2024 Statement from Asia expert operators on transcatheter pulmonary valve replacement.

Transcatheter pulmonary valve replacement (TPVR), also known as percutaneous pulmonary valve implantation, refers to a minimally invasive technique that replaces the pulmonary valve by delivering an artificial pulmonary prosthesis through a catheter into the diseased pulmonary valve under the guidance of X-ray and/or echocardiogram while the heart is still beating not arrested. In recent years, TPVR has achieved remarkable progress in device development, evidence-based medicine proof and clinical experience. To update the knowledge of TPVR in a timely fashion, and according to the latest research and further facilitate the standardized and healthy development of TPVR in Asia, we have updated this consensus statement. After systematical review of the relevant literature with an in-depth analysis of eight main issues, we finally established eight core viewpoints, including indication recommendation, device selection, perioperative evaluation, procedure precautions, and prevention and treatment of complications.

Effect of transcatheter closure by AmplatzerTM Duct Occluder II in patients with small ventricular septal defect.

Transcatheter closure of ventricular septal defect (VSD) is an alternative treatment of surgery. However, because of the potential risk of the complete atrioventricular block (AVB) and the lack of available dedicated devices for VSD, this procedure rarely used most centers. Recently in Thailand, with distinct device design that may cause less clamp force and radial force, the AmplatzerTMDuct Occluder (ADO) II has become available for the closure of small VSDs. This is a retrospective review of 49 patients who underwent transcatheter VSD closure using ADO II at Songklanagarind hospital and Queen Sirikit National Institute of Child Health between January 2014 and December 2016 with an intention to report procedural success rate and 12-months-outcomes. Twenty-six cases were female. The median age and weight at treatment were 7.8 year (ranged from 1.9 to 25 year) and 25.1 kg (ranged from 11 and 57 kg). The majority (65.3%) of the VSD was a perimembranous defect. The mean diameter of LV entry was 5.2 ± 2.5 mm and the mean RV exit was 3.2 ± 0.8 mm. Four patients had more than 1 exit at RV orifice. The mean length of the VSD was 5.3 ± 1.8 mm. The mean aortic valve-VSD distance was 3.9 ± 2.0 mm. The mean tricuspid valve-VSD distance was 3.8 ± 2.5 mm. The procedural success achieved in all patients (100%), most of the implantations were performed in a retrograde approach (53.1%). The ADO II was successfully implanted in 2 cases (5%) who failed other devices in the first attempts. There were no major complications. There were no changes in the severity of tricuspid and aortic valve regurgitation immediate after implantation and at 12 months' follow-up. Three patients (6%) had tiny to small residual shunt at 12 months' follow-up. None of the patients had a complete heart block. However, new-onset of left bundle branch block and right bundle branch block occurred in 2 (5%) cases and 5 (10%) cases, respectively. With symmetrical double-disc design and softness of the device, the ADO II can be safely and effectively used for closure of the VSDs with the defect size less than 6 mm.

Feasibility and early outcomes of aortic coarctation treatments with BeGraft Aortic stent.

OBJECTIVES: To assess feasibility and early outcomes of using BeGraft Aortic stent in the treatment of aortic coarctation (CoA). BACKGROUND: BeGraft Aortic stent (Bentley InnoMed, Hechingen, Germany) allows large postdilation diameter up to 30 mm. With availability of lengths of 19-59 mm and lower stent profile, they can be used in native and recurrent CoA in adults and in pediatric patients. MATERIALS AND METHODS: This is a multicentre retrospective analysis of 12 implanted BeGraft Aortic stents in CoA between May 2017 and April 2019. RESULTS: Twelve patients aged 7.7-38 years (median 18.3 years) with body weight of 19.9-56 kg (median 45.5 kg). Eight patients (66%) had native juxtaductal CoA while four had recurrent CoA after previous surgical or transcatheter treatments. The stents were implanted successfully in all the patients with no serious adverse events. The length of the stents ranged from 27 to 59 mm and the implanted stent diameter varied from 12 to 18 mm. The median intraprocedural CoA pressure gradient decreased from 25 mmHg (range 16-66 mmHg) to 2 mmHg (range 0-13 mmHg). The mean follow-up duration was 10.2 months. Two patient (16.6%) had residual stent narrowing requiring staged redilation. One patient (8%) had pseudoaneurysm formation at 1 year cardiac CT follow-up. CONCLUSIONS: The BeGraft Aortic stent may be considered to be safe and effective in the short term in treatment of CoA from childhood to adulthood. Long-term follow-up is needed.

Early clinical experience with the straight design of Venus P-valve™ in dysfunctional right ventricular outflow tracts.

OBJECTIVES: To assess the initial procedural and short to medium-term experience with the straight design of the Venus P-valve™ (Venus MedTech, Hangzhou, China) in dysfunctional right ventricular outflow tracts (RVOT). BACKGROUND: The Melody™ valve (Medtronic, Minneapolis, Minnesota) has been the only percutaneous valve option for smaller RVOT conduits. The straight Venus P-valve™ may provide an alternative to the Melody™ valve. METHODS: Retrospective data collection of patient characteristics, procedural data, clinical and imaging follow-up of the straight Venus P-valve™. RESULTS: Nine patients (four female) with a mean age of 23.1 ± 7.5 years and a mean weight of 72.7 ± 29.4 kg underwent straight Venus P-valve™ implantation between 03/2014 and 06/2016. All patients had right ventricle-to-pulmonary artery conduits which were pre-stented before the valve implantation. All valves were deployed successfully without any significant procedural complications. During the mean follow-up of 24 ± 9.1 months, there were no valve related re-interventions or deterioration in valve performance. There was one case of insignificant, single wire frame fracture and no cases of endocarditis. The cohort demonstrated a reduction in pulmonary regurgitation and tricuspid regurgitation, which was sustained throughout the follow-up. Similarly the gradient across the RVOT tract did not significantly increase. CONCLUSIONS: Implantation of the straight Venus P-valve™ has provided satisfactory short to mid-term results with high success rates and no complications and may be considered as an alternative option in patients with RVOT dysfunction.

Feasibility and 1-year outcome of transcatheter closure of perimembranous ventricular septal defects with different devices.

OBJECTIVE: To analyze feasibility of closing perimembranous ventricular septal defect (pmVSD) with different devices by percutaneous approach and determining initial 1-year outcome of the procedure. BACKGROUND: Transcatheter closure of pmVSD remains controversial due to a previous higher incidence of complete heart block (CHB), especially with the Amplatzer pmVSD occluder. Recently, several devices have been used to minimize the procedure-related complications. METHODS AND MATERIALS: A retrospective longitudinal cohort study of 133 patients who underwent transcatheter closure of pmVSD from September 2009 to March 2015. The median age and weight at intervention were 7.1 years (ranging from 9 months to 28 years) and 21.2 kg (ranging from 6.4 to 93 kg). RESULTS: Transcatheter pmVSD closure was successfully performed in 129 cases (97%) using 13 different devices. There were two new onset severe aortic regurgitation (AR) (1.5%), one new onset severe tricuspid regurgitation (0.7%), and one CHB (0.7%). Immediately after procedure, 41 patients (30.8%) had mild to moderate residual shunt and 27 patients (20.8%) had trivial to mild AR. At 1 year, there was no CHB and 10 patients (9.6%) had tiny to mild residual shunt and 10 patients (9.6%) had trivial AR. CONCLUSION: With proper case selection, good expertise, and judicious use of various devices with respect to anatomic details of pmVSD, transcatheter closure is feasible with satisfactory early outcome.

Medium-term results of percutaneous pulmonary valve implantation using the Venus P-valve: international experience.

AIMS: The aim of this study was to assess the international procedural and short-term to midterm experience with the new percutaneous Venus P-valve. METHODS AND RESULTS: Retrospective data of patient characteristics, clinical and imaging follow-up of Venus P-valve implantation outside China were collected. Thirty-eight patients underwent attempted Venus P-valve implantation between October 2013 and April 2017. Thirty-seven valves were successfully implanted during 38 procedures. There was one unsuccessful attempt and there were two valve migrations, one of which required surgical repositioning. The mean follow-up was 25 months with no short-term or midterm valve failure or deterioration in performance. Frame fractures occurred in 27% of patients. The cohort demonstrated a statistically significant reduction in pulmonary regurgitation fraction and indexed right ventricular diastolic volumes at six and 12 months. CONCLUSIONS: Implantation of the Venus P-valve has provided satisfactory short-term to midterm results with high success and low complication rates in an inherently challenging patient substrate.

Percutaneous pulmonary valve implantation with the Venus P-valve: clinical experience and early results.

BACKGROUND: At present, the exclusion for percutaneous pulmonary valve implantation for free pulmonary regurgitation after tetralogy of Fallot repair includes an unfavourably large right ventricular outflow tract. OBJECTIVE: To report feasibility and early experience with a recently developed transcatheter heart valve, Venus P-valveTM, implanted in six patients with severe pulmonary regurgitation with large right ventricular outflow tracts. Patients There were two female patients and four male patients. The median age of the patients was 18.5 years, and the mean body weight was 53.8 kg. All the patients were in NYHA class II and had severe pulmonary regurgitation after previous transannular patch repair of tetralogy of Fallot. The median time after the last surgical operation was 13.5 years. RESULTS: The Venus P-valveTM was successfully implanted in all the patients with implanted valve diameters ranging from 24 to 32 mm. The mean fluoroscopy time was 29.8 minutes. None of the patients had significant outflow tract gradient or pulmonary regurgitation immediately after valve implantation. Only one patient had unexpected mild proximal valve migration to the right ventricular body during withdrawal of the delivery system. It caused mild paravalvar leak and significant tricuspid regurgitation. At 6 months follow-up, the median of right ventricular end-diastolic volume indices decreased from 146 to 108 ml/m2 (p-value=0.046). The Doppler systolic peak gradient across the valve ranged from 4 to 40 mmHg, and there was no evidence of stent fracture on fluoroscopy or structural valve failure. CONCLUSION: The Venus P-valveTM can be implanted successfully and effectively in patients with severe pulmonary regurgitation and a large right ventricular outflow tract. The early results with this valve are encouraging.

Progressive aneurysmal dilation of coronary arterial fistula after transcatheter closure: successfully treated by a second occlusion device.

We report on a 6-year-old boy with a huge right coronary artery to the right ventricle fistula, who had previously been treated by device closure at the right ventricular exit point. However, 3 years later, the right coronary artery aneurysm showed progressively dilation and compressed the right ventricle. To prevent further complications related to the aneurysm, the proximal part of the aneurysm was successfully occluded by a vascular plug.

Survival analysis of Down syndrome with congenital heart disease: a 5-years registry at QSNICH.

BACKGROUND: Down syndrome (DS) is the most common chromosomal abnormality in children. Atrio-ventricular septal defect (AVSD) is the most common congenital heart disease (CHD) reported in DS. The mortality rate of DS with congenital heart disease (CHD) is 5-7 times higher than normal population. The survival rate in DS has improved with time and has reported up to 91% and 85% at one and ten years of age, respectively. OBJECTIVE: To study the prevalence of CHD, clinical course, treatment, the overall survival in patients with DS compare with those who are associated with CHD. STUDY DESIGNS: DS registry, multidisciplinary approach, single centre. MATERIAL AND METHOD: All DS patients at QSNICH with parental signed consent were enrolled in the registry. The study was conducted for 5 years starting from May 2007 to April 2012. All patients were followed-up according to schedule modified from American Academic of Pediatrics (AAP) health supervision guideline. Standard treatment was given to all those children with diagnosis of CHD and/or other associated diseases. RESULTS: Four hundred and two cases of DS were enrolled. Two cases were excluded due to the parental inconvenience. The mode and mean age of the patients at registration were 1 and 7 months (1-62). Two hundred and seventy-one cases had an initial echocardiographic diagnosis of CHD, which included 91 of Patent ductus arteriosus (PDA), 49 of Ventricular septal defect (VSD), 34 of AVSD, 34 of secondum Atrial septal defect (ASD), 6 of Tetralogy of Fallot (TOF), 2 of Coractation of Aorta (CoA), 11 of other CHD and 44 of combined lesions. During the follow-up period, spontaneous closure of PDA, VSD and ASD occurred in 46, 12 and 15 cases, respectively. After the exclusion of those who had spontaneous closure, the prevalence rate of CHD in DS was 49.8%. VSD was the most common lesion, slightly more than PDA. Ninety-eight cases underwent cardiac surgery; including 39 of associated VSD, 24 of AVSD, 26 of PDA, 3 of TOF, 4 of CoA/AA repaired, one each of Cor triatriatum and primum ASD. Twelve cases had catheter interventions, including PDA occlusion (10 cases), and 1 case of balloon dilatation of re-CoA and coils embolization of isolated major aorto-pulmonary collateral artery. Seven cases were lost at follow-up. By using the hospital information and National Health Security Office (NHSO) database, 47 infants died during the follow-up period. The causes of death were immediate post cardiac surgery in 10 cases (AVSD in 9, VSD in 1) and not associated with surgery in 37 cases including CHF in 8, pneumonia in 7, leukemia in 4, airway disease in 3, others 7 and unknown 8 cases. The overall survival at 1 and 5 years of age were 96% and 86%, respectively. DS with CHD had a significantly lower survival rate than those without CHD (p < 0.001). CONCLUSION: According to this study, the prevalence rate of CHD in DS was high. VSD was the most common lesion and has better prognosis than AVSD. The main cause of death was a cardiac problem. Follow-up patients by using modified AAP guideline and standard treatment can improve their overall survival.

An unusual cause of progressive cyanosis post Fontan operation: congenital extra-hepatic porto-systemic shunt.

OBJECTIVE: To report an unusual case of progressive cyanosis post Fontan operation due to porto-systemic venous shunt and the result of its treatment. MATERIAL AND METHOD: A patient with diagnosis of progressive cyanosis post Fontan operation from porto-systemic venous shunt at QSNICH RESULTS: This is a case of twelve years old girl, who had diagnosis of situs solitus, levocardia, atrio-ventricular concordant, ventriculo-arterial concordant, hypoplastic right ventricle with large ventricular septal defect. She had pulmonary artery banding at 4 months of age followed by a non-fenestrated extra-cardiac conduit Fontan performed at 7 years and 7 months of age. During the first year of post operation, her systemic oxygen saturation (SpO2) was 93-94% after which it decreased to 87%, 84%, 75% at 1.5, 2.5 and 3 years after surgery, respectively. Clinically she also had progressive dyspnea on exertion. Diffuse pulmonary arterio-venous malformation was demonstrated by contrast echocardiogram during cardiac catheterization. Cardiac magnetic resonance angiography showed abnormal extra-hepatic portal vein to inferior vena cava shunt. After balloon test occlusion in the cath lab, which showed no change in the portal venous pressure, complete occlusion of this porto-systemic venous shunt was performed by using Amplatzer Vascular Plug II. Her systemic oxygen saturation increased to 83% with functional class I at one-year post occlusion. CONCLUSION: The present report an unusual case of progressive cyanosis post Fontan operation due to pulmonary arteriovenous malformation, which was secondary to congenital extra-hepatic porto-systemic shunt. The venous blood from the intestinal and splenic veins was partially bypassing the liver into inferior vena cava. The patient's clinical condition and SpO2 improved after transcatheter occlusion of the shunt with the device.

Outcomes of straightforward extracardiac Fontan operation in advanced-age group.

INTRODUCTION: Fontan operation is a palliative procedure used in patients with univentricular heart (UVH). There have been numerous modifications to the technique. The appropriate strategy for treating patients with UVH in Thailand is still a major concern. OBJECTIVE: To describe the outcomes of patients after straightforward Fontan operation at advanced-age and evaluate their post-surgical cardiac performance. MATERIAL AND METHOD: A cross sectional study was performed in post-Fontan operation patients at Rajvithi Hospital between 2000 and 2009. Post-operative cardiac performance was analyzed using the echocardiogram and treadmill exercise stress test (EST). RESULTS: Thirty-nine patients were enrolled in the present study. The mean age was 11.7 years and the mean age at operation was 7.4 years. The predominant systemic chamber was morphologic left ventricle. Straightforward Fontan operation was performed in 60% of cases. Mean of Fontan index, mean of McGoon ratio, and mean of Nakata index were 2.63, 2.32, and 414.15 sqmm/sqm, respectively. Mean of the PVR and PAP were 1.98 U/m2 and 11.05 mmHg. Eighteen percent of patients died in the early post-operative period. Most of the patients died from septicemia. The mean EFwas 58.43%. Systolic dysfunction in 17.9% of the cases was abnormal, whereas diastolic dysfunction was present in half of the cases. Thirty-nine percent had MPI abnormality. During EST three cases developed arrhythmias and 3 cases had hypotension. Patients were categorized into 3 groups (Group 1: normal systolic and diastolic functions, Group 2: diastolic dysfunction, Group 3: impairment of both systole and diastole). Pre-operative cardiac catheterization parameters, surgical data and cardiac performance showed no statistical significance. However, there was a significant correlation between those with abnormal cardiac performance and arrhythmias or hypotension during EST (p = 0.003). CONCLUSION: The selection of suitable cases and good pre-operative evaluation could decrease the morbidity and mortality in patients undergoing the Fontan procedure. This study also found a correlation between abnormal cardiac performance and transient cardiac arrhythmia during exercise. The evaluation of cardiac performance and EST remains to be performed for following-up of patients who have undergone the Fontan operation, even for the asymptomatic cases.