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Prof. Hubert Seggewiss,
This session was co-chaired by Prof. Hubert SEGGEWISS (Schweinfurt, DE) and Prof Ales LINHART (Prague, CZ). The aim of the session was to give an update of the management of difficult situations in patients with hypertrophic cardiomyopathy.
Constantinos O'MAHONY (London, GB) gave an excellent overview of Left ventricular outflow tract obstruction: indications and limitations of current therapies. Treating HCM requires assessment of the obstruction with respect to condition (resting or provocable obstruction), level (subaortic or mid-cavitary, involvement of mitral valve and papillary muscle), and amount of LV hypertrophy. Symptomatic patients should be treated either by medication and in case of treatment failure, invasive treatments (alcohol septal ablation or surgical myectomy). Symptomatic relief is comparable. Furthermore, the author pointed out that up to now, no data are available which show superiority of any one method regarding mortality during long-term follow-up.
Pier LAMBIASE (London, GB) reported on the Management of atrial fibrillation. Atrial fibrillation worsens the prognosis of HCM. Treatment stratgies should consider stroke prophylaxis, optimizing rate control, rhythm control with or without radiofrequency ablation or substrate modification. Before dealing with AF itself, existing obstruction should be treated first. Early consideration of anticoagulation is warranted in patients with left atrial dilatation (LA volume > 41ml/m2) and documentation of short runs of atrial tachycardia. Left atrial appendage closure devices should be more intensely discussed in the near future.
Juan Ramon GIMENO BLANES (Murcia, ES) presented hints on Management of severely symptomatic but non-obstructive disease. Such patients are characterised either by left ventricular systolic impairment, or severe diastolic dysfunction, or right ventricular failure in conjuction with pulmonary hypertension or tricuspid regurgitation. Apart from the exclusion of secondary causes that lead to similar phenotypes such as storage diseases or HCM phenocopies, such patients should be treated according to the guidelines for patients with heart failure. The future will show whether metabolic substrate modification, with various agents that are now under scrutiny, will obtain substantial therapeutic merit.
Franco CECCHI (Florence, IT) gave a well-balanced talk on Prevention of sudden death: dilemmas in risk statification. The risk stratification scheme that is presently used is not perfect, as the assessed risk factors are characterised by low positive predictive value, although the aggregation of multiple risk factors in one patient is associated with increased risk of sudden death. Available data about the epidemiology of sudden death in HCM are somewhat outdated and therefore, the risk of sudden death should be reassessed in large patient cohorts in conjuction with mutation analysis, disease staging with the aid of biomarkers, patient management strategies and other disease characteristics that up to now have been overlooked, such as specific ECG patterns. The implantation of ICDs in HCM patients is lifesaving but has to be carefully considered as it is associated with a high frequency of inappropriate shocks and significant peri-implantation complications that can sometimes be lethal.
Hypertrophic cardiomyopathy: treatment frontiers
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