Cases reported "Crisscross Heart"

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1/8. Twisted atrioventricular valves in double inlet left ventricle.

    Twisted atrioventricular connections usually occur in hearts with biventricular artioventricular connections. Here, we describe a case of twisted atrioventricular valves associated with double inlet left ventricle and discordant ventriculo-arterial connections. color Doppler echocardiography, and cine magnetic resonance imaging, clearly demonstrated that the right atrioventricular valve was located anterior and superior to the left atrioventricular valve, and that the axes of the two atrioventricular valves crossed each other within the dominant left ventricle.
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2/8. Coronary arteries in crisscross heart.

    crisscross heart, or superoinferior ventricles, is a complex and often confusing congenital anomaly. We report a heretofore unreported presentation of "isolated" crisscross heart in situs inversus levocardia, which allows us to more clearly define the typical features of crisscross ventricles. The case of this 41-year-old woman, who had a peculiar coronary anatomy, underscores the concept that coronary artery anatomy is strictly related to the myocardial mass served. In complex congenital heart defects, development of an anterior descending artery is possible (as a primary artery, along with the circumflex and right coronary arteries) only if the ventricular septum develops properly and is aligned with the semilunar valves. We use the present case of crisscross heart to illustrate the spectrum of anomalies that can occur during formation of the cardiac apex; this spectrum ranges from a normal apex, to a diverted apex (as in dextroversion in situs solitus), to a crisscross anomaly.
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3/8. A criss-cross heart with twisted atrioventricular connections, "perfect streaming," and double discordance.

    At 24 weeks gestational age, a term female infant was diagnosed with complex congenital heart disease. The antenatal cardiac diagnosis was uncertain and included univentricular heart. Following delivery, the child remained well and was normally saturated. echocardiography and angiocardiography revealed an unusual relationship between atria and ventricles.
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4/8. Criss-cross heart evaluated by colour Doppler echocardiography and magnetic resonance imaging.

    Two-dimensional colour Doppler echocardiography was performed on a 1-month-old male infant with criss-cross heart, double outlet right ventricle, ventricular septum defect and pulmonary stenosis. Complex structural abnormalities were suspected after two-dimensional echocardiography (2-D echo) and confirmed by colour Doppler and magnetic resonance imaging (MRI). We stress that the blood streams in the ventricular inflow tracts revealed by colour Doppler and the spatial relationships of the cardiac segments disclosed by MRI are essential to make an accurate non-invasive diagnosis of this complex malformation.
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5/8. An unusual ventricular loop associated with right juxtaposition of the atrial appendages.

    We report two autopsied cases of an unusual ventricular loop in hearts with right-sided juxtaposition of the atrial appendages. Case 1 showed usual atrial arrangement, a concordant atrioventricular connexion with a disharmonious ventricular loop showing left-hand topology and double outlet right ventricle with normally related arterial trunks. The atrioventricular connexions were crossing, with the inlet to the left-sided morphologically right ventricle being posterior to that of left ventricle. Mitral hypoplasia and coarctation of the aorta were the associated lesions. The second case showed usual atrial arrangement, a discordant atrioventricular connexion with an imperforate left atrioventricular orifice and double outlet right ventricle with the aorta in the right-sided position. A large right atrium was connected to right-sided morphologically left ventricle. A prominent dimple in the left atrial floor was firmly attached to the hypoplastic right ventricle which was left-sided and anterior. A small but discrete inlet portion of the right ventricle could be traced towards the anteriorly located left atrial dimple. Thus, despite the presence of a discordant atrioventricular connexion with the usual atrial arrangement, there was righ-hand ventricular topology. In each case the inlet component of the ventricular septum was displaced, being to the right in case 1 and anteriorly in case 2. We suggest that the embryologic mechanism producing disharmony between the atrioventricular connexion and the segmental combinations be interpreted on the basis of posterior ventricular looping, since they are best explained on the basis of a hypothetical heart with posteriorly located outflow tracts.
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6/8. Disharmony between atrioventricular connections and segmental combinations: unusual variants of "crisscross" hearts.

    Two congenitally malformed hearts are described, one studied in life and the other at autopsy, in which the topology of the ventricular mass was not as expected for the atrioventricular (AV) connection present. In Case 1, studied at autopsy, there was the usual atrial arrangement with a concordant AV connection. The morphologically right ventricle, however, was left-sided and the ventricular mass was of left-hand topology (l loop) rather than the expected right-hand pattern (d loop). In Case 2, studied during life, there was the usual atrial arrangement and a discordant AV connection. Rather than the anticipated left-hand topology (l loop), the ventricular mass was arranged in right-hand fashion (d loop) and the morphologically right ventricle was right-sided. The cases emphasize that for a full description of a congenitally malformed heart, it is often necessary to account for the topology of each segment as well as the connections (or alignment) among the segments.
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7/8. Can concordant criss-cross heart be ameliorated by hemodynamic changes?

    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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8/8. Superoinferior ventricles with criss-cross atrioventricular connections and intact ventricular septum.

    We report a 6-year-old girl with superoinferior ventricles, criss-cross atrioventricular (AV) relationships with solitus atria, a D-loop, L-transposition of the great arteries, subpulmonary stenosis, but without a ventricular septal defect (VSD). The diagnosis was made by echocardiographic examination and was strengthened by cardiac catheterization and angiocardiography. Balloon atrial septostomy in the neonatal period and a modified Blalock-Taussig shunt at the age of 16 months were performed to alleviate hypoxia. Following the shunt operation, the clinical condition of the patient deteriorated with progressive cardiac enlargement, congestive heart failure, and tricuspid regurgitation. We assume that the absence of a VSD contributed to this deterioration. Subsequent improvement was obtained with balloon enlargement of the atrial septal defect and a bidirectional Glenn anastomosis.
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