Cardiology Practice Questions

Q: Which of the following vasculitides is predominantly seen in children?

Kawasaki disease. ***Kawasaki disease*** - This is the **most common vasculitis in children**, with approximately 90% of cases occurring in children **under 5 years of age**. - It is **predominantly a pediatric condition** and rarely occurs in adults. - Characterized by fever lasting more than 5 days, along with conjunctivitis, oral changes (strawberry tongue, cracked lips), rash, cervical lymphadenopathy, and extremity changes. - The major complication is **coronary artery aneurysms**, which can lead to myocardial infarction if untreated. - Treatment with **IVIG and aspirin** reduces the risk of coronary complications. *Henoch-Schönlein purpura (HSP)* - While this is a **common vasculitis in children** (peak age 4-6 years), it also occurs in adults. - Presents with palpable purpura, arthritis, abdominal pain, and glomerulonephritis. - Caused by **IgA-mediated immune complex deposition** in small vessels. - However, it is not as **exclusively pediatric** as Kawasaki disease. *Takayasu arteritis* - A **large vessel vasculitis** affecting the aorta and its major branches. - Predominantly affects **young women** between 10-40 years of age, not specifically children. - Presents with absent pulses, hypertension, and vascular bruits. *Susac syndrome* - A rare microangiopathy affecting the brain, retina, and inner ear. - Typically occurs in **young adults** (mean age 30-40 years), not in children. - Characterized by encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss.

Q: Which of the following congenital heart defects is least likely to cause recurrent pulmonary infections?

Tetralogy of Fallot (TOF). ***Tetralogy of Fallot (TOF)*** - This is a **cyanotic heart defect** characterized by **right-to-left shunting** of blood, leading to reduced pulmonary blood flow. - Reduced pulmonary blood flow means less blood congests the lungs, thus decreasing the risk of **pulmonary edema** and subsequent infections. *Ventricular Septal Defect (VSD)* - VSD results in a **left-to-right shunt**, increasing blood flow to the pulmonary artery and causing **pulmonary overcirculation**. - This **pulmonary congestion** makes the lungs more susceptible to recurrent infections due to increased fluid and pressure in the pulmonary vasculature. *Patent Ductus Arteriosus (PDA)* - PDA also causes a **left-to-right shunt** from the aorta to the pulmonary artery, leading to **pulmonary overcirculation**. - The increased blood flow and pressure in the pulmonary system contribute to **pulmonary edema** and heightened risk of respiratory infections. *Atrial Septal Defect (ASD)* - An ASD typically causes a **left-to-right shunt** at the atrial level, increasing blood flow to the lungs and resulting in **pulmonary overcirculation**. - While generally less severe than VSD or PDA, significant pulmonary blood flow can still predispose individuals to recurrent **pulmonary infections**.

Q: Which of the following is the most common cause of syncope in children?

Neurocardiogenic syncope. ***Neurocardiogenic syncope*** - This is the **most common cause of syncope** in children and adolescents, often triggered by prolonged standing, pain, or emotional stress. - It results from a **reflex-mediated drop in heart rate and blood pressure**, leading to temporary cerebral hypoperfusion. *Breath holding spells* - While common in infants and toddlers (6 months to 6 years), these are typically **self-limiting, benign events** related to anger or pain, and not the most common cause of syncope across all pediatric ages. - They are characterized by **cyanosis** or pallor followed by a brief loss of consciousness, but differ from true syncope in their underlying mechanism. *Hypoglycemia* - Although it can cause **lightheadedness, confusion, and sometimes loss of consciousness**, it is not the most frequent cause of syncope in generally healthy children without underlying metabolic disorders or diabetes. - Diagnosis requires demonstrating **low blood sugar levels** at the time of the event. *Hypovolemia* - This can cause syncope due to **decreased circulating blood volume** and reduced cerebral perfusion, often seen in cases of severe dehydration or hemorrhage. - However, in the general pediatric population, it is a **less common cause of syncope** compared to neurocardiogenic mechanisms.

Q: A five-day-old, full-term male infant who was severely cyanotic at birth, showed improvement in oxygenation after initial administration of prostaglandin E1 and subsequent balloon atrial septostomy, is most likely diagnosed with which of the following conditions?

Transposition of great vessels. ***Transposition of great vessels*** - Severe **cyanosis at birth** that improves with **prostaglandin E1 (PGE1)** and **balloon atrial septostomy** is highly characteristic of transposition of the great arteries (TGA), as these interventions improve mixing of oxygenated and deoxygenated blood. - PGE1 maintains **ductus arteriosus** patency, while balloon atrial septostomy creates or enlarges an **atrial septal defect**, both crucial for survival in TGA by allowing blood mixing. *Tetralogy Fallot* - While it causes cyanosis, Tetralogy of Fallot typically presents with **hypercyanotic spells** that worsen with activity and is not typically managed acutely with a balloon atrial septostomy for severe immediate cyanosis at birth. - The primary defect in Tetralogy of Fallot is **right ventricular outflow tract obstruction**, which is not directly addressed by PGE1 or atrial septostomy as definitive treatment. *Truncus Arteriosus* - Truncus arteriosus involves a single great artery overriding the ventricular septum and typically results in severe **congestive heart failure** and **pulmonary overcirculation** rather than severe, isolated cyanosis at birth requiring PGE1 and atrial septostomy for acute improvement. - Cyanosis may be present but is usually not the predominant life-threatening feature in the immediate newborn period in the same way as TGA. *Tricuspid Atresia* - Although tricuspid atresia causes cyanosis due to an absent tricuspid valve, it often presents with **right ventricular hypoplasia** and requires either an atrial septal defect or patent foramen ovale for survival, which may be enlarged by septostomy. - However, the severity of cyanosis and the dramatic response to both PGE1 and septostomy, particularly in a *severe* cyanotic newborn, is more indicative of the circulatory defect in TGA.

Q: What is the most common atrial septal defect in Down syndrome?

Ostium primum. ***Ostium primum*** - **Ostium primum atrial septal defects** are the most common type of atrial septal defect seen in individuals with **Down syndrome** (Trisomy 21). - This defect is typically associated with **atrioventricular septal defects (AVSDs)**, also known as **endocardial cushion defects**, which are highly prevalent in Down syndrome. *Ostium secondum* - **Ostium secundum ASDs** are the most common type of **isolated** atrial septal defect in the general population, not specifically in Down syndrome. - While they can occur in Down syndrome, they are less characteristic than ostium primum defects which constitute part of the AVSD spectrum. *Absent atrial septum* - An **absent atrial septum**, or **common atrium**, is a very rare and severe cardiac anomaly. - It is not considered the most common type of atrial septal defect in Down syndrome, although individuals with Down syndrome can have complex congenital heart defects. *Sinus venosum* - **Sinus venosum ASDs** are less common and are typically located near the superior or inferior vena cava. - They are often associated with **anomalous pulmonary venous return** but are not the predominant type of ASD in Down syndrome.

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

Ventricular septal defect (VSD). ***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.

Q: Most common cardiac anomaly in Turner's syndrome?

Coarctation of aorta. ***Coarctation of aorta*** - **Coarctation of the aorta** is considered the **most common clinically significant cardiac anomaly** in Turner syndrome, occurring in **10-15%** of patients - It involves narrowing of the aorta, typically **near the ductus arteriosus** (juxtaductal), leading to upper extremity hypertension and left ventricular hypertrophy - From a **clinical and examination perspective**, coarctation is emphasized as the classic cardiac association with Turner syndrome due to its diagnostic and therapeutic importance - Often found in conjunction with bicuspid aortic valve *Bicuspid aortic valve* - **Bicuspid aortic valve (BAV)** is actually the **most prevalent** cardiac abnormality in Turner syndrome, affecting **30-50%** of individuals - However, it is often **asymptomatic in childhood** and may only manifest with complications (stenosis, regurgitation, endocarditis) later in life - In **examination contexts**, coarctation is typically considered the primary answer due to its greater clinical significance and need for early intervention - BAV is frequently an incidental finding on echocardiography *Ventricular septal defect* - **Ventricular septal defects (VSDs)** are **not characteristic** of Turner syndrome - VSDs are more commonly associated with **Down syndrome** and other chromosomal abnormalities - They involve a hole between the ventricles causing a left-to-right shunt *Atrial septal defect* - **Atrial septal defects (ASDs)**, particularly **partial anomalous pulmonary venous return (PAPVR)**, can occur in Turner syndrome but are less common than coarctation - Standard secundum ASDs are not a primary association with Turner syndrome

Q: A newborn presents with central cyanosis and decreased oxygen saturation. What is the most likely category of diagnosis?

Congenital cyanotic heart defect. **Congenital cyanotic heart defect** - **Central cyanosis** and **decreased oxygen saturation** in a newborn are hallmark signs of **cyanotic congenital heart defects**, which involve right-to-left shunting of deoxygenated blood or inadequate pulmonary blood flow. - These defects lead to arterial hypoxemia, which manifests as a bluish discoloration of the skin and mucous membranes. *Acyanotic congenital heart defect* - **Acyanotic heart defects** typically involve left-to-right shunting (e.g., VSD, ASD) or obstructions (e.g., coarctation), which usually do not cause **central cyanosis** in the newborn period. - While some acyanotic defects can lead to cyanosis later in life due to pulmonary hypertension reversing the shunt (Eisenmenger syndrome), initial presentation in a newborn is not typically cyanotic. *Acquired heart disease* - **Acquired heart disease** in a newborn is rare and usually refers to conditions like myocarditis or endocarditis, which are typically infectious or inflammatory in origin, not congenital structural abnormalities. - While it can manifest with heart failure symptoms, **central cyanosis** in a newborn is far more indicative of a congenital structural anomaly affecting oxygenation. *Transposition of the Great Arteries (TGA)* - **Transposition of the Great Arteries (TGA)** is a specific type of **cyanotic congenital heart defect** where the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. - While TGA is a highly likely diagnosis given the symptoms, the question asks for the most likely *category* of diagnosis, which is the broader "congenital cyanotic heart defect" group that includes TGA.

Q: The MANDATORY diagnostic criterion for Kawasaki disease is which of the following?

Fever. **Fever** - **Fever** lasting at least five days is an **absolute prerequisite** for diagnosing Kawasaki disease. - Without this prolonged fever, other symptoms, even if present, are not sufficient for a diagnosis of Kawasaki disease. *Cervical lymphadenopathy* - While **cervical lymphadenopathy** is one of the classic diagnostic criteria for Kawasaki disease, it is not mandatory. - It is present in a significant number of cases but can be absent, especially in infants. *Bilateral conjunctivitis* - **Bilateral conjunctivitis** is a common diagnostic criterion but is not always present in every patient. - It is typically non-exudative and painless. *Polymorphous rash* - A **polymorphous rash** is a characteristic feature of Kawasaki disease but is not mandatory for diagnosis, as it can be transient or mild. - The rash can take various forms, including maculopapular, scarlatiniform, or erythema multiforme-like.

Q: Which of the following is the MOST COMMON manifestation of congenital rubella syndrome?

Sensory neural hearing loss. ***Sensorineural hearing loss*** - **Sensorineural hearing loss** is the **most common manifestation** of congenital rubella syndrome, affecting **60-90% of infected infants**, and is often the **only manifestation** present. - It can be **unilateral or bilateral** and is typically **permanent**, ranging from mild to profound. - It is the **most frequent sequela** and may be the sole defect in many cases. *Cataract* - While **cataracts** are a classic component of the congenital rubella syndrome **triad** (cataracts, cardiac defects, deafness), they occur in **30-50% of cases**. - They are **less common** than sensorineural hearing loss. - Other ocular manifestations include **retinopathy**, **glaucoma**, and **microphthalmia**. *Congenital heart disease* - **Congenital heart disease**, particularly **patent ductus arteriosus (PDA)** and **peripheral pulmonary artery stenosis**, is a classic manifestation. - It occurs in **40-60% of cases**, making it **less common** than hearing loss. - Cardiovascular defects are part of the classic triad but not the most frequent finding. *None of the options* - This option is incorrect because **sensorineural hearing loss** is the well-established **most common manifestation** of congenital rubella syndrome. - It is the **single most frequent defect** identified in affected children.

Question 1

Which of the following vasculitides is predominantly seen in children?

Accepted Answer

Kawasaki disease

Answer

Takayasu arteritis

Answer

Susac syndrome

Answer

Henoch-Schonlein purpura

Question 2

Which of the following congenital heart defects is least likely to cause recurrent pulmonary infections?

Accepted Answer

Tetralogy of Fallot (TOF)

Answer

Ventricular Septal Defect (VSD)

Answer

Atrial Septal Defect (ASD)

Answer

Patent Ductus Arteriosus (PDA)

Question 3

Which of the following is the most common cause of syncope in children?

Accepted Answer

Neurocardiogenic syncope

Answer

Breath holding spells

Answer

Hypoglycemia

Answer

Hypovolemia

Question 4

A five-day-old, full-term male infant who was severely cyanotic at birth, showed improvement in oxygenation after initial administration of prostaglandin E1 and subsequent balloon atrial septostomy, is most likely diagnosed with which of the following conditions?

Accepted Answer

Transposition of great vessels

Answer

Tetralogy Fallot

Answer

Truncus Arteriosus

Answer

Tricuspid Atresia

Question 5

What is the most common atrial septal defect in Down syndrome?

Accepted Answer

Ostium primum

Answer

Ostium secondum

Answer

Absent atrial septum

Answer

Sinus venosum

Question 6

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

Accepted Answer

Ventricular septal defect (VSD)

Answer

Persistent truncus arteriosus (PTA)

Answer

Common ventricle (CV)

Answer

Atrial septal defect (ASD)

Question 7

Most common cardiac anomaly in Turner's syndrome?

Accepted Answer

Coarctation of aorta

Answer

Ventricular septal defect

Answer

Atrial septal defect

Answer

Bicuspid aortic valve

Question 8

A newborn presents with central cyanosis and decreased oxygen saturation. What is the most likely category of diagnosis?

Accepted Answer

Congenital cyanotic heart defect

Answer

Acyanotic congenital heart defect

Answer

Acquired heart disease

Answer

Transposition of the Great Arteries (TGA)

Question 9

The MANDATORY diagnostic criterion for Kawasaki disease is which of the following?

Accepted Answer

Fever

Answer

Cervical lymphadenopathy

Answer

Bilateral conjunctivitis

Answer

Polymorphous rash

Question 10

Which of the following is the MOST COMMON manifestation of congenital rubella syndrome?

Accepted Answer

Sensory neural hearing loss

Answer

None of the options

Answer

Congenital heart disease

Answer

Cataract

Cardiology — MCQs

Cardiology — MCQs

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