title: Syncope--an unusual presentation of ventricle dysfunction in a patient with Fontan circulation.
abstract: We report a case of recurrent syncope in association with moderate ventricular dysfunction and mild AV-valve regurgitation in an 18-year-old girl, 4 years after she underwent total cavopulmonary connection surgery. Cardiac catheterization revealed a transpulmonary gradient of 1-2 mmHg. During exercise, a dramatic fall in blood pressure and blood oxygenation was observed, paralleled by an increase in heart rate and central venous pressure. Although a slight increase in pulmonary vascular resistance could not be excluded, the reaction was interpreted in terms of an extremely low transpulmonary gradient in association with ventricular dysfunction. Five months after heart transplantation, the patient has been completely free from syncope. Fontan circulation usually involves a delicate haemodynamic situation which may necessitate haemodynamic re-evaluations, including dynamic measurements of the central venous pressure during exercise. Also a moderate ventricular dysfunction may result in compromised pulmonary circulation which in turn may lead to syncope during exercise as a result of insufficient systemic circulation.
authors.affiliation: The Department of Paediatrics, Division of Paediatric Cardiology, Queen Silvia's Children's Hospital, Göteborg, Sweden. daniel.holmgren@sahlgrenska.se
authors.forename: Daniel
authors.initials: D
authors.lastname: Holmgren
authors.forename: Staffan
authors.initials: S
authors.lastname: Redfors
authors.forename: Annika
authors.initials: A
authors.lastname: Rydberg
authors.forename: Håkan
authors.initials: H
authors.lastname: Wåhlander
mesh_terms: D000293:Adolescent; D001794:Blood Pressure; D002302:Cardiac Output; D002496:Central Venous Pressure; D004562:Electrocardiography; D005260:Female; D018729:Fontan Procedure; D006331:Heart Diseases / Q000150:complications* / Q000175:diagnosis* / Q000601:surgery; D006339:Heart Rate; D016027:Heart Transplantation; D006352:Heart Ventricles / Q000503:physiopathology; D006801:Humans; D005082:Physical Exertion; D013575:Syncope / Q000175:diagnosis* / Q000209:etiology*