Melody transcatheter pulmonary valve replacement: a single-center case series in Southeast Asia.

BACKGROUND: Studies of transcatheter pulmonary valve replacement (TPVR) with the Melody valve have demonstrated good clinical and hemodynamic outcomes. Our study analyzes the midterm clinical and hemodynamic outcomes for patients who underwent Melody valve implantation in Southeast Asia. METHODS: Patients with circumferential conduits or bioprosthetic valves and experiencing post-operative right ventricular outflow tract (RVOT) dysfunction were recruited for Melody TPVR. RESULTS: Our cohort (n = 14) was evenly divided between pediatric and adult patients. The median age was 19 years (8-38 years), a male-to-female ratio of 6:1 with a median follow-up period of 48 months (16-79 months), and the smallest patient was an 8-year-old boy weighing 18 kg. All TPVR procedures were uneventful and successful with no immediate mortality or conduit rupture. The primary implant indication was combined stenosis and regurgitation. The average conduit diameter was 21 ± 2.3 mm. Concomitant pre-stenting was done in 71.4% of the patients without Melody valve stent fractures (MSFs). Implanted valve size included 22-mm (64.3%), 20-mm (14.3%), and 18-mm (21.4%). After TPVR, the mean gradient across the RVOT was significantly reduced from 41 mmHg (10-48 mmHg) to 16 mmHg (6-35 mmHg) at discharge, p < 0.01. Late follow-up infective endocarditis (IE) was diagnosed in 2 patients (14.3%). Overall freedom from IE was 86% at 79 months follow-up. Three patients (21.4%) developed progressive RVOT gradients. CONCLUSION: For patients in Southeast Asia with RVOT dysfunction, Melody TPVR outcomes are similar to those reported for patients in the US in terms of hemodynamic and clinical improvements. A pre-stenting strategy was adopted and no MSFs were observed. Post-implantation residual stenosis and progressive stenosis of the RVOT require long term monitoring and reintervention. Lastly, IE remained a concern despite vigorous prevention and peri-procedural bacterial endocarditis prophylaxis.

Clinical Presentation and Therapy of d-Transposition of the Great Arteries.

d-Transposition of the great arteries (d-TGA) is the most common form of congenital heart disease that presents with cyanosis in a newborn. The aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. It constitutes 3-5% of all congenital heart defects. In a simple d-TGA (about two-thirds of patients), there is no other cardiac abnormality other than a patent foramen ovale (PFO) and a patent ductus arteriosus (PDA). In a complex d-TGA additional cardiac abnormalities such as VSD, pulmonary stenosis or coronary abnormalities are present. About one-third to 40% of patients with d-TGA have an associated ventricular septal defect. Among patients with d-TGA, 6% of those with intact ventricular septum and 31% of those with ventricular septal defect have associated pulmonary stenosis. Coronary abnormalities are of importance with regard to the complexity of surgical repair.

First reported long-term two- and three-dimensional echocardiographic follow-up with histopathological analysis of a transcatheter pulmonary valve implantation in a pet dog.

Transcatheter pulmonary valve implantation (TPVI) is indicated for use in the management of failing pulmonary valves in humans. We report here the long-term follow-up of the first documented transcatheter pulmonary valve implanted in a client-owned dog. A one-year-old Beagle dog with severe congenital type A valvular pulmonic stenosis first underwent percutaneous balloon pulmonary valvuloplasty, leading two years later to severe pulmonary regurgitation. A TPVI using a Melody™ bioprosthetic valve was then successfully performed, with normalization of the right heart cavities. Repeated two- and three-dimensional transthoracic echocardiographic examinations combined with Doppler modes confirmed the appropriate position and function of the valve for four years. Mitral myxomatous valvular degeneration led to refractory left-sided congestive heart failure, and the dog was humanely euthanized. After postmortem examination, X-ray imaging and histopathological evaluation of the stent and the valve were performed. Ex-vivo imaging of the implanted valve using a Faxitron® Path radiography system and microscopic evaluation of the implanted stent and bioprosthetic leaflets did not show any relevant leaflet or stent alterations. This case provides a proof of concept in interventional veterinary cardiology, showing that TPVI can be performed in dogs with subsequent long-term maintaining normal pulmonary valve function.

Pulmonary artery dissection secondary to ventricular septal defect and pulmonary valve stenosis.

The aortic short axis view demonstrated the widening of the pulmonary artery and the membrane-like echo in the pulmonary artery divided it into true lumen and false lumen. And the flow of the ruptured openings on the band-like echo was clearly revealed by Color Doppler.

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.

Prenatal Predictors for Pulmonary Balloon Valvuloplasty in the Newborn.

Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1-58) days and 11 (22%) underwent outpatient PBV at 51.8 (11-174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02-94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS.

64-Slice ECG-gated computed tomographic angiography for assessment of coronary arteries in brachycephalic dogs with pulmonary stenosis.

BACKGROUND: Brachycephalic dogs with pulmonary stenosis are known to have a higher incidence of concurrent coronary artery abnormalities than non-brachycephalic breeds, which increases risk when performing balloon valvuloplasty. The use of ECG-gated CT angiography has been reported for the evaluation of coronary arteries in normal dogs and dogs with pulmonary stenosis. The purpose of this study was to report findings of coronary artery origination and morphology of main branches using ECG-gated CT angiography in brachycephalic dogs with pulmonary stenosis. METHODS: An ECG-gated CT angiographic protocol was used to image coronary artery anatomy in nine brachycephalic dogs with pulmonary stenosis. Images were assessed for quality as well as coronary artery morphology by one veterinary radiologist, one veterinary cardiologist and one veterinary radiology resident. RESULTS: All nine dogs had good to excellent image quality. Coronary artery anomalies were identified in three of nine dogs: one R2A anomaly, one L2A anomaly and one L2C anomaly. Two dogs were assessed to be poor balloon valvuloplasty candidates based on CT angiographic images. LIMITATION: Coronary artery morphology was not confirmed via postmortem examination in all patients. CONCLUSION: ECG-gated CT angiography is a minimally invasive imaging modality capable of diagnosing various coronary artery anomalies in brachycephalic dogs with pulmonary stenosis and aiding in the determination of patient candidacy for balloon valvuloplasty.

[Analysis of echocardiographic features and postnatal management of fetal pulmonary valve stenosis].

Objective: To investigate the echocardiographic features, consistency of diagnosis between fetal and postnatal periods and postnatal clinical outcomes of fetal pulmonary valve stenosis (PS) with different degrees. Methods: This study was a retrospective cohort study comprising 108 cases of fetal PS diagnosed during the fetal period and followed up postnatally at Xinhua Hospital, Shanghai Jiaotong University School of Medicine from November 2012 to February 2023. Echocardiographic characteristics, including morphological and hemodynamic features were collected for all fetuses who were then were followed up to at least 6 months after birth. One-way analysis of variance and Kruskal-Wallis test were used to compare the differences in the echocardiographic features among fetuses with different degrees of PS. Subsequently, McNemar test was used to assess the consistency of diagnosis between the fetal and postnatal periods. Furthermore, Logistic regression analysis was applied to explore the risk factors for neonatal intervention in fetuses with moderate PS and the receiver operating characteristic (ROC) curve was utilized to ascertain the optimal cut-off value for continuous variables. Results: The age of the mothers of the 108 fetuses at the initial assessment was (30.8±4.0) years, and the gestational age was 26.5 (24.6, 30.0) weeks. The fetuses were categorized into mild (17 cases), moderate (49 cases), and severe groups (42 cases) based on the initial echocardiographic features. Mild PS was characterized by valve thickening and hyperechogenicity combined with systolic flow acceleration or dilation of main pulmonary artery. Moderate PS exhibited both restricted valve motion and a colorful blood flow pattern at the valve orifice. The peak flow velocities of fetuses with moderate and critical PS were notably higher than those in the mild group ((2.66±0.86) and (2.77±1.30) vs. (1.43±0.59)m/s, F=14.52, P<0.001). In critical PS, all cases showed retrograde ductal flow, with a significantly higher proportion of a small right ventricle compared to the mild and moderate PS (42.9% (18/42) vs. 0 and 2.0% (1/49), χ2=31.73, P<0.001). The proportion of severe tricuspid regurgitation was also higher (35.7% (15/42) vs. 0 and 10.2% (5/49), χ2=36.94, P<0.001). Compared to mild and severe PS, the consistency of diagnosis between fetal and postnatal periods in moderate PS was lower (40.8% (20/49) vs.13/17 and 80.3% (35/42), χ2=12.45, P=0.006). The systolic flow velocity was identified as an independent risk factor for neonatal intervention in fetuses with moderate PS (OR=7.21, 95%CI2.11-24.62). A flow velocity of ≥2.18 m/s in second trimester and ≥3.15 m/s in third trimester indicated the necessity of neonatal intervention for fetal moderate PS. Among the 108 fetuses, 68 underwent surgical intervention and all survived. Additionally, 39 fetuses were regularly followed up. A sole non-surgical fatality occurred, leading to a 6-month survival rate of 99.1% (107/108). Conclusions: Various degrees of fetal PS demonstrate distinctive morphological and hemodynamic alterations in echocardiography. The disparity in severity between the postnatal and fetal stages requires ongoing monitoring for fetal PS. The prognosis for fetal PS is generally favorable.

Fetal Diagnosis of Supravalvular Aortic Stenosis and Pulmonary Stenosis in a Family with Non-Syndromic Elastin Mutation.

Supravalvular aortic stenosis (SVAS) has been well described in Williams-Beuren Syndrome and non-syndromic elastin (ELN) mutations. Non-syndromic ELN mutations are inherited in an autosomal dominant pattern with incomplete penetrance and variable expressivity. ELN haploinsufficiency leads to progressive arteriopathy, typically affecting the aortic sinotubular junction. Multi-level pulmonary stenosis has also been reported and biventricular obstruction may portend a worse prognosis. Fetal presentation of ELN mutation with SVAS has not been previously reported in the literature. We present a case of fetal diagnosis of SVAS and multi-level pulmonary stenosis in a family with a known pathogenic ELN mutation (Exon 6, c.278del [p.Pro93Leufs*29]). On the fetus' initial fetal echo, there was only mild flow acceleration through the aortic outflow tract, however, she went on to develop progressive bilateral obstruction. In the early post-natal period, the child was clinically asymptomatic and showed similar mild SVAS and mild valvar and supravalvular pulmonary stenosis. Our case highlights the need for serial monitoring of fetuses with suspected or confirmed ELN arteriopathy.

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.

Intracardiac Three-Dimensional Image as Surgical Decision-Making Tool of Congenital Heart Disease.

This study aimed to evaluate the anatomical reproducibility of a preoperative intracardiac 3D image (IC image) created using computed tomography, and to investigate its usefulness as a surgical decision-making tool. Between 2012 and 2022, ventricular septal defect (VSD) patients, and double outlet right ventricle (DORV) or transposition of the great arteries (TGA) with pulmonary stenosis (PS) patients who underwent cardiac surgery and had preoperative computed tomography were enrolled. SYNAPSE VINCENT® (Fujifilm) was used to create an IC image which was analyzed retrospectively. In 14 VSD patients, the diagnostic consistency rate in the Soto classification with intraoperative findings was 100% (14/14) for IC image versus 64% (9/14) for transthoracic echocardiography (P = 0.04). The defect size showed a higher correlation coefficient with IC image (0.837, P = 0.001) than with transthoracic echocardiography (0.567, P = 0.034). In 11 DORV/TGA with PS patients, the diagnostic consistency rate in the Lev classification was 100% (9/9) for IC image versus 77% (7/9) for transthoracic echocardiography (P = 0.47). The secondary interventricular foramen (SVF)/left ventricular outflow tract (LVOT) ratio by IC image was significantly smaller in the biventricular-repair group (median 0.71, IQR 0.67-1.06) than in the univentricular-repair group (median 1.79, IQR 1.53-2.42) (P = 0.006). An IC image is useful as a surgical decision-making tool for simple VSDs and complex congenital heart diseases such as DORV or TGA with pulmonary stenosis. The SVF/LVOT ratio determined from the IC image may be a useful indicator for avoiding LVOT obstruction.

Successful balloon angioplasty in a cat with unilateral left pulmonary artery branch stenosis.

A 9-month-old intact male Ragdoll cat was presented for evaluation of a left-sided systolic murmur that was first auscultated during examination for a newly developed cough. Transthoracic echocardiography revealed a narrowed left pulmonary artery and an increase in flow velocities at the level of the narrowing, consistent with left pulmonary artery branch stenosis. The right pulmonary artery appeared normal. Balloon angioplasty was performed and successfully reduced pressure gradient across the stenosis. The patient continues to do well 14 months after the procedure.

Echocardiography: conotruncal anomaly: a case of common arterial trunk with intact ventricular septum and hypoplastic left heart complex with unbalanced pulmonary stenoses.

We describe the echocardiographic findings of a common arterial trunk with intact ventricular septum, mitral and left ventricular hypoplasia, atretic left ventricular outlet and bilateral, and unbalanced pulmonary artery stenoses.

Surgical correction of an infundibular pulmonic stenosis and ventricular septal defect in a Shetland Sheepdog.

A four-month-old male Shetland Sheepdog presented with exercise intolerance. Physical examination revealed an IV/VI left cranial systolic heart murmur. Echocardiography showed a severe infundibular pulmonic stenosis and a concomitant restrictive ventricular septal defect. As clinical signs of congestive right-sided heart failure worsened and were refractory to medical treatment, surgical correction was advised. Via sternotomy, with cardiopulmonary bypass and cardioplegic cardiac arrest, ventricular septal defect closure and resection of the stenotic infundibular band were performed through right ventriculotomy, followed by patch enlargement. Postoperative recovery was uneventful and echocardiography showed complete resolution of the stenosis and successful closure of the ventricular septal defect. Follow-up echocardiography revealed restenosis after seven weeks and recurrence of right-sided heart failure three months postoperatively. Stenting of the restenosis was attempted via a hybrid procedure with sternotomy and direct transventricular approach. The dog developed fatal ventricular fibrillation during stent deployment. This is the first dog in which surgical right ventricular patch enlargement under cardiopulmonary bypass is reported for the treatment of a primary infundibular pulmonic stenosis.

Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects.

Pulmonary stenosis (PS) is mainly a congenital defect that accounts for 7-12% of congenital heart diseases (CHD). It can be isolated or, more frequently, associated with other congenital defects (25-30%) involving anomalies of the pulmonary vascular tree. For the diagnosis of PS an integrated approach with echocardiography, cardiac computed tomography and cardiac magnetic resonance (CMR) is of paramount importance for the planning of the interventional treatment. In recent years, transcatheter approaches for the treatment of PS have increased however, meaning surgery is a possible option for complicated cases with anatomy not suitable for percutaneous treatment. The present review aims to summarize current knowledge regarding diagnosis and treatment of PS.

Fate of transposition of the great arteries with pulmonary stenosis after double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire.

OBJECTIVES: For transposition of the great arteries with unrestricted ventricular septal defect and pulmonary stenosis, double-root translocation is reported to reconstruct ideal double artery roots with growth potential. However, prospective long-term studies describing the long-term outcomes are still scarce. Therefore, the aim was to assess development of double artery roots, hemodynamics, and freedom from death and heart failure 17 years after double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire procedures. METHODS: In this prospective population-based study, 266 patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis (from July 2004 to August 2021) were consecutively included before surgery. All patients were divided into 3 groups based on the type of operation: double-root translocation (174), Rastelli (68), and Réparation à l'Etage Ventriculaire (24), who accepted postoperative evaluations annually. Generalized linear mixed model analysis was performed to determine growth potential of artery roots. RESULTS: Longitudinal repeated computed tomography measurements show the pulmonary root has significantly increased diameter (0.62 [0.03] mm/y, P < .001) over time and an adequate Z-score (-0.18) at the last follow-up only in the double-root translocation group. The pressure gradients of double outflow tracts in the double-root translocation group were the least among 3 groups. The probabilities of freedom from death/heart failure at the 15th year were 73.1%, 59.3%, and 60.9% in the double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire groups, respectively (double-root translocation vs Rastelli, P = .026; double-root translocation vs Réparation à l'Etage Ventriculaire, P = .009; Rastelli vs Réparation à l'Etage Ventriculaire, P = .449). CONCLUSIONS: By reconstructing ideal double artery roots, double-root translocation can provide postoperative long-term excellent hemodynamics and minimal death and heart failure for patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis.