Long-term outcome after treatment of isolated pulmonary valve stenosis

doi:10.1016/j.ijcard.2010.10.038

International Journal of Cardiology

Volume 156, Issue 1, 5 April 2012, Pages 11-15

https://doi.org/10.1016/j.ijcard.2010.10.038 Get rights and content

Abstract

Background

Few data are available on very long-term follow-up after treatment for isolated pulmonary valve stenosis (PVS), either surgically or by percutaneous balloon angioplasty (PBA).

Methods and results

All patients with isolated PVS were selected from our database of congenital heart defects. Their records were reviewed systematically. We identified 79 surgically treated patients with a median follow-up of 22.5 years (range 0–45 years) and 139 PBA patients with median follow-up of 6.0 years (range 0–21 years). Echocardiographic and catheterization parameters indicate excellent results of both techniques in relieving the transpulmonary gradient. However, after initial surgery 20.3% of patients needed a cardiac re-intervention: 81% for severe pulmonary valve regurgitation, but none for residual pulmonary stenosis. After initial PBA a cardiac re-intervention was needed in 9.4% of patients. In 85% the indication was residual pulmonary stenosis, in none of them pulmonary regurgitation, although almost all patients developed a mild pulmonary regurgitation. Freedom of re-intervention after surgery was 98.4%, 93.5%, 87.7%, 70.9% and 55.7% at 5, 10, 20, 30 and 40 years postoperatively. Freedom of re-intervention in the PBA group was 95.1%, 87.5% and 84.4% at 5, 10 and 20 years post-procedure.

Conclusions

Both surgery and PBA are safe and successful in relieving the acute transpulmonary gradient. Long-term results of surgery are worse than previously thought due to severe PR. After PBA re-interventions for residual stenosis are frequently needed and the incidence of mild PR is high. Very long-term results of PBA are still unknown.

Introduction

Pulmonary valve stenosis (PVS) is a common disorder and accounts for up to 10% of all congenital heart defects. Until a few decades ago, surgical intervention was the treatment of choice for a moderate to severe PVS. However, since the first balloon valvuloplasty has been performed in 1982 by Kan [1], it has been further established as a valuable alternative. Although isolated PVS is relatively common, few data are available on very long-term follow-up after treatment, either surgically or by balloon angioplasty. Therefore, we aimed at evaluating retrospectively the outcome of both PVS treatment choices in our centre.

Section snippets

Patients' selection

An automatic search in the database of all patients with congenital heart disease followed in our centre was performed. After looking for every case of PVS, those patients with isolated PVS were selected. Patients with associated anomalies (Table 1) were not considered for further analysis. Patients found to have a PVS in combination with a patent foramen ovale or atrial septal defect (ASD) without any further anomalies, were not excluded. Patients with Noonan syndrome were selected for

Selection of patients

In our centre's database, 120 patients underwent surgical repair for PVS between 1960 and 2009. Twenty-seven patients were excluded from further analysis because of associated anomalies, as listed in Table 1. The patient with mitral valve dysplasia also had a problem of left ventricular non-compaction. One patient had first undergone balloon angioplasty and in thirteen patients, no follow-up data were available. Subsequently, a total of 79 surgically treated patients were eligible for further

Surgery

Survival data after surgical repair for isolated PVS are excellent. In our series of 79 patients we reported two in-hospital fatal cases and one late death due to myocarditis, unrelated to the preceding surgical intervention. These results confirm the data found in the literature. In a series of 191 patients with a follow-up time between 20 and 30 years, a survival (excluding hospital mortality) of 90% was reported at 25 years postoperatively [2]. Life expectancy was normal in patients who

Conclusions

Both surgery and PBA have low mortality rates and appear to be successful in relieving the acute transpulmonary gradient. Life-long follow-up after surgery is warranted because long-term results are worse than previously thought, due to severe PR. After PBA re-interventions for pulmonary stenosis are more frequently needed and the incidence of mild PR is high. Long-term results of PBA are still unknown.

Acknowledgement

The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology [18].

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Les interventions chez les adultes atteints de cardiopathie congénitale (AACC) sont axées sur des interventions chirurgicales et percutanées, tenant compte de l'évolution rapide de la pratique clinique dans ce domaine. Afin d'apporter une certaine rigueur à notre démarche et d'amplifier la propension cumulative des données probantes concernant les AACC, nous avons utilisé le processus ADAPTE; nous avons systématiquement évalué, mis à jour et adapté les lignes directrices existantes des sociétés de cardiologie canadiennes, américaines et européennes de 2010 à 2020. Nous avons appliqué cette méthode aux interventions liées à l'obstruction du débit ventriculaire droit et gauche, à la tétralogie de Fallot, à la coarctation, à l'aortopathie associée à une dysfonction de la valve aortique bicuspide, aux anomalies du canal auriculo-ventriculaire, à l'anomalie d'Ebstein, à la transposition congénitale complète corrigée, et aux patients ayant subi une intervention de Fontan. En plus des tables indexées aux évidences, des diagrammes de flux cliniques sont inclus pour chaque affection afin de faciliter une approche pratique de la prise de décision clinique. En sont exclues : les recommandations concernant les stimulateurs cardiaques, les défibrillateurs et les interventions dirigées envers les arythmies, qui font l'objet de documents distincts. De la même façon, en cas de chevauchement avec d'autres directives sur les interventions valvulaires, il est fait référence à des publications parallèles. Il y a une pénurie d’évidences de haute qualité sous forme d'essais cliniques randomisés pour appuyer les lignes directrices concernant les AACC. Nous en avons tenu compte dans la formulation de l’amplitude des recommandations formulées par nos experts nationaux et internationaux. Au fur et à mesure que les données sur le suivi à long terme s'accumulent, nous nous attendons à ce que les preuves à la base de la pratique clinique deviennent de plus en plus granulaires. Ces recommandations sont destinées à guider la discussion entre les cliniciens, les cardiologues interventionnels, les chirurgiens et les patients qui doivent prendre des décisions complexes concernant les interventions chez les AACC.
2020 ESC Guidelines for the management of adult congenital heart disease: The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Adult Congenital Heart Disease (ISACHD)
2021, Revista Espanola de Cardiologia
Repaired isolated pulmonary valve stenosis: living happily ever after?
2020, Revista Espanola de Cardiologia
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Aunque la estenosis de la válvula pulmonar (EVP) se considera una cardiopatía congénita de bajo riesgo, se ha descrito la aparición de complicaciones y necesidad de reintervenir durante el seguimiento. Los objetivos del estudio fueron evaluar los resultados a largo plazo de la EVP reparada e identificar predictores de complicaciones cardiovasculares y reintervención.
Se estudió a 158 pacientes adultos con EVP reparada (reparaciones practicadas de 1957 a 2010) con seguimiento activo en un centro terciario de referencia.
Se sometió a cirugía a 95 pacientes (60%) y a valvuloplastia percutánea con balón a 63 (40%). Tras una mediana de seguimiento de 27 [20-33] años, la mayoría de los pacientes (n = 134 [84,8%]) estaban en clase funcional I de la New York Heart Association, pero 61 (38.6%) requirieron reintervención, principalmente reemplazo de la valvular pulmonar (n = 28 [17,7%]), y 19 (12%) presentaron al menos una complicación cardiovascular: 13 (8,2%), arritmias supraventriculares; 6 (3,8%), insuficiencia cardiaca; 5 (3,2%), accidente cerebrovascular; 1 (0,6%), muerte; 1 (0,6%) tromboembolia, y 1 (0,6%), arritmia ventricular. El análisis multivariante mostró que la edad en el momento de la reparación de la EVP (HR = 1,08; IC95%, 1,04-1,12; p < 0,001) y la presencia de cianosis antes de la reparación (HR = 5,23; IC95%, 1,99-13,78; p = 0,001) fueron predictores independientes de complicaciones cardiovasculares.
Se puede esperar un buen resultado a largo plazo tras de la reparación de la EVP, pero pueden aparecer complicaciones y necesidad de reintervenir. Una edad más avanzada y la presencia de cianosis en el momento de la reparación de la EVP son predictores de complicaciones cardiovasculares e identifican a una población que requiere un control más estricto.
Although pulmonary valve stenosis (PVS) is considered a low risk congenital heart disease, there have been reports of complications and the need for reintervention throughout follow-up. The aims of this study were to evaluate the long-term outcome of repaired PVS and to identify predictors of cardiovascular complications and reintervention.
We studied 158 adult patients with repaired PVS (repair procedures performed from 1957 to 2010) receiving active follow-up in a tertiary referral center.
A total of 95 patients (60%) received surgical treatment, and 63 patients (40%) received percutaneous pulmonary balloon valvuloplasty. At the end of follow-up (27 years, IQR, 20-33 years), most patients (n = 134, 84.8%) were in New York Heart Association functional class I, but 61 patients (38.6%) required a reintervention, mainly pulmonary valve replacement (17.7%, n = 28), and 19 patients (12%) had at least one cardiovascular complication: 13 (8.2%) supraventricular arrhythmias, 6 (3.8%) heart failure, 5 (3.2%) stroke, 1 (0.6%) death, 1 (0.6%) thromboembolism, and 1 (0.6%) ventricular arrhythmia. Multivariate analysis showed that age at PVS repair (HR, 1.08; 95%CI, 1.04-1.12; P < .001) and the presence of cyanosis before PVS repair (HR, 5.23; 95%CI, 1.99-13.78; P = .001) were independent predictors for cardiovascular complications.
Good long-term outcome can be expected after PVS repair, but complications and the need for reintervention may appear. Older age and the presence of cyanosis at PVS repair emerged as predictors of cardiovascular complications and identified a population that may merit stricter control.
2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines
2019, Journal of the American College of Cardiology
2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines
2019, Journal of the American College of Cardiology

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View full text

Long-term outcome after treatment of isolated pulmonary valve stenosis

Abstract

Background

Methods and results

Conclusions

Introduction

Section snippets

Patients' selection

Selection of patients

Surgery

Conclusions

Acknowledgement

Percutaneous balloon valvuloplasty: a new method for treating congenital pulmonary valve stenosis

New Engl J Med

Long-term outcome of patients undergoing surgical repair of isolated pulmonary valve stenosis. Follow-up at 20–30 years

Circulation

25-year mortality after surgical repair of congenital heart defect in childhood. A population-based cohort study

JAMA

Long-term outcome after surgery for pulmonary stenosis (a longitudinal study of 22–33 years)

Eur Heart J

Second Natural History Study of Congenital Heart Defects. Results of treatment of patients with pulmonary valvar stenosis

Circulation

Long-term follow-up of patients after surgical treatment for isolated pulmonary valve stenosis

Mayo Clin Proc

Balloon pulmonary valvuloplasty: results of the valvuloplasty and angioplasty of congenital anomalies registry

J Cardiol

Long-term results after balloon pulmonary valvuloplasty

Circulation

Long-term results (up to 17 years) of pulmonary balloon valvuloplasty in adults and its effects on concomitant severe infundibular stenosis and tricuspid regurgitation

Am Heart J