Prenatal diagnosis of right ventricular outflow tract obstruction with intact ventricular septum, and detection of ventriculocoronary connections

Abstract

Objectives-To determine the accuracy of prenatal diagnosis of pulmonary atresia and intact ventricular septum (PAIVS), and pulmonary stenosis, including prenatal detection of ventriculocoronary connections, to evaluate heart size during the prenatal period, and to evaluate the outcome. Design and patients-Medical records of 20 cases with prenatally diagnosed PAIVS and pulmonary stenosis were reviewed retrospectively. Prenatal and postnatal echocardiography were also reviewed and dimensions of the ventricles and vessels were measured retrespectively. Results-Of 20 prenatal diagnoses (15 PAIVS and five pulmonary stenosis), 16 were confirmed as correct. One critical pulmonary stenosis case had been diagnosed as PAIVS prenatally; three had no confirmation. Eight pregnancies were terminated, three had no active treatment, and nine were treated; all survived. Of 13 assessed with ventriculocoronary connections prenatally, seven were diagnosed correctly (four with, three without ventriculocoronary connections), but one was falsely positive; five had no confirmation. The more prominent hypoplasia of the main pulmonary artery and the tricuspid valve annulus, and the sigmoid shape of the ductus arteriosus, seemed to be associated with the presence of ventriculocoronary connections. Conclusions-Current prenatal echocardiography can accurately diagnose right ventricular outflow tract obstruction and ventriculocoronary connections. Prenatal detection of this constellation of abnormalities aids in family counselling and decisions on postnatal management. (Heart 1999;81:661-668)

Prediction of Therapeutic Strategy and Outcome for Antenatally Diagnosed Pulmonary Atresia/Stenosis With Intact Ventricular Septum

“…Maeno et al reported that current high resolution ultrasound provides accurate information for prenatal diagnosis of right ventricular outflow obstruction and ventriculocoronary connection. 6 In the present study, all 18 patients had correct fetal diagnoses confirmed by postnatal echocardiography and angiographic examination. With regard to ventriculocoronary connection, we diagnosed 3 cases of right-ventricle-dependent coronary circulation.…”

Atresia of Proximal Coronary Arteries in Pulmonary Atresia with Intact Ventricular Septum – Fetal and Neonatal Findings

“…In several reports, ventriculocoronary connections with PA+IVS have been demonstrated in fetal scans resulting in termination of pregnancy in half of them [12][13][14][15][16][17][18] . During the fetal period, these connections have been associated with prominent hypoplasia of the main pulmonary artery, a small tricuspid valve annulus as well as a sigmoid-shaped ductus arteriosus [16] . The extent of stenosis, interruptions or ostial atresia of the coronary arteries occurring with ventriculocoronary connections are related both to the size of the RV and outcome.…”

Fetal Echocardiography

“…• Development/progression of ventricular inflow, outflow and arch obstruction [53][54][55][56][98][99][100][101][102][103][104] • Development/progression of structural hypoplasia [53][54][55][56][105][106][107][108][109][110] • Development/progression of valvular regurgitation [98] • Development/progression of dysrhythmia [51,[75][76][77]98,111,112] • Development of foramen ovale restriction [66,113] • Diminution and closure of ventricular septal defect [114] • Development of ductus arteriosus aneurysm or spontaneous constriction [67,115] • Development of primary myocardial disease [31,[116][117][118][119] • Development of congestive heart failure [120][121][122][123][124] • Development of, progression or regression of cardiac tumors [125]…”