Management of prenatally detected vascular rings: a United Kingdom national survey.

OBJECTIVE: To investigate UK variability in prenatal and postnatal management strategy of right aortic arch and double aortic arch (RAA/DAA). METHODS: Online surveys were sent to senior physicians (consultants) of the National Fetal Cardiology Working Group regarding prenatal diagnosis, counselling, and perinatal management of antenatally diagnosed RAA/DAA and to the British Congenital Cardiovascular Association regarding postnatal management strategies. RESULTS: There were 28 prenatal and 90 postnatal surveys completed. Prenatally, there was consensus for potential associated chromosomal/genetic anomalies, but there was variation in the risk quoted. Confidence in defining aortic arch morphology was reported by 43% (12/28) of fetal cardiologists. There was variation in what was felt to be possible symptoms/signs of a compressive vascular ring, postnatal investigation, postnatal management, follow-up duration of asymptomatic patients, and indications for surgical intervention. CONCLUSION: This study has highlighted important areas for future research: improving accuracy of prenatal diagnosis, clarification of potential symptoms, optimal investigation strategies, and indications for surgery.

Fetal aortic valve stenosis: a critique of case selection criteria for fetal intervention.

OBJECTIVE: Selection of fetuses with aortic stenosis (AS) for prenatal intervention has been influenced by published scoring systems. This study aimed to test these scoring systems by retrospective application to consecutive cases of fetal AS. METHODS: Retrospective analysis of the echocardiographic findings of 31 consecutive fetuses with AS evaluated at a tertiary fetal cardiology centre. Published 'eHLHS' scores and threshold scores were applied to the group and compared to postnatal management, in terms of biventricular repair versus single ventricle palliation. RESULTS: Thirty-one fetuses were identified with AS, and eHLHS was identified in 17 at the initial echocardiogram. No fetus with a full eHLHS score (3/3 or 4/4) achieved a biventricular repair. Three fetuses had a favourable threshold score (≥4), one of whom had a successful biventricular outcome. Seven fetuses had an unfavourable threshold score (<4) and underwent a univentricular pathway. CONCLUSION: The eHLHS score is a reliable predictor for the progression to HLHS at term. The score identifies those who would achieve a biventricular repair postnatally without prenatal intervention. A minority of fetuses with favourable threshold scores may achieve a biventricular repair postnatally without prenatal intervention, but eHLHS and an unfavourable threshold score (<4) predict a single ventricle pathway postnatally.

EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Prenatal diagnosis of congenital heart disease.

This review article will guide the reader through the background of prenatal screening for congenital heart disease. The reader will be given insight into the normal screening views, common abnormalities, risk stratification of lesions and also recent advances in prenatal cardiology.

Atrioventricular block during fetal life.

Congenital complete atrioventricular (AV) block occurs in approximately 1 in 20,000 live births and is known to result in significant mortality and morbidity both during fetal life and postnatally. Complete AV block can occur as a result of an immune or a non-immune mediated process. Immune mediated AV block is a multifactorial disease, but is associated with the trans-placental passage of maternal autoantibodies (anti-Ro/SSA and/or anti-La/SSB). These autoantibodies attach to and subsequently damage the cardiomyocytes and conduction tissue in susceptible fetuses. In this report, we examine the evidence in reference to means of assessment, pathophysiology, and potential prenatal therapy of atrioventricular block.

Prenatal screening for structural congenital heart disease.

Congenital heart defects can be diagnosed during fetal life using echocardiography. Prenatal diagnosis allows full investigation of affected fetuses for coexisting abnormalities, and gives time for parents to be informed about the prognosis of the fetus and treatments that might be required. In a minority of cases, where the natural history suggests an unfavourable outcome, prenatal diagnosis provides an opportunity for fetal cardiac intervention. For some cardiac lesions, notably hypoplastic left heart syndrome, transposition of the great arteries, and coarctation of the aorta, prenatal diagnosis has been shown to reduce postnatal morbidity and mortality. Some costs of care, notably the transport of critically ill infants, are reduced by prenatal diagnosis. Prenatal screening programmes typically recommend detailed assessment of fetuses judged to be at high risk of congenital heart disease. However, most cases of congenital heart disease arise in the low-risk population, and detection of affected fetuses in this setting depends on recognizing abnormalities of the heart during the midtrimester scan. Evidence supports the use of structured training interventions and feedback to those undertaking sonographic examinations, to improve the prenatal detection of congenital heart disease.