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Rupture of an ascending aortic aneurysm is the most common cause of death in patients with Marfan syndrome, while type-A aortic dissection is the second most common fatal lesion. The aortic root dimension and the rate at which it increases are the best indications for surgical treatment. We regard aortic enlargement to 5.5 cm as the upper limit of safety and we are inclined to advocate surgery at an earlier stage in high-risk families. We report our results of the surgical management of aortic aneurysm in the two siblings; a third sibling is currently being followed medically.

The aim of this study was to evaluate the incidence of thromboembolic events in patients with giant left atrium (> 6.5 cm) after mitral valve replacement. From January 2000 to September 2002, a total of 126 patients who had undergone mitral valve replacement were divided into two groups according to the presence or absence of giant left atrium. Group A comprised 34 patients with left atrium over 6.5 cm without compression symptoms and Group B comprised 92 patients. The preoperative variables did not distinguish the patients in each group, except for atrial fibrillation; Group A 85.2% and Group B 61.9% (p < 0.01). After mitral valve replacement, left atrium mean diameter was significantly decreased in Group A from 8.1 ± 1.3 mm to 6.2 ± 1.6 mm ( p < 0.01). There were no significant differences in thrombosis, hemorrhage and thromboembolism rates in both groups. Postoperative clinical and hemodynamic parameters demonstrated a positive clinical response to mitral valve replacement in patients with giant left atrium. During follow-up no direct relationship between thromboembolism and giant left atrium was evident.

Our objective was to compare the results of revascularization by sequential radial artery (RA) grafting with a left anterior descending left internal mammary artery (LIMA)-RA t-composite grafting technique. Patients were grouped as those with proximal anastomoses performed on the ascending aorta (Group A; n = 38), and those with proximal anastomoses performed on the LIMA as t-grafts (Group T; n = 13). Neither of the two groups revealed any mortality. The average number of grafts was lower in Group T (2.23 ± 0.43 in group T and 2.85 ± 0.69 in group A, p < 0.05). The results of the control coronary artery angiographies were superior in Group A. The patency rate of the RA grafts was 96.8% in Group A. Of the 20 distal anastomoses performed with RA grafts in 8 patients from Group T, nine (45%) were found to be patent. The patency rates of RA grafts with sequential distal anastomoses were found to be better when the proximal anastomosis was performed on the ascending aorta rather than on the LIMA. In conclusion, sequential distal anastomosis of RA grafts seem to be safe and effective when proximal anastomoses are performed on the ascending aorta.

The aim of this study was to evaluate the usefulness of repairing significant tricuspid regurgitation (or = grade 2) without severe pulmonary hypertension (or = 50 mm Hg). Between 1993 and June 2001, 88 consecutive patients were operated on for rheumatic mitral valve disease associated with significant tricuspid regurgitation and without severe pulmonary hypertension. The severity of the tricuspid valve disease was assessed by echocardiography. Sixty-three patients had severe (or = grade 3) tricuspid regurgitation (Group I), and 25 patients had moderate (grade 2) tricuspid regurgitation (Group II). There was no hospital mortality. six patients died during follow-up. The overall actuarial survival rate for 8 years was 92.1% +/- 3.1%. Cox proportional hazard regression analysis showed that age ( p = 0.006) and pulmonary complication ( p = 0.01) were associated with increased late mortality. Freedom from death was similar in both groups at 8 years (93.1% +/- 3.3% versus 88% +/- 8%, p = 0.7). Severe postoperative tricuspid regurgitation (or = grade 3), caused by the failure of tricuspid repair or leaving the valve untouched, impaired long-term survival after surgery, and actuarial survival was 96.1% +/- 2.7% and 83% +/- 7.8% at 7 years ( p = 0.048), respectively. Severe tricuspid regurgitation, functional or organic, should be corrected at the time of mitral valve surgery, whereas untouched functional moderate tricuspid regurgitation improves after mitral valve surgery.

Various surgical procedures have been employed to treat a greatly enlarged left atrium. We review the use of partial cardiac autotransplantation to reduce left atrial volume in 7 patients with rheumatic mitral valve disease and left atrial and ventricular volume in 2 patients with idiopathic dilated cardiomyopathy. There were 5 males and 4 females aged 25 to 62 years. The patients with rheumatic etiology had atrial fibrillation, while those with dilated cardiomyopathy had sinus rhythm. The mitral valve was replaced in 6 patients and reconstructed in 3. Mean aortic cross clamp time in the operations involving isolated left atrial resection was 119 ± 44 min. Mean left atrial volume fell from 331 mL to 92 mL, while mean left atrial diameter decreased from 8.6 cm to 4.7 cm. Sinus rhythm was restored in 5 of the 7 patients who had preoperative atrial fibrillation. There was no operative mortality. The patients with dilated cardiomyopathy died in the postoperative period, one on the 14th day from low cardiac output and the other on the 113th day from multiorgan failure. Partial cardiac autotransplantation can be effective in reducing heart chamber size in selected patients, especially those with giant left atrium.

The aim of this study was to evaluate the clinical outcome of surgical treatment in patients with Marfan syndrome. Between 1985 and November 2001, 33 patients with Marfan syndrome were operated for chronic aneurysm of the aortic root with involvement of the ascending aorta in 20 patients and type A dissection in 13 patients. The patients comprised 24 males and 9 females with a mean age of 31.9 ± 9.7 years (range, 18 to 54 years). The mean diameter of the ascending aorta was 6.6 ± 1.6 cm and that of the aortic root was 5.4 ± 1.2 cm. Hemodynamic instability was observed in 11 patients. The aortic arch was replaced in 7 patients. There was no hospital mortality. Late mortality was 6%, involving 2 patients who had aortic valve replacement. Actuarial freedom from death was 92.3% ± 7.4% at 12 years and from late aortic complications was 86.4% ± 9.4% at 13 years. Aortic aneurysm was a significant univariate adverse factor for late aortic complications. Aortic surgery can be performed in Marfan patients with low morbidity and mortality. Aggressive surgical intervention does not impair surgical outcome while it decreases reoperation risk.

An asymptomatic 24-year-old man underwent surgical correction of an aorto-left ventricular tunnel. A 1-cm opening was found 1 cm above the commissure of the left and right coronary cusps, which communicated with the left ventricle. It was successfully closed by direct suture.

Partial left atrial resection was performed in 8 males and 12 females, aged 19 to 63 years, with giant left atrium and mitral valve disease. Preoperatively, 18 patients had atrial fibrillation, and 2 had normal sinus rhythm. Echocardiography revealed left atrial thrombosis in 3 patients and spontaneous echo contrast in 5. The lateral wall of the left atrium, the region between the pulmonary veins, the roof of the atrium, and the tissue parallel to the mitral annulus were resected. Resection was performed using the cardiac autotransplantation technique in 6 patients. The mitral valve was replaced in 9 patients and reconstructed in 11. Mean aortic crossclamp time was 101 ± 35 minutes and total perfusion time was 135 ± 26 minutes. Mean follow-up was 20.4 ± 1.1 months. There was no operative mortality. One patient (5%) died suddenly in the late postoperative period. Left atrial volume was reduced from 265.3 ± 125 mL to 83 ± 43 mL (p < 0.01). Left atrial diameter decreased from 8.02 ± 1.31 cm to 4.4 ± 1.23 cm (p < 0.01). Sinus rhythm was detected in 13 patients (65%) postoperatively. No left atrial thrombosis or spontaneous echo contrast were found during follow-up. Statistically significant reductions in cardiac size and improvements in functional capacity were observed.

Between March 1996 and September 1997, videothoracoscopy was performed in 50 of 140 patients who underwent minimally invasive coronary artery bypass grafting. Mean age was 45.3 ± 6.8 years. The left internal mammary artery was harvested by thoracoscopy alone in 21 patients and by both thoracoscopy and direct vision in 29. Coronary artery bypass was then performed through a left anterior minithoracotomy. In 48 patients, the internal mammary artery was grafted directly to the left anterior descending artery; a small saphenous vein graft was interposed in the other 2 patients. The diagonal branch was bypassed with saphenous vein in 2 patients, the first obtuse marginal in 1, the right posterior descending branch in 1, and the right ventricular branch of the right coronary artery in 1. Concomitant carotid endarterectomy was performed in 1 patient. There was no mortality. Two patients had perioperative myocardial infarction. It was concluded that videothoracoscopy can help to achieve complete mobilization of the left internal mammary artery for minimally invasive coronary artery bypass grafting. These techniques can be regarded as safe and effective, giving excellent results and a shortened hospital stay with the advantage of avoiding some morbidity due to costal cartilage resection.

Combined coronary bypass and lung surgery was performed in 3 patients. Through a median sternotomy or a left thoracotomy, bypass grafting was performed on beating heart or under cardiopulmonary bypass, followed by the lung operation. The lung lesion was diagnosed as carcinoma in 2 patients and hydatid cyst in 1 patient. With few exceptions, beating heart coronary bypass through a median sternotomy can be performed in a combined operation.

The rare cardiac anomaly of atresia of the coronary sinus ostium with a large communication between the coronary sinus and the left atrium was discovered during a mitral valve replacement operation in a 44-year-old woman.