Experience of the regional program for the patients with pulmonary hypertension: one more step towards registry of patients
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Abstract
The aim – to analyze the results of the 3-month experience of the regional program developed to provide medical care for the patients with pulmonary hypertension (PH) in Lviv region (Ukraine).
Material and methods. Since December 2015 until April 2016 18 patients with idiopathic and associated forms of pulmonary arterial hypertension (PAH) (n=12, 66.7 %) and Eisenmenger syndrome (n=6, 33.3 %) who have been previously treated with generic sildenafil were enrolled into the regional program. Mean age of the patients was 36.1±11.9 years, female/male ratio was 3.5:1. Mean pulmonary artery pressure by right heart catheterization was 62±14 mm Hg and pulmonary vascular resistance 13.6±5.4 WU. The majority of patients (61.1 %) presented with the signs of functional class (FC) III while FC II was diagnosed in seven (38.9 %) cases. During participation in the program sildenafil monotherapy was prescribed to eight (44.4 %) patients, combination with iloprost to ten (55.6 %) participants. Treatment efficacy was monitored by the change in 6-minute walk test and parameters of pulmonary hemodynamics and right ventrivular function were assessed by echocardiography.
Results. At the time patients were included into the program the mean duration of disease for all participants was
59 (30; 124) months. Subjects with Eisenmenger syndrome had lower SaO2 and higher tricuspid valve pressure
gradient (TVPG), while PAH subjects presented with larger right atrium area and slightly larger right ventricle diameter and lower TAPSE. After three months of treatment self-reported improvement of symptoms was accompanied by increase in 6-minute walk test (from 374 to 392 m), decrease in heart rate (89±14 to 74±12 beats per minute) with mild increase in SaO2 (from 89.8±9.3 % to 93.8±4.5 %) in all patients. Echocardiographic evaluation demonstrated slight decrease in TVPG (from 71.1±17.9 to 69.9±17.9 mm Hg), which was accompanied by moderate decrease in the area of the right atrium (from 29.4±10.4 to 28.8±10.4 cm2) and increase in right ventrivular diameter, especially, in the PAH group.
Conclusions. Use of the original sildenafil or its combination with inhaled iloprost in prevalent PH patients with the history of irregular or generic treatment was accompanied by increase in tolerance to physical load but did not provide significant improvement of pulmonary hemodynamics or right ventricular performance assessed by echocardiography.
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Живило І.О., Радченко Г.Д., Сіренко Ю.М. Створення загальнодержавного реєстру хворих із легеневою артеріальною гіпертензією – вимога сучасності? // Укр. кардіол. журн.– 2016.– № 1.– С. 41–46.
Конопльова Л.Ф., Коваленко В.М., Амосова К.М. та ін. Діагностика та лікування легеневої гіпертензії. Рекомендації робочої групи з легеневих гіпертензій // Укр. кардіол. журн.– 2014.– Додаток № 3.– С. 2–39.
Легенева гіпертензія у дорослих: уніфікований клінічний протокол екстреної, первинної, вторинної (спеціалізованої) та третинної (високоспеціалізованої) медичної допомоги // Наказ Міністерства охорони здоров’я України 21.06.2016 р. № 614 http://www.dec.gov.ua/mtd/reestr.html.
Семен Х.О. Сучасні підходи до лікування легеневої артеріальної гіпертензії: акцент та початковому комбіновану лікуванні та корекції дисфункції правого шлуночка // Медицина транспорту України.– 2014.– № 4.– C. 64–70.
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.– Vol. 142 (2).– P. 448–456.
Bhatt A.В. Survival in Eisenmenger syndrome: a paradigm shift in outcomes research for adult congenital heart disease? // Heart.– 2014.– Vol. 100.– P. 1308–1310.
D’Alonzo G.E., Barst R.J., Ayres S.M. et al. Survival in patients with primary pulmonary hypertension // Ann. Intern. Medicine.– 1991.– Vol. 115 (5).– P. 343–349.
Dimopoulos K., Inuzuka R., Goletto S. et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension // Circulation.– 2010.– Vol. 121.– P. 20–25.
Del Pozo R., Hernandez Gonzalez I., Escribano-Subias P. The prostacyclin pathway in pulmonary arterial hypertension: a clinical review // Expert Rev. Respir. Med. – 2017.– Vol. 24.– P. 1–13.
Galiè N., Ghofrani H.A., Torbicki A. et al. Sildenafil citrate therapy for pulmonary arterial hypertension // New Engl. J. Med.– 2005.– Vol. 353 (20).– P. 2148–2117.
Galié N., Humbert M., Vachiery J.–L. 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. Respiratory J.– 2015.– Vol. 46 (4).– P. 903–975.
Galiè N., Barberà J.A., Frost A.E. et al. AMBITION Investigators. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension // New Engl. J. Med.– 2015.– Vol. 373 (9).– P. 834–844.
Gall H., Sommer N., Milger K. et al. Survival with sildenafil and inhaled iloprost in a cohort with pulmonary hypertension: an observational study // BMC Pulm. Med.– 2016.– Vol. 16 (5).– P. 1–9.
Garin M.C., Clark L., Chumney E.C. et al. Cost-utility of treatments for pulmonary arterial hypertension: a Markov state-transition decision analysis model // Clin. Drug Investig.– 2009.– Vol. 29 (10).– P. 635–646.
Gremeaux V., Troisgros O., Benaim S. et al. Determining the minimal clinically important difference for the six-minute walk test and the 200-meter fast-walk test during cardiac rehabilitation program in coronary artery disease patients after acute coronary syndrome // Arch. Phys. Med. Rehabil.– 2011.– Vol. 92 (4).– P. 611–618.
Humbert M., Sitbon O., Chaouat A. et al. Pulmonary arterial hypertension in France: results from a national registry // Amer. J. Respir. Crit. Care Med.– 2006.– Vol. 173 (9).– P. 1023–1030.
McGoon M.D., Benza R.L., Escribano-Subias P. et al. Pulmonary arterial hypertension: epidemiology and registries // J. Am. Coll. Cardiol.– 2013.– Vol. 62 (Suppl. 25).– P. D51–D59.
Olschewski H., Simonneau G., Galiè N. et al. Inhaled iloprost for severe pulmonary hypertension // New Engl. J. Med.– 2002.– Vol. 347 (5).– P. 322–329.
Peacock A.J., Murphy N.F., McMurray J.J. et al. An epidemiological study of pulmonary arterial hypertension // Eur. Respir. J.– 2007.– Vol. 30 (1).– P. 104–109.
Perera S., Mody S., Woodman R. et al. Meaningful change and responsiveness in common physical performance measures in older adults // J. Amer. Geriatrics Society.– 2006.– Vol. 54 (5).– P. 743–749
Redelmeier D., Bayoumi A., Goldstein R. et al. Interpreting small differences in functional status: the Six Minute Walk test in chronic lung disease patients // Amer. J. Respir. Critical Care Medicine.– 1997.– Vol. 155.– P. 1278–1282.
Rubin L.J., Badesch D.B., Fleming T.R. et al. SUPER-2 Study Group. Long-term treatment with sildenafil citrate in pulmonary arterial hypertension: the SUPER-2 study // Chest.– 2011.– Vol. 140 (5).– P. 1274–1283.
Semen K., Yelisyeyeva O., Jarocka-Karpowicz I. et al. Sildenafil reduces signs of oxidative stress in pulmonary arterial hypertension: evaluation by fatty acid composition, level of hydroxynonenal and heart rate variability // Redox Biol.– 2016.– Vol. 7.– P. 48–57.
Strange G., Gabbay E., Kermeen F. et al. Time from symptoms to definitive diagnosis of idiopathic pulmonary arterial hypertension: The delay study // Pulm. Circ.– 2013.– Vol. 3 (1).– P. 89–94.
Vonk-Noordegraaf A., Haddad F., Chin K.M. et al. Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology // J. Am. Coll. Cardiol.– 2013.– Vol. 62 (Suppl. 25).– P. D22–33.