Transmitral flow was studied using Doppler echocardiography with the A/E ratio as a parameter of left ventricular diastolic function in 21 patients (ages 2.5-30.0 years) who had undergone early surgical correction of an isolated secundum type atrial septal defect (ASD) compared to a healthy cohort of 21 subjects. Pre- and postoperative M-mode recordings were compared in 12 of the 21 patients to evaluate the effect of operation on interventricular septal motion (IVS) and left ventricular (LV) and right ventricular (RV) end-diastolic diameter ratio (LVDD/RVDD) as parameters of right ventricular volume overload. No significant difference in A/E ratio was found between the patient and control groups. IVS was shown to normalize in 11 of 12 patients postoperatively and to improve from paradox to flattened in 1 of 12. LVDD/RVDD increased from 1.26 +/- 0.31 to 2.10 +/- 0.51 (p = 0.0008), with no significant difference remaining between the control and patient groups postoperatively. These findings support the conclusion that an intrinsic abnormality of the left ventricle is not responsible for its diastolic dysfunction in patients with ASD who develop left ventricular failure, thereby implicating an acquired abnormality. Mechanical sequelae of right ventricular volume overload were shown to normalize in all patients subsequent to operation in the present study, and therefore cannot be excluded as a cause of left ventricular failure in ASD.
The case is described of a boy with some right ventricular dysplasia and episodes of ventricular tachycardia of left bundle branch block pattern who had symptoms from the age of 1 month. Angiography and cardiac biopsy demonstrated major involvement of the left ventricular myocardium. A sister of the patient presented at the age of 2 months with predominantly left ventricular cardiomyopathy; clinical signs and angiography suggested the presence of right ventricular dysplasia as well. She died suddenly at the age of 9 years. Her brother, now aged 14 years, is being treated with antiarrhythmic drugs. The hypothesis of this cardiomyopathy being a variant of "arrhythmogenic right ventricular dysplasia syndrome" is discussed.
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