An Intriguing Case of a Twisted and Tilted Heart

Background: Diagnosing congenital cardiac malpositions can be complex, especially when multiple anomalies coexist. We report an unusual case of supero-inferior (upstairs-downstairs) ventricular arrangement with a criss-cross inlet, in a neonate with D-looped transposition of the great arteries (D-TGA), double outlet right ventricle (DORV), and hypoplastic aortic arch with coarctation of the isthmus. Case Presentation: The patient, diagnosed antenatally with DORV, D-TGA, and hypoplastic arch, was born in stable condition and started on prostaglandin E1 infusion. Postnatal echocardiography and CT angiography revealed a criss-cross heart (CCH) with supero-inferior ventricles. At 2-3 weeks, the patient underwent aortic arch repair and pulmonary artery (PA) banding. Subsequently, balloon atrial septostomy caused an intimal tear in the inferior vena cava (IVC), requiring IVC stenting. Over several months, PA band revision and selective left PA banding were necessary due to recurrent band loosening and stenosis. A Jatene arterial switch and ventricular septal defect (VSD) repair are planned after the infant reaches appropriate weight. Investigations: Imaging confirmed situs solitus with atrioventricular concordance and ventriculoarterial discordance. A large VSD without inlet valve straddling, double outlet right ventricle, and plethoric lung fields were noted. The criss-cross inlet was characterized by a misaligned right atrioventricular connection, with the right ventricle positioned superiorly and anteriorly to the left ventricle (upstairs-downstairs arrangement). Management and Outcome: Despite three sternotomies, including PA banding and multiple revisions, the child remains in high-dependency care, with plans for future surgery. Multidisciplinary team management was essential in navigating this challenging case. Conclusion: This case highlights the intricate nature of criss-cross heart with supero-inferior ventricular arrangement. Diagnosing these rare congenital anomalies requires a combination of imaging modalities, and management involves tailored surgical interventions to address the associated malformations. Early recognition and multidisciplinary collaboration are key to improving outcomes in such complex cases.