Lost in transition: a case report of very late palliation of a double outlet right ventricle

Children with congenital heart disease remain at risk of being lost to follow-up. They may present at a later stage with complications related to underlying condition that usually require in-depth phenotyping for optimal management. We describe an unusual case of a double outlet right ventricle (DORV) who presented three decades after initial intervention.

A 32-year-old female sought attention for worsening breathlessness. Available data suggested that she had double outlet right ventricle (DORV) and underwent pulmonary artery (PA) banding in infancy. On examination, she was frail, clubbed and cyanosed with oxygen saturation reduced to 75% at rest. Blood tests demonstrated elevated haemoglobin (208 g/L) and thrombocytopenia (70 *10⁹/L). Echocardiography was limited but demonstrated DORV with increased gradient across both outflow tracts. Cardiac magnetic resonance (CMR) imaging clearly demonstrated DORV with doubly committed interventricular communication, subvalvular aortic valve stenosis and supravalvular pulmonary stenosis due to a previous PA band. CMR was also remarkable for demonstrating severely impaired left ventricle and difficulty achieving optimal contrast between myocardium and blood pool on late enhancement images. She underwent relief of subaortic stenosis with immediate improvement in left ventricular (LV) function and PA plasty with application of a new PA band.

Lost to follow-up congenital patients present a particular challenge due to complications of chronic unusual physiology. This requires careful assessment by a multidisciplinary team to define optimal management strategy.