Propranolol therapy in 55 infants with infantile hemangioma: dosage, duration, adverse effects, and outcome.

Propranolol has recently been reported to be a highly effective treatment for infantile hemangioma (IH) and is emerging as a first-line therapy. This study reports the observations after completed propranolol therapy in 55 patients. Propranolol was administered in a dosage of 2 mg/kg per day with initial monitoring of vital signs. Therapy duration was planned for 4 to 6 months; if there was significant relapse, the period of treatment was extended. The mean age of 55 patients at the beginning of the treatment was 6 months (52.7% <4 mos, 30.9% 4-9 mos, 16.3% >9 mos). Thirteen patients (21.7%) showed a reaction possibly due to the medication, but we did not observe any life-threatening adverse effects. The therapy was interrupted due to temporary aggravation of preexisting bronchial asthma in one child. The initially administered dosage was adjusted to the increase of weight in 21 patients (38.2%), but most did not require a dosage adjustment despite somatic growth. Mean duration of treatment was 6 months; younger patients needed longer treatment periods. Response to treatment was favorable; eight (14.5%) showed total regression and 46 (83.4%) partial regression, and one (1.8%) had no response. Propranolol is an efficacious therapy for severe IH. Risks and complications appear moderate. If indicated, therapy should be initiated early to minimize the extent of residual changes. Young patients show quick and extended benefit. Prospective controlled trails are necessary to observe the effects on a long-term basis.

Outcome after mechanical aortic valve replacement in children and young adults.

BACKGROUND: We asked whether aortic valve replacement using a mechanical prosthesis would allow normalization of left ventricular function and structure in children and young adults. METHODS: We performed a clinical follow-up examination in 30 patients with aortic valve replacement at 25 years of age or younger, including conventional and tissue Doppler echocardiography and magnetic resonance imaging. RESULTS: Aortic valve replacement was performed at the median age of 14.3 years (range, 7.6 to 24.3 years) using a mechanical prosthesis (St. Jude Medical; median diameter, 23 mm; range, 17 to 27 mm). Indications were severe aortic stenosis in 6 of 30 patients, aortic regurgitation in 20 of 30 patients, or a combination of aortic stenosis and regurgitation (4 of 30 patients). Aortic valve replacement was a reoperation in 12 of 30 patients who primarily underwent aortic valvotomy at a median of 7.1 years (range, 1.0 to 11.3 years). In-hospital mortality was 0%. Follow-up was a median of 6 years (range, 1.2 to 14.5 years). Twenty-nine of 30 patients were in New York Heart Association functional class I without thromboembolic complications, cerebrovascular accidents, or major bleeding on oral anticoagulation. Left ventricular dilatation before aortic valve replacement was present in 20 of 30 patients but normalized in all but 4 patients on follow-up. Most patients showed a normal end-diastolic volume on magnetic resonance imaging, and 23 of 26 patients showed a normal left ventricular ejection fraction (median, 0.53; range, 0.33 to 0.75). Peak systolic strain of the left ventricular myocardium was a median of -13.3% (range, -0.5% to -31%), and was normal in 28 of 30 patients. CONCLUSIONS: Aortic valve replacement in children and young adults offers a good treatment option and may lead to normalization of left ventricular size and function in most patients.