Article Text
Abstract
Aims Total Anomalous Pulmonary Venous Connexion (TAPVC) is a rare congenital heart disease. Signs and symptoms of TAPVC can sometimes mimic a primary pulmonary pathology in infancy such as bronchiolitis. Diagnosing TAPVC during the winter period can be challenging.
Methods We present the case of a 3-week old infant presenting with failure to thrive and respiratory symptoms suggesting bronchiolitis, but eventually was found to have an infracardiac TAPVC.
Results A 3-week old infant, presented to hospital due to failure to regain her birth weight. At presentation she was hypoxic with symptoms and signs of a bronchiolitis (tachypnoea, inspiratory crackles, normal heart size and pulmonary plethora on chest x-ray) (Figure 1). Following a profound desaturation episode, she was intubated and ventilated and admitted to Paediatric Intensive Care Unit (PICU). In PICU, cardiovascular examination was normal, except for a 4cm hepatomegaly. She required moderate ventilator support with oxygen requirements between 35 and 60%, all pointing towards a bronchiolitic type of illness. However, she displayed an unusually high number of bradycardic and hypoxic episodes with minimal stimulation. A hyperoxia test yielded a PaO2 31kPa reassuring clinicians that there was no intracardiac right to left shunt. 3 days after admission, she deteriorated clinically requiring higher ventilator support and had a worsening radiological picture (Figure 2). Virology and inflammatory markers did not reveal an infective cause. A 12-lead ECG showed right ventricular hypertrophy. An echocardiogram was done and showed an infracardiac TAPVC with no obvious obstruction and an Atrial Septal Defect. She was transferred to a Tertiary Cardiac centre, where she underwent a TAPVC repair 5 days after her initial presentation to hospital.
Conclusion Diagnosing TAPVC can be quite challenging for health professionals as a number of clinical signs and symptoms are similar to the more common childhood respiratory infections such as bronchiolitis. Every child presenting with symptoms of respiratory illness should have a thorough history and examination to rule out an underlying cardiac pathology. It is also important to recognise that a hyperoxia test suggestive of a lung pathology and oxygen saturations that can rise up to 100%, do not exclude a diagnosis of TAPVC.