Management of Grown Up Congenital Heart Disease

Eisenmenger Syndrome: Not Always Inoperable

“…Pharmacologic treatment often involved the use of digitalis, diuretics, anti-arrhythmics, and/or anticoagulants; however, none of these approaches significantly modified survival or risk of deterioration. 14,15 Research into the underlying pathophysiology of PAH has identified, and continues to identify, several important pathways and mechanisms that are involved in the vascular changes characteristic of the disease, and has led to the development of successful, targeted therapies. Results from clinical trials of these therapies in patients with Eisenmenger syndrome are challenging the notion that it is a stable disease not amenable to treatment.…”

“…1 The structural abnormalities of the lung circulation are histologically similar to those seen in other forms of PAH. 2 Eisenmenger syndrome is a multisystem disorder associated with numerous life-threatening complications, including hemoptysis, cerebrovascular accidents, brain abscesses, arrhythmias, and syncope. 3 Exercise capacity is severely impaired in most patients with Eisenmenger syndrome.…”

Pulmonary Hypertension in Congenital Shunts

“…30 Series reflective of contemporary practice suggest that pulmonary hypertension complicates 2% of patients undergoing home for a minimum of 12-15 hours per day may improve symptoms, but has not been shown to modify survival. 36 Patient education, behavioural modifications and awareness of potential medical risk factors are all important aspects of management. Patients with ES are at particular risk during cardiac and noncardiac surgery and anaesthesia, and as a result of dehydration, chest infections, high altitude and intravenous lines.…”

“…Digoxin has been used in palliative therapy for right heart failure in ES, although available evidence supporting its use is particularly weak. 36 The use of anticoagulation in patients with ES is controversial, as ES patients have a high incidence of pulmonary artery thrombosis, haemoptysis, stroke and an increased risk of haemorrhage. 36 One recent study estimated the prevalent pulmonary artery thrombosis in ES to be 20%, with risk correlating with increasing age, biventricular dysfunction, dilatation of the pulmonary arteries, and concomitantly decreased pulmonary flow velocity.…”

“…36 The use of anticoagulation in patients with ES is controversial, as ES patients have a high incidence of pulmonary artery thrombosis, haemoptysis, stroke and an increased risk of haemorrhage. 36 One recent study estimated the prevalent pulmonary artery thrombosis in ES to be 20%, with risk correlating with increasing age, biventricular dysfunction, dilatation of the pulmonary arteries, and concomitantly decreased pulmonary flow velocity. 37 Although evidence suggests a benefit of such treatment in patients with idiopatic PAH, no data exist in ES, and the haemorrhage associated risks of treatment in these patients may outweigh potential benefits.…”