Eisenmenger syndrome

Published on 02/04/2015 by admin

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28 Eisenmenger syndrome

Instruction

Examine this patient’s heart.

Examine this patient’s cardiovascular system.

Salient features

History

• Symptoms may not appear until early late childhood or early adulthood

• Cyanosis (appears as right-to-left shunting develop)

• Dyspnoea on exercise and impaired exercise tolerance

• Palpitations (common and usually caused by atrial fibrillation or flutter)

• Angina of effort

• Haemoptysis (may occur as a result of pulmonary infarction, or rupture of dilated pulmonary arteries or aorticopulmonary vessels)

• Syncope (owing to inadequate cardiac output or, less commonly, an arrhythmia)

• Symptoms of hyperviscosity including visual disturbances, fatigue, headache, dizziness and paraesthesia

• Symptoms of heart failure are uncommon until the disease is in advanced stages.

Examination

• Clubbing of fingers and central cyanosis

• An ‘a’ waves in the JVP, ‘v’ wave if tricuspid regurgitation is also present

• Left parasternal heave and palpable P2

• Loud P2, pulmonary ejection click, early diastolic murmur of pulmonary regurgitation (Graham Steell murmur)

• Loud pansystolic murmur of tricuspid regurgitation

• Listen carefully to the second sound. The clinical findings from the underlying defect are as follows:

• Ventricular septal defect (VSD): single second sound
• Atrial septal defect (ASD): fixed, wide split second sound
• Patent ductus arteriosus (PDA): reverse split of second sound and differential cyanosis where lower-limb cyanosis is marked.

Diagnosis

This patient has Eisenmenger syndrome with a shunt at the ventricular level (lesion) that is congenital in origin (aetiology), and severe pulmonary hypertension (functional status).

Questions

What do you understand by the term Eisenmenger syndrome?

Pulmonary hypertension with a reversed or bidirectional shunt. It matters very little where the shunt happens to be (e.g. VSD, ASD, PDA, persistent truncus arteriosus, single ventricle or common atrioventricular canal).

Advanced-level questions

What do you understand by the term Eisenmenger complex?

Eisenmenger complex is a VSD with a right-to-left shunt in the absence of pulmonary stenosis. The onset of Eisenmenger syndrome is often heralded by a softening of the murmur, a decrease in the left heart size and in increase in the second pulmonic sound.

Mention some cyanotic heart diseases of infancy

• Tetralogy of Fallot

• Transposition of the great vessels

• Tricuspid regurgitation

• Total anomalous pulmonary venous connection.

What is the age of onset of Eisenmenger syndrome?

In the case of PDA and VSD, about 80% occur in infancy, whereas in the case of ASD over 90% occur in adult life.

What factors worsen deteriorate pulmonary hypertension in these patients?

• Pregnancy

• Dehydration or acute vasodilation (e.g. sauna, hot tub)

• Increased fluid volume

• Worsened renal or hepatic function

• Chronic environmental hypoxia

• Increased left-sided filling pressure:

• Left ventricular diastolic dysfunction
• Obstructive congenital lesion
• Myocardial restriction
• Systemic hypertension with increased left ventricular afterload.

• Erythrocytosis and increased blood viscosity; anaemia

• Hypercoagulability: thrombosis

• Acute infection

• Arrhythmias.

(See Clin Chest Med 2007;28:243–53.)

What are the complications of Eisenmenger syndrome?

• Haemoptysis

• Erythrocytosis

• Right ventricular failure

• Cerebrovascular accidents (as a result of paradoxical embolization, venous thrombosis of cerebral vessels or intracranial haemorrhage)

• Sudden death

• Brain abscess

• Bleeding and thrombosis (patients at increased risks for both as a consequence of an abnormal haemostasis secondary to chronic arterial desaturation)

• Paradoxical embolization

• Infective endocarditis

• Hyperuricaemia

• Recurrent haemoptysis.

How would you investigate this patient?

• Electrocardiogram shows right ventricular hypertrophy; atrial arrhythmias particularly in those with underlying ASD.

• Chest radiograph shows conspicuous dilatation of the pulmonary artery (Fig. 28.1) with narrowed ‘pruned’ peripheral vessels (caused by pulmonary hypertension); slight to moderate enlargement of the heart (predominantly RV) may be seen in ASD whereas the size of the heart is normal in VSD or PDA.

• Echocardiography provides evidence of right ventricular overload and pulmonary hypertension; the underlying cardiac defect can be visualized, although shunting may be difficult to demonstrate by colour Doppler imaging because of low velocity jet; contrast echocardiography permits localization of shunt.

• Cardiac catheterization determine the extent and severity of pulmonary vascular disease and accurately quantify the magnitude of the intracardiac shunting; assessment of reversibility of shunting is done using pulmonary vasodilators (e.g. oxygen, inhaled nitrous oxide, intravenous adenosine or epoprostenol).

image

Fig. 28.1 Chest radiograph demonstrating gross dilatation of the main, left and right pulmonary arteries in a patient with Eisenmenger ASD.

(With permission from Adam et al. 2008.)

What is the prognosis in these patients?

• Survival is 80% 10 years after diagnosis, 77% at 15 years, and 42% at 25 years.

• Death is usually sudden; other causes include heart failure, haemoptysis, brain abscess or stroke.

• Poor prognostic factors include syncope, clinically evident right ventricular systolic dysfunction, low cardiac output and severe hypoxaemia.

Is pregnancy safe in this patient?

Pregnancy is associated with a high incidence of early spontaneous abortion and rarely results in the birth of a healthy child. Mortality of the mother is high (30–60%) in those with underlying VSD, particularly in late pregnancy and postpartum period. Pregnancy is, therefore, contraindicated and if occurs is best terminated at an early stage. If pregnancy proceeds to term, a vaginal delivery is the preferred route with careful management of hydration, arrhythmias and hypoxaemia. Epidural anaesthesia is preferred over general anaesthesia in complicated cases.

What treatment is available for Eisenmenger syndrome?

• Phlebotomy done cautiously in symptomatic hyperviscosity due to secondary erythrocytosis when the haematocrit >0.65 (but not due to dehydration). Always replace fluid intravenously following phlebotomy

• Long-term intravenous epoprostenol (Circulation 1999;99:1858–65; Ann Intern Med 1999;130:740–3)

• Uncontrolled studies suggest that prostacyclin analogues and phosphodiesterase 5 inhibitors (e.g. sildenafil) may have benefits in advanced pulmonary vascular disease

• In a randomized, controlled trial of bosentan versus placebo, bosentan showed a significant improvement in exercise capacity based on 6-minute walk distance, with serious safety concerns (Circulation 2006;114:48–54)

• Combined heart–lung transplantation (limited success and hence patients should be carefully selected).

Victor Eisenmenger was a German physician who described this condition in an infant in 1897. His patient had cyanosis since infancy and a fairly good quality of life until he succumbed at the age of 32 years. The patient was active until the age of 29 years when he developed right heart failure and died 3 years later following a massive haemoptysis. Post-mortem revealed a large VSD (2.5 cm) with both the LV and RV with equally thick walls. The pulmonary arteries revealed atheroma with multiple thrombi leading to pulmonary infarctions.

Paul Wood at the Brompton Hospital in 1958 published a study of 127 patients and was the first to suggest that Eisenmenger reaction occurred with defects other than the ventricular level (BMJ 1958;2:701, 709).

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