Transcatheter Pulmonary Valve Implantation in Repaired Tetralogy of Fallot and Anomalous Left Coronary Artery Origin.

We describe a rare and extremely challenging case of transcatheter pulmonary valve implantation in repaired tetralogy of Fallot and anomalous origin of the left main coronary artery from the right coronary sinus. Procedural planning based on advanced multimodality imaging and 3-dimensional technology proved to be the key to procedural success.

Right disc thrombosis of the new Gore Cardioform ASD Occluder.

BACKGROUND: The GCA is a well performing device in terms of efficacy despite complex anatomies (aortic rim <5 mm and ASD diameter >17 mm) with a good safety profile. AIMS: To evaluate atrial septal defect (ASD) features impacting on right disc device thrombosis in patients who underwent Gore Cardioform ASD Occluder (GCA) implantation. METHODS: A total of 44 consecutive patients undergoing percutaneous ASD with GCA device from January 2020 to September 2022 at our tertiary care Center were evaluated. The minimum follow-up was 6 months. RESULTS: The patients were stratified in two groups according to a cut-off value of ASD diameter equal to 20 mm at sizing balloon, derived from ROC analysis (AUC = 0.894; p = 0.024). Baseline characteristics were comparable between groups in terms of age, sex, weight, height, and interatrial septum dimensions. Patients with ASD > 20 mm (n = 9) had a higher ASD/device dimension ratio, both at echocardiography (p = 0.009) and at sizing balloon (p = 0.001), longer fluoroscopic time (p = 0.022), and higher incidence of device thrombosis (0.006). Right disc thrombosis was observed in three patients of the ASD > 20 mm group, always in the inferior portion of the right disc. On univariate analysis, ASD diameter at sizing balloon (OR 1.360; p = 0.036) was the only positive predictor of device thrombosis. CONCLUSIONS: Right disc thrombosis of the GCA device may be under-recognized at follow-up, hence deserving clinical attention, especially in those patients with larger ASD diameters.

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.

[Proposal of a common model for informed consent for interventional procedures in congenital heart disease patients]. / Proposta di un modello comune al consenso informato per le procedure di emodinamica nel cardiopatico congenito.

BACKGROUND: A multidisciplinary study group involving physicians and jurists was established to review and approve an informed consent about the most frequent interventional procedures for congenital heart diseases. METHODS: The authors worked together with representatives of the Italian Society of Pediatric Cardiology and Congenital Heart Disease (SICP) Council and Jurist's expert in the field of health case-law. The final draft was shared with the major Italian centers involved in congenital interventional procedures and with AICCA, the Italian Patients Association of Congenital Heart Diseases - Adults and Children. RESULTS: At the end of this review process, a final informed consent form was developed for the most frequent procedures performed in our catheterization laboratories. All of them consist of two parts: a general statement and a procedure-related one. CONCLUSIONS: The work performed by this multidisciplinary study group, under the supervision of the SICP, resulted in a new dedicated informed consent about interventional procedures in the field of congenital cardiology, taking into account the new legal requests. This informed consent is intended to be both a document that can be used as such and a document from which to derive a specific document for each center. We believe that using similar informed consents in all Congenital Heart Disease Centers or at least have informed consents all inspired by the same setting, could be a further improvement in taking care of the patients and their families.

Case report: Personalized transcatheter approach to mid-aortic syndrome by in vitro simulation on a 3-dimensional printed model.

An 8-year-old girl, diagnosed with mid-aortic syndrome (MAS) at the age of 2 months and under antihypertensive therapy, presented with severe systemic hypertension (>200/120 mmHg). Computed tomography (CT) examination revealed aortic aneurysm between severe stenoses at pre- and infra-renal segments, and occlusion of principal splanchnic arteries with peripheral collateral revascularization. Based on CT imaging, preoperative three-dimensional (3D) anatomy was reconstructed to assess aortic dimensions and a dedicated in vitro planning platform was designed to investigate the feasibility of a stenting procedure under fluoroscopic guidance. The in vitro system was designed to incorporate a translucent flexible 3D-printed patient-specific model filled with saline. A covered 8-zig 45-mm-long Cheatham-Platinum (CP) stent and a bare 8-zig, 34-mm-long CP stent were implanted with partial overlap to treat the stenoses (global peak-to-peak pressure gradient > 60 mmHg), excluding the aneurysm and avoiding risk of renal arteries occlusion. Percutaneous procedure was successfully performed with no residual pressure gradient and exactly replicating the strategy tested in vitro. Also, as investigated on the 3D-printed model, additional angioplasty was feasible across the frames of the stent to improve bilateral renal flow. Postoperative systemic pressure significantly reduced (130/70 mmHg) as well as dosage of antihypertensive therapy. This is the first report demonstrating the use of a 3D-printed model to effectively plan percutaneous intervention in a complex pediatric MAS case: taking full advantage of the combined use of a patient-specific 3D model and a dedicated in vitro platform, feasibility of the stenting procedure was successfully tested during pre-procedural assessment. Hence, use of patient-specific 3D-printed models and in vitro dedicated platforms is encouraged to assist pre-procedural planning and personalize treatment, thus enhancing intervention success.

DATA in BRIEF of: Interventional Cardiac Catheterization in Neonatal Age: Results in a Multi-centre Italian Experience.

A comprehensive description of morbidity and mortality as well as risk factors of interventional cardiac catheterization performed in neonatal age was reported in our paper recently published on the International Journal of Cardiology (IJCA28502; PII: S0167-5273(20)30384-3; DOI: 10.1016/j.ijcard.2020.04.013). Eight Italian high-volume centres of Paediatric Cardiology were involved in this observational, retrospective data collection and analysis. In this dataset, clinical and procedural characteristics of 1423 newborns submitted to 1551 interventional cardiac catheterization procedures were analyzed. Primary outcomes were considered procedure and in-hospital mortality as well as major adverse event and procedural failure rates. Secondary outcomes were considered minor adverse events and need for blood transfusion. Targets of this data analysis were: 1) to evaluate the overall major risk factors of interventional cardiac catheterization; 2) to identify the most hazardous interventional procedures; 3) to assess possible trends of individual procedures as well as their outcome over time; 4) to find possible relationships between the volume activity of any centre and the procedure and follow-up outcome. In particular, this Data in Brief companion paper aims to report the specific statistic highlights of the multivariable analysis (binary logistic regression) used to assess the impact of any potential risk factors on the type of procedure over a short-term follow-up.

Prediction of post-stenting biomechanics in coarcted aortas: A pilot finite element study.

Endovascular stenting has recently become a standard treatment for native coarctation of the aorta (CoA) in children and young adults, given the efficacy in relieving vessel obstruction with a low incidence of adverse events. Yet, despite the short-term success of the technique, late hypertension remains an endemic risk. To assess the impact of the percutaneous procedure on the aortic wall biomechanics, we designed a novel finite element (FE) protocol for the simulation of endovascular stenting in three patient-specific CoA anatomies, developing a remeshing procedure that allows for coping with different CoA severities. Our FE protocol was able to yield numerical results on stent distortions and stresses, as well as on changes in aortic wall stresses and distensibility. These results were consistent with intraprocedural in-vivo evidences and with previous findings from the literature, and they suggest that our numerical approach could be used to understand the role of patient specific anatomical features (CoA severity and arch type) on the post-stenting aortic biomechanics. If soundly validated on a vast cohort of patients, our approach could support patient selection for the procedure.

Interventional cardiac catheterization in neonatal age: results in a multicentre Italian experience.

BACKGROUND: Despite recent technical advances, interventional cardiac catheterization is still challenging in neonatal age and no specific data concerning early outcome are so far published in literature. METHODS: Neonatal trans-catheter cardiac interventions performed in high-volume Italian referral centers were retrospectively analyzed. Primary outcomes were procedural major adverse events, in-hospital mortality and procedural failure. Secondary outcomes were minor adverse events and need for blood transfusion. RESULTS: From January 2000 to December 2017, 1423 newborns (mean weight 3.0 ± 0.6 kg, range 1.0-5.8; median age 2.0 days) underwent interventional cardiac catheterization. Overall, global procedure adverse event rate and in-hospital mortality were 10.2% and 5.2%, respectively. At multi-variable analysis, primary composite outcome was significantly related to low-weight (<2.5 kg) (p < 0.01) and younger age (≤7 days) (p < 0.01) at the procedure, prematurity (p < 0.01), uni-ventricular physiology (p < 0.01), associated genetic syndromes (p < 0.01) and procedure risk category (p < 0.01). No relationship between volume of activity of any single center and procedure outcome was found. Over time, a trend toward an increased number of procedures and their complexity was recorded. Trans-catheter management of cardiac malformations with critical, duct-dependent pulmonary blood flow by arterial duct stenting or right ventricular outflow tract stenting showed the highest increase. CONCLUSIONS: Interventional cardiac catheterization is relatively safe and feasible in neonatal age. Peri-natal age, low weight, uni-ventricular physiology and genetic syndromes still significantly contribute to procedural morbidity and in-hospital mortality of this approach.

Percutaneous Pulmonary Valve Implantation.

Percutaneous pulmonary valve implantation (PPVI) is recognized as a feasible and low risk alternative to surgery to treat dysfunctional right ventricular outflow tract (RVOT) in usually pluri-operated patients. Evolving technology allowed to develop different kind of prosthesis and to go from an initial treatment exclusively of stenotic conduit to an actual approach extended also to wide native RVOT. The Melody transcatheter pulmonary valve (TPV) and the Edwards Sapien valve are nowadays the most commonly implanted prostheses. However, other devices have been developed to treat large RVOT (i.e., the Venus p-valve, the Medtronic Harmony TPV, the Alterra Adaptive Prestent, and the Pulsta valve). Indications for PPVI are the same as for surgical interventions on pulmonary valve, with limits related to the maximum diameter of the available percutaneous prosthesis. Therefore, an accurate preoperative evaluation is of paramount importance to select patients who could benefit from this procedure. The overall periprocedural mortality incidence is around 1.4%, while freedom from RVOT reintervention ranges from 100% at 4 months to 70% at 70 months, according to the different published studies.

Surgical Rescue After Failed Percutaneous Closure of an Aorto-Atrial Tunnel.

Congenital sinus of Valsalva aneurysm is a rare disorder, mostly involving the right and noncoronary sinuses, in which intracardiac rupture is more likely foreseen into the right chambers. Because of the unfavorable prognosis, which includes heart failure and sudden death, detection itself is an indication for treatment, which may be performed either surgically or percutaneously. We present a case of a four-year-old patient with aorto-right atrial tunnel, in which a transcatheter attempt of closure was performed, complicated by new onset of aortic valve regurgitation, requiring surgical intervention.

3-Dimensional personalized planning for transcatheter pulmonary valve implantation in a dysfunctional right ventricular outflow tract.

BACKGROUND: Identification of adequate landing zone for transcatheter pulmonary valve implantation (TPVI) is crucial to successfully treat an aneurysmatic native right ventricle outflow tract (RVOT); three-dimensional (3D) patient-tailored digital and physical printed models are available but their actual strengths and weaknesses still not well documented. The aim of the study was to tackle TPVI planning in the dysfunctional and borderline RVOT exploiting both digital and physical printed 3D patient-specific models. METHODS: Electrocardiographically gated computed tomography (CT) angiography was segmented and anatomical RVOT geometrical changes dynamically tracked throughout the cardiac cycle using in-house processing. A compliant 3D-printed model was manufactured from the diastolic rest phase to test in vitro the catheter-based procedure feasibility; results were compared against CT-derived in vivo measurements and the actual catheterization outcome. RESULTS: CT-gated analysis successfully quantified in vivo RVOT dynamic changes corroborating the feasibility of non-conventional pulmonary jailing percutaneous intervention. Clinicians used the 3D-printed model to test the steps of the jailing procedure; yet, the deformable 3D model printed at diastole underestimated the final implant dimensions obtained during cardiac catheterization by the same operators. CONCLUSIONS: Multidisciplinary synergy between CT-gated analysis and pre-procedural tests on 3D-printed phantoms can help the interventional team to tackle complex TPVI procedures. To fully exploit 3D-printed models, adequate selection of the still frame to print and tuning of printing material properties is crucial and can be aided by 3D dynamic virtual models.

Use of 65 cm large caliber Dryseal sheaths to facilitate delivery of the Edwards SAPIEN valve to dysfunctional right ventricular outflow tracts.

BACKGROUND: The Edwards SAPIEN valve and its delivery system may complicate transit through the right heart during transcatheter pulmonary valve replacement (tPVR). We report our early experience using a large diameter, 65 cm delivery sheath to facilitate delivery of the SAPIEN valve to the right ventricular outflow tract (RVOT). METHODS: Retrospective analysis of all patients from three large congenital heart centers undergoing tPVR with the Edwards SAPIEN valve delivered with the 65 cm Gore Dryseal Sheath. RESULTS: Over a 12 month period, 30 patients (17 female) with median age 17.5 years (range 8-72) underwent attempted tPVR with the SAPIEN valve delivered using the 65 cm Dryseal sheath (20-26Fr). All procedures resulted in successful valve delivery to the target area. Twenty patients had a native RVOT. The most commonly used valve diameter was 29 mm (n = 15) with the majority of cases requiring a 26Fr Dryseal sheath (n = 20). One patient with severe RVOT stenosis underwent prestenting. Median procedure time was 100 min (59-225). No patient had increase in tricuspid valve regurgitation as a consequence of valve delivery. One patient required a synchronous cardioversion for intraprocedural VT and another required ECMO postprocedure due to severe pre-existing left ventricular dysfunction. On median follow-up of 5 months, all patients had mild or less pulmonary regurgitation. Median peak Doppler velocity across the pulmonary valve was 2.2 m/s (1.7-4). There were no clinically relevant complications relating to vascular access. CONCLUSIONS: Using 65 cm Dryseal sheaths facilitates delivery of SAPIEN valves in patients with dysfunctional RVOTs.

Prediction of stenting related adverse events through patient-specific finite element modelling.

Right ventricular outflow tract (RVOT) calcific obstruction is frequent after homograft conduit implantation to treat congenital heart disease. Stenting and percutaneous pulmonary valve implantation (PPVI) can relieve the obstruction and prolong the conduit lifespan, but require accurate pre-procedural evaluation to minimize the risk of coronary artery (CA) compression, stent fracture, conduit injury or arterial distortion. Herein, we test patient-specific finite element (FE) modeling as a tool to assess stenting feasibility and investigate clinically relevant risks associated to the percutaneous intervention. Three patients undergoing attempted PPVI due to calcific RVOT conduit failure were enrolled; the calcific RVOT, the aortic root and the proximal CA were segmented on CT scans for each patient. We numerically reproduced RVOT balloon angioplasty to test procedure feasibility and the subsequent RVOT pre-stenting expanding the stent through a balloon-in-balloon delivery system. Our FE framework predicted the occurrence of CA compression in the patient excluded from the real procedure. In the two patients undergoing RVOT stenting, numerical results were consistent with intraprocedural in-vivo fluoroscopic evidences. Furthermore, it quantified the stresses on the stent and on the relevant native structures, highlighting their marked dependence on the extent, shape and location of the calcific deposits. Stent deployment induced displacement and mechanical loading of the calcific deposits, also impacting on the adjacent anatomical structures. This novel workflow has the potential to tackle the analysis of complex RVOT clinical scenarios, pinpointing the procedure impact on the dysfunctional anatomy and elucidating potential periprocedural complications.

Heart failure in grown-up congenital heart disease.

The increasing survival to adulthood of patients with congenital heart disease (CHD) has changed the epidemiology of adult CHD (ACHD) patients and has led to an increment in hospitalization rates due to heart failure (HF). ACHD patients hospitalized for HF have a five-fold higher risk of death than those compensated. HF occurs predominantly in patients with tetralogy of Fallot, single ventricles, and after the Mustard operation for transposition of the great arteries. Diagnostic strategies applied in acquired HF patients are usually used to evaluate ACHD patients, but sometimes this can postpone the identification of HF that can become manifest with unusual and peculiar signs or symptoms. In the same way, therapeutic management resembles the acquired HF one, even if no large randomized clinical trials have been conducted in ACHD patients. Therefore, a close monitoring in dedicated units is mandatory in order to identify in time HF manifestations and manage them adequately.

Percutaneous management of failed bioprosthetic pulmonary valves in patients with congenital heart defects.

AIMS: We reviewed our center experience in the field of transcatheter pulmonary valve-in-valve implantation (TPViV), that is emerging as a treatment option for patients with pulmonary bioprosthetic valve (BPV) dysfunction. METHODS: Between April 2008 and September 2015, a total of six patients with congenital heart disease (four men) underwent TPViV due to stenosis of preexisting BPV. Four patients received a Melody Medtronic Transcatheter Pulmonary Valve and two an Edward Sapien Valve. RESULTS: No procedural-related complications occurred. After valve implantation, right ventricular systolic pressure (RVSP, 80.5 ±â€Š25.3-41.2 ±â€Š8.35 mmHg, P < 0.05), right ventricular outflow tract (RVOT) gradient (55.3 ±â€Š23.4-10.6 ±â€Š3.8 mmHg, P < 0.05), and RVSP-to-aortic pressure (0.75 ±â€Š0.21-0.38 ±â€Š0.21, P = 0.01) fell significantly. Echocardiograms at follow-up revealed a significant reduction in estimated RVSP (88.7 ±â€Š22-21.7 ±â€Š4.7 mmHg, P < 0.05), in RVOT (76.2 ±â€Š17.9-25.7 ±â€Š6.1 mmHg, P = 0.005), and in mean RVOT (40.7 ±â€Š9.9-15.5 ±â€Š4.8 mmHg, P < 0.05) gradients. Cardiac magnetic resonance showed no significant change in biventricular dimensions and function. Symptomatic patients reported improvement of symptoms, although cardiopulmonary exercise did not show any significant differences. CONCLUSION: TPViV is an effective and well tolerated treatment for BPV dysfunction, improving freedom from surgical reintervention. Long-term studies will redefine the management of dysfunctional RVOT, either native or surrogate.

Partial abnormal drainage of superior and inferior caval veins into the left atrium: two case reports.

Abnormal connection of the right superior caval vein to the left atrium is an uncommon systemic vein drainage anomaly, with only a few cases reported among congenital heart disease (CHD), around 20 cases published in the medical literature. The inferior vena cava connection with the left atrium, also very rare, can appear directly or in heterotaxy. Clinical suspicion arises due to the presence of cyanosis in the absence of other specific clinical signs (without other associated CHD). We present the cases of two children with abnormal superior and inferior systemic venous return. The first case is an abnormal connection of right superior vena cava to the left atrium associated with persistent left superior vena cava draining into the right atrium through the coronary sinus. The second case is an interruption of the inferior vena cava with hemiazygos continuation, drained into the left superior vena cava, which drained into the left atrium. The diagnosis was imagistic - echocardiography and angiography. Surgical treatment solutions vary from one case to another, usually following anatomic correction. Hypoxia accompanied by cyanosis must bring into question the pathology of systemic venous drainage anomaly, after other common causes have been excluded. Surgery is indicated in all cases due to the risk associated with the presence of right-to-left shunt.

Coronary-cameral fistulas: indications and methods for closure.

Coronary-cameral fistulas (CCF) are anomalous connections between one or more coronary arteries and a cardiac chamber, most commonly the right ventricle or right atrium. The major indications for closure are: significant left to right shunt, myocardial ischaemia, prevention of endoarteritis or rupture. Nowadays, the first option for treatment is transcatheter closure. According to the morphology of the fistulas the most appropriate occluder device should be selected: coils (e.g., Gianturco coils, controlled-release coils, PFM coils), vascular plugs or a patent ductus arteriosus (PDA) device or muscular ventricular septal defect (VSD) device. The way to deploy the occluders could be direct arterial or venous through an arteriovenous loop, according to the anatomy of the fistulas assessed by multiple angiograms in different projections. A test occlusion of the fistula with balloon catheter and simultaneous coronary angiogram is recommended for choosing the proper device type and size and the best position for deployment to achieve complete occlusion of the fistula without compromising the flow in coronary side branches.