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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]

Aims: To evaluate the feasibility of a transthoracic echocardiogram using an apical-subcostal protocol in invasive mechanical ventilation (IMV) and prone position. Methods: Prospective study of adults who required a prone position during IMV. A pillow was placed only under the left hemithorax in the prone position to elevate and ease the apical and subcostal windows. A critical care cardiologist (prone group) acquired and evaluated the images using the apical-subcostal protocol. Besides, we used ambulatory echocardiograms performed as a comparative group (supine group). Results: 86 patients were included, 43 in the prone and 43 in the supine. In the prone group, the indication to perform an echocardiogram was hemodynamic monitoring. All patients were ventilated with protective parameters, and the mean end-expiratory pressure was 10.6 cmH2O. The protocol was performed entirely in 42 of 43 patients in the prone group because one patient did not have any acoustic window. In the 43 patients in the prone group analyzed and compared to the supine group, global biventricular function was assessed in 97.7% (p = 1.0), severe heart valve disease in 88.4% (p = 0.055), ruled out of the presence of pulmonary hypertension in 76.7% (p = 0.80), pericardial effusion in 93% (p = 0.12), and volume status by inferior vena cava in 93% (p = 0.48). Comparing prone versus supine position, a statistical difference was found when evaluating the left ventricle apical 2-chamber view (65.1 versus 100%, p < 0.01) and its segmental function (53.4 versus 100%, p < 0.01). Conclusion: The echocardiogram using an apical-subcostal protocol is feasible in patients in the IMV and prone position. [ABSTRACT FROM AUTHOR]

Cardiac magnetic resonance imaging (CMR) often shows discrepancies between right ventricular outflow tract (RVOT) flow and left ventricular outflow tract flow in patients with late-stage repaired tetralogy of Fallot (rTOF), leading to potential errors in pulmonary regurgitation fraction (PRF) assessment. This study aimed to identify the conditions under which RVOT flow can be acutely evaluated using four-dimensional (4D) flow CMR. Twenty-seven consecutive patients with rTOF underwent both two-dimensional phase-contrast (2D PC) and 4D flow CMR between 2016 and 2018, excluding those with peripheral pulmonary artery stenosis, RVOT conduit replacement, unknown surgical method, and an aortic valve regurgitation greater than 20%. Seven healthy controls also underwent only 4D Flow CMR. All healthy controls and fifteen patients with rTOF showed laminar RVOT flow, while seven patients exhibited helical, and four patients exhibited vortical RVOT flow in 4D flow CMR visualization. Flow-volume concordance between the pulmonary artery and aortic flow was significantly lower in patients with rTOF and PRF > 40% in 2D PC CMR. This concordance rate in the suprapulmonary valve was high in both the TOF and control groups, comparing at five RVOT locations in 4D flow CMR. Regarding RVOT flow regurgitation in 4D flow, the whole bulk evaluation exhibited greater variation depending on the flow type compared to the whole pixel-wise evaluation. The study confirmed the flow volume at the upper section of the pulmonary valve as the most accurate correlate of aortic flow volume. Furthermore, the 4D flow CMR using the pixel-wise method demonstrated superior accuracy compared to the traditional bulk flow method. [ABSTRACT FROM AUTHOR]

Background. Preliminary results with the recently certified self-expandable Venus P-Valve™ designed for percutaneous pulmonary valve implantation in patients with dilated right ventricular outflow tracts are encouraging, but experience is limited. We therefore assessed our early and midterm outcomes with the Venus P-Valve™. Methods. Twenty patients who underwent Venus P-Valve™ implantation in our institution were included in this retrospective study. Procedural data and clinical, imaging, and hemodynamic data at baseline and last follow-up were recorded and analyzed. Results. Mean patient age was 35.0 ± 16.8 years, and five patients were <18 years of age. Procedural success was 100%, and there was no major valve-related procedural complication. At last follow-up (median 0.5 (range 0.1–6.6) years), valve function was excellent in all patients. Two patients had mild regurgitation after 6.2 and 6.6 years, respectively, while all other patients had no or only trace regurgitation. Flow was unobstructed with a mean gradient estimated by echocardiography of 12 ± 4 mmHg. NYHA functional class improved significantly (p = 0.009), and right ventricular dimensions significantly decreased (right ventricular end-diastolic diameter 56±9 mm vs. 44±8 mm) (p < 0.001). Transient benign ventricular arrhythmias were frequent. One patient experienced a severe arrhythmia with sustained ventricular tachycardia during follow-up. Conclusions. Early and midterm results with the Venus P-Valve™ are excellent. It considerably extends the interventional options and offers a safe and effective alternative to surgery in patients with large right ventricular outflow tracts. Larger multi-institutional studies with longer follow-up duration are required to reliably assess the long-term performance and possible long-term complications of the Venus P-Valve™. [ABSTRACT FROM AUTHOR]

Background: Conflicting data exist on the occurrence and outcome of infective endocarditis (IE) after pulmonary valve implantation. Objectives: This study sought to assess the differences between transcatheter pulmonary valve implantation (TPVI) and surgical pulmonary valve replacement (SPVR). Methods: All patients ≥ 4 years who underwent isolated pulmonary valve replacement between 2005 and 2018 were analyzed based on the data of a major German health insurer (≈9.2 million insured subjects representative of the German population). The primary endpoint was a composite of IE occurrence and all-cause death. Results: Of 461 interventions (cases) in 413 patients (58.4% male, median age 18.9 years [IQR 12.3–33.4]), 34.4% underwent TPVI and 65.5% SPVR. IE was diagnosed in 8.0% of cases during a median follow-up of 3.5 years. Risk for IE and all-cause death was increased in patients with prior IE (p < 0.001), but not associated with age (p = 0.50), sex (p = 0.67) or complexity of disease (p = 0.59). While there was no difference in events over the entire observational time period (p = 0.22), the time dynamics varied between TPVI and SPVR: Within the first year, the risk for IE and all-cause death was lower after TPVI (Hazard Ratio (HR) 95% CI 0.19 (0.06–0.63; p = 0.006) but increased over time and exceeded that of SPVR in the long term (HR 10.07 (95% CI 3.41–29.76; p < 0.001). Conclusions: Patients with TPVI appear to be at lower risk for early but higher risk for late IE, resulting in no significant difference in the overall event rate compared to SPVR. The results highlight the importance of long-term specialized care and preventive measures after both interventions. [ABSTRACT FROM AUTHOR]

Despite many advances in surgical repair during the past few decades, the majority of tetralogy of Fallot patients continue to experience residual hemodynamic and electrophysiological abnormalities. The actual issue, which has yet to be solved, is understanding how this disease evolves in each individual patient and, as a result, who is truly at risk of sudden death, as well as the proper timing of pulmonary valve replacement (PVR). Our responsibility should be to select the most appropriate time for each patient, going above and beyond imaging criteria used up to now to make such a clinically crucial decision. Despite several studies on timing, indications, procedures, and outcomes of PVR, there is still much uncertainty about whether PVR reduces arrhythmia burden or improves survival in these patients and how to appropriately manage this population. This review summarizes the most recent research on the evolution of repaired tetralogy of Fallot (from adolescence onwards) and risk factor variables that may favor or delay PVR. [ABSTRACT FROM AUTHOR]

Purpose: This study evaluated the elastic characteristics of the pulmonary trunk and distal branches in fetuses diagnosed with tetralogy of Fallot (TOF) using Doppler echocardiography. Methods: Data on 42 fetuses diagnosed with TOF and 84 gestational age-matched normal fetuses were prospectively collected from the Second Xiangya Hospital of Central South University between August 2022 and January 2023. The severity of TOF was classified into three categories based on the z-score of the pulmonary annulus diameter: mild (z-score =-2), moderate (-40.05). Conclusion: Fetuses diagnosed with TOF exhibited increased vascular stiffness in the MPA and reduced stiffness in the distal pulmonary artery (PA). Larger-scale follow-up studies are required to elucidate the relationships between these changes in vascular stiffness and PA development in patients with TOF. [ABSTRACT FROM AUTHOR]

Brugada syndrome is a rare arrhythmogenic syndrome associated mainly with pathogenic variants in the SCN5A gene. Right ventricle outflow tract fibrosis has been reported in some cases of patients diagnosed with Brugada syndrome. Pulmonary atresia with an intact ventricular septum is characterized by the lack of a functional pulmonary valve, due to the underdevelopment of the right ventricle outflow tract. We report, for the first time, a 4-year-old boy with pulmonary atresia with an intact ventricular septum who harbored a pathogenic de novo variant in SCN5A, and the ajmaline test unmasked a type-1 Brugada pattern. We suggest that deleterious variants in the SCN5A gene could be implicated in pulmonary atresia with an intact ventricular septum embryogenesis, leading to overlapping phenotypes. [ABSTRACT FROM AUTHOR]

Introduction: Partial heart transplants are a new type of pediatric transplant that replace defective heart valves with the parts of matched donor hearts containing the necessary valves. Short‐term outcomes of partial heart transplants are excellent, but long‐term outcomes are unknown. In order to predict the long‐term outcomes of partial heart transplants, we evaluated long‐term growth and function of semilunar heart valves transplanted in infancy as part of a heart transplant. Methods: All children who underwent infant heart transplantation at a single center from 1997 to 2014 were included in this study. Children in whom echocardiograms after heart transplantation and after 10 years were not available for review were excluded. The echocardiograms were reviewed by two authors to analyze semilunar valve annulus diameters, Z‐scores, peak valve gradients, and valve regurgitation. Statistical difference was determined using two‐tailed, paired sample t‐tests with Bonferroni correction for multiple comparisons. Results: Data from 15 patients were analyzed. The aortic valve annulus averaged 1.3 cm (range 0.7–1.8 cm) immediately after transplantation and grew to an average of 1.7 cm (range 1.4–2.3 cm) after 10 years (p <.001). After 10 years, the aortic valve peak gradient avereraged 5.1 mmHg (range 2.1–15.5 mmHg) and none of the valves had more than trivial regurgitation. The pulmonary valve annulus averaged 1.5 cm (range 1.1–2.5 cm) immediately after transplantation and grew to an average of 2.1 cm (range 1.0–2.9 cm) after 10 years (p <.001). After 10 years, the pulmonary valve peak gradient averaged 4.3 mmHg (range 1.1–13.8 mmHg), and 7% of valves had moderate regurgitation. Discussion: Semilunar heart valves transplanted in infancy as part of a heart transplant demonstrate statistically significant growth and excellent function after 10 years. This predicts excellent long‐term outcomes of partial heart transplants. [ABSTRACT FROM AUTHOR]

Introduction: Bronchoscopic lung volume reduction (BLVR) with endobronchial valves has been shown to be a safe and effective treatment for patients with severe lung emphysema. Previous studies have reported a benefit in pulmonary function, exercise capacity, and quality of life after BLVR-treatment. The effect of BLVR with valves on the pulmonary gas exchange and its association with clinical outcomes has not been analyzed to date. The primary goal of this study was to investigate the impact of BLVR on the pulmonary gas exchange and the impact of the target lobe selection in patients with discordant target lobes in high-resolution computed tomography (HRCT) scan and perfusion scan on the pulmonary gas exchange and clinical outcomes. Methods: In this single-center study, we retrospectively analyzed pulmonary function tests, 6-min-walk-tests, HRCT scans, perfusion scans, and blood gas analyses in 77 patients over the course of 6 months following BLVR treatment. Results: We observed that complete lobar occlusion with bronchoscopic valves leads to a transient impairment of pulmonary gas exchange. Despite this, an overall positive clinical outcome could be shown in patients treated with endobronchial valves. If the target lobe selection based on HRCT and perfusion scans is discrepant, a selection based on the HRCT scan tends to be associated with a better outcome than a selection based on the perfusion scan. Conclusions: Complete lobar occlusion with bronchoscopic valves leads to a transient impairment of pulmonary gas exchange but nevertheless results in an overall positive clinical outcome. [ABSTRACT FROM AUTHOR]

Background: Maternal diabetes adversely affects fetal cardiovascular system development. Previous studies have reported that the fetuses of mothers with diabetes exhibit both structural and functional changes; nevertheless, prior studies have not examined the association between glucose control and fetal cardiac morphology and performance. Thus, the objective was to determine the association between fetal cardiac morphology and function and maternal glucose control in type 1 diabetes and to compare the differences in measured cardiac parameters between the fetuses of mothers with diabetes and healthy controls. Methods: In this prospective, longitudinal case-control study — including 62 pregnant women with type 1 diabetes mellitus and 30 healthy pregnant women — fetal cardiac assessment using B-mode, M-mode, and spectral pulsed-wave Doppler was performed in the second and third trimesters. In women with T1DM, glycated hemoglobin and data obtained from glucose sensors — including the percentage of time in, below, and above the range (TIR, TBR, and TAR, respectively), and coefficient of variation (CV) — were analyzed across three time periods: the last menstrual period to 13 (V1), 14–22 (V2), and 23–32 weeks (V3) of gestation. Fetal cardiac indices were compared between groups, and the correlation between glucose control and fetal cardiac indices was assessed. Results: At 28–32 weeks, the fetuses of women with T1DM exhibited increased left ventricular end-diastolic length, relative interventricular septum thickness, right ventricular cardiac output, and pulmonary valve peak systolic velocity compared with healthy controls. At 18–22 weeks, pulmonary and aortic valve diameters, left and right ventricular stroke volumes, and left cardiac output inversely correlated with the CV and glycated hemoglobin levels at V1 and V2. Furthermore, at 28–32 weeks, pulmonary and aortic valve diameters, left ventricular stroke volume, cardiac output, and right/left atrioventricular valve ratio inversely correlated with the TBR at V1, V2, and V3. Moreover, diastolic functional parameters correlated with the TAR and glycated hemoglobin levels, particularly after the first trimester. Conclusion: In women with T1DM, maternal hyperglycemia during pregnancy correlates with fetal diastolic function, whereas glucose variability and hypoglycemia inversely correlate with fetal left ventricular systolic function in the second and third trimesters. Key message: This prospective longitudinal study demonstrates that in women with T1DM, maternal hyperglycemia during pregnancy correlates with fetal diastolic function. In contrast, glucose variability and hypoglycemia inversely correlate with fetal left ventricular systolic function in the second and third trimesters. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021. In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience. This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S. centers. The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention. A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2). In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes. [Display omitted] [ABSTRACT FROM AUTHOR]

Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1–58) days and 11 (22%) underwent outpatient PBV at 51.8 (11–174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02–94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS. [ABSTRACT FROM AUTHOR]

Methicillin-resistant Staphylococcus aureus (MRSA) bacteremia can be persistent and refractory; however, the optimal approach for its treatment has not been determined. Although fosfomycin (FOM) has been shown to have synergistic effects with anti-MRSA agents in vitro, clinical experience with FOM combination therapy is limited. Thus, we present cases of persistent MRSA bacteremia that improved with the addition of FOM. In case 1, a 48-year-old man with prosthetic vascular graft infection developed persistent MRSA bacteremia despite vancomycin (VCM) and daptomycin (DAP) administration. On day 46, after the first positive blood culture, we added FOM to DAP. The blood culture became negative on day 53. In case 2, an 85-year-old woman presented with pacemaker-related MRSA bacteremia. She was treated with VCM, followed by DAP and DAP plus rifampicin. However, the bacteremia persisted for 32 days because of difficulties in immediate pacemaker removal. After adding FOM to DAP, the blood culture became negative on day 38. In case 3, a 57-year-old woman developed persistent MRSA bacteremia due to pulmonary valve endocarditis and pulmonary artery thrombosis after total esophagectomy for esophageal cancer. The bacteremia continued for 50 days despite treatment with DAP, followed by VCM, VCM plus minocycline, DAP plus linezolid (LZD), and VCM plus LZD. She was managed conservatively because of surgical complications. After adding FOM to VCM on day 51, the blood culture became negative on day 58. FOM combination therapy may be effective in eliminating bacteria and can serve as salvage therapy for refractory MRSA bacteremia. [ABSTRACT FROM AUTHOR]

Background Decellularized pulmonary homografts are being increasingly adopted for right ventricular outflow tract reconstruction in adult patients undergoing the Ross procedure. Few reports presented Matrix PplusN xenograft (Matrix) in a negative light. The objective of this study was to compare our midterm outcomes of Matrix xenograft versus standard cryopreserved pulmonary homograft (CPHG). Methods Eighteen patients received Matrix xenograft between January 2012 and June 2016, whereas 66 patients received CPHG. Using nonparametric statistical tests and survival analysis, we compared midterm echocardiographic and clinical outcomes between the groups. Results Except for significant age difference (the Matrix group was significantly older with 57 ± 8 years than the CPHG group, 48 ± 9 years, p = 0.02), the groups were similar in all other baseline characteristics. There were no significant differences in cardiopulmonary bypass times (208.3 ± 32.1 vs. 202.8 ± 34.8) or in cross-clamp times (174 ± 33.9 vs. 184.4 ± 31.1) for Matrix and CPHG, respectively. The Matrix group had significantly inferior freedom from reintervention than the CPHG group with 77.8 versus 98.5% (p = 0.02). Freedom from pulmonary valve regurgitation ≥ 2 was not significantly different between the groups with 82.4 versus 90.5% for Matrix versus CPHG, respectively. After median follow-up of 4.9 years, Matrix xenograft developed significantly higher peak pressure gradients compared with CPHG (20.4 ± 15.5 vs. 12.2 ± 9.0 mm Hg; p = 0.04). Conclusion After 5 years of clinical and echocardiographic follow-up, the decellularized Matrix xenograft had inferior freedom from reintervention compared with the standard CPHG. Closer follow-up is necessary to avoid progression of valve failure into right ventricular deterioration. [ABSTRACT FROM AUTHOR]

Sotos syndrome is an autosomal dominant condition characterized by overgrowth with advanced bone age, macrodolicocephaly, motor developmental delays and learning difficulties, and characteristic facial features caused by heterozygous pathogenetic variants in the NSD1 gene located on chromosome 5q35. The prevalence of heart defects (HDs) in individuals with Sotos syndrome is estimated to be around 15–40%. Septal defects and patent ductus arteriosus are the most commonly diagnosed malformations, but complex defects have also been reported. The aim of our study was to analyze the prevalence of HD, the anatomic types, and the genetic characteristics of 45 patients with Sotos syndrome carrying pathogenetic variants of NSD1 or a 5q35 deletion encompassing NSD1, who were followed at Bambino Gesù Children's Hospital in Rome. Thirty-nine of the forty-five patients (86.7%) had a mutation in NSD1, while six of the forty-five (13.3%) had a deletion. Most of the patients (62.2%, 28/45) were male, with a mean age of 14 ± 7 years (range 0.2–37 years). A total of 27/45 (60.0%) of the patients had heart defects, isolated or combined with other defects, including septal defects (12 patients), aortic anomalies (9 patients), mitral valve and/or tricuspid valve dysplasia/insufficiency (1 patient), patent ductus arteriosus (3 patients), left ventricular non-compaction/hypertrabeculated left ventricle (LV) (4 patients), aortic coarctation (1 patient), aortopulmonary window (1 patient), and pulmonary valve anomalies (3 patients). The prevalences of HD in the two subgroups (deletion versus intragenic mutation) were similar (66.7% (4/6) in the deletion group versus 58.91% (23/39) in the intragenic variant group). Our results showed a higher prevalence of HD in patients with Sotos syndrome in comparison to that described in the literature, with similar distributions of patients with mutated and deleted genes. An accurate and detailed echocardiogram should be performed in patients with Sotos syndrome at diagnosis, and a specific cardiological follow-up program is needed. [ABSTRACT FROM AUTHOR]

Hypereosinophilic syndrome (HES) is considered to be a rare myeloproliferative disease that is characterized by persistent eosinophilia with associated multiple-organ damage. The heart is often involved in HES, representing a major cause of morbidity and mortality. HES is a heterogeneous group of disorders; the majority of the cases are idiopathic. Summarizing the findings regarding myocardial, valvular, and vascular abnormalities in a series of patients with HES, most studies found normal left ventricular (LV) volumes with reduced LV global longitudinal strain and LV apical rotation and twist in HES cases, accompanied by increased left atrial (LA) volumes and stroke volumes, reduced peak LA circumferential strain (representing systolic abnormalities), and mitral annular dilation and functional deterioration. Regarding the right heart, preserved right ventricular volumes and functional properties, increased right atrial volumes, mild RA functional abnormalities, and dilated tricuspid annular dimensions without functional impairment could be seen in these studies. Aortic and pulmonary valves showed no specific disease-related alterations. Vascular abnormalities included increased aortic stiffness without dilation of the aorta and pulmonary hypertension in some cases. These results suggest disease-specific but relatively mild myocardial, valvular, and vascular abnormalities in HES. The present review aimed to summarize the available clinical data about cardiac mechanics and valvular and vascular abnormalities in a series of patients with HES. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12–19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31–63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10–53 mmHg) pre-procedure to 10 mmHg (IQR 5–16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5–44 min). The large sizes—mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120–300 mL). Patients were followed for a median period of 360 days (IQR 164–525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes. [ABSTRACT FROM AUTHOR]

The maned wolf, Chrysocyon brachyurus, is the largest South American canid, with a natural distribution that stretches across Peru, Bolivia, Brazil, Argentina, Paraguay and Uruguay. The present study reports the case of a rescued specimen of maned wolf that underwent a rehabilitation process in Paraguay, starting in October 2020 with its rescue, and finalising in May 2021 with the reintroduction. Herein, we document findings regarding the general management, biometrics, feeding and environmental enrichment; chemical immobilisation and monitoring; haematology, blood biochemistry and specific serology‐relevant pathogens; skin examination and bone marrow cytology; orthopaedic, ophthalmological and dental evaluation; abdominal and cardiac ultrasonography; radiology and copro‐parasitology. Main findings include the feeding habits of the individual and enrichment opportunities. The animal weighed 7 kg on arrival, with an estimated age of 5 months, and 18 kg on reintroduction, with an estimated age of 1 year. The animal tested negative to serologic tests for Brucella canis, Dirofilaria, canine distemper, Toxoplasmosis and canine parvovirus. Leptospira testing showed antibodies against L. grippotyphosa on both samplings, L. wolffi and L. ictero on the first sampling, and L. pomona on the second sampling. Abdominal organs were examined and measured through ultrasound evaluation and kidneys showed no alterations. Echocardiography showed preserved mitral, tricuspid and aortic valve flows, but turbulent pulmonary valve flow. Copro‐parasitology reported the presence of Lagochilascaris sp. and Balantidium sp. All the information gathered aided in diagnosing the health status of the individual, and the response to environmental enrichment helped assess the behaviour, which led to the suggestion of reintroducing the animal. These data constitute the first published health check of a maned wolf in Paraguay, which can contribute to the species' conservation in the country. The protocol presented in this study can serve as a basis for developing an action plan for the maned wolf in Paraguay. [ABSTRACT FROM AUTHOR]

In patients with congenital heart disease, transcatheter pulmonary valve implantation is the treatment of choice in dysfunctional right ventricular outflow tracts (RVOT). The self-expandable Venus-P valve was approved recently for use in large native tracs. It is reported the experience of the first case of successful implantation of Venus-P pulmonary valve in Colombia. Ours was a 16-year-old patient with Tetralogy of Fallot, with complete correction at 11 months of life. The patient has severe pulmonary insufficiency and functional class deterioration. Cardiac catheterization was performed to place a 34 x 30 mm Venus-P valve, a procedure carried out without complications, evidencing significant improvement in the diastolic pressure of the pulmonary artery, without final gradient between the right ventricle and pulmonary artery. Reports exist of experiences in Venus-P valve implant globally with favorable results; percutaneous valve placement represents decreased procedure times and hospital stays. We believe percutaneous valve replacement in pulmonary position in native RVOTs is a possible and safe option to improve pulmonary insufficiency and right ventricular dysfunction in these types of patients. [ABSTRACT FROM AUTHOR]

Background: Percutaneous pulmonary valve implantation has become an attractive method of dysfunctional right ventricle outflow tract treatment. Case presentation: We describe a unique case of a 20-year-old Caucasian male patient with a complex cyanotic heart defect, namely pulmonary atresia, with congenitally corrected transposition of the great arteries and ventricular septal defect after Rastelli-like surgery at the age of 5 years with homograft use. At the age of 20 years, the patient needed percutaneous pulmonary valve implantation owing to homograft dysfunction. Despite unusual course of the coronary arteries, balloon testing in the landing zone of the right ventricle outflow tract excluded potential coronary artery compression. Then, after presentation, a Melody valve was implanted successfully in the pulmonary valve position. The 8-year follow-up was uneventful. Conclusion: This is likely the first description of a percutaneous pulmonary valve implantation in such anatomy. Such a procedure is feasible; however, it requires exceptional caution owing to the anomalous coronary arteries course, which can be the reason for their compression. [ABSTRACT FROM AUTHOR]

Background: Native pulmonary valve (PV) infective endocarditis (IE) is a rare condition with an incidence of 1.5–2%. Although medical therapy is the mainstay of treatment, surgical intervention is still indicated in cases that fail to respond to antibiotics. To date, there is lack of consensus about the best surgical approach for isolated native PV IE. While valve repair is sometimes feasible, most of the cases require valve replacement depending on the extent of tissue damage. A stented bioprosthesis can be used when infection is confined to the valve leaflets. However, extension of damage to the pulmonary root or right ventricular outflow tract usually requires debridement and root replacement. Case presentation: A 30-year-old lady with a long history of restrictive ventricular septal defect (VSD) presented with fever and shortness of breath shortly after vaginal delivery that was diagnosed as isolated PV IE with pulmonary septic emboli. After 1 week of antibiotic therapy, there was no response with persistent infection and complete valve destruction. Heart team discussion recommended PV replacement using a Freestyle valve along with VSD repair. On follow-up after 1 year, she was doing fine with well-functioning Freestyle valve. Conclusion: Unrepaired restrictive VSD can be a predisposing factor for native PV IE. A Freestyle valve in the pulmonary position is a valid option for such cases, with good early outcomes and reasonable durability, particularly when there is extensive tissue infection and homograft is not available. More evidence is still required to evaluate the long-term outcomes of PV Freestyle in cases of IE. [ABSTRACT FROM AUTHOR]

Background: Recently, improvements in the repair of tetralogy of Fallot have increased the need for reoperation in adulthood, and it's not rare that these reoperation candidates suffer from biventricular failure. However, there are no firm treatment guidelines, and each country, and even each facility, treats each case individually. Case presentation: We report the successful staged treatment of pulmonary regurgitation and pacemaker-induced cardiomyopathy with biventricular failure in adulthood in a case of complete atrioventricular block after tetralogy of Fallot repair in childhood. We planned a staged therapeutic strategy with preoperative left ventricular volume reduction with medication, following surgical pulmonary valve replacement concomitant epicardial lead implantation on the lateral basal wall, placed just beneath the generator pocket through 3rd intercostal space. in addition to postoperative intervention with a defibrillator to adjust cardiac resynchronization therapy, resulted in improvement of symptoms. Conclusion: In a patient with biventricular failure after TOF repair, a staged treatment strategy involving medication, PVR, and CRT with a combination of epicardial and intravenous leads could be a useful treatment worth trying before heart transplantation. [ABSTRACT FROM AUTHOR]

Background: Recently, improvements in the repair of tetralogy of Fallot have increased the need for reoperation in adulthood, and it's not rare that these reoperation candidates suffer from biventricular failure. However, there are no firm treatment guidelines, and each country, and even each facility, treats each case individually. Case presentation: We report the successful staged treatment of pulmonary regurgitation and pacemaker-induced cardiomyopathy with biventricular failure in adulthood in a case of complete atrioventricular block after tetralogy of Fallot repair in childhood. We planned a staged therapeutic strategy with preoperative left ventricular volume reduction with medication, following surgical pulmonary valve replacement concomitant epicardial lead implantation on the lateral basal wall, placed just beneath the generator pocket through 3rd intercostal space. in addition to postoperative intervention with a defibrillator to adjust cardiac resynchronization therapy, resulted in improvement of symptoms. Conclusion: In a patient with biventricular failure after TOF repair, a staged treatment strategy involving medication, PVR, and CRT with a combination of epicardial and intravenous leads could be a useful treatment worth trying before heart transplantation. [ABSTRACT FROM AUTHOR]

Fallot-type absent pulmonary valve is a rare and complex congenital heart disease. Repair surgery for this condition during the neonatal period has a mortality rate of over 50%. We reported a neonate with Fallot-type absent pulmonary valve and occlusion of the left main bronchus. The patient's pulmonary artery had unusual anatomy of a type that has not previously been reported. This case report outlines a successful treatment strategy for patients with complex congenital heart disease and airway occlusion during the neonatal period and the effect of these unusual anatomical conditions on postoperative outcomes. [ABSTRACT FROM AUTHOR]

Background: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post‐transcatheter pulmonary valve replacement (TPVR). Methods: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post‐TPVR. Patients were dichotomized into three groups based on RVSP: 0%–49%, 50%–74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4‐chamber using TomTec software (TOMTEC IS, Germany). Results: Forty‐eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p =.001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p =.014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. Conclusion: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed. [ABSTRACT FROM AUTHOR]

Pulmonary hypertension due to left heart disease (PH-LHD) is commonly seen in patients with heart failure (HF), but there are limited treatment options. Recent studies have shown an association between aldehyde dehydrogenase 2 (ALDH2) rs671 polymorphisms and pulmonary hypertension (PH). Therefore, this study aimed to investigate the occurrence of ALDH2 rs671 polymorphisms, and the association between ALDH2 and risk of PH-LHD in patients with HF. It also investigated different ALDH2 genotypes and examined their association with cardiac structure and function in HF patients with PH-LHD. A total of 178 HF patients were consecutively enrolled in this study: 102 without PH-LHD and 76 with PH-LHD. Clinical data, parameters of echocardiography, and relevant biochemical indexes were recorded in both groups. Differences in data obtained between groups were compared, and the risk of variant ALDH2 polymorphisms with PH-LHD in HF patients was analysed using univariate and multivariate logistic regression. The prevalence of ALDH2 rs671 GA/AA polymorphisms (variant ALDH2) was 24 of 102 patients (23.53%) in the HF without PH-LHD group, and 32 of 76 patients (42.10%) in the HF with PH-LHD group, with a statistically significant difference. Univariate and multivariate logistical regression showed that variant ALDH2 is an independent risk factor for HF combined with PH-LHD. A higher proportion of patients with variant ALDH2 in the HF with PH-LHD group had a tricuspid regurgitation velocity >2.8 m/s, and they had higher values of peak early diastolic velocity of the mitral orifice/peak velocity of the early diastolic wave of the mitral orifice, maximum frequency shift of pulmonary valve flow, and pulmonary artery stiffness. Variant ALDH2 may be an independent risk factor for HF combined with PH-LHD. Variant ALDH2 may also be involved in pulmonary artery remodelling and is a potential new target for clinical treatment of PH-LHD. [ABSTRACT FROM AUTHOR]

Duplicate measure of hemoglobin mass by carbon monoxide (CO)-rebreathing is a logistical challenge as recommendations prompt several hours between measures to minimize CO-accumulation. This study investigated the feasibility and reliability of performing duplicate CO-rebreathing procedures immediately following one another. Additionally, it was evaluated whether the obtained hemoglobin mass from three different CO-rebreathing devices is comparable. Fifty-five healthy participants (22 males, 23 females) performed 222 duplicate CO-rebreathing procedures in total. Additionally, in a randomized cross-over design 10 participants completed three experimental trials, each including three CO-rebreathing procedures, with the first and second separated by 24 h and the second and third separated by 5–10 min. Each trial was separated by >48 h and conducted using either a glass-spirometer, a semi-automated electromechanical device, or a standard three-way plastic valve designed for pulmonary measurements. Hemoglobin mass was 3 ± 22 g lower (p < 0.05) at the second measure when performed immediately after the first with a typical error of 1.1%. Carboxyhemoglobin levels reached 10.9 ± 1.3%. In the randomized trial, hemoglobin mass was similar between the glass-spirometer and three-way valve, but ∼6% (∼50 g) higher for the semi-automated device. Notably, differences in hemoglobin mass were up to ∼13% (∼100 g) when device-specific recommendations for correction of CO loss to myoglobin and exhalation was followed. In conclusion, it is feasible and reliable to perform two immediate CO-rebreathing procedures. Hemoglobin mass is comparable between the glass-spirometer and the three-way plastic valve, but higher for the semi-automated device. The differences are amplified if the device-specific recommendations of CO-loss corrections are followed. [ABSTRACT FROM AUTHOR]

Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making. [ABSTRACT FROM AUTHOR]

Key Clinical Message: The case highlights the good survival after radical surgery and chemotherapy of a cardiac sarcoma, and the need for close follow‐up due to possible early postsurgical complications. [ABSTRACT FROM AUTHOR]

This article discusses a case of a left ventricular outflow tract (LVOT) aneurysm in a patient who had undergone a Senning procedure for isolated ventricular inversion. The patient presented with a left precordial bulge and a chest X-ray revealed a prominent left para-cardiac shadow. Echocardiogram and cardiac CT confirmed the presence of a large aneurysm arising from the lateral wall of the LVOT. The patient underwent surgical repair and remained well at the 6-month follow-up. This case highlights the importance of long-term follow-up after cardiac surgery and the relevance of chest X-ray in postoperative evaluation. [Extracted from the article]

This article discusses a case study of a newborn with tetralogy of Fallot and features similar to DiGeorge syndrome. The child underwent genetic testing, including chromosomal microarray analysis and whole exome sequencing, which identified a de novo variant in the HIRA gene resulting in the loss of the start codon. The HIRA gene has been associated with hematopoiesis, neurodevelopment, and cardiac development. This case adds to the current knowledge of HIRA-related phenotypes and suggests a potential association between HIRA gene variants and tetralogy of Fallot. [Extracted from the article]

Blood-filled cysts of the heart valves are frequently reported at postpartum autopsies of infants. They are seen as round nodules mostly in the paediatric age group in infants less than 2 months of age and disappear spontaneously within 6 months of life. We report a unique case of an 11-month-old girl who presented at a tertiary healthcare hospital in 2022 with a blood-filled cyst on the posterior leaflet of the pulmonary valve that was successfully treated. This case report highlights the characteristics and course of a paediatric patient with bloodfilled cysts. Further studies are yet needed to better understand the diagnostic approaches to blood-filled cysts as well as treatment modalities to fill the gap in clinical settings. [ABSTRACT FROM AUTHOR]

Background: Orexins are hypothalamic neuropeptides associated with various neurophysiological activities such as sleep, arousal, and reward. However, there are few studies investigating the relationships between orexin receptors in the paraventricular nucleus and sexual behaviors. Objectives: To explore the roles of orexin receptors in the paraventricular nucleus on sexual behaviors and uncover its potential mechanisms in males. Materials and methods: Orexin A, orexin 1 receptor antagonist SB334867, and orexin 2 receptor antagonist TCS‐OX2‐29 were microinjected into the paraventricular nucleus to investigate the effects of orexin receptors on copulatory behavior testing of C57BL/6 mice. To explore if ejaculation could activate orexin 1 receptor‐expressing neurons in the paraventricular nucleus, fluorescence immunohistochemical double staining was utilized. The levels of serum norepinephrine were measured and the lumbar sympathetic nerve activity was recorded to reflect the sympathetic nervous system activity. Moreover, the bulbospongiosus muscle‐electromyogram was recorded and analyzed. To test whether perifornical/lateral hypothalamic area orexinergic neurons directly projected to the paraventricular nucleus, virus retrograde tracing technology was utilized. Results: Orexin A significantly enhanced sexual performance by shortening the intromission and ejaculation latencies, and increasing the mount and intromission frequencies, while the opposite outcomes appeared with SB334867. However, TCS‐OX2‐29 had no significant effects on sexual behaviors. Moreover, orexin A increased lumbar sympathetic nerve activity and the levels of serum norepinephrine, while SB334867 decreased lumbar sympathetic nerve activity and norepinephrine, which caused a significant decrease in sympathetic nervous system outflow. Meanwhile, a robust increase in the bulbospongiosus muscle‐electromyogram activity was identified after microinjecting orexin A. Furthermore, cFos immunopositive cells were increased and double stained with orexin 1 receptor‐expressing neurons in the mating group. Additionally, the retrograde tracing results demonstrated that orexinergic neurons in the perifornical/lateral hypothalamic area directly projected to the paraventricular nucleus. Conclusions: Orexin 1 receptor in the paraventricular nucleus could influence the ejaculatory reflex via mediating the sympathetic nervous system activity, which might be of great importance in the treatment of premature ejaculation in the future. [ABSTRACT FROM AUTHOR]

Absent pulmonary valve syndrome (APVS) is a rare congenital anomaly characterized by rudimentary PV tissue with variable degrees of PV stenosis and regurgitant pulmonary blood flow. In most cases, it is associated with tetralogy of Fallot. In a minority of APVS cases, with an unknown frequency, intact ventricular septum (IVS), patent ductus arteriosus, and possible tricuspid atresia are present. This condition is known as non-Fallot type APVS. We describe a case of an antenatal diagnosis of APVS with IVS, a large patent ductus arteriosus, and ascending aorta dilatation. The mother was referred to our center at 32 weeks of gestation due to cardiomegaly on sonography. Fetal echocardiography revealed cardiomegaly, right atrial and ventricular enlargement, aneurysmal dilatation of the main pulmonary artery, and mild dilatation of the pulmonary artery branches. Also observed were IVS, rudimentary PV tissue with severe to-and-fro turbulence across the PV, a widely open ductus arteriosus, ascending aorta dilatation, and levorotation of the heart. After childbirth, our diagnosis was confirmed by echocardiography and surgery. The baby experienced severe respiratory distress. At 15 days of life, surgical intervention in the form of pulmonary artery arterioplasty was performed, resulting in good outcomes. The patient underwent follow-up for 6 months and showed reasonable health. [ABSTRACT FROM AUTHOR]

Appropriate load‐bearing function of soft connective tissues is provided by their nonlinear and often anisotropic mechanics. Recapitulating such complex mechanical behavior in tissue‐engineered structures is particularly crucial, as deviation from native tissue mechanics can trigger pathological biomechanical pathways, causing adverse tissue remodeling and dysfunction. Here, a novel method combining computational modeling, melt electrowriting (MEW), and design of experiments (DOE) is reported to generate scaffolds composed of sinusoidal fibers with prescribed biaxial mechanical properties, recapitulating the distinct nonlinear, anisotropic stress–strain behavior of three model tissues: adult aortic valve, pediatric pulmonary valve, and pediatric pericardium. Finite element analysis is used to efficiently optimize scaffold architecture over a broad parameter space, representing up to 65 conditions, to define MEW print parameters to achieve polycaprolactone scaffolds with target mechanical properties. Architectural parameters are further optimized experimentally using DOE and regression to account for uncertainties involved in the simulation, yielding functional scaffolds with accurate, prescribed mechanics. The prescribed architecture also primarily governs the mechanics of hybrid structures generated by casting cell‐laden fibrin hydrogel within the scaffolds. This high‐fidelity approach recapitulates biaxial mechanical properties over a broad range of mechanical nonlinearity and anisotropy and is generalizable for programmed biofabrication in a variety of tissue engineering applications. [ABSTRACT FROM AUTHOR]

Various transcatheter interventions for the right ventricular outflow tract (RVOT) have been introduced and developed in recent decades. Transcatheter pulmonary valve perforation was first introduced in the 1990s. Radiofrequency wire perforation has been the approach of choice for membranous pulmonary atresia in newborns, with high success rates, although complication rates remain relatively common. Stenting of the RVOT is a novel palliative treatment that may improve hemodynamics in neonatal patients with reduced pulmonary blood flow and RVOT obstruction. Whether this option is superior to other surgical palliative strategies or early primary repair of tetralogy of Fallot remains unclear. Transcatheter pulmonary valve replacement has been one of the biggest innovations in the last two decades. With the success of the Melody and SAPIEN valves, this technique has evolved into the gold standard therapy for RVOT abnormalities with excellent procedural safety and efficacy. Challenges remain in managing the wide heterogeneity of postoperative lesions seen in RVOT, and various technical modifications, such as pre-stenting, valve ring modification, or development of self-expanding systems, have been made. Recent large studies have revealed outcomes comparable to those of surgery, with less morbidity. Further experience and multicenter studies and registries to compare the outcomes of various strategies are necessary, with the ultimate goal of a single-step, minimally invasive approach offering the best longer-term anatomical and physiological results. [ABSTRACT FROM AUTHOR]

Pulmonary regurgitation usually leads to right heart dilatation and eventually right heart dysfunction, which is associated with a poor prognosis. Transcatheter pulmonary valve replacement is a developing treatment for pulmonary valve dysfunction that can take the place of traditional surgery and make up for the shortcomings of a large injury. Echocardiography plays a significant role in assessing ventricular function; however, conventional echocardiographic parameters have several limitations. Speckle tracking echocardiography has been regarded as a more accurate tool for quantifying cardiac function than conventional echocardiography. Therefore, the aim of this review was to summarize the application of speckle tracking echocardiography for evaluating right and left ventricular functions in patients after transcatheter pulmonary valve replacement. [ABSTRACT FROM AUTHOR]

Key Points: Question: Do partial heart transplants deliver growing heart valve implants for neonates? Findings: The first-in-human partial heart transplant demonstrated valve growth during follow-up for more than 1 year. Meaning: Partial heart transplant is a new approach to treat neonates with irreparable heart valve dysfunction. Importance: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until an adult-sized valve can fit. Objective: To deliver the first heart valve implant that grows. Design, Setting, and Participants: Case report from a pediatric referral center, with follow-up for more than 1 year. Participants were a recipient neonate with persistent truncus arteriosus and irreparable truncal valve dysfunction and a donor neonate with hypoxic-ischemic brain injury. Intervention: First-in-human transplant of the part of the heart containing the aortic and pulmonary valves. Main Outcomes and Measures: Transplanted valve growth and hemodynamic function. Results: Echocardiography demonstrated adaptive growth and excellent hemodynamic function of the partial heart transplant valves. Conclusions and Relevance: In this child, partial heart transplant delivered growing heart valve implants with a good outcome at age 1 year. Partial heart transplants may improve the treatment of neonates with irreparable heart valve dysfunction. This case report documents the first partial heart transplant in a neonate with irreparable heart valve dysfunction to deliver aortic and pulmonary valves that grow with the developing child. [ABSTRACT FROM AUTHOR]

This article discusses the development of a Procedure Pack (PP) that allows for the fabrication of an autologous heart valve replacement during surgery. The pack consists of various components, including a stent, catheter delivery system, crimping tool, crosslinker, and mold. The article describes the results of several design iterations and in-vivo testing in sheep, which led to the successful fabrication of fully functional valves. However, the high cost and time required for certification of all the components made translation to the market impossible. The authors suggest prioritizing existing products and minimal in-house development to facilitate device translation and certification. [Extracted from the article]

A study published in the journal Thoracic & Cardiovascular Surgeon discusses a novel stent design for intraoperatively manufactured autologous transcatheter pulmonary valve replacement (THVR) for both adults and children. The study focuses on the feasibility of valve integration and deformation behavior of the stent through computer-aided design and finite element analysis. The researchers developed a stent design with more suture points by adding auxiliary fixation elements, allowing for the manufacturing of THVR during surgery. Prototype testing showed promising results, and further optimization and long-term evaluation are planned. [Extracted from the article]

This article discusses the risk of ventricular tachycardias (VT) in patients who have undergone repaired Tetralogy of Fallot (rTOF) surgery. The study included 39 patients who underwent electrophysiological study for VT risk stratification. The researchers found that slow conducting anatomic isthmuses (SCAI) were present in the majority of patients, with the most common SCAI being isthmus 3 and isthmus 4. Ablation procedures were successful in 87% of patients, with only one patient experiencing recurrence of VT/VF. The study suggests that implantable cardioverter-defibrillator (ICD) implantation may be necessary in some cases. [Extracted from the article]

This article, published in the journal Thoracic & Cardiovascular Surgeon, examines the acute changes in biventricular intrinsic myocardial function that occur during percutaneous pulmonary valve implantation (PPVI) in patients with right ventricular outflow tract (RVOT) dysfunction. The study involved twenty patients who underwent PPVI, and their biventricular pressure-volume loops were assessed using a conductance catheter technique. The results showed that after PPVI, there was a significant decrease in right ventricular contractility and pulmonary arterial elastance, while left ventricular contractility remained unchanged. The study suggests that further investigation is needed to understand the long-term effects of these response patterns on biventricular remodeling. [Extracted from the article]

This article discusses the use of exercise tests to aid in the decision-making process for pulmonary valve replacement in patients with pulmonary valve regurgitation (PVR). The study evaluated cardiopulmonary exercise tests (CPET) performed on patients with PVR and compared them to a control group without PVR. The researchers introduced new CPET parameters to analyze the O2pulse before and after exercise. The results showed that classic CPET parameters did not provide any benefit in the decision process, but the duration of elevated O2pulse after peak exercise was significantly different between the two groups. The authors suggest that the paradoxical course of the O2pulse may be indicative of RV remodeling and increased stiffness. [Extracted from the article]

This article discusses the use of decellularized porcine heart valves as an alternative option for pediatric patients and young adults with heart valve disease. The study focuses on the application of sodium hydroxide (NaOH) for decellularization and the removal of xenoantigens, which are antigens recognized upon implantation into humans. The results show that decellularization with NaOH effectively removes cells from the porcine heart valves and reduces the amount of αGal, a prominent xenoantigen. The study concludes that decellularization using NaOH results in porcine heart valves with significantly reduced xenoreactivity. [Extracted from the article]

This article discusses the use of the Ross/Ross-Konno procedure in pediatric cardiac surgery for aortic valve diseases and left ventricle outflow tract obstruction. The study analyzes the mid- and long-term clinical results of over 20 years of experience with this procedure. The results show successful long-term durability, low risk of complications, good hemodynamic performance, and potential for growth. The procedure offers a longer-lasting solution and avoids the need for lifelong anticoagulant therapy. However, there is a relatively common occurrence of homograft degenerations that require interventions, especially after the Ross-Konno procedure. [Extracted from the article]

Background: Congenital heart disease poses a therapeutic challenge, specifically pulmonary valve stenosis. This has been treated for many years with invasive procedures and bioprostheses, which over time, become dysfunctional due to the accumulation of fibrous tissue and calcification. Objective: The aim of this study is to describe the use of endovascular management in the right ventricular outflow tract, as the beginning of an ongoing effot to improve pediatric outcomes in developing countries. Methods: Seven pediatric patients with endovascular management of the right outflow tract are presented. Three of them underwent surgical valvuloplasty with persistent pulmonary stenosis. They decided to insert a percutaneous transcatheter pulmonary valve (PPVI) with a Melody valve using the valve-in-valve technique, with 100% stenosis and no complications associated with the procedure. Results: Four patients with successful percutaneous valve implantation had different congenital heart diseases. In addition, the case of a patient in whom an intentional pulmonary valve fracture was performed, an innovative procedure in pediatric endovascular management in the country, is highlighted. Conclusions: The procedure was minimally invasive, safe, and effective. The IVPP technique could be a viable option in our country for managing failed primary valve implantations or even in native tracts. [ABSTRACT FROM AUTHOR]

Background: Ultrasonography had diagnostic importance in the evaluation of different diseases in buffaloes, including cardiovascular diseases. Aim: The current work describes the normal echocardiographic findings in healthy buffaloes, along with establishing reference values for echocardiographic dimensions for both sides of the heart, i.e., left and right ones. Methods: About 30 healthy adult buffaloes that belonged to private farms in Assiut, Egypt, were included in this study. Each animal underwent a complete clinical evaluation as well as hematological analyses, lipid profile indices, liver functions, cardio-thoracic radiography, and echocardiography to confirm no diseased conditions were detected. The study was conducted on healthy buffaloes (n = 30) in Assiut Governorate, Egypt. Results: The obtained results reported healthy buffaloes with normal clinical findings as well as indices of blood pictures and serum biochemicals that were within the reference intervals. Radiography revealed a free reticulum and a welldefined diaphragm. The heart was seen as a typical radio-opaque organ. Ultrasonographically, using grayscale B-mode and M-mode, the heart was commonly imaged from the left fourth intercostal space. Different echocardiographic views were described, including the four chamber view, i.e., right atrium (RA), right ventricle (RV), tricuspid valve (TCV), left atrium, left ventricle, mitral valve, and interventricular septum (IVS), and the right ventricular outflow tract, i.e., RA, TCV, RV, pulmonary artery (PA), and pulmonary valve. Cross sections in each of the apex and base of the heart were described. Echocardiographic dimensions during cardiac diastole and systole, including diameters and wall thickness of each of the atria and ventricles, were demonstrated. Interventricular septal thickness wall thickness as well as diameters of the aorta and PA, were stated. Conclusion: The work tried to put reference values on the normal echocardiographic dimensions using 2-D B-mode gray scale ultrasonography in healthy adult buffaloes. These echocardiographic reference dimensions with normal echocardiographic imaging will be very helpful in enhancing the diagnostic efficacy of ultrasounds for recognizing abnormal findings related to cardiac disorders. [ABSTRACT FROM AUTHOR]

Introduction: Percutaneous pulmonary valve implantation (PPVI) with a SAPIEN 3 valve is effective for treating treat right ventricle outflow (RVOT) dysfunction. A modified technique was developed without prestenting using a protective valve delivery method. We aimed to compare the procedural results of the modified technique group (MTG) to those of patients in a conventional technique group (CTG). Methods: We designed a matched before–after study. All consecutive PPVI with SAPIEN 3 performed in the MTG over 9 months were matched, based on the RVOT type and size, to consecutive procedures performed previously with SAPIEN 3. Results: A total of 54 patients were included, equally distributed in the two groups. The sizes of the SAPIEN 3 valves were 23 mm (n = 9), 26 mm (n = 9), 29 mm (n = 36). The two groups were similar regarding demographic data, RVOT type, and pre-procedure hemodynamics. PPVI was performed in a single procedure in all patients of the MTG, whereas six (22.2%) patients of the CTG group underwent prestenting as a first step and valve implantation later (p = 0.02). The procedures were successful in all cases. Stent embolization was reported in two patients (7.4%) in the CTG, which were impacted in pulmonary arteries. In one case (3.7%), in the MTG, an unstable 29 mm SAPIEN 3 valve was stabilized with two stents and additional valve-in-valve implantation. The hemodynamics results were good in all cases, without significant differences between the two groups. The procedures' durations and fluoroscopy times were significantly reduced in the MTG (48.1 versus 82.6 min, p < 0.0001; 15.2 versus 29.8 min, p = 0.0002). During follow-up, neither stent fracture nor valve dysfunction was noticed in either group. Conclusion: PPVI without prestenting and with a protective delivery method of the SAPIEN 3 valve significantly reduces the procedure's complexity, the duration, and the irradiation while maintaining excellent hemodynamics results in selected cases. [ABSTRACT FROM AUTHOR]

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