Cardiology Practice Questions

Q: A child presenting to the clinic with features of Down syndrome. What is the most common cardiac lesion in children with Down syndrome?

Atrioventricular Septal Defect. ***Atrioventricular Septal Defect*** - **Atrioventricular septal defect (AVSD)**, also known as **endocardial cushion defect**, is the most common cardiac anomaly in children with **Down syndrome**, occurring in about 40-50% of those with congenital heart disease. - It involves defects in both the atrial and ventricular septa, often with a common AV valve, leading to significant **shunting** and potential **pulmonary hypertension**. *VSD* - While **ventricular septal defects (VSDs)** are common congenital heart lesions, they are less prevalent than AVSDs in patients with Down syndrome. - VSDs involve a hole in the septum separating the ventricles, leading to a left-to-right shunt. *Coarctation of aorta* - **Coarctation of the aorta** is a narrowing of the aorta, typically occurring distal to the left subclavian artery. - This lesion is not specifically associated with Down syndrome and is much less common than septal defects in this population. *Transposition of great vessels* - **Transposition of the great arteries (TGA)** involves the aorta arising from the right ventricle and the pulmonary artery from the left ventricle, leading to parallel circulations. - This complex cyanotic heart defect is not typically associated with Down syndrome and has a different embryological origin.

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: A 4-year-old girl is brought to the pediatrician's office, where her father reports that she suddenly became pale and stopped running while playfully chasing her and her pet Chihuahua. After 30 minutes, she was no longer pale and wanted to resume the game. She has never had a previous episode and has never been cyanotic. Her physical examination was normal, as were her chest x-ray and echocardiogram. An ECG shows a specific pattern indicating which of the following?

Wolff-Parkinson-White syndrome. ***Wolff-Parkinson-White syndrome*** - The ECG shows a **short PR interval** and a **delta wave** (slurring of the initial upstroke of the QRS complex), which are classic signs of **pre-excitation** seen in Wolff-Parkinson-White (WPW) syndrome. - The child's symptoms of sudden pallor and stopping activity, followed by spontaneous recovery, suggest a brief episode of **tachycardia** due to WPW, which can cause symptoms stemming from reduced cardiac output. *Paroxysmal ventricular tachycardia* - **Ventricular tachycardia** is characterized by wide, bizarre QRS complexes originating from the ventricles, often without a preceding P wave, which is not clearly depicted in the provided ECG. - While it can cause sudden symptoms like pallor due to reduced cardiac output, the distinctive **delta wave** and **short PR interval** seen in the ECG point away from primary VT and towards pre-excitation. *Stokes-Adams syndrome* - This syndrome involves recurrent episodes of **syncope** due to a transient **heart block** or **bradyarrhythmia**, leading to a sudden decrease in cerebral perfusion. - The ECG in Stokes-Adams syndrome would typically show evidence of severe bradycardia or asystole during an event, which is not consistent with the depicted ECG or the likely tachyarrhythmic nature of the symptoms. *Paroxysmal supraventricular tachycardia* - While **paroxysmal supraventricular tachycardia (PSVT)** is common in children and can cause symptoms like pallor, the ECG would typically show a **narrow QRS complex tachycardia** with absent or abnormal P waves, but **lacks the delta wave and short PR interval** characteristic of WPW. - WPW syndrome is a common cause of PSVT in children, but the ECG findings specifically indicate the underlying Wolff-Parkinson-White pattern, rather than just the generic PSVT.

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 type of VSD in Tetralogy of Fallot is

Perimembranous type. ***Perimembranous type*** - The **perimembranous ventricular septal defect (VSD)** is the most common type encountered in Tetralogy of Fallot. - This defect is located adjacent to the **membranous septum**, often extending into the inlet, outlet, or muscular septum [1]. *Muscular type* - **Muscular VSDs** are located within the muscular portion of the ventricular septum [1]. - While present in some cases of Tetralogy of Fallot, they are significantly less common than perimembranous defects. *Inlet type* - **Inlet VSDs** are located posterior to the septal leaflet of the tricuspid valve, within the inlet septum [1]. - Although possible, they are not the most frequent type of VSD observed in Tetralogy of Fallot. *Outlet type* - **Outlet VSDs** are found beneath the semilunar valves, in the conal or outlet septum [1]. - While relevant to the outflow obstruction in Tetralogy of Fallot, the **perimembranous defect** is the predominant VSD morphology.

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.

Question 1

A child presenting to the clinic with features of Down syndrome. What is the most common cardiac lesion in children with Down syndrome?

Accepted Answer

Atrioventricular Septal Defect

Answer

VSD

Answer

Transposition of great vessels

Answer

Coarctation of aorta

Question 2

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 3

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 4

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 5

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 6

A 4-year-old girl is brought to the pediatrician's office, where her father reports that she suddenly became pale and stopped running while playfully chasing her and her pet Chihuahua. After 30 minutes, she was no longer pale and wanted to resume the game. She has never had a previous episode and has never been cyanotic. Her physical examination was normal, as were her chest x-ray and echocardiogram. An ECG shows a specific pattern indicating which of the following?

Accepted Answer

Wolff-Parkinson-White syndrome

Answer

Paroxysmal ventricular tachycardia

Answer

Stokes-Adams syndrome

Answer

Paroxysmal supraventricular tachycardia

Question 7

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 8

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 9

Most common type of VSD in Tetralogy of Fallot is

Accepted Answer

Perimembranous type

Answer

Muscular type

Answer

Inlet type

Answer

Outlet type

Question 10

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)

Cardiology — MCQs

Cardiology — MCQs

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