Partial Anomalous Pulmonary Venous Return

1. Normally, the right pulmonary vein enters the left atrium here. 2. In PAPVR, the right pulmonary vein abnormally enters the right atrium.

This is one of the best illustrations I have found for explaining PAPVR.

Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart defect in which blood flow from one or more of the pulmonary veins return to the right atrium instead of to the left atrium.  This is a problem because the right atrium shoots this already oxygenated blood right back to the lungs to be oxygenated again.

Of course, this is a very inefficient way for the heart to function. The heart must work extra hard to provide an adequate amount of oxygenated blood to the body; and over time, this causes a variety of consequences, including arrhythmias, pulmonary hypertension, and right-sided heart failure.

If 50% or more of the pulmonary veins anomalously return, this is considered clinically significant enough to warrant surgical correction. If the patient has an Atrial Septal Defect, the surgeon will close the defect with a patch and redirect blood from the anomalous pulmonary vein to the left atrium.  If there is no ASD, a baffle or tunnel will be created to redirect blood to the left atrium.

PAPVR associated with Scimitar Syndrome has the added complication of dextrocardia. The surgery will likely be performed on a heart that is rotated backwards.  The atrial septum is usually intact.

Our first look at Nathan after heart surgery

My own son, Nathan, had this surgery on July 25, 2006, at C.S. Mott Children’s Hospital at the University of Michigan Medical Center. Our experience included a full day of preliminary testing, surgery the following morning, and a stay in PICU.  After extubation, he was moved to a regular hospital room where he spent the first few days on strong pain medication.  Removal of the chest tubes occurred on about the third day–this was somewhat emotionally challenging but was successful, nonetheless.  By that evening, his personality began to return.  On the fourth day, nurses encouraged him to take a brief walk down the hall.  By the fifth day, he was well enough to be released!

Nathan takes his first walk down the hall after heart surgery to correct PAPVR, Scimitar Syndrome. His surgery was performed at C.S. Mott Children's Hospital, an excellent facility for the treatment of congenital heart defects.

We used a number of child-friendly books to prepare Nathan for surgery.  One of his favorites was Franklin Goes to the Hospital.

Other related posts on this website include:  Scimitar Syndrome, Dextrocardia, Nathan’s Prayer, Remembering C.S. Mott, and Dr. Edward Bove.

Also see my article in Parents magazine:  “Nathan’s Wonder Slide”

If your child has an upcoming heart surgery, consider adding him/her to our Prayer List.

Tags: C.S. Mott Children's Hospital, congenital heart defect, Congenital Heart Defects, dextrocardia, Dr. Edward Bove, Nathan, Nathan's Prayer, Nathan's Wonder Slide, PAPVR, Parents Magazine, Partial Anomalous Pulmonary Venous Return, Scimitar Syndrome, University of Michigan Medical Center