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A case of fetal tuberous sclerosis with multiple intracardiac rhabdomyomas exhibited persistent supraventricular tachycardia. The tachycardia was terminated by the use of cardiac version. The largest tumor almost occluded the right ventricular inlet portion. The obstruction was relieved after surgical removal of the largest tumor. No arrhythmia was noted after 3 months of follow-up.

Objectives: We investigated the influence of cardiopulmonary bypass on cardiomyocyte gap junctions and connexins. Methods: Samples were collected at intervals during operation from the right atrial appendage in 21 patients (mean [± SD] age 55 ± 21 years). Immunodetection of connexins was conducted by Western blotting and confocal microscopy with parallel electron microscopic examination of gap junctions. Results: Downregulation of connexin 43 during the course of operation occurred in more than half of the patients. The mean densitometric value of connexin 43 decreased by 23%, with samples from patients with coronary artery disease showing a greater reduction than seen in those from patients with other diseases (31% ± 22% vs 10% ± 24%, P =.04). Such alterations were confirmed by confocal microscopy, which also demonstrated reduced connexin 45 immunolabeling in most patients. Electron microscopy revealed a reduction in the dimensions of cell membrane-located gap junctions and more frequent intracytoplasmic gap junctional membrane in samples from later time points ( P =.04). Conclusions: Downregulation of connexins accompanied by a reduction in gap junctions is common in the cardiomyocytes of the right atrial appendage during cardiopulmonary bypass. The association of a marked reduction in connexin 43 with coronary artery disease may imply inadequate intraoperative cardiac protection in patients with this disease. J Thorac Cardiovasc Surg 2002;124:1106-12

A rare case of combined aortic and mitral regurgitation resulting from downward displacement of the aortic anulus is reported. The patient was treated successfully with an aortic valve replacement with a Bjork-Shiley valve prosthesis, which was anchored at the normal site of the aortic anulus. No procedures were performed on the mitral valve, but mitral regurgitation was not evident postoperatively.

The incidence of aortic valve prolapse and aortic regurgitation (AR) among Chinese with ventricular septal defect (VSD) has not been studied, and controversies still exist regarding optimal surgical treatment and timing of operation for this condition. A prospective study of 332 consecutive patients with VSD showed that aortic valve prolapse and AR occurred in 43 (11.9%) patients. Valve lesions occurred more commonly among patients with subpulmonic VSD (28.0%) than with subaortic VSD (8.8%) (P less than 0.005). A retrospective study of another 306 patients revealed that 37 (12.1%) had valve complications. Of the 80 patients with valve lesions, 60 were males and 20 were females. The youngest ages of prolapse and AR in subpulmonic VSD were 7 months and 3 years 8 months, respectively; those in subaortic VSD were 2 years and 3 years 6 months, respectively. Valve prolapse occurred mostly before the age of 6-10 years, leading progressively to AR. The coronary cusps prolapsed in subpulmonic VSD were limited to the right cusp; and those in subaortic VSD were the right cusp, noncoronary cusp, or both. The magnitude of left-to-right shunts was small and the pulmonary artery pressure was normal in the majority of patients. Seventy-two patients underwent open-heart closure of the VSD, with additional valvuloplasty in 15 and valve replacement in three patients. It is concluded that Chinese with VSD are prone to develop aortic valve complications. Surgical closure of the subpulmonic VSD may restore the prolapsed valve to normal. Closure of the subaortic VSD has little effect. Valvuloplasty in subaortic VSD may palliate AR, but in all probability cannot restore valve competency.