Greater Pulmonary Regurgitation Research Articles

Short- and Mid-Term Results of Pulmonary Valve Replacement with the Inspiris Valve

BackgroundVarious bioprosthetic valves are used off-label for pulmonary valve replacement (PVR), but there is no consensus on whether a particular valve is best for this application. Recently, the Inspiris Resilia valve (Edwards Lifesciences Inc) was approved for aortic valve replacement, and surgeons have begun using it for PVR. There is limited evidence on the performance of the Inspiris valve compared with other valves in the pulmonary position. MethodsThis study reviewed all patients who underwent PVR with a size 19- to 29-mm Inspiris valve or Mosaic valve (Medtronic Inc) from 2007 to 2022 at Lucile Packard Children's Hospital Stanford (Palo Alto, CA). Midterm outcomes included freedom from moderate or severe pulmonary regurgitation (PR), a maximum Doppler gradient ≥36 mm Hg, and freedom from reintervention. ResultsA total of 225 consecutive patients who underwent PVR with a size 19- to 29-mm Mosaic (n = 163) or Inspiris (n = 62) valve were included. There was no difference in baseline characteristics. Early postoperative gradients were low in both groups but higher in the Mosaic cohort, and neither group had more than mild PR on discharge. On univariable and multivariable analysis, Inspiris valves were significantly more likely to develop moderate or greater PR over time. There was no significant difference between the valves in freedom from reintervention or from a maximum gradient ≥36 mm Hg. ConclusionsEarly and short-term gradients were similar in patients undergoing PVR with Inspiris and Mosaic valves, but significant PR was more common in patients who received an Inspiris valve. These preliminary findings suggest that the durability of the Inspiris valve in the pulmonary position may not be superior to that of other bioprosthetic valves used for PVR.

Electromechanical Dyssynchrony Is Associated With Right Ventricular Remodeling and Dysfunction Independently of Pulmonary Regurgitation Late After Tetralogy of Fallot Repair

The aim of this study was to investigate the relationship among right ventricular (RV) dilatation, dysfunction, and electromechanical dyssynchrony (EMD) in patients with repaired tetralogy of Fallot (rTOF). Data from a prospective rTOF registry of subjects with moderate or greater pulmonary regurgitation (PR) and contemporary imaging were analyzed. Electrocardiograms and echocardiograms were analyzed for EMD (prolonged QRS duration [QRSd], echocardiographic septal flash, and mechanical delay) and mechanical dispersion. The relationship among these, RV measurements on cardiac magnetic resonance, exercise capacity, and incident arrhythmia or death was analyzed with adjustment for PR. In total, 271 patients with rTOF (42% women; median age, 32years; interquartile range [IQR], 23-34 years) were included. Patients had moderate to severe PR (median PR fraction, 38%; IQR, 30%-47%), moderate to severe RV enlargement (median RV end-diastolic volume index, 161 mL/m2; IQR, 138-186 mL/m2) and mild RV systolic dysfunction (median RV ejection fraction [RVEF], 44%; IQR, 38%-48%). Eleven patients (4%) experienced ventricular arrhythmia or death. Presence of EMD was associated with larger RV size (RV end-diastolic volume index and RV end-systolic volume index, P=.006 and P<.001, respectively) and lower RVEF (P<.001). A sharp inflection in the relation among QRSd, RV size, and RVEF was observed when QRSd exceeded 150msec (3.1% decrease in RVEF for every 20-msec increase in QRSd between 160 and 200msec). Similar inflection points were observed for the mechanical delay between the RV basal-lateral and midseptal segments. The mechanical delay was higher in patients with vs without incident atrial arrhythmia (371 vs 276msec, P=.014). In adults with rTOF, EMD is independently associated with larger RV size, lower RVEF, and incident atrial arrhythmias.

Patient-Reported Outcomes After Tetralogy of Fallot Repair

BackgroundComprehensive assessment of tetralogy of Fallot (TOF) outcomes extends beyond morbidity and mortality to incorporate patient-reported outcomes (PROs), including quality of life (QOL) and health status (HS). ObjectivesThis study explored PROs in adolescents and adults with TOF and delineated variables associated with PROs. MethodsThis was a cross-sectional observational study within a larger prospective registry of adolescents and adults with repaired TOF and moderate or greater pulmonary regurgitation from North America, Europe, and Asia. Participants completed PROs, including a QOL linear analogue scale (QOL-LAS) and an HS visual analogue scale (HS-VAS). Scores were classified according to age cohorts: <18, 18 to 25, 26 to 40, and >40 years. ResultsThe study included 607 patients (46.3% female; median age 28.5 years). Median QOL-LAS scores (0-100) were similar across age cohorts (85, 80, 80, 80; P = 0.056). Median HS-VAS scores (0-100) were lowest for the oldest cohort (77) compared with the 3 younger cohorts (85, 80, 80) (P = 0.004). With advancing age, there were increased reports of poor mobility (P < 0.001) and pain or discomfort (P = 0.004); problems in these dimensions were reported by 19.1% and 37.2% of patients aged >40 years, respectively. Of factors associated with superior PROs on multivariable regression modeling (ie, being White, being nonsyndromic, having employment, and having better left ventricular function; P < 0.05), asymptomatic status (functional class I) was the variable associated with the greatest number of QOL and HS measures (P < 0.001). ConclusionsStrategies to improve TOF outcomes should consider PROs alongside conventional clinical variables. Factors associated with poorer PROs represent opportunities to intervene to improve the lives of patients with TOF.

Outcomes of pulmonary valve leaflet augmentation for transannular repair of tetralogy of Fallot

ObjectiveTo evaluate the midterm results of pulmonary valve leaflet augmentation in transannular repair of tetralogy of Fallot (TOF). MethodsFrom 2007 to 2019, 131 patients underwent a transannular repair with pulmonary leaflet augmentation for TOF (n = 120) and double outlet right ventricle with pulmonary stenosis (n = 11). Patch material was expanded polytetrafluoroethylene (n = 76), glutaraldehyde-treated autologous pericardium (n = 47) and bovine pericardium (n = 8). ResultsMedian age at repair was 8.9 months (interquartile range, 5.4-14.8). There was no operative mortality. Median follow-up was 6.25 years (interquartile range, 2.77-7.75). Freedom from severe pulmonary regurgitation (PR) was 85% (95% confidence interval [CI], 77%-90%) and 76% (95% CI, 66%-83%) at 1 and 5 years, respectively. Freedom from moderate or greater PR was 69% (95% CI, 60%-76%) and 30% (95% CI, 21%-39%) at 5 and 10 years, respectively. Three patients required pulmonary valve replacement for PR. Nine patients required pulmonary balloon valvuloplasty. Freedom from intervention for pulmonary valve stenosis was 98% (95% CI, 93%-99%) and 94% (95% CI, 87%-97%) at 1 and 5 years, respectively. One patient with severe PR had an indexed right ventricular volume >160 mL/m2. Use of expanded polytetrafluoroethylene resulted in a greater freedom from moderate or greater PR (log-rank test P < .001; Cox regression hazard ratio, 0.40; 95% CI, 0.25-0.63; P < .001). ConclusionsAt midterm follow-up of transannular repair with pulmonary valve leaflet augmentation, severe PR occurs in less than 50% of patients. The expanded polytetrafluoroethylene patch performs better than pericardium.

Transcatheter Pulmonary Valve Replacement With the Sapien Prosthesis

BackgroundThere are limited published data focused on outcomes of transcatheter pulmonary valve replacement (TPVR) with either a Sapien XT or Sapien 3 (S3) valve. ObjectivesThis study sought to report short-term outcomes in a large cohort of patients who underwent TPVR with either a Sapien XT or S3 valve. MethodsData were entered retrospectively into a multicenter registry for patients who underwent attempted TPVR with a Sapien XT or S3 valve. Patient-related, procedural, and short-term outcomes data were characterized overall and according to type of right ventricular outflow tract (RVOT) anatomy. ResultsTwenty-three centers enrolled a total of 774 patients: 397 (51%) with a native/patched RVOT; 183 (24%) with a conduit; and 194 (25%) with a bioprosthetic valve. The S3 was used in 78% of patients, and the XT was used in 22%, with most patients receiving a 29-mm (39%) or 26-mm (34%) valve. The implant was technically successful in 754 (97.4%) patients. Serious adverse events were reported in 67 patients (10%), with no difference between RVOT anatomy groups. Fourteen patients underwent urgent surgery. Nine patients had a second valve implanted. Among patients with available data, tricuspid valve injury was documented in 11 (1.7%), and 9 others (1.3%) had new moderate or severe regurgitation 2 grades higher than pre-implantation, for 20 (3.0%) total patients with tricuspid valve complications. Valve function at discharge was excellent in most patients, but 58 (8.5%) had moderate or greater pulmonary regurgitation or maximum Doppler gradients >40 mm Hg. During limited follow-up (n = 349; median: 12 months), 9 patients were diagnosed with endocarditis, and 17 additional patients underwent surgical valve replacement or valve-in-valve TPVR. ConclusionsAcute outcomes after TPVR with balloon-expandable valves were generally excellent in all types of RVOT. Additional data and longer follow-up will be necessary to gain insight into these issues.

Alterra Adaptive Prestent and SAPIEN 3 THV for Congenital Pulmonic Valve Dysfunction

The aim of this study was to demonstrate the safety and functionality of the Alterra Adaptive Prestent and SAPIEN 3 transcatheter heart valve (THV) in patients with dysfunctional, dilated right ventricular outflow tract (RVOT) greater or equal to moderate pulmonary regurgitation (PR). Significant variations in the size and morphology of the RVOT affect the placement of transcatheter pulmonary valves. The Alterra Prestent internally reduces and reconfigures the RVOT, providing a stable landing zone for the 29-mm SAPIEN 3 THV. Eligible patients had moderate or greater PR, weighed >20kg, and had RVOT diameter 27 to 38mm and length >35mm. The primary endpoint was device success, a 5-item composite: 1 Alterra Prestent deployed in the desired location, 1 SAPIEN 3 THV implanted in the desired location within the Prestent, right ventricular-to-pulmonary artery peak-to-peak gradient<35mmHg after THV implantation, less than moderate PR at discharge, and no explantation 24h post-implantation. The secondary composite endpoint was freedom from THV dysfunction (RVOT/pulmonary valve (PV) reintervention, greater or equal to moderate total PR, mean RVOT/PV gradient ≥ 35 mm Hg at 30days and 6months. Descriptive statistics are reported. Enrolled patients (N=15) had a median age and weight of 20 years and 61.7kg, respectively; 93.3% were in New York Heart Association functional class I or II. Device success was 100%. No staged procedures were necessary. No THV dysfunction was reported to 6months. No serious safety signals were reported. This early feasibility study demonstrated the safety and functionality of the Alterra Adaptive Prestent in patients with congenital RVOT dysfunction and moderate or greater PR. Durability and long-term outcome data are needed.

Experience and Outcomes of Surgically Implanted Melody Valve in the Pulmonary Position

Multiple congenital cardiac malformations require pulmonary valve replacement and/or right ventricular outflow (RVOT) reconstruction. Pulmonary valve replacement remains challenging in children owing to the limited growth potential of prosthetic valves. We evaluated outcomes in patients undergoing surgical implantation of a Melody valve in the RVOT. Data were retrospectively collected for 23 patients undergoing surgical Melody valve implantation at Boston Children's Hospital between 2009 and 2019. We assessed postoperative valve function, reintervention rates, and mortality. Median age was 1.7 years (range, 2 months to 6 years); 12 patients were aged greater than 2 years (52%). Diagnosis was tetralogy of Fallot in 15 patients (65%); 15 had a prior RVOT operation (65%). The Melody valve was dilated before surgery to a median diameter of 14 mm (range, 10-20 mm). No patients had acute pulmonary regurgitation. One required transcatheter RVOT reintervention before discharge. Median follow-up was 3.7 years (range, 0.02-8.7 years) with moderate or greater pulmonary regurgitation in 2 patients. Catheter-based interventions (mean, 0.83 ± 1.07/patient) occurred at a median of 1 year (range, 16 days to 5.4 years) and included valve expansion for somatic growth (n= 10) and subsequent valve-in-valve replacement (n= 3). Three patients (13%) required surgical valve explant or replacement at a median of 1.0 year (range, 0.6-3.7 years) for Melody-specific indications. One-, 3-, and 5-year freedom from Melody-driven reoperation was 90%, 90%, and 83%, respectively. The Melody valve can be surgically implanted in the RVOT of young patients with acceptable early results. These valves can be successfully dilated through transcatheter reintervention to accommodate growth.

Usefulness of Pulmonary Arterial End-Diastolic Forward Flow Late After Tetralogy of Fallot Repair to Predict a “Restrictive” Right Ventricle

The functional significance of pulmonary arterial end-diastolic forward flow (EDFF) in patients with repaired tetralogy of Fallot (rTOF) is not fully understood, with conflicting reports regarding its associations with pulmonary regurgitation (PR), right ventricular (RV) size and function, and so-called restrictive RV physiology. To examine these associations, we retrospectively analyzed 399 patients with rTOF who had contemporaneous echocardiography (Echo) and cardiovascular magnetic resonance (CMR) studies. The median age at TOF repair was 0.7 years (0.21, 2.66), age at CMR was 19.8 years (13.0, 29.4), and interval between Echo and CMR was 48 days (0, 182). Doppler identified EDFF in 122 (31%) patients and CMR in 113 patients (28%). Compared with those without EDFF, patients with EDFF were younger, had greater PR, and higher RV end-diastolic volume, stroke volume, and ejection fraction. Markers of RV restriction such as right atrial size did not differ between groups. On multivariable regression, EDFF was associated with higher RV stroke volume and lower left ventricular end-diastolic volume. The association between Echo and CMR measurements of EDFF was modest (area under the receiver operating characteristic curve = 0.684, r = 0.374, p < 0.001). In conclusion, EDFF was common in this large cohort of patients with rTOF, but its presence and extent varied between Echo and CMR. EDFF was associated with greater PR and larger RV size, but not with markers of poor RV compliance such as right atrial enlargement. Mechanisms beyond RV noncompliance may contribute to the presence of EDFF.

Long-Term Outcomes of Balloon Valvuloplasty for Isolated Pulmonary Valve Stenosis.

To evaluate the long-term cumulative incidence of ≥ moderate pulmonary regurgitation (PR) and re-intervention following balloon pulmonary valvuloplasty (BPV). While BPV for pulmonary valve stenosis (PS) relieves obstruction acutely, long-term outcomes are not well documented. Between 1982 and 2002, 211 patients had BPV for isolated PS. Follow-up data were available for 103 patients. Cumulative incidence of ≥ moderate PR and re-intervention was evaluated and risk factors for ≥ moderate PR assessed in univariate and multivariable analyses. Median age at BPV was 0.7years (range 1day-42.2years); peak catheter gradient was 65mmHg (range 31-169mmHg); 23% had critical PS. Sixty-two patients had a recent echocardiogram with median follow-up 15.1years (range 10.1-26.3years); 60% had ≥ moderate PR. Three patients had pulmonary valve replacement following BPV due to symptomatic severe PR. In univariate analysis, critical PS, younger age, smaller BSA, and smaller pulmonary annulus at the time of BPV, as well as greater baseline PS gradient by catheterization, were associated with ≥ moderate PR (all p<0.05). In multivariable analysis, only BSA<0.3m2 was independently associated with ≥ moderate PR (adjusted odds ratio 6.4, 95% confidence interval 1.2-33.6). In the largest study to date of > 10-year outcomes following BPV, 60% of patients with available follow-up data developed ≥ moderate PR. Few patients had pulmonary valve replacement. Patients with lower BSA at the time of BPV were more likely to have greater PR at late follow-up.

Abstract 12430: Vicious Circle Between Progressive Right Ventricular Dilatation and Pulmonary Regurgitation in Patients After Tetralogy of Fallot Repair? Right Heart Enlargement Promotes Flow Reversal in the Left Pulmonary Artery

Introduction: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well known. We hypothesized that unilaterally increased pulmonary vascular resistance (PVR), resulting from lung compression by the enlarged and levorotated heart leads to greater PR in the LPA. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: This is a single-center retrospective analysis of 50 magnetic resonance studies in patients after TOF repair. Patients with more than mild discrete branch pulmonary artery stenosis were excluded. Blood flow was measured by phase-contrast velocity encoding within the branch pulmonary arteries. On the axial image with the largest total cardiac surface area, cardiac angle (α) between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured (Figure). Results: There was no difference in LPA and RPA diameters. The LPA showed significantly less total forward flow (p=0.04), smaller net forward flow (p=&lt;0.001), and greater RF (p=0.001) than the RPA. Left lung area was smaller than the right (p&lt;0.001). RVEDVi correlated with LPA RF (R=0.48, p&lt;0.001), but not with RPA RF. Larger RVEDVi correlated with a larger α angle (R=0.46, p&lt;0.001), i.e. a more leftward cardiac axis and with smaller left lung area (R=-0.58, p&lt;0.001). LPA RF, but not RPA RF, correlated inversely with left lung area indexed to the left hemithorax area (R=-0.34, p=0.02). Conclusions: An enlarged and levorotated heart - as a result of PR - is associated with smaller left lung size, and augments diastolic flow reversal in the LPA, presumably via increased left PVR. By imposing a further volume load on the RV, LPA regurgitation may thus close a positive feed-back loop of PR and RV dilatation.

Long-term outcomes after definitive repair for tetralogy of Fallot with preservation of the pulmonary valve annulus

The aim of our study was to evaluate the long-term outcomes after definitive repair of tetralogy of Fallot with preservation of the pulmonary valve (PV) annulus. From 1989 to 2000, 84 of 222 patients (37.8%) with tetralogy of Fallot and PV stenosis underwent definitive repair with preservation of the PV annulus without right ventriculotomy. PV commissurotomy was concomitantly performed in 74 patients (88.1%). The PV was bicuspid in 56 patients (66.7%); the mean Z value was -1.2 ± 1.5 (range, -4.9 to 2.4). The mean follow-up period was 15.8 ± 5.7 years (maximum, 22.8), and follow-up data were complete for 75 patients (89.3%). The actuarial survival and freedom from reoperation rates at 20 years was 98.6% and 95.8%. The freedom from ventricular arrhythmia at 5, 10, 15, and 20 years was 98.7%, 89.6%, 74.1%, and 58.0%, respectively. All detected ventricular arrhythmias were isolated monofocal premature ventricular contractions. Freedom from moderate or greater pulmonary regurgitation at 5, 10, 15 and 20 years was 50.4%, 44.9%, 38.4%, and 35.7%, respectively. A bicuspid PV (hazard ratio, 2.910; 95% confidence interval, 1.404-6.204, P = .004) and a Z-value of less than -2 (hazard ratio, 1.948; 95% confidence interval, 0.915-5.857; P = .034) were the risk factors for developing moderate or greater pulmonary regurgitation. The long-term outcomes after definitive repair of tetralogy of Fallot with preservation of the PV annulus were excellent. Although isolated, monofocal premature ventricular contractions were frequently observed, fatal ventricular arrhythmia was not. The indication should not only be decided by the PV annulus size, but also by the valvular morphology to maintain long-term PV competency.

Pulmonary Valve Replacement Function in Adolescents: A Comparison of Bioprosthetic Valves and Homograft Conduits

Data comparing function and reintervention rates of various pulmonary valve replacements in adolescents are sparse. New transcatheter therapies-including transcatheter pulmonary valves-have been developed, and their utilization could be justified by these data. We performed a retrospective review that included baseline and operative data with cross-sectional follow-up on adolescents aged 10 to 21 years who underwent pulmonary valve replacement (n=254). We compared homograft conduits (n=84) with bioprosthetic pulmonary valves (n=170) in terms of freedom from valve dysfunction--right ventricular outflow tract obstruction greater than 50 mm Hg, or moderate or greater pulmonary regurgitation--and reintervention. Median follow-up duration was 4.4 years and was longer in the homograft cohort. Freedom from valvar dysfunction was 72%±4% at 5 years and 48%±8% at 10 years. Freedom from right ventricular outflow tract reintervention was 90%±3% at 5 years, 67%±5% at 10 years, and 47%±8% at 15 years. No differences existed for dysfunction or reintervention between the homograft and bioprosthetic valve cohorts. Shorter freedom from dysfunction was associated with younger age, smaller implanted valve Z-score, valve replacement after prior homograft, and higher preoperative right ventricular outflow tract (RVOT) gradient. Shorter freedom from reintervention was associated with younger age, smaller implanted valve Z-score, and more recent valve implant. Adolescents represent an important age group as an increasing number are undergoing pulmonary valve replacement, and they may be particularly impacted by new transcatheter therapies. This study provides baseline comparative data for current surgical options that should help inform medical decision making as longer term data become available for transcatheter pulmonary valve replacement and other new technologies.