Hayes C J, Gersony W M, Driscoll D J, Keane J F, Kidd L, O'Fallon W M, Pieroni D R, Wolfe R R, Weidman W H
Department of Pediatric Cardiology, Columbia-Presbyterian Medical Center-Babies Hospital, New York, NY 10032.
Circulation. 1993 Feb;87(2 Suppl):I28-37.
From 1958 to 1969, 592 patients (mostly children) with pulmonary 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 pulmonary valvotomy. Among these with gradients of 50-79 mm Hg, some were managed medically, and some were managed surgically. Most had a second cardiac catheterization at conclusion of NHS-1. More than 15 years had elapsed since NHS-1, and most of the cohort are adults. This report (Second Natural History Study) addresses the long-term results of medical and surgical management.
Of the original cohort of 592 patients, 580 were alive at completion of NHS-1. New data were obtained on 464 (78.4%) of the original cohort. Probability of 25-year survival was 95.7%. Probability of survival was less (80%) in a subgroup of patients entering NHS-1 > 12 years old with cardiomegaly. Less than 20% of patients managed medically during NHS-1 subsequently required a valvotomy. Only 4% of operated patients required a second operation. Bacterial endocarditis occurred rarely. Ninety-seven percent were in New York Heart Association class I. There was a small but higher-than-normal prevalence of serious arrhythmias. Most patients, whether medically or surgically managed, had low Doppler maximum gradients. Final clinical status was excellent or good in 83%.
Probability of survival is similar to that of the general population, and the vast majority of patients are asymptomatic. If valvotomy or valvuloplasty is required in a child, reoperation is rarely necessary. Patients with gradients < 25 mm Hg do not experience an increase in gradient. Patients with a gradient > or = 50 mm Hg should have valvotomy or valvuloplasty. Choice of management of patients with gradients of 40-49 mm Hg remains debatable.
1958年至1969年期间，592例（大多为儿童）肺动脉狭窄患者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的患者接受了肺动脉瓣切开术。在压差为50-79 mmHg的患者中，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，该队列中的大多数患者已成年。本报告（第二次自然史研究）阐述了药物治疗和手术治疗的长期结果。
在最初的592例患者队列中，580例在NHS-1结束时存活。获得了原始队列中464例（78.4%）患者的新数据。25年生存率为95.7%。在NHS-1入组时年龄>12岁且有心脏扩大的患者亚组中，生存率较低（80%）。在NHS-1期间接受药物治疗的患者中，随后需要进行瓣膜切开术的不到20%。仅4%的手术患者需要再次手术。细菌性心内膜炎很少发生。97%的患者为纽约心脏协会I级。严重心律失常的患病率虽低但高于正常水平。大多数患者，无论接受药物治疗还是手术治疗，多普勒最大压差都较低。83%的患者最终临床状况为优良。
生存率与普通人群相似，绝大多数患者无症状。如果儿童需要进行瓣膜切开术或瓣膜成形术，很少需要再次手术。压差<25 mmHg的患者压差不会增加。压差>或=50 mmHg的患者应进行瓣膜切开术或瓣膜成形术。压差为40-49 mmHg的患者的治疗选择仍存在争议。
