Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan.
Department of Cardiovascular Surgery, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan.
Cardiol Young. 2021 Apr;31(4):556-561. doi: 10.1017/S1047951120004424. Epub 2020 Dec 11.
Infants with complete atrioventricular septal defect occasionally accompany pulmonary hypertension; however, the pulmonary circulation can be altered by pulmonary vascular conditions as well as the left heart lesions. This study aimed to explore whether the left heart lesions were related to the pulmonary circulation among them.
We performed echocardiography and cardiac catheterisation in 42 infants with complete atrioventricular septal defect and studied relationships between the pulmonary haemodynamic parameters and the left heart morphology.
Age and weight at preoperative evaluation were 65 days (47-114) (the median following interquartile range) and 5.5 kg (4.0-7.1), respectively. There were 27 individuals with Down syndrome. Gestational age was 38 weeks (37-39). Catheterisation showed mean pulmonary arterial pressure: 36 (29-46) mmHg, the ratio of pulmonary to systemic blood flow: 3.45 (2.79-4.98), pulmonary vascular resistance: 2.20 Wood units·m2 (1.53-3.65), and pulmonary arterial compliance: 2.78 (1.86-4.10) ml/Hg/m2. Echocardiography showed the Rastelli classification type A in 28 and type C in 14, moderate or severe left atrioventricular valve regurgitation in 19 patients (45%), atrioventricular valve index of 0.67 (0.56-0.79), left ventricular end-diastolic volume z score of 4.46 (1.96-7.78), and aortic valve diameter z score of -0.70 (-1.91 to 0.20). Multivariable regression analysis revealed that preoperative pulmonary vascular resistance was significantly correlated to gestational age (p = 0.002), and that preoperative pulmonary arterial compliance was significantly correlated to gestational age (p = 0.009) and Down syndrome (p = 0.036).
The pulmonary circulation does not depend upon the presence of left heart lesions but gestational age and Down syndrome in infants with complete atrioventricular septal defect.
完全性房室间隔缺损的婴儿偶尔伴有肺动脉高压；然而，肺循环也可能因肺血管状况和左心病变而改变。本研究旨在探讨其中左心病变是否与肺循环有关。
我们对 42 例完全性房室间隔缺损婴儿进行了超声心动图和心导管检查，并研究了肺血流动力学参数与左心形态之间的关系。
术前评估时的年龄和体重分别为 65 天（47-114）（中位数）和 5.5kg（4.0-7.1），其中 27 例为唐氏综合征。胎龄为 38 周（37-39）。导管检查显示平均肺动脉压为 36（29-46）mmHg，肺血流量与体循环血流量之比为 3.45（2.79-4.98），肺血管阻力为 2.20 伍德单位·m2（1.53-3.65），肺动脉顺应性为 2.78（1.86-4.10）ml/Hg/m2。超声心动图显示 Rastelli 分类 A 型 28 例，C 型 14 例，19 例（45%）中度或重度房室瓣反流，房室瓣指数 0.67（0.56-0.79），左室舒张末期容积 z 评分 4.46（1.96-7.78），主动脉瓣直径 z 评分-0.70（-1.91 至 0.20）。多变量回归分析显示，术前肺血管阻力与胎龄显著相关（p=0.002），而术前肺动脉顺应性与胎龄（p=0.009）和唐氏综合征（p=0.036）显著相关。
在完全性房室间隔缺损的婴儿中，肺循环不依赖于左心病变的存在，而依赖于胎龄和唐氏综合征。
