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Texas Heart Institute Journal Sep 2022In this retrospective study, we compared the results from 2 pulmonary valve augmentation techniques in patients undergoing surgical repair of tetralogy of Fallot.
BACKGROUND
In this retrospective study, we compared the results from 2 pulmonary valve augmentation techniques in patients undergoing surgical repair of tetralogy of Fallot.
METHODS
Between 2015 and 2018, 18 patients had anterior pulmonary valve repair at our institution, and 26 patients had both anterior and posterior pulmonary valve repair.
RESULTS
Patients ranged from 6 months to 30 years of age. The median follow-up period was 8 months in the anterior augmentation group and 5 months in the anterior and posterior augmentation group. Postoperative echocardiograms indicated that only 2 patients (11%) in the anterior augmentation group had moderate or severe pulmonary insufficiency, compared with no patients in the anterior and posterior augmentation group. At follow-up, pulmonary insufficiency was seen in 3 patients (17%) in the anterior augmentation group and no patients in the anterior and posterior augmentation group.
CONCLUSION
Reconstruction of the native pulmonary valve accompanied by pulmonary cusp augmentation can decrease or even circumvent postoperative pulmonary insufficiency. Both anterior augmentation and anterior and posterior augmentation techniques are easily applied; however, we believe that the anterior and posterior augmentation technique is superior in terms of early postoperative and follow-up pulmonary insufficiency outcomes.
Topics: Humans; Infant; Pulmonary Valve; Pulmonary Valve Insufficiency; Retrospective Studies; Tetralogy of Fallot; Treatment Outcome
PubMed: 36223246
DOI: 10.14503/THIJ-20-7368 -
Anatolian Journal of Cardiology Apr 2020
Topics: Aneurysm; Computed Tomography Angiography; Diagnosis, Differential; Dyspnea; Humans; Male; Middle Aged; Pulmonary Artery; Pulmonary Valve Stenosis
PubMed: 32352412
DOI: 10.14744/AnatolJCardiol.2020.57598 -
BMC Veterinary Research Jul 2020Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy currently described in humans and cats. It consists of a spongy myocardium characterized by...
BACKGROUND
Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy currently described in humans and cats. It consists of a spongy myocardium characterized by prominent trabeculation and deep recesses involving more than 50% of the ventricular thickness. We describe the clinical and pathological features of LVNC combined with tricuspid valve dysplasia, double-orifice tricuspid valve and severe pulmonary stenosis in a puppy. In addition, we briefly review the LVNC causes, pathogenesis, forms and current diagnostic criteria.
CASE PRESENTATION
A seven-week-old intact German Shorthaired Pointer-cross male was presented with a poor body condition, exercise intolerance and dyspnea. Clinical exam identified a bilateral systolic murmur (grade IV/VI over the right heart base and grade III/VI over the left heart base). Echocardiography identified tricuspid valve dysplasia, mild mitral regurgitation, and severe pulmonic stenosis with a trans-valvar systolic pressure gradient of 106 mmHg. Left ventricular noncompaction was diagnosed by necropsy and further confirmed histopathologically by the presence of two distinct myocardial layers: an inner noncompacted zone covering more than 50% of ventricular thickness containing prominent trabeculation and deep recesses, and an outer zone of compact myocardium.
CONCLUSIONS
This is the first case describing LVNC in a canine patient, supporting the introduction of this form of heart disease as a differential diagnosis for cardiomyopathies in juvenile and adult dogs.
Topics: Animals; Cardiomyopathies; Dog Diseases; Dogs; Echocardiography; Heart Defects, Congenital; Heart Ventricles; Male; Pulmonary Valve Stenosis; Tricuspid Valve
PubMed: 32703195
DOI: 10.1186/s12917-020-02480-7 -
Interactive Cardiovascular and Thoracic... Jul 2022Given the anatomical variations of tetralogy of Fallot (TOF), different surgical techniques can be used to achieve correction. Transannular patches (TAPs) are the most...
OBJECTIVES
Given the anatomical variations of tetralogy of Fallot (TOF), different surgical techniques can be used to achieve correction. Transannular patches (TAPs) are the most commonly used technique; they are associated with right ventricular dysfunction, the incidence of which can be reduced through pulmonary valve preservation.
METHODS
Between January 2010 and July 2019, we performed 274 surgical corrections of tetralogy of Fallot at Fundación Cardioinfantil; 63 patients (23%) underwent repair with a TAP in addition to a pulmonary neovalve (Group I), 66 patients (24.1%) received a TAP without a pulmonary valve (Group II) and 145 patients (52.9%) had a repair with valve preservation (Group III). We analysed patient's characteristics before, during and after surgery at a 30-day follow-up.
RESULTS
We found that patients in Group III were older (P = 0.04). Group II had the lowest level of O2 saturation before surgery (82%, P = 0.001). Cardiopulmonary bypass and aortic cross-clamp times were longer in Group I (P < 0.001). Right ventricular dysfunction was less frequent in Group III (15.9%, P = 0.011). Severe residual pulmonary regurgitation was more common in Group II (21.9%, P = 0.001).
CONCLUSIONS
Preservation of the pulmonary valve is an important factor for immediate postoperative management of tetralogy of Fallot. Patients who were repaired with a TAP with or without a pulmonary neovalve had a higher incidence of right ventricular dysfunction than those with pulmonary valve preservation.
Topics: Follow-Up Studies; Humans; Infant; Pulmonary Valve; Pulmonary Valve Insufficiency; Retrospective Studies; Tetralogy of Fallot; Treatment Outcome; Ventricular Dysfunction, Right
PubMed: 35640540
DOI: 10.1093/icvts/ivac155 -
Frontiers in Pediatrics 2021Pulmonary valve (PV) stenosis affects cardiac pulmonary function and exercise performance. A cardiopulmonary exercise test (CPET) combined with a transthoracic...
OBJECTIVE
Pulmonary valve (PV) stenosis affects cardiac pulmonary function and exercise performance. A cardiopulmonary exercise test (CPET) combined with a transthoracic echocardiogram (TTE) can measure exercise performance, disease progression, and treatment effects. We assessed the exercise capacity in children with PV stenosis by conducting CPET and TTE.
METHODS
From 2005 to 2021, 84 patients with PV stenosis aged 6-18 years were enrolled; 43 were treated with balloon pulmonary valvuloplasty (BPV) (Group A), and 41 received follow-up care (Group B), and their CPET and pulmonary function test results were compared with 84 healthy, matched individuals (Control). We also conducted TTE to compare the peak pulmonary artery pulse wave velocity and pulmonary valve (PV) area before and after catheterization and follow-up care.
RESULTS
There were no significant differences among the CPET parameters of the patient groups and controls in anaerobic metabolic equivalent (MET) (group A: 6.44 ± 0.58; group B: 6.28 ± 0.47, control: 6.92 ± 0.39, = 0.110), peak MET (group A: 9.32 ± 0.74; group B: 9.13 ± 0.63; control: 9.80 ± 0.52, = 0.263), and heart rate recovery (group A: 28.04 ± 4.70; group B: 26.44 ± 3.43, control:26.10 ± 2.42, = 0.718). No significant differences were found in the pulmonary functions between the three groups. The pulmonary artery pulse wave velocity significantly decreased after catheterization (3.97 ± 1.50 vs. 1.95 ± 0.94, < 0.0001), but not after follow-up care (1.67 ± 0.77 vs. 1.75 ± 0.66, = 0.129). The pulmonary vale area significantly improved in group A (0.89 ± 0.71 vs. 1.16 ± 0.58, < 0.0001), whereas only insignificant progression of PV stenosis was observed in group B (1.60 ± 0.64 vs. 1.57 ± 0.65, = 0.110).
CONCLUSIONS
Patients treated with BPV had a similar exercise capacity with that of patients under follow-up care and the healthy controls. Larger or multi-center studies should be conducted to confirm the physical fitness of pediatric patients with PV stenosis after management.
PubMed: 35096713
DOI: 10.3389/fped.2021.802645 -
The Journal of Thoracic and... Dec 2022The Ross procedure is an excellent option for children or young adults who need aortic valve replacement because it can restore survival to that of the normal...
OBJECTIVE
The Ross procedure is an excellent option for children or young adults who need aortic valve replacement because it can restore survival to that of the normal aged-matched population. However, autograft remodeling can lead to aneurysmal formation and reoperation, and the biomechanics of this process is unknown. This study investigated postoperative autograft remodeling after the Ross procedure by examining patient-specific autograft wall stresses.
METHODS
Patients who have undergone the Ross procedure who had intraoperative pulmonary root and aortic specimens collected were recruited. Patient-specific models (n = 16) were developed using patient-specific material property and their corresponding geometry from cine magnetic resonance imaging at 1-year follow-up. Autograft ± Dacron for aneurysm repair and ascending aortic geometries were reconstructed to develop patient-specific finite element models, which incorporated material properties and wall thickness experimentally measured from biaxial stretching. A multiplicative approach was used to account for prestress geometry from in vivo magnetic resonance imaging. Pressure loading to systemic pressure (120/80) was performed using LS-DYNA software (LSTC Inc, Livermore, Calif).
RESULTS
At systole, first principal stresses were 809 kPa (25%-75% interquartile range, 691-1219 kPa), 567 kPa (485-675 kPa), 637 kPa (555-755 kPa), and 382 kPa (334-413 kPa) at the autograft sinotubular junction, sinuses, annulus, and ascending aorta, respectively. Second principal stresses were 360 kPa (310-426 kPa), 355 kPa (320-394 kPa), 272 kPa (252-319 kPa), and 184 kPa (147-222 kPa) at the autograft sinotubular junction, sinuses, annulus, and ascending aorta, respectively. Mean autograft diameters were 29.9 ± 2.7 mm, 38.3 ± 5.3 mm, and 26.6 ± 4.0 mm at the sinotubular junction, sinuses, and annulus, respectively.
CONCLUSIONS
Peak first principal stresses were mainly located at the sinotubular junction, particularly when Dacron reinforcement was used. Patient-specific simulations lay the foundation for predicting autograft dilatation in the future after understanding biomechanical behavior during long-term follow-up.
Topics: Humans; Child; Young Adult; Aged; Autografts; Transplantation, Autologous; Polyethylene Terephthalates; Aortic Valve; Aortic Valve Insufficiency; Pulmonary Valve
PubMed: 34538420
DOI: 10.1016/j.jtcvs.2021.08.058 -
Circulation. Cardiovascular... Apr 2021The folded valve is a manual shortening of the Melody device, which has been validated as a valuable therapeutic option for the management of dysfunctional right...
BACKGROUND
The folded valve is a manual shortening of the Melody device, which has been validated as a valuable therapeutic option for the management of dysfunctional right ventricular outflow tracts needing a short valved stent. In this article, we aimed to evaluate, in a multicenter cohort, the mid-term outcomes of patients in whom a percutaneous pulmonary valve implantation was performed using the folded valve technique.
METHODS
A 2012 to 2018 retrospective multicenter study was performed in 7 European institutions. All patients who benefit from percutaneous pulmonary valve implantation with a folded Melody valve were included.
RESULTS
A total of 49 patients (median age, 19 years [range 4–56], 63% male) were included. The primary percutaneous pulmonary valve implantation indication was right ventricular outflow tract stenosis (n=19; 39%), patched native right ventricular outflow tracts were the most common substrate (n=15; 31%). The folded technique was mostly used in short right ventricular outflow tracts (n=28; 57%). Procedural success was 100%. After a median follow-up of 28 months (range, 4–80), folded Melody valve function was comparable to the immediate postimplantation period (mean transvalvular peak velocity=2.6±0.6 versus 2.4±0.6 m/s, P>0.1; only 2 patients had mild pulmonary regurgitation). Incidence rate of valve-related reinterventions was 2.1% per person per year (95% CI, 0.1%–3.9%). The probability of survival without valve-related reinterventions at 36 months was 90% (95% CI, 76%–100%).
CONCLUSIONS
The folded Melody valve is a safe technique with favorable mid-term outcomes up to 6.5 years after implantation, comparable with the usual Melody valve implantation procedure. Complications and reinterventions rates were low, making this technique relevant in selected patients.
Topics: Adolescent; Adult; Cardiac Catheterization; Child; Child, Preschool; Female; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Male; Middle Aged; Prosthesis Design; Pulmonary Valve; Pulmonary Valve Insufficiency; Retrospective Studies; Treatment Outcome; Young Adult
PubMed: 33726503
DOI: 10.1161/CIRCINTERVENTIONS.120.009707 -
Journal of the American College of... Feb 2020Transcatheter pulmonary valve replacement (TPVR) is associated with a risk of procedural serious adverse events (SAE) and exposure to ionizing radiation.
BACKGROUND
Transcatheter pulmonary valve replacement (TPVR) is associated with a risk of procedural serious adverse events (SAE) and exposure to ionizing radiation.
OBJECTIVES
The purpose of this study was to define the risk of, and associations with, SAE and high-dose radiation exposure using large-scale registry data.
METHODS
The analysis of the multicenter C3PO-QI registry was limited to patients who underwent TPVR from January 1, 2014, to December 31, 2016. SAE were defined as the occurrence of ≥1 moderate, major, or catastrophic events. Radiation dose was reported as dose area product adjusted for weight. Associations with outcome measures were explored in univariate and multivariable analyses.
RESULTS
A total of 530 patients (59% male) underwent TPVR at a median age of 18.3 years (interquartile range [IQR]: 12.9 to 27.3 years) and weight of 58 kg (IQR: 43 to 77 kg) at 14 centers. Implant substrate included homograft (41%), bioprosthesis (30%), native right ventricular outflow tract (RVOT) (27%) and other (2%). TPVR indications were pulmonary insufficiency (28%), stenosis (23%), and mixed (49%). AE and SAE occurred in 26% and 13% of cases, respectively, including 1 mortality. SAE were more frequent in homograft conduit than other RVOT substrates, although SAE type and severity differed between implant substrates. Median radiation dose was 198 μGy·m/kg (IQR: 94 to 350 μGy·m/kg). Higher radiation dose was associated with older age, greater RVOT obstruction, and concomitant interventions (p < 0.001). During a median follow-up duration of 1 year, 13.3% underwent catheterization, surgery, or both, unrelated to infection. Younger age, smaller size, and hemodynamic and anatomic factors indicative of greater RVOT obstruction were associated with TPV reintervention.
CONCLUSIONS
The incidence of SAE during TPVR in the C3PO-QI registry is high, but mortality is uncommon. Radiation dose is greater than for other congenital interventions and is associated with patient and procedural factors. Reintervention is common during early follow-up.
Topics: Adolescent; Adult; Cardiac Catheterization; Cardiac Surgical Procedures; Catheterization; Child; Endocarditis; Female; Heart Defects, Congenital; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Incidence; Male; Multivariate Analysis; Pulmonary Valve; Pulmonary Valve Insufficiency; Radiation Exposure; Radiation, Ionizing; Registries; Risk; Tetralogy of Fallot; Young Adult
PubMed: 32000947
DOI: 10.1016/j.jacc.2019.11.042 -
SAGE Open Medical Case Reports 2023Primary cardiac tumours are rare and most of them are benign. Myxomas, fibroelastomas and lipomas are common in adults. Primary valvular cardiac tumours are even more...
Primary cardiac tumours are rare and most of them are benign. Myxomas, fibroelastomas and lipomas are common in adults. Primary valvular cardiac tumours are even more rare and affect all four valves in a similar proportion. Valvular lipomas are very rare. In the pulmonary valve there is only one described. Lipomas can be spindle-cell varieties. But of these, there is only one described in a valve, and it is placed in the aortic valve. Pulmonary valve lipomas can produce obstruction to the right ventricular outflow tract as well as pulmonary valve regurgitation, or pulmonary embolism. Symptoms may be dyspnoea, angina, arrhythmias, or syncope. We aim to illustrate with this case report how we came into this very rare pathology, so we present a 54-year-old woman with a giant spindle-cell lipoma located in the anterior pulmonary leaflet and severe dyspnoea. Total resection of the tumour was performed and restoration of valve function was obtained by means of bicuspidization of the remaining pulmonary leaflets. She had a good recovery after surgery and no complication during the postoperative evolution, being discharged from hospital after 7 days from surgery, with echocardiographic control showing good biventricular function, absence of tumour or obstruction, and minimal pulmonary valve regurgitation.
PubMed: 38033915
DOI: 10.1177/2050313X231216544 -
Circulation. Arrhythmia and... Jun 2023Ventricular arrhythmias and sudden death are recognized complications in tetralogy of Fallot. Electrophysiological studies (EPS) before pulmonary valve replacement...
BACKGROUND
Ventricular arrhythmias and sudden death are recognized complications in tetralogy of Fallot. Electrophysiological studies (EPS) before pulmonary valve replacement (PVR), the most common reintervention in tetralogy of Fallot, could potentially inform therapy to improve arrhythmic outcomes.
METHODS
A prospective multicenter study was conducted to systematically assess EPS with programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR from January 2020 to December 2021. A standardized stimulation protocol was used across all centers.
RESULTS
A total of 120 patients were enrolled, mean age 39.2±14.5 years, 53.3% males. Sustained ventricular tachycardia was induced in 27 (22.5%) patients. When identifiable, the critical isthmus most commonly implicated (ie, in 90.0%) was between the ventricular septal defect patch and pulmonary annulus. Factors independently associated with inducible ventricular tachycardia were history of atrial arrhythmia (odds ratio, 8.56 [95% CI, 2.43-34.73]) and pulmonary annulus diameter >26 mm (odds ratio, 5.05 [95% CI, 1.47-21.69]). The EPS led to a substantial change in management in 23 (19.2%) cases: 18 (15.0%) had catheter ablation, 3 (2.5%) surgical cryoablation during PVR, and 9 (7.5%) defibrillator implantation. Repeat EPS 5.1 (4.8-6.2) months after PVR was negative in 8 of 9 (88.9%) patients. No patient experienced a sustained ventricular arrhythmia during 13 (6.1-20.1) months of follow-up.
CONCLUSIONS
Systematically performing programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR yields a high rate of inducible ventricular tachycardia and carries the potential to alter management. It remains to be determined whether a standardized treatment approach based on the results of EPS will translate into improved outcomes.
REGISTRATION
URL: https://clinicaltrials.gov/ct2/show/NCT04205461; Unique identifier: NCT04205461.
Topics: Male; Humans; Young Adult; Adult; Middle Aged; Female; Pulmonary Valve; Tetralogy of Fallot; Heart Valve Prosthesis Implantation; Prospective Studies; Treatment Outcome; Tachycardia, Ventricular; Arrhythmias, Cardiac; Pulmonary Valve Insufficiency
PubMed: 37170812
DOI: 10.1161/CIRCEP.122.011745