Letter to the Editor, regarding Rakha S, Hammad A, Elmarsafawy H, Korkor MS, et al. (2023). A deeper look into the functions of right ventricle using three-dimensional echocardiography: the forgotten ventricle in children with systemic lupus erythematosus. Eur J Pediatr. 182:2807–2819

Excerpt

The interesting paper by Rakha et al. about three-dimensional (3-D) echocardiography in children with systemic lupus erythematosus correlates with equivalent observations, made in cases of drepanocytosis, and mainly focused on right ventricle (RV) dysfunction [1]. In our experience, consisting of a cohort of 46 children between the ages of 3 and 21 years with drepanocytosis, 3-D echocardiography revealed primary RV dysfunction, with a severity proportional to that of the hematological disease and, above all, to the resulting pulmonary hypertension. This condition is induced by progressive microvascular thromboses, inadequate release of nitric oxide, and pulmonary microvessel vasoconstriction secondary to hypoxia. Consequently, the RV end-diastolic and end-systolic volumes increase, often associated with a progressively greater tricuspid annular plane systolic excursion, and, importantly, RV free wall and global longitudinal strain decrease. Moreover, the myocardial fibers, given the recurrent hypoxic crises, hyperkynetic circulation and iron tissues deposition, typical of drepanocytosis, undergo an accelerated thickening and lengthening, which decrease or cancel the functional benefits of the Frank-Starling law [2]. This chain of negative events affects also the left ventricle (LV), partly wrapped by the same bundles of myocardial fibers of the RV and sometimes damaged also by the abnormal displacement of the interventricular septum, induced by the high hypertension present inside the right cardiac chambers [3]. It results in a corresponding LV enlargement, eccentric parietal hypertrophy, increased end-systolic and end-diastolic volumes, and decreased global and peak systolic wall stresses. The final result of this pathology leads to an impending heart failure, where the primary RV function damage, correlated with pulmonary hypertension, plays a triggering role. From a speculative point of view, the cardiac pathology connected with drepanocytosis provides a typical model to study the RV impact on LV dysfunction. …