pediatrics
Eisenmenger Syndrome
Treatment of Eisenmenger Syndrome
Medications to treat pulmonary arterial hypertension (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) "Treat and repair" approach Supportive care Heart and lung transplantation Ideally, corrective operations should have been done earlier to prevent Eisenmenger syndrome. There is no specific treatment once the syndrome develops, other than heart and lung transplantation, but medications that may lower pulmonary artery pressure have been shown to have at least a modest beneficial effect on survival ( Treatment references ). Prostacyclin analogs (eg,treprostinil,epoprostenol), endothelin antagonists (eg,bosentan), and phosphodiesterase-5 inhibitors (eg,sildenafil,tadalafil) have been shown to improve performance on 6-minute walk tests and to reduce N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. In a small number of patients, aggressive therapy with pulmonary vasodilating drugs has resulted in net left-to-right shunting, allowing surgical repair of the underlying cardiac defect and significant reduction in mean pulmonary artery pressure. This has been called the treat and repair approach. Supportive treatment includes avoidance of conditions that may exacerbate the syndrome (eg, pregnancy, volume depletion, isometric exercise, high altitudes, smoking). Supplemental oxygen may provide some benefit. Symptomatic polycythemia can be treated by cautious phlebotomy to lower hematocrit to 55 to 65% plus simultaneous volume replacement with normal saline. However, compensated and asymptomatic polycythemia does not require phlebotomy, regardless of hematocrit. Phlebotomy eventually leads to iron deficiency, which is associated with a higher risk of adverse outcomes. In case of confirmed iron deficiency, supplemental iron should be carefully administered to replenish iron stores. Hyperuricemia can be treated withallopurinol300 mg orally once a day. therapy is potentially harmful due to the risk of pulmonary hemorrhage, so anticoagulant use should be individualized ( Treatment references ). Although there is no clear evidence, low-dose Life expectancy depends on type and severity of the underlying congenital anomaly and ranges from 20 to 50 years. In untreated patients, low exercise tolerance and secondary complications severely limit quality of life. Use of advanced pulmonary vasodilator therapies has been shown to improve functional capacity and appears to improve survival. Lung and Heart-Lung Transplantation or All patients should be given Prevention before dental or surgical procedures that are likely to cause bacteremia. 1.Diller GP, Korten MA, Bauer UMM, et al: Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects.Eur Heart J37(18):1449–1455, 2016. doi: 10.1093/eurheartj/ehv743 2.Kempny A, Hjortshoj CS, Gu H, et al: Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicenter study.Circulation135(15): 1432–1440, 2017. doi: 10.1161/CIRCULATIONAHA.116.023033 