No abstract

Topsy-turvy heart is a rare congenital anomaly; it has a specific characteristic of cardiac malrotation and superior-inferior arrangement of right/left ventricles. A five-day-old patient was diagnosed antenatally with hypoplastic left heart and right hydronephrosis and had been admitted to the hospital with symptoms of respiratory distress. The postnatal imaging studies revealed an abnormal ventricular arrangement with a large aortopulmonary window, ventricular septal defect (VSD), and the upside-down orientation resulting in the posterior-inferior displacement of the common arterial confluence and, in turn, the left main bronchial stenosis. For the first time, the diagnosis of a topsy-turvy heart with the intracardiac anomaly (VSD) was confirmed.

Background The trans-catheter closure of atrial septal defect (ASD) usually has a rapid impact on biventricular remodelling and functions. Whether the transcatheter closure of ASD at early childhood or at adulthood age would affect the improvement in biventricular dimensions and functions remains an area of active research. Results This prospective observational study enrolled 70 subjects (50 ASD cases and 20 control subjects). Tissue Doppler imaging (TDI) and strain (S) were performed for the control group and ASD patients at baseline and at 24 h and 1 month after ASD device closure. The total ASD group was subdivided into two subgroups: group-1—children and adolescent with ASD, who underwent transcatheter closure at age ≤ 19 years; group-2—adult who underwent ASD device closure at age > 19 years old. The right and left ventricular global longitudinal systolic strain (RV/LV-GLS) and RV free wall longitudinal strain (RV free wall LS) showed a significant decline after 24 h of device closure (RVGLS-P = 0.001, LVGLS-P = 0.048, RV free wall LS-P < 0.001). However, after a 1-month follow-up, the LVGLS increased in comparison with 24 h changes after device closure (P = 0.038). The baseline mean value of RV free wall LS of G2 was significantly lower than G1 value (P < 0.001). There was no statistically significant difference between the 2 age subgroups regarding biventricular GLS and RV free wall LS changes after device closure. The changes in LV diastolic function immediately and after 1 month of device closure showed a statistically significant change in e′ and its delta change value in group-2 in comparison with its baseline values and to group-1 delta changes (P = 0.002, P = 0.011, P = 0.019, respectively). Conclusion The ASD transcatheter closure reduced biventricular global and RV free wall longitudinal systolic strain within 1 day of intervention and was associated with a short-term improvement in the LV-GLSS after a 1-month duration. The progressive increase in LV preload results in its strain growth and reduction in diastolic function after transcatheter ASD closure. The older age at the time of ASD device closure was associated with a significant decrease in the RV free wall LS and septal e′ velocity towards abnormality.