INVESTIGATION OF CENTRAL HEMODYNAMICS VIA RIGHT HEART AND PULMONARY ARTERY CATHETERIZATION IN PATIENTS WITH SYSTEMIC CONNECTIVE TISSUE DISEASES

Pulmonary arterial hypertension (PAH) associated with systemic connective tissue diseases (SCTD) is a poor prognostic manifestation of the latter that result in death if untreated. The invasive determination of hemodynamic parameters is prominent in diagnosing the disease and determining its treatment policy and prognosis.

Objective: to analyze the results of catheterization in PAH-SCTD patients admitted to the V.A. Nasonova Research Institute of Rheumatology.

Subjects and methods. The investigation included 59 patients admitted to the V.A. Nasonova Research Institute of Rheumatology from September 2009 to September 2014. PAH was diagnosed in accordance with the conventional guidelines. All the patients underwent right heart and pulmonary artery (PA) catheterization at the diagnosis and over time during treatment.

Results and discussion. All the patients included in the trial met the pre-capillary pulmonary hypertension (PH) criteria: mean pulmonary artery pressure (MPAP) ≥25 mm Hg; and PA wedge pressure (PAWP) <15 mm Hg. The exclusion of other causes of PH (pulmonary fibrosis, left heart disease, and thromboembolism), as well as a high transpulmonary pressure gradient >15 mm Hg and pulmonary vascular resistance (PVR) >3 Wood units could diagnose PAH in all our patients. There was a statistically highly significant association between pathological hemodynamic changes and functional class (FC). FC was found to be most closely correlated with right atrial pressure (RAP), cardiac output (CO), PVR, and cardiac index (CI). Among the most common manifestations of heart failure, only the presence of peripheral edemas was associated with worse hemodynamic parameters in PAH. It should be noted that out of two biomarkers (N-terminal pro-brain natriuretic peptide and uric acid), the former is largely related to the magnitude of changes in hemodynamic factors. The critical values of hemodynamic parameters were due to extreme edema – anasarca (RAP >17 mm Hg, PVR >20 Wood units, CI <14 ml/m2). Analysis of clinical and instrumental parameters in relation to FC revealed a linear relationship of RAP, CO, and PVR between the level of these parameters and FC; moreover, the highest correlation coefficients were observed for the hemodynamic parameters characterizing right ventricular systolic function. It is remarkable that no MPAP changes were found; only patients with FC IV showed its slight increase (from 51±8 to 55±8 mm Hg; р = 0.087). PAWP remained unchanged regardless of FC.

Conclusion. Thus, the hemodynamic parameters determined in patients with PAH-SCTD during right heart and LA catheterization are closely related to the manifestations of respiratory and heart failure, the biomarkers, and FC of PAH.

1. Чазова ИЕ, Мартынюк ТВ, редакторы. Легочная гипертензия. Москва: Практика; 2015 [Chazova IE, Martynyuk TV, editors. Legochnaya gipertenziya [Pulmonary hypertension]. Moscow: Praktika; 2015].

2. Волков АВ. Легочная артериальная гипертензия при системных заболеваниях соединительной ткани. Научно-практическая ревматология. 2015;53(1):69–77 [Volkov AV. Pulmonary arterial hypertension in connective tissue diseases. Nauchno-prakticheskaya revmatologiya =Rheumatology Science and Practice. 2015;53(1):69–77 (In Russ.)]. doi: 10.14412/1995-4484-2015-69-77

3. Волков АВ, Мартынюк ТВ, Юдкина НН и др. Выживаемость пациентов с легочной артериальной гипертензией, ассоциированной с системной склеродермией. Терапевтический архив. 2012;(5):24–8 [Volkov AV, Martynyuk TV, Yudkina NN, et al. The survival rate of patients with pulmonary arterial hypertension associated with systemic scleroderma. Terapevticheskii arkhiv. 2012;(5):24–8 (In Russ.)].

4. Koh ET, Lee P, Gladman DD, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol. 1996;35:989–93. doi: 10.1093/rheumatology/35.10.989

5. Fagan KA, Badesch DB. Pulmonary hypertension associated with connective tissue disease. Prog Cardiovasc Dis. 2002;45:225–34. doi: 10.1053/pcad.2002.129975

6. Yang X, Mardekian K, Sanders KN, et al. Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature. Clin Rheumatol. 2013;32:1519–31. doi: 10.1007/s10067-013-2307-2

7. 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;72:1940–6. doi: 10.1136/annrheumdis-2012-202489

8. Simonneau G, Galie N, Jansa P, et al. Long-term results from the EARLY study of bosentan in WHO functional class II pulmonary arterial hypertension patients. Int J Cardiol. 2014 Mar 15;172(2):332–9. doi: 10.1016/j.ijcard.2013.12.179

9. Humbert M, Yaici A, de Groote P, et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum. 2011 Nov;63(11):3522–30. doi: 10.1002/art.30541

10. Campo A, Mathai SC, Le Pavec J, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. 2010 Jul 15;182(2):252–60. doi: 10.1164/rccm.200912-1820OC

11. Berger M, Haimowitz A, van Tosh A, et al. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol. 1985;6:359–65. doi: 10.1016/S0735-1097(85)80172-8

12. D’Alto M, Romeo E, Argiento P, et al. Accuracy and precision of echocardiography versus right heart catheterization for the assessment of pulmonary hypertension. Int J Cardiol. 2013;168:4058–62. doi: 10.1016/j.ijcard.2013.07.005

13. Farber HW, Foreman AJ, Miller DP, McGoon MD. REVEAL Registry: correlation of right heart catheterization and echocardiography in patients with pulmonary arterial hypertension. Congest Heart Fail. 2011;17:56–64. doi: 10.1111/j.1751-7133.2010.00202.x

14. Mukerjee D, St George D, Knight C, et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford). 2004;43:461–6. doi: 10.1093/rheumatology/keh067

15. Galie N, Hoeper MM, Humbert M, et al. 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. 2009;30:2493–537. doi: 10.1093/eurheartj/ehp297

16. McLaughlin VV, Archer SL, Badesch DB, et al. 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. Circulation. 2009;119:2250–94. doi: 10.1161/CIRCULATIONAHA. 109.192230

17. Чазова ИЕ, Авдеев СН, Царева НА и др. Клинические рекомендации по диагностике и лечению легочной гипертонии. Терапевтический архив. 2014;(9):4–23 [Chazova IE, Avdeev SN, Tsareva NA, et al. Clinical guidelines for diagnosis and treatment of pulmonary hypertension. Terapevticheskii arkhiv. 2014;(9):4–23 (In Russ.)].

18. Schwaiger JP, Khanna D, Coghlan GJ. Screening patients with scleroderma for pulmonary arterial hypertension and implications for other at-risk populations. Eur Respir Rev. 2013;22:515–25. doi: 10.1183/09059180.00006013

19. Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005;52:3792–800. doi: 10.1002/art.21433

20. Coghlan JC, Denton CP, Grü nig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73:1340–9. doi: 10.1136/annrheumdis-2013-203301

21. Kawut SM, Taichman DB, Archer-Chicko CL, et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest. 2003;123:344–50. doi: 10.1378/chest.123.2.344

22. Benza RL, Miller DP, 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 Jul 13;122(2):164–72. doi: 10.1161/CIRCULATIONAHA. 109.898122

23. Mauritz GJ, Rizopoulos D, Groepenhoff H, et al. Usefulness of serial N-terminal pro-B-type natriuretic peptide measurements for determining prognosis in patients with pulmonary arterial hypertension. Am J Cardiol. 2011 Dec 1;108(11):1645–50. doi: 10.1016/j.amjcard.2011.07.025

24. McLaughlin VV, Presberg KW, Doyle R, et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:78–92. doi: 10.1378/chest.126.1_suppl.78S