Introduction: Rheumatic heart disease (RHD) remains a major public health issue affecting children and young adults in developing countries. This study aimed to evaluate the clinical characteristics, management, and reactivation of RHD among children and young adults. Patients and Methods: This was a hospital-based retrospective study conducted at the National Cardiovascular Center Harapan Kita, Indonesia; we retrieved relevant data from patients diagnosed with RHD between 2012 and 2018. Results: Two hundred and seventy-nine patients were diagnosed with rheumatic heart disease, of whom 108 were children (mean age of 12.02 ± 3.36 years) and 171 were young adults (mean age was 24.9 ± 3.84). RHD was more common in female than in male young adults (1.5:1). Hospitalization due to RHD complications such as congestive heart failure was seen in 11.11% of cases in children, while pulmonary hypertension was present in 19.95% young adult cases. Reactivation of RHD occurred in 17.2% (48/279) cases, significantly in children (p < 0.001). Overall, the mitral valve (either isolated or combined) was the organ most affected in children (39.13%) and young adults (44.81%). Isolated mitral regurgitation was more common in children (13/21, 61.9%), while isolated mitral stenosis was more common in young adults (19/47, 40.42%). There was a high rate of rheumatic tricuspid valve disease in all populations (193/279, 69.17%) and reported involvement of pulmonary regurgitation (46/279, 16.48%). Multivalve lesions were more common than single lesions in both groups, with a combination of mitral and tricuspid regurgitation the predominant type in children (32/43, 74.41%) and mixed mitral lesion and tricuspid regurgitation in young adults (22/72, 30.56%). We observed a significant occurrence of quadrivalve lesions in children (p = 0.039). Valve repair was more common in children (49.07%) and replacement in young adults (32.16%), with low in-hospital mortality. Compliance with secondary prophylaxis was a significant challenge. Conclusion: Chronic RHD often presented with complications of the disease or reactivation of rheumatic fever (RF). Inadequate treatment of RF/RHD leads to extensive valvular damage and consequent disabilities. Efforts toward active early diagnosis and prompt treatment of RF/RHD and effective preventive measures are essential.

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This multi-center, multi-vendor study demonstrated variations in radiation dose metrics of pediatric cardiac CT reflecting current practice in Asia. Gender, tube voltage, scan mode and cardiac function assessment should be considered as essential radiation dose-influencing factors in developing optimal pediatric cardiac CT protocols.

Background This study aims to assess the cost-effectiveness and budget impact of adopting sildenafil to the benefits package for the indication of pulmonary arterial hypertension (PAH), compared to beraprost. Methods Based on a societal perspective, a model-based economic evaluation was performed using local and international data to quantify the potential costs and health-related outcomes in terms of life years (LYs) and quality-adjusted life years (QALYs). Results The economic model calculated the incremental cost-effectiveness ratio (ICER) per QALY gained for using sildenafil as first-line therapy compared to beraprost for the patient in functional class (FC) II and III, i.e. USD 3098 and USD 2827, respectively. The results indicated that in spite of sildenafil being more expensive than beraprost, generic sildenafil could potentially be a good value for money since ICER per QALY is below one times gross domestic product (GDP) per capita in Indonesia. Furthermore, budget impact analysis estimated that the incremental budget needed within 5 years for including sildenafil compared to beraprost for PAH patients starting in FC II and FC III was USD 436,775 and USD 3.6 million, respectively. Conclusions Compared to beraprost, sildenafil would be preferable for the treatment of PAH patients in FC II and FC III in Indonesia. The additional budget for adopting sildenafil compared to beraprost as the treatment of PAH in the benefits package was estimated at around USD 4.0 million. Electronic supplementary material The online version of this article (10.1186/s12913-019-4422-5) contains supplementary material, which is available to authorized users.

Background The Fontan procedure has been applied in many patients with single-ventricle physiology, with quite low mortality rates all over the world, and a 8.6%–9.1% rate in Indonesia. Structural and characteristic differences in the morphologically left and right ventricles influence the role of the systemic ventricle in the functionally univentricular heart and impact on postoperative outcomes. Mid- and long-term survival based on systemic ventricle morphology remains controversial. This study aimed to investigate the impact of systemic ventricle morphology on ten-year survival after the Fontan procedure. Methods The 162 patients who underwent a Fontan operation at our institution between 2008 and February 2018 and survived to discharge were reviewed and followed up until March 2018. Data were extracted from the registry and pediatric surgical conferences, medical records, surgery reports, echocardiography and catheterization reports, and follow-up to the end of the study period. Median follow-up was 26.5 months (range 10.75–54 months). Results The patients were divided into 2 groups based on systemic ventricle morphology. Seventy-four patients were included in the left morphology group and 88 in the right morphology group. There was no difference in 10-year survival rates between the two type of systemic ventricle morphology. Postoperative thromboembolic events influenced the 10-year survival rate after the Fontan procedure (hazard ratio 4.84, 95% confidence interval: 1.26–18.55, p = 0.021). Conclusion Systemic ventricle morphology was not associated with the 10-year survival rate after the Fontan procedure. Postoperative thromboembolic events accounted for a 4.84-times higher mortality risk after the Fontan procedure.

IntroductionSince the first successful percutaneous closure under transesophageal echocardiographic (TEE) guidance, many centers explored transcatheter procedures without fluoroscopy. This single-center study is aimed to show the feasibility and safety of percutaneous patent ductus arteriosus (PDA) closure under echocardiography-only guidance during our 1-year experience.MethodsPatients with PDA were recruited for percutaneous PDA closure guided by either fluoroscopy or echocardiography-only in National Cardiovascular Center Harapan Kita (ClinicalTrials.gov Identifier: NCT05321849, clinicaltrials.gov/ct2/show/NCT05321849). Patients were evaluated clinically and radiologically using transthoracic echocardiography (TTE) at 6, 24, and 48 h after the procedure. The primary endpoint was the procedural success. Secondary endpoints were the procedural time and the rate of adverse events.ResultsA total of 60 patients underwent transcatheter PDA closure, 30 patients with fluoroscopy and 30 patients with echocardiography guidance. All patients had successful PDA closure. There were only residual shunts, which were disappeared after follow-up in both groups, but one patient with a fluoroscopy-guided procedure had moderate tricuspid regurgitation with suspected thrombus in the tricuspid valve. The procedural time was not significantly different between the fluoroscopy and echocardiography groups.

Recent advances in the diagnosis and management of pulmonary arterial hypertension (PAH) have led to a significant improvement in the outcomes for patients with PAH. However, prompt and accurate diagnosis of PAH remains an unmet challenge due to lack of awareness and lack of meticulous data to profile the etiology and pathophysiology of this rare progressive disease, especially in low- and middle-income country. In Indonesia, the true prevalence and incidence of different subtypes of PAH in general population is still unknown. The Congenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry was the first single-center prospective registry in Indonesia, which indicated that almost 80% of adult patients with congenital heart disease (CHD) had experienced PAH and even Eisenmenger syndrome due to delayed diagnosis. Screening for early detection of asymptomatic CHD in children is yet to be systematically established in Indonesia, leading to undiagnosed and uncorrected CHD in adulthood. There are no specific national guidelines focusing on diagnostic workup and treatment of PAH in Indonesia. Furthermore, the lack of adequate diagnostic facilities, limited treatment availability, and limited drug coverage under the National Health Insurance Scheme are key issues that remain unaddressed. This review focuses on the diagnosis, treatment, and management of PAH associated with CHD in Indonesia as per international guidelines. We have proposed recommendations to effectively control and prevent PAH associated with CHD in Indonesia. The paper should be of interest to readers in the area of medical management and policy makers especially in low- and middle-income countries. Key Highlights: Pulmonary arterial hypertension (PAH) is a rare progressive subtype of pulmonary hypertension with poor overall prognosis and outcomes. Prompt and accurate diagnosis of PAH remains an unmet challenge in low- and middle-income countries due to poor knowledge about the etiology and pathophysiology of this syndrome. Also, the symptoms and signs of early-stage PAH are usually nonspecific or undetectable in newborn and infants, thus presenting a challenge for physicians to establish early diagnoses of PAH. The challenging factors in low- and middle-income countries, especially Indonesia archipelago are limitations of healthcare infrastructure, limited expertise, lack of awareness, lack of timely PAH screening strategies, poor antenatal care and unpredictable availability of PAH medications. There are no specific national guidelines focusing on diagnostic workup and treatment of PAH in Indonesia. Under-utilization of treatment guidelines and lack of adequate diagnostic treatment facilities have resulted in sub-optimal management of PAH patients in Indonesia. Adherence to international guidelines is an important aspect of PAH management in Indonesia. Updated disease and functional classifications of PAH as per internati...

Introduction: Low cardiac output syndrome is one of the postoperative complications that are associated with significant morbidity and mortality after surgical closure of atrial septal defect (ASD) with small-sized left ventricle (LV). This study investigated whether preoperative left ventricular end-diastolic volume index (LVEDVi) could accurately predict low cardiac output syndrome (LCOS) after surgical closure of ASD with small-sized LV.Method: This retrospective cohort study involved adult ASD patients with small-sized LV from January 2018 to December 2019 in National Cardiovascular Center Harapan Kita. Preoperative MRI data to assess the left and right ventricle volume were collected. A bivariate analysis using independent Student's t-test was done. Diagnostic test using receiver operating characteristic (ROC) curve was also done to obtain the area under the curve (AUC) value. The best cutoff point was determined by Youden's index.Result: Fifty-seven subjects were involved in this study [age (mean ± SD) 32.56 ± 13.15 years; weight (mean ± SD) 48.82 ± 12.15 kg]. Subjects who had post-operative LCOS (n = 30) have significantly lower LVEDVi (45.0 ± 7.42 ml/m2 vs. 64.15 ± 13.37 ml/m2; p &lt; 0.001), LVEDV (64.6 ± 16.0 ml vs. 85.9 ± 20.7 ml; p &lt; 0.001), LVSV (38.97 ± 11.5 ml vs. 53.13 ± 7.5 ml; p &lt; 0.001), and LVSVi (27.28 ± 8.55 ml/m2 vs. 37.42 ± 5.35 ml/m2; p &lt; 0.001) compared to subjects who did not have post-operative LCOS (n = 27). ROC analysis showed that the best AUC was found on LVEDVi (AUC 95.3%; 95% confidence interval: 90.6–100%). The best cutoff value for LVEDVi to predict the occurrence of LCOS after surgical closure of ASD was 53.3 ml/m2 with a sensitivity of 86.7% and a specificity of 85.2%.Conclusion: This study showed that preoperative LVEDVi could predict LCOS after surgical closure of ASD with small-sized LV with a well-defined cutoff. The best cutoff value of LVEDVi to predict the occurrence of LCOS after surgical ASD closure was 53.5 ml/m2.