Purpose or Case Report: Percutaneous lymphatic interventions (PLI) target the pulmonary lymphatic abnormalities seen in patients with plastic bronchitis (PB). Existing data in patients with congenital heart disease(CHD)indicates poor transplant-free survival after the diagnosis of PB. We describe various techniques of PLI and characterize intermediate-term survival and complications after PLI.
Methods & Materials: We reviewed 70 patients with CHD and PB that underwent PLI at our institution between 8/2013 and 8/2019. We collected imaging, procedural, intermediate-term clinical outcome, and complication data.
Results: In the review of 70 patients, the median age was 8.8 years and 60 patients (86%) had Fontan physiology. Most patients had daily or weekly cast production prior to PLI for PB. Abnormal lymphatic perfusion was seen in both lungs in 62 patients (88%), and 50 patients (71%) underwent complete thoracic duct embolization, 16 (22%) patients had selective branch embolisation and 4 patients (6%) had direct percutaneous transthoracic branch embolisation. After index procedure, 11 patients (15.7%) required repeat PLI. Transplant-free survival was 89% at 19 months and 82% at 30 months. Median length of hospital stay for PLI was 6 days. Common complications included transient abdominal pain (77%), hypotension requiring IV fluids or pressor support (51%), and systemic inflammatory response syndrome (21%). Major complications were rare; 7 patients were reintubated, 4 had gastrointestinal bleeding with one requiring intervention, 3 had cardiac arrest, and 2 had embolic stroke. Two patients died prior to discharge (1acute cardiac arrest after an aspiration and 1multi-organ failure). Intermediate follow-up data were available on 63 patients (90%) from chart review or telephone survey. 81.0% of patients had no casting at follow-up. Casting frequency (p<0.001) and respiratory support (p=0.002) decreased post-intervention. Most reported improvement in their child’s overall health, quality of life, and physical activity.
Conclusions: PLI is an effective treatment for PB in CHD patients. The majority of patients had cessation of casting and favorable outcomes which demonstrates PLI’s feasibility and safety.