Initial clinical manifestations and mid- and long-term results after surgical repair of double-chambered right ventricle in children and adults.

OBJECTIVE: By means of retrospective analysis of our institutional experience, we reviewed the clinical manifestation and outcomes of patients subsequent to surgical repair of double-chambered right ventricle. METHODS: Between 1988 and 2005, we performed surgical repair in 35 of 37 patients diagnosed with double-chambered right ventricle. The patients ranged in age from 4 to 69 years, with a mean of 21.3 years. Most presented in infancy, with initial manifestation of a short systolic murmur in 34 (92%) of all cases. Pressure gradients were measured invasively across the right ventricular outflow tract of between 30 and 140 mmHg, with a median of 60 mmHg. An associated ventricular septal defect was present in 26 patients (70%). Of the group, 4 patients were aged over 40 years, and 2 had previously undergone operative closure of a ventricular septal defect. RESULTS: The operative interval ranged from 2 months to 41 years, with a median of 9 years. In all, we resected muscular bundles and enlarged the right ventricular outflow tract. There was no hospital or late death. Median follow-up subsequent to surgery was 7 years, with a range from 0.4 to 11 years. No patient required further surgery to relieve any obstruction of the right ventricular outflow tract, nor long term medical therapy or pacing because of cardiac arrhythmia. CONCLUSIONS: Surgical repair of a double-chambered right ventricle yields excellent haemodynamic and functional results over the mid to long term.

Assessment of regional atrial function in patients with hypertrophic cardiomyopathies using tissue Doppler imaging.

BACKGROUND: This study applied tissue Doppler imaging and color tissue Doppler imaging to study atrial function changes in patients with hypertrophic cardiomyopathy (HCM). The profile of the segmental atrial velocities and the strain rate were determined and compared with those of normal matched control subjects. METHODS: This study investigated 20 patients with HCM and 20 age-matched healthy control subjects. In a four-chamber apical view, tissue Doppler imaging was used to measure the lateral left and right atrial (LA and RA) and interatrial septal (IAS) wall systolic, early, and late diastolic velocities. Similarly, the atrial strain rate during ventricular systole (SR(S)) and the early (SR(E)) and late (SR(A)) diastolic phases in patients and control subjects were measured. The interventricular septal tissue Doppler-derived isovolumic relaxation time was calculated. RESULTS: Only the IAS annular and middle segments showed a significant reduction in the early diastolic velocity (mean, 4.01 +/- 2.2 vs 8.7 +/- 1.1, p = 0.001; 3.23 +/- 2 vs 6.01 +/- 1.9, p = 0.001, respectively) for the patients with HCM in comparison with the control subjects. Generally, the atrial strain rate was clearly reduced. The systolic strain rate (SR(S)) was significantly reduced in the LA wall in the annular (p = 0.007) and middle (p = 0.001) segments and in the IAS middle segment (p = 0.007). Similarly, there was a reduction of the early diastolic strain rate (SR(E)) in the LA annular (p = 0.001) and middle (p = 0.01) segments and in the IAS annular (p = 0.05) and middle (p = 0.001) segments, as well as in the RA annular segment (p = 0.02). The RA middle segments showed insignificant changes. CONCLUSION: Atrial function may be affected by HCM due to impairment of myocardial diastolic function. Strain rate imaging is reproducible, yields readily obtained parameters that provide unique data about global and longitudinal segmental atrial contraction, and can quantify the atrial dysfunction in patients with HCM.