Search

Background: Nikaidoh translocation operation is increasingly being used for patients with TGA, VSD and LVOTO. The early results of this operation are excellent and possibly better than the Rastelli repair. However, the long-term results remain inadequately defined. Methods and results: Detailed follow up data of a patient, 12 years after Nikaidoh operation, using computerized image analysis are reported. The patient complained of chest pain on exertion. Imaging showed severe dilatation of the non-coronary sinus of the aortic root, compressing the RVOT and pulmonary artery branches resulting in RV dilatation. The dilated aortic sinus showed marked pulsatility with expansion during systole and diastole. As the child was symptomatic, with a risk of aneurysm rupture, excision was performed. This complication is thought to be due to interruption of the innervation of the root as well as cutting vasa vasorum. Conclusion: Nikaidoh operation is a viable solution for the repair of TGA, VSD, PS. Dilatation of the neo-aortic root should be carefully followed and causes of the dilatation investigated.

Background/purpose: Prognostic factors remain unclear in patients undergoing transcatheter implantation of Venus P-valve for their severe pulmonary regurgitation associated with native right ventricular (RV) outflow tract. Method: Between January 2017 and October 2018, we prospectively collected data of patient characteristics, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) before and 6–12 months after valve graft implantation. Results: Fifteen patients (male: 8, median age: 24.8 years) were enrolled. The procedure success rate was 100%. The median follow-up was 16.3 months without any dysfunction of the valve graft. The cohort demonstrated a significant improvement in cardiac index (from 3.3 to 3.9 L/min/m2) and increase of percentage of New York Heart Association functional class I (P

Aim: Exercise capacity is altered in congenital heart diseases (CHD) with potentially impaired pulmonary perfusion adaptation during exercise, such as in single ventricle or in significant pulmonary regurgitation. This study aimed to evaluate the value of double gas transfer factor analysis, at rest in conjunction with postural manoeuvres, to explore the various components of pulmonary gas transfer and its association with exercise capacity. Methods: A total of 40 subjects (24 CHD, 16 controls) underwent a combined measurement of lung diffusing capacity for carbon monoxide and nitric oxide (DLCO-DLNO) to determine pulmonary membrane diffusion (Dm) and Vcap, in sitting then supine position. CHD patients performed a maximal cardiopulmonary exercise test. Results: Compared to normal controls, the CHD group's DLNO, DLCO, Vcap, and alveolar volume (AV) at rest, in the sitting position were depressed, whereas the DLCO/AV and DLNO/AV were similar. The magnitude of Dm and Vcap adaptation induced by postural change was similar in both groups, indicating a preserved pulmonary capillary recruitment capacity in CHD. In the CHD group, at rest, for each ml of postural-induced increase in Vcap we observed during exercise a VE/VCO2 slope decrease of 0.46 (95% CI[0.83; 0.098]), indicating a better ventilatory response to exercise. Conclusion: CHD patients with impaired pulmonary circulation have a reduced Dm and Vcap mainly due to decreased pulmonary volume but maintain a normal capacity to adapt these parameters through a simple recruitment manoeuver. Vcap adaptation evaluated at rest predicts the level of ventilatory efficiency during exercise, which represents a main limiting factor in these CHD patients.

Background: Patients undergoing complete repair of tetralogy of Fallot generally have an excellent prognosis. Unfortunately, pulmonary valve reconstruction with a transannular patch is generally required. Resulting pulmonary regurgitation is associated with late right ventricular dysfunction and morbidity. Early pulmonary regurgitation may also be poorly tolerated. This study compares immediate outcomes of non-valved transannular patch repair (NV-TAP) to a valve-sparing (V-Sp) approach. Methods: 67 patients, with tetralogy of Fallot and severe pulmonary annular hypoplasia underwent complete repair between 2010 and 2018.23 patients had a NV-TAP repair while a V-Sp technique was used in 44 patients. Mortality, length of stay in the intensive care unit and hospital, use of inotropes, duration of mechanical ventilation, use and duration of dialysis, chest drainage duration, reinterventions, and postoperative complications were compared. Immediate outcomes were assessed by biological data and postoperative echocardiography. Results: ICU stay was shorter in the V-Sp group (5 days vs 8 days, p = 0.009). At postoperative day 1, Vasoactive Inotropic Score and mean dosage of adrenaline were significantly lower in the V-Sp group. Duration of inotrope use was significantly shorter. Hospital stay, mechanical ventilation, and chest drainage duration, the incidence and duration of dialysis all showed a trend towards being lower in the V-Sp group. Surgical complications were similar despite longer cardiopulmonary bypass and aortic cross-clamp durations. Conclusion: Repair of the native pulmonary valve by a V-Sp technique reduces length of stay in the ICU and the use of inotropic agents in the immediate post-operative period.

Competing Interests: Disclosures Dr O’Leary previously received research grant support from Abbott Cardiovascular. Dr Maschietto is a consultant and proctor for Medtronic Inc and Edwards Lifesciences.

Background: A dilated native right ventricular outflow tract (RVOT) presents unique challenges for transcatheter management using balloon-expandable valves. The Alterra Adaptive Prestent was designed to expand transcatheter therapy to treat patients with dilated RVOTs., Objectives: The aim of this study was to report 2-year outcomes of the main cohort of the ALTERRA (Multicenter Study of Congenital Pulmonic Valve Dysfunction Studying the SAPIEN 3 THV With the Alterra Adaptive Prestent) pivotal trial using the prestent with transcatheter pulmonary valve replacement., Methods: The prestent device used with the 29 mm SAPIEN 3 transcatheter heart valve (THV) was evaluated for the management of patients with moderate or greater pulmonary valve regurgitation (PR). The primary endpoint was THV dysfunction at 6 months, defined as a nonhierarchical composite of RVOT/pulmonary valve reintervention, moderate or greater total PR on transthoracic echocardiography, and mean RVOT/pulmonary valve gradient 35 mm Hg or greater on transthoracic echocardiography. The primary endpoint and outcomes through 2 years are presented in this analysis., Results: Of 97 patients screened, 60 underwent prestent and THV implantation. There was 1 staged procedure. No patients had THV dysfunction at 6 months. At 2 years, the majority of patients (92.5%) had mild or less PR, with no reports of coronary compression, stent fractures warranting reintervention, or endocarditis. Of the 21 patients (34.4%) who experienced early (days 0-1) arrhythmias, 12 had episodes of nonsustained ventricular tachycardia that resolved with medication. One patient underwent reintervention secondary to an iatrogenic RVOT obstruction; there were no deaths or explantations through 2 years., Conclusions: The Alterra prestent in combination with the SAPIEN 3 THV has excellent outcomes at 2 years, with no significant valve dysfunction in the main pivotal cohort., Competing Interests: Funding Support and Author Disclosures Edwards Lifesciences sponsored this study. Dr Dimas is a proctor and consultant for Edwards Lifesciences, Medtronic, and B. Braun; and is a consultant for Abbott. Dr Babaliaros is a consultant for Edwards Lifesciences and Abbott Vascular; and holds stock options in TransMural Systems. Dr Kim is a consultant for Edwards Lifesciences. Dr Lim has received research grants to his institution on his behalf from Abbott, atHeart, Boston Scientific, Corvia, Edwards Lifesciences, Medtronic, V-Wave, and W.L. Gore; and has received personal consulting fees from LagunaTech, Philips, Valgen, and Venus. Dr Morgan is a consultant and proctor for Edwards Lifesciences. Dr Jones is a consultant for Edwards Lifesciences and atHeart Medical; and is a consultant and proctor for Medtronic, Abbott, and W.L. Gore. Dr Armstrong is a consultant for Abbott, Medtronic, Edwards Lifesciences, and Starlight Cardiovascular. Dr Berman is a consultant and proctor for Abbott, B. Braun, Edwards Lifesciences, and Medtronic. Dr Aboulhosn is a consultant and proctor for Edwards Lifesciences and Medtronic. Dr Mahadevan is principal investigator for clinical trials sponsored by Abbott, and RECOR Medical; and is a proctor for Edwards Lifesciences. Dr Gillespie is a consultant and proctor for Abbott, Medtronic, and W.L. Gore. Dr Balzer is a consultant and proctor for Abbott, Edwards Lifesciences, and Medtronic. Dr Yu is an employee of Edwards Lifesciences. Dr Shirali has received institutional grant funding from Edwards Lifesciences. Dr Parthiban has received grant funding to the institution from Edwards Lifesciences. Dr Leipsic has institutional computed tomography core laboratory contracts with Edwards Lifesciences, Medtronic, Abbott, and Boston Scientific. Dr Blanke is a consultant for Edwards Lifesciences and LARALAB. Dr Zahn is a consultant for Abbott, Edwards Lifesciences, and Medtronic. Dr Shahanavaz is a consultant for Edwards Lifesciences and Medtronic. Dr Zellers has reported that he has no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Cardiopulmonary exercise testing (CPET) provides a comprehensive objective assessment in patients with repaired Tetralogy of Fallot (rTOF). However, the evidence underpinning this practice is scanty as are the mechanisms which drive exercise ability. Objectives: To describe the current evidence linking CPET data and prognosis in addition to review the most important determinants of exercise. Methods: The preferred reporting items (PRISMA) guidelines were followed. A systematic search of CPET studies, with/without echocardiography was carried out on PubMed MEDLINE, EBM review-Cochrane Database, Wiley Online library and EBM reviews. Results: Of 400 studies identified, 21 met the inclusion criteria. 17 articles (81%) were reported in young adults, and 4 articles (19%) in younger group. The sample size ranged from 15 to 875, and the publication year ranged from 2002 to 2019. Mean age was 25 ​± ​7 years. Overall mean predicted VO2 peak was 68 ​± ​2.8% (95% CI. 62.3–74%). There was no difference in mean predicted VO2 between older and younger groups (68 ​± ​2.7 vs 69 ​± ​2.9,%, p ​> ​0.05). Peak predicted VO2 was found to be higher in contemporary studies than historic investigations (76 ​± ​6 vs 59 ​± ​13,%, p.001). Submaximal measures were rarely reported. Determinants of exercise capacity were reported in 9 studies (43%). Prognostic findings qualitatively suggested that mild exercise intolerance with preserved ventilatory-equivalent for carbon dioxide is associated with better outcomes and lower mortality rate. Conclusions: The literature showed a high degree of heterogeneity which limited comparability. Marked reduction in functional capacity in patients with repaired TOF seems to be more dependent on surgical selection and developing technique than advancing age.

Background: Transcatheter pulmonary valve replacement (TPVR) has expanded and evolved since its initial commercial approval in the United States in 2010., Objectives: This study sought to characterize real-world practice, including patient selection, procedural outcomes, complications, and off-label usage., Methods: Characteristics and outcomes for patients undergoing balloon-expandable TPVR were collected from the American College of Cardiology National Cardiovascular Data Registry IMPACT (Improving Pediatric and Adult Congenital Treatment) Registry., Results: Between April 2016 and March 2021, 4,513 TPVR procedures were performed in patients with a median age of 19 years, 57% with a Melody (Medtronic Inc) and 43% with a SAPIEN (Edwards Lifesciences) valve. Most implanting centers performed <10 cases annually. One-third of transcatheter pulmonary valve implants were into homograft conduits, one-third were into bioprosthetic valves (BPVs), 25% were in native or patched right ventricular outflow tracts (RVOTs), and 6% were into Contegra (Medtronic Inc) conduits. Over the course of the study period, SAPIEN valve use grew from ∼25% to 60%, in large part because of implants in patients with a native/patched RVOT. Acute success was achieved in 95% of patients (95.7% in homografts, 96.2% in BPVs, 94.2% in native RVOTs, and 95.4% in Contegra conduits). Major adverse events occurred in 2.4% of procedures, more commonly in patients with a homograft (2.9%) or native RVOT (3.4%) than a prior BPV (1.4%; P = 0.004)., Conclusions: This study describes novel population data on the use and procedural outcomes of TPVR with balloon-expandable valves. Over time, there has been increasing use of TPVR to treat regurgitant native RVOT anatomy, with the SAPIEN valve more commonly used for this application., Competing Interests: Funding Support and Author Disclosures A portion of this work was supported by the National Institutes of Health (T32HL007604) to Dr Stefanescu Schmidt. The manuscript contents are solely the responsibility of the authors and do not necessarily represent the official views of the National Institutes of Health. Dr Stefanescu Schmidt is an investigator for Occlutech. Dr Armstrong is a consultant and proctor for Medtronic and Edwards Lifesciences and an investigator for Edwards Lifescience. Dr Aboulhosn is a consultant and proctor for Medtronic and Edwards Lifesciences. Dr Jones is an investigator, consultant, proctor, and Clinical Event Committee member for Medtronic; and is an investigator and consultant for Edwards Lifesciences. Dr Levi is a consultant for Medtronic and Edwards Lifesciences. Dr McElhinney is a consultant and proctor for Medtronic. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

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., Competing Interests: Funding Support and Author Disclosures This study was supported by Medtronic. Dr Gillespie serves as a consultant for Medtronic; as an advisory board member and a consultant for Abbott; and as a consultant and principal investigator for W. L. Gore & Associates. Dr McElhinney serves as a proctor and consultant for Medtronic. Dr Jones is an investigator, a consultant, and a proctor for Medtronic; and is an investigator and a consultant for Edwards Lifesciences. Dr Levi serves as a consultant for Edwards Lifesciences and Medtronic. Dr Asnes is a consultant for Medtronic. Dr Gray receives research support as an investigator for Medtronic and Edwards Lifesciences. Dr Cabalka is an investigator and a consultant for Medtronic and Edwards Lifesciences. Dr Qureshi serves as a consultant for Medtronic and W.L. Gore & Associates. Dr Justino serves as a consultant for Abbott, Abiomed, Baylis Medtech, Chiesi USA, Edwards Lifesciences, Janssen Research and Development, Medtronic, and Pediastent; and is a cofounder of PolyVascular. Dr Benson serves as consultant for Medtronic. Mr Haugan is a full-time employee and shareholder of Medtronic. Dr Cheatham serves as a consultant, a principal investigator, a proctor, and an advisory board member for Medtronic. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease. After surgical repair, the excellent survival rate has led to long-term complications dominated by pulmonary regurgitation (PR). Our study aimed to identify echocardiographic criteria for assessment of right ventricular (RV) function and quantification of PR correlated with cardiac MRI indices., Methods: We conducted a descriptive study between June 2021 and March 2022 including patients followed for repaired ToF since 2016 or earlier. All patients were seen for clinical, electrocardiographic, and ultrasound evaluation. Cardiac MRI was performed with a maximum delay of 1 month from Doppler echocardiography., Results: 38 patients were included, with a mean age at imaging of 18 ± 9 years. Our study showed that the echocardiographic parameters of PR quantification correlated with a pulmonary regurgitation fraction on MRI greater than 40% were: the PHT value (p = 0.005) and the ratio of PR jet width to pulmonary valve annulus diameter (p = 0.032). The only ultrasound parameter estimating RV systolic function correlated with RVEF on cardiac MRI was FAC, with p = 0.007., Conclusion: Quantitative assessment of RV parameters in patients followed for repaired ToF, as well as the assessment of the severity of PR, is feasible with a standardized ultrasound examination, with good correlation with cardiac MRI., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Background: Many patients with repaired tetralogy of Fallot require reoperation in the medium to long-term for residual pulmonary valve regurgitation. Best timing for pulmonary valve replacement remains controversial. A balance needs to be found between protecting the patients from permanent right ventricular damage and insertion of a prosthetic valve with its inherent issues. In the current study we sought to investigate the right and left ventricular functional recovery following valve replacement in our tertiary care institution., Results: In a retrospective cross-sectional study patients with history of total correction of tetralogy of Fallot who had undergone pulmonary valve replacement due to severe pulmonary regurgitation between 2003-2018 were evaluated for post intervention right and left ventricular functional recovery. Clinical and full echocardiographic data before and after the surgery were recorded and compared. There was statistically significant improvement in RV size and function post pulmonary valve replacement. There was no statistically significant improvement in left ventricular systolic function. Twenty percent of patient had persisting severe right ventricular enlargement at least twelve months post-surgery. No patient had fully normalized right ventricular size and function in follow-up., Conclusions: Pulmonary valve replacement leads to improvement in right ventricular size and function in patients with repaired tetralogy of Fallot. However normalization of functional parameters did not occur and the majority of the patients have residual right and left ventricular dysfunction following redo valve replacement surgery., Competing Interests: Declaration •Ethics approval and consent to participate : Written Patient consent was obtained, Ethics committee of Rajaei Cardiovascular Medical Research Center, Tehran, Iran approved of the study protocol (committee's reference number : 99053) • Consent for publication : Not applicable • Availability of data and material : presented in the main paper • Competing interests : None • Funding : None • Authors' contributions Case concept and design : SS,HM Acquisition of data : HM,SR Drafting of manuscript : S S,SR Revision : SS,HM,SR • All authors have read and approved the manuscript • Acknowledgements : None, (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Carcinoid disease is caused by neuroendocrine tumors, most often located in the gut, and leads in approximately 20% of cases to specific, severe heart disease, most prominently affecting right-sided valves. If cardiac disease occurs, it determines the patient's prognosis more than local growth of the tumor. Surgical treatment of carcinoid-induced valve disease has been found to improve survival in observational studies. Cardiac imaging is crucial for both diagnosis and management of carcinoid heart disease; in the past, imaging was accomplished largely by echocardiography, but more recently, imaging for carcinoid heart disease has increasingly become multimodal and warrants awareness of the particular diagnostic challenges of this disease. This paper reviews the pathophysiology and manifestations of carcinoid heart disease in light of the different imaging modalities., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Pulmonary regurgitation (PR) and right ventricular (RV) dilatation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF)., Aims: To compare Echo data with the gold standard CMR in a paediatric population of r-TOF with significant PR, to assess the reliability of standard and advanced echo parameters. In addition, to evaluate their correlation with peak oxygen consumption (VO 2 )., Methods and Results: All patients underwent standard echo-Doppler study, speckle tracking analysis, and CMR to assess PR and RV size and function. Thirty-six patients underwent also cardiopulmonary exercise test. Fourty-six patients (aged 13.7 ± 3.0) were included. Echo derived RV areas correlated with CMR RV volumes (p < .0001, r = 0.72). RV end-diastolic area > 21.9 cm 2 /m 2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume ≥ 150 ml/m 2 . RVEF was preserved in all patients, while TAPSE was reduced in 78.2% and RVGLS in 60.8%. Flow-reversal in pulmonary branches showed a sensitivity of 95.8% and a specificity of 59.1% to identify CMR pulmonary regurgitant fraction (RF) ≥ 35%. None of the CMR parameters correlated with peak VO 2 . Among the Echo data only right atrial strain (RAS) correlated with peak VO 2. CONCLUSION: In children, flow-reversal in pulmonary branches identifies hemodynamically significant RF with a good sensitivity but poor specificity. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. RV longitudinal systolic dysfunction coexists with a still preserved EF. RAS correlates strongly with peak VO 2 and should be added in their follow up., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objectives: The aim of this study was to test the hypothesis that narrowing the landing zone using commercially available endografts would enable transcatheter pulmonary valve replacement (TPVR) using commercially available transcatheter heart valves., Background: TPVR is challenging in an outsized native or patch-repaired right ventricular outflow tract (RVOT). Downsizing the RVOT for TPVR is currently possible only using investigational devices. In patients ineligible because of excessive RVOT size, TPVR landing zones were created using commercially available endografts., Methods: Consecutive patients with native or patch-repaired RVOTs and high or prohibitive surgical risk were reviewed, and this report describes the authors' experience with endograft-facilitated TPVR (EF-TPVR) offered to patients ineligible for investigational or commercial devices. All EF-TPVR patients were surgery ineligible, with symptomatic, severe pulmonary insufficiency, enlarged RVOTs, and severe right ventricular (RV) enlargement (>150 ml/m 2 ). TPVR and surgical pulmonary valve replacement (SPVR) were compared in patients with less severe RV enlargement., Results: Fourteen patients had large RVOTs unsuitable for conventional TPVR; 6 patients (1 surgery ineligible) received investigational devices, and 8 otherwise ineligible patients underwent compassionate EF-TPVR (n = 5 with tetralogy of Fallot). Three strategies were applied on the basis of progressively larger RVOT size: single-barrel, in situ fenestrated, and double-barrel endografts as required to anchor 1 (single-barrel and fenestrated) or 2 (double-barrel) transcatheter heart valves. All were technically successful, without procedure-related, 30-day, or in-hospital deaths. Two late complications (stent obstruction and embolization) were treated percutaneously. One patient died of ventricular tachycardia 36 days after EF-TPVR. Compared with 48 SPVRs, RV enlargement was greater, but 30-day and 1-year mortality and readmission were no different. The mean transvalvular pressure gradient was lower after EF-TPVR (3.8 ± 0.8 mm Hg vs. 10.7 ± 4.1 mm Hg; p < 0.001; 30 days). More than mild pulmonary insufficiency was equivalent in both (EF-TPVR 0.0% [n = 0 of 8] vs. SPVR 4.3% [n = 1 of 43]; p = 1.00; 30 days)., Conclusions: EF-TPVR may be an alternative for patients with pulmonic insufficiency and enlarged RVOTs ineligible for other therapies., Competing Interests: Author Disclosures Dr. Babaliaros is a consultant for and has received research grant support from Abbott Vascular and Edwards Lifesciences; and has equity in Transmural Systems. Dr. Greenbaum is a proctor for Edwards Lifesciences, Medtronic, and St. Jude Medical; and has equity in Transmural Systems. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2020 American College of Cardiology Foundation. All rights reserved.)

Objectives: The aim of this study was to demonstrate the safety and functionality of the Alterra Adaptive Prestent and SAPIEN 3 transcatheter heart valve (THV) in patients with dysfunctional, dilated right ventricular outflow tract (RVOT) greater or equal to moderate pulmonary regurgitation (PR)., Background: Significant variations in the size and morphology of the RVOT affect the placement of transcatheter pulmonary valves. The Alterra Prestent internally reduces and reconfigures the RVOT, providing a stable landing zone for the 29-mm SAPIEN 3 THV., Methods: Eligible patients had moderate or greater PR, weighed >20 kg, and had RVOT diameter 27 to 38 mm and length >35 mm. The primary endpoint was device success, a 5-item composite: 1 Alterra Prestent deployed in the desired location, 1 SAPIEN 3 THV implanted in the desired location within the Prestent, right ventricular-to-pulmonary artery peak-to-peak gradient <35 mm Hg after THV implantation, less than moderate PR at discharge, and no explantation 24 h post-implantation. The secondary composite endpoint was freedom from THV dysfunction (RVOT/pulmonary valve (PV) reintervention, greater or equal to moderate total PR, mean RVOT/PV gradient ≥ 35 mm Hg at 30 days and 6 months. Descriptive statistics are reported., Results: Enrolled patients (N = 15) had a median age and weight of 20 years and 61.7 kg, respectively; 93.3% were in New York Heart Association functional class I or II. Device success was 100%. No staged procedures were necessary. No THV dysfunction was reported to 6 months. No serious safety signals were reported., Conclusions: This early feasibility study demonstrated the safety and functionality of the Alterra Adaptive Prestent in patients with congenital RVOT dysfunction and moderate or greater PR. Durability and long-term outcome data are needed., Competing Interests: Author Relationship With Industry Edwards Lifesciences sponsored this study. Dr. Shahanavaz is a consultant for Medtronic; and is a proctor for Edwards Lifesciences. Dr. Balzer is a consultant and proctor for Medtronic and Edwards Lifesciences. Dr. Babaliaros is a consultant for Edwards Lifesciences; and has equity in TransMural Systems. Dr. Dimas is a consultant for Medtronic and Abbott Laboratories/St. Jude Medical. Dr. Reddy is a consultant for B. Braun Interventional Systems. Dr. Leipsic is a core laboratory consultant for Edwards Lifesciences, Medtronic, and Abbott Laboratories; and is a consultant for Circle CVI. Dr. Blanke is a consultant for Edwards Lifesciences. Dr. Gorelick is an employee of Edwards Lifesciences. Dr. Zahn is a proctor and consultant for Medtronic and Edwards Lifesciences. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Right ventricular outflow tract diseases are historically outcomes of surgical reconstruction for heart defects in neonates or children (Tetralogy of Fallot, Ross surgery). This kind of surgery performed during childhood lead often to dysfunctional right ventricular outflow tract (stenosis, leak) in older infants or young adults. In this case, reintervention on the right ventricular outflow tract would be associated with a high surgical risk. Development of the first percutaneous valves in the year 2000 paved the way for the pulmonary revalvulation. This percutaneous procedure has emerged as a credible alternative to the surgery for multioperated high risk patients with congenital cardiopathies. Two valves are currently available on the French market (Melody®, Sapien®); they cover all therapy indications, except the example of very wide outflow tracts which remain a surgical issue. Medical teams in charge of these young patients have to be enough trained in order to limit risks during the procedure. To this end, several teams promote the percutaneous pulmonary revalvulation over surgical revalvulation, this latter becoming limited only to cases in which percutaneous treatment is not feasible., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Congenital heart defects that involve obstruction to the right ventricular outflow tract are common. Surgical repair involves early relief of right ventricular outflow tract obstruction, which typically results in pulmonary regurgitation and large irregularly shaped "native" right ventricular outflow tract. This type of anatomy represents the majority of patients who could potentially benefit from transcatheter pulmonary valve therapy. Currently approved balloon-expandable devices were not designed for this application and the unique anatomy of these patients presents tremendous challenges for designing a valve that is. This article explores those challenges and the newest self-expanding devices designed to treat this challenging population., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy., Methods and Results: A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs -0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S' lateral [1.0 (2.0) cm/s vs -0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E' lateral [0.57 (2.4) cm/s vs -3.3 (3.9) cm/s; p < 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E' lateral [-0.7 (1.9) vs 1.5 (1.9); p < 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs -5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [-0.18 (0.42) vs 0.23 (0.48); p = 0.037]., Conclusion: Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objectives: The aim of this study was to describe and analyze data from patients treated in France with the Edwards SAPIEN transcatheter heart valve (Edwards Lifesciences LLC, Irvine, California) in the pulmonary position., Background: The Edwards SAPIEN valve has recently been introduced for percutaneous pulmonary valve implantation (PPVI)., Methods: From April 2011 to May 2017, 71 patients undergoing PPVI were consecutively included., Results: The median age at PPVI was 26.8 years (range 12.8 to 70.1 years). Primary underlying diagnoses were conotruncal malformations (common arterial trunk, tetralogy of Fallot and variants; n = 45), Ross procedure (n = 18), and other diagnoses (n = 8). PPVI indication was pure stenosis in 33.8% of patients, pure regurgitation in 28.1%, and mixed lesions in 38.1%. PPVI was successfully implemented in 68 patients (95.8%). Pre-stenting of the right ventricular outflow tract was performed in 70 patients (98.6%). Early major complications occurred in 4 subjects (5.6%), including 1 death, 1 coronary compression, and 2 pulmonary valve embolizations. Three of the 4 major complications occurred in the first 15 operated patients. No significant regurgitation was recorded after the procedure. Transpulmonary gradient was significantly reduced from 34.5 to 10.5 mm Hg (p < 0.0001). No patient died during a 1-month follow-up period. At 1-year follow-up, the death rate was 2.9%, and 3 patients had undergone surgical reintervention (44%)., Conclusions: Early results with the Edwards SAPIEN valve in the pulmonary position demonstrate an ongoing high rate of procedural success., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: To investigate the feasibility and mid-term results of percutaneous pulmonary valve implantation (PPVI) in patients with conduit free or "native" right ventricular outflow tracts (RVOT)., Methods and Results: We identified all 18 patients with conduit free or "native" right ventricular outflow tract, who were treated with percutaneous pulmonary valve implantation (PPVI) in our institution. They were divided into two groups - these in whom the central pulmonary artery was used as an anchoring point for the preparation of the landing zone (n=10) for PPVI and these, in whom a pulmonary artery branch was used for this purpose (n=8). PPVI was performed successfully in all patients with significant immediate RVOT gradient and pulmonary regurgitation grade reduction. Four patients had insignificant paravalvular regurgitation. In one patient the valve was explanted after 4months because of bacterial endocarditis. A follow-up of 19 (4-60) months showed sustained good function of the other implanted valves. The MRI indexed right ventricular end diastolic volume significantly decreased from 108(54-174) ml/m 2 before the procedure to 76(60-126) ml/m 2 six months after PPVI, p=0.01., Conclusions: PPVI is feasible with good mid-term results in selected patients with a "native" RVOT without a previously implanted conduit. Creating a stable landing zone with a diameter less than the largest available valve (currently 29mm) is crucial for the technical success of the procedure. Further studies and the development of new devices could widen the indications for this novel treatment., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Transcatheter pulmonary valve replacement (TPVR) is an important treatment option in repaired tetralogy of Fallot (TOF) and right ventricular outflow tract (RVOT) dysfunction. Indications for timing of TPVR are extrapolated from surgical pulmonary valve replacement guidelines, which are themselves controversial as published evidence is scarce and expert opinion therefore prevails. We review current indications for PVR following TOF repair, focusing on those for TPVR specifically, and discuss anatomical and functional considerations as these pertain to determination of candidacy for TPVR. Hemodynamic assessment surrounding PVR has focused on assessment of the right ventricle (RV) size and systolic function, with the goal of intervening in the asymptomatic patient prior to the development of irreversible RV deterioration and right heart failure. The impact of abnormal RV mechanics on the LV has been appreciated, with the assessment of LV function assuming higher priority in decision-making regarding possible PVR. In addition to the standard volumetric assessment, evaluation with indices of myocardial wall strain, tissue velocities, diastology, and ventricular response to exercise is emerging as tools with potential to further refine timing of PVR. We conclude that, at present, current evidence, although limited, supports a more aggressive approach in those who meet inclusion for TPVR in patients with repaired TOF and RVOT dysfunction guided by the discussed hemodynamic assessment, however, more importantly this review should lay the framework for future investigations regarding hemodynamic assessment of this population., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well understood. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR., Methods: Forty-eight Cardiovascular Magnetic Resonance (CMR) studies in patients after TOF repair were analyzed. In addition to the routine blood flow and ventricular volume quantification cardiac angle between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured on an axial image. Statistical analysis was performed to compare flow parameters between RPA and LPA and to assess correlation among right ventricular volume, pulmonary blood flow parameters and lung area., Results: There was no difference between LPA and RPA diameters. The LPA showed significantly less total forward flow (2.49 ± 0.87 L/min/m(2) vs 2.86 ± 0.89 L/min/m(2); p = 0.02), smaller net forward flow (1.40 ± 0.51 vs 1.89 ± 0.60 mL/min/m(2); p = <0.001), and greater regurgitant fraction (RF) (34 ± 10 % vs 43 ± 12 %; p = 0.001) than the RPA. There was no difference in regurgitant flow volume between RPA and LPA (p = 0.29). Indexed right ventricular end-diastolic volume (RVEDVi) correlated with LPA RF (R = 0.48, p < 0.001), but not with RPA RF (p = 0.09). Larger RVEDVi correlated with a more leftward cardiac axis (R = 0.46, p < 0.001) and with smaller left lung area (R = -0.58, p < 0.001). LPA RF, but not RPA RF, correlated inversely with left lung area (R = -0.34, p = 0.02). The follow-up CMRs in 20 patients showed a correlation of the rate of RV enlargement with the rates of LPA RF worsening (R = 0.50, p = 0.03), and of increasing left lung compression (R = -0.55, p = 0.012)., Conclusion: An enlarged and levorotated heart is associated with left lung compression and impaired flow into the left lung.

Repaired tetralogy of Fallot (TOF) is one of the most common diagnoses encountered when caring for adults with congenital heart disease. Although long-term survival after childhood TOF repair is excellent, morbidity is common and most patients require reintervention in adulthood. This review provides an overview of key surveillance and management issues for adults with TOF, including residual right ventricular outflow tract disease and timing of pulmonary valve replacement, arrhythmias and risk stratification, left-sided heart disease and heart failure, and pregnancy management., (Copyright © 2015 Elsevier Inc. All rights reserved.)

The recovery of cardiopulmonary variables from peak exercise in patients with pulmonary stenosis (PS) or regurgitation (PR) is delayed, but the impact of treating PS or PR on exercise recovery kinetics is unknown. 43 patients (median age 14 years) with PS (n = 23) or PR (n = 20) after repair of congenital heart disease underwent successful percutaneous pulmonary valve implantation (PPVI). Cardiopulmonary exercise tests (CPET) were performed both before and within 1 month after PPVI. Apart from peak oxygen uptake (VO2), the constant decay of VO2, CO2 output (VCO2), minute ventilation (VE), and heart rate (HR) and oxygen pulse were calculated for the first minute of recovery as the first-degree slope of a single linear relation. PPVI led to a significant improvement in NYHA functional class in the PS and PR groups (p<0.001 and p=0.0015, respectively). On CPET, peak VO2 improved post-PPVI only in the PS (25.6 ± 6.2 vs. 27.8 ± 7.9 ml/kg/min; p = 0.01) but not PR group (29.0 ± 9.8 vs. 28.6 ± 8.9 ml/kg/min; p = 0.6). However, VO2 slope improved in the PS (0.40 ± 0.23 vs. 0.65 ± 0.27, p < 0.001) as well as in the PR group (0.56 ± 0.37 vs. 0.67 ± 0.37, p = 0.003) as did VCO2 slope (0.39 ± 0.2 vs. 0.55 ± 0.24, p = 0.002 and 0.42 ± 0.33 vs. 0.53 ± 0.35, p = 0.02: for the PS and PR groups, respectively). The VE and HR slopes did not change after PPVI. Despite the lack of improvement in exercise capacity in the PR group, treatment of PS and PR by PPVI induces significant and similar improvements in the ability of recovering from maximal exercise in the 2 groups., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Objectives: This study sought to confirm that the short-term hemodynamic effectiveness of the Melody transcatheter pulmonary valve (TPV) (Medtronic, Inc., Minneapolis, Minnesota) achieved by real-world providers is equivalent to the historical results established in the initial 5-center Investigational Device Exemption trial., Background: TPV replacement has been used to treat right ventricular outflow tract (RVOT) conduit dysfunction for >10 years. The Melody TPV received U.S. Food and Drug Administration approval in 2010 as a Humanitarian Use Device., Methods: Patients with dysfunctional RVOT conduits were entered in this prospective, nonrandomized study at 10 centers. The primary endpoint was acceptable hemodynamic function at 6 months post-implantation, defined as a composite of RVOT echocardiographic mean gradient ≤30 mm Hg, pulmonary regurgitation less than moderate as measured by echocardiography, and freedom from conduit reintervention and reoperation., Results: Cardiac catheterization was performed in 120 patients for potential implantation of the Melody TPV; of these, 100 patients were implanted, with a 98.0% procedural success rate. There were no procedure-related deaths. Acceptable hemodynamic function at 6 months was achieved in 96.7% of patients with evaluable data (87.9% of the entire implanted cohort), with results maintained through 1 year. No patient had moderate or severe pulmonary regurgitation after implantation. No patient required catheter reintervention in the first year after implantation, and 2 patients required reoperation for conduit replacement. The rate of freedom from TPV dysfunction was 96.9% at 1 year., Conclusions: This first prospective, real-world experience with the Melody TPV in the United States demonstrates continued high procedural success, excellent short-term TPV function, and low reintervention and reoperation rates at 1 year. (Melody Transcatheter Pulmonary Valve Post-Approval Study; NCT01186692)., (Copyright © 2014. Published by Elsevier Inc.)

Objectives: The aim of this study was to better understand the quantitative volumetric changes associated with pregnancy in women with repaired tetralogy of Fallot (TOF), utilizing sequential cardiovascular magnetic resonance (CMR) imaging., Background: An increasing number of women with repaired TOF are reaching childbearing age. Limited echocardiographic studies suggest accelerated remodeling of the right ventricle (RV) in women with repaired TOF after pregnancy., Methods: Sequential CMRs from a group of women with repaired TOF who completed pregnancy and from a matched comparison group of nulliparous women with repaired TOF were evaluated. The two groups were matched according to baseline QRS duration, RV end-diastolic volume (EDV), age at CMR and time between CMRs. Longitudinal change of CMR parameters was compared between the groups., Results: Thirteen women (mean age 26.6 ± 7.4 years) with repaired TOF who completed pregnancy and 26 nulliparous women with repaired TOF (mean age 22.6 ± 8.0 years) were included in this analysis. The rate of increase of RV EDV in the pregnancy group was higher than the comparison group (4.1 ± 1.1 ml/m(2)/year vs. 1.6 ± 0.6 ml/m(2)/year, p=0.07). RV EF did not change significantly in either group. No definitive interaction between degree of pulmonary regurgitation and increase of RV EDV was identified., Conclusions: Women with repaired TOF who have completed pregnancy appear to experience an accelerated rate of right ventricular remodeling, defined as an increase in end-diastolic volume; however RV systolic function does not deteriorate. Further investigations with a prospective study design, larger cohorts, and longer follow-up are needed to confirm these initial observations., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: Free pulmonary regurgitation (PR) after surgical correction of Tetralogy of Fallot (ToF) with transannular patching can lead to irreversible right ventricular (RV) failure. However, the optimal timing of valve replacement is still debated., Methods and Results: Thirty six pigs were included in the study. Twenty one pigs had a bare metal stent placed in the pulmonary annulus inducing free PR and 9 animals served as control. Six animals died prematurely due to procedural complications. The 21 animals were divided into 3 groups with differential duration of PR (1, 2, 3 months, respectively) after which PPVR was performed. After 1 month with competent valve the animals were euthanized. Cardiac magnetic resonance (CMR) and right heart catheterization were performed serially. Free PR led to severe dilation of the RV in all three groups compared to matched controls (p<0.001). Final RV volume after one month with competent pulmonary valve was modeled. Increase in RV volume from baseline to valve replacement (ΔRV) was the only predictor of RV recovery (p<0.001) and increases in ΔRV beyond 120 mL/m2 were predictive of very low probability of recovery. A total of 5 animals did not recover., Conclusions: Recovery of right ventricular function after free PR by treatment with PPVR was successful in the majority of animals. Increases in RV volume during PR were the only predictor of non-recovery after PPVR and duration of PR did not in itself predict treatment success., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: Fragmented QRS complexes (fQRS) correlate with myocardial scar, and may predict arrhythmias in patients with repaired tetralogy of Fallot (TOF). We investigated the relationship between fQRS in operated TOF patients with right ventricular (RV) dysfunction and RV outflow tract (RVOT) aneurysm., Methods: We studied 56 operated TOF patients with moderate/severe pulmonary regurgitation, referred for cardiac magnetic resonance imaging (MRI) over a 4.5 year period. The presence of fQRS (additional notches in the R/S wave in ≥ 2 contiguous leads on the ECG) was correlated with MRI findings., Results: fQRS was observed in 44 (78.6%) patients. Patients with fQRS had significantly larger RV end diastolic volume index (RVEDVi; 162 ml vs 141 ml, p=0.028) and RV end systolic volume index (RVESVi; 88 ml vs 70 ml, p=0.031). Increasing number of leads with fragmentation was independently associated with increasingly lower RV ejection fraction (adjusted co-efficient -0.97, 95%CI -1.83 to -0.12, p=0.026), greater pulmonary regurgitation fraction (1.65, 0.28 to 3.01, p=0.019), larger RVEDVi (6.78, 2.00 to 11.56, p=0.006) and RVESVi (5.41, 1.66 to 9.15, p=0.005). Anterior fragmentation correlated most significantly with RV dysfunction (p<0.05). fQRS had no significant association with LV dysfunction. Presence of any fQRS (OR 17.5, 95%CI 2.1-147.8, p=0.009) and inferior fQRS (OR 9.0, 95%CI 2.7-30.1, p<0.001) were found to be significant predictors for RVOT aneurysm., Conclusions: The presence of fQRS on the ECG is significantly associated with RV dysfunction and RVOT aneurysms in repaired TOF patients. Increasing burden of fragmentation, especially in the anterior leads, is associated with increasing RV dysfunction., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)