Prenatal echocardiographic classification and prognostic evaluation strategy in fetal pulmonary atresia with intact ventricular septum

Autor: Hongdan Wang, Taibing Fan, Ying Wang, Bangtian Peng, Yanan Li, Cun-Ying Cui, Yuanyuan Liu, Lin Liu
Rok vydání: 2019
Předmět:
Adult
Heart Defects
Congenital

Aortic arch
medicine.medical_specialty
Observational Study
Gestational Age
fetal echocardiography
postnatal evaluation
Sensitivity and Specificity
Ultrasonography
Prenatal

Young Adult
03 medical and health sciences
Fetal Heart
0302 clinical medicine
Predictive Value of Tests
Pregnancy
Reference Values
Internal medicine
medicine.artery
Mitral valve
medicine
Humans
030212 general & internal medicine
prenatal diagnosis
Tricuspid valve
medicine.diagnostic_test
business.industry
Gestational age
Umbilical artery
General Medicine
Prognosis
medicine.disease
congenital heart diseases
medicine.anatomical_structure
Echocardiography
Pulmonary Atresia
Ventricle
030220 oncology & carcinogenesis
Cardiology
Female
pulmonary atresia with intact ventricular septum
business
Pulmonary atresia
Fetal echocardiography
Research Article
Zdroj: Medicine
ISSN: 1536-5964
0025-7974
DOI: 10.1097/md.0000000000017492
Popis: Fetal pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart disease. The present study aimed to classify PA/IVS and determine the relationship between prenatal echocardiographic characteristics and postnatal biventricular or univentricular repair strategies. A total of 51 fetuses with PA/IVS were examined from 2012 to 2019. Data on prenatal echocardiography, associated anomaly, karyotype, and outcome were collected. Two-dimensional measurements included tricuspid valve (TV) z-score, mitral valve (MV) z-score, TV/MV ratio, and ratio of right to left ventricle (RV/LV) length, whereas color Doppler measurements included degree of tricuspid regurgitation (TR), ventriculo-coronary artery communication (VCAC), tricuspid inflow duration (TID), cardiac cycle duration (CCD), middle cerebral artery pulsatility index (MCA PI), and umbilical artery pulsatility index (UA PI). Diagnostic classification was based on the development of RV and the presence or absence of VCAC. Postnatal evaluation was divided according biventricular or univentricular repair. Of the 51 fetuses with PA/IVS, 20 were type I, 17 were type II, and 14 were type III. Only one fetus exhibited right aortic arch. The karyotype of all the fetuses was normal. Of the 28 patients who underwent postnatal surgery, 13 (46%) underwent biventricular repair and 15 (54%) underwent univentricular repair. TV z-score was significantly higher for the biventricular repair group compared with univentricular repair group (−1.20 ± 0.98 vs −4.33 ± 0.80, P = .000). TV/MV, RV/LV length, and TID/CCD were significantly higher for the biventricular repair group than the univentricular repair group (0.81 ± 0.14 vs 0.54 ± 0.09, 0.71 ± 0.11 vs 0.49 ± 0.09, 39.20 ± 3.84 vs 29.16 ± 4.58, P = .000). Moderate or severe TR and VCAC were significantly different between the 2 groups (P = .000). Gestational age, MCA PI, and UA PI did not differ between the 2 groups (P = .72, P = .36, P = .06). The cutoff values for the biventricular repair characteristic curves were TV z-score >−3.28, TV/MV ratio >0.71, RV/LV length >0.62, and TID/CCD >33.95%. The sensitivities of the TV z-score, TV/MV, RV/LV length, and TID/CCD were 100%, 77%, 85%, and 92%, respectively. The specificities of the TV z-score, TV/MV, RV/LV length, and TID/CCD were 94%, 100%, 100%, and 94%, respectively. Fetal echocardiography was able to classify PA/IVS according to variable degree of RV and VCAC. In fetal PA/IVS, TV z-score >−3.28, TV/MV >0.71, RV/LV length >0.62, TID/CCD >33.95%, moderate and severe TR, and the absence of VCAC were associated with postnatal biventricular repair strategy. These findings may have implications for prenatal counseling and prediction of fetal outcome.
Databáze: OpenAIRE