Fetal pulmonary atresia with ventricular septal defect: Features, associations, and outcome in fetuses with different pulmonary circulation supply types

Abstract

Objectives To assess features and outcome in fetuses with pulmonary atresia with ventricular septal defect (PA‐VSD). Methods Fetuses with PA‐VSD were prospectively enrolled and grouped on the basis of the pulmonary blood supply, including type A (only arterial duct [DA]), type B (both DA and major aortopulmonary collateral arteries [MAPCAs] present), and type C (MAPCAs only). The echocardiography features, associated chromosomal/genetic malformations, and postnatal outcome were compared among the three groups. Results Fifty‐five fetuses with PA‐VSD were enrolled. The presence of confluent PAs varied, with the highest displaying rate in type A and lowest rate in type C (100% vs 41.1%). The intrapericardial pulmonary arteries in all groups were hypoplastic but smaller in types B and C than in type A (P < .05). Deletion of 22q11.2 and right aortic arch were more frequently observed in types B and C than in type A. At the end of the study, overall survival rates in type C were lower than those in type A (22.1% vs 77.3%). Conclusion There are great differences in the size of pulmonary arteries, associated genetic malformations, and perinatal outcomes among fetuses with PA‐VSD. These results could be used for family counseling and surgical planning.

Pulmonary atresia and ventricular septal defect: How accurate is the fetal echocardiography, and do the major aortopulmonary collateral arteries matter?

“…and Naimi I et al. reported similar rates with us in recent studies 7,8 . The improvement seems to be related to the advanced ultrasound technology.…”

“…The impact of MAPCAs on perinatal and postnatal outcomes is debatable in the literature 3,7 . MAPCA‐dependent newborns can present cyanosis in case of narrow collaterals, or those with adequate collaterals may be acyanotic with subtle symptoms for months until stenosis of the collaterals occurs.…”

“…The presence or absence of confluent PAs transforms the method of surgery. Non‐confluent PAs have narrow RPA, LPA, and larger MAPCAs which may lead to pulmonary parenchymal disease secondary to incomplete intrapulmonary arborization 7 . Therefore, assessment of the confluence of PAs and the size and anatomy of PAs on fetal echocardiography is as necessary as identifying the origin of pulmonary blood supply for proper prenatal counseling.…”

Tetralogy of Fallot—A literature review

“…3 There is known to be an array of levels of pulmonary stenosis, ranging from partial to complete. 3,4 In partial pulmonary valve stenosis, only a slight narrowing of the right pulmonary artery may be observed on ultrasound, whereas in complete atresia, the pulmonary artery is shown as just a thin hypoechoic linear structure with no flow. 4,5 In terms of ultrasound appearances, the size of the pulmonary artery and its ratio to the ascending aorta are a sign of the severity of right outflow tract obstruction.…”

“…3,4 In partial pulmonary valve stenosis, only a slight narrowing of the right pulmonary artery may be observed on ultrasound, whereas in complete atresia, the pulmonary artery is shown as just a thin hypoechoic linear structure with no flow. 4,5 In terms of ultrasound appearances, the size of the pulmonary artery and its ratio to the ascending aorta are a sign of the severity of right outflow tract obstruction. 6 There are a number of variants of Tetralogy of Fallot, with variations in types of VSDs, coronary arterial patterns, and differences in aortic override.…”