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Cardiology in the Young Apr 2024Pulmonary regurgitation is the most common complication in repaired tetralogy of Fallot patients. Severe chronic pulmonary regurgitation can be tolerated for decades,...
BACKGROUND AND AIM
Pulmonary regurgitation is the most common complication in repaired tetralogy of Fallot patients. Severe chronic pulmonary regurgitation can be tolerated for decades, but if not treated, it can progress to symptomatic, irreversible right ventricular dilatation and dysfunction. We investigated clinical associations with pulmonary valve replacement among patients with significative pulmonary regurgitation and how interventional developments can change their management.
METHODS
All adult patients with repaired tetralogy of Fallot who were followed at an adult CHD Clinic at a single centre from 1980 to 2022 were included on their first outpatient visit. Follow-up was estimated from the time of correction surgery until one of the following events occurred first: pulmonary valve replacement, death, loss to follow-up or conclusion of the study.
RESULTS
We included 221 patients (116 males) with a median age of 19 (18-25). At a median age of 33 (10) years old, 114 (51%) patients presented significant pulmonary regurgitation. Among patients with significant pulmonary regurgitation, pulmonary valve replacement was associated with male gender, older age at surgical repair, and longer QRS duration in adulthood. Pulmonary valve replacement was performed in 50 patients, including four transcatheter pulmonary valve implantations, at a median age of 34 (14) years.
CONCLUSION
Pulmonary regurgitation affects a large percentage of tetralogy of Fallot adult patients, requiring a long-term clinical and imaging follow-up. Sex, age at surgical repair and longer QRS are associated with the need of PVR among patients with significative pulmonary regurgitation. Clinical practice and current literature support TPVI as the future gold standard intervention.
Topics: Adult; Humans; Male; Pulmonary Valve; Pulmonary Valve Insufficiency; Tetralogy of Fallot; Heart Valve Prosthesis Implantation; Cardiac Surgical Procedures; Treatment Outcome; Retrospective Studies
PubMed: 37921218
DOI: 10.1017/S1047951123003608 -
Heart (British Cardiac Society) Jul 2022Complications and need for reinterventions are frequent in patients with pulmonary valve stenosis (PVS). Pulmonary regurgitation is common, but no data are available on...
BACKGROUND
Complications and need for reinterventions are frequent in patients with pulmonary valve stenosis (PVS). Pulmonary regurgitation is common, but no data are available on outcome after pulmonary valve replacement (PVR).
METHODS
We performed a retrospective analysis of 215 patients with PVS who underwent surgical valvotomy or balloon valvuloplasty. Incidence and predictors of reinterventions and complications were identified. Right ventricle (RV) remodelling after PVR was also assessed.
RESULTS
After a median follow-up of 38.6 (30.9-49.4) years, 93% of the patients were asymptomatic. Thirty-nine patients (18%) had at least one PVR. Associated right ventricular outflow tract (RVOT) intervention and the presence of an associated defect were independent predictors of reintervention (OR: 4.1 (95% CI 1.5 to 10.8) and OR: 3.6 (95% CI 1.9 to 6.9), respectively). Cardiovascular death occurred in 2 patients, and 29 patients (14%) had supraventricular arrhythmia. Older age at the time of first intervention and the presence of an associated defect were independent predictors of complications (OR: 1.0 (95% CI 1.0 to 1.1) and OR: 2.1 (95% CI 1.1 to 4.2), respectively). In 16 patients, cardiac magnetic resonance before and after PVR was available. The optimal cut-off values for RV volume normalisation were 193 mL/m for RV end-diastolic volume indexed(sensitivity 80%, specificity 64%) and 100 mL/m for RV end-systolic volume indexed(sensitivity 80%, specificity 56%).
CONCLUSIONS
Previous RVOT intervention, presence of an associated defect and older age at the time of first repair were predictors of outcome. More data are needed to guide timing of PVR, and extrapolation of tetralogy of Fallot guidelines to this population is unlikely to be appropriate.
Topics: Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Pulmonary Valve Stenosis; Retrospective Studies; Tetralogy of Fallot; Treatment Outcome; Ventricular Function, Right; Ventricular Remodeling
PubMed: 34815333
DOI: 10.1136/heartjnl-2021-320121 -
The Canadian Journal of Cardiology Sep 2017Few topics in adult congenital heart disease have approached the level of scrutiny bestowed on pulmonary valve replacement (PVR) strategies late after tetralogy of... (Review)
Review
Few topics in adult congenital heart disease have approached the level of scrutiny bestowed on pulmonary valve replacement (PVR) strategies late after tetralogy of Fallot (TOF) repair. Despite the successes of primary surgery for TOF, there is a growing group of adults with residual right ventricular outflow tract and pulmonary valve dysfunction. Patients with residual chronic pulmonic regurgitation as a consequence of earlier surgery can later develop symptoms of exercise intolerance and complications including heart failure, tachyarrhythmias, and sudden cardiac death. Optimal timing of PVR has sparked debate, which has catalyzed increasing research efforts over the past decade. Although performance of PVR in the absence of symptoms is currently on the basis of the rationale that achievement of complete reverse remodelling is highly desirable, whether this approach results in improvement in patient outcomes in the long-term has yet to be shown. Surgical PVR and percutaneous pulmonary valve intervention are different techniques with specific advantages and disadvantages that require careful consideration for each individual patient, alongside the need for requisite reinterventions over the course of a patient's lifetime. Criteria pertaining to referral strategies are ever being refined as newer technologies for percutaneous therapies continue to evolve. In this article we review the literature surrounding the indications for, the optimal timing of, and the approaches to pulmonary valve procedures in adults with previously repaired TOF.
Topics: Cardiac Surgical Procedures; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Reoperation; Tetralogy of Fallot
PubMed: 28843325
DOI: 10.1016/j.cjca.2017.06.011 -
Heart (British Cardiac Society) Oct 2016Residual right ventricular outflow tract and pulmonary valve disease is common in adults with repaired tetralogy of Fallot. Chronic severe pulmonary regurgitation as a... (Review)
Review
Residual right ventricular outflow tract and pulmonary valve disease is common in adults with repaired tetralogy of Fallot. Chronic severe pulmonary regurgitation as a result of surgical repair can lead to myriad complications including right ventricular dysfunction, decreased exercise tolerance, right heart failure and symptomatic arrhythmias. The aim of restoring pulmonary valve integrity is to preserve right ventricular size and function with the intent of mitigating the development of symptoms and poor long-term outcomes. Right ventricular size thresholds by cardiac MRI have emerged beyond which reverse right ventricular remodelling after pulmonary valve replacement is less likely. Though pulmonary valve replacement has been shown to improve right ventricular dimensions and symptoms, no consistent improvement in right ventricular ejection fraction or objective measures of exercise capacity have been demonstrated. Furthermore, there are no long-term studies showing that normalisation of right ventricular size results in improved clinical outcomes. New transcatheter techniques of percutaneous pulmonary valve replacement have emerged with good short-term and mid-term outcomes, further adding to the complexity in determining 'when' and 'how' right ventricular outflow tract and pulmonary valve intervention should occur. With improved survival of these patients, the trend towards earlier pulmonary valve replacement at smaller right ventricular size and rapidly evolving transcatheter pulmonary valve techniques, the clinician must balance the goals of preserving right ventricular size and function in an attempt to prevent untoward outcomes with risks of multiple interventions in a patient's lifetime.
Topics: Cardiac Catheterization; Cardiac Surgical Procedures; Chronic Disease; Exercise Tolerance; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Patient Selection; Pulmonary Valve; Pulmonary Valve Insufficiency; Risk Assessment; Risk Factors; Severity of Illness Index; Tetralogy of Fallot; Time Factors; Time-to-Treatment; Treatment Outcome; Ventricular Function, Right
PubMed: 27329296
DOI: 10.1136/heartjnl-2015-309067 -
JACC. Cardiovascular Interventions Nov 2014
Topics: Cardiac Catheterization; Cardiac Surgical Procedures; Female; Heart Defects, Congenital; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Male; Pulmonary Valve; Pulmonary Valve Insufficiency; Pulmonary Valve Stenosis
PubMed: 25459039
DOI: 10.1016/j.jcin.2014.10.002 -
JACC. Cardiovascular Interventions Aug 2023The Harmony transcatheter pulmonary valve (TPV) is the first U.S. Food and Drug Administration-approved device for severe pulmonary regurgitation (PR) in the native or... (Clinical Trial)
Clinical Trial
BACKGROUND
The Harmony transcatheter pulmonary valve (TPV) is the first U.S. Food and Drug Administration-approved device for severe pulmonary regurgitation (PR) in the native or surgically repaired right ventricular outflow tract (RVOT).
OBJECTIVES
One-year safety and effectiveness of the Harmony TPV were evaluated in patients from the Harmony Native Outflow Tract Early Feasibility Study, Harmony TPV Pivotal Study, and Continued Access Study, representing the largest cohort to date of Harmony TPV recipients.
METHODS
Eligible patients had severe PR by echocardiography or PR fraction ≥ 30% by cardiac magnetic resonance imaging and clinical indications for pulmonary valve replacement. The primary analysis included 87 patients who received a commercially available TPV22 (n = 42) or TPV25 (n = 45) device; 19 patients who received an early device iteration prior to its discontinuation were evaluated separately.
RESULTS
In the primary analysis, median patient age at treatment was 26 years (IQR: 18-37 years) in the TPV22 group and 29 years (IQR: 19-42 years) in the TPV25 group. At 1 year, there were no deaths; 98% of TPV22 and 91% of TPV25 patients were free from the composite of PR, stenosis, and reintervention (moderate or worse PR, mean RVOT gradient >40 mmHg, device-related RVOT reoperation, and catheter reintervention). Nonsustained ventricular tachycardia occurred in 16% of patients. Most patients had none/trace or mild PR (98% of TPV22 patients, 97% of TPV25 patients). Outcomes with the discontinued device are reported separately.
CONCLUSIONS
The Harmony TPV device demonstrated favorable clinical and hemodynamic outcomes across studies and valve types through 1 year. Further follow-up will continue to assess long-term valve performance and durability.
Topics: Humans; Cardiac Catheterization; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Prospective Studies; Pulmonary Valve; Pulmonary Valve Insufficiency; Treatment Outcome; Ventricular Outflow Obstruction
PubMed: 37278682
DOI: 10.1016/j.jcin.2023.03.002 -
Cardiology Journal 2017Transcutaneous pulmonary valve replacement (TPVR) has become an alternative to heart surgery for patients after previous right ventricular outflow tract (RVOT) or...
BACKGROUND
Transcutaneous pulmonary valve replacement (TPVR) has become an alternative to heart surgery for patients after previous right ventricular outflow tract (RVOT) or pulmonary artery (PA) surgical interventions. The objective was to present immediate and long-term outcomes of trans¬cutaneous pulmonary valve replacement.
METHODS
Between 06/2009 and 06/2016, 46 patients underwent TPVR. Initial diagnoses included tetralogy of Fallot, common arterial trunk, transposition of great arteries post Rastelli correction, left ventricle outflow obstruction after Ross operation, pulmonary atresia, and isolated dysplastic pulmonary valve stenosis. Thirty eight (78%) patients had previously implanted conduits in the pulmonary position, the rest had either RVOT patch reconstruction (n = 6; 13%) or biological valve implantation (n = 2; 4%). They presented primarily with pulmonary stenosis (n = 18; 39%) or regurgitation (n = 28; 60%).
RESULTS
All procedures were successful - 44 Melody and 2 Edwards-Sapien valves were implanted. Before each procedure exclusion of potential coronary compression and RVOT prestenting was performed. Significant RVOT systolic gradient reduction (from 35.3 ± 19.5 to 13.5 ± 7.1 mm Hg; p < 0.001) and decrease of right to left ventricle systolic pressure ratio from 0.58 ± 0.18 to mean 0.37 ± 0.1 (p < 0.001) was achieved. Also, in every patient PA-RVOT competence was restored, with minor in¬competence in only a few patients. Post procedure follow-up ranged from 2 to 86 (mean 35.2) months. Follow-up fluoroscopy or chest X-ray revealed 6 stent fractures (2 stent defragmentation - with only 1 significant valve stenosis).
CONCLUSIONS
Transcutaneous pulmonary valve replacement is a safe procedure with encouraging results, it also enables deferring surgical reintervention in the majority of patients.
Topics: Adolescent; Adult; Cardiac Catheterization; Child, Preschool; Female; Fluoroscopy; Follow-Up Studies; Heart Valve Prosthesis Implantation; Humans; Male; Prosthesis Design; Pulmonary Valve; Pulmonary Valve Insufficiency; Pulmonary Valve Stenosis; Retrospective Studies; Time Factors; Treatment Outcome
PubMed: 28248409
DOI: 10.5603/CJ.a2017.0023 -
JACC. Cardiovascular Interventions Aug 2019
Topics: Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Treatment Outcome
PubMed: 31202945
DOI: 10.1016/j.jcin.2019.04.006 -
Texas Heart Institute Journal Sep 2022In this retrospective study, we compared the results from 2 pulmonary valve augmentation techniques in patients undergoing surgical repair of tetralogy of Fallot.
BACKGROUND
In this retrospective study, we compared the results from 2 pulmonary valve augmentation techniques in patients undergoing surgical repair of tetralogy of Fallot.
METHODS
Between 2015 and 2018, 18 patients had anterior pulmonary valve repair at our institution, and 26 patients had both anterior and posterior pulmonary valve repair.
RESULTS
Patients ranged from 6 months to 30 years of age. The median follow-up period was 8 months in the anterior augmentation group and 5 months in the anterior and posterior augmentation group. Postoperative echocardiograms indicated that only 2 patients (11%) in the anterior augmentation group had moderate or severe pulmonary insufficiency, compared with no patients in the anterior and posterior augmentation group. At follow-up, pulmonary insufficiency was seen in 3 patients (17%) in the anterior augmentation group and no patients in the anterior and posterior augmentation group.
CONCLUSION
Reconstruction of the native pulmonary valve accompanied by pulmonary cusp augmentation can decrease or even circumvent postoperative pulmonary insufficiency. Both anterior augmentation and anterior and posterior augmentation techniques are easily applied; however, we believe that the anterior and posterior augmentation technique is superior in terms of early postoperative and follow-up pulmonary insufficiency outcomes.
Topics: Humans; Infant; Pulmonary Valve; Pulmonary Valve Insufficiency; Retrospective Studies; Tetralogy of Fallot; Treatment Outcome
PubMed: 36223246
DOI: 10.14503/THIJ-20-7368 -
European Journal of Cardio-thoracic... Jan 2017Fungal isolated native pulmonary valve endocarditis is extremely uncommon. Data are scarce and report high mortality and recurrence rates. Recommended management...
Fungal isolated native pulmonary valve endocarditis is extremely uncommon. Data are scarce and report high mortality and recurrence rates. Recommended management combines both medical and surgical approaches. We report herein a rare case of isolated pulmonary valve endocarditis caused by Candida albicans The patient did not display prior heart disease. Medical management was unsuccessful. Pulmonary valve replacement allowed rapid improvement.
Topics: Antifungal Agents; Candida albicans; Candidiasis; Endocarditis; Humans; Male; Middle Aged; Pulmonary Valve Insufficiency
PubMed: 27354256
DOI: 10.1093/ejcts/ezw219