Treatment of Pulmonic Stenosis with a Domed Pulmonic Valve and Moderate-to-Severe Obstruction (Peak Gradient ≥36 mmHg)

This protocol applies to a precisely defined form of valvular pulmonary stenosis: isolated obstruction arising from a domed pulmonic valve with a peak transvalvular gradient of 36 mmHg or greater, and less than moderate pulmonic valve regurgitation.

Clinical scenario

The patient population is strictly bounded. Eligibility requires confirmed isolated valvular stenosis with domed valve morphology and a haemodynamically significant gradient. Cases involving a dysplastic pulmonary valve, a hypoplastic pulmonary annulus, sub-valvular or supravalvular pulmonary stenosis, or severe pulmonary regurgitation are outside the scope of this protocol.

Domed pulmonic valve Peak gradient ≥36 mmHg Isolated valvular stenosis <Moderate pulmonic regurgitation

Treatment approach

For this patient population, the applicable intervention is pulmonary valve replacement. Both surgical and transcatheter approaches are addressed; which pathway applies and under what specific conditions is detailed in the full structured protocol.

Treatment goals

The objective is alleviation of right ventricular dilatation and dysfunction and resolution of the associated symptoms.

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References

DOI: 10.2147/VHRM.S380240

According to the American Heart Association/American College of Cardiology (AHA/ACC) guidelines, in case of domed pulmonic valve with moderate or severe valvular stenosis and less than moderate pulmonic valve regurgitation, balloon valvotomy is recommended.

Severe stenosis: peak gradient ≥64 mmHg; Moderate stenosis: peak gradient between 36 and 64 mmHg.

At last, pulmonary valve replacement may be necessary when there is a failure with repair or severe residual symptomatic PR, in particular in cases with marked dysplasia of the pulmonary valve or significant hypoplasia of the annulus.

The management of these diseases has been radically changed by the introduction of the transcatheter pulmonary valve replacement (tPVR), leading to a reduction in the number of surgical interventions over lifetime.

The main available valves for transcatheter replacement are the Melody Valve and the Edwards SAPIEN XT and S3 Valves.

Pulmonary valve replacement, if appropriately implemented, effectively alleviates right ventricular dilatation and dysfunction and resolves the related symptoms.

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