Pulmonic Stenosis Follow-up

Updated: Dec 22, 2014
  • Author: Xiushui (Mike) Ren, MD; Chief Editor: Richard A Lange, MD, MBA  more...
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Follow-up

Deterrence/Prevention

Infective endocarditis prophylaxis: The American Heart Association (AHA) Guidelines on Prevention of Bacterial Endocarditis considers all forms of isolated pulmonic stenosis (PS) to be in the moderate-risk category, and any PS associated with complex congenital heart disease to be in the high-risk category. Therefore, antibiotic prophylaxis is recommended for all forms of PS.

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Prognosis

Except for critically severe stenosis in neonates, survival is the rule for individuals with congenital PS. [6] The long-term course of individuals with mild PS is indistinguishable from that of the unaffected population. Mild PS does not tend to progress in severity; rather, pulmonic valve orifice size usually increases with body growth.

Severe PS may result in outflow obstruction that progresses over a period of years despite body growth (60% of patients require intervention within 10 y of diagnosis). With appropriate intervention, those with moderately severe PS have an excellent prognosis.

The functional effect of PS may change during an individual's lifetime such that symptoms or limitations occurring in childhood may resolve by adulthood.

Available data support relieving moderately severe and severe PS in childhood, with follow-up care through adolescence and into adulthood. When PS is corrected during childhood, the life expectancy of the affected individual matches that of the unaffected age- and sex-matched cohort. The more severe and protracted the course of PS, the less optimal the outcome of intervention, including death due to RV failure in the most severe cases.

Balloon valvuloplasty is preferred, provided the valve is compliant and mobile. Those with severe valvular fibrocalcific thickening are more likely to require a surgical approach. The recurrence rate of PS in patients who are treated surgically is approximately 4%. Long-term results of balloon valvuloplasty are comparable to the results of surgical repair, with the rate of recurrence of severe PS less than 5%. A recent study shows that long-term follow-up of patients after surgical treatment for isolated pulmonary valve stenosis resulted in a high rate of reinterventions (53% at a median follow-up of 34 years). Thus, close follow-up in postsurgical patients is needed.

Adult patients are more likely to present with subvalvular hypertrophic pulmonic stenosis or valvular fibrocalcific thickening. Secondary subvalvular hypertrophic stenosis regresses following correction of the primary valvular abnormality, and residual dilatation of the pulmonary trunk is not significant clinically, even when marked. Recognizing subvalvular hypertrophy is important, since it may lead to dynamic outflow obstruction during the acute phase following correction of valvular stenosis. With few exceptions, postvalvuloplasty pulmonic regurgitation is of mild-to-moderate severity.

A study by Zdradzinski et al indicated that specific guidelines for the timing of valve replacement need to be developed for patients with isolated PS who develop pulmonic regurgitation after surgical valvotomy. The study, which involved 109 adult patients, including 34 patients with isolated PS and 75 with tetralogy of Fallot, investigated whether recommendations for valve replacement in patients with tetralogy of Fallot who develop pulmonic regurgitation after complete repair can be applied when regurgitation develops after valvotomy for isolated PS. [7]

The report found that the degrees of pulmonic regurgitation and symptom severity were similar between the two groups of patients, but an analysis of biventricular systolic function and QRS width indicated that the morphologic changes that occur in association with tetralogy of Fallot and its repair involve more than just the effects of pulmonic regurgitation. [7]

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