Right ventricular dysfunction and pulmonary valve replacement after correction of tetralogy of fallot

doi:10.1016/S0003-4975(02)03586-5

The Annals of Thoracic Surgery

Volume 73, Issue 6, June 2002, Pages 1794-1800

Presented at the Forty-eighth Annual Meeting of the Southern Thoracic Surgical Association, San Antonio, TX, Nov 8–10, 2001.

https://doi.org/10.1016/S0003-4975(02)03586-5 Get rights and content

Abstract

Background. Correction of tetralogy of Fallot often leads to pulmonary regurgitation, sometimes warranting pulmonary valve replacement, for which indications and timing to achieve optimal results are not yet clear. This retrospective study describes follow-up and reinterventions in our tetralogy of Fallot population.

Methods. Review of all consecutive patients operated on for tetralogy of Fallot between 1977 and 2000 was conducted. Included are date and type of repair, Doppler echocardiography (two-dimensional echocardiography), electrocardiographs, reoperations, and physical condition.

Results. Total repair was performed in 171 patients at a mean age 1.9 ± 2.5 years, follow-up time counted 9.6 ± 7.0 years. Right ventriculotomy was used in 92%, and transatrial ventricular septal defect closure was used in 8%; 74% received a transannular outflow patch. Twenty-year survival was 91%. Last follow-up electrocardiographs showed right bundle branch block in 67% and serious arrhythmias in 11%. Two-dimensional echocardiography demonstrated severe pulmonary insufficiency and dilated right ventricle in 31% and 38%, respectively, increasing with postrepair age (p < 0.001). Poor clinical condition (New York Heart Association class II+) and echocardiographic proof of right atrial dilatation (p = 0.012) and arrhythmias (p = 0.03) were significantly associated. Furthermore, the influence of residual hemodynamic lesions, such as a remaining ventricular septal defect or pulmonary stenosis, or right ventricular dilatation was important (p = 0.04). Reintervention was necessary in 32 patients (19%; 10-year freedom, 83%), including angioplasty for residual stenosis and pulmonary valve replacement. At a mean age of 9.2 years after correction, 14 patients received a homograft, and 2 patients received a heterograft. In 7 patients the right ventricle returned to normal dimensions and symptoms disappeared. The incidence of right ventricular dilatation was considerably higher (p = 0.020) in patients with a transannular patch; the transatrial approach showed the opposite (p = 0.03), and patients presented with lower QRS duration (p = 0.007), although no difference could be found between survival after both surgical techniques. Effects of early timing (correction < 6 months) on right ventricular dysfunction could not be established.

Conclusions. Severe right ventricular dilatation and pulmonary regurgitation secondary to outflow tract repair in tetralogy of Fallot are frequently occurring sequelae developing slowly over time. Indications for pulmonary valve replacement remain controversial because echocardiographic findings or arrhythmias are not always accompanied by deterioration of clinical condition. However, right atrial dilatation and additional hemodynamic lesions demand increased vigilance. Transatrial repair is associated with a favorable outcome.

Section snippets

Population

The reports of all consecutive surviving patients, operated on in the Children’s Heart Center Utrecht between 1977 and 2000 and residing in the Netherlands, were examined retrospectively. The population (n = 171) consisted of 106 (62%) male and 65 female patients with a mean follow-up time of 9.6 ± 7.0 years (range, 0.2 to 32.6 years; median, 8.0) and mean age of 13.8 ± 8.0 years (range, 0.2 to 37.8 years). Excluded from this survey were 99 patients with follow-up abroad directly after

Follow-up

A total of 16 patients died during follow-up after complete repair. However, of the 11 deaths almost immediately after the operation, 2 patients had tracheobronchomalacia, 1 suffered from cystic fibrosis combined with septic shock, and 2 from trisomy 13 and VATER association. Late deaths included 3 unrelated deaths (eg, car accident) and 2 patients with sudden cardiac death. Actuarial survival was 91% at both 10 and 20 years (Fig 1). Total mortality attributed only to the heart disease itself

Comment

In conjunction with recent large studies, the prognosis of a newborn with tetralogy of Fallot is excellent 1, 2, 3, 4, 21. Most patients were in New York Heart Association functional class I, and we found a 20-year actuarial survival of 91%. This is lower than the general population, as pointed out by Murphy and colleagues [1], but it is still a very reasonable outcome compared with other serious congenital heart defects. The group of patients with pulmonary atresia needs to be distinguished,

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Cited by (124)

Outcomes in Young Adults With Tetralogy of Fallot and Pulmonary Annular Preserving or Transannular Patch Repairs
2021, Canadian Journal of Cardiology
Early surgical tetralogy of Fallot (ToF) repair involved patching across the pulmonic annulus (transannular patch [TAP] repair), which resulted in severe pulmonic regurgitation. Long-term outcome improvements were anticipated with modifications that preserved the pulmonic annulus (annulus-preserving [AP] repair). The objective of the present study was to evaluate the need for late reintervention in adults with AP repair and those with TAP repair.
We conducted a retrospective review of adults (born 1981-1996) with childhood intracardiac ToF repairs at a tertiary care center. The primary cardiovascular outcome was need for reintervention after primary intracardiac repair of ToF. Secondary outcomes included a composite of death, heart failure, and ventricular arrhythmias.
Two hundred thirty adults were included: 104 with AP repair and 126 with TAP repair. The median age at last follow up was 25 years (interquartile range [IQR] 20-28) and the median follow-up duration was 7.9 years (IQR 3.5-12). Reintervention of any type was significantly more common in the TAP group during both childhood and adulthood (72.2% TAP vs 20.2% AP, HR 5.5, 95% CI 3.4-9.0; P < 0.001). Pulmonary valve replacement (PVR) was almost 6 times more likely in adults with TAP repair (65.1% TAP vs 16.3% AP, HR 5.7, 95% CI 3.4-9.7; P < 0.001).
Patients who had AP ToF repair had significantly fewer late reinterventions compared with TAP repair, with the majority of reinterventions due to PVR. More long-term follow-up is required.
La réparation chirurgicale précoce de la tétralogie de Fallot (TF) exigeait un patch dans l’anneau pulmonaire (réparation par patch transannulaire [PTA]), qui entraînait une régurgitation pulmonaire grave. Les modifications qui permettaient de préserver l’anneau pulmonaire (la réparation avec préservation de l’anneau [PA]) laissaient anticiper l’amélioration à long terme des critères de jugement. L’objectif de la présente étude était d’évaluer la nécessité de réintervention tardive chez les adultes qui subissaient une réparation avec PA et ceux qui subissaient une réparation par PTA.
Nous avons mené une revue rétrospective auprès d’adultes (nés entre 1981-1996) qui avaient subi des réparations intracardiaques de la TF durant l’enfance dans un centre de soins tertiaires. Le principal critère de jugement cardiovasculaire était le besoin de réintervention après la réparation intracardiaque primaire de la TF. Les critères de jugement secondaires incluaient un critère composite de décès, d’insuffisance cardiaque et d’arythmies ventriculaires.
Parmi les 230 adultes sélectionnés, 104 avaient subi une réparation avec PA et 126 avaient subi une réparation par PTA. L’âge médian au dernier suivi était de 25 ans (écart interquartile [EIQ] 20-28) et la durée médiane des suivis était de 7,9 ans (EIQ 3,5-12). Les réinterventions de tout type étaient significativement plus fréquentes dans le groupe PTA durant l’enfance et l’âge adulte (72,2 % PTA vs 20,2 % PA, RR 5,5, IC à 95 % IC 3,4-9,0; P < 0,001). Le remplacement de la valve pulmonaire (RVP) était presque six fois plus probable chez les adultes qui avaient subi une réparation par PTA (65,1 % PTA vs 16,3 % PA, RR 5,7, IC à 95 % 3,4-9,7; P < 0,001).
Les patients qui avaient subi une réparation de la TF avec PA subissaient significativement moins de réinterventions tardives que les patients qui avaient subi une réparation par PTA. La majorité de ces interventions était réalisée en raison du RVP. D’autres suivis à long terme sont nécessaires.
The TRIVIA Cohort for Surgical Management of Tetralogy of Fallot: Merging Population and Clinical Data for Real-World Scientific Evidence
2020, CJC Open
Guidelines for surgical management of tetralogy of Fallot (TOF) are often based on low-quality evidence due to the many challenges of congenital heart disease: heterogeneous cardiac anatomy, consequences from surgical interventions arising years later, and scarcity of hard outcomes. The overarching goal of the Tetralogy of Fallot Research for Improvement of Valve replacement Intervention: A Bridge Across the Knowledge Gap (TRIVIA) study is to evaluate the long-term impact of the surgical management strategies in TOF. The specific objectives are: (1) to describe the long-term outcomes of TOF according to the native anatomy and the presence of genetic conditions, (2) to evaluate the long-term outcomes of surgical repair according to associated residual lesions, and (3) to evaluate the impact of paediatric pulmonary valve replacements on the long-term outcomes.
The TRIVIA study is a population-based cohort including all subjects with TOF in the province of Québec between 1980 and 2017. It links patient-level granular clinical data with long-term administrative health care data. We will evaluate mortality, cardiovascular interventions, and hospitalizations for adverse cardiovascular events using survival Cox models and marginal mean/rates models for recurrent events, respectively. Multivariate multilevel models will correct for potential confounders, and risk score matching will mitigate the potential of confounding by indication.
The current TRIVIA cohort includes 1001 eligible subjects with TOF with complete lifelong follow-up for > 98%. The median follow-up is 17.1 years, totalling > 17,000 patient-years.
Universal health insurance data combined with granular clinical data enable the development of population-based cohorts, to which contemporary statistical methods are applied to address important research questions in congenital heart disease research.
Les lignes directrices pour la prise en charge chirurgicale de la tétralogie de Fallot (TdF) sont souvent basées sur des données de faible qualité en raison des nombreux défis posés par les maladies cardiaques congénitales : anatomie cardiaque hétérogène, répercussions des interventions chirurgicales survenant des années plus tard et rareté de conclusions solides. L'objectif principal de l’étude TRIVIA (Tetralogy of Fallot Research for Improvement of Valve replacement Intervention: A Bridge Across the Knowledge Gap) est d'évaluer l'impact à long terme des stratégies de prise en charge chirurgicale dans les TdF. Les objectifs spécifiques sont les suivants : (1) décrire les issues à long terme de la TdF en fonction de l'anatomie native et de la présence de conditions génétiques, (2) évaluer les conséquences à long terme de la chirurgie réparatrice en fonction des lésions résiduelles associées, et (3) évaluer l'impact des remplacements valvulaires pulmonaires pédiatriques sur le pronostic à long terme.
L'étude TRIVIA est une étude de cohorte basée sur une population comprenant tous les sujets atteints de TdF dans la province du Québec entre 1980 et 2017. Elle fait le lien entre les données cliniques détaillées des patients et les données administratives des soins de santé à long terme. Nous évaluerons la mortalité, les interventions cardiovasculaires et les hospitalisations pour troubles cardiovasculaires en utilisant respectivement des analyses de survie par les modèles de Cox et des modèles de moyennes/taux marginaux pour les événements récurrents. Des modèles multivariés à plusieurs niveaux permettront de corriger les facteurs de confusion potentiels, et l'appariement par des scores de risque atténuera le potentiel de confusion par indication.
La cohorte de l’étude TRIVIA comprend actuellement 1001 sujets admissibles atteints de TdF avec un suivi complet tout au long de la vie pour > 98 %. Le suivi médian est de 17,1 ans, pour un total de > 17 000 patients-années.
Les données de l'assurance maladie universelle combinées à des données cliniques détaillées permettent la mise en place de cohortes populationnelles, auxquelles les méthodes statistiques contemporaines pourront être appliquées afin de répondre aux questions importantes pour la recherche portant sur les maladies cardiaques congénitales.
When “Blue Babies” Grow Up: Complications After Surgical Repair of Tetralogy of Fallot
2020, JACC: Case Reports
Multicenter Comparison of Percutaneous and Surgical Pulmonary Valve Replacement in Large RVOT
2020, Annals of Thoracic Surgery
A percutaneous approach for pulmonary valve replacement (PVR) is a feasible alternative to surgical PVR in selected patients with severe pulmonary regurgitation after repair of tetralogy of Fallot. However, large right ventricular outflow tract (diameter ≥ 25 mm) remains challenging.
This retrospective multicenter study enrolled consecutive patients with large right ventricular outflow tract who underwent percutaneous PVR (Venus P-valve, Venus MedTech Inc, Hangzhou, China) (n = 35) or surgical PVR (homograft valve; n = 30) between May 2014 and April 2017. Patients were followed up at 1, 3, 6, and 12 months, and yearly thereafter. Main study outcomes were pulmonary valve function and right ventricular function at discharge and midterm follow-up.
PVR was successful in all patients. Percutaneous compared with surgical PVR group had: similarly distributed baseline characteristics; shorter hospitalization, intensive care unit stay, and endotracheal intubation duration; lower cost; lower pulmonary valve gradient before discharge; lower pulmonary valve regurgitant grade (mean difference, –0.63; 95% CI –1.11 to –0.20, P = .022), pulmonary valve gradient (mean difference, –5.7 mm Hg; 95% CI –9.4 to –2.2 mm Hg, P = .005), and right ventricular end-diastolic volume index (mean difference, –9.5 mL/m²; 95% CI –16.9 to –3.1 mL/m², P = .022); and greater right ventricular ejection fraction (mean difference, 5.4%; 95% CI 2.4%-8.3%, P = .002) at median 36 months follow-up, without deaths in either group.
Percutaneous PVR using Venus P-valve appeared to be a safe, efficacious and minimally invasive alternative to surgical PVR in selected patients with large right ventricular outflow tract yielding better right ventricular and pulmonary valve function at midterm follow-up.
Outcome after surgical repair of tetralogy of Fallot: A systematic review and meta-analysis
2020, Journal of Thoracic and Cardiovascular Surgery
Citation Excerpt :
The search yielded 4105 unique articles of which 143 matched our inclusion criteria (Figure 1). These reported on 137 different cohorts comprising 21,427 unique patients spanning operative cohorts from 1951 until 2017 (Table 1).15-167 The pooled overall mean age at correction was 3.7 ± 5.6 years, and mean follow-up time was 8.6 ± 6.1 years (total follow-up 147,430 patient-years).
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Intracardiac correction was pioneered by Walton Lillehei in 1955 and has since then gone through major developments. The aim of this study was to provide a systematic literature review of published results on the long-term outcome of complete surgical correction of TOF.
MEDLINE, PubMed, Embase, Web of Science, Cochrane, and Google Scholar were systematically searched for literature published between January 2000 and July 2018. Pooled estimates with a random effects model after log-transformation were calculated for mortality and reintervention. Potential heterogeneity was assessed by subgroup analyses and meta-regression.
A total of 143 papers of 137 distinct cohorts comprising 21,427 patients and total follow-up duration of 147,430 patient-years were included. Overall mean age at correction was 3.7 ± 5.6 years, but excluding papers exclusively focusing on correction in adults yielded a mean age of 0.5 ± 2.5 years at correction. Previous palliative shunts (107 studies), a transventricular approach (81 studies), and a transannular patch (124 studies) were used in 16% (range 0%-78%), 39% (range 0%-100%), and 49% (range 0%-100%) of the patients. respectively. In case a transannular patch was used, monocusp reconstruction was applied in 15% (range 0%-100%) (49 studies). The most common genetic abnormality was Down syndrome, with a pooled estimated prevalence of 4.6% (range 0%-12.3%). The pooled estimates of early and late mortality were 2.84% (95% confidence interval [CI], 2.34-3.45) and 0.42%/year (95% CI, 0.33-0.54), respectively. The pooled estimate of late cardiac mortality was 0.26%/year (95% CI, 0.21-0.34). Valve-related mortality and non–valve-related mortality had pooled estimates of 0.20%/year (95% CI, 0.15-0.26) and 0.17%/year (95% CI, 0.12-0.22), respectively. The pooled estimate of reintervention was 2.26%/year (95% CI, 1.86-2.75).
TOF can be surgically corrected at a young age with low perioperative and long-term mortality. Life-long intensive follow-up and substantial reintervention rates characterize the clinical course.
Anesthetic Considerations for Transcatheter Pulmonary Valve Replacement
2018, Journal of Cardiothoracic and Vascular Anesthesia
The introduction of transcatheter therapy for valvular heart disease has revolutionized the care of patients with valvular disorders. Pathologic regurgitation or stenosis of the pulmonary valve, right ventricular outflow tract, or a right ventricle-to-pulmonary artery conduit represent emerging indications for transcatheter therapy. To date, minimal literature exists detailing the anesthetic management of patients undergoing transcatheter pulmonary valve replacement. In this review, the pathophysiology and indications for transcatheter pulmonary valve replacement and possible complications unique to this procedure are reviewed. Anesthetic management, including preoperative assessment, intraoperative considerations, and early postoperative monitoring, are discussed.

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Original article: cardiovascularRight ventricular dysfunction and pulmonary valve replacement after correction of tetralogy of fallot

Abstract

Section snippets

Population

Follow-up

Comment

Ann Thorac Surg

J Thorac Cardiovasc Surg

Am J Cardiol

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Am J Cardiol

J Am Coll Cardiol

Ann Thorac Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

Original article: cardiovascular
Right ventricular dysfunction and pulmonary valve replacement after correction of tetralogy of fallot