Pulmonary Screening in Subjects after the Fontan Procedure

• Published in: The Journal of pediatrics Vol. 199; pp. 140 - 143
• Main Authors: Liptzin, Deborah R., Di Maria, Michael V., Younoszai, Adel, Narkewicz, Michael R., Kelly, Sarah L., Wolfe, Kelly R., Veress, Livia A.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.08.2018
• Subjects: altitude; congenital; executive function; heart defects; hypoplastic left heart syndrome; neuropsychology; sleep apnea syndromes; sternotomy; altitude; sternotomy; heart defects; hypoplastic left heart syndrome; neuropsychology; sleep apnea syndromes; FEV1; TLC; congenital; executive function
• ISSN: 0022-3476; 1097-6833; 1097-6833
• DOI: 10.1016/j.jpeds.2018.03.050

To review the pulmonary findings of the first 51 patients who presented to our interdisciplinary single-ventricle clinic after undergoing the Fontan procedure. We performed an Institutional Review Board–approved retrospective review of 51 patients evaluated following the Fontan procedure. Evaluation included history, physical examination, pulmonary function testing, and 6-minute walk. Descriptive statistics were used to describe the population and testing data. Sixty-one percent of the patients had a pulmonary concern raised during the visit. Three patients had plastic bronchitis. Abnormal lung function testing was present in 46% of patients. Two-thirds (66%) of the patients had significant desaturation during the 6-minute walk test. Patients who underwent a fenestrated Fontan procedure and those who underwent unfenestrated Fontan were compared in terms of saturation and 6-minute walk test results. Sleep concerns were present in 45% of the patients. Pulmonary morbidities are common in patients after Fontan surgery and include plastic bronchitis, abnormal lung function, desaturations with walking, and sleep concerns. Abnormal lung function and obstructive sleep apnea may stress the Fontan circuit and may have implications for cognitive and emotional functioning. A pulmonologist involved in the care of patients after Fontan surgery can assist in screening for comorbidities and recommend interventions.