Cardiology Practice Questions

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.

Q: Which one of the following is a criterion of Kawasaki disease?

Rash. ***Rash*** - A **polymorphous rash**, which can be macular, papular, or scarlatiniform, is one of the **five principal diagnostic criteria** for **Kawasaki disease**. - This rash typically appears early in the course of the illness and can affect any part of the body, often involving the trunk and extremities. *Edema* - **Edema of the hands and feet**, especially when accompanied by **erythema** (redness), is actually one of the **principal diagnostic criteria** for Kawasaki disease under "extremity changes." - This finding typically occurs in the acute phase, followed by **desquamation** (peeling) in the convalescent phase, particularly in the periungual region. - Note: While edema is a valid criterion, **rash** is considered the most characteristic and commonly used criterion among the options listed. *Purulent conjunctivitis* - **Kawasaki disease** characteristically presents with **bilateral non-purulent (non-exudative) conjunctival injection** - red eyes without discharge or exudate. - **Purulent conjunctivitis** (conjunctivitis with pus/discharge) indicates a bacterial infection and actually argues **against** the diagnosis of Kawasaki disease. - This is the only option that is definitively **not** a criterion. *Strawberry tongue* - **Strawberry tongue** (red, swollen tongue with prominent papillae) is part of the **oral changes criterion** in Kawasaki disease, which includes red cracked lips, strawberry tongue, and erythema of the oropharyngeal mucosa. - While also seen in scarlet fever and toxic shock syndrome, strawberry tongue is a **recognized feature** of Kawasaki disease. - Note: This is technically a valid criterion, though less specific than the polymorphous rash.

Q: What is the pathophysiologic basis for cyanosis in congenital heart disease?

Decreased oxygenation from right-to-left shunt. ***Decreased oxygenation from right-to-left shunt*** - In cyanotic congenital heart disease, a **right-to-left shunt** allows **deoxygenated blood** from the right side of the heart to bypass the lungs and enter the systemic circulation directly. - This results in **decreased systemic arterial oxygen saturation (hypoxemia)**, which causes the characteristic bluish discoloration of the skin and mucous membranes known as **cyanosis**. - Cyanosis becomes clinically visible when **deoxygenated hemoglobin exceeds 3-5 g/dL** in capillary blood, which occurs due to the mixing of deoxygenated and oxygenated blood. *Increased pulmonary blood flow due to left-to-right shunt* - A **left-to-right shunt** (seen in ASD, VSD, PDA) directs oxygenated blood from the left heart back into the pulmonary circulation. - This causes **acyanotic heart disease** with increased pulmonary blood flow, potentially leading to heart failure or pulmonary hypertension, but **not cyanosis**. *Increased systemic vascular resistance* - Increased **systemic vascular resistance (SVR)** affects cardiac afterload and blood pressure but does not directly cause cyanosis. - While elevated SVR may worsen right-to-left shunting in certain conditions (e.g., Tetralogy of Fallot), it is not the pathophysiologic basis for cyanosis. *Right-to-left shunt* - While a **right-to-left shunt** is the anatomic/structural abnormality that permits deoxygenated blood to enter systemic circulation, the question asks for the **pathophysiologic basis** (the functional consequence). - The shunt itself is the mechanism, but **decreased oxygenation resulting from the shunt** is what directly produces the clinical finding of cyanosis.

Question 1

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 2

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 3

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 4

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 5

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 6

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 7

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

Question 8

Which one of the following is a criterion of Kawasaki disease?

Accepted Answer

Rash

Answer

Edema

Answer

Purulent conjunctivitis

Answer

Strawberry tongue

Question 9

What is the pathophysiologic basis for cyanosis in congenital heart disease?

Accepted Answer

Decreased oxygenation from right-to-left shunt

Answer

Increased pulmonary blood flow due to left-to-right shunt

Answer

Increased systemic vascular resistance

Answer

Right-to-left shunt

Question 10

What is the most appropriate management step for a 6-year-old child with a history of repaired tetralogy of Fallot who presents with exercise intolerance, palpitations, and cyanosis, and has an ECG showing ventricular arrhythmias?

Accepted Answer

Holter monitoring and beta-blocker therapy

Answer

Repeat surgical intervention for tetralogy of Fallot

Answer

Catheter ablation and ACE inhibitors

Answer

Electrophysiological study followed by ICD placement

Cardiology — MCQs

Cardiology — MCQs

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