Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy

Improved current era outcomes in patients with heterotaxy syndromes☆

“…Historically visceral heterotaxy has been associated with significant morbidity and mortality [2,[4][5][6][7]. Right atrial isomerism has been described by some as one of the worst forms of contemporary heart disease [8] with overall 5-year survival ranging from 30% to 74% [9,10]. The results are better in left atrial isomerism with 5-year survival rates ranging between 65% and 84%, which is still considerably lower than survival for most other forms of congenital heart disease [2,10].…”

Clinical Features, Management, and Outcome of Children With Fetal and Postnatal Diagnoses of Isomerism Syndromes

“…Although antenatal diagnosis often represents a more severe spectrum of cardiovascular disease, 14 fetal diagnosis may permit more options, including termination of pregnancy or more tailored neonatal treatment. [15][16][17][18][19][20] We sought to assess the impact of prenatal diagnosis on management and outcome in a large cohort of cases with a diagnosis of either LAI or RAI.…”

Prenatal Diagnosis of Total Anomalous Pulmonary Venous Connection with Asplenia

“…The omi nous complications following pulmonary venous obstruc tion in the heterotaxic heart increases the mortality and morbidity of the patients in the neonatal period, even applying surgical palliative procedures [6,8], Sudden death may occur in the neonatal period because of pulmo nary venous obstruction [8], In both our cases, the pro gression of pulmonary venous obstruction could be moni tored by observing the flow velocity and profile measured by pulse Doppler throughout the pregnancy and perinatal period. Based on this measurement, optimal therapeutic interventions and management could be provided result ing in avoiding unnecessary palliation and in improving the surgical outcome of this complicated cardiac ano maly.…”

“…In contrast, the heterotaxic heart, especially asplenia (right isomerism), is one of the complex cardiac anoma lies often associated with TAPVC [6], usually presenting with significant unbalanced intracardiac chambers which are easily detected by fetal échocardiographie scan even in early pregnancy. Both our cases were initially found to have cardiac anomalies because of failure to visualize the well-balanced four chamber view on the fetal scan.…”

“…Since heterotaxic heart are often associated with an anomalous pulmonary venous connection [6], our cases underwent repeated and serial fetal scans in order to study the anomalous pulmonary venous connection using color Doppler flow mapping, and were finally found to have an anomalous pulmonary venous connection in the third tri mester. The first suspicious finding of TAPVC in our cases was the abnormal localization of continuous flow on color Doppler echocardiography behind the heart.…”