Cardiac rhythm abnormalities - An underestimated cardiovascular risk in adult patients with Mucopolysaccharidoses.

Patients with Mucopolysaccharidosis (MPS) have an increased risk of cardiovascular complications, conduction tissue abnormalities and arrhythmia; all rare but underestimated. It has been reported that conduction system defects are progressive in this group of patients and may result in sudden cardiac death. The aim of this study is to review our current practice and suggest best practice guidelines regarding the frequency of cardiac rhythm monitoring in this patient group. Seventy-seven adult MPS patients who attended metabolic clinics between 2013 and 2019 were included in this retrospective observational study. Patients were affected with different MPS types: MPS I (n = 33), MPS II (n = 16), MPS IV (n = 19), VI (n = 8) and VII (n = 1). The assessments included: 12­lead electrocardiogram (ECG), 24-h ECG (Holter monitor), loop recorder/pacemaker interrogation assessment. Data from 12­lead ECG (available from 69 patients) showed a variety of abnormalities: T wave inversion in a single lead III (n = 19), left ventricular hypertrophy (n = 14), early repolarization (n = 14), right axis deviation (RAD, n = 11), partial RBBB (n = 9), right bundle branch block (RBBB) (n = 1) and first degree AV block (n = 1). ECG changes of bundle branch block, RAD (left posterior fascicular block) could represent conduction tissue abnormality and equally could be related to the underlying lung tissue abnormality which is present in most of the patients with MPS. T wave abnormality in a single lead is usually insignificant in healthy individuals; however in MPS patients it could be as a result of chest shape. Among the 34 patients for who 24-hour ECG was available, sinus tachycardia was the most common rhythm noted (n = 9), followed by sinus bradycardia (n = 4), atrial fibrillation (AF) (n = 1) and atrio-ventricular nodal re-entry tachycardia (AVNRT) (n = 1). Permanent pacemaker was inserted in two patients. AF was observed in one patient with MPS II. In conclusion, we postulate that regular cardiac monitoring is required to warrant early detection of underlying conduction tissue abnormalities. In addition, 12­lead ECG is the first line investigation that, if abnormal, should be followed up by 24-hour Holter monitoring. These findings warrant further research studies.

Idiopathic left ventricular apical hypoplasia.

A 46-year-old man was found to have an abnormal ECG taken during a routine health and blood pressure check. His only symptom was non-specific central chest discomfort, unrelated to exertion. His ECG showed sinus rhythm, a normal axis and poor R wave progression across the chest leads and lateral T wave flattening. An echocardiogram showed a dilated left ventricle with a thin and hypokinetic septum bulging to the right. The apex was 'not well seen' but also appeared thin and hypokinetic. The right heart and valves were normal. The patient was further investigated for left ventricular hypoplasia.

Aeromedical transfer to reduce delay in primary angioplasty.

BACKGROUND: Aeromedical transfer can reduce transfer times for primary percutaneous coronary intervention (PPCI). Delays in dispatch of the helicopter and landing-reperfusion can reduce the benefits of air travel. The ad hoc nature of these transfers may compound delays. A formal aeromedical transfer service, with rapid dispatch protocols and rapid landing to balloon times could significantly reduce reperfusion times. METHODS: A standard operating procedure (SOP) was developed using a field assessment team (doctor, aircrew paramedic) and a cardiologist-led multidisciplinary team meeting the incoming aircraft. The aeromedical SOP for STEMI care was implemented when anticipated land journey >30 min to the nearest PPCI centre. Reperfusion times for actual air travel and estimated virtual land journeys from the same location were compared. RESULTS: Between April and December 2009, 8 patients were managed according to the aeromedical SOP. Median air distance 49 miles and road, 40 miles. All subsequent data shown in median minutes (range). Call-balloon time 109 (97-116). Call-aeromedical activation 13 (9-26). Aeromedical activation-arrive scene 12 (9-16). Time at scene 29 (24-52). Call-depart scene 57 (45-75). Air journey 25 (18-30) and landing-balloon 21 (8-22). Call-arrive at PPCI centre for air 85 (70-95); estimated virtual road call-arrive at PPCI centre 102 (85-104). CONCLUSIONS: This SOP delivered sub 120 min call-balloon times in all cases undergoing PPCI from difficult locations where anticipated land journeys were >30 min. With longer anticipated land journeys (or more remote locations) the proportional gains with air transfer will be greater. Subject to a formal SOP and very rapid landing-balloon times, aeromedical transfer can significantly reduce the number of patients suffering long reperfusion delays in acute myocardial infarction.