Objective: Prenatal diagnosis of total (TAPVC) or partial (PAPVC) anomalous pulmonary venous connection in isolation or associated with other cardiac disease is important for appropriate prenatal counseling and perinatal management. We sought to assess the echocardiographic clues to the fetal diagnosis of TAPVC and PAPVC in a cohort of affected fetuses.Methods: We retrospectively reviewed 29 fetal echocardiograms performed in 16 pregnancies with fetal TAPVC or PAPVC, systematically analyzing heart chamber size, presence of a confluence behind the left atrium or of a vertical vein, and Doppler flow patterns.Results: Prenatal diagnosis was made at a mean gestational age of 27 ± 7 weeks. TAPVC was found in 11 cases; five cases for each of supracardiac and infracardiac types and one mixed type. PAPVC was diagnosed in five fetuses, four of which had scimitar syndrome. Ten fetuses had an additional major cardiac defect, including hypoplastic left heart syndrome and right atrial isomerism. In three cases the prenatal diagnosis was only made at follow‐up assessment. Among TAPVC cases, visualization of a confluence behind the left atrium (10/11) and a vertical vein (11/11) were the most consistent echocardiographic clues. Dextrocardia and a small right pulmonary artery suggested scimitar syndrome. The diagnosis was confirmed postnatally or at autopsy in 12 cases. In six fetuses with TAPVC and obstruction confirmed postnatally, continuous turbulent flow in the vertical vein and monophasic continuous flow in the pulmonary veins were demonstrated by color and spectral Doppler.Conclusions: Fetal echocardiography permits prenatal diagnosis of TAPVC or PAPVC. Spectral and color Doppler provide clues to the presence of an obstructed pulmonary venous pathway. Copyright © 2003 ISUOG. Published by John Wiley & Sons, Ltd.

Objective: Decreased middle cerebral artery (MCA) pulsatility index (PI) is a marker of fetal brain‐sparing in placental insufficiency and it is also found in fetuses with severe congenital heart disease. This study sought to explore the impact of anatomical subtypes in fetal heart disease on MCA‐PI and head growth.Methods: We retrospectively reviewed fetal echocardiograms of pregnancies complicated by fetal hypoplastic left heart syndrome (HLHS; n = 42) with and without anatomic coarctation (n = 28 and n = 10, respectively), isolated severe aortic coarctation (n = 21), D‐transposition of the great arteries (TGA; n = 11) and pulmonary outflow tract obstruction without forward flow across the pulmonary valve (POTO; n = 15), comparing observations with gestational age‐matched controls (n = 89). No fetus had major extracardiac pathology or aneuploidy. MCA and umbilical artery (UA) PI, the cerebral placental ratio (CPR = MCA‐PI/ UA‐PI) and neonatal head circumference were obtained and expressed as Z‐scores.Results: Lower MCA‐PI, higher UA‐PI and lower CPR were observed in fetal HLHS and isolated coarctation with reversed arch flow (n = 6) (P < 0.001) but not TGA, POTO or isolated coarctation with antegrade arch flow (n = 15) compared with controls. No difference was found between HLHS with anatomical coarctation and those without; however, MCA‐PI correlated positively with neonatal head circumference in HLHS with reversed distal arch flow (r = 0.33, P < 0.05).Conclusions: Severe left heart obstruction with reversed aortic arch flow is associated with altered fetal cerebral blood flow, and in these conditions, MCA‐PI positively correlates with head growth. Anatomical arch obstruction itself may not be a contributing factor to altered MCA flow in fetal HLHS. Copyright © 2013 ISUOG. Published by John Wiley & Sons Ltd.