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Eisenmenger Syndrome

An advanced state in which untreated large left-to-right shunts progress to pulmonary hypertension with shunt reversal.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Kardiyoloji department. Book Appointment →

What is Eisenmenger Syndrome?

Eisenmenger syndrome develops when uncorrected large left-to-right shunts (such as large VSDs, ASDs, or PDAs) impose prolonged high flow and pressure on the pulmonary bed, leading to advanced pulmonary arterial hypertension.

Right-to-left shunting produces hypoxemia, cyanosis, and secondary erythrocytosis. The condition becomes a multisystem disorder with bleeding, thrombosis, kidney dysfunction, and gallstone risk.

Prevention depends on early diagnosis and closure of the underlying shunt. Once Eisenmenger physiology develops, shunt closure is contraindicated and management is palliative.

Symptoms

Progressive cyanosis and digital clubbing
Exercise intolerance and dyspnea
Syncope and chest pain
Hemoptysis
Features of erythrocytosis (headache, blurred vision)
Right upper-quadrant pain from gallstones
High mortality risk in pregnancy

Risk Factors

Uncorrected large VSD, ASD, or PDA
Variants of tetralogy of Fallot
Truncus arteriosus
Living at high altitude
Late-diagnosed congenital heart disease
Incompletely treated PDA
Large childhood shunts

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Cyanosis developing in a patient with a known shunt
  • New-onset hemoptysis
  • Mandatory counseling for Eisenmenger patients considering pregnancy
  • Recurrent syncope and palpitations

Treatment Methods

01
Pulmonary vasodilator therapy (endothelin receptor antagonists, PDE5 inhibitors, prostacyclins)
02
Oxygen therapy (for hypoxemia)
03
Phlebotomy (in symptomatic hyperviscosity)
04
Anticoagulant therapy (in selected cases)
05
Avoidance of pregnancy and effective contraception
06
Heart-lung transplantation in advanced disease

Which Department to Visit?

You can visit our Kardiyoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.