Response of the stroke volume and blood pressure of young patients with nonobstructive hypertrophic cardiomyopathy to exercise.

Stroke volume (SV), cardiac output (CO) and systolic blood pressure (SBP) were measured during maximal symptom-limited bicycle exercise testing in 13 young patients (age, 11-26 years) with nonobstructive hypertrophic cardiomyopathy (HCM). SV was measured by impedance plethysmocardiography; %SVend, %COend, and %SBPend represent the ratio of the value at termination of the exercise to the respective value at rest. In all patients of HCM-I (the Cardiac Event Group, 3 patients) and 3 of HCM-II (the Non-Cardiac Event Group, 10 patients), the %SVend was less than 100%. The %SVend of HCM-I was significantly lower than the respective values of the HCM-II and Control groups. The %COend values of the HCM-I and HCM-II groups were each significantly lower than that of the Control. The %SBPend values of the HCM-I and HCM-II groups were each significantly lower than that of the Control. Among the HCM patients, the %SVend value was positively correlated with the %SBPend value. The patients who had more severe HCM had poorer exercise-induced increases in SV and SBP. These results suggest that sudden cardiac death in young HCM patients is associated with inhibition of the increase in SV upon exercise.

Comparison of three-dimensional contrast-enhanced magnetic resonance angiography and axial radiographic angiography for diagnosing congenital stenoses in small pulmonary arteries.

Accuracy of 3-dimensional contrast-enhanced magnetic resonance angiography (MRA) in diagnosing morphology of the branch pulmonary artery (PA) was evaluated in 73 patients (aged 7.2 +/- 6.4 years [mean +/- SD]) with various congenital heart diseases. The presence or absence of localized stenosis of branch PAs, PA diameter, and Nakata's PA index were determined on MRA and axial radiographic angiography, and the results were compared. Sensitivity, specificity, and overall accuracy in detecting branch PA stenoses were 92.7%, 96.2%, and 95.2%, respectively. Correlations between axial radiographic angiography and MRA were excellent in measuring PA diameter (r = 0.956, SEE = 1.49 mm, n = 139) as well as PA index (r = 0.839, SEE = 48.9, n = 37); both p < 0.0001. Bland-Altman plots showed a mean difference +/- SD for PA diameter of 0.17 +/- 1.51 mm and for PA index of 8.5 +/- 50.1. When the main right and left PAs were taken as the first generation, the most distal branches visible on MRA were the 4.7 +/- 0.7 generation with breath-holding (n = 23) and the 3.7 +/- 0.5 without breath-holding (n = 50), respectively (p < 0.0001). Both intra- and interobserver variabilities of MRA measurements were few (9.5 +/- 11.6% and 13.5 +/- 15.0%, respectively, n = 139). In conclusion, 3-dimensional contrast-enhanced MRA enables us to document branch PA morphology clearly in infants and adult patients with congenital cardiovascular defects.

Idiopathic sustained left ventricular tachycardia in pediatric patients.

BACKGROUND: Idiopathic sustained ventricular tachycardia originating from the left ventricle (ILVT) has been an indication for catheter ablation. The present study evaluated the clinical features, long-term prognosis and indications for treatment in pediatric patients with ILVT. METHODS: The subjects of the present study were eight patients (four males and four females) with a mean age at onset of 11.0 years (range 3-15 years). The mean follow-up period was 7.7 years (range 2.1-11.3 years). RESULTS: In electrophysiologic studies, intravenously administered verapamil was effective for the termination of tachycardia in all six patients who received this treatment and for the prevention of tachycardia in four of five patients. Oral administration of verapamil was effective in five of seven patients. Propranolol or flecainide was added to the treatment protocol for two patients who did not respond to verapamil alone. Tachycardia disappeared without drugs in four patients during the follow-up period and became non-sustained in another patient. Two of three patients with persistent tachycardia underwent catheter ablation. Pharmacologic treatment was very effective for ILVT among these patients. CONCLUSIONS: Pharmacologic therapy, such as with verapamil, is still the treatment of choice for ILVT because of a good long-term prognosis and potential risks and complications by manipulation of catheter ablation.