Long-Term Outcomes After Melody Transcatheter Pulmonary Valve Replacement in the US Investigational Device Exemption Trial.

BACKGROUND: The Melody valve was developed to extend the useful life of previously implanted right ventricular outflow tract (RVOT) conduits or bioprosthetic pulmonary valves, while preserving RV function and reducing the lifetime burden of surgery for patients with complex congenital heart disease. METHODS: Enrollment for the US Investigational Device Exemption study of the Melody valve began in 2007. Extended follow-up was completed in 2020. The primary outcome was freedom from transcatheter pulmonary valve (TPV) dysfunction (freedom from reoperation, reintervention, moderate or severe pulmonary regurgitation, and/or mean RVOT gradient >40 mm Hg). Secondary end points included stent fracture, catheter reintervention, surgical conduit replacement, and death. RESULTS: One hundred seventy-one subjects with RVOT conduit or bioprosthetic pulmonary valve dysfunction were enrolled. One hundred fifty underwent Melody TPV replacement. Median age was 19 years (Q1-Q3: 15-26). Median discharge mean RVOT Doppler gradient was 17 mm Hg (Q1-Q3: 12-22). The 149 patients implanted >24 hours were followed for a median of 8.4 years (Q1-Q3: 5.4-10.1). At 10 years, estimated freedom from mortality was 90%, from reoperation 79%, and from any reintervention 60%. Ten-year freedom from TPV dysfunction was 53% and was significantly shorter in children than in adults. Estimated freedom from TPV-related endocarditis was 81% at 10 years (95% CI, 69%-89%), with an annualized rate of 2.0% per patient-year. CONCLUSIONS: Ten-year outcomes from the Melody Investigational Device Exemption trial affirm the benefits of Melody TPV replacement in the lifetime management of patients with RVOT conduits and bioprosthetic pulmonary valves by providing sustained symptomatic and hemodynamic improvement in the majority of patients. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT00740870.

Comparison of the investigational device exemption and post-approval trials of the Melody transcatheter pulmonary valve.

OBJECTIVE: We compared 5-year outcomes of transcatheter pulmonary valve (TPV) replacement with the Melody TPV in the post-approval study (PAS) and the investigational device exemption (IDE) trial. BACKGROUND: As a condition of approval of the Melody TPV after the IDE trial, the Food and Drug Administration required that a PAS be conducted to evaluate outcomes of TPV replacement in a "real-world" environment. The 5-year outcomes of the PAS have not been published, and the IDE and PAS trials have not been compared. METHODS: The cohorts comprised all patients catheterized and implanted at 5 IDE sites and 10 PAS sites. Differences in trial protocols were detailed. Time-related outcomes and valve-related adverse events were compared between the two trials with Kaplan-Meier curves and log-rank testing. RESULTS: 167 patients (median age, 19 years) were catheterized and 150 underwent TPV replacement in the IDE trial; 121 were catheterized (median age, 17 years) and 100 implanted in the PAS. Freedom from hemodynamic dysfunction (p = .61) or any reintervention (p = .74) over time did not differ between trials. Freedom from stent fracture (p = .003) and transcatheter reintervention (p = .010) were longer in PAS, whereas freedom from explant (p = .020) and TPV endocarditis (p = .007) were shorter. Clinically important adverse events (AEs) were reported in 14% of PAS and 7.2% of IDE patients (p = .056); the incidence of any particular event was low in both. CONCLUSIONS: Hemodynamic and time-related outcomes in the PAS and IDE trials were generally similar, confirming the effectiveness of the Melody TPV with real-world providers. There were few significant complications and limited power to identify important differences in AEs. The lack of major differences in outcomes between the two studies questions the usefulness of mandated costly post-approval studies as part of the regulatory process for Class III medical devices.

Endocarditis After Transcatheter Pulmonary Valve Replacement.

BACKGROUND: Endocarditis has emerged as one of the most concerning adverse outcomes in patients with congenital anomalies involving the right ventricular outflow tract (RVOT) and prosthetic valves. OBJECTIVES: The aim of this study was to evaluate rates and potential risk factors for endocarditis after transcatheter pulmonary valve replacement in the prospective Melody valve trials. METHODS: All patients in whom a transcatheter pulmonary valve (TPV) was implanted in the RVOT as part of 3 prospective multicenter studies comprised the analytic cohort. The diagnosis of endocarditis and involvement of the TPV were determined by the implanting investigator. RESULTS: A total of 309 patients underwent transcatheter pulmonary valve replacement (TPVR) and were discharged with a valve in place. The median follow-up duration was 5.1 years, and total observation until study exit was 1,660.3 patient-years. Endocarditis was diagnosed in 46 patients (median 3.1 years after TPVR), and a total of 35 patients were reported to have TPV-related endocarditis (34 at the initial diagnosis, 1 with a second episode). The annualized incidence rate of endocarditis was 3.1% per patient-year and of TPV-related endocarditis was 2.4% per patient-year. At 5 years post-TPVR, freedom from a diagnosis of endocarditis was 89% and freedom from TPV-related endocarditis was 92%. By multivariable analysis, age ≤12 years at implant (hazard ratio: 2.3; 95% confidence interval: 1.2 to 4.4; p = 0.011) and immediate post-implant peak gradient ≥15 mm Hg (2.7; 95% confidence interval: 1.4 to 4.9; p = 0.002) were associated with development of endocarditis and with development of TPV-related endocarditis (age ≤12 years: 2.8; 95% confidence interval: 1.3 to 5.7; p = 0.006; gradient ≥15 mm Hg: 2.6; 95% confidence interval: 1.3 to 5.2; p = 0.008). CONCLUSIONS: Endocarditis is an important adverse outcome following TVPR in children and adults with post-operative congenital heart disease involving the RVOT. Ongoing efforts to understand, prevent, and optimize management of this complication are paramount in making the best use of TPV therapy. (Melody Transcatheter Pulmonary Valve [TPV] Study: Post Approval Study of the Original Investigational Device Exemption [IDE] Cohort; NCT00740870; Melody Transcatheter Pulmonary Valve Post-Approval Study; NCT01186692; and Melody Transcatheter Pulmonary Valve [TPV] Post-Market Surveillance Study; NCT00688571).

Percutaneous transcatheter pulmonary valve replacement in children weighing less than 20 kg.

BACKGROUND: Since FDA approval of the Melody valve, transcatheter pulmonary valve replacement (TPVR) has been offered to an expanding population. Limited data exist regarding the safety and feasibility of TPVR in smaller patients. METHODS: All patients weighing <20 kg who underwent catheterization for percutaneous TPVR at four centers were reviewed. RESULTS: Of the 51 patients reviewed, 43 (84%) had successful valve implantation and 8 (16%) were found not to be candidates due to access veins too small (n = 3), coronary artery/aortic root compression (n = 3), and RVOT too large (n = 2). The 43 patients who underwent successful percutaneous TPVR had a median age and weight of 5.8 years (3.3-10) and 17.7 kg (13.5-19.8), and most had tetralogy of Fallot (TOF). TPVR was performed via femoral vein (FV) access in 24 patients and internal jugular vein (IJV) access in 19 patients. Median weight in patients with IJV implant was 1.1 kg lower than those who underwent transfemoral implant (17.0 vs. 18.1 kg, P = 0.05). There were four adverse events: one iliac vein injury, one contained MPA tear, and two patients with post-procedure femoral vein bleeding. All patients were alive at recent follow-up, a median of 2.0 years (0.1-6.0) after TPVR. There was excellent valve function with mean Doppler gradients of 3-20 mm Hg, and trivial or mild pulmonary regurgitation in all but one patient. There were no RVOT reinterventions and no cases of endocarditis. CONCLUSIONS: Percutaneous TPVR can be safely performed in patients <20 kg. The procedure frequently requires IJV access, and access site/bleeding complications may be more common in this cohort.

Comparison of valvar and right ventricular function following transcatheter and surgical pulmonary valve replacement.

OBJECTIVE: Trans-catheter (TC) pulmonary valve replacement (PVR) has become common practice for patients with right ventricular outflow tract obstruction (RVOTO) and/or pulmonic insufficiency (PI). Our aim was to compare PVR and right ventricular (RV) function of patients who received TC vs surgical PVR. DESIGN: Retrospective review of echocardiograms obtained at three time points: before, immediately after PVR, and most recent. PATIENTS: Sixty-two patients (median age 19 years, median follow-up 25 months) following TC (N = 32) or surgical (N = 30) PVR at Yale-New Haven Hospital were included. OUTCOME MEASURES: Pulmonary valve and right ventricular function before, immediately after, and most recently after PVR. RESULTS: At baseline, the TC group had predominant RVOTO (74% vs 10%, P < .001), and moderate-severe PI was less common (61% vs 100%, P < .001). Immediate post-procedural PVR function was good throughout. At last follow-up, the TC group had preserved valve function, but the surgical group did not (moderate RVOTO: 6% vs 41%, P < .001; >mild PI: 0% vs 24%, P = .003). Patients younger than 17 years at surgical PVR had the highest risk of developing PVR dysfunction, while PVR function in follow-up was similar in adults. Looking at RV size and function, both groups had a decline in RV size following PVR. However, while RV function remained stable in the TC group, there was a transient postoperative decline in the surgical group. CONCLUSIONS: TC PVR in patients age <17 years is associated with better PVR function in follow-up compared to surgical valves. There was a transient decline in RV function following surgical but not TC PVR. TC PVR should therefore be the first choice in children who are considered for PVR, whenever possible.

Patient Selection Process for the Harmony Transcatheter Pulmonary Valve Early Feasibility Study.

This early feasibility study was designed to obtain in vivo data to confirm assumptions on device loading conditions of the Medtronic Harmony transcatheter pulmonary valve (TPV). Secondary objectives included procedural feasibility, safety, and valve performance. The Harmony TPV was developed for nonsurgical pulmonary valve replacement in non-right ventricle-pulmonary artery conduit patients. The Native Outflow Tract TPV Research Clinical Study was the first study approved under the Food and Drug Administration Early Feasibility Study guidance. Enrollment required that patient anatomy be precisely matched to the single-size Harmony TPV implant, necessitating a rigorous selection process. The study was nonrandomized, prospective, and performed at 3 sites. All patients met standard indications for surgical pulmonary valve replacement. The goal of the screening committee was to match the candidate anatomy to predetermined engineering criteria thought to be predictive of secure Harmony TPV implantation for the single-size device under study. A majority of the screening committee was required to recommend a patient as eligible for implant. A total of 270 patients underwent prescreening cardiac magnetic resonance imaging, 66 were enrolled and received a computed tomography scan (24%), 21 met criteria for implant and were catheterized (8%), and 20 underwent implant. Nineteen of 20 met criteria for implant success. In conclusion, the Medtronic Harmony TPV represents an emerging therapeutic option for patients with complex postoperative right ventricular outflow tract failure. The initial clinical evaluation of this technology was unique, and the highly variable anatomy of this population required careful screening to ensure acceptable device fit.

Relationships Among Conduit Type, Pre-Stenting, and Outcomes in Patients Undergoing Transcatheter Pulmonary Valve Replacement in the Prospective North American and European Melody Valve Trials.

OBJECTIVES: This study sought to evaluate the incidence of and risk factors for conduit and stent-related outcomes following transcatheter pulmonary valve replacement (TPVR). BACKGROUND: Stent fracture (SF) and right ventricular outflow tract (RVOT) reintervention are among the most important adverse outcomes after TPVR using the Melody valve (Medtronic, Minneapolis, Minnesota). The conduit environment and conduit preparation practices vary among patients who undergo TPVR. METHODS: Data from 3 prospective Melody valve multicenter studies were pooled and analyzed. All patients who had successful implant of a Melody valve that was present at hospital discharge comprised the study cohort; patients who had TPVR into a stentless conduit comprised the analysis cohort. SF was diagnosed using protocol-specified or clinical fluoroscopy or radiography, and classified as major or minor. RESULTS: Of 358 patients who underwent catheterization with intent to perform TPVR, 309 were discharged with the Melody valve in place (study cohort) of which 251 patients had TPVR into a stentless conduit (analysis cohort). Median follow-up was 5 years. New pre-stents were placed in 68% of patients with a stentless conduit, and 22% received multiple pre-stents. At 3 years, freedom from any SF and major SF was 74 ± 3% and 85 ± 2%, respectively, and freedom from RVOT reintervention was 85 ± 2%. New pre-stents were associated with longer freedom from SF and RVOT reintervention than was no pre-stent. CONCLUSIONS: Risks of SF and reintervention after TPVR with a Melody valve were reduced by implantation of pre-stents, which has become standard practice. This study supports pre-stenting as an important component of TPVR therapy.

Transcatheter Pulmonary Valve Replacement Reduces Tricuspid Regurgitation in Patients With Right Ventricular Volume/Pressure Overload.

BACKGROUND: Tricuspid regurgitation (TR) is a common and important comorbidity in patients with postoperative right ventricular outflow tract (RVOT) obstruction or pulmonary regurgitation (PR). Transcatheter pulmonary valve replacement (TPVR) has become a useful tool in the management of postoperative RVOT obstruction and PR, but it is unknown whether relief of the right ventricular volume and/or pressure overload by TPVR will have a beneficial effect on TR, as is often seen with surgical pulmonary valve replacement. OBJECTIVES: This study sought to assess the prevalence of and factors associated with significant TR in patients undergoing TPVR for RVOT obstruction or PR. METHODS: Data were combined from 3 prospective multicenter trials of patients referred for TPVR. Follow-up data through 5 years post-implantation were analyzed. RESULTS: Of 300 patients studied, 77 (25.6%) had moderate or severe TR at baseline. After TPVR, TR severity was improved in 65% of those patients, and more than one-half had mild TR or less TR at discharge. Of 13 patients with severe TR pre-implantation, only 1 had severe TR at 1-year follow-up and beyond. Moderate or severe baseline TR was associated with shorter freedom from RVOT reintervention after TPVR. CONCLUSIONS: In this prospective multicenter study of post-operative patients with RVOT obstruction and/or PR, TR was common. In patients with significant baseline TR, TPVR resulted in clinically relevant acute reductions in TR that persisted over at least 5 years of follow-up. These observations support the application of TPVR therapy in patients with RVOT obstruction or PR who are anatomically suitable, even in the setting of significant concomitant TR.

Evaluation of the Melody transcatheter pulmonary valve and Ensemble delivery system for the treatment of dysfunctional right ventricle to pulmonary artery conduits.

Synthetic conduits and bioprosthetic valves are used in the treatment of patients with congenital heart disease involving the right ventricular outflow tract and pulmonary valve. In-situ time-dependent degradation uniformly results in conduit and valve dysfunction. The abnormal hemodynamics imposed by valve and conduit dysfunction have been linked to exercise intolerance, arrhythmia, right heart failure, and sudden death. Starting in childhood, affected patients are subjected to repeated open-heart surgeries to restore valve function and potentially reduce morbidity and mortality. Percutaneous transcatheter pulmonary valve replacement with the Melody Transcatheter Pulmonary Valve (Medtronic, Inc., Minneapolis, MN) has been performed in ≈8000 patients worldwide. The valve and implant procedure provide a far less invasive means of restoring valve and conduit function and allow patients to forego multiple operations. Recent clinical trials have shown excellent and durable results in terms of valve function, relief of obstruction, and improvement in functional class up to 7 years from implant.

Transcatheter Pulmonary Valve Replacement for Right Ventricular Outflow Tract Conduit Dysfunction After the Ross Procedure.

BACKGROUND: Right ventricular outflow tract (RVOT) conduit dysfunction is a limitation of the Ross procedure. Transcatheter pulmonary valve replacement (TPVR) could alter the impact of conduit dysfunction and the risk-benefit balance for the Ross procedure. METHODS: Retrospective review of databases from 3 prospective Melody TPV (Medtronic Inc, Minneapolis, MN) trials. RESULTS: Among 358 patients who were catheterized with the intent to implant a Melody TPV for RVOT conduit stenosis or regurgitation (PR) as part of 3 prospective multicenter studies, 67 (19%) had a prior Ross procedure. Of these, 56 (84%) received a Melody valve; in 5 of the 11 patients who did not, the implant was aborted due to concern for coronary artery compression, and 1 implanted patient required emergent surgery for left coronary compression. The RVOT gradient decreased from a median 38 mm Hg to 13.5 mm Hg (p < 0.001). There was no or trivial PR in all but 4 patients, in whom it was mild. At a median follow-up of 4.0 years, 1 patient died from sepsis. Twelve patients underwent 14 transcatheter (n = 8) or surgical (n = 6) TPV reinterventions for obstruction with stent fracture (n = 9), endocarditis with conduit obstruction (n = 3), or reoperation (n = 2). Freedom from TPV explant was 89% ± 5% at 4 years. Among patients who did not undergo reintervention for obstruction, there was no change in RVOT gradient over time, and all but 1 patient had mild or less PR at last follow-up. CONCLUSIONS: The TPVR with the Melody valve provides acceptable early outcomes and durable valve function in the majority of Ross patients. Recurrent RVOT obstruction associated with stent fracture was the main reason for reintervention. Coronary compression is not uncommon in Ross patients and should be assessed prior to TPVR.

Clinical and hemodynamic outcomes up to 7 years after transcatheter pulmonary valve replacement in the US melody valve investigational device exemption trial.

BACKGROUND: Studies of transcatheter pulmonary valve (TPV) replacement with the Melody valve have demonstrated good short-term outcomes, but there are no published long-term follow-up data. METHODS AND RESULTS: The US Investigational Device Exemption trial prospectively enrolled 171 pediatric and adult patients (median age, 19 years) with right ventricular outflow tract conduit obstruction or regurgitation. The 148 patients who received and were discharged with a TPV were followed up annually according to a standardized protocol. During a median follow-up of 4.5 years (range, 0.4-7 years), 32 patients underwent right ventricular outflow tract reintervention for obstruction (n=27, with stent fracture in 22), endocarditis (n=3, 2 with stenosis and 1 with pulmonary regurgitation), or right ventricular dysfunction (n=2). Eleven patients had the TPV explanted as an initial or second reintervention. Five-year freedom from reintervention and explantation was 76±4% and 92±3%, respectively. A conduit prestent and lower discharge right ventricular outflow tract gradient were associated with longer freedom from reintervention. In the 113 patients who were alive and reintervention free, the follow-up gradient (median, 4.5 years after implantation) was unchanged from early post-TPV replacement, and all but 1 patient had mild or less pulmonary regurgitation. Almost all patients were in New York Heart Association class I or II. More severely impaired baseline spirometry was associated with a lower likelihood of improvement in exercise function after TPV replacement. CONCLUSIONS: TPV replacement with the Melody valve provided good hemodynamic and clinical outcomes up to 7 years after implantation. Primary valve failure was rare. The main cause of TPV dysfunction was stenosis related to stent fracture, which was uncommon once prestenting became more widely adopted. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00740870.

Normal pediatric data for isovolumic acceleration at the lateral tricuspid valve annulus-a heart rate - dependent measure of right ventricular contractility.

BACKGROUND: Quantitative assessment of right ventricular (RV) function is important for the management of patients with congenital heart disease. Tissue Doppler (TDI)-derived myocardial acceleration during isovolumic contraction (IVA) is an echocardiographic measure of contractility that is independent of loading conditions. The aim of this study was to establish normative data for IVA at the lateral tricuspid valve annulus (RV IVA). METHODS: This is a retrospective study of 340 children who had normal echocardiograms. We analyzed RV IVA in relation to age, body surface area (BSA), gender, heart rate (HR), tricuspid annular plane systolic excursion (TAPSE), and TDI-derived S' (RV S'). RESULTS: RV IVA showed a positive correlation with HR (r = 0.58, P<0.001) and a negative correlation with age and BSA (r = -0.44, P<0.001, for both). RV IVA ranged from 2.3 m/sec(2) (±2 SD: 1-3.7 m/sec(2) ) at a HR of <60 beats per minute (bpm) to 4.9 m/sec(2) (±2 SD: 3.5-6.4 m/sec(2) ) at a HR of >160 bpm. When RV IVA was corrected for HR (RV IVAc), it became independent of HR, age, BSA, and gender. The mean RV IVAc was 2.3 m/sec(1.5) (±2 SD: 1.1-3.6 m/sec(1.5) ). There was a correlation of normalized RV IVAc with normalized TAPSE and RV S'. CONCLUSION: RV IVA, a marker of RV contractility, shows a strong dependence on HR. HR corrected RV IVA (RV IVAc) is independent of age, BSA, gender, and HR. We suggest measuring RV IVAc routinely in patients at risk for RV dysfunction.

Iatrogenic left pulmonary artery to left atrium fistula.

Trans-catheter balloon angioplasty is a well-established treatment modality for pulmonary artery (PA) stenosis in children with congenital heart disease. We report a case of an unusual complication where a fistula developed between the left PA and the left atrium during balloon angioplasty in a patient with history of tetralogy of Fallot. This was successfully treated with placement of a covered stent.

Feasibility and short-term outcomes of percutaneous transcatheter pulmonary valve replacement in small (<30 kg) children with dysfunctional right ventricular outflow tract conduits.

BACKGROUND: In 2010, the Melody transcatheter pulmonary valve (TPV) received Food and Drug Administration approval for treatment of dysfunctional right ventricular outflow tract conduits in patients ≥30 kg. Limited data are available regarding use of this device in smaller patients. METHODS AND RESULTS: We evaluated technical and short-term clinical outcomes of 25 patients <30 kg (10 patients <20 kg) who underwent TPV replacement for treatment of conduit dysfunction at 3 centers. Median age and weight were 8.0 years (3.4-14.4) and 21.4 kg (13.8-29.0). The median conduit diameter at the time of surgical implant was 17 mm (12-23). Two patients did not undergo TPV implant (risk of coronary compression in 1; inability to advance the delivery sheath beyond the common femoral vein in 1). After successful TPV implant, the peak conduit gradient fell from 29±16 to 9±6 mm Hg (P<0.001), and all but 2 patients had no/trivial regurgitation (down from moderate or severe preimplant in 20). TPV implant was via the femoral vein in 17 patients, the right internal jugular vein in 4, and the left subclavian vein in 2 patients. At a median follow-up of 16 months, 1 patient underwent conduit replacement for recurrent conduit stenosis, 2 developed stent fracture requiring a second TPV, and 2 developed bacterial endocarditis treated with antibiotics, 1 of whom then underwent conduit replacement. The average Melody valve mean Doppler gradient and conduit regurgitation were unchanged from early postimplant. CONCLUSIONS: Percutaneous TPV replacement can be performed in small children with good procedural and early hemodynamic results in the majority of patients.

Melody valve implant within failed bioprosthetic valves in the pulmonary position: a multicenter experience.

BACKGROUND: Transcatheter pulmonary valve implantation using the Melody valve has emerged as an important therapy for the treatment of postoperative right ventricular outflow tract dysfunction. Melody-in-bioprosthetic valves (BPV) is currently considered an off-label indication. We review the combined experience with transcatheter pulmonary valve implantation within BPVs from 8 centers in the United States and discuss technical aspects of the Melody-in-BPV procedure. METHODS AND RESULTS: A total of 104 patients underwent Melody-in-BPV in the pulmonary position at 8 US centers from April 2007 to January 2012. Ten different types of BPVs were intervened on, with Melody valve implantation at the intended site in all patients. Following Melody valve implant, the peak right ventricle-to-pulmonary artery gradient decreased from 38.7 ± 16.3 to 10.9 ± 6.7 mm Hg (P<0.001), and the right ventricular systolic pressure fell from 71.6 ± 21.7 to 46.7 ± 15.9 mm Hg (P<0.001). There was no serious procedural morbidity, and no deaths related to the catheterization or implant. At a median follow-up of 12 months (1-46 months), no patients had more than mild regurgitation, and 4 had a mean right ventricular outflow tract gradient ≥30 mm Hg. During follow-up, there were 2 stent fractures, 3 cases of endocarditis (2 managed with surgical explant), and 2 deaths that were unrelated to the Melody valve. CONCLUSIONS: Transcatheter pulmonary valve implantation using the Melody valve within BPVs can be accomplished with a high rate of success, low procedure-related morbidity and mortality, and excellent short-term results. The findings of this preliminary multicenter experience suggest that the Melody valve is an effective transcatheter treatment option for failed BPVs.

Pulmonary venous obstruction in the atrial switch operation: a forgotten complication.

The reported case involved a 7-year-old girl with congenitally corrected transposition of the great arteries (ccTGA) and situs inversus who after surgical management experienced symptoms of dyspnea and left-sided obstructive heart disease. Her symptoms, chest X-ray, and cardiac catheterization demonstrated pulmonary venous obstruction, a known but rare complication associated with intra-atrial baffle obstruction. The incidence of the reported disorder, the follow-up assessment, and the current literature regarding complications of Senning/Mustard surgery are discussed in the context of patients with ccTGA.

Reliability and accuracy of echocardiographic right heart evaluation in the U.S. Melody Valve Investigational Trial.

BACKGROUND: Appropriate patient selection for transcatheter pulmonary valve (TPV) replacement requires accurate evaluation of right ventricular (RV) performance. The aim of this study was to evaluate the reliability and accuracy of echocardiography for evaluating RV parameters in patients in the five-center Melody TPV trial. METHODS: Echocardiographic data were compared with cardiac magnetic resonance (CMR) and catheterization; interobserver comparisons were made using site and core laboratory data. RESULTS: Doppler echocardiographic assessments of RV outflow tract obstruction and RV pressure showed excellent interobserver agreement; mean Doppler gradients were correlated most closely with gradients at catheterization (R = 0.66), and Doppler RV pressure estimates were correlated well with catheterization data (R = 0.58). Assessment of pulmonary regurgitation (PR) using a three-point severity scale showed good agreement with CMR-derived PR fraction (86%). The tricuspid annular Z score was highly reproducible but correlated weakly with CMR RV end-diastolic volume (R = 0.21). However, RV apical diastolic area was highly reproducible (R = 0.87) and had an excellent correlation with CMR RV end-diastolic volume (R = 0.78); all patients with indexed RV apical diastolic areas ≥30 cm(2)/m(2) had CMR RV end-diastolic volumes ≥160 mL/m(2). RV function using the fractional area change method showed a fair correlation with CMR RV ejection fraction (R = 0.48). CONCLUSIONS: In patients with dysfunctional RV outflow tract conduits, echocardiography provided reproducible, accurate estimates of pressure overload and RV size. Echocardiographic assessment of PR correlated less closely with CMR PR fraction but showed good categorical agreement; assessment of RV function by these methods was suboptimal. Echocardiography alone may be a suitable screening test for some TPV replacement candidates; CMR may be indicated for TPV replacement decisions hinging on assessment of RV function.

Short-term performance of the transcatheter Melody valve in high-pressure hemodynamic environments in the pulmonary and systemic circulations.

BACKGROUND: The Melody valve is approved for percutaneous pulmonary valve replacement in dysfunctional right ventricular outflow tracts. The function of this valve when subjected to high-pressure loads in humans is unknown. The aim of this study was to describe the immediate and short-term results of Melody valves implanted in a high-pressure environment. METHODS AND RESULTS: Definitions of a high-pressure system were established for Melody valves implanted in the systemic (ie, aortic or mitral position) and pulmonary (ie, right ventricular outflow tract conduit or tricuspid valve annulus) circulations. Implants in these environments were ascertained from databases of the 5 centers that participated in the US Investigational Device Exemption trial. Thirty implants met the inclusion criteria: 23 pulmonary circulation implants (all in the pulmonary position) systemic circulation implants (5 in the native aortic position, 1 in a left ventricle-to-descending aorta conduit, and 1 in the mitral annulus). All pulmonary circulation implants were performed percutaneously in the catheterization laboratory. A hybrid approach (surgical exposure for transcatheter implant) was used for 4 of the aortic implants. There were no procedure-related deaths. Three patients died of nonprocedure- and nonvalve-related causes. At 1 year, freedom from moderate to severe regurgitation was 100%, and freedom from mild regurgitation was 90%. Freedom from moderate to severe stenosis was 86% at 1 year. CONCLUSIONS: Short-term performance of the Melody valve in high-pressure environments is encouraging, with good valve function in all patients.