Rupture of a calcified right ventricle to pulmonary artery homograft by balloon dilation- emergency rescue by venus P-Valve.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is a recognized alternative treatment to surgery for patients with dysfunctional right ventricular outflow tracts. Patient selection is essential to avoid serious complications from attempted treatment, such as rupture or dissection, especially of the calcified outflow tracts. We describe a case with an unexpected rupture of a calcified homograft valve and main pulmonary artery, which was treated successfully by emergency implantation of a self-expanding Venus P-Valve (Venus MedTech, Hangzhou, China) without the need for pre-stenting with a covered stent. CASE DETAILS: A 13-year-old boy had two previous operations of tetralogy of Fallot, one a total repair and the other a homograft valved conduit for pulmonary regurgitation. He presented with dyspnea and severe right ventricular outflow tract obstruction (RVOTO) and had a calcified outflow tract and main pulmonary artery. In the catheter laboratory, a non-compliant balloon dilation resulted in a contained rupture of the conduit. The patient remained hemodynamically stable, and the rupture was treated with a self-expandable Venus P-Valve without the need for a covered stent combined with a balloon-expandable valve or a further surgical procedure. DISCUSSION: Preprocedural evaluation with an inflating balloon is necessary to examine tissue compliance and determine suitability for PPVI. However, this condition is accompanied by a risk of conduit rupture. Risk factors of this complication are calcification and homograft use. These ruptures are mostly controlled with a prophylactic or therapeutic covered stent, with a low rate of requiring surgery. However, there are severe ruptures which lead to hemothorax and death. In the available literature, there was no similar reported case of conduit rupture, which a self-expandable Pulmonary valve stent has managed. It seems that fibrosis and collagen tissue around the heart, formed after open surgeries, can contribute to the control of bleeding in these cases. CONCLUSION (CLINICAL LEARNING POINT): The suitability of patients for the PPVI procedure should be examined more carefully, specifically patients with homograft and calcification in their conduit. Furthermore, conduit rupture might be manageable with self-expandable artificial pulmonary valves, specifically in previously operated patients, and the applicability of this hypothesis is worth examining in future research.

Total Correction for Tetralogy of Fallot in Patients Weighing Over 10 kg: Experiences and Follow-Up Outcomes.

Background Although the recommended time for total correction of tetralogy of Fallot (TOF) is during infancy, sometimes TOF cases present to healthcare setups after pre-school age, with some cases presenting even beyond adolescence in developing countries. The objective of this study was to assess patients with TOF weighing 10 kg and above who underwent definitive corrective surgical techniques such as transannular patch (TAP), valve-sparing right ventricular outflow tract (RVOT) pericardial patch augmentation, non-ventriculotomy infundibular resection for postoperative complications, hospital stay, and right ventricular (RV) dysfunction in the immediate postoperative period and subsequent outpatient department follow-ups. Methodology This comprehensive, retrospective cohort study included single-center data collected between January 16, 2018, and January 15, 2024. The study included 63 patients diagnosed with TOF weighing 10 kg and above, ensuring a robust and representative sample. Results Of the 119 patients who underwent total correction for TOF, 63 met the study's inclusion criteria of TOF weighing above 10 kg. Of the 63 patients, 55.6% were males, and 44.4% were females. The mean weight of the study participants was 33.4 kg. The mean age was 15.9 years. Of the 63 patients, 39 underwent TAP surgery, 18 underwent RVOT patch augmentation, and six underwent total correction by non-ventriculotomy infundibular resection. There was a significant difference between the type of surgery groups and RV dysfunction, with the TAP group showing a higher incidence of RV dysfunction, indicating a potential risk factor associated with this technique. Conclusions Although TAP has significant immediate postoperative complications compared to other techniques, its long-term follow-up suggests that long-term survival and quality of life, as measured by major adverse cardiac events such as heart failure, arrhythmias, and reoperation rates, are commensurable in adulthood. This indicates that despite the initial challenges, TAP can provide satisfactory outcomes in the long run.

Transcatheter Pulmonary Valve in Congenital Heart Disease.

Over the last 2 decades, experience with transcatheter pulmonary valve replacement (TPVR) has grown significantly and has become an effective and reliable way of treating pulmonary valve regurgitation, right ventricular outflow (RVOT) obstruction, and dysfunctional bioprosthetic valves and conduits. With the introduction of self-expanding valves and prestents, dilated native RVOT can be addressed with the transcatheter approach. In this article, the authors review the current practices, technical challenges, and outcomes of TPVR.

Right ventricular outflow tract stenting for late presenter unrepaired Fallot physiology: a single-center experience.

Objectives: The purpose of this study was to assess the clinical outcome after right ventricular outflow tract (RVOT) stenting in late presenter patient with unrepaired Fallot physiology. Background: In younger patients, RVOT stenting is an alternative to mBTT shunt; however, there have been few reports of this palliative technique in late presenter population, including adults. Methods: This was a single-center, retrospective study of nonrandomized, palliated Fallot patients. Clinical outcomes such as left ventricular ejection fraction and saturation were measured in 32 individuals following RVOT stenting in adults (n = 10) and children (n = 22). The Statistical Package for Social Science (SPSS) 26.0 software was used to analyze the statistical data. Results: During the procedure, the average stent diameter and length were 8.84 ± 1.64 mm and 35.46 ± 11.23 mm, respectively. Adult patients received slightly longer stents than pediatric patients (43.60 ± 11.64 mm vs. 31.77 ± 9.07 mm). Overall, patients' saturation increased from 58.56 ± 19.03% to 91.03 ± 8.98% (p < 0.001), as did their left ventricular ejection fraction (LVEF) from 64.00 ± 18.21% to 75.09 ± 12.98% (p = 0.001). Three patients improved their LVEF from 31 to 55%, 31 to 67%, and 26 to 50%. The median length of stay was 8 (2-35) days, with an ICU stay of 2 (0-30) days. The median time from RVOT stent palliation to total repair was 3 months (range: 1 month-12 months). Conclusions: RVOT stenting is a safe and effective method for increasing saturation and ejection fraction not only in newborn infants but also in late presenters, including adults with unrepaired Fallot physiology.

Cine MRI-derived radiomics features indicate hemodynamic changes in the pulmonary artery.

Although cine MRI-derived radiomics features in the cardiac blood pool have been used to represent cardiac function and motion, the clinical relevance of radiomics features in the great vessels is still unknown. The aim of the present study was to test the hypothesis that cine MRI-derived radiomics features of the pulmonary artery (PA) can represent hemodynamic abnormalities in pulmonary hypertension (PH). With the approval of the institutional review board (IRB), 50 PH patients (21 males, 36-89 years old, diagnosed with right heart catheterization [RHC]) and 23 healthy volunteers (14 males, 26-80 years old) were retrospectively enrolled in this study. All participants underwent cardiac 4D flow and cine MRI (25 retrospective phases) at the right ventricular (RV) outflow tract (RVOT). A total of 93 radiomics features were extracted from RVOT cine images through a fixed size region of interest (ROI) at the proximal part of the PA. The peak values of the 6 first order features were different between the PH patients and controls. 4D flow-derived mean velocity in PA was related to 'Kurtosis' (r = 0.452,), 'Range' (r = 0.426), 'Autocorrelation' (r = 0.407), 'Joint Average' (r = 0.459), 'Sum Average' (r = 0.459), 'High Gray Level Emphasis' (r = 0.41), 'Large Dependence High Gray Level Emphasis' (r = 0.44), 'High Gray Level Run Emphasis' (r = 0.422), 'Gray Level Variance' (r = 0.419), 'High Gray Level Zone Emphasis' (r = 0.451), and 'Small Area High Gray Level Emphasis' (r = 0.415). Mean RV pressure was related to 'Inverse Variance' (r = 0.43) and 'Run Percentage' (r = 0.403). All p values < 0.05. Cine MRI-derived PA radiomics features have the potential to serve as novel imaging biomarkers for representing hemodynamic changes in pulmonary circulation.

Stenting of native right ventricular outflow tract obstructions in symptomatic infants: histological work-up of explanted specimen.

BACKGROUND: Stenting of stenotic right ventricular outflow tract is a palliative measure for severely impaired small babies with Tetralogy of Fallot or similar pathologies. Little is known about the histopathological fate of the stents in the right ventricular outflow tract. METHODS: Eight samples of surgically removed right ventricular outflow tract stents were histologically analysed according to a predefined protocol. RESULTS: The most frequent diagnosis was Tetralogy of Fallot in four patients, pulmonary atresia with ventricular septal defect in two patients, double outlet right ventricle with pulmonary obstruction in one patient, and muscular obstruction of the right ventricular outflow tract in one patient with a syndromic disease with hypertrophic cardiomyopathy. Stents mean implantation duration was 444 days ranging from 105 to 1117 days (median 305.5 days). Histology revealed a variable degree of pseudointima formation consisting of fibromuscular cells surrounded by extracellular matrix. Four of the specimen contained adjacent myocardial tissue fragments, which showed regressive changes. Neither myocardium nor pseudointima tissue or tissue parts locally related to stent struts were infiltrated by inflammatory cells. CONCLUSIONS: Histological analysis after explantation of early-in-life implanted right ventricular outflow tract stents revealed predominantly pronounced neo-intimal proliferation with a visible endothelial layer, no signs of inflammation, and no prolapse of muscular tissue through the stent struts. Thus, implantation of stents in early life seems to interfere little with the hosts' immune system and might help to open up the right ventricular outflow tract by mechanical forces and regressive changes in adjacent muscular tissue.

The Utility of Invengenx® Bovine Patch for Right Ventricular Outflow Tract (RVOT) Reconstruction and Augmentation in the Surgical Management of Tetralogy of Fallot (TOF): A Contemporary Study and Review of the Literature.

Background and objective Complex congenital heart diseases (CHDs), such as the tetralogy of Fallot (TOF), often warrant reconstruction and augmentation of the right ventricular outflow tract (RVOT). This procedure requires the use of both synthetic and natural materials. However, finding the ideal material for tissue implants can be challenging. Biological materials often face issues such as tissue degeneration, calcium deposition, antigenicity, rejection, shrinkage, and fibrosis. These issues can lead to complications such as stenosis and insufficiency, potentially requiring early reoperations. In light of this, this study aimed to investigate the effectiveness of the Invengenx® bovine patch for RVOT reconstruction and augmentation. Methods This was a retrospective observational study conducted among eight children who underwent TOF correction cardiac surgery. Their demographic and clinical characteristics, intraoperative findings, and postoperative follow-up results at six months were collected from the hospital patient database. Results There were no deaths or complications in this study. We observed a significant reduction in the gradient across the pulmonary valve and the outflow tract at six months post-procedure. The analysis demonstrated that the Invengenx® bovine patch was successful and did not lead to any complications. Conclusions This study demonstrates the safety and efficacy of this engineered bovine pericardial patch (Invengenx®) as a cardiovascular substitute for surgical repair of both simple and more complex congenital cardiac defects.

Techniques for Cardiac Resynchronization Therapy in Patients with Congenital Heart Disease.

Cardiac resynchronization therapy (CRT) for congenital heart disease has shown promising suucess as an adjunct to medical therapy for heart failure. While cardiac conduction defects and need for ventricular pacing are common in congential heart disease, CRT indications, techniques and long term outcomes have not been well establaished. This is a review of the techniques nad short term outcomes of CRT for the following complex congenital heart disease conditions: single ventricle physiology, systemic right ventricle, and the subpulmonic right ventricle.

Transcatheter Interventions for Neonates with Congenital Heart Disease: A Review.

Newborns with congenital heart disease often require interventions linked to high morbidity and mortality rates. In the last few decades, many transcatheter interventions have become the first-line treatments for some critical conditions in the neonatal period. A catheter-based approach provides several advantages in terms of procedural time, length of hospitalization, repeatability and neurodevelopmental issues (usually related to cardiopulmonary bypass). The main transcatheter procedures will be reviewed, as they are now valid alternatives to conventional surgical management.

Pentagalloyl glucose-stabilized decellularized bovine jugular vein valved conduits as pulmonary conduit replacement.

Congenital heart diseases (CHD) are one of the most frequently diagnosed congenital disorders, affecting approximately 40,000 live births annually in the United States. Out of the new patients diagnosed with CHD yearly, an estimated 2,500 patients require a substitute, non-native conduit artery to replace structures congenitally absent or hypoplastic. Devices used for conduit replacement encounter limitations exhibiting varying degrees of stiffness, calcification, susceptibility to infection, thrombosis, and a lack of implant growth capacity. Here, we report the functionality of pentagalloyl glucose (PGG) stabilized decellularized valved bovine jugular vein conduit (PGG-DBJVC). The PGG-DBJVC tissues demonstrated mechanical properties comparable to native and glutaraldehyde fixed tissues, while exhibiting resistance to both collagenase and elastase enzymatic degradation. Subcutaneous implantation of tissues established their biocompatibility and resistance to calcification, while implantation in sheep in the pulmonary position demonstrated adequate implant functionality, and repopulation of host cells, without excessive inflammation. In conclusion, this PGG-DBJVC device could be a favorable replacement option for pediatric patients, reducing the need for reoperations required with current devices. STATEMENT OF SIGNIFICANCE: Congenital Heart Disease (CHD) is a common congenital disorder affecting many newborns in the United States each year. The use of substitute conduit arteries is necessary for some patients with CHD who have missing or underdeveloped structures. Current conduit replacement devices have limitations, including stiffness, susceptibility to infection and thrombosis, and lack of implant growth capacity. Pentagalloyl glucose-stabilized bovine jugular vein valved tissue (PGG-DBJVC) offers a promising solution as it is resistant to calcification, and biocompatible. When implanted in rats and as pulmonary conduit replacement in sheep, the PGG-DBJVC demonstrated cellular infiltration without excessive inflammation, which could lead to remodeling and integration with host tissue and eliminate the need for replacement as the child grows.

Unmet Clinical Needs for Transcatheter Pulmonary Valves.

A common feature of congenital heart disease is the presence of right ventricular outflow tract (RVOT) obstruction that can range from mild to severe and can lead to atresia of the pulmonary valve, in extreme conditions. RVOT abnormalities can frequently be corrected surgically or via interventional means. However, most of these patients will ultimately develop pulmonary valve insufficiency and eventual right ventricular dilation, which will require a pulmonary valve replacement at some point in their life to mitigate the detrimental effects of pulmonary valve regurgitation (PVR) on the right ventricle (RV). The evolution from the studies done by Philip Bonhoeffer to implant a pulmonary valve via transcatheter means, have provided a bedrock for transcatheter pulmonary valve replacement (TPVR). Yet, several areas of unmet need for a demographic of patients still exist. Here, we discuss the clinical unmet needs in children under 20 Kg and expand the use of hybrid and other TPVR approaches along with the current indications and contraindications for pulmonary valve replacement. The constraints and limitations from commercially available pulmonary valves will be discussed from a clinical standpoint. Finally, we explore the use of hybrid and periventricular delivery of transcatheter pulmonary valves in younger patients.

Clinical characteristics of tetralogy of Fallot with left pulmonary artery acute angulation on computed tomography.

Left pulmonary artery (LPA) stenosis with acute angulation commonly necessitates surgical revision in the treatment of tetralogy of Fallot (TOF). We investigated the clinical characteristics of acute angulation of the LPA in patients with TOF via computed tomography (CT). Between 2011 and 2022, 160 patients were diagnosed with TOF using CT. After excluding 28 patients due to insufficient records or age, 132 patients were included in the present analysis. The patients were divided into two groups according to the presence or absence of acute angulation of the LPA: group I (TOF with an acute angle of the LPA, n = 53) and group II (TOF without an acute angle of the LPA, n = 79). We retrospectively collected clinical data from electronic medical records. T-tests were used to analyze continuous variables (i.e., age, sex, weight, right ventricular outflow tract [RVOT] pressure on echocardiography, and distance to bifurcation), and Fisher's exact and chi-square tests were used to analyze categorical data (i.e., presence of the right aortic arch, pulmonary arterial atresia/hypoplasia, atrial septal defect [ASD], patent ductus arteriosus [PDA], and pulmonary valve). The groups did not differ significantly in terms of sex, weight, presence of the right aortic arch, pulmonary arterial atresia/hypoplasia, ASD, or distance to the bifurcation. Moreover, there was no significant difference between the presence and absence of PDA (P = 0.057); however, patients in group I were significantly older (143.2 ± 97.8 days) than those in group II (91.1 ± 76.0 days, P = 0.002). Furthermore, RVOT pressure was significantly higher among patients in group I (45.1 ± 22.5 mmHg) than in group II (25.0 ± 12.4 mmHg, P < 0.001). In the current study, acute angulation of the LPA in patients with TOF, as observed on CT, was associated with older age and higher RVOT pressure on echocardiography.

Transannular bovine jugular vein monocusp for the reconstruction of severe right ventricular outflow tract obstruction.

Objective: Severe right ventricular outflow tract obstruction in tetralogy of Fallot and variants necessitates the use of transannular patch in a significant proportion of children undergoing repair. We have used a Contegra monocusp together with delamination of native leaflet tissue in order to create a functioning pulmonary valve. Methods: In total, 18 (2017-2022) consecutive Contegra monocusp implantations were included. Median age and weight were 3.65 [2.00; 9.43] months and 6.12 [4.30; 8.22] kg, respectively. Nine of 18 patients had undergone palliation. Native pulmonary leaflet tissue was recruited to create a single posterior cusp. Contegra monocusp selection was based on the goal to achieve a neoannulus of Z value ≈ 0. Monocusp sizes implanted were 16 [14; 18] mm. Patch plasty of left pulmonary artery (LPA) (9), right pulmonary artery (RPA) (2), and both LPA-RPA (5) were often performed. Results: All patients survived the operation and were discharged home in good health. Median ventilation time and hospital stay were 2 [1; 9] and 12.5 [9; 54] days, respectively. Follow-up duration was 30.68 [3.47; 60.47] months and 100% complete. One patient with well-corrected right ventricular outflow tract died 9.4 months postoperatively, possibly of aspiration. One child with membranous pulmonary atresia needed reoperation (conduit insertion) at 3.5 months of follow-up. Five needed catheter interventions: supravalvar stent (2), LPA stent (3), and RPA stent (1), most of them in the earlier half of the experience. Pulmonary annulus changed from preoperative -3.91 [-5.98; -2.23] to -0.10 [-1.44; 1.92] at discharge; growing proportionally to -0.13 [-3.52; 2.73] at follow-up. Kaplan-Meier freedom from composite dysfunction was 79.25 (95% confidence interval, +13.68%, -31.44%) at 36 months. Conclusions: Recruitment of native leaflets, optimal Contegra monocusp, and commissuroplasty provide an easily replicable technique for achieving a competent, proportionally growing neopulmonary valve. Longer follow-up is needed to determine its impact on delaying a pulmonary valve replacement.

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.

A mid- and long-team follow-up study of the application of single-valved bovine pericardium patches in right ventricular outflow tract reconstruction.

Background: Pulmonary regurgitation following right ventricular outflow tract (RVOT) reconstruction may cause right heart dysfunction and even right heart failure. Installation of a single valve at this time point can effectively reduce pulmonary regurgitation, thereby protecting right heart function. Here, we analyzed the outcomes and mid- and long-term follow-up data of patients undergoing single-valved bovine pericardium patch (svBPP) placement for reconstruction and explored the effectiveness and gaps of svBPP in preventing right heart failure. Methods: A retrospective analysis was performed on patients undergoing RVOT reconstruction using BalMonocTM svBPP from October 2010 to August 2020. The follow up procedures included outpatient visits and collection of outcomes. The cardiac ultrasound-related indicators during the follow-up visits included ejection fraction (EF), right ventricular end-diastolic diameter (EDD), pulmonary regurgitation, and pulmonary artery stenosis. The survival rates and reoperation-free rate were analyzed by Kaplan-Meier method. Results: Patients includes tetralogy of Fallot, pulmonary atresia and other complex congenital heart disease. A total of 5 patients (5.7%) died during the perioperative period. Early complications included pleural effusion, cardiac insufficiency, respiratory insufficiency, chylothorax, and atelectasis, all of which were cured. After discharge, 83 patients (94.3%) were effectively followed up. During follow-up, 1 patient died and 1 patient underwent reoperation. The 1-, 5-, and 10-year survival rates were 98.8%, 98.8%, and 98.8%, respectively, and the reintervention-free rates for the same intervals were 98.8%, 98.8%, and 98.8%, respectively. The last follow-up ultrasound revealed severe pulmonary stenosis in 0 cases, moderate stenosis in 2 cases, mild stenosis in 7 cases, and no stenosis in 73 cases. Pulmonary regurgitation was not found in 12 patients; however, there were 2 cases of severe pulmonary regurgitation, 20 cases of moderate pulmonary regurgitation, and 48 cases of mild pulmonary regurgitation. Conclusions: As shown in the mid- and long-term follow-up studies, BalMonocTM svBPP has good performance in RVOT reconstruction. It can effectively eliminate or reduce pulmonary valve regurgitation and protect right heart function. Both réparation à l'Etage ventriculaire (REV) and the modified Barbero-Marcial procedure can bring growth potential and reduce reoperation rate.

A Novel Mutation in the ADAMTS10 Associated with Weil-Marchesani Syndrome with a Unique Presentation of Developed Membranes Causing Severe Stenosis of the Supra Pulmonic, Supramitral, and Subaortic Areas in the Heart.

Weill-Marchesani syndrome (WMS) is a rare genetic inherited disorder with autosomal recessive and dominant modes of inheritance. WMS is characterized by the association of short stature, brachydactyly, joint stiffness, eye anomalies, including microspherophakia and ectopia of the lenses, and, occasionally, heart defects. We investigated the genetic cause of a unique and novel presentation of heart-developed membranes in the supra-pulmonic, supramitral, and subaortic areas, creating stenosis that recurred after their surgical resection in four patients from one extended consanguineous family. The patients also presented ocular findings consistent with Weill-Marchesani syndrome (WMS). We used whole exome sequencing (WES) to identify the causative mutation and report it as a homozygous nucleotide change c. 232T>C causing p. Tyr78His in ADAMTS10. ADAMTS10 (ADAM Metallopeptidase with Thrombospondin Type 1 Motif 10) is a member of a family of zinc-dependent extracellular matrix protease family. This is the first report of a mutation in the pro-domain of ADAMTS10. The novel variation replaces a highly evolutionary conserved tyrosine with histidine. This change may affect the secretion or function of ADAMTS10 in the extracellular matrix. The compromise in protease activity may thus cause the unique presentation of the developed membranes in the heart and their recurrence after surgery.

Retrograde Pulmonary Valve Perforation and Stenting through a Major Aorto-Pulmonary Collateral Artery.

Management of pulmonary atresia, ventricular septal defect with major aorto-pulmonary collateral arteries, and hypoplastic native pulmonary arteries focuses on growth of the native pulmonary arteries. One strategy to grow the native pulmonary arteries is through pulmonary valve perforation followed by right ventricular outflow tract stenting, if suitable. We present a unique case of retrograde pulmonary valve perforation and stenting of the right ventricular outflow tract through a major aorto-pulmonary collateral artery.

RVOT premature ventricular contractions induce significant anatomical displacement during 3D mapping: A cause of mid-term ablation failure?

BACKGROUND: Catheter ablation is a first-line treatment for symptomatic right ventricular outflow tract (RVOT) premature ventricular complexes (PVCs). There is evidence of displacement of the ablation target site during PVCs relative to the location in sinus rhythm (SR). AIM: To analyse the extent of displacement induced by RVOT PVCs and its effect on the ablation sites and the mid-term efficacy of ablation. METHODS: In this multicentre French study, we retrospectively included 18 consecutive adults referred for ablation of RVOT PVCs using a three-dimensional (3D) mapping system. PVC activation maps were performed conventionally (initial map), then each PVC activation point was manually reannotated considering the 3D location on a previous SR beat (corrected map). The ablation-site locations on the initial or the corrected area, including the 10 best activation points, were analysed. Mid-term efficacy was evaluated. RESULTS: The direction of map shift during PVCs relative to the map in SR occurred along a vertical axis in 16 of 18 patients. The mean activation-point displacement for each of the 18 mapped chambers was 5.6±2.2mm. Mid-term recurrence of RVOT PVCs occurred in 5 (28%) patients. In all patients with recurrences, no significant ablation lesion was located on the corrected (true) site of origin. CONCLUSIONS: RVOT PVCs induce a vertical anatomical shift that can mislead physicians about the true location of the arrhythmia's site of origin. Our study highlights the association between mid-term PVC recurrence and the absence of spatial overlap between ablation points and the corrected site of origin.

The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot.

OBJECTIVE: Despite decades of experience, aspects of the management of tetralogy of Fallot with pulmonary stenosis (TOF) remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision making. Therefore, the TOF Clinical Practice Standards Committee was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. In addition, the group was tasked with identifying pertinent research questions for future investigations. It is recognized that variability in institutional experience could influence the application of this framework to clinical practice. METHODS: The TOF Clinical Practice Standards Committee is a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF. With the assistance of a medical librarian, a citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to TOF and its management; the search was restricted to the English language and the year 2000 or later. Articles pertaining to pulmonary atresia, absent pulmonary valve, atrioventricular septal defects, and adult patients with TOF were excluded, as well as nonprimary sources such as review articles. This yielded nearly 20,000 results, of which 163 were included. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of the member votes with 75% agreement on each statement. RESULTS: In asymptomatic infants, complete surgical correction between age 3 and 6 months is reasonable to reduce the length of stay, rate of adverse events, and need for a transannular patch. In the majority of symptomatic neonates, both palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities such as intracranial hemorrhage, sepsis, or other end-organ compromise as high-risk patients. In these high-risk patients, palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical palliation. CONCLUSIONS: Ongoing research will provide further insight into the role of catheter-based interventions. For complete surgical correction, both transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be utilized. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention. Given our current knowledge and the gaps identified, we propose several key questions to be answered by future research and potentially by a TOF registry: When to palliate or proceed with complete surgical correction, as well as the ideal type of palliation; the optimal surgical approach for complete repair for the best long-term preservation of right ventricular function; and the utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques.

Fate of the Right Ventricular Outflow Tract Following Valve-Sparing Repair of Tetralogy of Fallot.

Valve-sparing repair (VSR) of tetralogy of Fallot (TOF) tends to result in higher residual right ventricular outflow tract (RVOT) gradients. We evaluated the progression and clinical implications of RVOT gradients following VSR of TOF. Demographic, clinical, and operative data were retrospectively collected from consecutive TOF patients who underwent VSR at our institution between 01/2010 and 06/2021. RVOT gradient, pulmonary valve annulus (PVA) diameter and Boston Z-scores were recorded from serial echocardiograms. Data are presented as median and interquartile range or number and percentage. A total of 156 children (boys 92, 59%) underwent VSR at 6.5 (4.9-8.4) months of age and 6.6 kg (5.6- 7.7) weight. There was 1 (0.6%) operative mortality. The remaining 155 patients were followed for 69.4 months (4-106.2). RVOT gradient was 2.4m/s (1.7-2.9) at discharge. It transiently increased, then declined and stabilized during follow-up. PVA Z-score was -1.7 (-3.1 to 0.5) at discharge and 'grew' to -0.8 (-1.7 to 0.4) at last follow-up. Freedom from RVOT re-intervention was 97%, 94% and 91% at 1, 5 and 10-year follow-up. Among 67 (43%) patients with PVA Z-score < -2, a similar RVOT gradient pattern was observed and freedom from RVOT re-intervention was 97%, 95% and 95% at 1, 5 and 8-year follow-up. Following VSR of TOF, RVOT gradients transiently increase and then fall as PVA growth catches up, resulting in durable intermediate outcomes. Patients with PVA Z-score < -2 demonstrated a similar pattern of hemodynamics in the RVOT and excellent freedom from reintervention.