Abnormalities of right ventricular long axis function after atrial repair of transposition of the great arteries

Abstract

Background-While volume derived global indices of right ventricle (RV) function are frequently abnormal after the Mustard procedure, the mechanism for these abnormalities is poorly understood. RV muscle fibres are predominantly arranged longitudinally and thus indices derived in the long axis may better describe RV function. Methods-20 survivors of the Mustard operation were studied (age 7.8-37.3 years, median 14.2 years). Long axis recordings from the apical four chamber view were obtained with the M mode cursor positioned through the lateral angle of the tricuspid valve annulus. M mode traces were recorded on paper and later digitised to derive total atrioventricular ring excursion, peak lengthening rate, and peak shortening rate. These data were averaged and compared with control data for the normal RV and left ventricle (LV). Results-RV total atrioventricular ring excursion was lower than that for the RV (p < 0.0001) or LV (p < 0.005) of controls. Peak lengthening rate was lower than the normal RV (p < 0.0001) and LV (p < 0.0001) rates. Furthermore, peak shortening rate was less than that of normal RV (p < 0.0001) and normal LV (p < 0.005) controls. Conclusion-Systemic RV long axis function is notably reduced compared with that of either the normal subpulmonary RV or the systemic LV. This presumably reflects the response of the predominantly longitudinally arranged myocardial fibres to increased afterload. However, such measurements may provide a more sensitive marker for progressive changes in global function during long term follow up.

Comprehensive evaluation of right ventricular function in children with different anatomical subtypes of hypoplastic left heart syndrome after Fontan surgery

“…Though we found no difference between groups, TAPSE values in our collective of HLHS patients in general were lower (mean 0.9 cm) than the described normal values for the same age group for the subpulmonary RV (mean 1.9 cm). In 20 patients with atrial switch operation for transposition of the great arteries, Derrick et al found the mean TAPSE of the systemic RV to be 1.2 cm [18] which is similar to that of our cohort. The lower values when compared to subpulmonary RVs could be explained by chronic adjustment of the systemic RV to unnatural loading conditions.…”

Incidence and results of reoperations following the Senning operation: 27 years of follow-up in 314 patients at a single center☆

“…The long-term outcome in terms of functional status and quality of life was reported to be reasonably good in the majority of the patients [4,12,13]. However, there is significant morbidity and mortality associated with specific complications following atrial switch operations, such as systemic ventricular dysfunction [3,14], and baffle complications [3,10]. Reoperations for those late sequelae are demanding, and mortality rate of up to 37% associated to reoperations have been reported [10].…”

Carvedilol for Children and Adolescents With Heart Failure

“…Evidence is increasing that systemic ventricles that are not of left ventricle morphology respond differently to medical interventions. [26][27][28][29] No significant benefit could be detected in young adults with systemic right ventricles who were treated with angiotensinconverting enzyme inhibitors or angiotensin receptor blockers, 30,31 leading to speculation that vasodilators might be poorly tolerated in a circulation with intrinsically limited ventricular filling. 32 The high level of placebo response we observed, as well as the interaction between systemic ventricular anatomy and the primary outcome, are important considerations for the design of future clinical trials.…”