Ann Thorac Surg 2003;75:1963-1965
© 2003 The Society of Thoracic Surgeons
Case report
A case of acute aortic dissection type a in a patient with situs inversus
Tetsuya Niino, MDa*,
Motomi Shiono, MDa,
Tatsuya Inoue, MDa,
Mitsumasa Hata, MDa,
Akira Sezai, MDa,
Nanao Negishi, MDa
a Second Department of Surgery, Nihon University School of Medicine, Tokyo, Japan
Accepted for publication November 26, 2002.
* Address reprint requests to Dr Niino, Second Department of Surgery, Nihon University School of Medicine, 30-1 Ooyaguchi Kamimachi, Itabashi-ku, Tokyo 173-0081, Japan
e-mail: xj8t-nin{at}asahi-net.or.jp
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Abstract
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We report a case of acute aortic dissection type A in a patient with situs inversus. A 33-year-old male, complaining of sudden chest pain, visited our institute. Contrast-enhanced computed tomography and echocardiography suggested Stanford type A acute aortic dissection and dextrocardia. Aortic root and ascending aortic replacement were successfully performed. Antegrade brain-isolated extracorporeal circulation was established. The aortic branch arteries were mirror-image reversed. Anatomic positional relationships and presence or absence of concurrent anomalies should be sufficiently investigated preoperatively in patients with dextrocardia. His postoperative course was uneventful and a postoperative computed tomographic scan confirmed a good result.
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Introduction
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Dextrocardia describes a condition in which the cardiac apex is in the right thorax with reversal of the axis of cardiac inclination. Situs inversus totalis is a rare congenital cardiac malformation characterized by a perfect mirror image of the normal anatomy of the visceral organs.
A 33-year-old male was hospitalized with sudden back pain. Contrast computed tomography (Fig 1)
and echocardiography revealed Stanford type A acute aortic dissection and dextrocardia. He did not appear to have Marfan’s syndrome and echocardiography did not reveal aortic valve regurgitation or concurrent cardiac anomaly.
Emergency surgery was carried out. The three arch vessels were completely reversed in mirror image (Fig 2a).
In addition, the ventricular apex was forced to the right side, and the superior vena cava and inferior vena cava were located on the left side. All operative findings were completely reversed compared with normal arteries. Cardiopulmonary bypass was established from the left-sided right (venous) atrium to the right femoral artery. Circulation was stopped at a rectal temperature of 24°C. A left ventricular vent was inserted through the left superior pulmonary vein. Selective cerebral perfusion was carefully established by introducing balloon cannulas into the brachiocephalic and carotid arteries. The intimal tear was located just proximal to the innominate artery. Aortic root and ascending aortic replacement were finally required because the dissection involved the right coronary orifice and aortic valve sinuses. The open distal anastomosis was initially carried out using a 26-mm prosthesis. Antegrade systemic flow was then initiated from the sidearm of the prosthesis. Aortic root replacement was performed with a valved conduit. Finally, two prosthesis were anastomosed to each other (Fig 2b) Pathologic findings of the aortic wall demonstrated arteriosclerotic changes. The postoperative course was uneventful. Postoperative echocardiogram indicated the mechanical aortic valve was working well. At present, this patient is followed in the outpatient clinic and is without complications.
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Fig 2. Intraoperative findings. (a) The three arch vessel positions were completely turned around compared with normal positions. (b) Aortic root and ascending aortic replacement were performed.
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Comment
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Dextrocardia represents a positional abnormality in which the heart is present in the right thorax. The incidence has been reported to be 0.02% [1]. Dextrocardia is classified into three types by the positional relationship between the visceral organ and the atrium: specular type dextrocardia in which the visceral organ has a specular relation to the normal position, solitary dextrocardia in which the atrium and visceral organ are present in the normal position, and atrial-visceral dislocation syndrome with asplenia or polysplenia. In patients with dextrocardia, the incidence of concurrent cardiac anomaly has been reported to be high. In patients with solitary dextrocardia or atrial-visceral dislocation syndrome, the incidence has been reported to range from 50% to 90% [2]. Furthermore, the incidence of concurrent cardiac anomaly in patients with specular type dextrocardia varies: 0.05% or less to 50% or more in a total number of patients with dextrocardia [3–5]. Therefore, it is important to preoperatively evaluate concurrent cardiac anomaly. In particular, dextrocardia causes a high incidence of venous perfusion abnormalities; thus, caution is needed [3]. When emergency surgery is indicated, as in the present patient, sufficient detailed examination may be difficult. However, preoperatively, abnormalities such as aortic valve regurgitation should be confirmed by echocardiography, and congenital heart disease and the position of the inferior vena cava should be investigated if possible. A study has reported that among dextrocardia types, specular type dextrocardia is associated with autosomal recessive inheritance [6]. However, it remains to be clarified. In our patient, family history did not suggest this disorder. The surgical technique is not special, but surgeons may be confused due to the specular findings. The anatomic positional relationship and presence or absence of concurrent anomaly should be sufficiently investigated preoperatively. None of the previous studies have reported emergency surgery for acute aortic dissection complicated by dextrocardia. Our patient was an extremely rare case.
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References
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- Van P.R., Weinberg P.M., Smith S., et al. Malposition of the heart. . Moss‘s Heart diseases in infant, children, and adolescents, 4th ed Baltimore: Williams & Wilkins, 1989:530-589.
- Momma K. Dextrocardia, mesocardia, levocardia. . Rinsho Hattatsu-Sinnzoubyou Gaku, 2nd ed Japan: Chagai-Igakasha, 1997:546-555.
- Grant R.P. The syndrome of dextroversion of the heart. Circulation 1958;18:25-36.[Medline]
- Kuribayashi R., Abe T. Mirror image dextrocardia and its associated congenital cardiac anomalies: a report of a case and review of 185 cases collected from literature. Jpn J Thorac Surg 1972;25:179-182.
- Arcilla R.A., Gasul B.M. Congenital dextracardia: clinical, angiocardiographic, and autopsy studies on 50 patients. J Pediat 1961;58:39-58.
- Cockayne E.A. The genetics of transposition of the viscera. Q J Med 1938;7:479-493.
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Abstract
Introduction
