Jinjin Wu1, Yi Lu2, Wenzhuo Zhao1, Jie Shen1, Fen Li1, Hao Zhang3, Qimin Chen4, Lijun Fu5. 1. Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China. 2. Department of Internal Medicine, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China. 3. Department of cardiothoracic surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China. 4. Department of Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China. chenqimin@scmc.com.cn. 5. Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China. fulijun@scmc.com.cn.
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.
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.
Authors: Myung Jin Kim; Jae Sung Ko; Jeong Kee Seo; Hye Ran Yang; Ju Young Chang; Gi Beom Kim; Jung-Eun Cheon; Woo Sun Kim Journal: Eur J Pediatr Date: 2011-09-13 Impact factor: 3.183
Authors: Anna Baiges; Fanny Turon; Macarena Simón-Talero; Stephanie Tasayco; Javier Bueno; Kamal Zekrini; Aurélie Plessier; Stéphanie Franchi-Abella; Florent Guerin; Amar Mukund; C E Eapen; Ashish Goel; Nidugala Keshava Shyamkumar; Sandra Coenen; Andrea De Gottardi; Avik Majumdar; Simona Onali; Akash Shukla; Flair José Carrilho; Lucas Nacif; Massimo Primignani; Giulia Tosetti; Vicenzo La Mura; Frederik Nevens; Peter Witters; Dhiraj Tripathi; Luis Tellez; Javier Martínez; Carmen Álvarez-Navascués; Miguel López Fraile López; Bogdan Procopet; Fabio Piscaglia; Barbara de Koning; Elba Llop; Mario Romero-Cristobal; Eric Tjwa; Alberto Monescillo-Francia; Marco Senzolo; Mercedes Perez-LaFuente; Antonio Segarra; Shiv Kumar Sarin; Virginia Hernández-Gea; David Patch; Wim Laleman; Hermien Hartog; Dominique Valla; Joan Genescà; Juan Carlos García-Pagán Journal: Hepatology Date: 2019-08-19 Impact factor: 17.425