Eisenmenger syndrome: medical prevention and management strategies

Abstract

The original definition of Eisenmenger syndrome refers to an unrestrictive post-tricuspid valve congenital systemic-to-pulmonary shunt. When the pulmonary arterial systolic pressure becomes equal to the systemic arterial systolic pressure, the direction of the shunt becomes pulmonary-to-systemic. The latter leads to progressive cyanosis, and exercise intolerance is initially proportional to the degree of hypoxaemia. Later, congestive heart failure may occur . The management principle is to avoid any factors that destabilise this delicately balanced physiology. Until recently, this could only be achieved by symptomatic therapy; however, when patients are severely incapacitated, transplantation is needed. At present, new drugs, which are more selective pulmonary vasodilators, are available to interfere with the ongoing disease process to improve functional capacity and delay the decision for transplantation.

Massive epistaxis in a patient with Eisenmenger syndrome: illustrating the clot-versus-bleed conundrum

“…Jensen et al 9 presented a similar case describing haemoptysis with PAT in 2007. Epistaxis has been mentioned in review articles10 11 but anti-platelet drug induced heavy epistaxis, although logically feasible, has not been reported.…”

What do Cardiovascular Nurses Know about the Hematological Management of Patients with Eisenmenger Syndrome?

“…It is estimated that only 1% to 4% of patients with congenital heart disease develop Eisenmenger syndrome [5,6]. The fact that less than half of the CHD nurses knew the procedure of isovolumic phlebotomy may be because some centers do not use phlebotomy in their management, although it is recommended by international experts [1,7,8], or because phlebotomies are performed by nurses other than CHD ones. Since CHD nurses are often the resource person for coaching and guiding patients and for consultation by other nurses [9], they should be well informed about the condition and its appropriate management.…”

Eisenmenger Syndrome: A Clinical Review

“…Reduced stroke volume increase the right-to-left shunt which can lead to increased cyanosis and tissue hypoxia. Calcium channel blockers, as well as other afterload reducing drugs, should be avoided in patients with Eisenmenger syndrome [3].…”

“…Pulmonary vasodilating therapy consists of either prostacyclins, endothelin receptor antagonists, or phosphodiesterase inhibitors (PDE-inhibitor). Prostacyclin can be administered intravenously (epoprostenol), subcutaneously (treprostinil), orally (Beraprost sodium), or via inhalation (iloprost); endothelin antagonists (bosentan, sitaxsentan) are administered orally; and PDE-inhibitors (sildenafil) can be administered both orally as intravenously [3]. More studies are ongoing to determine the efficacy of newer molecules, and the timing when to start with these specific agents.…”

“…It wasn't until 1958 that Dr. Paul Wood broadened the anatomic concept of the Eisenmenger complex and redefined to describe the underlying pathophysiology as “pulmonary hypertension at systemic level, due to a high pulmonary vascular resistance (> 800 dynes-sec/cm 5 ), with reversed or bidirectional shunt through a large ventricular septal defect (1.5–3 cm across)” [2]. Since then, the term Eisenmenger syndrome has been used to describe a variety of cardiac lesions which are hemodynamically similar because of the presence of pulmonary vascular disease and cyanosis resulting from any systemic-to-pulmonary circulation communication with a left-to-right shunts, such as atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, truncus arteriosus and single ventricle anomalies [3]. Eisenmenger physiology may also occur in patients with large surgically created extracardiac left-to-right shunts which initially cause increased pulmonary blood flow.…”