A case of criss-cross heart with situs inversus associated with subvalvular aortic stenosis and mitral regurgitation in a 9 year old boy is reported. Surgical correction of the valvular abnormalities gave an excellent clinical and haemodynamic result. A review of the literature shows that this abnormality of rotation of the ventricles coexists with a concordant atrioventricular connection in 70 % cases; the ventriculoarterial connection is only discordant in 12 % cases. The right ventricle is always situated above the left ventricle. The most common associated lesions are usually accessible to surgery; ventricular septal defect (98 % cases); stenosis or atresia of the pulmonary artery (70% cases). However, right ventricular hypoplasia is also common (65 % cases). Six patients underwent surgical correction of the associated lesions with 4 successes and 2 deaths. Surgery is possible in these complex cardiac malformations with good results providing a precise preoperative anatomical diagnosis is made.

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