Clinical Implications of Airway Anomalies and Stenosis in Patients with Heterotaxy Syndrome

In heterotaxy syndrome, bronchopulmonary situs usually reflects atrial situs, resulting in either right (RAI) or left atrial isomerism (LAI). This study determines airway anomalies and its implications in patients with heterotaxy. This retrospective study included 223 patients with heterotaxy syndrome who received an integrated cardiac computed tomography evaluation. Patient database from 1995 to 2020 was reviewed. The patients were examined by a congenital heart disease team comprising pediatric cardiologists, radiologists, pulmonologists, and cardiovascular surgeons. Among the 223 patients, 189 (84.8%, M/F=1.66) had RAI and 29 had LAI (13.0%, M/F=0.71). Five patients had indeterminate isomerism (2.2%, M/F=1.5). Discordant bronchopulmonary and atrial situs occurred in 4% patients, while discordant bronchopulmonary, atrial, and splenic situs occurred in 23.2% patients. Lower airway stenosis was observed in 61 patients (27.4%), including 27.5%, 20.7%, and 60% RAI, LAI, and indeterminate isomerism patients, respectively (p=0.189). One patient had an intrinsic long segment lower tracheal stenosis and received slide tracheoplasty. Initial cardiac operation was performed in 213 patients. Higher surgical mortality occurred in patients with RAI (19.5% vs. none for LAI and indeterminate isomerism, p=0.038). In patients with RAI, lower airway anomaly/stenosis increased the duration of ventilator usage (p=0.030) but did not affect surgical mortality. Total anomalous pulmonary venous return to systemic veins and pulmonary venous stenosis were major surgical risk factors. Bronchopulmonary isomerism shares a similar isomeric pattern to cardiac atrial appendage. Lower airway anomalies/stenosis was common in patients with heterotaxy, resulting in prolonged ventilator therapy in patients with RAI.

TOTALLY ANOMALOUS PULMONARY VENOUS CONNECTION OUTCOMES FROM A SINGLE CENTER

Objectives: There is limited data on totally anomalous pulmonary venous connection (TAPVC) repair results in our country. The aim of this study is to evaluate the early postoperative results of a large series of TAPVC patients operated in our clinic. Patients and Methods: The data of the patients who underwent TAPVC repair in our clinic between May 2005 and May 2021 were retrospectively reviewed using hospital records Results: A total of 150 TAPVC patients aged between 0 days and 39 years (median 3 months, IQR: 18 days – 9 months), 92 males (61%) and 58 females (39%) underwent surgical intervention. The median length of hospital stay was 12 (IQR:7-16) days. Overall mortality was 14% (21 patients). Seventeen out of 32 patients with additional cardiac anomalies (53%) and 4 out of 118 patients with isolated TAPVC (3%) died. The presence of additional cardiac anomalies was associated with mortality (p<0.00001). Mortality rate in univentricular patients was 59% (10 patients). This rate was higher in comparison to the mortality rate of biventricular patients (8%; p<0,00001). Three patients were reoperated due to postoperative pulmonary venous stenosis. Conclusion: The TAPVC outcomes in isolated and biventricular patients were favorable with a low mortality and postoperative pulmonary venous stenosis in this cohort. Patients with a single ventricle physiology and/or heterotaxia had a significantly increased risk of mortality, which might be due to the intrinsic challenges of the univentricular physiology regarding the balance between the pulmonary and systemic circulations. Tendency for increased mortality in the cases with preoperative pulmonary venous obstruction is a potential target for improvement.

Short-term results of sutureless closure for primary total anomalous pulmonary venous connection: seventeen cases

Introduction: Total anomalous pulmonary venous connection (TAPVC) is a rare condition. Mortality of TAPVC has improved dramatically in the present. Sutureless closure had initially used for congenital pulmonary venous stenosis (PVS) or post-repair PVS. Recently, it had been adopted for primary TAPVC to prevent postoperative pulmonary venous obstruction, a leading cause of death. This technique had gradually applied at our institute for primary TAPVC since 2018. Methods: Prospective case series was conducted from mid-2019. Including criteria consists of patients with primary TAPVC. These are all supracardiac, infracardiac, mixed type and intracardiac TAPVC with pulmonary venous stenosis. Excluding measures are TAPVC with single ventricle, isomerism or heterotaxy. End-point outcomes were mortality and short-term PVS. Results: Seventeen TAPVC cases were enrolled in this study from 06/2019-12/2020 at Children's Hospital 1. Male/female ratio was 12/5. Median age at admission was 44 (3-1010) days old. The median operative weight was 3.9 kg (2.4-11). Of those, fourteen (82.4%) cases were supracardiac TAPVC and 3 (17.6%) cases were infracardiac TAPVC. Nine (52.9%) cases were emergency operations. Median aortic clamp time was 66 (32-138) mins. Median bypass time was 112 (86-212) mins. There were 9 (52.9%) cases with arrhythmias, 2 (11.8%) cases with chylothorax. Mean ventilation time was 3.6±0.5 days; mean CICU time was 6.5 (1-20), median LOS was 14.5 (8-39). In-hospital mortality was 5.9% (1). Mean follow-up time was 12.3±6.4 months (range 4-24), there was no PVS with median pulmonary venous score of 0 (range 0-1). Conclusions: Sutureless technique could be a safe option for primary TAPVC repair.

Fetal Case of Rare Association of Hypoplastic Left Heart Syndrome and Absent Atrial Septum Accompanied by Mixed Form of Supracardiac Total Anomalous Pulmonary Venous Connection

Abnormal pulmonary venous flow patterns on fetal echocardiography and a nutmeg lung pattern on fetal magnetic resonance imaging are seen in patients with pulmonary venous stenosis. The association between these findings and the degree of pulmonary venous stenosis remains unknown. We report an extremely rare case of a fetus diagnosed with hypoplastic left heart syndrome complicated by an absent atrial septum and supracardiac total anomalous pulmonary venous connection with left pulmonary venous congestion. This case suggests that compared to non-pulsatile continuous pulmonary venous flow, the nutmeg lung pattern can only be observed with severe pulmonary congestion and advanced pulmonary lymphangiectasia.

Superior Vena Cava Flap to Reroute Partial Anomalous Pulmonary Venous Connection

A 46-year-old man who had undergone atrial septal defect closure during childhood was referred for surgery for residual partial anomalous pulmonary venous connection. The anomalous pulmonary veins were connected to the higher (cephalad) segment of the superior vena cava. As the usual caval division technique was not applicable, we chose to utilize the anterior wall of the superior vena cava as a flap for anomalous pulmonary vein rerouting. Bovine pericardium was used to reconstruct the systemic venous pathway. Systemic or pulmonary venous stenosis was not detected. The patient was discharged with a sinus rhythm.