An Unusual Presentation of Double-Outlet Right Atrium.

We describe an unusual example of double-outlet right atrium with separate atrioventricular junctions. The straddling and overriding tricuspid valve had two orifices, and the mitral valve was morphologically normal. An appropriate understanding of the morphology of the atrioventricular junctions, the valves, and the subvalvar apparatus, along with the location of the atrioventricular conduction axis, allowed for successful biventricular repair.

Experience with surgical correction of double outlet both ventricles.

Double outlet both ventricles is a rare abnormal ventriculo-arterial malformation in which both great arterial trunks are committed to both the ventricles, albeit now being recognized with increasing frequency. Patients with the lesion present with a spectrum of clinical manifestations. The size and location of the interventricular communication dictate the feasibility of biventricular repair.

Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.

So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect.

Effect of sex, age and body measurements on heart weight, atrial, ventricular, valvular and sub-epicardial fat measurements of the normal heart.

Aims: Descriptive morphological studies of the normal heart are lacking. Previous autopsy studies have focused mainly on heart weight. We characterize the normal heart by providing normal dimensions of the atria, ventricles, valves and sub-epicardial fat, comparing the findings in terms of sex, age and body measurements.

Functionally univentricular heart with systemic venous anomalies: surgical palliation and pulmonary arterial reconstruction with a roll of left atrial appendage.

In this report, we describe a 3-year-old patient with a functionally univentricular heart (UVH), who had a combination of double outlet right ventricle (DORV) along with an unrouteable interventricular communication (VSD), severe infundibular and pulmonary valvar stenosis, and severe left pulmonary artery (LPA) ostial stenosis. This patient also had an interrupted inferior caval vein (IVC) with bilateral superior caval veins (SVC). We were able to undertake a successful Kawashima procedure with interruption of the antegrade pulmonary blood flow, reconstructing the LPA using a pedicled roll of the left atrial appendage (LAA).