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Pulmonary hypertension (PH)-associated with interstitial lung disease (ILD) develops as a consequence of progressive underlying lung disease or disproportionately to the underlying disease. The PH investigation by right heart catheterization (RHC) for defining PH severity is recommended in patients with ILD who show more severe symptoms than expected from lung disease, appearance of right heart failure, and clinical deterioration not matched by the declining lung function. In patient with progressive ILD, RHC is only considered if it affects the future treatment such as lung transplantation or enrollment in clinical trial/registry. The decision to undertake the RHC in progressive ILD was still fraught with doubts. Here we reported a young adult male patient with ILD whom developed progressive signs and symptoms. By RHC, he had severe precapillary PH with hemodynamic parameters indicated the presence of pulmonary vascular disease. A PH-specific treatment, sildenafil citrate, was administered, and patient responded well and was clinically stable during the addition of sildenafil citrate. This case highlights the clinical implication of performing RHC in progressive ILD, which can change the treatment decision by PH-specific drugs. Therefore, the RHC decision making in patient with progressive ILD need reconsideration.

Objective To assess the effect of combined hospital and home-based exercise programs on functional capacity and quality of life (QoL) among uncorrected atrial septal defect-associated pulmonary arterial hypertension (ASD-PAH) patients.Methods This study was a randomized controlled trial with uncorrected ASD-PAH patients as the subjects. They were allocated randomly into control and exercise groups. Exercise group subjects performed hospital and home-based exercise programs, completing baseline 6-minute walking test (6MWT) and EQ-5D-3L QoL test (Utility Index and EQ-VAS scores), and were followed up for 12 weeks. The primary outcomes were 6MWT distance and EQ-5D-3L score at week 12. The N-terminal pro B-type natriuretic peptide (NT-proBNP) level was also assessed. A repeated-measure ANOVA was performed to detect endpoint differences over time.Results The exercise group contained 20 subjects and control group contained 19. In total, 19 exercise group subjects and 16 control group subjects completed the protocol. The 6MWT distance, Utility Index score, and EQ-VAS score incrementally improved significantly in the exercise group from baseline until week 12, with mean differences of 76.7 m (p

Backgrounds The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry is the first registry for congenital heart disease (CHD) and CHD-related pulmonary hypertension (PH) in adults in Indonesia. The study aims to describe the demographics, clinical presentation, and hemodynamics data of adult CHD and CHD-related PH in Indonesia. Methods The COHARD-PH registry is a hospital-based, single-center, and prospective registry which includes adult patients with CHD and CHD-related PH. The patients were enrolled consecutively. For this study, we evaluated the registry patients from July 2012 until July 2019. The enrolled patients underwent clinical examination, electrocardiography, chest x-ray, 6-min walking test, laboratory measurement, and transthoracic and transesophageal echocardiography. Right heart catheterization was performed to measure hemodynamics and confirm the diagnosis of pulmonary artery hypertension (PAH). Results We registered 1012 patients during the study. The majority were young, adult females. The majority of CHD was secundum ASD (73.4%). The main symptom was dyspnea on effort. The majority of patients (77.1%) had already developed signs of PH assessed by echocardiography. The Eisenmenger syndrome was encountered in 18.7% of the patients. Based on the right heart catheterization, 66.9% of patients had developed PAH. Patients with PAH were significantly older, had lower peripheral oxygen saturation, had lower 6-min walking distance, and higher NTproBNP. The NTproBNP level independently predicted the development of PAH among CHD. Conclusions The COHARD-PH registry is the first Indonesian adult-CHD and CHD-related PH registry. The demographics, clinical presentation, and hemodynamics dataof this registry reflect the situation in developing countries which needs to be compared with similar registries from developed countries.

Right-sided infective endocarditis (IE) is very rare and mostly occurs in the tricuspid valve, which pulmonary valve involvement in very rare. Most IE of the pulmonary valve occurs in patients with congenital heart defects. we report a case of the complications of pulmonary valve IE and pulmonary embolism which then causes pulmonary infarction in patients with VSD who have not been corrected. This case successfully underwent surgery for endocarditis/vegetation removal and septal defect closure.

Background NKX2-5 variant in atrial septal defect patients has been reported. However, it is not yet been described in the Southeast Asian population. Here, we screened the NKX2-5 variants in patients with atrial septal defect (ASD) in the Indonesian population. Method We recruited 97 patients with ASD for genetic screening of the NKX2-5 variant using Sanger sequencing. Results We identified three variants of NKX2-5: NM_004387.4:c.63A>G at exon 1, NM_004387.4:c.413G>A, and NM_004387.4:c.561G>C at exon 2. The first variant is commonly found (85.6%) and benign. The last two variants are heterozygous at the same locus. These variants are rare (3.1%) and novel. Interestingly, these variants were discovered in familial atrial septal defects with a spectrum of arrhythmia and severe pulmonary hypertension. Conclusion Our study is the first report of the NKX2-5 variant in ASD patients in the Southeast Asian population, including a novel heterozygous variant: NM_004387.4:c.413G>A and NM_004387.4:c.561G>C. These variants might contribute to familial ASD risk with arrhythmia and severe pulmonary hypertension. Functional studies are necessary to prove our findings.

Women adapt to pregnancy through multi-organ system physiologic changes, including cardiovascular adaptations. These changes affect those with pre-existing cardiovascular problems differently, and subsequently lead to higher probability of death caused by cardiovascular diseases during pregnancy. Therefore, detection of cardiovascular disease early in pregnancy is important to lower maternal morbidity and mortality by providing prompt and adequate management. This study aimed to evaluate and test the feasibility of integrating 12-lead electrocardiogram (ECG) examination and antenatal care (ANC) screening as a simple and effective method for early detection of heart abnormality in pregnant woman. Pregnant women were recruited in this study in any trimester who attended ANC for a routine pregnancy examination in Puskesmas Tegalrejo Yogyakarta. The subjects underwent primary screening which focused on cardiac auscultation and 12-lead ECG examinations, and those who had abnormal findings were further followed-up in secondary screening by using trans-thoracic echocardiography to confirm heart abnormality. A total of 523 pregnant women from Puskesmas Tegalrejo were included in this study. 15 (2.8%) pregnant woman were suspected to have heart abnormalities; from those, 3 (0.5%) were found with heart murmurs with abnormal ECG readings, 1 (0.19%) had heart murmurs with normal ECG results, and 11 (2.1%) had abnormal ECG readings only. The secondary screening of those patients resulted in 1 (0.19%) pregnant woman who was diagnosed with Atrial Septal Defect. Our study found that among 15 patients identified with suspected ECG abnormalities, one mother who underwent ANC was newly diagnosed with a pre-existing cardiac abnormality. Our study concluded this screening method is a simple and feasible integrated heart screening program that can be implemented widely. We hope this integrated heart screening program may benefit pregnant women who may have cardiac abnormalities to be detected as early as possible, thus reducing maternal morbidity and mortality.

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 international guidelines along with new research findings on prognostic factors can help in making better management decisions for PAH patients at different stages of the disease.

Pulmonary hypertension (PH) due to atrial septal defect (ASD) may cause a decline in right ventricle (RV) function. Soluble ST2 isa prognostic biomarker for left ventricle dysfunction. However, its role in RV function has not been investigated. This study aimed to investigate the association between serumsoluble ST2 with RV systolic function in patients withASD–associatedPH. This was a cross sectional study. Subjects were patients participated in the COHARD-PH registry performed in Dr. Sardjito General Hospital, Yogyakarta Indonesia. The patients with ASD and PH confirmed by right heart catheterization (RHC) were enrolled in this study. The soluble ST2 level was measured in the serum collected from pulmonary artery during RHC. Right ventricle systolic function was determined by transthoracic echocardiography using peak systolic velocity of tricuspid annulus (S’) parameter. This study was performed in 32 adults with uncorrected ASD. They predominantly females [n=29 (90.6%)] with median age of 31(22.5-44.0) years old. Mean ASD diameter was 2.69±0.53 cm. Median mean pulmonary artery pressure (mPAP) 45.0 (36.25-70.0) mmHg. Median soluble ST2 level was 23.28 ng/mL. There were no significant correlations between soluble ST2 level with S’value (r=0.071; p=0.35), with mPAP (r=0.043; p=0.815), with pulmonary vasculer resistance (PVR) (r=0.025; p=0.893) and with right ventricle (RV) diameter (r=0.200; p=0.273). Soluble ST2 level was found higher in subject with RV dysfunctionbut not statistically significant. In conclusion, serum soluble ST2 level did not associate withRV systolic function, measured by S’, in adult ASD-associated PH.

Background Atrial Septal Defect (ASD) is a congenital heart disease that often under-diagnosed in youth. Atrial septal defect has a poor clinical manifestation at Pulmonary Arterial Hypertension (PAH) condition. Cardiac autonomic dysfunction may occur in PAH due to over sympathetic activation. This condition in general population is associated with increase risk of sudden cardiac death. Despite the case, there are only limited studies which associated cardiac autonomic dysfunction and PAH in adult patient with uncorrected ASD. Purpose This study aims to compare cardiac autonomic dysfunction, using heart rate variability, in adult patient with uncorrected ASD between PAH and non-PAH group. Methods Adult (18+) patient with uncorrected ASD who listed in ASD registry in Indonesia, were selected in this analytical observational study. Patients in whom no other congenital defect and atrial fibrillation in electrocardiogram were eligible for inclusion. Diagnose of PAH was established when mean pulmonary arterial pressure value from right heart catheterization is ≥ 25 mmHg. Arrhythmias and heart rate variability, using Standard Deviation of Normal-Normal (SDNN), High Frequency (HF) and Low Frequency (LF) parameters, were collected from holter monitoring. These procedures were performed on the same day. Results We enrolled 55 adult patients with uncorrected ASD (mean age: 33.3 ± 10.3 years, 89.1% women), consist of 51 secundum ASD, 3 sinus venosus ASD and 1 primum and secundum ASD patient. They were divided into PAH group (28 patients) and non-PAH group (27 patients). Patients in PAH group had a higher incidence of paroxysmal atrial fibrillation and ventricular extrasystole compare to those in non-PAH group (28.5% vs. 14.8% and 75% vs. 70%). All parameters of heart rate variability was significantly reduced in PAH group compare to non-PAH group (SDNN : mean difference 14.5 ± 2.98 ms, CI 95%, P < 0.001; HF : median difference 99.5 ms2, P < 0.001; LF : median difference 129.6 ms2, P < 0.001). Conclusion A marked reduction in cardiac autonomic function occur in adult uncorrected ASD patient with PAH compare to patient with non-PAH, that might be the result of decreasing baroreceptor and increasing chemoreceptor function in PAH.

Uncorrected atrial septal defect undergoes right ventricle chronic volume overload which may lead to pulmonary hypertension and Eisenmenger Syndrome. The soluble suppression of tumorigenicity-2 is a left ventricle strain biomarker; however, its role in right ventricle strain is unclear. This study aimed to investigate the implication of serum soluble suppression of tumorigenicity-2 in adult uncorrected atrial septal defect. This was a cross-sectional study. We enrolled 81 adult uncorrected secundum atrial septal defect patients. Clinical and hemodynamic data were collected. Serum samples were withdrawn from the pulmonary artery during right heart catheterization. Serum soluble suppression of tumorigenicity-2 and NT-proBNP levels were measured. Subjects were divided into three groups based on clinical and hemodynamic severity. The correlation of soluble suppression of tumorigenicity-2 with patients' data and comparison among groups were analyzed. A p value

The coronavirus disease of 2019 (COVID-19) is a current pandemic of viral infection which mainly involves respiratory system and may progress into severe multiple organ dysfunction and mortality. Pulmonary artery hypertension (PAH) is a disease marked by increased mean pulmonary artery pressure and pulmonary vascular resistance due to pulmonary panvascular remodeling. Although rare, the prevalence of PAH is currently escalating in Indonesia due to increased diagnostic capacity and referral, treatment availability and improved survival. Despite chronic cardiac and pulmonary diseases are at increased risk to develop severe COVID-19, patients with PAH are considered to be not in higher risk to develop severe COVID-19. However, whether this population is protected from severe COVID-19 is unclear. There are protective and offensive factors need to be considered in PAH patients in respect to COVID-19.

Background: Assessment of health-related quality of life (HRQoL) are often measured as an important patient-reported outcome (PRO) in clinical studies. Pulmonary arterial hypertension (PAH) is a common complication of atrial septal defect (ASD). This study aimed to compare the HRQoL of PAH related uncorrected secundum ASD at pre and post therapy with oral sildenafil therapy. Methods: We conducted quasi experimental study at Sardjito General Hospital Yogyakarta since April 2016 to August 2017. Adults with PAH related uncorrected secundum ASD, listed on Congenital Heart Disease and Pulmonary Hypertension (COHARD-PH) registry, and met the inclusion and exclusion criteria were recruited as subject. Interview was done at pre and 12 weeks post oral sildenafil therapy 3 x 20 mg using the EQ-5D-3L questionnaire. Statistical analysis was done using paired T-test to determine the differences of EQ5D utility and EQVAS score at pre and post therapy. Results: A total of 18 adult patients with PAH related to uncorrected secundum ASD were enrolled in this study (83.33% female; mean age 38.72 ± 10.81 years old). The most frequent reported problems pre therapy were pain/discomfort A total of 18 adult patients with PAH related to uncorrected secundum ASD were enrolled in this study (83.33% female; mean age 38.72 ± 10.81 years old). The most frequent reported problems pre therapy were pain/discomfort (83%) and anxiety/depression (78%). Wilcoxon signed rank test showed the median of EQ-5D utility score were increased after sildenafil therapy (before= 0.604, after=0.664; Z= -2,703; p:0.007), respectively. Meanwhile, the paired T-test results showed an increase of EQ-VAS mean difference 6.67±8.75 (p:0.005; 95% CI 2.32-11.02) after sildenafil therapy.(83%) and anxiety/depression (78%). Paired T-test results showed the mean value of EQ5D utility score pre and post therapy were 0.558 and 0.664 post therapy (p:0.014; 95% CI 0.024-0.187). Meanwhile, the mean of EQ-VAS pre and post therapy were 65 and 71.67, respectively (p:0.005; 95% CI 2.32-11.02). Conclusion: The administration of oral sildenafil therapy 3 x 20 mg during 12 weeks in adult patients with PAH related uncorrected secundum ASD gives better HRQoL.

Background and Objectives. Pulmonary arterial hypertension (PAH) pathomechanism involves an increased plasma level of endothelin-1 and a reduced plasma level of prostacyclin and nitric oxide. Whether similar mechanisms prevail in PAH associated with atrial septal defect (ASD) is unclear. This study aimed to investigate the relationship of endothelin-1, prostacyclin, and nitric oxide with PAH in uncorrected ASD in Indonesian population. Methods. The study design was cross-sectional. The subjects were adult uncorrected secundum ASD with PAH. Pulmonary artery pressure was measured with right heart catheterization. Pulmonary venous blood was obtained during catheterization for measuring endothelin-1, prostacyclin, and nitric oxide. Correlation tests were performed to determine any association between biomarkers and mean pulmonary artery pressure (mPAP). The levels of biomarkers were compared based on the severity of PAH. Statistical significance was determined at p<0.05. Results. Forty-four subjects were enrolled in this study. Endothelin-1 level and mPAP had significant moderate positive correlation (r = 0.423 and p value = 0.004). However, no significant correlation was observed between prostacyclin, nitric oxide levels, and mPAP. The pattern of endothelin-1, prostacyclin, and nitric oxide was distinctive. Levels of endothelin-1 were incrementally increased from mild, moderate, to severe PAH. The levels of prostacyclin and nitric oxide had similar pattern in association with the severity of PAH, which was increased in mild-to-moderate PAH but decreased in severe PAH. Conclusions. There was a distinctive pattern of endothelin-1, prostacyclin, and nitric oxide based on severity of PAH in adult uncorrected ASD. Significant correlations existed between endothelin-1 and the severity of PAH and mPAP.

Key Clinical Message Uncorrected left‐to‐right shunt congenital heart defect is a predisposing factor for infective endocarditis (IE), especially right‐sided IE which has different clinical manifestations and complications from left‐sided IE. Prompt diagnosis by means of transthoracic echocardiography and timely antibiotics management for IE are encouraged to prevent multiorgan failure and fatal pulmonary embolism.

One of the risk factors of Acute Coronary Syndrome (ACS) still controversial is Lipoprotein(a). Lp(a) is one of the lipid componentshighly homologous to plasminogen and which may compete with it in the fibrinolytic pathway and has an atherogenic effect. Prior to thestudy many variaties in results have been shown. These variations are related to different population and ethnics, thus, the researcherswere triggered to investigate the role of Lp(a) on the ACS in the Indonesian population. This case control study was conducted at theSardjito General Hospital, Yogyakarta, Indonesia consisting of 40 participants in ACS as the case group and other 40 persons sufferingfrom Stable Angina Pectoris (SAP) as a control group. This study lasted from May−December 2011. The Lp(a) was measured usingturbidimetric immunoassay method while other laboratory results were obtained from the medical records. The results of this studyshowed that high Lp(a) level (more than 30 mg/dL) was the risk factor of ACS (RR=2.818, CI: 1.069–7.426). There was no difference ofthe baseline characteristics such as: the history of hypertension, diabetes mellitus, smoking, as well as in other laboratory parameters suchas: lipid profile, hemoglobine and uric acid level in the case as well as the control group. Significant differences were found in leucocytenumber, creatinine and blood glucose level. The median level of those parameters was found higher in the case group.

One of the causes of pulmonary arterial hypertension (PAH) is connective tissue disease, including systemic lupus erythematosus (SLE). The prevalence of PAH in patients with SLE according to cohort studies varies widely between 0.5% and 43%. In some cases, PAH is the initial manifestation leading to the diagnosis of SLE. However, PAH can develop as a complication in patients who are initially diagnosed with SLE. We report two cases with a different approach to the diagnosis of pulmonary hypertension in SLE patients. These cases emphasize the importance of investigating examination results according to the guidelines to establish the diagnosis of PAH in SLE.

Key Clinical Message Persistent Left Superior Vena Cava (PLSVC) should be suspected if we find dilatation of coronary sinus. Sophisticated imaging is not always available in each health care provider. Transthoracic echocardiography (TTE) with agitated saline injections through the left and right antecubital veins provides a simple, and inexpensive, but effective study for a rapid bedside diagnosis of PSLVC.

Background Congenital heart diseases (CHD) cause declining of functional capacity, cyanosis, arrhythmia, stroke, and death in adulthood. The screening for congenital heart disease in children has not been established in Indonesia. This results in a relatively high prevalence of uncorrected CHD in adulthood, who mostly have developed pulmonary artery hypertension. The screening program by cardiac auscultation and electrocardiography (ECG) in schoolchildren has been proven to be feasible in developed countries, which may also be feasible in Indonesia.Aim This study was planned to test and assess CHD screening methods based on electrocardiography examination in school children.Methods We conducted a screening program in first grade elementary school children in the Province of Yogyakarta, Indonesia. The schools were determined by purposive sampling. Trained general practitioners in community health centers participated in the screening. The physical examinations were done, which focused on cardiac auscultation and ECG examination. Those who had abnormal findings underwent trans-thoracic echocardiography to confirm the CHD.Results We collaborated with local governments in recruiting the participating community health centers and elementary schools. A total of 130 elementary schools from 5 districts in Yogyakarta Province were recruited, all without prior information on students’ presentation of symptoms.Conclusions This study is the first screening program that focuses on heart diseases in elementary school students in Indonesia. The success and feasibility of this program will give a valuable impact on preventing CHD complications during adulthood.

Backgrounds The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry is the first congenital heart disease (CHD) and PH registry in Indonesia. The study aims to describe prevalence, demographics, and hemodynamics data of adult CHD and PH in Indonesia.Methods The COHARD-PH registry is a hospital-based, single-center, and prospective cohort registry which includes adult patients with CHD and CHD-related PH. The patients were enrolled consecutively. We evaluate the registry patients from July 2012 until July 2018. The enrolled patients underwent clinical examination, electrocardiography, chest x-ray, 6 minute walking test, laboratory measurement, and transthoracic and transoesofageal echocardiography. Right Heart Catheterization (RHC) was performed to measure hemodynamics and confirmed the diagnosis of pulmonary artery hypertension (PAH).Results We registered 803 patients during the study. The majority were young-adult females. The majority of CHD was secundum ASD (79.0%). The main symptom was dyspneu on effort. The majority (78.1%) already develops signs of PH assessed by echocardiography. The Eisenmenger syndrome was encountered in 17.3% subjects. Based on the RHC, 67.8% subjects had developed PAH. Patients with PAH were significantly older, lower peripheral oxygen saturation, lower 6 minute walking distance, and higher NTproBNP. There was an increased of PAH prevalence according to age range, with the highest prevalence between 51 and 60 years old.Conclusions The COHARD-PH registry is the first Indonesian adult-CHD and PH registry. The incidence, demographics data, and hemodynamics data of this registry reflects situation in developing countries which need to be compared with similar registries from developed countries.

Heart Failure is now considered as one of the leading cause for mortality and morbidity. It is affecting several organs and cause organ damages due to the myocardial failure to pump inadequate oxygenated blood to the body including metabolites, to end organs and peripheral tissues. Heart failure results from multifactorial mechanism including neurohumoral activations including increased activity of the sympathetic nervous system, renin-angiotensin aldosteron system, vasopression and the atrial natriuretic peptide. This neurohumoral pathway has significant contribution to the development of myocardial dysfunction that lead to clinical manifestation of heart failure. Some of the markers in these pathways have now been considered as an independent predictors of prognosis in heart failure patient.

Background: Right atrium (RA) enlargement in uncorrected atrial septal defect (ASD) is due to chronic volume overload. Several electrocardiogram (ECG) criteria had been proposed for screening RA enlargement. This study aimed to compare the accuracy of ECG criteria in detecting RA enlargement in adults with uncorrected ASD. Methods: This was a cross-sectional study involving 120 adults with uncorrected secundum ASD. The subjects underwent ECG examination, transthoracic echocardiography, and right heart catheterization. An RA enlargement was determined with RA volume index by transthoracic echocardiography. Various ECG and combined ECG criteria were evaluated. Statistical analysis was performed to analyze the sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV). Results: An RA enlargement was detected in 64.2% subjects. The P wave height > 2.5 mm in lead II criterion had the best specificity (100%) and PPV (100%), but low sensitivity (19%) and accuracy (48%). The combined 2 ECG criteria (QRS axis > 90°, R/S ratio > 1 in V1) had 82% sensitivity, 56% specificity, 73% accuracy, 77% PPV, and 63% NPV. The combined 3 ECG criteria (QRS axis > 90°, R/S ratio > 1 in V1, and P wave height > 1.5 mm in V2) had 35% sensitivity, 86% specificity, 53% accuracy, 82% PPV, and 43% NPV. Conclusions: The combined 2 ECG criteria (QRS axis > 90° and R/S ratio > 1 in V1) had increased sensitivity, better accuracy, and more balance of PPV and NPV as compared with P wave > 2.5 mm in II criterion and combined 3 ECG criteria to diagnose RA enlargement in adults with uncorrected ASD.

Pericardial effusion associated with malignacy has poor prognosis. The prompt identification of etiology is mandatory, such that timely management can be performed and survival can be increased. However, difficulty in etiology determination is commonly encountered. In this case, we report female patient with relapse stage IV breast cancer who develop massive pericardial and bilateral pleural effusion. The similar characteristics were found in both effusion fluids, however the identification of etiology was not similar. Metastatic cells were found in pleural effusion, whereas they were absent in pericardial effusion.

Background: Secundum type atrial septal defect (ASD) is the most common adult congenital heart disease. Hemodynamically, ASD result in a left-to-right shunt that causes right heart volume overload. This condition affects interventricular septal position and shape. Left ventricular (LV) eccentricity index (EI) suggests right ventricle (RV) overload when this ratio is >1.0. The aim of this study was to assess correlation between LV EI and mean pulmonary arterial pressure (mPAP) in secundum type ASD patients. Methods: We conducted a cross sectional study from ASD patients registry data. We enrolled subjects with secundum type ASD from the complete registry data. The LV EI was calculated from transthoracal echocardiography. The mPAP was measured by right heart catheterization as a gold standart. The correlation was performed by Spearman correlation test. Results: There were 40 adult secundum type ASD patients participated. The mean LV EI in enddiastolic was 1.55±0.39 and LV EI in end-systolic was 1.75±0.58. The median value of mPAP was 29 (12-99) mmHg. There was a moderate strength, positive and significant correlation between LV EI in end-diastolic and mPAP (r=0.37, p=0.018). The correlation was more significant and stronger between LV EI in end-systolic and mPAP (r=0.52, p=0.001). Conclusion: There was sufficiently strong and significant correlation between LV EI in both end-diastolic and end-systolic phase with mPAP in adult secundum type ASD.

Atrial septal defect (ASD) is the most frequent congenital heart disease in adulthood. Pulmonary hypertension (PH) complicating ASD compels patients seeking medical assistance because of its disabling symptom. Most adult ASD develop PH which render significant morbidity and mortality. The aim of the research is to characterize the clinical profiles of adult patients with ASD. The research design was cross sectional. The subjects were enrolled consecutively from outpatient clinics and inpatient wards. The demography, medical and imaging data were collected and recorded in case report form. Descriptive statistics was applied to characterize the subjects. Seventy-six subjects were enrolled. The majority were women (77.6 %) in the productive and child-bearing ages (63.2%). The most common symptoms were dyspneu on effort, fatigue, and palpitation. Most subjects had functional capacity of WHO class functional II (70.2 %). The mean oxygen saturation was 96.4 %. Based on the echocardiography examination, 77.6% of subjects had suffered from PH. The mean longest diameter of defects were 2.7 centimeter. The direction of blood flow was mostly left to right (77.6 %). Left and right ventricle function were within normal limit. Right heart catheterization showed mean left atrial pressure 11.5 mmHg, which confirmed the precapillary or arterial PH. Mean pulmonary artery pressure was 42.0 mmHg. The pulmonary artery resistance index mostly less than 4 Wood Unit/m2 (63.7 %), indicating the feasibility to close the defect. Whereas 24.6 % of subjects were contraindicated for closing. Pulmonary artery hypertension (PAH) was diagnosed in 77.6 % subjects, meanwhile 13.2 % had borderline PAH. In conclusion, most adult ASD patients had developed PAH, mostly young women in productive ages, mainly visited hospital due to symptom of PH, the direction of flow predominantly left to right side and mostly had reduced functional capacity. Keywords : atrial septal defect, pulmonal hypertension, pulmonary artery hypertension Abstrak Defek septum atrium (DSA) merupakan penyakit jantung bawaan yang paling sering dijumpai pada usia dewasa karena tanda dan gejalanya yang samar. Hipertensi paru (HP) merupakan komplikasi DSA yang mendorong pasien mendatangi rumah sakit karena munculnya gejala yang berat. Hampir semua pasien DSA dewasa mengalami HP yang menyebabkan angka kesakitan dan kematian yang bermakna. Tujuan penelitian ini adalah untuk mengetahui karakteristik klinis pasien dengan DSA dan HP. Disain penelitian adalah potong lintang. Subjek dicuplik secara berurutan dari klinik rawat jalan dan rawat inap. Data demografi, medis dan pencitraan dikumpulkan dan dicatat dalam suatu case report form. Penelitian deskriptif dilakukan untuk menilai karakteristik subjek. Sebanyak tujuh puluh enam subjek dicuplik. Mayoritas subjek adalah wanita (77,6 %) dalam usia muda, produktif dan subur (63,2 %). Gejala paling sering adalah sesak saat aktivitas, mudah lelah, dan berdebar. Sebagian besar pasien masuk dalam kelas fungsional WHO II (70,2 %). Rerata saturasi oksigen 96,4 %. Berdasar hasil ekhokardiografi, sebanyak 77,6 subjek telah mengalami HP. Rerata diameter terpanjang defek adalah 2,7 centimeter. Arah aliran darah melewati defek sebagian besar kiri ke kanan (77,6 %). Fungsi ventrikel kanan dan kiri dalam batas normal. Kateterisasi jantung kanan menunjukkan rerata tekanan atrium kiri sebesar 11,5 mmHg yang menandakan HP prekapiler/arteri. Rerata tekanan arteri pulmonalis sebesar 42.0 mmHg. Nilai indeks tahanan arteri pulmonalis sebagian besar kurang dari 4 Wood unit/m2 (63,7 %) yang menunjukkan kemungkinan bisa dilakukan penutupan defek. Sedangkan 24,6 % subjek kontraindikasi untuk penutupan. Hipertensi arteri pulmonalis (HAP) didiagnosis pada 77,6 % subjek, sedangkan 13,2 % subjek mengalami HAP borderline. Sebagai kesimpulan, sebagian besar DSA dewasa telah mengalami HAP, sebagian besar wanita muda yang berusia produktif, sebagian besar mengunjungi rumah sakit karena gejala HP, arah aliran sebagian besar dari kiri ke kanan dan sebagian besar telah mengalami penurunan kapasitas fungsional.

Following plaque rupture, activated platelet will induce subsequent inflammatory process including neutrophil recruitment. In vitro study demonstrated an interaction between neutrophils and platelets via a mechanism involving CD40-CD40 ligand. However, whether this mechanism exists in the clinical setting remains unknown. Fifty-four patients with acute myocardial infarction (AMI) and 25 with unstable angina of pain onset ofor = 24 h were enrolled consecutively. Acute myocardial infarction was diagnosed from three diagnostic criteria, i.e., anginal pain, electrocardiogram ST-T changes, and cardiac enzyme elevation. Unstable angina was diagnosed in patients without elevated cardiac enzymes. Peripheral venous blood was drawn at admission for routine blood count and soluble CD40 ligand (sCD40L) measurement. Neutrophil count was determined by an automated blood cell counter. Circulating sCD40L was measured using a standard enzyme-linked immunosorbent assay. Neutrophil count was significantly higher in AMI as compared with unstable angina (P0.001), whereas circulating sCD40L did not significantly differ. Despite marked elevation, no correlation was observed between neutrophil count and circulating sCD40L in AMI. Interestingly, we observed a strong and positive significant correlation between neutrophil count and circulating sCD40L level (r = 0.607, P = 0.002) in unstable angina. Circulating sCD40L is associated with neutrophil count and may mediate interaction between neutrophils and platelets in acute coronary syndrome, particularly in unstable angina.

Aims Although endothelin-1 (ET-1) has been suggested to contribute to the pathogenesis of neointima formation and atherosclerosis, the individual roles of ET-1 derived from certain cell types in this disease remain unclear. In this study, we determined the role of vascular endothelial ET-1 on vascular inflammation and neointima formation using vascular endothelial ET-1-knockout [ET-1f/f; Tie2-Cre (+)] mice. Methods and results Intimal hyperplasia was induced by complete ligation of the left carotid artery in 12-week-old male ET-1f/f;Tie2-Cre (+) mice ( n = 35) and the wild-type (WT) littermates ( n = 34). Following this intervention, neointima formation was reduced in ET-1f/f;Tie2-Cre (+) mice compared with the WT mice, independent of the difference in blood pressure. This reduction was associated with a decrease in inflammatory cell recruitment to the vessel wall, which was accompanied by reduced expression levels of endothelial adhesion molecules as well as chemokines and a decrease in vascular smooth muscle cell proliferation. Conclusion The results of our study provide direct evidence for the role of vascular endothelial ET-1 in mediating vascular inflammation and neointima formation following vascular injury in addition to promoting vasoconstriction and cell proliferation. Furthermore, this study suggests a strategy for the efficient design of ET receptor antagonists with targeted inhibition of ET-1 signalling in vascular endothelial cells.

Background: In STEMI patients, the duration of action potential dispersion occurs between normal and ischemic tissue due to the lengthening of the refractory period, causing transmural dispersion of repolarization, which could be detected with Tp-Te interval prolongation on the electrocardiogram (ECG). Benefits of fibrinolytic therapy in patients with STEMI has been demonstrated, with reduced mortality significantly and improve coronary patency in order to increase myocardial perfusion. The goal of this study was to determine Tp-Te interval alteration in STEMI patients before and after the fibrinolytic therapy between successful fibrinolysis compared to failed fibrinolysis. Method: Cross-sectional study was conducted to collect ECG from medical records at Dr. Sardjito General Hospital in January–September 2016. STEMI patients with onset less than 12hours whom reperfused with fibrinolytic therapy were registered. Tp-Te interval was measured before, soon after (0 minute), and 30 minutes after fibrinolysis with successful and failed results. The unpaired t-test analysis was used to compare Tp-Te interval alteration after fibrinolysis. Then, ΔTp-Te cut-off value was determined to find sensitivity and specificity based on ROC. Result: Among 84 patients enrolled in this study, 46 patients with successful fibrinolysis and 38patients with failed fibrinolysis. Both of groups had Tp-Te interval prolongation before fibrinolysis, with mean value of 120.30 ± 13.02 ms in successful fibrinolysis group and 118.57 ± 15.24 ms in failed fibrinolysis group. In successful fibrinolysis group, Tp-Te interval reduced significantly with ΔTp-Te value of 17.55 ± 13.35 ms on 0 minute and 20.85 ± 15.62 ms on 30 minutes after fibrinolysis, while in failed fibrinolysis group there was not a decrease of Tp-Te interval with ΔTp-Te value of -0.77 ± 11.00 ms on 0 minute (p

Acute coronary syndrome is a clinical condition of partial or total obstruction of blood flow in the coronary artery due to acute thrombus formation. Culprit vessel, coronary artery segment within which the site of origin of thrombus formation lies, is occupied by eroded or ruptured atherosclerotic plaque. Direct contact between circulating blood constituent and atherosclerotic plaque content owing to loss of endothelial cell barrier orchestrates the haemostasis events, i.e. thrombus formation and coagulation activation. Evolved within years of human life span, atherosclerotic undergoes three main steps: initiation, progression and finally complication (Libby, 2002). Atherosclerotic plaque development involves cellular and molecular interactions as well as blood flow dynamic alterations in the affected area. Although these steps affect all individual, some gather the risk factors to develop progression and complication of coronary atherosclerotic lesion faster and more prominent than others. Given the dynamic nature of these steps, understanding several mechanisms engage in every step will provide insight into therapeutic approach. Here, we review the last two steps of coronary atherosclerotic plaque development, with the focus in the role of platelets, anucleated cells being the target for therapeutic advancement in atherosclerosis and acute coronary syndrome.

Endothelium plays important role in protective mechanism of vascular wall. The balance between endothelin-1 (ET-1) and nitric oxide provide endothelial barrier to lipoprotein retain and macrophage recruitment. In contrasts, ET-1 is also a strong vasoconstrictor. In this study, we aim to determine the role of vascular endothelial cells-derived ET-1 in the development of atherosclerosis. For that purpose, we crossbred Vascular Endothelial Cells-specific ET-1 Knockout (VEETKO) mice to ApoEKO mice. ApoE/VEET-DKO exhibited significantly lower ET-1 plasma and mRNA level as compared to ApoEKO mice. No significant differences of blood pressure, plasma cholesterol or lipid profiles were observed in both mice. Surprisingly, after 8 weeks of western diet, we found that the atherosclerotic lesion was exaggerated in the aortic sinus and brachiochepalic artery of ApoE/VEET-DKO mice (n=7) as compared to those of ApoEKO mice (n=7) (ratio/vessel wall, 0.93±0.13vs.0.49±0.09, p

Complex mechanism of the chronic inflammatory process in vascular injury and atherosclerosis involves various types of cells and cytokines. Previous in vitro studies suggest that Endothelin-1 (ET-1) is produced by those cells, induces expression of several adhesion molecules and mediates the leukocyte-endothelial interaction. In the present study, we sought to determine whether local endothelial ET-1 plays a direct effect on early inflammation and neointimal formation as a response to vascular injury in vivo . Using Cre-loxP system and Tie-2 Cre promoter, Vascular Endothelial ET-1 Knockout (VEETKO) mice was generated. The mice showed 60% reduction of ET-1 mRNA level at major organs and undetected serum ET-1 level. To induce vascular inflammation, complete ligation of the left common carotid artery was performed in 12-weeks-old male VEETKO (n=35) and Wild Type (WT) littermates (n=34). Inhibition of vascular endothelial ET-1 prevented the 4-fold increase in ET-1 mRNA level of carotid artery after ligation, in contrast to that of WT mice. This prevention resulted in lower vascular inflammation revealed by lower mRNA and protein level of PECAM-1, ICAM-1, and MCP-1 in VEETKO mice. The lack of ET-1 in endothelium further inhibited the adhesion of inflammatory cells at early days after ligation as compared to that adhered in endothelium of WT mice (cell numbers, 20.7±4.9 vs. 54.6±3.9 respectively, p