Case Study

Pulmonary atresia with ventricular septal defect and pulmonary sequestration

Asian Cardiovascular & Thoracic Annals 21(4) 460–463 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312456692 aan.sagepub.com

Tomohiro Nakata1, Tadashi Ikeda1, Hiraku Doi2, Shiro Baba2 and Ryuzo Sakata1

Abstract A 5-day-old girl who had pulmonary atresia with ventricular septal defect, patent ductus arteriosus, intralobar pulmonary sequestration of the right lower lobe, and retroesophageal innominate vein, was referred to our institution. We successfully performed a systemic-to-pulmonary shunt. During cardiopulmonary bypass, the arteries supplying the sequestrated lung were ligated above the diaphragm through the median sternotomy, without a thoracotomy. The postoperative course was uneventful, and we avoided the potential complications of lung infection and ventricular overload.

Keywords Multiple abnormalities, bronchopulmonary sequestration, patent ductus arteriosus, pulmonary atresia with ventricular septal defect

Introduction Pulmonary sequestration is a rare condition in which a portion of lung tissue receives its blood supply from an anomalous systemic artery. It is treated by surgical removal of the sequestrated lung tissue or coil embolization, to prevent infections and progressive respiratory symptoms.1–4 We encountered a neonate who had pulmonary atresia with ventricular septal defect and intralobar pulmonary sequestration of the right lower lung. The supplying artery arose just adjacent to the celiac trunk, therefore, it was not suitable for coil embolization. To prevent possible infection and ventricular overload caused by the pulmonary sequestration, we ligated the anomalous arteries through a median sternotomy, with concomitant creation of a systemic-to-pulmonary shunt.5,6

Case report A 5-day-old girl was referred to our institution for surgical intervention. A chest radiograph (Figure 1(a)) revealed an abnormal boot-like appearance of the heart, but no parenchymal changes. Echocardiography revealed pulmonary atresia with ventricular septal defect (Figure 1(b)), patent ductus arteriosus, and a

right aortic arch. The ductus arteriosus was maintained by continuous lipo-prostaglandin E1 infusion. Preoperative cardiac catheterization revealed sequestration of the right lower lung (Figure 1(c)). The supplying artery arose from the descending aorta, just adjacent to the celiac trunk. In the venous phase, blood from this area drained into the right pulmonary veins. Based on these findings, we diagnosed intralobar pulmonary sequestration. Multidetector-row computed tomography (MDCT) showed the detailed configuration of the supplying artery (Figure 2(a) and (b)), and revealed an extremely rare anomaly of the innominate vein: retroesophageal innominate vein (Figure 2(c)).7,8 As the supplying artery arose near the major abdominal artery (i.e. the celiac trunk), coil embolization entailed 1 Department of Cardiovascular Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan 2 Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan

Corresponding author: Tomohiro Nakata, MD, PhD, Department of Cardiovascular Surgery, Kyoto University Graduate School of Medicine, 54 Shogoin-KawaharaCho, Sakyo-Ku, Kyoto, 606-8507, Japan. Email: [email protected]

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Nakata et al.

461 median sternotomy. The patient was placed on cardiopulmonary bypass in the standard fashion, using ascending aortic and right atrial cannulation. After division of the ductus arteriosus, the supplying arteries in the right pleural cavity were approached through a right pleurotomy. Ligation of 2 major and 1 minor supplying arteries was carried out without performing a thoracotomy. An expanded polytetrafluoroethylene graft (3.5 mm in diameter) was sewn to the left subclavian artery and the left pulmonary artery. The duration of cardiopulmonary bypass was 111 min. Postoperative MDCT showed obstruction of the supplying arteries above the diaphragm. At 10 months of age, the patient successfully underwent a total correction, which included ventricular septal defect closure, resection of the hypertrophied right ventricular muscle, and right ventricular outflow tract reconstruction using a tanned autologous pericardial patch with an expanded polytetrafluoroethylene monocusp valve. She has had no bouts of pneumonia on follow-up.

Discussion

Figure 1. Preoperative imaging: (a) chest radiograph, (b) echocardiogram, and (c) cardiac catheterization. The supplying artery of the pulmonary sequestration (black arrows) arose from the descending aorta, just adjacent to the celiac trunk (white arrow). Ao: aorta; LV: left ventricle; RV: right ventricle.

considerable risk. Therefore, we decided to ligate the supplying arteries surgically, concomitant with the cardiac operation. On the 33rd day after birth (weight, 3.2 kg), cardiac surgery was performed through a

Pulmonary sequestration is a rare congenital malformation characterized by dysplastic and nonfunctioning lung tissue, with abnormal connection to the tracheobronchial tree and the pulmonary arteries.1,2 It commonly results in recurrent respiratory problems, and occasionally causes congestive heart failure due to excessive blood flow through the abnormal lobe.5,6 Conventional treatment involves surgical removal of the sequestrated tissue (lobectomy or wedge resection) or coil embolization of the supplying artery.1,2 Coil embolization is less invasive than surgical lung resection, and can effectively prevent late complications or recurrent pneumonia.3 Therefore, this approach has been used widely in recent years, especially in neonates and young infants. In our patient, because the supplying artery was large and arose close to the celiac trunk (Figure 2(b)), surgical ligation of the supplying arteries concomitant with a systemic-to-pulmonary shunt was thought to be the safest and best way to treat the sequestration.6 However, evaluation of volume load incurred by pulmonary sequestration is difficult in the presence of intracardiac malformations or an extracardiac left-to-right shunt. To prevent hemodynamic instability associated with a systemic-to-pulmonary shunt, the supplying arteries should be ligated before creation of the shunt. To the best of our knowledge, such a concomitant operation has not been reported previously. In our experience, lung decompression during cardiopulmonary bypass makes it easy to dissect, identify, and ligate the supplying arteries in the pleural cavity, without single-lung ventilation.

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Figure 2. Preoperative multidetector-row computed tomography ((a) anterior view, (b) right anterior oblique view) showing the detailed configuration of the supplying artery of the pulmonary sequestration (black arrows) and celiac trunk (black arrowhead), and ((c) posterior view) the retroesophageal innominate vein (white arrowheads).

An anomalous retroesophageal innominate vein is very rare.7,8 It travels posteriorly to the trachea and esophagus, subsequently joining the azygos vein before draining into the superior vena cava. Because we confirmed the diagnosis by MDCT preoperatively, we were able to leave the innominate vein untouched, and avoid needless dissection. MDCT is a good modality for evaluating both pulmonary sequestration and anomalous retroesophageal innominate vein.

pleural cavity through a median sternotomy and thereby avoided the potential complications of volume overload and lung infection. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflicts of interest statement None declared.

Conclusion In conclusion, we successfully performed a systemic-topulmonary shunt with concomitant ligation of the supplying arteries of a pulmonary sequestration in the

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6. Levine MM, Nudel DB, Gootman N, Wolpowitz A and Wisoff BG. Pulmonary sequestration causing congestive heart failure in infancy: a report of two cases and review of the literature. Ann Thorac Surg 1982; 34: 581–585. 7. Ming Z, Aimin S and Rui H. Evaluation of the anomalous retroesophageal left brachiocephalic vein in Chinese children using multidetector CT. Pediatr Radiol 2009; 39: 343–347. 8. Choi JY, Jung MJ, Kim YH, Noh CI and Yun YS. Anomalous subaortic position of the brachiocephalic vein (innominate vein): an echocardiographic study. Br Heart J 1990; 64: 385–387.

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Pulmonary atresia with ventricular septal defect and pulmonary sequestration.

A 5-day-old girl who had pulmonary atresia with ventricular septal defect, patent ductus arteriosus, intralobar pulmonary sequestration of the right l...
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