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J Thorac Cardiovasc Surg 2002;124:176-178
© 2002 The American Association for Thoracic Surgery
Brief Communications |
From the Departments of Pediatric Cardiovascular Surgerya and Pediatric Cardiology,b The Heart Institute of Japan, Women's Medical University, Tokyo, Japan.
Received for publication Sept 18, 2001. Accepted for publication Dec 10, 2001. Address for reprints: Junichi Kashiwagi, MD, Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, 8-1 Kawada, Shinjuku, Tokyo, 186-8666, Japan (E-mail: skasiwag{at}hij.twmu.ac.jp).
Crisscross heart is a rare cardiac malformation that results from the abnormal rotation of the ventricles.
1 We performed a successful arterial switch operation in a patient with a concordant crisscross heart that was complicated by a complete transposition of the great arteries. To our knowledge, an arterial switch operation to treat a concordant crisscross heart has not been reported.
Clinical summary
A 2-year-old boy with a concordant crisscross heart, a complicated complete transposition of the great arteries, a ventricular septal defect (VSD), an atrial septal defect, and a patent ductus arteriosus was referred after a balloon atrioseptostomy at 1 day of age and pulmonary artery (PA) banding at 31 days of age. A chest radiograph revealed levocardia with a cardiothoracic ratio of 59%. An electrocardiogram exhibited a sinus rhythm and a mean frontal QRS axis of -70°. Preoperative cardiac catheterization showed a mean PA pressure of 18 mm Hg, a pulmonary/systemic flow ratio of 1.3, and a pulmonary vascular resistance of 2.0 Wood units (Table 1). The systolic pressures in the ventricles were identical, and the pressure gradient at the PA banding site was 63 mm Hg. Left and right ventriculograms are shown in Figure 1. An inferior ventricular injection showed an anatomically left ventricle and an intraventricular septum. The PA originated from the left ventricle. A superior ventricular injection showed an anatomically right ventricle and an ascending aorta that originated from the right ventricle. The PA was well developed, with a PA index of 406. The left ventricular end-diastolic volume was 133% of normal, with a left ventricular ejection fraction of 61%. The right ventricular end-diastolic volume was 102% of normal, with a right ventricular ejection fraction of 51%.
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Discussion
Only two cases of definitive biventricular repairs
2,3 and a few cases of modified Fontan procedures
4,5 for the surgical treatment of concordant crisscross heart have been reported. We have performed two biventricular repairs,
2 including this case, and 17 modified Fontan procedures in patients with concordant crisscross heart anomalies from June 1983 to March 2001. The 17 modified Fontan procedures were indicated for hypoplastic right ventricles (n = 3), hypoplastic left ventricles (n = 2), straddling atrioventricular valves (n = 4), and ventricular anatomic features (n = 8). In the 8 patients with ventricular anatomic features, VSDs were present that occupied almost the entire inflow of the right ventricle and reached its apex, where the chordae of the mitral valve were observed. Among the 17 modified Fontan procedures, 1 late death occurred (of congestive heart failure at 13.8 months after the operation).
In the case of a concordant crisscross heart with a complicated straddling of the atrioventricular valve, a biventricular repair is not suitable. Such cases require a modified Fontan procedure. Important considerations in deciding to adapt a biventricular repair are the presence of hypoplastic one ventricle, a straddling of the atrioventricular valve, and the other ventricular anatomic features.
References
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