Neonatal Repair of Total Anomalous Pulmonary Venous Connection with Goldenhar Syndrome and Unilateral Lung Agenesis (cid:0) 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.

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.

Background
Goldenhar syndrome is a rare congenital disease characterized by craniofacial abnormalities included the incomplete development of the eye, ear, jaw [1]. The average incidence rate of this syndrome is estimated to be between 1:3000 and 1:5000 live births. Cardiovascular malformations have been reported in 5-58% of patients [1]. The prevalence of lung agenesis is reported to 34 per 1,000,000 live births [2]. Cases of repair for total anomalous pulmonary venous connection with lung agenesis have previously been reported, operative mortality was high. In addition, total anomalous pulmonary venous connection with unilateral lung agenesis and Goldenhar syndrome were reported only 2 cases. We report a rare case of total anomalous pulmonary venous connection with unilateral lung agenesis and Goldenhar syndrome successfully repaired at 3 day after birth. To the best of our knowledge, this is the rst report survived more than 1 year after surgery.

Case Presentation
A 25-year-old pregnant woman at 25 weeks of gestation was diagnosed with right lung agenesis and right hypoplastic eye, but heart malformation was not diagnosed.
A female neonate was delivered at 37 weeks with caesarean section. The birth weight of the neonate was 2101g, postnatally, she was noticed craniofacial abnormalities and suspected Goldenhar syndrome. She deteriorated requiring intratracheal ventilation. Chest radiograph, Transthoracic echocardiography and enhanced Computed Tomography con rmed the diagnosis. 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( Fig.1 A,B,C). In addition, she had a large arterial septal defect, a small ventricle septal defect and a patent ductus arteriosus. There was apparent indication of pulmonary venous obstruction, the operation was performed at 3 days after birth.
The heart was exposed through a median sternotomy. Cardiopulmonary bypass was instituted with ascending aortic perfusion, direct bicaval venous drainage. The patent ductus arteriosus was ligated. The pulmonary artery venting via the left pulmonary artery, cardiac arrest was achieved using antegrade cardioplegia infusion. We approached from superior of the left pulmonary artery without inversion. The vertical vein was divided and incised to the common pulmonary venous chamber, the posterior wall of left atrium was incised towards the left atrial appendage longitudinally. The common pulmonary venous chamber with vertical vein and the left atrium was anastomosed using 7-0 PDS running sutures. The atrial septal defect was closed directly. The aortic clamp times was 48 minutes. The left and right ventricular function were good with catecholamine supports and nitric oxide, weaning from cardiopulmonary bypass was smooth and the sternum was closed. Postoperative echocardiography and Computed Tomography 1 year after the surgery, between the common pulmonary venous chamber and the left atrium was no stenosis (Fig.2).

Discussion
Goldenhar syndrome is a rare congenital disease and has also been reported cases with cardiac and lung malformations. Most frequent is tetralogy of Fallot, followed by septal defects and situs inversus. Total anomalous pulmonary venous connection with lung agenesis have been reported only 6 cases [2,3,4], The combination of total anomalous pulmonary venous connection, unilateral lung agenesis, and Goldenhar syndrome were only two cases, however surgical outcomes were unknown [3,4]. To the best of our knowledge, this is the only case survived more than 1 year after surgery. Hasegawa and colleagues [4] reported cardiac procedures with lung agenesis were performed through a median sternotomy or a posterolateral thoracotomy, however the posterolateral thoracotomy approach were high mortality. Therefore, this case was performed through a median sternotomy, the total anomalous pulmonary venous connection and the posterior wall of left atrium were well in sight through a superior approach without inversion. The common pulmonary venous chamber and the left atrium was anastomosed using the vertical vein because the common pulmonary venous chamber was small. Following up more than 1 year after surgery, pulmonary venous obstruction due to use of the vertical vein was none.

Conclusion
A 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 pulmonary chamber.

Consent for publication
Informed consent was obtained from the patient.

Availability of data and materials
The data that support the ndings of this report are available from Chiba Children's Hospital. The author can make it available upon reasonable request.

Competing interests
There are no competing interests for all authors.