Pulmonary Atresia With Intact Ventricular Septum

Abstract

Background —Fetal echocardiography is widely established in the United Kingdom for prenatal diagnosis of congenital heart disease. This may result in a substantial reduction in incidence at birth because of selected termination of pregnancy. The objective of this population-based study was to determine the incidence of pulmonary atresia with intact ventricular septum (PAIVS) at birth, the impact of fetal echocardiography on this incidence, and to compare the outcome of cases with and those without prenatal diagnosis. Methods and Results —From 1991 to 1995, all infants born with PAIVS and all fetal diagnoses in the United Kingdom and Eire were studied. There were 183 live births (incidence 4.5/100 000 live births). The incidence was 4.1 cases per 100 000 live births in England and Wales, 4.7 in Scotland, 6.8 in Eire, and 9.6 in Northern Ireland ( P =0.01). There were 86 fetal diagnoses made at a mean of 22.0 weeks of gestation leading to 53 terminations of pregnancy (61%), 4 intrauterine deaths (5%), and 29 live births (34%). The incidence at birth would be 5.6 per 100 000 births in England and Wales, 5.3 in Scotland, and unchanged in Eire and Northern Ireland, if there were no terminations of pregnancy and assuming no further spontaneous fetal deaths ( P =0.28). An initial diagnosis of critical pulmonary stenosis was made in 6 cases, at a mean of 22.3 weeks of gestation with progression to PAIVS by 31.4 weeks. Probability of survival at 1 year was 65% and was the same for live-born infants whether or not a fetal diagnosis had been made. Conclusions —PAIVS is rare, occurring in 1 in 22 000 live births in the United Kingdom and Eire. Termination of pregnancy has resulted in an important reduction in the live-born incidence in mainland Britain.

Prenatal Tricuspid Valve Size as a Predictor of Postnatal Outcome in Patients with Severe Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum

“…As prenatal diagnosis of PA/IVS has been associated with a high rate of terminations of pregnancy ranging between 61 and 82% due to concerns regarding RV growth [14,15], early and accurate prediction of postnatal outcome is of great importance as many parents decide to continue the pregnancy when BV repair is likely. Several studies have looked at prenatal determinants of postnatal outcome in fetuses with PA/IVS (table 2), many of which have had findings similar to ours.…”

Immediate effects and outcome of in‐utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis

“…During fetal life, progression of CPS and development of PAIVS have been reported2, 3. In the absence of high‐grade tricuspid regurgitation (TR) this lesion leads to a hypoplastic right ventricle (RV), which may preclude a biventricular circulation after birth1, 4, 5. Even if biventricular repair can be achieved postnatally, RV function may remain abnormal and the potential of postnatal RV growth is limited6, 7.…”

Fetal Intelligent Navigation Echocardiography (FINE) Detects 98% of Congenital Heart Disease

“…The lack of prenatal identification of CHD is associated with adverse consequences for the neonate, including abnormal neurological development, and even death. 19–22 In contrast, the prenatal diagnosis of specific cardiac anomalies improves the preoperative condition, 23–27 pre-surgical mortality rate, 28–30 survival after surgery, 19,23,31,32 and long-term neurocognitive function and outcome. 33,34 …”