Transcatheter-Based Interventions for Tetralogy of Fallot Across All Age Groups.

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. Palliative procedures, either surgical or transcatheter, aim to improve oxygen saturation, affording definitive procedures at a later stage. Transcatheter interventions have been used before and after surgical palliative or definitive repair in children and adults. This review aims to provide an overview of the different catheter-based interventions for TOF across all age groups, with an emphasis on palliative interventions, such as patent arterial duct stenting, right ventricular outflow tract stenting, or balloon pulmonary valvuloplasty in infants and children and transcatheter pulmonary valve replacement in adults with repaired TOF, including the available options for a large, dilated native right ventricular outflow tract.

Letter to the Editor Ross procedure valve function, clinical outcomes and predictors after 25 years follow up.

The following letter presents an answer of a comment of our work titled "Ross procedure: valve function, clinical outcomes and predictors after 25 years' follow-up," recently published in your journal by Rangwala et al.1 As our colleagues point out, the Ross procedure has excellent survival rates but a significant risk of valve dysfunction and therefore reintervention at follow-up. Although the survival advantage with the Ross procedure appears to be consistent compared with mechanical valve substitutes, this benefit is not as clear compared with biological valve substitutes. However, biological valve substitutes also have significant reintervention rates during follow-up. The different surgical modifications of the Ross procedure have not clearly demonstrated better results in follow-up in terms of autograft reintervention. This procedure can be performed in a medium-volume center with good results as long as adequate patient selection and adequate surgical training are carried out.

Surgical Heritage: You Had to Be There, Ross: The Comeback Kid.

Half a century after the first pulmonary autograft operation (Ross operation), performed in 1967 by Donald Ross in central London, there is a very strong conviction that the Ross operation is the best available valve substitute today, not only for children, but also for younger and older adults. The Ross operation has stimulated a lot of science to do with tissue-engineering and biology of heart valves, which is a promising avenue for the future. For one of us (M.Y.), it has certainly been a privilege to be associated with the comeback of the Ross operation.

Late Pulmonary Autograft Dilation: Can We Make a Good Operation Great? The Tailored Approach.

While it is the main viable option in the growing child and young adult, the Ross procedure has expanded its applicability to older patients, for whom long-term results are equivalent, if not superior, to prosthetic aortic valve replacement. Strategies aiming at mitigating long-term autograft failure from root enlargement and valve regurgitation have led some to advocate for root reinforcement with prosthetic graft material. On the contrary, we will discuss herein the rationale for a tailored approach to the Ross procedure; this strategy is aimed at maintaining the natural physiology and interplay between the various autograft components. Several technical maneuvers, including careful matching of aortic and autograft annuli and sino-tubular junction as well as external support by autologous aortic tissue maintain these physiologic relationships and the viability of the autograft, and could translate in a lower need for late reintervention because of dilation and/or valve regurgitation.

Late Pulmonary Autograft Dilation: Can We Make a Good Operation Great? The Supported Ross.

The Ross procedure is an excellent option for aortic valve replacement resulting in outstanding hemodynamic performance and the ability to avoid systemic anticoagulation. The long-term durability of the autograft is generally good but concerns for later aortic root dilation with ensuing neoaortic insufficiency have prompted efforts to stabilize the autograft, root, sinuses and Sino-tubular junction in order to delay or entirely avoid late reinterventions on the neoaortic root. We have employed an inclusion technique, supporting the Auto-graft in a Terumo Gelweave™ Valsalva graft. We performed a retrospective study of all 129 patients undergoing the Ross procedure from 1992 to 2019 at Children's Wisconsin. Fifty-one underwent the supported Ross (SR) and 78 underwent unsupported Ross (UR). Structured clinical data was collected and echocardiograms were reviewed. Median follow-up was 4.9 years (up to 22.6 years) for UR patients and 3.6 years (up to 11.4 years) for SR patients. In order to provide a fair comparison, we sub -analyzed patients aged 10 to 18 years who underwent the Ross procedure, 16 who underwent the UR and 18 patients who underwent the SR. Change in aortic annulus diameter (P = 0.002), aortic sinus diameter (P = 0.001) change in left ventricular function (P = 0.039) and change in aortic insufficiency (P = 0.008) were all worse in UR. The SR is simple, reproducible, and predictable. It seems to prevent change in annulus diameter, sinus diameter and to reduce late neoaortic insufficiency. Longer follow-up with a larger group of patients is required to draw definitive conclusions.

Risk Factors for Coronary Artery Complications After Prosthetic Pulmonary Valve Implantation in Patients With Congenital Heart Disease.

BACKGROUND: Coronary artery complications (CACs) in patients who undergoing prosthetic pulmonary valve implantation for congenital heart disease can lead to fetal outcomes. However, the incidence of and risk factors for CACs in these patients remain unknown.Methods and Results: A retrospective cohort study was conducted on patients who underwent cardiac computed tomography or invasive coronary angiography after prosthetic pulmonary valve implantation at Seoul National University Hospital from June 1986 to May 2021. Among 341 patients, 25 (7.3%) were identified with CACs, and 2 of them died. Among the patients with CACs, congenital coronary anomalies and an interarterial course of the coronary artery were identified in 11 (44%) and 18 (72%) patients, respectively. Interarterial and intramural courses of the coronary artery were associated with a 4.4- and 10.6-fold increased risk of CACs, respectively. Among patients with tetralogy of Fallot and pulmonary atresia, the aortic root was rotated further clockwise in patients with coronary artery compression compared to those without it (mean [±SD] 128.0±19.9° vs. 113.5±23.7°; P=0.024). The cut-off rotation angle of the aorta for predicting the occurrence of coronary artery compression was 133°. CONCLUSIONS: Perioperative coronary artery evaluation and prevention of CACs are required in patients undergoing prosthetic pulmonary valve implantation, particularly in those with coronary artery anomalies or severe clockwise rotation of the aortic root.

A rare case of a neonate with fallot-type absent pulmonary valve and occlusion of the left main bronchus.

Fallot-type absent pulmonary valve is a rare and complex congenital heart disease. Repair surgery for this condition during the neonatal period has a mortality rate of over 50%. We reported a neonate with Fallot-type absent pulmonary valve and occlusion of the left main bronchus. The patient's pulmonary artery had unusual anatomy of a type that has not previously been reported. This case report outlines a successful treatment strategy for patients with complex congenital heart disease and airway occlusion during the neonatal period and the effect of these unusual anatomical conditions on postoperative outcomes.

Percutaneous pulmonary valve implantation in a patient with congenitally corrected transposition of the great arteries: a case report.

BACKGROUND: Percutaneous pulmonary valve implantation has become an attractive method of dysfunctional right ventricle outflow tract treatment. CASE PRESENTATION: We describe a unique case of a 20-year-old Caucasian male patient with a complex cyanotic heart defect, namely pulmonary atresia, with congenitally corrected transposition of the great arteries and ventricular septal defect after Rastelli-like surgery at the age of 5 years with homograft use. At the age of 20 years, the patient needed percutaneous pulmonary valve implantation owing to homograft dysfunction. Despite unusual course of the coronary arteries, balloon testing in the landing zone of the right ventricle outflow tract excluded potential coronary artery compression. Then, after presentation, a Melody valve was implanted successfully in the pulmonary valve position. The 8-year follow-up was uneventful. CONCLUSION: This is likely the first description of a percutaneous pulmonary valve implantation in such anatomy. Such a procedure is feasible; however, it requires exceptional caution owing to the anomalous coronary arteries course, which can be the reason for their compression.

Transcatheter pulmonary valve replacement after pulmonary homograft dysfunction.

Pulmonary homograft dysfunction is challenging to treat in patients with a previous Ross procedure, and results in significant morbidity and mortality in case of reoperation. We report the case of a patient with early severe pulmonary homograft stenosis 18 months after a Ross procedure and successful management using transcatheter pulmonary valve replacement.

Bacterial cellulose as a promising material for pulmonary valve prostheses: In vivo study in a sheep model.

OBJECTIVES: Currently, no consensus exists regarding the most durable prosthesis for pulmonary valve replacement. Bacterial cellulose is a resistant, nonbiodegradable, nonpyrogenic bioimplant with low hemolysis and clotting properties. We hypothesized that bacterial cellulose heart valve prostheses could be an attractive alternative for pulmonary valve replacement. METHODS: We conducted a large animal model experiment in three adult sheep. The animals underwent open-heart surgery and cardiopulmonary bypass for bacterial cellulose conduit implantation in the pulmonary position. The sheep were followed for seven months, and clinical and laboratory parameters were analyzed. Echocardiographic evaluations were performed at 3 and 7 months. After seven months, the sheep were sacrificed and an autopsy was performed. The explanted conduits were radiologically and histopathologically analyzed. RESULTS: All sheep survived the operation, showing good recovery and normal health status; no adverse events were noted during the 7-month postoperative follow-up. Interval laboratory findings were normal with no signs of hemolysis or infection. Echocardiographic analysis after 7 months revealed a normal mean pressure gradient with excellent cusp motion and coaptation; a trace of regurgitation was found in two sheep. X-ray analysis of the explanted conduits revealed no structural defects in the leaflets with minimal calcification. Histological examination showed slight thickening of the conduit by pannus formation. No material failure, no calcification inside the material, and only minor calcification extrinsic to the matrix were observed. CONCLUSIONS: This pilot study provides evidence that bacterial cellulose may be suitable for pulmonary valve prostheses and surgical pulmonary artery plasty. Further studies on the high pressure side of the left heart are needed.

Multicenter Experience for Early and Mid-Term Outcome of MyVal Transcatheter Pulmonary Valve Implantation.

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12-19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31-63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10-53 mmHg) pre-procedure to 10 mmHg (IQR 5-16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5-44 min). The large sizes-mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120-300 mL). Patients were followed for a median period of 360 days (IQR 164-525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Use of Three-Dimensional Intracardiac Echocardiography Catheter in the Evaluation of Prosthetic Pulmonary Valves after Transcatheter Replacement.

Transcatheter pulmonary valve replacement (TPVR) is commonly performed in patients with congenital heart disease as a safe alternative to replacement via open heart surgery. Intracardiac echocardiography (ICE) is a useful technique for evaluating multiple structures that are difficult to assess by other echocardiographic techniques, particularly the pulmonary valve. To our knowledge, the use of three-dimensional (3D) ICE catheters to evaluate prosthetic valves after TPVR has not been reported. Three-dimensional ICE catheters offer a comprehensive evaluation of transcatheter-deployed pulmonary valves through 3D, 3D color, xPlane, and multiplane reconstruction. The aim of this study is to demonstrate the feasibility of using 3D ICE catheters, outline their role in evaluating post-TPVR deployment success and complications, consider their additive value to two-dimensional ICE, and present our institutional experience with it in 50 cases of TPVR.

Reconstruction of right ventricular outflow tract with severe calcification: lantern procedure.

Right ventricular outflow tract reconstruction is repeatedly required after the Rastelli procedure. However, standard right ventricular outflow tract reconstruction using direct anastomosis on the posterior right ventricular outflow tract wall is unfeasible in cases with severe calcification. Herein, we present a novel technique called the "lantern procedure," which can fix the prosthetic pulmonary valve without anastomosis to the calcified right ventricular outflow tract wall.

Early Outcomes of Pulmonary Valve Replacement With the Edwards Inspiris Resilia Pericardial Bioprosthesis.

BACKGROUND: Controversy regarding the optimal pulmonary valve substitute remains, with no approved surgical valve for pulmonary valve replacement (PVR). Furthermore, unfavorable anatomy often precludes transcatheter PVR in patients with congenital heart disease. We therefore sought to evaluate the feasibility of the Edwards Inspiris pericardial aortic bioprosthesis in the pulmonary position in pediatric and adult patients requiring PVR. METHODS: Data from consecutive patients who underwent PVR from February 2019 to February 2021 at our institution were retrospectively reviewed. Postoperative adverse events included paravalvular or transvalvular leak, endocarditis, explant, thromboembolism, valve thrombosis, valve-related bleeding, hemolysis, and structural valve degeneration. Progression of valve gradients was assessed from discharge to 30 days and one year. RESULTS: Of 24 patients with median age of 26 years (interquartile range [IQR]: 17-33; range: 4-60 years), 22 (91.7%) patients had previously undergone tetralogy of Fallot repair and 2 (8.3%) patients had undergone double-outlet right ventricle repair in the neonatal period or infancy. All patients had at least mild right ventricular (RV) dilatation (median RV end-diastolic volume index 161.4, IQR: 152.3-183.5 mL/m2) and at least moderate pulmonary insufficiency (95.8%) or stenosis (8.3%). Median cardiopulmonary bypass and cross-clamp times were 71 (IQR: 63-101) min and 66 (IQR: 60-114) min, respectively. At a median postoperative follow-up of 2.5 years (IQR: 1.4-2.6; range: 1.0-3.0 years), there were no mortalities, valve-related reoperations, or adverse events. Postoperative valve gradients and the severity of pulmonary regurgitation did not change significantly over time. CONCLUSIONS: At short-term follow-up, the bioprosthesis in this study demonstrated excellent safety and effectiveness for PVR. Further studies with longer follow-up are warranted.

Long-term Outcomes of Polytetrafluoroethylene Bicuspid Pulmonary Valve Replacement.

BACKGROUND: In 2016 we reported promising midterm outcomes of bicuspid pulmonary valve replacement using 0.1-mm polytetrafluoroethylene (PTFE) membrane. This follow-up study analyzes long-term outcomes and risk factors for reintervention and structural valve deterioration (SVD). METHODS: We performed a retrospective review of the original 119 patients who underwent PTFE bicuspid pulmonary valve replacement. Median patient age was 16.9 years (range, 0.4-57.1). Reintervention was defined as any surgical or percutaneous catheter procedure on the PTFE valve. SVD was defined as development of a peak pressure gradient ≥ 50 mm Hg or at least a moderate amount of pulmonary regurgitation on follow-up echocardiography. RESULTS: The median follow-up duration was 9.5 years. The survival rate was 96.5% at 5 and 10 years, with 2 early and 2 late mortalities. Freedom from reintervention was 90.0% at 5 years and 63.3% at 10 years. Freedom from SVD was 92.8% at 5 years and 51.1% at 10 years, with regurgitation the predominant mode (64.6%). Freedom from both reintervention and SVD at 5 and 10 years were 89.1% and 49.5%, respectively. Multivariable analysis identified smaller valve diameter (hazard ratio, 0.82; P < .001) and more than trivial pulmonary regurgitation at discharge (hazard ratio, 5.81; P < .001) as risk factors for reintervention or SVD. CONCLUSIONS: Long-term results of the PTFE bicuspid pulmonary valve replacement were acceptable. However, improvements may be needed to reduce technical error and improve durability. Smaller valve diameter and more than trivial pulmonary regurgitation at discharge were risk factors for reintervention or SVD, warranting careful follow-up for timely reintervention.

Three-Patch Reconstruction of Hourglass Supravalvar Pulmonary Artery Stenosis Mid-Term Results.

BACKGROUND: Some patients with pulmonary stenosis present with a complex multilevel disease that involves the valve, the leaflets, and the sinotubular junction (STJ) forming an hourglass appearance. We herein report the mid-term results of our experience with the reconstruction of the supravalvar narrowing using three pericardial patches. MATERIAL AND METHODS: Retrospective analysis of patient charts and echocardiography studies of patients who underwent three-patch reconstruction of the pulmonary valve (PV) from 2013 to 2022. After PV transection distal to STJ, vertical incisions into the sinuses were performed, and leaflets were trimmed and thinned. The three sinuses were augmented using three pericardial patches. RESULTS: Nineteen patients underwent repair of hourglass supravalvar pulmonary stenosis. Mean weight at surgery was 9.4 kg (median 7.2, range 4.7-35); 16 patients underwent previous catheterization with unsuccessful balloon dilatation of the PV (13 pts.). Preoperative aortic/pulmonary annulus ratio was 1.02 (median 1, range 0.89-1.25). After surgery, gradients across the PV were significantly reduced (94 ± 26 vs 29 ± 9 mm Hg, P = .02). Postoperatively, 14 patients had mild or no pulmonary insufficiency (PI) and five had mild to moderate PI. At a mean follow-up of 71 months (median 78 months, range 8-137), gradients continued to decrease (29 ± 9 vs 15 ± 5 mm Hg, P < .001). CONCLUSION: The three-patch technique for the repair of supravalvar pulmonary stenosis is simple, reproducible, and achieves excellent and long-standing relief of the right ventricular outflow tract gradient.