1st Cardiology Department, University of Medical Sciences, Poznań, Poland.
Cardiol J. 2009;16(6):500-6.
Patients with Eisenmenger syndrome form a small percentage of congenital heart disease patients. The rarity of this syndrome, combined with its complex pathophysiology, account for the insufficient understanding of the principles underlying its proper treatment. The main clinical symptoms are: cyanosis due to secondary erythrocytosis, resulting in increased blood viscosity, iron deficiency anemia (enhanced by unnecessary phlebotomies), blood clotting disturbances, heart failure and serious supraventricular and ventricular arrhythmias. Recent decades have seen developments in pulmonary hypertension pathophysiology which have led to the introduction of new groups of drugs: prostacycline analogs (Epoprostenol, Treprostinil, Beraprost, Illoprost), phosphodiesterase inhibitors (Sildenafil, Tadalafil), endothelin receptor antagonists (Bosentan, Sitaxantan, Ambrisentan) and nitric oxide. These drugs should be administered to patients in III-IV NYHA class. Despite successful early results, the therapeutic effect on patients with Eisenmenger syndrome has not been conclusively established. Our therapeutic efforts should be directed mainly towards preventing complications. As a rule, we should avoid agents with no established therapeutic efficacy and try to alleviate symptoms without any additional risk, so as not to disrupt the existing clinical balance.
艾森曼格综合征患者在先天性心脏病患者中占比较小。该综合征较为罕见，且其病理生理学较为复杂，这导致其治疗原则尚未被充分理解。其主要临床症状为：继发红细胞增多导致的发绀，进而引起血液黏度增加、缺铁性贫血（因不必要的放血而加重）、凝血障碍、心力衰竭和严重的室上性和室性心律失常。近几十年来，肺动脉高压的病理生理学的发展导致了新的药物类别出现：前列环素类似物（依前列醇、曲前列尼尔、贝前列素、伊洛前列素）、磷酸二酯酶抑制剂（西地那非、他达拉非）、内皮素受体拮抗剂（波生坦、司他生坦、安立生坦）和一氧化氮。这些药物应在 III-IV 级纽约心脏协会（NYHA）心功能分级的患者中使用。尽管早期治疗效果成功，但艾森曼格综合征患者的治疗效果尚未得到明确证实。我们的治疗重点应主要放在预防并发症上。一般来说，我们应避免使用疗效尚未确定的药物，并尽量在不增加任何额外风险的情况下缓解症状，以免破坏现有的临床平衡。
