Intravascular Lithotripsy in an Adult With Fontan Pathway Obstruction.

Fontan pathway obstruction is a potentially serious complication characterized by an anatomical or functional narrowing anywhere in the cavo-pulmonary pathways. Here, we report the first case in the literature where an innovative Fontan conduit rehabilitation procedure with intravascular lithotripsy was used achieving a dramatic increase in the pathway size.

Early Outcomes From a Multicenter Transcatheter Self-Expanding Pulmonary Valve Replacement Registry.

BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021. OBJECTIVES: In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience. METHODS: This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S. CENTERS: The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention. RESULTS: A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2). CONCLUSIONS: In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes.

Transcatheter Pulmonary Valve Replacement With the Harmony Valve in Patients Who Do Not Meet Recommended Oversizing Criteria on the Screening Perimeter Plot.

BACKGROUND: Anatomic selection for Harmony valve implant is determined with the aid of a screening report and perimeter plot (PP) that depicts the perimeter-derived radius along the right ventricular outflow tract (RVOT) and projects device oversizing. The PP provides an estimation of suitability for implant, but its sensitivity as a screening method is unknown. This study was performed to describe anatomic features and outcomes in patients who underwent Harmony TPV25 implant despite a PP that predicted inadequate oversizing. METHODS: We reviewed RVOT anatomic features and measurements in patients who underwent transcatheter pulmonary valve replacement with the Harmony TPV25 device despite a PP that predicted inadequate oversizing. RESULTS: This study included 22 patients. There were no unsuccessful implants or adverse valve-related events. Anatomic features varied, but all patients fit into 1 of 3 anatomic types characterized by differences in RVOT dimensions. Type 1 patients (n=9) had a long RVOT with a choke point and a wide main pulmonary artery. Type 2 patients (n=6) had a short RVOT that was pyramidal in shape, with no choke point, and extensive main pulmonary artery lengthening/expansion during systole. Type 3 patients (n=7) had a short, bulbous main pulmonary artery with a choke point and an open pulmonary artery bifurcation. CONCLUSIONS: Transcatheter pulmonary valve replacement with the Harmony valve is feasible in some patients whose PP fit analysis predicts inadequate oversizing. All cases in this series fit into 1 of 3 anatomic patterns, which are not identified in the screening report. Implanters must review cases individually to assess the feasibility of the implant.

Bioptome-assisted blunt dissection facilitates endovascular retrieval of a chronically embolized stent.

We present a 19-year-old female with history of d-transposition of the great arteries status post-arterial switch operation.

Right ventricular outflow tract obstruction associated with neointimal tissue accumulation and distortion of the Harmony TPV25 stent frame: Potential mechanisms and treatment.

BACKGROUND: The Harmony TPV25 transcatheter pulmonary valve (Medtronic Inc.) is constructed with a self-expanding stent frame comprising six zigged nitinol wires sewn together and covered with knitted polyester fabric, with flared inflow and outflow ends and a porcine pericardial valve sutured to the central portion of the device. It was approved for treatment of pulmonary regurgitation after prior right ventricular outflow tract repair in 2021. Early outcomes of this procedure have been excellent, but little is known about valve durability or ultimate mechanisms of dysfunction. METHODS: We collected data on patients who underwent reintervention for TPV25 dysfunction and described findings related to distortion of the stent frame and tissue accumulation. RESULTS: We describe six patients who underwent valve-in-valve implant for TPV25 obstruction (peak catheterization gradient peak 28-73 mmHg) 10-28 months after implant. In all cases, there was tissue accumulation within the inflow and valve-housing segments of the device and deformation of the self-expanding valve frame characterized by variable circumferential narrowing at the junction between the valve housing and the inflow and outflow portions of the device, with additional geometric changes in all segments. All six patients underwent valve-in-valve implant that results in a final peak gradient ≤10 mmHg and no regurgitation. DISCUSSION: The occurrence of short-term Harmony TPV25 dysfunction in multiple patients with a similar appearance of frame distortion and tissue accumulation within the inflow and valve housing portions of the device suggests that this may be an important failure mechanism for this valve. Potential causes of the observed findings are discussed. It is possible to treat this mechanism of TPV25 dysfunction with valve-in-valve implant using balloon expandable transcatheter valves.

1-Year Outcomes in a Pooled Cohort of Harmony Transcatheter Pulmonary Valve Clinical Trial Participants.

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.

Clinical Outcomes of Percutaneous Fontan Stenting in Adults.

BACKGROUND: Fontan pathway stenosis is a well-known complication after palliation. Percutaneous stenting is effective for angiographic/hemodynamic relief of Fontan obstruction, but its clinical impact in adults remains unknown. METHODS: This was a retrospective cohort of 26 adults undergoing percutaneous stenting for Fontan obstruction from 2014 to 2022. Procedural details, functional capacity, and liver parameters were reviewed at baseline and during follow-up. RESULTS: Median age was 22.5 years (interquartile range [IQR] 19-28.8 y); 69% were male. After stenting, Fontan gradient significantly decreased (2.0 ± 1.9 vs 0 [IQR 0-1] mm Hg; P < 0.005), and minimal Fontan diameter increased (11.3 ± 2.9 vs 19.3 [IQR 17-20] mm; P < 0.001). One patient developed acute kidney injury periprocedurally. During a follow-up of 2.1 years (IQR 0.6-3.7 y), 1 patient had thrombosis of the Fontan stent and 2 underwent elective Fontan re-stenting. New York Heart Association functional class improved in 50% of symptomatic patients. Changes in functional aerobic capacity on exercise testing were directly related to pre-stenting Fontan gradient (n = 7; r = 0.80; P = 0.03) and inversely related to pre-stenting minimal Fontan diameter (r = -0.79; P = 0.02). Thrombocytopenia (platelet count < 150 109/L) was present in 42.3% of patients before and in 32% after the procedure (P = 0.08); splenomegaly (spleen size > 13 cm) was present in 58.3% and 58.8% (P = 0.57), respectively. Liver fibrosis (aspartate transaminase to platelet ratio index and Fibrosis-4) scores were unchanged after the procedure compared with baseline. CONCLUSIONS: Percutaneous stenting in adults is safe and effective in relieving Fontan obstruction, resulting in subjective improvement in functional capacity in some. A subset of patients demonstrated improvement in markers of portal hypertension, suggesting that Fontan stenting could improve Fontan-associated liver disease in select individuals.

Pre- and Postprocedure Imaging of Transcatheter Pulmonary Valve Implantation.

Transcatheter pulmonary valve replacement (TPVR) is a minimally invasive procedure for treatment of right ventricular outflow tract (RVOT) dysfunction in surgically repaired congenital heart diseases. TPVR is performed in these patients to avoid the high risk and complexity of repeat surgeries. Several TPVR devices are now available to be placed in the right ventricle (RV) to pulmonary artery (PA) conduit, native RVOT, or surgical bioprosthetic valves. Imaging is used before TPVR to determine patient eligibility and optimal timing, which is critical to avoid irreversible RV dilatation and failure. Imaging is also required for evaluation of contraindications, particularly proximity of the RVOT to the left main coronary artery and its branches. Cross-sectional imaging provides details of the complex anatomy in which the TPVR device will be positioned and measurements of the RVOT, RV-PA conduit, or PA. Echocardiography is the first-line imaging modality for evaluation of the RVOT or conduit to determine the need for intervention, although its utility is limited by the complex RVOT morphology and altered anatomy after surgery. CT and MRI provide complementary information for TPVR, including patient eligibility, assessment of contraindications, and key measurements of the RVOT and PA, which are necessary for procedure planning. TPVR, performed using a cardiac catheterization procedure, includes a sizing step in which a balloon is expanded in the RVOT, which also allows assessment of the risk for extrinsic coronary artery compression. Follow-up imaging with CT and MRI is used for evaluation of postprocedure remodeling and valve function and to monitor complications. ©RSNA, 2022 Online supplemental material is available for this article.

Reintervention and Survival After Transcatheter Pulmonary Valve Replacement.

BACKGROUND: Transcatheter pulmonary valve (TPV) replacement (TPVR) has become the standard therapy for postoperative pulmonary outflow tract dysfunction in patients with a prosthetic conduit/valve, but there is limited information about risk factors for death or reintervention after this procedure. OBJECTIVES: This study sought to evaluate mid- and long-term outcomes after TPVR in a large multicenter cohort. METHODS: International registry focused on time-related outcomes after TPVR. RESULTS: Investigators submitted data for 2,476 patients who underwent TPVR and were followed up for 8,475 patient-years. A total of 95 patients died after TPVR, most commonly from heart failure (n = 24). The cumulative incidence of death was 8.9% (95% CI: 6.9%-11.5%) 8 years after TPVR. On multivariable analysis, age at TPVR (HR: 1.04 per year; 95% CI: 1.03-1.06 per year; P < 0.001), a prosthetic valve in other positions (HR: 2.1; 95% CI: 1.2-3.7; P = 0.014), and an existing transvenous pacemaker/implantable cardioverter-defibrillator (HR: 2.1; 95% CI: 1.3-3.4; P = 0.004) were associated with death. A total of 258 patients underwent TPV reintervention. At 8 years, the cumulative incidence of any TPV reintervention was 25.1% (95% CI: 21.8%-28.5%) and of surgical TPV reintervention was 14.4% (95% CI: 11.9%-17.2%). Risk factors for surgical reintervention included age (0.95 per year [95% CI: 0.93-0.97 per year]; P < 0.001), prior endocarditis (2.5 [95% CI: 1.4-4.3]; P = 0.001), TPVR into a stented bioprosthetic valve (1.7 [95% CI: 1.2-2.5]; P = 0.007), and postimplant gradient (1.4 per 10 mm Hg [95% CI: 1.2-1.7 per 10 mm Hg]: P < 0.001). CONCLUSIONS: These findings support the conclusion that survival and freedom from reintervention or surgery after TPVR are generally comparable to outcomes of surgical conduit/valve replacement across a wide age range.

Multicenter Study of Endocarditis After Transcatheter Pulmonary Valve Replacement.

BACKGROUND: Endocarditis has emerged as one of the most impactful adverse events after transcatheter pulmonary valve replacement (TPVR), but there is limited information about risk factors for and outcomes of this complication. OBJECTIVES: The purpose of this study was to evaluate risk factors for and outcomes of endocarditis in a large multicenter cohort. METHODS: The authors established an international registry focused on characterizing endocarditis after TPVR, including the incidence, risk factors, characteristics, and outcomes. RESULTS: Investigators submitted data for 2,476 patients who underwent TPVR between July 2005 and March 2020 and were followed for 8,475 patient-years. In total, 182 patients were diagnosed with endocarditis a median of 2.7 years after TPVR, for a cumulative incidence of 9.5% (95% CI: 7.9%-11.1%) at 5 years and 16.9% (95% CI: 14.2%-19.8%) at 8 years (accounting for competing risks: death, heart transplant, and explant) and an annualized incidence of 2.2 per 100 patient-years. Staphylococcus aureus and Viridans group Streptococcus species together accounted for 56% of cases. Multivariable analysis confirmed that younger age, a previous history of endocarditis, and a higher residual gradient were risk factors for endocarditis, but transcatheter pulmonary valve type was not. Overall, right ventricular outflow tract (RVOT) reintervention was less often to treat endocarditis than for other reasons, but valve explant was more often caused by endocarditis. Endocarditis was severe in 44% of patients, and 12 patients (6.6%) died, nearly all of whom were infected with Staphylococcus aureus. CONCLUSIONS: The incidence of endocarditis in this multicenter registry was constant over time and consistent with prior smaller studies. The findings of this study, along with ongoing efforts to understand and mitigate risk, will be critical to improve the lifetime management of patients with heart disease involving the RVOT. Although endocarditis can be a serious adverse outcome, TPVR remains an important tool in the management of RVOT dysfunction.

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.

Management and Outcomes of Transvenous Pacing Leads in Patients Undergoing Transcatheter Tricuspid Valve Replacement.

OBJECTIVES: The aim of this study was to determine the prevalence of pacemaker lead-related complications following transcatheter tricuspid valve replacement (TTVR). BACKGROUND: The rate of permanent pacemaker implantation following tricuspid valve (TV) surgery is high, and many patients have transvenous leads. The feasibility, safety, and outcomes of subsequently performing TTVR in the setting of transvenous pacemaker leads have not been established. METHODS: The VIVID (Valve-in-Valve International Database) registry was used to review 329 patients who underwent TTVR following TV repair or replacement. Patients were subdivided into 3 cohorts for intergroup comparisons: no lead, epicardial lead, and transvenous lead (entrapped or not entrapped during the TTVR procedure). RESULTS: Of 329 patients who underwent TTVR, 128 (39%) had prior pacing systems in place, 70 with epicardial and 58 with transvenous leads. A total of 31 patients had leads passing through the TV. Three patients had the right ventricular (RV) lead extracted prior to TTVR. The remaining 28 patients had the RV lead entrapped between the transcatheter TV implant and the surgical valve (n = 22) or the repaired TV (n = 6). One patient had displacement of the RV lead during the procedure, and 2 experienced lead failure during follow-up. Overall, there was no significant difference in the cumulative incidences of competing outcomes (death, TV reintervention, TV dysfunction) between patients with and those without pacing leads or entrapped RV leads. CONCLUSIONS: TTVR in the setting of trans-TV pacemaker leads without lead extraction or re-replacement can be performed safely with a low risk for complications, offering an alternative to surgical TV replacement.

Thirty-Day Outcomes of Transcatheter Mitral Valve Replacement for Degenerated Mitral Bioprostheses (Valve-in-Valve), Failed Surgical Rings (Valve-in-Ring), and Native Valve With Severe Mitral Annular Calcification (Valve-in-Mitral Annular Calcification) in the United States: Data From the Society of Thoracic Surgeons/American College of Cardiology/Transcatheter Valve Therapy Registry.

BACKGROUND: Transcatheter mitral valve replacement using aortic transcatheter heart valves has recently become an alternative for patients with degenerated mitral bioprostheses, failed surgical repairs with annuloplasty rings or severe mitral annular calcification who are poor surgical candidates. Outcomes of these procedures are collected in the Society of Thoracic Surgeons/American College of Cardiology/Transcatheter Valve Therapy Registry. A comprehensive analysis of mitral valve-in-valve (MViV), mitral valve-in-ring (MViR), and valve-in-mitral annular calcification (ViMAC) outcomes has not been performed. We sought to evaluate short-term outcomes of early experience with MViV, MViR, and ViMAC in the United States. METHODS: Retrospective analysis of data from the Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry. RESULTS: Nine hundred three high-risk patients (median Society of Thoracic Surgeons score 10%) underwent MViV (n=680), MViR (n=123), or ViMAC (n=100) between March 2013 and June 2017 at 172 hospitals. Median age was 75 years, 59.2% female. Technical and procedural success were higher in MViV. Left ventricular outflow tract obstruction occurred more frequently with ViMAC (ViMAC=10%, MViR=4.9%, MViV=0.7%; P<0.001). In-hospital mortality (MViV=6.3%, MViR=9%, ViMAC=18%; P=0.004) and 30-day mortality (MViV=8.1%, MViR=11.5%, ViMAC=21.8%; P=0.003) were higher in ViMAC. At 30-day follow-up, median mean mitral valve gradient was 7 mm Hg, most patients (96.7%) had mitral regurgitation grade ≤1 (+) and were in New York Heart Association class I to II (81.7%). CONCLUSIONS: MViV using aortic balloon-expandable transcatheter heart valves is associated with a low complication rate, a 30-day mortality lower than predicted by the Society of Thoracic Surgeons score, and superior short-term outcomes than MViR and ViMAC. At 30 days, patients in all groups experienced improvement of symptoms, and valve performance remained stable. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT02245763.

Transcatheter closure of coronary artery fistula: A 21-year experience.

OBJECTIVES: To evaluate the outcomes of transcatheter coronary artery fistula (CAF) closure and to identify anatomic/procedural factors that may impact outcomes. BACKGROUND: Due to the rarity of CAF, reported experience with transcatheter closure remains limited and anatomic and procedural factors that may lead to unsuccessful closure, complications, or recanalization of CAF are unclear. METHODS: All patients who underwent transcatheter CAF closure at Mayo Clinic from 1997 to 2018 were retrospectively reviewed. CAF anatomic characteristics, procedural techniques, and clinical/angiographic outcomes were assessed. RESULTS: A total of 45 patients underwent transcatheter closure of 56 CAFs. The most commonly used devices were embolization coils in 40 (71.4%) CAFs, vascular occluders in 10 (17.8%), or covered stent in 2 (3.6%). Acute procedural success with no or trivial residual flow occurred in 50 (89.3%) CAFs. Residual flow was small in three (5.4%) and large in three (5.4%). Eight (17.8%) patients had complications, including device migration in three, intracranial hemorrhage from anticoagulation in one, and myocardial infarction (MI) in four. MI was a result of covered stent thrombosis or stagnation of flow after closure of large distal CAF. Twenty-two patients with 27 CAFs had follow-up angiography after successful index procedure at median time of 423 (IQ 97-1348) days. Of these, 23 (85.2%) had no/trace flow and 4 had large flow from recanalization. CONCLUSIONS: Transcatheter CAF closure is associated with a favorable acute procedural success and complication rate in selected patients. Procedural success and risk for complication are highly dependent on CAF anatomy and closure technique.

Transcatheter Tricuspid Valve-in-Valve and Valve-in-Ring Implantation for Degenerated Surgical Prosthesis.

With explosive growth in the field of transcatheter therapies in recent years, transcatheter valve-in-valve implantation has rapidly emerged as a viable, low-risk alternative to high-risk redo surgical interventions. The authors review the fundamental clinical principles of transcatheter tricuspid valve-in-valve and valve-in-ring implantation as pertinent to the procedural steps and technique.

Tricuspid Valve Imaging and Intervention in Pediatric and Adult Patients With Congenital Heart Disease.

Tricuspid valve abnormalities in congenital heart disease comprise a wide spectrum, with the most common being Ebstein anomaly and tricuspid valve dysplasia. Tricuspid valve dysfunction may also be secondary to other types of congenital heart disease, including functional tricuspid regurgitation seen in right heart volume overload conditions, such as atrial septal defect and repaired tetralogy of Fallot with severe pulmonary valve regurgitation. Congenitally corrected transposition and Mustard and Senning procedures maintain the right ventricle as the systemic ventricle, and the tricuspid valve is subject to unique hemodynamic stress not typically seen in normal circulation. Surgical treatment of tricuspid valve disease remains the mainstay of therapy; primary catheter-based interventions are uncommon. However, once a tricuspid valve has been replaced with a bioprosthesis, the patient may be a candidate for tricuspid valve-in-valve catheter-based replacement.