Abstract
Pulmonary vein stenosis after repair of total anomalous pulmonary venous connection (TAPVC) is a life-threatening complication that frequently recurs, particularly in patients with asplenia. Although intrinsic mechanisms such as ostial narrowing and progressive neointimal proliferation are well recognized, extrinsic mechanical factors are less commonly emphasized in congenital heart disease. We report a case of recurrent pulmonary vein stenosis in a patient with asplenia in whom intrinsic susceptibility and possible posterior extrinsic compression may have acted synergistically. Serial computed tomography demonstrated persistent proximity between the left pulmonary vein and indwelling enteral feeding tubes. Sustained improvement was achieved after surgical relocation of the left pulmonary vein orifice to a site remote from the tubes. This case suggests that external mechanical influences may represent an underrecognized and potentially modifiable contributor to postoperative pulmonary vein stenosis in high-risk pediatric patients.
Keywords: Pulmonary vein stenosis, Asplenia, Total anomalous pulmonary venous connection, Posterior compression, Enteral feeding tube
Case report
A 2-year-old girl was prenatally diagnosed with asplenia and single ventricle physiology. She was born at 38 weeks and 5 days of gestation with a birth weight of 2,479 g. Postnatal echocardiography revealed double-outlet right ventricle, total anomalous pulmonary venous connection (TAPVC, type 1b), a ventricular septal defect, moderate common atrioventricular valve regurgitation, and bilateral superior vena cavae. Computed tomography (CT) and chest radiography further demonstrated situs inversus and a sliding hiatal hernia. In this case, the vertical vein of the TAPVC drained into the left superior vena cava and exhibited significant stenosis at the entry site. Accordingly, surgical repair of the TAPVC by direct anastomosis to the left atrium and pulmonary artery banding was performed on the first day of life. To manage the hiatal hernia, gastric and duodenal tubes(6Fr) were placed immediately after birth for gastric decompression and nutritional support, followed by definitive surgical repair on day of life (DOL) 17. Although oral feeding was initiated thereafter, enteral nutrition via the duodenal tube was continued because of the patient’s intrinsically low gastric capacity. Subsequent echocardiography demonstrated progressively increased pulmonary venous flow velocity, and CT imaging revealed stenosis at the orifices of both pulmonary veins. Reoperation was performed on DOL 49. Despite initial improvement, recurrent stenosis of the left pulmonary vein orifice was detected around DOL 110, necessitating a second reoperation. Throughout this period, continuous placement of both gastric and duodenal tubes was required. Detailed CT imaging revealed that the left pulmonary vein orifice was consistently located in close proximity to the esophagus containing indwelling tubes, with the vertebral body positioned posterior to these structures (Fig. 1A, B). Therefore, it was postulated that prior to the second reoperation, the left pulmonary vein orifice had been persistently compressed posteriorly by the two relatively rigid feeding tubes (Fig. 1C). The posteriorly positioned vertebra prevented the tubes from displacing posteriorly, resulting in continuous anterior pressure on the vein. After the second reoperation, the left pulmonary vein orifice was repositioned laterally, away from the intraesophageal tubes. No further pulmonary vein stenosis has been observed (Fig. 1D).
Fig. 1.
Schematic illustration of the relationship among the vertebral body, indwelling feeding tubes, and the left pulmonary vein at representative time points. Timeline of anatomical changes by day of life (DOL). The red arrow indicates the left pulmonary vein, and the blue arrow indicates feeding tubes. A DOL 0, before development of pulmonary vein stenosis. B DOL 41, progressive narrowing of the left pulmonary vein orifice. C DOL 97, persistent posterior compression prior to the second reoperation. D DOL 154, after reoperation with release of posterior compression
Discussion
Pulmonary vein stenosis after TAPVC repair remains one of the most challenging complications in pediatric congenital heart disease, particularly in patients with asplenia. The pathogenesis of postoperative pulmonary venous obstruction has traditionally been attributed to intrinsic mechanisms, including ostial narrowing related to surgical factors and progressive neointimal or fibrotic proliferation [1, 2]. Procedure-related injury has also been reported as a cause of pulmonary vein stenosis, such as iatrogenic narrowing following catheter ablation procedures [3]. In addition, several reports have demonstrated that extrinsic mechanical forces can contribute to pulmonary venous obstruction. Documented examples include compression by vertebral osteophytes, descending aortic aneurysms, and patent ductus arteriosus, suggesting that external anatomical structures may influence the development or progression of pulmonary vein stenosis [4–6].
In our patient, intrinsic susceptibility associated with asplenia likely contributed to restenosis; however, the clinical course and serial imaging suggested that external mechanical factors may also have played a role. The left pulmonary vein orifice repeatedly aligned with the esophagus containing indwelling gastric and duodenal tubes, and computed tomography demonstrated persistent posterior proximity between these relatively rigid tubes and the pulmonary vein. Given the posterior constraint of the vertebral body, the tubes may have exerted sustained anterior pressure on the vein. Although causality cannot be definitively established, this anatomical configuration raises the possibility that extrinsic compression may have aggravated the recurrent obstruction. Because gastrointestinal anomalies and prolonged enteral tube placement are common in patients with asplenia, this mechanism may represent an underrecognized but clinically relevant contributor to postoperative pulmonary vein stenosis.
Catheter-based interventions, including balloon angioplasty and stent implantation, play an important role in the contemporary management of pulmonary vein stenosis. However, in this neonate, transcatheter therapy was not pursued due to the small vessel size, the proximity of the stenosis to the pulmonary venous confluence [1], and concerns regarding the long-term implications of stent placement in a rapidly growing infant. Surgical revision was therefore considered the most feasible and durable option. After surgical relocation of the left pulmonary vein orifice to a site remote from the enteral tubes, no further restenosis has been observed.
This case highlights that both intrinsic and extrinsic factors may interact in the development of recurrent pulmonary vein stenosis after TAPVC repair. Awareness of potentially modifiable external influences, such as device-related compression, may help optimize perioperative management in high-risk pediatric patients.
Conclusion
This case highlights that multiple factors may contribute to the development of pulmonary vein stenosis after TAPVC repair in patients with asplenia. In addition to the intrinsic atrial properties that predispose these patients to restenosis, possible extrinsic mechanical compression from prolonged enteral tube placement may have contributed to the recurrence in this case. Recognition of such potentially modifiable external influences underscores the importance of careful anatomical assessment and interdisciplinary coordination in the perioperative management of high-risk pediatric patients.
Acknowledgements
We would like to thank the radiology team at Kanazawa Medical University for their assistance in acquiring and interpreting the CT images used in this report. We also appreciate the support of the pediatric intensive care staff involved in the patient’s perioperative management.
Author contributions
Conceptualizing, Takako Nishino and Makoto Ando; writing-Original draft preparation, TN; writing-Review and editing, TN. All authors have read and agreed to the published version of the manuscript.
Funding
This research received no external funding.
Data availability
No datasets were generated or analysed during the current study.
Declarations
Ethics approval and consent to participate
Not applicable. Informed consent for publication was obtained from the patient’s guardians.
Competing interests
The authors declare no competing interests.
Footnotes
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References
- 1.Callahan R, Gauvreau K, Marshall AC, et al. Outcomes in establishing individual vessel patency for pediatric pulmonary vein stenosis. Children. 2021;8(3):210. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Liu X, Liufu R, Liu T, et al. Supracardiac total anomalous pulmonary venous connection type 1b: morphology and outcomes. J Thorac Cardiovasc Surg. 2023;166:193–200. [DOI] [PubMed] [Google Scholar]
- 3.Teunissen C, Velthuis BK, Hassink RJ, et al. Incidence of pulmonary vein stenosis after radiofrequency catheter ablation of atrial fibrillation. JACC Clin Electrophysiol. 2017;3(6):589–98. [DOI] [PubMed] [Google Scholar]
- 4.Kindzelski BA, Ghobrial J, Schlenk R, et al. Multidisciplinary approach to isolated pulmonary vein compression by an enlarging vertebral osteophyte. JACC Case Rep. 2022;4(3):145–9. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 5.Dewilde W, Ector J, Voigt J. Right pulmonary vein and bilateral compression by a massive descending aorta aneurysm. Int J Cardiol. 2007;122(2):e8-9. [DOI] [PubMed] [Google Scholar]
- 6.Bagaria V, Suresha SN, Hiremath CS, et al. Cardiomegaly secondary to patent ductus arteriosus causing extrinsic compression of left pulmonary vein. Eur J Cardiothorac Surg. 2022;62(5):455. [DOI] [PubMed] [Google Scholar]
Associated Data
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Data Availability Statement
No datasets were generated or analysed during the current study.