Clinical characterization and natural history of chemotherapy-induced dilated cardiomyopathy.

AIMS: Chemotherapy-induced dilated cardiomyopathy (CI-DCM) is a well-recognized phenotype of non-ischemic dilated cardiomyopathy (DCM), characterized by poor outcomes. However, a detailed comparison between idiopathic DCM (iDCM) and CI-DCM is still lacking. METHODS AND RESULTS: All consecutive DCM patients enrolled in the Trieste Muscle Heart Disease Registry were analysed. CI-DCM and iDCM were defined according to current recommendations. The primary study outcome measure was all-mortality death and secondary outcomes were a) a composite of cardiovascular death/heart-transplantation/ventricular-assist-device implantation, and b) major ventricular arrhythmias. The study included 551 patients (499 iDCM and 52 CI-DCM). At enrolment, compared with iDCM, CI-DCM patients were older (51 ± 14 years vs. 58 ± 3 years, respectively, P < 0.001) and had a higher left ventricular ejection fraction (32% ± 9 vs. 35% ± 10, respectively, P = 0.03). Over a median follow-up of 90 months (IQR 54-140 months), CI-DCM patients had a higher incidence of all-cause mortality compared with iDCM (36.5% vs. 8.4% in CI-DCM and iDCM respectively, P < 0.001), while the incidence of major ventricular arrhythmias was higher in the iDCM group compared with CI-DCM (4% vs. 0%, in CI-DCM and iDCM respectively, P = 0.03). The risk of the composite outcome was comparable between the two groups (P = 0.91). At Cox multivariable analysis, the diagnosis of CI-DCM emerged as independently associated to primary outcome (HR 6.42, 95% C.I. 2.52-16.31, P < 0.001). CONCLUSIONS: In a well-selected DCM cohort, patients with a chemotherapy-induced aetiology had a higher incidence of all-cause mortality compared with iDCM. Conversely, the incidence of life-threatening ventricular arrhythmic events was higher among patients with iDCM.

Exposure to Elevated Nitrogen Dioxide Concentrations and Cardiac Remodeling in Patients With Dilated Cardiomyopathy.

BACKGROUND: Empirical evidence suggests a strong link between exposure to air pollution and heart failure incidence, hospitalizations, and mortality, but the biological basis of this remains unclear. We sought to determine the relationship between differential air pollution levels and changes in cardiac structure and function in patients with dilated cardiomyopathy. METHODS AND RESULTS: We undertook a prospective longitudinal observational cohort study of patients in England with dilated cardiomyopathy (enrollment 2009-2015, n = 716, 66% male, 85% Caucasian) and conducted cross sectional analysis at the time of study enrollment. Annual average air pollution exposure estimates for nitrogen dioxide (NO2) and particulate matter with diameter of 2.5 µm or less (PM2.5) at enrolment were assigned to each residential postcode (on average 12 households). The relationship between air pollution and cardiac morphology was assessed using linear regression modelling. Greater ambient exposure to NO2 was associated with higher indexed left ventricular (LV) mass (4.3 g/m2 increase per interquartile range increase in NO2, 95% confidence interval 1.9-7.0 g/m2) and lower LV ejection fraction (-1.5% decrease per interquartile range increase in NO2, 95% confidence interval -2.7% to -0.2%), independent of age, sex, socioeconomic status, and clinical covariates. The associations were robust to adjustment for smoking status and geographical clustering by postcode area. The effect of air pollution on LV mass was greatest in women. These effects were specific to NO2 exposure. CONCLUSIONS: Exposure to air pollution is associated with raised LV mass and lower LV ejection fraction, with the strongest effect in women. Although epidemiological associations between air pollution and heart failure have been established and supported by preclinical studies, our findings provide novel empirical evidence of cardiac remodeling and exposure to air pollution with important clinical and public health implications.

Prevalence and prognostic relevance of myocardial inflammation and cardiotropic viruses in non-ischemic dilated cardiomyopathy.

BACKGROUND: Non-ischemic dilated cardiomyopathy (DCM) is a heterogeneous disease with a spectrum of etiological factors. However, subsets of the disease are not well-characterized with respect to these factors. The aim of this study was to evaluate the prevalence of myocardial inflammation and cardiotropic viruses in DCM patients and their impact on clinical outcome. METHODS: Fifty-seven patients with DCM underwent endomyocardial biopsy between 2010 and 2013. Biopsies were analyzed by polymerase chain reaction (PCR) for the presence of cardiotropic viruses, and inflammatory cell infiltration was assessed by immunohistochemistry. During a 5-year follow-up, 27 (47%) patients reached the composite outcome measure: heart transplantation, left ventricle assist device implantation or cardiovascular-related death. RESULTS: Thirty-one (54%) patients had myocardial inflammation and cardiotropic viruses were detected in 29 (52%). The most frequent viruses were parvovirus B19 and human herpesvirus type-6. Four specific sub-groups were distinguished by PCR and immunohistochemistry: virus-positive (chronic) myocarditis, autoreactive inflammatory DCM, viral DCM, non-inflammatory DCM. The presence of a viral genome in myocardium or diagnosis of inflammatory DCM did not predict the outcome of composite outcome measures (p > 0.05). However, univariate Cox regression and survival function estimation revealed an association between inflammation by a high number of T-cells and poor prognosis. CONCLUSIONS: This study has shown that two markers - cardiotropic viruses and myocardial inflammation - are prevalent among DCM patients. They are also helpful in identifying sub-groups of DCM. An increased number of T-lymphocytes in the myocardium is a predictor of poor mid-term and long-term prognosis.

Echocardiographic reference intervals in healthy UK deerhounds and prevalence of preclinical dilated cardiomyopathy: a prospective, longitudinal study.

BACKGROUND: Sighthounds have high echocardiographic (ECHO) left ventricular volumes. Establishing robust breed-specific ECHO reference intervals (RI) for screening is important. End-diastolic volume index (EDVI), end-systolic volume index (ESVI) and ejection fraction (EF) reference ranges derived by Simpson's method of discs are not available for deerhounds. The influence of sex or body weight (BW) on left ventricular diameter during diastole (LVDd) and systole (LVDs) has never been reported. OBJECTIVES: Prospectively determine ECHO RI and assess prevalence of dilated cardiomyopathy (DCM) in healthy UK deerhounds. ANIMALS: Ninety-nine deerhounds. METHODS: Deerhounds scored on ECHO and ECG variables then classified as normal (NORM), equivocal (EQUIV) or affected (AFF) with DCM. Fifty-nine NORM deerhounds used to determine ECHO RI. RESULTS: Prevalence of DCM was 21.6%. There were significant differences in BW (P<0.001), LVDd (P<0.001) and LVDs (P<0.05) between female and male deerhounds. Cut-off values for EDVI (≥140.2 mL/m2: 79% sensitivity/97% specificity), ESVI (≥71.9 mL/m2: 94.7% sensitivity/94.2% specificity) and EF (≤42.1%: 84.2% sensitivity/92.8% specificity) were proposed to help diagnose DCM. The most reliable ECHO variables to identify AFF dogs were LVDs indexed to BW by allometric scaling and ESVI; one of the least reliable was sphericity index. Ventricular arrhythmias (VA) were identified in 13.6% of the population, with the highest prevalence in AFF deerhounds (42%). CONCLUSIONS: Preclinical DCM in deerhounds is common and VA may be associated with DCM. Healthy deerhounds have higher LVDd, LVDs and EDVI compared with other breeds. This study provides ECHO RIs for deerhounds; sex or BW RIs should be used when screening.

Heavy alcohol drinking and subclinical echocardiographic abnormalities of structure and function.

OBJECTIVE: The aim of the study is to assess changes in heart structure and function associated with heavy alcohol use by comparing echocardiographic indices in a population-based sample to those in patients admitted to an inpatient facility with severe alcohol problems. METHODS AND RESULTS: We used data from the Know Your Heart study (2015-2017) which is a cross-sectional study that recruited 2479 participants aged 35-69 years from the general population of the city of Arkhangelsk in Northwest Russia and 278 patients from the Arkhangelsk Regional Psychiatric Hospital with a primary diagnosis related to chronic alcohol use (narcology clinic subsample). The drinking patterns of the population-based sample were characterised in detail. We used regression models controlling for age, sex, smoking, education and waist to hip ratio to evaluate the differences in echocardiographic indices in participants with different drinking patterns. The means of left ventricular end-diastolic diameter and indexed left atrial systolic diameter were increased among heavy drinkers (narcology clinic subsample), while mean left ventricular ejection fraction was decreased in this group compared with the population-based sample. In contrast, the harmful and hazardous drinkers in the population-based sample did not differ from non-problem drinkers with respect to echocardiographic indices of systolic and diastolic function. CONCLUSIONS: Extremely heavy drinking is associated with a specific set of structural and functional abnormalities of the heart that may be regarded as precursors of alcohol-related dilated cardiomyopathy.

Thyrotoxicosis and dilated cardiomyopathy in developing countries.

BACKGROUND: Thyrotoxicosis is the state of thyroid hormone excess. But, in sub-Saharan Africa (SSA), specifically Northern Ethiopia, scientific evidence about thyrotoxicosis and its cardiac complications like dilated cardiomyopathy is limited. Therefore, this study aimed to explore the thyrotoxicosis presentation and management and identify factors associated with dilated cardiomyopathy in a tertiary hospital in Northern Ethiopia. METHODS: An institution-based cross-sectional study was conducted in Ayder Comprehensive Specialized Hospital from 2017 to 2018. Data from 200 thyrotoxicosis cases were collected using a structured questionnaire. After describing variables, logistic regression was conducted to identify independent predictors of dilated cardiomyopathy. Statistical significance was declared at p < 0.05. RESULTS: Mean age at presentation of thyrotoxicosis was 45 years and females accounted for 89 % of the cases. The most frequent etiology was multinodular toxic goiter (51.5 %). As well, the most common symptoms and signs were palpitation and goiter respectively. Thyroid storm occurred in 6 % of the cases. Out of 89 patients subjected to echocardiography, 35 (39.3 %) of them had dilated cardiomyopathy. And, the odds of dilated cardiomyopathy were higher in patients who had atrial fibrillation (AOR = 15.95, 95 % CI:5.89-38.16, p = 0.001) and tachycardia (AOR = 2.73, 95 % CI:1.04-7.15, p = 0.040). All patients took propylthiouracil and 13.0 % of them experienced its side effects. Concerning ß-blockers, propranolol was the most commonly (78.5 % of the cases) used drug followed by atenolol (15.0 %). Six patients underwent surgery. CONCLUSIONS: In developing countries like Ethiopia, patients with thyrotoxicosis have no access to methimazole which is the first-line anti-thyroid drug. Besides, they greatly suffer from dilated cardiomyopathy (due to late presentation) and side effects of propylthiouracil. Therefore, we recommend that patients should get adequate health information about thyrotoxicosis and anti-thyroid drugs including their side effects. Additionally, hospitals and other concerned bodies should also avail of TSH tests and methimazole at an affordable cost. Furthermore, community awareness about iodized salt and iodine-rich foods should be enhanced.

Drugs of Abuse and Heart Failure.

Substance use is common among those with heart failure (HF) and is associated with worse clinical outcomes. Alcohol, tobacco, cannabis, and cocaine are commonly abused substances that can contribute to the development and worsening of HF. Heavy alcohol consumption can lead to dilated cardiomyopathy, whereas moderate intake may decrease incident HF. Tobacco increases the risk of HF through coronary artery disease and coronary artery disease-independent mechanisms. Continued smoking worsens outcomes for those with HF and cessation is associated with an improved risk of major adverse cardiac events. Cannabis has complex interactions on the cardiovascular system depending on the method of consumption, amount consumed, and content of cannabinoids. Delta-9-tetrahydrocannabinol can increase sympathetic tone, cause vascular dysfunction, and may increase the risk of myocardial infarction. Cannabidiol is cardioprotective in preclinical studies and is a potential therapeutic target. Cocaine increases sympathetic tone and is a potent proarrhythmogenic agent. It increases the risk of myocardial infarction and can also lead to a dilated cardiomyopathy. The use of beta-blockers in those with HF and cocaine use is likely safe and effective. Future studies are needed to further elucidate the impact of these substances both on the development of HF and their effects on those who have HF.

Comparison of periodontal parameters between patients with ischemic and dilative cardiomyopathy.

BACKGROUND: This cross-sectional study aimed in the comparison of periodontal parameters, number of remaining teeth and oral behaviour between patients with ischemic- (ICM) and non-ischemic dilative cardiomyopathy (DCM). METHODS: Patients with HF from the Department for Cardiac Surgery at the Heart Center Leipzig were included. The two groups (ICM and DCM) were composed by matching according to age, gender and smoking habits. All participants received a comprehensive periodontal examination, including a periodontal probing on six measurement points of each tooth. RESULTS: A total of 226 patients (n = 113 each group) was included. Patients in DCM group used interdental cleaning significantly more often than ICM (23.9% vs. 12.5%, p = 0.04). The majority of patients in both groups (ICM: 83.6%, DCM: 84.6%, p = 0.23) were diagnosed with stage III-IV periodontitis. Periodontal parameters were comparable between groups (p > 0.05). Variance analysis revealed no influence of the group (ICM vs. DCM) on the number of remaining teeth (p = 0.16), periodontitis stage (p = 0.27) or the periodontal inflamed surface area (p = 0.62). CONCLUSIONS: Patients with severe HF show high periodontal burden, without any differences between ICM and DCM group. Therefore, increased attention should be payed to periodontal health of patients with severe heart disease, irrespective of their underlying disease.

Psoriasis as risk factor for non-ischemic dilated cardiomyopathy: a population-based cross-sectional study.

BACKGROUND: Psoriasis is a chronic inflammatory skin condition commonly associated with psoriatic arthritis, malignancy, diabetes, inflammatory bowel disease, and cardiovascular disease. Several reports and studies have reported an association between psoriasis and non-ischemic dilated cardiomyopathy (NIDCM). We aim to study the relationship between psoriasis and non-ischemic dilated cardiomyopathy in a large population-based study. METHODS: We utilized the Healthcare Cost and Utilization Project National Inpatient Sample 2017 database, which represents a 20% sample of all payer hospitalizations in the United States. We investigated hospitalizations for patients aged 18 years old or older with diagnoses of any type of psoriasis and non-ischemic dilated cardiomyopathy. Psoriasis, cardiomyopathy, and other comorbidities were identified through their international classification of diseases, 10th revision codes recorded in the discharge record for each hospitalization. RESULTS: Of a total of 6,084,184 all-cause admissions, 0.5% were admissions for patients with psoriasis (n = 32,807). Of the patients with and without psoriasis who had non-ischemic dilated cardiomyopathy, after adjusting for age, sex, race, diabetes mellitus, hypertension, alcohol abuse, cocaine abuse, arrhythmias, and obesity in a multivariate analysis, the presence of psoriasis was not significantly associated with non-ischemic dilated cardiomyopathy. CONCLUSION: Psoriasis is a chronic autoimmune disorder which carries a higher cardiovascular events and more prevalent traditional atherosclerotic risk factors in comparison to the general population. However, association with non-ischemic cardiomyopathy or NIDCM in particular has not been studied sufficiently. Our study, being one of the first larger studies to assess this correlation, indicated no relationship between psoriasis and non-ischemic dilated cardiomyopathy.

Epidemiological and pathological aspects of cardiomyopathies in cats in Southern Brazil / Aspectos epidemiológicos e patológicos das cardiomiopatias em gatos no Sul do Brasil

Cardiomyopathies are considered one of the most important causes of heart failure in cats and are subdivided into three main morphological types: hypertrophic (HCM), dilated (DCM), and restrictive (RCM). This study aimed to determine the frequency and types of cardiomyopathies in cats diagnosed in southern Brazil, with an emphasis on their epidemiological and pathological aspects. Necropsy reports filed in a veterinary pathology laboratory were reviewed, and cats diagnosed with cardiomyopathy were selected for the study. Animal identification data, history and clinical signs, and gross lesions, were reviewed and compiled. During the study period, 1.594 cat necropsies were performed, of which 72 (4.5%) comprised a diagnosis of cardiomyopathy. HCM was the most frequent followed by CMR and CMD, representing 77.8%, 12.5% and 9.7%, respectively. Age ranged from three months to 18 years, with a median age of seven years. In relation to sex, 62.5% were males and 37.5% females. In 76.4% of the cases, it affected cats without a breed defined. Restrictive mixed dyspnea and hydrothorax were the main signs or findings of the clinical examination. Sudden death and acute paresis of the pelvic limbs due to aortic thromboembolism have also been described. In HCM, myocardial thickening was observed, with a reduction in the ventricular chamber. Hypertrophy, disarray, and fibrosis of the myofibers were the main histological findings. In RCM, whitish and thickened endocardium was seen in most cases. DCM was characterized by dilated cardiac chambers, and microscopic examination revealed no significant findings. The main extra cardiac lesions revealed pulmonary edema and congestion, hydrothorax and chronic passive congestion of the liver. Cardiomyopathies are important causes of death in cats and should be included in the differential diagnosis of patients with cardio respiratory clinical signs and in cases related to sudden death and acute paresis of the pelvic limbs.(AU)

As cardiomiopatias são consideradas umas das mais importantes causas de insuficiência cardíaca em gatos e são subdivididas morfologicamente em três principais tipos: cardiomiopatia hipertrófica (CMH), dilatada (CMD) e restritiva (CMR). Este trabalho teve como objetivo determinar a frequência e os tipos de cardiomiopatias em gatos diagnosticados no Sul do Brasil, abordando seus aspectos epidemiológicos e patológicos. Foram revisados os laudos de necropsias de gatos e selecionados para o estudo de diagnóstico de cardiomiopatia. Os dados referentes à identificação do animal, o histórico/sinais clínicos e lesões macroscópicas foram revisados e compilados. No período estudado, foram realizadas 1.594 necropsias de gatos, destas, 72 (4,5%) compreenderam diagnóstico de cardiomiopatia. A CMH foi a mais frequente seguida pela CMR e CMD, representando 77,8%, 12,5% e 9,7%, respectivamente. A idade variou de três meses a 18 anos, com a idade mediana de sete anos. Em relação ao sexo, 62,5% eram machos e 37,5% fêmeas. Em 76,4% dos casos, afetou gatos sem raça definida. Dispneia mista restritiva e hidrotórax foram os principais sinais ou achados do exame clínico. Morte súbita e paresia aguda de membros pélvicos em razão do tromboembolismo aórtico também foram descritos. Na CMH, observou-se espessamento do miocárdio, com redução da câmara ventricular. Hipertrofia, desarranjo e fibrose das miofibras foram os principais achados histológicos. Na CMR, visualizou-se endocárdio esbranquiçado e espessado na maioria dos casos. A CMD caracterizou-se pela dilatação das câmaras cardíacas, e sem lesão histológica significante. As principais lesões extracardíacas encontradas foram edema e congestão pulmonares, hidrotórax e congestão passiva crônica do fígado. As cardiomiopatias são causas importantes de morte em gatos, devem ser incluídas no diagnóstico diferencial de pacientes com sinais clínicos cardiorrespiratórios e também em casos relacionados a morte súbita e paresia aguda dos membros pélvicos.(AU)

Risk of sudden cardiac death in New York Heart Association class I patients with dilated cardiomyopathy: A competing risk analysis.

AIMS: Primary prevention implantable cardioverter defibrillator (ICD) is not generally recommended in New York Heart Association (NYHA) I class patients with dilated cardiomyopathy (DCM). This study sought to assess the competing risk of sudden cardiac death (SCD) in DCM patients with left ventricular ejection fraction (EF) ≤35% and NYHA I class. METHODS: A total of 272 DCM patients with EF ≤35% and NYHA class I-III after ≥3 months of guideline-directed medical therapy were included. The risk of SCD and SCD/malignant ventricular arrhythmias (MVA) was assessed in NYHA I vs. NYHA II and NYHA III groups by competing risk analysis. RESULTS: NYHA I patients were younger, had higher EF and smaller left atrium, were less likely receiving mineral corticoid receptor antagonists. The cumulative incidence of SCD (p = 0.92) and SCD/MVA (p = 0.42) did not differ between NYHA I vs NYHA II-III classes. NYHA class did not influence the association between ICD and SCD risk (p for interaction = 0.125). CONCLUSIONS: In this cohort of DCMs, patients with EF ≤35% and NYHA I class were exposed to a risk of SCD and life-threatening arrhythmias not different from NYHA II-III. Therefore, inclusion of asymptomatic patients with DCM and systolic dysfunction should be strongly considered in future randomized studies on primary prevention ICD.

Body Mass Index in Young Women and Risk of Cardiomyopathy: A Long-Term Follow-Up Study in Sweden.

BACKGROUND: Incidence rates of cardiomyopathies, which are a common cause of heart failure in young people, have increased during the last decades. An association between body weight in adolescence and future cardiomyopathy among men was recently identified. Whether or not this holds true also for women is unknown. The aim was therefore to determine whether for young women being overweight or obese is associated with a higher risk of developing cardiomyopathy. METHODS: This was a registry-based national prospective cohort study with data collected from the Swedish Medical Birth Register, 1982 to 2014, with up to 33 years of follow-up. Included women were of childbearing age (18-45 years) during the initial antenatal visit in their first or second pregnancy (n=1 393 346). We obtained baseline data on body mass index (BMI), smoking, education, and previous disorders. After exclusions, mainly because of previous disorders, the final sample was composed of 1 388 571 women. Cardiomyopathy cases were identified by linking the Medical Birth Register to the National Patient and Cause of Death registers. RESULTS: In total, we identified 1699 cases of cardiomyopathy (mean age at diagnosis, 46.2 [SD 9.1] years) during the follow-up with an incidence rate of 5.9 per 100 000 observation years. Of these, 481 were diagnosed with dilated cardiomyopathy, 246 had hypertrophic cardiomyopathy, 61 had alcohol/drug-induced cardiomyopathy, and 509 had other forms. The lowest risk for being diagnosed with a cardiomyopathy was detected at a BMI of 21 kg/m2, with a gradual increase in risk with higher BMI, particularly for dilated cardiomyopathy, where a hazard ratio of 4.71 (95% CI, 2.81-7.89) was found for severely obese subjects (BMI ≥35 kg/m2), as compared with BMI 20 to <22.5. CONCLUSIONS: Elevated BMI among young women was associated with an increased risk of being diagnosed with a subsequent cardiomyopathy, especially dilated cardiomyopathy, starting already at mildly elevated body weight, whereas severe obesity entailed an almost 5-fold increase in risk. With the increasing numbers of persons who are overweight or obese, higher rates of cardiomyopathy can be expected in the future, along with an altered disease burden related to adiposity.

Sex Differences in the Long-term Prognosis of Dilated Cardiomyopathy.

BACKGROUND: Dilated cardiomyopathy (DCM) represents a specific phenotype of heart failure. Sex differences in the long-term prognosis of patients with DCM are unknown. The aim of this study is to investigate the long-term prognostic role of gender in a large cohort of patients with DCM. METHODS: A total of 1113 patients with DCM were prospectively enrolled. To investigate the impact of sex, a propensity score-matching analysis was performed on a sample of 586 patients. Univariable and multivariable Cox models and competing-risk analyses were estimated on both cohorts for the following outcome measures: (1) all-cause mortality/heart transplantation (HTx)/ventricular assist device (VAD); (2) cardiovascular mortality/HTx/VAD; and (3) sudden cardiac death or malignant ventricular arrhythmias. RESULTS: Women were older than men (50 ± 15 years vs 47 ± 15 years, respectively, P = 0.004) and more frequently had moderate to severe left ventricular dilation (P < 0.001) and left bundle branch block (P = 0.019). At multivariable analyses, male sex was independently associated with all considered outcome measures in the total cohort. At propensity score-matching analysis, over a median follow-up of 126 months (interquartile range, 62-201), 96 men (33%) vs 66 women (22%) experienced all-cause mortality/HTx/VAD (P = 0.03), 95 men (32%) vs 57 women (20%) experienced cardiovascular mortality/HTx/VAD (P = 0.025), and 46 men (16%) vs 28 women (10%) experienced sudden cardiac death/malignant ventricular arrhythmias (P = 0.07). CONCLUSION: The long-term outcomes of women affected by DCM are more favourable than those of men, and sex emerged as an important independent factor, particularly for cardiovascular outcomes.

Diagnostic characteristics of acute myocarditis.

BACKGROUND: Acute myocarditis is an inflammation of the heart muscle. Its unpredictable evolution justifies the importance of its early recognition. The clinical polymorphism associated with the lack of sensitivity of conventional diagnostic means make diagnosis a challenge for the clinician. However, the magnetic resonance imaging has been of great interest for the differential diagnosis as well as for the evolutionary follow-up of this pathology. AIM: Determine the clinical profile of acute myocarditis and the contribution of multimodal imaging in its diagnosis. METHODS: This was a descriptive, retrospective study, including 31 patients hospitalized for acute myocarditis at the cardiology department of the Internal Security Forces Hospital-La Marsa between 2011 and 2017. RESULTS: The mean age of our patients was 36.5 ± 13.3 years [17-63] with a clear male predominance (sex ratio = 6.75). Smoking was the most common cardiovascular risk factor (60%). Fifty percent of the population had only two cardiovascular risk factors. The most common clinical picture was acute chest pain (84%) preceded by influenza-like illness (53%). The electrocardiogram was pathological in 97% of cases. Hyperleukocytosis was objectified in 33% of cases. Elevation of C-reactive protein was present in 80% of cases. As for troponins, they were high in 94% of cases. Overall myocardial contractility was conserved in 84% of cases with segmental kinetic disorders in 45%. Cardiac magnetic resonance imaging showed pericardial effusion (10%), spontaneous myocardial hypersignal in triple-reversal-T2 recovery (42%) and which corresponded to the zones of late enhancement. Late contrast enhancement was predominant at the lateral wall (39%), with epicardial involvement occurring in 100% of cases.28 patients had a favorable outcome after a follow-up of 24 months. However, there was only one case of death at 3 months and two cases that progressed to dilated cardiomyopathy. CONCLUSION: Acute myocarditis is mainly a disease of the young male subject. Cardiac magnetic resonance imaging is of crucial interest for positive diagnosis.

Role of family history and clinical screening in the identification of families with idiopathic dilated cardiomyopathy in Johannesburg, South Africa.

BACKGROUND: Familial disease is implicated in 20 - 50% of cases of idiopathic dilated cardiomyopathy (IDCM) worldwide. The contribution of familial factors to IDCM in the Johannesburg area, South Africa, is unknown. OBJECTIVES: To describe the demographic details of patients with IDCM who presented at Charlotte Maxeke Johannesburg Academic Hospital (CMJAH), and to determine if there is evidence of familial disease through family history assessment and clinical screening of relatives. METHODS: This was a single-centre, cohort study performed at a quaternary care centre at CMJAH. Fifty unrelated probands diagnosed with IDCM and available first- and second-degree relatives were included in the study. A three-generation family pedigree was drawn up for all 50 probands. The pedigrees were analysed to identify the presence or absence of familial disease and categorised as positive, intermediate, negative or unreliable according to the family history obtained. From the 50 proband cases, there were 21 family members available for screening for features of IDCM. Eighty-two family members (55 first-degree and 27 second-degree relatives) were screened clinically. Screening included a personal history, full physical examination, electrocardiogram (ECG) and echocardiogram. RESULTS: The mean age at diagnosis of IDCM in the probands was 41.7 (standard deviation (SD) 12.4) years. The majority of probands were males (n=38; 76%). Of 50 pedigrees analysed, 14 (28%) were positive and likely to be indicative of familial dilated cardiomyopathy (DCM), and 9 (18%) patients were at intermediate risk of familial disease. Eighty-two asymptomatic family members were screened, with a median age of 33 (range 11 - 76) years. No asymptomatic family members were identified with features of DCM or presymptomatic DCM. Eleven of the 21 families screened had relatives with possible presymptomatic DCM identified by abnormalities on the echocardiogram in 3 families (14.3%) (4 individuals; all first-degree relatives of the index case) or identified on the basis of a conduction defect (an arrhythmia or first-/ second-/third-degree heart block) in 8 families (72.7%) (11 individuals; 9 first-degree and 2 second-degree relatives). CONCLUSIONS: Screening for IDCM should include a three-generation family history and clinical screening of all first-degree family members. As IDCM has an age-related penetrance, at-risk family members should receive follow-up for screening to assess symptoms and signs of IDCM. Genetic testing would potentially identify family members at high risk, who would benefit from screening; this might be a less expensive option.

Arrhythmic risk stratification in patients with dilated cardiomyopathy and intermediate left ventricular dysfunction.

AIMS: Arrhythmic risk stratification is a challenging issue in patients with dilated cardiomyopathy (DCM), particularly when left ventricular ejection fraction (LVEF) is more than 35%. We studied the prevalence and predictors of sudden cardiac death or malignant ventricular arrhythmias (SCD/MVAs) in DCM patients categorized at low arrhythmic risk because of intermediate left ventricular dysfunction under optimal medical treatment (OMT). METHODS: DCM patients considered at low arrhythmic risk (LVEF >35% and New York Heart Association class I-III after 6 ±â€Š3 months of OMT) were analysed. An arrhythmogenic profile was defined as the presence of at least one among a history of syncope, nonsustained ventricular tachycardia, at least 1000 premature ventricular contractions/24 h, at least 50 ventricular couplets/24 h at Holter ECG monitoring. SCD/MVAs was considered as the study end-point. RESULTS: During a median follow-up of 152 months (interquartile range 100-234), 30 out of 360 (8.3%) patients at low arrhythmic risk (LVEF 47 ±â€Š7%) experienced the study end-point [14 (3.9%) SCD and 16 (4.4%) MVA]. Compared with survivors, patients who experienced SCD/MVAs had more frequently an arrhythmogenic profile and a larger left atrium. Their LVEF at the last available evaluation before the arrhythmic event was 36 ±â€Š12%. At multivariable analysis, left atrial end-systolic area [hazard ratio 1.107; 95% confidence interval (95% CI) 1.039-1.179, P = 0.002 for 1 mm increase] and arrhythmogenic profile (hazard ratio 3.667; 95% CI 1.762-7.632, P = 0.001) emerged as predictors of SCD/MVAs during follow-up. CONCLUSION: A consistent quota of DCM patients with intermediate left ventricular dysfunction receiving OMT experienced SCD/MVA during follow-up. Left atrial dilatation and arrhythmogenic pattern were associated with a higher risk of SCD/MVA.

Outcomes of heart transplantation in patients with human immunodeficiency virus.

Human immunodeficiency virus-positive (HIV+) patients are not routinely offered heart transplantation (HT) due to lack of adequate outcomes data. Between January 2004 and March 2017, we identified 41 adult (≥18 years) HT recipients with known HIV+ serostatus at the time of transplant in UNOS and evaluated post-HT outcomes. Overall, Kaplan-Meier (KM) estimates of survival at 1 and 5 years were 85.9% and 77.3%, respectively, with no significant difference in bridge-to-transplant ventricular-assist device (BTT-VAD, n = 22) and no-BTT-VAD (n = 19). KM estimates of cardiac allograft vasculopathy (CAV) and malignancy at 5 years were 32% and 19%, respectively. Using propensity scores, 41 HIV+ HT recipients were matched to 41 HIV- HT recipients for idiopathic dilated-cardiomyopathy; and there was no significant difference in post-HT survival up to 5 years. Furthermore, only 24 centers in the United States had performed HIV+ HT during the study period, indicating that >80% of HT centers in the United States had not performed any HIV+ HT. In a cohort representative of the current status of HIV+ HTs in the United States, we found that the posttransplant survival was excellent and rates of CAV and malignancy were comparable to the overall HT population. These results should encourage greater number of centers to offer HT to suitable HIV+ candidates and help reduce unequal access to HT for HIV+ patients.

Atrial fibrillation in patients with inherited cardiomyopathies.

Atrial fibrillation (AF) often complicates the course of inherited cardiomyopathies and, in some cases, may be the presenting feature. Each inherited cardiomyopathy has its own peculiar pathogenetic characteristics that can contribute to the development and maintenance of AF. Atrial fibrillation may occur as a consequence of disease-specific defects, non-specific cardiac chamber changes secondary to the primary illness, or a combination thereof. The presence of AF can denote a turning point in the progression of the disease, promoting clinical deterioration and increasing morbidity and mortality. Furthermore, the management of AF can be particularly challenging in patients with inherited cardiomyopathies. In this article, we review the current information on the prevalence, pathophysiology, risk factors, and treatment of AF in three different inherited cardiomyopathies: hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia/cardiomyopathy, familial dilated cardiomyopathy, and left ventricular non-compaction cardiomyopathy.

Genetic architecture of recent-onset dilated cardiomyopathy in Moravian region assessed by whole-exome sequencing and its clinical correlates.

AIMS: Recent-onset dilated cardiomyopathy (RODCM) is a disease of heterogeneous aetiology and clinical outcome. In this pilot study, we aimed to assess its genetic architecture and correlate genotype with left ventricular reverse remodelling (LVRR). PATIENTS AND METHODS: In this multi-centre prospective observational study, we enrolled 83 Moravian patients with RODCM and a history of symptoms of less than 6 months, for whole-exome sequencing (WES). All patients underwent 12-month clinical and echocardiographic follow-up. LVRR was defined as an absolute increase in left ventricular ejection fraction > 10% accompanied by a relative decrease of left ventricular end-diastolic diameter > 10% at 12 months. RESULTS: WES identified at least one disease-related variant in 45 patients (54%). LVRR occurred in 28 patients (34%), most often in carriers of isolated titin truncated variants, followed by individuals with a negative, or inconclusive WES and carriers of other disease-related variants (56% vs. 42% vs. 19%, P=0.041). CONCLUSION: A substantial proportion of RODCM cases have a monogenic or oligogenic genetic background. Carriers of non-titin disease-related variants are less likely to reach LVRR at 12- months than other individuals. Genetic testing could contribute to better prognosis prediction and individualized treatment of RODCM.