Preliminary results from a nationwide adult cardiology perspective for pulmonary hypertension: RegiStry on clInical outcoMe and sUrvival in pulmonaRy hypertension Groups (SIMURG)

Abstract

Objective:The present study was designed to evaluate the characteristics of pulmonary hypertension (PH) and adult cardiology practice patterns for PH in our country.Methods:We evaluated preliminary survey data of 1501 patients with PH (females, 69%; age, 44.8±5.45) from 20 adult cardiology centers (AdCCs).Results:The average experience of AdCCs in diagnosing and treating patients with PH was 8.5±3.7 years. Pulmonary arterial hypertension (PAH) was the most frequent group (69%) followed by group 4 PH (19%), group 3 PH (8%), and combined pre- and post-capillary PH (4%). PAH associated with congenital heart disease (APAH-CHD) was the most frequent subgroup (47%) of PAH. Most of the patients’ functional class (FC) at the time of diagnosis was III. The right heart catheterization (RHC) rate was 11.9±11.6 per month. Most frequently used vasoreactivity agent was intravenous adenosine (60%). All patients under targeted treatments were periodically for FC, six-minute walking test, and echo measures at 3-month intervals. AdCCs repeated RHC in case of clinical worsening (CW). The annual rate of hospitalization was 14.9±19.5. In-hospital use of intravenous iloprost reported from 16 AdCCs in CWs. Bosentan and ambrisentan, as monotreatment or combination treatment (CT), were noted in 845 and 28 patients, respectively, and inhaled iloprost, subcutaneous treprostinil, and intravenous epoprostenol were noted in 283, 30, and four patients, respectively. Bosentan was the first agent used for CT in all AdCCs and iloprost was the second. Routine use of antiaggregant, anticoagulant, and pneumococcal and influenza prophylaxis were restricted in only two AdCCs.Conclusion:Our nationwide data illustrate the current status of PH regarding clinical characteristics and practice patterns.

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

“…Our registry shows that patients are predominantly young adult females. This observation is consistent with other registries [3,6,7,13,[16][17][18]. The mean age of patients in our registry during first enrollment is 34.7 years old.…”

“…Most patients come to our medical facility when they already had a complaint, with shortness of breath and easily fatigued among the most common complaints. These symptoms are similar to previous studies where the patients are already in an advanced condition and had limited activity [13,15]. Since the most prevalent CHD in our registry is ASD, the patients remained asymptomatic for decades.…”

“…The prevalence and incidence of CHD-related PH are still unknown; nevertheless in clinical practice, adult patients with undetected and delayed diagnosis of CHD are frequent [10]. Compared with registries from developed countries, the situation regarding the adult CHDrelated PH in developing countries are very different [11][12][13]. The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry was initiated in 2012 to be the first registry done in Indonesia to describe adult CHD and CHD-related PH populations.…”

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

“…This characteristic is similar to previous study where the patient was already in advanced condition and had limited activity. 13,14 Since in our registry the most prevalent CHD was ASD, the patients remain asymptomatic for decades. Therefore, the symptoms that appear later will urge the patients to consult doctor and visit hospital.…”

“…10 Compared with registry from developed countries, the situation regarding the adult CHD-related PH in developing countries are quite different. 11,12,13 The COngenital HeARt Diseases in adult and Pulmonary Hypertension (COHARD-PH) was performed in patients with confirmed ASD and VSD by TTE examination. The TTE and TOE examination were conducted with G.E Vivid 7 (G.E Healthcare, U.S.A), G.E Vivid S6 (G.E Healthcare, U.S.A) or Phillips HD 15 (Philips N.V, The Netherland).…”