International Journal of Cardiology 191 (2015) 235–236
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Letter to the Editor
Compression of superior caval vein — New clinical problem of aortopathy Tomoaki Murakami a,b,⁎, Takeaki Shirai a, Yumi Shiina a, Koichiro Niwa a a b
Department of Cardiology, St. Luke's International Hospital, Japan Department of Cardiology, Chiba Children's Hospital, Japan
a r t i c l e
i n f o
Article history: Received 5 May 2015 Accepted 6 May 2015 Available online 8 May 2015 Keywords: Aortopathy Tetralogy of Fallot Pulmonary atresia Right aortic arch
Aortopathy has been recognized as one of the complications in patients with tetralogy of Fallot (TOF) [1]. However, there were not many reports concerning the clinical problems that arise from the aortopathy. Although aortic dissection is a concern, it has been rarely reported in association with the aortopathy in patients with TOF [2]. However, problems with aortopathy have been reported little by little with a
widely spread concept of aortopathy. Bradley and colleagues reported a high incidence of major adverse maternal cardiovascular-related events in patients with aortopathy [3]. Now, we report another complication of aortopathy. A 26-year-old student from China was referred to our cardiology clinic, because his primary care doctor diagnosed him with TOF. He was pointed out with the cardiac abnormalities at the age of 19 in China, but he refused further examinations. After he came to Japan to study, he sometimes felt palpitation and consulted his primary care doctor. The doctor diagnosed him as TOF and referred to us. In our outpatients' clinic, he was diagnosed with TOF, pulmonary atresia, major aortopulmonary collateral arteries and right aortic arch. Moreover, his ascending aorta was extraordinarily dilated (59 mm in diameter, Fig. 1A) and it compressed his superior caval vein (Fig. 1B). Cardiac catheterization was performed in order to judge the operability. The mean blood pressure in the superior caval vein was 11 mm Hg and that in his inferior caval vein was 6 mm Hg (Fig. 2). He is waiting for Rastelli type operation with aortoplasty. In this case, the aorta was so dilated that it compressed the superior caval vein, which has never been reported. Aortopathy is one of the
Fig. 1. Computed tomography (CT) images of the thorax. A. CT thorax sagittal plane image demonstrating the extraordinary dilated ascending aorta (59 mm in diameter). B, CT thorax coronal plane image demonstrating the dilated aorta compressing the superior caval vein. AAo indicates ascending aorta; MAPCA, major aortopulmonary collateral artery; and SCV, superior caval vein.
⁎ Corresponding author at: Department of Cardiology, Chiba Children's Hospital, 579-1 Heta-cho, Midori-ku, Chiba 266-0007, Japan. E-mail address:
[email protected] (T. Murakami).
http://dx.doi.org/10.1016/j.ijcard.2015.05.031 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
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T. Murakami et al. / International Journal of Cardiology 191 (2015) 235–236
Fig. 2. Pressure waveforms in superior and inferior caval veins. The mean blood pressure in the superior caval vein was 5 mm Hg higher than that in the inferior caval vein.
common problems in patients with TOF [1]. It is frequent in patients with right aortic arch, pulmonary atresia and a history of an aorto-pulmonary shunting, and some patient's demographics, such as male sex and the association of chromosome 22q11.2 deletion [4,5]. Our patient has several risk factors for aortopathy, that is, male sex, right aortic arch and pulmonary atresia. The compression made the 5 mm Hg of mean pressure difference between the superior and inferior caval veins. It seems not to be significant for normal circulation. However, aortopathy also could occur in patients with a single ventricle, who need Fontan type operation. In Fontan circulation, the pressure difference of 5 mm Hg in the venous system could become a serious problem. In patients with aortopathy, the dilated aorta could compress surrounding organs, such as the superior caval vein. Careful evaluation is essential, especially before surgical procedure. Conflict of interest The authors report no relationships that could be construed as a conflict of interest.
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