Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary Center

Aim: To define the clinical characteristics, hemodynamics, and adverse events for pediatric patients with pulmonary arterial hypertension (PAH) undergoing right heart catheterization (RHC). Methods: The large referral single center data of 591 diagnostic RHC procedures performed between 2005 and 202...

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Main Authors: Zhuoyuan Xu, Hongsheng Zhang, Alexandra Arvanitaki, Chen Zhang, Qiangqiang Li, Bradley B. Keller, Hong Gu
Format: Article
Language:English
Published: MDPI AG 2022-09-01
Series:Journal of Clinical Medicine
Subjects:
Online Access:https://www.mdpi.com/2077-0383/11/18/5374
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author Zhuoyuan Xu
Hongsheng Zhang
Alexandra Arvanitaki
Chen Zhang
Qiangqiang Li
Bradley B. Keller
Hong Gu
author_facet Zhuoyuan Xu
Hongsheng Zhang
Alexandra Arvanitaki
Chen Zhang
Qiangqiang Li
Bradley B. Keller
Hong Gu
author_sort Zhuoyuan Xu
collection DOAJ
description Aim: To define the clinical characteristics, hemodynamics, and adverse events for pediatric patients with pulmonary arterial hypertension (PAH) undergoing right heart catheterization (RHC). Methods: The large referral single center data of 591 diagnostic RHC procedures performed between 2005 and 2020 on pediatric PAH patients was retrospectively collected and analyzed. Results: A total of 591 RHC procedures performed on 469 patients with congenital heart disease (CHD)-PAH (median age 8.8 years, 7.9% New York Heart Association (NYHA) class > II, 1.5% with syncope) and 122 patients with idiopathic PAH (median age of 9.0 years, 27.0% NYHA class > II, 27.0% with syncope) were included. Of those, 373 (63.1%) procedures were performed under general anesthesia. Eighteen patients (18/591, 3.0%) suffered adverse events (mainly pulmonary hypertensive crisis, PHC, n = 17) during the RHC procedure, including 14 idiopathic pulmonary arterial hypertension (IPAH) patients and 4 CHD-PAH patients, and one IPAH patient died in hospital 63 hours after RHC. The risk of developing PHC was significantly increased in patients with IPAH (OR = 14.02, 95%CI: 4.49–43.85, <i>p</i> < 0.001), atrial blood gas pH < 7.35 (OR = 12.504, 95%CI: 3.545–44.102, <i>p</i> < 0.001) and RAP > 14 mmHg (OR = 10.636, 95%CI: 3.668–30.847, <i>p</i> < 0.001). Conclusions: RHC is generally a low-risk procedure in pediatric patients with PAH. However, PHC occur in approximately 3% of patients. Therefore, RHC should be performed in a large, experienced referral pediatric cardiology center, especially in pediatric patients with IPAH requiring general anesthesia.
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spelling doaj.art-d118104ec52d4d02bfaf23274984b2f12023-11-23T16:59:38ZengMDPI AGJournal of Clinical Medicine2077-03832022-09-011118537410.3390/jcm11185374Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary CenterZhuoyuan Xu0Hongsheng Zhang1Alexandra Arvanitaki2Chen Zhang3Qiangqiang Li4Bradley B. Keller5Hong Gu6Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, ChinaDepartment of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China1st Department of Cardiology, AHEPA University Hospital, 54621 Thessaloniki, GreeceDepartment of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, ChinaDepartment of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, ChinaGreater Louisville and Western Kentucky Practice, Cincinnati Children’s Heart Institute, Louisville, KY 40202, USADepartment of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, ChinaAim: To define the clinical characteristics, hemodynamics, and adverse events for pediatric patients with pulmonary arterial hypertension (PAH) undergoing right heart catheterization (RHC). Methods: The large referral single center data of 591 diagnostic RHC procedures performed between 2005 and 2020 on pediatric PAH patients was retrospectively collected and analyzed. Results: A total of 591 RHC procedures performed on 469 patients with congenital heart disease (CHD)-PAH (median age 8.8 years, 7.9% New York Heart Association (NYHA) class > II, 1.5% with syncope) and 122 patients with idiopathic PAH (median age of 9.0 years, 27.0% NYHA class > II, 27.0% with syncope) were included. Of those, 373 (63.1%) procedures were performed under general anesthesia. Eighteen patients (18/591, 3.0%) suffered adverse events (mainly pulmonary hypertensive crisis, PHC, n = 17) during the RHC procedure, including 14 idiopathic pulmonary arterial hypertension (IPAH) patients and 4 CHD-PAH patients, and one IPAH patient died in hospital 63 hours after RHC. The risk of developing PHC was significantly increased in patients with IPAH (OR = 14.02, 95%CI: 4.49–43.85, <i>p</i> < 0.001), atrial blood gas pH < 7.35 (OR = 12.504, 95%CI: 3.545–44.102, <i>p</i> < 0.001) and RAP > 14 mmHg (OR = 10.636, 95%CI: 3.668–30.847, <i>p</i> < 0.001). Conclusions: RHC is generally a low-risk procedure in pediatric patients with PAH. However, PHC occur in approximately 3% of patients. Therefore, RHC should be performed in a large, experienced referral pediatric cardiology center, especially in pediatric patients with IPAH requiring general anesthesia.https://www.mdpi.com/2077-0383/11/18/5374pediatricright heart catheterizationpulmonary arterial hypertensionoutcome
spellingShingle Zhuoyuan Xu
Hongsheng Zhang
Alexandra Arvanitaki
Chen Zhang
Qiangqiang Li
Bradley B. Keller
Hong Gu
Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary Center
Journal of Clinical Medicine
pediatric
right heart catheterization
pulmonary arterial hypertension
outcome
title Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary Center
title_full Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary Center
title_fullStr Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary Center
title_full_unstemmed Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary Center
title_short Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary Center
title_sort right heart catheterization in pediatric pulmonary arterial hypertension insights and outcome from a large tertiary center
topic pediatric
right heart catheterization
pulmonary arterial hypertension
outcome
url https://www.mdpi.com/2077-0383/11/18/5374
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