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Open Access 24.09.2024 | Cardiology/CT Surgery (K Gist, Section Editor)

Percutaneous and Surgical Pulmonary Valve Replacement Options in Adult Congenital Heart Disease: a Review

verfasst von: Awais Ashfaq, Muhammad Faateh, Shabana Shahanavaz

Erschienen in: Current Treatment Options in Pediatrics | Ausgabe 4/2024

Abstract

Purpose of the Review

Adult congenital heart disease (ACHD) patients are increasing. Valve replacements are one of the most common procedures performed in these subset of patients, especially pulmonary valve replacement. The purpose of the review is to discuss the indications, pre-procedure planning, spectrum of surgical and percutaneous valve replacement options, choice of prosthetic valves and post procedure management in ACHD.

Recent Findings

The choice of prosthesis for pulmonary valve replacement is an ever-evolving process. Various options exist and none have proven to be the standard of care. We discuss different valve prosthesis and their indications.

Summary

Pulmonary valve replacements in ACHD patients continue to increase. Bioprosthetic valves are the most common replacement options, however newer valves with novel technologies are on the horizon. Transcatheter valve replacement has proven to be the preferred approach wherever feasible.
Hinweise

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Introduction

Improvements in outcomes of pediatric congenital cardiac care are leading to an increasing prevalence of adult congenital heart disease (ACHD) patients. Tetralogy of Fallot (TOF) is the most common cyanotic heart defect. The primary goals of TOF repair are to eliminate intracardiac shunting and relieve the right ventricular outflow tract obstruction (RVOT). Pulmonary regurgitation (PR), a common occurrence following TOF repair, was originally considered harmless. However, long-term studies have shown that chronic PR leads to right ventricular (RV) dilatation, biventricular dysfunction, arrhythmias, exercise intolerance, and sudden death [16]. Consequently, TOF management has shifted towards preserving the pulmonary valve, whenever allowed. The optimal timing and technique of pulmonary valve replacement (PVR) requires careful multidisciplinary attention and has been demonstrated to play a role in the long-term survival and quality of life of patients with TOF [3, 7, 8].
There are a variety of available prosthesis types and technical approaches for PVR. This review will focus on different percutaneous and surgical options related to PVR in ACHD.

Indications

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 [913].
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.
 
b.
 ≥ moderate TR.
 
c.
Residual shunt with Qp:Qs ≥ 1.5.
 
d.
Patients undergoing aortic valve or root operations.
 
 

Special considerations in ACHD patients

In addition to the standard preprocedural screening, specific evaluation for acquired comorbidities, arrhythmias, coronary artery disease, renal and hepatic dysfunction, prior sensitization to blood products, layering of acquired vascular risk factors onto native disease might impact the procedure offered. Adults with CHD are known to have higher rates of diabetes and hypertension as compared to the general population [1417].

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.

Role of advanced imaging in transcatheter pulmonary valve replacement

Preprocedural computed tomography (CT) has become a crucial method in identifying suitable candidates for Transcatheter Pulmonary valve replacement (TPVR), as well as providing guidance for interventions. In addition to evaluating vascular occlusions, CT can help assess tortuous vascular pathways and anatomical details, such as bilateral branch PA stenosis, that may necessitate internal jugular or bilateral femoral access [20]. Key aspects of the anatomical position of the conduit in relation to sternum and the aorta as well as characteristics of the conduit, such as degree of calcification, presence of concentric calcification, length of narrowing assessed via CT, helps plan the rehabilitation. Severe concentric calcification and diffuse narrowing may increase the risk of conduit tears during balloon angioplasty and need for placement of covered stents. Coronary artery proximity to the stenotic region and the extent of conduit calcification influence candidacy [21].

Surgical valve choices

a)
Homografts
Cryopreserved aortic and pulmonary homografts may be used for RVOT reconstruction. The advantages of homografts include availability in a wide range of sizes and favorable handling characteristics during implantation. Bifurcating pulmonary or aortic grafts can be directly anastomosed to branch pulmonary arteries in the absence of adequate central pulmonary arteries or in need of augmentation. Major disadvantages include the limited shelf life of each homograft (approximately 2 years), high cost and durability. Freedom from reintervention rates reported in the literature range widely, from 30% to over 80% at 10 years [22, 23]. Smaller conduit size (or younger age at operation) has been consistently shown to be a risk factor for homograft conduit failure. Freedom from reoperation at 10 years is < 50% for homografts of < 19 mm diameter. Other factors that have less consistently been shown to increase the risk of failure include use of aortic homografts, residual branch pulmonary artery stenosis, ABO mismatch, and non-Ross operations (particularly operation for truncus arteriosus) [23, 24].
 
b)
Xenograft conduits
The most commonly used xenograft conduits include bovine jugular vein grafts, porcine pulmonary valved conduit (Shelhigh Inc., Milburn, NJ, USA), and porcine aortic root (Medtronic, Inc., Minneapolis, MN, USA). Advantages of xenograft conduits include abundant supply, availability of small conduits for neonatal applications, excellent handling characteristics, and low cost. The Medtronic Freestyle porcine aortic root has been used for RVOT reconstruction and is available in sizes ranging from 19 to 29 mm [25]. The Medtronic Contegra graft, obtained from bovine jugular vein, consists of a venous valve within a jugular vein conduit. It is available in multiple sizes ranging from 12 to 22 mm. The Medtronic Freestyle porcine aortic root (> 19 mm) appears to have excellent durability at short-term follow-up, but long-term data are pending [26]. Data reported in the literature regarding durability of the bovine jugular vein graft varies significantly, with freedom from reintervention ranging between 66% at 3 years to 90% at 7 years [27].
 
c)
Synthetic valved conduits
Conduits made of Dacron or Polytetrafluoroethylene (PTFE) tube grafts with bioprosthetic, or mechanical valves are available commercially, or can be constructed manually at the time of the operation using PTFE tube and PTFE valve leaflets. This is our preference at Cincinnati Children’s Hospital. The first polymeric valve was reported in 1958 and the first in-human implantation occurred in 1960 [28, 29]. The conduits can be made in a bileaflet or a trileaflet configuration. For larger conduits, we have adopted using a trileaflet configuration and these are constructed using a predefined valve leaflet template and a 0.1 mm thick PTFE membrane. The leaflets are sutured onto their corresponding sinuses on the conduits interior surface [30, 31] (Fig. 1). Advantages of the synthetic valved conduits include excellent longevity, virtually unlimited shelf life making them readily available, and avoidance of anticoagulation. A comparison of PTFE vs homograft after ross procedure in 90 patients over 17 years by Castrillon et al. revealed encouraging midterm results with regards to freedom from reintervention and without differences in hemodynamics or valve function [31].
 
d)
Mechanical valves
Despite the theoretical advantages of long-term durability of mechanical valves, concerns regarding risks of long-term anticoagulation and valve thrombosis, even with adequate anticoagulation, have limited extensive use of these prostheses especially in the pulmonary position. Risk of thrombosis may be higher in patients with right-ventricular dysfunction [32]. Use of mechanical valves may be justified in patients requiring anticoagulation for other reasons, in patients likely to be compliant with long-term anticoagulation, and those in whom risk of reoperation is deemed to be unusually high.
 
e)
Bioprosthetic valves
Bioprosthetic valves have been the most common choice for PVR and are available as stented porcine aortic valves or bovine pericardial valves. They offer several advantages: They do not require anticoagulation, are readily available, easy to implant, and have improved freedom from regurgitation compared with most other valves [33, 34]. Another important advantage of stented bioprosthetic valves is their ability to provide a landing zone for transcatheter valve-in-valve interventions. Placement of a stented bovine pericardial valve in the orthotopic position with transannular patch augmentation is shown in Fig. 2 [35]. Most bioprosthetic PVRs were originally manufactured for use in the aortic position. The largest available bovine pericardial valve for aortic implantation is 29 mm. However, larger mitral bioprosthetic valves can be “reversed” and used in pulmonary position. Various bioprosthetics have historically been used as PV replacements, including the Hancock II (a stented porcine aortic valve), the Carpentier-Edwards PERIMOUNT (a stented bovine pericardial valve) and the Freestyle (a porcine aortic root). In 1999, the bovine jugular vein conduit (BJVC) emerged as a promising graft for RVOT reconstruction due to the valve being naturally integrated within the conduit. A BJVC for surgical delivery was commercialized as the Contegra valve (Medtronic, USA), with utility in RVOT diameters between 12 and 22 mm. The longevity of bioprosthetic valves is limited by their susceptibility to structural valve deterioration (SVD). Calcification is typical of SVD and leads to leaflet stiffening, while various other hallmarks include immune responses and foreign body reaction. Bioprostheses may also fail due to infective endocarditis or thrombosis. It is predominantly a concern after transcatheter procedures, with an incidence between 7.5% and 17% after TPVR [36]. Endocarditis occurs more in BJVCs than in homografts, and the transcatheter Melody valve exhibits significantly greater susceptibility than the surgical Contegra valve [37].
 
f)
New valves
Additional new valves include the Foldax valve, a stented valve with leaflets constructed from siloxane-based poly urethane and has encouraging early results [38].
 

Transcatheter pulmonary valve choices

a)
Balloon expandable valves
i.
Melody valve
The Medtronic Melody TPV, first implanted in 2000 and FDA-approved in 2010, is used for dysfunctional RV-PA conduit and BPV (Fig. 3). Made from a bovine jugular vein sewn into a covered Cheatham Platinum stent, it comes in sizes TPV 20 and TPV 22, with unexpanded heights of 28 mm and 30 mm. The maximum diameter is 22 mm, but dilation to 24 mm has been reported. It is delivered using the Medtronic Ensemble II system (sizes 18, 20, and 22), which simplifies the introduction, delivery, and deployment of the valve [3941]. It includes an integrated balloon, long-sheath, and introducer, allowing percutaneous introduction, guidewire delivery to the RVOT, and deployment without extra sheaths or catheters. The Melody valve is manually crimped onto the balloon, then the sheath is advanced over the balloon-mounted valve. Once implanted, the balloons are deflated, and the delivery system is removed over the guidewire.
 
ii.
Sapien valve
The Edward Sapien transcatheter heart valve, originally for TAVR, was used in an Right ventricle to Pulmonary Artery (RV-PA) homograft in 2006. The Sapien XT and S3, approved in 2016 and 2020, are made from bovine pericardium in a cobalt-chromium stent. The S3, available in sizes 20, 23, 26, and 29 mm, is delivered using the Edward Commander system, though its uncovered valve can risk tricuspid valve injury. Sapien valves, with an outer skirt to minimize leaks, are suitable for larger conduits and native/patched RVOT [4244]. Their higher radial force reduces stent fractures and often negates the need for pre-stenting in the presence of discrete stenosis (Fig. 4).
 
 
b)
Self-expandable valves and adaptive pre-stent systems
i.
Harmony valve
The Medtronic Harmony, the first self-expanding valve for native/patched RVOT, received FDA approval in 2021. Made from treated porcine pericardium in a nitinol wire frame, it comes in two sizes: TPV 22 and TPV 25. TPV 22 is 55 mm long with an inflow diameter of 41 mm and an outflow diameter of 32 mm, housing a 22 mm valve. TPV 25 is 51 mm long with an inflow diameter of 54 mm and an outflow diameter of 43 mm, housing a 25 mm valve. Both are delivered using a 25F system [45] (Fig. 5).
 
ii.
Alterra adaptive prestent
The Alterra Adaptive Prestent, approved by the FDA in 2021, was designed by Edwards to configure RVOT morphology and create a landing zone for the 29 mm Sapien 3 valve. It features a self-expanding nitinol frame partially covered with PTFE, with inflow and outflow diameters of 40 mm and a total length of 49 mm. The covered portion is about 30 mm long. The stent’s uncovered distal end allows placement near pulmonary artery orifices in short landing zones. Its 27 mm waist provides a rigid landing zone for the Sapien valve. Delivered with a 16 F eSheath, it requires a landing zone length of ≥ 35 mm and a diameter between 27 and 38 mm [46] (Fig. 6).
 
iii.
Other self-expanding valve systems
Several other self-expanding valve systems designed and used for dysfunctional native/patched RVOT in various regions in the world with acceptable short and mid-term outcomes [47-49]. None of these are commercially available in the United States yet.
The Venus P-valve (by Venus MedTech, China) is another self-expanding valve made of nitinol stent, with porcine pericardial valve sewn inside it. The valve is covered with pericardial tissue except for the distal flare. The valve diameter ranges from 18–36 mm, in 2 mm increments and the length ranges from 20–35 mm in 5 mm increments. The middle portion where the valve is house is straight, with radiopaque markers at the junction with a proximal and distal flare portion. The flare portion is 10 mm larger than the straight segment. The valve is delivered via a 22- 24F system. Measurements from CMR are used for screening, and selection of the valve [47].
The Med-Zenith PT-valve (by Med-Zenith, China) is made of a self-expanding nitinol frame, covered with porcine pericardium in its entirety. A porcine pericardial valve is sewn in the middle portion, with available valve diameters of 20, 23 and 26 mm. The inflow and outflow portion are equal in size, and come in sizes 28, 32, 36, 40 and 44 mm. The length is 38–54 mm [48].
The Pulsta valve, made by TaeWoong Medical, South Korea is a self-expanding valve made of knitted nitinol wire, covered partially with porcine pericardium. It is available in diameters ranging from 18–28 mm, in 2 mm increments. Both ends are flared symmetrically to 4 mm larger than the middle segment. The length is 28 – 38 mm. The valve is delivered with an 18–20 F delivery system [49].
 
 

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 [5052].
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.

Conclusion

Pulmonary valve replacements in adult congenital heart disease patients continue to increase. Bioprosthetic valves are the most common replacement options, however newer valves with novel technologies are on the horizon. Transcatheter valve replacement has proven to be the preferred approach wherever feasible.

Declarations

Disclosures

Awais Ashfaq: PI for Pyrames.

Competing interests

The authors declare no competing interests.
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Metadaten
Titel
Percutaneous and Surgical Pulmonary Valve Replacement Options in Adult Congenital Heart Disease: a Review
verfasst von
Awais Ashfaq
Muhammad Faateh
Shabana Shahanavaz
Publikationsdatum
24.09.2024
Verlag
Springer International Publishing
Erschienen in
Current Treatment Options in Pediatrics / Ausgabe 4/2024
Elektronische ISSN: 2198-6088
DOI
https://doi.org/10.1007/s40746-024-00313-5

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