The patient is a twenty-three-month-old boy with total anomalous pulmonary venous return (TAPVR), ventricular septal defect (VSD), and secundum atrial septal defect (ASD II) initially treated with a TAPVR correction with an anastomosis of the confluence to the LA, VSD closure, and fenestrated ASD II closure in the first month of life. The patient also had suspected VACTERL association and primary cilial dyskinesia.
After the primary surgery, the patient developed pulmonary artery hypertension (PAH) and received a balloon dilation of the fenestrated ASD patch to alleviate right ventricular pressure. A stenosis of the ostial collector was diagnosed, and the patient received a patch reconstruction of the left atrial appendage. The patient was then presented at the authors’ department after several infectious respiratory decompensations aggravated by severe PAH.
A CT angiography revealed stenosis of all four pulmonary veins as well as the confluence and bilateral pneumonic infiltrates. The patient had to be intubated and treated with antibiotics for seven days until he was deemed a candidate for surgery. The patient then underwent redo pulmonary vein stenosis correction with resection of the confluence, resection of all four stenotic ostia, a modified atriopericardial anastomosis, and a fenestrated ASD closure.
Upon inspection, the anastomosis of the confluence, or collector to the left atrium, was without stenosis but seemed to be small. The individual pulmonary veins were stenotic and there was excess fibrous tissue in between the ostia. The anastomosis itself was displaced leftwards.
To begin, the confluence and the stenotic pulmonary veins were excised from the left atrium and the pulmonary veins were incised until the segment veins in intermittent deep hypothermic arrest. When cardiac bypass was reestablished, all pulmonary veins showed satisfactory return of venous blood.
An atriopericardial anastomosis was fashioned starting at the deepest leftward portion of the left atrium and keeping careful distance to the pulmonary venous orifices. The atrial septum was reconstructed with a fenestrated Gortex patch. The patch was shifted to the righthand side to enable free flow from the right pulmonary veins.
The postoperative course was hemodynamically uneventful. Respiratory weaning, however, was complicated and the patient was transferred to another acute care clinic which specializes in prolonged respiratory weaning in small children. Respiratory weaning was successful after several weeks and the patient is scheduled for routine coronary angiography in the authors’ department in the next month.
References
- Viola N, Caldarone CA: Surgical Repair of Post-Repair Pulmonary Vein Stenosis Using “Sutureless“ Techniques. Operative Techniques in Thoracic and Cardiovascular Surgery 2011, 16(2):112-121.
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