樽谷 朋晃

A newborn with right atrial isomerism, single-ventricle physiology, supracardiac total anomalous pulmonary venous connection, and pulmonary atresia underwent vertical vein stenting, resulting in ductus arteriosus compression. Subsequent ductal stenting restored patency, preventing haemodynamic compromise and ensuring survival. This case highlights the risk of secondary ductal obstruction in vertical vein stenting and the importance of early recognition and intervention.

Severe Ebstein's disease often necessitates early neonatal surgical intervention, although, in some patients, surgery may be delayed until infancy or later, and medical management may be undertaken instead. Various indicators, such as tricuspid regurgitation flow velocity, have been studied to predict single or biventricular circulation; however, indicators predicting the need for neonatal surgery have not been addressed. We aimed to identify predictive indices for neonatal surgical necessity by analysing the clinical characteristics of those requiring and not requiring neonatal surgery. We enrolled eight patients with severe Ebstein's disease who were born at our hospital from 2018 to 2023. Four patients underwent neonatal surgery (Group S), whereas four did not (Group F). We compared pregnancy, delivery, and postnatal clinical courses and laboratory indices before and after birth between the groups. The systolic/diastolic time ratio, an index of ventricular function calculated from the TR waveform of echocardiography at birth, was significantly prolonged in Group S [2.18 ± 0.31 vs. 1.72 ± 0.20 in Group F (p = 0.043)]. The area under the receiver operating characteristic curve was 0.94, with a threshold systolic/diastolic time ratio value of 1.99 using Youden's method. This ratio predicted neonatal surgery necessity with 100% sensitivity and 75% specificity. Despite the small sample size, we demonstrated that the systolic/diastolic time ratio is a valuable predictive index of ventricular function and prognosis in severe Ebstein's disease patients. A systolic/diastolic time ratio <2, determined from the tricuspid regurgitation waveform on echocardiography at birth, supports patient monitoring without surgical intervention through the newborn period and beyond.