One-stage surgical repair of infracardiac total anomalous pulmonary venous connection and coarctation of the aorta in a neonate

Abstract

Total anomalous pulmonary venous connection is a rare congenital anomaly that requires prompt surgical repair. Associated anomalies are common; however, its coexistence with coarctation of the aorta is extremely rare. Herein, we present a rare case of a neonate who underwent successful one-stage repair of infracardiac total anomalous pulmonary venous connection associated with coarctation of the aorta.

Introduction

Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart defect (CHD) (0.4%–2% of all CHDs) in which the four pulmonary veins do not drain into the left atrium (LA), but into a common pulmonary confluence (CPC) that connects to the right atrium (RA) or one of its tributaries [1]. In its infracardiac type, the CPC drains via a vertical vein (VV) which crosses the diaphragm and connects to the portal vein, ductus venosus, hepatic vein, or the inferior vena cava (IVC) [2]. The pathway of CPC in infracardiac TAPVC is usually associated with obstruction resulting in an early presentation during the first days of life with severe respiratory distress and poor feeding, and without urgent surgical repair, the mortality rate is very high [3]. However, there are quite a few reported cases of infracardiac TAPVC without obstructions [4]. TAPVC may be associated with other defects such as tetralogy of Fallot, ventricular septal defect, and coarctation of the aorta (CoA) [5]. It has been reported that TAPVC was associated with CoA in only 1.4% of cases, and in only two cases the TAPVC was of the infracardiac type [6]. Herein, we present a very rare case of a neonate who underwent successful one-stage surgical repair of infracardiac TAPVC and CoA.

Case presentation

A 20-day-old boy with an unremarkable perinatal history was referred to the pediatric emergency department with respiratory distress. The physical examination revealed poor general condition and central cyanosis. The pulses were bounding in both brachial arteries but the femoral arteries were not palpable. A systolic murmur grade 4/6 was heard along the left sternal edge. The liver was enlarged 2 cm below the costal margin. Transthoracic echocardiography (TTE) was performed and revealed infracardiac TAPVC with associated CoA. Computed tomography angiography (CTA) confirmed the diagnosis of CoA (Fig. 1), and for technical issues, the pathway of CPC was not apparent on CTA. TTE was repeated and the drainage of the CPC into the portal vein was confirmed. The heart team at our hospital decided for urgent surgical repair. At first, the repair of CoA was performed through left thoracotomy with patch plasty technique since the stenosis was too long, and it was not possible to perform resection and end-to-end anastomosis. Then, through median sternotomy, the pericardium was opened, and a total cardiopulmonary bypass (CPB) was prepared. The heart was arrested with an antegrade cold blood cardioplegic solution. The CPC was seen behind the pericardium (Fig. 2). The VV was descending crossing the diaphragm, and it was dissected and controlled (Fig. 3). The heart was lifted out of the pericardium and a long incision was made in LA, and a parallel incision was made in the CPC (Fig. 4). A wide anastomosis was performed between these two incisions thus connecting the CPC into LA. The VV was permanently closed. RA was opened, and a small ASD was closed. The remainder of the operation was completed uneventfully. The patient was weaned off the CPB with stable hemodynamic conditions on low-dose inotropes (milrinone, epinephrin). Postoperative TTE showed normal pulmonary venous drainage without any gradient across the anastomosis, and excellent repair of CoA. After 48 hours on mechanical ventilation, the patient was extubated, and discharged from the ICU after 5 days. On one year follow-up, the patient was with normal growth, and in very good general condition without any complaints. TTE during the follow-up period revealed normal flow in the pulmonary veins.

Discussion

The combination of TAPVC with CoA is extremely rare, and it has been reported that only 6 of 422 patients (1.4%) with TAPVC had associated CoA [6]. Neonates with infracardiac TAPVC usually present early in the first days of life with severe symptoms due to obstruction in the pathway of the CPC [5]. When associated with CoA, the blood supply to the lower body comes from ductal flow, and diagnosis may be missed preoperatively since TTE may not detect the acceleration of flow at the coarctation site [7], and thus the severity of CoA is often underestimated. In our case, TTE was performed and was crucial as it revealed the association of infracardiac TAPVC with CoA which was confirmed by CTA. To the best of our knowledge, this is only the second reported case with this combination of infracardiac TAPVC and COA [5], although some similar cases with other types of TAPVC have also been reported [7, 8].

Conclusion

The association of TAPVC with coarctation of the aorta is extremely rare, and by then prompt one-stage repair is indicated. Precise surgical repair of such complex cases can save the lives of such moribund neonates.

Author contributions

None of the authors listed in the manuscript are employed by a government agency that has a primary function other than research and/or education. Moreover, none of the authors are submitting this manuscript as an official representative or on behalf of the government.

Conflict of interest statement

The authors have no conflict of interest.

Funding

There are no funding resources for writing this manuscript.

Data availability

The data that support the findings of this study are available from the corresponding author, upon reasonable request.

References