What is a Ross procedure in cardiac pediatrics?

What is the Ross Procedure in Cardiac Pediatrics

The Ross procedure is a surgical technique where the patient's own pulmonary valve (pulmonary autograft) is transplanted to replace a diseased aortic valve, and a homograft is then used to reconstruct the right ventricular outflow tract (RVOT), creating a living valve substitute that avoids anticoagulation and has growth potential. 1

Surgical Technique and Components

The operation involves two key steps that convert single-valve disease into a two-valve operation:

• Autograft transplantation: The native pulmonary valve is harvested and implanted into the aortic position, with total aortic root replacement being the preferred method of implantation 1, 2
• RVOT reconstruction: A pulmonary or aortic homograft is placed to replace the harvested pulmonary valve 1, 3
• Coronary reimplantation: The coronary arteries must be reimplanted into the neoaortic root 4

Primary Indications in Pediatric Patients

The Ross procedure should be reserved for younger patients (typically <18 years, though extended to <50 years in some cases) with congenital aortic valve disease when anticoagulation is contraindicated or undesirable. 1, 4

Specific indications include:

• Congenital aortic stenosis in children requiring permanent valve replacement 2, 5
• Bicuspid aortic valve disease with stenosis, regurgitation, or mixed lesions 5, 6
• Small aortic annulus, particularly in young women of childbearing age who wish to avoid anticoagulation 2, 4
• Complex left ventricular outflow tract obstruction 2
• Active endocarditis (native or prosthetic valve) due to resistance to infection 2

Key Advantages

The procedure offers several unique benefits in the pediatric population:

• Growth potential: The autograft demonstrates real potential for growth as the child develops, with serial measurements showing the left ventricular outflow tract increases proportionally with somatic growth 5, 3
• Avoidance of anticoagulation: Eliminates lifelong warfarin therapy and associated bleeding/thromboembolic risks 1, 3
• Excellent hemodynamics: Provides superior hemodynamic performance compared to mechanical or biological prostheses 5, 2
• Low thrombogenicity: Minimal risk of thromboembolic complications (0.00-0.58%/year) 3
• Resistance to infection: Lower endocarditis rates (0.00-1.68%/year) compared to prosthetic valves 3, 2

Critical Limitations and Risks

The major concern is that this procedure converts single-valve disease into potential double-valve disease, with significant reintervention rates over time. 3, 1

Autograft Failure

• Neoaortic regurgitation is the most common mode of failure, typically occurring in the second decade post-operatively 1
• Autograft dilatation can develop, particularly in patients with bicuspid aortic valve and aortic regurgitation 6
• Reoperation for autograft failure occurs at rates of 0.37-2.81%/year 3

Homograft Failure

• At least half of pulmonic homograft valves require reintervention within 10-20 years 1
• RVOT reconstruction failure requiring reoperation occurs at rates of 0.34-4.76%/year 3
• Calcification and adhesions may complicate future reoperations 1

Mortality Rates

• Early mortality: 0-17% (higher in neonates, lower in older children; overall modern era mortality ~2.5-5.6%) 5, 3, 2
• Late mortality: Very low at 0.04-1.83%/year, with 10-year survival of 94-98% 5, 3

Absolute Contraindications

Marfan syndrome is an absolute contraindication due to the risk of progressive aortic root dilatation 2

Use with extreme caution in:

• Rheumatic valve disease: Higher incidence of autograft dysfunction and increased reoperation risk 7, 2
• Dysplastic dilated aortic root: Increased risk of autograft failure 2

Surgical Expertise Requirements

This procedure should ONLY be performed at Comprehensive Valve Centers by surgeons with exceptional expertise and extensive experience in the Ross operation. 1, 4

The technical demands include:

• Complex coronary reimplantation techniques 4
• Potential need for concomitant procedures (Ross-Konno for annulus enlargement, annulus reduction techniques) 2
• Management of graft-host size mismatch 2

Long-term Follow-up Requirements

Lifelong yearly follow-up is mandatory to monitor both valve systems 4

Essential monitoring includes:

• Echocardiography: Assessment of autograft function, neoaortic root dimensions, and RVOT conduit function 8, 4
• Aortic root surveillance: Continued monitoring of the entire aorta, particularly in patients with associated aortopathy 8
• Functional assessment: Exercise testing for surgical decision-making regarding reintervention 4
• Arrhythmia screening: ECG and ambulatory monitoring, as arrhythmias may occur with LV hypertrophy 4

Reintervention Spectrum

When reoperations are required, they can be complex:

• 55% are isolated procedures (single valve replacement) 7
• 45% involve 2-4 simultaneous cardiac procedures (multivalvular surgery) 7
• Despite complexity, reoperation mortality is very low with good mid-term results 7
• Freedom from additional cardiac surgery: 82% at 10 years after initial reoperation 7

Pregnancy Considerations

The Ross procedure is particularly advantageous for women of childbearing age:

• Avoids anticoagulation-related embryopathy that occurs with mechanical valves 4
• Pregnancy is generally well-tolerated in patients with good autograft function 4
• Fetal echocardiography is recommended in the second trimester due to 5% or higher recurrence risk of congenital heart disease 4

Comparative Context

The Ross procedure should be preferred over mechanical valve replacement in younger patients with few comorbidities when performed by an experienced surgeon, as it offers mortality benefits at 20-year follow-up despite higher operative complexity. 4

Alternative options include:

• Mechanical valves: More durable but require lifelong anticoagulation 4
• Biological valves/homografts: Avoid anticoagulation but have limited durability and no growth potential 4
• Balloon valvuloplasty: May be attempted in adolescents with non-calcified valves before proceeding to Ross 4