References

  1. Home /
    2. What is pulmonary hypertension? / Diagnostics (scientific) /
  2. References

References

1. Atrial septostomy

The creation of this right-left shunt is indicated in cases of right heart failure and syncope despite maximum conservative therapy. The aim is to reduce the right ventricular end-diastolic pressure. This procedure may also be used as a bridging measure before other forms of treatment.

2. Pulmonary thrombendarterectomy (PEA)

PEA is the treatment of choice for patients with CTEPH. In this operation, the blood clot and some of the vascular media are dissected out of the pulmonary vessel. The indication is based on functional restriction, haemodynamics and location of the blood clots (more central or more peripheral). Whether thromboembolic lesions can be reached depends very much on the experience of the surgical team. In the ideal case, thrombotic material can be removed even from subsegmental vascular sections. The prerequisite for PEA is oral anticoagulation for at least 3 months. Perioperative mortality is reported as between 5 and 24% depending on the centre. With a 5-year survival rate of 75–80%, PEA is clearly superior to drug therapy and also to lung transplant and should therefore be undertaken in all patients who meet the criteria for surgery.

3. Lung transplant

If the condition of a patient does not improve despite the maximum drug therapy, lung transplant constitutes a further option. The 5-year survival rate for this procedure is about 45%.

Because of the complexity of both the disease and the treatment, patients with PAH should be very closely monitored. For patients in the early stages of the disease, being given oral therapy, six-monthly check-ups are recommended. Patients at an advanced stage of the disease, particularly those receiving parenteral or combined therapy, should be observed in specialist centres at three-monthly intervals.

References:

  1. Humbert, M., Sitbon, O., Chaouat, A., Bertocchi, M., Habib, G., Gressin, V., Yaici, A., Weitzenblum, E., Cordier, J.F., Chabot, F., et al. (2006). Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 173, 1023-1030.
  2. D’Alonzo, G.E., Barst, R.J., Ayres, S.M., Bergofsky, E.H., Brundage, B.H., Detre, K.M., Fishman, A.P., Goldring, R.M., Groves, B.M., Kernis, J.T., et al. (1991). Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 115, 343-349.
  3. Kovacs, G., Berghold, A., Scheidl, S., and Olschewski, H. (2009). Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 34, 888-894.
  4. Humbert, M., Sitbon, O., and Simonneau, G. (2004). Treatment of pulmonary arterial hypertension. N Engl J Med 351, 1425-1436.
  5. Trembath, R.C., Thomson, J.R., Machado, R.D., Morgan, N.V., Atkinson, C., Winship, I., Simonneau, G., Galie, N., Loyd, J.E., Humbert, M., et al. (2001). Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. N Engl J Med 345, 325-334.
  6. Machado, R.D., Aldred, M.A., James, V., Harrison, R.E., Patel, B., Schwalbe, E.C., Gruenig, E., Janssen, B., Koehler, R., Seeger, W., et al. (2006). Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat 27, 121-132.
  7. Chambers, C.D., Hernandez-Diaz, S., Van Marter, L.J., Werler, M.M., Louik, C., Jones, K.L., and Mitchell, A.A. (2006). Selective serotonin-reuptake inhibitors and risk of persistent pulmonary hypertension of the newborn. N Engl J Med 354, 579-587.
  8. Hachulla, E., Gressin, V., Guillevin, L., Carpentier, P., Diot, E., Sibilia, J., Kahan, A., Cabane, J., Frances, C., Launay, D., et al. (2005). Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 52, 3792-3800.
  9. de Groote, P., Gressin, V., Hachulla, E., Carpentier, P., Guillevin, L., Kahan, A., Cabane, J., Frances, C., Lamblin, N., Diot, E., et al. (2008). Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis. Ann Rheum Dis 67, 31-36.
  10. Krowka, M.J., Swanson, K.L., Frantz, R.P., McGoon, M.D., and Wiesner, R.H. (2006). Portopulmonary hypertension: Results from a 10-year screening algorithm. Hepatology 44, 1502-1510.
  11. Stauber, R.E., and Olschewski, H. Portopulmonary hypertension: short review. Eur J Gastroenterol Hepatol 22, 385-390.
  12. de Cleva, R., Herman, P., Pugliese, V., Zilberstein, B., Saad, W.A., Rodrigues, J.J., and Laudanna, A.A. (2003). Prevalence of pulmonary hypertension in patients with hepatosplenic Mansonic schistosomiasis–prospective study. Hepatogastroenterology 50, 2028-2030.
  13. Chaouat, A., Bugnet, A.S., Kadaoui, N., Schott, R., Enache, I., Ducolone, A., Ehrhart, M., Kessler, R., and Weitzenblum, E. (2005). Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 172, 189-194.
  14. Pengo, V., Lensing, A.W., Prins, M.H., Marchiori, A., Davidson, B.L., Tiozzo, F., Albanese, P., Biasiolo, A., Pegoraro, C., Iliceto, S., et al. (2004). Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 350, 2257-2264.
  15. Lang, I.M. (2004). Chronic thromboembolic pulmonary hypertension–not so rare after all. N Engl J Med 350, 2236-2238.
  16. Bonderman, D., Wilkens, H., Wakounig, S., Schafers, H.J., Jansa, P., Lindner, J., Simkova, I., Martischnig, A.M., Dudczak, J., Sadushi, R., et al. (2009). Risk factors for chronic thromboembolic pulmonary hypertension. Eur Respir J 33, 325-331.
  17. Bonderman, D., Turecek, P.L., Jakowitsch, J., Weltermann, A., Adlbrecht, C., Schneider, B., Kneussl, M., Rubin, L.J., Kyrle, P.A., Klepetko, W., et al. (2003). High prevalence of elevated clotting factor VIII in chronic thromboembolic pulmonary hypertension. Thromb Haemost 90, 372-376.
  18. Bonderman, D., Skoro-Sajer, N., Jakowitsch, J., Adlbrecht, C., Dunkler, D., Taghavi, S., Klepetko, W., Kneussl, M., and Lang, I.M. (2007). Predictors of outcome in chronic thromboembolic pulmonary hypertension. Circulation 115, 2153-2158.
  19. Wensel, R., Opitz, C.F., Anker, S.D., Winkler, J., Hoffken, G., Kleber, F.X., Sharma, R., Hummel, M., Hetzer, R., and Ewert, R. (2002). Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation 106, 319-324.
  20. Fijalkowska, A., Kurzyna, M., Torbicki, A., Szewczyk, G., Florczyk, M., Pruszczyk, P., and Szturmowicz, M. (2006). Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest 129, 1313-1321.
  21. Nagaya, N., Uematsu, M., Satoh, T., Kyotani, S., Sakamaki, F., Nakanishi, N., Yamagishi, M., Kunieda, T., and Miyatake, K. (1999). Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 160, 487-492.
  22. Torbicki, A., Kurzyna, M., Kuca, P., Fijalkowska, A., Sikora, J., Florczyk, M., Pruszczyk, P., Burakowski, J., and Wawrzynska, L. (2003). Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation 108, 844-848.
  23. Ahearn, G.S., Tapson, V.F., Rebeiz, A., and Greenfield, J.C., Jr. (2002). Electrocardiography to define clinical status in primary pulmonary hypertension and pulmonary arterial hypertension secondary to collagen vascular disease. Chest 122, 524-527.
  24. Fuster, V., Steele, P.M., Edwards, W.D., Gersh, B.J., McGoon, M.D., and Frye, R.L. (1984). Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 70, 580-587.
  25. Bonderman, D., Wexberg, P., Martischnig, A.M., Heinzl, H., Lang, M.B., Sadushi, R., Skoro-Sajer, N., and Lang, I.M. A non-invasive algorithm to exclude precapillary pulmonary hypertension. Eur Respir J.
  26. Moser, K.M. (1994). Diagnosing pulmonary embolism. BMJ 309, 1525-1526.
  27. Rich, S., Kaufmann, E., and Levy, P.S. (1992). The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 327, 76-81.
  28. Galie, N., Hoeper, M.M., Humbert, M., Torbicki, A., Vachiery, J.L., Barbera, J.A., Beghetti, M., Corris, P., Gaine, S., Gibbs, J.S., et al. (2009). Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30, 2493-2537.
  29. Rich, S., Seidlitz, M., Dodin, E., Osimani, D., Judd, D., Genthner, D., McLaughlin, V., and Francis, G. (1998). The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest 114, 787-792.
  30. Bogaard, H.J., Natarajan, R., Mizuno, S., Abbate, A., Chang, P.J., Chau, V.Q., Hoke, N.N., Kraskauskas, D., Kasper, M., Salloum, F.N., et al. Adrenergic receptor blockade reverses right heart remodeling and dysfunction in pulmonary hypertensive rats. Am J Respir Crit Care Med 182, 652-660.
  31. Delcroix, M., and Naeije, R. Optimising the management of pulmonary arterial hypertension patients: emergency treatments. Eur Respir Rev 19, 204-211.
  32. McLaughlin, V.V., Benza, R.L., Rubin, L.J., Channick, R.N., Voswinckel, R., Tapson, V.F., Robbins, I.M., Olschewski, H., Rubenfire, M., and Seeger, W. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol 55, 1915-1922.
  33. Olschewski, H., Simonneau, G., Galie, N., Higenbottam, T., Naeije, R., Rubin, L.J., Nikkho, S., Speich, R., Hoeper, M.M., Behr, J., et al. (2002). Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 347, 322-329.
  34. Galie, N., Beghetti, M., Gatzoulis, M.A., Granton, J., Berger, R.M., Lauer, A., Chiossi, E., and Landzberg, M. (2006). Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 114, 48-54.
  35. Sitbon, O., Gressin, V., Speich, R., Macdonald, P.S., Opravil, M., Cooper, D.A., Fourme, T., Humbert, M., Delfraissy, J.F., and Simonneau, G. (2004). Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 170, 1212-1217.
  36. Jais, X., D’Armini, A.M., Jansa, P., Torbicki, A., Delcroix, M., Ghofrani, H.A., Hoeper, M.M., Lang, I.M., Mayer, E., Pepke-Zaba, J., et al. (2008). Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol 52, 2127-2134.
  37. Barst, R.J., Langleben, D., Badesch, D., Frost, A., Lawrence, E.C., Shapiro, S., Naeije, R., and Galie, N. (2006). Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J Am Coll Cardiol 47, 2049-2056.
  38. Galie, N., Olschewski, H., Oudiz, R.J., Torres, F., Frost, A., Ghofrani, H.A., Badesch, D.B., McGoon, M.D., McLaughlin, V.V., Roecker, E.B., et al. (2008). Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 117, 3010-3019.
  39. Galie, N., Ghofrani, H.A., Torbicki, A., Barst, R.J., Rubin, L.J., Badesch, D., Fleming, T., Parpia, T., Burgess, G., Branzi, A., et al. (2005). Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 353, 2148-2157.
  40. Ghofrani, H.A., Voswinckel, R., Gall, H., Schermuly, R., Weissmann, N., Seeger, W., and Grimminger, F. Riociguat for pulmonary hypertension. Future Cardiol 6, 155-166.
  41. Galie, N., Rubin, L., Hoeper, M., Jansa, P., Al-Hiti, H., Meyer, G., Chiossi, E., Kusic-Pajic, A., and Simonneau, G. (2008). Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 371, 2093-2100.
  42. Hoeper, M.M., Barbera, J.A., Channick, R.N., Hassoun, P.M., Lang, I.M., Manes, A., Martinez, F.J., Naeije, R., Olschewski, H., Pepke-Zaba, J., et al. (2009). Diagnosis, assessment, and treatment of non-pulmonary arterial hypertension pulmonary hypertension. J Am Coll Cardiol 54, S85-96.

Correspondence: Prof. Dr. Irene Lang, Klinische Abteilung für Kardiologie, Universitätsklinik für Innere Medizin II, Medizinische Universität Wien, Währinger Gürtel 18–20, 1090 Wien, Austria, e-mail: irene.lang@meduniwien.ac.at