Ann Thorac Surg 2008;85:1441-1443. doi:10.1016/j.athoracsur.2007.10.013
© 2008 The Society of Thoracic Surgeons
Case Reports
Heart Failure Due to Severe Supravalvular Aortic Stenosis in Painless Type A Aortic Dissection
Hiroaki Sakamoto, MD, PhD*,
Yasunori Watanabe, MD, PhD,
Haruhiko Sugimori, MD
Department of Cardiovascular Surgery, Hitachi General Hospital, Hitachi, Ibaraki, Japan
Accepted for publication October 2, 2007.
* Address correspondence to Dr Sakamoto, Department of Cardiovascular Surgery, Hitachi General Hospital, 2-1-1 Jonan, Hitachi, Ibaraki, 317-0077, Japan (Email: sakamotoh{at}aol.com).
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Abstract
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Severe supravalvular aortic stenosis is a rare complication of aortic dissection. Painless aortic dissection is also relatively rare. We report the case of a 67-year-old man who had New York Heart Association class III heart failure without chest pain and was found to have supravalvular aortic stenosis secondary to chronic type A aortic dissection. The patient underwent ascending aorta and hemiarch replacement with selective antegrade cerebral perfusion under deep hypothermic circulatory arrest. The patient was discharged to home with complete resolution of heart failure and has been carefully followed up for 1 year, during which he has experienced no symptoms.
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Introduction
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Slitlike supravalvular aortic stenosis is a rare complication of aortic dissection. Painless aortic dissection is also relatively rare. We present the case of a patient who had heart failure due to severe supravalvular aortic stenosis secondary to painless type A aortic dissection.
A 67-year-old man was referred to our hospital for evaluation of persistent cough. The patient had a medical history remarkable for type B aortic dissection and a 1-month history of cough. At admission, blood pressure was 184/93 mm Hg, and room air oxygen saturation was 87%. He denied any chest or back pain. A 3/6 systolic murmur was noted at the second left intercostal space. A chest radiograph demonstrated bilateral lung congestion, mild cardiomegaly, and dilatation of the ascending aorta. Blood chemistry values were in the normal range except for a brain natriuretic peptide (BNP) concentration of 1,388 pg/mL. The upper normal limit of BNP is about 20 pg/mL. The clinical findings were consistent with New York Heart Association (NYHA) class III disease. A computed tomography scan of the chest showed a type A aortic dissection with an intimal flap in the ascending aorta extending into the arch, and bilateral pleural effusion. The false lumen of the dissection in the proximal portion of the ascending aorta was completely thrombosed, causing severe compression of the true aortic lumen (Fig 1). An antecedent type B aortic dissection did not show any change. Transthoracic echocardiography was performed to measure the pressure gradient across the compressed portion of the ascending aorta, but the gradient could not be measured because the angle of the echocardiographic probe in relation to the heart was not good. Preoperative cardiac catheterization was not performed, to avert iatrogenic complications such as aortic rupture.
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Fig 1. Computed tomography scan shows completely thrombosed false lumen of ascending aorta, which produced slitlike narrowing of true lumen (arrow), and bilateral pleural effusion.
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The patient was taken to the operating room. Intraoperative transesophageal echocardiography demonstrated mild aortic valve regurgitation and severe supravalvular aortic stenosis due to the thrombosed false lumen, but there were no left ventricular wall motion abnormalities and no pericardial effusion (Fig 2). The chest was opened with a median sternotomy. Cardiopulmonary bypass was established with femoral artery and bicaval cannulation. The patient was cooled to 20° C, and circulatory arrest with selective antegrade cerebral perfusion was initiated. On opening the ascending aorta, the large false lumen was observed to be filled with an organized thrombus that compressed the true lumen, causing severe supravalvular aortic stenosis. An intimal tear was found in a proximal segment of the aortic arch, with subsequent retrograde dissection to the aortic root. The thrombosed false lumen extended to just above the aortic valve annulus of the noncoronary cusp, which resulted in mild aortic valve regurgitation due to prolapse of the noncoronary cusp. Ascending aorta and hemiarch replacement was performed using a 28-mm Dacron graft (DuPont, Wilmington, DE). The thrombus was removed from the false lumen, and the dissected proximal ascending aorta was glued together with gelatin-resorcin-formol and buttressed with Teflon felt. After the repair, an intraoperative transesophageal echocardiogram demonstrated coaptation of the aortic valve leaflets, and there were no signs of aortic regurgitation.
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Fig 2. Transesophageal echocardiogram demonstrates mild aortic valve regurgitation and severe supravalvular aortic stenosis due to thrombosed false lumen (arrow).
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Extubation was performed on the fifth postoperative day using an endotracheal tube. A postoperative echocardiogram showed no signs of supravalvular aortic stenosis or aortic valve regurgitation. After the operation, lymphorrhea developed in the femoral artery wound, which was the cannulation site. The femoral wound healed after a few weeks. The patient was discharged to home after a 4-week postoperative course, with complete resolution of heart failure. He has been carefully followed up for 1 year, during which he has experienced no symptoms. One year after the operation, the BNP concentration decreased to 100 pg/mL and heart failure improved to NYHA class I.
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Comment
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Severe supravalvular aortic stenosis is a rare complication of aortic dissection; there are only three reports in the literature [1-3]. The mechanisms of supravalvular aortic stenosis described in previous reports are as follows. Vilacosta and colleagues [1] reported that the large thrombosed false lumen obstructed the true lumen of the ascending aorta. Roan and colleagues [2] reported that the large thickened dissected intimal flap with a ball valve–like movement obstructed the ascending aorta. Sato and colleagues [3] reported that the dissected intimal flap and false lumen of the ascending aorta caused supravalvular aortic stenosis without ball valve–like movement of the dissected flap or thrombus within the false lumen. The mechanism of supravalvular aortic stenosis in our case patient was similar to that reported by Vilacosta and colleagues [1].
Painless aortic dissection is relatively rare. The most common clinical symptom of aortic dissection is severe chest or back pain. However, other symptoms related to the course of the dissection may complicate the clinical findings of this disease, making diagnosis difficult [4].
Our patient denied chest or back pain and had a systolic heart murmur. Moreover, he had NYHA class III congestive heart failure accompanied by an elevated BNP concentration and bilateral pleural effusion. Our initial suspicion was that heart failure was caused by valvular heart disease. However, a CT scan showing a type A dissection with a thrombosed false lumen causing severe compression of the true lumen was instrumental in discerning the true cause of the heart failure.
On opening the ascending aorta, the false lumen was found to be filled with organized old thrombi, which produced a slitlike narrowing of the true lumen. In addition, the aortic wall was thickened, rather than thin and fragile, which is characteristic of acute aortic dissection. Therefore, this patient had chronic type A dissection that probably occurred at the onset of cough 1 month earlier.
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References
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- Vilacosta I, San Roman JA, Aragoncillo P, et al. Supravalvular aortic stenosis in aortic dissection Am J Cardiol 1998;81:1271-1273.[Medline]
- Roan PG, Buja LM, Grammer JC. Ascending aortic obstruction produced by dissected intimal flap Br Heart J 1981;46:452-454.[Free Full Text]
- Sato Y, Matsukage T, Matsumoto N, et al. Painless acute aortic dissection presenting as discrete supravalvular aortic stenosis Int J Cardiol 2007;114:125-126.[Medline]
- Hagan PG, Nienaber CA, Isselbacher EM, et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease JAMA 2000;283:897-903.[Abstract/Free Full Text]
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Abstract
Introduction
