Search Results (32)

Functionally single-ventricle (FSV) defects are complex congenital heart anomalies that require Fontan palliation, a surgical procedure redirecting systemic venous blood directly to the pulmonary arteries, bypassing the heart. Despite improvements in surgical techniques and perioperative care leading to enhanced survival rates, patients remain vulnerable to significant long-term complications, due to the unique Fontan circulation physiology. This circulation relies on low pulmonary vascular resistance and preserved single-ventricle function but predisposes patients to venous congestion and reduced cardiac output, resulting in multi-organ dysfunction. Key cardiovascular complications include systolic and diastolic dysfunction of the single ventricle, atrioventricular valve regurgitation, arrhythmias, pulmonary vascular disease, and thromboembolic events. Systemic complications encompass Fontan-associated liver disease (FALD), protein-losing enteropathy (PLE), plastic bronchitis (PB), renal impairment, and endocrine and psychosocial burdens. All the problems induce frequent hospitalizations, psychological challenges, and impaired educational and employment opportunities. Comprehensive management requires multidisciplinary approaches addressing the complex interplay of hemodynamic, organ-specific problems, and psychosocial factors inherent to Fontan physiology. Full article

Mitral valve prolapse represents the most common cause of primary mitral regurgitation in Western countries and has traditionally been viewed as a disorder driven by valvular incompetence and chronic volume overload. Within this paradigm, left ventricular enlargement was expected to correlate with regurgitant severity. However, patients with myxomatous bileaflet prolapse often exhibit left ventricular dilatation disproportionate to the degree of regurgitation, leading to the hypothesis of an intrinsic myocardial disease process. Cardiovascular magnetic resonance imaging has challenged this concept through the identification of doming volume, a previously unrecognized systolic blood compartment located between the mitral annular plane and the ventricular surface of prolapsing leaflets. This volume is mechanically coupled to ventricular contraction and contributes to total ventricular volume load independently of transvalvular regurgitation. Recognition of doming volume provides a physiological explanation for excessive ventricular remodeling observed in bileaflet prolapse and Barlow disease. Doming volume has important implications for imaging assessment. Its common exclusion from echocardiographic volumetric measurements may result in underestimation of left ventricular end-systolic volume, overestimation of ejection fraction, and underestimation of regurgitant burden, contributing to discordance between echocardiographic and cardiovascular magnetic resonance-derived measurements. Cardiovascular magnetic resonance enables comprehensive assessment, allowing accurate quantification of ventricular volumes, mitral regurgitation severity, doming volume, and myocardial tissue characteristics. Integration of doming volume into the evaluation of mitral valve prolapse improves physiological consistency between imaging findings and ventricular remodeling. However, further evidence is required before doming volume assessment can be incorporated into operative clinical indications or decision-making thresholds. Full article

Increasing recognition of the clinical impact of isolated tricuspid regurgitation has led to rapid expansion of surgical and transcatheter tricuspid valve interventions. Given the close anatomic relationship between the tricuspid valve and the atrioventricular conduction system, both surgical and transcatheter approaches carry a significant risk of new conduction disturbances and permanent pacemaker implantation. A three-dimensional understanding of the atrioventricular conduction axis is essential to anticipate and mitigate these complications. This review provides a comprehensive overview of conduction system anatomy and physiology in the context of tricuspid valve interventions, highlighting the mechanisms underlying procedure-related conduction abnormalities. We also discuss contemporary management strategies, including approaches to pre-existing transvalvular leads, valve-sparing pacing alternatives, and the evolving role of electrophysiologists within the multidisciplinary heart team. Full article

Introduction: Hypertrophic cardiomyopathy (HCM) is a common myocardial disease worldwide and is associated with heart failure symptoms and sudden cardiac death. In a subset of patients, it may produce dynamic left ventricular outflow tract obstruction (LVOTO) and systolic anterior motion (SAM)-related mitral valve dysfunction through drag forces and altered mitral–septal geometry. In contrast, subaortic stenosis caused by a subaortic membrane is an uncommon congenital lesion that may lead to fixed subvalvular LVOTO in adulthood. The coexistence of these entities is rare and can substantially complicate diagnosis and management. Case presentation: A 51-year-old woman with HCM, paroxysmal atrial fibrillation, and heart failure presented with acute decompensation and cardiogenic shock. After initial hemodynamic stabilization and cardioversion for atrial fibrillation with rapid ventricular response, multimodality imaging with transthoracic and transesophageal echocardiography, coronary computed tomography angiography, and cardiac magnetic resonance demonstrated dual LVOTO, with a dynamic component related to HCM/SAM physiology and a fixed component caused by an elongated subaortic membrane, accompanied by severe SAM-related mitral regurgitation. Echocardiography showed a resting peak LVOT gradient of 49 mmHg, increasing to 85 mmHg with the Valsalva maneuver. After exclusion of obstructive coronary artery disease and evaluation for selected phenocopies, the patient underwent septal myectomy, subaortic membrane resection, and adjunctive mitral valve plication. Early postoperative echocardiography showed reduction in the maximum provoked LVOT gradient to 38 mmHg and improvement of mitral regurgitation from severe to mild. At 3-month follow-up, she remained in sinus rhythm, improved to New York Heart Association functional class II, and had no documented readmissions for heart failure. Conclusions: Combined fixed and dynamic LVOTO due to concomitant subaortic membrane and HCM is exceedingly rare. Accurate diagnosis requires a high index of suspicion and a multimodality imaging strategy to define the obstructive mechanisms and support mechanism-based surgical management and avoid incomplete treatment when a coexisting fixed lesion is present. Full article

Background/Objectives: This study compared the hemodynamic performance of fenestrated (FEVAR), branched (BEVAR), and chimney endovascular aortic aneurysm repair (chEVAR) in patients with complex aortic aneurysms. Methods: The pre- (native) and post-endovascular repair (endograft-defined) blood lumen was reconstructed from computed tomography angiographies of nine (9) elective patients treated with FEVAR (n = 3), BEVAR (n = 3), and chEVAR (n = 3). Computational fluid dynamics (CFD) simulations obtained blood flow properties. Velocity magnitude, wall shear stress (WSS), time-averaged wall shear stress (TAWSS), oscillatory shear index (OSI), relative residence time (RRT), and local normalized helicity (LNH) were computed at peak systole and mid-diastole. The hemodynamic data were statistically analyzed to evaluate correlations between FEVAR, BEVAR, and chEVAR, focusing on targeted visceral arteries. Results: Only slight differences were observed regarding RRT, OSI, and TAWSS between FEVAR and BEVAR, whereas the chEVAR group demonstrated a marked deviation from both. In FEVAR, the postoperative helical flow structures appeared more compact, while in BEVAR they were more developed and exhibited a more rotational configuration. The LNH of the visceral vessel patterns exhibited similar qualitative features across groups. Regarding TAWSS, higher values were found in BEVAR, whereas chEVAR showed the lowest. Conclusions: FEVAR, BEVAR, and chEVAR improved postoperative blood flow characteristics toward near-physiological conditions, reducing undesired flow patterns and recirculation zones. FEVAR showed more stable visceral flow, and BEVAR demonstrated higher flow rates and fewer recirculation zones, while chEVAR exhibited more streamlined visceral artery flow with reduced regurgitation at bridging stent entries. Despite variations, all approaches effectively preserved visceral artery perfusion. Full article

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

Mitral annular disjunction (MAD) is a structural abnormality of the mitral valve increasingly detected with advanced cardiac imaging, particularly cardiac magnetic resonance (CMR). However, the clinical impact of different degrees of disjunction and the lack of standardized measurement criteria remain controversial. This study aimed to describe a series of patients with MAD assessed by CMR and to discuss, in the context of current literature, potential cut-off values that may distinguish physiological from pathological MAD. We retrospectively identified all CMR examinations performed at our institution over a 6-month period in which MAD was visible in at least two cine steady-state free precession (SSFP) projections. For each patient, we recorded MAD extent, presence of mitral valve prolapse/regurgitation, late gadolinium enhancement (LGE) pattern, and main clinical presentation. Nine patients (mean age 57 years; 5 men) were included. Larger MAD distances (>4 mm) were frequently associated with non-ischemic LGE in the basal lateral wall and with valvular abnormalities, whereas smaller disjunctions (≤3 mm) were often observed in patients without significant structural disease. Non-ischemic LGE was present in 6/9 patients, all with MAD > 5 mm. These observations, together with published data, support the hypothesis that small degrees of MAD may represent a frequent anatomical variant, while more extensive disjunction, especially when associated with fibrosis, may indicate a pathological substrate for arrhythmias. Standardized CMR-based criteria and validated MAD cut-off values are needed to improve risk stratification and to incorporate MAD assessment into routine clinical practice. Full article

Background: This study aims to establish standardized reference values for myocardial T1 and T2 relaxation times in a clinically and imaging-defined real-world patient cohort, evaluating their variability in relation to age, sex, and comorbidities. By identifying key physiological and pathological influences, this investigation seeks to enhance CMR-based myocardial mapping for improved differentiation between normal and pathological myocardial conditions. Methods: This retrospective observational study analyzed T1 and T2 relaxation times using CMR at 1.5 Tesla in a cohort of 491 subjects. T1 and T2 times were measured using MOLLI and GRASE sequences, and statistical analyses assessed intra- and interindividual variations, including the influence of age, sex, and comorbidities, to establish reference values and improve myocardial tissue characterization. Results: T1 and T2 relaxation times were analyzed in 291 and 200 participants, respectively. The mean global T1 time was 1004.7 ± 49.8 ms, with no significant differences between age groups (p = 0.81) or sexes (p = 0.58). However, atrial fibrillation (AF) and mitral regurgitation (MR) were associated with significantly prolonged T1 times (p < 0.05). The mean global T2 time was 67.4 ± 8.6 ms, with age-related prolongation (p < 0.05), but no sex differences (p = 0.46). Comorbidities did not significantly influence T2 times, except for NYHA Class III–IV patients, who exhibited prolonged T2 values (p < 0.05). Conclusions: Standardized T1 and T2 reference values are essential to improve diagnostic accuracy and risk stratification in CMR-based myocardial tissue characterization. Future research should focus on multicenter validation, AI-driven analysis, and the development of age- and comorbidity-adjusted normative databases to enhance individualized cardiovascular care. Full article

Tricuspid regurgitation (TR) is a well-recognized factor contributing to adverse outcomes and mortality. Recent developments in transcatheter valve repair techniques, with the emergence of tricuspid transcatheter edge-to-edge repair (TEER) devices, have altered the treatment algorithm of TR and now offer a safe and feasible alternative for the effective management of the disease and an improvement in patient symptoms. Evidence from large studies and registries showcases the benefit of tricuspid interventions in terms of heart failure hospitalization and quality of life; however, most studies do not report a significant benefit in terms of hard outcomes. Even though longer-term follow-up may be needed to identify such differences, it is important to also identify distinct patient phenotypes that would benefit the most from such interventions, moving from pure anatomical criteria to an overall assessment of the patient’s clinical status. Therefore, the aim of this review is to provide updates on potential moderators of the effect of tricuspid TEER, focusing on novel anatomical criteria, right cardiac function, and renal physiology, in order to guide patient selection and provide an insightful discussion on the optimal patient phenotype for future trial design. Full article

Background/Objectives: Optimal feeding position may contribute to improving the quality and safety of bottle-feeding in premature infants. The aim of this study was to compare the advantages of right side-lying (R-SLP) and left side-lying (L-SLP) positioning during the bottle-feeding of preterm infants. Methods: The randomized study included eight neonates (n = 8) born at ≤34 weeks of gestational age (GA). Four bottle-feeding sessions were conducted for each newborn: two in the R-SLP and two in the L-SLP position. Levels of oxygen saturation (SpO₂) and heart rate (HR) were measured as indicators of physiological stability. The qualitative aspects of feeding included total time of SpO₂ declines to ≤85%, the newborn’s alertness level based on the Neonatal Behavioral Assessment Scale (NBAS), and the number of possetings, regurgitations, and choking episodes. The volume of milk consumed and the duration of each feeding session were also recorded. Results: The L-SLP position was characterized with higher SpO₂ (p = 0.042) at the 10th minute after feeding and lower HR (p = 0.022) at the end of feeding. Greater milk intake (p = 0.042), shorter feeding duration (p = 0.021), and shorter duration of SpO₂ declines to ≤85% (p = 0.025) were also observed in L-SLP. No differences were found in alertness level, or in the number of choking episodes, possetings, or regurgitations compared to R-SLP. Conclusions: This pilot study suggests the potential efficacy of the L-SLP position during bottle-feeding of premature infants. The results require the need for larger studies to confirm the potential benefits of using L-SLP. Full article

Background: Tricuspid regurgitation (TR) is increasingly recognized as a detectable finding during routine fetal echocardiography. Although previous studies have explored its potential role as an indirect marker for congenital heart disease (CHD) in the first trimester, the prognostic significance of isolated mild TR in chromosomally normal and low-risk fetuses during the second and third trimesters remains unclear. Clarifying the clinical relevance of this commonly encountered Doppler finding is essential to guide appropriate prenatal management and avoid unnecessary interventions in low-risk pregnancies. Materials and Methods: This retrospective study reviewed fetal echocardiography reports of 1592 pregnant women referred to a pediatric cardiology clinic after the 20th gestational week between 1 January 2024 and 1 January 2025. Following exclusion criteria, 1072 low-risk pregnancies were included. A total of 136 fetuses with TR were identified, and among them, postnatal echocardiographic outcomes of 60 neonates who underwent transthoracic echocardiography within the first 10 days after birth were analyzed. Results: Among the 1072 low-risk pregnancies included in the study, a total of 136 fetuses were diagnosed with TR on fetal echocardiography. The majority of these cases were characterized as mild and isolated, without accompanying structural abnormalities. Postnatal echocardiographic assessments revealed no major congenital cardiac anomalies, reinforcing the interpretation that isolated mild TR in the context of low-risk pregnancies represents a benign and likely transient physiological finding. Conclusion: Isolated mild TR, particularly in low-risk and chromosomally normal pregnancies, appears to be a transient and clinically insignificant finding. These results support the interpretation of fetal TR in the context of overall clinical and structural evaluation, helping to avoid unnecessary interventions and reduce parental anxiety. Full article

The co-evolution between plants and herbivorous insects has led to a continuous arms race on defense and anti-defense mechanisms. In this process, insect-derived effectors are crucial for suppressing plant defense. Despite considerable progress in plant–insect interaction studies, the functional role of heat shock cognate protein 70 (HSC70) as an effector in herbivorous insects remains poorly characterized. This study provides evidence that HSC70-3 functions as an effector in interactions between the cruciferous specialist diamondback moth (Plutella xylostella) and its host plant radish (Raphanus sativus ‘Nanpan Prefecture’). Using immunofluorescence labeling and in situ Western blot (WB), we demonstrated that HSC70-3 is secreted into plant wound sites through larval gut regurgitant during feeding. Short-term host transfer experiments revealed tissue-specific hsc70-3 expression changes, indicating a dynamic response to plant-derived challenges. These findings suggest hsc70-3 is differentially regulated at transcriptional and translational levels to facilitate insect adaptation to host plant shifts. Knockout of hsc70-3 using CRISPR/Cas9 technology significantly impaired larval growth, prolonged development duration, and reduced pupal weight on host plants, indicating its involvement in host adaptation. However, knockout mutants exhibited no significant developmental defects when reared on an artificial diet, suggesting that hsc70-3 primarily functions in modulating plant-induced defense responses rather than directly affecting insect physiology. Collectively, these findings provide evidence for the functional roles of HSC70-3 in P. xylostella and plant interactions, laying a foundation for further investigations into insect effectors and their mechanisms in modulating plant defense responses. Full article

Mitral valve regurgitation is among the most prevalent valvular heart diseases and increases with age. Percutaneous therapy has emerged for the management of mitral regurgitation in high surgical risk patients. However, the long-term consequences of these interventions are still not fully understood due to their novelty and the difficulty of developing a strategy specific to the patient’s anatomy and/or pathology. To optimize these outcomes, an in vitro patient-specific approach could provide important insights for the most suitable strategy to use according to the patient profile. To ensure the reliability of this in vitro approach, the aim of this study was to reproduce the physiological behavior of the healthy native mitral valve for future applications. To do so, different silicon combinations reproducing the physiological anatomy of a healthy mitral valve were developed and tested under physiological hemodynamic conditions in a cardiac simulator. The hemodynamic and biomechanical behaviors of each mitral valve model were analyzed and compared to the physiological values provided in the literature. This study identified EcoFlex 00-50 and DragonSkin 10 (Smooth-On Inc., Easton, PA, USA) as the optimal silicon combination resulting in physiological strain values and hemodynamic parameters. These findings could be useful for future patient-specific applications, helping in the optimization of percutaneous mitral valve therapy. Full article

Background and Objectives: Left ventricular aneurysm (LVA) causes geometric changes, including reduced systolic function and a more spherical shape, which is quantified by the sphericity index (SI), the ratio of the short to long axis in the apical four-chamber view. This study aimed to assess SI’s value in A-LVA and B-LVA, identify influencing factors, and evaluate its clinical relevance. Materials and Methods: This clinical study included 54 patients with post-infarction LVA and used echocardiography to determine LVA locations (A-LVA near the apex and B-LVA in the basal segments), with SI and other echocardiographic measures assessed in both systole and diastole for the entire cohort and stratified by A-LVA and B-LVA groups. Results: Among the 54 patients, 41 had A-LVA and 13 had B-LVA. The mean SI was 0.55 in diastole and 0.47 in systole for the cohort. Patients with A-LVA had a mean SI of 0.51 in diastole and 0.44 in systole, while B-LVA patients exhibited significantly higher SI values, with 0.65 in diastole and 0.57 in systole, due to lower long-axis (L) values in both phases. The mean left ventricular ejection fraction (EF) was 23.95% in A-LVA and 30.85% in B-LVA, with no significant difference. However, apical aneurysms were larger (greater LVAV and LVAA) and more significantly reduced functional myocardium. LVEDV, LVESV, LVEDA, and LVESA did not differ significantly between A-LVA and B-LVA. In cases of severe mitral regurgitation (MR), SI was notably higher (0.75 in diastole) due to a marked reduction in the L axis. Conclusions: SI is key in differentiating A-LVA and B-LVA on echocardiography. B-LVA has lower volume and area values, but similar aneurysm and left ventricular volumes and EF. Higher SI in B-LVA is due to a reduced L-axis, and is worsened by severe mitral regurgitation (MR). Surgical ventricular reconstruction (SVR) compensates for L-axis reduction, with preservation of the L axis critical for achieving a more physiological shape. SI thus serves as a marker for left ventricular geometry and surgical outcomes. Full article

Background: Papillary muscles are structures integrated into the mitral valve apparatus, having both electrical and mechanical roles. The importance of the papillary muscles (PM) is mainly related to cardiac arrhythmias and mitral regurgitation. The aim of this review is to offer an overview of the anatomy and physiology of the papillary muscles, along with their involvement in cardiovascular pathologies, including arrhythmia development in various conditions and their contribution to secondary mitral regurgitation. Methods: A literature search was performed on PubMed using the following relevant keywords: papillary muscles, mitral valve, arrhythmia, anatomy, and physiology. Results: During the cardiac cycle, papillary muscles have continuous dimensional and pressure changes. On one hand, their synchrony or dyssynchrony impacts the process of mitral valve opening and closure, and on the other hand, the pressure changes can trigger electrical instability. There is increased awareness of papillary muscles as an arrhythmic source. Arrhythmias arising from PM were found in patients with or without structural heart disease, via Purkinje fibres, due to increased automaticity or triggered activity. Conclusions: Despite the interest in mitral valve physiology, there are still many unknowns in relation to the papillary muscles, especially with regard to their role in arrhythmogenesis and the pathogenesis of mitral regurgitation. Full article