Epilepsy and Brugada Syndrome: Association or Uncommon Presentation?

Brugada syndrome (BrS) is a rare genetic disease, of which its clinical manifestations include, but not limited to, syncope or sudden cardiac death. A 30-year-old Bangladeshi male patient with a past medical history of epilepsy was admitted following successful resuscitation from an out of hospital cardiac arrest secondary to ventricular fibrillation. Electrocardiogram (ECG) upon admission was suggestive of BrS type I. His old medical record showed similar ECG 2 months earlier when he had presented with syncope and was diagnosed with seizure. The correlation between BrS and epilepsy has been reported in the literature, discussing whether seizure is an uncommon presentation of BrS or whether epilepsy and BrS share similar genetic mutations that have the potential to cause both arrhythmia and seizures in some patients. Patients who present with seizure and ECG suggestive of Brugada pattern should be evaluated to rule out associated or underlying cardiac arrhythmia.

Visualizing Localized Reentry With Ultra-High Density Mapping in Iatrogenic Atrial Tachycardia: Beware Pseudo-Reentry.

BACKGROUND: The activation pattern of localized reentry (LR) in atrial tachycardia remains incompletely understood. We used the ultra-high density Rhythmia mapping system to study activation patterns in LR. METHODS AND RESULTS: LR was suggested by small rotatory activations (carousels) containing the full spectrum of the color-coded map. Twenty-three left-sided atrial tachycardias were mapped in 15 patients (age: 64±11 years). 16 253±9192 points were displayed per map, collected over 26±14 minutes. A total of 50 carousels were identified (median 2; quartiles 1-3 per map), although this represented LR in only n=7 out of 50 (14%): here, rotation occurred around a small area of scar (<0.03 mV; 12±6 mm diameter). In LR, electrograms along the carousel encompassed the full tachycardia cycle length, and surrounding activation moved away from the carousel in all directions. Ablating fractionated electrograms (117±18 ms; 44±13% of tachycardia cycle length) within the carousel interrupted the tachycardia in every LR case. All remaining carousels were pseudo-reentrant (n=43/50 [86%]) occurring in areas of wavefront collision (n=21; median 0.5; quartiles 0-2 per map) or as artifact because of annotation of noise or interpolation in areas of incomplete mapping (n=22; median 1, quartiles 0-2 per map). Pseudo-reentrant carousels were incorrectly ablated in 5 cases having been misinterpreted as LR. CONCLUSIONS: The activation pattern of LR is of small stable rotational activations (carousels), and this drove 30% (7/23) of our postablation atrial tachycardias. However, this appearance is most often pseudo-reentrant and must be differentiated by interpretation of electrograms in the candidate circuit and activation in the wider surrounding region.

The changing face of cardiovascular disease 2000-2012: An analysis of the world health organisation global health estimates data.

The pattern and global burden of disease has evolved considerably over the last two decades, from primarily communicable, maternal, and perinatal causes to non-communicable disease (NCD). Cardiovascular disease (CVD) has become the single most important and largest cause of NCD deaths worldwide at over 50%. The World Health Organisation (WHO) estimates that 17.6 million people died of CVD worldwide in 2012. Proportionally, this accounts for an estimated 31.43% of global mortality, with ischaemic heart disease (IHD) accounting for approximately 7.4 million deaths, 13.2% of the total. IHD was also the greatest single cause of death in 2000, accounting for an estimated 6.0 million deaths. The global burden of CVD falls, principally, on the low and middle-income (LMI) countries, accounting for over 80% of CVD deaths. Individual populations face differing challenges and each population has unique health burdens, however, CVD remains one of the greatest health challenges both nationally and worldwide.

Rumpel-Leede phenomenon in a hypertensive patient due to mechanical trauma: a case report.

BACKGROUND: In this report, we present an interesting case of a patient with Rumpel-Leede phenomenon, a rare occurrence that can result in significant delays in medical treatment. This phenomenon is characterized by the presence of a petechial rash that results from acute dermal capillary rupture. In our patient, it occurred secondary to raised pressure in the dermal vessels caused by repeated inflation of a sphygmomanometer cuff. Contributory factors in Rumpel-Leede phenomenon include prevalent conditions such as diabetes mellitus, hypertension, thrombocytopenia, chronic steroid use, antiplatelets, and anticoagulants. CASE PRESENTATION: A 58-year-old Russian woman with diabetes and hypertension presented to our hospital with a non-ST elevation myocardial infarction, and she subsequently developed a petechial rash on her distal upper limbs. A vasculitic screen was performed, with normal results. CONCLUSIONS: Given the timing and distribution of the rash, it was felt that this was an example of Rumpel-Leede phenomenon in a susceptible individual. This is an important diagnosis to be aware of in patients with vascular risk factors presenting for acute medical care who subsequently develop a petechial rash.

A long-term follow-up of patients with prolonged asystole of greater than 15s on head-up tilt testing.

BACKGROUND: Head-up tilt (HUT) is used for diagnosis of vasovagal syncope (VVS), and can provoke cardioinhibition. VVS is usually considered benign, however pacemaker insertion may be indicated in some patients. We sought to characterize the long-term outcomes of patients with prolonged asystole (>15s) on HUT. METHODS: We conducted a retrospective study on patients with asystole >15s on HUT identified from 5133 patients who were investigated between 1998 and 2012 at our institution. Patients were mailed questionnaires or telephoned to ascertain outcomes. Where contact was not possible, the patients' general practitioners were contacted to request up-to-date information. RESULTS: A total of 26 patients with a mean age of 45 ± 18 years and a mean duration of asystole on HUT of 26 ± 7s were successfully followed up from a total of 77 patients identified. The follow-up duration was 99 ± 39 months. Six patients had undergone pacemaker (PPM) implantation. Of the patients without PPM, 16 reported spontaneously improved symptoms. Ten patients sustained injury prior to HUT compared with one after HUT, when a clear diagnosis was made and management advice was given. There were no major injuries or deaths after HUT. The 6 patients with PPMs had a mean age of 60 ± 16 (67% male) at HUT. Four patients had no further syncope after PPM and two demonstrated improvement but still experienced recurrent syncope. CONCLUSIONS: Prolonged asystole (>15s) on tilt does not necessarily predict adverse outcomes with most patients improving spontaneously over the long-term. Pacemaker insertion in selected patients may reduce syncope recurrence but does not always abolish it.

Malfunction of subpectorally implanted cardiac resynchronization therapy defibrillators due to weakened header bond.

BACKGROUND: Implantable cardioverter defibrillator (ICD) implantation has increased significantly over the last 10 years. Concerns about the safety and reliability of ICD systems have been raised, with premature lead failure and battery malfunctions accounting for the majority of reported adverse events. We describe the unique mode of presentation, diagnosis, and management of cardiac resynchronization therapy defibrillators (CRT-D) malfunctions that were caused by weakened bonding between the generator and header. METHODS AND RESULTS: Between June 2008 and December 2009, 22 Teligen™ ICDs and 24 Cognis™ CRT-Ds were implanted subpectorally at our institution, until a product advisory was issued. Of 24 Cognis™ CRT-D implants, 3 patients presented with CRT-D malfunctions. All our cases presented with initially intermittent and then persisting increases in shock lead impedance, associated with nonphysiological noise in the shock electrogram channels. These issues were rectified by generator change. Postexplant laboratory analysis confirmed inadequate bonding between device header and titanium casing in all cases, resulting in loosening and rocking of the header followed by fatigue-induced fracture of the shock circuitry. CONCLUSION: Weakened bonding between the header and generator casing of subpectorally implanted CRT-Ds can result in fractures and malfunction of the HV circuit. Physicians monitoring patients with devices affected by the product advisory should remain vigilant in order to diagnose and manage similar device malfunction expediently.

Relationship between myocardial perfusion with myocardial contrast echocardiography and function early after acute myocardial infarction for the prediction of late recovery of function.

BACKGROUND: Following ST elevation acute myocardial infarction (STEMI) and reperfusion therapy, there are often persistent wall thickening (WT) abnormalities and perfusion defects due to variable degree of myocardial stunning and necrosis. We hypothesised that following STEMI and reperfusion therapy, the extent of residual perfusion assessed by myocardial contrast echocardiography (MCE) and not the extent of WT abnormalities would predict subsequent global recovery of left ventricular (LV) function. METHODS: Accordingly, 112 patients with STEMI underwent simultaneous assessment of WT abnormality and perfusion using MCE 7+/-2 days after AMI and reperfusion therapy. Both WT and perfusion were scored on a 16 segment LV model. Contrast perfusion index (CPI), and global LV function were calculated. Echocardiography was repeated 12 weeks after reperfusion to assess recovery of LV function. RESULTS: Of the 112 patients recruited, follow up echocardiography 12 weeks after reperfusion was available in 98 patients. CPI was significantly higher (p<0.0001) in the 66 patients, who showed late recovery of LV function (1.67+/-0.27) compared to those who did not show recovery of function (1.25+/-0.04). No significant difference was noted in the indices of baseline LV function in patients with (1.67+/-0.32) and without (1.80+/-0.36) recovery of LV function. The multivariable predictors of late recovery of function were MCE (p=0.02), absence of diabetes (p=0.02) and lower peak creatine kinase (p=0.01). CONCLUSION: The extent of residual contrast perfusion and not WT abnormalities predicts late recovery of global LV function after acute myocardial infarction and reperfusion therapy.

Comparison between myocardial contrast echocardiography and (99m)technetium sestamibi single photon emission computed tomography determined myocardial viability in predicting hard cardiac events following acute myocardial infarction.

The extent of residual myocardial viability (MV) after acute myocardial infarction (AMI) is an important determinant of the outcome. Single photon emission computed tomography (SPECT) is widely used to assess MV after an AMI. However, myocardial contrast echocardiography (MCE), a relatively new technique for the assessment of MV, has better spatial and temporal resolution than SPECT. The present study evaluated whether MV determined by MCE is comparable to that determined using SPECT for the prediction of hard cardiac events after an AMI. Accordingly, 99 patients who had undergone simultaneous rest low-power MCE and nitrate-enhanced SPECT 7 days after an AMI were followed up for cardiac death and AMIs. Both MCE perfusion (1 = normal; 2 = reduced; and 3 = absent) and SPECT tracer uptake (0 = normal; 1 = mildly reduced; 2 = moderately reduced; 3 = severely reduced; and 4 = absent) were scored on a 16-segment left ventricular model. The contrast perfusion index and SPECT perfusion index were calculated by adding the respective scores in the 16 segments and dividing by 16. The contrast perfusion index and SPECT perfusion index were used as a measure of the residual MV on MCE and SPECT, respectively. Of the 99 patients recruited, 95 were available for the follow-up examination (follow-up 46 +/- 16 months). A total of 15 events (16%) occurred (8 cardiac deaths and 7 AMIs). Of the clinical, biochemical, echocardiographic, and SPECT markers of prognosis, the only independent predictors of cardiac death and cardiac death or AMI were age and MV as determined by MCE (p = 0.01 and p = 0.002, respectively). In conclusion, MV determined by MCE at rest was superior to nitrate-enhanced SPECT for the prediction of hard cardiac events after AMI.

Improved prediction of outcome by contrast echocardiography determined left ventricular remodelling parameters compared to unenhanced echocardiography in patients following acute myocardial infarction.

AIMS: Contrast echocardiography has been shown to be a more accurate method of assessing left ventricular (LV) remodelling compared with unenhanced echocardiography after acute myocardial infarction (AMI). However, whether this translated into improved prediction of outcome is not known. METHODS AND RESULTS: Accordingly, a total of 89 consecutive patients undergoing contrast echocardiography and unenhanced echocardiography 7 to 10 days after AMI and reperfusion therapy were followed up for cardiac death (CD) and AMI. LV ejection fraction (LVEF), LV end-systolic volume (ESV), and LV end-diastolic volume were assessed by the two methods independently. Outcome data were obtained (mean 46 +/- 16 months).There were 15 (17%) events (eight CDs and seven AMIs). LVEF and ESV with contrast echocardiography were found to be independent multivariable predictors of CD (P = 0.04 and P = 0.02, respectively) and CD or AMI (P = 0.02 and P = 0.01, respectively). Furthermore, LVEF and ESV with contrast echocardiography provided incremental information for the prediction of CD (P = 0.004 and P = 0.004, respectively) and CD or AMI (P = 0.02 and P = 0.03, respectively). CONCLUSION: Contrast echocardiography provided improved prediction of outcome compared with unenhanced echocardiography following AMI.

Treatment of ruptured coronary aneurysm with a novel covered stent.

Coronary artery aneurysms are relatively rare but have been diagnosed with increasing frequency since the advent of coronary arteriography. Their reported incidence varies from 1.5% to 5% with male dominance and a predilection for the right coronary artery (RCA), accounting for over 40% of all cases. The most common etiology amongst adults remains atherosclerosis accounting for 50% of coronary aneurysms. We describe the first use of a novel flexible pericardium covered stent for successful treatment of a ruptured coronary aneurysm in 76 year old lady.

Myocardial contrast echocardiography in ST elevation myocardial infarction: ready for prime time?

Acute myocardial infarction (AMI) continues to be a significant public health problem in industrialized countries and an increasingly significant problem in developing countries. ST elevation myocardial infarctions (STEMI) constitute approximately 40% of all AMIs with approximately 670,000 cases yearly in the United States alone. The risk of further cardiac complications such as re-infarction, sudden death, and heart failure for those who survive AMI is substantial. Thus, early assessment and risk stratification during the acute phase of STEMI is important. Furthermore, it is essential to assess the efficacy early after any initial therapeutic intervention, not only to facilitate further management, but also to enable development of new treatment algorithms/approaches to further improve the outcome. The aim of reperfusion therapy in AMI is not only to rapidly restore epicardial coronary blood flow but also to restore perfusion at the microcirculatory level. Myocardial contrast echocardiography (MCE) which utilizes microbubbles can assess myocardial perfusion in real time. Its ability to assess myocardial perfusion and function in one examination allows it to ascertain the extent of myocardial reperfusion achieved in the risk area. Furthermore, in stable patients after AMI, MCE allows assessment of LV function, residual myocardial viability, and ischaemia which are all powerful prognostic markers of outcome. Its portability, rapid acquisition and interpretation of data, and the absence of radiation exposure make it an ideal bedside technique.

Prognostic value of myocardial viability detected by myocardial contrast echocardiography early after acute myocardial infarction.

OBJECTIVES: This study sought to determine whether residual myocardial viability determined by myocardial contrast echocardiography (MCE) after acute myocardial infarction (AMI) can predict hard cardiac events. BACKGROUND: Myocardial viability detected by MCE has been shown to predict recovery of left ventricular (LV) function in patients with AMI. However, to date no study has shown its value in predicting major adverse outcomes in AMI patients after thrombolysis. METHODS: Accordingly, 99 stable patients underwent low-power MCE at 7 +/- 2 days after AMI. Contrast defect index (CDI) was obtained by adding contrast scores (1 = homogenous; 2 = reduced; 3 = minimal/absent opacification) in all 16 LV segments divided by 16. At discharge, 65 (68%) patients had either undergone or were scheduled for revascularization independent of the MCE result. The patients were subsequently followed up for cardiac death and nonfatal AMI. RESULTS: Of the 99 patients, 95 were available for follow-up. Of these, 86 (87%) underwent thrombolysis. During the follow-up time of 46 +/- 16 months, there were 15 (16%) events (8 cardiac deaths and 7 nonfatal AMIs). Among the clinical, biochemical, electrocardiographic, echocardiographic, and coronary arteriographic markers of prognosis, the extent of residual myocardial viability was an independent predictor of cardiac death (p = 0.01) and cardiac death or AMI (p = 0.002). A CDI of < or = 1.86 and < or = 1.67 predicted survival and survival or absence of recurrent AMI in 99% and 95% of the patients, respectively. CONCLUSIONS: The extent of residual myocardial viability predicted by MCE is a powerful independent predictor of hard cardiac events in patients after AMI.

Contrast echocardiography for the assessment of myocardial viability.

PURPOSE OF REVIEW: The availability of an accurate, non-invasive method for distinguishing viable from irreversibly damaged myocardium, after acute myocardial infarction or in chronic coronary artery disease, is important in clinical decision making. Such a tool would enable physicians to identify patients most likely to benefit from revascularization strategies in patients with coronary artery disease and left ventricular dysfunction. Myocardial contrast echocardiography is a new technique that utilizes acoustically active gas-filled microspheres (microbubbles), which remain exclusively in the intravascular space and allow the simultaneous assessment of global and regional myocardial structure, function, and perfusion. An increasing body of data supports its role in assessing myocardial viability and predicting the recovery of function. RECENT FINDINGS: Myocardial contrast echocardiography accurately differentiates 'stunning' from necrosis, delineates transmural extent of infarction, predicts recovery of regional and global left ventricular systolic function in the recuperative phase, identifies patients at high risk of left ventricular remodelling, and provides incremental viability data when performed in conjunction with low-dose dobutamine echocardiography. SUMMARY: Technological advances have positioned myocardial contrast echocardiography as a safe, practical bedside technique for the evaluation of myocardial viability. It has comparable accuracy with other non-invasive imaging techniques, such as dobutamine stress echocardiography, radionuclide scintigraphy and cardiac magnetic resonance imaging.

Comparison between myocardial contrast echocardiography and single-photon emission computed tomography for predicting transmurality of acute myocardial infarction.

Contrast-enhanced cardiovascular magnetic resonance imaging (CMR) has been shown to accurately assess transmural extent of infarction, which is an excellent predictor of long-term improvement in contractile function. We assessed the relative accuracy of myocardial contrast echocardiography (MCE) and single-photon emission computed tomography (SPECT) to predict transmural extent of infarction after acute myocardial infarction. MCE, SPECT, and CMR were performed in 40 patients with acute myocardial infarction 7 to 10 days after thrombolysis. CMR was used to divide the transmural extent of infarction into 5 groups: 0%, 1% to 25%, 26% to 50%, 51% to 75%, and 76% to 100% in dysfunctional segments. MCE and SPECT were compared with assessment grades of transmural extent of infarction. There was a significant relation (p<0.0001) between decreasing contrast intensity as assessed qualitatively by MCE and increasing transmural extent of infarction on CMR as was the case for SPECT. The accuracy of MCE (77%) to predict>50% transmural extent of infarction (nonviable myocardium) was significantly (p=0.02) superior to that of SPECT (70%). Absence of uptake on MCE and SPECT virtually ruled out 25% transmural extent of infarction (84% vs 76%, p=0.03). MCE and SPECT correlate well with the transmural extent of infarction. However, MCE is significantly more accurate in predicting >50% of the transmural extent of infarction and more sensitive in identifying

Safety: the heart of the matter.

An integral part of evaluation of cardiac disease in modern day medicine is echocardiography. It has made great strides since the initial collaboration of Dr. Helmut Hertz and Dr. Inge Edler. In its modern day form, echocardiography maintains a legacy of a bedside utility while adopting many of the technologic advances ushered in by the digital era. As a result, it boasts a broad and growing spectrum of application including routine use in primary cardiac diagnosis and screening, therapeutic assessment, and guidance of interventional and surgical procedures. With the advent of ultrasound contrast agents it is now arguably the most complete 'one-stop' investigational tool to assess cardiac structure, function and perfusion. However, has it maintained its safety profile? The familiar and oft quoted dictum in medicine of "first do no harm" is of great importance for any diagnostic tool and patient safety should remain a primary consideration for any new investigational technique. In this issue Cosyns' et al. have examined whether some of the theoretical and in vitro experimental concerns surrounding myocardial injury during and following contrast echocardiography result in any detectable change in cardiac function.

Diabetic cardiomyopathy: mechanisms, diagnosis and treatment.

Independent of the severity of coronary artery disease, diabetic patients have an increased risk of developing heart failure. This clinical entity has been considered to be a distinct disease process referred to as 'diabetic cardiomyopathy'. Experimental studies suggest that extensive metabolic perturbations may underlie both functional and structural alterations of the diabetic myocardium. Translational studies are, however, limited and only partly explain why diabetic patients are at increased risk of cardiomyopathy and heart failure. Although a range of diagnostic methods may help to characterize alterations in cardiac function in general, none are specific for the alterations in diabetes. Treatment paradigms are very much limited to interpretation and translation from the results of interventions in non-diabetic patients with heart failure. This suggests that there is an urgent need to conduct pathogenetic, diagnostic and therapeutic studies specifically in diabetic patients with cardiomyopathy to better understand the factors which initiate and progress diabetic cardiomyopathy and to develop more effective treatments.

Offloading diabetic foot wounds using the scotchcast boot: a retrospective study.

A retrospective study was conducted to evaluate treatment outcomes associated with the treatment of diabetic foot ulcers using the Scotchcast boot. Data were extracted from the records of 180 patients with diabetes, 150 (83.3%) male, with a mean age of 55.3 +/- 10.9 years undergoing treatment for noninfected, non-ischemic neuropathic diabetic foot wounds at a university teaching hospital's tertiary care outpatient clinic. All patients who met the criteria were treated with the Scotchcast boot as the sole form of pressure relief. The average follow-up for each patient was 85.9 +/- 30.6 months (range 34.2 to 147.7 months). The mean time to healing for all patients was 130.5 +/- 106.7 days. Superficial (Grade 1) wounds healed significantly faster than deep (Grade 3) wounds (111.5 +/- 98.2 versus 180.8 +/- 138.8 days, P = 0.01) and 144 (80%) wounds healed during the follow-up period. Of the 36 (20%) that did not heal with Scotchcast boot therapy, 10 went on to more proximal amputation (5.6% of total population), two required surgical intervention to heal their wounds (0.6% of total population), one patient died with an unhealed wound (0.6% of total population), 23 patients (12.8% of total population) were lost to follow-up, and 47 (26%) died. Experience and the results of this and other studies suggest that this removable modality may be useful in outpatient care of deeper or complex wounds that require frequent inspection, but a device that ensures compliance may be the preferred treatment of superficial wounds.