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Ann Thorac Surg 1999;67:1843-1845
© 1999 The Society of Thoracic Surgeons
a Section of Thoracic and Cardiovascular Surgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA
Address reprint requests to Dr Elkins, Thoracic Surgery, University of Oklahoma Health Sciences Center, PO Box 26901, Oklahoma City, OK 73190
Presented at the Aortic Surgery Symposium VI, April 30May 1, 1998, New York, NY.
| Abstract |
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Methods. We reviewed the medical records of 18 (5.9%) patients with aortic valve disease and an ascending aortic aneurysm and 26 (8.5%) patients with dilation of the ascending aorta, subgroups of 307 patients who had a Ross operation between August 1986 and February 1998. We examined operative and midterm results, including recent echocardiographic assessment of autograft valve function and ability of the autograft root and ascending aortic repair or replacement to maintain normal structural integrity.
Results. There was one operative death (2%) related to a perioperative stroke. Forty-two of 43 survivors have normal autograft valve function, with trace to mild autograft valve insufficiency, and one patient has moderate insufficiency at the most recent echocardiographic evaluation. None of the patients has dilatation of the autograft root or of the replaced or reduced ascending aorta.
Conclusions. Early results with extension of the Ross operation to include replacement of an ascending aortic aneurysm or vertical aortoplasty for reduction of a dilated ascending aorta are excellent, with autograft valve function equal to that seen in similar patients without ascending aortic disease.
| Introduction |
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| Patients and methods |
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Contact for clinical evaluation was made with all patients within 6 months of the closing date of this study, and echocardiographic evaluation of autograft valve function and ascending aorta size was obtained for all patients within 1 year of closure.
Age ranged from 7 to 51 years (median, 31 years) in the aneurysm group, and 3 to 60 years (median, 36 years) in the dilatation group. Seven of the 18 patients with aortic aneurysms (39%) were female, and 5 of the patients with aortic dilatation (19%) were female. Aortic valve insufficiency was the predominant abnormality in 10 patients, 7 had aortic stenosis, 26 had aortic stenosis and aortic insufficiency, and 1 patient had an ascending aortic aneurysm after aortic valve replacement with a prosthetic valve. Aortic valve morphology was bicuspid in 34, tricuspid in 3, and unicuspid in 7 patients. Fourteen patients had required previous aortic valve operations, and 4 of them had previous aortic valve replacement. Coarctation had been repaired in 4 patients, and 1 patient had had a patent ductus closed as a child.
Before acceptance of these patients for a Ross operation, they were screened carefully by detailed medical and family history for evidence suggestive of a connective tissue disorder that might affect their pulmonary valve. No genetic testing was done. One patient with an ascending aortic aneurysm was known to have Turners syndrome; however, we have not considered this a contraindication for a Ross operation.
Ascending aortic aneurysm size ranged from 3 cm (in a 7-year-old child) to 9.5 cm, with a median of 6 cm in the patients who had an interposition Dacron graft replacement of their ascending aorta. In the patients treated with a vertical aortoplasty, the ascending aortic diameter was 3.3 to 5.5 cm, with a median of 4 cm. Twenty-four patients had significant dilatation of the aortic valve annulus and had an aortic valve annuloplasty and fixation of the aortic annulus with an external Dacron cuff, using a technique previously described [2]. Thirteen additional patients had the aortic valve annulus supported with a pericardial strip (n = 8) or a Dacron cuff (n = 5). Forty-two patients had the Ross operation as a root replacement, and 2 had an inclusion cylinder [3].
Follow-up is complete for all patients, and ranges from 0.3 to 2.2 years in the aortic aneurysm group and 0.3 to 2.5 years in the aortic dilatation group. Total follow-up time is 43 patient-years.
| Results |
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One patient required reoperation for autograft insufficiency. This patient, who had aortic stenosis and a coarctation of the aorta, previously had a balloon valvuloplasty of the coarctation and of the aortic valve at age 7 years. At age 20, he had a Ross operation with replacement of his 6-cm aortic aneurysm with a 24-mm Dacron graft. His aortic annulus was 23 mm, which is normal for his body surface area, and the proximal suture line was not supported with an external cuff. Two months postoperatively, annular dilatation developed with moderate autograft insufficiency and recurrent symptoms. At reoperation, the aortic annulus was reduced from 29 mm to 22 mm with restoration of autograft valve function to mild insufficiency.
Autograft valve function as assessed by the most recent echocardiographic evaluation (within 1 year of closure) demonstrates retained normal function in 42 of the 43 survivors. One patient has moderate autograft valve insufficiency that developed shortly after a significant motor vehicle accident with sternal injury. Enlargement of the ascending aorta has not been demonstrated by postoperative transthoracic echocardiography in the patients with a reduction vertical aortoplasty. Measurement of the aortic root diameter, a diameter that corresponds to the maximal sinus diameter of the autograft valve, was greater than the upper limit for normal aortic valve sinus diameter (35 mm) in 13 of 43 patients. Although these patients have a sinus diameter that approximates 40 mm, neither progressive change in the sinus diameter nor autograft valve dysfunction has been seen. Actuarial freedom from moderate aortic insufficiency is similar in patients with ascending aortic replacement or ascending aortic repair to all other patients who had a Ross operation at our institution (Fig 1).
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| Comment |
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The presence of an abnormal aortic wall in patients with coarctation of the aorta and a bicuspid aortic valve has been suggested by McKusick [10], and others have noted an increased incidence of ascending aortic aneurysm or dilatation in patients with aortic insufficiency and a bicuspid aortic valve [11]. Histologic examination of aortic tissues has shown that these conditions may be formes frustes of Marfan syndrome, primarily based on the finding of cystic medial necrosis. The pathologic finding of cystic medial necrosis is not necessarily indicative of a generalized connective tissue disorder because it is found in older patients who have no gross abnormality of the aorta [12]. Patients with a stenotic bicuspid aortic valve and dilatation of the ascending aorta or aneurysm formation are at an increased risk for dissection, and surgeons have suggested replacement of the ascending aorta at the time of aortic valve replacement. As there is no known genetic basis for the combination of bicuspid aortic valve disease and ascending aortic dilatation and there is no evidence of a generalized elastin or fibrillin disorder that might also involve the pulmonary artery and valve, we used the Ross operation for aortic replacement in patients thought to have aneurysmal changes in the ascending aorta and a reduction aortoplasty in patients with a dilated ascending aorta and a normal-appearing aortic wall.
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