Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.

BACKGROUND AND AIMS: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI. METHODS: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries. RESULTS: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively. CONCLUSIONS: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs.

Mind the gap-missing device on the shelf? Retrospective experience with 5/7 Occlutech duct Occluder.

AIM: To describe our initial experience with the indications and results of the 5/7 Occlutech® duct Occluder (ODO, Occlutech International AB, Helsingborg, Sweden). A small incremental increase in occluder sizes is of utmost importance for successful outcomes, especially in smaller patients in whom protrusion of the distal disk towards the aorta should be minimised. METHODS: Retrospective study of all patients undergoing PDA closure with the 5/7 ODO in three institutions since 2018. RESULTS: The 5/7 ODO was used in 18 patients with median age and weight at the time of the procedure of 17.5 months (interquartile range 25th to 75th percentile 8 months- 4.4 years) and 13.6 kg (interquartile range 25th to 75th percentile 6.4-22.5 kg) respectively. All cases were successful. There were no cases of device embolisation, haemolysis, or flow disturbance of the LPA or the aorta. CONCLUSIONS: This small retrospective study demonstrated an excellent outcome of transcatheter PDA closure with the 5/7 ODO. The device is a beneficial complement to the existing sizes of PDA devices, filling the gap between the 4/6 and 6/8 ODO and avoiding protrusion of a larger disk in the aortic isthmus.

Transcatheter correction of sinus venosus defect in a patient with a challenging anatomical configuration: From bench testing to clinical success.

We report successful transcatheter correction of a sinus venosus defect in a 72-year-old woman with anomalous pulmonary venous return in a challenging anatomical configuration. The procedure was facilitated by hands-on simulation training on a newly developed, perfused, 3D-printed model.

Discordance between 2D and 4D flow in the assessment of pulmonary regurgitation severity: a right ventricular remodeling follow-up study.

OBJECTIVES: Pulmonary regurgitation (PR) is common in adult congenital heart disease (ACHD). 2D phase contrast MRI is the reference method for the quantification of PR and helps in the decision of pulmonary valve replacement (PVR). 4D flow MRI can be an alternative method to estimate PR but more validation is still needed. Our purpose was to compare 2D and 4D flow in PR quantification using the degree of right ventricular remodeling after PVR as the reference standard. METHODS: In 30 adult patients with a pulmonary valve disease recruited between 2015 and 2018, PR was assessed using both 2D and 4D flow. Based on the clinical standard of care, 22 underwent PVR. The pre PVR estimate of PR was compared using the post-operative decrease in right ventricle end-diastolic volume on follow-up exam as reference. RESULTS: In the overall cohort, regurgitant volume (Rvol) and regurgitant fraction (RF) of PR measured by 2D and 4D flow were well correlated but with moderate agreement in the overall cohort (r = 0.90, mean diff. -14 ± 12.5 mL; and r = 0.72, mean diff. -15 ± 13%; all p < 0.0001). Correlations between Rvol estimates and right ventricle end-diastolic volume decrease after PVR was higher with 4D flow (r = 0.80, p < 0.0001) than with 2D flow (r = 0.72, p < 0.0001). CONCLUSIONS: In ACHD, PR quantification from 4D flow better predicts post-PVR right ventricle remodeling than that from 2D flow. Further studies are needed to evaluate the added value of this 4D flow quantification for guiding replacement decision. KEY POINTS: • Using 4D flow MRI allows a better quantification of pulmonary regurgitation in adult congenital heart disease than 2D flow when taking right ventricle remodeling after pulmonary valve replacement as a reference. • A plane positioned perpendicular to the ejected flow volume as allowed by 4D flow provides better results to estimate pulmonary regurgitation.

Lympho-hematopoietic malignancies risk after exposure to low dose ionizing radiation during cardiac catheterization in childhood.

Pediatric patients with congenital heart disease (CHD) often undergo low dose ionizing radiation (LDIR) from cardiac catheterization (CC) for the diagnosis and/or treatment of their disease. Although radiation doses from a single CC are usually low, less is known about the long-term radiation associated cancer risks. We aimed to assess the risk of lympho-hematopoietic malignancies in pediatric CHD patients diagnosed or treated with CC. A French cohort of 17,104 children free of cancer who had undergone a first CC from 01/01/2000 to 31/12/2013, before the age of 16 was set up. The follow-up started at the date of the first recorded CC until the exit date, i.e., the date of death, the date of first cancer diagnosis, the date of the 18th birthday, or the 31/12/2015, whichever occurred first. Poisson regression was used to estimate the LDIR associated cancer risk. The median follow-up was 5.9 years, with 110,335 person-years. There were 22,227 CC procedures, yielding an individual active bone marrow (ABM) mean cumulative dose of 3.0 milligray (mGy). Thirty-eight incident lympho-hematopoietic malignancies were observed. When adjusting for attained age, gender and predisposing factors to cancer status, no increased risk was observed for lympho-hematopoietic malignancies RR/mGy = 1.00 (95% CI: 0.88; 1.10). In summary, the risk of lympho-hematopoietic malignancies and lymphoma was not associated to LDIR in pediatric patients with CHD who undergo CC. Further epidemiological studies with greater statistical power are needed to improve the assessment of the dose-risk relationship.

Risk factors for early occurrence of malnutrition in infants with severe congenital heart disease.

To identify the risk factors of early occurrence of malnutrition in infants with severe congenital heart disease (CHD) during their first year of life. Retrospective longitudinal multicenter study carried out from January 2014 to December 2020 in two tertiary care CHD centers. Four CHD hemodynamic groups were identified. Malnutrition was defined by a Waterlow score under 80% and/or underweight under -2 standard deviations. A total of 216 infants with a severe CHD, e.g., requiring cardiac surgery, cardiac catheterization, or hospitalization for heart failure during their first year of life, were included in the study. Malnutrition was observed among 43% of the cohort, with the highest prevalence in infants with increased pulmonary blood flow (71%) compared to the other hemodynamic groups (p < 0.001). In multivariate analysis, low birthweight (OR 0.62, 95% CI 0.44-0.89, p = 0.009), CHD with increased pulmonary blood flow (OR 4.80, 95% CI 1.42-16.20, p = 0.08), heart failure (OR 9.26, 95% CI 4.04-21.25, p < 0.001), and the number of hospitalizations (OR 1.35, 95% CI 1.08 l-1.69, p = 0.009) during the first year of life were associated with malnutrition (AUC 0.85, 95% CI 0.79-0.90).  Conclusions: In infants with a severe CHD, early occurrence of malnutrition during the first year of life affected a high proportion of subjects. CHD with increased pulmonary blood flow, low birthweight, heart failure, and repeated hospitalizations were risk factors for malnutrition. Further studies are required to identify optimal nutritional support in this population. What is Known: • Malnutrition is a known morbidity and mortality factor in children with severe congenital heart disease. What is New: • Early occurrence of malnutrition during the first year of life in infant severe congenital heart disease (CHD) was high (43%). • CHD with increased pulmonary blood flow, low birthweight, heart failure, and repeated hospitalizations during the first year of life were risk factors for malnutrition.

Three centers experience with device closure of congenital Gerbode-type perimembranous ventricular septal defects.

OBJECTIVES: We aim to evaluate our experience with interventional closure of Gerbode-type perimembranous ventricular septal defects (pmVSDs). METHODS: We performed three-center retrospective data review of patients with congenital indirect Gerbode-type pmVSDs treated percutaneously between August 2017 and May 2021. Standard safety and latest follow-up outcomes were assessed. RESULTS: Ten patients (six females) were identified with a median age of 6.8 years (range: 2.5-54) and a median weight of 26.5 kg (range: 12-88). The median left ventricular defect size was 10 mm (range: 3-15.5). On baseline ultrasound, 6 patients had absent subaortic rim , 6 patients had trivial aortic regurgitation, and 3 patients had tear-drop-type (small) aortic cusp prolapse. The tricuspid regurgitation was graded II (n = 5) and III (n = 5). Five Lifetech Konar-Multifunctional occluders, four Amplatzer duct occluders II and one Amplatzer duct occluder I were implanted. The median fluoroscopy time was 10.4 min (range: 4.3-20.2). Pre-existing aortic regurgitations remained identical. One new aortic regurgitation was identified before discharge and remained trivial after 48 months of follow-up. No heart block or tricuspid stenosis was observed on a median follow-up of 17 months (range: 3-48). All patients are symptom-free with complete shunt closure and significant regression or resolution of tricuspid regurgitation. CONCLUSIONS: Despite anatomical challenges, interventional closure of congenital indirect Gerbode-type pmVSD appears to be feasible, safe, and most importantly clinically effective using different commercially available devices. Amplatzer duct occluder II and Lifetech Konar-Multifunctional occluder offer interesting specifications to retrogradely target this specific defect with success.

Edwards SAPIEN XT transcatheter pulmonary valve implantation: 5-year follow-up in a French Registry.

OBJECTIVES: This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. BACKGROUND: The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. METHODS: From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. RESULTS: The primary efficacy outcome was met for 87.1% patients after a mean follow-up of 4.6 ± 1.8 years, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8-95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular regurgitation was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01-2.00%). At 5 years, freedom from infective endocarditis was 98.4% (95% CI 89.1-99.8%). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. CONCLUSIONS: This study documents the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance.

Feasibility and accuracy of printed models of complex cardiac defects in small infants from cardiac computed tomography.

BACKGROUND: Three-dimensional (3-D) printed models are increasingly used to enhance understanding of complex anatomy in congenital heart disease. OBJECTIVE: To assess feasibility and accuracy of 3-D printed models obtained from cardiac CT scans in young children with complex congenital heart diseases. MATERIALS AND METHODS: We included children with conotruncal heart anomalies who were younger than 2 years and had a cardiac CT scan in the course of their follow-up. We used cardiac CT scan datasets to generate 3-D models. To assess the models' accuracy, we compared four diameters for each child between the CT images and the printed models, including the largest diameters (Dmax) of ventricular septal defects and aortic annulus and their minimal diameters (Dmin). RESULTS: We obtained images from 14 children with a mean age of 5.5 months (range 1-24 months) and a mean weight of 6.7 kg (range 3.4-14.5 kg). We generated 3-D models for all children. Mean measurement difference between CT images and 3-D models was 0.13 mm for Dmin and 0.12 mm for Dmax for ventricular septal defect diameters, and it was 0.16 mm for Dmin and -0.13 mm for Dmax for aortic annulus diameter, indicating a non-clinically significant difference. CONCLUSION: Three-dimensional printed models could be feasibly generated from cardiac CT scans in a small pediatric population with complex congenital heart diseases. This technique is highly accurate and reliably reflects the same structural dimensions when compared to CT source images.

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. BACKGROUND: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. METHODS: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018­2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. CONCLUSIONS: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.

The usefulness of 3D printed heart models for medical student education in congenital heart disease.

BACKGROUND: Three-dimensional (3D) printing technology enables the translation of 2-dimensional (2D) medical imaging into a physical replica of a patient's individual anatomy and may enhance the understanding of congenital heart defects (CHD). We aimed to evaluate the usefulness of a spectrum of 3D-printed models in teaching CHD to medical students. RESULTS: We performed a prospective, randomized educational procedure to teach fifth year medical students four CHDs (atrial septal defect (ASD, n = 74), ventricular septal defect (VSD, n = 50), coarctation of aorta (CoA, n = 118) and tetralogy of Fallot (ToF, n = 105)). Students were randomized into printing groups or control groups. All students received the same 20 min lecture with projected digital 2D images. The printing groups also manipulated 3D printed models during the lecture. Both groups answered an objective survey (Multiple-choice questionnaire) twice, pre- and post-test, and completed a post-lecture subjective survey. Three hundred forty-seven students were included and both teaching groups for each CHD were comparable in age, sex and pre-test score. Overall, objective knowledge improved after the lecture and was higher in the printing group compared to the control group (16.3 ± 2.6 vs 14.8 ± 2.8 out of 20, p < 0.0001). Similar results were observed for each CHD (p = 0.0001 ASD group; p = 0.002 VSD group; p = 0.0005 CoA group; p = 0.003 ToF group). Students' opinion of their understanding of CHDs was higher in the printing group compared to the control group (respectively 4.2 ± 0.5 vs 3.8 ± 0.4 out of 5, p < 0.0001). CONCLUSION: The use of 3D printed models in CHD lectures improve both objective knowledge and learner satisfaction for medical students. The practice should be mainstreamed.

Usefulness of perioperative transoesophageal echocardiography during paediatric cardiac surgery.

BACKGROUND: Paediatric transoesophageal echocardiography probes allow perioperative evaluation during paediatric congenital heart disease surgery. AIM: To assess the usefulness of perioperative transoesophageal echocardiography in evaluating the severity of residual lesions, based on the type of congenital heart disease repaired in paediatric patients. METHODS: A retrospective analysis was conducted on paediatric patients who underwent open-heart surgery at our tertiary centre over a four-year period. Perioperative transoesophageal echocardiography studies were performed, and residual lesions were classified as mild, moderate or severe. RESULTS: Overall, 323 procedures involving 310 patients with a median age of 13.8 (0.07-214.4) months and a median weight of 8.2 (2-96) kg at intervention were enrolled in the study. Twenty-one (6.5%) residual lesions led to immediate reintervention: severe right ventricular outflow tract obstruction (n=12); severe aortic regurgitation (n=3); superior vena cava stenosis (n=2); moderate residual ventricular septal defect (n=2); severe mitral regurgitation (n=1); and severe mitral stenosis (n=1). Three (0.9%) neonates had ventilation difficulties caused by the transoesophageal echocardiography probe having to be removed, but experienced no sequelae. CONCLUSION: Perioperative transoesophageal echocardiography is a safe procedure, providing information on severe residual lesions, leading to the immediate revision of several paediatric congenital heart disease cases.

Efficacy of a Transition Program in Adolescents and Young Adults With Congenital Heart Disease: The TRANSITION-CHD Randomized Controlled Trial.

PURPOSE: Currently, nearly 90% of patients with congenital heart disease (CHD) reach adulthood in relatively good health. Structured transition programs have emerged to support adolescents and young adults in transitioning to adult care structures, improve their autonomy, and limit healthcare ruptures. The TRANSITION-CHD randomized controlled trial aimed to assess the impact of a transition program on health-related quality of life (HRQoL) in adolescents and young adults with CHD. METHODS: From January 2017 to February 2020, 200 subjects with a CHD, aged 13-25 years, were enrolled in a prospective, controlled, multicenter study and randomized in two balanced groups (transition program vs. standard of care). The primary outcome was the change in PedsQL self-reported HRQoL score between baseline and 12-month follow-up, using an intention-to-treat analysis. The secondary outcomes were the change in disease knowledge, physical health (cardiopulmonary fitness, physical activity), and mental health (anxiety, depression). RESULTS: The change in HRQoL differed significantly between the transition group and the control group (mean difference = 3.03, 95% confidence interval (CI) = [0.08; 5.98]; p = .044; effect size = 0.30), in favor of the intervention group. A significant increase was also observed in the self-reported psychosocial HRQoL (mean difference = 3.33, 95% CI = [0.01; 6.64]; p = .049; effect size = 0.29), in the proxy-reported physical HRQoL (mean difference = 9.18, 95% CI = [1.86; 16.51]; p = .015; effect size = 0.53), and in disease knowledge (mean difference = 3.13, 95% CI = [1.54; 4.72]; p < .001; effect size = 0.64). DISCUSSION: The TRANSITION-CHD program improved HRQoL and disease knowledge in adolescents and young adults with CHD, supporting the generalization and systematization of similar preventive interventions in pediatric and congenital cardiology.

Cardiac Drugs in ACHD Cardiovascular Medicine.

Adult congenital heart disease (ACHD) is a growing population that requires life-long care due to advances in pediatric care and surgical or catheter procedures. Despite this, drug therapy in ACHD remains largely empiric due to the lack of clinical data, and formalized guidelines on drug therapy are currently lacking. The aging ACHD population has led to an increase in late cardiovascular complications such as heart failure, arrhythmias, and pulmonary hypertension. Pharmacotherapy, with few exceptions, in ACHD is largely supportive, whereas significant structural abnormalities usually require interventional, surgical, or percutaneous treatment. Recent advances in ACHD have prolonged survival for these patients, but further research is needed to determine the most effective treatment options for these patients. A better understanding of the use of cardiac drugs in ACHD patients could lead to improved treatment outcomes and a better quality of life for these patients. This review aims to provide an overview of the current status of cardiac drugs in ACHD cardiovascular medicine, including the rationale, limited current evidence, and knowledge gaps in this growing area.

Long-term outcomes of transcatheter pulmonary valve implantation with melody and SAPIEN valves.

BACKGROUND: Transcatheter pulmonary valve implantation (TPVI) is effective for treating right ventricle outflow tract (RVOT) dysfunction. Factors associated with long-term valve durability remain to be investigated. METHODS: Consecutive patients successfully treated by TPVI with Melody valves (n = 32) and SAPIEN valves (n = 182) between 2008 and 2020 at a single tertiary centre were included prospectively and monitored. RESULTS: The 214 patients had a median age of 28 years (range, 10-81). The RVOT was a patched native pulmonary artery in 96 (44.8%) patients. Median follow-up was 2.8 years (range, 3 months-11.4 years). Secondary pulmonary valve replacement (sPVR) was performed in 23 cases (10.7%), due to stenosis (n = 22, 95.7%) or severe regurgitation (n = 1, 4.3%), yielding an incidence of 7.6/100 patient-years with melody valves and 1.3/100 patient-years with SAPIEN valves (P = 0.06). The 5- and 10-year sPVR-freedom rates were 78.1% and 50.4% with Melody vs. 94.3% and 82.2% with SAPIEN, respectively (P = 0.06). The incidence of infective endocarditis (IE) was 5.5/100 patient-years with Melody and 0.2/100 patient-years with SAPIEN (P < 0.0001). Factors associated with sPVR by univariate analysis were RV obstruction before TPVI (P = 0.04), transpulmonary maximal velocity > 2.7 m/s after TPVI (p = 0.0005), valve diameter ≤ 22 mm (P < 0.003), IE (P < 0.0001), and age < 25 years at TPVI (P = 0.04). By multivariate analysis adjusted for IE occurrence, transpulmonary maximal velocity remained associated with sPVR. CONCLUSIONS: TPVI is effective for treating RVOT dysfunction. Incidence of sPVR is higher in patients with residual RV obstruction or IE. IE add a substantial risk of TPVI graft failure and is mainly linked to the Melody valve. SOCIAL MEDIA ABSTRACT: Transcatheter pulmonary valve implantation is effective for treating right ventricular outflow tract dysfunction in patients with congenital heart diseases. Incidence of secondary valve replacement is higher in patients with residual obstruction or infective endocarditis.