Ann Thorac Surg 2010;89:963-965. doi:10.1016/j.athoracsur.2009.07.095
© 2010 The Society of Thoracic Surgeons
Case Reports
Giant Coronary Artery Aneurysm With Pulmonary Artery Fistula in a Patient on Chronic Hemodialysis
Chiho Tokunaga, MD, PhDa,*,
Akito Imai, MDa,
Yoshiharu Enomoto, MD, PhDa,
Yumiko Oishi Tanaka, MD, PhDb,
Shonosuke Matsushita, MD, PhDa,
Yuji Hiramatsu, MD, PhDa,
Yuzuru Sakakibara, MD, PhDa
a Department of Cardiovascular Surgery, University of Tsukuba, Ibaraki, Japan
b Department of Radiology, University of Tsukuba, Ibaraki, Japan
Accepted for publication July 27, 2009.
* Address correspondence to Dr Tokunaga, 1-1-1 Tennoudai, Tsukuba-shi, Ibaraki, 305-8575, Japan (Email: chiho-t{at}md.tsukuba.ac.jp).
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Abstract
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The combination of coronary artery aneurysm and pulmonary artery fistula is extremely rare and its common cause is atherosclerosis. A 61- year-old woman presented with a giant coronary artery aneurysm with pulmonary artery fistula and intramyocardial calcifications of the left ventricle associated with progressive atherosclerosis due to chronic hemodialysis. The coronary artery aneurysm was resected under cardiopulmonary bypass because of hemodynamic instability due to restrictive cardiac dysfunction. The patient's restrictive cardiac dysfunction was improved after aneurysm resection. Surgical resection should be considered for giant coronary artery aneurysm with restrictive cardiac dysfunction.
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Introduction
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The combination of coronary artery aneurysm and pulmonary artery fistula is extremely rare and its common cause is atherosclerosis [1, 2]. Here we report the case of a 61-year-old woman on chronic hemodialysis who showed restrictive cardiac dysfunction induced by the giant coronary artery aneurysm with pulmonary artery fistula.
A 61-year-old woman who had been undergoing hemodialysis for 18 years due to Alport syndrome presented with progressive shortness of breath and hypotension during hemodialysis.
A chest roentgenogram showed cardiomegaly and a densely marginal calcified mass overlapping onto the cardiac contour. An echocardiogram showed a severely calcified posterior wall of the left ventricle and restrictive diastolic dysfunction. Left posterior wall motion was akinetic due to severe calcification, although the global ejection fraction was maintained at 68%. A coronary computed tomography angiographic scan performed with a 16-detector row showed an 8 cm x 8 cm intrapericardial aneurysm with severe calcification oppressing the ascending aorta and right ventricle (Figs 1 and 2).
The aneurysm was connected to the right coronary artery; however, the coronary flow to the distal artery was preserved (Fig 3A). A dilated and calcified circumflex coronary artery running into the ventricle muscle formed intramyocardial calcification. A coronary angiographic scan revealed a giant coronary aneurysm arising from the branch of the right coronary artery and draining into the pulmonary artery (Fig 3B). For the patient to continue with hemodialysis, surgical correction was indicated to correct hemodynamic instability due to restrictive cardiac dysfunction induced by the calcified aneurysm and intramyocardial left ventricle calcifications. Under cardiopulmonary bypass, the aneurysm was resected, and communication to the branch of the right coronary artery and the pulmonary artery was closed with pledgetted sutures. The patient's restrictive cardiac dysfunction was improved and her hemodynamics during hemodialysis became stable after aneurysm resection, even though we left the calcified left ventricle as it was before surgery. Pathologic examination revealed a right coronary artery aneurysm with extensive wall sclerosis.
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Fig 1. Four-chamber view of computed tomographic scan shows a giant aneurysm oppressing the right ventricle and calcified left ventricle wall.
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Fig 3. (A) Coronary angiography revealed a branch into the aneurysm arising from the right coronary artery (RCA). (B) The flow from the aneurysm draining into the pulmonary artery (PA) (white arrows).
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Comment
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Although coronary artery aneurysms have been more frequently diagnosed due to advances in imaging technology, giant coronary artery aneurysms remain rare [2]. Aneurysm of the coronary artery is defined as coronary dilatation, which exceeds the diameter of normal adjacent segment by 1.5 times [1], and an aneurysm more than 20 mm in diameter called a giant coronary artery aneurysm [2]. The most common cause for coronary aneurysms is atherosclerosis, which accounts for 50% of all cases, whereas congenital heart disease causes 17% and Kawasaki disease 10% [2]. Coronary-to-pulmonary artery fistula is a congenital heart malformation, and the combination of coronary artery aneurysm and pulmonary artery fistula is extremely rare. Hirose and colleagues [3] reported that the incidence of coronary artery aneurysm associated with fistula was found in only 0.02% of patients who had undergone coronary angiography [3]. Li and colleagues [2] also reported that coronary artery aneurysm was found in 5.9% of patients with congenital coronary artery fistula, which was found in only 0.2% of patients who had undergone heart surgery.
Our understanding of the pathology of coronary artery aneurysms is still limited, although there is a consensus that the essential component in the formation of coronary aneurysm is an abnormal vessel media that may be secondary to an extension of the intimal arteriosclerosis process [1].
It is well known that patients on hemodialysis have a much higher prevalence of atherosclerosis than the general population. A number of uremia-associated factors and disturbances of the calcium-phosphate metabolism may enhance the rate of atherosclerosis progression [4]. In the present case, our patient showed severe vascular calcifications, including the left circumflex coronary artery and those that may have been due to dysregulation of the mineral metabolism related to her chronic hemodialysis. Therefore, the growth of her huge coronary artery aneurysm associated with the pulmonary artery fistula may have been accelerated by her progressive atherosclerosis.
Small coronary artery aneurysms without symptoms may be treated with simple observation, whereas giant coronary artery aneurysms with significant symptoms should be considered for surgical resection. Various surgical approaches have been reported to effectively close the fistula and resect the aneurysm. Hirose and colleagues [3] reported the resection of a left anterior descending coronary artery aneurysm through the pulmonary artery and coronary artery bypass grafting to the distal left anterior descending coronary artery for suspected distal occlusion [3]. Said and colleagues [5] simply ligated the proximal ends of the fistula and sutured the distal ends through the pulmonary artery. A surgical strategy or technique must be selected in accordance with the size and anatomy of the aneurysm to perform safe and effective corrections, and the contribution of imaging is essential for preoperative evaluation.
Our patient was suffering from restrictive cardiac dysfunction not only because of a giant aneurysm oppressing the right ventricle, but also because of the intramyocardial calcification of the left ventricle wall due to calcified circumflex arteries. We believe that this is the first report of this complicated hemodynamic physiology in a hemodialysis patient. We believe that complete removal of the aneurysm would be necessary to release the ventricle restriction; therefore, we performed a resection of the aneurysm and closed the fistula under direct vision through the aneurysm during cardioplegic arrest. The complete removal of the aneurysm was effective in improving the patient's hemodynamics during hemodialysis, even though we left the calcified left ventricle as it was before the surgery.
In summary, we have reported a successful surgical treatment of a rare giant calcified coronary artery aneurysm with a pulmonary artery fistula in a chronic hemodialysis patient. Surgical resection should be considered for giant coronary artery aneurysm with a restrictive cardiac dysfunction.
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References
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- Syed M, Lesch M. Coronary artery aneurysm: a review Prog Cardiovasc Dis 1997;40:77-84.[Medline]
- Li D, Wu Q, Sun L, et al. Surgical treatment of giant coronary artery aneurysm J Thorac Cardiovasc Surg 2005;130:817-821.[Abstract/Free Full Text]
- Hirose H, Amano A, Yoshida S, et al. Coronary artery aneurysm associated with fistula in adults: collective review and a case report Ann Thorac Cardiovasc Surg 1999;5:258-264.[Medline]
- Krasniak A, Drozdz M, Pasowicz M, et al. Factors involved in vascular calcification and atherosclerosis in maintenance haemodialysis patients Nephrol Dial Transplant 2007;22:515-521.[Abstract/Free Full Text]
- Said SA, de Voogt WG, Hamad MS, et al. Surgical treatment of bilateral aneurysmal coronary to pulmonary artery fistulas associated with severe atherosclerosis Ann Thorac Surg 2007;83:291-293.[Abstract/Free Full Text]
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Abstract
Introduction
