Kun Zhang, Shuguang Tao, Wei Gao, Jiangrong Gu, Linlin Wen, Yun Fan
Hebei Children’s Hospital.
China
Frontiers in Pediatrics
Front Pediatr 2025; 13:
DOI: 10.3389/fped.2025.1590620
Abstract
Common pulmonary vein atresia (CPVA) is a rare congenital heart disease characterized by the absence of functional connectivity between the pulmonary vein and any other heart cavity or systemic venous structure. A 13-h-old newborn (G3P3) was admitted to the department of pediatrics of a local maternity hospital and given tracheal intubation ventilator for assisted breathing due to systemic cyanosis, respiratory distress, and poor response 4 h after birth. He was transferred to Handan Maternal and Child Health Hospital 7 h after birth. After entering the department of neonatology, the child was considered to have anomalous pulmonary venous drainage (subcardiac type) after undergoing color Doppler examination. There was no obvious effect on the treatment, such as liquid replenishment and acid correction, so he was transferred to our hospital. After admission, respiratory failure complicated with heart failure, metabolic acidosis, and hyperlactatemia were initially diagnosed. After two-dimensional echocardiography, emergency surgery was decided after consideration of CPVA. Complete pulmonary venous malformation drainage and cardiopulmonary bypass-assisted repair were performed 9 h after admission. The patient was successfully discharged from the ICU on the 30th day after surgery, but on the 52nd day after surgery, pulmonary vein obstruction was found again. After emergency cardiopulmonary bypass-assisted repair, the pulmonary vein obstruction was corrected, but the child had multiorgan failure (MOF), coagulation function was abnormal, and vital signs were still difficult to maintain with cardiopulmonary bypass assistance after the second operation, and the family gave up treatment. Although we have made significant progress in understanding and processing CPVA, the early diagnosis, surgical treatment, and prognosis remain challenging. The changes in examination data we found in the evolution of the patient’s condition during this period and the corresponding changes required constant alertness to the possibility of various problems, such as the occurrence of pulmonary vein obstruction and occlusion, and timely targeted treatment strategies. The treatment experience and lessons during this period can be referred to by everyone, hoping to provide some help in improving the treatment of CPVA patients.
Category
Absence or Atresia of Normal Pulmonary Venous Connections
Surgical Interventions to Prevent or Limit Disease Associated with Repair of Anomalous Pulmonary Venous Connections or Heart Transplantation
Year of Publication: 2025
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