A Note From Carl L. Backer M.D.:

We have used the sutureless pulmonary vein stenosis repair technique in five patients at Children’s Memorial Hospital.  Four of these were reoperations after neonatal repair of total anomalous pulmonary venous connection, one was for primary congenital pulmonary vein stenosis.  I have not used this as a primary procedure in patients with total anomalous pulmonary venous connection.  The procedure was initially successful in the child with congenital pulmonary vein stenosis but the child developed relentless progressive stenosis of the pulmonary veins and died of pulmonary hypertension 3 months postoperatively.

I have used transesophageal echocardiography in all cases both to assess the preoperative pulmonary vein stenosis and to assess the adequacy of the postoperative repair.  I have also measured the pulmonary artery pressure directly following the procedure.  This has been helpful in predicting the postoperative course.  I have sized the pulmonary veins with dilators intraoperatively and recorded this for later analysis. 

A few technical points:  I have used running 7.0 Prolene suture in all of these procedures.  The right-sided suture line has been placed anterior and superior to the right phrenic nerve.  Isolated right-sided pulmonary vein stenosis can be addressed without actually entering the right atrium and simply incising the dilated plump pulmonary veins and carrying the incision into the left atrium.  In contrast, the left-sided suture line I have found is best placed posterior and inferior to the left phrenic nerve which tends to be more superior in relationship to the pulmonary veins than the right phrenic nerve.  In a similar fashion, isolated left pulmonary vein stenosis can be addressed by retracting the heart to the left and again incising into the pump dilated pulmonary veins proximal to the stenosis and carrying this incision into the left atrium.  On reoperations I attempt to preserve the inferior and superior adhesions in the pericardium to prevent bleeding at the superior and inferior aspect of the neoatrium.  These adhesions I have found can be quite helpful in containing the blood within the neo-left atrium. 

Many of these patients will have significant preoperative pulmonary hypertension.  Postoperatively they should be managed expectantly for possible pulmonary hypertensive crisis.  Despite measuring a normal pulmonary artery pressure intraoperatively, I have treated all these patients for the first 24 to 48 hours with muscle paralysis, hyperventilation, and routine use of pulmonary vasodilators such as milrinone and low dose dobutamine.  I would not hesitate to use inhaled nitric oxide or prostacyclin should the pulmonary artery pressures be borderline postoperatively or should the patient show any evidence of postoperative low output secondary to pulmonary hypertension.  Although I have not managed these patients with a pulmonary artery catheter, this should be a consideration. 

In summary the sutureless repair of pulmonary vein stenosis which was independently performed by Lacour-Gayet in France and the group at Sick Children’s Hospital in Toronto is a distinct advance in the management of infants with pulmonary veins stenosis following repair of total anomalous pulmonary venous connection.  In my view, it is a tremendous advance over the previous attempts to patch these with pericardium and suturing directly to the vein which almost always resulted in a recurrent pulmonary vein stenosis secondary to the inflammation along the suture line.  Despite the neoatrium being essentially a controlled bleed into the pericardium, violating the surgical principle of suturing “intima to intima,” the technique appears to have excellent intermediate results in these patients and I predict will have a good long-term outcome as well.