Cardiology Practice Questions

Q: Most common conotruncal anomaly

Tetralogy of Fallot. ***Tetralogy of Fallot*** - Tetralogy of Fallot is the **most common conotruncal anomaly** and the **most common cyanotic congenital heart defect**, accounting for approximately 5-7% of all congenital heart diseases. - Its four classic features (ventricular septal defect, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy) all result from an **anterior and cephalad displacement of the conotruncal septum**. - Among the classic conotruncal anomalies (TOF, TGA, Truncus arteriosus, DORV), TOF has the highest prevalence. *TGA* - **Transposition of the Great Arteries (TGA)** is another significant conotruncal anomaly, but it is less common than Tetralogy of Fallot. - It involves an **abnormal septation** and rotation of the great arteries, leading to two parallel circulations. *Truncus arteriosus* - **Truncus arteriosus** is a rare and severe conotruncal anomaly where a single great artery arises from both ventricles, instead of separate aorta and pulmonary arteries. - This condition involves a failure of the **truncal ridges to fuse** and form a septum. *Double outlet right ventricle* - **Double outlet right ventricle (DORV)** is a complex anomaly where both great arteries (aorta and pulmonary artery) arise entirely or predominantly from the right ventricle. - This condition is less common than Tetralogy of Fallot and has a **wide spectrum of anatomical variations**.

Q: Most common cardiac defect in Turner syndrome

Coarctation of aorta. ***Coarctation of aorta*** - **Coarctation of the aorta** is the most frequent cardiac defect found in individuals with **Turner syndrome**, occurring in approximately 10-20% of cases [1]. - This congenital narrowing of the aorta typically presents with **hypertension in the upper extremities** and diminished or absent pulses in the lower extremities [1]. *VSD* - **Ventricular septal defects (VSDs)** are common congenital heart defects but are not the *most common* defect associated with Turner syndrome. - VSDs involve a hole in the wall separating the two lower chambers of the heart, resulting in a **left-to-right shunt**. *ASD* - **Atrial septal defects (ASDs)** are relatively common congenital heart defects but are less frequent in Turner syndrome compared to coarctation of the aorta [2]. - An ASD involves a hole in the wall separating the two upper chambers of the heart, leading to a **left-to-right shunt**. *TOF* - **Tetralogy of Fallot (TOF)** is a complex congenital heart defect involving four specific abnormalities, but it is **rarely associated with Turner syndrome**. - TOF typically presents with **cyanosis** and can be diagnosed by an abnormal heart murmur and characteristic findings on echocardiography.

Q: In the context of hypercyanotic spells (tet spells) in children with congenital heart disease, when does crying typically stop?

Forced inspiration. ***Forced inspiration*** - During a hypercyanotic spell (Tet spell), the infant exhibits **inconsolable, forceful crying that characteristically stops at forced inspiration**. - The child takes a **deep, forceful inspiratory breath** which temporarily increases intrathoracic negative pressure and systemic vascular resistance. - This distinctive breathing pattern is a **hallmark clinical feature** of Tet spells and helps differentiate them from other causes of cyanosis. - The forced inspiration is often followed by a brief pause before the cycle may repeat. *Forced Expiration* - Crying does not typically stop at forced expiration during hypercyanotic spells. - The expiratory phase is part of the crying effort but not where the characteristic cessation occurs. - The key clinical observation is the deep inspiratory effort that interrupts the crying pattern. *Mid inspiration* - While inspiration is involved, the cessation is specifically at **forced/deep inspiration**, not mid-inspiration. - Mid-inspiration would not capture the characteristic forceful, deep inspiratory effort that defines the pattern. - The clinical significance lies in the maximal inspiratory effort, not a partial one. *Crying is continuous* - Crying during Tet spells is **not continuous**; it has a characteristic pattern of interruption. - The crying is forceful and prolonged but punctuated by periods of forced inspiration. - Recognition of this non-continuous pattern with forced inspiratory pauses is clinically important for diagnosis.

Q: Most common type of TAPVC is -

Supracardiac. ***Supracardiac*** - This is the **most common type** of Total Anomalous Pulmonary Venous Connection (TAPVC), accounting for about 50% of cases. - Pulmonary veins drain into a **common vertical vein** that ascends to connect with the **innominate vein** or superior vena cava. *Cardiac* - In this type, the pulmonary veins drain directly into the **right atrium** or a coronary sinus. - It is relatively less common than the supracardiac type. *Infracardiac* - This is the **least common** and most severe type, where the pulmonary veins drain below the diaphragm, typically into the portal vein, ductus venosus, or inferior vena cava. - It is often associated with **pulmonary venous obstruction**, leading to cyanosis and pulmonary hypertension. *Multiple* - While it is possible to have anomalous drainage sites, **multiple sites** draining into different systemic veins are less common than a single primary site for TAPVC. - TAPVC is typically classified into specific anatomic types rather than 'multiple' as a primary category.

Q: Most common syndrome associated with A-V canal defect -

Down syndrome. ***Down syndrome*** - **Down syndrome (Trisomy 21)** is the most common syndrome associated with **atrioventricular (AV) canal defects** (endocardial cushion defects) - Occurs in approximately **40-50% of individuals with Down syndrome**, making it the hallmark cardiac anomaly in this condition - AV canal defects range from partial to complete defects involving atrial and ventricular septa and AV valves *Klinefelter syndrome* - **Klinefelter syndrome (47,XXY)** is not characteristically associated with AV canal defects - May have **mitral valve prolapse** or **aortic root dilation**, but AV canal defects are not a typical feature *Turner syndrome* - **Turner syndrome (45,X)** has distinct cardiovascular associations including **coarctation of the aorta** and **bicuspid aortic valve** - AV canal defects are **not** characteristic of Turner syndrome *Marfan syndrome* - **Marfan syndrome** is a connective tissue disorder with **aortic root dilation**, **aortic aneurysms**, and **mitral valve prolapse** - **AV canal defects are not a feature** of Marfan syndrome

Q: What is the most common complication of a large Patent Ductus Arteriosus (PDA)?

Congestive Heart Failure (CHF). ***Congestive Heart Failure (CHF)*** - A large PDA results in a significant **left-to-right shunt**, increasing pulmonary blood flow and leading to **pulmonary overcirculation**. [1] - This increased workload on the heart, particularly the left atrium and ventricle, can lead to **ventricular dysfunction** and ultimately CHF. [1] *Eisenmenger syndrome* - While a severe complication of an untreated large PDA, it represents a **late stage** where the left-to-right shunt has reversed due to **pulmonary hypertension**. [1] - It is not the *most common* initial complication, as CHF often develops earlier in the disease progression. *Infective endocarditis* - This is a potential long-term complication of a PDA, especially if untreated, due to the **turbulent blood flow** across the ductus. - However, it is less common than CHF, which results directly from the hemodynamic burden imposed by a large shunt. *None of the options* - This option is incorrect because **Congestive Heart Failure** is indeed a very common and significant complication of a large Patent Ductus Arteriosus.

Q: Double aortic arch is associated with which syndrome?

DiGeorge syndrome. ***DiGeorge syndrome*** - **DiGeorge syndrome** is caused by a **22q11.2 deletion** affecting the development of the third and fourth pharyngeal pouches, leading to **thymic hypoplasia**, **parathyroid hypoplasia**, and **cardiac anomalies**. - Common cardiac defects include **interrupted aortic arch type B**, **truncus arteriosus**, **tetralogy of Fallot**, and **VSD**. - **Double aortic arch** can occur in 22q11.2 deletion syndrome, though it is less common than other cardiac anomalies; however, among the syndromes listed, this represents the most appropriate association. - The question tests recognition that various cardiac arch anomalies, including double aortic arch, may be seen in this genetic syndrome. *CATCH 22 syndrome* - This is an **acronym** for DiGeorge syndrome: **C**ardiac defects, **A**bnormal facies, **T**hymic hypoplasia, **C**left palate, **H**ypocalcemia, and **22q11 deletion**. - It is **essentially the same condition** as DiGeorge syndrome, just using different nomenclature. - While technically correct, "DiGeorge syndrome" is the more standard medical terminology currently used. *Shprintzen syndrome* - **Shprintzen syndrome** (also called **velocardiofacial syndrome or VCFS**) is caused by the **same 22q11.2 deletion** as DiGeorge syndrome. - It represents a **phenotypic variant within the 22q11.2 deletion syndrome spectrum**, with more emphasis on palatal and facial features. - Since it shares the same genetic basis, it can also present with similar cardiac anomalies, but "DiGeorge syndrome" is the more commonly recognized term for this genetic disorder. *None of the options* - This is incorrect because the three syndromes listed above (DiGeorge, CATCH 22, and Shprintzen) all refer to **22q11.2 deletion syndrome** or its variants, which can be associated with various cardiac anomalies including double aortic arch. - Among the listed options, **DiGeorge syndrome** is the most appropriate and widely recognized answer.

Q: A 9-month-old child of a diabetic mother presents with tachypnea and hepatomegaly. Echocardiography shows normal cardiac morphology with asymmetric septal hypertrophy. Which of the following medications is indicated for the management of this child's condition?

Propranolol. ***Propranolol*** - **Propranolol** is a **beta-blocker** that is indicated for **hypertrophic cardiomyopathy** (HCM) in infants, especially those of diabetic mothers. - It works by reducing the **heart rate** and **myocardial contractility**, which decreases the **left ventricular outflow tract (LVOT) obstruction** caused by the hypertrophied septum. *Digoxin* - **Digoxin** is a **positive inotrope**, meaning it increases the force of myocardial contraction. - This effect would worsen the **outflow tract obstruction** in hypertrophic cardiomyopathy and is therefore contraindicated. *Frusemide* - **Frusemide** is a **diuretic** used to manage **fluid overload** and **congestive heart failure**. - While fluid management can be part of heart failure treatment, frusemide does not directly address the underlying **asymmetric septal hypertrophy** or **LVOT obstruction** in this context. *Isoptin* - **Isoptin** (verapamil) is a **non-dihydropyridine calcium channel blocker**. - While some calcium channel blockers can be used in adult hypertrophic cardiomyopathy, verapamil is generally avoided in infants with HCM due to its potential for **negative inotropic effects** and worsening hypotension, especially in the presence of outflow obstruction, and the risk of significant **bradycardia** and **atrioventricular block**.

Q: In children, which of the following is a key diagnostic sign of congestive heart failure (CHF)?

Hepatomegaly. ***Hepatomegaly*** - In children, **hepatomegaly** is a crucial indicator of **right-sided heart failure** due to congestion of the hepatic venous system. - The liver is a compressible organ and can accommodate a significant increase in blood volume, causing it to enlarge considerably before other signs of **venous congestion** become apparent. *Raised JVP* - **Raised jugular venous pressure (JVP)** is often difficult to assess reliably in infants and young children due to their short necks and uncooperative nature. - While present in older children with CHF, it is not considered as sensitive or specific as other signs in younger pediatric patients. *Pedal edema* - **Pedal edema** is less common in pediatric CHF compared to adults, particularly in infants and toddlers. - Their shorter hydrostatic columns and tendency to spend more time supine make dependent edema less prominent. *Basal crepitations* - **Basal crepitations** (rales) indicate **pulmonary edema**, which is a sign of **left-sided heart failure**. - While a part of CHF, **hepatomegaly** is a more consistent and often earlier sign that can be detected across different forms of pediatric CHF (right or left-sided).

Question 1

Most common conotruncal anomaly

Accepted Answer

Tetralogy of Fallot

Answer

TGA

Answer

Truncus arteriosus

Answer

Double outlet right ventricle

Question 2

Most common cardiac defect in Turner syndrome

Accepted Answer

Coarctation of aorta

Answer

VSD

Answer

ASD

Answer

TOF

Question 3

An 11-year-old child with a history of streptococcal pharyngitis presents you with fever and arthralgia. There is no past history of rheumatic heart disease or features of carditis or valvular disease. How often is 600,000 IU of benzathine penicillin recommended for prophylaxis of rheumatic heart disease?

Accepted Answer

Once in three weeks for 5 years or till the age of 18, whichever is longer

Answer

Immediately

Answer

Thrice weekly lifelong

Answer

Once in three weeks for 10 years or till the age of 25, whichever is longer

Question 4

In the context of hypercyanotic spells (tet spells) in children with congenital heart disease, when does crying typically stop?

Accepted Answer

Forced inspiration

Answer

Forced Expiration

Answer

Mid inspiration

Answer

Crying is continuous

Question 5

Most common type of TAPVC is -

Accepted Answer

Supracardiac

Answer

Cardiac

Answer

Infracardiac

Answer

Multiple

Question 6

Most common syndrome associated with A-V canal defect -

Accepted Answer

Down syndrome

Answer

Klinefelter syndrome

Answer

Turner syndrome

Answer

Marfan syndrome

Question 7

What is the most common complication of a large Patent Ductus Arteriosus (PDA)?

Accepted Answer

Congestive Heart Failure (CHF)

Answer

Eisenmenger syndrome

Answer

Infective endocarditis

Answer

None of the options

Question 8

Double aortic arch is associated with which syndrome?

Accepted Answer

DiGeorge syndrome

Answer

CATCH 22 syndrome

Answer

Shprintzen syndrome

Answer

None of the options

Question 9

A 9-month-old child of a diabetic mother presents with tachypnea and hepatomegaly. Echocardiography shows normal cardiac morphology with asymmetric septal hypertrophy. Which of the following medications is indicated for the management of this child's condition?

Accepted Answer

Propranolol

Answer

Digoxin

Answer

Frusemide

Answer

Isoptin

Question 10

In children, which of the following is a key diagnostic sign of congestive heart failure (CHF)?

Accepted Answer

Hepatomegaly

Answer

Pedal edema

Answer

Raised JVP

Answer

Basal crepitations

Cardiology — MCQs

Cardiology — MCQs

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