Search Results (32)

Background: Patients with single-ventricle physiology represent a high-risk group for heart transplantation. Due to complex anatomical and physiological challenges, including multiple prior sternotomies, pulmonary artery abnormalities, and systemic consequences of altered circulation, they represent both a surgical and a clinical challenge. We aimed to analyze perioperative challenges, as well as early and long-term complications, in this specific group of patients. Methods: We performed a retrospective data analysis of a high-volume heart transplant center, focusing on patients with single-ventricle physiology who were scheduled for heart transplantation due to end-stage heart failure. We retrospectively analyzed the period from the beginning of the transplant program in November 1985 to the end of November 2024. Results: Among 1553 transplanted patients (adults and children), 29 were transplanted due to congenital heart disease (congenital valvular disease not included). In this group, nine patients were transplanted due to end-stage heart failure in the course of single-ventricle physiology. Age at transplantation ranged from 7 to 31 years (median, 17 years), and body weight ranged from 15 to 69 kg (median, 47.9 kg). All nine patients referred for heart transplantation presented with single-ventricle physiology. Their underlying congenital heart defects were heterogeneous and included hypoplastic left heart syndrome (HLHS), double-outlet left ventricle (DOLV), transposition of the great arteries (TGA) with associated ventricular septal defects (VSDs), atrial septal defects (ASDs), valvular abnormalities such as tricuspid and or pulmonary valve atresia or stenosis, systemic or atrioventricular valve regurgitation, and vascular abnormalities, including right-sided aortic arch, aortic coarctation, and pulmonary artery hypoplasia, stenosis, or occlusion, as well as associated pulmonary vascular abnormalities such as left pulmonary artery stenosis and MAPCAs. All patients had previously undergone staged palliative procedures, including Norwood, Hemi-Fontan, Fontan, bidirectional Glenn, modified Blalock–Taussig shunts, Bjork–Fontan, or pulmonary artery banding, often with repeated interventions such as balloon angioplasty, stent placement, or MAPCA closure. Extracardiac comorbidities were common and included coagulopathies, protein-losing enteropathy, hepatic dysfunction, and chronic venous insufficiency. Preoperative functional status was markedly impaired in all patients (NYHA III-IV, INTERMACS 3-4), with severely reduced exercise capacity and thrombotic events in several individuals. Perioperative transplant surgical strategies included femoral cannulation in four cases and standard aortic and caval cannulation in five cases. Pulmonary artery reconstruction was required in all patients. Extended donor pulmonary arteries were applied in eight cases, while a bifurcated Dacron prosthesis was utilized in one patient. Perioperative mortality was 33%, with three deaths attributed to bleeding and hemodynamic instability, while overall mortality was 44% including one late death unrelated to transplantation. Protein-losing enteropathy, although persistent in the immediate postoperative period, resolved in all surviving patients, underscoring the transformative impact of transplantation. Conclusions: These findings emphasize the importance of individualized surgical planning, extended donor pulmonary artery harvesting, and careful preoperative coordination. Heart transplantation remains a viable and life-extending option for selected single-ventricle patients, despite the significant technical and clinical challenges involved. Full article

Background: This case report describes the surgical management of pulmonary artery banding (PAB) in a cat diagnosed with an incomplete atrioventricular canal (AVC) defect and a concurrent muscular septal defect. It highlights the use of PAB as a palliative treatment to manage this rare congenital heart condition in companion animals. Case Presentation: A 9-month-old European long-haired male cat presented with clinical signs of heart failure. Pharmacological treatment with sildenafil and furosemide failed to stabilize the patient’s condition. Echocardiographic assessment revealed an incomplete AVC type A and a muscular septal defect. Pulmonary artery banding was performed to reduce pulmonary blood flow and alleviate heart failure symptoms. Pre- and postoperative echocardiographic evaluations were conducted to monitor structural and functional changes in the heart. Post-surgical outcomes included a marked reduction in the size of the right atrium and ventricle and a stable pulmonary artery flow velocity of 3.8 m/s. The cat has remained in very good condition without the need for pharmacotherapy for 13 months post-surgery and is still alive at the time of reporting. Conclusions: PAB proved effective as a palliative intervention for managing an incomplete AVC in this feline patient. The successful outcome suggests that PAB may offer significant long-term benefits and improved survival in selected cases of incomplete AVCs in cats. Full article

Right ventricular (RV) failure is a common complication in multiple congenital heart disease (CHD), significantly increasing morbidity and mortality. Despite its impact, no therapies specifically target the failing RV. The growing population of CHD patients underscores the need to understand the pathophysiology of RV failure through preclinical research. This study aimed to develop an ovine model of RV failure induced by pressure and volume overload. A total of 14 juvenile sheep randomly underwent pulmonary artery banding (n = 6), pulmonary leaflet perforation (n = 4), and pulmonary annulotomy with transannular patching (TAP) (n = 4). Detailed anesthetic and surgical protocols were described, and intraoperative and early postoperative complications were evaluated. Acute RV pressure overload resulted in a 120% increase in RV pressure (p = 0.0312). The stroke volume index and cardiac index significantly declined (p = 0.0312), and there was a significant decrease in ScvO₂ (p = 0.0312). Both TAP and leaflet perforation achieved moderate-to-severe pulmonary regurgitation. Both procedures led to an incremental trend in RV pressures and resulted in a 24% increase in the stroke volume index. All techniques demonstrated safety and feasibility, with low mortality. This comprehensive model could be reproducible in other large animal models, offering a robust platform for preclinical research into CHD-RV failure models. Full article

Background: The diet and physical activity of the world’s population determine the increase in the number of bariatric surgeries. The most common types of bariatric surgery are laparoscopic sleeve gastrectomy (LSG) and Roux-Y gastric bypass (RYGB). Surgical interventions are carried out in patients with numerous comorbidities, among which the most common are cardiovascular diseases. The aim of the present study was to review the literature regarding the safety and results of surgical treatment in patients with cardiac revascularization prior to surgery. Methods: We performed an online search in Pubmed in September 2023 to identify articles that reported cardiac revascularization prior to bariatric surgery. The extracted information included details of the working method, number of patients, types of cardiovascular disease—heart failure (HF) and cardiac artery disease (CAD), types of revascularization—coronary artery bypass graft (CABG), percutaneous coronary intervention (PCI) or both CABG + PCI, demographic data (age, gender, race), clinical characteristics (body mass index—BMI, smoking status), comorbidities (diabetes mellitus, hypertension, prior myocardial infarction), postoperative complications, and postoperative results. Results: A total of 171 records were identified by the initial search, and 165 papers were excluded after applying the exclusion criteria (types of cardiovascular disease, types of revascularization, and demographic data). We evaluated a group of 9479 patients of which 730 had HF, 2621 CAD, and 1426 underwent prior cardiac revascularization. The analysis of the demographic data showed an average age of 55.5 years and a fluctuation of the male gender between 39% and 71.1%, and the female gender between 28.9% and 61%. The main types of bariatric interventions were RYGB (3659 cases) and LSG (659 cases), to which adjustable gastric band (AGB) and bilio-pancreatic diversion—duodenal switch (BPD-DS) were added. Among the most postprocedural complications were ST-segment elevation myocardial infarction (2 patients), gastro-intestinal bleeding (51 cases), pulmonary embolism (1 patient), arrhythmia (3 patients) and pacemaker insertion (1 patient). The recorded postoperative mortality rate was 0.42% (6 cases). Conclusions: Bariatric surgery remains safe in patients with cardiac revascularization. These finding need to be confirmed in more large-scale randomized trials. Full article

Pulmonary arterial hypertension (PAH) is a chronic disorder characterized by excessive pulmonary vascular remodeling, leading to elevated pulmonary vascular resistance and right ventricle (RV) overload and failure. MicroRNA-146a (miR-146a) promotes vascular smooth muscle cell proliferation and vascular neointimal hyperplasia, both hallmarks of PAH. This study aimed to investigate the effects of miR-146a through pharmacological or genetic inhibition on experimental PAH and RV pressure overload animal models. Additionally, we examined the overexpression of miR-146a on human pulmonary artery smooth muscle cells (hPASMCs). Here, we showed that miR-146a genic expression was increased in the lungs of patients with PAH and the plasma of monocrotaline (MCT) rats. Interestingly, genetic ablation of miR-146a improved RV hypertrophy and systolic pressures in Sugen 5415/hypoxia (SuHx) and pulmonary arterial banding (PAB) mice. Pharmacological inhibition of miR-146a improved RV remodeling in PAB-wild type mice and MCT rats, and enhanced exercise capacity in MCT rats. However, overexpression of miR-146a did not affect proliferation, migration, and apoptosis in control-hPASMCs. Our findings show that miR-146a may play a significant role in RV function and remodeling, representing a promising therapeutic target for RV hypertrophy and, consequently, PAH. Full article

Background/Objectives: Hybrid palliation (HP) procedures for hypoplastic left heart syndrome (HLHS) are increasing. Our objective was to compare mortality and morbidity following HP and NP (Norwood palliation) procedures. Methods: Systematic review and meta-analysis of HLHS patients of peer-reviewed literature between 2000 and 2023. Mortality and/or heart transplantation in HP versus NP in the neonatal period, interstage period, and at 1, 3 and 5 years of age, and morbidity including completion of Stage II and Stage III palliation, unexpected interventions, pulmonary artery pressures, right ventricle function, neurodevelopmental outcomes and length of hospital stay were evaluated. Results: Twenty-one (meta-analysis: 16; qualitative synthesis: 5) studies evaluating 1182 HLHS patients included. HP patients had higher interstage mortality (RR = 1.61; 95% CI: 1.10–2.33; p = 0.01) and 1-year mortality (RR = 1.22; 95% CI: 1.03–1.43; p = 0.02) compared to NP patients without differences in 3- and 5-years mortality. HP procedure in high-risk HLHS patients had lower mortality (RR = 0.48; 95% CI: 0.27–0.87; p = 0.01) only in the neonatal period. HP patients underwent fewer Stage II (RR = 0.90; 95% CI: 0.81–1.00; p = 0.05) and Stage III palliation (RR = 0.78; 95% CI: 0.69–0.90; p < 0.01), had more unplanned interventions (RR = 3.38; 95% CI: 2.04–5.59; p < 0.01), and longer hospital stay after Stage I palliation (weighted mean difference = 12.88; 95% CI: 1.15–24.62; p = 0.03) compared to NP patients. Conclusions: Our study reveals that HP, compared to NP for HLHS, is associated with increased morbidity risk without an improved survival rate. Full article

Right-sided mechanical support of the Fontan circulation by existing devices has been compounded by the cross-sectional design of vena cava anastomosis to both pulmonary arteries. Our purpose was to investigate whether increasing inferior vena cava (IVC) flow with a rotary blood pump in the IVC only in an ovine animal model of Fontan would lead to acceptable superior vena cava (SVC) pressure. To achieve this, a Fontan circulation was established in four female sheep by anastomosing the SVC to the main pulmonary artery (MPA) and by interposing a Dacron graft between the IVC and the MPA. A rotary blood pump was then introduced in the graft, and the effect of incremental flows was observed at increasing flow regimen. Additionally, to stimulate increased pulmonary resistance, the experience was repeated in each animal with the placement of a restrictive band on the MPA distally to the SVC and Dacron graft anastomosis. Circulatory support of IVC flow alone increased the systemic cardiac output significantly, both with and without banding, indicating the feasibility of mechanical support of the Fontan circulation by increasing the flow only in the inferior vena cava. The increase in SVC pressure remained within acceptable limits, indicating the potential effectiveness of this mode of support. The findings suggest that increasing the flow only in the inferior vena cava is a feasible method for mechanical support of the Fontan circulation, potentially leading to an increase in cardiac output with acceptable increases in superior vena cava pressure. Full article

Increased mitochondrial reactive oxygen species (ROS) formation is important for the development of right ventricular (RV) hypertrophy (RVH) and failure (RVF) during pulmonary hypertension (PH). ROS molecules are produced in different compartments within the cell, with mitochondria known to produce the strongest ROS signal. Among ROS-forming mitochondrial proteins, outer-mitochondrial-membrane-located monoamine oxidases (MAOs, type A or B) are capable of degrading neurotransmitters, thereby producing large amounts of ROS. In mice, MAO-B is the dominant isoform, which is present in almost all cell types within the heart. We analyzed the effect of an inducible cardiomyocyte-specific knockout of MAO-B (cmMAO-B KO) for the development of RVH and RVF in mice. Right ventricular hypertrophy was induced by pulmonary artery banding (PAB). RV dimensions and function were measured through echocardiography. ROS production (dihydroethidium staining), protein kinase activity (PamStation device), and systemic hemodynamics (in vivo catheterization) were assessed. A significant decrease in ROS formation was measured in cmMAO-B KO mice during PAB compared to Cre-negative littermates, which was associated with reduced activity of protein kinases involved in hypertrophic growth. In contrast to littermates in which the RV was dilated and hypertrophied following PAB, RV dimensions were unaffected in response to PAB in cmMAO-B KO mice, and no decline in RV systolic function otherwise seen in littermates during PAB was measured in cmMAO-B KO mice. In conclusion, cmMAO-B KO mice are protected against RV dilatation, hypertrophy, and dysfunction following RV pressure overload compared to littermates. These results support the hypothesis that cmMAO-B is a key player in causing RV hypertrophy and failure during PH. Full article

Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy and is associated with considerable early mortality. Heart transplantation is often the only viable life-saving option. Pulmonary artery banding (PAB) has been recently proposed as a bridge or alternative to transplantation for DCM. In our cohort, PAB was selectively addressed to heritable DCM or DCM with congenital left ventricle aneurysm (CLVA). This study aimed to describe the clinical evolution and left ventricle reverse remodeling (LVRR) over time (6 months and 1 year after surgery). Ten patients with severe DCM received PAB between 2016 and 2021 and underwent clinical and postoperative echocardiography follow-ups. The median age at PAB was <1 year. The in-hospital mortality was zero. Two patients died two months after PAB of end-stage heart failure. The modified Ross class was improved in the eight survivors with DCM and remained stable in the two patients with CLVA. We observed a positive LVRR (LV end-diastolic diameter Z-score: 8.4 ± 3.7 vs. 2.8 ± 3; p < 0.05; LV ejection fraction: 23.8 ± 5.8 to 44.5 ± 13.1 (p < 0.05)). PAB might be useful as part of the armamentarium available in infants and toddlers with severe DCM not sufficiently responding to medical treatment with limited probability of spontaneous recovery. Full article

Pulmonary hypertension (PH) is a progressive condition that affects the pulmonary vessels, but its main prognostic factor is the right ventricle (RV) function. Many mice/rat models are used for research in PAH, but results fail to translate to clinical trials. This study reviews studies that test interventions on pulmonary artery banding (PAB), a model of isolated RV disfunction, and PH models. Multiple tested drugs both improved pulmonary vascular hemodynamics in PH models and improved RV structure and function in PAB animals. PH models and PAB animals frequently exhibited similar results (73.1% concordance). Macitentan, sildenafil, and tadalafil improved most tested pathophysiological parameters in PH models, but almost none in PAB animals. Results are frequently not consistent with other studies, possibly due to the methodology, which greatly varied. Some research groups start treating the animals immediately, and others wait up to 4 weeks from model induction. Treatment duration and choice of anaesthetic are other important differences. This review shows that many drugs currently under research for PAH have a cardioprotective effect on animals that may translate to humans. However, a uniformization of methods may increase comparability between studies and, thus, improve translation to clinical trials. Full article

Hypoplastic left heart syndrome is a spectrum of complex congenital cardiac defects. Although in borderline cases, biventricular repair is a viable option, in the majority of cases, univentricular palliation is the treatment of choice. Hybrid palliation can be a valid alternative to classic Norwood operation in the neonatal period, especially in selected cases such as high-risk patients or borderline left ventricles. Echocardiography is the main diagnostic modality in this pediatric population, from the fetal diagnosis to the subsequent surgical steps of palliative treatment. Hybrid palliation is performed after birth and is characterized by surgical banding of the pulmonary arteries along with transcatheter stenting of the ductus arteriosus. There are some peculiar aspects of cardiac imaging that characterize this type of palliation, and that should be considered in the different phases before and after the procedure. We aimed to review the current literature about the role of echocardiography in the management of patients with hypoplastic left heart undergoing hybrid palliation. Full article

Positional abnormalities per se do not require treatment, but in their place, the accompanying pulmonary pathology in dextroposition patients and pathophysiologic hemodynamic abnormalities resulting from multiple defects in patients with cardiac malposition should be the focus of treatment. At the time of the first presentation, treating the pathophysiologic aberrations caused by the defect complex, whether it is by improving the pulmonary blood flow or restricting it, is the first step. Some patients with simpler or single defects are amenable to surgical or transcatheter therapy and should be treated accordingly. Other associated defects should also be treated appropriately. Biventricular or univentricular repair dependent on the patient’s cardiac structure should be planned. Complications in-between Fontan stages and after conclusion of Fontan surgery may occur and should be promptly diagnosed and addressed accordingly. Several other cardiac abnormalities unrelated to the initially identified heart defects may manifest in adulthood, and they should also be treated. Full article

Background: Cardiomyopathies (CMs) are a heterogeneous and severe group of diseases that shows a highly variable cardiac phenotype and an incidence of app. 1/100.000. Genetic screening of family members is not yet performed routinely. Patients and methods: Three families with dilated cardiomyopathy (DCM) and pathogenic variants in the troponin T2, Cardiac Type (TNNT2) gene were included. Pedigrees and clinical data of the patients were collected. The reported variants in the TNNT2 gene showed a high penetrance and a poor outcome, with 8 of 16 patients dying or receiving heart transplantation. The age of onset varied from the neonatal period to the age of 52. Acute heart failure and severe decompensation developed within a short period in some patients. Conclusion: Family screening of patients with DCM improves risk assessment, especially for individuals who are currently asymptomatic. Screening contributes to improved treatment by enabling practitioners to set appropriate control intervals and quickly begin interventional measures, such as heart failure medication or, in selected cases, pulmonary artery banding. Full article

Pediatric heart failure (HF) is an important clinical condition with high morbidity and mortality. Compared to adults, pediatric HF shows different etiologies characterized by different physiology, a different clinical course, and deeply different therapeutic approaches. In the last few years, new drugs have been developed and new therapeutic strategies have been proposed with the goal of identifying an individualized treatment regimen. The aim of this article is to review the new potential drugs and non-pharmacological therapies for pediatric heart failure in children. Full article

Current treatment paradigms for end-stage dilated cardiomyopathy (DCM) in children include heart transplantation and mechanical support devices. However, waitlist mortality, shortage of smaller donors, time-limited durability of grafts, and thrombo-hemorrhagic events affect long-term outcomes. Moreover, both these options are noncurative and cannot preserve the native heart function. Pulmonary artery banding (PAB) has been reinvented as a possible “regenerative surgery” to retrain the decompensated left ventricle in children with DCM. The rationale is to promote positive ventricular–ventricular interactions that result in recovery of left ventricular function in one out of two children, allowing transplantation delisting. Although promising, global experience with this technique is still limited, and several surgical centers are reluctant to adopt PAB since its exact biological bases remain unknown. In the present review, we summarize the clinical, functional, and molecular known and supposed working mechanisms of PAB in children with DCM. From its proven efficacy in the clinical setting, we described the macroscopic geometrical and functional changes in biventricular performance promoted by PAB. We finally speculated on the possible underlying molecular pathways recruited by PAB. An evidence-based explanation of the working mechanisms of PAB is still awaited to support wider adoption of this surgical option for pediatric heart failure. Full article