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Address for reprints: Prashanth Vallabhajosyula, MD, Division of Cardiothoracic Surgery, University of Pennsylvania, 3400 Spruce St, 6 Silverstein, Philadelphia, PA.
A 72-year-old man with bicuspid aortic valve (BAV) disease underwent aortic valve replacement (AVR) in 1993, and subsequently developed cardiomyopathy secondary to sarcoidosis for which he underwent orthotopic heart transplantation in 1995. Posttransplantation, he developed mild hypertension in the range of 130 to 140 mm Hg (systolic), but otherwise was doing well clinically and undergoing routine echocardiographic surveillance. In late December 2017, he presented to an outside hospital with chest discomfort. Echocardiography at that time showed normal function of the transplanted heart and an ascending aortic diameter of 3.6 cm. As part of a workup for presumed pneumonia, he underwent a computed tomography (CT) scan of the chest, which incidentally showed a distal ascending aortic/arch aneurysm in the native aorta with maximal diameter of 7.2 cm.
It is difficult to establish the temporal progression of the aneurysm based on echocardiography reports alone. For example, in 2015 the mid ascending aorta was “mildly dilated,” and a subsequent report in 2016 failed to mention the ascending aorta at all, demonstrating the limitations of echocardiography as an imaging modality to adequately delineate the distal ascending aorta and arch.
Upon completion of the preoperative evaluation, the patient underwent reoperative cardiac surgery for aneurysm resection. Right common femoral artery and vein were accessed for cardiopulmonary bypass before entering the chest, because the aorta was right underneath the sternum. Intraoperative inspection revealed a chronic pseudoaneurysm of the native ascending aorta directed posteriorly between the right pulmonary artery and superior vena cava. The donor aorta was nonaneurysmal and not involved in the disease process. After careful dissection, the ascending aorta was freed and a crossclamp was applied. The aneurysm was resected and transverse hemiarch replacement was performed under deep hypothermic circulatory arrest with retrograde cerebral perfusion. Upon completion of the distal anastomosis, cardiopulmonary bypass was resumed, and ascending aortic replacement was performed (Video 1). Follow-up clinic visit at 1 and 6 months showed stable repair by CT imaging (Figure 1).
Video 1Dr Prashanth Vallabhajosyula describes the operative steps of aneurysmal resection in a patient with delayed bicuspid aortic valve related aneurysm formation after heart transplantation, and discusses the importance of computed tomography scan surveillance of the thoracic aorta in patients with bicuspid aortic valve disease. Video available at: https://www.jtcvs.org/article/S0022-5223(19)30591-4/fulltext.
Figure 1Computed tomography scan of the chest in a patient with a delayed aneurysmal complication of bicuspid aortic valve disease more than 20 years after heart transplantation. A, Preoperative. B, Postoperative.
To our knowledge, this is the first reported case in the literature of a recipient native ascending aortic aneurysm in a patient with BAV disease after cardiac transplantation. Other reports have described thoracic aortic aneurysms in the recipients' native aorta unrelated to BAV disease,
Nevertheless, a retrospective review of 243 explanted hearts found that 7 patients had native (recipient) BAV disease, suggesting that the prevalence of BAV disease in the heart transplantation population may be as high as 2.8%.
The etiology of this aneurysm is likely a combination of risk factors related to both BAV disease and heart transplantation. Current theory suggests that ascending aortic dilatation in BAV disease results from hemodynamic and embryologic influences.
Because any hemodynamic abnormalities associated with the morphologically bicuspid valve should have been eliminated at the time of this patient's AVR, this case underscores the importance of the embryologic component of aortopathy in BAV disease. Aneurysm formation is also more prevalent after heart transplantation than in the general population due to factors unrelated to BAV disease.
For example, immunosuppression may directly increase the rate of atherosclerosis and weaken aortic wall tissue. The patient was taking prednisone (5 mg daily), which has been associated with aneurysm expansion in animal models,
and tacrolimus (0.5 mg twice per day), which is known to induce hypertension. Moreover, hemodynamic changes related to improved cardiac output after heart transplantation could exacerbate the problem.
Although the exact cause for aneurysm formation in this patient may not be known, this case demonstrates the necessity for follow-up thoracic aortic imaging in BAV patients. The 2018 American Association for Thoracic Surgery guidelines for BAV aortopathy recommend regular interval imaging of the thoracic aorta regardless of baseline diameter to screen for aneurysm formation.
At our institution, surveillance CT imaging is not a routine part of follow-up care after heart transplantation. Therefore, future studies should assess the need for surveillance imaging of the thoracic aorta in patients after heart transplantation, especially in the presence of BAV disease and other risk factors for aneurysm formation.
Conclusions
Surgery on the ascending aorta can be done safely with minimal surgical complications many years after cardiac transplantation. Routine surveillance with follow up CT imaging of the thoracic aorta may be warranted in BAV patients after cardiac transplantation.
Dr Prashanth Vallabhajosyula describes the operative steps of aneurysmal resection in a patient with delayed bicuspid aortic valve related aneurysm formation after heart transplantation, and discusses the importance of computed tomography scan surveillance of the thoracic aorta in patients with bicuspid aortic valve disease. Video available at: https://www.jtcvs.org/article/S0022-5223(19)30591-4/fulltext.
References
Fukuhara S.
Stephens E.H.
Glotzbach J.P.
Borger M.A.
Repair of ascending aortic aneurysms following cardiac transplantation.
We read with great interest the case report by Bojko and colleagues1 of a patient with aortic aneurysm very late after heart transplantation (HTx). The patient, who had a history of aortic valve replacement for bicuspid aortic valve (BAV) disease, underwent HTx for developed cardiomyopathy secondary to sarcoidosis. An incidental computed tomographic image of the chest taken to rule out pneumonia revealed a distal ascending aortic aneurysm with maximal diameter of 7.2 cm. The patient had been followed up with transthoracic echocardiography, which had shown no evidence of dilatation of the thoracic aorta.
In this issue of the Journal, Bojko and colleagues1 describe the development of a pseudoaneurysm in native aortic tissue 22 years after heart transplantation on the background of aortic valve replacement for bicuspid aortic valve (BAV) in the recipient. They recommend regular surveillance in this clinical setting. With an incidence of 1% to 2% in the population, more than an occasional heart transplant recipient will fall into the BAV category—one report has the rate as high as 2.8%2—so this recommendation has some importance.
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