Clinical Question-12

Clinical Question-12

56 year old female presents to emergency department with complains of cyanosis, exertion dyspnoea, syncope and chest pain. He has a history of atrial septal defect. She was advised for ASD Closure but she lost follow up and did not undergo the ASD closure procedure. What is the complication of untreated ASD?

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[A] Right-sided heart failure
[B] Cryptogenic stroke
[C] Eisenmenger syndrome
[D] Pulmonary infarction

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Eisenmenger syndrome

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Key Concept	Summary
Etiology	Uncorrected left-to-right shunt (e.g., ASD) causes pulmonary hypertension and shunt reversal.
Shunt Reversal	Becomes right-to-left due to increased pulmonary vascular resistance.
Hallmark Features	Central cyanosis, digital clubbing, and secondary erythrocytosis.
ECG Findings	Right axis deviation and right ventricular hypertrophy.
Echocardiography	RV enlargement, paradoxical septal motion, signs of pulmonary hypertension.
Definitive Test	Right heart catheterization to assess pulmonary artery pressure.
Hemoptysis	Due to ruptured pulmonary or bronchial vessels.
Polycythemia	Result of chronic hypoxia; monitor for hyperviscosity syndrome.
Stroke Risk	Paradoxical embolism via right-to-left shunt.
Pregnancy	Strongly contraindicated due to high maternal mortality.
Management	Supportive care, oxygen therapy, and pulmonary vasodilators (e.g., bosentan).
Phlebotomy	Used only for symptomatic hyperviscosity; routine use discouraged.
Oxygen Therapy	May improve symptoms but doesn’t reverse the shunt.
Prognosis	Survival into 3rd–4th decade is common with good care.
Transplant Indication	Progressive symptoms despite maximal therapy.
Contraindicated Drugs	Systemic vasodilators used cautiously due to systemic hypotension.
Neurologic Risk	Brain abscess, stroke due to bypassing pulmonary filter.
Diuretics	May be used cautiously for volume overload.
Monitoring	Echo, SpO₂, hematocrit, functional class regularly.

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Atrial Septal Defect (ASD) with Eisenmenger Syndrome: A Summary

Feature	Atrial Septal Defect (ASD) with Eisenmenger Syndrome: A Summary- Description
Definition	An atrial septal defect is a hole in the wall between the heart’s upper chambers. Eisenmenger syndrome is a complication where high pressure in the pulmonary arteries causes a reversal of the blood flow through the defect, leading to deoxygenated blood circulating to the body.
Causes	Untreated ASD, especially large ones, can lead to increased blood flow to the lungs, damaging pulmonary vessels and increasing pressure, notes the NIH. Over time, this leads to pulmonary hypertension and shunt reversal. Genetic predisposition also plays a role in the development of Eisenmenger syndrome in patients with ASD, according to Cureus.
Symptoms	Symptoms typically develop in puberty or early adulthood and worsen over time. Common symptoms include: • Cyanosis (bluish or grayish skin) • Shortness of breath • Fatigue and dizziness • Chest pain • Clubbing (enlarged fingernails and toenails) • Coughing up blood • Swelling in the legs, ankles, or feet
Complications	Eisenmenger syndrome can lead to serious complications such as: • Arrhythmias • Heart failure • Pulmonary hemorrhage/hemoptysis • Stroke • Brain abscess • Gout • Heart infection (endocarditis) • Pregnancy complications (high risk for both mother and baby)
Management	Focus is on managing symptoms and preventing further heart and lung damage. Surgical correction of the ASD is usually not recommended once Eisenmenger syndrome has developed. Management options may include: • Medications (e.g., pulmonary vasodilators, anticoagulants, diuretics) • Oxygen therapy • Lifestyle modifications (e.g., avoiding strenuous activity, high altitudes, dehydration) • In severe cases, heart-lung or lung transplantation may be considered.

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