Cardiology Practice Questions

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 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: 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).

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: A 7-year-old child with rheumatic fever would require the following investigations, except:

Urine examination. ***Urine examination*** - A urine examination is **not typically required** for the diagnosis or monitoring of acute rheumatic fever. - While some systemic inflammatory conditions can affect the kidneys, it is **not a primary diagnostic or prognostic tool** for rheumatic fever. *Electrocardiogram* - An **ECG** is crucial to assess for cardiac involvement, such as **conduction abnormalities** (e.g., prolonged PR interval due to carditis). - It helps detect inflammation of the heart muscle, a major manifestation of rheumatic fever. *Echocardiogram* - An **echocardiogram** is essential for diagnosing **rheumatic carditis**, especially valvular damage. - It visualizes the heart's structure and function, identifying effects like **mitral or aortic regurgitation**. *Blood culture* - A **blood culture** is often done to rule out other infectious causes of similar symptoms like **septic arthritis** or **infective endocarditis**. - While rheumatic fever is triggered by *Streptococcus pyogenes*, the bacteria are typically no longer present in the bloodstream at the time of acute rheumatic fever.

Q: Which among the following diagnostic criteria is most characteristic of Kawasaki disease?

Non-exudative bilateral conjunctival injection. ***Non-exudative bilateral conjunctival injection*** - **Non-exudative bilateral conjunctival injection** is a key diagnostic criterion for Kawasaki disease, indicating inflammation without pus or discharge. - This finding is one of the five principal clinical features used to diagnose the condition, alongside fever of at least five days. *Unilateral cervical lymphadenopathy* - **Unilateral cervical lymphadenopathy** is a common diagnostic criterion for Kawasaki disease, but it must be larger than **1.5 cm** to be considered significant. - While present in many cases, its unilateral nature and specific size requirement make it less universally characteristic than bilateral conjunctival injection. *Gastrointestinal symptoms* - **Gastrointestinal symptoms**, such as diarrhea, vomiting, and abdominal pain, can occur in Kawasaki disease but are **non-specific** and not primary diagnostic criteria. - These symptoms are often present due to systemic inflammation but do not directly help in distinguishing Kawasaki from other febrile illnesses. *Periungual desquamation (peeling of skin around the nails)* - **Periungual desquamation** is a characteristic feature of **subacute or convalescent phase** of Kawasaki disease, occurring typically *after* the acute febrile stage. - While highly suggestive of Kawasaki disease, it is not an acute diagnostic criterion used to initially identify the illness during its active phase.

Q: In which of the following conditions is an atrial septal defect (ASD) commonly observed?

Holt-Oram syndrome. ***Correct: Holt-Oram syndrome*** - Holt-Oram syndrome has the **strongest and most specific association with secundum atrial septal defects (ASDs)** among the options listed - Approximately **75% of patients have congenital heart defects**, with **ASD being the most common** (followed by VSD) - Classic presentation: **upper limb abnormalities** (radial ray defects, triphalangeal thumb) + **ASD** - Autosomal dominant inheritance with mutations in TBX5 gene - When a question asks about "commonly observed" ASD, Holt-Oram is the classic teaching association *Incorrect: Ellis-van Creveld syndrome* - While Ellis-van Creveld syndrome does have a high incidence of cardiac defects (50-60%), the **most characteristic cardiac finding is a common atrium (single atrium)** rather than a typical secundum ASD - A common atrium is an extreme form of ASD where there is complete absence of the atrial septum - The syndrome is primarily characterized by **chondroectodermal dysplasia**: short limbs, short ribs, polydactyly, and dental/nail abnormalities - Much rarer than Holt-Oram syndrome *Incorrect: Down syndrome* - Down syndrome (Trisomy 21) has a high prevalence of congenital heart defects (40-50%), but the **most characteristic defect is an atrioventricular septal defect (AVSD)**, also known as endocardial cushion defect - While AVSDs involve the atrial septum, they are **distinct from isolated ASDs** and involve both the atrial and ventricular septa with abnormal AV valves - Other common defects: VSD, PDA, and tetralogy of Fallot *Incorrect: Turner's syndrome* - Turner's syndrome (45,XO) is characterized by congenital heart defects in about 25-50% of cases - The **most common cardiac abnormalities are coarctation of the aorta (30%) and bicuspid aortic valve (30%)** - Left-sided obstructive lesions predominate; **ASDs are less commonly associated** with Turner's syndrome - Other features: short stature, webbed neck, lymphedema, and gonadal dysgenesis

Q: What is the most characteristic auscultatory location for a patent ductus arteriosus (PDA) murmur?

Prominent in left 2nd intercostal space. ***Prominent in left 2nd intercostal space*** - The classic **"machinery" murmur** of a **patent ductus arteriosus (PDA)** is best heard at the **left upper sternal border** (left 2nd intercostal space). - This location corresponds to the anatomical position where the **ductus arteriosus** connects the **pulmonary artery** to the **descending aorta**. - The murmur is **continuous**, heard throughout systole and diastole, with peak intensity around the second heart sound. *Prominent in left infraclavicular space* - While the PDA murmur may radiate to the **left infraclavicular area**, this is not the primary auscultatory location. - The **left 2nd intercostal space** remains the **loudest and most diagnostic point** for detecting this murmur. *Prominent in right 2nd intercostal space* - The right 2nd intercostal space is the classic location for **aortic valve** murmurs, not PDA. - PDA is a **left-sided finding** due to the anatomical position of the ductus between the pulmonary artery and aorta. *Prominent at apex (5th intercostal space, midclavicular line)* - The apex is the primary location for **mitral valve** pathology, not PDA. - While some cardiac murmurs may radiate to multiple areas, the PDA murmur is characteristically **loudest at the left upper sternal border**, not the apex.

Question 1

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 2

Most common syndrome associated with A-V canal defect -

Accepted Answer

Down syndrome

Answer

Klinefelter syndrome

Answer

Turner syndrome

Answer

Marfan syndrome

Question 3

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 4

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 5

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

Question 6

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 7

A 7-year-old child with rheumatic fever would require the following investigations, except:

Accepted Answer

Urine examination

Answer

Electrocardiogram

Answer

Echocardiogram

Answer

Blood culture

Question 8

Which among the following diagnostic criteria is most characteristic of Kawasaki disease?

Accepted Answer

Non-exudative bilateral conjunctival injection

Answer

Unilateral cervical lymphadenopathy

Answer

Gastrointestinal symptoms

Answer

Periungual desquamation (peeling of skin around the nails)

Question 9

In which of the following conditions is an atrial septal defect (ASD) commonly observed?

Accepted Answer

Holt-Oram syndrome

Answer

Down syndrome

Answer

Turner's syndrome

Answer

Ellis-van Creveld syndrome

Question 10

What is the most characteristic auscultatory location for a patent ductus arteriosus (PDA) murmur?

Accepted Answer

Prominent in left 2nd intercostal space

Answer

Prominent in left infraclavicular space

Answer

Prominent at apex (5th intercostal space, midclavicular line)

Answer

Prominent in right 2nd intercostal space

Cardiology — MCQs

Cardiology — MCQs

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