Ross Procedure Sections: Introduction | History | Anatomy | Pros & Cons | Patient Selection | Technique | Results | Pediatric Ross

Pediatric Ross

The most common valve problem in children is aortic stenosis. The small, malformed aortic valves in these children are very difficult to repair. Stenotic valves can either be dilated with balloon catheters or surgically incised to increase the opening. If the valve cannot be opened sufficiently, the child continues to suffer from aortic stenosis. After balloon dilatation or surgery, the opening may become larger but as a consequence the valve may also leak a lot more (i.e.regurgitation. These children continue to have problems with strain on the pumping chamber until a better solution can be found.

Replacement of the abnormal aortic valve in a small child is a unique challenge, particularly in sizing the new valve. There are no manufactured valves that perform well in very small children. The smallest successful artificial valve is roughly 17 millimeters in diameter. Very young children with congenital aortic stenosis cannot even fit a valve of this size. The root of the heart can sometimes be surgically enlarged enough to tolerate a 17 or 19 millimeter valve (using the Konno procedure), but not without consequence. Even with enlargement techniques, it is often not possible to implant a full adult sized valve in very small children. These patients will then outgrow the implant, requiring further surgery later in life.

Mechanical valve substitutes also require protection against clotting with anticoagulant medications. To prevent clot formation on the new valve (which can cause strokes or dysfunction of the valve), oral anticoagulants must be taken for the remainder of the patients life. The issue of reliable anticoagulation administration is troublesome in small children and adolescents. Implantation of animal tissue, or xenograft, valves (which do not require blood thinners) does not solve this dilemna entirely since tissue valves are very prone to premature calcification and degeneration in growing children. Even human homograft (cadaver) valves used in small infants tend to calcify before the patient reaches adult size.

To overcome these significant problems with surgical treatment of congenital aortic valve disease, the Ross procedure has been proposed as a desirable alternative. Over the last decade, Dr. Ronald Elkins at the University of Oklahoma has shown that the Ross procedure is truely the best long term treatment for children with aortic valve and root abnormalities. Notably, Dr. Elkins was the first to demonstrate that the autotransplant grew along with the child, making this operation the only aortic valve replacement with growth potential. In addition, the native tissue reconstruction provided by the Ross made it possible to eliminate the burden of anticoagulation altogether. The Ross also had clear advantages over xenograft tissue valve replacements since the pulmonary autograft neither calcified nor degenerated over time.

Dr. Elkins has now reported on over 112 children undergoing Ross procedures from the age of 8 months or older. Operative mortality was only 4.4%, a remarkable accomplishment considering the complexity of the valve problems typically seen in these small patients. Actuarial survival was 96.5% at seven years from the date of surgery . Freedom from failure in the transplanted pulmonary graft was 92%, and as high as 96% when used as a full root replacement.

Dr. Elkins success with the Ross in small children has prompted other surgeons to extend the benefits of the autotransplant into even younger ages. Dr. John Calhoun of the University of Texas, San Antonio was the first to use a Ross reconstruction in a newborn infant. In these newborns, the potential for growth of the aortic reconstruction is a vital advantage of the Ross procedure over any other form of replacement. As a further extension of the procedure in patients with very small roots, Dr. Steven Gundry at Loma Linda University has reported a modified Ross autotransplant done in concert with annular enlargement as first described by Konno, et. al. The Konno-Ross is performed by first harvesting additional muscle from the anterior right ventricular outflow tract as the autograft is procurred. After removal of the diseased aortic valve, the aortic annulus is split open between the right and left coronary arteries, as described by Konno, et. al. Then the pulmonic donor graft is sewn into this enlarged annulus, including the additional muscle skirt harvested with the autograft. Finally, the pulmonary homograft reconstruction of the right heart is purposely oversized to permit growth of the child and reduce the need for secondary operations. In 8 Konno-Ross procedures performed by Dr. Gundry, all patients are doing well.

In conclusion, the root replacement style of Ross procedure is feasible to perform in very small children and neonates with minimal operative risk. Unlike previous aortic valve repairs or replacements, the potential for growth without degeneration, calcification, or outgrowing the surgical result is provided by the autotransplant operation. The freedom from anticoagulation is another clear advantage. Although technically more demanding, the use of the Ross operation in pediatric patients with aortic valve disease is clearly a major step forward in the surgical management of these patients.

Ross Procedure Sections: Introduction | History | Anatomy | Pros & Cons | Patient Selection | Technique | Results | Pediatric Ross

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