Adolescents and young adults with congenital aortic valve disease (either aortic regurgitation and/or stenosis) present a significant challenge in terms of surgical strategy and technical approach for cardiac surgeons. The primary goal is to preserve the native valve whenever technically feasible. Even if the repair is not perfect, it allows for greater flexibility in subsequent surgeries compared to valve replacement strategies, without increasing the risk of complications. Moreover, preserving the native valve enables annular growth in younger patients and avoids the need for lifelong anticoagulation or antiplatelet therapy, which would significantly impact their daily lives.

This expert review provides a comprehensive analysis of surgical options for congenital aortic valve disease, evaluating the benefits and drawbacks of each technique and defining their role within a structured treatment plan. The review primarily focuses on four options: aortic valve repair, the Ross procedure, the Ozaki technique, and prosthetic valve replacement.

1. Aortic Valve Repair

Aortic valve repair is the preferred initial approach in young patients and adolescents. In cases of stenosis, surgical intervention should focus on improving leaflet mobility through techniques such as commissurotomy, shaving, or leaflet expansion with a pericardial patch. In cases where the primary dysfunction mechanism is regurgitation, the same repair techniques used in adults can be applied. Annuloplasty is the cornerstone of valve repair in these patients. In aortic annuloectasia, aortic root reimplantation (David procedure) has demonstrated a lower risk of annular dilation and reoperation compared to aortic root remodeling (Yacoub technique). Subannular rings (internal, such as HAART 2000 and 3000®, or external, using pericardial/PTFE strips like Coroneo®) have shown promising initial results, though long-term data are still unavailable.

Survival rates in expert centers range from 91% to 100% at 10 years, with virtually no major complications such as valve thrombosis, major bleeding, or endocarditis. Some series report reoperation rates of up to 40% at 10 years, but an imperfect repair remains preferable over immediate valve replacement. Risk factors for valve dysfunction and reoperation include unicuspid aortic valve, bicuspid valve with asymmetric commissures, and the use of pericardial patches.

2. Ross Procedure

The Ross procedure is the preferred option when valve repair is not feasible. It has demonstrated excellent outcomes, particularly in well-selected cases, such as patients with stenosis rather than regurgitation, normal or small annular size, adolescent or older patients, and those with a pulmonary annulus similar to the aortic annulus. Autograft reinforcement techniques, including subcoronary implantation, autograft placement within the left ventricular outflow tract, and external annuloplasty with a pericardial strip, have been developed to reduce the risk of dilation and dysfunction.

Compared to prosthetic aortic valve replacement in patients with non-repairable valves, the Ross procedure is associated with better survival and lower reoperation rates. The autograft behaves less obstructively, maintains better hemodynamic performance, and preserves physiological valve motion. Additionally, it allows for aortic valve growth as the patient matures, minimizing the risk of prosthesis-patient mismatch. When left ventricular outflow tract obstruction is present at multiple levels, the Ross procedure can be combined with annular enlargement techniques, with the Konno procedure being the most appropriate option.

Outcomes vary significantly based on patient age and perioperative conditions, but in adolescents and young adults, results are superior compared to those in neonates or infants. Survival rates in these age groups are excellent, with hospital mortality between 0% and 3%. Some studies report that long-term freedom from reoperation (>20 years) does not exceed 25%. Despite this, the Ross procedure remains a safe technique, with an annual complication rate (thrombosis, endocarditis, or major bleeding) of less than 1%.

Nevertheless, reoperation remains the Achilles’ heel of the Ross procedure, particularly regarding the right ventricular outflow tract. These reoperations are more complex and technically demanding than those for other patient groups and should be performed in experienced centers specializing in congenital heart disease.

3. Ozaki Technique

The Ozaki technique offers excellent hemodynamics, with a large effective orifice area, laminar flow, and a high coaptation height. It allows annular growth and has demonstrated a low incidence of valve-related complications. Compared to bioprosthetic valves, its durability may be comparable, though long-term data are still lacking. Short- to mid-term results remain inconclusive, with some series reporting a reoperation rate of approximately 10% within 14–28 months of follow-up.

Unresolved technical aspects of the Ozaki technique include the optimal patch material for leaflet reconstruction, the recommended glutaraldehyde fixation time, and the primary mechanisms of reconstruction failure. The exact role of the Ozaki technique in adolescent patients with congenital aortic valve disease remains uncertain. However, this technique may be particularly useful in specific cases, such as adolescents or young adults with an irreparable aortic valve and an unfavorable anatomy for the Ross procedure.

4. Prosthetic Valve Replacement

In young patients, prosthetic valve replacement should be considered a last resort, reserved for cases where all other techniques have failed. This is due to the significant disadvantages associated with prosthetic valves, particularly in terms of long-term outcomes. In cases of small left ventricular outflow tracts and/or small aortic annuli, annular enlargement should be considered to prevent severe prosthesis-patient mismatch as the child grows.

Mechanical prostheses are generally the preferred choice in this age group due to their durability. Alternative options include bioprostheses, homografts, and xenografts, though their long-term benefits remain controversial. Mechanical valves have low perioperative morbidity and mortality, but reoperation rates are high: 15–20% at 10 years and 40% at long-term follow-up. The main causes of reoperation include pannus formation, endocarditis, valve thrombosis, and prosthesis-patient mismatch. Additionally, lifelong anticoagulation therapy with vitamin K antagonists is required, leading to limitations such as contraindications during pregnancy, restrictions in physical activity, and the need for continuous dose adjustments.

Although bioprostheses initially show lower reoperation rates compared to mechanical valves, all bioprostheses eventually degenerate and require replacement. Consequently, long-term survival benefits are inferior compared to mechanical prostheses. Transcatheter aortic valve implantation (TAVI) has been briefly mentioned, but no long-term data are available for young patients. While TAVI could potentially be useful in high-risk surgical cases, clear indications remain undefined.

Homografts are also discussed, with evidence suggesting a lower risk of endocarditis compared to prosthetic valves. However, they present a higher risk of reoperation than mechanical prostheses and tend to undergo significant calcification over time, making future interventions more complex.

COMMENTARY:

There are multiple surgical options for managing congenital aortic valve disease in adolescents and young adults, reflecting the broad anatomical variability of this condition. The choice of surgical strategy must be individualized, weighing the risks and benefits of each approach. However, the main objectives should always be to preserve the native valve whenever possible, maximize durability, minimize surgical complications, and optimize hemodynamics to improve the patient’s quality of life.

REFERENCE:

Frankel WC, Robinson JA, Roselli EE, Unai S, Tretter JT, Fuller S, et al. Lifetime Management of Adolescents and Young Adults with Congenital Aortic Valve Disease. Ann Thorac Surg. 2025 Jan;119(1):59-69. doi: 10.1016/j.athoracsur.2024.04.038. Epub 2024 Jun 11. PMID: 38871162.