Ten years of experience from a reference center in pulmonary arterial hypertension in Uruguay
DOI:
https://doi.org/10.29193/RMU.35.3.3Keywords:
PULMONARY HYPERTENSION, RISK ASSESSMENT, OUTCOME AND PROCESS ASSESSMENTAbstract
Introduction: Pulmonary Arterial Hypertension (PAH) is a clinical condition that leads to failure in the right ventricle and death. The epidemiology of patients with pulmonary hypertension was characterized in a reference center in Uruguay.
Method: 52 patients with a diagnose of invasive pulmonary hypertension (January 2006-December 2016) were included in the study. Risk was estimated by means of a four variable model (functional class, functional capacity, distance covered in six minutes, right atrium pressure and cardiac output). Values of 1, 2 and 3 were allocated (low, medium and high risk respectively) to each variable (ESC/ERS 2015 PAH guidelines). Risk was categorized by rounding the numbers to the closest integer value of the average of the addition of values allocated for each variable.
Results: age ranged 46±2 years old, 85% of patients were women. Idiopathic hypertension, PAH associated to congenital heart disease (PAH-CHD) and PAH associated to connective tissue diseases (PAH-CTD) prevailed. Patients with PAH-CHD evidenced the greatest distance covered in 6 minutes and the lowest proportion of FC III/IV (p <0.05).
Survival was lowet in the PAH-CTD (p =0.069). Mortality after one year was 0%, 6% and 20% for patients with low (n=17), intermediate (n=28) and high (n=7) risk, respectively, regardless of age, sex and subgroup of PAH. 51% of patients with intermediate and high risk received combined treatment.
Conclusions: the characteristics and survival of patients with PAH of a reference center in Uruguay were analysed in the study. The risk model allowed the discrimination of the patients’ mortality. 51% of patients with intermediate and high risk received combined treatment.
References
(1) Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37:67-119.
(2) Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013; 62:D34-41.
(3) Lau EMT, Giannoulatou E, Celermajer DS, Humbert M. Epidemiology and treatment of pulmonary arterial hypertension. Nat Rev Cardiol 2017; 14:603-14.
(4) Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 2013; 62:D42-50.
(5) Jiang X, Jing ZC. Epidemiology of pulmonary arterial hypertension. Curr Hypertens Rep 2013; 15:638-49.
(6) McGoon MD, Benza RL, Escribano-Subias P, Jiang X, Miller DP, Peacock AJ, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 2013; 62:D51-9.
(7) D’Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115:343-9.
(8) Hoeper MM, Humbert M, Souza R, Idrees M, Kawut SM, Sliwa-Hahnle K, et al. A global view of pulmonary hypertension. Lancet Respir Med 2016; 4:306-22.
(9) Valverde AB, Soares JM, Viana KP, Gomes B, Soares C, Souza R. Pulmonary arterial hypertension in Latin America: epidemiological data from local studies. BMC Pulm Med 2018; 18:106.
(10) Alves JL, Jr., Gavilanes F, Jardim C, Fernandes C, Morinaga LTK, Dias B, et al. Pulmonary arterial hypertension in the southern hemisphere: results from a registry of incident Brazilian cases. Chest 2015; 147:495-501.
(11) Grignola JC, Salisbury JP, Pascal G, Trujillo P, Parma G, Curbelo P. Tratamiento específico de la hipertensión arterial pulmonar: experiencia de la policlínica de hipertensión pulmonar del Hospital Maciel, período 2009-2011. Rev Urug Cardiol 2012; 27:132-42.
(12) Sitbon O, Humbert M, Jaïs X, Ioos V, Hamid AM, Provencher S, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111:3105-11.
(13) Hoeper MM, Kramer T, Pan Z, Eichstaedt CA, Spiesshoefer J, Benjamin N, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J 2017; 50(2) doi: 10.1183/13993003.00740-2017.
(14) Boucly A, Weatherald J, Savale L, Jais X, Cottin V, Prevot G, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J 2017; 50(2) doi: 10.1183/13993003.00889-2017.
(15) Pocock SJ, Clayton TC, Altman DG. Survival plots of time-to-event outcomes in clinical trials: good practice and pitfalls. Lancet 2002; 359:1686-9.
(16) Lajoie AC, Bonnet S, Provencher S. Combination therapy in pulmonary arterial hypertension: recent accomplishments and future challenges. Pulm Circ 2017; 7:312-25.
(17) Zhang R, Dai LZ, Xie WP, Yu ZX, Wu BX, Pan L, et al. Survival of Chinese patients with pulmonary arterial hypertension in the modern treatment era. Chest 2011; 140:301-9.
(18) Berra G, Noble S, Soccal PM, Beghetti M, Lador F. Pulmonary hypertension in the elderly: a different disease? Breathe (Sheff) 2016; 12:43-9.
(19) Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173:1023-30.
(20) Ling Y, Johnson MK, Kiely DG, Condliffe R, Elliot CA, Gibbs JS, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med 2012; 186:790-6.
(21) Brida M, Gatzoulis MA. Pulmonary arterial hypertension in adult congenital heart disease. Heart 2018; 104:1568-74.
(22) Hascoet S, Baruteau AE, Humbert M, Simonneau G, Jais X, Petit J, et al. Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome. J Heart Lung Transplant 2017; 36:386-98.
(23) Weatherald J, Boucly A, Sitbon O. Risk stratification in pulmonary arterial hypertension. Curr Opin Pulm Med 2018; 24:407-15.
(24) Kylhammar D, Kjellstrom B, Hjalmarsson C, Jansson K, Nisell M, Soderberg S, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J 2018; 39:4175-81.
(25) Raina A, Humbert M. Risk assessment in pulmonary arterial hypertension. Eur Respir Rev 2016; 25:390-8.
(26) Galiè N, Channick RN, Frantz RP, Grunig E, Jing ZC, Moiseeva O, et al. Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J 2018; 53(1) doi: 10.1183/13993003.01889-2018.
(27) Fondo Nacional de Recursos. Política y gestión de la cobertura de medicamentos de alto costo. Montevideo: FNR, 2010. (Publicación técnica, 13).
(28) Fondo Nacional de Recursos. Tratamiento de la hipertensión arterial pulmonar con bosentan e iloprost. Montevideo: FNR, 2010.
