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.

Transcatheter versus surgical treatment for isolated superior sinus venosus atrial septal defect.

BACKGROUND: The superior sinus venosus atrial septal defect is a congenital communication between the left and right atria. Open surgical approach by patch closure has historically been the only treatment option. Recently, a transcatheter approach has been developed. This study aims to compare the efficacy and safety of surgical and transcatheter approach in treatment of sinus venosus atrial septal defect. METHODS: Between March 2010 and December 2020, 58 patients (median age: 45.4, range 14.8-73.8) underwent either surgical or transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage. RESULTS: Twenty-four patients (median age: 35.4, range 14.8-66.8) underwent surgery while 34 patients (median age: 46.8, range 15.5-73.8) had a transcatheter treatment. During the catheterization era, 41 patients was considered suitable for a transcatheter closure. In 5 patients, surgery was the patient's or referring physician's choice. In 2 cases, the procedure was unsuccessful; the remaining 34 were successfully closed (94.4% of cases). Intensive care unit stay (median of 1 day, range 0.5-4, vs. 0, range 0-2, p < 0.0001) and hospital stay (median 7 days, range 2-15 vs. 2 days, range 1-12, p < 0.0001), were significantly longer in the surgery group. Total early complication rate, consisted on procedural and in-hospital complication, were higher in the surgical group (62.5% vs. 23.5%; p = 0.005). However, complications in both groups were clinically mild. At follow-up, a small residual shunt was present in 6 patients (surgery group: 2 pts; catheterization group: 4 pts; p: NS). Imaging studies showed significant improvement of right ventricular size and unobstructed pulmonary venous return in all patients. No late complications occurred at follow-up. CONCLUSIONS: Transcatheter correction of sinus venosus atrial septal defect is effective and safe in selected patients and may be considered as a valid alternative to surgery.

Sinus node dysfunction during transcatheter assessment and stent correction of sinus venosus atrial septal defects.

Covered stent correction of a superior sinus venosus atrial septal defect is increasingly performed as an alternative to surgical repair. While sinus node dysfunction requiring pacemaker implantation may be required after surgical repair, this has not been previously reported after covered stent implantation. We reviewed the experience in two interventional centers. Balloon inflation in the superior vena cava was used to confirm the anomalous pulmonary vein drainage would be unobstructed after stent implantation. During balloon testing in 62 consecutive patients, we assessed gradients across the pulmonary vein to left atrium while monitoring the rhythm. We observed the outcomes after covered stent correction in 51 patients. In a single patient, significant bradycardia and pauses developed on repeat balloon testing and the procedure was abandoned without stent implantation. In another patient, there was no sign of sinus node dysfunction during balloon testing but several hours after stent implantation, the patient became symptomatic from sinus bradycardia and pauses and had a pacemaker implanted 3 days later. Over a year later there are some signs of improvement in sinus node function. While sinus node dysfunction has not been described previously during balloon testing or after stent implantation, this report demonstrates for the first time that it may occur. Larger registries are therefore required to monitor for this uncommon complication.

Invasive Hemodynamic Evaluation of the Fontan Circulation: Current Day Practice and Limitations.

PURPOSE OF REVIEW: Establishing the Fontan circulation has led to improved survival in patients born with complex congenital heart diseases. Despite early success, the long-term course of Fontan patients is complicated by multi-organ dysfunction, mainly due to a combination of low resting and blunted exercise-augmented cardiac output as well as elevated central venous (Fontan) pressure. Similarly, despite absolute hemodynamic differences compared to the normal population with biventricular circulation, the "normal" ranges of hemodynamic parameters specific to age-appropriate Fontan circulation have not been well defined. With the ever-increasing population of patients requiring Fontan correction, it is of utmost importance that an acceptable range of hemodynamics in this highly complex patient cohort is better defined. RECENT FINDINGS: Multiple publications have described hemodynamic limitations and potential management options in patients with Fontan circulation; however, an acceptable range of hemodynamic parameters in this patient population has not been well defined. Identification of "normal" hemodynamic parameters among patients with Fontan circulation will allow physicians to more objectively define indications for intervention, which is a necessary first step to eliminate institutional and regional heterogeneity in Fontan management and potentially improve long-term clinical outcomes.

Correction of sinus venosus atrial septal defects with the 10 zig covered Cheatham-platinum stent - An international registry.

BACKGROUND: Covered stent correction of sinus venosus ASDs (SVASD) is a relatively new technique. Challenges include anchoring a sufficiently long stent in a nonstenotic superior vena cava (SVC) and expanding the stent at the wider SVC-RA junction without obstructing the anomalous right upper pulmonary vein (RUPV). The 10-zig covered Cheatham-platinum (CCP) stent has the advantage of being available in lengths of 5-11 cm and dilatable to 34 mm in diameter. METHODS: An international registry reviewed the outcomes of 10-zig CCP stents in 75 patients aged 11.4-75.9 years (median 45.4) from March 2016. Additional stents were used to anchor the stent in the SVC or close residual shunts in 33/75. An additional stent was placed in 4/5 (80%) with 5/5.5 cm CCPs, 18/29 (62%) with 6 cm CCPs, 5/18 (28%) with 7 cm CCPs, 5/22 (23%) with 7.5/8 cm CCPs and 0/1 with an 11 cm CCP. A "protective" balloon catheter was inflated in the RUPV in 17. RESULTS: Early stent embolization in two patients required surgical removal and defect repair and tamponade was drained in one patient. The CT at 3 months showed occlusion of the RUPV in one patient. Follow up is from 2 months to 5.1 years (median 1.8 years). QP:QS has reduced from 2.5 ± 0.5 to 1.2 ± 0.36 (p < .001) and RVEDVi from 149.1 ± 35.4 to 95.6 ± 21.43 ml/m2 (p < .001). CONCLUSIONS: Ten-zig CCPs of 7-8 cm appear to provide reliable SVASD closure with a low requirement for additional stents. Careful selection of patients and meticulous attention to detail is required to avoid complications.

Intravascular Ultrasound for Assessment of Residual Coarctation of the Aorta after Balloon Angioplasty in Infants.

Intravascular ultrasound (IVUS) has been introduced as an accurate and minimally invasive diagnostic technique for the assessment of vascular anatomy and its abnormalities. We believe that IVUS can be used for clarifying the reasons for failure of balloon angiography in infantile coarctation of the aorta (CoA), because post-balloon angioplasty tearing, intimal flap, thrombosis and pseudoaneurysm of the aorta can be evaluated by IVUS with greater sensitivity and specificity. We aimed to assess the outcome of balloon angioplasty of CoA using angiography as the gold standard and IVUS as a new method in infants, comparing the two techniques for the evaluation of the diameter and area of CoA segment pre- and post-procedure. This cross-sectional study was performed on 18 infants hospitalized with a final diagnosis of CoA. All the infants underwent angiography and were also assessed by IVUS to measure the preoperative and postoperative diameter of the narrow segment in the two anterior-posterior and lateral views. In assessment by IVUS, the mean diameter of the coarctation site increased from 2.10 ± 0.30 mm to 4.50 ± 0.94 mm (P < 0.001). Similarly, the average minimum area of the coarctation level increased from 5.26 ± 1.50 mm2 to 13.77 ± 3.48 mm2 after angioplasty (P < 0.001). Comparing these findings, angiography and IVUS showed a high level of agreement. In the assessment of a dissection flap, there was a high level of agreement between angioplasty and IVUS before the procedure, but IVUS had higher accuracy after the procedure. Our study showed that IVUS was more reliable than angiography in the assessment of residual coarctation. IVUS yielded high sensitivity (58.3%) and specificity (100%) for discriminating the presence and absence of residual coarctation as well as the need for repeating the procedure. The assessment of coarctation before and after angioplasty procedures in children is possible using the IVUS method, with high accuracy. IVUS can offer greater accuracy than angiography in the evaluation of the coarctation area, detecting tears, dissection and flaps, and assessment of residual coarctation.

Transcatheter creation of a pulmonary artery to left atrial fenestration in a failing Fontan circulation using the Atrial Flow Regulator (AFR).

Transcatheter creation of an interatrial communication using the Occlutech Atrial Flow Regulator Device for pulmonary hypertension or heart failure is well described. We report a case of an 8-year-old boy with a failing Fontan circulation, in whom the Atrial Flow Regulator was used to successfully create a fenestration between the pulmonary artery and left atrium, improving his clinical condition.

Initial experience with a novel ePTFE-covered balloon expandable stent in patients with near-atretic or severe aortic coarctation and small femoral arterial access.

OBJECTIVES: We report our experience of using the Bentley BeGraft Aortic stent in patients with severe or near-atretic aortic coarctation and small femoral arterial access. BACKGROUND: Use of covered stent is recommended in some settings such as aortic coarctation with associated aneurysm, Turner syndrome, and coarctation with aortic atresia. However, currently available covered stents need larger sheaths that may limit their use in children and patients with smaller arterial access. Newer stents may overcome this limitation. METHODS: Single-centre retrospective study of patients with severe or near-atretic aortic coarctation and small femoral arterial access. RESULTS: Between July and October, 2019, five patients (median age 15 years) with near-atretic or severe coarctation were treated with a Bentley BeGraft Aortic stent. Long sheaths between 9 and 11 Fr were used to implant stents, which were dilated up to 12-16 mm. None of the patients had residual coarctation (gradient >20 mm of mercury) after stenting. None of the patients developed acute vascular injuries or local access related complications at the end of the procedure or during follow-up (range 6-10 months). CONCLUSIONS: Bentley BeGraft aortic stents are important to consider in patients with severe coarctation and provided acute procedural success in patients with small femoral arterial access and widen the applicability in this patient population.

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.

The Future of Paediatric Heart Interventions: Where Will We Be in 2030?

PURPOSE OF REVIEW: Cardiac catheterization therapies to treat or palliate infants, children and adults with congenital heart disease have developed rapidly worldwide in both technical innovation and device development in the previous three decades. By reviewing of current status of novel or development of devices and techniques, we will discuss what is likely to happen in paediatric heart intervention in the next decade. RECENT FINDINGS: Recently, biodegradable stents and devices, transcatheter pulmonary valve implantation for the native right ventricle outflow tract and MRI-guided interventions have been progressing rapidly with good immediate to early results. These are expected to be introduced and spread in the next decade although there are still challenges to overcome. The future of paediatric heart intervention is very promising with rapid development of technological progress.

A low threshold for neonatal intervention yields a high rate of biventricular outcomes in pulmonary atresia with intact ventricular septum.

AIMS: Management strategies for pulmonary atresia with intact ventricular septum are variable and are based on right ventricular morphology and associated abnormalities. Catheter perforation of the pulmonary valve provides an alternative strategy to surgery in the neonatal period. We sought to assess the long-term outcome in terms of survival, re-intervention, and functional ventricular outcome in the setting of a 26-year single-centre experience of low threshold inclusion criteria for percutaneous valvotomy. METHODS AND RESULTS: Retrospective analysis of patients diagnosed with pulmonary atresia with intact ventricular septum from 1990 to 2016 at a tertiary referral centre, was performed. Of 71 patients, 48 were brought to the catheterisation laboratory for intervention. Catheter valvotomy was successful in 45 patients (94%). Twenty-three patients (51%) also underwent ductus arteriosus stenting. The length of intensive care and hospital stay was significantly shorter, and early re-interventions were significantly reduced in the catheterisation group. There were eight deaths (17%); all within 35 days of the procedure. Of the survivors, only one has required a Fontan circulation. Twenty-eight patients (74%) have undergone biventricular repair and nine patients (24%) have one-and-a-half ventricle circulation. Following successful valvotomy, 80% of patients required further catheter-based or surgical interventions. CONCLUSIONS: A low threshold for initial interventional management yielded a high rate of successful biventricular circulations. Although mortality was low in patients who survived the peri-procedural period, the rate of re-intervention remained high in all groups.

Radiation protection knowledge and practices in interventional cardiologists practicing in Africa: a cross sectional survey.

We conducted a survey of doctors working in the cardiac catheterisation laboratories in Africa on their knowledge, attitude and practice with respect to radiation protection. Of seventy-two respondents contacted, 61 (84.7%) completed the questionnaire. Twenty-eight, (45.9%) were younger than 45 years. Thirty-seven, (60.6%) had less than 10 years of experience in the laboratory. Only 28 (45.9%) had undertaken radiation protection training. Fifty-eight, (95.1%) consistently used lead aprons. Forty-seven, (77%) reported consistently using thyroid shields. Ten (16.4%) consistently used radiation protection eyeglasses, whilst 36 (59%) never used them. Thermoluminescent Dosimeter badges were consistently used in 23 (37.7%). Forty-two, (68.9%) reported having ceiling mounted lead/acrylic shields. Level of radiation exposure in the most recent one year was ≤2 mSv in 14, between 2 and 20 mSv in 8 and between 20 and 30 mSv in 2, whilst 33 did not know their dose readings. The use of basic radiation protection tools as well as the knowledge and measurement of radiation exposure among interventional cardiologists working in Africa is low. The unavailability of some of the protective tools and a knowledge gap in terms of radiation protection and monitoring of self-exposure were some of the reasons for suboptimal self-protection against ionising radiation among our respondents. We suggest that initiatives be taken by all stakeholders to train this group of medical professionals in basic radiation protection to avoid unnecessary exposure to themselves, co-workers and patients.

Novel use of a 3D printed heart model to guide simultaneous percutaneous repair of severe pulmonary regurgitation and right ventricular outflow tract aneurysm.

We describe percutaneous repair of severe pulmonary regurgitation and a right ventricular outflow tract pseudoaneurysm in a 19-year-old patient after repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. A 3D printed model of his heart was used to simulate percutaneous repair with a closure device in the aneurysm neck and a Venus P-valve in the right ventricular outflow tract. The encouraging findings from the simulation allowed us to plan the complex procedure effectively with a successful outcome and avoidance of surgery.

Molding the shape of congenial and structural interventional cardiology: interviews with directors of major congresses.

The range and number of educational and networking events that are available for fellows, trainees, and junior faculty to attend grows every year. Each meeting useful in its own way; each adding value to the development and the growth of an interventionist. Within paediatric, congenital, and structural heart disease, three of the standout meetings are: Pediatric and Interventional Cardiac Symposium (PICS-AICS), Congenital and Structural Interventions (CSI), and International Workshop on Interventional Pediatric and Adult Congenital Cardiology (IPC). All of these were started by leaders in our field; people known to be passionate educators and innovators. International congresses focusing more broadly on congenital cardiac disease in children and adults are rare. These forums allow more interdisciplinary discussions between the interventionist, surgeon, and non-invasive specialists. Purely interventional meetings are essential to allow colleagues to debate and explore the nuances and intricacies of technique and approach, developing concepts to be challenged in wider forums. During the recent 21st PICS-AICS meeting Prof. Ziyad M. Hijazi, Shakeel A. Qureshi, Mario Carminati, and Dr Damien Kenny shared their time to engage in frank, recorded conversations which provide a unique insight in to the process and concepts behind three of our most important educational congresses.

Stenting of the inter-atrial septum in infants and small children: Indications, techniques and outcomes.

OBJECTIVES: To evaluate the procedural success and outcome of inter-atrial stenting. BACKGROUND: Inter-atrial stenting has been shown to be an effective way to maintain inter-atrial blood flow, however it is considered a high risk procedure, usually performed urgently in patients with significant hemodynamic compromise. METHODS: Between September 2004 and August 2016, inter-atrial stenting was attempted in 29 children. Procedural, clinical, and follow-up data were collected retrospectively. RESULTS: The procedures were completed successfully in 27 patients. Twenty-five procedures were undertaken percutaneously, with the remaining four being performed as hybrid procedures. The patients were considered as high risk for adverse events (82% scored as CRISP 4 and 5) with four deaths during the first 24 hr (14%). Procedural complications occurred in eight patients (28%) with related death in three patients (10%). One further patient died after an uncomplicated technically successful stent implantation performed as a salvage procedure. Procedural complications (71% vs. 14%) and mortality (43% vs. 5%) were higher in those, who weighed 3 kg or less (P < 0.05). Patency of the stents was maintained until planned staged surgery in 22 patients at a mean of 302 days. Three patients underwent further balloon dilation for flow restriction at 58-201 days. In two un-operated patients the stents remained patent at follow-up. One patient with severe pulmonary hypertension died with a patent stent. CONCLUSIONS: Inter-atrial stenting produces reliable patency with a very good success rate. Morbidity and mortality were related to low weight at the time of the procedure.

Catheter, MRI and CT Imaging in Newborns with Pulmonary Atresia with Ventricular Septal Defect and Aortopulmonary Collaterals: Quantifying the Risks of Radiation Dose and Anaesthetic Time.

A comprehensive understanding of the native pulmonary blood supply is crucial in newborns with pulmonary atresia with ventricular septal defect and aortopulmonary collaterals (PA/VSD/MAPCA). We sought to describe the accuracy in terms of identifying native pulmonary arteries, radiation dose and anaesthetic time associated with multi-modality imaging in these patients, prior to their first therapeutic intervention. Furthermore, we wanted to evaluate the cumulative radiations dose and anaesthetic time over the study period. Patients with PA/VSD/MAPCA diagnosed at < 100 days between 2004 and 2014 were identified. Cumulative radiation dose and anaesthetic times were calculated, with imaging results compared with intraoperative findings. We then calculated the cumulative risks to date for all surviving children. Of 19 eligible patients, 2 had echocardiography only prior to first intervention. The remaining 17 patients underwent 13 MRIs, 4 CT scans and 13 cardiac catheterization procedures. The mean radiation dose was 169 mGy cm2 (47-461 mGy cm2), and mean anaesthetic time was 111 min (33-185 min). 3 children had MRI only with no radiation exposure, and one child had CT only with no anaesthetic. Early cross-sectional imaging allowed for delayed catheterisation, but without significantly reducing radiation burden or anaesthetic time. The maximum cumulative radiation dose was 8022 mGy cm2 in a 6-year-old patient and 1263 min of anaesthetic at 5 years. There is the potential to generate very high radiation doses and anaesthetic times from diagnostic imaging alone in these patients. As survival continues to improve in many congenital heart defects, the important risks of serial diagnostic imaging must be considered when planning long-term management.

Results of balloon pulmonary valvoplasty in children with Noonan's syndrome.

Pulmonary valve stenosis is common in patients with Noonan's syndrome. The response to balloon valvoplasty varies.We assessed the correlation between re-intervention rate, immediate response, and the progress of the valve gradient over time after intervention. METHODS: This is a retrospective study conducted from 1995 to 2014. RESULTS: Of 14 patients identified, seven had re-intervention 28±54 months (range 3-149, median 3.3) after valvoplasty. These patients did not have a significant decrease in gradient after intervention. Their gradient subsequently decreased during follow-up and then became static before increasing years after intervention. In contrast, the gradient of patients not requiring further intervention continually reduced over time. Demographics did not differ between these groups. CONCLUSION: We could not identify predisposing factors for long-term success of pulmonary valvoplasty in Noonan's patients, but the trajectory of gradients differs significantly between patients needing re-intervention from those who remain free from re-intervention.

Percutaneous Device Closure of Paravalvular Leak: Combined Experience From the United Kingdom and Ireland.

BACKGROUND: Paravalvular leak (PVL) occurs in 5% to 17% of patients following surgical valve replacement. Percutaneous device closure represents an alternative to repeat surgery. METHODS: All UK and Ireland centers undertaking percutaneous PVL closure submitted data to the UK PVL Registry. Data were analyzed for association with death and major adverse cardiovascular events (MACE) at follow-up. RESULTS: Three hundred eight PVL closure procedures were attempted in 259 patients in 20 centers (2004-2015). Patient age was 67±13 years; 28% were female. The main indications for closure were heart failure (80%) and hemolysis (16%). Devices were successfully implanted in 91% of patients, via radial (7%), femoral arterial (52%), femoral venous (33%), and apical (7%) approaches. Nineteen percent of patients required repeat procedures. The target valve was mitral (44%), aortic (48%), both (2%), pulmonic (0.4%), or transcatheter aortic valve replacement (5%). Preprocedural leak was severe (61%), moderate (34%), or mild (5.7%) and was multiple in 37%. PVL improved postprocedure (P<0.001) and was none (33.3%), mild (41.4%), moderate (18.6%), or severe (6.7%) at last follow-up. Mean New York Heart Association class improved from 2.7±0.8 preprocedure to 1.6±0.8 (P<0.001) after a median follow-up of 110 (7-452) days. Hospital mortality was 2.9% (elective), 6.8% (in-hospital urgent), and 50% (emergency) (P<0.001). MACE during follow-up included death (16%), valve surgery (6%), late device embolization (0.4%), and new hemolysis requiring transfusion (1.6%). Mitral PVL was associated with higher MACE (hazard ratio [HR], 1.83; P=0.011). Factors independently associated with death were the degree of persisting leak (HR, 2.87; P=0.037), New York Heart Association class (HR, 2.00; P=0.015) at follow-up and baseline creatinine (HR, 8.19; P=0.001). The only factor independently associated with MACE was the degree of persisting leak at follow-up (HR, 3.01; P=0.002). CONCLUSION: Percutaneous closure of PVL is an effective procedure that improves PVL severity and symptoms. Severity of persisting leak at follow-up is independently associated with both MACE and death. Percutaneous closure should be considered as an alternative to repeat surgery.