Pulmonary vascular resistance/systemic vascular resistance (PVR/SVR) ratio changes after sildenafil therapy in uncorrected congenital heart disease-associated pulmonary arterial hypertension


Evita Devi Noor Rahmawati(1), Putrika Prastuti Ratna Gharini(2), Anggoro Budi Hartopo(3), Lucia Kris Dinarti(4), Dyah Wulan Anggrahini(5*)

(1) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(2) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(3) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(4) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(5) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(*) Corresponding Author


Pulmonary vascular resistance (PVR) to systemic vascular resistance (SVR) ratio is a prognostic predictor in congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) after defect correction. Sildenafil, widely used as a PAH drug, can decrease PVR with minimal or without changes in SVR, resulting in decreased PVR/SVR ratio after treatment. However, there is limited evidence that PVR/SVR ratio reduced after sildenafil therapy in uncorrected CHD-associated PAH patients. This study aimed to investigate the decreasing of the PVR/SVR ratio after ≥ 1-year oral sildenafil therapy in adult uncorrected CHD-associated PAH. A total of 30 uncorrectable CHD-associated PAH subjects derived from the COHARD-PH registry were included in this study. Right heart catheterization (RHC) was performed during the first visit and further evaluations were conducted after ≥1-year oral sildenafil therapy. The PVR/SVR ratio at the baseline and after the evaluation was collected. The primary outcome of this study was the changes in PVR/SVR ratio from baseline to evaluated RHC. Characteristic analysis of subjects with decreased PVR or PVR/SVR ratio was perforemd as the secondary outcome. The mean PVR and SVR were not different from baseline and evaluated RHC (15.98± 10.67 vs. 18.38±13.93 WU, p=0.206 and 36.65±13.99 vs. 39.34±15.46 WU, p=0.262). There was no significant difference in the baseline PVR/SVR ratio and the evaluated PVR/SVR ratio after ≥1-year sildenafil therapy (0.48 ±0.32 vs. 0.49±0.36; p=0.882). As much as 15 subjects (50%) experienced decreased PVR/SVR ratio. However, there was no significant difference in the characteristics, including age, Eisenmenger syndrome, type of shunts, baseline PVR, PAH-specific treatment, and baseline NT-proBNP level (p>0.05). In conclusion, sildenafil therapy does not change PVR/SVR ratio in adults with uncorrected CHD-associated PAH.


congenital heart disease; pulmonary arterial hypertension; pulmonary vascular resistance; sildenafil; systemic vascular resistance

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1.Galiè N, Chairperson ESC, Humbert M, Chairperson ERS, Vachiery J, Gibbs S, 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 Respir J 2015; 46(4):903-75.
2.McLaughlin VV, Davis M, Cornwell W. Pulmonary arterial hypertension. Curr Probl Cardiol 2011; 36(12):461-517.
3.Dinarti LK, Hartopo AB, Kusuma AD, Satwiko MG, Hadwiono MR, Pradana AD, et al. The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry: a descriptive study from single-center hospital registry of adult congenital heart disease and pulmonary hypertension in Indonesia. BMC Cardiovasc Disord 2020; 20(1):163.
4.Schwerzmann M, Pfammatter JP. Approaching atrial septal defects in pulmonary hypertension. Expert Rev Cardiovasc Ther 2015; 13(6):693-701.
5.Myers PO, Tissot C, Beghetti M. Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease. Circ J 2014; 78(1):4-11.
6.Palma G, Giordano R, Russolillo V, Cioffi S, Palumbo S, Mucerino M, et al. Sildenafil therapy for pulmonary hypertension before and after pediatric congenital heart surgery. Texas Hear Inst J 2011; 38(3):238-42.
7.Galie N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353(20):2148-57.
8.Galie N, McLaughlin VV, Rubin LJ, Simonneau G. An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J 2019; 53(1):1802148.
9.Garg N, Sharma MK, Sinha N. Role of oral sildenafil in severe pulmonary arterial hypertension: clinical efficacy and dose response relationship. Int J Cardiol 2007; 120(3):306-13.
10.Abbas AE, Fortuin FD, Schiller NB, Appleton CP, Moreno CA, Lester SJ. A simple method for noninvasive estimation of pulmonary vascular resistance. J Am Coll Cardiol 2003; 41(6):1021-7.
11.Stefadouros MA, Dougherty MJ, Grossman W, Craige E. Determination of systemic vascular resistance by a noninvasive technic. Circulation 1973; 47(1):101-7.
12.Davies GG, Jebson PJ, Glasgow BM, Hess DR. Continuous Fick cardiac output compared to thermodilution cardiac output. Crit Care Med 1986; 14(10):881-5.
13.Lau EMT, Giannoulatou E, Celermajer DS, Humbert M. Epidemiology and treatment of pulmonary arterial hypertension. Nat Rev Cardiol 2017; 14(10):603-14.
14.Huang JB, Liang J, Zhou LY. Eisenmenger syndrome: not always inoperable. Respir Care 2012; 57(9):1488-95.
15.Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010; 122(2):156-63.
16.Hascoët S, Baruteau AE, Humbert M, Simonneau G, Jais X, Petit J, et al. Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome. J Hear Lung Transplant 2017; 36(4):386-98.
17.Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Hear J 2020;42(6):563-645.
18.Humbert M, Ghofrani HA. The molecular targets of approved treatments for pulmonary arterial hypertension. Thorax 2016; 71(1):73-83.
19.Souza R, Jardim C, Fernandes CJC, Lapa MS, Rabelo R, Humbert M. NT-proBNP as a tool to stratify disease severity in pulmonary arterial hypertension. Respir Med 2007; 101(1):69-75.
20.Wolfson AM, Maitland ML, Thomeas V, Glassner C, Gomberg-Maitland M. A serial NT-proBNP model to improve prognostication in patients with pulmonary arterial hypertension. J Clin Exp Cardiol 2017; 8(11):1000555.

DOI: https://doi.org/10.19106/JMedSci005501202305

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Journal of the Medical Sciences (Berkala Ilmu Kedokteran) by  Universitas Gadjah Mada is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Based on a work at http://jurnal.ugm.ac.id/bik/.