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[A successful re-operation in emergency for a case of total anomalous pulmonary venous connection]
Insights
A child with total anomalous pulmonary venous connection experienced severe stenosis of an autologous pericardium channel, requiring revision with equine pericardium. This case highlights the importance of careful follow-up after congenital heart defect repair.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Congenital Heart Defects
Background:
- Total anomalous pulmonary venous connection (TAPVC) into the coronary sinus is a rare congenital heart defect.
- Intracardiac repair using autologous pericardium for the intra-atrial channel is a recognized surgical approach.
Observation:
- A five-month-old girl developed cyanotic attacks and respiratory distress two months post-TAPVC repair.
- Echocardiography revealed severe stenosis of the coronary sinus to left atrium channel, with pulmonary artery pressure exceeding systemic levels.
Findings:
- Reoperation showed the autologous pericardium channel was stenotic ('pinhole') due to significant thickening.
- Revision involved replacing the autologous pericardium with equine pericardium, leading to a satisfactory post-operative course.
Implications:
- Autologous pericardium may undergo significant thickening and stenosis, potentially related to hemodynamic load and growth.
- Meticulous long-term follow-up is crucial for patients undergoing TAPVC repair with autologous pericardium channels.
- Equine pericardium presents a viable alternative for intra-atrial channel reconstruction in complex TAPVC cases.
Abstract:
A five-month-old-girl underwent the intra cardiac repair for total anomalous pulmonary venous connection into coronary sinus. The intra-atrial channel from the coronary sinus to the atrial septal defect enlarged with cut-back method was built with the autologous pericardium. About two months after the first operation, she suffered cyanotic attacks associated with bradycardia and respiratory distress. in echocardiogram the channel from the coronary sinus to the left atrium showed severe stenosis. The pulmonary artery pressure elevated over systemic arterial pressure in cardiac catheterization. When she was referred and transferred to our hospital, 4 months after the first operation, her condition deteriorated and required reoperation in emergency for shock. In the second operation, under cardiopulmonary bypass, the channel from the coronary sinus to the left atrium became stenotic like pinhole with heavy thickening of autologous pericardium and atrial septum. Then, autologous pericardium was detached and cut-back of the atrial septum was added widely. New channel was established with the preserved equine pericardium. Although respiratory care extended for respiratory distress, post-operative course was satisfied. We considered that hemodynamic load seemed mainly to contribute to the thickening of the autologous pericardium, but the effect produced with the growth could not be negated. The meticulous follow-up should be recommended.