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Right ventricular outflow dysfunction, manifesting as stenosis, regurgitation, or both, is nearly universal in patients with repaired tetralogy of Fallot, precipitating a complex pathophysiological cascade that leads to increasing rates of morbidity and mortality with advancing age. As the number of adolescent and adult patients with repaired tetralogy of Fallot continues to grow as a result of excellent survival during infancy, the need to improve late outcomes has become an urgent priority. This American Heart Association scientific statement provides an update on the current state of knowledge of the pathophysiology, methods of surveillance, risk stratification, and latest available therapies, including transcatheter and surgical pulmonary valve replacement strategies, as well as management of life-threatening arrhythmias. It reviews emerging evidence on the roles of comorbidities and patient-reported outcomes and their impact on quality of life. In addition, this scientific statement explores contemporary evidence for clinical choices such as transcatheter or surgical pulmonary valve replacement, discusses criteria and options for intervention for failing implanted bioprosthetic pulmonary valves, and considers a new approach to determining optimal timing and indications for pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

In the last decades, the easy genetic manipulation, the external fertilization, the high percentage of homology with human genes and the reduced husbandry costs compared to rodents, made zebrafish a valid model for studying human diseases and for developing new therapeutical strategies. Since zebrafish shares with mammals the same bone cells and ossification types, it became widely used to dissect mechanisms and possible new therapeutic approaches in the field of common and rare bone diseases, such as osteoporosis and osteogenesis imperfecta (OI), respectively. OI is a heritable skeletal disorder caused by defects in gene encoding collagen I or proteins/enzymes necessary for collagen I synthesis and secretion. Nevertheless, OI patients can be also characterized by extraskeletal manifestations such as dentinogenesis imperfecta, muscle weakness, cardiac valve and pulmonary abnormalities and skin laxity. In this review, we provide an overview of the available zebrafish models for both dominant and recessive forms of OI. An updated description of all the main similarities and differences between zebrafish and mammal skeleton, muscle, heart and skin, will be also discussed. Finally, a list of high- and low-throughput techniques available to exploit both larvae and adult OI zebrafish models as unique tools for the discovery of new therapeutic approaches will be presented. [ABSTRACT FROM AUTHOR]

Right ventricular assist devices (RVADs) have been extensively used to provide hemodynamic support for patients with end-stage right heart (RV) failure. However, conventional in-parallel RVADs can lead to an elevation of pulmonary artery (PA) pressure, consequently increasing the right ventricular (RV) afterload, which is unfavorable for the relaxation of cardiac muscles and reduction of valve complications. The aim of this study is to investigate the hemodynamic effects of the pulsatile frequency of the RVAD on pulmonary artery. Firstly, a mathematical model incorporating heart, systemic circulation, pulmonary circulation, and RVAD is developed to simulate the cardiovascular system. Subsequently, the frequency characteristics of the pulmonary circulation system are analyzed, and the calculated results demonstrate that the pulsatile frequency of the RVAD has a substantive impact on the pulmonary artery pressure. Finally, to verify the analysis results, the hemodynamic effects of the pulsatile frequency of the RVAD on pulmonary artery are compared under diffident support modes. It is found that the pulmonary artery pressure decreases by approximately 6% when the pulsatile frequency changes from 1 to 3 Hz. The increased pulsatile frequency of RA-PA support mode may facilitate the opening of the pulmonary valve, while the RV-PA support mode can more effectively reduce the load of RV. This work provides a useful method to decrease the pulmonary artery pressure during the RVAD supports and may be beneficial for improving myocardial function in patients with end-stage right heart failure, especially those with pulmonary hypertension. [ABSTRACT FROM AUTHOR]

During the Ross procedure, an aortic heart valve is replaced by a patient's own pulmonary valve. The pulmonary autograft subsequently undergoes substantial growth and remodeling (G&R) due to its exposure to increased hemodynamic loads. In this study, we developed a homogenized constrained mixture model to understand the observed adaptation of the autograft leaflets in response to the changed hemodynamic environment. This model was based on the hypothesis that tissue G&R aims to preserve mechanical homeostasis for each tissue constituent. To model the Ross procedure, we simulated the exposure of a pulmonary valve to aortic pressure conditions and the subsequent G&R of the valve. Specifically, we investigated the effects of assuming either stress- or stretch-based mechanical homeostasis, the use of blood pressure control, and the effect of root dilation. With this model, we could explain different observations from published clinical studies, such as the increase in thickness, change in collagen organization, and change in tissue composition. In addition, we found that G&R based on stress-based homeostasis could better capture the observed changes in tissue composition than G&R based on stretch-based homeostasis, and that root dilation or blood pressure control can result in more leaflet elongation. Finally, our model demonstrated that successful adaptation can only occur when the mechanically induced tissue deposition is sufficiently larger than tissue degradation, such that leaflet thickening overrules leaflet dilation. In conclusion, our findings demonstrated that G&R based on mechanical homeostasis can capture the observed heart valve adaptation after the Ross procedure. Finally, this study presents a novel homogenized mixture model that can be used to investigate other cases of heart valve G&R as well. [ABSTRACT FROM AUTHOR]

The article in Clinical Genetics explores ABL1 deficiency syndromes, highlighting how mutations in different isoforms can lead to distinct diseases. The study focuses on a Lebanese consanguineous family with cardiac abnormalities, revealing a novel likely pathogenic variant in ABL1. The findings suggest that variants affecting different ABL1 isoforms may result in unique phenotypes, emphasizing the need for further research to fully understand ABL1-associated disorders. [Extracted from the article]

An 8‐h‐old Arabian foal was presented for evaluation of progressive weakness since birth. The foal presented laterally recumbent with pale and muddy mucous membranes, a grade III/VI left basilar murmur, and an irregularly, irregular rhythm. Transthoracic echocardiogram along with an electrocardiogram (EKG) was performed. Atrial fibrillation was recorded throughout the examination. The left and right atria were both significantly enlarged. A right to left shunt across the atrial septum was confirmed via an intravenous injection of agitated saline. The tricuspid valve was rudimentary without apparent atrioventricular flow and no ventricular septal defect was appreciated. The animal was euthanased, and necropsy found dysplasia of the tricuspid valve, a hypoplastic right ventricle with pulmonary valve atresia, and an intact ventricular septum. This condition is similar, both haemodynamically and in terms of prognosis, to tricuspid atresia, which has been described previously in Arabian foals, with the exception that there is the complete absence of connection between the atrium and ventricle in the case of tricuspid atresia. Of 14 previously reported cases of tricuspid atresia in neonatal foals, nine were Arabian foals. Further investigation into the possibility of hypoplastic right ventricle as a familial condition in the Arabian breed should be considered. [ABSTRACT FROM AUTHOR]

The Myval is a balloon-expandable transcatheter heart valve (THV) developed by Meril Life Sciences Pvt. Ltd. (Vapi, Gujarat, India) that has an innovative operator-friendly design that aids in improving deliverability and features precise deployment. Various clinical studies demonstrate its effectiveness and safety, making it a promising choice in valvular interventions. Myval has been successfully utilized as a transcatheter aortic valve implantation (TAVI) device in cases with conduction disturbances, bicuspid aortic valve anatomy, non-calcified aortic regurgitation, dysfunctional stenosed right ventricular outflow tract (RVOT) conduits, pulmonary valve replacement, mitral valve replacement, and valve-in-valve and valve-in-ring implantation procedures. Myval's diverse sizes are also of key importance in complex cases of large annuli and complex anatomy. Further long-term studies are needed to consolidate these results. Its introduction signifies a significant advancement in cardiology, aiming to enhance patient outcomes and quality of life. In the present review, we provide an update on new-generation Myval THV series and review the available clinical data published to date with an emphasis on diverse use in specific clinical scenarios. [ABSTRACT FROM AUTHOR]

Objectives: This study primarily aimed to compare the mechanical ventilation durations between pulmonary valve-sparing and transannular patch repair techniques in the surgical correction of Tetralogy of Fallot. Secondary objectives included comparison of demographic characteristics, cardiopulmonary bypass parameters, postoperative vasoactive inotrope requirements, incidence of cardiac conduction abnormalities, echocardiographic findings, intensive care unit and hospitalisation durations, reoperations rates, morbidity and mortality between the 2 approaches. Methods: This retrospective cohort study was conducted at the Royal Hospital, Muscat, Oman, between January 2016 and December 2019. This study included 102 paediatric patients who underwent complete surgical correction of Tetralogy of Fallot over 3 years, either by a pulmonary valve-sparing technique (Group 1, n = 43) or by transannular patch repair (Group 2, n = 59). Data for both primary and secondary outcomes were extracted from hospital records. Results: Mechanical ventilation duration was significantly shorter in Group 1 (P = 0.039). Patients in Group 1 were generally older, with shorter cardiopulmonary bypass and aortic clamp times, lower inotrope scores, and shorter chest tube retention, intensive care unit and hospitalisation periods. Junctional ectopic tachycardia and severe pulmonary regurgitation were significantly more common in Group 2, while right ventricular outflow tract peak pressure gradients were higher in Group 1. Multivariate analysis identified patient weight as the only independent predictor of mechanical ventilation duration. Conclusion: Pulmonary valve preservation was associated with better early outcomes, including reduced mechanical ventilation duration, lower vasoactive inotrope scores, decreased postoperative arrhythmias and shorter hospital stay. [ABSTRACT FROM AUTHOR]

OBJECTIVES The impact of residual pulmonary stenosis (rPS) or right ventricular (RV) outflow tract obstruction on prognosis after surgical pulmonary valve insertion) in repaired tetralogy of Fallot patients with pulmonary regurgitation (PR) remains controversial. rPS assessment is partially dependent on RV contractility. We investigated the impact of rPS according to RV ejection fraction (RVEF). METHODS In this multicentre, retrospective study, 117 repaired tetralogy of Fallot patients who underwent surgical pulmonary valve insertion for more than moderate PR between 2003 and 2021 were examined. Regarding rPS, the threshold for PR with rPS (PSR) and PR was 25 mmHg. For RVEF, the threshold for preserved RVEF (pEF) and reduced RVEF (rEF) was 40%. The patients were divided into 4 groups: patients with PR and pEF (PR-pEF) (n  = 48), those with PR and rEF (PR-rEF) (n  = 44), those with PSR and pEF (PSR-pEF) (n  = 16), and those with PSR and rEF (PSR-rEF) (n  = 9). Clinical parameters, postoperative adverse event rates and their associations were studied. RESULTS The 5-year freedom from adverse cardiovascular events was the highest in the PSR-pEF and the lowest in the PSR-rEF groups. The PSR-rEF group had the highest RV end-diastolic pressure (RVEDP) (12 ± 2.2 mmHg) (P = 0.006). From multivariable analysis, RVEDP was associated with postoperative adverse events (P = 0.016). RVEDP > 8 mmHg was associated with a lower freedom from adverse events. CONCLUSIONS The freedom from adverse events was the lowest in the PSR-rEF group, with the highest RVEDP, suggesting RV systolic and diastolic dysfunction. Reduced RVEF may mask the intrinsic degree of residual stenosis, delay surgical pulmonary valve insertion timing and increase adverse events. [ABSTRACT FROM AUTHOR]

Background: Atrial septal defect (ASD) is a prevalent congenital heart condition in adults, which finally leads to pulmonary hypertension and right heart failure if left untreated. Right heart catheterization (RHC), the current gold standard for determining ASD closure feasibility, is invasive. Thus, a noninvasive prescreening tool is urgently needed. Methods and Results: In a multicenter, retrospective study, we assessed 924 ASD patients (2012–2022) to determine their suitability for ASD closure. Using LASSO regression, we identified predictors for a correctable shunt, enabling us to create the ASD model. The ASD model, comprising of estimated pulmonary artery systolic pressure (ePASP), peak velocity through the pulmonary valve (PV), peak E‐wave velocity through the tricuspid valve (TVE), and right atrial longitudinal dimension (RA) by echocardiography, was constructed and exhibited favorable discriminative capability with an area under the curve (AUC) of 0.941 (95% CI: 0.920–0.961) in the derivation group. The model also demonstrated good calibration and discriminative abilities in the validation cohort. When juxtaposed with the earlier congenital heart disease (CHD) model, the newly developed ASD model demonstrated superior predictive capabilities for correctable shunt, supported by the net reclassification index (NRI) [0.063 (95% CI: 0.001–0.127, p = 0.047)] and integrated discrimination improvement (IDI) [0.023 (95% CI: 0.011–0.036, p < 0.001)]. Conclusion: In summary, our research advocates the ASD model as a superior tool for screening suitable ASD defect closure candidates. [ABSTRACT FROM AUTHOR]

Aim: Two‐dimensional phase‐contrast magnetic resonance imaging (2D flow MRI) and its multidimensional alternatives, 4D and 5D flow MRI, measure blood flow in the heart and great vessels. While 2D flow MRI is the standard technique, it has limitations regarding need for precise image plane prescribing and long scan time. In contrast, 4D and 5D flow MRI acquire 3D volumes, enabling retrospective assessment of all vessels. This review evaluates these three techniques for quantification of blood flow of the aortic and pulmonary valves in congenital heart disease. Methods: A systematic literature search was conducted in August 2024 using the PUBMED database, including articles comparing 2D, 4D, and 5D flow MRI. Results: Fifteen articles comparing 2D and 4D, one comparing 2D and 5D and three articles comparing 4D and 5D flow MRI were included. No study compared all three techniques. 2D, 4D and 5D flow MRI demonstrated a good agreement for flow quantification. 4D flow MRI, however, tends to present a better accuracy and internal consistency than 2D flow MRI for determination of peak velocities and flow in stenotic lesions, particularly when comparing velocities to echocardiography. 4D and 5D flow MRI are associated with shorter scan times than 2D flow MRI. Conclusions: 4D and 5D flow MRI appear to offer promising alternatives to 2D flow MRI with the advantage of reduced scan times. Larger and prospective studies including echocardiography are needed to evaluate the potential of 4D and 5D to replace 2D flow MRI for flow quantification and peak velocity determination. [ABSTRACT FROM AUTHOR]

The article discusses the recent recommendations from the International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) regarding the third-trimester obstetric ultrasound scan, focusing on the evaluation of the fetal heart. It suggests additional recommendations for clinicians implementing these guidelines, particularly in measuring the size of the fetal heart and assessing ventricular and outflow tract disproportion. The article provides detailed methods for measuring the fetal heart, calculating ratios, and identifying abnormalities associated with cardiomegaly and ventricular disproportion, offering calculators to aid in the evaluation process. [Extracted from the article]

Background The quadricuspid pulmonary valve (QPV) is a relatively rare heart congenital anomaly. It is usually asymptomatic and incidentally detected. Case summary A 52-year-old woman presented with paroxysmal palpitations and chest tightness after exertion. After a series of examinations, we finally diagnosed her with QPV and pulmonary hypertension. The symptoms have improved significantly with medications, and subsequently, the patient was discharged. Discussion This case demonstrated the crucial role of multimodality imaging in evaluating the non-invasive depiction of pulmonary valve disease. [ABSTRACT FROM AUTHOR]

Background: Volatile substance (thinner) addiction can cause serious cardiac events, such as malignant ventricular arrhythmias, acute coronary syndromes, sudden death syndrome, and dilated cardiomyopathy, as reported in many case studies. We aimed to find echocardiographic and electrocardiographic parameters that could foresee these adverse outcomes in clinical settings. Methods: We enrolled 32 healthy young adult patients with at least 1 year of thinner addiction and no cardiac symptoms. We also recruited a control group of 30 healthy individuals without any medical problems. Both groups received standard echocardiography and ECG tests. We analyzed the following echocardiographic parameters: LVEDd (left ventricular end‐diastolic diameter), LVESd (left ventricular end‐systolic diameter), mitral valve EF slope, E/A ratio, and aortic and pulmonary valve VTI (velocity time integral). We also measured the corrected (QTc), uncorrected QT intervals, and widest P‐wave values in the ECG. We used the SPSS 13 software for statistical analysis. Results: The echocardiographic findings did not differ significantly between the groups. However, the ECG results showed that the thinner addicts had higher values of corrected (QTc), uncorrected QT intervals, and widest P‐wave values than the control group, according to Mann–Whitney U and Student's T test. Conclusion: Corrected QT (QTc) and P‐wave duration are increased in individuals with a thinner addiction. These findings may suggest a higher risk of sudden cardiac death, atrial, and ventricular dysrhythmias in the future. [ABSTRACT FROM AUTHOR]

RASopathies are a group of genetic syndromes caused by germline mutations in genes involved in the RAS/Mitogen-Activated Protein Kinase signaling pathway, which regulates cellular proliferation, differentiation, and angiogenesis. Despite their involvement at different levels of this pathway, RASopathies share overlapping clinical phenotypes. Noonan syndrome is the most prevalent RASopathy, with an estimated incidence of 1 in 2500 live births, and it is typically inherited in an autosomal dominant manner, with 50% of cases involving gain-of-function mutations in the PTPN11 gene. De novo mutations are common, accounting for 60% of cases. The phenotype of Noonan syndrome includes characteristic facial and physical features, congenital cardiac defects, lymphatic and cerebrovascular anomalies, renal malformations, hematological abnormalities, developmental issues, and an increased risk of cancer. Severe congenital cardiac defects and lymphatic abnormalities significantly impact prognosis, contributing to increased morbidity and mortality. Recent therapeutic advancements have introduced trametinib, an MEK1/2 inhibitor, for treating Noonan syndrome patients with severe cardiac and lymphatic complications. To assess its efficacy, here, we present a case of a newborn with Noonan syndrome who exhibited refractory chylothorax, ventricular hypertrophy, and pulmonary stenosis who was treated with trametinib. The patient demonstrated significant improvement in chylothorax and left ventricular hypertrophy, though pulmonary stenosis persisted. This case further confirms trametinib's potential as a therapeutic option for severe Noonan syndrome complications, emphasizing the need for further clinical trials to optimize treatment protocols and evaluate long-term outcomes. [ABSTRACT FROM AUTHOR]

Objective: Multisystem inflammatory syndrome in children (MIS-C) is a critical childhood disease that is associated with coronavirus disease (COVID-19). We aimed to describe the clinical, laboratory, and echocardiographic characteristics and outcome of critical MIS-C cases in Egyptian children during the first wave of the COVID-19 pandemic. Design: A retrospective, observational study. Setting: A single-center tertiary pediatric intensive care unit (PICU).In Ain Shams university hospitals Cairo Egypt Methods: Children admitted to the PICU diagnosed with severe MIS-C as per the Centers for Disease Control's definition from June 23, 2020, to August 22, 2020, were included. Results: The patient's mean age was 7.45 (interquartile range [IQR], 4.23) years, and the cause of PICU admission was hypotension and shock. All patients had a fever for 4.8 (IQR, 2.5) days before shock developed. Overall, 68% had a gastrointestinal manifestation, and 55.6% had a rash. Thirty-five of 45 patients had ≥ 4 elevated inflammatory markers. The cardiac troponin I level was elevated in 35 of 45 patients. The most common cardiac condition was valvulitis (tricuspid regurgitation, 29/45; mitral valve regurgitation, 28/45; pulmonary valve regurgitation, 5/45; atrial valve regurgitation, 4/45). Twenty-one patients had an impaired ejection fraction < 50%, and 17 patients had coronary dilatation. Six patients had pericardial effusion, 1 patient had dilated pulmonary arteries, and 6 patients (13.3%) died of their associated comorbidities. The mean PICU length of stay among patients with no associated comorbidities was 7 days. Conclusions: Critical cases of MIS-C had a spectrum of different cardiac conditions, with valvulitis being the most common one. The worst outcome occurred in patients with comorbidities and infants. [ABSTRACT FROM AUTHOR]

The Journal of Clinical Medicine's editorial on structural cardiovascular interventions in 2024 highlights key insights from recent studies. The document discusses advancements in structural heart valve interventions, such as transcatheter aortic valve intervention (TAVI) and mitral and tricuspid transcatheter edge-to-edge repair (TEER). Notable studies include the NOTION trials on TAVI durability, the RHEIA trial showing TAVI superiority over SAVR in women, and the MATTERHORN trial demonstrating non-inferiority of mitral TEER to surgery. The document emphasizes the importance of original cohort studies and real-world data in complementing randomized clinical trials in the field of cardiovascular interventions. [Extracted from the article]

Heart valve tissue engineering holds the potential to transform the surgical management of congenital heart defects affecting the pediatric pulmonary valve (PV) by offering a viable valve replacement. While aiming to recapitulate the native valve, the minimum requirement for tissue engineered heart valves (TEHVs) has historically been adequate mechanical function at implantation. However, long-term in situ functionality of TEHVs remains elusive, suggesting that a closer approximation of the native valve is required. The realization of biomimetic engineered pediatric PV is impeded by insufficient characterization of healthy pediatric tissue. In this study, we comprehensively characterized the planar biaxial tensile behaviour, extracellular matrix (ECM) composition and organization, and valvular interstitial cell (VIC) phenotypes of PVs from piglets to provide benchmarks for TEHVs. The piglet PV possessed an anisotropic and non-linear tension-strain profile from which material constants for a predictive constitutive model were derived. The ECM of the piglet PV possessed a trilayer organization populated by collagen, glycosaminoglycans, and elastin. Biochemical quantification of ECM content normalized to wet weight and DNA content of PV tissue revealed homogeneous distribution across sampled regions of the leaflet. Finally, VICs in the piglet PV were primarily quiescent vimentin-expressing fibroblasts, with a small proportion of activated α-smooth muscle actin-expressing myofibroblasts. Overall, piglet PV properties were consistent with those reported anecdotally for pediatric human PVs and distinct from those of adult porcine and human PVs, supporting the utility of the properties determined here to inform the design of tissue engineered pediatric PVs. Heart valve tissue engineering has the potential to transform treatment for children born with defective pulmonary valves by providing living replacement tissue that can grow with the child. The design of tissue engineered heart valves is best informed by native valve properties, but native pediatric pulmonary valves have not been fully described to date. Here, we provide comprehensive characterization of the planar biaxial tensile behaviour, extracellular matrix composition and organization, and valvular interstitial cell phenotypes of pulmonary valves from piglets as a model for the native human pediatric valve. Together, these findings provide standards that inform engineered heart valve design towards generation of biomimetic pediatric pulmonary valves. [Display omitted] [ABSTRACT FROM AUTHOR]

Large-scale analyses of surgical outcomes after surgical pulmonary valve replacement (sPVR) as part of re-do surgery in adults with congenital heart disease (ACHD) are rare. Therefore, we present our outcomes of sPVR in ACHD patients over the last decade and demonstrate our standardized surgical approach. All ACHD patients who underwent sPVR between January 2013 and August 2022 were included. Primary diagnoses, peri-operative data, post-operative echocardiography, pre- and post-operative RV MRI and in-hospital mortality were examined. Pre- and postoperative MRI parameters were compared using paired testing. Standardized surgery was documented. Normality of continuous variables was tested using Shapiro-Wilk test. 79 patients (male 59.5% (n = 47), 71 re-operations (89.9%)) at a median age of 41.7 (52.2–28.8) years were included. Main underlying disease was Tetralogy of Fallot (TOF; n = 47, 59.5%). After removal of degenerated valve/conduit parts, right ventricular outflow tract (RVOT) patch augmentation and implantation of a larger stented bioprosthesis (25mm in 78.5%) were conducted. In 57% of cases, concomitant surgery was performed (mainly tricuspid valve surgery: n = 28, 35.4%). 25 patients (31.6%) were operated with beating heart technique. Echocardiographic outcomes showed no moderate or severe insufficiency (median Vmax of 2 m/s (2.3–1.77 m/s)) upon discharge. Available MRI data showed significantly lower indexed RV-EDV (p = 0.0006) and RV-ESV (P = 0.0017) after surgery. In-hospital mortality was 5.1% (n = 4). SPVR is a safe therapeutic option with low surgical risk and satisfying post-operative results. It can serve as a solid therapeutic option for patients who need future valve-in-valve interventions. [ABSTRACT FROM AUTHOR]

Background: Tetralogy of Fallot with an absent pulmonary valve is a very rare variant of tetralogy. It is characterized by absent valve tissue, severe pulmonary regurgitation, and secondary aneurysmal dilatation of the pulmonary arteries. Aim: In this study, we aim to investigate the clinical presentations, management strategies, and outcomes of patients with tetralogy of Fallot and absent pulmonary valve. Methodology: We retrospectively reviewed the charts of all patients who presented to the American University of Beirut Medical Center between January 2010 and December 2020 and who were diagnosed with this anomaly. Results: A total of 300 cases of tetralogy of Fallot were identified, of which 18 patients had absent pulmonary valves. They were followed up for an average of 8.2 years. Prenatal diagnoses were made in four patients, while 13 patients were identified in the neonatal period, with an average age of 4.5 days. Genetic testing confirmed DiGeorge syndrome in one patient. Five patients underwent surgical intervention in the neonatal period, while the remaining patients were operated on during their early childhood. While overall there were no surgical mortalities nor any need for reinterventions, a variety of morbidities were encountered. Conclusion: This study provides an overview of this rare anomaly and its management in a developing country. [ABSTRACT FROM AUTHOR]

Various surgical approaches address complex heart disease with arch anomalies. Bilateral pulmonary artery banding (bPAB) is a strategy for critically ill patients with complex arch anomalies. Some reports argued the potential effect of bPAB on the growth of the left ventricular outflow tract (LVOT) during inter-stage after bPAB. This study aimed to analyze the LVOT growth for biventricular repair candidates with arch anomaly and systemic ventricular outflow tract (SVOT) for univentricular repair candidates with arch anomaly. This retrospective study analyzed 17 patients undergoing initial bPAB followed by arch repair. The Z-scores of LVOT and SVOT were compared between pre-bPAB and pre-arch repair. Patient characteristics, transthoracic echocardiogram data, and PAB circumferences were reviewed. The diameter of the minimum LVOT for biventricular repair (BVR) candidates, the pulmonary valve (neo-aortic valve, neo-AoV) and the pulmonary trunk (the neo-ascending aorta, neo-AAo) for univentricular repair (UVR) candidates, and the degree of aortic or neo-aortic insufficiency in each candidate was statistically analyzed. 17 patients were divided into the UVR candidates (group U) with 9 patients and the BVR candidates (group B) with 8 patients. In group B, the median value of the Z-score of the minimum LVOT increased from -3.2 (range: − 4.1 ~ − 1.0) at pre-PAB to -2.8 (range: − 3.6 ~ − 0.3) at pre-arch repair with a significant difference (p = 0.012). In group U, the median value of the Z-score of the neo-AoV increased from 0.5 (range: − 1.0 ~ 1.7) at pre-bPAB to 1.2 (range: 0.2 ~ 1.9) at pre-arch repair with a significant difference (p < 0.01). The median value of the Z-score of the neo-AAo was also increased from 3.1 (range: 1.5 ~ 4.6) to 4.3 (range: 3.1 ~ 5.9) with a significant difference (p = 0.028). The growth of the LVOT for BVR candidates and SVOT for UVR candidates during the inter-stage between bPAB and arch repair was observed. These results suggest the potential advantage of bPAB in surgical strategies. Further research is needed to validate these findings and refine surgical approaches. [ABSTRACT FROM AUTHOR]

Background: The ductus arteriosus (DA) is critical in maintaining postnatal circulation in neonates with obstructed systemic circulation (OSC) and pulmonary circulation (OPC). We hypothesized that the size of the DA and aortic isthmus (AoI) undergoes adaptive growth in utero to counteract the hemodynamic challenges in these congenital heart diseases (CHD). Methods: Postnatal echocardiograms of neonates diagnosed prenatally with ductal-dependent CHD who were started on prostaglandins within 24 h of birth were reviewed. We assessed the cross-sectional area of the aortic valve opening, pulmonary valve opening, AoI, and DA by calculating (diameter)2/body surface area. Neonates were classified into OSC or OPC then subgrouped depending upon the patency of semilunar valves: OSC with and without aortic atresia (OSC-AA and OSC-nAA, respectively) and OPC with and without pulmonary atresia (OPC-PA and OPC-nPA, respectively). Results: Ninety-four cases were studied. The DA in OSC was significantly larger than OPC, and the DA in OSC-AA was significantly larger than OSC-nAA. The size of the AoI was significantly larger in OPC than OSC and larger in OSC-AA than OSC-nAA. Within the OSC-nAA group, there was no significant difference in the size of the DA, AoI, or pulmonary valve opening between those with retrograde flow (RF) at the AoI and without (nRF) except the aortic valve opening was significantly larger in nRF. All groups had comparable cross-sectional areas of systemic output. Conclusions: Our findings suggest that DA and AoI show compensatory growth to maintain critical blood flow to vital organs against primary anatomical abnormalities in ductus-dependent CHD. (249 words) [ABSTRACT FROM AUTHOR]

Introduction: Delivery systems are crucially important for the implantation of medical devices in patients with congenital heart disease. However, very little data is available comparing the advantages and disadvantages of the various delivery systems. Areas covered: This article describes the delivery systems and methods used for delivery of atrial septal occluder devices, ventricular septal occluder devices, devices to occlude patent arterial ducts, and transcatheter pulmonary valves. Delivery systems are compared relating to prepping and loading, positioning of the delivery sheath/catheter, deployment, ability to recapture and reposition, as well as device release. Expert opinion: For most ASD/VSD/PDA occluder devices, the basic delivery mechanism has changed very little over the preceding 20 years. Future modifications may focus on meaningful modifications to the cable systems that reduce stiffness and improve angulation at the connection to the device. Over the next 5–10 years, it is expected to see significant changes to delivery systems used for transcatheter pulmonary valve implantation, that result in improvements in the ability to recapture and reposition self-expandable transcatheter valves during the deployment process, combined with kink resistant sheaths that facilitate easy tracking across often complex right ventricular outflow tracts. [ABSTRACT FROM AUTHOR]

Premature ventricular contractions (PVCs) are a common arrhythmia characterized by ectopic excitations within the ventricles. Accurately estimating the ablation site using an electrocardiogram (ECG) is crucial for the initial classification of PVC origins, typically focusing on the right and left ventricular outflow tracts. However, finer classification, specifically identifying the left cusp (LC), anterior cusp (AC), and right cusp (RC), is essential for detailed preoperative planning. This study aims to improve the accuracy of cardiac waveform source estimation and classification in 27 patients with PVCs originating from the pulmonary valve. We utilized an anatomical human model and electromagnetic simulations to estimate wave source positions from 12-lead ECG data. Time-series source points were identified for each measured ECG waveform, focusing on the moment when the distance between the estimated wave source and the pulmonary valve was minimal. Computational analysis revealed that the distance between the estimated wave source and the pulmonary valve was reduced to less than 1 cm, with LC localization achieving errors under 5 mm. Additionally, 74.1% of the subjects were accurately classified into the correct origin (LC, AC, or RC), with each origin demonstrating the highest percentage of subjects corresponding to the targeted excitation origin. Our findings underscore the novel potential of this source localization method as a valuable complement to traditional waveform classification, offering enhanced diagnostic precision and improved preoperative planning for PVC ablation procedures. [ABSTRACT FROM AUTHOR]

A common feature of congenital heart disease is the presence of right ventricular outflow tract (RVOT) obstruction that can range from mild to severe and can lead to atresia of the pulmonary valve, in extreme conditions. RVOT abnormalities can frequently be corrected surgically or via interventional means. However, most of these patients will ultimately develop pulmonary valve insufficiency and eventual right ventricular dilation, which will require a pulmonary valve replacement at some point in their life to mitigate the detrimental effects of pulmonary valve regurgitation (PVR) on the right ventricle (RV). The evolution from the studies done by Philip Bonhoeffer to implant a pulmonary valve via transcatheter means, have provided a bedrock for transcatheter pulmonary valve replacement (TPVR). Yet, several areas of unmet need for a demographic of patients still exist. Here, we discuss the clinical unmet needs in children under 20 Kg and expand the use of hybrid and other TPVR approaches along with the current indications and contraindications for pulmonary valve replacement. The constraints and limitations from commercially available pulmonary valves will be discussed from a clinical standpoint. Finally, we explore the use of hybrid and periventricular delivery of transcatheter pulmonary valves in younger patients.

Pulmonary sinus aneurysm (PSA) is an abnormal dilatation of the sinus of the pulmonary valve. This condition is exceedingly rare. A few reports of pulmonary artery aneurysms and just 2 reports of pulmonary sinus aneurysms have been reported in the literature. Various factors contribute to pulmonary artery aneurysms, including pulmonary valve stenosis, pulmonary hypertension, and other diseases that induce arterial wall debilitation, such as connective tissue disorders, vasculitis, rheumatologic maladies, and infections. It can present with some symptoms linked with other congenital anomalies or remain asymptomatic. Recently, we encountered a case of asymptomatic pulmonary sinus aneurysm in neonatal. Echocardiography showed that the aneurysm of the anterior and right cusps of the pulmonary valve mimicked tumors at the pulmonary root.

Decellularized grafts have shown promising results in tissue engineering. The aim of decellularization is to create a scaffold that is devoid of immunogenic components, has natural tissue architecture, and can provide cellularity again. The aim of our study is to obtain a living/regenerative valve by coculture of HUVEC and human dermal fibroblast cells on the pulmonary heart valves of decellularized young sheep and subsequently perform their in vitro characterization to determine its suitability for clinical studies. Various characterization tests were applied to sheep pulmonary heart valves decellularized by the detergent-based method. HUVEC and dermal fibroblast cells were seeded on the scaffold, and their viability was determined by MTT analysis, adhesion by SEM, and cell infiltration by histological staining. Finally, to determine the regenerative ability of the resulting live cap, collagen type I (Col1), collagen type III (Col3), and elastin (Eln) gene expressions were analyzed by PCR. The results showed that cell proliferation increased day by day on the scaffold. In histological findings, it was observed that cellular regeneration was almost completely achieved, especially in arterial wall samples. According to PCR findings, a significant increase in Col1, Col3, and Eln gene expressions was observed in arterial wall samples. In this study, for the first time in the literature, the regeneration potential of differentiated human cells on decellularized sheep heart valves was observed, and markers related to new ECM production were analyzed. In conclusion, we suggest that the decellularized heart valves of young sheep can be used as a starting matrix in tissue engineering studies, and clinical studies are needed. [ABSTRACT FROM AUTHOR]

Cardiac catheterization for congenital heart disease (CHD) performed under fluoroscopic guidance still lacks definition and requires exposure to ionizing radiation and contrast agents, with most patients needing multiple procedures through their lifetime, leading to cumulative radiation risks. While fusion overlay techniques have been employed in the past to aid, these have been limited to a single plane, while interventions are traditionally performed under biplane fluoroscopy. We describe our initial experience performing cardiac catheterizations guided by an enhanced biplane GuideCCI system© (Siemens Healthcare, Germany) augmented by 3D magnetic resonance imaging and computed tomography modeling. Twenty-one children and young adults with CHD undergoing catheterization procedures between October 2019 and May 2021 were chosen based on their degree of complexity of cardiac anatomy. 3D stereolithography models were generated, overlayed, and displayed in real time, alongside angiographs in both planes on the screen during these procedures. We report successful implementation of this novel technology for performance of 26 interventions including stent placements, balloon dilations, vessel occlusion and percutaneous valve and transvenous pacemaker implantation all in patients with various complex cardiac anatomies. A statistically significant reduction in radiation and contrast use was noted for coarctation of the aorta stent angioplasty and transcatheter pulmonary valve replacement when compared with national benchmarks and local institutional metrics (with and without single plane overlay). No complications were encountered with the use of this technology. Use of a tracheal registration technique provided very good correlation in most cases. Operators preferred using biplane augmented catheterization over traditional fluoroscopy in patients with complex cardiac anatomy undergoing interventions. [ABSTRACT FROM AUTHOR]

Background: Abiotrophia (ABI) and Granulicatella (GRA) are rare causative pathogens in infective endocarditis (IE). This study aims to describe the epidemiology, clinical characteristics, and outcome of ABI/GRA-IE. The main features of ABI/GRA-IE were compared with Viridans group streptococci (VGS) IE. Methods: From January 2015 to December 2023, a total of 1531 definite IE in Zhongshan Hospital, Fudan University, Shanghai, China were retrospectively enrolled in this study. Clinical and laboratory data were collected. Results: Forty-five ABI/GRA-IE cases were identified, representing 2.9% of all IE cases in Zhongshan Hospital between 2015 and 2023, compared to 20.1% of VGS-IE. ABI and GRA IE shared similar clinical characteristics. Congenital valvulopathy was reported in 21 (46.7%) ABI/GRA-IE and 85 (28.8%) VGS-IE (P = 0.025). Pulmonary valve was more frequently affected in ABI/GRA-IE (6 [13.3%]) than VGS-IE (7 [2.4%]) (P = 0.002). Congestive heart failure was observed in 30 (66.7%) ABI/GRA-IE and 103 (34.9%) VGS-IE (P < 0.001). Systemic embolization excluding central nervous system (CNS) occurred in 13 (28.9%) ABI/GRA-IE and 39 (13.2%) VGS-IE (P = 0.012). In-hospital mortality was reported as 4.4% in ABI/GRA-IE and 3.7% in VGS-IE (P = 0.854). Conclusion: GRA/ABI-IE was approximately one-seventh as prevalent as VGS-IE. Congestive heart failure and systemic embolization (excluding CNS) were more frequent in GRA/ABI-IE compared to VGS-IE. Mortality of ABI/GRA-IE in this study was comparable to that of VGS-IE and lower than previously reported results. [ABSTRACT FROM AUTHOR]

Background: Cardiac symptoms are a characteristic feature of chronic obstructive pulmonary disease (COPD), a disorder with lately achieved recognition as a systemic illness according to established standards. The research's objective was aimed at investigating the transthoracic echocardiography role in assessing right ventricular (RV) dysfunction among individuals diagnosed with COPD, as well as to examine the potential correlation between blood pro B-type natriuretic peptide (BNP) concentrations + and this diagnostic procedure. Methods: Arterial blood gasses, spirometry, echocardiography, and serum pro BNP were done for 80 COPD patients and 20 healthy smokers, and some echocardiographic parameters were correlated with degree of airflow limitation. Results: 77.5% of COPD cases exhibited pulmonary hypertension (PHTN), and on classifying our COPD patients, it was found that 65% of patients in group II had PHTN and 90% of patients in group III had PHTN. Systolic pulmonary artery pressure (SPAP), mean pulmonary arterial pressure (MPAP), right ventricular mid diameter (RVMD), right ventricular basal diameter (RVBD), right ventricular longitudinal diameter (RVLD), right ventricular outflow tract (RVOT) above pulmonary and aortic valve, and tricuspid annular plane systolic excursion (TAPSE) along with RVEF exhibited a significant variance among all groups. A negative correlation was observed among forced expiratory volume (FEV) 1% and (RVMD as well as RVBD) and between Pro BNP and (TAPSE and RVEF). Conclusions: PHTN was found in 77.5% of COPD patients and is negatively correlated with FEV1%. Serum pro BNP level exhibits a negative correlation with FEV1% in stable COPD patients. [ABSTRACT FROM AUTHOR]

Background: Branch pulmonary artery (PA) stenosis is one of the most common indications for percutaneous interventions in patients with transposition of the great arteries (TGA), tetralogy of Fallot (ToF), and truncus arteriosus (TA). However, the effects of percutaneous branch PA interventions on exercise capacity remains largely unknown. In addition, there is no consensus about the optimal timing of the intervention for asymptomatic patients according to international guidelines. This trial aims to identify the effects of percutaneous interventions for branch PA stenosis on exercise capacity in patients with TGA, ToF, and TA. In addition, it aims to assess the effects on RV function and to define early markers for RV adaptation and RV dysfunction to improve timing of these interventions. Methods: This is a randomized multicenter interventional trial. TGA, ToF, and TA patients ≥ 8 years with a class IIa indication for percutaneous branch PA intervention according to international guidelines are eligible to participate. Patients will be randomized into the intervention group or the control group (conservative management for 6 months). All patients will undergo transthoracic echocardiography, cardiac magnetic resonance (CMR) imaging, and cardiopulmonary exercise testing at baseline, 6 months, and 2–4 years follow-up. Quality of life (QoL) questionnaires will be obtained at baseline, 2 weeks post intervention or a similar range for the control group, and 6 months follow-up. The primary outcome is exercise capacity expressed as maximum oxygen uptake (peak VO2 as percentage of predicted). A total of 56 patients (intervention group n = 28, control group n = 28) is required to demonstrate a 14% increase in maximum oxygen uptake (peak VO2 as percentage of predicted) in the interventional group compared to the control group (power 80%, overall type 1 error controlled at 5%). Secondary outcomes include various parameters for RV systolic function, RV functionality, RV remodeling, procedural success, complications, lung perfusion, and QoL. Discussion: This trial will investigate the effects of percutaneous branch PA interventions on exercise capacity in patients with TGA, ToF, and TA and will identify early markers for RV adaptation and RV dysfunction to improve timing of the interventions. Trial registration: ClinicalTrials.gov NCT05809310. Registered on March 15, 2023. [ABSTRACT FROM AUTHOR]

The study investigated the relationship between the histological compositions of the tricuspid, pulmonary, mitral, and aortic valves, and age. All 85 fresh human hearts were obtained with an age range between 20 and 90 years. The central area of the valves was conducted to analyze the density of collagen and elastic fibers by using an image analysis program. Neural network function in MATLAB was used for classification data and accuracy test of the age predictive model. Overall, a gradual increase in the density of collagen and elastic fibers was demonstrated with age in all valve types. The pulmonary valve cusps had the least density of collagen and elastic contents, whereas the most dense of collagen was found in the mitral leaflets. A similarity was noted for the elastic fibers in the tricuspid, mitral, and aortic valves. The highest correlation between the collagen (r = 0.629) and elastic fibers (r = 0.713) and age was found in the noncoronary cusp of the aortic valve. The established predictive equations using collagen and elastic fibers in the noncoronary cusp provided the standard error of ± 14.0 and 12.5 years, respectively. A 60.9% of accuracy was found in all age groups using collagen, while accuracy in elastic fibers showed 70.0% in the classification process using the neural networks. The current study provided additional data regarding age-associated changes of collagen and elastic fibers in the human heart valves in Thais and the benefits and application in age forensic identification. [ABSTRACT FROM AUTHOR]

Pulmonary valve replacement (PVR) consists of substituting a patient's original valve with a prosthetic one, primarily addressing pulmonary valve insufficiency, which is crucially relevant in Tetralogy of Fallot repairment. While extensive clinical and computational literature on aortic and mitral valve replacements is available, PVR's post‐procedural haemodynamics in the pulmonary artery and the impact of prosthetic valve dynamics remain significantly understudied. Addressing this gap, we introduce a reduced Fluid–Structure Interaction (rFSI) model, applied for the first time to the pulmonary valve. This model couples a three‐dimensional computational representation of pulmonary artery haemodynamics with a one‐degree‐of‐freedom model to account for valve structural mechanics. Through this approach, we analyse patient‐specific haemodynamics pre and post PVR. Patient‐specific geometries, reconstructed from CT scans, are virtually equipped with a template valve geometry. Boundary conditions for the model are established using a lumped‐parameter model, fine‐tuned based on clinical patient data. Our model accurately reproduces patient‐specific haemodynamic changes across different scenarios: pre‐PVR, six months post‐PVR, and a follow‐up condition after a decade. It effectively demonstrates the impact of valve implantation on sustaining the diastolic pressure gradient across the valve. The numerical results indicate that our valve model is able to reproduce overall physiological and/or pathological conditions, as preliminary assessed on two different patients. This promising approach provides insights into post‐PVR haemodynamics and prosthetic valve effects, shedding light on potential implications for patient‐specific outcomes. [ABSTRACT FROM AUTHOR]

Background: Percutaneous pulmonary valve implantation (PPVI) is a recognized alternative treatment to surgery for patients with dysfunctional right ventricular outflow tracts. Patient selection is essential to avoid serious complications from attempted treatment, such as rupture or dissection, especially of the calcified outflow tracts. We describe a case with an unexpected rupture of a calcified homograft valve and main pulmonary artery, which was treated successfully by emergency implantation of a self-expanding Venus P-Valve (Venus MedTech, Hangzhou, China) without the need for pre-stenting with a covered stent. Case details: A 13-year-old boy had two previous operations of tetralogy of Fallot, one a total repair and the other a homograft valved conduit for pulmonary regurgitation. He presented with dyspnea and severe right ventricular outflow tract obstruction (RVOTO) and had a calcified outflow tract and main pulmonary artery. In the catheter laboratory, a non-compliant balloon dilation resulted in a contained rupture of the conduit. The patient remained hemodynamically stable, and the rupture was treated with a self-expandable Venus P-Valve without the need for a covered stent combined with a balloon-expandable valve or a further surgical procedure. Discussion: Preprocedural evaluation with an inflating balloon is necessary to examine tissue compliance and determine suitability for PPVI. However, this condition is accompanied by a risk of conduit rupture. Risk factors of this complication are calcification and homograft use. These ruptures are mostly controlled with a prophylactic or therapeutic covered stent, with a low rate of requiring surgery. However, there are severe ruptures which lead to hemothorax and death. In the available literature, there was no similar reported case of conduit rupture, which a self-expandable Pulmonary valve stent has managed. It seems that fibrosis and collagen tissue around the heart, formed after open surgeries, can contribute to the control of bleeding in these cases. Conclusion (clinical Learning Point): The suitability of patients for the PPVI procedure should be examined more carefully, specifically patients with homograft and calcification in their conduit. Furthermore, conduit rupture might be manageable with self-expandable artificial pulmonary valves, specifically in previously operated patients, and the applicability of this hypothesis is worth examining in future research. [ABSTRACT FROM AUTHOR]

Background: The application of z-scores in normalizing the cardiac size function and structural dimension will be of immense benefit to the clinician, especially in evaluating children with cardiac anomalies. However, heterogeneity in the obtained z- score results is high, thus a subgroup analysis by region (or continent) to assist healthcare practitioners is necessary. Objectives: The review aimed to ascertain the overall mean z-scores for cardiac structures and function. Methods: A thorough search of several databases, EMBASE, PubMed/MEDLINE, and Google Scholar was made. Articles published between January 1999 and December 2023 were recruited, of which the last search was done in December 2023. Keywords used in the search were "z-scores", Children; echocardiography; cardiac structures; cardiac function; and body surface area (BSA)". We restricted our search to children. Besides, additional relevant articles were manually searched. PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) was used to highlight selected studies using a pre-defined search protocol. The I2 statistics were used to ascertain statistical heterogeneity. Results: Two hundred and forty citations were identified in our search strategy, of which a total of 34 studies were identified. Twenty-four were excluded from the thirty-four studies. A total of 11 studies met our inclusion criteria shown in the PRISMA. Apart from different z scores reading obtained from various countries and regions, some authors focused on few cardiac parameters while others were exhaustive. The mean z-scores of the cardiac structures from various countries/regions range as follows; The range of Z scores obtained by different studies and regions above are as follows; MV;-1.62-0.7 AV: -1.8 -0.5 TV: -2.71 -0.7; PV ; -1.52- (-0.99) MPA; -1-81 -0.8 LPA;-1.07-0.4; RPA;-0.92- 0.1 IVSD; -0.1.77–1.89 LVPWD; -0.12-1.5 LVPWS; -0.1-0.15 LVPWS; 0.03–0.18 LVIDD; -1.13- (-0.98) LVIDS; -0.84-10.3 respectively. The mean z-score from the pooled studies showed mitral valve diameter as -0.24 ± 0.9 and pulmonary valve annuls as -1.10 ± 0.3. The left ventricular end diastolic diameter is -0.93 ± 0.3 while the left ventricular end systolic diameter is -0.05 ± 0.5. The total pooled sample size of the eleven included studies was 9074 and the mean at 95% interval was 824.9 ± 537.344. The pooled mean is presented under the model of the Mean raw (MRAW) column. The heterogeneity discovered among the selected studies was statistically significant. Conclusion: Due to heterogeneity involved in the reportage of the z-scores of cardiac structures and function, it may be necessary for every region to use their z-scores domiciled in their locale. However, having a pooled mean z-score of cardiac structures and function may be useful in the near future. [ABSTRACT FROM AUTHOR]

Background: Isolated pulmonary valve endocarditis (IPE) accounts for less than 2% of all infective endocarditis patients. It is commonly associated with several predisposing factors, including intravenous drug use (IVDU) and congenital heart disease. The most common causative pathogens of IPE are Staphylococcus aureus and Streptococcus viridans. We report a Down's syndrome patient with IPE and with no standard risk factors caused by the rare pathogen Acinetobacter spp. This led to respiratory failure and systemic infection due to septic pulmonary emboli. Early elective surgery was decided upon as the patient was no longer responding to medical therapy, and his clinical condition was worsening over time. Case presentation: A 15-year-old male with Down syndrome and no underlying heart defect presented with a 3-month history of episodic fever, nausea, vomiting, and diarrhea. Transthoracic echocardiography (TTE) revealed large vegetation on the pulmonary valve leaflet, another mobile mass at the pulmonary artery bifurcation, and severe pulmonary regurgitation. Serial blood cultures isolated Acinetobacter spp. Despite initial antibiotic therapy, the patient continued to have sepsis, unresolved vegetations, and developed life-threatening complications and respiratory distress, which convinced us to perform a pulmonary valve replacement surgery with a homograft. After surgery, the patient recovered and was discharged on the ninth postoperative day (POD). Conclusion: This report highlights IPE's diagnostic and therapeutic challenges, alongside the importance of a comprehensive cardiopulmonary workup in patients with unexplained fever, sepsis, and pulmonary symptoms, even without typical risk factors. Based on the patient's aggravating condition despite medical treatment, early surgical intervention and pulmonary valve replacement were deemed crucial. However, there still needs to be a definitive guideline on when and how surgery should be performed in patients with complicated IPE, especially in pediatric patients. [ABSTRACT FROM AUTHOR]

Infective endocarditis (IE) affects the endothelium of the heart, with the heart valves most commonly involved. It has been documented that the annual incidence of infective endocarditis is 3-10 per 100,000 patient-years1. However, it can be underestimated since the incidence in developing countries cannot be determined accurately. Here, we present a case of a 37-year-old male who was referred from a local health facility with shortness of breath on presentation; the patient was anuric for one day and initial laboratory investigations showed metabolic acidosis, hyperkalemia, sepsis, and deranged renal function tests. The patient had received a three-week course of intravenous (IV) piperacillin-tazobactam at the previous health facility, being diagnosed as a case of infective endocarditis. An initial transthoracic echocardiogram (TTE) showed vegetation on the pulmonary valve; however, the patient was neither an IV drug abuser nor did he have any history of implantation of intracardiac devices or central venous catheters. There was no recent or remote history of dental or surgical procedures. Due to the acute kidney injury, hemodialysis sessions and IV imipenem were started. As the patient’s hemodynamic profile improved by the fifth day of admission, TTE was repeated, revealing a small ventricular septal defect (VSD). This case report highlights the importance of even small VSD that could potentially lead to right-sided IE. Surgical correction of VSD could prevent such a life-threatening condition. [ABSTRACT FROM AUTHOR]

Background: Diagnosis of infective endocarditis (IE) can be challenging due to negative blood cultures and diagnostic limitations of various imaging modalities. Transesophageal echocardiography (TEE) is the gold standard imaging modality for visualization of valvular vegetations. However, due to the anterior location of the pulmonary valve, post-surgical changes, and sedation requirement, TEE can be challenging in the pediatric population. The aim of this study was to assess the value of Cardiac CT (CCT) for diagnosis of IE in children and young adults with congenital heart disease (CHD). Methods: This is a single-center retrospective study of pediatric patients with CHD and diagnosis of IE who underwent CCT from 2018 to 2022. Data collected included age, gender, cardiac diagnosis, clinical presentation, echocardiographic/CCT findings, and blood culture results. In addition, modified Duke criteria (MDC) for the diagnosis of IE were applied with and without CCT findings as the diagnostic imaging criterion. Results: Fourteen patients were included in this study with a median age of 11 years old. Nine patients were female. Ten patients had IE of the RV-PA conduit and four patients had IE of the aortic valve. Using MDC, 4 patients had definite IE. After including CCT findings, 11 patients (79%) met MDC for definite IE. Blood cultures were positive in 12 patients. CCT revealed the following complications: thromboembolic findings/pseudoaneurysms in 5 patients each and prosthetic valve perforation/prosthetic valve leak in one patient each. Conclusions: This study reinforces the complimentary role of CCT to echocardiography in the work-up and diagnosis of IE in patients with CHD. With further improvement in lowering radiation exposure, CCT may have a key role in the diagnostic work-up of endocarditis and could be implemented in the diagnostic criteria of IE. [ABSTRACT FROM AUTHOR]

Objectives Patients with congenital heart disease frequently require surgical or percutaneous interventional valve replacement after initial congenital heart defect (CHD) repair. In some of these patients, simultaneous replacement of both semilunar valves is necessary, resulting in increased procedural complexity, morbidity, and mortality. In this study, we analyze the outcomes of simultaneous aortic and pulmonary valve replacements following multiple surgical interventions for CHD. Methods This was a retrospective study of 24 patients who after initial repair of CHD underwent single-stage aortic and pulmonary valve replacement at our institution between 2003 and 2021. Results The mean age of the patients was 28 ± 13 years; the mean time since the last surgery was 15 ± 11 years. Decellularized valved homografts (DVHs) were used in nine patients, and mechanical valves were implanted in seven others. In eight patients, DVHs, biological, and mechanical valves were implanted in various combinations. The mean cardiopulmonary bypass time was 303 ± 104 minutes, and aortic cross-clamp time was 152 ± 73 minutes. Two patients died at 12 and 16 days postoperatively. At a maximum follow-up time of 17 years (mean 7 ± 5 years), 95% of the surviving patients were categorized as New York Heart Association heart failure class I. Conclusion Single-stage aortic and pulmonary valve replacement after initial repair of CHD remains challenging with substantial perioperative mortality (8.3%). Nevertheless, long-term survival and clinical status at the latest follow-up were excellent. The valve type had no relevant impact on the postoperative course. The selection of the valves for implantation should take into account operation-specific factors—in particular reoperability—as well as the patients' wishes. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the vascular impedance of the pulmonary arteries in fetuses with tetralogy of Fallot (TOF) by Doppler echocardiography. Methods: A total of 42 fetuses with TOF (TOF group) and 84 gestational age‐matched normal fetuses (control group) were prospectively collected from the Second Xiangya Hospital of Central South University from August 2022 to January 2023. The severity of TOF was classified into mild TOF (z score ≥−2), moderate TOF (−4 < z score < −2), or severe TOF (z score ≤−4) according to the z score value of the pulmonary annulus diameter. The pulsatility index (PI) of the main pulmonary artery (MPA), distal left pulmonary artery (DLPA), and distal right pulmonary artery (DRPA) were measured by pulsed‐wave Doppler. The differences in clinical data and echocardiographic parameters between TOF group, control group, and TOF subgroups were compared. Results: Compared with the control group, MPA‐PI increased significantly, whereas DLPA‐PI and DRPA‐PI decreased in TOF group (all P <.001). There were no significant differences in MPA‐PI and DRPA‐PI among mild TOF, moderate TOF, and severe TOF (all P >.05). However, DLPA‐PI decreased significantly in severe TOF compared with mild TOF (P <.05). Conclusion: Fetuses with TOF presented increased vascular impedance in the pulmonary trunk and decreased impedance in distal pulmonary artery branches. Further large and follow‐up studies are needed to demonstrate the associations between those changed vascular impedances and the development of PA in patients with TOF. [ABSTRACT FROM AUTHOR]

Transcatheter heart valve replacements (TVR) are mostly designed in a closed position (c) with leaflets coaptating. However, recent literature suggests fabricating valves in semi-closed (sc) position to minimize pinwheeling. With about 100,000 children in need of a new pulmonary valve each year worldwide, this study evaluates both geometrical approaches in adult as well as pediatric size and condition. Three valves of each geometry were fabricated in adult (30 mm) and pediatric (15 mm) size, using porcine pericardium. To evaluate performance, the mean transvalvular pressure gradient (TPG), effective orifice area (EOA), and regurgitation fraction (RF) were determined in three different annulus geometries (circular, elliptic, and tilted). For both adult-sized valve geometries, the TPG (TPGC = 2.326 ± 0.115 mmHg; TPGSC = 1.848 ± 0.175 mmHg)* and EOA (EOAC = 3.69 ± 0.255 cm2; EOASC = 3.565 ± 0.025 cm2)* showed no significant difference. Yet the RF as well as its fluctuation was significantly higher for valves with the closed geometry (RFC = 12.657 ± 7.669 %; RFSC = 8.72 ± 0.977 %)*. Recordings showed that the increased backflow was caused by pinwheeling due to a surplus of tissue material. Hydrodynamic testing of pediatric TVRs verified the semi-closed geometry being favourable. Despite the RF (RFC = 7.721 ± 0.348 cm2; RFSC = 5.172 ± 0.679 cm2), these valves also showed an improved opening behaviour ((TPGC = 20.929 ± 0.497 cm2; TPGSC = 15.972 ± 1.158 cm2); (EOAC = 0.629 ± 0.017 cm2; EOASC = 0.731 ± 0.026 cm2)). Both adult and pediatric TVR with semi-closed geometry show better fluiddynamic functionality compared to valves with a closed design due to less pinwheeling. Besides improved short-term functionality, less pinwheeling potentially prevents early valve degeneration and improves durability. *Results are representatively shown for a circular annulus geometry. [ABSTRACT FROM AUTHOR]

Introduction: Pulmonary regurgitation (PR) remains a common sequela in patients following surgically corrected TOF, and may lead to progressive right ventricle dilatation and dysfunction. The conventional approach of redo-sternotomy for pulmonary valve replacement (PVR) is associated with increased operative time as well as risks of bleeding and injury to the heart and great vessels. Thus, left anterior mini-thoracotomy has become an alternative approach in eliminating the risks of redo-sternotomy in these patients. This series aimed to determine the outcomes of minimally invasive pulmonary valve replacement after surgical TOF correction. Methods: A retrospective analysis was conducted on 24 patients with severe PR post-surgical TOF correction who underwent left anterior mini-thoracotomy PVR in Penang General Hospital from January 2021 to January 2023. Results: The median age was 23.5 years (I.Q.range 17.6-36.3), with a male:female ratio of 1:4. Majority of patients had mild to moderate symptoms prior to surgery and 19 patients (79.1%) were on regular diuretics medication. All patients had severe free-flow PR with evidence of right ventricular dilatation and dysfunction. Magnetic Resonance Imaging and computed tomography of pulmonary artery were performed prior to surgery. Minimally invasive PVR was performed on all patients via left upper anterior mini-thoracotomy and femoral-femoral bypass without cardioplegic arrest. The operative time and cardiopulmonary bypass time were 208 (I.Q.range 172-324) and 98.6 minutes(I.Q.range 87.4-152.4) respectively. The time to wean off inotropes postoperatively was 6.2 hours (I.Q.range1.4-14.8), and no postoperative arrhythmia and chest re-exploration were reported. Most patients stayed in Intensive Care Unit (ICU) for 10.8 hours (I.Q.range 8.4-36.5), and the total hospital stay was 4.2 days (I.Q.range 3.4-7.6). 2 patients (11.1%) required blood transfusion postoperative. There was no paravalvular leak and no mortality during the follow-up period of up to 28 months. Conclusion: Minimally invasive PVR after surgical correction of TOF is a safe alternative to the conventional redo-sternotomy approach in patients with favorable anatomy. This approach is able to reduce the risks associated with redo-sternotomy, particularly bleeding and injury to mediastinal structures, with the additional benefit of expedited recovery and hospital discharge. Our series has shown a safe and efficient approach in these patients with favorable outcomes. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve implantation (TPVI) is the standard of care in patients with repaired tetralogy of Fallot (rTOF) presenting with right ventricular outflow tract (RVOT) dysfunction. However, the feasibility of TPVI is limited by the high cost and nonavailability of larger-sized valves for dilated native RVOT of rTOF patients. We report the first successful TPVI with a custom-made 35 mm balloon-expandable valve (Myval™) in a 30-year-old rTOF patient with severe pulmonary regurgitation and RV dysfunction. [ABSTRACT FROM AUTHOR]

The gross morphological examination of native caprine heart valves revealed distinctive structural characteristics of the caprine's cardiac anatomy. Four primary orifices were identified, each protected by thin, valve‐like structures. Atrioventricular orifices featured tricuspid and bicuspid valves, while the aorta and pulmonary arteries were guarded by semilunar valves. Within the atrioventricular apparatus, distinct features were observed including the tricuspid valve's three leaflets and the bicuspid valve's anterior and posterior leaflets. Ultrasonography provided insights into valve thickness and chordae tendineae lengths. Morphometric studies compared leaflets/cusps within individual native valves, showcasing significant variations in dimensions. Comparative analysis between native and decellularized valves highlighted the effects of decellularization on leaflet thickness and chordae tendineae lengths. Decellularized valves exhibited reduced dimensions compared to native valves, indicating successful removal of cellular components. While some dimensions remained unchanged post‐decellularization, significant reductions were observed in leaflet thicknesses and chordae tendineae lengths. Notably, semilunar valve cusps displayed varying responses to decellularization, with significant reductions in cusp lengths observed in the aortic valve, while the pulmonary valve exhibited more subtle changes. These findings underscore the importance of understanding structural alterations in heart valves post‐decellularization, providing valuable insights for tissue engineering applications and regenerative medicine. [ABSTRACT FROM AUTHOR]

Congenital heart disease (CHD) is the most common congenital anomaly, with an overall incidence of approximately 1% in the United Kingdom. Exome sequencing in large CHD cohorts has been performed to provide insights into the genetic aetiology of CHD. This includes a study of 1891 probands by our group in collaboration with others, which identified three novel genes—CDK13, PRKD1, and CHD4, in patients with syndromic CHD. PRKD1 encodes a serine/threonine protein kinase, which is important in a variety of fundamental cellular functions. Individuals with a heterozygous mutation in PRKD1 may have facial dysmorphism, ectodermal dysplasia and may have CHDs such as pulmonary stenosis, atrioventricular septal defects, coarctation of the aorta and bicuspid aortic valve. To obtain a greater appreciation for the role that this essential protein kinase plays in cardiogenesis and CHD, we have analysed a Prkd1 transgenic mouse model (Prkd1em1) carrying deletion of exon 2, causing loss of function. High‐resolution episcopic microscopy affords detailed morphological 3D analysis of the developing heart and provides evidence for an essential role of Prkd1 in both normal cardiac development and CHD. We show that homozygous deletion of Prkd1 is associated with complex forms of CHD such as atrioventricular septal defects, and bicuspid aortic and pulmonary valves, and is lethal. Even in heterozygotes, cardiac differences occur. However, given that 97% of Prkd1 heterozygous mice display normal heart development, it is likely that one normal allele is sufficient, with the defects seen most likely to represent sporadic events. Moreover, mRNA and protein expression levels were investigated by RT‐qPCR and western immunoblotting, respectively. A significant reduction in Prkd1 mRNA levels was seen in homozygotes, but not heterozygotes, compared to WT littermates. While a trend towards lower PRKD1 protein expression was seen in the heterozygotes, the difference was only significant in the homozygotes. There was no compensation by the related Prkd2 and Prkd3 at transcript level, as evidenced by RT‐qPCR. Overall, we demonstrate a vital role of Prkd1 in heart development and the aetiology of CHD. [ABSTRACT FROM AUTHOR]

Background: Following surgical repair in infancy, a high proportion of children born with tetralogy of Fallot are surviving into adulthood, and it is important that these patients are monitored by an adult congenital heart disease specialist for problems and complications that can occur in the adult patient. Objective: To provide an overview on the management of adult patients with repaired tetralogy of Fallot and emphasise the need for specialist follow-up. Discussion: Following surgical repair of tetralogy of Fallot, it is important to have lifelong surveillance in adulthood for complications such as pulmonary valve dysfunction. Modern imaging modalities have helped with monitoring and the early detection of problems. Cardiac surgery has been refined over the years, leading to improved longevity. Over the past 23 years, percutaneous interventions have been developed to deal with valve complications and the results have been promising. Healthy behaviour, such as regular exercise, and behaviours to minimise the risk of endocarditis should be encouraged. Regular follow-up with an adult congenital heart disease specialist is important. Patients wishing to undertake a pregnancy should be supervised through a high-risk pregnancy clinic. [ABSTRACT FROM AUTHOR]

Radiofrequency (RF) perforation of an atretic pulmonary valve is commonly performed in patients with pulmonary atresia with intact ventricular septum with specifically designed RF wires. In difficult anatomy or low-resource centers, this may instead be successfully performed with a modified coronary guide wire and an electrocautery surgical pencil. [ABSTRACT FROM AUTHOR]