Los criterios actuales y las estrategias de abordaje | 05 FEB 17
Perspectiva general de la hipertensión pulmonar
La hipertensión pulmonar es un importante tema sanitario, que afecta a todos los grupos etarios, con importancia creciente en los ancianos. Se asocia con deterioro clínico y aumento considerable del riesgo de mortalidad.
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Referencias
1 Hoeper MM, Bogaard HJ, Condliff e R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 2013; 62 (25 suppl): D42–50.
2 Galie N, Humbert M, Vachiery JL, 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.
3 Moreira EM, Gall H, Leening MJ, et al. Prevalence of pulmonary hypertension in the general population: the Rotterdam study. PLoS One 2015; 10: e0130072.
4 Tuder RM, Archer SL, Dorfmuller P, et al. Relevant issues in the pathology and pathobiology of pulmonary hypertension. J Am Coll Cardiol 2013; 62 (25 suppl): D4–12.
5 Humbert M, Sitbon O, Yaici A, et al, and the French Pulmonary Arterial Hypertension Network. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 2010; 36: 549–55.
6 Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107: 216–23.
7 D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115: 343–49.
8 Thenappan T, Shah SJ, Rich S, Gomberg-Maitland M. A USA-based registry for pulmonary arterial hypertension: 1982–2006. Eur Respir J 2007; 30: 1103–10.
9 Kawut SM, Horn EM, Berekashvili KK, et al. New predictors of outcome in idiopathic pulmonary arterial hypertension. Am J Cardiol 2005; 95: 199–203.
10 Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007; 30: 104–09.
11 Abenhaim L, Moride Y, Brenot F, et al, and the International Primary Pulmonary Hypertension Study Group. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. N Engl J Med 1996; 335: 609–16.
12 Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173: 1023–30.
13 Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010; 122: 156–63.
14 Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 2010; 137: 376–87.
15 Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest 2012; 142: 448–56.
16 McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 2013; 62 (25 suppl): D51–59.
17 Frost AE, Badesch DB, Barst RJ, et al. The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL diff ers from historic and non-US Contemporary Registries. Chest 2011; 139: 128–37.
18 Escribano-Subias P, Blanco I, Lopez-Meseguer M, et al, and the REHAP investigators. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J 2012; 40: 596–603.
19 Ling Y, Johnson MK, Kiely DG, 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: 790–96.
20 Korsholm K, Andersen A, Kirkfeldt RE, Hansen KN, Mellemkjaer S, Nielsen-Kudsk JE. Survival in an incident cohort of patients with pulmonary arterial hypertension in Denmark. Pulm Circ 2015; 5: 364–69.
21 Hoeper MM, Huscher D, Pittrow D. Incidence and prevalence of pulmonary arterial hypertension in Germany. Int J Cardiol 2016; 203: 612–13.
22 Health and Social Care Information Centre. Fifth annual report: key fi ndings from the National Audit of Pulmonary Hypertension of the UK, Channel Islands, Gibraltar and Isle of Man. Report for the audit period April 2013–March 2014.
23 Jing ZC, Xu XQ, Han ZY, et al. Registry and survival study in Chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest 2007; 132: 373–79.
24 Zhang R, Dai LZ, Xie WP, et al. Survival of Chinese patients with pulmonary arterial hypertension in the modern treatment era. Chest 2011; 140: 301–09.
25 Alves JL Jr, Gavilanes F, Jardim C, et al. Pulmonary arterial hypertension in the southern hemisphere: results from a registry of incident Brazilian cases. Chest 2015; 147: 495–501.
26 Idrees M, Alnajashi K, Abdulhameed J, et al, and the Registry Taskforce SAPH. Saudi experience in the management of pulmonary arterial hypertension; the outcome of PAH therapy with the exclusion of chronic parenteral prostacyclin. Ann Thorac Med 2015;10: 204–11.
27 Soubrier F, Chung WK, Machado R, et al. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol 2013;62 (25 suppl): D13–21.
28 Girerd B, Montani D, Eyries M, et al. Absence of infl uence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension. Respir Res 2010; 11: 73.
29 Ventetuolo CE, Praestgaard A, Palevsky HI, Klinger JR, Halpern SD, Kawut SM. Sex and haemodynamics in pulmonary arterial hypertension. Eur Respir J 2014; 43: 523–30.
30 Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol 2013;168: 871–80.
31 Benza RL, Gomberg-Maitland M, Naeije R, Arneson CP, Lang IM. Prognostic factors associated with increased survival in patients with pulmonary arterial hypertension treated with subcutaneous treprostinil in randomized, placebo-controlled trials. J Heart Lung Transplant 2011; 30: 982–89.
32 Idrees M, Al-Najashi K, Khan A, et al, and the SAPH Registry Taskforce. Pulmonary arterial hypertension in Saudi Arabia: Patients’ clinical and physiological characteristics and hemodynamic parameters. A single center experience. Ann Thorac Med 2014; 9: 209–15.
33 Olsson KM, Delcroix M, Ghofrani HA, et al. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation 2014; 129: 57–65.
34 Hao YJ, Jiang X, Zhou W, et al. Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients. Eur Respir J 2014; 44: 963–72.
35 Coghlan JG, Denton CP, Grunig E, et al, and the DETECT study group. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014;73: 1340–49.
36 Al-Harbi A, Abdullah K, Al-Abdulkareem A, Alghamdi A, Al-Jahdali H. Prevalence of portopulmonary hypertension among liver transplant candidates in a region highly endemic for viral hepatitis. Ann Transplant 2014; 19: 1–5.
37 Krowka MJ, Swanson KL, Frantz RP, McGoon MD, Wiesner RH. Portopulmonary hypertension: Results from a 10-year screening algorithm. Hepatology 2006; 44: 1502–10.
38 Kawut SM, Krowka MJ, Trotter JF, et al, and the Pulmonary Vascular Complications of Liver Disease Study Group. Clinical risk factors for portopulmonary hypertension. Hepatology 2008;48: 196–203.
39 van der Linde D, Konings EE, Slager MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol 2011; 58: 2241–47.
40 Marelli AJ, Ionescu-Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation 2014; 130: 749–56.
41 Duff els MG, Engelfriet PM, Berger RM, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 2007; 120: 198–204.
42 Lowe BS, Therrien J, Ionescu-Ittu R, Pilote L, Martucci G, Marelli AJ. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol 2011; 58: 538–46.
43 Verheugt CL, Uiterwaal CS, van der Velde ET, et al. Mortality in adult congenital heart disease. Eur Heart J 2010; 31: 1220–29.
44 Ross AG, Bartley PB, Sleigh AC, et al. Schistosomiasis. N Engl J Med 2002; 346: 1212–20.
45 Chitsulo L, Engels D, Montresor A, Savioli L. The global status of schistosomiasis and its control. Acta Trop 2000; 77: 41–51.
46 Colley DG, Bustinduy AL, Secor WE, King CH. Human schistosomiasis. Lancet 2014; 383: 2253–64.
47 Lapa M, Dias B, Jardim C, et al. Cardiopulmonary manifestations of hepatosplenic schistosomiasis. Circulation 2009; 119: 1518–23.
48 Papamatheakis DG, Mocumbi AO, Kim NH, Mandel J. Schistosomiasis-associated pulmonary hypertension. Pulm Circ 2014; 4: 596–611.
49 dos Santos Fernandes CJ, Jardim CV, Hovnanian A, et al. Survival in schistosomiasis-associated pulmonary arterial hypertension. J Am Coll Cardiol 2010; 56: 715–20.
50 de Cleva R, Herman P, Pugliese V, et al. Prevalence of pulmonary hypertension in patients with hepatosplenic mansonic schistosomiasis—prospective study. Hepatogastroenterology 2003;50: 2028–30.
51 Bertrand E, Dalger J, Ramiara JP, Renambot J, Attia Y. Bilharzial pulmonary arterial hypertension. Clinical and hemodynamic study in 37 patients. Arch Mal Coeur Vaiss 1978;71: 216–21 (in French).
52 Watt G, Long GW, Calubaquib C, Ranoa CP. Cardiopulmonary involvement rare in severe Schistosoma japonicum infection. Trop Geogr Med 1986; 38: 233–39.
53 Speich R, Jenni R, Opravil M, Pfab M, Russi EW. Primary pulmonary hypertension in HIV infection. Chest 1991; 100: 1268–71.
54 Sitbon O, Lascoux-Combe C, Delfraissy JF, et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med 2008; 177: 108–13.
55 Murray CJ, Ortblad KF, Guinovart C, et al. Global, regional, and national incidence and mortality for HIV, tuberculosis, and malaria during 1990–2013: a systematic analysis for the Global Burden of Disease Study 2013. Lancet 2014; 384: 1005–70.
56 Global Burden of Disease Study 2013 Collaborators. Global, regional, and national incidence, prevalence, and years lived with disability for 301 acute and chronic diseases and injuries in 188 countries, 1990–2013: a systematic analysis for the Global
Burden of Disease Study 2013. Lancet 2015; 386: 743–800.
57 Lloyd-Jones DM, Larson MG, Leip EP, et al, and the Framingham Heart Study. Lifetime risk for developing congestive heart failure: the Framingham Heart Study. Circulation 2002; 106: 3068–72.
58 Bursi F, Weston SA, Redfi eld MM, et al. Systolic and diastolic heart failure in the community. JAMA 2006; 296: 2209–16.
59 Lam CS, Roger VL, Rodeheff er RJ, Borlaug BA, Enders FT, Redfi eld MM. Pulmonary hypertension in heart failure with preserved ejection fraction: a community-based study. J Am Coll Cardiol 2009; 53: 1119–26.
60 Kjaergaard J, Akkan D, Iversen KK, et al. Prognostic importance of pulmonary hypertension in patients with heart failure. Am J Cardiol 2007; 99: 1146–50.
61 Bui AL, Horwich TB, Fonarow GC. Epidemiology and risk profile of heart failure. Nat Rev Cardiol 2011; 8: 30–41.
62 Roger VL, Weston SA, Redfi eld MM, et al. Trends in heart failure incidence and survival in a community-based population. JAMA 2004; 292: 344–50.
63 Owan TE, Hodge DO, Herges RM, Jacobsen SJ, Roger VL, Redfi eld MM. Trends in prevalence and outcome of heart failure with preserved ejection fraction. N Engl J Med 2006; 355: 251–59.
64 Bhatia RS, Tu JV, Lee DS, et al. Outcome of heart failure with preserved ejection fraction in a population-based study. N Engl J Med 2006; 355: 260–69.
65 Borlaug BA, Redfi eld MM. Diastolic and systolic heart failure are distinct phenotypes within the heart failure spectrum. Circulation 2011; 123: 2006–14.
66 Galie N, Humbert M, Vachiery JL, 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 Respir J 2015; 46: 903–75.
67 Ghio S, Gavazzi A, Campana C, et al. Independent and additive prognostic value of right ventricular systolic function and pulmonary artery pressure in patients with chronic heart failure. J Am Coll Cardiol 2001; 37: 183–88.
68 Grigioni F, Potena L, Galie N, et al. Prognostic implications of serial assessments of pulmonary hypertension in severe chronic heart failure. J Heart Lung Transplant 2006; 25: 1241–46.
69 Miller WL, Grill DE, Borlaug BA. Clinical features, hemodynamics, and outcomes of pulmonary hypertension due to chronic heart failure with reduced ejection fraction: pulmonary hypertension and heart failure. JACC Heart Fail 2013; 1: 290–99.
70 Gerges C, Gerges M, Lang MB, et al. Diastolic pulmonary vascular pressure gradient: a predictor of prognosis in “out-of-proportion” pulmonary hypertension. Chest 2013; 143: 758–66.
71 Bursi F, McNallan SM, Redfi eld MM, et al. Pulmonary pressures and death in heart failure: a community study. J Am Coll Cardiol 2012; 59: 222–31.
72 Barbash IM, Escarcega RO, Minha S, et al. Prevalence and impact of pulmonary hypertension on patients with aortic stenosis who underwent transcatheter aortic valve replacement. Am J Cardiol 2015; 115: 1435–42.
73 Cam A, Goel SS, Agarwal S, et al. Prognostic implications of pulmonary hypertension in patients with severe aortic stenosis. J Thorac Cardiovasc Surg 2011; 142: 800–08.
74 Roselli EE, Abdel Azim A, Houghtaling PL, Jaber WA, Blackstone EH. Pulmonary hypertension is associated with worse early and late outcomes after aortic valve replacement: implications for transcatheter aortic valve replacement. J Thorac Cardiovasc Surg 2012; 144: 1067–74.e2.
75 Ben-Dor I, Goldstein SA, Pichard AD, et al. Clinical profile, prognostic implication, and response to treatment of pulmonary hypertension in patients with severe aortic stenosis. Am J Cardiol 2011; 107: 1046–51.
76 O’Sullivan CJ, Wenaweser P, Ceylan O, et al. Eff ect of pulmonary hypertension hemodynamic presentation on clinical outcomes in patients with severe symptomatic aortic valve stenosis undergoing transcatheter aortic valve implantation: insights from the new proposed pulmonary hypertension classification. Circ Cardiovasc Interv 2015; 8: e002358.
77 Mohammed SF, Hussain I, AbouEzzeddine OF, et al. Right ventricular function in heart failure with preserved ejection fraction: a community-based study. Circulation 2014; 130: 2310–20.
78 Sakata Y, Shimokawa H. Epidemiology of heart failure in Asia. Circ J 2013; 77: 2209–17.
79 Bocchi EA, Braga FG, Ferreira SM, et al, and the Sociedasde Brasileira de Cardiologia. III Brazilian Guidelines on Chronic Heart Failure. Arq Bras Cardiol 2009; 93 (suppl 1): 3–70 (in Portuguese).
80 Makubi A, Hage C, Lwakatare J, et al. Contemporary aetiology, clinical characteristics and prognosis of adults with heart failure observed in a tertiary hospital in Tanzania: the prospective Tanzania Heart Failure (TaHeF) study. Heart 2014; 100: 1235–41.
81 Ogah OS, Sliwa K, Akinyemi JO, Falase AO, Stewart S. Hypertensive heart failure in Nigerian Africans: insights from the Abeokuta Heart Failure Registry. J Clin Hypertens (Greenwich) 2015; 17: 263–72.
82 Damasceno A, Mayosi BM, Sani M, et al. The causes, treatment, and outcome of acute heart failure in 1006 Africans from 9 countries. Arch Intern Med 2012; 172: 1386–94.
83 Stewart S, Wilkinson D, Hansen C, et al. Predominance of heart failure in the Heart of Soweto Study cohort: emerging challenges for urban African communities. Circulation 2008; 118: 2360–67.
84 Bloomfi eld GS, Barasa FA, Doll JA, Velazquez EJ. Heart failure in sub-Saharan Africa. Curr Cardiol Rev 2013; 9: 157–73.
85 Ntusi NB, Mayosi BM. Epidemiology of heart failure in sub-Saharan Africa. Expert Rev Cardiovasc Ther 2009; 7: 169–80.
86 Stewart S, Mocumbi AO, Carrington MJ, Pretorius S, Burton R, Sliwa K. A not-so-rare form of heart failure in urban black Africans: pathways to right heart failure in the Heart of Soweto Study cohort. Eur J Heart Fail 2011; 13: 1070–77.
87 Sliwa K, Davison BA, Mayosi BM, et al. Readmission and death after an acute heart failure event: predictors and outcomes in sub-Saharan Africa: results from the THESUS-HF registry. Eur Hear
1 Hoeper MM, Bogaard HJ, Condliff e R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 2013; 62 (25 suppl): D42–50.
2 Galie N, Humbert M, Vachiery JL, 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.
3 Moreira EM, Gall H, Leening MJ, et al. Prevalence of pulmonary hypertension in the general population: the Rotterdam study. PLoS One 2015; 10: e0130072.
4 Tuder RM, Archer SL, Dorfmuller P, et al. Relevant issues in the pathology and pathobiology of pulmonary hypertension. J Am Coll Cardiol 2013; 62 (25 suppl): D4–12.
5 Humbert M, Sitbon O, Yaici A, et al, and the French Pulmonary Arterial Hypertension Network. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 2010; 36: 549–55.
6 Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107: 216–23.
7 D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115: 343–49.
8 Thenappan T, Shah SJ, Rich S, Gomberg-Maitland M. A USA-based registry for pulmonary arterial hypertension: 1982–2006. Eur Respir J 2007; 30: 1103–10.
9 Kawut SM, Horn EM, Berekashvili KK, et al. New predictors of outcome in idiopathic pulmonary arterial hypertension. Am J Cardiol 2005; 95: 199–203.
10 Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007; 30: 104–09.
11 Abenhaim L, Moride Y, Brenot F, et al, and the International Primary Pulmonary Hypertension Study Group. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. N Engl J Med 1996; 335: 609–16.
12 Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173: 1023–30.
13 Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010; 122: 156–63.
14 Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 2010; 137: 376–87.
15 Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest 2012; 142: 448–56.
16 McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 2013; 62 (25 suppl): D51–59.
17 Frost AE, Badesch DB, Barst RJ, et al. The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL diff ers from historic and non-US Contemporary Registries. Chest 2011; 139: 128–37.
18 Escribano-Subias P, Blanco I, Lopez-Meseguer M, et al, and the REHAP investigators. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J 2012; 40: 596–603.
19 Ling Y, Johnson MK, Kiely DG, 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: 790–96.
20 Korsholm K, Andersen A, Kirkfeldt RE, Hansen KN, Mellemkjaer S, Nielsen-Kudsk JE. Survival in an incident cohort of patients with pulmonary arterial hypertension in Denmark. Pulm Circ 2015; 5: 364–69.
21 Hoeper MM, Huscher D, Pittrow D. Incidence and prevalence of pulmonary arterial hypertension in Germany. Int J Cardiol 2016; 203: 612–13.
22 Health and Social Care Information Centre. Fifth annual report: key fi ndings from the National Audit of Pulmonary Hypertension of the UK, Channel Islands, Gibraltar and Isle of Man. Report for the audit period April 2013–March 2014.
23 Jing ZC, Xu XQ, Han ZY, et al. Registry and survival study in Chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest 2007; 132: 373–79.
24 Zhang R, Dai LZ, Xie WP, et al. Survival of Chinese patients with pulmonary arterial hypertension in the modern treatment era. Chest 2011; 140: 301–09.
25 Alves JL Jr, Gavilanes F, Jardim C, et al. Pulmonary arterial hypertension in the southern hemisphere: results from a registry of incident Brazilian cases. Chest 2015; 147: 495–501.
26 Idrees M, Alnajashi K, Abdulhameed J, et al, and the Registry Taskforce SAPH. Saudi experience in the management of pulmonary arterial hypertension; the outcome of PAH therapy with the exclusion of chronic parenteral prostacyclin. Ann Thorac Med 2015;10: 204–11.
27 Soubrier F, Chung WK, Machado R, et al. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol 2013;62 (25 suppl): D13–21.
28 Girerd B, Montani D, Eyries M, et al. Absence of infl uence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension. Respir Res 2010; 11: 73.
29 Ventetuolo CE, Praestgaard A, Palevsky HI, Klinger JR, Halpern SD, Kawut SM. Sex and haemodynamics in pulmonary arterial hypertension. Eur Respir J 2014; 43: 523–30.
30 Hoeper MM, Huscher D, Ghofrani HA, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol 2013;168: 871–80.
31 Benza RL, Gomberg-Maitland M, Naeije R, Arneson CP, Lang IM. Prognostic factors associated with increased survival in patients with pulmonary arterial hypertension treated with subcutaneous treprostinil in randomized, placebo-controlled trials. J Heart Lung Transplant 2011; 30: 982–89.
32 Idrees M, Al-Najashi K, Khan A, et al, and the SAPH Registry Taskforce. Pulmonary arterial hypertension in Saudi Arabia: Patients’ clinical and physiological characteristics and hemodynamic parameters. A single center experience. Ann Thorac Med 2014; 9: 209–15.
33 Olsson KM, Delcroix M, Ghofrani HA, et al. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation 2014; 129: 57–65.
34 Hao YJ, Jiang X, Zhou W, et al. Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients. Eur Respir J 2014; 44: 963–72.
35 Coghlan JG, Denton CP, Grunig E, et al, and the DETECT study group. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis 2014;73: 1340–49.
36 Al-Harbi A, Abdullah K, Al-Abdulkareem A, Alghamdi A, Al-Jahdali H. Prevalence of portopulmonary hypertension among liver transplant candidates in a region highly endemic for viral hepatitis. Ann Transplant 2014; 19: 1–5.
37 Krowka MJ, Swanson KL, Frantz RP, McGoon MD, Wiesner RH. Portopulmonary hypertension: Results from a 10-year screening algorithm. Hepatology 2006; 44: 1502–10.
38 Kawut SM, Krowka MJ, Trotter JF, et al, and the Pulmonary Vascular Complications of Liver Disease Study Group. Clinical risk factors for portopulmonary hypertension. Hepatology 2008;48: 196–203.
39 van der Linde D, Konings EE, Slager MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol 2011; 58: 2241–47.
40 Marelli AJ, Ionescu-Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation 2014; 130: 749–56.
41 Duff els MG, Engelfriet PM, Berger RM, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 2007; 120: 198–204.
42 Lowe BS, Therrien J, Ionescu-Ittu R, Pilote L, Martucci G, Marelli AJ. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol 2011; 58: 538–46.
43 Verheugt CL, Uiterwaal CS, van der Velde ET, et al. Mortality in adult congenital heart disease. Eur Heart J 2010; 31: 1220–29.
44 Ross AG, Bartley PB, Sleigh AC, et al. Schistosomiasis. N Engl J Med 2002; 346: 1212–20.
45 Chitsulo L, Engels D, Montresor A, Savioli L. The global status of schistosomiasis and its control. Acta Trop 2000; 77: 41–51.
46 Colley DG, Bustinduy AL, Secor WE, King CH. Human schistosomiasis. Lancet 2014; 383: 2253–64.
47 Lapa M, Dias B, Jardim C, et al. Cardiopulmonary manifestations of hepatosplenic schistosomiasis. Circulation 2009; 119: 1518–23.
48 Papamatheakis DG, Mocumbi AO, Kim NH, Mandel J. Schistosomiasis-associated pulmonary hypertension. Pulm Circ 2014; 4: 596–611.
49 dos Santos Fernandes CJ, Jardim CV, Hovnanian A, et al. Survival in schistosomiasis-associated pulmonary arterial hypertension. J Am Coll Cardiol 2010; 56: 715–20.
50 de Cleva R, Herman P, Pugliese V, et al. Prevalence of pulmonary hypertension in patients with hepatosplenic mansonic schistosomiasis—prospective study. Hepatogastroenterology 2003;50: 2028–30.
51 Bertrand E, Dalger J, Ramiara JP, Renambot J, Attia Y. Bilharzial pulmonary arterial hypertension. Clinical and hemodynamic study in 37 patients. Arch Mal Coeur Vaiss 1978;71: 216–21 (in French).
52 Watt G, Long GW, Calubaquib C, Ranoa CP. Cardiopulmonary involvement rare in severe Schistosoma japonicum infection. Trop Geogr Med 1986; 38: 233–39.
53 Speich R, Jenni R, Opravil M, Pfab M, Russi EW. Primary pulmonary hypertension in HIV infection. Chest 1991; 100: 1268–71.
54 Sitbon O, Lascoux-Combe C, Delfraissy JF, et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med 2008; 177: 108–13.
55 Murray CJ, Ortblad KF, Guinovart C, et al. Global, regional, and national incidence and mortality for HIV, tuberculosis, and malaria during 1990–2013: a systematic analysis for the Global Burden of Disease Study 2013. Lancet 2014; 384: 1005–70.
56 Global Burden of Disease Study 2013 Collaborators. Global, regional, and national incidence, prevalence, and years lived with disability for 301 acute and chronic diseases and injuries in 188 countries, 1990–2013: a systematic analysis for the Global
Burden of Disease Study 2013. Lancet 2015; 386: 743–800.
57 Lloyd-Jones DM, Larson MG, Leip EP, et al, and the Framingham Heart Study. Lifetime risk for developing congestive heart failure: the Framingham Heart Study. Circulation 2002; 106: 3068–72.
58 Bursi F, Weston SA, Redfi eld MM, et al. Systolic and diastolic heart failure in the community. JAMA 2006; 296: 2209–16.
59 Lam CS, Roger VL, Rodeheff er RJ, Borlaug BA, Enders FT, Redfi eld MM. Pulmonary hypertension in heart failure with preserved ejection fraction: a community-based study. J Am Coll Cardiol 2009; 53: 1119–26.
60 Kjaergaard J, Akkan D, Iversen KK, et al. Prognostic importance of pulmonary hypertension in patients with heart failure. Am J Cardiol 2007; 99: 1146–50.
61 Bui AL, Horwich TB, Fonarow GC. Epidemiology and risk profile of heart failure. Nat Rev Cardiol 2011; 8: 30–41.
62 Roger VL, Weston SA, Redfi eld MM, et al. Trends in heart failure incidence and survival in a community-based population. JAMA 2004; 292: 344–50.
63 Owan TE, Hodge DO, Herges RM, Jacobsen SJ, Roger VL, Redfi eld MM. Trends in prevalence and outcome of heart failure with preserved ejection fraction. N Engl J Med 2006; 355: 251–59.
64 Bhatia RS, Tu JV, Lee DS, et al. Outcome of heart failure with preserved ejection fraction in a population-based study. N Engl J Med 2006; 355: 260–69.
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