Search

Homozygous familial hypercholesterolaemia (FH) causes severe cardiovascular disease from childhood. Conventional drug therapy is usually ineffective; lipoprotein apheresis (LA) is often required. Liver transplantation (LT) can correct the metabolic defect but is considered a treatment of last resort. Newer drugs including lomitapide and evinacumab might reduce the need for apheresis and LT. We sought to determine the long-term outcomes following LT in Australia and New Zealand. We analysed demographic, biochemical and clinical data from all patients in Australia and New Zealand who have received LT for homozygous FH, identified from the Australia and New Zealand Liver and Intestinal Transplant Registry. Nine patients (five female; one deceased; seven aged between 3 and 6 years at the time of LT and two aged 22 and 26 years) were identified. Mean follow-up was 14.1 years (range 4–27). Baseline LDL-cholesterol off all treatment was 23 ± 4.1 mmol/L. Mean LDL-cholesterol on medical therapy (including maximal statin therapy in all patients, ezetimibe in three and LA in five) was 11 ± 5.7 mmol/L (p < 0.001). After LT, mean LDL-cholesterol was 2.6 ± 0.9 mmol/L (p = 0.004) with three patients remaining on statin therapy and none on LA. One patient died from acute myocardial infarction (AMI) three years after LT. Two patients required aortic valve replacement, more than 10 years after LT. The remaining six patients were asymptomatic after eight to 21 years of follow-up. No significant adverse events associated with immunosuppression were reported. LT for homozygous FH was highly effective in achieving substantial long-term reduction in LDL-cholesterol concentrations in all nine patients. LT remains an option for severe cases of homozygous FH where drug therapy combined with apheresis is ineffective or unfeasible. [Display omitted] • Liver transplantation has low morbidity in experienced centres. • Modern immunosuppression facilitates long-term graft survival. • Liver transplantation lowers LDL-cholesterol more than any other treatment. • Aortic stenosis can progress despite liver transplantation. • Post-transplant familial hypercholesterolaemia cardiovascular guidelines are needed. [ABSTRACT FROM AUTHOR]

Background: Guidelines previously recommended use of dual antiplatelet therapy, statins, angiotensin-converting enzyme inhibitors or angiotensin receptor blockers (ACEI/ARB) and beta blockers (five classes of drugs) in patients without contraindications or intolerance after acute coronary syndrome (ACS). However, recent guidelines have taken a more nuanced view regarding the use of ACEI/ARB and beta blockers. Our aim was to develop a composite post-discharge medication indicator, based on available evidence, to support quality improvement.Methods: 4,112 consecutive post-ACS patients who underwent coronary angiography and left ventricular ejection fraction (LVEF) assessment in 2015-16 were recorded in the All New Zealand ACS Quality Improvement (ANZACS-QI) registry. Patients receiving coronary artery bypass grafting were excluded. Three composite indicator algorithms that took into account known contraindications/intolerances were compared across NZ District Health Boards (DHBs): RESULTS: Overall and individual DHB performance was highest (74%, DHB range 52-84%) when reported using the NHFA/CSANZ indicator, and slightly lower (69%, DHB range 48-78%) on the ANZACS-QI indicator. Performance was lowest using the older five-drug-class indicator (65%, DHB range 48-77%).Conclusions: We have developed a composite post-discharge medication indicator appropriate for use in identifying gaps in evidence-based management across NZ, which is now being reported regularly to DHBs. [ABSTRACT FROM AUTHOR]

Background: Cardiac troponin (T and I) are considered the standard markers for detection of myocardial damage and the diagnosis of acute coronary syndrome (ACS) among patients who present to an emergency department with chest pain. However, these markers can be released in other situations and may be associated with short- and long-term clinical outcomes. In this study, we examine late mortality rates among patients presenting with a suspected ACS due to an unstable coronary plaque and those patients having a non-ACS.Methods: 4388 patients were hospitalised with suspected ACS, between 14 and 27 May 2012 in the Australia and New Zealand SNAPSHOT ACS study. Those patients were categorised in five diagnostic groups: 1) ST elevation MI (n=419); 2) non-ST elevation MI (n=1012); 3) unstable angina (n=925); 4) non-ACS diagnoses (n=837); and 5) chest pain considered unlikely ischaemic (not otherwise specified, n=1195).Result: The respective mortality rates at 18 months in these groups were 16.2%, 16.3%, 6.8%, 12.8%, and 4.8%; Pearson χ2=110 p<0.001. Among non-ACS diagnoses patients (group 4) those with the highest mortality rates (cardiac (14.4%), respiratory (18.2%), sepsis (15.4%) and neoplastic (67%) diagnoses) had the highest rates of elevated troponin levels (48%, 31%, 38% and 67% respectively). By contrast, those with the lowest mortality rates (musculoskeletal (2.9%), gastrointestinal disorders (3.9%) and non-specific chest pain (7.4%)) had the lowest rate of elevated troponin levels (9%, 18% and 15.8% respectively). However, after adjusting for baseline clinical and demographic characteristics, the mortality rate at 18 months for patients with elevated troponin was similar for ACS or non-ACS diagnoses (Hazard Ratio, 95% C.I.0.98-1.07, p=0.333).Conclusions: Among patients in the 2012 SNAPSHOT ACS study, non-ACS diagnoses characterised by high rates of elevated troponin levels had high mortality rates similar to those diagnosed with ACS. Therapies known to be effective in ACS patients, including early invasive management, should be examined in these non-ACS patients with troponin elevations within adequately powered randomised trials. [ABSTRACT FROM AUTHOR]