Cause Of Cardiac Death Research Articles

Amiodarone versus other pharmacological interventions for prevention of sudden cardiac death.

Sudden cardiac death (SCD) is one of the main causes of cardiac death. There are two main strategies to prevent it: managing cardiovascular risk factors and reducing the risk of ventricular arrhythmias. Implantable cardiac defibrillators (ICDs) constitute the standard therapy for both primary and secondary prevention; however, they are not widely available in settings with limited resources. The antiarrhythmic amiodarone has been proposed as an alternative to ICD. To evaluate the effectiveness of amiodarone for primary or secondary prevention in SCD compared with placebo or no intervention or any other antiarrhythmic drugs in participants at high risk (primary prevention) or who have recovered from a cardiac arrest or a syncope due to Ventricular Tachycardia/Ventricular Fibrillation, or VT/VF (secondary prevention). We searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE (OVID), EMBASE (OVID), CINAHL (EBSCO) and LILACS on 26 March 2015. We reviewed reference lists of included studies and selected reviews on the topic, contacted authors of included studies, screened relevant meetings and searched in registers for ongoing trials. We applied no language restrictions. Randomised and quasi-randomised trials assessing the efficacy of amiodarone versus placebo, no intervention, or other antiarrhythmics in adults. For primary prevention we considered participants at high risk for SCD. For secondary prevention we considered participants recovered from cardiac arrest or syncope due to ventricular arrhythmias. Two authors independently assessed the trials for inclusion and extracted relevant data. We contacted trial authors for missing data. We performed meta-analyses using a random-effects model. We calculated risk ratios (RR) for dichotomous outcomes with 95% confidence intervals (CIs). Three studies included more than one comparison. We included 24 studies (9,997 participants). Seventeen studies evaluated amiodarone for primary prevention and six for secondary prevention. Only three studies used an ICD concomitantly with amiodarone for the comparison (all of them for secondary prevention).For primary prevention, amiodarone compared to placebo or no intervention (17 studies, 8383 participants) reduced SCD (RR 0.76; 95% CI 0.66 to 0.88), cardiac mortality (RR 0.86; 95% CI 0.77 to 0.96) and all-cause mortality (RR 0.88; 95% CI 0.78 to 1.00). The quality of the evidence was low.Compared to other antiarrhythmics (three studies, 540 participants), amiodarone reduced SCD (RR 0.44; 95% CI 0.19 to 1.00), cardiac mortality (RR 0.41; 95% CI 0.20 to 0.86) and all-cause mortality (RR 0.37; 95% CI 0.18 to 0.76). The quality of the evidence was moderate.For secondary prevention, amiodarone compared to placebo or no intervention (two studies, 440 participants) appeared to increase the risk of SCD (RR 4.32; 95% CI 0.87 to 21.49) and all-cause mortality (RR 3.05; 1.33 to 7.01). However, the quality of the evidence was very low. Compared to other antiarrhythmics (four studies, 839 participants) amiodarone appeared to increase the risk of SCD (RR 1.40; 95% CI 0.56 to 3.52; very low quality of evidence), but there was no effect in all-cause mortality (RR 1.03; 95% CI 0.75 to 1.42; low quality evidence).Amiodarone was associated with an increase in pulmonary and thyroid adverse events. There is low to moderate quality evidence that amiodarone reduces SCD, cardiac and all-cause mortality when compared to placebo or no intervention for primary prevention, and its effects are superior to other antiarrhythmics.It is uncertain if amiodarone reduces or increases SCD and mortality for secondary prevention because the quality of the evidence was very low.

Neutrophil Gelatinase-Associated Lipocalin, but Not Kidney Injury Marker 1, Correlates with Duration of Delayed Graft Function

Background: No specific early biomarker is available to measure kidney injury after kidney transplantation (KT). Both neutrophil gelatinase-associated lipocalin (NGAL) and kidney injury marker 1 (KIM-1) increase after oxidative injury. Their potential as early biomarkers was evaluated in this one-arm pilot study. Materials and Methods: Twenty consecutive KT patients receiving a kidney from a donation after circulatory death donor were included. Graft perfusate was collected, as well as serum samples before transplantation, at the end of surgery, and 1, 4, and 7 days after transplantation. NGAL and KIM-1 were measured using ELISA. Kidney function and delayed graft function (DGF) were monitored. Results: In this cohort, 85% of the KT patients developed DGF. Perfusate NGAL correlated with donor age (r² = 0.094, p = 0.01) and serum creatinine (r² = 0.243, p = 0.05). A cardiac cause of death was associated with higher NGAL in the perfusate (p = 0.03). Serum NGAL at day 1 was significantly higher in patients with DGF (730 ng/ml, range 490-1,655, vs. 417 ng/ml, range 232-481; p = 0.01). Serum NGAL levels at day 1, 4, and 7 correlated with the duration of DGF. KIM-1 was not detectable in the perfusate or in the serum until postoperative day 4 in 80% of patients. Conclusions: NGAL in the perfusate correlates with known donor risk factors for DGF. For the first time, we describe that serum NGAL at day 1 can discriminate between DGF and immediate graft function. Also, serum NGAL levels at day 1, 4, and 7 correlate with the duration of DGF. No association with KIM-1 was found. These data suggest that NGAL may be used as an early biomarker to detect DGF and warrants further study.

Introduction to special issue on: the David Barker commemorative meeting, September 2014; the future of the science he inspired.

Introduction to special issue on: the David Barker commemorative meeting, September 2014; the future of the science he inspired.

Sudden unexpected death in children with congenital heart defects.

Congenital heart defects (CHDs) are the most common birth defects and are an important cause of death in children. The fear of sudden unexpected death has led to restrictions of physical activity and competitive sports. The aim of the present study was to investigate the rate of sudden unexpected deaths unrelated to surgery in children 2-18 years old with CHDs and, secondarily, to determine whether these deaths were related to cardiac disease, comorbidity, or physical activity. To identify children with CHDs and to determine the number of deaths, data concerning all 9 43 871 live births in Norway in 1994-2009 were retrieved from the Medical Birth Registry of Norway, the Cardiovascular Disease in Norway project, the Oslo University Hospital's Clinical Registry for Congenital Heart Defects and the Norwegian Cause of Death Registry. Survivors were followed through 2012, and information for the deceased children was retrieved from medical records at Norwegian hospitals. Among 11 272 children with CHDs, we identified 19 (0.2%) children 2-18 years old who experienced sudden unexpected deaths unrelated to cardiac surgery. A cardiac cause of death was identified in seven of these cases. None of the children died during physical activity, whereas two children survived cardiac arrest during sports. Sudden unexpected death was infrequent among children with CHDs who survived 2 years of age. Comorbidity was common among the children who died. This study indicates that sudden unexpected death in children with CHDs rarely occurs during physical activity.

Rigor mortis at the myocardium investigated by post-mortem magnetic resonance imaging

IntroductionPost-mortem cardiac MR exams present with different contraction appearances of the left ventricle in cardiac short axis images. It was hypothesized that the grade of post-mortem contraction may be related to the post-mortem interval (PMI) or cause of death and a phenomenon caused by internal rigor mortis that may give further insights in the circumstances of death. Method and materialsThe cardiac contraction grade was investigated in 71 post-mortem cardiac MR exams (mean age at death 52y, range 12–89y; 48 males, 23 females). In cardiac short axis images the left ventricular lumen volume as well as the left ventricular myocardial volume were assessed by manual segmentation. The quotient of both (LVQ) represents the grade of myocardial contraction. LVQ was correlated to the PMI, sex, age, cardiac weight, body mass and height, cause of death and pericardial tamponade when present. In cardiac causes of death a separate correlation was investigated for acute myocardial infarction cases and arrhythmic deaths. ResultsLVQ values ranged from 1.99 (maximum dilatation) to 42.91 (maximum contraction) with a mean of 15.13. LVQ decreased slightly with increasing PMI, however without significant correlation. Pericardial tamponade positively correlated with higher LVQ values. Variables such as sex, age, body mass and height, cardiac weight and cause of death did not correlate with LVQ values. There was no difference in LVQ values for myocardial infarction without tamponade and arrhythmic deaths. ConclusionBased on the observation in our investigated cases, the phenomenon of post-mortem myocardial contraction cannot be explained by the influence of the investigated variables, except for pericardial tamponade cases. Further research addressing post-mortem myocardial contraction has to focus on other, less obvious factors, which may influence the early post-mortem phase too.

Presentation of Sudden Death in Emergency Department of a Tertiary Care Hospital of Bangladesh: A Retrospective Study

Background: The incidence and causes of sudden death vary in different societies, and these differences are influenced by demographic and clinical factors such as age, gender, risk factors, past medical history and presumed cause of death. This three years long retrospective study describes the influence of these factors on death from sudden cardiopulmonary arrest.Materials and Methods: This is a retrospective study of sudden death among all the age groups who were seen at Emergency Room (ER) of Ibrahim Cardiac Hospital & Research Institute, Dhaka, Bangladesh. Total 414 cases of death were reported between 1stJanuary, 2012 to 31st December, 2014. They were investigated retrospectively and subsequently analyzed on age, sex, risk factors, past medical history, endo-tracheal intubation with resuscitation and cause of death. The statistical analysis was performed as appropriate to illustrate any possible association between different demographic variables and cardiopulmonary arrest.Results: Out of 414 deaths, 72.5% were male, & 27.5% were female. The mean age of subjects was 60.60 ± 13.34 years with male mean age of 60.35 ± 13.62 years and female mean age of 59.70 ± 13.24 years. There were three different age groups in this study. 52 deaths were in 17-45 years of age group, 233 death patients were in 46-65 years age group & 129 deaths were more than 65 years age group. Among 46-65years age group 161 deaths were presumed to be due to cardiac cause which is higher than the other two groups. 75.12% of all the study patients had hypertension, 84.06% had diabetes mellitus, 23.43% had CKD & 69.32% had dyslipidaemia. 31.40% patients were active smoker, & 20.53% had prior h/o smoking. Patients who suffered cardiac cause of death had significant history of hypertension (p<0.001), dyslipidaemia (p<0.001), smoking habit (p<0.001), & CKD (p<0.001). 40.8% (169) of deceased patients had previous ischemic heart disease, 10.4% (43) had previous cerebrovascular disease and 28% (116) had prior morbidity like malignancy, multi organ dysfunction. 73.67% subjects needed endotracheal intubation with urgent cardio pulmonary resuscitation. Out of 414 study patients, 65% had presumed precipitating cardiac cause of death, 15% had presumed neurological cause of death and rest 20% had other causes.Conclusion: The current study indicates a definite influence of age, gender, medical risk factors and past medical history has causal relationship with sudden death. The highest incidence occurred in the middle age group as compared to other age groups. Incidence was also higher in men than in women. Subjects with hypertension, diabetes mellitus and previous coronary artery disease had higher incidence of sudden deathsBangladesh Crit Care J March 2015; 3 (1): 14-16

Presence of brain pathology in deceased subjects with and without chronic obstructive pulmonary disease.

Patients with chronic obstructive pulmonary disease (COPD) have extrapulmonary co-morbidities, such as cardiovascular disease, musculoskeletal wasting and neuropsychological conditions. To date, it remains unknown whether and to what extent COPD is associated with a higher prevalence of brain pathology. Therefore, the aim of this retrospective study was to compare the prevalence of neuropathological brain changes between deceased donors with and without COPD. Brain autopsy reports of age-matched donors with (n = 89) and without COPD (n = 89) from the Netherlands Brain Bank were assessed for demographics, cause of death, co-morbidities and brain pathology. The prevalence of degenerative brain changes was comparable for donors with and without COPD (50.6% vs. 61.8%, p > 0.05). Neoplastic brain changes were reported in a minority of the donors (5.6% vs. 10.1%, p > 0.05). After correction for cerebrovascular accident or cardiac cause of death and Charlson co-morbidity index score, the prevalence of vascular brain changes was higher among control versus COPD donors (27.0% vs. 11.2%, adjusted p = 0.013, odds ratio = 2.98). Brain autopsy reports of donors with and without COPD did not reveal differences in the presence of degenerative or neoplastic brain changes. Vascular brain changes were described more often in controls. Prospective studies including spirometry and structural and functional brain imaging should corroborate our findings.

The hybrid approach for palliation of hypoplastic left heart syndrome: Intermediate results of a single-center experience

IntroductionHypoplastic left heart syndrome (HLHS) is a major cause of cardiac death during the first week of life. The hybrid approach is a reliable, reproducible treatment option for patients with HLHS. Herein we report our results using this approach, focusing on its efficacy, safety and late outcome. MethodsWe reviewed prospectively collected data on patients treated for HLHS using a hybrid approach between July 2007 and September 2014. ResultsNine patients had a stage 1 hybrid procedure, with seven undergoing a comprehensive stage 2 procedure. One patient completed the Fontan procedure. Five patients underwent balloon atrial septostomy after the hybrid procedure; in three patients, a stent was placed across the atrial septum. There were three deaths: two early after the hybrid procedure and one early after stage two palliation. Overall survival was 66%. ConclusionsIn our single-center series, the hybrid approach for HLHS yields intermediate results comparable to those of the Norwood strategy. The existence of dedicated teams for the diagnosis and management of these patients, preferably in high-volume centers, is of major importance in this condition.

The hybrid approach for palliation of hypoplastic left heart syndrome: Intermediate results of a single-center experience

IntroductionHypoplastic left heart syndrome (HLHS) is a major cause of cardiac death during the first week of life. The hybrid approach is a reliable, reproducible treatment option for patients with HLHS. Herein we report our results using this approach, focusing on its efficacy, safety and late outcome. MethodsWe reviewed prospectively collected data on patients treated for HLHS using a hybrid approach between July 2007 and September 2014. ResultsNine patients had a stage 1 hybrid procedure, with seven undergoing a comprehensive stage 2 procedure. One patient completed the Fontan procedure. Five patients underwent balloon atrial septostomy after the hybrid procedure; in three patients, a stent was placed across the atrial septum. There were three deaths: two early after the hybrid procedure and one early after stage two palliation. Overall survival was 66%. ConclusionsIn our single-center series, the hybrid approach for HLHS yields intermediate results comparable to those of the Norwood strategy. The existence of dedicated teams for the diagnosis and management of these patients, preferably in high-volume centers, is of major importance in this condition.

Causes of death and predictors of survival after aortic valve replacement in low flow vs. normal flow severe aortic stenosis with preserved ejection fraction.

Reduced stroke volume index (SVI) in patients with severe aortic stenosis (AS) and preserved ejection fraction (EF) is associated with adverse outcomes even after aortic valve replacement (AVR), although specific reasons for impaired survival in this group are unknown. We investigated predictors of post-AVR survival and specific cause of death in patients with AS according to SVI. Among 1120 consecutive patients with severe AS (aortic valve area <1.0 cm(2)) and preserved EF (≥50%) using 2-D and Doppler echocardiography who had AVR, 61 (5%) patients had reduced SVI [<35 mL/m(2) (low flow, LF)] and 1059 (95%) had normal SVI [≥35 mL/m(2) (normal flow, NF)]. Survival post-AVR was lower in patients with LF compared with NF [3-year survival in LF group 76% (95% CI 70-82) vs. 89% (95% CI 88-90%), P = 0.03] primarily due to higher cardiac mortality [3-year event rate 13% (95% CI 8-18%) in LF vs. 5% (95% CI 5-7%) in NF, P = 0.02]. Congestive heart failure (CHF) was the most common cause of cardiac death in the LF group (57% of post-AVR cardiac deaths) and was a more frequent cause of death in LF compared with NF (3-year risk 7 vs. 2%, P = 0.008). Multivariable predictors of post-AVR mortality included age, creatinine, haemoglobin, right ventricular systolic pressure, SVI, and cognitive impairment. Reduced SVI is associated with higher cardiac mortality after AVR. CHF is the predominant cause of cardiac mortality after AVR in patients with LF, suggesting the presence of persistent myocardial impairment in this population.

Transient receptor potential melastatin-4 is involved in hypoxia-reoxygenation injury in the cardiomyocytes.

Ischemic heart disease still remains the most common cause of cardiac death. During ischemia-reperfusion (I/R), reactive oxygen species (ROS) are produced in excess in cardiac tissue, where they induce cell death. Our previous study showed that 9-phenanthrol (9-Phe), a specific inhibitor of the TRPM4 channel, preserves cardiac contractile function and protects the heart from I/R injury-related infarction in the excised rat heart. Accordingly, we hypothesized that TRPM4 channels are involved in the 9-Phe-mediated cardioprotection against ROS-induced injury. In rats, intravenous 9-Phe mitigated the development of myocardial infarction caused by the occlusion of the left anterior descending artery. Immunohistochemical analysis indicated that TRPM4 proteins are expressed in ventricular myocytes susceptible to I/R injury. Hydrogen peroxide (H2O2) is among the main ROS overproduced during I/R. In the cardiomyocyte cell line H9c2, pretreatment with 9-Phe prevented cell death induced by conditions mimicking I/R, namely 200 μM H2O2 and hypoxia-reoxygenation. Gene silencing of TRPM4 preserved the viability of H9c2 cardiomyocytes exposed to 200 μM H2O2. These results suggest that the cardioprotective effects of 9-Phe are mediated through the inhibition of the TRPM4 channels.

Characteristics and outcome of congenital left ventricular aneurysm and diverticulum: Analysis of 809 cases published since 1816

BackgroundCongenital left ventricular aneurysm (LVA) or diverticulum (LVD) is rare cardiac anomalies. We aimed to analyse the clinical characteristics and outcome in all ever published patients. MethodsMEDLINE, Web of science, Google and EMBASE, and reference lists of relevant articles were searched for publications reporting on LVA or LVD patients. ResultsWe identified 809 patients published since 1816 [354 (49.1%) LVA, 453 (50.6%) LVD, 2 (0.3%) both]. Mean age at diagnosis was 34.1±27 (LVA) and 29.7±27.6years (LVD; p=0.05). 48.9% were male. LVA was larger (38.7±22.5mm versus 31.4±21.2mm; p=0.002) and frequently found in submitral location (33% versus 4.9%; p<0.001), LVD was frequently located at the LV-apex (61.2% versus 28.7%; p<0.001). LVD was often associated with cardiac (34.2% versus 11%; p<0.001) or extracardiac anomalies (32.7% versus 3%; p<0.001). LVA patients presented more frequently with ventricular tachycardia/fibrillation (18.1% versus 13.1%; p=0.01), the incidences of rupture (4% versus 4.5%; p=0.9), syncope (8.3% versus 5.1%; p=0.1), and embolic events (4.9% versus 3.6%; p=0.4) at presentation were not different between LVA and LVD.Mean follow-up was 56.3±43months. Cardiac death occurred more frequently in the LVA group (11.5% versus 5.0%; p=0.05) at a median age of 0.8 [LVA] and 2.5 [LVD] years. The leading cause of cardiac death was congestive heart failure in the LVA-group (50.0% versus 0.0%; p=0.01), and rupture in the LVD-group (75.0% versus 27.3%; p=0.04). ConclusionsLVA and LVD are distinct congenital anomalies with different clinical and morphological characteristics. The prognosis of LVA is significantly worse during long-term follow-up.

A Case of Congenitally Corrected Transposition of Great Arteries (CCTGA)

Recent years, much scientific attention has been given to congenital heart diseases (CHD) and probable complications. Congenitally corrected transposition of the great arteries (CCTGA) is a rare, complex form of congenital heart defects. CCTGA is characterized by atrioventricular (AV) and ventriculoarterial (VA) discordance and hence by a physiologically normal direction of blood flow sometimes called "double discordance". The development of complete AV block and global ventricular dysfunction has been identified as the cause of cardiac death. This paper presents a case of CCTGA with rhythm disorders and exertional dyspnea.&#x0D; CBMJ 2015 January: Vol. 04 No. 01 P: 37-40

Modern chronic heart failure therapy in context of pulmonary banding to avoid heart transplantation

s of the 51st Workshop for Pediatric Research 51st Workshop for Pediatric Research Gottingen, Germany 16-17 April 2015 This supplement has not been sponsored. Meeting abstracts Background Dilated cardiomyopathy (DCM) is a leading cause of cardiac death in children. Approximately 30% of children die or need cardiac transplantation in the first year after diagnosis. We established a protocol to improve the outcome in this high-risk population. Patients and methods We present our experience in 21 patients (mean age 8 month, mean weight 6 kg) treated in our institution from 2006 to 2015. The patients were diagnosed with DCM with a highly impaired function of the LV (mean EF 17%) and a conserved function of the RV (mean EF 52%). Our protocol for medical enhancement of left ventricular recovery in association with pulmonary artery banding involves the use of a highly specific β1-blocker, an angiotensin-converting enzyme inhibitor and an aldosterone antagonist. Our therapy aims to reduce oxygen consumption and improve oxygen delivery. Heart rate control is the most important goal; therefore clonidine is given after surgical procedure and digoxin in long-term treatment if heart rate remains high despite adequate β1-blocker therapy. To improve oxygen delivery our goal is to archive haemoglobin levels of 12-14 g/dl, therefore Erythropoietin is given as long term treatment. Additional treatments include supplementation of carnitine, coenzyme Q, riboflavin or thiamine. Results At a mean follow up of 36 month (range 2-120) freedom from death was 91% and freedom from heart transplantation was 85%. Surviving patients showed a significant improvement in left ventricular ejection fraction (from 17 ± 6 to 50 ± 11%) and LVEdD (z-score from +7 ± 2 to +1.7 ± 1.9). The levels of BNP improved significantly (from 3222 ± 2756 to 70 ± 56 pg/ml). Conclusion Our data suggest that the medical and surgical approach described may result in a markedly improved medium-term outcome in children with DCM. Further studies are required to evaluate the long-term-outcome of these patients.

Upgraded heart failure therapy leads to an improved outcome of dilated cardiomyopathy in infants and toddlers.

Dilated cardiomyopathy is a leading cause of cardiac death in children. Approximately 30% of children die or need cardiac transplantation in the first year after establishing the diagnosis. New strategies are needed to improve the outcome in this high-risk patient population. We present our experience in 38 patients below the age of three years, who were diagnosed with dilated cardiomyopathy and who were treated at our institution between 2006 and 2012. The treatment strategy involved institution of β-blockers and angiotensin-converting enzyme inhibitors as soon as feasible. In selected cases, pulmonary artery banding or intracoronary autologous bone marrow-derived cell therapy was performed. The median age at presentation was six months (range 1-26 months). The median follow-up age was 16 months (range 2-80 months). Kaplan-Meier analysis of survival after dilated cardiomyopathy diagnosis revealed a one-year survival of 97% and a five-year survival of 86%. The rate of freedom from death or heart transplantation was 82% at one year and 69% at five years. Surviving patients who were free of transplantation, at the follow-up at 25 months (3-80 months), showed a significant improvement in left ventricular ejection fraction (from 19±11 to 46±16%) and left ventricular end-diastolic diameter (z-score from 4.6±2.4 to 1.4±1.6). In addition, the levels of B-type natriuretic peptide improved significantly (from 3330±3840 to 171±825 pg/ml). Our data suggest that the clinical approach described here may result in a markedly improved medium-term outcome in young children with dilated cardiomyopathy. Further studies are required to evaluate whether these approaches reduce end-points such as transplantation or death.

Sickle Cell Disease Mortality in California and Georgia 2004-2008

▪On-going public health surveillance efforts in sickle cell disease (SCD) are critical for understanding the course and outcomes of this disease over time. Once nearly universally fatal by adolescence, many patients are living well into adulthood and sometimes into retirement years. Previous SCD mortality estimates have relied on data from death certificates alone or from deaths of patients receiving care in high volume hematology clinics, resulting in gaps in reporting and potentially biased conclusions.The Registry and Surveillance System for Hemoglobinopathies (RuSH) project collected and linked population-based surveillance data on SCD in California and Georgia from a variety of sources for years 2004-2008. These data sources included administrative records, newborn screening reports and health insurance claims as well as case reports of adult and pediatric patients receiving care in the following large specialty treatment centers: Georgia Comprehensive Sickle Cell Center, Georgia Regents University, Georgia Comprehensive Sickle Cell Center at Grady Health Systems and Children’s Healthcare of Atlanta in Georgia, and Children’s Hospital Los Angeles and UCSF Benioff Children’s Hospital Oakland in California. Cases identified from these combined data sources were linked to death certificates in CA and GA for the same years.Among 12,143 identified SCD cases, 640 were linked to death certificates. Combined SCD mortality rates by age group at time of death are compared to combined mortality rates for all African Americans living in CA and GA. (Figure 1). SCD death rates among children up to age 14 and among adults 65 and older were very similar to those of the overall African American population. In contrast, death rates from young adulthood to midlife were substantially higher in the SCD population.Overall, only 55% of death certificates linked to the SCD cases had SCD listed in any of the cause of death fields. Thirty-four percent (CA) and 37% (GA) had SCD as the underlying cause of death. An additional 22% and 20% (CA and GA, respectively) had underlying causes of death that were not unexpected for SCD patients, including related infections such as septicemia, pulmonary/cardiac causes of death, renal failure and stroke. The remaining 44% (CA) and 43% (GA) had underlying causes of death that were either not related to SCD (e.g., malignancies, trauma) or too vague to be associated with SCD (e.g., generalized pulmonary or cardiac causes of death. Figure 2 shows the number of deaths by state, age group at death and whether the underlying cause of death was SCD specific, potentially related to SCD or not clearly related to SCD. While the number of deaths was too small to use for life expectancy calculations, there were more deaths over age 40 than under age 40 during this five year period.This effort represents a novel, population-based approach to examine mortality in SCD patients. These data suggest that the use of death certificates alone to identify deceased cases may not capture all-cause mortality among all SCD patients. Additional years of surveillance are needed to provide better estimates of current life expectancy and the ability to track and monitor changes in mortality over time. On-going surveillance of the SCD population is required to monitor changes in mortality and other outcomes in response to changes in treatments, standards of care and healthcare policy and inform advocacy efforts.This work was supported by the US Centers for Disease Control and Prevention and the National Heart, Lung and Blood Institute, cooperative agreement numbers U50DD000568 and U50DD001008. [Display omitted] [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Utility and importance of new echocardiographic screening methods in diagnosis of anomalous coronary origins in the pediatric population: assessment of quality improvement.

Anomalous aortic origin of coronaries from the contralateral sinus (AAOCA) is rare but an important cause of cardiac death in the otherwise healthy young athlete. This necessitates prompt, accurate identification; transthoracic echocardiography (TTE) remains the primary screening tool. Assessment of accuracy is difficult since the true prevalence of the disease is unknown, with estimates at 0.3-1.07%. The incidence by TTE remains much lower, between 0.09 and 0.17% even with sophisticated equipment and a high index of suspicion. Our goal was to incorporate two new screening views to our standard TTE protocol and assess improvement in diagnosis of AAOCA by TTE in our laboratory. Recently (2011), we incorporated two new screening methods to standard protocol. The parasternal short axis sweep is extended to visualize the anomalous segment arising superiorly from the ascending aorta before exiting the root at a site close to a 'normal' origin. Secondly, the anomalous, interarterial coronary demonstrates an anterior, steep-angled course visualized in the parasternal long axis between the aorta and pulmonary artery. The echocardiogram database was searched for patients newly diagnosed with AAOCA in 2010 (prior to incorporation of new methods) and 2012. AAOCA incidence in our patient population improved from 0.02% (2010) to 0.22% (2012), age range from 4 days to 17 years. Teenagers and symptomatic patients with anomalous right coronary origin (6) underwent additional confirmatory imaging, and three underwent surgery. One patient with anomalous left coronary origin underwent surgical repair. Addition of the screening views can significantly increase the sensitivity of TTE in diagnosing AAOCA in the asymptomatic patients. We propose that these views be incorporated into the standard TTE evaluation of coronary arteries.

Contrast-enhanced postmortem computed tomography in clinical pathology: enhanced value of 20 clinical autopsies.

Postmortem computed tomography (PMCT) is a modern tool that complements autopsy diagnostics. In clinical autopsies, a major cause of death is cardiovascular disease. To improve the performance of PMCT in cardiovascular disease, full body angiography was developed (PMCT angiography [PMCTA]). Twenty PMCTA scans generated before autopsy were compared with native PMCT and clinical autopsy. The objective of the study was to quantify the additional diagnostic value of adding angiography to native imaging and to compare PMCT and PMCTA findings to autopsy findings. The diagnosis of the cause of death was identical or overlapped in 80% of the cases that used PMCTA and 70% that used PMCT. The additional diagnostic yield given by PMCT and PMCTA in combination with autopsy was 55%. PMCT yielded additional diagnoses in the musculoskeletal system. The greatest additional diagnostic value of PMCTA was in association with cardiovascular diagnoses. The accuracy of PMCTA for cardiac causes of death was 80%, and the positive predictive value was 90%. The findings indicate that native PMCT cannot display the cardiovascular system sufficiently clearly for high-quality diagnostic assessment. However, PMCTA is a powerful tool in autopsy cases with a history of cardiovascular disease and/or a suspected cardiovascular cause of death. The combination of PMCTA and clinical autopsy enhances diagnostic quality and completeness of the autopsy report. Furthermore, in cases without consent or with a restricted consent for clinical autopsy, PMCTA has the potential to provide information on cardiovascular causes of death.

Risk Scores for 30-Day Mortality After Percutaneous Coronary Intervention: New Insights Into Causes and Risk of Death

ObjectiveTo determine the causes and risk of death after percutaneous coronary interventions (PCIs) and to compare the discriminatory ability of the New York State Risk Score (NYSRS) with the Mayo Clinic Risk Score (MCRS). Patients and MethodsWe studied in-hospital and 30-day mortality after PCI in 4898 patients treated at Mayo Clinic in Rochester, Minnesota, from January 1, 2007, through December 31, 2010, to validate the NYSRS equation with recalibrated predicted probabilities of death. ResultsOf the 4898 patients studied, 93 (1.9%) died during the index hospitalization, and 36 (0.7%) died within 30 days after discharge. For the in-hospital and 30-day mortality, respectively, the area under the receiver operating characteristic curve was 0.92 and 0.88 for the NYSRS and 0.93 and 0.90 for the MCRS, indicating excellent discrimination. The NYSRS model underpredicted event rates when applied in Mayo Clinic data (2.6% observed [127 of 4898 patients] vs 2.3% predicted [114 of 4898 patients]), even after recalibration. The instantaneous hazard over time revealed the highest risk of death in the first 3 days after PCI (daily probability, >0.2%), declined to 0.1% until about day 12, and then decreased below 0.1%. Cardiac causes (mainly myocardial infarction) dominated in the first week (83 of 85 deaths [97.6%]) and then decreased to 59.5% (25 of 42 deaths) between 8 and 30 days after PCI. ConclusionThe discriminatory ability of the NYSRS and the MCRS for in-hospital and 30-day mortality after PCI is roughly interchangeable. The risk of death is highest during the first 2 weeks and is dominated by cardiac causes of death.

Investigation of cardiac death - what is (technically) possible?

In searching for genetic causes of cardiac death, the key requirements are collection of the appropriate sample and selection of the appropriate test. It is essential to obtain a good source of DNA; occasionally, establishing a fibroblast culture may be possible and worthwhile. Once DNA is available, options for mutation screening are changing rapidly, as new sequencing technologies move into the diagnostic laboratory. Selecting a genetic test involves not just the history and post-mortem findings, but ideally should also rely on evaluation of the family, including family history and potentially investigation of family members. The results of testing may have important implications for family members. Options range from no genetic testing through sequencing a single gene, sequencing (increasingly large) panels of genes, and even sequencing essentially all of the known genes (exome sequencing). A negative result on a test done today is not necessarily the end of the story - the options available in 5 years (and our ability to interpret the results) are likely to be substantially different from the situation today. In addition to discussion of these issues, this talk will include a shopper's guide to currently available tests, mosquitoes in amber and a 700,000 year old horse.