Eisenmenger syndrome and complications

Eisenmenger syndrome and complications

Eisenmenger syndrome and complications: Eisenmenger syndrome can be considered as the final common pathway for all significant left to shunts in which unrestricted pulmonary blood flow leads to obstructive changes in the pulmonary vascular tree. This leads to progressive pulmonary hypertension, which is initially reactive and reversible, but later on obliterative and irreversible. In this stage there is reversal of the shunt which becomes right to left with systemic desaturation. Generally this process occurs only with pulmonary to systemic flow ratio (Qp:Qs) of more than 2:1. Eisenmenger syndrome with ventricular septal defect is known as Eisenmenger complex.

Due to chronic hypoxia as a result of systemic desaturation, erythropoietin levels are elevated and causes polycythemia in Eisenmenger syndrome. Excessive polycythemia can cause hyperviscosity syndrome. Symptomatic cases need phlebotomy, with care to avoid microcytosis due to iron deficiency which is the strongest predictor for cerebrovascular events. Microcytosis also causes decompensated erythrocytosis as they are less deformable and produce renal ischemia, being unable to negotiate the renal microvasculature. Renal ischemia in turn increases erythropoietin levels, further worsening polycythemia.

Platelet function abnormalities and coagulopathy are also common in Eisenmenger syndrome. Thrombotic complications like stroke can occur, especially in the setting of dehydration or following contrast angiography. Brain abscesses are also potentially serious complications to be looked for especially when they present with neurological deficit. Cerebral microembolism and airway hemorrhages can occur. Airway hemorrhages have to be thought of specially when moving from lower to a higher altitude as during air travel or while ascending a mountain.

Even though it is very important to rule out correctable causes in a patient with suspected Eisenmenger syndrome, it is equally important to avoid aggressive testing as patients can die during cardiovascular procedures. Adequate hydration prior and during any contrast angiography is essential to prevent stroke. Oxygen therapy may have a role in reducing the levels of pulmonary hypertension.

In a study of 1098 patients with Eisenmenger syndrome, 405 were on advanced therapy for pulmonary arterial hypertension at baseline [1]. Most of these patients were on endothelin receptor antagonist, phosphodiesterase 5 inhibitor or prostacyclin analog. Only about 10% of the patients were on dual therapy. As patients were recommended advanced therapy during the study period, by the end of the study, 690 patients were on advanced therapy for pulmonary hypertension. There were 278 deaths in the study period, of which 51 were in pre tricuspid defects, 130 in post tricuspid defects and 97 in complex cases. Median follow up period of the study was 3.1 years.

Definitive therapy of Eisenmenger syndrome can be in the form of heart lung transplantation if feasible.

Reference

1. Kempny A, Hjortshøj CS, Gu H, Li W, Opotowsky AR, Landzberg MJ, Jensen AS, Søndergaard L, Estensen ME, Thilén U, Budts W, Mulder BJ, Blok I, Tomkiewicz-Pająk L, Szostek K, D’Alto M, Scognamiglio G, Prokšelj K, Diller GP, Dimopoulos K, Wort SJ, Gatzoulis MA. Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study. Circulation. 2017 Apr 11;135(15):1432-1440.