Surgical Management of Left Ventricular Outflow Tract Obstruction after Biventricular Repair of Double Outlet Right Ventricle

Regardless of the preoperative morphology and the type of operation, left ventricular outflow tract obstruction (LVOTO) after biventricular repair of double outlet right ventricle (DORV) may develop. This report presents our 10-yr experience with surgical management of LVOTO after biventricular repair of DORV. Between 1996 and 2006, 15 patients underwent reoperation for subaortic stenosis after biventricular repair of DORV. The mean age at biventricular repair was 23.3+/-18.3 months (1.1-64.2). Biventricular repairs included tunnel constructions from the left ventricle to the aorta in 14 cases and an arterial switch operation in one. The mean left ventricle-to-aorta peak pressure gradient was 54.0+/-37.7 mmHg (15-140) after a mean follow-up of 9.5+/-6.3 yr. We performed extended septoplasty in nine patients and fibromuscular resection in six. There were no early or late mortality. There was one heart block and one aortic valve injury after an extended septoplasty, and two and one after a fibromuscular resection. No patient required reoperation for recurrent subaortic stenosis. The mean pressure gradient was 11.2+/-11.4 mmHg (0-34) after a mean follow-up of 5.6+/-2.7 yr. Extended septoplasty is a safe and effective method for the treatment of subaortic stenosis, especially in cases with a long-tunnel shaped LVOTO.

Aplicación del análisis secuencial segmentario en 100 especímenes cardiopulmonares / Sequential segmental analysis in 100 cardiopulmonary specimens

OBJECTIVE: To evaluate the feasibility and accuracy of the sequential segmental analysis (SSA) to diagnose congenital heart diseases. MATERIAL AND METHODS: 100 cardiopulmonar specimens were analyzed according with the sequential segmental analysis. The atrial situs, atrioventricular (AVC) and ventriculoarterial connections (VAC) were defined, finally associated lesions were observed. RESULTS: From 100 specimens, 91 had situs solitus (SS), 6 inversus (SI) and 3 isomerism (2 right isomerism and 1 left isomerism). The AVC in these hearts were: 89 biventricular, 80 (90%) concordant, 6 (6.7%) discordant and 3 (3.3%) ambiguous (2 right topology an 1 left topology). The univentricular AVC were 11, 4 (36%) had absent left AVC (mitral atresia), 3 (27%) absent right AVC (tricuspid atresia), 2 (18%) double inlet right ventricle (DIRV), 1 double inlet left ventricle (DILV) and 1 (9%) double inlet undetermined ventricle. The VAC in SS were: 52 (57%) concordant, 13 (14%) discordant, 6 (6.6%) double outlet right ventricle (DORV), 20 (22%) with single outlet (13 pulmonary atresia , 5 aortic atresia and 2 common arterial trunk). The VAC in SI were: concordant in one, (16.7%), discordant 3 (50%) and DORV in 2 (33.3%). In the hearts with isomerism, the VAC were concordant in one, DORV in one and single outlet in another (pulmonary atresia). The associated anomalies were ASD (67%), PAD (51%) and VSD (53%). CONCLUSIONS: The SSA was and effective and accurate tool to codify clear and completely the congenital heart disease. It is suggested that if the SSA is normal to describe this situation as a Normal Basic Heart Organization and then to describe the associated anomalies.

Tetralogia de Fallot: consideraçöes sobre aspectos morfológicos de uma entidade descrita com perfeiçäo / Tetralogy of Fallot. Considerations About Morphological Features of an Entity Well Described

PURPOSE--To analyze some morphological aspects of the tetralogy of Fallot which have been raising controversies in the literature, due to the different approach to the nomenclature of congenital heart defects by different authors regarding the definition of double outlet right ventricle and its concomitance with tetralogy. METHODS--We reviewed the original description of the anomaly, and also analyzed the morphology of 22 anatomical specimens, describing the degree of aortic overriding, the type of ventricular septal defect, the degree of sub-pulmonary stenosis, etc. RESULTS--Regarding the degree of aortic overriding, there was only one case where that vessel connected predominantly to the left ventricle. In the remaining hearts, the degree of overriding was between 50 per cent and 75 per cent in 10 cases and greater than 75 per cent in 11. The intensity of infundibular stenosis was evaluated as mild in half the available hearts, and the pulmonary valve was bicuspid in 11 cases. Right aortic arch was present in 40 per cent of the available hearts and the ventricular septal defect had muscular borders in just one case (5 per cent). CONCLUSION--We could conclude that if double outlet is only a type of ventricle-arterial connexion, and so, depending on the degree of overriding of the aorta over the trabecular septum, we may have the malformation coexisting with the tetralogy

Taussig-Bing complex--a pathologic study of eight cases.

Taussig-Bing Complex, an uncommon anomaly, was found in 8 out of 110 hearts of double outlet right ventricle (7.2%). The mean age at death was 23.5 months with maximum survival upto 10 years. There was only one case of dextrocardia; viscero-atrial and atrio-ventricular concordance was seen in all. Subpulmonary ventricular septal defect (VSD) was restrictive in two cases, in one of whom it was due to spontaneous closing process. Additional VSD was found in 2 cases. The great vessels showed side by side relationship with bilateral conii in 7 cases. In the remaining case there was L malposition with absent subpulmonary conus. Subpulmonary stenosis was noted in 3 cases. Tubular hypoplasia of aorta was seen in 2 cases with subaortic obstruction in one. Mitral valve abnormalities were present in 5 cases of which stenotic lesions were seen in 3. Coronary artery abnormalities were noted in 3 cases.