medigraphic.com
ISSN 2007-2783 (Print)
Órgano Oficial del Instituto Científico Pfizer

2015, Number 1

<< Back Next >>

Residente 2015; 10 (1)

Pulmonary arterial hypertension

Ordoñez-Vázquez AL, Montes-Castillo C, Mora-Soto JR, Sánchez-Hernández JD

Language: Spanish
References: 45
Page: 18-30
PDF size: 280.63 Kb.


ABSTRACT

Pulmonary arterial hypertension (PAH) is a complex condition with a poor prognosis. It is defined as a mean pulmonary arterial pressure greater than 25 mmHg and a pulmonary wedge pressure less than 15 mmHg. The prevalence worldwide is of 15 cases per million people; in Mexico the prevalence is unknown. The classification of the 4th Symposium of Pulmonary Arterial Hypertension held in Dana Point, California, is currently used. The idiopathic and familiar forms correspond to 40% of the etiology; the remaining 60% corresponds to patients with comorbidities. The multifactorial interaction generates endothelial dysfunction in pulmonary arteries of small caliber, altered vasodilator and vasoconstrictor substances, platelet aggregation tendency with risk of thrombosis in situ, vascular remodeling, and progressively increased pulmonary vascular resistance. As a result, right ventricular afterload increases, resulting in heart failure. In clinical suspicion of PAH, an echocardiography should be performed, and diagnosis must be done with right catheterization. Assessing the severity and determining the functional class of the World Heath Organization will determine its treatment.


REFERENCES

  1. Vanhoof JMM, Delcroix M, Vandevelde E, Denhaerynck K, Wuyts W, Belge C et al. Emotional symptoms and quality of life in patients with pulmonary arterial hypertension. J Heart Lung Transplant. 2014; 33: 800-808.

  2. Benza RL, Gomberg-Maitland M, Miller DP, Frost A, Frantz RP, Foreman AJ et al. The REVEAL registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012; 141: 354-362.

  3. Morales BJ, Carmona RA, Rosas RM. Hipertensión arterial pulmonar, una entidad rara. Rev Invest Clin. 2014; 66: 65-78.

  4. Swiston J, Johnson R, Granton J. Factors that prognosticate mortality in idiopathic pulmonary arterial hypertension: a systematic review of the literature. Respir Med. 2010; 104: 1588-1607.

  5. Bossone E, D’Andrea A, D’Alto M, Citro R, Argiento P, Ferrara F et al. Echocardiography in pulmonary arterial hypertension: from diagnosis to prognosis. J Am Soc Echocardiogr. 2013; 26: 1-14.

  6. Bolaños-Campos, I. Hipertensión arterial pulmonar, Guías de manejo. Rev Costarric Cardiol [online]. 2012, vol.14, n.1-2, pp. 26-54. ISSN 1409-4142.

  7. Integerafrica Research and Development. (2013-2014). RELAHP. Recuperado el 16 de diciembre de 2014. Disponible en: https://www.relahp.org

  8. Lai YC, Potoka KC, Champion HC, Mora AL, Gladwin MT. Pulmonary arterial hypertension: the clinical syndrome. Circ Res. 2014; 115: 115-130.

  9. Naval N. Clasificación actual de la hipertensión pulmonar. Insuf Card. 2011; 6: 30-38.

  10. Taichman BD, Mandel J. Epidemiology of pulmonary arterial hypertension. Clin Chest Med. 2013; 34: 619-637.

  11. Melero JM. Estado actual de la hipertensión arterial pulmonar. Insuf Card. 2009; 4: 23-26.

  12. Brown ML, Chen H, Halpern S. Delay in recognition of pulmonary arterial hypertension. Factors identified from the REVEAL Registry. Chest. 2011; 140: 19-26.

  13. Cogan JD, Pauciulo MW, Batchman AP, Prince MA, Robbins IM, Hedges LK et al. High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006; 174: 590-598.

  14. Aldred MA, Vijayakrishnan J, James V, Soubrier F, Gomez-Sanchez MA, Martensson G et al. BMPR2 gene rearrangements account for a significant proportion of mutations in familial and idiopathic pulmonary arterial hypertension. Hum Mutat. 2006; 27: 212-213.

  15. Machado RD, Aldred MA, James V, Harrison RE, Patel B, Schwalbe EC et al. Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat. 2006; 27: 121-132.

  16. Thomson JR, Machado RD, Pauciulo MW, Morgan NV, Humbert M, Elliott GC et al. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-b family. J Med Genet. 2000; 37: 741-745.

  17. Morrell NW, Yang X, Upton PD, Jourdan KB, Morgan N, Sheares KK et al. Altered growth responses of pulmonary artery smooth muscle cells from patients with primary pulmonary hypertension to transforming growth factor-beta1 and bone morphogenetic proteins. Circulation. 2001; 104: 790-795.

  18. Masri FA, Xu W, Comhair SA, Asosingh K, Koo M, Vasanji A et al. Hyperproliferative apoptosis-resistant endothelial cells in idiopathic pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol. 2007; 293: L548-554.

  19. Gambaryan N, Perros F, Montani D, Cohen-Kaminsky S, Mazmanian M, Renaud JF et al. Targeting of c-kit + haematopoietic progenitor cells prevents hypoxic pulmonary hypertension. Eur Respir. J. 2011; 37: 1392-1399.

  20. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004; 351: 1425-1436.

  21. O’Callaghan DS, Savale L, Montani D, Jaïs X, Sitbon O, Simonneau G et al. Treatment of pulmonary arterial hypertension with targeted therapies. Nat Rev Cardiol. 2011; 8: 526-538.

  22. Stenmark KR, Gerasimovskaya E, Nemenoff RA, Das M. Hypoxic activation of adventitial fibroblasts: role in vascular remodeling. Chest. 2002; 122: 326S-334S.

  23. Montani D, Perros F, Gambaryan N, Girerd B, Dorfmuller P, Price LC et al. C-Kit positive cells accumulate in remodeled vessels of idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. 2011; 184: 116-123.

  24. Gambaryan N, Cohen-Kaminsky S, Montani D, Girerd B, Huertas A, Seferian A et al. Circulating fibrocytes and pulmonary arterial hypertension. Eur Respir J. 2012; 39: 210-212.

  25. Kherbeck N, Tamby MC, Bussone G, Dib H, Perros F, Humbert M et al. The role of inflammation and autoimmunity in the pathophysiology of pulmonary arterial hypertension. Clin Rev Allergy Immunol. 2013; 44: 31-38.

  26. Perros F, Dorfmüller P, Montani D, Hammad H, Waelput W, Girerd B et al. Pulmonary lymphoid neogenesis in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. 2012; 185: 311-321.

  27. Montani D, Günther S, Dorfmüller P, Perros F, Girerd B, Garcia G et al. Pulmonary arterial hypertension. Orphanet J of Rare Dis. 2013; 8: 97.

  28. Forfia PR, Trow TK. Diagnosis of pulmonary arterial hypertension. Clin Chest Med. 2013; 34: 665-681.

  29. Humbert M, Gerry Coghlan J, Khanna D. Early detection and management of pulmonary arterial hypertension. Eur Respir Rev. 2012; 21: 306-312.

  30. Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009; 54: S43-S45.

  31. Escribano P, Barberà J, Suberviola V. Evaluación diagnóstica y pronóstica actual de la hipertensión Pulmonar. Rev Esp Cardiol. 2010; 63: 583-596.

  32. Guerra RFJ. Papel de la ecocardiografía ante la sospecha de hipertensión pulmonar. Arch Bronconeumol. 2011; 47: 7-11.

  33. Blanco I, Barbera JA. Diagnóstico clínico y hemodinámico de la hipertensión pulmonar tromboembólica crónica. Arch Bronconeumol. 2009; 45: 15-20.

  34. Amaro A. El estudio hemodinámico: indicaciones del cateterismo derecho e izquierdo en el diagnóstico y seguimiento de la hipertensión pulmonar. Arch Bronconeumol. 2011; 47: 12-14.

  35. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004; 351: 1425-1436.

  36. Mazzei JA, Cáneva JO, Perrone SV, Melero MJ, Scali JJ, Botman G. Actualización en el diagnóstico y tratamiento de la hipertensión pulmonar. Medicina (B Aires). 2011; 71: 1-48.

  37. Taichman DB, Ornelas J, Chung L, Klinger JR, Lewis S, Mandel J et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest. 2014; 146: 449-475.

  38. Ilarouranga A, Ibarra-Barrueta O, Oribe-Ibáñez M, Lertxundi EU, Santiago C, Iglesias-Lambarri A et al. Tratamiento de la hipertensión pulmonar. Farm Hosp (Madrid). 2004; 28: 275-285.

  39. Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet. 2001; 358: 1119-1123.

  40. Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002; 346: 896-903.

  41. Galiè N, Olschewski H, Oudiz RJ, Torres F, Frost A, Ghofrani HA et al. Ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo controlled, multicenter, efficacy studies (ARIES) Group. Circulation. 2008; 117: 3010-3019.

  42. Pulido T, Adzerikho I, Channick RN, Delcroix M, Galiè N, Ghofrani HA et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013; 369: 809-818.

  43. Pombo JM, Escribano SP, Tello MR, Gómez-Sánchez MA, Delgado JJ, González-Gallarza RD et al. Experiencia de 10 años en el tratamiento con epoprostenol en perfusión intravenosa continua en hipertensión pulmonar arterial grave. Rev Esp Cardiol. 2003; 56: 230-235.

  44. Hiremath J, Thanikachalam S, Parikh K, Shanmugasundaram S, Bangera S, Shapiro L et al. Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trial. J Heart Lung Transplant. 2010; 29: 137-149.

  45. Olschewski H, Simonneau G, Galiè N, Higenbottam T, Naeije R, Rubin LJ et al. Aerosolized Iloprost Randomized Study Group. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002; 347: 322-329.




2020     |     www.medigraphic.com