title: Extracorporeal membrane oxygenation as a bridge to diagnosis in a 20-month old girl with pulmonary hypertension and right ventricular failure.
abstract: A 20-month old girl with severe pulmonary hypertension and cardiomegaly was admitted to the paediatric intensive care unit with right ventricular failure of unknown origin. Only after decompression of the heart chambers under extracorporeal membrane oxygenation (ECMO), did the pathognomonic membrane of Cor triatriatum become visible on echocardiography. The patient underwent successful surgical correction and subsequently cardiac function recovered completely. Cor triatriatum remains a rare congenital cardiac disorder with a variable presentation, often including recurrent respiratory infections before right-sided heart failure occurs. This case illustrates that ECMO can serve not only as a bridge to diagnosis, but can also facilitate correct diagnosis. Given the excellent outcome after surgical treatment, it is crucial that cardiologists rule out the possibility of cor triatriatum when assessing a child with unexplained pulmonary hypertension.
authors.affiliation: Paediatric Critical Care Research Group, Pediatric Intensive Care Unit, Mater Children's Hospital, Brisbane, Australia. luregn.schlapbach@mater.org.au
authors.forename: Luregn J
authors.initials: LJ
authors.lastname: Schlapbach
authors.forename: Martin
authors.initials: M
authors.lastname: Grips
authors.forename: Rob
authors.initials: R
authors.lastname: Justo
authors.forename: Tom
authors.initials: T
authors.lastname: Karl
mesh_terms: D006348:Cardiac Surgical Procedures; D006332:Cardiomegaly / Q000209:etiology; D003310:Cor Triatriatum / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D018618:Echocardiography, Doppler, Color; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D007223:Infant; D011237:Predictive Value of Tests; D012720:Severity of Illness Index; D016896:Treatment Outcome; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D016278:Ventricular Function, Right