Search Results (13)

Pulmonary disfunction is frequent in repaired congenital heart diseases. Both pulmonary regurgitation and pulmonary stenosis are possible complications over time. In the past, the surgical approach was the only feasible management but exposed the patient to a redo-surgery with its consequent risks. Nowadays, the development of novel devices and techniques has made possible a transcatheter pulmonary valve implantation. The Melody Transcatheter Pulmonary Valve (TPV) (Medtronic Inc., Minneapolis, MN, USA) and the Edwards Sapien XT and S3 Transcatheter Heart Valve (Edwards Lifesciences LLC, Irvine, CA, USA) are balloon-expandable valvular bioprostheses approved for pulmonary position. Venus P-Valve (Venus Medtech, Shanghai, China) and Harmony TPV (Medtronic Inc., Minneapolis, MN, USA) are self-expandable pulmonary valves. Alterra Adaptive Prestent (Edwards Lifesciences LLC, Irvine, CA, USA) is an hourglass self-expandable stent that reduces the size of large right ventricular outflow tracts, creating a suitable landing zone to implant an Edwards Sapien S3 THV 29 mm. Novel stents and percutaneous valves are being planned and experimented with to widen the field of transcatheter approach. The aim of this review is to describe both the current approaches, strategies, and techniques as well as the future perspective to deal with the patients with significant pulmonary stenosis and/or regurgitation. Full article

The purpose: Evaluation of the short-term and long-term results of a phased correction of the tetralogy of Fallot (ToF) with stenting of the right ventricular outflow tract (RVOT) in comparison with a one-stage total correction (TC) of the defect. Materials and methods: Two groups of patients with classical ToF were formed. Group 1 (n = 25; median age = 72 days) was initially represented by children with ToF with a more severe clinical status (median weight = 3.6 kg, with more pronounced cyanosis and with comorbidities). The children of group 1 underwent the first stage of RVOT stenting and the second stage of TC of ToF. Group 2 (n = 25) was represented by older patients, with a higher body weight and SpO2 level, and they underwent a single-stage TC of the defect. Results: The application of a step-by-step ToF correction approach with RVOT stenting in low-weight newborns with severe hypoxemia demonstrated an equivalent effect on SpO2 dynamics—reverse remodeling of the heart—when compared with a less severe cohort of patients who underwent simultaneous TC of classical ToF. After RVOT stenting in children from group 1, the median SpO2 increased from 80% to 94.5%, the median Z value of the pulmonary artery trunk decreased from −3.46 mm to −2.54 mm, and the median index of end-diastolic volume of the left ventricle decreased from 23.07 mm/m² to 57.6 mL/m². TC of ToF in children from group 1 with a phased strategy of correction of the defect was no less successful than in children who underwent simultaneous TC. In the long-term follow-up period after TC of ToF, children from both groups, who were obviously unequal in their initial status, were practically comparable in clinical characteristics, exhibiting features of cardiac remodeling and achieving endpoints. And there were no significant differences between the two groups in the frequency of reaching the endpoints such as re-operations, cerebrovascular events, and death during the annual, three-year, and five-year follow-up period. Conclusions: The strategy of RVOT stenting followed by TC of ToF in a severe group of children demonstrated comparable results compared with the results of simultaneous TC of ToF in a more stable group of patients during the in-hospital, annual, three-year, and five-year follow-up periods. Full article

Despite significant improvements in techniques, the treatment of neonates and infants with congenital heart disease resulting in duct-dependent pulmonary circulation is still significantly challenging. Despite current trends toward early primary surgical repair, temporary palliation is still necessary in those patients who are at high surgical risk for complete correction due to unfavorable clinical or anatomic characteristics. Recent advances in interventional cardiology have led to the emergence of right ventricular outflow tract and arterial duct stenting as cost-effective alternatives to surgical palliation in high-risk surgical candidates or whenever short-term pulmonary blood flow support is anticipated. This review aims to explore the evolving landscape of these transcatheter approaches, highlighting their role, efficacy and potential complications in the context of duct-dependent pulmonary circulation anatomies. Full article

Transcatheter pulmonary valve replacement is a minimally-invasive alternative treatment for right ventricular outflow tract dysfunction and has been rapidly evolving over the past years. Heart valve prostheses currently available still have major limitations. Therefore, one of the significant challenges for the future is the roll out of transcatheter tissue engineered pulmonary valve replacement to more patients. In the present study, biodegradable poly-ε-caprolactone (PCL) nanofiber scaffolds in the form of a 3D leaflet matrix were successfully seeded with human endothelial colony-forming cells (ECFCs), human induced pluripotent stem cell-derived MSCs (hMSCs), and porcine MSCs (pMSCs) for three weeks for the generation of 3D tissue-engineered tri-leaflet valved stent grafts. The cell adhesion, proliferation, and distribution of these 3D heart leaflets was analyzed using fluorescence microscopy and scanning electron microscopy (SEM). All cell lineages were able to increase the overgrown leaflet area within the three-week timeframe. While hMSCs showed a consistent growth rate over the course of three weeks, ECFSs showed almost no increase between days 7 and 14 until a growth spurt appeared between days 14 and 21. More than 90% of heart valve leaflets were covered with cells after the full three-week culturing cycle in nearly all leaflet areas, regardless of which cell type was used. This study shows that seeded biodegradable PCL nanofiber scaffolds incorporated in nitinol or biodegradable stents will offer a new therapeutic option in the future. Full article

Background: Severe pulmonary regurgitation (PR) often occurs following a transannular patch repair of tetralogy of Fallot, resulting in an enlarged native right ventricular outflow tract (nRVOT) with varying shapes. Methods: We conducted a single-center study with eight patients having severe PR and enlarged nRVOT (diameters ≥ 29 mm). Transcatheter pulmonary valve replacement (TPVR) was performed using the self-expanding PT-Valve. Preoperative evaluation included echocardiography, computed tomography, and magnetic resonance imaging. A 3D-printed model of the nRVOT was used for preoperative assessment. Follow-up data were collected in 1-year follow-up. Results: PT-Valve was successfully implanted in all patients, resulting in immediate improvement of severe PR. Pulmonary artery diastolic pressure increased significantly (p < 0.001). No deaths or coronary compression occurred during the procedure. Over a 1-year follow-up, no stent displacement or fracture occurred. Only two patients had trace paravalvular leaks. Magnetic resonance imaging revealed a reversal of right ventricular remodeling, with a significant reduction in right ventricular end-diastolic volume index (p < 0.001) and improved right ventricular ejection fraction (p < 0.001). All patients achieved primary endpoints. Conclusion: 3D printing-guided PT-Valve implantation in enlarged nRVOT for severe PR is safe and effective, expanding TPVR indications and offering potential treatment for a broader patient population. Full article

Introduction: Takotsubo cardiomyopathy (TCM) is a reversible form of cardiomyopathy characterized by transient regional systolic dysfunction of the left ventricle. Case outline: A 78-year-old woman was admitted to the general hospital due to acute inferior STEMI late presentation. Two days after admission, the patient reported intense chest pain and an ECG registered diffuse ST-segment elevation in all leads with ST-segment denivelation in aVR. The patient also showed clinical signs of cardiogenic shock and was referred to a reference institution for further evaluation. Echocardiography revealed akinesia of all medioapical segments, dynamic obstruction of the left ventricular outflow tract (LVOT), moderate mitral regurgitation, and pericardial effusion. Coronary angiography showed the suboccluded right coronary artery, and a primary percutaneous coronary intervention was performed, which involved implanting a drug-eluting stent. The patient’s condition worsened as pericardial effusion increased and led to tamponade. Pericardiocentesis was performed, resulting in the patient’s stabilization. At this point, significant gradients at the LVOT and pericardial effusion were not registered. After eight days without symptoms and stable status, the patient was discharged. Conclusions: The simultaneous presence of AMI and TCM increases the risk of developing cardiogenic shock. The cardio-circulatory profile of these patients is different from those with AMI. Full article

Background: Adults with congenital heart disease (ACHD) are a growing population needing ongoing care. The aim of this study was to investigate if a dedicated ACHD team impacted the timing and indication of invasive cardiology procedures in these patients at our hospital. Methods: Our retrospective single-center study enrolled adult patients with moderate or complex congenital heart disease and with at least one cardiac catheterization between January 2010 and December 2021. According to the period, procedures were labeled as group A (2010 to 2015) or group B (2016 to 2021) and further divided into diagnostic (DCC) and interventional cardiac catheterizations (ICC). Results: 594 patients were eligible for the study. Both DCC (p < 0.05) and ICC increased between groups A and B (p < 0.05). In group B: Fontan patients accounted for the majority of DCC (p < 0.001), while DCC decreased in arterial switch repair (p < 0.001). In Fontan patients, conduit stenting was prevalent (p < 0.001), while fenestration closures dropped (p < 0.01). In patients with tetralogy of Fallot and native outflow tract, percutaneous pulmonary valve implantations (PPVI) increased, with a concurrent reduction in pulmonary valve replacements (p < 0.001 vs. surgical series). In right ventricular conduits, ICC increased (p < 0.01), mainly due to PPVI. Among Mustard/Senning patients, baffle stenting increased from Group A to Group B (p < 0.001). In patients with pulmonary atresia and biventricular repair, ICC often increased for pulmonary artery stenting. Conclusions: A dedicated working group could improve ACHD patients’ indications for interventional procedures, leading to tailored treatment, better risk stratification and optimizing time until heart transplantation. Full article

Performance of cardiovascular magnetic resonance (CMR) in the planning phase of percutaneous pulmonary valve implantation (PPVI) is needed for the accurate delineation of the right ventricular outflow tract (RVOT), coronary anatomy and the quantification of right ventricular (RV) volume overload in patients with significant pulmonary regurgitation (PR). This helps to find the correct timings for the intervention and prevention of PPVI-related complications such as coronary artery compression, device embolization and stent fractures. A defined CMR study protocol should be set for all PPVI candidates to reduce acquisition times and acquire essential sequences that are determinants for PPVI success. For correct RVOT sizing, contrast-free whole-heart sequences, preferably at end-systole, should be adopted in the pediatric population thanks to their high reproducibility and concordance with invasive angiographic data. When CMR is not feasible or contraindicated, cardiac computed tomography (CCT) may be performed for high-resolution cardiac imaging and eventually the acquisition of complementary functional data. The aim of this review is to underline the role of CMR and advanced multimodality imaging in the context of pre-procedural planning of PPVI concerning its current and potential future applications. Full article

Cardiac computed tomography angiography (C-CTA) is crucial in assessing the right ventricular outflow tract (RVOT) prior to a transcatheter pulmonary valve replacement (TPVR), as an incorrect evaluation can make the procedure more challenging and can lead to device-related complications. This study aimed to evaluate the feasibility and accuracy of 4D straightened segmentation for a landing zone analysis over anatomical segmentation. Seven pre-operative CTAs and seven post-operative CTAs were used to measure the cross-sectional area, circumference, and diameters at five selected planes as the landing zone for TPVR and compared these to the 4D straightened model with the anatomical model. Furthermore, the right ventricular volume, stent volume, and 4D ellipticity index were calculated from the 4D straightened model. The 4D straightened segmentation had comparable accuracy and efficacy for the measurements at the landing zone. The cross-sectional area and the circumference varied greatly at the RVOT and the basal plane of the pulmonary valve compared with the other three planes of the 4D straightened models from the pre-operative CTAs; however, only the values at the RVOT were found to vary greatly from the post-operative CTAs. The 4D straightened model can provide accurate measurements and is thus a useful method for the periprocedural evaluation of TPVR. Full article

Patients with the complex congenital heart disease (CHD) are usually associated with right ventricular outflow tract dysfunction and typically require multiple surgical interventions during their lives to relieve the right ventricular outflow tract abnormality. Transcatheter pulmonary valve replacement was used as a non-surgical, less invasive alternative treatment for right ventricular outflow tract dysfunction and has been rapidly developing over the past years. Despite the current favorable results of transcatheter pulmonary valve replacement, many patients eligible for pulmonary valve replacement are still not candidates for transcatheter pulmonary valve replacement. Therefore, one of the significant future challenges is to expand transcatheter pulmonary valve replacement to a broader patient population. This review describes the limitations and problems of existing techniques and focuses on decellularized tissue engineering for pulmonary valve stenting. Full article

Background. Despite current trends toward early primary repair, the surgical systemic-to-pulmonary shunt is still considered the first-choice palliation in patients with critical tetralogy of Fallot (TOF) and duct-dependent pulmonary circulation unsuitable for primary repair. However, stenting of the right ventricular outflow tract (RVOT) is nowadays emerging as an effective alternative to surgical palliation in selected patients. Methods and results. RVOT stenting is usually performed from a venous route, either femoral or, in selected cases, the right internal jugular vein. Less frequently, mostly in pulmonary infundibular/valvar atresia, this procedure can be performed using a hybrid surgical/interventional approach by surgical exposure of the RVOT, puncture of the atretic valve, and stent deployment under direct vision. The size and type of the most appropriate stent may be chosen, based on ultrasound measurements of the RVOT, to cover the right ventricular infundibulum completely and, at the same time, sparing the pulmonary valve, unless significant pulmonary valve annulus hypoplasia and/or supra-valvular stenosis is a significant component of the obstruction. In the large series so far published, early mortality of RVOT stenting is less than 2%, comparing favourably with either Blalock-Thomas-Taussig shunt or early primary repair. In addition, morbidity and clinical sequelae of this approach do not significantly differ from surgical palliation, even if RVOT stenting shows lesser durability and a higher rate of trans-catheter re-interventions over a mid-term follow-up. Finally, similar but more balanced pulmonary artery growth than surgical palliation following RVOT stenting is reported over a mid-term follow-up. Conclusions. RVOT stenting is a technically feasible, well-tolerated, and effective palliation in critical TOF. This approach is cost-effective with respect to surgical palliation either in high-risk neonates or whenever a short-term pulmonary blood flow source is anticipated due to the early surgical repair. It effectively increases pulmonary blood flow, improves arterial saturation, and promotes balanced pulmonary artery growth over a mid-term follow-up. Full article

The current treatment practice for Tetralogy of Fallot (TOF) is to undertake complete surgical repair between 6–9 months of age with excellent immediate and long-term results. In patients with increased cyanosis or frequent cyanotic spells, younger age of complete repair as early as 3 months is usually acceptable. Although neonatal TOF repair is reported with good immediate survival from a few centres, post-operative morbidity is significant, and the hospital stay is prolonged. Hence, in neonates and small term and preterm infants with severe cyanosis, palliative procedures such as modified Blalock-Taussig (BT) shunt, ductal stenting, right ventricular outflow tract (RVOT) stenting and balloon pulmonary valvotomy (BPV) have been reported. With the development of low-profile stents, an increasing clinician experience and more predictable outcome with both duct and RVOT stenting, these procedures have gained popularity as the preferred palliations for TOF at most centres. We reviewed the literature to analyse whether BPV for TOF palliation, a technique first reported three decades ago, still has a role in the present era of paediatric cardiac care. In this review, we have concluded that BPV has very specific indications in patients with TOF, with predominantly valvar pulmonary stenosis, and may be a preferred option over other palliative strategies in hypoxemic preterm or low-birth weight infants. Full article

Percutaneous pulmonary valve implantation (PPVI) is used to treat pulmonary stenosis (PS) or pulmonary regurgitation (PR). We described our experience with PPVI, specifically valve-in-valve transcatheter pulmonary valve replacement using the Melody valve and novel self-expandable systems using the Pulsta valve. We reviewed data from 42 patients undergoing PPVI. Twenty-nine patients had Melody valves in mostly bioprosthetic valves, valved conduits, and homografts in the pulmonary position. Following Melody valve implantation, the peak right ventricle-to-pulmonary artery gradient decreased from 51.3 ± 11.5 to 16.7 ± 3.3 mmHg and right ventricular systolic pressure fell from 70.0 ± 16.8 to 41.3 ± 17.8 mmHg. Thirteen patients with native right ventricular outflow tract (RVOT) lesions and homograft underwent PPVI with the new self-expandable Pulsta valve—a nitinol wire stent mounted with a trileaflet porcine pericardial valve. Following Pulsta valve implantation, cardiac magnetic resonance imaging showed a decreased PR fraction and that the right ventricular end-diastolic volume index decreased from 166.1 ± 11.9 to 123.6 ± 12.4 mL/m². There were no mortality, severe procedural morbidity, or valve-related complications. At the mean 14.2 month (4–57 months) follow-up, no patients had more than mild PR. PPVI using Melody and Pulsta valves was first shown to provide excellent early outcomes without serious adverse event in most patients with RVOT dysfunction in Korea. Full article