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Arrhythmogenic cardiomyopathy (AC) is a broad term denoting subclinical or clinical heart muscle disease which presents predominantly with serious arrhythmias. Heart muscle disease or cardiomyopathy can present as an abnormal electrocardiogram, heart failure, syncope, arrhythmias, or sudden cardiac death. Ventricular arrhythmogenesis is the hallmark of this entity. A small but significant number of athletes experiencing sudden cardiac death have AC. AC has complex and ill-understood pathophysiology. Usually, arrhythmogenic cardiomyopathies are genetic or hereditary. In these patients, muscle hypertrophy, dilatation, cardiac fat metaplasia, and fibrosis provide the anatomic substrate for arrhythmias. Hereditary cardiomyopathies are considered primary diseases of the cardiac myocytes and their intercellular junctions. Secondary diseases of the myocardium due to coronary artery disease, hypertension, valvular heart disease, etc., are excluded from this nomenclature. Channelopathies are also not included in the spectrum of AC. Initially, AC was reported as a single phenotype and labeled arrhythmogenic right ventricular dysplasia, and it was considered a desmosomal disorder. Our current knowledge about this entity has expanded to include multiple variants of this entity with diverse genetic mutations. Its genotypes and phenotypes are still evolving. As of now, distinct desmosomal, cytoskeletal, nuclear, and sarcomeric ACs have been reported. Geography-specific syndromic ACs have also been described. It is a cell-to-cell disjunction cardiomyopathy impairing the inter- and intracellular signal transduction. Gap junctions make AC unique in the sense that arrhythmias precede structural alterations or heart failure in the vast majority. There is also a distinct arrhythmogenic atrial cardiomyopathy. Diagnostic algorithms include morphofunctional, depolarization and repolarization abnormalities, distinct genetic mutations, and typical arrhythmias which serve as the basis of scoring systems devised to diagnose AC. This review discusses salient aspects of AC focusing on imaging observations.

Extraluminal migration of a drug-eluting intracoronary stent into pericardial space with or without aneurysm formation is a rare complication. It is usually symptomatic although rare asymptomatic cases have been described. Herein, we report a 49-year-old male who presented with atypical chest pain 9 years after intracoronary stent implantation in the right coronary artery and the ramus intermediate branch. Echocardiographic examination showed a 7.4 cm × 3.8 cm large oblong cystic mass along the lateral wall of the left ventricle with inward bulging of the adjacent segments. Computed tomography and invasive coronary angiography showed this mass to be a partly thrombosed giant oblong coronary aneurysm arising from the bifurcation of the left main coronary artery with extraluminal migration of the stent from the ramus branch.

Background: Left atrial (LA) volume indexing for body surface area (BSA) is the common practice. Since LA volume index is of cardiovascular pathophysiologic significance, it is suggested that indexing for other body size parameters be explored to evaluate a more appropriate alternative method. The aims of this study were to find normal and the best cutoff values for LA volume indexed for multiple body size parameters in normal Indian subjects. Methods: Data from the multicentric prospective INDEA study conducted through 2018 to 2020 was reviewed and subjects without known cardiac disease and completely normal echocardiograms that had the left atrial volume (LAV) measured by biplane Simpson's method were included. LAV was indexed by BSA (ml/m2), by height (LAV/m), by height raised to exponent 1.72 (mL/m 1.72 and 2.7 (ml/m2.7), by body weight, by ideal body weight (IBW), by ideal body surface area (IBSA) and by height squared (ml/h2). Results: A total of 1046 healthy volunteers (382 female, 38%), mean age 38 ± 10.4 years (range 30–48 years) and body mass index 23.6 kg/m2 (22–25 kg/m2) were analyzed. Mean and normal values were: LAV/BSA 18.7 + 3.15 ml/m2 (range 15–21 ml/m2), LAV/ht 26.0 ± 4.5 ml/m, (range 17–35 ml/m), LAV/ht2 16 ± 2.8 ml/m2 (range 10.4–21.6 ml/m2) and LAV/ht2.7 8.71 ± 2.2 ml/m2.7 (range 6.98–13.58 ml/m2.7). Using ROC curve analysis, LAV/h 1.72 had the highest AUC and the best predictive value to identify LA enlargement but not very different from LAV/BSA. Ideal BSA and ideal body weight as a denominator did not provide any incremental value. Conclusion: Normal values for LAV indexed for height, weight, body surface area by three different methods of height as an allometric parameter are described in normal Indian individuals. We reinforce that LA volume indexation for BSA is an acceptable and robust method in non-obese Indian subjects. Indexing for height 1.72 is probably slightly superior method to evaluate LAV.

A 68-year-old male developed cardiac tamponade secondary to coronary artery perforation following percutaneous coronary artery intervention. He underwent thoracotomy to seal the perforation of the acute marginal branch of the right coronary artery. Following sealing of perforation with application of cyanoacrylate to the right atrioventricular groove, the patient remained stable with minimal pericardial effusion for 1 month and later developed a large (6 cm × 5 cm) intrapericardial hematoma compressing the right ventricular inflow with intermittent fever. The patient declined repeat surgery and showed some regression of hematoma and a decrease in trans-tricuspid pressure gradients with colchicine after 1 month. The case is reported for its rarity with a review of literature.

Isolated congenital aneurysm-like deformity of the muscular interventricular septum is rare and may be associated with other congenital heart defects, ventricular dysfunction, conduction disturbances, and chromosomal abnormalities. It may be a partial or near-total defect. We report a tunnel-like congenital anomaly of the mid-muscular ventricular septum without other congenital anomalies or conduction disturbance in a 17-year-old asymptomatic girl. The partial ventricular septal defect was lined on the outer side by the right ventricular endocardium and showed partial obliteration during systole.

Vildagliptin, a dipeptidyl peptidase-4 (DPP-4) inhibitor is effective in reducing HbA1c levels in patients with type 2 diabetes (T2DM) when administered as monotherapy, dual or triple combination therapy. In India, Vildagliptin is commonly prescribed in T2DM patients because it reduces mean amplitude of glycemic excursion (MAGE), has lower risk of hypoglycemia and is weight neutral. Early combination therapy with vildagliptin and metformin is effective and well-tolerated in patients with T2DM, regardless of age or ethnicity. In view of already existing data on vildagliptin and the latest emerging clinical evidence, a group of endocrinologists, diabetologists and cardiologists convened for an expert group meeting to discuss the role and various combinations of vildagliptin in T2DM management. This practical document aims to guide Physicians and Specialists regarding the different available strengths and formulations of vildagliptin for the initiation and intensification of T2DM therapy.

A neonate with type 1a tricuspid valve atresia with quadrivalvular involvement is described. There were other associated abnormalities as well. Such a combination is extremely unusual and is being reported here for its uniqueness.

Abstract The current recommendations by Indian experts who are focused on the challenges in the management of patients with acute coronary syndrome (ACS) in rural areas, due to limited catheterization (CATH) lab facilities and interventional cardiologist coverage across the country, are described. 120 cardiologist experts drafted recommendations during ten advisory board meetings conducted from April to May 2022. Experts framed statements based on experience, collective clinical judgment from practical experience, and available scientific evidence regarding ACS. The consensus positioned fondaparinux as highly useful in non-CATH-lab-based hospitals for patients diagnosed with non-ST elevation acute coronary syndrome (NSTE-ACS) and ST elevation acute coronary syndrome (STE-ACS) patients who cannot be shifted to percutaneous coronary intervention (PCI)-capable centres, or for patients who are thrombolysed at peripheral centres.

Heart failure with preserved ejection fraction (HFpEF) is a heterogeneous clinical syndrome of effort intolerance based on structural and functional abnormalities of the cardiovascular system (CVS). Its prevalence is increasing progressively in comparison to that of heart failure with reduced ejection fraction due to aging, obesity, metabolic stress, and hypertension. Multiple domains of the CVS and peripheral organs have reduced reserve capacity and increased stiffness in patients with HFpEF. This high-gain CVS exhibits increased filling pressures and reduced filling volumes under stress despite the left ventricular ejection fraction, commonly estimated parameter of contractile performance, being normal, i.e., >50%. The cost of increasing cardiac output in terms of left ventricular filling pressures is increased and their relationship shows an upward and more left-directed slope due to reduced ventricular and arterial compliance. At the tissue level, there is myocytic hypertrophy and increased extracellular matrix with capillary rarefaction. There are many phenogroups of HFpEF based on the heart's ability to secrete natriuretic peptides, degree of dysmetabolism, age, renal function, body fat, rhythm, underlying etiology, and subclinical systolic dysfunction. The left ventricle may be pressure-loaded, volume-loaded, or have equipoise with regard to remodeling. Myocardial performance estimated by parameters other than those based on distance or volume displacement may be abnormal in more than half of the patients underlying the presence of subtle systolic dysfunction. This review looks at myocardial performance and characteristics in HFpEF by deformation imaging using acoustic speckle tracking and its diagnostic and prognostic significance. Research points toward the utility of global longitudinal strain in early detection, biological characterization, and risk stratification of HFpEF. Echocardiographic speckle-tracking-based longitudinal strain analysis represents a method of relatively high value and for sensitive phenotyping of HFpEF which is yet to be utilized optimally. Other dimensions of strain, although extensively studied in HFpEF, do not add much value. The focus is on systolic deformation since there is limited utility of diastolic strain and its rate.

A 27-year-old male was admitted with dyspnea of sudden onset and was initially diagnosed to have acute pulmonary embolism, bilateral pleural effusion, and mild pericardial effusion. Detailed echocardiographic examination revealed echocardiographic features of constrictive pericarditis (CP), marked spontaneous contrast in inferior vena cava, and a right atrial thrombus. The patient had bilateral lung consolidation and pleural effusion presumably of tubercular origin. Intracardiac thrombi are rare in patients with CP and may impact prognosis and management strategy.

This report describes a 68-year-old female who, immediately after the primary percutaneous coronary intervention of the left anterior descending artery for acute anterior myocardial infarction, developed an apical aneurysm and a “neo-chamber” within the left ventricular cavity separated from the main cavity by a linear thick hyperechoic free-floating fenestrated membrane. This was an excoriated but perforated subendocardial spiral myocardial layer separated from the subepicardial layer due to ischemia and infarction. Multiple orifices on the inner surface were observed, possibly due to patchy necrosis. In addition, there was a large remote intramyocardial hematoma in the inferolateral wall of indeterminate pathogenesis.

A 21-year-old girl presented with an episode of ventricular tachycardia in September 1991. She was found to have a large (8 cm × 5 cm) mass in the right ventricle and was operated. The mass was full of thrombi and fresh blood that was evacuated. She remained asymptomatic till May 2022, when she presented with another episode of ventricular tachycardia. She also had a history of effort angina of 2 months' duration. Multimodality imaging evaluation revealed a large calcific cystic mass in the right ventricular free wall with an unruptured aneurysm of the right sinus of Valsalva, a markedly distorted right ventricular cavity, and the Brugada pattern of electrocardiogram. The case is reported for its rarity, long follow-up, and strikingly high-quality images.

PURPOSE: There is a lack of studies evaluating the antimicrobial effect of riboflavin and ultraviolet-A (UVA) radiation using an epithelium-off cross-linking technique (Dresden protocol) in the literature. This study aimed to demonstrate the antimicrobial properties of riboflavin and UVA radiation and combined riboflavin/UVA radiation on various ocular pathogens Dresden protocol. MATERIALS AND METHODS: This was a prospective and comparative study. This study included four groups: Staphylococcus aureus, Pseudomonas aeruginosa, Candida albicans, and Acanthamoeba. All the groups were tested 12 different times with riboflavin alone, UVA light alone, and combined riboflavin with UVA. The growth inhibition zones were measured. Descriptive statistics were expressed as mean and standard deviation. The main outcome was measurement and analysis of growth inhibition zones. RESULTS: The mean growth inhibition zones following riboflavin and UVA exposure were 9.70 ± 1.63 mm for P. aeruginosa and 7.70 ± 1.08 mm for S. aureus. The mean growth inhibition zones for P. aeruginosa were greater than for S. aureus (t = 2.395, P = 0.038). The mean growth inhibition zones for C. albicans and Acanthamoeba were 0. The mean growth inhibition zones following UVA exposure alone and riboflavin alone for all organisms were also 0. CONCLUSION: Combined riboflavin and UVA in the clinical dose was effective against P. aeruginosa and S. aureus but ineffective against C. albicans and Acanthamoeba. Our study shows the potential of combined riboflavin and UVA in the treatment of bacterial keratitis.

Objective Dermatophytoses, one of the most ancient diseases, is becoming a menace in recent times. This has made the knowledge of antifungal susceptibility a priority in today's times. Material and Methods This is a prospective study conducted over 18 months including all dermatophytes isolated during the period. Dermatophytes were identified by routine phenotypic methods. Antifungal susceptibility testing was performed for griseofulvin, terbinafine, and itraconazole as per the Clinical Laboratory Standard Institute M38 A2, and minimum inhibitory concentrations (MICs) were read after 5 days. Results Patient details and associated risk factors were recorded. Fixed dose combinations with steroids were associated with 79.3% (46 out of 58) of patients with dermatophytosis of skin. Among the 72 dermatophytes isolated during the study period, 58 (80.5%) were isolated from skin scrapings and 14 (19.4%) from nail samples. Tinea corporis with cruris was the most common presentation. The most common dermatophyte isolated from skin scrapings was Trichophyton mentagrophytes complex (70.6%, 41 out of 58), while from nail samples it was Trichophyton rubrum complex (78.57%, 11 out of 14). Based on the MIC50 and MIC90 results, itraconazole showed the lowest MICs, followed by terbinafine and then griseofulvin. Conclusion With the changing epidemiology of species distribution and antifungal resistance, there is a need for continuous surveillance of these parameters of dermatophytes.

Transthyretin cardiac amyloidosis (ATTR-CM) is a rare and under-recognized disorder characterized by the aggregation of transthyretin-derived insoluble amyloid fibrils in the myocardium. Heterogeneity of symptoms at presentation, makes its diagnosis often delayed. An expert panel gathered on a virtual platform across India to conduct a meeting for developing a guiding tool for ATTR-CM diagnosis. The panel recommended younger age (≥40 years) for suspecting ATTR-CM and thick-walled non-dilated hypokinetic ventricle was considered as one of the important red flags. Electrocardiogram (ECG) and echocardiography (ECHO) findings were recommended as primary tests to raise the suspicion while nuclear scintigraphy and hematological tests were recommended to confirm the diagnosis and rule out amyloid light-chain (AL) amyloidosis. Cardiac magnetic resonance (CMR) and biopsy were recommended in case of ambiguity in the presence of red flags. Considering the lack of expert guidelines in the Indian scenario, a standardized diagnostic algorithm was also proposed.

Right ventricular myocardial infarction (RVMI) occurs in up to half of the patients of inferior wall myocardial infarction. RVMI results in edema and increased right ventricular (RV) mass, and hence, it should be possible to visualize microvascular obstruction as a manifestation of reperfusion injury. Reperfusion injury of the RV myocardium following primary percutaneous coronary intervention has been rarely reported. This report describes a patient with the left ventricular inferior and RVMI who showed echocardiographically detected reperfusion injury of the RV-free wall and left ventricular basal inferolateral wall and conformed by myocardial contrast perfusion study despite successful percutaneous revascularization of the right coronary artery.

This case report describes a 69-year-old male who presented with acute anterior myocardial infarction and was detected to have a hyperdominant right coronary artery (RCA) with a large posterior descending branch coursing from the posterior interventricular groove into the apical part of the anterior interventricular space and posterior left ventricular branch coursing in the left atrioventricular groove and supplying the entire free wall. Primary percutaneous intervention of the proximally occluded posterior descending artery resulted in resolution of ST-segment elevation in anterior and inferior leads and also revealed the large vascular territory of the RCA. The occurrence of superdominant RCA and intact left circumflex artery presenting with acute anterior wall myocardial infarction has not been described earlier. Precise morphological and physiological knowledge and evaluation of such a variation assists in opting for the best available therapeutic modality and prognosis.

A 63-year-old female with hypertension, evaluated for atypical chest pain and continuous precordial murmur, was found to have an insignificant stenosis in the proximal right coronary artery with a large tortuous fistula originating from the right sinus of Valsalva to the coronary sinus draining into the right atrium with an unusual course parallel to but distinct from the right coronary artery. Multidetector computed tomographic coronary angiography and cardiac catheterization confirmed the diagnosis. Such an anomaly has not been reported earlier.

A 26-year-old female patient who had surgical closure of the ventricular septal defect with an autologous pericardial patch and mitral valve repair for the cleft mitral valve in early childhood at age 4 and then subsequently mitral valve replacement with a bioprosthetic valve in 2011 at the age of 16 years, was found to have a large cystic mass protruding into the right ventricular inflow on echocardiography during a late routine examination in 2012. The cystic mass identified on echocardiography was shown to be an aneurysmal dilatation of the autologous pericardial patch used for the repair of the ventricular septal defect. As the third corrective surgery would have been inherently complicated with risk to the conduction system, she has been followed conservatively for the last 10 years without any significant complaints except mild dyspnea. This case illustrates that the pericardial patch aneurysm is relatively stable and surgery is rarely indicated unless the aneurysm causes mechanical compression or obstruction of the right ventricular inflow or outflow.

This report describes a 62-year-old postmenopausal female patient who presented with cardiogenic shock, wide QRS complete heart block (CHB), left ventricular apical ballooning with severe systolic dysfunction, and normal coronary angiogram. She underwent permanent pacemaker implantation and showed a disappearance of apical dyskinesis and significant improvement in left ventricular function over the next 1 week. CHB was persistent at follow-up. Relevant literature with regard to this unique combination is discussed.

Genetically determined deficiency of connective tissue predisposes to the prolapsed mitral valve and an abnormal fibrous annulus including mitral annulus disjunction and dehiscence of aortomitral annular fibrosa. In a more severe phenotype, there may be abnormal aortic tissue, leading to annuloaortic ectasia. This report describes a 2-year-old male child who presented with failure to thrive and was found to have Barlow's syndrome with bileaflet mitral valve prolapse and mitral annulus disjunction, annuloaortic ectasia, and a pseudoaneurysm of interannular fibrosa. The case is reported for its interesting unifying linkage among various defects.