Based on the data, we found that the mean age of subjects diagnosed as PAH in uncorrected secundum ASD was 38.72 years. These results are in accordance with the study of Haque et al. (2015) which stated that PAH development in secundum ASD mostly occurred in the third decade.18 Vogel et al. (1999) noted that the incidence of PAH in secundum ASD were increased in patients at the age of 18 to 40 year old.19 The majority of the subjects of this study were female (83.33 %) which similar to the previous study.19 Euro Heart Survey registry also support this findings. They concluded that the incidence of PAH in female ASD was 76.6%, higher than male patients.20All subjects have many problems in various EQ5D dimensions before starting the specific PAH therapy. In general, subjects reported their best performance on the self-care dimension and their worst on pain/discomfort dimension. Severe problems experienced by 11% of subjects on both pain/discomfort and anxiety/depression dimensions, while 6% of the subjects felt severe problems on performing usual activities. This result is in accordance with the study by Thompson et al. (2001) in Germany who found that the best performance of both primary and secondary PAH patients was experienced in the self-care dimension while the worst performance was reported on the dimension of usual activities. In this study, 20% of subjects experienced severe problems while performing usual activities and less than 10% experienced severe problems in other dimensions.21 Mychaskiw et al. (2010) who examined the health status of PAH patients in the subjects of SUPER 1 clinical trial found that moderate to severe problems mostly occur in the usual activity dimension (77%), while the least occur in self-care dimension (24%).22After 12 weeks of treatment, severe-problem response level was no longer experienced in the dimensions of pain/discomfort, anxiety/depression, and usual activities. The percentage of subjects who did not had any complain in all of the 5 EQ5D dimensions were increased. The severity of each EQ5D dimension problem were decreased. In line with these results, Pepke-Zaba et al. (2008) proved in his study that improvement was achieved in all dimensions of EQ5D after 12 weeks of sildenafil therapy.8A clinical trial by Pepke-Zaba et al. (2008) found that the mean of EQ-5D utility score changed 0.10±0.04 (p<0.01) and the mean of EQ-VAS changed 8±2 (p<0.01).8 Our study showed similar changes that after sildenafil therapy, the median of EQ-5D utility score was increased after therapy (before=0.604, after=0.664; Z= -2.703, p:0,007), while the mean of EQ-VAS score also were increased 6.67±8.75 (95% CI, 2.32 to 11.02) p-value 0.005. Determination of drug effects on HRQoL is an important component in evaluating the effect of drugs on clinical outcomes and health care. Several previous studies that evaluated HRQoL after sildenafil administration showed consistent results with this study. A study in patients with primary pulmonary hypertension (n=22) conducted by Sastry et al. (2004) mentioned that there was an increase in dyspnea and fatigue components of heart failure questionnaire after receiving 12 weeks sildenafil therapy (dose: 3 x 25 mg, 3 x 50 mg and 3 x 100 mg).23 Another study by Wong et al. (2007), involving 19 HAP patients (idiopathic, connective tissue disease, and CHD, also evaluated HRQoL changes 3 months after sildenafil therapy (dose: 3 x 25 mg and 3 x 50 mg) using SF-36 questionnaire. There was an increase on physical, social, and general health score.24 Tay et al. (2011) used CAMPHOR questionnaire to evaluate HRQoL in 12 patients with Eisenmenger syndrome who were given sildenafil 3x20 mg. The result showed an improvement in HRQoL after 3 months of therapy.25Demographic factors such as age, sex, marital status, education level, and employment status have an influence on HRQoL. A systematic review by Gu et al. (2016) who assessed factors affecting HRQoL of PAH patients explained that HRQoL is influenced by demographic characteristics (such as living alone, decreased social support), mental health (such as anxiety, depression, stress), physical health (such as exercise and symptomatic capacity), and pharmacologic therapy.4 In this study, the utility score mean of subjects aged <38 years was lower than subjects aged ≥ 38 years, but the mean of EQ-VAS score was the opposite (p>0.05). Study by Matura et al. (2014) that examined the difference in symptoms severity and HRQoL of young, middle, and older PAH patients concluded that the decrease in HRQoL component was experienced by all age groups, but the younger age group had slightly better physical function compared to other groups.26 Symptom severity, therapy complexity, or psychological stressors made the patients hard to work. This led to job losses, disrupted economic conditions and social isolation.27Nilsson (2012) mentioned that in a group of unmarried / single patients had a low HRQoL.28 Similarly, this study found that the mean of both utility scores and EQ-VAS of unmarried subjects were lower than those married subjects (p>0.05). Living alone and minimal social support worsen HRQoL emotional dimension score. In contrast, working actively is associated with a better HRQoL physical dimension score.4 The mean of both utility scores and EQ-VAS in unemployed subgroup in this study, were lower than those that worked (p>0.05).Delcroix and Howard (2015) published an article review on the burden of PAH disease and its impact on quality of life. It was explained that PAH patients who aged > 50 years old were reported to have more comorbid diseases such as ischemic heart disease, coronary artery disease, hypertension, atrial fibrillation, diabetes, and hypothyroidism than younger patients. The presence of comorbidities results in delayed diagnosis of PAH in older patients. The higher burden of comorbidities also contributes to the lower survival rates of older PAH patients in the UK and Ireland, which is about 3 times higher than the younger population (≤50 years).29 In line with this, in this study, the mean of EQ5D utility score and EQ-VAS of subjects with comorbidities were lower than those without comorbidities (p>0.05).Supportive therapies (such as diuretics, digoxin, oral anticoagulants, or oxygen) is one of the PAH patient’s management strategies mentioned in the ESC guidelines on PAH (2015). Right heart failure leads to fluid retention, increased central venous pressure, hepatic congestion, ascites, and peripheral edema. Clinical experience shows the benefits of diuretics to reduce fluid retention symptoms, but no randomized trials related to diuretic use in PAH patients. Diuretic therapy is recommended in PAH patients with signs of right heart failure and fluid retention, with recommendation class Ic. Aldosterone antagonist therapy may be considered, along with plasma electrolyte levels and renal function monitoring, to prevent hypokalemia and pre-renal kidney disease.30High prevalence of vascular thrombotic lesions was found on postmortem examination of idiopathic PAH patients. In addition, the abnormalities of the coagulation and fibrinolysis cascades in the PAH patient population have also been reported. Oral anticoagulants are administered to PAH patients with consideration of increased risk of venous thromboembolism (heart failure and immobilization). The benefits of oral anticoagulant therapy are limited to idiopathic PAH, hereditary PAH, and anorexigen-related PAH (recommendation class IIb). This is largely derived from retrospective single center studies. In a contrary, the research result conducted from registry and randomized clinical trials are heterogenous and inconclusive.30 Current evidence of anticoagulant drugs’ efficacy and safety in PAH patient populations is limited. The clinical guidelines do not recommend routine anticoagulant treatment in Eisenmenger syndrome patients and suggest to give anticoagulant treatment in atrial fibrillation and pulmonary artery thrombosis without major haemorrhage.31In this study 44,44% (8) subjects received other therapy, including PAH support therapy. A subject (5.56%) was treated with oral furosemide if necessary, six (33.34%) subjects treated with oral furosemide 1x20 mg and oral digoxin 1x0.125 mg, and a subject (5.56%) with accompanying atrial fibrillation received oral furosemide 1x40 mg, digoxin 1x0,125 mg, spironolactone 1x25 mg, and warfarin 1x2 mg. The mean of EQ5D utility score and EQ-VAS in group of subjects receiving other therapies were lower than without comorbidities (p>0.05). This is in line with cross-sectional studies by Zlupko et al (2008) that evaluate HRQoL PAH patients with various etiologies such as idiopathic, familial, systemic sclerosis, CHD, human immunodeficiency virus (HIV), liver disease, anorexigen, and obstructive pulmonary venous disease. A total of 93 subjects who were recruited received epoprostenol therapy (28%), bosentan (49%), calcium channel blockers (47%), sildenafil (3%), digoxin (33%), diuretics (57%) and warfarin (49%). The HRQoL evaluation was measured by a specific PAH disease questionnaire (MLHF-PH). The study found a severe HRQoL disorder in all subjects. There were no HRQoL differences in patients treated with diuretics, digoxin, and oxygen.32