Management of injured patients who were Jehovah's Witnesses, where blood transfusion may not be an option: a retrospective review.

INTRODUCTION: Management of major haemorrhage as a result of trauma is particularly challenging when blood is not an option (BNAO). Evidence on therapeutic strategies in this situation is limited. The aim of this study was to evaluate the management and outcomes of patients who identified themselves as Jehovah's Witnesses (who usually refuse blood products) with traumatic haemorrhage at an Australian major trauma centre. METHODS: A retrospective review of patients from The Alfred Trauma Registry was conducted, including patients who were Jehovah's Witnesses presenting between January 2010 and January 2017. We examined demographics, injury characteristics, clinical progress, therapeutic interventions and outcomes at hospital discharge. RESULTS: There were 34 patients meeting inclusion criteria, with 50% suffering major trauma. Anaemia was a clinical problem for 13 (38·2%) patients, with haemoglobin levels reaching a nadir of 69·7 g/l (95% CI: 56·7-82·7) on average 5·1 days (95% CI: 2·5-7·7) post admission. Various strategies were employed to reduce blood loss including six (46·2%) patients receiving tranexamic acid, nine (29·2%) patients receiving oral or intravenous iron and five (38·5%) receiving erythropoietin. Three patients received packed red cells, and two patients received synthetic haemoglobin-based oxygen carriers. CONCLUSIONS: Numerous therapeutic strategies were employed inconsistently in this unique population of patients. Augmenting circulatory volume with an oxygen carrier acceptable to JW patients presents a novel approach to be considered in adjunct to other strategies. An international resource centre would assist clinicians faced with anaemia and BNAO.

Clinical benefits of a specialised clinic for hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is the most common monogenic cardiac condition, with a range of investigation and management options available. Recent research has recommended management within multidisciplinary-dedicated HCM centres. AIM: To assess the clinical impact of attendance at a specialised clinic on the management of HCM. METHODS: A retrospective study of patients attending the HCM Clinic @ The Alfred during the first 2 years of operation was performed. Outcome measures comprised improvements in management of HCM as a result of attending the clinic including risk stratification and further investigations, medication changes, referral for implantable cardioverter-defibrillators (ICD) and septal reduction therapy, and screening of first-degree relatives (FDR). RESULTS: Following specialised clinic attendance, there was an increase in patients investigated with 24-h Holter monitoring by 39%, stress echocardiography by 21% and cardiac magnetic resonance imaging by 70%. Medications were optimised in 47% of patients. Six per cent of patients underwent ICD implantation and 13% underwent septal myectomy. Seventy new FDR were screened identifying 10 new cases of HCM. CONCLUSIONS: Attendance at our specialised HCM clinic leads to facilitation of cardiac investigations, optimisation of medical therapy, streamlining of referrals for ICD implantation and septal reduction therapy, and improved family screening. Referral to a specialised clinic offering comprehensive management should be considered for all patients with HCM.

Family screening in hypertrophic cardiomyopathy is underperformed, but can be improved by a specialised clinic.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) causes significant morbidity and sudden death. First-degree relatives (FDR) of affected patients are at risk due to autosomal dominant inheritance. Guidelines recommend clinical screening, including echocardiography, for all FDR. AIM: We sought to determine adherence to these guidelines, and whether a specialised HCM clinic improves screening rates. METHOD: This 12-month prospective follow-up study obtained family pedigrees from all patients referred to the HCM Clinic @ The Alfred. The number of living FDR was determined, and whether they had previously been assessed by echocardiography. One year after a coordinated clinic-based family screening approach was instituted, the number of additionally screened FDR was recorded. RESULTS: Three hundred and eight living FDR of 61 HCM patients were identified. Of these, echocardiography had previously been performed in only 80 (26%), yielding 13 (16%) additional cases of HCM. Twelve months after attendance at our clinic, 51 additional FDR were screened (64% improvement) and 8 new cases of HCM were identified. CONCLUSIONS: Recommended family screening for HCM is underperformed, resulting in missed opportunities to detect subclinical HCM. A coordinated approach through a specialised HCM clinic improves screening rates, thus referral to such a service should be considered for all patients with HCM and their families.