Background Children with hypertrophic cardiomyopathy (HCM) have historically been considered to be high-risk candidates for general anaesthesia (GA), but there is currently a paucity of evidence regarding the safety of anaesthesia and perioperative outcomes in this population.
Methods Clinical features and outcomes of all paediatric patients (<18 years) with HCM undergoing GA between 2000 and 2016 were reviewed.
Results 86 patients (median 12.4 years (IQR 6.5, 14.9)) underwent 164 separate GA procedures. Aetiology included non-syndromic disease (n=44, 56%), malformation syndromes (n=22, 26%), inborn error of metabolism (n=10, 12%) and neuromuscular disease (n=4, 5%). At the time of GA, mean maximal wall thickness (MWT) on echocardiography was 19 mm (SD±8 mm), 23 (14%) patients had severe left ventricular hypertrophy (MWT>30 mm) and 35 (21%) patients had a haemodynamically significant left ventricular outflow tract (LVOT) gradient (>50 mm Hg). The majority (n=143, 87%) had no perioperative complications. 20 (12%) patients had minor perioperative complications: bradycardia (n=4), hypotension (n=15) or transient ST segment changes (n=1). One (0.6% of GA procedures) patient experienced a cardiac arrest during anaesthetic induction with death occurring 3 days later. Clinical parameters (including age, MWT, LVOT gradient, systolic and diastolic dysfunction) were not associated with an increased risk of complications
Conclusions This is the largest published series to date of paediatric patients with HCM undergoing GA, which demonstrates that, in an expert centre, patients can be anaesthetised with a relatively low perianaesthetic mortality (0.6%) and prevalence of minor complications (12%). Future studies are required to systematically identify clinical features that may predict anaesthetic risk.
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Contributors GN and NF conceptualised and designed the study, collected data, carried out the initial analyses, drafted the initial manuscript, and reviewed and revised the manuscript. CW and MCA conceptualised and designed the study, collected data, and reviewed and revised the manuscript. EF designed the data collection instruments, and reviewed and revised the manuscript. EC and AI conceptualised and designed the study, and critically reviewed the manuscript for important intellectual content. JPK conceptualised and designed the study, and reviewed and revised the manuscript.
Funding This work was supported by the British Heart Foundation.
Competing interests None declared.
Patient consent Not required.
Ethics approval Great Ormond Street Hospital/ University College London Institute of Child Health Research and Development Office.
Provenance and peer review Not commissioned; externally peer reviewed.
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