Markedly dilated right atrium in an infant: what is the diagnosis?

An 18-month-old boy presented with recurrent episodes of irritability and documented fast heart rate suggestive of supraventricular tachycardia. Cardiovascular examination revealed significant cardiomegaly, normal heart sounds and no murmurs. The differential diagnosis of marked right atrial dilatation and management principles of idiopathic dilatation of the right atrium are described.

Automatic identification of hypertension and assessment of its secondary effects using artificial intelligence: A systematic review (2013-2023).

Artificial Intelligence (AI) techniques are increasingly used in computer-aided diagnostic tools in medicine. These techniques can also help to identify Hypertension (HTN) in its early stage, as it is a global health issue. Automated HTN detection uses socio-demographic, clinical data, and physiological signals. Additionally, signs of secondary HTN can also be identified using various imaging modalities. This systematic review examines related work on automated HTN detection. We identify datasets, techniques, and classifiers used to develop AI models from clinical data, physiological signals, and fused data (a combination of both). Image-based models for assessing secondary HTN are also reviewed. The majority of the studies have primarily utilized single-modality approaches, such as biological signals (e.g., electrocardiography, photoplethysmography), and medical imaging (e.g., magnetic resonance angiography, ultrasound). Surprisingly, only a small portion of the studies (22 out of 122) utilized a multi-modal fusion approach combining data from different sources. Even fewer investigated integrating clinical data, physiological signals, and medical imaging to understand the intricate relationships between these factors. Future research directions are discussed that could build better healthcare systems for early HTN detection through more integrated modeling of multi-modal data sources.

Ebstein's anomaly: an electrophysiological perspective.

Ebstein's anomaly of the tricuspid valve (EA) is an uncommon congenital cardiac malformation. It can present with atrioventricular tachycardia (AVRT), atrioventricular nodal re-entrant tachycardia (AVNRT), atrial arrhythmias, and rarely with ventricular tachycardia. The 12-lead electrocardiogram (ECG) is critically important and often diagnostic even prior to an electrophysiology study (EPS). Due to its complex anatomy, it poses particular challenges for mapping and ablation, even for an experienced electrophysiologist. In this review, we aim to provide insight into the electrophysiological perspective of EA and an in-depth analysis of the various arrhythmias encountered in diverse clinical scenarios.

Prevalence of metabolic syndrome and its association with atrial arrhythmias in patients with implanted permanent pacemaker for cardiac conduction abnormality.

The objective of the study was to find the prevalence of metabolic syndrome along with identifying the atrial arrhythmias, QTC interval, and coronary artery disease among these patients during follow-ups. Among 171 subjects who were implanted with permanent pacemakers, metabolic syndrome was present in 90 (52.6 %). Prevalence of Arrhythmias was 49 (28.7 %), atrial tachycardia (AT)/atrial fibrillation (AF) was seen in 29 (17 %) patients. Our study showed that there is a strong association between metabolic syndrome and atrial arrhythmias. Metabolic syndrome, age, coronary artery disease and Systolic blood pressure were good independent predictors of atrial arrhythmias among patients with pacemaker implantation.

Left cardiac sympathetic denervation in children with Jervell Lange-Nielsen syndrome and drug refractory torsades - A case series.

INTRODUCTION: Long QT syndrome is an inherited malignant channelopathy which leads to life-threatening arrhythmia, with multiple genotypes. Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive subtype of this disease, characterized by congenital sensorineural deafness and a high incidence of sudden cardiac death (SCD). METHODOLOGY: We prospectively followed up six children who underwent left cardiac sympathetic denervation (LCSD) for JLNS in view of high-risk features despite being on maximally tolerated doses of oral propranolol. RESULTS: Mean age at diagnosis was 2.75 ± 0.39 years, with a significant delay between onset of symptoms and diagnosis (mean 7.2 ± 3.5 months). All had sensorineural hearing loss, conforming to the JLNS phenotype. Mean QTc interval was 603 ± 93 ms, with T wave alternans (TWA) seen in all cases. All were started on propranolol and subsequently subjected to LCSD, and 3 underwent AAI permanent pacemaker implantation. Over a mean follow-up of 20 months, there was a significant reduction in QTc (603 ± 93 ms to 501 ± 33 ms, p = .04), which was persistent on follow-up (525 ± 41 ms) and only two out of six had persistent T wave alternans on ECG (p < .01). None of these children had presyncope, syncope, seizures, torsades de pointes, cardiac arrest or death on follow up following LCSD. CONCLUSION: Jervell Lange-Nielsen syndrome is a subtype of LQTS with high-risk features. LCSD, an effective therapeutic option for those having symptoms despite being on propranolol, results in significant reduction of QTc interval and amelioration of symptoms.

Utility of late gadolinium enhancement on magnetic resonance imaging in hypertrophic cardiomyopathy patients in an Indian cohort.

This prospective observational study sought to correlate segmental late gadolinium enhancement (LGE) seen in cardiac magnetic resonance imaging with occurrence of ventricular arrhythmias (VAs) in patients with hypertrophic cardiomyopathy. LGE was assessed in a 17-segmental model of heart. Of 57 patients, VAs were present in 26.3% of patients and 10.5% had sustained ventricular tachycardia. LGE was present in 43.9% of patients. Presence of LGE in 4 or more segments was associated with VAs with a sensitivity of 73% and specificity of 76% with area under curve of 0.733 in C-statistics.

Mitigation of Fibrosis after Myocardial Infarction in Rats by Using a Porcine Cholecyst Extracellular Matrix.

Fibrosis that occurs after nonfatal myocardial infarction (MI) is an irreversible reparative cardiac tissue remodeling process characterized by progressive deposition of highly cross-linked type I collagen. No currently available therapeutic strategy prevents or reverses MI-associated fibrotic scarring of myocardium. In this study, we used an epicardial graft prepared of porcine cholecystic extracellular matrix to treat experimental nonfatal MI in rats. Graft-assisted healing was characterized by reduced fibrosis, with scanty deposition of type I collagen. Histologically, the tissue response was associated with a favorable regenerative reaction predominated by CD4-positive helper T lymphocytes, enhanced angiogenesis, and infiltration of proliferating cells. These observations indicate that porcine cholecystic extracellular matrix delayed the fibrotic reaction and support its use as a potential biomaterial for mitigating fibrosis after MI. Delaying the progression of cardiac tissue remodeling may widen the therapeutic window for management of scarring after MI.

Outcomes of neonatal critical congenital heart disease: results of a prospective registry-based study from South India.

OBJECTIVES: Congenital heart disease (CHD) is now a leading contributor of infant and neonatal mortality in many low/middle-income countries including India. We established a prospective neonatal heart disease registry in Kerala to understand presentation of CHD, proportion of newborns with critical defects who receive timely intervention, outcomes at 1 month, predictors of mortality and barriers to timely management. METHODS: The congenital heart disease registry for newborns (≤28 days) in Kerala (CHRONIK) was a prospective hospital-based registry involving 47 hospitals from 1 June 2018 to 31 May 2019. All CHDs, except small shunts with a high likelihood of spontaneous closure, were included. Data on demographics, complete diagnosis, details of antenatal and postnatal screening, mode of transport and distance travelled and need for surgical or percutaneous interventions and survival were collected. RESULTS: Of the 1474 neonates with CHD identified, 418 (27%) had critical CHD, 22% of whom died at 1 month. Median age at diagnosis of critical CHD was 1 (0-22) day. Pulse oximeter screening identified 72% of critical CHD and 14% were diagnosed prenatally. Only 8% of neonates with duct-dependent lesions were transported on prostaglandin. Preoperative mortality accounted for 86% all deaths. On multivariable analysis, only birth weight (OR 2.7; 95% CI 2.1 to 6.5; p<0.0005) and duct-dependent systemic circulation (OR 6.43; 95% CI 5 to 21.8, p<0.0005) were predictive of mortality. CONCLUSIONS: While systematic screening, especially pulse oximetry screening, enabled early identification and prompt management of a significant proportion of neonates with critical CHD, important health system challenges like low use of prostaglandin need to be overcome to minimise preoperative mortality.

Electroconvulsive Therapy in Patients With Cardiac Implantable Electronic Devices: A Case Report and Systematic Review of Published Cases.

OBJECTIVE: The aims of the study were to report the case of a 54-year-old man with recurrent depressive disorder with multiple medical comorbidities having a dual-chamber pacemaker, treated successfully with 11 sessions of electroconvulsive therapy, and to conduct a systematic review of published cases documenting the use of electroconvulsive therapy (ECT) in patients with cardiac implantable electronic devices (CIEDs) for treating major psychiatric disorders. METHODS: We searched electronic databases (MEDLINE, PubMed, Google Scholar, Embase, Cochrane Library, PsycINFO, and Crossref) and included studies reporting on the use of electroconvulsive therapy in patients with CIEDs. RESULTS: Thirty-five publications across 53 years (1967-2021) reported on 76 patients (including current report) who received a pooled total of 979 modified ECT sessions. The most common adverse events were premature ventricular contraction and hypertension. There have been no reports of serious adverse effects that necessitated the cessation of ECT. CONCLUSIONS: Electroconvulsive therapy is a safe and efficacious treatment for major psychiatric disorders, and the presence of CIEDs should not delay or deter the use of ECT in these patients.

Novel Radiomics Features for Automated Detection of Cardiac Abnormality in Patients with Pacemaker.

Cardiac pacemakers are used in the treatment of patients with symptomatic bradycardia. The pacemaker paces the heart at the predetermined rate to maintain uninterrupted cardiac activity. Usually, pacemaker lead will be connected to the right atrium (RA) and right ventricle (RV) in dual-chamber pacemaker implantation and RV alone in single-chamber pacemaker implantation. This alters the route of proper conduction across the myocardial cells. The cell-to-cell conduction transmission in pacing delays the activation of selected intraventricular myocardial activation. Pacing-induced cardiomyopathy (PICM) is most commonly defined as a drop in left ventricle ejection fraction (LVEF) in the setting of chronic, high-burden right ventricle (RV) pacing. Currently, very few effective treatments are standard for PICM which rely on the detection of the RV pacing. Such treatments have primarily focused on upgrading to cardiac resynchronization therapy (CRT) when LVEF has dropped. However, the early and accurate detection of these stress factors is challenging. Cardiac desynchrony and interventricular desynchrony can be determined by various echocardiographic techniques, including M-mode, Doppler method, tissue Doppler method, and speckle tracking echocardiography which is subjective measures and shows a significant difference between RV and LV preejection period where the activation of LV is delayed considerably. Computer-aided diagnosis (CAD) is a noninvasive technique that can classify the ultrasound images of the heart in pacemaker-implanted patients and healthy patients with normal left ventricular systolic function and further detect the variations in pacemaker functions in its early stage using heart ultrasound images. Developing such a system requires a vast and diverse database to reach optimum performance. This paper proposes a novel CAD tool for the accurate detection of pacemaker variations using machine learning models of decision tree, SVM, random forest, and AdaBoost. The models have been used to extract radiomics features in terms of textures and then screened by their Relief-F scores for selection and ranking to be classified into nine groups consisting of up to 250 radiomics features. Ten best features were fed to the machine learning models. The R-wave dataset achieved a maximum test performance accuracy of 97.73% with four features in the random forest model. The T-wave dataset achieved a maximum test performance accuracy of 96.59% with three features in the SVM model. Our experimental results demonstrate the system's robustness, which can be developed as an early and accurate detection system for pacing-induced cardiomyopathy.

A porcine cholecystic extracellular matrix conductive scaffold for cardiac tissue repair.

Cardiac tissue engineering using cells, scaffolds or signaling molecules is a promising approach for replacement or repair of damaged myocardium. This study addressed the contemporary need for a conductive biomimetic nanocomposite scaffold for cardiac tissue engineering by examining the use of a gold nanoparticle-incorporated porcine cholecystic extracellular matrix for the same. The scaffold had an electrical conductivity (0.74 ± 0.03 S/m) within the range of native myocardium. It was a suitable substrate for the growth and differentiation of cardiomyoblast (H9c2) as well as rat mesenchymal stem cells to cardiomyocyte-like cells. Moreover, as an epicardial patch, the scaffold promoted neovascularisation and cell proliferation in infarcted myocardium of rats. It was concluded that the gold nanoparticle coated cholecystic extracellular matrix is a prospective biomaterial for cardiac tissue engineering.

Point density exclusion mapping-A useful tool for mapping arrhythmias arising from the endocavitary structures.

Ventricular tachycardia arising from the papillary muscles and other endocavitary structures are preferably ablated under intracardiac echocardiographic (ICE) guidance whenever feasible. However, the availability, need of trained operators, and the expenses involved restrict the routine use of ICE in many cath labs. Point density exclusion (PDX) mapping is a simple technique that doesn't demand any additional expense or tool apart from the routine electroanatomical mapping and thus can be widely applied in mapping of arrhythmias arising from endocavitary structures. The following report describes such a case and explains the method of performing PDX mapping.

Fragmented QRS on electrocardiography as a predictor of myocardial scar in patients with hypertrophic cardiomyopathy.

Background: Fragmented QRS (fQRS) and Q waves are ECG findings in patients with myocardial scar. fQRS is more sensitive than pathological Q waves in detecting myocardial fibrosis in patients with coronary artery disease (CAD). Cardiac magnetic resonance (CMR) imaging is used for the diagnosis and for quantifying scar tissue in patients with HCM. Our aim was to correlate ECG parameters like fQRS and Q waves with the presence of late gadolinium enhancement (LGE) assessed by contrast CMR imaging to elucidate ECG markers which might predict scar tissue in HCM.Methods: This study is a retrospective analysis which included 39 patients who were diagnosed/suspected to have HCM on echocardiography and referred for contrast CMR imaging at our centre between 2010 and 2016. Presence of fQRS was correlated with scar demonstrated by LGE on CMR.Results: Twenty four (66.67%) patients had asymmetrical septal hypertrophy, 7 (19.44%) patients had apical involvement while 5 (13.89%) had concentric pattern. Only 4 (11.11%) patients had pathological Q waves in contiguous leads on surface ECG while fQRS in two contiguous leads was present in 23 (63.89%) patients. Presence of fQRS was more in patients with LGE on CMR than those without (84.61 versus 10%, p<.001). When presence of LGE in specific segments (anterior, lateral and inferior) was correlated with corresponding ECG leads, all the three segments showed significant correlation. The overall sensitivity, specificity, PPV and NPV of fQRS for predicting scar tissue were 84.6, 90.0, 95.6 and 69.2%, respectively.Conclusion: fQRS on surface ECG can be used as an indirect marker to predict the presence of fibrosis in HCM.

What are the atrioventricular delays in right ventricular apical and septal pacing for optimal hemodynamics in patients with normal left ventricular function?

INTRODUCTION: There is a surge of interest in alternate site pacing to prevent pacing-induced left ventricular dysfunction. However, little is known regarding the appropriate atrioventricular (AV) delay between right ventricular (RV) septal and RV apical pacing for optimal hemodynamic benefit. OBJECTIVES: To determine the programmed values of atrial sensed and atrial paced AV delays in basal RV septal and apical RV pacing that results in the maximum delivered stroke volume (SV). METHODS: We calculated the Doppler-derived SV at various sensed and paced AV delays in 50 patients with complete AV block implanted with a dual-chamber pacemaker (group A: 25 RV apical pacing; group B: 25 RV septal pacing). The hemodynamic difference in terms of the SV between sensed and paced AV delay, corresponding to the site of RV pacing was then compared for statistical significance. RESULTS: In group A, maximal SV was derived at a sensed AV delay of 123.2 ± 11 ms and paced AV delay of 129.2 ± 10 ms, and in group B, at a sensed AV delay of 123.6 ± 8 ms and paced AV delay of and 132.8 ± 7 ms. At these intervals, there was no difference in the SV between septal and apical RV pacing (P = .28 and .22, respectively). CONCLUSION: The atrial sensed and atrial paced AV delays for septal and apical RV pacing for optimal hemodynamics are similar. For optimal hemodynamics, the atrial paced AV delay is longer than the atrial sensed AV delay.

A novel prediction model for risk stratification in patients with a type 1 Brugada ECG pattern.

BACKGROUND: Risk stratification in Brugada syndrome remains a controversial and unresolved clinical problem, especially in asymptomatic patients with a type 1 ECG pattern. The purpose of this study is to derive and validate a prediction model based on clinical and ECG parameters to effectively identify patients with a type 1 ECG pattern who are at high risk of major arrhythmic events (MAE) during follow-up. METHODS: This study analysed data from 103 consecutive patients with Brugada Type 1 ECG pattern and no history of previous cardiac arrest. The prediction model was derived using logistic regression with MAE as the primary outcome, and patient demographic and electrocardiographic parameters as potential predictor variables. The model was externally validated in an independent cohort of 42 patients. RESULTS: The final model (Brugada Risk Stratification [BRS] score) consisted of 4 independent predictors (1 point each) of MAE during follow-up (median 85.3 months): spontaneous type 1 pattern, QRS fragments in inferior leads≥3,S wave upslope duration ratio ≥ 0.8, and T peak - T end ≥ 100 ms. The BRS score (AUC = 0.95,95% CI 0.0.92-0.98) stratifies patients with a type 1 ECG pattern into low (BRS score ≤ 2) and high (BRS score ≥ 3) risk classes, with a class specific risk of MAE of 0-1.1% and 92.3-100% across the derivation and validation cohorts, respectively. CONCLUSIONS: The BRS score is a simple bed-side tool with high predictive accuracy, for risk stratification of patients with a Brugada Type 1 ECG pattern. Prospective validation of the prediction model is necessary before this score can be implemented in clinical practice.

Short-term response to phenytoin sodium in Andersen-Tawil syndrome-1 with a cardiac-dominant phenotype.

BACKGROUND: Andersen-Tawil syndrome (ATS) is a rare familial periodic paralysis that typically also affects the heart and skeletal system. Ventricular arrhythmias (VAs) are profound and difficult to control, but minimally symptomatic. In this report, we describe an atypical phenotype of ATS in two related families. We also report our experience with phenytoin sodium for the control of resistant VAs in these patients. METHODS AND RESULTS: Between 2014 and 2018, seven siblings were diagnosed with ATS on the basis of cardiac arrhythmias and genetic evaluation. Heterozygous mutation with c.431G > C (p.G144A) in exon 2 of KCNJ2 gene was observed in all patients. Characteristic cardiac manifestations were noted in all patients but periodic paralysis or objective neurological involvement was distinctly absent. Phenytoin was considered for control of symptomatic VA in three patients. Intake of oral phenytoin (5 mg/kg/day) for 1 month completely suppressed VA (<1% in 24-h Holter monitoring) in two patients, and significantly in the third (8% per 24 h) patient. Phenytoin was well-tolerated in all three patients. CONCLUSIONS: We describe a cardiac-predominant phenotype in ATS. ATS should be suspected in patients with typical cardiac manifestations even in the absence of periodic paralysis. Our initial experience with short-term use of phenytoin for control of resistant VAs is encouraging.