Yilmaz Ahmet Turan, Ucak Alper, Temizkan Veysel, Ak Koray, Sen Huseyin, Inan Kaan
GATA Haydarpasa Training Hospital, Cardiovascular Surgery, Cardiovascular Surgery Clinic, Istanbul, Turkey.
J Card Surg. 2009 Nov-Dec;24(6):742-7. doi: 10.1111/j.1540-8191.2009.00913.x. Epub 2009 Sep 30.
Ventricular septal defects (VSDs) are one of the most common congenital heart defects in adults. In adult patients with an anatomically large VSD and relatively preserved pulmonary vascular system, several pulmonary flow-limiting cardiac morphologic alterations (PFMA) are encountered.
Ninety-eight male patients (mean age 22.5 +/- 2 years) operated for an anatomically large VSD in our institution were retrospectively reviewed. PFMA in patients with an anatomically large but functionally mild-to-moderate VSD (when ratio of pulmonary to systemic flow (Q(p)/Q(s)) < 2.2 and ratio of pulmonary to systemic vascular resistance (R(p)/R(s)) < 0.3) were recorded.
Thirty patients (31.2%) revealed a mild-to-moderate VSD in functional severity. Five PFMA were encountered in these patients: (1) ostium (os) infundibulum (n = 10, 33.3%), (2) aneurysm of the membranous septum (AMS) (n = 10, 33.3%), (3) systolic bulging of the conal septum toward the right ventricular outflow tract (n = 6, 20%), (4) prolapse of the aortic cusps (n = 2, 6.7%), and (5) attachment of the tricuspid septal leaflet to the septal crest (n = 2, 6.7%). Double-chambered right ventricle was encountered in four patients with os infundibulum and classic tetralogy-type septal malalignment in one patient with aortic cusp prolapse. Concurrent to VSD repair, resection of the os infundibulum and the AMS and aortic valve repair were performed.
Presence of a large VSD and relatively preserved pulmonary vascular system in adults is associated with several PFMA. Preoperative awareness and concurrent surgical treatment of these alterations seem to be crucial to improve the expected benefit of surgical repair of VSD in this subgroup of the patients.
室间隔缺损（VSD）是成人中最常见的先天性心脏缺陷之一。在解剖学上存在大型VSD且肺血管系统相对保留的成年患者中，会出现几种限制肺血流的心脏形态学改变（PFMA）。
回顾性分析了我院98例接受解剖学上大型VSD手术的男性患者（平均年龄22.5±2岁）。记录了解剖学上大型但功能上轻度至中度VSD患者（肺循环与体循环血流量之比（Q(p)/Q(s)）<2.2且肺循环与体循环血管阻力之比（R(p)/R(s)）<0.3）的PFMA情况。
30例患者（31.2%）功能严重程度为轻度至中度VSD。这些患者出现了5种PFMA：（1）漏斗部开口（os）（n = 10，33.3%），（2）膜周部室间隔瘤（AMS）（n = 10，33.3%），（3）圆锥间隔向右心室流出道的收缩期膨出（n = 6，20%），（4）主动脉瓣叶脱垂（n = 2，6.7%），以及（5）三尖瓣隔叶附着于室间隔嵴（n = 2，6.7%）。4例漏斗部开口患者出现了双腔右心室，1例主动脉瓣叶脱垂患者出现了典型的四联症型室间隔排列不齐。在VSD修复的同时，进行了漏斗部开口和AMS的切除以及主动脉瓣修复。
成人中存在大型VSD且肺血管系统相对保留与几种PFMA有关。术前认识并同时对这些改变进行手术治疗似乎对于提高该亚组患者VSD手术修复的预期获益至关重要。
