Bilateral Harmony™ valve placement in branch pulmonary arteries.

While newer self-expanding pulmonic valves were primarily designed for larger right ventricular outflow tracks, there are instances where even larger anatomies cannot accommodate these devices. In this report, we describe the successful implantation of two Harmony™ valves in bilateral branch pulmonary arteries after exhausting other options.

Transcatheter pulmonary valve replacement in surgically-created double-barrel right ventricular outflow tracts: A single center case-series.

This manuscript describes the feasibility and approach to the assessment and performance of transcatheter pulmonary valve replacement (tPVR) in patients with surgically-created "double-barrel" right ventricular outflow tracts (RVOT). Patients with tetralogy of Fallot may have coronary anomalies which prohibit the performance of traditional tetralogy of Fallot repair. In certain cases, this may necessitate the placement of a right ventricle to pulmonary artery conduit in addition to the native RVOT, which is left in situ, creating so-called "double-barrel" RVOTs. When these patients develop RVOT dysfunction later in life, they would typically be referred for reoperation due to concerns for risk of coronary compression associated with a transcatheter approach. However, whether a transcatheter approach with valve replacement in the native RVOT is feasible or safe is unknown. This was a retrospective review of patients with a surgically created "double-barrel" RVOTs who underwent cardiac catheterization for assessment of tPVR at Boston Children's Hospital. From July 2012 to July 2022, there were four patients with "double-barrel" RVOTs who underwent assessment for tPVR. The age at catheterization ranged between 22 and 39 years. In three out of four patients, coronary compression testing was negative. These three patients had successful tPVR in the native RVOT. At follow up, all three patients were free of greater than mild regurgitation by echocardiogram and had a maximum instantaneous gradient across the RVOT ranging between 20 and 33 mmHg. Performance of tPVR in patients with surgically created "double-barrel" RVOTs is feasible. The safety of this procedure depends crucially on coronary artery assessment at all stages.

Long-term function of a novel autologous transcatheter pulmonary heart valve implant in an adult animal model.

BACKGROUND: Current heart valve implants entail major disadvantages in the treatment for younger patients or those with congenital heart defects. AIM: Evaluation of novel transcatheter pulmonary valve implant made from autologous pericardium with natural crosslinking agent in an in vitro setup and in vivo animal model METHODS: Valves were tested in a pulse duplicator according to ISO-standard 5840. For in vivo studies computer tomography was performed to measure sheep's native pulmonary valve dimensions. Pericardium was harvested by thoracotomy, personalized implants were manufactured and deployed in pulmonary valve position of the same sheep. Every 3 months implant functionality was evaluated by intracardiac echocardiography, intracardiac pressure measurements and cardiac magnetic resonance imaging (cMRI). Implants were explanted for macroscopic and histological examination. RESULTS: In vitro experiments showed compliance with regulatory requirements in terms of valve opening and insufficiency. Five sheep successfully received an autologous valve implant. Two animals had to be euthanized due to trauma sustained in the stable. Long-term valve function was excellent in three out of four animals with median implant cMRI regurgitation fraction of 9% (n = 4) at 3 months, 8% (n = 3) at 6, 8% (n = 3) at 9, 12% (n = 3) at 13, 8% (n = 2) at 17% and 8% (n = 2) at 20.5 months after implantation. Despite good adherence to neighboring tissue and endothelization, histological assessment revealed some signs of degeneration. CONCLUSION: Transcatheter pulmonary valve implants showed promising function for up to 20.5 months encouraging research to further improve this approach.

Hybrid approach for harmony transcatheter pulmonary valve replacement.

The Harmony™ Transcatheter Pulmonary Valve (Medtronic) was recently approved by the Food and Drug Administration for transcatheter pulmonary valve replacement in native right ventricular outflow tracts. Despite this milestone, some patients have main pulmonary arteries that are severely dilated and continue to require surgical pulmonary valve replacement. The hybrid approach combines surgical creation of a landing zone, transcatheter valve deployment, and suture stabilization of the implanted valve. In this case series, we report the first use of a hybrid approach for Harmony™ transcatheter pulmonary valve replacement. Two cases are reported with varying approaches for surgical creation of a landing zone followed by successful placement of a Harmony™ valve.

An unexpected circular shunt: Novel method to treat semilunar valve insufficiency in a single ventricle patient on mechanical circulatory support.

Severe semilunar valve insufficiency in single ventricle patients supported with mechanical circulatory support while awaiting transplant remains a complex clinical scenario with few favorable options for management. We present the first case, to our knowledge, of transcatheter closure of the pulmonic valve in a patient palliated with a hybrid stage 1 procedure for hypoplastic left heart syndrome.

Bilateral branch pulmonary artery Pulsta valve implantation for treatment of large right ventricular outflow tract in a high-risk patient.

Percutaneous pulmonary valve implantation (PPVI) has been implemented as a novel alternative strategy to surgical pulmonary valve replacement. However, PPVI has an inevitable limitation: the large right ventricular outflow tract (RVOT) lesions exhibit variable geometry and significant pulmonary regurgitation (PR). To overcome this limitation, bilateral branch pulmonary artery (PA) valve implantations using Melody or Sapien valves have been attempted and have shown a reduction in right ventricular volume with clinical benefits in the intermediate term. Nevertheless, these trials also have constraints of large branch PA size. Recently, a feasibility study using the Pulsta valve (Tae Woong Medical Co, Gyeonggi-do, South Korea) for native RVOTs was reported; the diameter of the Pulsta valve ranges from 18 to 32 mm. Herein, we present a successful percutaneous bilateral branch PA valve implantation using two 32 mm Pulsta valves in a 59-year-old man who showed right heart failure with severe pulmonary regurgitation despite several open heart surgeries for tetralogy of Fallot. The main PA was measured to be 49 mm, and both the right and left PAs were measured to be 30 mm.

Mid-term outcomes of the Pulsta transcatheter pulmonary valve for the native right ventricular outflow tract.

OBJECTIVES: The aim of this study is to present the mid-term outcomes of Pulsta valve. BACKGROUND: The Pulsta valve is a Self-expandable knitted nitinol-wire stent mounted with a treated tri-leaflet α-Gal-free porcine pericardial valve for percutaneous pulmonary valve implantation (PPVI) in patients with native right ventricular outflow tract (RVOT) lesions. METHODS: A multi-center clinical trial using Pulsta valve® was designed for patients with severe pulmonary regurgitation (PR) in the native RVOT in multiple centers in South Korea and 25 patients were enrolled. Before PPVI, severe PR (mean PR fraction: 45.5 ± 6.9%) and enlarged RV volume (mean indexed RV end-diastolic volume; 169.7 ± 13.0 ml/m2 ) was present. The mean age was 21.6 ± 6.6 years old. RESULTS: All patients were successfully implanted with 26, 28, or 32 mm diameter of Pulsta valve loaded on the 18 or 20 French delivery catheters. At 6 months follow up, indexed RV end-diastolic volume was decreased to 126.9 ± 16.9 ml/m2 . At mean 33.1 ± 14.3 months follow-up, the mean value of mean pressure gradient in Pulsta valve was 6.5 ± 3.0 mmhg without significant PR. There was no serious device-related adverse event. CONCLUSIONS: A multi-center clinical trial was completed successfully with planned Pulsta valve implantation and demonstrated good mid-term effectiveness without device-related serious adverse events.

Edwards SAPIEN XT transcatheter pulmonary valve implantation: 5-year follow-up in a French Registry.

OBJECTIVES: This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. BACKGROUND: The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. METHODS: From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. RESULTS: The primary efficacy outcome was met for 87.1% patients after a mean follow-up of 4.6 ± 1.8 years, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8-95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular regurgitation was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01-2.00%). At 5 years, freedom from infective endocarditis was 98.4% (95% CI 89.1-99.8%). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. CONCLUSIONS: This study documents the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance.

Combined hybrid pulmonary valve placement and atrial septal defect closure: case report and literature review.

We report a case of a 15-year-old female who underwent combined hybrid pulmonary valve replacement and transcatheter atrial septal defect device closure, which was performed due to severe volume overload of the right side of the heart secondary to pulmonary regurgitation and atrial septal defect.

Treatment of severe pulmonary insufficiency with bilateral branch pulmonary artery Melody valve implantation.

Branch pulmonary artery valve implantation has been reported in larger patients with dysfunctional right ventricular outflow tracts via routine femoral access. Here, we report treatment of severe pulmonary insufficiency with bilateral branch pulmonary artery Melody valve implantation (Medtronic, Minneapolis, Minnesota). To the best of our knowledge, this is the first report of bilateral valve implantation utilising the hybrid approach in a small-size patient.

Contemporary Outcomes of Pulmonary Valve Endocarditis: A 16-Year Single Centre Experience.

BACKGROUND: Limited data exist regarding the clinical characteristics and contemporary outcomes of patients with pulmonary valve (PoV) infective endocarditis (IE). METHODS: This is a retrospective cohort study of patients with a confirmed diagnosis of IE affecting the PoV at our centre between January 2002 and October 2018. Electronic medical records were reviewed to gather the clinical and echocardiographic variables. The population was subdivided according to risk factor profiles: group 1: miscellaneous risk factors; group 2: patients with congenital heart disease (CHD); and group 3: patients who inject drugs (PWID). The primary outcome was all-cause mortality. RESULTS: Out of 2,124 cases of IE during the study period, 24 (1.1%) patients had PoV IE. The majority of cases of PoV IE occurred in patients with prosthetic valves (54.2%). Coagulase-negative Staphylococci species were the most common micro-organisms. Seventy-five per cent (75%) of the patients required surgical management. The median follow-up was 2.8 years (interquartile range: 0.2-5.3 years). Patients with miscellaneous risk factors were older (p<0.01), and had higher rates of hypertension (p=0.01) and hyperlipidaemia (p=0.04). There was a statistically significant difference in survival between the groups (p=0.03), mainly driven by better outcomes of patients with CHD, compared to those with miscellaneous risk factors. CONCLUSIONS: In a contemporary 16-year series, a high proportion of patients with PoV IE required surgical management. Patients with PoV IE and CHD had better survival, compared to patients with miscellaneous risk factors at a median follow-up of 2.8 years.

Risk factors for infective endocarditis following transcatheter pulmonary valve replacement in patients with congenital heart disease.

OBJECTIVES: We sought to delineate the risk factors for infective endocarditis (IE) in patients undergoing transcatheter pulmonary valve replacement (TCPVR). BACKGROUND: Despite the therapeutic benefits of TCPVR for treatment of dysfunctional right ventricular outflow tracts, IE is a major complication of the approach. Specific hemodynamic gradients and patient immune status as predisposing factors for IE are largely unexplored. METHODS: We performed a retrospective review of patients who had undergone TCPVR at UCLA between October 2010 and October 2017. Cases of IE were diagnosed based on the modified Duke criteria. RESULTS: Two hundred and thirty-five cases of TCPVR were performed with a mean follow-up of 2.6 years (range 0.0-8.0 years). Sixteen distinct IE events developed in 13 patients (Melody™ n = 12, SAPIEN n = 1), with a median time from implant to IE of 3.3 years (range 2.0-7.2 years). Univariate Cox regression showed that immunocompromised status was significantly associated with the development of IE hazard ratios (HR 5.43 [1.80-16.4], p = .003). Kaplan-Meier curves show that the 5-year freedom from IE among immunocompetent patients was 87% (95% CI 78-96%) versus 64% (95% CI 39-89%) among immunocompromised patients (log-rank p = .02). Postimplant right ventricular systolic pressure was higher among immunocompromised patients (p = .03). The risk of IE post-TCPVR in immunocompromised patients with residual pulmonary stenosis was 43%. CONCLUSIONS: Among the risk factors examined in this study, immunocompromised status was the most significant predictor of IE development post-TCPVR. Patients with the lowest risk of IE are those with competent immune systems, without a history of IE, and with minimal residual pulmonary valve gradients post-TCPVR.

Use of 65 cm large caliber Dryseal sheaths to facilitate delivery of the Edwards SAPIEN valve to dysfunctional right ventricular outflow tracts.

BACKGROUND: The Edwards SAPIEN valve and its delivery system may complicate transit through the right heart during transcatheter pulmonary valve replacement (tPVR). We report our early experience using a large diameter, 65 cm delivery sheath to facilitate delivery of the SAPIEN valve to the right ventricular outflow tract (RVOT). METHODS: Retrospective analysis of all patients from three large congenital heart centers undergoing tPVR with the Edwards SAPIEN valve delivered with the 65 cm Gore Dryseal Sheath. RESULTS: Over a 12 month period, 30 patients (17 female) with median age 17.5 years (range 8-72) underwent attempted tPVR with the SAPIEN valve delivered using the 65 cm Dryseal sheath (20-26Fr). All procedures resulted in successful valve delivery to the target area. Twenty patients had a native RVOT. The most commonly used valve diameter was 29 mm (n = 15) with the majority of cases requiring a 26Fr Dryseal sheath (n = 20). One patient with severe RVOT stenosis underwent prestenting. Median procedure time was 100 min (59-225). No patient had increase in tricuspid valve regurgitation as a consequence of valve delivery. One patient required a synchronous cardioversion for intraprocedural VT and another required ECMO postprocedure due to severe pre-existing left ventricular dysfunction. On median follow-up of 5 months, all patients had mild or less pulmonary regurgitation. Median peak Doppler velocity across the pulmonary valve was 2.2 m/s (1.7-4). There were no clinically relevant complications relating to vascular access. CONCLUSIONS: Using 65 cm Dryseal sheaths facilitates delivery of SAPIEN valves in patients with dysfunctional RVOTs.

First human implant of the Alterra Adaptive PrestentTM : A new self-expanding device designed to remodel the right ventricular outflow tract.

Current balloon expandable transcatheter valves have limited applicability to patients with "native" right ventricular outflow tracts (RVOT), meaning those who have had previous surgery and are left with large, compliant, irregular RVOT. The Alterra Adaptive PrestentTM is a self-expanding, partially covered stent that was designed to internally reconfigure these types of RVOT, making them suitable for implantation of a commercially available balloon expandable heart valve, the SAPIEN 3. Herein, we describe the first human implant of this device.

A new one-step procedure for pulmonary valve implantation of the melody valve: Simultaneous prestenting and valve implantation.

OBJECTIVES: To describe a new modification, the one-step procedure, that allows interventionists to pre-stent and implant a Melody valve simultaneously. BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is the standard of care for managing patients with dysfunctional right ventricular outflow tract, and the approach is standardized. METHODS: Patients undergoing PPVI using the one-step procedure were identified in our database. Procedural data and radiation exposure were compared to those in a matched group of patients who underwent PPVI using the conventional two-step procedure. RESULTS: Between January 2016 and January 2017, PPVI was performed in 27 patients (median age/range, 19.1/10-55 years) using the one-step procedure involving manual crimping of one to three bare metal stents over the Melody valve. The stent and Melody valve were delivered successfully using the Ensemble delivery system. No complications occurred. All patients had excellent hemodynamic results (median/range post-PPVI right ventricular to pulmonary artery gradient, 9/0-20 mmHg). Valve function was excellent. Median procedural and fluoroscopic times were 56 and 10.2 min, respectively, which significantly differed from those of the two-step procedure group. Similarly, the dose area product (DAP), and radiation time were statistically lower in the one-step group than in the two-step group (P < 0.001 for all variables). After a median follow-up of 8 months (range, 3-14.7), no patient underwent reintervention, and no device dysfunction was observed. CONCLUSIONS: The one-step procedure is a safe modification that allows interventionists to prestent and implants the Melody valve simultaneously. It significantly reduces procedural and fluoroscopic times, and radiation exposure.

Fracturing a dysfunctional Edwards Perimount bioprosthetic valve to facilitate percutaneous valve-in-valve placement of SAPIEN 3 valve with modified delivery system.

Pulmonary valve replacement via surgical implantation of a bioprosthetic valve (BPV) is a well-established treatment for patients with dysfunctional RV outflow tracts. BPVs are prone to structural deterioration, and will eventually require replacement. Recently, percutaneous valve-in-valve (VIV) placement of transcatheter valves has established itself as a safe and effective alternative to surgical revision. Unfortunately, VIV therapy is inherently limited by the inner diameter of the BPV, which restricts the number of eligible patients. Other centers have reported on the feasibility of cracking certain BPVs with ultra high-pressure balloons in bench testing. We now report cracking an Edwards Perimount BPV in the pulmonary position to facilitate VIV placement of an Edwards SAPIEN 3. The ability to crack the Perimount valve allowed placement of a larger valve than previously considered and minimized the final valve gradient. In an effort to avoid the morbidity and mortality of surgical pulmonary valve replacement, this new strategy will expand the number of patients eligible for percutaneous VIV therapy.

Incidence and outcome of infective endocarditis following percutaneous versus surgical pulmonary valve replacement.

OBJECTIVES: To provide a comparison of the outcome of infective endocarditis (IE) in patients undergoing transcatheter pulmonary valve replacement (TPVR) versus surgical pulmonary valve replacement (SPVR). BACKGROUND: Although TPVR is thought to be associated with a higher risk of IE than SPVR, there is paucity of data to support this. METHODS: Patients who underwent TPVR or SPVR at UCLA between October 2010 and September 2016 were included and retrospectively analyzed. RESULTS: Three hundred forty-two patients underwent PVR at UCLA including 134 SPVR and 208 TPVR. Patients undergoing TPVR were more likely to have had a history of endocarditis than those undergoing SPVR (5.3% vs. 0.7%, P = 0.03) and a right ventricle to pulmonary artery (RV to PA) conduit (37% vs. 17%, P = 0.0001). Two SPVR and seven TPVR patients developed IE with a 4-year freedom from endocarditis of 94.0% in the SPVR versus 84% in the TPVR group (P = 0.13). In patients who underwent TPVR and developed endocarditis, the mean gradient across the RVOT prior to intervention was higher (28.1 ± 4.5 vs. 17.4 ± 0.6 mmHg, P = 0.02) and were more likely to have a conduit (71% vs. 36%, P = 0.049). CONCLUSIONS: In this study, patients undergoing TPVR were not at a higher risk of IE than patients undergoing SPVR. TPVR patients were more likely to have had a prior history of IE and RV-PA conduit. The patients at highest risk were those with stenotic RV to PA conduits who were treated with TPVR.

Bilateral branch pulmonary artery valve implantation in repaired tetralogy of fallot.

BACKGROUND: Transcatheter, bilateral branch pulmonary artery (PA) valve implantation is a novel treatment for patients with severe pulmonary insufficiency and oversized right ventricle (RV) outflow tract. There is scarce data on efficacy and safety of this approach. METHODS: This was a retrospective study of 8 patients with repaired tetralogy of fallot (TOF) who underwent bilateral branch PA valve implantation. Demographics, echocardiography, cardiac catheterization, and axial imaging data were reviewed. Variables were compared by a paired sample t-test. RESULTS: All patients were adult sized (weight 43-99 kg) with oversized RV outflow tract not suitable for conventional transcatheter pulmonary valve implantation. Staged bare metal PA stenting followed by valve implantation (interval 3-5 months) was technically successful in 7 patients with one stent embolization. In another patient, proximal stent migration prevented placement of bilateral pulmonary valve stents. There were a total of 14 valved branch PA stents placed (Melody valve n = 9, Sapien XT n = 2, Sapien 3 n = 3). In the 7 patients undergoing successful branch pulmonary valve placement, at median follow up of 10 months (range 3 months to 6 years), 13 (93%) valves had none/trivial insufficiency on echocardiography. Prevalve and postvalve implantation cardiac magnetic resonance imaging in five patients showed significant reduction of indexed RV end-diastolic volume (152 ± 27 to 105 ± 15 mL/m2 , P < .001). CONCLUSIONS: Transcatheter, bilateral branch PA valve implantation was technically feasible with satisfactory efficacy and safety in patients with repaired TOF, severe pulmonary insufficiency, and oversized RV outflow tracts. Elimination of pulmonary insufficiency with this method resulted in reduced RV end-diastolic volume. This approach can be offered as an alternative to surgery, particularly in patients considered high risk for standard surgical placement and who are not candidates for the newer self-expanding valve prosthesis for placement in RV outflow tracts larger than 30 mm diameter.

Ventricular arrhythmias immediately following transcatheter pulmonary valve implantation: A cause for concern?

BACKGROUND: Transcatheter pulmonary valve implantation (TPVI) has revolutionized the care of patients with congenital disorders of the right ventricular outflow tract (RVOT) and is increasingly being used in patients with native outflow tracts. This is the first study to specifically report the occurrence of ventricular arrhythmias in the immediate post-TPVI period. METHODS AND RESULTS: Medical records of all adult and pediatric patients who underwent TPVI at our institution between May 1, 2011 and March 1, 2016, were reviewed for the presence of clinically significant ventricular arrhythmias occurring within 30 days of TPVI. We defined a clinically significant arrhythmia as any ventricular arrhythmia that led to hemodynamic instability, resulted in a change of dose or addition of a new anti-arrhythmic medication, caused a delay in discharge, or was the primary reason for readmission. Seventy-five patients, with a median age of 19 years (range 4-65 years), underwent TPVI. In total, 12 (16%) patients had a clinically significant ventricular arrhythmia within 30 days following TPVI. Patients with native outflow tracts were at higher risk of post-TPVI arrhythmias than non-native outflow tract (29% vs. 9%, P = 0.02, adjusted OR 4.8, 95%CI 1.2-20.2). There were no cases of hemodynamic compromise or sudden cardiac death. The arrhythmias were well controlled with beta-blocker therapy. CONCLUSION: In this single center study, ventricular arrhythmias were common following TPVI, particularly in native outflow tract patients. However, the arrhythmias were generally benign and responded well to medical therapy when indicated. Long term therapy was often not needed.