Emergency surgical treatment of total anomalous pulmonary venous connection

Background: This study explores the strategy and effect of emergency surgical treatment for total anomalous pulmonary venous connection (TAPVC). Methods: From March 2009 to February 2020, 78 patients with TAPVC underwent emergency surgical correction. There were 51 males and 27 females. The median age was 39.5 days, and the median weight was 4.0 kg. The preoperative percutaneous oxygen saturation was 80.8±4.5%. Results: Of the cases investigated, seven died during the perioperative period, 16 had delayed chest closure, 19 had early pulmonary vein obstruction, two had secondary tracheal intubation, one had a brain complication, and one had third-degree atrioventricular block. Low weight, younger age, cardiopulmonary bypass time, and aortic cross-clamp time were identified as risk factors for early mortality. During the follow-up from four to 137 months, 12 cases did not respond to follow up. Ten patients died within one to six months after discharge. One patient underwent reoperation due to pulmonary vein obstruction. The longer hospital stays after operation and intensive care unit time were identified as risk factors for late mortality. Conclusions: Emergency surgery for severe TAPVC patients after admission had achieved good results in the near future. Prenatal diagnosis should be strengthened to save more patients. The higher late mortality rate indicates that such patients should strengthen post-discharge management to reduce the occurrence of post-discharge deaths.

Partial anomalous pulmonary venous connection and pulmonary venous varix coexist in Turner syndrome: a case report

Turner syndrome is a clinical syndrome caused by autosomal abnormalities in women. It is often accompanied by congenital cardiovascular malformations commonly including a bicuspid aortic valve malformation and aortic coarctation, but the presence of multiple pulmonary venous abnormalities is extremely rare. We present a 27-year-old woman who was diagnosed with Turner syndrome. She was revealed an anomalous right upper pulmonary venous connection, left upper pulmonary vein obstruction, and varicose vein malformations of the left pulmonary veins by a series of examination. Cardiac catheterisation and selective pulmonary angiography can further confirm the diagnosis, morphological characteristics, haemodynamic significance and provide a reference for the next step of treatment.

Impact of intrapulmonary-artery septation to pulmonary vein obstruction for two-lung Fontan

OBJECTIVES Patients with unbalanced pulmonary artery (PA) growth and decreased unilateral pulmonary circulation are considered unsuitable candidates for the Fontan procedure. Following our previous study on the utility of intrapulmonary-artery septation for patients with PA hypoplasia, we investigated its use in patients with pulmonary venous obstruction (PVO). METHODS We recruited 42 patients who underwent intrapulmonary-artery septation for unilateral PA hypoplasia and/or PVO between 1998 and 2018 and classified them into no PVO or PVO group. We analysed overall survival, success of the Fontan procedure and data from catheterization and echocardiography. In PVO, we evaluated the functional lung area before the Fontan procedure and the relevance of this parameter to operative outcomes. RESULTS The PVO and no-PVO group included 24 and 18 patients, respectively. One patient in the no-PVO and 6 patients in the PVO group died during follow-up; this difference was statistically significant (log-rank P = 0.040). In the no-PVO group, 15 (83%) patients achieved two-lung Fontan circulation. In the PVO group, 12 (50%) patients achieved two-lung Fontan circulation. Multivariate analysis revealed that functional lung area and shunt size [significantly larger in patients with functional lung area ≥50% in affected lung (P = 0.040)] were significant factors for successful two-lung Fontan procedure (P = 0.030). CONCLUSIONS Intrapulmonary-artery septation may contribute to increase functional lung area after PVO release to establish two-lung Fontan circulation in patients with PVO who have unbalanced PA growth and/or decreased unilateral pulmonary circulation.