Long QT syndrome with AV Wenckebaching & bundle branch block in a neonate.

We present a case of 21-day-old neonate brought with history of 3 episodes of syncope. Evaluation revealed congenital long QT syndrome associated with long cycle atypical AV Wenkebaching with a long short cycle sequence related left bundle branch aberrancy. Syncope was attributed to multiple episodes of Torsades de Pointes, necessitating emergency epicardial pacemaker implantation. In addition, child was started on oral propranolol therapy. On 2 months follow up, child was stable with no ventricular high rate episodes during pacemaker interrogation.

Right bundle branch block pattern after uncomplicated right ventricular outflow tract pacing in a patient with a left sided superior vena cava and corrected tetralogy of Fallot.

Usually an electrocardiogram after right ventricular (RV) pacing should yield left bundle branch block (LBBB) pattern. However, the presence of right bundle branch block (RBBB) pattern after pacemaker implantation should alert the physician to a malposition of lead. We report a case of 18-year-old female who underwent dual chamber pacemaker implantation and had RBBB pattern post implantation. Detailed evaluation revealed an uncomplicated right ventricular outflow tract pacing. The possible causes of this abnormal pattern after an uncomplicated RV pacing are also reviewed.

Correlation of pacing site in right ventricle with paced QRS complex duration.

BACKGROUND: Pacing from RV mid septum and outflow tract septum has been proposed as a more physiological site of pacing and narrower paced QRS complex duration. The paced QRS morphology and duration in different RV pacing sites is under continued discussion. Hence, this study was designed to address the correlation of pacing sites in right ventricle with paced QRS complex duration. METHODS: Two hundred fifty-two consecutive patients who underwent pacemaker implantation were enrolled. Baseline clinical characteristics were recorded for each patient. All patient underwent fluoroscopy, electrocardiogram and echocardiography post pacemaker implantation. Paced QRS duration was calculated from the leads with maximum QRS duration. RESULTS: Mean paced QRS (pQRS) duration was significantly higher in apical septum group with a mean of 148.9 ±â€¯14.8 m s compared to mid septum (139.6 ±â€¯19.9 m s; p-value 0.003) and RVOT septum (139.6 ±â€¯14.8 m s; p-value 0.002) groups, respectively. There was no significant difference between mid-septal and RVOT septal pQRS duration. On multivariate analysis, female gender, baseline QRS duration and RVOT septal pacing were the only predictors for narrow pQRS duration (<150 msec). CONCLUSION: RV mid-septal and RVOT septal pacing were associated with significantly lower pQRS duration as compared with apical pacing. Based on multivariate analysis RVOT septal pacing appears to be preferred and more physiological pacing site.

Tilted ECG - An unusual tilt test response.

Tilt testing is a commonly used diagnostic tool to evaluate syncope of undetermined etiology. We hereby describe a twelve year old male child who developed presyncope during provocative head up tilt testing along with ST segment elevation in cardiac monitor during presyncope. Patient underwent repeat head up tilt testing along with holter monitoring which showed a left bundle branch block escape rhythm. Hence development of ST elevation was due to the improper filter application in single lead ECG monitor. This case highlights the importance of filters in electrocardiographic monitoring.

The complex metabolism of trimethylamine in humans: endogenous and exogenous sources.

Trimethylamine (TMA) is a tertiary amine with a characteristic fishy odour. It is synthesised from dietary constituents, including choline, L-carnitine, betaine and lecithin by the action of microbial enzymes during both healthy and diseased conditions in humans. Trimethylaminuria (TMAU) is a disease typified by its association with the characteristic fishy odour because of decreased TMA metabolism and excessive TMA excretion. Besides TMAU, a number of other diseases are associated with abnormal levels of TMA, including renal disorders, cancer, obesity, diabetes, cardiovascular diseases and neuropsychiatric disorders. Aside from its role in pathobiology, TMA is a precursor of trimethylamine-N-oxide that has been associated with an increased risk of athero-thrombogenesis. Additionally, TMA is a major air pollutant originating from vehicular exhaust, food waste and animal husbandry industry. The adverse effects of TMA need to be monitored given its ubiquitous presence in air and easy absorption through human skin. In this review, we highlight multifaceted attributes of TMA with an emphasis on its physiological, pathological and environmental impacts. We propose a clinical surveillance of human TMA levels that can fully assess its role as a potential marker of microbial dysbiosis-based diseases.

South African flag sign to a giant coronary artery aneurysm.

Background: Coronary arteritis leading to aneurysm is one of the unusual presentations of IgG4-related disease. Acute myocardial infarction as a complication of IgG4-related giant coronary artery aneurysm is even rarer. Case summary: We describe the case of a 56-year-old gentleman who presented to our institute with Canadian Cardiovascular Society (CCS) class III angina. His symptoms were persistent even with high-dose antianginal medications. He had an acute coronary syndrome two weeks back for which he was treated conservatively in a peripheral health centre. His 12-lead electrocardiogram at the time of the event was suggestive of high lateral ST-segment elevation myocardial infarction (South African flag sign). His transthoracic echocardiography showed mild left ventricular dysfunction and a large echogenic mass lateral to the left ventricle. Coronary angiography followed by cardiac computed tomography revealed a giant pseudoaneurysm of the proximal and mid-left anterior descending coronary artery. FDG-PET scan showed significant metabolic activity in the aneurysm wall and mediastinal lymph nodes suggesting active inflammation. IgG4-related coronary arteritis was suspected, and the patient underwent aneurysmectomy and coronary artery bypass (CABG) surgery. The histopathology of the resected segment showed diffuse IgG4-secreting plasma cells confirming the diagnosis. Discussion: Atherosclerosis is the most common cause of coronary aneurysms in adults. However, cardiologists should be aware of atypical causes like IgG4-related disease that can even present with acute coronary syndrome. Although multimodality imaging is beneficial during early evaluation, histopathological analysis is the cornerstone for the diagnosis of IgG4-related disease. The management involves both immunosuppressive medication and endovascular or surgical repair.

Delineating the NOX-Mediated Promising Therapeutic Strategies for the Management of Various Cardiovascular Disorders: A Comprehensive Review.

Cardiovascular disorders (CVDs) are reported to occur with very high rates of incidence and exhibit high morbidity and mortality rates across the globe. Therefore, research is focused on searching for novel therapeutic targets involving multiple pathophysiological mechanisms. Oxidative stress plays a critical role in the development and progression of various CVDs, such as hypertension, pulmonary hypertension, heart failure, arrhythmia, atherosclerosis, ischemia-reperfusion injury, and myocardial infarction. Among multiple pathways generating reactive oxygen species (ROS), NADPH defines all abbreviations oxidases of the NOX family as the major source of ROS generation and plays an intricate role in the development and progression of CVDs. Therefore, exploring the role of different NADPH oxidase isoforms in various cardiovascular pathologies has attracted attention to current cardiovascular research. Focusing on NADPH oxidases to reduce oxidative stress in managing diverse CVDs may offer unique therapeutic approaches to prevent and treat various heart conditions. The current review article highlights the role of different NADPH oxidase isoforms in the pathophysiology of various CVDs. Moreover, the focus is also to emphasize different experimental studies that utilized various NADPH oxidase isoform modulators to manage other disorders. The present review article considers new avenues for researchers/scientists working in the field of cardiovascular pharmacology utilizing NADPH oxidase isoform modulators.

Pseudo-aneurysm of mitral aortic intervalvular fibrosa: two case reports.

The fibrous body between the mitral and aortic valve, known as mitral-aortic intervalvular fibrosa (MAIVF) is prone to infection and injury resulting in pseudo-aneurysm formation. Because of its relative rarity, we are far from making any conclusion regarding the natural history and appropriate therapeutic strategy for this condition. We report two cases of this condition with two different and rare etiologies with strikingly different natural courses, providing insight into the natural course and timing of surgery in this rare entity.

Effect of ivabradine vs atenolol on heart rate and effort tolerance in patients with mild to moderate mitral stenosis and normal sinus rhythm.

BACKGROUND: Patients with mitral stenosis become symptomatic at a higher heart rate. We studied the comparative efficacy of heart rate control with ivabradine or atenolol and its effect on effort tolerance in patients with mild-moderate mitral stenosis in normal sinus rhythm. METHODS AND RESULTS: Fifty patients with mild-moderate mitral stenosis in sinus rhythm were randomized to receive ivabradine or atenolol for 4 weeks each in an open-label, randomized, crossover design trial. A 24-hour Holter and treadmill test was performed at baseline and after each active treatment period. In the first treatment period, 23 patients were allocated to ivabradine (22 analyzed), and 27 were allocated to atenolol (26 analyzed). In the second period, all 48 patients were analyzed. Ivabradine increased the mean total exercise time to 500.7 seconds (SD 99.7) from a baseline of 410.3 seconds (SD 115.4), and atenolol increased it to 463.7 seconds (SD 113.1). The point estimate (absolute difference between ivabradine and atenolol) was 35.27 seconds (95% CI 15.24-55.20; P = .0009). The point estimate for decrease in the maximum exercise heart rate and mean heart rate were 7.64/min (95% CI 0.37-15.9; P = .04) and 5.61/min (95% CI 2.51-8.71; P = .0007), respectively. CONCLUSIONS: Ivabradine is more effective than atenolol for effort related symptoms in patients with mild-moderate mitral stenosis and normal sinus rhythm.

Indigenous telemedicine.

We are describing a simple and innovative way of documenting tachycardia. This patient came with video recording of neck pulsation done with the help of mobile phone camera. No other documentation of this tachycardia was available as patient was living in a remote area away from even basic health facilities.

Rate control with ivabradine: angina pectoris and beyond.

The importance of heart rate in human health and disease has been well known to clinicians for quite some time. Recent epidemiologic studies have further strengthened this concept. Modulation of heart rate by pharmacologic as well as non-pharmacologic means has affected cardiovascular mortality and morbidity in various trials and observational studies. Conventional rate-control agents, such as ß-adrenoceptor antagonists (ß-blockers), calcium channel blockers, and digoxin, have contributed greatly to the management of various diseases where heart-rate reduction is required; however, these agents have effects beyond rate control that may be unacceptable. Ivabradine has recently been recognized as a pure heart-rate-reducing agent and is being extensively studied. It is the latest addition to the class of drugs used to control angina. It is indicated in cases of ß-blocker intolerance or when ß-blockers fail to achieve a heart rate of <60 beats/min. The pure heart-rate-reducing effect of ivabradine has also been reported in smaller studies and anecdotal case reports. The theoretical possibilities of the utility of ivabradine are many and have opened up a whole new field of research for the future. The BEAUTIFUL trial enrolled approximately 10,000 patients with coronary artery disease (CAD) and left ventricular dysfunction, with the aim of assessing the effect of ivabradine versus atenolol on various cardiovascular outcomes. Although ivabradine failed to achieve favorable results for primary endpoints, it appeared effective in achieving a favorable secondary endpoint in a subgroup of patients who had a heart rate of >70 beats/min. Other large trials are also underway to assess the effects of ivabradine on heart failure, acute coronary syndromes, CAD, and other cardiovascular disorders. In this review, we discuss the pharmacologic basis of the action of ivabradine and its role in angina control, as well as in other conditions being actively studied or in which a role for ivabradine has been hypothesized.

Spontaneous demonstration of counterclockwise right atrial activation following successful isthmus ablation.

We describe an uncommon case of typical flutter with symptomatic sinus node dysfunction, in which a permanent junctional rhythm developed following ablation of the cavo-tricuspid isthmus. This rhythm activated the right atrium in counter clockwise manner thus providing spontaneous proof of unidirectional isthmus block, a phenomenon that is usually demonstrated by proximal coronary sinus pacing.

Detecting sub-clinical disease activity in patients with chronic rheumatic valvular heart disease.

OBJECTIVE: Valve disease progression in rheumatic heart disease(RHD) is generally attributed to recurrent attacks of acute rheumatic fever(ARF). However, persistence of chronic sub-clinical inflammation remains a plausible but unproven cause. Non-invasive means to identify sub-clinical inflammation may facilitate research efforts towards understanding its contribution to disease progression. METHODS: Patients with chronic RHD, without clinical evidence of ARF, undergoing elective valve surgery were enrolled. Sub-clinical inflammation was ascertained by histological evaluation of left atrial appendage and valve tissue excised during surgery. We assessed the diagnostic utility of Gallium-67 scintigraphy imaging, and inflammatory biomarkers, hsCRP, IL-2, IL-6, Tumor Necrosis Factor-Alpha(TNF-α), Interferon-gamma(IFN-γ), and Serum Amyloid A(SAA), in identifying patients with sub-clinical inflammation. RESULTS: Of the 93 RHD patients enrolled(mean age 34 ± 11 years, 45% females), 86 were included in final analysis. Sub-clinical inflammation was present in 27 patients(31.4%). Patients with dominant regurgitant lesions were more likely to have sub-clinical inflammation compared to those with stenotic lesions, though this association was not statistically significant(dominant regurgitant lesions vs isolated mitral stenosis: OR 3.5, 95%CI 0.68-17.96, p = 0.133). Inflammatory biomarkers were elevated in the majority of patients: hsCRP, IL-2, IL-6, TNF-α, and IFN-γ in 44%, 89%, 90%, 79%, and 81% patients, respectively. However, there was no significant association between biomarker elevation and histologically ascertained sub-clinical inflammation. Ga-67 imaging was unable to identify inflammation in the 15 patients in whom it was performed. CONCLUSION: Sub-clinical inflammation is common in RHD patients. Conventional inflammatory markers are elevated in the majority, but aren't discriminatory enough to identify the presence of histologic inflammation.

Comparison of 68Ga-DOTANOC PET/CT with cardiac MRI in patients with clinical suspicion of cardiac sarcoidosis.

BACKGROUND: 68Ga-DOTA-NaI-octreotide (DOTANOC) is a promising new alternative to 18F-fluorodeoxyglucose (FDG) for imaging inflammation in cardiac sarcoidosis. The aim of the study was to compare 68Ga-DOTANOC positron emission tomography/computed tomography (PET/CT) with cardiac magnetic resonance imaging (CMR) in patients with clinical suspicion of cardiac sarcoidosis. METHODS AND RESULTS: Patients with extracardiac sarcoidosis and clinical suspicion of cardiac involvement underwent 68Ga-DOTANOC cardiac PET/CT, myocardial perfusion single photon emission computed tomography (MPS) and CMR (T2-weighted and delayed gadolinium-enhanced T1-weighted images). The patients were screened using revised criteria of Japanese circulation society. Presence of perfusion defects on MPS, abnormal myocardial uptake on 68Ga-DOTANOC PET/CT and characteristic pattern of late gadolinium enhancement (LGE) with or without T2 hyperintensity on CMR was considered positive. RESULTS: Seventeen patients (13 male and 4 female) were included in the study. Out of the 17 patients, both CMR and PET were positive in 11 and both were negative in 2. In the remaining 4 patients, CMR was positive but PET was normal. Thus, PET and CMR were concordant in 13 (76.5%) patients and discordant in 4 (23.5%). Intermodality agreement was fair (Cohen's kappa = 0.39). CONCLUSION: LGE on CMR is superior to 68Ga-DOTANOC PET/CT for detecting cardiac involvement in sarcoidosis and there is fair concordance between the two. However, since LGE does not specifically differentiate between inflammation and fibrosis, 68Ga-DOTANOC PET/CT may be better than CMR in identifying patients with active inflammation, since it directly targets inflammatory cells and can have a complementary role to CMR.