The association of right ventricular-pulmonary arterial coupling and pulmonary vascular resistance in adult patients with uncorrected atrial septal defect.

BACKGROUND: Atrial septal defects (ASD) are the most common type of adult congenital heart disease (ACHD) associated with a high risk developing of pulmonary arterial hypertension (PAH). ASD closure is not recommended in patients with PAH and Pulmonary Vascular Resistance (PVR) ≥ 5 Wood Unit (WU). Noninvasive methods have been proposed to measure PVR; however, their accuracy remains low. Right Ventricle (RV) - Pulmonary Artery (PA) coupling is defined as the ability of the RV to adapt to high-resistance conditions. Tricuspid Annular Plane Systolic Excursion (TAPSE)/estimated pulmonary artery systolic pressure (ePASP) calculation using echocardiography is a noninvasive technique that has been proposed as a surrogate equation to evaluate RV-PA coupling. Currently, no research has demonstrated a relationship between RV-PA coupling and PVR in patients with ASD. METHODS: The study participants were consecutive eligible patients with ASD who underwent right heart catheterization (RHC) and echocardiography at Hasan Sadikin General Hospital, Bandung. Both the procedures were performed on the same day. RV-PA Coupling, defined as TAPSE/ePASP > 0.31, was assessed using echocardiography. The PVR was calculated during RHC using the indirect Fick method. RESULTS: There were 58 patients with ASD underwent RHC and echocardiography. Among them, 18 had RV/PA Coupling and 40 had RV/PA Uncoupling. The PVR values were significantly different between the two groups (p = 0.000). Correlation test between TAPSE/ePASP with PVR showed moderate negative correlation (r= -0.502, p = 0.001). TAPSE/ePASP ≤ 0.34 is the cutoff point to predict PVR > 5 WU with sensitivity of 91.7% and specificity 63.6%. CONCLUSION: This study showed a moderate negative correlation between TAPSE/ePASP and PVR. TAPSE/ePASP ≤ 0.34 could predict PVR > 5 WU with good sensitivity.

Clinical Presentation and 6-Month Outcomes of Patients with Peripartum Cardiomyopathy in Indonesia.

Background: Due to the rarity of peripartum cardiomyopathy (PPCM) globally, baseline characteristic data for PPCM patients are still scarce. Therefore, this study aims to determine the baseline characteristics and 6-month outcomes of PPCM patients in Indonesia. Methods: From January 2014 to December 2021, all PPCM patients aged ≥18 years who were admitted to Dr. Hasan Sadikin General Hospital in Bandung, Indonesia, participated in this single-center, prospective cohort study. All patients were re-evaluated within 6 months of PPCM diagnosis. Results: A total of 138 patients with PPCM were admitted to Dr. Hasan Sadikin General Hospital in Bandung. The mean age of all patients was 30.4 ± 6.4 years old. Approximately 60% patients were multipara and had preeclampsia. All guideline-directed medical therapy for heart failure was received by most patients, excluding mineralocorticoid receptor antagonists (25.2%) and bromocriptine (14.1%). The neonatal mortality rate was 5.1%. Among those who survived, 61.2% had normal weight, 31.8% had low birth weight, and 7% had very low birth weight. At the 6-month follow-up, 6.7% of the patients died, 63.3% recovered, and 1.9% were rehospitalized. Conclusion: The present study found a high incidence of PPCM in Indonesia. Our patients frequently had preeclampsia, which contributed to the higher rate of miscarriage and low birth weight. Our liberal use of beta-blockers and ACEi/ARB may have contributed to the higher 6-month recovery rate than that in other countries.

A Meta-Analysis of Atrial Septal Defect Closure in Patients With Severe Pulmonary Hypertension: Is There a Room for Poking Holes Amid Debate?

Severe pulmonary arterial hypertension (PAH) associated with atrial septal defect (ASD) poses a challenge to a closure of ASD, particularly severe PAH that persists even after pharmacological therapeutic strategy. Our study was aimed to evaluate this matter. A systematic literature search from several databases was conducted up until August 1st, 2023. A meta-analysis was undertaken on studies that reported hemodynamic measurements in ASD patients with severe PAH before and after closure. The primary objectives were the extent of improvement in all hemodynamic parameters following closure, and the secondary outcomes were major adverse cardiac events (MACEs) during follow-up. Our study comprised 10 studies with a total of 207 participants. Patients were divided into treat-and-repair and straight-to-repair groups based on the therapeutic strategy. Meta-analysis of all studies demonstrated significant improvement in mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), pulmonary vascular resistance index (PVRI), 6-minutes walking distance (6MWD), and lower prevalence of World Health Organization functional classes (WHO fc), particularly in the treat-and-repair strategy subgroup. Additionally, merely 4 of the 156 individuals died from cardiac causes, and only 1 required rehospitalization, indicating a low likelihood of MACEs arising. Our new findings support the notion that effective shunt closure can improve various hemodynamic parameters in carefully chosen patients with noncorrectable ASD-PAH. Further large and prospective observational studies are still warranted to validate these findings.

Eisenmenger syndrome with left main compression syndrome: a case report.

BACKGROUND: Left main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes if left untreated. It presents with typical angina but is frequently mistaken for pulmonary hypertension (PH) symptoms. It is now recognized as one of the few important causes of angina in PH. CASE PRESENTATION: A 37-year-old man with a history of unoperated atrial septal defect and Eisenmenger syndrome came to the outpatient department with a chief complaint of angina on exertion. Electrocardiogram showed regular sinus rhythm with right axis deviation, right ventricular hypertrophy, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete right bundle branch block. Cardiac CT showed compression of the left main coronary artery due to a dilated main pulmonary artery. Therefore, this patient was diagnosed with Eisenmenger syndrome with left main compression due to dilated pulmonary artery. He was treated successfully with IVUS-guided stent implantation. The patient experienced marked improvement in regular activities, with no recurrence of angina symptoms. Angiography 3 months after the procedure revealed good patency of the stent, without significant stenosis. CONCLUSIONS: Left main coronary artery compression is a complication that should be suspected in patients with Eisenmenger syndrome presenting with angina symptoms. Non-invasive modalities are recommended for diagnostic evaluation, but the gold-standard technique remains coronary angiography. The best treatment is not well-established, with either myocardial revascularization or PH treatment, but a left main coronary artery stenting procedure is considered an ideal emergent treatment to provide a better quality of life for patients in this condition.

A Case of Complex Pulmonary Hypertension: the Importance of Diagnostic Investigation.

Pulmonary hypertension (PH) encompasses several heterogeneous groups of multiple diseases characterized by abnormal pulmonary arterial blood pressure elevation. Unrepaired atrial septal defect (ASD) may be associated with pulmonary arterial hypertension (PAH), indicating pulmonary vascular remodeling. Furthermore, unrepaired ASD could also be associated with other conditions, such as left heart disease or thromboembolism, contributing to the disease progression. We present a case of a 61-years-old woman with complex PH comprising several etiologies, which are PAH due to unrepaired Secundum ASD, mitral regurgitation caused by mitral valve prolapse as a group 2 PH, pulmonary embolism (PE) which progress to chronic thromboembolism PH (CTEPH) and post-acute sequelae of SARS Cov-2. We highlighted the importance of diagnostic investigation in PH, which is crucial to avoid misdiagnosis and inappropriate treatment that could be detrimental for the patient.

A case of endocarditis and spondylodiscitis associated with Mycobacterium tuberculosis.

Tuberculosis (TB) is a global health problem, in which the majority of cases occur in population-dense developing countries. Despite advances in various diagnostic TB modalities, extrapulmonary TB remains a challenge due to complexities related to its diagnostic approach. Hereby, we present a rare case of endocarditis and spondylodiscitis associated with Mycobacterium tuberculosis (MTB). This case report highlighted the challenges faced in diagnosing blood culture-negative infective endocarditis (BCNIE). We also emphasized the importance of considering MTB as etiology of BCNIE, particularly in endemic TB areas.

Superior Vena Cava Syndrome due to pericardial hematoma: A case report and mini-review of literature.

Superior vena cava syndrome is a life-threatening condition. Typically, the clinical presentations are gradual; hence, the diagnosis is often delayed until critical compression or obstruction has occurred. Pericardial hematoma is a rare condition that could occur after cardiac surgery. An asymptomatic, 25-year-old female, who underwent surgical atrial septal defect closure 5 days ago, was sent for routine echocardiography examination before discharge. An intrapericardiac hematoma was detected at the right atrium's free wall without any intracardiac hemodynamic consequences. The patient was discharged and planned for monthly evaluation. During follow-up, the intrapericardiac hematoma was expanding. In the third month's follow-up, the patient complained of shortness of breath, headaches, and coughs. Echocardiography evaluation revealed enlarged pericardial hematoma, which compressed the right atrium and superior vena cava orifice, without echo' sign of cardiac tamponade. Computed tomography scan revealed superior vena cava compression by the pericardial hematoma and appearance of the collateral vessel. The patient was diagnosed with superior vena cava syndrome and sent for surgical evacuation. Pericardial hematoma after cardiac surgery should be evaluated meticulously. Chronic expanding hematoma could cause superior vena cava syndrome, which is fatal. Early diagnosis and appropriate treatment are essential in managing this condition.

A fatal case of early prosthetic valve endocarditis caused by multidrug-resistant (MDR) - Sphingomonas paucimobilis.

Sphyngomonas paucimobilis (S. paucimobilis) is a low-pathogenicity, gram-negative bacilli (GNB) that are previously known as an opportunist microorganism. Recent studies have shown that S. paucimobilis is an emerging pathogen causing various infections. Multidrug-resistant GNB has emerged as a major clinical and therapeutic dilemma in various hospital-associated infections. Although rare, S. paucimobilis could be associated with infective endocarditis (IE). Prosthetic valve endocarditis (PVE) is the most severe type of IE, which has high mortality rates despite diagnostic and treatment advances. We report a fatal case of early PVE associated with multidrug-resistant (MDR) - S. paucimobilis complicated with perivalvular abscess, complete heart block, valve detachment, and septic arthritis.

Multiple pericardial hematomas: a case report and mini-review in multimodality imaging.

BACKGROUND: Pericardial hematoma is blood accumulation in the pericardial space. Although rare, it could arise in various conditions, such as after cardiac surgery. Clinical diagnosis of pericardial hematoma is implausible; thus, cardiac imaging plays a pivotal role in identifying this condition. We presented a case of multiple pericardial hematomas, which was found as an incidental finding in post-cardiac surgery evaluation. We highlighted the diagnostic challenge and the key features of multi-modality cardiac imaging in pericardial hematoma evaluation. CASE PRESENTATION: An asymptomatic, 35-years old male, who underwent surgical closure of secundum atrial septal defect (ASD) one month ago, came for routine transthoracic echocardiography evaluation. An intrapericardiac hematoma was visualized at the right ventricle (RV) 's free wall side. Another mass with an indistinct border was visualized near the right atrium (RA). This mass was suspected as pericardial hematoma differential diagnosed with intracardiac thrombus. Cardiac computed tomography (CT) scan showed both masses have an attenuation of 30-40 HU; however, the mass's border at the RA side was still not clearly delineated. Mild superior vena cava (SVC) compression and multiple mediastinal lymphadenopathies were also detected. These findings are not typical for pericardial hematomas nor intracardiac thrombus; hence another additional differential diagnosis of pericardial neoplasm was considered. We pursued further cardiac imaging modalities because the patient refused to undergo an open biopsy. Single-photon emission computer tomography (SPECT)/CT with Technetium-99 m (Tc-99 m) macro-aggregated albumin (MAA) and Sestamibi showed filling defect without increased radioactivity, thus exclude the intracardiac thrombus. Cardiac magnetic resonance imaging (MRI) reveals intrapericardial masses with low intensity of T1 signal and heterogeneously high intensity on T2 signal weighted imaged and no evidence of gadolinium enhancement, which concluded the diagnosis as subacute pericardial hematomas. During follow-up, the patient remains asymptomatic, and after six months, the pericardial hematomas were resolved. CONCLUSION: Pericardial hematoma should be considered as a cause of pericardial masses after cardiac surgery. When imaging findings are atypical, further multi-modality cardiac imaging must be pursued to establish the diagnosis. Careful and meticulous follow-up should be considered for an asymptomatic patient with stable hemodynamic.

Cardiac manifestations, treatment characteristics, and outcomes of paediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2: A systematic review.

BACKGROUND: Paediatric inflammatory multisystem syndrome (PIMS) temporally associated with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) (PIMS-TS) is a rare clinical syndrome associated with a multiorgan system dysfunction, especially acute cardiac injury, and mandates a higher level of care. AIM OF REVIEW: To investigate cardiac manifestations, treatment characteristics, and outcomes of PIMS-TS. KEY SCIENTIFIC CONCEPTS OF REVIEW: Twenty-six studies were included with 1228 pooled subjects, with a mean age of 8.6 years, which were dominated by male gender (53%), and African ethnicity (31%). 732 (38%) patients were reactive on a serological test, and 457 patients (45%) were positive on SARS-CoV-2 RT-PCR. ST-segment abnormalities were the most common ECG findings (16%, n/N: 34/212). Various markers of troponin and the pooled mean of BNP and NT-pro-BNP levels were elevated. Cardiomegaly and pericardial effusion (21.8%, n/N: 164/751) were the most common chest X-ray findings. In echocardiography, the majority of patients' left ventricular ejection fraction was reduced (59.0%, n/N: 180/305), with pericardial effusion/ pericarditis seen the most (17.44%, n/N: 221/1267), and Z score ≥ 2 in 28% (n/N: 42/139). Cardiac MRI findings were consistent with acute myocarditis. Intravenous immunoglobulin, corticosteroids, and vasoactive drugs were frequently utilized. The mean length of stay was 6 days, with most patients (71%, n/N: 834/1163) were admitted to the ICU. However, the overall prognosis was favorable, with 98% alive (n/N: 1235/1260), and more than 50% of patients experienced recovery of left ventricular systolic functions at discharge (116 out of 206 patients).

Pediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2: a New Challenge amid the Pandemic.

Initially, SARS-CoV-2 infection had been reported as a relatively mild case in children than in adults. Nevertheless, recent evidence found that a subset of children then developed a significant systemic inflammatory response that resembles atypical/typical Kawasaki's disease (KD) and toxic shock syndrome. This novel clinical syndrome later identified as pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS). In contrast with KD, PIMS-TS appears to occur in children at an older age with a predominance of gastrointestinal symptoms, hemodynamic instability, and myocardial dysfunction. However, the exact pathomechanism remains to be understood. Nevertheless, the post-viral immunological reaction is postulated to be the underlying mechanistic underpinnings. The multifaceted nature of the PIMS-TS' course underlines the need for early recognition and multispecialty care and management.