Cardiovascular magnetic resonance predictors of heart failure in hypertrophic cardiomyopathy: the role of myocardial replacement fibrosis and the microcirculation

Introduction: Heart failure (HF) in hypertrophic cardiomyopathy (HCM) is associated with high morbidity and mortality. Predictors of HF, in particular the role of myocardial fibrosis and microvascular ischemia remain unclear. We assessed the predictive value of cardiovascular magnetic resonance (CMR) for development of HF in HCM in an observational cohort study. Methods: Serial patients with HCM underwent CMR, including adenosine first-pass perfusion, left atrial (LA) and left ventricular (LV) volumes indexed to body surface area (i) and late gadolinium enhancement (%LGE- as a % of total myocardial mass). We used a composite endpoint of HF death, cardiac transplantation, and progression to NYHA class III/IV. Results: A total of 543 patients with HCM underwent CMR, of whom 94 met the composite endpoint at baseline. The remaining 449 patients were followed for a median of 5.6 years. Thirty nine patients (8.7%) reached the composite endpoint of HF death (n = 7), cardiac transplantation (n = 2) and progression to NYHA class III/IV (n = 20). The annual incidence of HF was 2.0 per 100 person-years, 95% CI (1.6–2.6). Age, previous non-sustained ventricular tachycardia, LV end-systolic volume indexed to body surface area (LVESVI), LA volume index ; LV ejection fraction, %LGE and presence of mitral regurgitation were significant univariable predictors of HF, with LVESVI (Hazard ratio (HR) 1.44, 95% confidence interval (95% CI) 1.16–1.78, p = 0.001), %LGE per 10% (HR 1.44, 95%CI 1.14–1.82, p = 0.002) age (HR 1.37, 95% CI 1.06–1.77, p = 0.02) and mitral regurgitation (HR 2.6, p = 0.02) remaining independently predictive on multivariable analysis. The presence or extent of inducible perfusion defect assessed using a visual score did not predict outcome (p = 0.16, p = 0.27 respectively). Discussion: The annual incidence of HF in a contemporary ambulatory HCM population undergoing CMR is low. Myocardial fibrosis and LVESVI are strongly predictive of future HF, however CMR visual assessment of myocardial perfusion was not

Functional Outcomes after Inpatient Rehabilitation in a Prospective Stroke Cohort

Aim: Rehabilitation is proven to improve function following a stroke. The functional outcomes of stroke patients and benefits from rehabilitation are highly variable. We aim to describe functional outcomes and gains following inpatient rehabilitation post-stroke and identify factors associated with improved outcomes. Methods: In this five-years prospective study, data for 1332 consecutive stroke patients admitted to the Singapore General Hospital inpatient rehabilitation unit were charted into a custom-designed rehabilitation database. The primary outcome measure was the Functional Independence Measure (AFIM) and discharge (DFIM). The functional gain was defined as the DFIM-AFIM. Results: The mean age was 64.1±12.5 years, 58.9% were male patients and 78.9% consisted of ischaemic (versus haemorrhagic) strokes. The average rehabilitation length of stay (RLOS) was 18.7±13.9 days and the majority (87.7%) were discharged home. The most common risk factor was hypertension (78.4%) and urinary tract infection (21.2%) was the commonest post-stroke complication. The mean AFIM and DFIM scores were 67.9±23.0 and 83.2±23.5 respectively with a mean functional gain of 15.4±12.3 FIM points. Younger, male, and haemorrhagic stroke patients had better functional outcomes. Multiple regression analysis results revealed that higher DFIM score was associated with higher admission motor and cognitive FIM scores, younger age, male gender, employment at admission, single patients, presence of a caregiver, haemorrhagic stroke, right-sided motor impairments, absence of urinary tract infection or depression, acupunction treatment, and a longer RLOS. The regression model on functional gain was associated with similar independent predictors on DFIM scores except that a higher AFIM was associated with lower functional gains. Conclusion: In this large cohort study, stroke patients make significant functional gains and should be offered rehabilitation to improve outcomes. A comprehensive set of multiple interacting demographic, clinical, cultural, and social factors significanctly impact on stroke functional outcomes after inpatient rehabilitation. The identification of these factors allows optimising rehabilitation for individual patients, and is important for discharge and resource planning

CASE REPORT Huntington Disease: Report of First Case Documented in Malaysia

Huntington disease (HD), described by George Huntington in 1872, is a genetic disorder which is characterised by involuntary movements and dementia beginning in midlife, with unrelenting progress t

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Abstract Objective: We assessed the psychometric properties of a Singaporean Chinese version of the EQ-5D, a health-related quality of life (HRQoL) instrument. Materials and Methods: Consecutive outpatients with rheumatic diseases seen for routine follow-up consultations at the National University Hospital, Singapore were interviewed twice within 2 weeks using a standardised questionnaire containing the EQ-5D, the Short-Form 36 Health Survey (SF-36), the Learned Helplessness Subscale, a pain Visual Analogue Scale (VAS) and assessing demographic and psychosocial characteristics. To assess the validity of the EQ-5D, 13 hypotheses relating the EQ-5D self-classifier (5 dimensions) or visual analogue scale (EQ-VAS) to SF-36 scores or other variables were examined using the Mann-Whitney U test, KruskalWallis or Spearman's correlation coefficient. Test-retest reliability was assessed using Cohen's kappa. Results: Forty-eight subjects were studied (osteoarthritis: 16; rheumatoid arthritis: 22; systemic lupus erythematosus: 8; spondyloarthropathy: 2; female: 93.8%; mean age: 56.4 years). Seven of 13 a-priori hypotheses relating EQ-5D to external variables were fulfiled, supporting the validity of the EQ-5D. For example, subjects reporting moderate or extreme problems for EQ-5D dimensions generally had lower median SF-36 scores than those without such problems. Cohen's kappa for test-retest reliability of the self-classifier ranged from 0.41 to 1.00 (n = 42; median interval: 7 days, interquartile range: 7 to 11 days). Conclusions: The Singaporean Chinese EQ-5D self-classifier appears to be a valid measure of HRQoL in Singaporeans with rheumatic diseases; however, the reliability of the EQ-VAS requires further investigation. These data provide a basis for further studies of the Singaporean Chinese EQ-5D. Ann Acad Med Singapore 2003; 32:685-9

ON T2* MAGNETIC RESONANCE AND CARDIAC IRON

Background: Measurement of myocardial iron is key to the clinical management of patients at ris