{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T06:43:47Z","timestamp":1762065827279,"version":"build-2065373602"},"reference-count":24,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2022,9,13]],"date-time":"2022-09-13T00:00:00Z","timestamp":1663027200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>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 &gt; II, 1.5% with syncope) and 122 patients with idiopathic PAH (median age of 9.0 years, 27.0% NYHA class &gt; 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\u201343.85, p &lt; 0.001), atrial blood gas pH &lt; 7.35 (OR = 12.504, 95%CI: 3.545\u201344.102, p &lt; 0.001) and RAP &gt; 14 mmHg (OR = 10.636, 95%CI: 3.668\u201330.847, p &lt; 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.<\/jats:p>","DOI":"10.3390\/jcm11185374","type":"journal-article","created":{"date-parts":[[2022,9,13]],"date-time":"2022-09-13T22:37:28Z","timestamp":1663108648000},"page":"5374","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Right Heart Catheterization in Pediatric Pulmonary Arterial Hypertension: Insights and Outcome from a Large Tertiary Center"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2529-1943","authenticated-orcid":false,"given":"Zhuoyuan","family":"Xu","sequence":"first","affiliation":[{"name":"Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China"},{"name":"Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hongsheng","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China"},{"name":"Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3180-7280","authenticated-orcid":false,"given":"Alexandra","family":"Arvanitaki","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, AHEPA University Hospital, 54621 Thessaloniki, Greece"},{"name":"Pulmonary Hypertension Service, Royal Brompton Hospital, London SW3 6NP, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chen","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China"},{"name":"Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qiangqiang","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China"},{"name":"Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5476-6050","authenticated-orcid":false,"given":"Bradley B.","family":"Keller","sequence":"additional","affiliation":[{"name":"Greater Louisville and Western Kentucky Practice, Cincinnati Children\u2019s Heart Institute, Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hong","family":"Gu","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China"},{"name":"Beijing Institute of Heart Lung and Blood Vessel Diseases, Beijing 100029, China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,9,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/j.recesp.2016.01.002","article-title":"2015 ESC\/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension","volume":"69","author":"Galie","year":"2016","journal-title":"Rev. Esp. Cardiol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2551","DOI":"10.1016\/j.jacc.2017.03.575","article-title":"Pulmonary Hypertension in Infants, Children, and Young Adults","volume":"69","author":"Hansmann","year":"2017","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1801916","DOI":"10.1183\/13993003.01916-2018","article-title":"Paediatric pulmonary arterial hypertension: Updates on definition, classification, diagnostics and management","volume":"53","author":"Rosenzweig","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Kubiak, G.M., Ciarka, A., Biniecka, M., and Ceranowicz, P. (2019). Right Heart Catheterization-Background, Physiological Basics, and Clinical Implications. J. Clin. Med., 8.","DOI":"10.3390\/jcm8091331"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1800738","DOI":"10.1183\/13993003.00738-2018","article-title":"Right heart catheterisation is still a fundamental part of the follow-up assessment of pulmonary arterial hypertension","volume":"52","author":"Humbert","year":"2018","journal-title":"Eur. Respir. J."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2034","DOI":"10.1093\/eurheartj\/ehx034","article-title":"Pulmonary arterial hypertension in congenital heart disease: Translational opportunities to study the reversibility of pulmonary vascular disease","volume":"38","author":"Bartelds","year":"2017","journal-title":"Eur. Heart J."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/j.healun.2016.07.017","article-title":"Incidence of right heart catheterization in patients initiated on pulmonary arterial hypertension therapies: A population-based study","volume":"36","author":"Duarte","year":"2017","journal-title":"J. Heart Lung Transplant."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"2546","DOI":"10.1016\/j.jacc.2006.07.061","article-title":"Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers","volume":"48","author":"Hoeper","year":"2006","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1093\/bja\/aem059","article-title":"Risk of cardiac catheterization under anaesthesia in children with pulmonary hypertension","volume":"98","author":"Taylor","year":"2007","journal-title":"Br. J. Anaesth."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1111\/ped.13161","article-title":"Cardiac catheterization in children with pulmonary arterial hypertension","volume":"59","author":"Matsuura","year":"2017","journal-title":"Pediatr. Int."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"280","DOI":"10.4103\/2045-8932.83445","article-title":"Functional classification of pulmonary hypertension in children: Report from the PVRI pediatric taskforce, Panama 2011","volume":"1","author":"Lammers","year":"2011","journal-title":"Pulm. Circ."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"ii86","DOI":"10.1136\/heartjnl-2015-309132","article-title":"Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK","volume":"102","author":"Hansmann","year":"2016","journal-title":"Heart"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"3105","DOI":"10.1161\/CIRCULATIONAHA.104.488486","article-title":"Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension","volume":"111","author":"Sitbon","year":"2005","journal-title":"Circulation"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1016\/j.jacc.2015.07.032","article-title":"Predictors of Catastrophic Adverse Outcomes in Children With Pulmonary Hypertension Undergoing Cardiac Catheterization: A Multi-Institutional Analysis From the Pediatric Health Information Systems Database","volume":"66","author":"Glatz","year":"2015","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"2159","DOI":"10.1016\/j.jacc.2014.02.575","article-title":"Survival differences in pediatric pulmonary arterial hypertension: Clues to a better understanding of outcome and optimal treatment strategies","volume":"63","author":"Zijlstra","year":"2014","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1378\/chest.10-2327","article-title":"Survival of Chinese patients with pulmonary arterial hypertension in the modern treatment era","volume":"140","author":"Zhang","year":"2011","journal-title":"Chest"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1183\/09031936.00056110","article-title":"Pulmonary arterial hypertension: A comparison between children and adults","volume":"37","author":"Barst","year":"2011","journal-title":"Eur. Respir. J."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1326","DOI":"10.1161\/hc3601.095274","article-title":"Pulmonary and systemic arterial pressure changes during syncope in primary pulmonary hypertension","volume":"104","author":"Mikhail","year":"2001","journal-title":"Circulation"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1097\/MJT.0b013e3181f94c02","article-title":"Pulmonary hypertension: A review of pathophysiology and anesthetic management","volume":"19","author":"Salehi","year":"2012","journal-title":"Am. J. Ther."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e105","DOI":"10.1093\/ejcts\/ezu445","article-title":"Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: Updated data from the first 24 patients","volume":"47","author":"Baruteau","year":"2015","journal-title":"Eur. J. Cardiothorac. Surg."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.chest.2019.03.003","article-title":"Use of Balloon Atrial Septostomy in Patients With Advanced Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis","volume":"156","author":"Khan","year":"2019","journal-title":"Chest"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1097\/CRD.0000000000000270","article-title":"Right Heart Catheterization-Related Complications: A Review of the Literature and Best Practices","volume":"28","author":"Chen","year":"2020","journal-title":"Cardiol. Rev."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1097\/PCC.0000000000000724","article-title":"Pediatric Pulmonary Hypertensive Crisis Medications: A Stepping Stone for the Future","volume":"17","author":"Cunningham","year":"2016","journal-title":"Pediatr. Crit. Care Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1007\/s00246-020-02535-4","article-title":"Risk Factors for Adverse Events in Children with Pulmonary Hypertension Undergoing Cardiac Catheterization","volume":"42","author":"Vaiyani","year":"2021","journal-title":"Pediatr. Cardiol."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/18\/5374\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:30:42Z","timestamp":1760142642000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/18\/5374"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9,13]]},"references-count":24,"journal-issue":{"issue":"18","published-online":{"date-parts":[[2022,9]]}},"alternative-id":["jcm11185374"],"URL":"https:\/\/doi.org\/10.3390\/jcm11185374","relation":{},"ISSN":["2077-0383"],"issn-type":[{"type":"electronic","value":"2077-0383"}],"subject":[],"published":{"date-parts":[[2022,9,13]]}}}