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Questions and Answers

Which symptom is NOT a characteristic that raises suspicion for congenital heart disease?

Cyanosis since birth

Murmur since birth

Recurrent chest infection

History of rheumatic fever (correct)

What is the primary consequence of a left to right shunt in congenital heart disease?

Decreased pressure in the left atrium

Cyanosis from birth

Reversal of blood flow

Volume overload of the right heart (correct)

Which congenital heart defect is most commonly associated with an atrial septal defect (ASD)?

Coarctation of the aorta

Tricuspid atresia

Tetralogy of Fallot

Mitral valve disease (correct)

What is a likely long-term outcome if an atrial septal defect is left untreated?

Eisenmenger syndrome Signup and view all the answers

In an atrial septal defect, what condition is characterized by the direction of blood flow?

Blood flows from LA to RA Signup and view all the answers

Which type of shunt is primarily responsible for increased right ventricle pressure over time?

Left to right shunt Signup and view all the answers

Which of the following complications can arise from untreated left to right shunting?

Right ventricle hypertrophy Signup and view all the answers

Which congenital heart disease is characterized by a right to left shunt during advanced stages?

Eisenmenger syndrome Signup and view all the answers

What is a common symptom in mild cases of right ventricle hypertrophy?

Asymptomatic Signup and view all the answers

Which of the following is an associated anomaly with secundum ASD?

Tri-phalangeal thumb Signup and view all the answers

What is the expected auscultation finding in right ventricle hypertrophy?

Accentuated, wide fixed splitting of S2 Signup and view all the answers

Which complication of right ventricle hypertrophy is characterized by cyanosis?

Eisenmenger's syndrome Signup and view all the answers

In ventricular septal defect (VSD), which type is considered hemodynamically insignificant?

Small muscular type Signup and view all the answers

What is the typical pressure in the left ventricle (LV) during systole?

120 / 0 mmHg Signup and view all the answers

What characteristic murmur is associated with a sub-aortic VSD?

Harsh pansystolic murmur with thrill over the 3rd, 4th intercostal spaces Signup and view all the answers

What is a common X-ray finding in patients with right ventricle hypertrophy?

Dilated pulmonary artery Signup and view all the answers

Which investigation is expected to reveal right bundle branch block (RBBB) in cases of right ventricle hypertrophy?

ECG Signup and view all the answers

Which of the following complications can arise from a ventricular septal defect (VSD)?

Paradoxical embolism Signup and view all the answers

What is the primary investigation that can reveal biventricular enlargement and the presence of anomalies?

Chest X-ray Signup and view all the answers

In cases of aortic coarctation, what is a common finding in blood pressure measurements?

Higher blood pressure in the upper half compared to the lower half Signup and view all the answers

What is a clinical sign indicative of collateral circulation in aortic coarctation?

Rib notching on X-ray Signup and view all the answers

What condition is characterized by the narrowing of the aorta and is usually found distal to the left subclavian artery?

Aortic coarctation Signup and view all the answers

What murmur is typically heard due to aortic coarctation?

Continuous murmur over collaterals Signup and view all the answers

Which of the following is an important aspect of the treatment for congenital heart defects like VSD?

Prophylaxis against infective endocarditis Signup and view all the answers

What is the most common type of pulmonary stenosis?

Valvular stenosis Signup and view all the answers

What is a likely consequence of untreated severe pulmonary stenosis?

Right-sided heart failure Signup and view all the answers

Which investigation is considered diagnostic for pulmonary stenosis?

Echocardiogram Signup and view all the answers

What does a continuous thrill over the left infraclavicular area indicate?

Patent ductus arteriosus Signup and view all the answers

What happens to the pulmonary arteries in the case of patent ductus arteriosus?

Increased blood flow leading to lung plethora Signup and view all the answers

What type of heart sound may be heard in severe pulmonary stenosis?

Ejection click and S4 Signup and view all the answers

What is a potential risk associated with pulmonary stenosis and structural heart conditions?

Bacterial endocarditis Signup and view all the answers

Which of the following is a common symptom of patent ductus arteriosus (PDA)?

Palpitations and general throbbing Signup and view all the answers

What type of murmur is typically heard in patients with a left-to-right shunt?

Continuous 'machinery' murmur Signup and view all the answers

Which condition is associated with paradoxical embolism and differential cyanosis?

Eisenmenger's syndrome Signup and view all the answers

Which imaging technique is most effective for detecting chamber dilatation in congenital heart disease?

Echocardiogram Signup and view all the answers

What is the recommended treatment for symptomatic patients with Eisenmenger's syndrome?

Heart-lung transplantation Signup and view all the answers

In the context of congenital heart defects, what does 'PS' refer to?

Pulmonary stenosis Signup and view all the answers

What distinguishing feature characterizes triology of Fallot compared to tetralogy of Fallot?

Presence of atrial septal defect (ASD) Signup and view all the answers

Which form of dextrocardia refers only to the heart being transposed while other organs remain in their normal orientation?

Isolated dextrocardia Signup and view all the answers

What is the main reason that closure of the defect is contraindicated in Eisenmenger's syndrome?

It increases right heart pressure Signup and view all the answers

Study Notes

Congenital Heart Disease Definition & Sources

Congenital heart disease (CHD) is a condition that affects the structure of the heart before birth, contributing to various problems like blood flow issues, oxygen levels, and heart function.
There are many possible causes of CHD, including family history, environmental factors, and genetic predispositions.

Clinical Presentation of CHD

Suspect CHD in cases with cyanosis (blue discoloration) since birth, family history of heart disease, murmur (unusual heart sound) since birth, and associated birth defects.

Investigations for CHD

X-ray: Used to assess heart size, shape, and blood flow patterns.
ECG: Detects abnormalities in heart rhythm and electrical activity.
Echo: Provides detailed images of the heart's structure and function.
Catheterization: Allows for direct examination of the heart chambers and vessels, measuring pressure and blood flow.

Atrial Septal Defect (ASD)

A hole in the wall (septum) between the two upper chambers of the heart, leading to blood flow from the left atrium to the right, known as a left-to-right shunt.
High ASD (ostium secundum): The most common type.
Low ASD (ostium primum): May be associated with mitral valve disease (Lutembacher's Syndrome)

Hemodynamics of ASD

Increased blood flow to the right ventricle and pulmonary arteries.
Right ventricle enlargement occurs.
Pulmonary hypertension (high pressure in the lungs) can lead to a reversal of the shunt.
Left ventricular failure can occur due to decreased cardiac output.

Clinical Picture of ASD

Symptoms: Asymptomatic in early life, exertional dyspnea (difficulty breathing with effort), and recurrent chest infections can all occur.
Signs : Right ventricle hypertrophy, accentuated and fixed splitting of the second heart sound, and murmurs, often of relative tricuspid and pulmonary stenosis

Complications of ASD

RSHF (right-sided heart failure)
Paradoxical embolism (blood clot that travels from the right side of the heart to the left, potentially causing a stroke).
Eisenmenger's syndrome: Cyanosis (blue discoloration) due to a reversal of the shunt.
Infective endocarditis (infection of the heart lining): Rare due to low pressure gradients.
Arrhythmia (irregular heartbeat), primarily atrial fibrillation (AF).

Ventricular Septal Defect (VSD)

An opening in the wall between the two lower chambers of the heart, causing a left-to-right shunt.
Membranous type: Occurs in the membranous part of the interventricular septum.
Muscular type (Roger's disease): Occurs in the muscular part of the interventricular septum, often resolves spontaneously.

Hemodynamics of VSD

Blood flow from the left ventricle (higher pressure) to the right ventricle (lower pressure) during systole (contraction of the heart).
Increased blood flow to the pulmonary arteries, causing lung plethora (excessively full lungs) and potential pulmonary hypertension.
Increased blood flow to the left atrium and left ventricle, leading to left ventricular enlargement.
Decreased cardiac output due to less blood flow through the aorta.

Clinical Picture of VSD

Symptoms: Asymptomatic in mild cases and Roger’s disease. May include signs of lung plethora, left-sided heart failure, and associated complications.
Signs: Lung plethora, left-sided heart failure, giant (a) wave in the jugular vein (a sign of increased pressure in the right ventricle).

Cardiac Examination of VSD

Biventricular enlargement with a hyperdynamic apex.
Auscultation: Accentuated pulmonary component of the second heart sound, wide splitting of S2.
Murmur: Harsh pansystolic murmur with a thrill over the third and fourth intercostal spaces, ejection systolic murmur of pulmonary hypertension, sub-aortic VSD may be associated with aortic regurgitation, and a mid-diastolic murmur of relative mitral stenosis.

Complications of VSD

Heart failure
Infective endocarditis
Paradoxical embolism
Eisenmenger's syndrome: Usually at 2nd-3rd decade of life.

Aortic Coarctation

Narrowing of a part of the aorta, typically distal to the left subclavian artery, potentially interfering with blood flow to the lower body.
Associated congenital anomalies: Bicuspid aortic valve, patent ductus arteriosus (PDA), ventricular septal defect (VSD), congenital aneurysm of the Circle of Willis, Turner's syndrome.

Symptoms of Aortic Coarctation

Asymptomatic in mild cases.
Symptoms of hemodynamics: Hypertension in the upper body, fatigue, intermittent claudication (pain in legs during exercise), pain around the left shoulder.

Signs of Aortic Coarctation

No signs in mild cases.
Signs of hemodynamics: High blood pressure in the arms, prominent carotid pulsation, low blood pressure in the legs, weak pulses in the lower extremities.
Collaterals (new blood vessels developing in the body to compensate for the narrowing in the aorta) may be seen in the interscapular area (Suzman's sign).

Cardiac Examination of Aortic Coarctation

Left ventricular hypertrophy.
Auscultation: Accentuated S2, ejection systolic murmur due to coarctation itself, associated aortic stenosis, hypertension, early diastolic murmur due to associated aortic regurgitation, and continuous murmur over the collaterals.

Complications of Aortic Coarctation

Complications of hypertension: Cerebral hemorrhage, heart failure, infective endocarditis.

Pulmonary Stenosis

Narrowing of the pulmonary valve, often impacting blood flow from the right ventricle to the pulmonary artery.
Types: Valvular, subvalvular (infundibular), supravalvular (rare).

Hemodynamics of Pulmonary Stenosis

Increased resistance to blood flow to the pulmonary artery.
Right ventricle hypertrophy, resulting in right-sided heart failure.
Low cardiac output.

Clinical Picture of Pulmonary Stenosis

Symptoms: Asymptomatic in mild cases, may experience fatigue, shortness of breath, and signs of right-sided heart failure.
Signs: Giant (a) wave in the jugular vein, right ventricle hypertrophy.

Cardiac Examination of Pulmonary Stenosis

Auscultation: Weak pulmonary component of S2 with wide splitting, ejection click in valvular type, S4 on the tricuspid area, and ejection systolic murmur on the pulmonary area.

Complications of Pulmonary Stenosis

Right-sided heart failure
Infective endocarditis

Patent Ductus Arteriosus (PDA)

Persistence of the ductus arteriosus after birth, a blood vessel connecting the aorta and pulmonary artery.
Normal during fetal life, allowing blood to bypass the lungs.
Common in premature infants, particularly female infants.

Hemodynamics of PDA

Blood flow from the aorta (higher pressure) to the pulmonary artery (lower pressure) in both systole and diastole.
Increased blood flow in the pulmonary arteries (leading to lung plethora).
Increased blood flow in the left atrium and left ventricle, causing left ventricular enlargement.
Increased blood flow to the aorta in systole, causing high cardiac output and high systolic blood pressure.
Low diastolic blood pressure due to blood escaping from the aorta to the pulmonary artery during diastole.
Hyperdynamic circulation with widened pulse pressure.
Pulmonary hypertension and shunt reversal (Eisenmenger's Syndrome).

Clinical Picture of PDA

Symptoms: Asymptomatic in mild cases, may experience symptoms of lung plethora or hyperdynamic circulation: palpitation, generalized throbbing, widened pulse pressure, chest pain, and shortness of breath.
Signs: Lung plethora, signs of hyperdynamic circulation, giant (a) wave in the jugular vein due to pulmonary hypertension.

Cardiac Examination of PDA

Left ventricular enlargement
Continuous thrill over the left infraclavicular area (site of PDA) .
Auscultation: Accentuated and reversed splitting of S2, continuous "machinery" murmur over the left infraclavicular area.

Complications of PDA

Left-sided heart failure
Paradoxical embolism (strokes)
Infective endocarditis
Eisenmenger's Syndrome: Differential cyanosis (cyanosis in the lower extremities, normal in the upper extremities)

Tetralogy of Fallot (F4)

A combination of four heart defects:
- Pulmonary stenosis (subvalvular)
- Mild right ventricular enlargement
- Ventricular septal defect (not clinically significant)
- Overriding aorta (aorta sits over both ventricles instead of only the left)

Trilology of Fallot (F3)

A combination of three heart defects:
- Pulmonary stenosis (valvular)
- Marked right ventricular enlargement
- Atrial septal defect

Eisenmenger's Syndrome

A condition where a left-to-right shunt (blood flows from left to right) leads to pulmonary hypertension and a reversal of the shunt, causing right-to-left blood flow.
Causes: VSD, PDA, ASD

Clinical Picture of Eisenmenger's Syndrome

History of congenital heart disease (VSD, PDA, ASD).
Pulmonary infections and hemoptysis (coughing up blood).
Clinical presentation of pulmonary hypertension.
Decrease in the original murmur of the shunt due to lower pressure gradients.

Treatment of Eisenmenger's Syndrome

Prevention is the best course.
Closure of the defect is contraindicated, as it can increase pressure on the right side of the heart.
Symptomatic treatment is often necessary.
Heart-lung transplantation may be considered in severe cases.

Dextrocardia

Displacement of the heart to the right side of the chest, either congenitally or acquired.
Types:
- Situs inversus totalis: Mirror-like transposition of the heart and all other internal organs.
- Isolated dextrocardia: Mirror-like transposition of the heart only.
- Dextroversion: The heart is displaced to the right, but the right ventricle remains on the right and the left ventricle remains on the left.
- Acquired dextrocardia: Displacement of the heart to the right due to a condition, like fibrosis.

Treatment of Dextrocardia

Treatment depends on the underlying cause and the severity of the condition.
Some cases are asymptomatic and may not require treatment, while others may necessitate surgery or medical management.

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