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Infracardiac total anomalous pulmonary venous drainage with concomitant hypoplastic aortic arch in a preterm neonate: a rare and complex surgical challenge

Published online by Cambridge University Press:  10 July 2025

Munavvar Gasimova Open the ORCID record for Munavvar Gasimova [Opens in a new window] ,
Bahruz Aliyev ,
Aynur Abbasaliyeva ,
Cabir Gulmaliyev ,
Khayala Mammadova  and
Shiraslan Bakhshaliyev Open the ORCID record for Shiraslan Bakhshaliyev [Opens in a new window]
Munavvar Gasimova*
Affiliation:
Anesthesiology and Intensive care, Liv Bona adea, Baku, Azerbaijan
Bahruz Aliyev
Affiliation:
Pediatric Cardiac Surgery Department, Liv Bona Dea Hospital, Baku, Azerbaijan
Aynur Abbasaliyeva
Affiliation:
Anesthesiology and Intensive care, Liv Bona adea, Baku, Azerbaijan
Cabir Gulmaliyev
Affiliation:
Cardiovascular surgery, Liv Bona Dea, Baku, Azerbaijan
Khayala Mammadova
Affiliation:
Pediatric Cardiac Surgery Department, Liv Bona Dea Hospital, Baku, Azerbaijan
Shiraslan Bakhshaliyev
Affiliation:
Pediatric Cardiac Surgery Department, Liv Bona Dea Hospital, Baku, Azerbaijan
*
Corresponding author: Munavvar Gasimova; E-mail: munavvar.gasimova1990@gmail.com
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Abstract

Total anomalous pulmonary venous drainage associated with hypoplastic aortic arch represents an exceptionally rare and intricate congenital cardiac anomaly. We report the case of a 13-day-old late preterm neonate presenting with infracardiac total anomalous pulmonary venous drainage, severe pulmonary hypertension, and a hypoplastic aortic arch. The patient underwent single-stage surgical correction involving rerouting of the pulmonary venous confluence to the left atrium and aortic arch reconstruction using autologous pericardial patch under cardiopulmonary bypass with selective cerebral perfusion. Postoperative recovery was favourable, marked by successful extubation, resolution of pulmonary hypertension, and absence of residual anatomical obstruction. This case highlights the feasibility and effectiveness of early, comprehensive surgical repair and the necessity of coordinated multidisciplinary perioperative care. Long-term follow-up remains vital for monitoring functional outcomes and detecting late complications.

Keywords

Total anomalous pulmonary venous drainagehypoplastic aortic archneonaterare case

Information

Type
Case Report
Information
Cardiology in the Young , Volume 35 , Issue 9 , September 2025 , pp. 1944 - 1946
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Total anomalous pulmonary venous connection is a rare congenital anomaly wherein the pulmonary veins fail to connect to the left atrium, Reference Files and Morray1 resulting in systemic desaturation and right heart volume overload. Total anomalous pulmonary venous connection is classified anatomically into supracardiac, cardiac, infracardiac, and mixed types. Reference Haas, Driscoll and Rickert-Sperling2 Infracardiac total anomalous pulmonary venous drainage, one of the least frequent subtype, is often associated with obstruction and carries a high perioperative risk. Reference Matsui, Adachi, Uemura, Gardiner and Ho3

Hypoplastic aortic arch, characterised by diffuse narrowing of the aortic arch, represents a severe form of left-sided obstructive lesion. Its coexistence with total anomalous pulmonary venous drainage is exceedingly uncommon and significantly complicates both diagnosis and surgical management. Reference Yun4

This report details a rare and complex case of infracardiac total anomalous pulmonary venous drainage with hypoplastic aortic arch in a late preterm neonate, highlighting the diagnostic challenges, surgical strategy, and successful postoperative outcome.

Case presentation

A 13-day-old male late preterm infant (gestational age: 36 weeks, birth weight: 2.8 kg) was admitted to the cardiovascular ICU with marked respiratory distress (Figure 1) (RR: 60/min), oxygen saturation of 85% on supplemental oxygen, and blood pressure of 65/34 mmHg. Clinical examination revealed cyanosis and a hyperdynamic precordium. Initial transthoracic echocardiography demonstrated infracardiac total anomalous pulmonary venous drainage with a large, non-restrictive atrial septal defect, severe pulmonary hypertension, right heart dilation, and mitral/aortic annulus Z-scores of -3, consistent with underdevelopment of left-sided cardiac structures. CT angiography confirmed infracardiac total anomalous pulmonary venous drainage with drainage via a vertical vein into the portal system, a large patent ductus arteriosus, and a hypoplastic aortic arch with diffuse narrowing (Figures 2 and 3).

Figure 1.

Pulmonary congestion and cardiomegaly consistent with volume overload secondary to total anomalous pulmonary venous drainage and pulmonary hypertension.

Figure 2.

Coronal contrast–enhanced CT scan showing anomalous pulmonary venous return. The right and left pulmonary veins drain into a vertical vein,which empties into the hepatic venous system,consistent with total anomalous pulmonary venous drainage.

Figure 3.

Coronal contrast-enhanced CT angiogram demonstrating aortic arch hypoplasia. The ascending aorta gives rise to a hypoplastic aortic arch, with a patent ductus arteriosus providing compensatory flow to the descending aorta. The narrowed aortic segment suggests significant arch obstruction, which, in the neonatal period, may lead to duct-dependent systemic circulation. This imaging is critical for preoperative assessment, aiding in surgical planning for arch reconstruction.

An alprostadil infusion (0.01 mcg/kg/min) was initiated to maintain ductal patency. Given the haemodynamic instability and complex anatomical findings, the patient was scheduled for early surgical intervention.

Surgical management

Via median sternotomy, aorto-bicaval cannulation was established, and the patient was cooled to 28°C. Following cross-clamping, cardioplegic arrest was achieved using antegrade Del Nido cardioplegia. The patent ductus arteriosus was ligated, and the aortic cannula advanced for selective cerebral perfusion. The hypoplastic aortic arch was excised, and reconstruction was performed using an autologous pericardial patch as a patch arterioplasty, restoring the arch continuity and calibre.

The vertical vein was ligated supradiaphragmatically. After left atriotomy, the pulmonary venous confluence was anastomosed to the posterior left atrial wall. The atrial septal defect was closed with an autologous pericardial patch incorporating a 3-mm fenestration to accommodate potential postoperative pressure gradients. An autologous pericardial patch treated with glutaraldehyde was used for both aortic arch reconstruction and atrial septal defect closure. The sternum was left open temporarily with skin coverage using a Dacron patch. Total CPB time was 144 minutes, with an aortic cross-clamp duration of 95 minutes. The patient was transferred to the cardiovascular ICU in stable condition on milrinone, norepinephrine, adrenaline, and inhaled iloprost.

Postoperative course

The sternum was closed 72 hours postoperatively. The patient was extubated on postoperative day 4 and demonstrated progressive improvement in oxygenation and feeding tolerance. Follow-up echocardiography revealed unobstructed pulmonary venous flow (mean velocity: 0.93 m/s), no gradient across the reconstructed aortic arch (peak gradient: 20 mmHg), and no evidence of persistent pulmonary hypertension. A small residual secundum atrial septal defect was noted. The patient was discharged on postoperative day 16 with stable haemodynamics and normal growth parameters.

Discussion

This case exemplifies the clinical and surgical complexity of managing infracardiac total anomalous pulmonary venous drainage in conjunction with a hypoplastic aortic arch. Both anomalies independently demand urgent intervention due to the risk of profound hypoxaemia and compromised systemic perfusion. Reference Konduri and Aggarwal5 Their coexistence poses significant technical and physiological challenges. The coexistence of infracardiac total anomalous pulmonary venous drainage with hypoplastic aortic arch may reflect a shared fetal haemodynamic basis, as impaired pulmonary venous return can result in reduced left atrial filling and subsequently hypoplastic development of the mitral valve, left ventricle, and aortic arch. Reference Doksöz, Güven and Demirpençe6

The infracardiac variant is particularly associated with pulmonary venous obstruction, while hypoplastic aortic arch contributes to systemic hypoperfusion. Reference Shhada, Nahle and Hamdar7 The presence of mitral and aortic annular hypoplasia, likely secondary to diminished left atrial inflow during fetal development, further complicates the perioperative course. Reference Kritzmire, Thomas and Horenstein8

Surgical correction involved precise timing and coordination. The strategy of simultaneous arch reconstruction and pulmonary venous rerouting was chosen to minimise cumulative bypass time and reduce the risk of prolonged hypoxia or left heart underloading. Reference Bernabei, Margaryan, Arcieri, Bianchi, Pak and Murzi9 Emerging strategies, including beating-heart arch reconstruction under selective myocardial perfusion, Reference Ergün, Onan and Yildiz10 are routinely applied by our team in isolated arch reconstructions. However, in this case, we elected to use conventional cardioplegic arrest to enable precise arch reconstruction and intracardiac repair

The selective cerebral perfusion time was 28 minutes. The use of selective cerebral perfusion and autologous materials likely contributed to the favourable neurological and haemodynamic outcomes. Reference Asou, Kado and Imoto11

Postoperative pulmonary hypertension was managed effectively with inotropes and inhaled iloprost, optimising right ventricular performance without the need for long-term central access. Reference Janjindamai, Thatrimontrichai, Maneenil, Chanvitan and Dissaneevate12 Although inhaled nitric oxide is often preferred due to its rapid onset and short half-life, we employed inhaled iloprost based on institutional protocol and availability. It provided effective pulmonary vasodilation without causing systemic hypotension in this case.

Alternative strategies for managing such complex presentations include staged repair (initial arch reconstruction followed by delayed total anomalous pulmonary venous drainage repair), or hybrid approaches incorporating ductal stenting. Reference Yerebakan, Murray and Valeske13 However, early single-stage repair offers the advantage of reduced cumulative bypass time and earlier physiologic restoration.

Meticulous follow-up is critical, as late complications such as pulmonary venous stenosis, recoarctation, or arrhythmia can occur.

Conclusion

This report documents a highly unusual and complex congenital presentation, successfully managed with a single-stage surgical repair. The favourable short-term outcome underscores the importance of early diagnosis, tailored surgical strategy, and expert perioperative management. Long-term surveillance remains essential to monitor for potential late sequelae and ensure continued haemodynamic stability.

References

Files, MD, Morray, B. Total anomalous pulmonary venous connection: preoperative anatomy, physiology, imaging, and interventional management of postoperative pulmonary venous obstruction. Semin Cardiothorac Vasc Anesth 2017; 21: 123–131.CrossRefGoogle ScholarPubMed
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Matsui, H, Adachi, I, Uemura, H, Gardiner, H, Ho, SY. Anatomy of coarctation, hypoplastic and interrupted aortic arch: relevance to interventional/surgical treatment. Expert Rev Cardiovasc Ther 2007; 5: 871–880.CrossRefGoogle ScholarPubMed
Yun, SW. Congenital heart disease in the newborn requiring early intervention. Korean J Pediatr 2011; 54: 183–191.CrossRefGoogle ScholarPubMed
Konduri, A, Aggarwal, S. Partial and Total Anomalous Pulmonary Venous Connection. In: StatPearls. StatPearls Publishing, Treasure Island (FL), 2019. [Updated 2023 Jul 17].Google Scholar
Doksöz, Ö., Güven, B, Demirpençe, S, et al. Coarctation of the aorta with infracardiac total anomalous pulmonary venous drainage: a rare combination. Ann Thorac Cardiovasc Surg 2014; 20: 778–780.Google ScholarPubMed
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Figure 0

Figure 1. Pulmonary congestion and cardiomegaly consistent with volume overload secondary to total anomalous pulmonary venous drainage and pulmonary hypertension.

Figure 1

Figure 2. Coronal contrast–enhanced CT scan showing anomalous pulmonary venous return. The right and left pulmonary veins drain into a vertical vein,which empties into the hepatic venous system,consistent with total anomalous pulmonary venous drainage.

Figure 2

Figure 3. Coronal contrast-enhanced CT angiogram demonstrating aortic arch hypoplasia. The ascending aorta gives rise to a hypoplastic aortic arch, with a patent ductus arteriosus providing compensatory flow to the descending aorta. The narrowed aortic segment suggests significant arch obstruction, which, in the neonatal period, may lead to duct-dependent systemic circulation. This imaging is critical for preoperative assessment, aiding in surgical planning for arch reconstruction.