Our experience confirms that patients with right isomerism have more complex cardiac defects than those with left isomerism. Overall, the presence of isomerism carries a poor prognosis, the more so for right isomerism, with this related to the complex cardiac abnormalities. In our cohort, extracardiac totally anomalous pulmonary venous connection with pulmonary arterial obstruction was always a fatal combination. The mapping of cardiac and abdominal morphologies is still essential for proper diagnosis of these syndromes, especially in fetal life.

Primary cardiac tumors in infancy and childhood are rare and usually benign. Fibroma is the second most common bening cardiac tumor after rhabdomyoma. Surgery is required when fibromas cause ventricular outflow tract obstruction, ventricular dysfunction and life-threatening arrhythmia. This case report describes a 9-year-old asymptomatic male presenting with a giant left ventricular cardiac fibroma who was followed up using conservative treatment.

Summary: Angiocardiographic appearance of a crisscross heart with straddling tricuspid valve has been presented. Atrial situs was normal and the right atrium was connected to the morphological right ventricle situated superiorly and to the left of the left ventricle. The left atrium was connected to the morphological left ventricle situated inferiorly and to the right of the right ventricle. The interventricular septum was horizontal in position. Both great arteries arose from the right ventricle with the anterior aorta. The case was concluded as a criss-cross heart with concordant atrioventricular (A-V) connection and double outlet right ventricle (DORV). Straddling of the right A-V valve was recognized in another institution by two-dimensional (2-D) echocardiography and confirmed by right atrial angiography in our institution. The value of combined investigation with selective four-chamber angiography, especially including right atrial angiography and 2-D echocardiography in the diagnosis of such complex cardiac anomalies has been stressed.

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From February 1992 to October 1995, 504 patients aged 3 to 69 years (median, 36 years) received 663 Ultracor prostheses (103 aortic, 259 mitral, 12 tricuspid, and 130 multiple). Early mortality was 2.77% with a rate of 3.88% for aortic valve replacement, 3.47% for mitral, 0% for tricuspid, and 0.99% for double valve replacement. Follow-up was 100% (719.5 patient-years) and the overall mortality (early and late deaths) was 3.76% with a rate of 4.85% after aortic valve replacement, 3.85% after mitral valve replacement, 8.33% after tricuspid valve replacement, and 2.97% after double valve replacement. There were no deaths among 29 patients who had triple valve replacement. All patients including 33 children (3.5 to 18 years of age) received sodium warfarin. The linearized risk per patient-year for all embolic events (major and minor) was 0.69%, 1.11%, and 0.69% for aortic, mitral, and multiple valve replacement respectively. When only major events were considered, the linearized risks were 0.27%, 0.13%, and 0% per patient-year respectively. Freedom from major systemic embolism was 99% ± 0.07% after aortic valve replacement, 99% ± 0.07% after mitral valve replacement, and 99% ± 0.06% after multiple valve replacement. Five patients suffered valve thrombosis, 4 of whom definitely received inadequate anticoagulation therapy. Sixteen patients had 25 episodes of anticoagulant-related hemorrhage. No structural failure was recorded and no significant hemolysis was found in the absence of periprosthetic leak. This experience encourages us to continue using the Ultracor prosthesis.