ĭuring 60 years following Eisenmenger´s report only little insight was gained into the pathophysiology of this disease. In this paper neither pulmonary artery pressure nor pulmonary vascular disease were discussed. Autopsy revealed a nonrestrictive membranous malalignment ventricular septal defect (VSD), marked right ventricular hypertrophy, an overriding aorta, and atheromatosis of the major pulmonary arteries. He succumbed suddenly after massive hemoptysis. This patient had a reasonably active life until 3 years before his death, when dyspnea increased and right heart failure began. In 1897 he reported on a 32-year-old man with cyanosis and dyspnea since infancy. The first clinical description originates from the Viennese physician Victor Eisenmenger (29. Jan. 1864 - 11. Dec. The most severe form of PAH related to congenital cardiac anomalies (CCD) is called Eisenmenger Syndrome (ES). Pulmonary artery wedge pressure, cardiac output and the derived parameters will not be taken into account, and PAH during exercise will not any longer be used for defining PAH. Now, the preassigned upper limit of the normal mean PAP at rest will be 20 mmHg, while 21-24 mmHg is termed “borderline-PAH”, and a mean PAP of more than 25 mmHg as “manifest” PAH. A short time ago, a new grading has been proposed during the 4 th World symposium on PAH 2008 in Dana Point, USA. Until recently pulmonary arterial hypertension (PAH) was defined by a mean pulmonary artery pressure (PAP) of more than 25 mmHg at rest or more than 30 mmHg during exercise. ○ WHO Group IV - Pulmonary hypertension due to chronic thrombotic and/ or embolic diseaseĬongenital cardiac anomalies, associated with PAH, are represented in Group 1 in this clinical classification system. ○ WHO Group III - Pulmonary hypertension associated with lung diseases and/ or hypoxemia ○ WHO Group II - Pulmonary hypertension associated with left heart disease ○ WHO Group I - Pulmonary arterial hypertension (PAH) Overall, the classification system of pulmonary hypertension can be summarized as: 1, Suppl S, 2009, Simonneau et al, Updated Clinical Classification of Pulmonary Hypertension, Pages S43-54, Copyright (2009), with permission from Elsevier. Reprinted from Journal of the American College of Cardiology, Volume 54 No. This medical update emphasizes the current diagnostic and therapeutic options for Eisenmenger patients with particularly focussing on epidemiology, clinical aspects and specific diagnostic options. In such centers, specific interdisciplinary management strategies of physicians specialized on congenital heart diseases and PAH should be warranted. To optimize the quality of life and the outcome, referral of Eisenmenger patients to spezialized centers is required. Unfortunately, data in Eisenmenger patients suffer from small patient numbers and a lack of randomized controlled studies. As new selective pulmonary vasodilators have become available and proven to be beneficial in various forms of pulmonary arterial hypertension, this targeted medical treatment has been expected to show promising effects with a delay of deterioration also in Eisenmenger patients. Due to the chronic slow progressive hypoxemia with central cyanosis, adult patients with the Eisenmenger syndrome suffer from a complex and multisystemic disorder including coagulation disorders (bleeding complications and paradoxical embolisms), renal dysfunction, hypertrophic osteoarthropathy, heart failure, reduced quality of life and premature death.įor a long time, therapy has been limited to symptomatic options or lung or combined heart-lung transplantation. Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension and arises on the basis of congenital heart disease with a systemic-to-pulmonary shunt.
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