factor for PDA because the ductus arteriosus in premature infants is less responsive to oxygen-induced constriction and has immature smooth muscle. - **Hypoxia** during fetal life and immediately after birth contributes to maintaining ductal patency by preventing the usual increase in oxygen levels that triggers closure.

Q: In morbus caeruleus (cyanotic congenital heart defects), foramen ovale typically closes:

Variable depending on defect. ***Variable depending on defect*** - In **morbus caeruleus** (cyanotic congenital heart defects), the closure of the **foramen ovale** depends on the specific cardiac defect and its hemodynamics. - In defects like **tricuspid atresia**, **pulmonary atresia with intact ventricular septum**, or **severe Ebstein's anomaly**, the foramen ovale must remain patent for survival, allowing essential right-to-left shunting. - In defects like **Tetralogy of Fallot** (the most common cyanotic CHD), the foramen ovale typically closes normally as the shunt occurs at the ventricular level. - **Clinical significance:** The need for PFO patency correlates with the degree of obstruction to pulmonary blood flow at the tricuspid or pulmonary valve level. *Never* - While some cyanotic heart defects require a **patent foramen ovale (PFO)** for survival, many cyanotic defects allow normal closure. - Stating "never" is an overgeneralization that doesn't account for conditions like **Tetralogy of Fallot** where foramen ovale closure typically occurs normally. *6-12 months* - This represents the normal timeline for **anatomical closure** of the foramen ovale in healthy infants after functional closure occurs shortly after birth. - In cyanotic heart defects requiring persistent shunting, this normal timeline is disrupted, but in others (like TOF), closure may proceed on schedule. *2-3 months* - This timeframe relates to early postnatal development when functional closure has occurred and anatomical fusion is progressing. - The actual closure pattern in **morbus caeruleus** is not determined by a fixed timeline but by the underlying cardiac anatomy and hemodynamics.

Q: Which is the most common congenital cardiac defect?

Ventricular septal defect. ***Ventricular septal defect*** - **Ventricular septal defect (VSD)** is the most common form of congenital cardiac defect, occurring in approximately 2 to 6 per 1000 live births. - It involves a **hole in the septum** separating the left and right ventricles, allowing oxygenated blood to shunt from the left to the right side of the heart. *Atrial septal defect* - While common, an **atrial septal defect (ASD)** is less frequent than VSD, typically accounting for about 10% of congenital heart diseases. - It involves a **hole in the septum** between the left and right atria, leading to a left-to-right shunt. *TAPVC* - **Total anomalous pulmonary venous connection (TAPVC)** is a rare and severe congenital heart defect where all four pulmonary veins drain abnormally. - It accounts for only about 1% of all congenital cardiac defects. *Transposition of great arteries* - **Transposition of the great arteries (TGA)** is another relatively rare and critical congenital heart defect. - In TGA, the **aorta originates from the right ventricle** and the **pulmonary artery from the left ventricle**, leading to two separate circulatory systems.

Q: Pulmonary plethora in a child presenting with cyanosis, is seen in?

Total Anomalous Pulmonary Venous Connection (TAPVC). ***Total Anomalous Pulmonary Venous Connection (TAPVC)*** - In **non-obstructed TAPVC**, all pulmonary veins drain anomalously into the right atrium (or its tributaries) instead of the left atrium. - This causes **complete mixing of oxygenated pulmonary venous blood with deoxygenated systemic venous blood** in the right atrium → **cyanosis**. - Since an obligatory **atrial septal defect (ASD)** allows blood to reach the left heart, and there is **increased volume load on the right heart**, there is **increased pulmonary blood flow → pulmonary plethora** on chest X-ray. - Key point: **Obstructed TAPVC** causes pulmonary venous congestion and oligemia, NOT plethora. *Coarctation of the aorta* - **Coarctation of the aorta** is an **acyanotic** congenital heart disease involving systemic outflow obstruction. - It does **not cause cyanosis** unless there is differential cyanosis (lower body only) with a PDA and pulmonary hypertension causing right-to-left shunt. - Does not cause pulmonary plethora. *Tetralogy of Fallot (TOF)* - **Tetralogy of Fallot** presents with **cyanosis** due to right-to-left shunting through a VSD. - However, it has **pulmonary oligemia (decreased pulmonary blood flow)** due to right ventricular outflow tract obstruction and pulmonary stenosis. - Chest X-ray shows **boot-shaped heart** with decreased pulmonary vascular markings, NOT plethora. *Tricuspid Atresia (TA)* - **Tricuspid atresia** causes **cyanosis** due to obligatory right-to-left shunting at the atrial level. - Pulmonary blood flow is typically **decreased or normal** (depending on presence of VSD/PDA), NOT increased. - Does not typically cause pulmonary plethora.

Q: A 6-year-old with congenital heart disease presents with fever, new-onset murmur, and petechiae. Blood cultures are pending, but initial Gram stain shows Gram-positive cocci. What is the most appropriate initial intervention?

Start broad-spectrum antibiotics. ***Start broad-spectrum antibiotics*** - The presentation of **fever**, **new-onset murmur**, **petechiae**, and **Gram-positive cocci** in a patient with **congenital heart disease** is highly suggestive of **infective endocarditis**. - Prompt initiation of **broad-spectrum antibiotics** is crucial to prevent further damage to the heart valves and systemic complications while awaiting definitive culture results. *Schedule for urgent valve replacement* - **Valve replacement** is a definitive treatment for severe valvular damage but is typically considered after initial medical management has failed or in cases of severe complications like heart failure or recurrent emboli. - It is not the initial intervention for suspected infective endocarditis. *Administer high-dose steroids* - **Steroids** are anti-inflammatory but are not indicated in the treatment of active bacterial infections like endocarditis. - Administering steroids could potentially worsen the infection by suppressing the immune response. *Wait for susceptibility testing* - **Waiting for susceptibility testing** to initiate treatment would delay critical care, allowing the infection to progress and increasing morbidity and mortality. - Initial treatment should be empiric, and antibiotics can be narrowed once susceptibility results are available.

Q: A 2-year-old presents with fever, cervical lymphadenopathy, strawberry tongue, and desquamating rash on fingers. What is the cardiac complication?

Coronary artery aneurysm. ***Coronary artery aneurysm*** - The constellation of **fever**, **cervical lymphadenopathy**, **strawberry tongue**, and **desquamating rash** is highly suggestive of **Kawasaki disease**. - **Coronary artery aneurysm** is the most significant and *life-threatening cardiac complication* of untreated or severe Kawasaki disease, leading to long-term morbidity and mortality. *Pericarditis* - While pericarditis can occur in some systemic inflammatory conditions, it is **not the hallmark cardiac complication** of Kawasaki disease. - Pericarditis is characterized by **chest pain** and **pericardial friction rub**, which are not typically the primary concerns in Kawasaki disease management. *Endocarditis* - **Infective endocarditis** involves inflammation of the *inner lining of the heart and heart valves*, often caused by bacterial or fungal infections. - It is **not a typical complication of Kawasaki disease**, which is a vasculitis, not primarily an endocardial infection. *Myocarditis* - Myocarditis (inflammation of the heart muscle) can occur in various viral infections, and mild myocarditis can be present in Kawasaki disease. - However, **coronary artery aneurysms** represent the most *specific and critical cardiac complication* that requires careful monitoring and treatment to prevent rupture or thrombosis.

Q: A child with Tetralogy of Fallot develops worsening cyanosis and fainting spells. Which immediate intervention is recommended?

Knee-chest position. ***Knee-chest position*** - The **knee-chest position** increases **systemic vascular resistance (SVR)**, which helps to reduce the right-to-left shunting of deoxygenated blood through the ventricular septal defect (VSD) in Tetralogy of Fallot. - This maneuver effectively alleviates **hypercyanotic spells** (tet spells) by increasing pulmonary blood flow and improving oxygenation. *Morphine* - While morphine can be used to sedate and reduce infundibular spasm during a tet spell, it is typically administered *after* initial attempts to increase SVR, such as the knee-chest position. - Its primary role is to reduce anxiety and calm the child, which can indirectly help, but it's not the immediate mechanical intervention of choice. *Oxygen therapy* - **Oxygen therapy** can be beneficial by increasing the partial pressure of oxygen in the blood, which may help to alleviate cyanosis. - However, in a severe tet spell, the primary issue is reduced pulmonary blood flow due to an outflow obstruction and right-to-left shunting, so simply providing oxygen without addressing the shunting is often insufficient as an immediate, stand-alone intervention. *IV fluids* - **IV fluids** are important for maintaining adequate **preload** and preventing dehydration, especially if the child is hyperpneic or agitated. - While supportive, IV fluids do not directly address the underlying pathophysiology of a tet spell, which involves a dynamic right ventricular outflow tract obstruction and right-to-left shunting.

Question 1

Which of the following is NOT a ductus dependent congenital heart disease?

Accepted Answer

Truncus arteriosus

Answer

Tricuspid atresia

Answer

Tetralogy of Fallot with pulmonary atresia

Answer

Interrupted Aortic Arch

Question 2

Which one of the following does not produce cyanosis in the first year of life -

Accepted Answer

Atrial septal defect

Answer

Truncus arteriosus

Answer

Double outlet right ventricle

Answer

Hypoplastic left heart syndrome

Question 3

All of the following about PDA are true except

Accepted Answer

More common in males

Answer

Common heart lesion in rubella

Answer

Treatment is closure of defect by ligation and division of ductus

Answer

Hypoxia and immaturity are important in maintaining the patency

Question 4

In morbus caeruleus (cyanotic congenital heart defects), foramen ovale typically closes:

Accepted Answer

Variable depending on defect

Answer

6-12 months

Answer

2-3 months

Answer

Never

Question 5

Which is the most common congenital cardiac defect?

Accepted Answer

Ventricular septal defect

Answer

Atrial septal defect

Answer

TAPVC

Answer

Transposition of great arteries

Question 6

Pulmonary plethora in a child presenting with cyanosis, is seen in?

Accepted Answer

Total Anomalous Pulmonary Venous Connection (TAPVC)

Answer

Coarctation of the aorta

Answer

Tetralogy of Fallot (TOF)

Answer

Tricuspid Atresia (TA)

Question 7

A 16-day-old baby girl is brought to the emergency department appearing ill. On examination, she has pallor and dyspnea with a respiratory rate of 85 per minute. Her heart rate is 200 bpm, heart sounds are distant, and a gallop is heard. Chest X-ray shows cardiomegaly. Echocardiogram reveals dilated ventricles and dilation of the left atrium. ECG shows ventricular depolarization complexes with low voltage. What is the most likely underlying diagnosis?

Accepted Answer

Congestive Heart Failure secondary to congenital heart disease

Answer

Hypoplastic Left Heart Syndrome

Answer

Critical Aortic Stenosis

Answer

Total Anomalous Pulmonary Venous Return

Question 8

A 6-year-old with congenital heart disease presents with fever, new-onset murmur, and petechiae. Blood cultures are pending, but initial Gram stain shows Gram-positive cocci. What is the most appropriate initial intervention?

Accepted Answer

Start broad-spectrum antibiotics

Answer

Schedule for urgent valve replacement

Answer

Administer high-dose steroids

Answer

Wait for susceptibility testing

Question 9

A 2-year-old presents with fever, cervical lymphadenopathy, strawberry tongue, and desquamating rash on fingers. What is the cardiac complication?

Accepted Answer

Coronary artery aneurysm

Answer

Pericarditis

Answer

Endocarditis

Answer

Myocarditis

Question 10

A child with Tetralogy of Fallot develops worsening cyanosis and fainting spells. Which immediate intervention is recommended?

Accepted Answer

Knee-chest position

Answer

Morphine

Answer

Oxygen therapy

Answer

IV fluids

Cardiology — MCQs

Cardiology — MCQs

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