Application value of fetal echocardiography in the evaluation of pulmonary atresia with intact ventricular septum

doi:10.3877/cma.j.issn.1672-6448.2021.08.008

Abstract

Abstract:

Objective

To assess the value of fetal echocardiography in the evaluation of pulmonary atresia with intact ventricular septum (PA/IVS).

Methods

Clinical data of 29 fetuses with PA/IVS diagnosed by prenatal echocardiography at Hebei Maternity Hospital from October 2017 to December 2020 were analyzed retrospectively. Right ventricle length/left ventricle length (RV/LV), tricuspid valve inflow duration/cardiac cycle length (TVID/CCL), tricuspid valve annulus/mitral valve annulus (TV/MV), and tricuspid Z score (TV-Z) were measured by echocardiography. The flow spectrum of the ductus venosus (DV), atrioventricular valve regurgitation, internal diameter and blood flow direction of the ductus arteriosus (DA), the presence of pericardial effusion or not, the presence of combined coronary artery abnormalities or not, and right ventricular dependent coronary circulation (RVDCC) were observed to carry out clinical typing for the 29 fetuses and analyze echocardiographic characteristics.

Results

Prenatal echocardiography in all the 29 cases of PA/IVS showed the reverse flow of the DA to the pulmonary artery. Massive tricuspid regurgitation was observed in 24 cases. Not obvious tricuspid regurgitation or low-to-moderate tricuspid regurgitation was observed in five cases. Missing or reverse A-wave of the DV was observed in 18 cases. Normal venous duct spectrum was observed in 11 cases. According to clinical typing results, there were 12 cases of type Ⅰ PA/IVS, 9 cases of type Ⅱ, 3 cases of type Ⅲ, and 5 cases of type Ⅳ among the 29 PA/IVS fetuses, and there were significant differences in RV/LV, TVID/CCL, TV/MV, TV-Z, and other parameters among fetuses with different types of PA/IVS. Abnormal coronary artery was found in 3 cases, 1 case with abnormal blood flow signal across the myocardium of the right ventricle (type Ⅳ), and 2 cases complicated with coronary artery-right ventricular fistula (1 case with type Ⅲ and 1 case with type Ⅳ); these three cases were considered to be complicated with RVDCC.

Conclusion

Echocardiography has significant value in prenatal diagnosis, clinical typing ,and prognosis evaluation of PA/IVS. Combined application of multiple echocardiographic indexes is helpful for the accurate evaluation of PA/IVS, thus providing a basis for postpartum treatment.

Key words: Echocardiography, Fetal heart, Pulmonary atresia with intact ventricle septum, Tricuspid valve Z score, Prenatal diagnosis

Kun Wang, Xiaohua Zhang, Fengrui Yi, Jie Liu, Fengqun Dong. Application value of fetal echocardiography in the evaluation of pulmonary atresia with intact ventricular septum[J]. Chinese Journal of Medical Ultrasound (Electronic Edition), 2021, 18(08): 759-764.

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References 17

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PA/IVS分型	例数	右心室大小	Roman评价体系（包括①②③④）^a	三尖瓣Z值	心胸面积比	水肿
Ⅰ型	2	右心室增大	无	2.53、4.20	0.40、0.48	心包积液
Ⅰ型	10	右心室偏小	TV/MV：0.72~0.93，RV/LV：0.66~0.85，其中1例符合③	-0.09~-2.92	正常	无
Ⅱ型	6	右心室明显减小，无RVDCC	符合①②	-3.15~-4.02	正常	无
Ⅱ型	3	右心室明显减小，无RVDCC	符合①②③	-3.56~-4.28	正常	无
Ⅲ型	1	右心室明显减小	符合①②③④，可见左冠状动脉-右心室交通	-5.21	正常	无
Ⅲ型	2	右心室明显减小	符合①②③	-3.64、-4.47	正常	无
Ⅳ型	2	右心室明显减小	符合①②③④，1例可见右冠状动脉-右心室交通，1例右心室窦状间隙开放，心肌可见远离心腔的血流信号穿行	-6.23、-6.69	正常	无
Ⅳ型	3	右心室明显减小	符合①②③	-3.85~-4.38	正常	无

PA/IVS分型	例数	右心室大小	Roman评价体系（包括①②③④）^a	三尖瓣Z值	心胸面积比	水肿
Ⅰ型	2	右心室增大	无	2.53、4.20	0.40、0.48	心包积液
Ⅰ型	10	右心室偏小	TV/MV：0.72~0.93，RV/LV：0.66~0.85，其中1例符合③	-0.09~-2.92	正常	无
Ⅱ型	6	右心室明显减小，无RVDCC	符合①②	-3.15~-4.02	正常	无
Ⅱ型	3	右心室明显减小，无RVDCC	符合①②③	-3.56~-4.28	正常	无
Ⅲ型	1	右心室明显减小	符合①②③④，可见左冠状动脉-右心室交通	-5.21	正常	无
Ⅲ型	2	右心室明显减小	符合①②③	-3.64、-4.47	正常	无
Ⅳ型	2	右心室明显减小	符合①②③④，1例可见右冠状动脉-右心室交通，1例右心室窦状间隙开放，心肌可见远离心腔的血流信号穿行	-6.23、-6.69	正常	无
Ⅳ型	3	右心室明显减小	符合①②③	-3.85~-4.38	正常	无

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