Concordant criss cross AV connection with ventricular septal defect (VSD)

Criss-cross heart (CCH) is a rare congenital anomaly of cardiac rotation resulting in crossing of ventricular inlets and drainage of the atria into contra-laterally located ventricles.¹ The atrio-ventricular (AV) and ventriculo-arterial (VA) connections can be concordant or discordant.² There can be either side-by-side or superior-inferior ventricular arrangement (superior-inferior ventricles, SIV); Lev and Rowlatt, in 1961, were the first to describe an unusual arrangement of ventricular inlets with the right ventricle abnormally positioned superior the left ventricle.³ In 1974 Anderson et al. used the term “criss-cross heart” for the first time.¹ The complex and distorted cardiac anatomy seen in CCH makes accurate diagnosis difficult. Because of its common association with diverse cardiac anomalies, CCH requires early diagnosis to provide timely operative management and achieve a good functional outcome. Transthoracic echocardiography is the preferred diagnostic modality, though cardiac magnetic resonance imaging can provide valuable additional information, especially in challenging cases. Cardiac catheterisation and angiography have little value in the management, except in patients with features of pulmonary hypertension and in indeterminate cases.⁴ The paucity of data on CCH from India provided the impetus to study its echocardiographic features in Indian patients.