ABSTRACT
Longitudinal right ventricular free wall strain (RVFWS) has been identified as an independent prognostic marker in patients with pulmonary hypertension. Little is known however about the prognostic value of RVFWS in patients with sickle cell (SC) disease, particularly during exercise. We therefore examined the prognostic significance of RVFWS both at rest and with exercise in patients with SC disease and normal resting systolic pulmonary artery pressure (SPAP). Consecutive patients with SC disease referred for bicycle ergometer stress echocardiography (SE) were enrolled ftom July 2019 to January 2021. All patients had measurable tricuspid regurgitation velocity (TRV). Conventional echocardiography parameters, left ventricular global longitudinal strain (LVGLS), RVFWS, and ventriculoarterial coupling indices (TAPSE/SPAP and RVFWS/SPAP) were assessed at rest and peak exercise. Repeat SE was performed at a median follow-up of 2 years. The cohort consisted of 87 patients (mean age was 31 ± 11 years, 66% females). All patients had normal resting TRV < 2.8 m/s, RVFWS and LVGLS at baseline. There were 23 (26%) patients who had peak stress RVFWS < 20%. They had higher resting and peak stress TRV and SPAP, but lower resting and peak stress TAPSE/SPAP, RVFWS/SPAP, and LVGLS as well as lower peak stress cardiac output when compared to patients with peak stress RVFWS ≥ 20% (p < 0.05). Patients with baseline peak stress RVFWS < 20% had a significant decrease in exercise performance at follow-up (7.5 ± 2.7 min at baseline vs. 5.5 ± 2.8 min at follow-up, p < 0.001). In the multivariate analysis, baseline peak stress RVFWS was the only independent predictor of poorer exercise performance at follow-up [odds ratio 8.2 (1.2, 56.0), p = 0.033]. Among patients with SC disease who underwent bicycle ergometer SE, a decreased baseline value of RVFWS at peak stress predicted poorer exercise time at follow-up.
ABSTRACT
Sudden death is the most frequent cause of late mortality in adults with congenital heart disease. This study reviews the experience of an Adult Congenital Heart Center with the use of implantable cardioverter defibrillators (ICDs). The charts of adults with congenital heart disease who had an ICD implantation were reviewed for diagnosis, residual lesions, reoperations, reason for implantation, complications, and recurrence of arrhythmias. Since 1995, 13 patients (mean age 43 years) had an ICD implantation for aborted sudden death (4), and spontaneous (6), or induced (3) ventricular tachycardia. Diagnosis were repaired (6) or palliated (1) tetralogy of Fallot, operated pulmonary stenosis (2), palliated complex pulmonary atresia (1), congenitally corrected transposition (1), operated ventricular (1), and atrial (1) septal defects. Significant residual lesions included severe pulmonary regurgitation (2), systemic ventricular dysfunction (2), and severe pulmonary hypertension (1). Five patients had a QRS > 180 ms, four had a QRS < 180 ms, four had a paced rhythm. Overall, four (31%) patients had no ventricular dysfunction, no residual lesion, and QRS < 180 ms. During a mean follow-up of 29 months, seven patients had recurrent ventricular tachycardia, three with normal ventricular function, no residual lesion, and QRS < 180 ms. ICD implantation is an important adjunct in the management of adults with congenital heart disease. As malignant arrhythmias occur even in patients with no residual lesion, no QRS prolongation and no ventricular dysfunction, the recognition of those who would benefit from an ICD remains a clinical challenge.
