The most common indications are related to the degree of PR as obtained from conventional echocardiography and more recently by right ventricle volume indices determined by cardiac magnetic resonance (CMR) imaging [
2]. It is crucial to take the overall clinical condition of the patient into account when planning any intervention, especially in the asymptomatic group of patients. The indications for PVR listed below are consistent with American Heart Association/American College of Cardiology guidelines on ACHD [
9‐
13].
1.
Exercise intolerance symptoms attributable to moderate/severe PR and RV volume overload or with syncope and/or tachyarrhythmias.
2.
In asymptomatic patients, RV dilation with RV end systolic volume greater than 80 mL/m2, RV end diastolic volume greater than 150 mL/m2, decreased RV or left ventricle (LV) ejection fraction (EF), or an RVOT aneurysm.
3.
At the time of other cardiac re-operations in TOF patients, PVR should be considered if patients have any of the following features:
a.
RVOT obstruction or severe branch pulmonary artery stenosis.
c.
Residual shunt with Qp:Qs ≥ 1.5.
d.
Patients undergoing aortic valve or root operations.
Preoperative decision-making for surgical PVR
PVR can be accomplished with low mortality in the contemporary era. However, to achieve this, careful patient screening and operative sequence has to be appropriately planned. A Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) analysis of 1,970 reoperations (with ≥ 1 prior sternotomy) in congenital heart disease patients reported a discharge mortality after PVR hospitalization to be 0.41% [
18]. In the sub-group of patients who were adult age (> 18 years), the mortality was 0.26%.
The majority of the risk of PVR is associated with preoperative patient characteristics and resternotomy. Preoperative imaging should be performed to evaluate the risks related to resternotomy, identify the location of key anatomy including location and relation to aorta, previous conduits, RVOT, and coronary anatomy. The presence or absence of atrial communication should be carefully determined by thoroughly reviewing prior operative notes. As most of these patients have undergone numerous cardiac catheterizations, peripheral arterial and venous structures can be comprised. It is important to review preoperative imaging studies to confirm patency of these structures as potential peripheral cannulation sites for cardiopulmonary bypass in the case resternotomy is prohibitive. In addition, peripheral cannulation sites should be pre-emptively prepped into the field and a clear alternate cannulation plan should be in place in the case of resternotomy injury [
19].
Preoperative decision making for transcatheter PVR
A detailed history of the patient’s previous procedures and operations, with a review of their original reports, including central access catheters, cardiac catheterizations, and surgeries, should be performed. Surgical reports should be reviewed in detail to understand the placement of conduits in the presence of abnormal coronary arteries or other perceived challenges. The presence of vascular occlusions might not be apparent on exam or vascular ultrasound due to collateral formation but can be noted based on previous procedural notes or dedicated cross-sectional imaging. Preoperative assessment should also include challenges to anticoagulation, the need for mechanical support, and backup surgical options.
Procedural considerations for TPVR
Dysfunctional RV-PA conduits often exhibit calcification and narrowing, with angiographic diameters notably smaller than their nominal sizes. Conduit tear during preparation for TPVR occurs in 4% to 22% of cases, are often stable and confined, and can be effectively managed with covered stents. Pre-stenting with a bare metal stent before Melody valve implantation reduces stent fracture risk and is now standard practice, necessitating coronary compression testing [
50‐
52].
For native or patched RVOT, the larger diameters required pose challenges for balloon-expandable valves. The advantage of the cobal-chromium frames in the Sapien XT and S3 are less prone to fracture than Melody valves. Pre-stenting may secure a landing zone but can complicate valve delivery. Simultaneous stenting with valve deployment is feasible, offering shorter procedures and reduced radiation [
53].
In dysfunctional bioprosthetic valves (BPV), transcatheter valve-in-valve implantation is viable, with intentional BPV ring fracture facilitating larger valve insertion [
54]. Coronary compression, a potential complication, requires pre-procedural testing to mitigate risks.
Outcomes of surgical PVR
ACHD patients with repaired TOF undergoing PVR have a low mortality risk. This risk is primarily related to resternotomy and the preoperative clinical status of the patient. An STS-CHD report of 6431 patients undergoing PVR with a median age of 17 years, revealed an in-hospital mortality rate of 0.9%. Major in-hospital complications (renal failure requiring persistent dialysis at the time of discharge, stroke, need for permanent pacemaker, postoperative ECMO, phrenic nerve injury, or reintervention before discharge) occurred in only 2.2% of patients [
55]. Another report from the STS Adult Cardiac Surgery Database, (median age at PVR 41 years) reported a higher in-hospital mortality of 4.1%, and major in-hospital complications occurred in 20.9% patients [
56].
Long term outcomes have a direct relationship with the severity of RV dysfunction at the time of the operation. Survival following PVR has been reported as excellent up to 20 years post-operatively. A large meta-analysis including a total of > 3000 patients reported a pooled 5-year mortality of 2.2% and the pooled 5-year re-PVR in < 5% patients [
57]. Sabate Rotes et al. published 40 years (1973–2012) of experience of PVR in TOF patients from Mayo Clinic (Rochester, MN) [
58]. Overall survival was 93% at 5 years, 83% at 10 years, and 80% at 15 years. Overall freedom from pulmonary valve reintervention was 97% at 5 years, 85% at 10 years, and 75% at 15 years.
Outcomes of transcatheter PVR
The Melody valve has the most extensive follow-up data among TPVRs [
59,
60]. The Melody IDE trial (
n = 171) reported a 5-year freedom from reintervention rate of 76% and a freedom from explant rate of 92%. Long-term data (
n = 149) showed a 10-year freedom from mortality rate of 90% and a freedom from reintervention rate of 60%. At 10 years, freedom from TPV dysfunction was 53%, with reoperation freedom at 79%. Higher reintervention rates were observed in patients under 21, without a protected conduit, or with stenosis as the primary TPVR indication. Freedom from infective endocarditis was 81% at 10 years, with an annual rate of 2.0%.
The COMPASSION trial demonstrated a 95.2% procedural success rate with no procedural mortality and a 93.7% freedom from reintervention at 3 years. In a registry of 774 patients, procedural success was 97.4% with an infective endocarditis (IE) incidence of 1.7% per patient-year. Long-term data for the Edward Sapien TPV are not yet available. Acute results from the COMPASSION trial (
n = 70) showed a 95.2% procedural success rate, no procedural mortality, and 100% and 98.4% freedom from all-cause mortality at 1 and 3 years, respectively. Freedom from reintervention at 3 years was 93.7%, and freedom from IE was 97.1%. In a large retrospective multicenter registry involving 774 patients treated with Sapien XT or S3, procedural success was 97.4%, with two procedural deaths and 10% reporting serious procedural adverse events. The IE incidence in this cohort was 1.7% per patient-year, with no IE-related deaths [
61,
62].
In a large multicenter registry of TPVR involving 2,476 patients (82% with Melody TPV and 18% with Sapien TPV), procedural mortality was 0.3%. Eight years post-TPVR, the cumulative incidence of death was 8.9% (95% CI: 6.9%—11.5%), with heart failure being the most common cause. Factors associated with increased mortality included age at implant, existing prosthetic valves in other positions, and the presence of a transvenous pacemaker/defibrillator. The cumulative incidence of any reintervention at 8 years was approximately 25%, with surgical reintervention at 14.4%.In the prospective non-randomized "Munich Comparative Study," comparing Melody TPVR (
n = 241) to SPVR (
n = 211), 10-year survival was 94% in the Melody group and 92% in the SPVR group, showing no significant difference. Freedom from reintervention was similar in both groups [
63,
64].
In the Harmony early feasibility study, 20 out of 21 patients received the Harmony TPV 22, with one patient excluded due to pulmonary hypertension. Three- and five-year outcomes showed no procedural mortality, though one patient with coronary artery disease died 3.5 years post-procedure, unrelated to the TPV. The pivotal study followed, leading to FDA approval in 2021. The continued-access phase included 87 patients, showing high procedural success and no mortality at one year. Freedom from PR, stenosis, and reintervention was 98% for TPV 22 and 91% for TPV 25 [
65].
In the Alterra early feasibility study [
66], fifteen out of 29 screened patients were enrolled. All procedures were successful, with the Alterra placed as intended, the 29 mm Sapien valve implanted, and no occurrences of significant stenosis or more than mild regurgitation. There were no incidents of mortality, reintervention, infective endocarditis, stroke, or myocardial infarction within the six-month follow-up period [
67].
In patients undergoing TPVR, including balloon expandable valves, infective endocarditis (IE) is a recognized risk associated with bioprosthetic materials. Large retrospective studies indicate an annual incidence ranging from 1.95% to 3.6% per patient-year, with cumulative incidences of 9.5% to 16.9% at 5 to 8 years post-implantation. Mortality rates from IE following TPVR range from 6.6% to 14%. Risk factors for IE include younger age, residual gradient (> 15 mmHg), and a history of previous IE [
68]. Comparatively, the Sapien valve shows a lower incidence of IE than other bioprosthetic materials used in TPVR. For instance, in the Munich Comparative Study, the annualized incidence of IE was 1.6% for the Melody valve compared to 0.5% for other surgical prosthetic valve replacements (SPVR). Studies also suggest that Sapien TPV has a lower incidence of IE compared to other conduits like Contegra.
Self-expanding valves have been associated with non-sustained ventricular tachycardia (VT), particularly due to interaction with the device's proximal aspect in the RV. In a study, VT occurred in 40% of patients within 24 h of valve implantation, with most cases being non-sustained except in one patient who experienced sustained VT. The location of annular implantation was identified as a contributing factor to VT incidence [
66].
In a multi-center retrospective study, researchers investigated transcatheter pulmonary valve (TPV) outcomes in patients over 40 years old. Key findings include successful TPV in 87% of cases, significant improvement in peak-to-peak gradient for PS/PS-PR patients, and no procedure-related deaths.
Given the safety of operative intervention and the growing number of catheter-based treatment options, the threshold for PVR is largely still evolving.