Cardiology Practice Questions

Q: What is the most common vasculitis seen in children?

Kawasaki disease. **Explanation:** The correct answer is **Kawasaki Disease (KD)**. While historically Henoch-Schönlein Purpura (HSP) was frequently cited as the most common, modern epidemiological data and global trends identify Kawasaki Disease as the most common primary vasculitis in children, particularly in those under 5 years of age. **Why Kawasaki Disease is correct:** Kawasaki Disease is an acute, self-limiting, medium-vessel vasculitis. It is the leading cause of acquired heart disease in children in developed nations. Its clinical significance lies in its predilection for the coronary arteries, leading to aneurysms if untreated. **Analysis of Incorrect Options:** * **Henoch-Schönlein Purpura (HSP):** Now commonly referred to as **IgA Vasculitis**, it is a small-vessel vasculitis. While it is extremely common and often the most frequent cause of *non-thrombocytopenic purpura*, current pediatric literature (including Nelson’s Textbook of Pediatrics) frequently ranks KD as the most common systemic vasculitis overall. * **Wegener’s Granulomatosis (Granulomatosis with Polyangiitis):** This is a small-vessel, ANCA-associated vasculitis that is rare in the pediatric population, typically presenting in older adolescents or adults. * **Polyarteritis Nodosa (PAN):** This is a medium-vessel vasculitis that is rare in children. It is distinguished from KD by the absence of the "mucocutaneous lymph node syndrome" features. **NEET-PG High-Yield Pearls:** * **Diagnostic Criteria for KD:** Fever for ≥5 days plus 4 out of 5 features: (1) Conjunctival injection, (2) Rash, (3) Edema/erythema of hands/feet, (4) Strawberry tongue/fissured lips, (5) Cervical lymphadenopathy. * **Treatment:** High-dose IVIG (2g/kg) and Aspirin. IVIG is most effective when given within the first 10 days to prevent coronary artery aneurysms. * **Most common cause of death in KD:** Myocardial Infarction due to coronary artery occlusion.

Q: Congenital heart disease is most likely in the newborn of mothers suffering from all except:

Rheumatoid arthritis. **Explanation:** The correct answer is **Rheumatoid Arthritis (B)** because it is not significantly associated with an increased risk of structural congenital heart disease (CHD) in the offspring. While RA is an autoimmune condition, it lacks the specific transplacental antibody-mediated effects or metabolic teratogenicity seen in the other options. **Analysis of Options:** * **Systemic Lupus Erythematosus (SLE):** Mothers with SLE (specifically those with anti-Ro/SSA and anti-La/SSB antibodies) are at a high risk of having infants with **Neonatal Lupus**. The most serious manifestation is **Congenital Complete Heart Block**, which is a permanent conduction defect caused by inflammatory damage to the fetal AV node. * **Diabetes in Pregnancy:** Pre-gestational diabetes is a potent teratogen. It is associated with a 3–5 fold increase in CHD. The most common defect is a **Ventricular Septal Defect (VSD)**, but the most specific (highly characteristic) defect is **Transposition of the Great Arteries (TGA)**. Additionally, these infants may develop Transient Hypertrophic Subaortic Stenosis. * **Congenital Heart Disease of the Mother:** Genetic predisposition plays a major role. If a mother has a CHD, the risk of recurrence in the offspring is approximately 2–10% (significantly higher than the general population risk of ~0.8%). **High-Yield Clinical Pearls for NEET-PG:** * **Most common CHD in offspring of diabetic mothers:** VSD. * **Most specific CHD in offspring of diabetic mothers:** TGA. * **Lithium intake** during pregnancy is associated with **Ebstein’s Anomaly**. * **Congenital Rubella Syndrome** is most commonly associated with **Patent Ductus Arteriosus (PDA)** and Peripheral Pulmonic Stenosis.

Q: Peripheral pulmonic stenosis is associated with which of the following conditions?

Williams syndrome. **Explanation:** **Williams Syndrome** (7q11.23 microdeletion) is a multisystem disorder characterized by elastin arteriopathy. The most common cardiovascular manifestation is **Supravalvular Aortic Stenosis (SVAS)**. However, because the elastin defect affects the entire arterial tree, **Peripheral Pulmonic Stenosis (PPS)**—narrowing of the peripheral pulmonary artery branches—is the second most common cardiac finding, occurring in approximately 35–40% of patients. **Analysis of Options:** * **Williams Syndrome (Correct):** The combination of SVAS and PPS is a classic diagnostic hallmark. These patients often present with "elfin" facies, hypercalcemia, and a "cocktail party" personality. * **Subaortic Stenosis:** This is typically an anatomical obstruction (like a subaortic membrane) below the valve, not associated with the generalized arteriopathy seen in Williams syndrome. * **Takayasu’s Arteritis:** This is a large-vessel vasculitis. While it can cause stenosis of the aorta and its primary branches (like the subclavian), it does not typically present with congenital peripheral pulmonic stenosis. * **Coarctation of the Aorta:** While Williams syndrome can occasionally feature coarctation, PPS is a much more specific and frequent association with the syndrome’s underlying pathology. **High-Yield Clinical Pearls for NEET-PG:** * **Alagille Syndrome:** Another high-yield association for **Peripheral Pulmonic Stenosis** (along with cholestasis and butterfly vertebrae). * **Noonan Syndrome:** Most commonly associated with **Valvular Pulmonic Stenosis** and Hypertrophic Cardiomyopathy (HCM). * **Congenital Rubella Syndrome:** Classically associated with **PDA** and **Peripheral Pulmonic Stenosis**. * **Williams Syndrome Triad:** SVAS + Elfin Facies + Idiopathic Infantile Hypercalcemia.

Q: NADA's criteria are used for the assessment of a child?

Presence of heart disease. **Explanation:** **Nada’s Criteria** is a clinical scoring system used to screen for the **presence of heart disease** in children. It was developed to help clinicians differentiate between innocent murmurs and those requiring further cardiac evaluation (like echocardiography). The criteria are divided into **Major** and **Minor** categories: * **Major Criteria:** Systolic murmur (Grade III or higher), Diastolic murmur, Cyanosis, and Congestive Heart Failure (CHF). * **Minor Criteria:** Systolic murmur (Grade II or less), Abnormal S2, Abnormal ECG, Abnormal Chest X-ray (cardiomegaly or increased pulmonary vascularity), and Abnormal Blood Pressure. **Diagnosis:** Heart disease is suspected if there is **1 Major** or **2 Minor** criteria present. **Why other options are incorrect:** * **Degree of dehydration:** Assessed using the WHO classification (No, Some, or Severe dehydration) based on signs like skin pinch, lethargy, and thirst. * **Degree of malnutrition:** Assessed using the **IAP Classification** (weight-for-age) or **Gomez Classification**. * **Degree of mental retardation:** Assessed using IQ scores (e.g., Stanford-Binet or Wechsler scales) and the **Vineland Social Maturity Scale (VSMS)**. **High-Yield Clinical Pearls for NEET-PG:** * **Jones Criteria:** Used for the diagnosis of Acute Rheumatic Fever. * **Duke Criteria:** Used for the diagnosis of Infective Endocarditis. * **Ross Classification:** Used to grade the severity of Heart Failure in infants. * **Modified Centor Criteria:** Used for Streptococcal pharyngitis.

Q: Holt-Oram syndrome is characterized by which of the following congenital heart defects?

Atrial Septal Defect (ASD). **Explanation:** **Holt-Oram Syndrome**, also known as **Heart-Hand Syndrome**, is an autosomal dominant condition caused by a mutation in the **TBX5 gene** on chromosome 12. This gene is critical for the developmental patterning of both the upper limbs and the cardiac septa. 1. **Why ASD is Correct:** The most characteristic cardiac manifestation of Holt-Oram syndrome is an **Atrial Septal Defect (ASD)**, specifically of the **ostium secundum** type (seen in ~60% of cases). The TBX5 protein is essential for the formation of the interatrial septum; its deficiency leads to septation failure. 2. **Why other options are incorrect:** * **VSD:** While Ventricular Septal Defects can occur in Holt-Oram syndrome, they are less common than ASDs. * **TGA and TAPVC:** These are complex cyanotic congenital heart diseases involving conotruncal or venous anomalies. They are not classically associated with the TBX5 mutation or the clinical spectrum of Holt-Oram syndrome. **Clinical Pearls for NEET-PG:** * **The "Hand" Component:** Patients always present with upper limb deformities. The most common is a **triphalangeal thumb** or an absent/hypoplastic thumb. Radial ray defects (hypoplasia of the radius) are also hallmark features. * **Symmetry:** Limb involvement is often bilateral but can be asymmetrical (the left side is typically more severely affected). * **Conduction Defects:** Beyond structural defects, these patients are prone to **cardiac conduction blocks** (e.g., first-degree AV block or sick sinus syndrome). * **Mnemonic:** Remember **"H"** for **H**olt-Oram, **H**eart (ASD), and **H**and (Thumb/Radial defects).

Q: Noonan syndrome is characterized by all of the following EXCEPT:

Coarctation of aorta. **Explanation:** Noonan syndrome is an autosomal dominant multisystem disorder, often referred to as the "Male Turner Syndrome" (though it affects both sexes), caused by mutations in the RAS-MAPK pathway (most commonly the **PTPN11 gene**). **Why Coarctation of Aorta is the correct answer:** Coarctation of the aorta is classically associated with **Turner Syndrome (45, XO)**, occurring in about 15-20% of those cases. In contrast, Noonan syndrome primarily affects the **right side** of the heart. While left-sided lesions can occur, coarctation is not a characteristic feature of Noonan syndrome. **Analysis of other options:** * **Dysplastic Pulmonary Stenosis (Option B):** This is the **most common** cardiac defect in Noonan syndrome (approx. 50-60%). Unlike isolated PS, the valve in Noonan is typically dysplastic/thickened rather than just fused. * **Hypertrophic Cardiomyopathy (Option A):** This is the second most common cardiac manifestation (approx. 20%). It is often asymmetrical and can be present at birth or develop in infancy. * **Pectus Excavatum (Option C):** Skeletal deformities are hallmark features. Patients typically present with pectus carinatum superiorly and pectus excavatum inferiorly. **High-Yield Clinical Pearls for NEET-PG:** * **Genetics:** PTPN11 mutation (50%); part of the "RASopathies." * **Karyotype:** Normal (46, XY or 46, XX), unlike Turner syndrome. * **Facial Features:** Low-set ears, hypertelorism, downward-slanting palpebral fissures, and webbed neck. * **Hematology:** Factor XI deficiency and bleeding diathesis are common. * **Mnemonic:** "Right for Noonan, Left for Turner" (Right-sided heart defects in Noonan; Left-sided in Turner).

Q: What is the initial surgical treatment for Tetralogy of Fallot (TOF)?

Modified Blalock-Taussig shunt. **Explanation:** Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease characterized by pulmonary stenosis, right ventricular hypertrophy, overriding of the aorta, and a VSD. The definitive treatment is total surgical correction; however, in neonates with severe cyanosis, hypercyanotic spells, or hypoplastic pulmonary arteries, an **initial palliative procedure** is required to increase pulmonary blood flow. **1. Why Modified Blalock-Taussig (mBT) Shunt is correct:** The mBT shunt is the preferred initial palliative surgery. It involves creating a connection between the **subclavian artery and the ipsilateral pulmonary artery** using a synthetic GORE-TEX graft. This mimics a Patent Ductus Arteriosus (PDA), ensuring adequate blood reaches the lungs for oxygenation, allowing the pulmonary arteries to grow before definitive repair. **2. Why other options are incorrect:** * **Fontan Procedure:** Used for "single ventricle" physiology (e.g., Tricuspid Atresia). It redirects systemic venous return directly to the pulmonary arteries, bypassing the right ventricle. * **Glenn Shunt:** A bidirectional superior vena cava-to-pulmonary artery anastomosis. It is a second-stage procedure for single ventricle physiology, not TOF. * **Rastelli Operation:** Used for TOF with Pulmonary Atresia or Transposition of Great Arteries (TGA) with VSD/PS. It involves using a valved conduit to connect the RV to the pulmonary artery. **High-Yield Clinical Pearls for NEET-PG:** * **Classic BT Shunt:** Direct anastomosis of the subclavian artery to the pulmonary artery (rarely done now due to limb ischemia). * **Boot-shaped heart (Coeur en sabot):** Classic X-ray finding in TOF due to RV hypertrophy and an upturned apex. * **Most important prognostic factor:** The severity of Right Ventricular Outflow Tract (RVOT) obstruction. * **Drug of choice for Tet Spells:** Morphine (reduces infundibular spasm) and IV Beta-blockers (Propranolol).

Q: Which of the following is NOT a feature of Holt-Oram Syndrome?

Thrombocytopenia. **Explanation:** **Holt-Oram Syndrome (HOS)**, also known as **Heart-Hand Syndrome**, is a classic multisystem disorder characterized by upper limb deformities and congenital heart defects. 1. **Why Thrombocytopenia is the correct answer:** Thrombocytopenia is **not** a feature of Holt-Oram Syndrome. It is, however, the hallmark of **TAR Syndrome (Thrombocytopenia-Absent Radius)**. While both syndromes involve radial limb defects, they are distinct clinical entities. In TAR syndrome, the thumb is typically **present**, whereas in Holt-Oram, thumb anomalies (triphalangeal or absent thumb) are common. 2. **Analysis of Incorrect Options:** * **Absent Radius (Option A):** Limb involvement is a core feature of HOS. It ranges from a triphalangeal thumb to a completely absent radius or phocomelia. The defects are often bilateral and asymmetric, typically affecting the left side more severely. * **Atrial Septal Defect (Option B):** ASD (specifically the *ostium secundum* type) is the most common cardiac manifestation of HOS, followed by Ventricular Septal Defects (VSD) and conduction blocks. * **Autosomal Dominant (Option D):** HOS is inherited in an autosomal dominant pattern, caused by mutations in the **TBX5 gene** on chromosome 12. **High-Yield Clinical Pearls for NEET-PG:** * **Gene:** TBX5 (Think: **T**humbs and **B**eats on chromosome **5**-like region/12). * **Classic Triad:** Radial ray defects + Congenital Heart Disease (ASD/VSD) + Cardiac conduction disturbances. * **Differentiating Point:** In **HOS**, the thumb is **absent/hypoplastic**. In **TAR syndrome**, the thumb is **present** but the radius is absent. * **Mnemonic:** "Holt-Oram = Heart & Hand."

Q: A 9-year-old girl is diagnosed with acute rheumatic fever. Instead of recovering as expected, her condition worsens and she dies. Which of the following is the most likely cause of death?

Myocarditis. **Explanation:** In the setting of **Acute Rheumatic Fever (ARF)**, the heart is the only organ where involvement leads to significant mortality during the acute phase. While ARF is characterized by pancarditis (involving the endocardium, myocardium, and pericardium), **Myocarditis** is the most common cause of death. **1. Why Myocarditis is correct:** During the acute stage of ARF, severe inflammation of the myocardium leads to cardiac dilatation and impaired contractility. This results in **acute congestive heart failure (CHF)**, which is the primary driver of mortality. Histologically, this is marked by the presence of **Aschoff bodies**, which are pathognomonic for rheumatic carditis. **2. Why other options are incorrect:** * **Central Nervous System (CNS) involvement:** Sydenham’s chorea is a major Jones criterion but is typically self-limiting and never fatal. * **Endocarditis:** While valvulitis (mitral/aortic regurgitation) occurs acutely, "Endocarditis" in a chronic sense leads to long-term morbidity (Rheumatic Heart Disease). Acute death is due to the muscle failure (myocarditis) rather than the valve inflammation itself. * **Streptococcal sepsis:** ARF is a non-suppurative, immune-mediated sequela that occurs *after* the streptococcal pharyngeal infection has cleared. The patient is not septic during the rheumatic flare. **Clinical Pearls for NEET-PG:** * **Most common cause of death in ARF:** Myocarditis. * **Most common cause of death in Chronic RHD:** Heart failure secondary to valvular lesions (Mitral Stenosis). * **Pathognomonic lesion:** Aschoff bodies (contain Anitschkow cells/Caterpillar cells). * **Most common valve involved:** Mitral valve (Regurgitation in acute phase, Stenosis in chronic phase).

Question 1

What is the most common vasculitis seen in children?

Accepted Answer

Kawasaki disease

Answer

Henoch-Schonlein purpura

Answer

Wegener's granulomatosis

Answer

Polyarteritis nodosa

Question 2

Congenital heart disease is most likely in the newborn of mothers suffering from all except:

Accepted Answer

Rheumatoid arthritis

Answer

Systemic lupus erythematosus

Answer

Diabetes in pregnancy

Answer

Congenital heart disease of the mother

Question 3

A severely ill 3-year-old child presents with fever, enlarged cervical lymph nodes, and a desquamating rash involving the palms, soles, and mouth. What complication should be monitored for in this child?

Accepted Answer

Coronary artery aneurysm

Answer

Abdominal aortic aneurysm

Answer

Aneurysm of the aortic root

Answer

Berry aneurysm

Question 4

Peripheral pulmonic stenosis is associated with which of the following conditions?

Accepted Answer

Williams syndrome

Answer

Subaortic stenosis

Answer

Takayasu's arteritis

Answer

Coarctation of the aorta

Question 5

NADA's criteria are used for the assessment of a child?

Accepted Answer

Presence of heart disease

Answer

Degree of dehydration

Answer

Degree of malnutrition

Answer

Degree of mental retardation

Question 6

Holt-Oram syndrome is characterized by which of the following congenital heart defects?

Accepted Answer

Atrial Septal Defect (ASD)

Answer

Ventricular Septal Defect (VSD)

Answer

Transposition of Great Arteries (TGA)

Answer

Total Anomalous Pulmonary Venous Connection (TAPVC)

Question 7

Noonan syndrome is characterized by all of the following EXCEPT:

Accepted Answer

Coarctation of aorta

Answer

Hypertrophic cardiomyopathy

Answer

Dysplastic pulmonary stenosis

Answer

Pectus excavatum

Question 8

What is the initial surgical treatment for Tetralogy of Fallot (TOF)?

Accepted Answer

Modified Blalock-Taussig shunt

Answer

Fontan procedure

Answer

Glenn shunt

Answer

Rastelli operation

Question 9

Which of the following is NOT a feature of Holt-Oram Syndrome?

Accepted Answer

Thrombocytopenia

Answer

Absent Radius

Answer

Atrial septal defect

Answer

Autosomal dominant inheritance

Question 10

A 9-year-old girl is diagnosed with acute rheumatic fever. Instead of recovering as expected, her condition worsens and she dies. Which of the following is the most likely cause of death?

Accepted Answer

Myocarditis

Answer

Central nervous system involvement

Answer

Endocarditis

Answer

Streptococcal sepsis

Cardiology — MCQs

Cardiology — MCQs

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