Anatomie et physiologie : recherches en cours
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Quantitative Anatomy of Taussig-Bing Anomaly

Leo A Bockeria, Osman A Makhachev, Margarita V Gorgeeva, Marina S Panova, Tatiana Yu Philippkina, Titalav Kh Khiriev and Sergey B Zaets

Background: Taussig-Bing is one of the variants of the double outlet right ventricle. The characteristic feature of the Taussig-Bing anomaly (TBA) that makes it different from partial transposition of the great arteries is sub pulmonary ventricular septal defect. Morphometric studies of hearts with TBA are limited and in the majority of cases do not contain quantitative assessment of intracardiac structures. The aim of this study was to measure intracardiac structures (e.g. cardiac mass, diameter of valves, thickness of the myocardium), to assess their deviation from the individual norm, as well to determine how these parameters are influenced by anatomical variant of the lesion, anthropometric variability or pulmonary hypertension.
Methods: Thirty pediatric hearts with TBA underwent morphometric assessment. The median age at the moment of death was 0.4 years. All patients had pulmonary hypertension of grade I-II (n=24) or grade III-IV (n=6) per Heath- Edwards. Morphometric assessment included the measurement of the following parameters: cardiac mass, diameter of the fibrous annulus of all cardiac valves (mitral, tricuspid, aortic, and pulmonary), myocardial thickness of the both right and left ventricle. Results of valvular morphometry were compared with individual normative data presented by Schulz DM and Giordano DA, and Z-score index was calculated.
Results: Cardiac mass, right ventricular myocardial thickness and the diameter of pulmonary valve significantly exceeded normal values in TBA hearts. Cardiac mass median Z-score in TBA hearts with pulmonary hypertension of III-IV grade was significantly higher if compared to pulmonary hypertension of I-II grade. Diameters of tricuspid and pulmonary valve were significantly bigger in TBA hearts without aortic obstruction.
Conclusions: There is an increase in cardiac mass, right ventricular myocardial thickness as well as pulmonary valve diameter in TBA hearts if compared to age adjusted normal values. The degree of pulmonary hypertension may contribute to the increase in cardiac mass.