DOI: https://doi.org/10.22141/2224-1485.3-4.64-65.2019.177848

Prognosis and its predictors for pulmonary hypertension associated with connective tissue diseases (data of the first Ukrainian register)

Yu.M. Sirenko, G.D. Radchenko, I.O. Zhyvylo, E.Yu. Titov, S.I. Deyak

Abstract


Background. This study is the first analysis of patient survival after treatment in the only Ukrainian pulmonary hypertension (PH) referral center that was organized in 2014 and mainly focused on pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). The aim of the study was: to evaluate the structure of patients who were treated in referral center; to evaluate Ukrainian reality in surviving of patients with PAH and CTEPH (we did not have any Ukrainian data before); to determine predictors of death. Materials and methods. Tree hundred and fifty-nine patients with PH were included in database: with idiopathic PAH — 72 (20.1 %), PAH associated with connective tissue diseases (CTD) — 20 (5.6 %), PAH associated with HIV — 8 (2.2 %), PAH associated with portal hypertension — 4 (1.1 %), PAH associated with congenital heart diseases (CHD) — 171 (47.6 %), PH due to left heart diseases — 8 (2.2 %), PH associated with lung diseases — 6 (1.7 %), CTEPH — 62 (17.3 %), with multifactorial PH — 8 (2.2 %). Two hundred and eighty-one patients were enrolled (52 (18.5 %) with CTEPH, 229 (81.5 %) with PAH) in survival analysis. Long-term survival (Kaplan-Meier) and its predictors (Stepwise binary logistic regression and Cox’s proportional hazards analyses) were evaluated in adult patients with PH (diagnosed by right heart catheterization) within a prospective registry at a single referral center in Kyiv, Ukraine. Follow-up period was up to 51 (mean 23.6 ± 14.1) months. Only two specific drugs are available in Ukraine for the treatment of PH: inhaled iloprost and sildenafil. Results. The Kaplan-Meier survival rate for the total cohort was 93.3, 86.8, and 81.5 % at 1, 2 and 3 years, respectively. Survival was better in patients with CHD as compared to idiopathic PAH (log-rank P = 0.002), CTD (log-rank P = 0.001) and CTEPH (log-rank P = 0.04). Univariate Cox’s predictors of death were: functional class IV (odds ratio (OR) = 4.94; 95% confidence interval (CI) 2.12–11.48), pre-sence of ascites (OR = 4.52; 95% CI 2.21–9.24), CTD etiology of PAH (OR = 3.07; 95% CI 1.07–8.87), CHD etiology of PAH (OR = 0.28; 95% CI 0.11–0.68), heart rate during treatment > 105 bpm (OR = 7.85; 95% CI 1.83–33.69), office systolic blood pressure < 100 mmHg (OR = 2.78; 95% CI 1.26–6.1), 6-minute walk test during treatment < 340 m (OR = 3.47; 95% CI 1.01–12.35), NT-proBNP > 300 pg/ml (OR = 4.98; 95% CI 1.49–16.6), right atrial area > 22 cm2 (OR = 14.2; 95% CI 1.92–104.89), right ventricular area in diastole (OR = 1.08; 95% CI 1.03–1.14), right ventricular area in systole (OR = 1.08; 95% CI 1.02–1.11), mean pressure in right atrium per each 1-mmHg increase (OR = 1.02; 95% CI 1.02–1.19). In multivariate Cox regression analysis, only presence of ascites, office systolic BP < 100 mmHg, CHD etiology of PAH, NT-proBNP > 300 pg/ml were associated with survival. Conclusions. This is the first Ukrainian single-center PH cohort study results. It was shown that the structure of PAH patients in Ukraine was different from that in other countries. However, our data of survival are almost similar to data of centers from other countries. The worst survival was observed in PAH associated with CTD as compared to all other its forms: survival was only 49.7 % over 3 years. The simple indicators were revealed that can be evaluated in routine practice and are independent predictors of death: the presence of ascites and elevated levels of NT-proBNP.


Keywords


pulmonary hypertension associated with connective tissue diseases; register of patients with pulmonary hypertension; survival; predictors of poor prognosis

References


Galie N., Humbert M., Vachiery J.L., Gibbs S., Lang I. et al.; ESC Scientific Document Group. 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.

Gabbay E., Yeow W., Playford D. Pulmonary arterial hypertension (PAH) is an uncommon cause of pulmonary hypertension (PH) in an unselected population: the Armadale echocardiography study. Am. J. Resp. Crit. Care Med. 2007. 175. A713.

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(9). 1023-1030.

Peacock A.J., Murphy N.F., McMurray J.J.V., Caballero L., Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur. Respir. J. 2007. 30(1). 104-109.

Condliffe R., Howard L.S. Connective tissue disease-associated pulmonary arterial hypertension. F1000 Prime Reports. 2015. 7. 06 (doi:10.12703/P7-06).

D’Alonzo G.E., Barst R.J., Ayres S.M. et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann. Intern. Med. 1991. 115. 343-349.

Rich S., Dantzker D.R., Ayres S.M. et al. Primary pulmonary hypertension. A national prospective study. Ann. Intern. Med. 1987. 107. 216-223.

Benza R.L., Miller D.P., Gomberg-Maitland M. et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010. 122. 164-172.

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. 156-163.

Raymond R.J., Hinderliter A.L., Willis P.W. et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J. Am. Coll. Cardiol. 2002 Apr 3. 39(7). 1214-1219.

Appelbaum L., Yigla M., Bendayan D. et al. Primary pulmonary hypertension in Israel: a national survey. Chest. 2001. 119. 1801-1806.

Miyamoto S., Nagaya N., Satoh T. et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise testing. Am. J. Respir. Crit. Care Med. 2000. 161. 487-492.

Sandoval J., Bauerle O., Palomar A. et al. Survival in primary pulmonary hypertension. Validation of a prognostic equation. Circulation. 1994. 89. 1733-1744.

Benza R., Lohmueller L., Kraisangka J., Kanwar M. Risk Assessment in Pulmonary Arterial Hypertension Patients: The Long and Short of it. Advances in Pulmonary Hypertension. 2018. 16. 125-135.

Markley R.R., Ali A., Potfay J., Paulsen W., Jovin I. Echocardiographic Evaluation of the Right Heart. J. Cardiovasc. Ultrasound. 2016 Sep. 24(3). 183-190.

Manes A., Palazzini M., Leci E. et al. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur. Heart J. 2014. 35(11). 716-24.

Benza R.L., Miller D.P., Barst R.J. et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL registry. Chest. 2012. 142(2). 448-456.

ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association Developed in Collaboration With the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J. ACC. 2009. 53(17). 1573-1619. doi: 10.1016/j.jacc.2009.01.004.

Marques-Alvesa P., Baptista R., Marinho da Silva A. еt al. Real-world, long-term survival of incident patients with pulmonary arterial hypertension. Rev. Port. Pneumol. 2017. 23(3). 124-131.

Zhang R., Dai L.-Z., Xie W.-P. et al. Survival of Chinese patients with pulmonary arterial hypertension in the modern treatment era. Chest. 2011. 140. 301-309.

Humbert M., Sitbon O., Yaïci A. et al.; French Pulmonary Arterial Hypertension Network. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur. Respir. J. 2010. 36. 549-555.

Peacock A.J., Murphy N.F., McMurray J.J.V. at al. An epidemiological study of pulmonary arterial hypertension. Eur. Respir. J. 2007. 30. 104-109.

Gall H., Felix J., Schneck F. еt al. The Giessen Pulmonary Hypertension Registry: Survival in pulmonary hypertension subgroups. J. Heart Lung Transplant. 2017. 36. 957-967.

Jansa P., Jarkovsky J., Al-Hiti H. et al. Epidemiology and long-term survival of pulmonary arterial hypertension in the Czech Republic: a retrospective analysis of a nationwide registry. BMC Pulm. Med. 2014. 14. 45.

Hopkins W.E. The remarkable right ventricle of patients with Eisenmenger syndrome. Coron. Artery Dis. 2005. 16. 19-25.

Thenappan T., Shah S.J., Rich S. еt al. Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur. Respir. J. 2010. 35. 1079-1087.

Launay D., Sitbon O., Hachulla E. et al. Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era. Ann. Rheum. Dis. 2013. 72. 1940-1946.

Yap L.B., Ashrafian H., Mukerjee D. et al. The natriuretic peptides and their role in disorders of right heart dysfunction and pulmonary hypertension. Clin. Biochem. 2004. 37. 847-856.

Andreassen A.K., Wergeland R., Simonsen S. et al.

N-terminal Pro-B-type natriuretic peptideas an indicator of dise-ase severity in a heterogeneous group of patients with chronic precapillary pulmonary hypertension. Am. J. Cardiol. 2006. 98. 525-529.

Park M.H., Scott R.L., Uber P.A. et al. Usefulness of B-type natriuretic peptide as a predictor of treatment outcome in pulmonary arterial hypertension. Congest. Heart Fail. 2004. 10. 221-225.

Galie N., Landzberg M., Beghetti M., Berger R.M., Efficace M., Gesang S., Papadakis K., Gatzoulis M.A. Evaluation of macitentan in patients with Eisenmenger syndrome: results from the randomised controlled MAESTRO study. P5462. European Heart Journal. 2017. 38(Suppl. 1). 1162-11631.

Austin C., Alassas K., Burger C. et al. Echocardiographic assessment of estimated right atrial pressure and size predicts mortality in pulmonary arterial hypertension. Chest. 2015. 147(1). 198-208.

Vijarnsorn C., Durongpisitkul K., Chungsomprasong P., Bositthipichet D., Ketsara S., Titaram Y., Chanthong P., Kanjanauthai S., Soongswang J. In Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts. PLoS ONE. 2018. 13(4). e0195092.

Mueller-Mottet S., Stricker H., Domeninghetti G. еt al. Long-Term Data from the Swiss Pulmonary Hypertension Registry. Respiration. 2015. 89. 127-140.




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