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.

Effectiveness of enteral ivabradine for heart rate control in septic shock: A randomised controlled trial.

Persistent tachycardia in patients with septic shock predicts poor outcome. This study sought to investigate the effect of the cardiac pacemaker current inhibitor ivabradine on heart rate and cardio-circulatory function in patients with septic shock. After informed consent, 60 patients with septic shock and persistent tachycardia (heart rate >95 /minute) were prospectively randomly assigned to receive either standard therapy for septic shock (group S) or standard therapy along with enteral ivabradine (group I) for the initial 96 hours after enrolment. Primary outcome was the difference in heart rate between the two groups during the first 96 hours. Secondary outcomes included the effect of ivabradine on haemodynamic, oxygenation, myocardial function and organ function parameters, incidence of adverse events and 30-day overall survival. Heart rate was lower in group I compared to group S (median difference in area under the curve -25.6 (95% confidence intervals -31.4 to -15.9) /minute; P <0.001). Vasopressor requirements, blood lactate levels, Sequential Organ Failure Assessment scores and E/e' ratio were lower in group I compared to group S. Stroke volume index and ejection fraction were higher in group I while cardiac index and oxygen delivery parameters were maintained similar to group S. There was no difference in 30-day mortality or in the incidence of serious adverse events. Enteral ivabradine is effective in reducing heart rate, and improving haemodynamic parameters and cardiac function in patients with septic shock and persistent tachycardia, without increasing the incidence of adverse events.

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.

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.

Wide-complex tachycardia in a patient with old myocardial infarction.

An elderly man with a history of coronary artery disease presented with sudden-onset palpitations. The electrocardiogram showed wide complex tachycardia with left bundle branch morphology which terminated spontaneously. Subsequent ECGs confirmed the diagnosis as atrial fibrillation with aberrant conduction because of a diseased left bundle.

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.

Incidence and predictors of pacemaker-induced cardiomyopathy with comparison between apical and non-apical right ventricular pacing sites.

BACKGROUND: Asynchronous activation of left ventricle (LV) due to chronic right ventricular (RV) pacing has been known to predispose to LV dysfunction. The predictors of LV dysfunction remain to be prospectively studied. This study was designed to follow up patients with RV pacing to look for development of pacing-induced cardiomyopathy (PiCMP), identify its predictors and draw comparison between apical vs non-apical RV pacing sites. METHODS: Three hundred sixty-three patients undergoing dual-chamber and single-chamber ventricular implants were enrolled and followed up. Baseline clinical parameters; paced QRS duration and axis; RV lead position by fluoroscopy; LV ejection fraction (LVEF) by Simpson's method on transthoracic echocardiography (TTE); intraventricular dyssynchrony (septal-posterior wall contraction delay) and interventricular dyssynchrony (aortopulmonary ejection delay) on TTE were recorded. The patients were followed up at 6-12 monthly interval with estimation of LVEF and pacemaker interrogation at each visit. Pacemaker-induced cardiomyopathy (PiCMP) was defined as a fall in ejection fraction of 10% as compared to the baseline LVEF. Patients developing PiCMP were compared to other patients to identify predictors. RESULTS: The mean age of study population was 59.8 years, 68.3% being males. Fifty-one percent and 49% patients underwent VVIR and DDDR pacemaker implantation, respectively. After attrition, 254 patients were analysed. PiCMP developed in 35 patients (13.8%) over a mean follow-up of 14.5 months. After multivariate analysis, burden of ventricular pacing > 60% [HR 4.26, p = 0.004] and interventricular dyssynchrony (aortopulmonary ejection delay > 40 msec) [HR 3.15, p = 0.002] were identified as predictors for PiCMP in patients undergoing chronic RV pacing. There was no effect of RV pacing site (apical vs non-apical) on incidence of PiCMP [HR 1.44, p = 0.353). CONCLUSIONS: Incidence of PiCMP with RV pacing was found to be 13.8% over a mean follow-up of 14.5 months. Burden of right ventricular pacing and interventricular dyssynchrony were identified as the most important predictors for the development of PiCMP. Non-apical RV pacing site did not offer any benefit in terms of incidence of PiCMP over apical lead position.

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.

Prognostic significance of fragmented QRS in patients with ST-elevation myocardial infarction undergoing revascularization.

BACKGROUND & OBJECTIVES: This longitudinal study was carried out to evaluate the prognostic significance of fragmented QRS (fQRS) in patients with acute ST elevation myocardial infarction (STEMI) undergoing revascularization. METHODS: This study included 103 STEMI patients belonging to Killip class I and II who underwent primary revascularization. All patients underwent twelve lead ECG at admission before PCI. Serial ECG were done after PCI at 3 hours, 6 hours, 24 hours, 48 hours and at discharge for detection of fQRS and echocardiography on day 3 post revascularization. Patients developing fQRS within 48 hours and with persistence of fQRS till discharge were included in "persistent fQRS" group. They were followed up after 30 days for major adverse cardiac events (MACE) and assessment of LV function by echocardiography. RESULTS: fQRS was present in 64 patients (61.5%) of study population with 37 patients (57.8%) having persistent fQRS. MACE rates were low (4.8%) and did not differ with respect to fQRS. fQRS significantly correlated with LV dysfunction at 30 days on univariate analysis (p-0.003) but not on multivariate analysis (p -0.10). fQRS was significantly related to impaired myocardial reperfusion as assessed by ΣSTR (percent of total ST segment resolution) (adjusted odds ratio, 95% CI [4.265 (1.034 - 17.58)], p = 0.04). CONCLUSION: In our study, fQRS did not predict MACE and LV dysfunction in acute STEMI patients belonging to Killip class I and II on short term follow-up of 30 days. But, fQRS independently predicted impaired microvascular myocardial reperfusion as assessed by ΣSTR.

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.

Clinical significance of intracoronary thrombus aspirated during primary percutaneous intervention: An immunohistopathological study.

BACKGROUND: Manual thrombus aspiration during primary percutaneous intervention provides us with aspirated thrombus sample, that may contain material from the disrupted plaque. Immunohistopathological analysis of thrombus can yield valuable information about the clinical and cardiovascular outcomes and possible mechanisms of myocardial infarction. MATERIAL AND METHODS: We studied and analysed the immunohistopathological features of coronary thrombus aspirated from patients undergoing primary percutaneous coronary angioplasty. Immunohistological staining included markers namely CD68, SMA and CD34 for macrophages, smooth muscle actin and endothelium, respectively. Major adverse cardiac events, angiographic outcome and infarct size were also noted. RESULTS: Fifty-three patients (Mean age - 51.3±13years; males-47) who underwent primary percutaneous coronary intervention with aspiration thrombectomy were enrolled. Thrombus was successfully aspirated in 40 of 53 patients (75.4%). Patients with successful thrombus aspiration had higher ST-segment resolution (≥50%) as compared to patients with failed thrombus aspiration. Presence of RBC-rich thrombus on microscopy was more commonly associated with post-procedure TIMI flow of <2 as compared to patients with fibrin-rich thrombus and a trend towards lower myocardial blush grade<2 (P=0.10), and a significantly higher final infarct size (37.5±5% vs 25±15%; P=0.04 of myocardium) on nuclear scan. Immunohistology revealed presence of plaque material in 72% (26/36) of the samples. CONCLUSIONS: Immunohistopathological evaluation of intracoronary thrombus may be of prognostic importance. High prevalence of plaque material in the aspirated intracoronary thrombus suggests plaque rupture as a possible etiology for vessel occlusion in these patients. SHORT SUMMARY: Immunohistopathological evaluation of intracoronary thrombus reveals high prevalence of plaque material in the aspirated intracoronary thrombus suggesting plaque rupture as a possible etiology for vessel occlusion in Indian STEMI patients.

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 Metabolite Trimethylamine-N-Oxide is an Emergent Biomarker of Human Health.

Trimethylamine-N-oxide (TMAO) is a low molecular weight metabolite whose production is dependent on metabolism of its precursors choline, carnitine, creatinine, betaine or lecithin by host gut microbes resulting in the synthesis of trimethylamine (TMA), which is subsequently oxidized to TMAO via hepatic flavin monooxygenase (FMO). TMAO is associated with microbial dysbiosis and is being studied for its linkage with cardiovascular disorders. In addition, dysregulated levels of TMAO have been linked with renal diseases, neurological disorders and cancer. Here we discuss the enzymatic and metabolic landscape that results in TMAO production, and in addition, collate data from numerous clinical studies that have assessed TMAO as a biomarker for various disease conditions. We also summarize the interaction of TMAO with modern and traditional drugs that together affect circulating TMAO levels in the human body.

Development of macaronic Hindi-English 'Hinglish' text message content for a coronary heart disease secondary prevention programme.

BACKGROUND: Coronary heart disease (CHD) is a leading cause of morbidity and mortality in India. Text message based prevention programs have demonstrated reduction in cardiovascular risk factors among patients with CHD in selected populations. Customisation is important as behaviour change is influenced by culture and linguistic context. OBJECTIVES: To customise a mobile phone text message program supporting behaviour and treatment adherence in CHD for delivery in North India. METHODS: We used an iterative process with mixed methods involving three phases: (1) Initial translation, (2) Review and incorporation of feedback including review by cardiologists in India to assess alignment with local guidelines and by consumers on perceived utility and clarity and (3) Pilot testing of message management software. RESULTS: Messages were translated in three ways: symmetrical translation, asymmetrical translation and substitution. Feedback from cardiologists and 25 patients was incorporated to develop the final bank. Patients reported Hinglish messages were easy to understand (93%) and useful (78%). The software located in Australia successfully delivered messages to participants based in Delhi-surrounds (India). CONCLUSIONS: Our process for customisation of a text message program considered cultural, linguistic and the medical context of potential participants. This is important in optimising intervention fidelity across populations enabling examination of the generalisability of text message programs across populations. We also demonstrated the customised program was acceptable to patients in India and that a centralised cross-country delivery model was feasible. This process could be used as a guide for other groups seeking to customise their programs. TRIAL REGISTRATION NUMBER: TEXTMEDS Australia (Parent study)-ACTRN 12613000793718.

Pulmonary arteriovenous malformation in chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension is a morbid condition associated with complications such as hemoptysis, right heart failure, paradoxical embolism, and even death. There is no known association of chronic thromboembolic pulmonary hypertension with pulmonary arteriovenous malformation. Possible hypothesis for this association is an increased pulmonary vascular resistance leading to the compensatory formation of pulmonary arteriovenous malformation. We present one such case presenting with hemoptysis that was managed with endovascular treatment.