Hypertrophic cardiomyopathy is a common, autosomal dominant cardiac disease with heterogeneity of expression. Although predominantly an obstructive lesion, it is also characterized by dysrhythmias, diastolic dysfunction, and subendocardial ischemia. Knowledge of the pathophysiology of the disease is imperative in order to provide safe anesthetic care. A comprehensive preoperative evaluation is important in identifying high-risk patients with more malignant forms of the disease. This chapter reviews the abnormalities associated with hypertrophic cardiomyopathy. In addition, strategies to maintain normal sinus rhythm and blood pressure and to prevent catecholamine surges are discussed with specific reference to anesthetic agents and their effects in patients with hypertrophic cardiomyopathy.

The risk of perioperative morbidity and mortality is increased in patients with pulmonary hypertension regardless of the procedure. This is particularly true for patients with multifactorial pulmonary hypertension. We present such a patient here with a history of Down syndrome and repaired congenital heart disease who is scheduled to undergo an elective tympanoplasty. Anesthetic issues relevant to the care of a patient with Down syndrome, atrioventricular septal defect, and pulmonary hypertension are discussed, with a focus on assessment and intraoperative planning.