We reported the first hospital-based registry of adults with CHD and CHD-related PH in Indonesia in which comprise the complete diagnostic work-up. The COHARD-PH registry is a single center registry in Dr. Sardjito Hospital, a national referral center for cardiovascular disease in Indonesia. The COHARD-PH registry has been started in July 2012 and still on going until recently. Within the duration of five years, July 2012 – July 2018, the number of patients enrolled were 803 adults with CHD. The majority of CHD in this registry was ASD, followed by VSD and PDA. Young adult females (ages between 21-40 years old) were predominant in our registry. Most patients were symptomatic, with majority in WHO functional class II. By echocardiography, the prevalence of probability of PH was 78.1 %. Further confirmation with RHC measurement, the COHARD-PH registry showed that 67.8% subjects had developed PAH.
In our registry, the majority of CHD was ASD. This is similar with other study that in adult registry, ASD was the most common.1 Most patients come to our medical facility when they already had a complaint, with shortness of breath and easily fatique among the most common complaint. 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. The main symptom of patients was associated with the development of PAH, which later confirmed with RHC procedure. The early finding of CHD-related PAH are often not easy to recognize due to the unknown precise period of PAH.11 The chronic systemic-to-pulmonary shunt is a congenital malformation causing blood overflow in the pulmonary vasculature from infancy, and if left untreated properly may give rise to PAH in adult life. The majority of our patients was untreated cases, and probably undetected cases in their childhood period. They come to our hospital in their delayed and progressed diseases.
Our registry showed that patients were predominantly young adult females. This observation was consistent with other registries.3,6,7,13,15,16,17 The mean age of patients in our registry during first enrollment was 35 years old. The facts that most cases were ASDs, in which at younger age there had been no complaints. In ASD clinical presentation, the pulmonary hypercirculation and right heart volume overload induces PAH after a longer period of time, which is different with VSD or PDA.6 The patients with VSD and PDA were symptomatic in earlier years of age and have more evident signs, probably before reaching adulthood, therefore mostly detected in childhood and adolescent period.12 Our hospital register in pediatric patients indicated that VSD was the most prevalence in childhood.18 Moreover, 72.7% patients were asymptomatic for a long period of time (> 2 decades). Mostly in the third decades of life, the PAH complication starts to clinically manifest and urge the patients to visit the hospital.
The echocardiographic data showed that majority of the patients was categorized in high probability of PH and confirmed PAH by RHC, which is a gold standard for diagnosis of PH and PAH. Almost 70% of our patients already developed PAH based on RHC. This data is much higher than data from other registries, especially registries from developed countries. The striking difference is likely due to late presentation and selection bias, because the patients were enrolled at our hospital mostly due to signs and symptoms they suffer. Currently, in Indonesia there was no early screening and detection of asymptomatic CHD, therefore many patients were undetected until they come to visit medical facilities due to complication.
The patients with PAH had worse clinical characteristics as compared to those without PAH. They were in young adult age and older than patients without PAH. Their functional capacities, measured by WHO class, 6 minute walking distance and peripheral oxygen saturation, were worse. The NTproBNP level, the sole biomarker for prognostication of PH, was higher in patients with PAH as compared with those without. Among CHD-associated PAH, it should be noted that four difference clinical subgroup have been proposed which reflects different pathophysiology and prognosis.4,12,16 Patients with small defect-associated PAH had similar outcome with Eisenmenger syndrome which was better than the outcome of patients in which PAH develops or persists after closure of the defect.16,17 The large defects with prevalent systemic-to-pulmonary shunts have better survival as compared with other clinical types.12,16
The definite management of CHD is closing the defect with either percutaneous defect closure or open heart surgery for those who still meet the closing criteria. While for those who have experienced CHD-PAH, medical therapy is mandatory by administration of pulmonary vasodilator medication as target drugs therapy.4 Recently, more than ten specific PAH drugs are available across the world.4 However, in Indonesia, only three drugs are available in the market, which are prostacyclin analogue (beraprost and illoprost) and PDE5 inhibitor (sildenafil). Unfortunalety, only beraprost is so far covered by the national insurance. This current study do not report the medications and procedures performed to the patients. The descriptive analysis of baseline data of this registry is reported here, whereas the intervention and outcome will be reported in the future. The registry data that showed predominant CHD-associated PAH in young adult female patients, give valuable evidence for further action by medical practitioner and government in developing countries such as the regulation in the prevention and early detection/screening for CHDs during infancy and childhood. Furthermore, the availability of more drugs for treating PAH complication also a consideration to be implemented.