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Context: A healthy, 22-year-old, male NCAA Division I baseball shortstop was experiencing confusion, chest pain, and tightness during an off-season intersquad scrimmage. The patient did not have any significant medical history or mechanism of head injury. After initial evaluation from the athletic trainer, the patient's cognitive status began to quickly decline. The emergency action plan was put in place rapidly and referred the patient to the local emergency clinic. Case Presentation: Upon arrival at the emergency department, an electrocardiogram was performed to rule out myocardial infarction or stroke. The first electrocardiogram results returned negative for any cardiac pathology, but a stroke alert was called. The patient was then transported to a level II trauma center due to continual cognitive decline. The patient was diagnosed with transient ischemic attack (TIA) secondary to an undiagnosed patent foramen ovale (PFO) that would later be diagnosed with further evaluation 2 months after the initial TIA incident. After multiple diagnostic and laboratory tests, the PFO went undetected until a 2D echocardiogram was performed and evaluated by a cardiologist. Management and Outcomes: After the confirmation of the congenital defect, surgical intervention was performed to correct the PFO using catheterization. Despite multiple preparticipation examinations, electrocardiograms, and examination of past family history, the PFO went undetected until the patient experienced symptoms of TIA. The discovery of PFO in this 22-year-old athletic individual is unusual because traditional screening techniques (electrocardiogram and preparticipation examinations) failed to detect the congenital defect. Conclusions: Due to the emergent and timely actions of the athletic trainer, the patient has made a full recovery and is able to compete fully in athletic events. This case study amplifies the need for athletic trainers at all sporting events, updated and reviewed emergency action plans, rapid recognition of TIA in athletic individuals, and return-to-play protocol for an athletic individual after TIA. [ABSTRACT FROM AUTHOR]

Background: Pulmonary artery banding (PAB) is performed as a palliative surgery for congenital heart diseases. Although pulmonary stenosis is one of the complications of PAB, symptomatic supravalvular pulmonary stenosis (SVPS) in adulthood after PAB is an extremely rare condition. Further, very few studies have focused on surgical cases of SVPS in adult. Herein, we describe a case of symptomatic SVPS in adulthood after PAB that was successfully managed with surgery. Case presentation: A 55-year-old male patient had been presenting with worsening shortness of breath for 10 years. Of note, he had been diagnosed with membranous ventricular septum defect at birth and he underwent PAB at 11 months of age. The patient underwent direct closure of ventricular septum defect and pulmonary artery debanding at 3 years and 6 months old. Medical examinations showed SVPS, with a pressure gradient of 60 mmHg. We planned pulmonary artery anterior wall plasty. However, based on the operative findings, the pulmonary stenosis was located in the whole circumference just above the annulus. Hence, it was challenging to completely relieve the stenosis with a pulmonary patch, and the Yacoub remodeling technique was applied to pulmonary artery reconstruction. Intraoperative pressure gradient between the right ventricle and the distal pulmonary artery dropped to 11 mmHg. Post operative course was uneventful. Conclusions: Patient with SVPS could present with symptoms in the long term after PAB in childhood. Therefore, due to the currently increasing number of adult congenital heart disease (ACHD) cases, attention should be paid to patients who underwent PAB. In this case, pulmonary artery was degenerated in the whole circumference because of the remained banding tape and the stenosis part was just above the annulus. Therefore, we applied Yacoub remodeling technique to this reconstruction. This pulmonary root remodeling procedure was an effective option for such SVPS. [ABSTRACT FROM AUTHOR]

Objective: Predicting whether postnatal balloon atrial septostomy (BAS) will be necessary for fetuses with dextro‐transposition of the great arteries (d‐TGA) remains challenging. We sought to determine whether fetal echocardiographic measurements obtained during maternal hyperoxygenation (MH) can improve our ability to predict the need for postnatal BAS. Methods: This was a retrospective, single‐institution review of d‐TGA fetuses whose mothers had undergone MH at 33–38 weeks' gestation at the Children's Hospital of Philadelphia, between 1 January 2017 and 30 March 2020. All fetal d‐TGA cases had either an intact ventricular septum or small ventricular septal defect measuring < 3 mm. Patent foramen ovale (PFO) size, measured by two‐dimensional imaging (2D) and by color Doppler, and patent ductus arteriosus (PDA) shunting (antegrade vs bidirectional) were assessed in room air (RA) and during MH. BAS status and timing were recorded. Results: Of 41 fetal d‐TGA cases in the cohort, 23 underwent postnatal BAS, while 18 did not, and 14 subjects underwent emergent BAS within 3 h of delivery. On univariate analysis, PFO size measured both in RA and during MH and antegrade shunting in the PDA during MH predicted BAS. During MH, median PFO size by 2D was significantly smaller in fetuses which underwent emergent BAS compared to fetuses which did not undergo BAS (2.5 (interquartile range (IQR), 2.0–3.0) mm vs 4.1 (IQR, 3.4–5.0) mm; P < 0.001). On cut‐point analysis, PFO size by 2D during MH of ≤ 3.2 mm predicted the need for emergent BAS with a sensitivity of 93% (95% CI, 66–100%) and a specificity of 78% (95% CI, 58–91%). Conclusions: In fetuses with d‐TGA, measurement of PFO size and direction of PDA shunting during MH improves our ability to predict the need for postnatal BAS, although additional study is needed. We propose incorporating third‐trimester MH when planning delivery of a d‐TGA fetus. © 2024 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Introduction: In children with myocarditis or dilated cardiomyopathy (DCM) on extracorporeal membrane oxygenation (ECMO) for cardiogenic shock, it is often necessary to decompress the left heart to minimize distension and promote myocardial recovery. We compare outcomes in those who underwent balloon atrial septostomy (BAS) versus direct left atrial (LA) drainage for left heart decompression in this population. Methods: Retrospective study of the Extracorporeal Life Support Organization (ELSO) multicenter registry of patients ≤ 18 years with myocarditis or DCM on ECMO who underwent LA decompression. Descriptive and univariate statistics assessed association of patient factors with decompression type. Multivariable logistic regression sought independent associations with outcomes. Results: 369 pediatric ECMO runs were identified. 52% myocarditis, 48% DCM, overall survival 74%. 65% underwent BAS and 35% LA drainage. Patient demographics including age, weight, gender, race/ethnicity, diagnosis, pre-ECMO pH, mean airway pressure, and arrest status were similar. 89% in the BAS group were peripherally cannulated onto ECMO, versus 3% in the LA drainage group (p <.001). On multivariable analysis, LA drainage (OR 3.96; 95% CI, 1.47-10.711; p =.007), renal complication (OR 2.37; 95% CI, 1.41-4.01; p =.001), cardiac complication (OR 3.14; 95% CI, 1.70-5.82; p <.001), and non-white race/ethnicity (OR 1.75; 95% CI, 1.04-2.94; p =.035) were associated with greater odds of mortality. There was a trend toward more episodes of pulmonary hemorrhage in BAS (n = 17) versus LA drainage group (n = 3), p =.08. Comparing only those with central cannulation, LA drainage group was more likely to be discontinued from ECMO due to recovery (72%) versus the BAS group (48%), p =.032. Conclusions: In children with myocarditis or DCM, there was a three times greater likelihood for mortality with LA drainage versus BAS for LA decompression. When adjusted for central cannulation groups only, there was better recovery in the LA drainage group and no difference in mortality. Further prospective evaluation is warranted. [ABSTRACT FROM AUTHOR]

Acute limb ischemia (ALI) is the sudden onset of decreased blood supply to the extremities and carries a poor prognosis for the affected limb and survival. A rare but well-recognized embolic etiology is a paradoxical embolism, the translocation of a thrombus from venous to arterial circulation through an intracardiac communication, most commonly a patent foramen ovale. The presentation of ALI secondary to a PFO-mediated paradoxical embolism is most often accompanied by combinations of deep vein thrombosis (DVT), pulmonary embolism (PE), and an acute cerebral or visceral ischemia. We present the first documented case of a Rutherford class I ALI secondary to a PFO-mediated paradoxical embolism, ipsilateral DVT, and PE in a 29-year-old female who was surgically managed for her disabling claudication rather than limb salvage. The overlapping presentation of a viable ALI and ipsilateral DVT created a challenging clinical diagnosis. Our review of the literature on PFO-mediated paradoxical emboli involved 43 reports including 51 patients with various arterial thromboses; 19 of these cases involved lower extremity ALI. This case report is the first case to date that demonstrates a paradoxical embolism causing acute lower extremity ischemia with ipsilateral DVT and no additional limb/visceral ischemia to suggest the diagnosis of ALI. We also highlight the role that quality of life plays in vascular surgical decision-making, extending ALI management goals to not only reducing mortality and major amputations, but also improving quality of life. [ABSTRACT FROM AUTHOR]

Aims Differentiating cardiac amyloidosis (CA) subtypes is important considering the significantly different therapies for transthyretin (ATTR)-CA and light chain (AL)-CA. Therefore, an echocardiographic method to distinguish ATTR-CA from AL-CA would provide significant value. We assessed a novel echocardiographic pixel intensity method to quantify myocardial calcification to differentiate ATTR-CA from phenocopies of CA and from AL-CA, specifically. Methods and results 167 patients with ATTR-CA (n = 53), AL-CA (n = 32), hypertrophic cardiomyopathy (n = 37), and advanced chronic kidney disease (n = 45) were retrospectively evaluated. The septal reflectivity ratio (SRR) was measured as the average pixel intensity of the visible anterior septal wall divided by the average pixel intensity of the visible posterior lateral wall. SRR and other myocardial strain-based echocardiographic measures were evaluated with receiver operator characteristic analysis to evaluate accuracy in distinguishing ATTR-CA from AL-CA and other forms of left ventricular hypertrophy. Mean SRR was significantly higher in the ATTR-CA cohort compared to the other cohorts (P < 0.001). SRR demonstrated the largest area under the curve (AUC) (0.91, P < 0.001) for distinguishing ATTR from all other cohorts and specifically for distinguishing ATTR-CA from AL-CA (AUC = 0.90, P < 0.001, specificity 96%, and sensitivity 63%). There was excellent inter- and intra-operator reproducibility with an ICC of 0.91 (P < 0.001) and 0.89 (P < 0.001), respectively. Conclusion The SRR is a reproducible and robust parameter for differentiating ATTR-CA from other phenocopies of CA and specifically ATTR-CA from AL-CA. [ABSTRACT FROM AUTHOR]

Cellular heterogeneity is a well-accepted feature of tissues, and both transcriptional and metabolic diversity have been revealed by numerous approaches, including optical imaging. However, the high magnification objective lenses needed for high-resolution imaging provides information from only small layers of tissue, which can result in poor cell statistics. There is therefore an unmet need for an imaging modality that can provide detailed molecular and cellular insight within intact tissue samples in 3D. Using GFP-tagged GLUT4 as proof of concept, we present here a novel optical mesoscopy approach that allows precise measurement of the spatial location of GLUT4 within specific anatomical structures across the myocardium in ultrathick sections (5 mm x 5 mm x 3 mm) of intactmouse heart.We reveal distinct GLUT4 distribution patterns across cardiac walls and highlight specific changes in GLUT4 expression levels in response to high fat dietfeeding, and we identify sex-dependent differences in expression patterns. This method is applicable to any target that can be labelled for light microscopy, and to other complex tissues when organ structure needs to be considered simultaneously with cellular detail. [ABSTRACT FROM AUTHOR]

Background: Routine Doppler study is a common tool for early diagnosis of Fetal Growth Restriction (FGR) and Small for Gestational Age (SGA) patients. It aimed to determine the role of the Foramen Ovale Pulsatility Index (FOPI) study beside routine Doppler study among patients with FGR and SGA fetuses. Methods: This prospective study was conducted on 35 FGR, 32 SGA, and 33 Appropriate for Gestational Age (AGA) fetuses. Demographic data, amniotic fluid index, neonatal outcome, and Doppler velocimetry, including Umbilical Artery Pulsatility Index (UMAPI), Uterine Artery Pulsatility Index (UTAPI), Middle Cerebral Artery Pulsatility Index (MCAPI), Ductus Venosus Pulsatility Index (DVPI), and FOPI were documented. Kolmogorov-Smirnov normality test, one-way ANOVA, Mann-Whitney U, Kruskal-Wallis, non-parametric pairwise comparisons adjusted for Bonferroni correction, Pearson correlation test, Chi square, Fisher's exact test, and Receiver Operating Characteristic Curve (ROC) analysis with Youden's Index (sensitivity+specificity-1) to estimate cut-off point were used to analyze the data at significance level <0.05 for all tests. Results: FOPI cut-off points were 2.24 (sensitivity=77%, specificity=94%) and 1.15 (sensitivity=90%, specificity=20%) to predict FGR and SGA, respectively. FOPI showed a positive correlation with UMAPI and UTAPI (r=0.52 and r=0.30, P<0.001 and P=0.006, respectively), but not with MCAPI and DVPI (r=0.08 and r=0.12, P=0.50 and P=0.30, respectively). Besides, UMAPI, UTAPI, and FOPI were altered among patients with stages I and II FGR. Umbilical cord potential hydrogen (umbilical cord pH), 1- and 5-min Apgar score significantly increased by Birth weight centile; however, UMAPI, FOPI, and UTAPI significantly decreased. Conclusion: UMAPI is recommended to predict short-term neonatal morbidities and demonstrate the early or late onset FGR. Besides, FOPI is suggested as the first-line Doppler study to detect abnormal growth velocity. More studies are warranted, especially considering long-term neonatal morbidities. [ABSTRACT FROM AUTHOR]

Introduction: Transthoracic (TTE) follow up guidelines after PFO device closure are vague. The primary goal of this study was to perform a literature search to characterize the timing of complications that occur after PFO device placement to determine the utility of long‐term routine TTE. Methods: A search was performed in Medline (PubMed) with English language and publication date (2000–2023) filters were applied. Studies were included if they reported on patients who underwent a PFO device closure. Studies were excluded if they only reported on ASD device closure patients, were meta‐analysis or review papers, or did not report any outcomes for the PFO device procedure. Results: A total of 305 articles met the criteria (observational cohort study, n = 196; case study, n = 99; and randomized control study, n = 10). Age at the time of PFO device closure was 47.5 ± 9.3 years. The incidence of complications was 6.9% (3358/48 348). Maximum range of follow‐up was 0–17 years. The plurality of complications was due to arrhythmias (46.3%, 1554/3358), followed by residual shunts (29.7%, 999/3358). The majority of complications occurred ≤6 months post‐device placement (57.8%, 1941/3358). Only 0.8% (27/3358) of complications took place >5 years. All patients with complications >5 years of device placement were presented with clinical symptoms related to their complications. Conclusion: The incidence of patients presenting with PFO‐reported complications beyond 5 years post‐procedure diminished. The utility of routine TTE beyond 5 years post‐PFO after device placement should be further assessed. [ABSTRACT FROM AUTHOR]

The halo-shape technique (HST) is an emerging approach for implanting a leadless pacemaker in scoliosis patients in recent years. Severe scoliosis and humpback made it challenging to push the tip of the delivery catheter towards the ventricular septum using the conventional gooseneck-shape technique. The feasibility and safety of the use of HST in an octogenarian with severe dextroscoliosis and humpback have not been well-assessed. Here, we report a case of high-degree atrioventricular block octogenarian with severe dextroscoliosis and humpback who successfully received a leadless pacemaker implantation using HST. Procedure-related complications were not observed, and the electrical parameters were stable at 6-month follow-up. [ABSTRACT FROM AUTHOR]

Introduction Methods Results Conclusion Left bundle branch area pacing (LBBAP) comprises pacing at the left ventricular septum (LVSP) or left bundle branch (LBBP). The aim of the present study was to investigate the differences in ventricular electrical heterogeneity between LVSP, LBBP, right ventricular pacing (RVP) and intrinsic conduction with different dyssynchrony measures using the ECG, vectorcardiograpy, ECG belt, and Ultrahigh frequency (UHF‐)ECG.Thirty‐seven patients with a pacemaker indication for bradycardia or cardiac resynchronization therapy underwent LBBAP implantation. ECG, vectorcardiogram, ECG belt and UHF‐ECG signals were recorded during RVP, LVSP and LBBP, and intrinsic activation. QRS duration (QRSd) was measured from the ECG, QRS area was calculated from the vectorcardiogram, LV activation time (LVAT) and standard deviation of activation time (SDAT) from ECG belt and electrical dyssynchrony (e‐DYS16) from UHF‐ECG.Both LVSP and LBBP significantly reduced ventricular electrical heterogeneity as compared to underlying LBBB and RV pacing in terms of QRS area (p < .001), SDAT (p < .001), LVAT (p < .001) and e‐DYS16 (p < .001). QRSd was only reduced as compared to RV pacing(p < .001). QRS area was similar during LBBP and normal intrinsic conduction, e‐DYS16 was similar during LVSP and normal intrinsic conduction, whereas SDAT was similar for LVSP, LBBP and normal intrinsic conduction. For all these variables there was no significant difference between LVSP and LBBP.Both LVSP and LBBP resulted in a more synchronous LV activation than LBBB and RVP. Especially LBBP resulted in levels of LV synchrony comparable to normal intrinsic conduction. [ABSTRACT FROM AUTHOR]

Introduction: Scar substrate in nonischemic cardiomyopathy (NICM) patients is often difficult to identify. Advances in cardiac imaging, especially using late iodine‐enhanced computed tomography (LIE‐CT), allow better characterization of scars giving rise to ventricular tachycardia (VT). Currently, there are limited data on clinical correlates of CT‐derived scar substrates in NICM. We sought assess the relationship between scar location on LIE‐CT and outcomes after radiofrequency catheter ablation (RFCA) in NICM patients with VT. Methods: From 2020 to 2022, consecutive patients with NICM undergoing VT RFCA with integration of cardiac CT scar modeling (inHeart, Pessac, France) were included at two US tertiary care centers. The CT protocol included both arterial‐enhanced imaging for anatomical modeling and LIE‐CT for scar assessment. The distribution of substrate on CT was analyzed in relation to patient outcomes, with primary endpoints being VT recurrence and the need for repeat ablation procedure. Results: Sixty patients were included (age 64 ± 12 years, 90% men). Over a median follow‐up of 120 days (interquartile range [IQR]: 41–365), repeat ablation procedures were required in 32 (53%). VT recurrence occurred in 46 (77%), with a median time to recurrence of 40 days (IQR: 8–65). CT‐derived total scar volume positively correlated with intrinsic QRS duration (r =.34, p = 0.008). Septal scar was found on CT in 34 (57%), and lateral scar in 40 (7%). On univariate logistic regression, septal scar was associated with increased odds of repeat ablation (odds ratio [OR]: 2.9 [1.0–8.4]; p = 0.046), while lateral scar was not (OR: 0.9 [0.3–2.7]; p = 0.855). Septal scar better predicted VT recurrence when compared to lateral scar, but neither were statistically significant (septal scar OR: 3.0 [0.9–10.7]; p = 0.078; lateral scar OR: 1.7 [0.5–5.9]; p = 0.391). Conclusion: In this tertiary care referral population, patients with NICM undergoing VT catheter ablation with septal LIE‐CT have nearly threefold increased risk of need for repeat ablation. [ABSTRACT FROM AUTHOR]

Congenital interventricular septal aneurysms (IVSA) of the muscular septum are rare and can be associated with other familial abnormalities of the ventricular septum, arrhythmias, additional congenital heart disease, and chromosomal abnormalities. IVSA is also linked to ventricular dysfunction and non-compaction, although there are limited reports of this association presenting in utero. We describe a case of fetal ventricular septal aneurysm associated with ventricular dysfunction and pericardial effusion. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Trans‐coronary ethanol ablation for ventricular tachycardia originating from the ventricular septum is effective, but there are cases with no septal perforator from left anterior descending artery. CT and angiography can reveal the optimal vessel. [ABSTRACT FROM AUTHOR]

Heart transplant prolongs life for patients with end-stage heart failure but rejection remains a complication that reduces long-term survival. The aim is to provide a comprehensive overview of the current status in HT rejection. EMB is an invasive diagnostic tool, consisting in the sampling of a fragment of myocardial tissue from the right ventricular septum using fluoroscopic guidance. This tissue can later be subjected to histopathological, immunohistochemical or molecular analysis, providing valuable information for cardiac allograft rejection, but this procedure is not without complications. To increase the accuracy of the rejection diagnosis, EMB requires a systematic evaluation of endocardium, myocardium, interstitium and intramural vessels. There are three types of rejection: hyperacute, acute or chronic, diagnosed by the histopathological evaluation of EMB as well as by new diagnostic methods such as DSA, ddcfDNA and gene expression profiling, the last having a high negative predictive value. More than 50 years after the introduction of EMB in medical practice, it still remains the "gold standard" in monitoring rejection in HT recipients but other new, less invasive diagnostic methods reduce the number of EMBs required. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the enhancement of image quality achieved through the utilization of SnapShot Freeze 2 (SSF2), a comparison was made against the results obtained from the original SnapShot Freeze algorithm (SSF) and standard motion correction (STND) in stent patients undergoing coronary CT angiography (CCTA) across the entire range of heart rates. Materials and methods: A total of 118 patients who underwent CCTA, were retrospectively included in this study. Images of these patients were reconstructed using three different algorithms: SSF2, SSF, and STND. Objective assessments include signal‐to‐noise ratio (SNR), contrast‐to‐noise ratio (CNR), diameters of stents and artifact index (AI). The image quality was subjectively evaluated by two readers. Results: Compared with SSF and STND, SSF2 had similar or even higher quality in the parameters (AI, SNR, CNR, inner diameters) of coronary artery, stent, myocardium, MV (mitral valve), TV (tricuspid valve), AV (aorta valve), and PV (pulmonary valve), and aortic root (AO). Besides the above structures, SSF2 also demonstrated comparable or even higher subjective scores in atrial septum (AS), ventricular septum (VS), and pulmonary artery root (PA). Furthermore, the enhancement in image quality with SSF2 was significantly greater in the high heart rate group compared to the low heart rate group. Moreover, the improvement in both high and low heart rate groups was better in the SSF2 group compared to the SSF and STND group. Besides, when using the three algorithms, an effect of heart rate variability on stent image quality was not detected. Conclusion: Compared to SSF and STND, SSF2 can enhance the image quality of whole‐heart structures and mitigate artifacts of coronary stents. Furthermore, SSF2 has demonstrated a significant improvement in the image quality for patients with a heart rate equal to or higher than 85 bpm. [ABSTRACT FROM AUTHOR]

Background: In this study, we examine the association between the hyperuricemia(HU) and hypertension(HTN) in Chinese young adults. Besides, the correlation between the occurrence of thickened left ventricular wall and HU was identified in patients with HTN. Methods: In all, 360 patients with HTN and 1991 young adults with normal blood pressure(NBP) were enrolled in the study. Participant characteristics were collected. Univariable and multivariable logistic regression tests were utilized to identify the correlation between the presence of HU and HTN, and the correlation between the occurrence of thickened ventricular septum and HU in patients with HTN. Results: The prevalence of HU in Chinese young adults with HTN was significantly higher than young adults with NBP(36.39% vs. 16.93%). Univariable analyses revealed that 8 factors were related with the presence of HTN with p value < 0.001, including HU, male, body mass index(BMI) ≥ 24 kg/m2, total cholesterol(TC) > 5.17mmol/L, triglyceride(TG) > 1.70mmol/L, high density lipoprotein cholesterol(HDL-C) < 1.0mmol/L, fasting blood glucose(FBG) > 6.10mmol/L and fatty liver. After adjusting these covariates, multivariable analysis revealed that HU[odds ratio(OR):1.47, 95% confidence interval(CI): 1.10–1.95, p = 0.008] remained independent association with HTN in young adults. Additionally, univariable and multivariable logistic analyses revealed that HU kept the independent effect on the presence of thickened interventricular septum(adjusted OR = 1.81, 95% CI: 1.05–3.11, P = 0.03) and thickened left ventricular posterior wall(adjusted OR = 2.28, 95% CI: 1.28–4.08, P = 0.005) in young adults with HTN. Conclusion: HU was independently associated with HTN in young adults. HU was independently correlated with thickened left ventricular wall, including interventricular septum and left ventricular posterior wall, in young adults with HTN. [ABSTRACT FROM AUTHOR]

Objective The aim of this study was to investigate fetal echocardiographic findings in predicting the need for surgical repair in fetuses with coarctation of the aorta (CoA) and to evaluate perinatal outcomes. Study Design In this retrospective study, fetuses diagnosed with CoA in a tertiary center between January 2015 and June 2021 were analyzed. Fetal echocardiographic measurements and quantitative findings, middle cerebral artery (MCA) and umbilical artery (UA) Doppler indices, and perinatal outcomes were recorded. Results A total of 57 fetuses with CoA were included in the study. In total, 51 (89.5%) pregnancies resulted in live births and 32 (62.8%) of the neonates underwent surgical repair. The left ventricle/right ventricle width ratio and aortic isthmus z -score were significantly lower in fetuses who underwent surgical repair (p = 0.004 0.001, respectively). Retrograde flow in the aortic isthmus (odds ratio [OR]:7.43; 95% confidence interval [CI]: 1.98–27.76), left-to-right foramen ovale shunt (OR: 8.50; 95% CI: 1.68–42.98), and ventricular septal defect (OR: 9.63; 95% CI: 1.90–48.74) were associated with the need for surgical repair. A new scoring system integrating these echocardiographic findings had 89% specificity and 54% sensitivity in predicting surgical repair. Fetal growth restriction rates, preterm birth rates, mean MCA pulsatility index (PI), and mean UA PI were similar in fetuses with and without surgical repair. Conclusion A scoring system integrating echocardiographic findings in fetuses with CoA may improve the prediction of surgical repair need. There is no evidence of an increased risk of FGR, preterm birth, and brain sparing effect in fetuses with CoA who require surgical repair. Key Points Coarctation of the aorta is one of the most difficult congenital heart defects to diagnose. A new scoring system may improve the prediction of surgical repair need. There is no increased risk of fetal growth restriction in fetuses requiring early surgical repair. [ABSTRACT FROM AUTHOR]

The aim of this study is to examine the frequency of maxillary sinus pathologies and their relationship with each other using cone beam computed tomography (CBCT) and to make a new grade according to the number of affected walls. 1000 maxillary sinuses of 500 patients with CBCT images were included in the study. Anatomical variations and pathological formations of the maxillary sinuses were examined. Images were evaluated for the presence of flat, polypoidal, partial and generalized mucosal thickening, partial and total opacification, polyps and mucous retention cysts. Maxillary sinus pathologies were graded according to the number of walls affected. In the examined CBCT images, no pathology was found in 54.2% of the maxillary sinuses, while pathology was observed in 45.8%. The most common sinus pathologies were mucous retention cyst (12.3%) and polypoidal thickening (12.2%). While pneumatization, ostium obstruction, and the presence of sinus-related roots were associated with sinus pathology, no relationship was found with nasal septum deviation and the presence of septa. Before dental implant and sinus surgery applications, the presence of sinus pathologies and their relationship with anatomical variations can be evaluated with CBCT, a three-dimensional technique, and complications such as sinus membrane perforation, infection, failure to break the bone window due to the presence of antral septa, graft loss and oroantral fistula formation can be reduced. [ABSTRACT FROM AUTHOR]

Whereas medical practice stems from Hippocrates, cardiovascular science originates with Aristotle. The Hippocratic philosophy was championed by Galen (129-216 CE), whose advocacy of a tripartite soul found favor in the early Christian Church. In contrast, Aristotle's works were banned as heresy by ecclesiastical authority, only to survive and prosper in the Islamic Golden Age (775-1258 CE). Galen theorized that the circulation consisted of separate venous and arterial systems. Blood was produced in the liver and traveled centrifugally through veins. When arriving in the right ventricle, venous blood passed through tiny pores in the ventricular septum into the left ventricle, where it became aerated by air passing from the lungs through the pulmonary veins to the left side of the heart. Following arrival at distal sites, arterial blood disappeared, being consumed by the tissues, requiring that the liver needed to continually synthesize new blood. The heart was viewed as a sucking organ, and the peripheral pulse was deemed to result from changes in arterial tone, rather than cardiac systole. Galen's framework remained undisputed and dominated medical thought for 1,300 years, but the reintroduction of Aristotelian principles from the Islamic world into Europe (through the efforts of the Toledo School of Translators) were nurtured by the academic freedom and iconoclastic environment uniquely cultivated at the University of Padua, made possible by Venetian rebellion against papal authority. At Padua, the work of Andreas Vesalius, Realdo Colombo, Hieronymus Fabricius ab Acquapendente, and William Harvey (1543-1628) methodically destroyed Galen's model, leading to the modern concept of a closed-ended circulation. Yet, due to political forces, Harvey was ridiculed, as was James Lind, who performed the first prospective controlled trial, involving citrus fruits for scurvy (1747); it took nearly 50 years for his work to be accepted. Even the work of William Withering (1785), the father of cardiovascular pharmacology, was tarnished by professional jealously and the marketing campaign of a pharmaceutical company. Today's cardiovascular investigators should understand that major advances are routinely derided by the medical establishment for political or personal reasons; and it may take decades or centuries for important work to be accepted. [Display omitted] • Medical practice stems from Hippocrates; cardiovascular science originates with Aristotle. • Galen's theory of separate venous and arterial systems—dominant for 1,300 years—was destroyed by scholars at the University of Padua (1500-1650 CE). • Landmark works by Harvey, Lind, and Withering were derided and tarnished by political and personal reasons. • Young investigators must challenge the status quo; if they do not rebel, nothing will change. [ABSTRACT FROM AUTHOR]

This article discusses the genetic determinants of atrial septal defect (ASD), a common congenital heart disease. The study identified five rare genetic variants in the FOXC1 gene in both familial and sporadic cases of ASD. Functional analysis confirmed that two of these variants, R173H and P79L, led to significant changes in FOXC1 transcriptional activity. Additionally, a mouse model with the R173H variant showed a thinning of the atrial septum, similar to the phenotype observed in humans. These findings provide new insights into the genetic causes of ASD and may contribute to improved diagnosis and counseling for affected individuals. [Extracted from the article]

To summarize echocardiographic characteristics of the anatomy and hemodynamic and clinical outcomes in fetuses with isolated pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS). This was a single-center retrospective study of fetuses with isolated PS or PA/IVS. Echocardiographic variables and clinical outcomes after delivery were evaluated and compared. Between 2016 and 2021, 115 livebirths with isolated PS or PA/IVS were included. Proportion of fetuses with mild, moderate and critical PS and PA/IVS was 41.7 %, 18.3 %, 26.1 % and 13.9 %. Fetuses with more severe PS had worse anatomic and hemodynamic profiles. Specifically, the cardiothoracic ratio, pulmonary valve (PV) velocity, degree and velocity of tricuspid regurgitation increased as PS severity increased; and the pulmonary artery/aorta ratio, right ventricle/left ventricle long-axis (TV/MV) ratio, tricuspid valve/mitral valve annulus (TV/MV) ratio, and tricuspid valve inflow duration/cardiac cycle ratio decreased as PS severity increased (P <0.001 for all). PV velocity ≥2 m/s predicted PV pressure ≥40 mm Hg after delivery, with an AUC of 0.81; TV/MV ratio combined with RV/LV ratio predicted clinical outcomes, with an AUC of 0.88. Live births with more severe PS had higher mortality rate (mild 0 vs. moderate 0 vs. critical 11 % vs. PA-IVS 36 %) and lower rate of developing bi-ventricles (mild 100 % vs. moderate 95 % vs. critical 89 % vs. PA-IVS 36 %). Findings of this study help better understand the anatomy and hemodynamic and clinical outcomes in fetuses with isolated PS or PA/IVS, which could have implications for prenatal counseling and prediction of fetal outcome. [ABSTRACT FROM AUTHOR]

Background Cardiac resynchronization therapy (CRT) with biventricular pacing is a well-established therapy. Left bundle branch area pacing (LBBAP) is a safe technique providing physiological pacing, and LBBAP-optimized CRT (LOT-CRT) has been shown to provide better electrical resynchronization than traditional CRT. However, there are few reports on shock lead placement in the left bundle branch area (LBBA) during CRT–defibrillator (CRT-D) implantation. Case summary A 76-year-old woman with heart failure from dilated cardiomyopathy presented with left bundle branch block pattern (QRS duration, 160 ms). Left ventricular ejection fraction was 21%. Cardiac resynchronization therapy–defibrillator implantation was performed due to worsening symptoms. By reshaping the Agilis HisPro catheter and adding a septal curve, the shock lead was placed deep into the ventricular septum, narrowing QRS duration to 114 ms. Left ventricular activation time was 84 ms. A defibrillation threshold test confirmed successful treatment without adverse events. At 6-month follow-up, left ventricular ejection fraction improved from 21 to 63%, with the patient's condition improving from New York Heart Association class III to class I. Discussion It was reported that QRS narrowing in CRT was related to long-term mortality, and LOT-CRT further decreased QRS duration as compared with LBBP only or biventricular pacing and increased the response rate. Combining LBBAP with coronary sinus pacing can potentially achieve superior electrical resynchronization. Lack of a suitable tool for direct shock lead placement in LBBA necessitated additional LBBAP lead in conventional LOT-CRT. Our successful LOT-CRT-D procedure with minimal number of leads through Agilis HisPro catheter reshaping enabled direct LBBA shock lead placement. [ABSTRACT FROM AUTHOR]

We describe the echocardiographic features of a 22-year-old female with a giant aneurysm of membranous ventricular septum (AMVS). Both transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) demonstrated significant dilatation of the aortic annulus and severe aortic regurgitation. A giant aneurysm was detected extending from a large membranous ventricular septal defect (MVSD) to the anterior surface of the aortic root. Contrast-enhanced CT and three-dimensional CT revealed a giant aneurysm located below the aortic root and connected to the left ventricular outflow tract (LVOT). The diagnosis was confirmed by surgery and postoperative pathological examination. [ABSTRACT FROM AUTHOR]

Objective: To analyze the outcome among neonates undergoing transcatheter baloon atrial septostomy for various kinds of congenital heart diseases. Study Design: Prospective longitudinal study. Place and Duration of Study: National Institute of Cardiovascular Diseases, Karachi Pakistan, from Feb 2020 to May 2021. Methodology: A total of 28 neonates of both genders with echocardiography proven diagnosis of Tricuspid Atresia with restrictive Patent Foramen Ovale, Dextro-Transposition of Great Arteries with Intact Ventricular Septum, complex cyanotic congenital heart disease with inadequate mixing or hypoplastic left heart syndrome with restrictive PFO and a non-significant Patent Ductus Arteriosus, undergoing transcatheter baloon atrial septostomy were included. Demographic data along with diagnosis, clinical presentation, echocardiographic findings, procedural outcome (successful/unsuccessful), duration of the procedure, complication (if any), post-procedural echocardiographic findings, duration of hospital stay, further treatment plan and 3-months folow up outcomes were recorded among al neonates. Results: Most common diagnosis was Dextro-Transposition of the Great Arteries with IVS observed in 14(50.0%) neonates. Procedural outcome was found to be successful among 23(82.1%) neonates. There were 19(67.9%) neonates who did not show any post-procedure complication. At 3-months folow up, improvement in blood saturation and weight gain were reported among 12(42.9%) neonates each. Overal, mortality was noted in 7(25.0%) neonates, of whom 4(57.1%) neonates were having hypoplastic left heart syndrome. Conclusion: Transcatheter atrial septostomy done in neonatal period was found to have positive outcomes. Mortality remains high among neonates undergoing transcatheter atrial septostomy having hypoplastic left heart syndrome. [ABSTRACT FROM AUTHOR]

Copyright of Anaesthesiologie & Intensivmedizin is the property of DGAI e.V. - Deutsche Gesellschaft fur Anasthesiologie und Intensivmedizin e.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article investigates the safety and efficacy of intramyocardial septal reduction for treating drug-refractory hypertrophic obstructive cardiomyopathy. Topics include significant reductions in left ventricular outflow tract gradient and maximum septal thickness after the procedure, improvements in patient symptoms and a low incidence of major adverse events and complications. The findings suggest that septal reduction is an effective treatment option with favorable safety outcomes.

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]

Background: Bow Hunter syndrome (BHS) is a rare, but important cause of posterior circulation stroke. It is also known as Rotational vertebral artery syndrome and is caused by transient dynamic vertebro-basilar insufficiency on movement of the neck in the presence of certain soft tissue or bone anomalies in the cranio-vertebral region. Case presentation: We present a case of Bow hunter syndrome in an 18-year-old adult male who presented with vomiting, occipital headache and loss of balance, with findings of posterior circulation stroke on imaging. Medical causes of young stroke, including vasculitis and clotting disorders were ruled out, following which a diagnostic conventional angiography and CT angiography was performed. The cause of vascular compromise in our case was the presence of vertebral anomalies, in particular, the presence of a partial ponticulus posticus with formation of an incomplete arcuate foramen. He was treated with cervical spine immobilisation and C1–C2 fixation. In addition, our patient had a single posterior inferior cerebellar artery (PICA) on the side of the dynamic insufficiency, which lead to bilateral cerebellar infarcts. Our case is unique because it demonstrates a combination of osseous and vascular developmental anomalies resulting in posterior circulation stroke. Conclusions: Though uncommon, BHS should be considered in the list of differentials in otherwise unexplained cases of posterior circulation stroke. Conventional angiography with dynamic manoeuvres is the modality of choice for documenting the rotational vertebral artery occlusion. [ABSTRACT FROM AUTHOR]

Left ventricular hypertrophy (LVH) is a common clinical manifestation associated with cardiovascular adverse events. Relying solely on the subjective judgment, it is challenging to promptly and accurately diagnose mild LVH. Quantitatively measuring the interventricular septum and left ventricular posterior wall to diagnose mild LVH is a time‐consuming and labor‐intensive process that is prone to errors. To propose a novel method for rapid and automatic end‐to‐end diagnosis of mild LVH. We propose a novel end‐to‐end automated method for detecting mild LVH. This method achieves rapid end‐to‐end detection of mild LVH in echocardiographic videos without the need for quantitative measurements. Initially, representative frames are extracted from echocardiographic videos, and these frames are then automatically diagnosed by the proposed network to detect LVH. The network architecture primarily consists of three key components: a feature extractor, bidirectional LSTM, and attention module. The Vit‐b model achieved 88% video classification accuracy in the experiment where 32 frames are extracted, and the ViT‐l model achieves 92% video classification accuracy in the experiment where 16 frames are extracted. It is shown experimentally that extracting fewer video frames can diagnose LVH more accurately. The experiments illustrate the superior performance and competitiveness of this method compared to other approaches, potentially applicable to the clinic. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the impact of soccer training on cardiac adaptations in mildly hypertensive middle-aged women. Methods: Hypertensive premenopausal women (n = 41; age (mean ± SD): 44 ± 7 years; height: 166 ± 6 cm; weight: 78.6 ± 11.6 kg; body fat: 43.3 ± 5.2%) were randomized to soccer training (SOC, n = 21) or control (CON, n = 20). SOC performed three weekly training sessions for 15 weeks, whereas CON had no training or lifestyle changes during the same period. Cardiac structure and function were assessed by echocardiography pre-intervention and post-intervention. Results: Soccer training increased (P = 0.001) left ventricular mass index by 10% [95% CI 4; 15], while no changes occurred in CON (time × group interaction, P = 0.005). In addition, only SOC demonstrated a within-group increase (P = 0.01) of 8% [95% CI 2; 14] in left ventricular septum diameter. For markers of right ventricular remodelling, a within-group increase (P = 0.02) occurred for tricuspid annulus plane systolic excursion of 8% [95% CI 1; 14] in SOC only. Left atrial diameter index increased (P < 0.001) by 6% [95% CI 3; 10] after SOC, while it was unaffected in CON (time × group interaction, P = 0.02). For makers of diastolic function, SOC demonstrated a within-group increase (P = 0.02) in the average early diastolic mitral annulus velocity of 10% [95% CI 2; 19]. In addition, a reduction (P < 0.001) in mitral valve A velocity of − 19% [95% CI − 29; − 10] was observed following soccer training, which manifested in increased (P < 0.001) mitral valve E/A ratio of 34% [95% CI 16; 53] in SOC. No within-group changes were apparent in CON. Conclusion: In sedentary, mildly hypertensive, middle-aged women, 15 weeks of soccer training increases left ventricular mass and left atrial diameter and improves indices of left ventricular diastolic function. [ABSTRACT FROM AUTHOR]

Key Clinical Message: This case highlights the pitfalls and provides tips for the extraction of deeply implanted lumenless leads, and encourages careful lead selection in the current era of widespread left bundle branch area pacing. The extraction of cardiovascular implantable electronic device leads is sometimes complicated. We describe a case with difficult but successful extraction of SelectSecure, a lumenless permanent pacemaker lead, implanted deep in the ventricular septum, highlighting its pitfalls and tips in the current era of left bundle branch area pacing. [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]

Copyright of Ahi Evran Medical Journal is the property of Ahi Evran University and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Iron overload is a major problem in beta thalassemia patients due to repeated blood transfusions. The liver is the first organ to be loaded with iron. An accurate assessment of iron overload is necessary for managing iron chelation therapy in such patients. Iron quantification by MRI scores over liver biopsy due to its non-invasive nature. Methods: Fifty-one patients with thalassemia major were subjected to 3.0-T MRI. Multiecho T2* sequence was used to cover the entire liver. Region of interest (ROI) was placed in three areas with maximum signal change, and an average T2* value was obtained. Similarly, a single ROI was placed at the mid-interventricular septum in the heart, and T2* value was obtained. T2* values so obtained were converted to iron concentration with the help of a T2* iron concentration calculator. The liver iron values were correlated with serum ferritin value. Results: There was a significant negative correlation between liver iron concentration (LIC) and T2* value of the liver (r = − 0.895, p < 0.01) and between cardiac iron concentration (CIC) and T2* value of the heart (r = − 0.959, p < 0.01). There was a slight positive correlation between LIC and serum ferritin (r = 0.642, p < 0.01) and no correlation between CIC and serum ferritin (r = − 0.137, p = 0.354). Conclusions: MRI is a useful tool to titrate the doses of chelating agents as it is accurate and non-invasive, does not involve radiation hazards and hence can be repeated as and when needed. Simultaneous assessment of cardiac iron overload is an added advantage of MRI. [ABSTRACT FROM AUTHOR]

Background: The Koolen-de Vries syndrome (KdVS) is a relatively new rare disease caused by micro-deletion of 17q21.31 which was first reported by Koolen in 2006. Typical phenotypes for KdVS include hypotonia, developmental delay, moderate intellectual disability, and characteristic facial dysmorphism. Up to now, there was only one case report about anesthesia management of patient diagnosed KdVS. It was a 2-year-old girl who experienced an MRI exam under anesthesia. Case presentation: We described a 21-month-old boy who planned to undergo an orchidopexy under general anesthesia diagnosed with KdVS. He had an intellectual disability, characteristic facial dysmorphism, tracheo/laryngomalacia, patent foramen ovale, and cryptorchidism related to KdVS. Due to the complex condition especially the presence of tracheo/laryngomalacia, we took some special measures, including reducing the amount of long-acting opioid, keeping the spontaneous breath, performing a caudal block, and applying the laryngeal mask. But the laryngeal mask was changed to an endotracheal tube because it failed to provide adequate ventilation. The boy experienced mild laryngeal spasm and hypoxia after extubation, but lateral position and etomidate eased his breathing problem and re-intubation was avoided. It is indicated that anesthesia management for patients with orphan disease is a real challenge for all anesthesia providers. Conclusions: The Koolen-de Vries syndrome is a relatively new orphan disease involving multiple systems. Keeping spontaneous breath, evaluating airway potency to anesthetics, applying endotracheal tube, and post-extubation lateral or prone position may be helpful for airway management for patient with hypotonia and tracheo/laryngomalacia. KdVS patient needs prolonged post-anesthesia monitoring and/or medication for airway complications. [ABSTRACT FROM AUTHOR]

Objective: Restrictive foramen ovale (FO) in dextro-transposition of the great arteries (d-TGA) with intact ventricular septum may lead to severe life-threatening hypoxia within the first hours of life, making urgent balloon atrial septostomy (BAS) inevitable. Reliable prenatal prediction of restrictive FO is crucial in these cases. However, current prenatal echocardiographic markers show low predictive value, and prenatal prediction often fails with fatal consequences for a subset of newborns. In this study, we described our experience and aimed to identify reliable predictive markers for BAS. Methods: We included 45 fetuses with isolated d-TGA that were diagnosed and delivered between 2010 and 2022 in two large German tertiary referral centers. Inclusion criteria were the availability of former prenatal ultrasound reports, of stored echocardiographic videos and still images, which had to be obtained within the last 14 days prior to delivery and that were of sufficient quality for retrospective re-analysis. Cardiac parameters were retrospectively assessed and their predictive value was evaluated. Results: Among the 45 included fetuses with d-TGA, 22 neonates had restrictive FO postnatally and required urgent BAS within the first 24 h of life. In contrast, 23 neonates had normal FO anatomy, but 4 of them unexpectedly showed inadequate interatrial mixing despite their normal FO anatomy, rapidly developed hypoxia and also required urgent BAS ('bad mixer'). Overall, 26 (58%) neonates required urgent BAS, whereas 19 (42%) achieved good O2 saturation and did not undergo urgent BAS. In the former prenatal ultrasound reports, restrictive FO with subsequent urgent BAS was correctly predicted in 11 of 22 cases (50% sensitivity), whereas a normal FO anatomy was correctly predicted in 19 of 23 cases (83% specificity). After current re-analysis of the stored videos and images, we identified three highly significant markers for restrictive FO: a FO diameter < 7 mm (p < 0.01), a fixed (p = 0.035) and a hypermobile (p = 0.014) FO flap. The maximum systolic flow velocities in the pulmonary veins were also significantly increased in restrictive FO (p = 0.021), but no cut-off value to reliably predict restrictive FO could be identified. If the above markers are applied, all 22 cases with restrictive FO and all 23 cases with normal FO anatomy could correctly be predicted (100% positive predictive value). Correct prediction of urgent BAS also succeeded in all 22 cases with restrictive FO (100% PPV), but naturally failed in 4 of the 23 cases with correctly predicted normal FO ('bad mixer') (82.6% negative predictive value). Conclusion: Precise assessment of FO size and FO flap motility allows a reliable prenatal prediction of both restrictive and normal FO anatomy postnatally. Prediction of likelihood of urgent BAS also succeeds reliably in all fetuses with restrictive FO, but identification of the small subset of fetuses that also requires urgent BAS despite their normal FO anatomy fails, because the ability of sufficient postnatal interatrial mixing cannot be predicted prenatally. Therefore, all fetuses with prenatally diagnosed d-TGA should always be delivered in a tertiary center with cardiac catheter stand-by, allowing BAS within the first 24 h after birth, regardless of their predicted FO anatomy. [ABSTRACT FROM AUTHOR]

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009–2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was − 3.06 (25%, 75%: − 4.00, − 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29–0.75, p < 0.001) and OR = 3.75 (95% CI 1.46–9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p < 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival. [ABSTRACT FROM AUTHOR]

This article explores the pathogenesis of ventricular hypoplasia in pulmonary atresia with intact ventricular septum (PA-IVS), a type of hypoplastic right heart syndrome. The study utilizes patient-specific induced pluripotent stem cells (iPSCs) and single-cell RNA sequencing to investigate the differentiation and proliferation of cardiomyocytes in PA-IVS. The findings suggest that impaired cardiomyocyte proliferation and abnormal lineage differentiation may contribute to the development of right ventricle hypoplasia in PA-IVS. Additionally, metabolic dysfunction and elevated mitochondrial activity are observed in PA-IVS iPSC-derived cardiomyocytes. Further research using 3-dimensional iPSC-derived cardiac organoids is recommended to better understand the specific defects in hypoplastic right heart syndrome. [Extracted from the article]

Background: Tetralogy of Fallot is a congenital heart disease mostly diagnosed and treated in early childhood. However, there are some adult cases receiving treatment. Case presentation: We describe a 78-year-old Japanese woman who presented with severely hypertrophic right ventricle, ventricular septum defect, overriding aorta, and severe infundibular stenosis in the right ventricular outflow tract. As hypoxemia was mild and daily exertion was sufficiently possible, home oxygen therapy was introduced. After 1 month, she was referred because of a positive blood culture. The blood culture test was positive four times, therefore, the antibacterial drug was administered according to active infective endocarditis. SpO2 repeatedly decreased during hospitalization, thus oxygen was needed. As there were infective endocarditis onset and progressive hypoxemia, we planned a surgical correction. Conclusion: Tetralogy of Fallot was diagnosed and successfully treated with complete surgical correction, and the development of infective endocarditis was the definitive indication for surgery at this late age. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES In this study, we aimed to compare infants with univentricular hearts who underwent an initial ductus stenting to those receiving a surgical systemic-to-pulmonary shunt (SPS). METHODS All infants with univentricular heart and ductal-dependent pulmonary blood flow who underwent initial palliation with either a ductus stenting or a surgical SPS between 2009 and 2022 were reviewed. Outcomes were compared after ductus stenting or SPS including survival, probability of re-interventions and the probability to reach stage II palliations. RESULTS A total of 130 patients were evaluated, including 49 ductus stenting and 81 SPSs. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19 patients. There was comparable hospital mortality (2.0% stent vs 3.7% surgery, P  = 0.91) between the groups, but shorter intensive care unit stay (median 1 vs 7 days, P  < 0.01) and shorter hospital stay (median 7 vs 17 days, P  < 0.01) were observed in patients with initial ductus stenting, compared to those with SPS. However, acute procedure-related complications were more frequently observed in patients with ductus stenting, compared with those with SPS (20.4 vs 6.2%, P  = 0.01), and 10 patients needed a shunt procedure after the initial ductus stent. The cumulative incidence of reaching stage II was similar between ductus stenting and SPS (88.0 vs 90.6% at 12 months, P  = 0.735). Pulmonary artery (PA) index (median 194 vs 219 mm2/m2, P  = 0.93) at stage II was similar between patients with ductus stenting and SPS. However, the ratio of the left to the right PA index [0.69 (0.45–0.95) vs 0.86 (0.51–0.84), P  = 0.015] was higher in patients who reached stage II with surgical shunt physiology, compared with patients with ductus stent physiology. CONCLUSIONS After initial ductus stenting in infants with univentricular heart, survival is comparable and post-procedural recovery shorter, but more acute stent dysfunctions and lower development of left PA are observed, compared to acute shunt dysfunctions. The less invasive procedure and shorter hospital stay are at the expense of more stent reinterventions. [ABSTRACT FROM AUTHOR]

This article, published in the journal Circulation, discusses the rapid evolution of congenital heart surgery. It highlights the historical development of surgical procedures for congenital heart defects and the shift towards earlier corrective procedures. The article also explores the management of patients with a single ventricle and the challenges associated with end-organ dysfunction. Additionally, it discusses advancements in imaging technology and surgical planning, as well as the growing population of adults living with congenital heart defects. The author emphasizes the need for continued surgical innovation and improved patient-specific management to enhance patient outcomes and quality of life. [Extracted from the article]

The aim of this study was to assess the impact of cardiac magnetic resonance (CMR) on the diagnosis in patients with known or suspected left ventricular noncompaction (LVNC). We retrospectively reviewed the medical charts of 12,811 consecutive patients who had CMR studies between 2008 and 2022 in a large tertiary center. We included patients referred for CMR because of known or suspected LVNC. The study sample consisted of 333 patients, 193 (58.0%) male, median age 39.0 (26.8–51.0) years. Among 74 patients fulfilling the echocardiographic LVNC criteria, the diagnosis was confirmed in 54 (73.0%) cases. In 259 patients with ultrasound-based suspicion of LVNC, CMR led to an LVNC diagnosis in 82 (31.7%) patients. In both groups, CMR led to a new diagnosis in 89 cases (10 (13.5%) and 79 (30.5%)). A quantity of 38 (5.4%) patients were diagnosed with dilated cardiomyopathy, 11 (1.4%) patients were diagnosed with hypertrophic cardiomyopathy, and 21 (4.1%) patients were diagnosed with unclassified cardiomyopathy. In four patients with suspected LVNC, a myocardial trabeculation was a secondary result of dilatation due to coronary heart disease. In five cases, valvular heart disease was found. Four patients were diagnosed with athlete's heart. Other diagnoses (arrhythmogenic right ventricular cardiomyopathy, peripartum cardiomyopathy, hypokinetic non-dilated cardiomyopathy, sarcoidosis, amyloidosis, and ventricular septum defect) were found in six patients. CMR is a valuable tool in the evaluation of cardiac muscle and in differentiating LVNC and other cardiac diseases. [ABSTRACT FROM AUTHOR]

Purpose: This study seeks to examine the influence of the heartbeat on the position, volume, and shape of the heart and its substructures during various breathing states. The findings of this study will serve as a valuable reference for dose-volume evaluation of the heart and its substructures in radiotherapy for treating thoracic tumors. Methods: Twenty-three healthy volunteers were enrolled in this study, and cine four-dimensional magnetic resonance images were acquired during periods of end-inspiration breath holding (EIBH), end-expiration breath holding (EEBH), and deep end-inspiration breath holding (DIBH). The MR images were used to delineate the heart and its substructures, including the heart, pericardium, left ventricle (LV), left ventricular myocardium, right ventricle (RV), right ventricular myocardium (RVM), ventricular septum (VS), atrial septum (AS), proximal and middle portions of the left anterior descending branch (pmLAD), and proximal portion of the left circumflex coronary branch (pLCX). The changes in each structure with heartbeat were compared among different respiratory states. Results: Compared with EIBH, EEBH increased the volume of the heart and its substructures by 0.25–3.66%, while the average Dice similarity coefficient (DSC) increased by − 0.25 to 8.7%; however, the differences were not statistically significant. Conversely, the VS decreased by 0.89 mm in the left–right (LR) direction, and the displacement of the RV in the anterior–posterior (AP) direction significantly decreased by 0.76 mm (p < 0.05). Compared with EIBH and EEBH, the average volume of the heart and its substructures decreased by 3.08–17.57% and 4.09–20.43%, respectively, during DIBH. Accordingly, statistically significant differences (p < 0.05) were observed in the volume of the heart, pericardium, LV, RV, RVM, and AS. The average DSC increased by 0–37.04% and − 2.6 to 32.14%, respectively, with statistically significant differences (p < 0.05) found in the right ventricular myocardium and interatrial septum. Furthermore, the displacements under DIBH decreased in the three directions (i.e.,− 1.73 to 3.47 mm and − 0.36 to 2.51 mm). In this regard, the AP displacement of the heart, LV, RV, RVM, LR direction, LV, RV, and AS showed statistically significant differences (p < 0.05). The Hausdorff distance (HD) of the heart and its substructures under the three breathing states are all greater than 11 mm. Conclusion: The variations in the displacement and shape alterations of the heart and its substructures during cardiac motion under various respiratory states are significant. When assessing the dose-volume index of the heart and its substructures during radiotherapy for thoracic tumors, it is essential to account for the combined impacts of cardiac motion and respiration. [ABSTRACT FROM AUTHOR]

To demonstrate possible functional changes in the frequently affected fetal interventricular septum (IVS) with spectral tissue Doppler imaging (TDI) and M-mode imaging to compare gestational diabetes mellitus (GDM) and control groups. A total of 63 pregnant women with GDM, 30 on diet (A1 GDM) and 33 on treated with insulin (A2 GDM), and 63 healthy pregnant women randomly selected and matched to the case group in the control group were included. The GDM fetuses had significantly thickened IVS, increased early diastole (E′), atrial contraction (A′), systole (S′), higher myocardial performance index (MPI′), prolonged isovolumetric relaxation time (IVRT′), shortened ejection time (ET′), and decreased septal annular plane systolic excursion (SAPSE) than the controls. The A2 GDM group fetuses had significantly thickened IVS, increased S′ and shortened ET′ than the A1 GDM group. In the GDM group, we found a significantly positive low correlation between glycated hemoglobin levels and maternal serum fasting glucose and one-hour postprandial glucose with fetal IVS thickness. We demonstrated a significantly negative low correlation between maternal serum one-hour postprandial glucose, glycated hemoglobin levels, and gestational weight gain with fetal IVS ET′. Fetal IVS diastolic and systolic functions were altered in the GDM group compared to controls, and systolic functions were altered in A2 GDM compared to A1 GDM. This may alert clinicians to possible cardiovascular diseases in the postnatal life, and early preventive strategies and long-term lifestyle changes may provide protection in fetuses with GDM. [ABSTRACT FROM AUTHOR]