Eisenmenger’s Syndrome

Eisenmenger's syndrome (or ES, Eisenmenger's reaction or tardive cyanosis) is defined as the process in which a left to right shunt caused by a congenital heart defect in the fetal heart causes increased flow through the pulmonary vasculature, causing pulmonary hypertension, which in turn causes increased pressures in the right side of the heart and reversal of the shunt into a shunt This occurs when increased pulmonary flow due to an ideal left-to-right shunt produced severe pulmonary hypertension and reversal of the shunt (i.e. right-to-left shunting)

Occurs in:

·         VSD (ventricular septal defect)

·         PDA (Patent ductus arteriosus) &

·         ASD (Arterial septal defect)

Clinical features:

Symptoms:

1.      Dyspnoea.

2.      Fatigue.

3.      Others (syncope, angina, oedema, haemoptysis)

Signs:

1.      Central cyanosis: In PDA cyanosis only present in lower limbs (differential cyanosis).

2.      Clubbing: Differential clubbing (clubbing in toes) in PDA.

3.      Pulse: Low volume.

4.      JVP: large ‘a’ wave.

5.      Left parasternal heave (due to RVH).

6.      On auscultation:

·         Pulmonary hypertension: Loud , prominent

·         Pulmonary ejection systolic click.

·         Early diastolic murmur over pulmonary area.

·         Pansystolic murmur over tricuspid area.

Investigations:

     

1.      Chest X-ray: Enlarged central pulmonary arteries and peripheral ‘pruning’ of the pulmonary vessels.

2.      ECG: right ventricular hypertrophy (RHV).

3.      Echocardiography: Demonstration of the defect.

Treatment: If the hole in the heart is identified before it causes pulmonary hypertension, it can normally be repaired through surgery preventing the disease. After pulmonary hypertension, a heart–lung transplant or a lung transplant with repair of the heart is a possible option. Transplantation is the final therapeutic option and only for patients with poor prognosis and quality of life. Timing and appropriateness of transplantation remain difficult decisions. 5-year and 10-year survival ranges between 70% and 80%, 50% and 70%, 30% and 50%, respectively. Since the average life expectancy of patients after lung transplantation is as low as 30% at 5 years, patients with reasonable functional status related to Eisenmenger syndrome have improved survival with conservative medical care compared with transplantation

Various medicines and therapies for pulmonary hypertension are under investigation for treatment of the symptoms.

Reference:

1.      Davidson’s Principles and practice of Medicine, 21^st edition.

2.      Wikipedia the free encyclopedia.