Vascular mechanisms of sudden death in hypertrophic cardiomyopathy, including blood pressure responses to exercise.

Approximately a third of patients with hypertrophic cardiomyopathy fail to increase blood pressure appropriately during exercise, a consequence of an inappropriate vasodilator response in nonexercising beds, leading to an exaggerated fall in systemic vascular resistance. The precise mechanism responsible for this abnormal vascular control in hypertrophic cardiomyopathy is still unclear, but is thought to be secondary to enhanced cardiac baroreceptor activity. However, alternate or synergistic mechanisms, including enhanced release of brain natriuretic peptide, may be involved. Normal exercise blood pressure responses have been shown to have a high (97%) negative predictive accuracy for sudden death during an average follow-up of approximately 3 years, providing considerable reassurance. Patients with abnormal blood pressure responses on exercise were at markedly increased risk of sudden cardiac death, although the positive predictive accuracy during this brief follow-up period was low (15%). It is likely that vascular instability may act as a trigger for sudden cardiac death in patients with an underlying electrophysiologic substrate. Recent evidence suggests that this vascular instability may also result in hypotension during ordinary daily activity, or even at rest, and may be an important cause of syncope in hypertrophic cardiomyopathy. Further studies are required to identify mechanisms of attenuating or reversing this vascular instability. Such measures might have the potential to improve symptoms of recurrent syncope and perhaps reduce the risk of sudden cardiac death.