Cardiology Practice Questions

Q: What could be the possible diagnosis for a newborn exhibiting weak lower limb pulses and strong upper limb pulses?

COA. ***COA*** (Coarctation of the Aorta) - The classic presentation of **Coarctation of the Aorta** involves a narrowing of the aorta, usually distal to the left subclavian artery, leading to increased pressure proximal to the coarctation (strong upper limb pulses) and decreased pressure distal to it (weak lower limb pulses). - This severe pressure gradient is manifested as **differential pulses** and potential **blood pressure discrepancy** between the arms and legs, making this the most likely diagnosis. *TGA* - **Transposition of the Great Arteries** is characterized by severe **cyanosis** presenting shortly after birth due to two parallel circulations (aorta from RV, pulmonary artery from LV). - It does not typically cause a significant differential in pulse strength between the upper and lower limbs unless complicated by underlying aortic arch anomalies. *TOF* - **Tetralogy of Fallot** typically presents with cyanosis and **hypercyanotic spells** (**tet spells**) due to severe right ventricular outflow tract obstruction and a large VSD. - While it is a common cyanotic heart disease, it primarily affects flow to the pulmonary circulation and does not cause obstruction in the systemic aorta leading to differential pulses. *Ebstein anomaly* - This condition involves the apical displacement of the **tricuspid valve** leaflets into the **right ventricle**, leading to tricuspid regurgitation and large right atrium. - Clinical features usually include varying degrees of cyanosis and signs of right heart failure but do not typically involve a differential in systemic arterial pulses.

Q: A 9-month-old infant presents with severe respiratory distress, central cyanosis not improving with oxygen, and a harsh systolic murmur. Chest X-ray shows boot-shaped heart with decreased pulmonary vascular markings. Echocardiography confirms the diagnosis. What is the most likely underlying cardiac defect?

Tetralogy of Fallot. ***Tetralogy of Fallot*** - The combination of **severe respiratory distress**, **central cyanosis** unresponsive to oxygen (a **cyanotic spell**), **harsh systolic murmur** (due to **pulmonary stenosis**), and a **boot-shaped heart** (coeur en sabot) on chest X-ray with **decreased pulmonary vascular markings** (due to reduced blood flow) is the classic presentation of **Tetralogy of Fallot** (TOF). - TOF is the most common cyanotic congenital heart disease and consists of four defects: large **VSD**, **pulmonary stenosis**, **overriding aorta**, and **right ventricular hypertrophy**. *Transposition of Great Arteries (TGA)* - TGA presents with severe cyanosis, but the chest X-ray typically shows a **'egg-on-a-string'** appearance due to a narrow mediastinum and cardiomegaly, not a boot shape. - Pulmonary vascular markings are often **increased** or normal, depending on whether there is associated pulmonary stenosis, and the murmur is typically non-specific or absent. *Total Anomalous Pulmonary Venous Return (TAPVR)* - This condition presents with cyanosis and can show a **'snowman'** or **'figure-of-eight'** sign on the chest X-ray if the supra-cardiac type is present, due to enlarged vertical vein and SVC. - Pulmonary vascular markings are usually **increased** due to pulmonary venous obstruction. *Truncus Arteriosus* - Although cyanotic, truncus arteriosus is characterized by a single great artery overriding the ventricular septum, leading to **increased pulmonary blood flow** and consequently **increased pulmonary vascular markings** on the chest X-ray. - The heart shape is typically **cardiomegaly** with a common trunk arising from the heart, often resulting in a late-onset or subtle cyanosis compared to TOF or TGA.

Q: A 4-year-old child presents with high-grade fever for 5 days, bilateral non-purulent conjunctival injection, strawberry tongue, cervical lymphadenopathy (>1.5 cm), and polymorphous rash. On day 6, desquamation of fingertips begins. Echocardiography shows coronary artery dilation. What is the most appropriate immediate treatment?

Intravenous immunoglobulin (IVIG) with aspirin. ***Intravenous immunoglobulin (IVIG) with aspirin*** - This is the **gold standard, immediate treatment** for acute Kawasaki disease to reduce systemic inflammation and prevent coronary artery complications - **IVIG** (2 g/kg as a single infusion over 10-12 hours) works by modulating the immune response and reducing inflammation - **High-dose aspirin** (80-100 mg/kg/day divided into 4 doses) provides anti-inflammatory effects during the acute febrile phase - This combination must be started **within 10 days of fever onset** (ideally within 7 days) to significantly reduce the risk of **coronary artery aneurysms** - Given that this child already has coronary artery dilation on echo, urgent IVIG + aspirin therapy is critical to prevent progression *High-dose aspirin alone* - Aspirin alone is **insufficient** to address the underlying immune-mediated vasculitis in Kawasaki disease - While aspirin has anti-inflammatory and antiplatelet effects, it does **not provide the immunomodulatory benefits** of IVIG - Monotherapy with aspirin has been shown to be **significantly less effective** at preventing coronary complications compared to combination therapy *Supportive care only* - Supportive care (hydration, antipyretics) addresses symptoms but **does not treat the underlying vasculitis** - Without specific immunomodulatory therapy, up to **25% of untreated KD patients** develop coronary artery aneurysms - This child already has coronary involvement, making specific therapy even more urgent *Oral corticosteroids* - Corticosteroids are generally reserved for **IVIG-refractory cases** (patients who remain febrile 36-48 hours after initial IVIG) or high-risk patients - They are **not recommended as first-line therapy** according to current AHA guidelines - IVIG remains superior for initial treatment in preventing coronary complications

Q: A 3-year-old child is brought to the clinic with a history of cyanosis since infancy. Which of the following is a component of Tetralogy of Fallot (TOF)?

Infundibular pulmonary stenosis. ***Infundibular pulmonary stenosis*** - This is the most common anatomic type of **pulmonary stenosis** seen in TOF, caused by hypertrophy of the muscle below the pulmonary valve (infundibulum). - The degree of this stenosis dictates the direction of flow across the VSD and, consequently, the severity of **cyanosis**. *Inter atrial septal defect* - An ASD is not a primary component of TOF. When TOF is associated with an ASD, the condition is termed **Pentalogy of Fallot**. - ASD typically causes a **left-to-right shunt** and is usually an acyanotic or late-onset cyanotic condition, unlike classic TOF. *Left ventricular hypertrophy (LVH)* - The pressure overload due to **pulmonary stenosis** and the large **VSD** leads to **Right Ventricular Hypertrophy (RVH)**. - LVH suggests conditions like severe **aortic stenosis** or systemic overloading, not the typical hemodynamics of TOF. *Transposition of the great arteries (TGA)* - **TGA** is a separate, distinct cyanotic congenital heart disease where the great arteries are transposed (aorta from RV, pulmonary artery from LV). - The components of TOF involve a single great artery relationship but with a large **Ventricular Septal Defect (VSD)** and overriding aorta.

Q: Which congenital heart disease is associated with the defect shown?

Endocardial cushion defect. ***Endocardial cushion defect*** - The image displays a **single palmar crease** (also known as a **simian crease**), which is a common dermatological feature observed in patients with **Down syndrome (Trisomy 21)**. - Approximately 40-50% of individuals with Down syndrome have **congenital heart disease**, with **endocardial cushion defects** (also known as atrioventricular septal defects) being the most common type. *Tricuspid atresia* - This is a complex cyanotic congenital heart defect characterized by the **absence of the tricuspid valve**, preventing blood flow from the right atrium to the right ventricle. - While it is a congenital heart defect, it is **not specifically associated with a single palmar crease** or Down syndrome as commonly as endocardial cushion defects. *Double outlet right ventricle* - This is a rare cyanotic congenital heart defect where **both the aorta and pulmonary artery arise primarily from the right ventricle**. - It is not typically associated with specific dermatological signs like a single palmar crease or specifically linked to Down syndrome. *Aortic regurgitation* - This is a condition where the **aortic valve does not close tightly**, causing blood to leak back into the left ventricle during diastole. - Aortic regurgitation is an **acquired or congenital valve defect**, but it is not directly linked to genetic syndromes like Down syndrome or the presence of a single palmar crease.

Q: Child presents with strawberry tongue, fever for 5 days, cracked lips, Periungual peeling of the skin and bulbar congestion. Which of the following cardiac lesions will be seen in this child?

Mitral regurgitation. ***Mitral regurgitation*** - The constellation of symptoms (strawberry tongue, fever, cracked lips, periungual peeling, bulbar congestion) is highly characteristic of **Kawasaki disease**. - **Coronary artery aneurysms** and **mitral regurgitation** are common cardiac complications of Kawasaki disease due to inflammation affecting the heart valves and coronary arteries. *Mitral stenosis* - Mitral stenosis is less common in acute Kawasaki disease and is more typically a long-term complication of rheumatic fever or a congenital anomaly. - The acute inflammatory nature of Kawasaki disease is more likely to cause valvular insufficiency (regurgitation) rather than narrowing (stenosis). *Tricuspid stenosis* - Tricuspid stenosis is a relatively rare valvular lesion and is not typically associated with Kawasaki disease. - Inflammatory conditions like Kawasaki disease primarily affect the left-sided heart valves and coronary arteries. *Pulmonary regurgitation* - While pulmonary regurgitation can occur congenitally or due to pulmonary hypertension, it is not a direct or common cardiac complication of Kawasaki disease. - Kawasaki disease primarily targets the systemic vasculature and coronary arteries, leading to problems like mitral regurgitation.

Q: What is the expected karyotype in this child with the following findings and having pulmonic stenosis on Echocardiography?

46,XY. ***46,XY*** - The image depicts a child with features suggestive of **Noonan Syndrome**, characterized by widely spaced eyes, low-set ears, a short webbed neck, and **pulmonic stenosis**. - Noonan Syndrome is a **cardiofacial cutaneous syndrome** that is genetically heterogeneous; however, it typically presents with a **normal karyotype of 46,XY** for males or 46,XX for females, as it arises from mutations in genes like *PTPN11*, *SOS1*, *RAF1*, or *KRAS*, rather than chromosomal aneuploidies. *46,XX* - This karyotype represents a **normal female**. While females can have Noonan Syndrome, the general characteristic features pointing towards a male child in the provided image makes this option less likely in this specific context. - The question asks for the expected karyotype in "this child," implying a specific gender based on the drawing; however, if the child were female with Noonan Syndrome, 46,XX would be expected. *45,X* - This karyotype corresponds to **Turner Syndrome**, which typically affects females and is characterized by features such as **short stature**, a webbed neck, and **coarctation of the aorta** (not pulmonic stenosis). - While there is some phenotypic overlap (e.g., webbed neck), the presence of pulmonic stenosis and other facial features are more consistent with Noonan syndrome, which is not associated with a monosomy of the X chromosome. *47,XXY* - This karyotype represents **Klinefelter Syndrome**, which affects males and is characterized by **tall stature**, **hypogonadism**, and **gynecomastia**. - The features in the image, including the **facial dysmorphism** and **pulmonic stenosis**, are inconsistent with the typical presentation of Klinefelter Syndrome.

Q: A 1-year-old child with severe acute malnutrition develops pneumonia which is not responding to treatment. Chest X-ray picture is given. What is the likely etiology?

Staphylococcus aureus. ***Staphylococcus aureus*** - The chest X-ray shows extensive **bilateral infiltrates** with areas of potential **abscess formation** or **necrotizing pneumonia**, suggested by the poorly defined lucencies within consolidation, which are characteristic of Staphylococcal infection, especially in immunocompromised individuals like a malnourished child. - Staphylococcal pneumonia is often severe, can lead to **necrosis**, **cavitation**, and is known for its **resistance to common antibiotics**, explaining the poor response to initial treatment in a severely malnourished child. *Mycoplasma* - Mycoplasma pneumoniae typically causes **"walking pneumonia"** with milder symptoms and **diffuse interstitial infiltrates** visible on Chest X-ray, which are not as severe or focal as seen in the image. - It's less common to cause rapidly progressing, severe, and necrotizing pneumonia in this age group, even in malnourished children. *Pneumococcus* - **Streptococcus pneumoniae (Pneumococcus)** usually causes **lobar pneumonia** with dense, homogeneous consolidation in one lobe, often with a visible air bronchogram, rather than the more patchy, bilateral, and potentially necrotizing pattern seen here. - While it can be severe, especially in malnourished children, the radiographic pattern is less typical for pneumococcal infection. *Adenovirus* - Adenovirus can cause a range of respiratory infections, from common colds to severe pneumonia, especially in young children. However, the Chest X-ray findings typically include **perihilar infiltrates**, **bronchial wall thickening**, and hyperinflation, which do not fully match the extensive, severe, and potentially cavitating pattern observed. - While it can cause severe pneumonia, the given X-ray features, particularly the suggestion of necrosis, are less characteristic of adenoviral infection.

Question 1

What could be the possible diagnosis for a newborn exhibiting weak lower limb pulses and strong upper limb pulses?

Accepted Answer

COA

Answer

TOF

Answer

Ebstein anomaly

Answer

TGA

Question 2

A 9-month-old infant presents with severe respiratory distress, central cyanosis not improving with oxygen, and a harsh systolic murmur. Chest X-ray shows boot-shaped heart with decreased pulmonary vascular markings. Echocardiography confirms the diagnosis. What is the most likely underlying cardiac defect?

Accepted Answer

Tetralogy of Fallot

Answer

Total anomalous pulmonary venous return

Answer

Transposition of great arteries

Answer

Truncus arteriosus

Question 3

A 4-year-old child presents with high-grade fever for 5 days, bilateral non-purulent conjunctival injection, strawberry tongue, cervical lymphadenopathy (>1.5 cm), and polymorphous rash. On day 6, desquamation of fingertips begins. Echocardiography shows coronary artery dilation. What is the most appropriate immediate treatment?

Accepted Answer

Intravenous immunoglobulin (IVIG) with aspirin

Answer

High-dose aspirin alone

Answer

Supportive care only

Answer

Oral corticosteroids

Question 4

A 3-year-old child is brought to the clinic with a history of cyanosis since infancy. Which of the following is a component of Tetralogy of Fallot (TOF)?

Accepted Answer

Infundibular pulmonary stenosis

Answer

Inter atrial septal defect

Answer

Left ventricular hypertrophy (LVH)

Answer

Transposition of the great arteries (TGA)

Question 5

A 6-week-old term baby presents with poor feeding. All are true about the condition shown except:

Accepted Answer

Ductal-dependent systemic circulation

Answer

Ibuprofen inhibits Prostaglandin production leading to closure

Answer

Left ventricular failure

Answer

Anatomical closure occurs by 2-3 weeks

Question 6

Which congenital heart disease is associated with the defect shown?

Accepted Answer

Endocardial cushion defect

Answer

Tricuspid atresia

Answer

Double outlet right ventricle

Answer

Aortic regurgitation

Question 7

A 1 day old neonate presents with central cyanosis. CXR and ECG are performed (shown in the image). Which diagnosis is most consistent with these findings?

Accepted Answer

Cardiomegaly with normal ECG suggestive of hypoplastic left heart syndrome

Answer

Cardiomegaly and Tall T waves in ECG suggestive of tetralogy of Fallot

Answer

Cardiomegaly with delta waves in ECG suggestive of Wolff-Parkinson-White syndrome

Answer

Cardiomegaly, Himalayan P waves in ECG suggestive of tricuspid atresia

Question 8

Child presents with strawberry tongue, fever for 5 days, cracked lips, Periungual peeling of the skin and bulbar congestion. Which of the following cardiac lesions will be seen in this child?

Accepted Answer

Mitral regurgitation

Answer

Mitral stenosis

Answer

Tricuspid stenosis

Answer

Pulmonary regurgitation

Question 9

What is the expected karyotype in this child with the following findings and having pulmonic stenosis on Echocardiography?

Accepted Answer

46,XY

Answer

46,XX

Answer

45,X

Answer

47,XXY

Question 10

A 1-year-old child with severe acute malnutrition develops pneumonia which is not responding to treatment. Chest X-ray picture is given. What is the likely etiology?

Accepted Answer

Staphylococcus aureus

Answer

Mycoplasma

Answer

Pneumococcus

Answer

Adenovirus

Cardiology — MCQs

Cardiology — MCQs

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