Cardiology Practice Questions

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.

Q: Which symptom is not typically associated with Kawasaki disease?

Hearing loss. ***Hearing loss*** - While various complications can arise from Kawasaki disease, **sensorineural hearing loss** is not a typical manifestation or diagnostic criterion. - Hearing loss is more commonly associated with conditions like **bacterial meningitis** or certain genetic syndromes, which are distinct from Kawasaki disease. *Coronary artery aneurysms* - **Coronary artery aneurysms** are a very serious and common complication of **untreated** or severe Kawasaki disease, leading to potential long-term cardiac issues. - This symptom is a major concern in the management of affected children due to the risk of **myocardial infarction** and future cardiac events. *Strawberry tongue* - **Strawberry tongue** (red, swollen, and bumpy tongue) is a characteristic mucosal change seen in Kawasaki disease, often appearing during the acute phase. - This finding is one of the key diagnostic criteria, reflecting the widespread **inflammation** associated with the illness. *Prolonged fever* - **Prolonged fever** (lasting at least five days) is the hallmark diagnostic criterion for Kawasaki disease, indicating a systemic inflammatory response. - The fever is typically **high-spiking** and unresponsive to antipyretics, driving the need for prompt evaluation and treatment.

Q: A child with Down syndrome presents with heart failure and a systolic murmur. What is the most likely cardiac lesion?

Atrioventricular septal defect. ***Atrioventricular septal defect*** - This is the **most common cardiac lesion** in children with Down syndrome, accounting for approximately 40-50% of defects. - It involves a defect in both the **atrial and ventricular septa** and often an abnormality of the **atrioventricular valves**, leading to significant shunting and early heart failure. *Ventricular septal defect* - While a common congenital heart defect, isolated **ventricular septal defects (VSDs)** are less frequently associated with Down syndrome compared to AVSDs. - A VSD alone would typically present as a harsh **systolic murmur** but may not cause early heart failure unless very large. *Atrial septal defect* - An **atrial septal defect (ASD)** is also a common congenital heart defect, but it is typically asymptomatic in early childhood and less likely to cause heart failure immediately. - The murmur associated with an ASD is usually a **systolic ejection murmur** at the upper left sternal border due to increased pulmonary flow, not necessarily a pansystolic murmur. *Patent ductus arteriosus* - A **patent ductus arteriosus (PDA)** can cause heart failure, but it is less common as the primary lesion in children with Down syndrome compared to AVSD. - The characteristic murmur of a PDA is a continuous **"machinery-like" murmur** rather than a purely systolic murmur as described.

Q: A 5-year-old child presents with shortness of breath, hepatomegaly, and elevated jugular venous pressure. What is the most likely diagnosis?

Congestive heart failure. ***Congestive heart failure*** - The combination of **shortness of breath**, **hepatomegaly**, and **elevated jugular venous pressure** in a 5-year-old child are classic signs of fluid overload due to **congestive heart failure**. - **Hepatomegaly** and **elevated jugular venous pressure** indicate systemic venous congestion, while **shortness of breath** points to pulmonary edema, common manifestations of heart failure. *Asthma* - While asthma can cause **shortness of breath**, it does not typically present with **hepatomegaly** or **elevated jugular venous pressure**. - Asthma is characterized by **bronchospasm** and airway inflammation, often with a history of recurrent wheezing. *Cystic fibrosis* - **Cystic fibrosis** primarily affects the respiratory and digestive systems, leading to chronic lung disease, malabsorption, and growth failure. - While it can cause **respiratory distress** due to mucus plugging, it is not typically associated with **acute hepatomegaly** or **elevated jugular venous pressure** as primary signs in this manner. *Pericarditis* - **Pericarditis** involves inflammation of the pericardium and can cause chest pain, fever, and sometimes **pericardial effusion**. - While severe pericardial effusion can lead to **cardiac tamponade** and elevated JVP, it is less likely to cause isolated hepatomegaly without other classic signs of chest pain or friction rub.

Q: A newborn exhibits cyanosis, tachypnea, and a murmur. Which diagnostic test would most accurately identify the underlying cardiac anomaly?

Echocardiogram. ***Echocardiogram*** - An **echocardiogram** provides a real-time, detailed view of the **heart's structure and function**, allowing for direct visualization of cardiac anomalies, blood flow patterns, and chamber sizes. - It is a **non-invasive** and highly accurate method for diagnosing congenital heart diseases, making it the most suitable initial diagnostic test for a newborn with cyanosis, tachypnea, and a murmur. *Chest X-ray (CXR)* - A CXR can show **cardiac silhouette size** and **pulmonary vascular markings**, which might suggest a cardiac anomaly but does not provide details on specific structural defects. - It is often used as an initial screening tool but lacks the resolution to accurately identify the underlying cardiac anomaly. *Electrocardiogram* - An **ECG** evaluates the **electrical activity of the heart**, detecting arrhythmias, chamber enlargement, and myocardial ischemia. - While it can provide clues about cardiac stress or hypertrophy, it cannot visualize the anatomical structure of the heart or precisely identify specific congenital anomalies. *Cardiac catheterization (invasive)* - **Cardiac catheterization** is an **invasive procedure** that provides very detailed information on blood pressures and oxygen saturation within the heart chambers, and can also be therapeutic. - However, due to its invasiveness and associated risks, it is typically reserved for cases where echocardiography is inconclusive, for pre-surgical planning, or for therapeutic interventions, not as the primary diagnostic tool in a neonate with suspected congenital heart disease.

Q: A 2-month-old infant presents with tachypnea and a harsh systolic murmur best heard over the left sternal border. Which congenital heart defect is most likely?

Ventricular septal defect. ***Ventricular septal defect*** - A **harsh systolic murmur** heard best at the **left sternal border** is a classic finding in VSD due to blood shunting from the left to the right ventricle. - **Tachypnea in an infant** with VSD often indicates **pulmonary overcirculation** and developing heart failure due to the significant left-to-right shunt. *Tetralogy of Fallot* - This condition typically presents with **cyanosis** and a **crescendo-decrescendo systolic ejection murmur** heard at the upper left sternal border due to right ventricular outflow tract obstruction. - While tachypnea can occur, the characteristic **cyanotic spells** (tet spells) and absence of a harsh VSD murmur make it less likely. *Atrial septal defect* - An ASD typically causes a **systolic ejection murmur** at the **pulmonic area** (second intercostal space, left sternal border) due to increased flow across the pulmonic valve, not a harsh murmur at the left sternal border. - Significant respiratory distress like tachypnea is less common in infancy unless there are other associated defects or severe pulmonary hypertension. *Coarctation of the aorta* - This defect presents with **blood pressure differences** between the upper and lower extremities and often a **systolic murmur** heard in the back or left infraclavicular area. - While it can cause heart failure symptoms including tachypnea, the murmur description and location are not typical for coarctation.

Q: A 6-year-old child presents with signs of congestive heart failure. An echocardiogram shows a complete atrioventricular septal defect. Which syndrome is most commonly associated with this congenital heart defect?

Down syndrome. **Down syndrome** - **Complete atrioventricular septal defect (AVSD)**, also known as endocardial cushion defect, is the most common and classic congenital heart defect seen in children with **Down syndrome (Trisomy 21)**. - It accounts for approximately 40-50% of the congenital heart diseases found in individuals with Down syndrome, often leading to early signs of **congestive heart failure**. *Turner syndrome* - **Turner syndrome (45, XO)** is most commonly associated with **coarctation of the aorta** and **bicuspid aortic valve**. - While other heart defects can occur, an atrioventricular septal defect is not the most common or characteristic finding. *Noonan syndrome* - **Noonan syndrome** is frequently associated with **pulmonary stenosis** (especially dysplastic pulmonary valve) and **hypertrophic cardiomyopathy**. - **AVSD** is not a common cardiac anomaly in patients with Noonan syndrome. *DiGeorge syndrome* - **DiGeorge syndrome** (22q11.2 deletion syndrome) is typically linked to defects involving the **conotruncal region** of the heart, such as **truncus arteriosus**, **interrupted aortic arch**, and **tetralogy of Fallot**. - An atrioventricular septal defect is not the primary cardiac anomaly associated with this syndrome.

Question 1

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

Accepted Answer

Rash

Answer

Edema

Answer

Purulent conjunctivitis

Answer

Strawberry tongue

Question 2

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 3

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

Question 4

Which symptom is not typically associated with Kawasaki disease?

Accepted Answer

Hearing loss

Answer

Strawberry tongue

Answer

Prolonged fever

Answer

Coronary artery aneurysms

Question 5

A child with Down syndrome presents with heart failure and a systolic murmur. What is the most likely cardiac lesion?

Accepted Answer

Atrioventricular septal defect

Answer

Ventricular septal defect

Answer

Atrial septal defect

Answer

Patent ductus arteriosus

Question 6

A 5-year-old child presents with shortness of breath, hepatomegaly, and elevated jugular venous pressure. What is the most likely diagnosis?

Accepted Answer

Congestive heart failure

Answer

Asthma

Answer

Cystic fibrosis

Answer

Pericarditis

Question 7

A child with Tetralogy of Fallot presents with cyanosis. Which imaging modality is essential for surgical planning?

Accepted Answer

Echocardiography

Answer

Chest X-ray

Answer

CT scan

Answer

MRI

Question 8

A newborn exhibits cyanosis, tachypnea, and a murmur. Which diagnostic test would most accurately identify the underlying cardiac anomaly?

Accepted Answer

Echocardiogram

Answer

Chest X-ray (CXR)

Answer

Electrocardiogram

Answer

Cardiac catheterization (invasive)

Question 9

A 2-month-old infant presents with tachypnea and a harsh systolic murmur best heard over the left sternal border. Which congenital heart defect is most likely?

Accepted Answer

Ventricular septal defect

Answer

Tetralogy of Fallot

Answer

Atrial septal defect

Answer

Coarctation of the aorta

Question 10

A 6-year-old child presents with signs of congestive heart failure. An echocardiogram shows a complete atrioventricular septal defect. Which syndrome is most commonly associated with this congenital heart defect?

Accepted Answer

Down syndrome

Answer

Turner syndrome

Answer

Noonan syndrome

Answer

DiGeorge syndrome

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