Multimodality diagnostic imaging for anomalous pulmonary venous connections: a pictorial essay.

Anomalous pulmonary venous connections represent a heterogeneous group of congenital heart diseases in which a part or all pulmonary venous flow drains directly or indirectly into the right atrium. Clinically, anomalous pulmonary venous connections may be silent or have variable consequences, including neonatal cyanosis, volume overload and pulmonary arterial hypertension due to the left-to-right shunt. Anomalous pulmonary venous connections are frequently associated with other congenital cardiac defects and their accurate diagnosis is crucial for treatment planning. Therefore, multimodality diagnostic imaging, comprising a combination (but not all) of echocardiography, cardiac catheterization, cardiothoracic computed tomography and cardiac magnetic resonance imaging, helps identify potential blind spots relevant to each imaging modality before treatment and achieve optimal management and monitoring. For the same reasons, diagnostic imaging evaluation using a multimodality fashion should be used after treatment. Finally, those interpreting the images should be familiar with the various surgical approaches used to repair anomalous pulmonary venous connections and the common postoperative complications.

A retrograde approach for transcatheter valvotomy procedure in infants with pulmonary atresia intact ventricular septum (PA-IVS): retrograde versus antegrade approach.

INTRODUCTION: This study evaluates the retrograde approach compared to the antegrade approach in infants with PA-IVS who underwent transcatheter pulmonary valvotomy procedure at National Cardiovascular Center Harapan Kita, Jakarta, Indonesia. MATERIAL AND METHOD: This is a single-centre retrospective study conducted from January 2017 to June 2019 consisting of infants undergoing transcatheter pulmonary valvotomy procedures from our centre. RESULTS: Among 3733 records of cardiac catheter procedure in paediatric patients during the last 3 years, there were 12 subjects with PA-IVS, where five subjects were done by antegrade approach and seven by retrograde approach. The retrograde approach is shown to excel the antegrade approach in terms of procedural time by 58.64 minutes (CI 95 % 32.97-84.29, p = 0.008) and PA-RV crossing time by 27 minutes (CI 95 % 14.01-39.99, p = 0.02). There was no significant difference in contrast used (120.23 ± 25.77 versus 150.27 ± 39.26 ml/BSA, p = 0.518), and right ventricle to pulmonary artery systolic pressure gradient after valvotomy (39.571 ± 5.814 versus 53.52 ± 29.15, p = 0.329) between the retrograde and the antegrade approach. CONCLUSION: The retrograde approach offered shorter procedural time and comparably satisfying results than the antegrade approach. The shorter procedural time was preferred due to the shorter duration of general anaesthesia, which may decrease the risk of neurodevelopmental deficits in the patient.

Economic evaluation of sildenafil for the treatment of pulmonary arterial hypertension in Indonesia.

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.

Asian consortium on radiation dose of pediatric cardiac CT (ASCI-REDCARD).

BACKGROUND: With incremental utilization of pediatric cardiac CT in congenital heart disease, it is imperative to define its current radiation dose levels in clinical practice in order to help imagers optimize CT protocols, particularly in Asia and other developing countries where CT physicists are not readily available. OBJECTIVE: To evaluate current radiation dose levels and influencing factors in cardiac CT in children with congenital heart disease in Asia by conducting a retrospective multi-center, multi-vendor study. MATERIALS AND METHODS: We included 1,043 pediatric cardiac CT examinations performed in 8 centers between January 2014 and December 2014 to evaluate congenital heart disease. In five weight groups, we calculated radiation dose metrics including volume CT dose index, size-specific dose estimate, dose-length product and effective dose. Age at CT exam, gender, tube voltage, scan mode, CT indication and image reconstruction algorithm were analyzed to learn whether they influenced CT radiation dose. RESULTS: Volume CT dose index, size-specific dose estimate, dose-length product and effective dose of pediatric cardiac CT showed variations in the range of 4.3-23.8 mGy, 4.9-17.6 mGy, 55.8-501.3 mGy∙cm and 1.5-3.2 mSv, respectively, within five weight groups. Gender, tube voltage, scan mode and cardiac function assessment significantly influenced CT radiation dose. CONCLUSION: 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.

Transoesophageal echocardiography-guided balloon-assisted percutaneous closure of a large secundum atrial septal defect in a pregnant woman: a case report.

Background: According to the 2018 European Society of Cardiology guidelines, atrial septal defect (ASD) closure can be performed during pregnancy but is rarely indicated. In this case, we demonstrate the viability of percutaneous balloon-assisted ASD closure without fluoroscopy in a pregnant woman. Case summary: A 23-year-old G3P2A0 woman who was 20 weeks pregnant had primary complaints of breathlessness [New York Heart Association functional class (NYHA fc) III and IV] for 1 week prior to admission. A transthoracic echocardiography showed a dilatation of the right atrium (RA), a dilated right ventricle, a dilated main pulmonary artery (28.1 mm), and an oval-shaped 22 × 33 mm-sized secundum ASD with a left-to-right shunt. Despite optimal pharmacological treatment, the NYHA fc persisted. Under transoesophageal echocardiography monitoring, we introduced a 40 mm Cera™ ASD Occluder (Lifetech, China) via the delivery sheath. The device was deployed in the usual position; however, despite numerous placement adjustments, the left atrium disc kept getting dislodged to the RA and could not engage correctly. Therefore, we decided to use a balloon-assisted approach using a sizing balloon of No. 34 mm. The device was successfully positioned, and a wiggle test was conducted to make sure that the device remained stable. The patient was able to give birth to the child normally several months later. Discussion: Despite the fact that pregnant women with ASD receive a very low dose of radiation, it is nevertheless recommended to avoid radiation because this demographic is particularly vulnerable to it. It is possible to treat a large ASD in pregnant women with a successful balloon-assisted approach.

Right ventricular fibrosis in adults with uncorrected secundum atrial septal defect and pulmonary hypertension: a cardiovascular magnetic resonance study with late gadolinium enhancement, native T1 and extracellular volume.

Introduction: Right ventricular (RV) fibrosis represents both adaptive and maladaptive responses to the overloaded RV condition. Its role in pulmonary hypertension (PH) associated with secundum atrial septal defect (ASD), which is the most common adult congenital heart disease (CHD), remains poorly understood. Methods: We enrolled 65 participants aged ≥18 years old with uncorrected secundum ASD who had undergone clinically indicated right heart catheterization (RHC), divided into the non-PH group (n = 7), PH group (n = 42), and Eisenmenger syndrome (ES) group (n = 16). We conducted cardiovascular magnetic resonance (CMR) studies with late gadolinium enhancement (LGE) imaging, native T1 mapping, and extracellular volume (ECV) measurement to evaluate the extent and clinical correlates of RV fibrosis. Results: LGE was present in 94% of the population and 86% of the non-PH group, mostly located at the right ventricular insertion point (RVIP) regions. LGE in the septal and inferior RV region was predominantly observed in the ES group compared to the other groups (p = 0.031 and p < 0.001, respectively). The mean LGE scores in the ES and PH groups were significantly higher than those in the non-PH group (3.38 ± 0.96 vs. 2.74 ± 1.04 vs. 1.57 ± 0.79; p = 0.001). The ES and PH groups had significantly higher degrees of interstitial RV fibrosis compared to those in the non-PH group, indicated by native T1 (1,199.9 ± 68.9 ms vs. 1,131.4 ± 47.8 ms vs. 1,105.4 ± 44.0 ms; p < 0.001) and ECV (43.6 ± 6.6% vs. 39.5 ± 4.9% vs. 39.4 ± 5.8%; p = 0.037). Additionally, native T1 significantly correlated with pulmonary vascular resistance (r = 0.708, p < 0.001), RV ejection fraction (r = -0.468, p < 0.001) and peripheral oxygen saturation (r = -0.410, p = 0.001). Conclusion: In patients with uncorrected secundum ASD, RV fibrosis may occur before the development of PH and progressively intensify alongside the progression of PH severity. A higher degree of RV fibrosis, derived from CMR imaging, correlates with worse hemodynamics, RV dysfunction, and poorer clinical conditions.

Right ventricular outflow tract stenting for late presenter unrepaired Fallot physiology: a single-center experience.

Objectives: The purpose of this study was to assess the clinical outcome after right ventricular outflow tract (RVOT) stenting in late presenter patient with unrepaired Fallot physiology. Background: In younger patients, RVOT stenting is an alternative to mBTT shunt; however, there have been few reports of this palliative technique in late presenter population, including adults. Methods: This was a single-center, retrospective study of nonrandomized, palliated Fallot patients. Clinical outcomes such as left ventricular ejection fraction and saturation were measured in 32 individuals following RVOT stenting in adults (n = 10) and children (n = 22). The Statistical Package for Social Science (SPSS) 26.0 software was used to analyze the statistical data. Results: During the procedure, the average stent diameter and length were 8.84 ± 1.64 mm and 35.46 ± 11.23 mm, respectively. Adult patients received slightly longer stents than pediatric patients (43.60 ± 11.64 mm vs. 31.77 ± 9.07 mm). Overall, patients' saturation increased from 58.56 ± 19.03% to 91.03 ± 8.98% (p < 0.001), as did their left ventricular ejection fraction (LVEF) from 64.00 ± 18.21% to 75.09 ± 12.98% (p = 0.001). Three patients improved their LVEF from 31 to 55%, 31 to 67%, and 26 to 50%. The median length of stay was 8 (2-35) days, with an ICU stay of 2 (0-30) days. The median time from RVOT stent palliation to total repair was 3 months (range: 1 month-12 months). Conclusions: RVOT stenting is a safe and effective method for increasing saturation and ejection fraction not only in newborn infants but also in late presenters, including adults with unrepaired Fallot physiology.

Efficacy and safety of transcarotid compared to transfemoral approach of patent ductus arteriosus stenting in duct-dependent cyanotic heart disease.

BACKGROUND: Minimally invasive transcatheter approaches were usually done for patent ductus arteriosus (PDA) with duct-dependent pulmonary circulation. There are two ways to establish vascular access, by using transfemoral either femoral vein (FV) or femoral artery (FA) and transcarotid artery (CA) with surgical cutdown approach to access the PDA and then provide good support for the balloon and the stent to be safely deployed. This study aims to compare the efficacy and safety of transcarotid with surgical cutdown compared to the transfemoral approach of patent ductus arteriosus stenting in duct-dependent cyanotic heart disease. RESULTS: Overall procedural complication rates were higher in the FA/FV approach than in the CA approach (51% vs. 30%). The incidence of acute limb ischemia in the FA approach is significantly higher than in the CA approach (P < 0.05). No acute thrombosis/occlusion of the carotid artery was assessed by carotid vascular ultrasound in 2-day series. CONCLUSIONS: The transcarotid approach with surgical cutdown may offer a secure and more efficient means of accessing the PDA, particularly for those emerging from the underside of the aortic arch.

Case Series of Berry syndrome: A rare constellation of fatal cardiac anomalies.

Berry syndrome is an extremely rare constellation of several congenital cardiac anomalies consisting of aortopulmonary window, aortic origin of the right pulmonary artery (AORPA), interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, and an intact ventricular septum with high neonatal mortality rates. The disease is fatal with high mortality (90%) in the neonatal period with surviving patients mostly developing pulmonary hypertension. We describe the clinical presentation and diagnostic clues in two patients with Berry syndrome.

Retrospective study of preoperative sildenafil in prolonged pleural effusion after Fontan.

Background: By rerouting the systemic venous return into the pulmonary circulation, the Fontan operation is perhaps the most effective palliative procedure in patients with complex congenital heart disease. The Fontan, however, still comes with several significant complications and morbidities, one of them being postoperative prolonged pleural effusion. In our center from 2017 to 2021, 10% of all our patients who underwent Fontan operation developed prolonged pleural effusion. With this study, we aimed to analyze the preoperative use of sildenafil for 6 months and its role in prolonged pleural effusion after the Fontan operation. Materials and Methods: In total, 158 patients were included in the analysis. The cohort was divided into patients who developed prolonged pleural effusion after Fontan surgery (n = 17) and those who did not (n = 141). We analyzed several risk factors and the history of sildenafil use in each group in the cohort. We found that patients who used sildenafil for at least 6 months prior to Fontan surgery versus those who did not use (n = 112 vs. n = 46) were found to be at lower risk of developing prolonged pleural effusion (odds ratio [OR]: 0.32 [confidence interval 95%: 0.11-0.88], P = 0.027). Conclusion: Preoperative administration of sildenafil for at least 6 months before Fontan might reduce the risk of postoperative prolonged pleural effusion.

Impact of older age at Fontan completion on mid-term survival.

BACKGROUND: The optimum age of Fontan completion remains unknown. Currently, the majority of centers worldwide are performing Fontan completion at 2-4 years of age. In Indonesia, lack of awareness and limited resources probably explain why patients seek treatment at advanced stage. This study aimed to evaluate the impact of older age at Fontan completion on mid-term survival. RESULTS: A single-center retrospective cohort study was performed on 261 patients who underwent Fontan completion between 2008 and 2019 and survived to discharge. The patients were followed up until April 2020, with a median follow-up period of 3 years (range 0-12 years). The median age was 5 years (range 2-24 years). The survival rates of patients with the age at operation ≤ 6 years and > 6 years were 92.1% and 82.8%, respectively. A subgroup analysis showed that the survival rates for age < 4 years, 4-6 years (reference age), 6-8 years, 8-10 years, 10-18 years, and > 18 years were 85.7%, 94.8%, 85.4%, 78.8%, 85.7%, and 66.7%, respectively. Age at Fontan completion of > 6 years (HR 3.84; p = 0.020) was associated with a lower 12-year survival rate. The age at operation of 8-10 years (HR 6.79; p = 0.022) and > 18 years (HR 15.30; p = 0.006) had the worst survival rates. CONCLUSIONS: An older age at Fontan completion (> 6 years) significantly reduced mid-term survival rate. The age at Fontan of 8-10 years and > 18 years had higher risk of mid-term death than age of 4-6 years.

Double Stenting in 19-Year-Old Patient With Tetralogy of Fallot With Prior Fractured Stent.

A 19-year-old patient with tetralogy of Fallot was referred to the cath lab because of persistent desaturation and a fractured right ventricular outflow tract stent. Restenting was done because the infundibular stenosis was very tight. Total repair was completed 1 week after the procedure, and at that time, the patient's hemodynamics had greatly improved. (Level of Difficulty: Advanced.).

Case report: Right atrial appendage hybrid access to bailout a stuck stent from the inferior vena cava of a small child.

A three-month-old baby boy (5. 4 Kg) with pulmonary atresia, subaortic ventricular septal defect (VSD), and patent ductus arteriosus (PDA) was sent for ductal stenting from the femoral vein. The route to the PDA was extremely tortuous and the procedure was complicated with a stent stuck in the abdominal inferior vena cava (IVC). Transfemoral stent recapture was technically laborious and the stent was successfully recaptured across a 10-Fr right atrial appendage (RAA) hybrid access avoiding a cardiopulmonary bypass (CBP). The PDA was subsequently stented for the femoral artery with satisfactory clinical outcomes.

Operability of atrial septal defect with borderline pulmonary vascular resistance index: A study in developing country.

Background: Pulmonary arterial hypertension secondary to atrial septal defect (ASD) is an important determinant of morbidity and mortality in defect closure. We aimed to compare perioperative outcome between preoperative borderline and low pulmonary vascular resistance index (≥4 WU.m2 and <4 WU.m2, respectively) in surgical closure of secundum atrial septal defect with concomitant pulmonary arterial hypertension. Methods and results: This was a single-center retrospective cohort study between January 2015 and January 2020. We classified patients with low and borderline PVRI who underwent ASD closure and recorded the perioperative outcomes. Results: We analyzed a total of 183 patients with atrial septal defect and pulmonary arterial hypertension; 92 patients with borderline PVRI and 91 patients with low PVRI. Borderline pulmonary vascular resistance index was not associated with increased risk of postoperative mortality (p = 0.621; OR0.48, 95% CI 0.04-5.48), but associated with higher risk of overall morbidity in bivariate analysis (p = 0.002; OR3.28, 95% CI 1.5-6.72). Multivariate analysis showed positive association of borderline pulmonary vascular resistance index (p = 0.045; OR2.63, 95% CI 1.02-6.77) and preoperative tricuspid valve gradient ≥64 mmHg (p = 0.034; OR2.77, 95% CI 1.08-7.13) with overall morbidity. Conclusion: There is no difference in incidence of in-hospital mortality between preoperative borderline and low pulmonary vascular resistance index patients. However, preoperative borderline pulmonary vascular resistance index and tricuspid valve gradient ≥64 mmHg are associated with increased overall morbidity after surgical closure in secundum atrial septal defect patients with pulmonary arterial hypertension.

Echocardiography-Guided Percutaneous Patent Ductus Arteriosus Closure: 1-Year Single Center Experience in Indonesia.

Introduction: Since 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. Methods: Patients 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. Results: A 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.

Preoperative Left Ventricle End Diastolic Volume Index as a Predictor for Low Cardiac Output Syndrome After Surgical Closure of Secundum Atrial Septal Defect With Small-Sized Left Ventricle.

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 < 0.001), LVEDV (64.6 ± 16.0 ml vs. 85.9 ± 20.7 ml; p < 0.001), LVSV (38.97 ± 11.5 ml vs. 53.13 ± 7.5 ml; p < 0.001), and LVSVi (27.28 ± 8.55 ml/m2 vs. 37.42 ± 5.35 ml/m2; p < 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.

Pulmonary Arterial Hypertension in Indonesia: Current Status and Local Application of International Guidelines.

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.

Pediatric Cardiothoracic CT Guideline Provided by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group: Part 2. Contemporary Clinical Applications.

The use of pediatric cardiothoracic CT for congenital heart disease (CHD) was traditionally limited to the morphologic evaluation of the extracardiac thoracic vessels, lungs, and airways. Currently, the applications of CT have increased, owing to technological advancements in hardware and software as well as several dose-reduction measures. In the previously published part 1 of the guideline by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group, we reviewed the prerequisite technical knowledge for clinical applications in a user-friendly and vendor-specific manner. Herein, we present the second part of our guideline on contemporary clinical applications of pediatric cardiothoracic CT for CHD based on the consensus of experts from the Asian Society of Cardiovascular Imaging CHD Study Group. This guideline describes up-to-date clinical applications effectively in a systematic fashion.

Clinical Profile and Management of Rheumatic Heart Disease in Children and Young Adults at a Tertiary Cardiac Center in Indonesia.

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.

Balloon pulmonary valvuloplasty in neonates with critical pulmonary stenosis: Jugular or femoral.

BACKGROUND: Critical pulmonary stenosis (PS) is one of the life-threatening congenital heart diseases which present during the neonatal period with cyanosis. Surgical valvotomy was once the procedure of choice for critical PS; however, balloon pulmonary valvuloplasty (BPV) has now become the standard treatment. Although the procedure is usually simple, crossing the pulmonary valve from the femoral vein can be difficult, especially when severe tricuspid regurgitation and right atrium dilatation are present. In such patients, the maneuver can be simplified by using the right internal jugular vein approach. However, many operators are reluctant to use this approach because of unfamiliarity with the technique, potential complications, and paucity of reports. Until now, there is no literature describing BPV using the transjugular approach in neonates, also none directly comparing the transfemoral and transjugular approaches. OBJECTIVE: We compared transjugular with the transfemoral approach in terms of procedure time and complications. MATERIALS AND METHODS: This was a retrospective cohort study. Participants were neonates with critical PS undergoing BPV in the National Cardiovascular Center Harapan Kita from 2013 to 2018. RESULTS: Of 15 neonates undergoing BPV, eight were done using the transjugular approach and seven using the femoral approach. Mean age and weight in both groups was similar. In all eight patients using transjugular approach, crossing the pulmonary valve was consistently quick and easy. The total procedural time, pulmonary crossing time, and fluoro time was significantly shorter using the transjugular approach (65 ± 8 vs. 108 ± 17.8 min, P < 0.05; 22 ± 3.1 vs. 45 ± 14.8 min, P < 0.01; 29 ± 13 vs. 67 ± 35 min, P < 0.05). There were no complications relating to vascular access. Moreover, the BPV procedure itself demonstrated comparable results in both groups. CONCLUSION: BPV using the transjugular approach is safe and effective to relieve critical PS in neonates compared to the transfemoral approach.