Cardiology Practice Questions

Q: Most common cardiac manifestation of Holt-Oram syndrome is?

ASD. ***ASD*** - **Atrial septal defect (ASD)** is the most frequently observed cardiac anomaly in patients with **Holt-Oram syndrome**. - This syndrome is characterized by upper limb abnormalities (especially radial ray defects) and congenital heart disease, with ASD or VSD being the most common defects, but **ASD is more prevalent overall**. *VSD* - **Ventricular septal defect (VSD)** is another common cardiac manifestation in Holt-Oram syndrome, but it occurs less frequently than ASD [1]. - While both are septal defects, **ASD is quantitatively more common** in this specific syndrome [1]. *tachycardia* - **Tachycardia**, or a fast heart rate, is a symptom or sign and not a structural cardiac manifestation itself. - While patients with **congenital heart defects** may experience arrhythmias, tachycardia is not the primary cardiac abnormality defining Holt-Oram syndrome. *PDA* - **Patent ductus arteriosus (PDA)** is a known congenital heart defect but is **not one of the most common** cardiac manifestations associated with Holt-Oram syndrome. - The characteristic defects in Holt-Oram syndrome primarily involve the **atrial and ventricular septa** [1].

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: 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: A 3-year-old boy is brought by his mother because he has had a high fever for the past week. Physical examination reveals bilateral injected conjunctivae, palmar erythema, oral mucositis, cervical lymphadenopathy, and solar erythema. What is the most likely diagnosis?

Kawasaki disease. ***Kawasaki disease*** - The constellation of **prolonged fever**, **bilateral conjunctival injection**, **oral mucositis** (strawberry tongue, cracked lips), **palmar erythema**, **cervical lymphadenopathy**, and a polymorphous rash (including solar erythema) are classic diagnostic criteria for **Kawasaki disease**. - This condition is an acute **vasculitis** of childhood that can lead to serious complications like **coronary artery aneurysms** if untreated. *Erysipelas* - This is a **superficial cellulitis** characterized by a well-demarcated, raised, red, and warm rash, typically on the face or lower extremities. - It does not present with bilateral conjunctival injection, oral mucositis, or systemic features seen in the patient, beyond fever. *Scarlet fever* - Caused by **Streptococcus pyogenes**, it presents with a characteristic **sandpaper-like rash**, **strawberry tongue**, and **pharyngitis**. - While it can cause fever and a strawberry tongue, it typically does not cause conjunctivitis, striking palmar erythema, or diffuse lymphadenopathy in the pattern observed. *Meningococcemia* - This is a severe systemic infection caused by **Neisseria meningitidis**, often presenting with features of **sepsis** and a **petechial or purpuric rash**. - While it causes high fever and can be rapidly progressive, it lacks the specific mucocutaneous findings like conjunctivitis, oral mucositis, and acral erythema that are characteristic of Kawasaki disease.

Q: Which congenital heart defect is characteristically associated with differential cyanosis?

Total Anomalous Pulmonary Venous Connection (TAPVC). Detailed understanding of congenital heart defects is essential for clinical diagnosis. ***Total Anomalous Pulmonary Venous Connection (TAPVC)*** - TAPVC can occasionally present with **differential cyanosis**, especially when there is **obstruction** to the pulmonary venous return and associated systemic-to-pulmonary shunts or right-to-left shunting at the atrial level. - While less common than in patent ductus arteriosus, severe forms of TAPVC with **pulmonary hypertension** and reversed shunting can lead to disparities in oxygen saturation between upper and lower extremities. *Ebstein anomaly (EA)* - While Ebstein anomaly can cause **cyanosis** due to a **right-to-left shunt** at the atrial level through a patent foramen ovale or ASD, it typically results in **generalized cyanosis**, not differential cyanosis. - The malformation of the **tricuspid valve** leads to atrialization of the right ventricle, causing severe right heart failure and elevated right atrial pressures, but usually uniform desaturation. *Tetralogy of Fallot (TOF)* - TOF is a common cyanotic heart defect characterized by **pulmonary stenosis**, VSD, overriding aorta, and right ventricular hypertrophy, leading to **generalized cyanosis** [1]. - Cyanosis in TOF is due to the **right-to-left shunt** across the VSD, allowing deoxygenated blood to enter the systemic circulation uniformly [1]. *Ventricular Septal Defect (VSD)* - An uncomplicated VSD typically causes a **left-to-right shunt**, leading to **acyanotic congenital heart disease**. - In cases of severe **pulmonary hypertension** (Eisenmenger syndrome), the shunt can reverse to right-to-left, causing generalized cyanosis, rather than differential cyanosis.

Q: In which condition is PGE most commonly used to maintain patent ductus arteriosus in an infant?

Hypoplastic left heart syndrome. ***Hypoplastic left heart syndrome*** - **HLHS** is the most common indication for PGE1 infusion to maintain a patent ductus arteriosus in neonates - In HLHS, the left ventricle and aorta are severely underdeveloped, making the **entire systemic circulation ductus-dependent** - The PDA allows right ventricular output to supply systemic blood flow by shunting blood from the pulmonary artery to the descending aorta - Without PGE1 to maintain ductal patency, these infants develop **severe circulatory shock and acidosis** as the ductus closes naturally - This is a **universally accepted life-saving indication** for PGE1 until surgical intervention (Norwood procedure or transplant) *TGA with intact ventricular septum* - While TGA with intact septum does require mixing of circulations and PGE1 is used, these infants typically have a patent foramen ovale (PFO) that provides some mixing at the atrial level - PGE1 helps maintain the PDA for additional mixing, but the primary issue is **circulatory separation**, not complete dependence on the ductus for systemic flow - Many centers perform urgent balloon atrial septostomy rather than relying solely on PGE1 *Pulmonary stenosis* - **Critical pulmonary stenosis** represents ductus-dependent pulmonary circulation - PGE1 maintains the PDA to provide pulmonary blood flow when right ventricular outflow is severely obstructed - While important, it is less commonly encountered than HLHS as the primary indication for PGE1 therapy *Tricuspid atresia* - In tricuspid atresia, there is no communication between the right atrium and right ventricle - Pulmonary blood flow is ductus-dependent if there is no VSD or if the VSD is restrictive - PGE1 may be required, but this is less common than HLHS overall

Q: Which one of the following conditions is associated with Kawasaki's syndrome?

Coronary artery aneurysm. ***Coronary artery aneurysm*** - **Coronary artery aneurysms** are a major and potentially life-threatening complication, occurring in 15-25% of untreated children with Kawasaki disease [1]. - This is due to the disease's predilection for causing **vasculitis** in medium-sized arteries, particularly the coronary arteries [1]. *Acute rheumatic fever* - Acute rheumatic fever is an **inflammatory disease** that can develop after an infection with Group A Streptococcus (strep throat or scarlet fever) [2]. - It primarily affects the **heart, joints, brain, and skin**, but its pathophysiology and clinical presentation are distinct from Kawasaki disease [2]. *Pulmonary embolus* - A **pulmonary embolus** is a block in one of the pulmonary arteries in your lungs, usually caused by blood clots that travel to the lungs from the legs or, rarely, other parts of the body. - It is not a characteristic feature or complication of **Kawasaki disease**. *Systemic lupus erythematosus* - **Systemic lupus erythematosus (SLE)** is a chronic autoimmune disease that can affect almost any part of the body, leading to a wide range of symptoms. - While it can cause vasculitis, its epidemiology, clinical presentation, and specific organ involvement are distinctly different from **Kawasaki disease**.

Q: Treatment of choice for Kawasaki disease is:

IV Immunoglobulins. ***IV Immunoglobulins*** - **Intravenous immunoglobulins (IVIG)** at a dose of **2 g/kg as a single infusion** are the cornerstone of therapy for Kawasaki disease, significantly reducing the risk of **coronary artery aneurysms** from ~25% to ~5%. - IVIG should be administered along with **high-dose aspirin (80-100 mg/kg/day)** within the first 10 days of fever onset for optimal effectiveness. - This combination therapy is the **gold standard treatment** for acute Kawasaki disease. *Corticosteroids* - While corticosteroids have anti-inflammatory properties, they are generally **not the primary treatment of choice** for acute Kawasaki disease. - They may be considered as **adjunctive therapy in IVIG-resistant cases** (persistent or recurrent fever ≥36 hours after initial IVIG) or in severe disease, but their routine use is debated. *Azathioprine* - **Azathioprine is an immunosuppressant** typically used in autoimmune diseases or organ transplantation. - It is **not indicated** for the acute management of Kawasaki disease. *Methotrexate* - **Methotrexate is an immunosuppressant and anti-inflammatory drug** used in conditions like rheumatoid arthritis and certain cancers. - It is **not used** for the acute treatment of Kawasaki disease.

Q: Infants of diabetic mothers are likely to have the following cardiac anomaly:

Transposition of great arteries. ***Transposition of great arteries*** - **Transposition of the great arteries (TGA)** is one of the most common cardiac anomalies in infants of diabetic mothers. - In TGA, the **aorta arises from the right ventricle** and the **pulmonary artery arises from the left ventricle**, resulting in two parallel circulations. - Infants of diabetic mothers have a **3-5 fold increased risk** of congenital heart defects, with TGA being among the most frequently observed. - Other common cardiac abnormalities in IDMs include **ventricular septal defect (VSD)** and **hypertrophic cardiomyopathy**. *Coarctation of aorta* - While coarctation can occur in infants of diabetic mothers, it is **less specifically associated** with maternal diabetes compared to TGA. - Coarctation involves **narrowing of the aorta**, typically near the ductus arteriosus. - More commonly associated with **Turner syndrome** and **bicuspid aortic valve**. *Fallot's tetralogy* - **Tetralogy of Fallot** is not specifically increased in incidence in infants of diabetic mothers. - It is characterized by four components: **pulmonary stenosis**, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. - Not a characteristic anomaly of maternal diabetes. *Ebstein's anomaly* - This anomaly involves the **tricuspid valve** being displaced into the right ventricle. - **Not associated with maternal diabetes** - instead, it is associated with maternal **lithium use** during pregnancy. - Presents with tricuspid regurgitation and right heart dysfunction.

Question 1

Most common cardiac manifestation of Holt-Oram syndrome is?

Accepted Answer

ASD

Answer

VSD

Answer

tachycardia

Answer

PDA

Question 2

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 3

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 4

Which of the following is a characteristic feature of Barth syndrome?

Accepted Answer

Dilated cardiomyopathy + skeletal myopathy + congenital neutropenia

Answer

Cardiac tamponade + skeletal myopathy + neutropenia

Answer

Restrictive cardiomyopathy + skeletal myopathy + neutropenia

Answer

Restrictive cardiomyopathy + skeletal myopathy + lymphopenia

Question 5

A 3-year-old boy is brought by his mother because he has had a high fever for the past week. Physical examination reveals bilateral injected conjunctivae, palmar erythema, oral mucositis, cervical lymphadenopathy, and solar erythema. What is the most likely diagnosis?

Accepted Answer

Kawasaki disease

Answer

Erysipelas

Answer

Scarlet fever

Answer

Meningococcemia

Question 6

Which congenital heart defect is characteristically associated with differential cyanosis?

Accepted Answer

Total Anomalous Pulmonary Venous Connection (TAPVC)

Answer

Ebstein anomaly (EA)

Answer

Tetralogy of Fallot (TOF)

Answer

Ventricular Septal Defect (VSD)

Question 7

In which condition is PGE most commonly used to maintain patent ductus arteriosus in an infant?

Accepted Answer

Hypoplastic left heart syndrome

Answer

TGA with intact ventricular septum

Answer

Pulmonary stenosis

Answer

Tricuspid atresia

Question 8

Which one of the following conditions is associated with Kawasaki's syndrome?

Accepted Answer

Coronary artery aneurysm

Answer

Acute rheumatic fever

Answer

Pulmonary embolus

Answer

Systemic lupus erythematosus

Question 9

Treatment of choice for Kawasaki disease is:

Accepted Answer

IV Immunoglobulins

Answer

Methotrexate

Answer

Corticosteroids

Answer

Azathioprine

Question 10

Infants of diabetic mothers are likely to have the following cardiac anomaly:

Accepted Answer

Transposition of great arteries

Answer

Coarctation of aorta

Answer

Fallot's tetralogy

Answer

Ebstein's anomaly

Cardiology — MCQs

Cardiology — MCQs

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