荷兰成年人先天性冠状动脉瘘调查。

Dutch survey of congenital coronary artery fistulas in adults.

作者信息

Said S A M, van der Werf T

出版信息

Neth Heart J. 2006 Jan;14(1):5-13.

PMID:25696548

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2557221/

Abstract

AIMS

This Dutch survey focused on the clinical presentation, noninvasive and invasive diagnostic methods, and treatment modalities of adult patients with congenital coronary artery fistulas (CAFs).

METHODS

Between 1996 and 2003, the initiative was taken to start a registry on congenital CAFs in adults. In total 71 patients from a diagnostic coronary angiographic population of 30,829 at 28 hospitals were collected from previously developed case report forms. Patient demographic data, clinical presentation, noninvasive and invasive techniques and treatment options were retrospectively collected and analysed.

RESULTS

Out of 71 patients with angiographically proven CAFs, 51 (72%) had 63 congenital solitary fistulas and 20 (28%) had 31 congenital coronary-ventricular multiple microfistulas. Patients with pseudofistulas were excluded from the registry. Coronary angiograms were independently re-analysed for morphology and specific fistula details. The majority (72%) of the fistulas were unilateral, 24% were bilateral and only 4% were multilateral. The morphological characteristics of these 94 fistulas were as follows: the origin was multiple in 47% and single in 53%; the termination was multiple in 52% and single in 48%; and the pathway of the fistulous vessels was tortuous/multiple in 66%, tortuous/single in 28%, straight/multiple in 3% and straight/single in 3%. Percutaneous transluminal embolisation (PTE) was performed in two (3%) patients; surgical ligation was undertaken in 13 (18%) patients. The overwhelming majority of the patients (56; 79%) were treated with conservative medical management. The total mortality was 6% (4/71) at a mean follow-up period of approximately five years. Cardiac mortality accounted for 4% (3/71); in all three patients, death could possibly be attributed to the presence of the fistula.

CONCLUSION

Registry of congenital coronary artery fistulas in adults in the Netherlands is feasible. In spite of restrictions imposed by the Dutch Privacy Law, it was possible to include 71 adult patients with congenital coronary artery fistulas who were eligible for thorough evaluation.

摘要

目的

这项荷兰的调查聚焦于成年先天性冠状动脉瘘（CAF）患者的临床表现、非侵入性和侵入性诊断方法以及治疗方式。

方法

1996年至2003年期间，发起了一项针对成年先天性CAF的登记工作。从28家医院的30829例诊断性冠状动脉造影人群中，根据先前制定的病例报告表共收集了71例患者。对患者的人口统计学数据、临床表现、非侵入性和侵入性技术以及治疗选择进行回顾性收集和分析。

结果

在71例经血管造影证实患有CAF的患者中，51例（72%）有63处先天性孤立性瘘，20例（28%）有31处先天性冠状动脉 - 心室多发性微瘘。假性瘘患者被排除在登记之外。对冠状动脉造影进行独立的形态学和特定瘘管细节再分析。大多数（72%）瘘为单侧，24%为双侧，仅4%为多侧。这94处瘘的形态学特征如下：起源多处占47%，单处占53%；终止多处占52%，单处占48%；瘘管血管走行迂曲/多处占66%，迂曲/单处占28%，笔直/多处占3%，笔直/单处占3%。2例（3%）患者接受了经皮腔内栓塞术（PTE）；13例（18%）患者进行了手术结扎。绝大多数患者（56例；79%）接受了保守药物治疗。平均随访约五年时，总死亡率为6%（4/71）。心脏死亡率占4%（3/71）；在所有3例患者中，死亡可能归因于瘘的存在。