Pulmonary Comorbidities in Children and Young Adults with Fontan Circulation: The Limitations of General Cardiology Clinics Compared with Dedicated Fontan Clinics.

Pathogenesis of pulmonary comorbidities post-Fontan surgery is multifactorial, including long-term, non-pulsatile blood flow, history of multiple sternotomies, abnormal airway lymphatics, associated scoliosis, and recurrent laryngeal nerve or vagal nerve injury as complications of cardiac surgery. A retrospective, cross-sectional analysis of 128 patients under the age of 30 years with a single ventricle status post-Fontan procedure. These patients were evaluated in our multidisciplinary Fontan clinic. Variables relating to demographics, pulmonary diseases, pulmonary function testing measurements, and imaging are described. Outcomes relating to elevated central venous pressure (CVP), pulmonary vascular resistance and transpulmonary gradient (TPG) are also portrayed. Restrictive lung disease was found in 41% of our cohort. Most patients with asthma were previously diagnosed; however, 39% of them required a controller medication change in the clinic. Most of the patients with a confirmed diagnosis of obstructive sleep apnea were screened for sleep disordered breathing and had a polysomnography done for the first time in the Fontan clinic. Reduced forced vital capacity (FVC) z-score < -1.645 was found in 67% of patients with elevated TPG > 6 mmHg (r = -0.143; p = .216.), 50% in patients with elevated pulmonary vascular resistance > 2 Wood units (r = -.137; p = .238), and 35% with elevated mean Fontan pressure > 15 mmHg (r = -.085; p = .464). Our Findings strongly emphasize the pivotal role of dedicated multidisciplinary Fontan clinics with pulmonary involvement in post Fontan care compared to general cardiology clinics given the high prevalence of pulmonary comorbidities post Fontan surgery.