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Article* "Development of the European Network in Orphan Cardiovascular Diseases" „Rozszerzenie Europejskiej Sieci Współpracy ds Sierocych Chorób Kardiologicznych” Title:Perioperative management of patients with pulmonary arterial hypertension RCD code: II.1.A Author: Grzegorz Kopeć Affiliation: Department of Cardiac and Vascular Diseases, Centre for Rare Cardiovascular Diseases, John Paul II Hospital, Krakow, Poland Date:2014.06.09 [* The article should be written in English John Paul II Hospital in Kraków Jagiellonian University, Institute of Cardiology 80 Prądnicka Str., 31-202 Kraków; tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88 e-mail: [email protected] www.crcd.eu Pulmonary arterial hypertension is a disease of low prevalence, and therefore rarely seen in surgical practice. The factors that increase the risk of postoperative complications include right ventricular failure, myocardial ischemia and postoperative hypoxia. In patients undergoing cardiopulmonary bypass mean pulmonary artery pressure before surgery > 30 mmHg is an independent risk factor of death. The European Society of Cardiology in 2014 issued new recommendations for the diagnosis and treatment of cardiac patients undergoing non-cardiac surgery. They referred also to patients with pulmonary arterial hypertension. In patients with pulmonary hypertension undergoing noncardiac surgery complications are predicted by functional class III or IV, right ventricular dysfunction, prolonged anesthesia and intermediate or high risk surgery. The incidence of perioperative cardiopulmonary complications reaches 38% and mortality of 7%. Therefore, if possible, it is advised to avoid elective surgical interventions. After the diagnosis of pulmonary arterial hypertension has been made a patient should be transferred to the center of reference in order to start treatment with drugs specific for pulmonary arterial hypertension. If a decision is made about surgery pharmacological treatment should be optimized and preparation for surgery should be discussed by a multidisciplinary team of experts. In the perioperative period the specific treatment should not be stopped. Sometimes it is necessary to switch the patient to the drugs used in nebulization or intravenously. Because most complications occur in the early hours after surgery, it is necessary to closely monitor patients for at least 24 hours after surgery. In the case of progression of heart failure diuretic treatment should be optimized and, if necessary, dobutamine infusion started. In refractory cases, it is recommended to include vasodilators by inhalation or intravenous injection under the supervision of a physician experienced in the treatment of pulmonary arterial hypertension. References 1. 1. Authors/Task Force Members, Kristensen SD, Knuuti J, Saraste A, Anker S, Bøtker HE, De Hert S, Ford I, Gonzalez-Juanatey JR, Gorenek B, Heyndrickx GR, Hoeft A, Huber K, Iung B, Kjeldsen KP, Longrois D, Lüscher TF, Pierard L, Pocock S, Price S, Roffi M, Sirnes PA, Sousa-Uva M, Voudris V, Funck-Brentano C; ESC Committee for Practice Guidelines, Zamorano JL, Achenbach S, Baumgartner H, Bax JJ, Bueno H, Dean V, Deaton C, Erol C, John Paul II Hospital in Kraków Jagiellonian University, Institute of Cardiology 80 Prądnicka Str., 31-202 Kraków; tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88 e-mail: [email protected] www.crcd.eu Fagard R, Ferrari R, Hasdai D, Hoes AW, Kirchhof P, Knuuti J, Kolh P, Lancellotti P, Linhart A, Nihoyannopoulos P, Piepoli MF, Ponikowski P, Sirnes PA, Tamargo JL, Tendera M, Torbicki A, Wijns W, Windecker S; ESA Clinical Guidelines Committee, Solca M, Brichant JF, De Hert S, de Robertis E, Longrois D, Langenecker SK, Wichelewski J; Document Reviewers, Piepoli MF, Wijns W, Agewall S, Ceconi C, Coca A, Corrà U, De Caterina R, Di Mario C, Edvardsen T, Fagard R, Germano G, Guarracino F, Hoes A, Joergensen T, Jüni P, Marques-Vidal P, Mueller C, Oto O, Pibarot P, Ponikowski P, Sellevold OF, Triposkiadis F, Windecker S, Wouters P. 2014 ESC/ESA Guidelines on non-cardiac surgery: cardiovascular assessment and management: The Joint Task Force on non-cardiac surgery: cardiovascular assessment and management of the European Society of Cardiology (ESC) and the European Society of Anaesthesiology (ESA). Eur Heart J. 2014 Aug 1. pii: ehu282. ……………………………………….. Author’s signature** John Paul II Hospital in Kraków Jagiellonian University, Institute of Cardiology 80 Prądnicka Str., 31-202 Kraków; tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88 e-mail: [email protected] www.crcd.eu [** Signing the article will mean an agreement for its publication] John Paul II Hospital in Kraków Jagiellonian University, Institute of Cardiology 80 Prądnicka Str., 31-202 Kraków; tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88 e-mail: [email protected] www.crcd.eu