JACC. Case reports · 2026
Clinical Characteristics of Patients With Fontan Circulation and Primary Ciliary Dyskinesia: A Case Series From the SV-ONE Registry.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Aldawsari Khalifah A, Bucholz Emily, Saba Thomas G, Schidlow David, Chen Sharon, Spurgin Stephen B, Khalid Omar, Jaggers James, Chatfield Kathryn C
Аннотация
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare genetic disorder associated with chronic respiratory disease. In single-ventricle palliation, pulmonary blood flow is driven in part by inspiratory effort and is critically dependent on low pulmonary vascular resistance. The coexistence of PCD and Fontan physiology is uncommon, and outcomes remain poorly defined.
METHODS: We performed a retrospective case series of patients with Fontan physiology and reported PCD within the multi-institutional Single-Ventricle Outcomes Network registry.
RESULTS: Among 1,535 patients with Fontan, only 5 had reported PCD. Median age at Fontan completion was 3 years, with a median follow-up of 7 years. Following Fontan, 2 patients developed chylous pleural effusions. No patient developed plastic bronchitis or protein-losing enteropathy.
CONCLUSIONS: In this case series, Fontan completion was only achieved in a small cohort of patients with reported PCD. These findings emphasize the need for multidisciplinary collaboration to appropriately describe, address, and manage multiorgan comorbidities.
METHODS: We performed a retrospective case series of patients with Fontan physiology and reported PCD within the multi-institutional Single-Ventricle Outcomes Network registry.
RESULTS: Among 1,535 patients with Fontan, only 5 had reported PCD. Median age at Fontan completion was 3 years, with a median follow-up of 7 years. Following Fontan, 2 patients developed chylous pleural effusions. No patient developed plastic bronchitis or protein-losing enteropathy.
CONCLUSIONS: In this case series, Fontan completion was only achieved in a small cohort of patients with reported PCD. These findings emphasize the need for multidisciplinary collaboration to appropriately describe, address, and manage multiorgan comorbidities.