Ose L, McKusick V A
Birth Defects Orig Artic Ser. 1977;13(3C):163-9.
Twenty-five patients, 16 men and 9 women, 5-59 years of age with the Marfan syndrome and cardiac complications were started on propranolol over a 6-year period. Propranolol was used as a beta-adrenergic blocking agent to reduce myocardial contractility in an attempt thereby to stay the progression of aortic dilatation and to prevent acute dissection of the aorta. The indications for prophylactic treatment were aortic dilatation, with aortic regurgitation in most cases. It was intended to keep the pulse rate below 70 at all times or below 60 at rest. Propranolol given in daily doses of 120-160 mg caused no side effects. The mean observation time for propranolol treatment in the 25 patients was 3.0 +/- 1.8 years ranging from 1-7 years. In spite of treatment, 5 patients (1 female and 4 males) experienced acute aortic dissection of rupture with fatal outcome. This occurred in 2 patients with the asthenic type, in 2 patients with the nonasthenic type, and in 1 patient with the marfanoid hypermobility syndrome. Serial echocardiograms showed that other patients on propranolol developed increasing dilatation of the aortic root. These observations indicate that propranolol does not necessarily protect against aortic dissection nor stop the progression of the aortic dilatation when cystic medial necrosis of the aorta is already present.
25例年龄在5至59岁之间、患有马方综合征并伴有心脏并发症的患者（16名男性和9名女性）在6年期间开始服用普萘洛尔。普萘洛尔作为一种β肾上腺素能阻滞剂，用于降低心肌收缩力，从而试图延缓主动脉扩张的进程并预防主动脉急性夹层形成。预防性治疗的指征是主动脉扩张，大多数情况下伴有主动脉反流。目标是使心率始终保持在70次/分钟以下或静息时在60次/分钟以下。每日剂量为120 - 160毫克的普萘洛尔未引起副作用。25例患者接受普萘洛尔治疗的平均观察时间为3.0±1.8年，范围为1至7年。尽管进行了治疗，仍有5例患者（1名女性和4名男性）发生了急性主动脉夹层破裂并导致死亡。这发生在2例无力型患者、2例非无力型患者以及1例马方样关节过度活动综合征患者身上。系列超声心动图显示，其他服用普萘洛尔的患者主动脉根部扩张加剧。这些观察结果表明，当主动脉中层囊性坏死已经存在时，普萘洛尔不一定能预防主动脉夹层形成，也无法阻止主动脉扩张的进展。
