What is the Ross-Konno procedure in congenital heart disease?

The Ross-Konno Procedure in Congenital Heart Disease

The Ross-Konno procedure is the gold standard surgical intervention for complex left ventricular outflow tract obstruction (LVOTO) with aortic valve disease and/or aortic annular hypoplasia in children and young adults, providing excellent hemodynamics with growth potential and minimal valve-related complications. 1

What is the Ross-Konno Procedure?

• The Ross-Konno procedure combines two surgical techniques: the Ross procedure (pulmonary autograft replacement of the aortic valve) with the Konno aortoventriculoplasty (enlargement of the left ventricular outflow tract) 1
• It addresses multilevel left ventricular outflow tract obstruction by enlarging both the aortic annulus and subvalvular area 1
• The procedure involves harvesting the patient's own pulmonary valve (autograft) with a muscle skirt and implanting it in the aortic position, while reconstructing the right ventricular outflow tract with a homograft or other conduit 2, 1

Indications

• Severe aortic stenosis with aortic annular hypoplasia 2, 1
• Complex multilevel left ventricular outflow tract obstruction 1
• Congenital aortic valve disease with associated left ventricular outflow tract obstruction 3
• Can be performed in all age groups, including infants and neonates 2

Advantages

• Provides complete relief of left ventricular outflow tract obstruction 3
• The pulmonary autograft has growth potential, making it ideal for children 3
• Avoids the need for lifelong anticoagulation therapy 2
• Excellent hemodynamic performance with minimal valve-related complications 4
• Significantly reduces and normalizes left ventricular diastolic diameter and mass index within 3-12 months after operation 2

Surgical Techniques and Modifications

• Traditional Ross-Konno involves creating a ventricular septal defect (VSD) that is then patched 1
• The Mini-Ross-Konno is a modification that effectively relieves LVOTO without creating a VSD, simplifying the procedure 5
• The procedure is typically performed as a complete root implantation, with coronary arteries harvested as buttons 1
• Modifications have been developed to minimize the risk of conduction tissue injury and complete heart block by altering the interventricular septal incision 1

Outcomes

• Survival rates of 94-98.5% at 10 years have been reported 4, 3
• Freedom from autograft reoperation is approximately 95% at 10 years 4
• Freedom from more than trivial neo-aortic regurgitation is 95% at 5 years 2
• Excellent functional outcomes with 99% of survivors in NYHA class I or II 4
• No significant bleeding or thromboembolic complications 4
• Freedom from endocarditis is 95% at 15 years 4

Potential Complications

• Potential for autograft failure requiring reoperation (rare, approximately 1.5% of cases) 2
• Right ventricle-pulmonary artery conduit may require intervention over time (balloon dilation or reoperation) 3
• Risk of complete heart block due to proximity of the septal incision to the conduction system 1
• Neo-aortic root dilatation with progression of aortic regurgitation is a concern, especially with geometric mismatch between aortic and pulmonary roots 2

Special Considerations

• The procedure is technically demanding and may be associated with higher surgical risk, particularly in very young patients 1
• Better outcomes are observed when palliative interventions (surgical or percutaneous) are performed prior to valve replacement in infants under 1 year of age 4
• Continued surveillance of the autograft and right ventricular outflow tract conduit is essential 4, 2
• The procedure should be performed at centers with expertise in congenital heart disease and experience with the Ross procedure 1

Long-term Follow-up

• Regular echocardiographic monitoring is essential to assess autograft function and right ventricular outflow tract conduit 6
• Serial measurements show that the left ventricular outflow tract increases in size as patients grow, correlating with expected somatic growth 3
• Most patients do not require anticoagulation therapy after the procedure 2
• Continued monitoring of the entire aorta is recommended, particularly in patients with associated aortopathy 7

The Ross-Konno procedure represents a significant advancement in the management of complex congenital aortic valve disease with left ventricular outflow tract obstruction, offering excellent long-term outcomes and quality of life for affected patients.