The outcome of surgical repair for total anomalous pulmonary venous return is generally excellent. The surgical mortality is less than 5 percent when repair is performed electively, in relatively healthy children without obstructed pulmonary veins.
As one might guess, the surgical mortality (death rate) is higher when surgery is performed emergently on critically ill newborns with obstructed pulmonary venous return. This is because they are very sick before going to surgery. Furthermore, critically ill newborns who do survive the surgery may require a prolonged period of post-operative intensive care. They often are on a ventilator (breathing machine) for an extended time as their lungs recover.
The long-term outcome after surgical repair of total anomalous pulmonary venous return is also excellent. Because the surgical repair results in a normal circulation, these children are typically expected to grow and develop normally. They usually have few symptoms, if any.
Rare complications of total anomalous pulmonary venous return can occur late following surgery. Regular follow-up by a qualified cardiologist is essential to detect these problems early, if they occur.
Rarely, obstruction to one or more pulmonary veins can develop. This can occur at the site of surgical repair, or due to abnormalities of the pulmonary veins themselves. Such pulmonary vein obstruction can lead to a shortness of breath or wheezing, particularly on exertion. The diagnosis can be somewhat difficult to make, and may require cardiac catheterization.
Abnormal cardiac rhythm is another late complication of total anomalous pulmonary venous return, but is also rare. Because of the extensive atrial surgery involved in the repair, some patients can experience abnormal electrical impulses arising in the atrium.
When these impulses occur in single beats, they are typically benign and do not require any treatment. On rare occasion, patients can have either sustained episodes of rapid heartbeat, or very slow heart rates. Either sustained fast heart rates or slow heart rates might require treatment. Fortunately, the results of such treatment (either medical or with a pacemaker) are excellent.