Subannular left ventricular aneurysms are rare in white persons. Six white patients have been reported in the literature who underwent surgical repair because of subaortic left ventricular aneurysm. The authors report on a further case, a 41 year-old woman who was operated on at their clinic. The rare occurrence of this disease inspired them to review the literature and to describe their case.
Action potential configuration and electrical restitution were studied in diseased human ventricular muscle by comparing the characteristics of hypertrophic (HYP) and dilated (DIL) human ventricular preparations. Conventional microelectrode techniques were used to evaluate action potentials evoked at increasingly longer diastolic intervals. The steady-state action potential duration (APD90) was significantly longer in DIL than in HYP preparations (393 +/- 5 ms, n = 4 and 296 +/- 11 ms, n = 4, respectively; P < 0.001, mean +/- SEM). In the dilated preparations studied at long diastolic intervals, the initial period of rapid repolarization (phase 1) was absent, and the rate of final repolarization (phase 3) was reduced. Electrical restitution relations in these preparations were fitted as the sum of two exponentials. The time constant of the fast component was significantly longer in DIL than in HYP preparations (242 +/- 9 ms and 121 +/- 4 ms, respectively; P < 0.001). No difference was observed in the time constants for the slow component of restitution in the two groups. Electrical restitution was also studied in single human ventricular myocytes by using patch clamp techniques. The initial 600 ms period of restitution was fitted in these cells to a monoexponential function. The time constant for this period of the restitution relation was significantly longer, while the estimated amplitude of this early rising phase was significantly lower in human cells obtained from DIL hearts than the respective parameters obtained in the healthy canine and guinea pig cells also examined. The observed changes in the restitution kinetics of the dilated human heart are, likely, the consequence of alterations in the ionic currents that underlie the cardiac action potential.
Combined valvular and coronary bypass surgery was performed in 1984-1988 in 62 patients. Their age range at operation was 38-75 (mean 59) years and more than 90% were in NYHA class III or IV. Valve replacement was performed in 55 cases--mitral in 13, aortic in 33, tricuspid in one and combined valve procedures in eight cases--and valvular correction in seven cases. The patients also received a total of 92 coronary artery grafts (1-4/patient, mostly as single grafts). The hospital mortality was 3% and there were two late deaths. Follow-up was 100% complete 2-52 (mean 22) months after surgery, and more than 90% of the survivors were improved.
This report is concerned with a patient successfully operated for Holt-Oram syndrome. The disorder is inherited dominantly with complete penetrance and variable expressivity. It is pointed out that in cases of congenital heart disease familial cumulation must be borne in mind which can easily be verified by pedigree investigation. After demonstrating autosomal dominant inheritance, suitable inferences must be drawn for genetic counseling. Attention is drawn to the heart-surgical significance of the rhythm disorders so often associated with the syndrome.
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