Ann Thorac Surg 1995;60:699-701
© 1995 The Society of Thoracic Surgeons
Case Report
Can Concordant Criss-Cross Heart Be Ameliorated by Hemodynamic Changes?
Masayoshi Nagatsu, MD,
Yorikazu Harada, MD,
Takamasa Takeuchi, MD,
Hirohisa Goto, MD,
Yoshinori Ota, MD
Department of Cardiovascular Surgery, Nagano Children Hospital, Nagano, Japan
Accepted for publication March 2, 1995.
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Abstract
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A 2-year-old female child with ``ameliorated'' concordant criss-cross heart, complicated superior-inferior ventricles, complete transposition of great arteries, hypoplastic right ventricle, ventricular septal defect, and aortic coarctation is described. The patient underwent subclavian flap, pulmonary artery banding, and balloon atrioseptostomy at age 1 month. The ameliorated concordant criss-cross anatomy was obtained after 2 years of follow-up. This dynamic morphologic change allowed us to perform a subsequent anatomic correction.
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Introduction
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Criss-cross heart is a rotational abnormality in which the systemic and pulmonary venous streams cross at the atrioventricular level without mixing [1, 2]. Regardless of whether the atrioventricular connection is concordant or discordant, each atrium connects with the contralateral ventricle and the ventricles are often arranged in a superior-inferior fashion [2–3]. Although the morphologic term of criss-cross heart has been described, to our knowledge it is not clearly shown whether a criss-cross morphology could be ameliorated by hemodynamic changes. We believe that these changes may be important for a surgical decision making in a criss-cross heart or superior-inferior ventricles.
Two years ago, a 31-day-old female infant was transported to us in a preshock status. According to the echocardiogram, a diagnosis of concordant criss-cross heart, complete transposition of great arteries with ventricular septal defect, patent ductus arteriosus and severe aortic coarctation, a subclavian flap, and ductus arteriosus ligation were performed immediately. Two days later the first cardiac catheterization revealed concordant criss-cross heart with superior-inferior ventricles, complicating situs solitus, atrioventricular concordance, and ventriculoarterial discordance, subpulmonary ventricular septal defect, and hypoplastic right ventricle as small as 34% of normal. One and 4 days later, respectively, pulmonary artery banding of 28 mm in circumference and balloon atrioseptostomy were carried out. The second catheterization at age 10 months revealed a mean pulmonary arterial pressure of 34 mm Hg and a growing right ventricular end-diastolic volume up to 99% of normal. The third catheterization data obtained at 2 years of age are summarized in Table 1
. At this point, obvious rewind of the axial rotation of the ventricular mass and the horizontally tilted down ventricular septum were shown (Fig 1). Two-dimensional echocardiography demonstrated the insertion of tricuspid valve chordae into the anterior deviated conal septum, but ruled out any straddling of chordae across the secondary interventricular foramen.
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Fig 1. . (A) Right ventriculograms and (B) left ventriculograms. (Top row) Angiograms at 33 days old; (middle row) angiograms at 10 months old; (bottom row) angiograms taken at 2 years old. Left angiograms show frontal views and the right ones show lateral views. As shown in the changes from top to bottom, the small right ventricular volume has obviously increased and the horizontal ventricular septum has tilted down, resulting in the amelioration of the criss-cross statement eventually. (AO = aorta; LV = left ventricle; PA = pulmonary artery; RV = right ventricle.)
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The anatomic correction was performed at age 2 years 7 months. A 25-mm longitudinal right ventriculotomy in the subaortic outflow exposed the infundibular septum between the aorta and the pulmonary trunk located in a sagittal plane with anterior malalignment. The ventricular septum was about 30 degrees below the horizontal plane and the pulmonary trunk overrode more than 80% on the ventricular septum. Fibrous continuity of the mitral and pulmonary valves was present. Chordae of the tricuspid valve inserted into the right side of the infundibular septum. The subaortic narrowing was relieved by massive infundibular muscle resection. An interventricular septation was carried out with a 25 x 30-mm patch so as to route blood from the left ventricle to the pulmonary trunk using 22 pledget-supported stitches placed along the resected infundibular septum, the posterior extension of the trabecula septomarginalis, and the fibrous continuity between the mitral and tricuspid valves. Then an arterial switch operation was performed using Lecompte maneuver. On the 26th postoperative day, the patient discharged in satisfactory condition.
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Comment
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Criss-cross hearts are produced by rotation of the ventricular mass along its long axis. This positional anomaly can coexist with a horizontal displacement of the ventricular mass along the horizontal plane of its long axis, which produces superior-inferior ventricles [1, 3, 4]. In this patient, the fundamental connection was situs solitus, atrioventricular concordance, and ventriculoarterial discordance. The initial appearance was clockwise rotation along its long axis and a horizontal tilt of its ventricular septum. A morphology of superior-inferior ventricles with criss-cross heart could be ameliorated by hemodynamic factors (Fig 2), as shown by this patient. The main defect was considered to be the hypoplastic right ventricle, which is not an unusual associated anomaly in superior-inferior ventricles and criss-cross heart [5], and was induced to normalize hemodynamically in this patient. The growth of the right ventricle made the subsequent anatomic correction possible, rather than the unwinding of the criss-cross itself, although the two events are obviously interlinked.
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Fig 2. . Ameliorated concordant criss-cross heart with superior-inferior ventricles. (Left and middle) The initial appearance of this single case can be produced by rotation of the ventricular mass along its long axis and horizontal tilt of the ventricular septum to the right. (Middle and right) The rewind of the criss-cross and tilted-down septum can be produced by enlargement of the hypoplastic morphologic right ventricle (MRV). The aorta was anterior, not to the right nor left, to the pulmonary trunk and the relationship of the great arteries had not be altered in this case. (A-V concordance = atrioventricular concordance; BAS = balloon atrioseptostomy; LA = left atrium; MLV = morphologic left ventricle; PAB = pulmonary artery banding; RA = right atrium; SCF = subclavian flap; V-A discordance = ventriculoarterial discordance.)
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Footnotes
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Address reprint requests to Dr Nagatsu, Department of Cardiovascular Surgery, Nagano Children's Hospital, 3100 Toyoshina, Toyoshina-cho, Minami-Azumi-gun, Nagano 399-82, Japan.
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References
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- Anderson RH. Criss-cross hearts revisited. Pediatr Cardiol 1982;3:305–13.[Medline]
- Anderson RH, Shinebourne EA, Gerlis LM. Criss-cross atrioventricular relationships producing paradoxical atrioventricular concordance or discordance: their significance to nomenclature of congenital heart disease. Circulation 1974;50: 176–81.[Abstract/Free Full Text]
- Van Praagh R, Weinberg PM, Van Praagh S. Malposition of the heart. In: Moss AJ, Adams FH, Emmanouilides GC, eds. Heart disease in infants, children and adolescents. Baltimore: Williams & Wilkins, 1977:394–417.
- Freedom RM, Culham G, Rowe RD. The criss-cross and superior-inferior ventricular heart. An angiographic study. Am J Cardiol 1984;42:620–8.
- Guthaner D, Higgins CB, Silverman JF, Hayden WG, Wexler L. An unusual form of the transposition complex. Uncorrected levo-transposition with horizontal ventricular septum. Report of two cases. Circulation 1976;53:190–5.[Abstract/Free Full Text]
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Abstract
Introduction
