Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2026;29:24-31. doi: 10.1053/j.pcsu.2026.02.004. Epub 2026 Mar 2.

ABSTRACT

The criss-cross heart with double outlet right ventricle (DORV) and combined atrioventricular (AV) and ventriculoarterial (VA) discordance presents one of the most formidable challenges in congenital cardiac surgery. The unique ventricular topology and complex inflow-outflow relationships often preclude standard approaches to biventricular repair, and single-ventricle palliation is frequently selected. However, in appropriately selected patients, anatomical repair through a double switch procedure may offer superior long-term outcomes by preserving systemic ventricular function. This report describes a surgical strategy for addressing a criss-cross heart with DORV and AV/VA discordance using a modified Senning procedure as part of a double switch operation. Preoperative imaging-including transthoracic echocardiography, computed tomography angiography, and cardiac catheterization-is critical for assessing operability. Particular attention must be paid to pulmonary vascular resistance, Qp/Qs, morphologic left ventricular end-diastolic pressure, and coronary anatomy. The surgical technique involves ventricular septal defect baffle closure to direct left ventricular output to the pulmonary artery, an arterial switch operation, and a modified Senning atrial switch. The Senning component is performed using a pedicled autologous pericardial baffle augmented with a large bovine pericardial patch to prevent pulmonary venous pathway narrowing, a frequent concern in patients with criss-cross anatomy. This approach allows for biventricular repair even in cases with severe atrial and ventricular malalignment. The technique aims to achieve unobstructed systemic and pulmonary venous pathways, minimize arrhythmia risk, and establish long-term left ventricular systemic circulation. Careful patient selection and meticulous surgical execution are essential for optimizing outcomes in this complex subgroup of congenital heart disease.

PMID:42091300 | DOI:10.1053/j.pcsu.2026.02.004