Yoshiko Nawata, Daisuke Toyomura, Seigo Okada, Yasuo Suzuki, Narumi Honda-Nakada, Yuji Ohnishi, Yuichiro Sugitani, Naoki Kawaguchi, Rui Tokitaka-Okada, Naoki Masaki, Eiji Ikeda, Shunji Hasegawa
Yamaguchi University Graduate School of Medicine. Fukuoka Children’s Hospital. Tohoku University Graduate School of Medicine.
Japan
International Journal of Cardiology
Int J Cardiol 2024; 65: 363-366
DOI: 10.1536/ihj.23-232
Abstract
Congenital pulmonary lymphangiectasia (CPL) is associated with fetal pulmonary venous obstructive physiology. The precise morbidity of CPL is unknown as CPL is generally fatal in neonates. Here, we report an infant with secondary CPL in total anomalous pulmonary venous connection (TAPVC). He developed severe pulmonary hypertension (PH) after corrective surgery for TAPVC. However, cardiac catheterization showed mild left pulmonary venous obstruction (PVO), which was deemed unnecessary for re-intervention. He died at 11 months-old due to an exacerbation of PH. Autopsy revealed medial hypertrophy of the pulmonary arteries, mild left PVO, and marked dilatation and proliferation of the pulmonary lymphatics which might have been involved in the PH, although CPL was not conclusively identified based on the previous biopsy findings. We should be aware of the possibility of CPL in addition to postoperative PVO when encountering patients with fetal pulmonary venous obstructive physiology. Furthermore, a cautious approach to the interpretation of lung biopsy results is warranted.
Category
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Symptoms and Quality of Life Associated with Pulmonary Venous Obstruction
Pulmonary Venous Pathology
Year of Publication: 2024
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes