Association Between Race/Ethnicity and Severity of Illness in Pediatric Cardiomyopathy and Myocarditis.

INTRODUCTION: Previous reports demonstrate racial/ethnic differences in survival for children hospitalized with cardiomyopathy and myocarditis. The impact of illness severity, a potential mechanism for disparities, has not been explored. METHODS: Using the Virtual Pediatric Systems (VPS, LLC), we identified patients ≤ 18 years old admitted to the intensive care unit (ICU) for cardiomyopathy/myocarditis. Multivariate regression models were used to evaluate the association between race/ethnicity and Pediatric Risk of Mortality (PRISM 3). Multivariate logistic and competing risk regression was used to examine the relationship between race/ethnicity and mortality, CPR, and ECMO. RESULTS: Black patients had higher PRISM 3 scores on first admission (𝛽 = 2.02, 95% CI: 0.15, 3.90). There was no difference in survival across race/ethnicity over multiple hospitalizations. Black patients were less likely to receive a heart transplant (SHR = 0.65, 95% CI: 0.45-0.92). Black and unreported race/ethnicity had higher odds of CPR on first admission (OR = 1.64, 95% CI: 1.01-2.45; OR = 2.12, 95% CI: 1.11-4.08, respectively). CONCLUSION: Black patients have higher severity of illness on first admission to the ICU, which may reflect differences in access to care. Black patients are less likely to receive a heart transplant. Additionally, Black patients and those with unreported race/ethnicity had higher odds of CPR, which was not mediated by severity of illness, suggesting variations in care may persist after admission.

Atypical presentation of Takayasu aortitis and myocarditis contributing to sudden death in a young Canadian first nations adult.

This is a rare presentation of Takayasu arteritis in a 30-year-old Canadian First Nations woman with cardiac and aortic root-predominant disease, which manifested in complete heart block. She had a past medical history significant for substance misuse. At presentation, cardiac magnetic resonance imaging identified diffuse thickening of the left atrium and ventricular outflow tract with left ventricular cavity dilation and preserved systolic function. A pacemaker was inserted at this time. Nine months later, the patient died following an out-of-hospital cardiac arrest in the context of cocaine intoxication. At autopsy, the cardiac thickening was also found to involve the proximal aortic root, which on microscopy demonstrated non-infectious aortitis and myocarditis with a granulomatous inflammatory pattern and dense fibrosis indicative of Takayasu arteritis. Important clinical clues to the diagnosis include age, sex, and Pacific Islands, American indigenous and Asian ethnicity. The case also underscores the need to rule out secondary causes of complete heart block, including systemic vasculitides, for all patients regardless of substance use history.

Racial Disparities in Hospital Mortality Among Pediatric Cardiomyopathy and Myocarditis Patients.

Racially disparate health outcomes exist for a multitude of populations and illnesses. It is unknown how race and ethnicity impact mortality for children with cardiomyopathy or myocarditis. This retrospective cross-sectional study employed the Kids' Inpatient Database to analyze 34,617 hospital admissions for patients ≤ 18 years old with cardiomyopathy, myocarditis, or both, without concomitant congenital heart disease. Multivariate logistic regression models investigated the impact of race/ethnicity on in-hospital mortality adjusting for age, calendar year, sex, insurance type, diagnostic category, treatment at a pediatric hospital, and non-cardiac organ dysfunction. African American race and Hispanic ethnicity were independent risk factors for mortality (African American: odds ratio (OR) 1.25, 95% confidence interval (CI) 1.01-1.53 and Hispanic: OR 1.29, 95% CI 1.03-1.60). African American race was also found to be significantly associated with the use of extracorporeal membrane oxygenation (ECMO), mortality while on ECMO, and cardiac arrest. Adjusting the regression model for ECMO and arrest attenuated the impact of African American race on mortality, suggesting that these variables may indeed play a role in explaining the impact of race on mortality for African American patients with myocardial disease. Hispanic ethnicity remained associated with higher risk of mortality despite controlling for all mechanical circulatory support and transplant (OR 1.30, 95% CI 1.04-1.63). Children of racial and ethnic minorities hospitalized with cardiomyopathy or myocarditis are more likely to die than their white counterparts, a trend that may be due at least in part to in-hospital differences in care or response to therapy.

Clinical features and outcome of lupus myocarditis in the Western Cape, South Africa.

BACKGROUND: African American ethnicity is independently associated with lupus myocarditis compared with other ethnic groups. In the mixed racial population of the Western Cape, South Africa, no data exists on the clinical features/outcome of lupus myocarditis. OBJECTIVES: The objective of this study was to give a comprehensive description of the clinical features and outcome of acute lupus myocarditis in a mixed racial population. METHODS: Clinical records (between 2008 and 2014) of adult systemic lupus erythematosus (SLE) patients at a tertiary referral centre were retrospectively screened for a clinical and echocardiographic diagnosis of lupus myocarditis. Clinical features, laboratory results, management and outcome were described. Echocardiographic images stored in a digital archive were reanalysed including global and regional left ventricular function. A poor outcome was defined as lupus myocarditis related mortality or final left ventricular ejection fraction (LVEF) <40%. RESULTS: Twenty-eight of 457 lupus patients (6.1%) met inclusion criteria: 92.9% were female and 89.3% were of mixed racial origin. Fifty-three per cent of patients presented within three months after being diagnosed with SLE. Seventy-five per cent had severely active disease (SLE disease activity index ≥ 12) and 67.9% of patients had concomitant lupus nephritis. Laboratory results included: lymphopenia (69%) and an increased aRNP (61.5%). Treatment included corticosteroids (96%) and cyclophosphamide (75%); 14% of patients required additional immunosuppression including rituximab. Diastolic dysfunction and regional wall motion abnormalities occurred in > 90% of patients. LVEF improved from 35% to 47% (p = 0.023) and wall motion score from 1.88 to 1.5 (p = 0.017) following treatment. Overall mortality was high (12/28): five patients (17.9%) died due to lupus myocarditis (bimodal pattern). Patients who died of lupus myocarditis had a longer duration of SLE (p = 0.045) and a lower absolute lymphocyte count (p = 0.041) at diagnosis. LVEF at diagnosis was lower in patients who died of lupus myocarditis (p = 0.099) and in those with a persistent LVEF < 40% (n = 5; p = 0.046). CONCLUSIONS: This is the largest reported series on lupus myocarditis. The mixed racial population had a similar prevalence, but higher mortality compared with other ethnic groups (internationally published literature). Patients typically presented with high SLE disease activity and the majority had concomitant lupus nephritis. Lymphopenia and low LVEF at presentation were of prognostic significance, associated with lupus myocarditis related mortality or a persistent LVEF < 40%.

Typhoid fever, complicated by myocarditis, in a traveller returning to the UK.

A 25-year-old male returning traveller presented with sudden onset chest pain. An ECG showed infero-lateral ischaemic changes, with an elevated troponin and inflammatory markers. An echocardiogram showed a normal size left ventricle, dynamic systolic function, structurally normal valves and no regional wall motion abnormality. Angiography revealed normal coronary arteries. A diagnosis of myocarditis was made. Five days later, he developed a significant pyrexia and diarrhoea. Salmonella typhi was isolated from blood cultures. The fever and symptoms resolved after 2 weeks of an intravenous third generation cephalosporin and the patient was discharged.

The persistent challenge of rheumatic fever in the Northern Mariana Islands.

OBJECTIVES: Acute rheumatic fever (ARF) is a major cause of morbidity and mortality in developing nations. The objective of this study was to determine the disease burden of ARF among the indigenous and non-indigenous populations of the Northern Mariana Islands. METHODS: This was a chart review of all pediatric outpatients seen from 1984 to 2006 with ICD-9 codes corresponding to rheumatic fever or any rheumatic cardiac sequelae. The study was set in the only comprehensive inpatient facility and only public pediatric clinic in the Northern Mariana Islands. RESULTS: One hundred fifty-eight cases of ARF were identified. Age at diagnosis ranged from 2.9 to 17.1 years (median 10.6 years). Fever and carditis were the most common presenting findings. The average annualized incidence of ARF was 85.8 per 100,000 person-years for those aged 5-14 years. Sixty-six percent of patients with ARF were of Chamorro or Carolinian ancestry, despite comprising only 39% of the total population, with a combined average annualized incidence of ARF of 167 per 100,000 person-years. CONCLUSIONS: This is the first documentation of the incidence of pediatric ARF in the Northern Mariana Islands, delineating the large disease burden in the indigenous and other Pacific Island ethnic groups. Impediments to diagnosis and primary and secondary prevention were identified. The data provide strong support for the need for primary and secondary prevention of ARF.

Associated factors and impact of myocarditis in patients with SLE from LUMINA, a multiethnic US cohort (LV). [corrected].

OBJECTIVE: To examine the factors associated with myocarditis and its impact on disease outcomes in SLE patients. METHODS: SLE patients aged > or = 16 yrs, disease duration < or = 5 yrs from LUMINA (LUpus in Minorities: NAture vs nurture), a multiethnic US cohort, were studied. Myocarditis was defined as per the category 3 of the pericarditis/myocarditis item of the SLAM-Revised (SLAM-R). Patients with concurrent pericardial involvement were excluded. Patients with myocarditis were compared with those without myocarditis or its sequelae in the preceding year. The association between myocarditis and baseline variables (T(0)) was first examined. The impact of myocarditis on disease activity over time (SLAM-R), damage accrual [SLICC Damage Index (SDI)] at last visit (T(L)) and mortality was evaluated. RESULTS: Fifty-three of the 496 patients studied had myocarditis. African American ethnicity [Odds ratio (OR) = 12.6; 95% CI 1.6, 97.8] and SLAM-R at diagnosis (OR = 1.1, 95% CI 1.0, 1.1) were significantly and independently associated with myocarditis. Myocarditis did not predict disease activity over time, but approached significance as a predictor of SDI at T(L) in multivariable analyses P = 0.051. Kaplan-Meier curves indicated that myocarditis was associated with shorter survival (log-rank = 4.87, P = 0.02), particularly in patients with > or = 5 yrs disease; however, myocarditis was not retained in the Cox proportional hazards regression model. CONCLUSIONS: Ethnicity and disease activity at diagnosis were associated with the occurrence of myocarditis in SLE. Myocarditis did not significantly impact on disease activity over time, but impacts some on damage accrual and survival, reflecting overall the more severe disease those patients experience.

Evidence of viral infection in the myocardium of American and Japanese patients with idiopathic dilated cardiomyopathy.

Enteroviruses have been implicated in the pathogenesis of idiopathic dilated cardiomyopathy (IDC). Recently, the association of adenovirus or parvovirus with IDC has been reported. Viral infection in the myocardium of American and Japanese patients with end-stage IDC was evaluated. Myocardial specimens from 30 American patients with IDC and 47 Japanese patients with IDC were analyzed for the presence of cardiotropic viruses. The strand-specific detection of enteroviral ribonucleic acid (RNA) was performed to determine viral activity in hearts with IDC. Established reverse transcription-polymerase chain reaction (PCR) or PCR techniques were used to detect genomic sequences of influenza viruses, mumps virus, adenovirus, parvovirus, herpes simplex viruses, varicella-zoster virus, and Epstein-Barr virus. Enteroviral RNA was detected in 7 of the 30 American patients (23%) and in 15 of the 47 Japanese patients (32%). Minus-strand enteroviral RNA, an indicator of active enteroviral RNA replication, was demonstrated in 5 of 7 plus-strand-positive American patients (71%) and in 12 of 15 plus-strand-positive Japanese patients (80%). Sequence analysis revealed that the viruses detected were Coxsackie B viruses. No genomic sequences of other viruses were detected in the myocardium of either American or Japanese patients with IDC. Therefore, active group B Coxsackie virus RNA replication in the myocardium was demonstrated in a significant proportion of American and Japanese patients with end-stage IDC. There was no evidence of persistent infection by other viruses in hearts with IDC. Specific therapy should be designed for Coxsackie virus positive patients with IDC.

Acute rheumatic fever in adults: a resurgence in the Hasidic Jewish community.

OBJECTIVE: To describe a series of adults diagnosed with acute rheumatic fever (ARF). METHODS: Retrospective chart review of 14 patients age > 18 years with suspected ARF between 1990 and 1994 in a private rheumatology practice setting. Four additional patients treated at our medical center were included in the study. RESULTS: Twelve patients met Jones criteria for rheumatic fever and were included in the study. Of these, only 3 had a childhood history of rheumatic fever. All had recent onset of arthritis and a history of antecedent sore throat. Only 4 patients, however, had throat cultures positive for B-hemolytic streptococcus. Nine patients were Hasidic Jews. Four patients had carditis. One patient had erythema marginatum, while chorea and subcutaneous nodules were not seen. Nine patients improved taking nonsteroidal antiinflammatory drugs or acetylsalicylic acid; 3 required steroid treatment to control severe arthritis. CONCLUSION: Our clinical experience suggests that ARF occurs frequently, especially among Hasidic Jewish adults. Due to the disabling nature of the arthritis and the significant incidence (33%) of carditis, strict adherence to penicillin prophylaxis guidelines is indicated.

Acute rheumatic fever in New York City (1969 to 1988): a comparative study of two decades.

One hundred fifteen attacks of acute rheumatic fever were observed during the past two decades (1969 to 1988) at the Columbia-Presbyterian Medical Center; 104 were initial attacks and 11 were recurrences. The maximum number of cases (18) occurred during the first year of this period, 1969; thereafter the annual incidence decreased until a resurgence was apparent in 1985 to 1986, with 25 cases. There were no differences in the frequency of major manifestations nor the severity of carditis in the 1970s compared with the 1980s. Polyarthritis alone was present in 51 cases, carditis alone in 31, and combined carditis and polyarthritis in 28; chorea was diagnosed in 5. Congestive heart failure occurred in 17 attacks of carditis, including one death from fulminant disease in 1982. In contrast to recent reports, the majority of the affected population was urban, low income, and from crowded communities: more than half were Hispanic, predominantly from Dominican Republic families. The prevalence of acute rheumatic fever underscores the need for early detection and treatment of streptococcal pharyngitis. The observation that 9.5% of the attacks were recurrent reflects failure to comply with antimicrobial prophylaxis and reaffirms the need for more effective secondary prevention programs.