Neonatal repair of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome: a case report

Background Total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare. Case presentation We present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7–0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction. Conclusion We report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

Abstract 17056: Report Of An Adult Living With Undiagnosed Total Anomalous Pulmonary Venous Return

Case Presentation: 25-year-old male presented with cellulitis and abscess of the lower extremity. He was an immigrant from Guatemala. He worked in landscaping and played soccer with limited exertion. His exam was notable for cyanosis and clubbing of all digits. A II/VI systolic ejection murmur at the left upper sternal border and a mildly accentuated P2 was auscultated. ECG was notable for right axis deviation and right ventricle hypertrophy. Chest x-ray showed a widened mediastinum with perihilar vascular prominence. CT chest with contrast demonstrated all pulmonary veins forming a large, coalescing venous structure draining into the left innominate vein. Transthoracic echocardiogram confirmed total pulmonary venous return by way of a large vertical vein draining into a dilated innominate vein and a 2.7 cm ostium secundum ASD. Discussion: Total anomalous pulmonary venous return (TAPVR) is a rare, often deadly condition. Most patients die in infancy. In the rare adult patient with unrepaired TAPVR, exercise intolerance, cyanosis, clubbing of the digits, and growth retardation is often noted. A right ventricular heave with auscultation of a fixed, split second heart sound with a loud pulmonic component is common. On chest x-ray, the classic “snowman” appearance is seen with vertical vein connections as in our patient. Right heart enlargement represents the base, and an enlarged connecting vertical vein and SVC make up the upper portion. Echocardiography can demonstrate the anomalous pulmonary venous conduit coursing behind the left atrium. CT angiography can also reveal TAPVR and the patient’s specific anatomy. Amongst the few surviving adult TAPVR patients, similar characteristics include large ASDs, short anomalous veins, and absence of pulmonary venous obstruction. A large interatrial connection provides adequate systemic flow. The latter two factors reduce pulmonary vascular resistance, hence improving pulmonary flow and systemic oxygen concentrations.

The Outcomes of Total Anomalous Pulmonary Venous Connection in Neonates−10-Year Experience at a Single Center

Background: Recent developments in surgical techniques and hospital care have led to improved outcomes following repair of total anomalous pulmonary venous connection (TAPVC). However, surgical repair of neonatal TAPVC remains associated with a high risk of postoperative mortality and pulmonary venous obstruction (PVO). We conducted this retrospective study to identify risk factors associated with surgical outcomes in the neonatal population.Methods: A retrospective review was conducted for all 127 neonates who underwent operations for isolated TAPVC from January 2009 to January 2019.Results: Preoperative PVO occurred in 33 (26.0%) of the 127 patients. Fifty patients (39.4%) required tracheal intubation before the operation. Twenty-three patients (18.1%) underwent emergency surgery. There were 11 (8.7%) early deaths. Significant risk factors were prolonged cardiopulmonary bypass (CPB) time (p = 0.013) and increased postoperative central venous pressure (CVP, p = 0.036). There were 5 (4.3%) late deaths within 1 year of repair. The risk factors for overall death were preoperative acidosis (p = 0.001), prolonged CPB time (p < 0.001) and increased postoperative CVP (p = 0.007). In particular, mortality was significantly higher (p = 0.007) with a postoperative CVP > 8 mmHg. With an increase in use of sutureless techniques (p = 0.001) and decrease in deep hypothermic circulatory arrest (p = 0.009) over the past 5 years, postoperative mortality greatly decreased (21.2%: 6.7%, p = 0.016). Postoperative PVO occurred in 15 patients (11.8%). Risk factors were mixed TAPVC (p = 0.037), preoperative acidosis (p = 0.001) and prolonged CPB time (p = 0.006).Conclusion: Although postoperative mortality of neonatal TAPVC has dropped to 6.7% over the past 5 years, it is still relatively high. Risk factors for postoperative death include preoperative acidosis, prolonged CPB time and increased postoperative CVP. Mortality was significantly higher for neonates with an average CVP > 8 mmHg 24 h after surgery.

Multimodality imaging in delineation of complex sinus venous defects and treatment outcomes over the last decade

Background: Diagnosis of sinus venosus defects, not infrequently associated with complex anomalous pulmonary venous drainage, may be delayed requiring multimodality imaging. Methods: Retrospective review of all patients from February 2008 to January 2019. Results: Thirty-seven children were diagnosed at a median age of 4.2 years (range 0.5−15.5 years). In 32 of 37 (86%) patients, diagnosis was achieved on transthoracic echocardiography, but five patients (14%) had complex variants (four had high insertion of anomalous vein into the superior caval vein and three had multiple anomalous veins draining to different sites, two of whom had drainage of one vein into the high superior caval vein). In these five patients, the final diagnosis was achieved by multimodality imaging and intra-operative findings. The median age at surgery was 5.2 years (range 1.6−15.8 years). Thirty-one patients underwent double patch repair, four patients a Warden repair, and two patients a single-patch repair. Of the four Warden repairs, two patients had a high insertion of right-sided anomalous pulmonary vein into the superior caval vein, one patient had bilateral superior caval veins, and one patient had right lower pulmonary vein insertion into the right atrium/superior caval vein junction. There was no post-operative mortality, reoperation, residual shunt or pulmonary venous obstruction. One patient developed superior caval vein obstruction and one patient developed atrial flutter. Conclusion: Complementary cardiac imaging modalities improve diagnosis of complex sinus venosus defects associated with a wide variation in the pattern of anomalous pulmonary venous connection. Nonetheless, surgical treatment is associated with excellent outcomes.

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.

Neonatal Repair of Total Anomalous Pulmonary Venous Connection with Goldenhar Syndrome and Unilateral Lung Agenesis：A Case Report

Background: Total anomalous pulmonary venous connection with unilateral lung agenesis and Goldenhar syndrome is extremely rare and high mortality.Case presentation: We present a case of total anomalous pulmonary venous connection with unilateral lung agenesis and Goldenhar syndrome. It was diagnosed from Transthoracic echocardiography and enhanced Computed Tomography. Total absence of the lung, the bronchial tree, and vascular structures were detected on the right side, and the left pulmonary veins returned abnormally to the innominate vein. There was apparent indication of pulmonary venous obstruction, the operation was performed at 3 days after birth. The common pulmonary venous chamber with vertical vein and the left atrium was anastomosed using 7-0 PDS running sutures through a median sternotomy. Postoperative echocardiography and Computed Tomography 1 year after the surgery, between the common pulmonary venous chamber and the left atrium was no stenosis.Conclusion: A extremely rare case of total anomalous pulmonary venous connection with unilateral lung agenesis and Goldenhar syndrome successfully repaired at 3 day after birth was reported. Anastomosis between the common pulmonary venous chamber and the left atrium using the vertical vein is a reasonable choice in patient with small common pulmonary venous chamber.

Apex rotation as a risk factor for total anomalous pulmonary connection repair in single ventricle

Background: The high incidence of postoperative pulmonary venous obstruction (PVO) is a major mortality-associated concern in patients with right atrial isomerism and extracardiac total anomalous pulmonary venous connection (TAPVC). We evaluated new anatomical risk factors for reducing the space behind the heart after TAPVC repair. Methods: 18 patients who underwent TAPVC repair between 2014 and 2020 were enrolled. Sutureless technique was used in 12 patients and conventional repair in six patients. The angle between the line perpendicular to the vertebral body and that from the vertebral body to the apex was defined as the “vertebral-apex angle (V-A angle).” The ratio of post- and preoperative angles, indicating the apex’s lateral rotation, was compared between patients with and without PVO. Results: The median (interquartile range) age and body weight at repair were 102 (79-176) days and 3.8 (2.6-4.8) kg, respectively. The 1-year survival rate was 83% (median follow-up, 29 [11-36] months). PVO occurred in seven patients (39%), who showed an obstruction of one or two branches in the apex side. The postoperative V-A angle (46° [45°-50°] vs. 36° [29°-38°], P = 0.001) and the ratio of post- and preoperative V-A angles (1.27 [1.24-1.42] vs. 1.03 [0.98-1.07], P = 0.001) were significantly higher in the PVO group than in the non-PVO group. The cut-off values of the postoperative V-A angle and ratio were 41° and 1.17, respectively. Conclusions: A postoperative rotation of the heart apex into the ipsilateral thorax was a risk factor for branch PVO after TAPVC repair.