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Clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension secondary to congenital portosystemic shunts

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Abstract

The aim of this retrospective study was to investigate the clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension (PAH) secondary to congenital portosystemic shunts (CPSS). Thirty-three pediatric patients diagnosed in our institution with CPSS between 2012 and 2019 were enrolled in this study. The patients were divided into PAH and non-PAH groups. The PAH group included 15 patients who presented with unexplained PAH when CPSS was diagnosed. Two patients with microangiopathic hemolytic anemia died of right heart failure shortly after diagnosis. One patient received a liver transplant at the age of 4.3 years and showed a mild decrease in pulmonary artery pressure (PAP) 4 years after the operation. Seven patients underwent one-stage shunt closure at a median age of 2.8 years (1.4–13 years). Follow-up examinations, from 1.6 to 4.1 years after intervention, showed marked reduction of PAP in one patient and stabilization of PAH in six others. However, in one patient who underwent two-stage shunt closure, a marked increase in PAP was noted after partial ligation of the shunt. The remaining four patients received only pulmonary vasodilator therapy, and one of them died of right heart failure 12 years after the PAH diagnosis. The non-PAH group included 18 patients without evidence of PAH upon CPSS diagnosis. Shunt closure was carried out in eight of these patients, but one patient subsequently developed PAH after the resolution of hepatopulmonary syndrome.

Conclusion: CPSS may be a more likely cause of unexplained PAH in pediatric patients than previously thought. Shunt closure or liver transplantation may prevent the progression of PAH, or even improve it for the majority of CPSS patients.

What is Known:

• Congenital portosystemic shunts (CPSS) are increasingly recognized as a cause of pulmonary arterial hypertension (PAH) in children, but the prevalence of CPSS in pediatric PAH patients is largely unknown, and the therapeutic outcomes of these conditions are not well described.

What is New:

• This is the largest cohort of CPSS-associated PAH ever published. CPSS may be a not uncommon cause of unexplained PAH in pediatric patients.

• Shunt closure or liver transplantation may prevent the progression of PAH, or even improve it for the majority of CPSS patients.

• Shunt closure might cause unique pulmonary hemodynamic changes in CPSS patients associated with hepatopulmonary syndrome (HPS), even leading to the transition from HPS to PAH.

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Abbreviations

CPSS:

Congenital portosystemic shunts

HPS:

Hepatopulmonary syndrome

PAH:

Pulmonary arterial hypertension

PAP:

Pulmonary artery pressure

RHC:

Right heart catheterization

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Funding

This research was supported by the National Natural Science Fund of China (81770380, 81974029).

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Author notes

  1. Jinjin Wu and Yi Lu contributed equally to this work.

Authors and Affiliations

  1. Department of Cardiology, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China

    Jinjin Wu, Wenzhuo Zhao, Jie Shen, Fen Li & Lijun Fu

  2. Department of Internal Medicine, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China

    Yi Lu

  3. Department of cardiothoracic surgery, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China

    Hao Zhang

  4. Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China

    Qimin Chen

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  1. Jinjin Wu
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Contributions

Fu Lijun and Chen Qimin designed the study and reviewed the manuscirpt. Lu Yi, Shen Jie, and Li Fen contributed to follow-up data collection. Zhao Wenzhuo run the data analysis. Wu Jinjin wrote the manuscript. Zhang Hao reviewed the manuscript.

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Correspondence to Qimin Chen or Lijun Fu.

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Wu, J., Lu, Y., Zhao, W. et al. Clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension secondary to congenital portosystemic shunts. Eur J Pediatr 180, 929–936 (2021). https://doi.org/10.1007/s00431-020-03817-y

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  • DOI: https://doi.org/10.1007/s00431-020-03817-y

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