Keane J F, Driscoll D J, Gersony W M, Hayes C J, Kidd L, O'Fallon W M, Pieroni D R, Wolfe R R, Weidman W H
Department of Pediatric Cardiology, Children's Hospital, Boston, Mass.
Circulation. 1993 Feb;87(2 Suppl):I16-27.
From 1958 to 1969, 462 patients (mostly children) with aortic stenosis were admitted to the First Natural History Study of Congenital Heart Defects (NHS-1) after cardiac catheterization. Most with gradients < 50 mm Hg were managed medically; most with gradients > or = 80 mm Hg had aortic valvotomy. Of those with gradients of 50-79 mm Hg, some were managed medically, and some were managed surgically. Most had a second cardiac catheterization at the conclusion of NHS-1. More than 15 years have elapsed since NHS-1, and most of the cohort are adults. This report (the Second Natural History Study [NHS-2]) addresses the long-term results of medical and surgical management.
Of the original cohort of 462 patients, 440 were alive at completion of NHS-1. New data were obtained on 371 (80.3%) of the original cohort. Probability of 25-year survival was 92.4% for those admitted with gradients < 50 mm Hg and 81.0% for those with admission gradients > or = 50 mm Hg. More than half of the cardiac deaths were sudden and unexpected. Forty percent of patients managed medically during NHS-1 subsequently required surgical management. Almost 40% of operated patients required a second operation. Three percent of the original cohort subsequently had bacterial endocarditis. There was a higher-than-normal prevalence of serious arrhythmias. Of NHS-2 full participants, 92.3% were in New York Heart Association functional class I. Most patients had low Doppler mean gradients. Clinically, 46.6% had aortic valve regurgitation. The final clinical status was excellent in 29.9%, good in 22.8%, fair in 28.6%, and poor in 18.7%.
Patients with gradients < 25 mm Hg can be followed medically; however, progressive stenosis can occur, and approximately 20% will require intervention. If the gradient is > or = 50 mm Hg, there is a risk of serious arrhythmias and, possibly, sudden death. If the gradient is > or = 80 mm Hg, intervention is clearly indicated; as techniques improve, delaying intervention in patients with gradients of 50-79 mm Hg may not be advantageous. Patients with gradients of 25-49 mm Hg can be followed medically with annual evaluation.
1958年至1969年，462例主动脉狭窄患者（大多为儿童）在接受心导管检查后被纳入先天性心脏病首次自然史研究（NHS - 1）。大多数压差<50 mmHg的患者接受药物治疗；大多数压差>或=80 mmHg的患者接受了主动脉瓣切开术。对于压差在50 - 79 mmHg的患者，一些接受药物治疗，一些接受手术治疗。大多数患者在NHS - 1结束时进行了第二次心导管检查。自NHS - 1以来已过去15年多，该队列中的大多数人已成年。本报告（第二次自然史研究[NHS - 2]）阐述了药物和手术治疗的长期结果。
在最初的462例患者队列中，440例在NHS - 1结束时存活。获取了原始队列中371例（80.3%）患者的新数据。入院时压差<50 mmHg的患者25年生存率为92.4%，入院时压差>或=50 mmHg的患者为81.0%。超过一半的心脏死亡是突然且意外的。在NHS - 1期间接受药物治疗的患者中，40%随后需要手术治疗。近40%接受手术的患者需要再次手术。原始队列中有3%的患者随后发生了感染性心内膜炎。严重心律失常的患病率高于正常水平。在NHS - 2的全部参与者中，92.3%处于纽约心脏协会心功能I级。大多数患者的多普勒平均压差较低。临床上，46.6%的患者有主动脉瓣反流。最终临床状况优秀的占29.9%，良好的占22.8%，中等的占28.6%，差的占18.7%。
压差<25 mmHg的患者可进行药物随访；然而，可能会发生进行性狭窄，约20%的患者将需要干预。如果压差>或=50 mmHg，存在严重心律失常甚至可能猝死的风险。如果压差>或=80 mmHg，显然需要进行干预；随着技术的改进，对于压差在50 - 79 mmHg的患者延迟干预可能并无益处。压差在25 - 49 mmHg的患者可每年进行一次评估并接受药物随访。
