Tricuspid atresia with congenital advanced AV nodal block: An extremely rare association

Abstract

Tricuspid atresia (TA) is a cyanotic congenital heart disease (CHD) with incidence of 0.06/1000 live birth with a prevalence rate of 1-3% of all CHDs. TA can be associated with either normally related (type I) or transposed great arteries (TGA) (type II and III) and can be associated with/without ventricular septal defect (VSD) ± pulmonary stenosis (PS). An atrial level shunt through atrial septal defect/ patent foramen ovale (ASD/PFO) is essential for life. Although advanced atrioventricular (AV) block including complete heart block is seen in few CHDs like with congenitally corrected TGA (CC-TGA), AV discordance (isolated ventricular inversion), endocardial cushion defect (ECD), and left isomerism (polysplenia), it is not described in TA. Here, we report a neonate having TA with transposition with VSD, no PS (type IIc) who also had advanced heart block since intrauterine life. His mother was worked for autoimmune disease, but did not reveal any evidence of such.