Diagnosis of secondary pulmonary lymphangiectasia in congenital heart disease: a novel role for chest ultrasound and prognostic implications

• Published in: Pediatric radiology Vol. 47; no. 11; pp. 1441 - 1451
• Main Authors: Lam, Christopher Z., Bhamare, Tanmay Anant, Gazzaz, Tamadhir, Manson, David, Humpl, Tilman, Seed, Mike
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01-10-2017; Springer Nature B.V
• Subjects: Biopsy; Cardiovascular disease; Chest; Children; Complications; Confidence intervals; Congenital diseases; Coronary artery disease; Diagnosis; Diagnostic systems; Female; Geriatrics; Heart; Heart Defects, Congenital - complications; Heart Defects, Congenital - surgery; Heart diseases; Humans; Imaging; Infant; Infant, Newborn; Likelihood ratio; Lung Diseases - congenital; Lung Diseases - diagnostic imaging; Lung Diseases - surgery; Lungs; Lymphangiectasis - congenital; Lymphangiectasis - diagnostic imaging; Lymphangiectasis - surgery; Lymphatic system; Male; Medical diagnosis; Medicine; Medicine & Public Health; Neuroradiology; Nuclear Medicine; Oncology; Original Article; Patients; Pediatrics; Prognosis; Prospective Studies; Pulmonary Veins - abnormalities; Radiology; Sensitivity and Specificity; Statistical analysis; Ultrasonic imaging; Ultrasonography - methods; Ultrasound; Pulmonary lymphangiectasia; Pulmonary venous obstruction; Chest; Congenital heart disease; Children; Ultrasound
• ISSN: 0301-0449; 1432-1998
• DOI: 10.1007/s00247-017-3892-z

Background
Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction.
Objectives
We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia.
Materials and methods
Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study. Thirteen children had pulmonary venous obstruction (62% male; median age: 17 days old, range: 1–430 days old) and 13 children did not have obstruction (62% male; median age: 72 days old, range: 4–333 days old). US features of secondary pulmonary lymphangiectasia were documented and diagnostic performance was determined. Clinical course of patients with secondary pulmonary lymphangiectasia was reviewed.
Results
Eleven of 13 (84.6%) patients in the obstructed group had a clinical and/or biopsy diagnosis of secondary pulmonary lymphangiectasia. Statistically significant chest US criteria for diagnosis were presence of irregular lung surface (likelihood ratio [LR] 6.8, 95% confidence interval [CI] 1.9–25.1), subpleural cystic appearing structures (LR 3.6, 95% CI 1.2–10.7), and combination of subpleural cystic appearing structures and surface irregularity together (LR 10.9, 95% CI 1.6–75.0). Seven of 11 (63.6%) patients with secondary pulmonary lymphangiectasia died during follow-up, the majority due to cardiopulmonary failure or complications.
Conclusion
Chest US is an accurate and reproducible bedside method for diagnosing secondary pulmonary lymphangiectasia in patients with pulmonary venous obstruction. These patients may have worse prognoses.