Utility of a Single 3-Vessel View in the Evaluation of the Ventricular Outflow Tracts.

OBJECTIVES: Prenatal diagnosis of congenital heart disease can improve neonatal outcomes. The purpose of this study was to evaluate the utility of an isolated 3-vessel view image in evaluating the ventricular outflow tracts. METHODS: Three-vessel view images were prospectively collected from consecutive patients referred to a regional fetal heart center for fetal echocardiography between gestational ages of 18 weeks and 23 weeks 6 days. Cardiac malformations were categorized as anomalies of the outflow tracts, the 4-chamber view, or combined lesions. A single representative still-frame 3-vessel view image was reviewed by 2 independent and blinded observers who were asked to label each image as "normal" or "abnormal." Test characteristics of the isolated 3-vessel view were calculated. RESULTS: During the study period, 122 consecutive patients (139 fetuses) underwent fetal echocardiography. Eight fetuses with fetal chest anomalies and 12 fetuses with oblique images were excluded. Thirty-four of 119 fetuses (28.6%) had abnormal echocardiograms, including 11 outflow tract anomalies and 16 combined anomalies. Using the 3-vessel view alone, both reviewers achieved 91% sensitivity for the detection of isolated outflow tract anomalies and mean sensitivity of 88% for combined anomalies. All cases of tetralogy of Fallot and transposition of the great arteries were identified in the 3-vessel view. CONCLUSIONS: A single 3-vessel view image can serve as a representative view of the outflow tracts and can show ventricular outflow tract anomalies with high sensitivity. Given that the conventional outflow tract views can be difficult to obtain, the 3-vessel view may serve as an effective first-line view when evaluating the ventricular outflow tracts for congenital heart disease.

Anatomic variability and outcome in prenatally diagnosed absent pulmonary valve syndrome.

BACKGROUND: We sought to describe current outcomes and risk factors for mortality for fetuses diagnosed with absent pulmonary valve syndrome (APV). Fetuses with APV were divided into two cohorts, those with underlying tetralogy of Fallot (TOF/APV) and those without underlying TOF and either an intact ventricular septum or small ventricular septal defect (APV/IVS). METHODS: The fetal echocardiographic database was reviewed from January 1, 2001, until June 1, 2010, and all subjects with a diagnosis of APV were included. Multiple clinical and fetal echocardiographic measurements were recorded. Statistical analysis was performed by χ2 analysis and t tests. Survival analysis was performed by Kaplan-Meier analysis. Significant relationships between variables were explored by regression analysis. Significance was set at p=0.05. RESULTS: The cohort consisted of 15 fetuses with TOF/APV and 6 fetuses with APV/IVS. There were no fetal demises in either cohort. Survival to birth was 71% in the TOF/APV cohort and 83% in the APV/IVS cohort (p=0.62). Of subjects born alive, survival was 80% for both cohorts (p=0.95). However, in the APV/IVS cohort, transplantation-free survival was only 20%. Underlying single-ventricle physiology strongly predicted those who underwent heart transplantation (p=0.003, R2=0.50). For the entire APV cohort, left ventricular dysfunction (p=0.005, R2=0.41) and a higher pulmonary artery valve-to-aortic valve ratio (p=0.02, R2=0.34) predicted mortality. CONCLUSIONS: Postnatal outcomes continue to improve for fetuses with APV syndrome. Left ventricular dysfunction and higher pulmonary artery valve-to-aortic valve ratio accurately predict postnatal mortality for fetuses with APV.

Right ventricular mechanics in the fetus with hypoplastic left heart syndrome.

BACKGROUND: Right ventricular mechanics influence outcomes in patients with hypoplastic left heart syndrome (HLHS). The aim of this study was to determine whether differences in right ventricular performance have their origins in fetal life and if the architectural character of the hypoplastic left ventricle affects right ventricular mechanics. METHODS: The first complete fetal echocardiograms after 17 weeks' gestation were reviewed in 84 fetuses with HLHS and in 115 gestational age-matched normal controls. Inflow, outflow, and myocardial tissue Doppler velocities were measured. E/A and E/e' ratios and right ventricular myocardial performance index were calculated. RESULTS: In fetuses with HLHS, there were lower tricuspid E/A ratios (mean, 0.6 ± 0.1 vs 0.7 ± 0.1; P < .001), higher E/e' ratios (mean, 8.1 ± 2.6 vs 7.0 ± 1.3; P = .006), and higher right ventricular myocardial performance indices (mean, 0.47 ± 0.14 vs 0.40 ± 0.10; P < .001) compared with controls. Among fetuses with HLHS grouped according to left ventricular architecture, those with left ventricular endocardial fibroelastosis had the most striking differences in right ventricular mechanics. CONCLUSIONS: Right ventricular mechanics are different from normal in fetuses with HLHS and are influenced by the presence of left ventricular endocardial fibroelastosis. These differences precede the imposition of undue loading conditions as a consequence of surgical palliation and may offer clues to the development of later right ventricular failure.