PMID- 36143021 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230308 IS - 2077-0383 (Print) IS - 2077-0383 (Electronic) IS - 2077-0383 (Linking) VI - 11 IP - 18 DP - 2022 Sep 13 TI - Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary Center. LID - 10.3390/jcm11185374 [doi] LID - 5374 AB - 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, p < 0.001), atrial blood gas pH < 7.35 (OR = 12.504, 95%CI: 3.545-44.102, p < 0.001) and RAP > 14 mmHg (OR = 10.636, 95%CI: 3.668-30.847, p < 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. FAU - Xu, Zhuoyuan AU - Xu Z AD - Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China. AD - Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China. FAU - Zhang, Hongsheng AU - Zhang H AD - Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China. AD - Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China. FAU - Arvanitaki, Alexandra AU - Arvanitaki A AUID- ORCID: 0000-0003-3180-7280 AD - 1st Department of Cardiology, AHEPA University Hospital, 54621 Thessaloniki, Greece. AD - Pulmonary Hypertension Service, Royal Brompton Hospital, London SW3 6NP, UK. FAU - Zhang, Chen AU - Zhang C AD - Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China. AD - Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China. FAU - Li, Qiangqiang AU - Li Q AD - Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China. AD - Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China. FAU - Keller, Bradley B AU - Keller BB AUID- ORCID: 0000-0001-5476-6050 AD - Greater Louisville and Western Kentucky Practice, Cincinnati Children's Heart Institute, Louisville, KY 40202, USA. FAU - Gu, Hong AU - Gu H AD - Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China. AD - Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China. LA - eng PT - Journal Article DEP - 20220913 PL - Switzerland TA - J Clin Med JT - Journal of clinical medicine JID - 101606588 PMC - PMC9500744 OTO - NOTNLM OT - outcome OT - pediatric OT - pulmonary arterial hypertension OT - right heart catheterization COIS- The authors declare no conflict of interest. EDAT- 2022/09/24 06:00 MHDA- 2022/09/24 06:01 PMCR- 2022/09/13 CRDT- 2022/09/23 01:27 PHST- 2022/08/16 00:00 [received] PHST- 2022/08/30 00:00 [revised] PHST- 2022/09/02 00:00 [accepted] PHST- 2022/09/23 01:27 [entrez] PHST- 2022/09/24 06:00 [pubmed] PHST- 2022/09/24 06:01 [medline] PHST- 2022/09/13 00:00 [pmc-release] AID - jcm11185374 [pii] AID - jcm-11-05374 [pii] AID - 10.3390/jcm11185374 [doi] PST - epublish SO - J Clin Med. 2022 Sep 13;11(18):5374. doi: 10.3390/jcm11185374.