Search

Hypertrophic cardiomyopathy (HCM) remains the most common cardiomyopathy in humans and cats with few preclinical pharmacologic interventional studies. Small-molecule sarcomere inhibitors are promising novel therapeutics for the management of obstructive HCM (oHCM) patients and have shown efficacy in left ventricular outflow tract obstruction (LVOTO) relief. The objective of this study was to explore the 6-, 24-, and 48-hour (h) pharmacodynamic effects of the cardiac myosin inhibitor, CK-586, in six purpose-bred cats with naturally occurring oHCM. A blinded, randomized, five-treatment group, crossover preclinical trial was conducted to assess the pharmacodynamic effects of CK-586 in this oHCM model. Dose assessments and select echocardiographic variables were assessed five times over a 48-h period. Treatment with oral CK-586 safely ameliorated LVOTO in oHCM cats. CK-586 treatment dose-dependently eliminated obstruction (reduced LVOTOmaxPG), increased measures of systolic chamber size (LVIDs Sx), and decreased select measures of heart function (LV FS% and LV EF%) in the absence of impact on heart rate. At all tested doses, a single oral CK-586 dose resulted in improved or resolved LVOTO with well-tolerated, dose-dependent, reductions in LV systolic function. The results from this study pave the way for the potential use of CK-586 in both the veterinary and human clinical setting.

Arachnoid cysts are relatively common, but rarely require intervention. While most arachnoid cysts in typical middle or posterior cranial fossa locations are seldom symptomatic, suprasellar cysts may become symptomatic due to the potential for ventricular outflow obstruction and hydrocephalus. Typical standard of care for the treatment of these lesions is endoscopic fenestration with third ventriculostomy, or the placement of ventriculoperitoneal or cystoperitoneal shunts. The surgical and anaesthetic risks of traditional interventions may be higher in the early neonatal period, including leak of cerebrospinal fluid, infection, and premature failure of ventriculostomy or shunts. This note describes a novel bedside ultrasound-guided technique to percutaneously fenestrate large suprasellar arachnoid cysts under local anaesthesia. The technique involves insertion of a 25-g spinal needle until contact with the membrane of the arachnoid cyst medially, followed by a lateral sweeping to widely incise/fenestrate the lesion into the ventricular space under continuous ultrasound visualisation. This note describes an example case which demonstrates durable radiological and clinical improvement after 2 years of follow-up. This may represent a management option to temporise, or perhaps definitively manage suprasellar arachnoid cysts in the neonatal period. [ABSTRACT FROM AUTHOR]

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

The optimal approach for supravalvar right ventricular outflow tract obstruction(RVOTO) after arterial switch operation(ASO) is unclear. The results of percutaneous balloon dilatation have been variable. We report the results of simultaneous double balloon dilation for RVOTO after ASO. Sixteen patients (1.3(0.7–3.8) years; 9.8(8.1–15.1) kgs underwent the procedure at 14(8–44.5) months after ASO. Salient technical features included placement of balloons over stiff guide-wires positioned in both branch pulmonary arteries to enable dilation of the distal-most main pulmonary artery (MPA) with high inflation pressures (~ 12–14 atmospheres) and short inflation-deflation cycles. Effective balloon size was based on the PA annulus or MPA distal to the narrowing. The final balloon: narrowest segment diameter ratio was 2.7. Following dilation, the right ventricle to systemic systolic pressure ratio decreased from 0.9 ± 0.18 to 0.52 ± 0.16 (p < 0.001) and mean RVOT gradient from 78 ± 18 to 34 ± 13.9 mmHg (p < 0.001). Narrowest diameter improved from 5.4 ± 2.2 to 9.2 ± 2.2 mm. There were no major complications. Two patients with inadequate relief (final RV-systemic ratios: 1.03 and 0.7) were referred for surgery. At median follow up of 9 months, IQR 7–22, range 5–73, others are free of re interventions with median RVOT gradient of 42, IQR 27–49, range 21–55 mmHg. The immediate and short-term follow up results of double balloon dilatation for supravalvar RVOTO is encouraging and may avoid the need for repeat surgery in the majority of patients. Further follow up is needed to determine the long-term durability of the results. [ABSTRACT FROM AUTHOR]

Background: Transcatheter mitral valve replacement (TMVR) has emerged as a minimally invasive alternative to mitral valve surgery for patients at high or prohibitive operative risk. Prospective studies reported favourable outcomes in patients with annulus calcification (valve-in-mitral annulus calcification; ViMAC), failed annuloplasty ring (mitral valve-in-ring; MViR), and bioprosthetic mitral valve dysfunction (mitral valve-in-valve; MViV). Multi-slice computed tomography (MSCT)-derived 3D-modelling and simulations may provide complementary anatomical perspectives for TMVR planning. Aims: We aimed to illustrate the implementation of MSCT-derived modelling and simulations in the workup of TMVR for ViMAC, MViR, and MViV. Methods: For this retrospective study, we included all consecutive patients screened for TMVR and compared MSCT data, echocardiographic outcomes and clinical outcomes. Results: Sixteen out of 41 patients were treated with TMVR (ViMAC n = 9, MViR n = 3, MViV n = 4). Eleven patients were excluded for inappropriate sizing, 4 for anchoring issues and 10 for an unacceptable risk of left ventricular outflow tract obstruction (LVOTO) based on 3D modelling. There were 3 procedure-related deaths and 1 non-procedure-related cardiovascular death during 30 days of follow-up. LVOTO occurred in 3 ViMAC patients and 1 MViR patient, due to deeper valve implantation than planned in 3 patients, and anterior mitral leaflet displacement with recurrent basal septum thickening in 1 patient. TMVR significantly reduced mitral mean gradients as compared with baseline measurements (median mean gradient 9.5 (9.0–11.5) mm Hg before TMVR versus 5.0 (4.5–6.0) mm Hg after TMVR, p = 0.03). There was no residual mitral regurgitation at 30 days. Conclusion: MSCT-derived 3D modelling and simulation provide valuable anatomical insights for TMVR with transcatheter balloon expandable valves in ViMAC, MViR and MViV. Further planning iterations should target the persistent risk for neo-LVOTO. [ABSTRACT FROM AUTHOR]

The Netherlands Heart Journal in 2024 showcased significant advancements in cardiovascular care and research, with a focus on innovations in imaging techniques, pharmacotherapy, interventions, and special populations. Notable studies included the CLEAR-CAD trial for coronary artery disease, the TAVI XS trial for aortic valve stenosis, and the use of telemedicine in heart failure and atrial fibrillation management. The journal emphasized collaboration, discussion, and innovation to enhance cardiovascular research and care both in the Netherlands and globally, with a commitment to open access for widespread knowledge sharing. [Extracted from the article]

Simple Summary: In recent years, intraventricular pressure gradient (IVPG) measured using color M-mode, which is excellent for evaluating diastolic function, has gained attention. In this study, the IVPG of cats with hypertrophic cardiomyopathy (HCM) was evaluated. The results showed that in non-obstructive HCM (HNCM), there were no changes in IVPG, whereas in hypertrophic obstructive cardiomyopathy (HOCM), IVPG increased despite a suspected decline in diastolic function. The fact that a significant difference was observed only with IVPG suggests the existence of pathophysiological differences that can only be detected by IVPG. By using IVPG for the evaluation of HOCM, it is expected that more accurate assessment of the condition, improvement of treatment methods, and new prognostic indicators, which were previously unattainable, will become possible. Hypertrophic cardiomyopathy (HCM) is a common form of cardiomyopathy in cats, and heart failure occurs as diastolic dysfunction progresses. HCM in cats is broadly classified as non-obstructive and obstructive hypertrophic cardiomyopathy, depending on the presence or absence of outflow tract obstruction. Measurement of the intraventricular pressure differences (IVPD) using color M-mode (CMM) has attracted attention as a reliable diastolic index as it correlates with catheterization, the gold standard for the assessment of diastolic performance. Because IVPD is affected by the size of the heart, the intraventricular pressure gradient (IVPG) index, which is unaffected by heart size, is by calculated by dividing IVPD by LV length. In the present study, CMM IVPG was used to non-invasively assess diastolic impairment in cats with obstructive hypertrophic cardiomyopathy. This study was conducted on 10 control cats and 18 cats in the HCM group. Although no severe left atrial enlargement was observed in the HCM group, the basal IVPG was significantly increased in the HOCM group compared to the control group. Although IVPD typically suggests impaired diastolic function and reduced ventricular compliance, the significant increase observed in the HOCM group compared to controls may suggest an indirect elevation in left atrial pressure, likely secondary to left ventricular outflow tract obstruction. The increase in IVPG in HOCM, as shown in this study, is a pathological effect of left ventricular outflow tract obstruction that cannot be detected by conventional echocardiographic indices, and evaluating IVPG is useful to evaluate cardiac function from a perspective that differs from conventional methods. [ABSTRACT FROM AUTHOR]

Copyright of Egyptian Heart Journal is the property of Springer Nature 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.)

Mitral regurgitation mediated by systolic anterior motion is a typical manifestation in patients with hypertrophic obstructive cardiomyopathy. Although septal myectomy is currently the preferred treatment for patients with drug-refractory hypertrophic obstructive cardiomyopathy, surgical management of associated mitral regurgitation remains controversial. Whether septal myectomy alone or additional valve procedures are required to correct significant mitral regurgitation depends largely on the pathophysiology of each individual. For intervention purposes, it is important to identify the real systolic anterior motion-mediated mitral regurgitation and clarify the mitral structural abnormalities, as well as the other conditions, which may indicate the degree of mitral valve competence. As a first-line imaging modality in the management of hypertrophic cardiomyopathy, echocardiography could provide crucial information for surgical decision making. In this article, we focus on elucidating the typical echocardiographic features of systolic anterior motion-mediated mitral regurgitation and offer an assessment process to determine other contributors to obstruction and mitral regurgitation. Additionally, guidance for surgical decision making for mitral regurgitation in patients with hypertrophic obstructive cardiomyopathy is discussed. [ABSTRACT FROM AUTHOR]

The document presents research abstracts from the 34th ECVIM-CA Congress in Lyon, France, covering various topics in veterinary internal medicine. The studies explore areas such as echocardiography in cats, risk factors for death in cats with hepatic lipidosis, and antimicrobial prescribing practices in canine acute diarrhea. Additionally, research on bronchoalveolar lavage in dogs and cats, the effects of pimobendan on echocardiographic parameters in dogs with MMVD, and the prevalence of feline blood pathogens in blood donor cats are discussed. The findings offer valuable insights into veterinary medicine practices and contribute to the understanding of various health conditions in animals. [Extracted from the article]

Introduction: 5p deletion syndrome, also called Cri-du-chat syndrome 5p is a rare genetic syndrome with reports up to 36% of patients are associated with congenital heart defects. We investigated the association between left outflow tract obstruction and Cri-du-chat syndrome. Methods: A retrospective review of the abnormal microarray cases with congenital heart defects in Children's Hospital of Pittsburgh and the Cytogenomics of Cardiovascular Malformations Consortium. Results: A retrospective review at nine pediatric centers identified 4 patients with 5p deletions and left outflow tract obstruction (LVOTO). Three of these patients had additional copy number variants. We present data suggesting an association of LVOTO with 5p deletion with high mortality in the presence of additional copy number variants. Conclusion: A rare combination of 5p deletion and left ventricular outflow obstruction was observed in the registry of copy number variants and congenital heart defects. [ABSTRACT FROM AUTHOR]

BACKGROUND: Septal reduction therapy (SRT) provides substantial symptomatic improvement in patients with obstructive hypertrophic cardiomyopathy (HCM). However, long-term disease course after SRT and predictors of adverse outcomes have not been systematically examined. METHODS: Data from 13 high clinical volume HCM centers from the international SHARE (Sarcomeric Human Cardiomyopathy Registry) were analyzed. Patients were followed from the time of SRT until last follow-up or occurrence of heart failure (HF) composite outcome (cardiac transplantation, implantation of a left ventricular assist device, left ventricular ejection fraction <35%, development of New York Heart Association class III or IV symptoms), ventricular arrhythmias composite outcome (sudden cardiac death, resuscitated cardiac arrest, or appropriate implantable cardioverter defibrillator therapy), or HCM-related death. Cox proportional hazards models were used to identify predictors of outcome. RESULTS: Of the 10 225 patients in SHARE, 1832 (18%; 968 [53%] male) underwent SRT, including 455 (25%) with alcohol septal ablation and 1377 (75%) with septal myectomy. The periprocedural 30-day mortality rate was 0.4% (8 of 1832) and 1499 of 1565 (92%) had a maximal left ventricular outflow tract gradient <50 mm Hg at 1 year. After 6.8 years (range, 3.4–9.8 years; 12 565 person-years) from SRT, 77 (4%) experienced HCM-related death (0.6% per year), 236 (13%) a composite HF outcome (1.9% per year), and 87 (5%) a composite ventricular arrhythmia outcome (0.7% per year). Among adults, older age at SRT was associated with a higher incidence of HCM death (hazard ratio, 1.22 [95 CI, 1.1–1.3]; P <0.01) and the HF composite (hazard ratio, 1.14 [95 CI, 1.1–1.2] per 5-year increase; P <0.01) in a multivariable model. Female patients also had a higher risk of the HF composite after SRT (hazard ratio, 1.4 [95 CI, 1.1–1.8]; P <0.01). De novo atrial fibrillation occurred after SRT in 387 patients (21%). Among pediatric patients followed for a median of 13 years after SRT, 26 of 343 (16%) developed the HF composite outcome, despite 96% being free of recurrent left ventricular outflow tract obstruction. CONCLUSIONS: Successful short- and long-term relief of outflow tract obstruction was observed in experienced multidisciplinary HCM centers. A subset of patients progressed to develop HF, but event-free survival at 10 years was 83% and ventricular arrhythmias were rare. Older age, female sex, and SRT during childhood were associated with a greater risk of developing HF. [ABSTRACT FROM AUTHOR]

Aims: The aim of this study was to investigate whether the H2FPEF score, which was developed to improve the diagnosis of heart failure (HF) with preserved ejection fraction, is associated with HF outcomes in patients with hypertrophic cardiomyopathy (HCM). Methods and results: Patients with HCM and preserved left ventricular ejection fraction (LVEF ≥50%) were included from a multicentre registry and the H2FPEF score was calculated. Patients were divided into three groups: low (0–1), intermediate (2–5) and high (6–9) H2FPEF score. The primary combined endpoint was a composite of all‐cause death and HF admissions, while the secondary endpoints were all‐cause death and HF admissions separately. A total of 955 patients were included (age 51 ± 17 years, 310 [32.5%] female). Patients with a high H2FPEF score (n = 105) were more often female, and presented with more symptoms and comorbidities. On echocardiography, patients with a high H2FPEF score had lower LVEF, more impaired diastolic function and more frequently left ventricular outflow tract obstruction. During follow‐up (median 90 months [interquartile range 49–176]), 103 (11%) patients died and 57 (6%) patients had a first HF hospitalization. Event‐free survival rate for the primary combined and secondary endpoints was lower for patients with an intermediate and high H2FPEF score. On multivariate Cox regression analysis, female sex (hazard ratio [HR] 1.670, 95% confidence interval [CI] 1.157–2.410; p = 0.006), Asian ethnicity (HR 6.711, 95% CI 4.076–11.048; p < 0.001), ischaemic heart disease (HR 1.732, 95% CI 1.133–2.650; p = 0.011), left atrial diameter (HR 1.028, 95% CI 1.005–1.051; p = 0.016) and intermediate (HR 2.757, 95% CI 1.612–4.713; p < 0.001) or high H2FPEF score (HR 3.689, 95% CI 1.908–7.134; p < 0.001) were independently associated with the primary combined endpoint. Conclusion: The H2FPEF score is independently associated with HF outcome in patients with HCM and may be considered for risk stratification. [ABSTRACT FROM AUTHOR]

Background: Left ventricular outflow tract stenosis and atrioventricular valve regurgitation are often problems encountered in adulthood after complete atrioventricular septal defect repair. The surgical approach and indications for managing long-term outcomes such as left atrioventricular valve regurgitation and left ventricular outflow tract stenosis after complete atrioventricular septal defect repair have been discussed. Case presentation: A 23-year-old woman with intellectual disability was diagnosed with complete atrioventricular septal defect and underwent two-patch repair without cleft closure in childhood. Follow-up examination in adulthood demonstrated moderate left-sided atrioventricular valve regurgitation and left ventricular outflow tract stenosis with a circumferential ridge (peak velocity, 3.7 m/s; pressure gradient, 54 mmHg). Intraoperative findings showed a circumferential ridge under the aortic valve, and we removed the ridge. In addition, a cleft was present at the anterior leaflet, and we completely closed the cleft. Anticoagulation therapy was not initiated, and no embolic complications occurred. Follow-up echocardiography demonstrated no ridge under the aortic valve and only mild-range left AVVR. Conclusions: We successfully performed surgical treatment without valve replacement or anticoagulation therapy in a patient with poor medical compliance. Delayed reoperation leads to degeneration of the valve structure and makes more difficult to repair. Atrioventricular valve regurgitation should be evaluated in combination with based on the etiology of the regurgitation especially cleft related or not, in addition to the dilatation annulus, cleft size, and depth of the leaflet coaptation depth, and associated other valve diseases. [ABSTRACT FROM AUTHOR]

Background Aortic stenosis (AS) is the most commonly acquired valvular pathology in the western world. Aortic stenosis severity is typically evaluated with Doppler echocardiography. Evaluation of aortic gradients using standard Doppler echocardiography from the apical window may underestimate the true gradient due to misalignment of blood flow to the ultrasound beam and is often better evaluated from other imaging windows using a non-imaging continuous wave Doppler (NI-CWD) probe. Herein, we describe a unique case of AS being underestimated by dynamic acoustic shadowing from the apical window rather than beam misalignment. Case summary The patient is a Hispanic 31-year-old male with a congenital bicuspid aortic valve who underwent a balloon aortic valvuloplasty at age 13. At age 31, the patient underwent a repeat transthoracic echocardiogram (TTE) that was largely unchanged from his prior TTE from 15 years prior. Notably on this TTE, there was acoustic shadowing of colour Doppler in the distal left ventricular outflow tract and aortic valve during systole. While gradients only suggested moderate AS, the degree of left ventricular hypertrophy was suspicious for more severe AS. Only by using the NI-CWD probe at the right sternal border were we able to identify very severe AS with a peak velocity of 6.5 m/s and a mean gradient of 100 mmHg. Discussion In our specific case, dynamic acoustic shadowing of the aortic valve from the apical window obscured both imaging and Doppler signals. This acoustic shadowing was not present from the right sternal border with the NI-CWD probe, leading to an over 100% increase in aortic valve peak velocity and proper correction of AS severity. This allowed for expedited care and underscores the importance of such techniques. [ABSTRACT FROM AUTHOR]

Copyright of Canadian Veterinary Journal / Revue Vétérinaire Canadienne is the property of Canadian Veterinary Medical Association 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.)

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

Congenitally corrected transposition of the great arteries (ccTGA) is an infrequent and complex congenital malformation, which accounts for approximately 0.5% of all congenital heart defects. This defect is characterized by both atrioventricular and ventriculoarterial discordance, with the right atrium connected to the morphological left ventricle (LV), ejecting blood into the pulmonary artery, while the left atrium is connected to the morphological right ventricle (RV), ejecting blood into the aorta. Due to this double discordance, the blood flow is physiologically normal. Most patients have coexisting cardiac abnormalities that require further treatment. Untreated natural course is often associated with progressive failure of the systemic right ventricle (RV), tricuspid valve (TV) regurgitation, arrhythmia, and sudden cardiac death, which occurs in approximately 50% of patients below the age of 40. Some patients do not require surgical intervention, but most undergo physiological repair leaving the right ventricle in the systemic position, anatomical surgery which restores the left ventricle as the systemic ventricle, or univentricular palliation. Various types of anatomic repair have been proposed for the correction of double discordance. They combine an atrial switch (Senning or Mustard procedure) with either an arterial switch operation (ASO) as a double-switch operation or, in the cases of relevant left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD), intra-ventricular rerouting by a Rastelli procedure. More recently implemented procedures, variations of aortic root translocations such as the Nikaidoh or the half-turned truncal switch/en bloc rotation, improve left ventricular outflow tract (LVOT) geometry and supposedly prevent the recurrence of LVOTO. Anatomic repair for congenitally corrected ccTGA has been shown to enable patients to survive into adulthood. [ABSTRACT FROM AUTHOR]

Objective: To analyze the clinical manifestations, imaging characteristics, treatment measures, and efficacy of mitral valve aneurysm (MVA), and to enhance the clinicians' understandings of MVA. Methods: The clinical data of one patient with aortic valve bicuspid malformation leading to mitral valve anterior leaflet aneurysm were collected. The clinical diagnosis was confirmed based on the clinical characteristics and imaging features, the treatment methods were selected, and the efficacy was analyzed. The relevant literatures were reviewed. Results: The patient, a 68-year-old female, was admitted due to palpitations and shortness of breath for 13 years, and the symptoms worsened one month ago. Thirteen years ago, the patient experienced palpitations and shortness of breath without any inducement and was diagnosed with "heart valve disease" in the local hospital. The symptoms worsened one month ago, leading to hospitalization. The transthoracic two-dimensional echocardiography (TTE) results showed the left ventricular hypertrophy, bicuspid aortic valve with thickened and echogenic leaflets, the forward flow velocity was increased, and the aortic valve orifice area was 2. 0 cm²; the mitral valve anterior leaflet margin was slightly thickened and echogenic, presenting a cystic "honeycomb-like" structure closely related to the aortic valve regurgitation jet, which appeared to enter the "sac". The preoperative transesophageal echocardiography (TEE) results showed the bicuspid aortic valve malformation with the anterior leaflet prolapsing into the left ventricular outflow tract during diastole; a "sac-like" structure was detected on the atrial surface of the mitral valve anterior leaflet, changing shape with the cardiac cycle and communicating with left ventricular blood flow. The ultrasound diagnosis was bicuspid aortic valve malformation with transverse fissure, severe regurgitation with mild stenosis, and mitral valve anterior leaflet aneurysm. Intraoperatively, the aortic valve annulus was enlarged with the significant leaflet regurgitation. The leaflets were excised, and a 23 mm bioprosthetic valve was implanted in the aortic position. Upon exploration through the aortic valve orifice, a "sac-like" structure was found on the mitral valve anterior leaflet, which was not specially treated. The postoperative TEE results showed good echo and activity of the aortic bioprosthetic valve, and the "sac-like" structure on the mitral valve remained unchanged. The follow-up results at 10 d and 4 months after operation showed good echo and activity of the aortic bioprosthetic valve, and compared with before operation, there was no significant change in the nature and size of the mitral valve cystic lesion. Conclusion: MVA is clinically rare. TTE is currently the most valuable imaging diagnostic method for MVA, especially when combined with TEE, which is the best diagnostic method and can assist in the treatment and efficacy evaluation. [ABSTRACT FROM AUTHOR]

Background Mavacamten in Phase 2 and 3 clinical trials was well tolerated, reduced left ventricular outflow tract obstruction (LVOTO), and improved exercise capacity and symptoms. However, due to its recent introduction in the market, there is limited evidence from real-world patients with severe/multiple comorbidities and/or who are exposed to potential treatment interactions. Hypertension is common in patients with hypertrophic cardiomyopathy (HCM), but its impact on the treatment of LVOTO is undefined. Case summary A 55-year-old man with severely obstructive symptomatic HCM and Grade I arterial hypertension underwent treatment with mavacamten 5 mg. He presented an accelerated hypertension from Day 10 of treatment. On admission, he reported improvement of his dyspnoea [New York Heart Association (NYHA) Class II] and NT-pro BNP decreased to 1646 ng/L. Echocardiography showed a left ventricular ejection fraction of 60% with reduced systolic anterior motion and LVOTO (max 21 mmHg). Causes of secondary hypertension were excluded, and blood pressure (BP) was controlled by eplerenone and amlodipine introduction. Accelerated hypertension was concluded as a final diagnosis, and a potential causal link with the introduction of mavacamten was made. Evolution up to Day 135 proved a stabilization of the BP profile and of the LVOT gradient (max 36 mmHg) as well as improvement in functional capacity (NYHA Class I). Discussion We hypothesize that rapid relief of excess afterload may induce alterations potentially leading to high BP in patients with impaired peripheral vascular resistances. Patients with severe obstructive HCM and hypertension should be given special attention during mavacamten titration and should self-monitor the BP during this phase. [ABSTRACT FROM AUTHOR]

The article "Stress echocardiography in daily clinical practice: an update from the European Association of Cardiovascular Imaging" discusses the evolution of stress echocardiography (SE) as a functional imaging test for myocardial ischemia beyond coronary artery disease. The article highlights the need for a paradigm shift in SE methodology to include new parameters and emphasizes the non-clinical advantages of SE, such as low cost and minimal environmental impact. The SE protocol involves a comprehensive assessment of various parameters, including regional wall motion, LV volumes, lung ultrasound, and coronary flow velocity reserve. The article also discusses the diverse indications for SE, its accuracy in predicting outcomes, and its role in assessing conditions beyond CAD, such as heart failure, valvular heart diseases, and congenital heart diseases. [Extracted from the article]

This article from the European Heart Journal discusses several studies related to cardiovascular health. The first study examines the association between periprocedural myocardial infarction (PMI) and mortality following percutaneous coronary intervention (PCI). The authors find that all definitions of PMI are associated with an increased risk of all-cause death after PCI. The second study evaluates the safety and efficacy of a leaflet modification device called ShortCut in patients at risk for coronary artery obstruction undergoing transcatheter aortic valve implantation (TAVI). The authors conclude that ShortCut is safe and associated with favorable clinical outcomes. The third study compares ticagrelor monotherapy with ticagrelor-based dual antiplatelet therapy (DAPT) in patients with acute coronary syndrome (ACS) undergoing drug-eluting stent (DES) implantation. The authors find that ticagrelor monotherapy after short-term DAPT is associated with less major bleeding without an increase in ischemic events. The fourth study examines the relationship between adulthood adiposity and cardiac structure and function in later life. The authors find that higher adiposity over adulthood is associated with adverse cardiac structure and function. The fifth study investigates the effects of a 20-year infancy-onset dietary intervention on retinal microvasculature in young adulthood. The authors find that the dietary intervention has favorable effects on retinal microvasculature. The article also includes commentaries and a discussion forum. [Extracted from the article]

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

Background: Transposition of the great arteries (TGA) is the most common cyanotic congenital heart defect in neonates but with low prenatal detection rate. This study sought to review the prenatal diagnosis, associated abnormalities, and mid-term postnatal outcomes of fetuses with TGA and investigate the integrated prenatal and postnatal management for TGA neonates. Methods: A total of 134 infants prenatally diagnosed with TGA in Guangdong Provincial People's Hospital, China, from January 2009 to December 2022 were included in the study. The prenatal ultrasound data and neonatal records were reviewed to assess the accuracy of prenatal diagnosis. Univariate and multivariate logistic and Cox analyses were used to identify risk factors associated with prognosis in such individuals. Results: The population originated from 40 cities in 10 provinces in China, with integrated antenatal and postnatal management rate reaching 94.0% (126/134) and a high accuracy rate (99.3%) of prenatal primary diagnosis. The median period of follow-up was 1.6 [interquartile range (IQR) 0.1–4.3] years. There were 3 (2.2%) postnatal deaths, 118 (88.1%) patients undergoing arterial switch operation (ASO), 3 (2.2%) undergoing Rastelli operations and 5 (3.7%) doing stage operations. Of 118 patients receiving ASO, the major morbidity occurred in 64 patients (54.2%), and right ventricular outflow tract obstruction (RVOTO) in 31 (26.3%). In the multivariate logistic analysis, gestational ages at birth (OR = 0.953, 95% CI 0.910–0.991; p = 0.025) and cardiopulmonary bypass (CPB) time (OR = 1.010, 95% CI 1.000–1.030; p = 0.038) were identified as independent risk factors associated with major morbidity. In the Cox multivariate analysis, aortic cross-clamping time (HR = 1.030, 95% CI 1.000–1.050; p = 0.017) was identified as independent risk factor associated with RVOTO. Conclusion: Earlier gestational ages at birth and longer CPB time are significantly associated with increased morbidity. Integrated prenatal and postnatal management is recommended for patients with prenatal diagnosis of TGA. [ABSTRACT FROM AUTHOR]

Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5–7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly's development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations. Correction of the defect during infancy is the preferred treatment for D-TGA. Balloon atrial septostomy (BAS) is necessary prior to the operation. The recommended surgical correction methods include arterial switch operation (ASO) and atrial switch operation (AtrSR), as well as the Rastelli and Nikaidoh procedures. The most common postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency and neopulmonic stenosis, right ventricular (RV) outflow tract obstruction (RVOTO), left ventricular (LV) dysfunction, arrhythmias, and heart failure. Early diagnosis and treatment of D-TGA is paramount to the prognosis of the patient. Improved surgical techniques have made it possible for patients with D-TGA to survive into adulthood. [ABSTRACT FROM AUTHOR]

Background: Calcified amorphous tumor of the heart is a rare non-neoplastic cardiac mass composed of calcified nodules over amorphous fibrous tissue with degeneration and some chronic inflammation. Calcified amorphous tumor is often associated with mitral annular calcification in patients with end-stage renal disease on dialysis. However, the exact etiology of calcified amorphous tumors remains uncertain. Case presentation: A 77-year-old female with lung cancer showed a tumor with large mobility in the left ventricular outflow tract on transthoracic echocardiography. She had mitral annular calcification, although her renal function was normal. The tumor was excised surgically. Pathologically, the extracted specimen consisted of a calcified lesion without tumor tissue and was diagnosed as a calcified amorphous tumor. Conclusions: As the patient had no other risk factors for calcified amorphous tumor except mitral annular calcification, we considered the association of blood coagulation abnormalities due to cancer-related thrombosis. This case suggests that calcified amorphous tumors may be associated with malignant tumors. [ABSTRACT FROM AUTHOR]

Rest imaging in hypertrophic cardiomyopathy may underestimate or miss left ventricular outflow tract obstruction, leading to suboptimal management decisions that negatively affect symptomatic patients. The 2024 hypertrophic cardiomyopathy guidelines describe exercise stress testing as an important tool to determine overall exercise tolerance and latent, exercise-provoked left ventricular outflow tract obstruction. [ABSTRACT FROM AUTHOR]

This article discusses the use of transvaginal SlowflowHD imaging technology to examine the development of embryonic and fetal hearts in the first trimester of pregnancy. The study found that by the 11th week of gestation, all major structures of the heart had developed. The article also mentions previous studies that used older technology to visualize cardiac structures, but the quality of the images was poor. The authors conclude that transvaginal SlowflowHD imaging provides a high-resolution and sensitive method for assessing cardiac structures in early pregnancy. The use of SlowflowHD has the potential to diagnose congenital heart anomalies and provide valuable diagnostic information on various heart conditions. However, it cannot determine flow direction and velocity, and further research is needed to explore its potential role in early fetal cardiac diagnosis. [Extracted from the article]

Left ventricular outflow tract obstruction is a rare complication following transcatheter mitral valve implantation. Diagnosing the underlying cause is mandatory to select from different treatment options. We report a case of stent-graft implantation into the left ventricular outflow tract for dynamic left ventricular outflow tract obstruction caused by systolic anterior motion of the anterior mitral valve leaflet (SAM). [ABSTRACT FROM AUTHOR]

Purpose: Cardiogenic shock still has a high mortality. In order to correctly manage these patients, it is useful to have available haemodynamic parameters, invasive and non‐invasive. The aim of this review is to show the current evidence on the use of echocardiographic aortic flow assessment by left ventricular outflow tract ‐ velocity time integral. Methods: Publications relevant to the discussion of echocardiographic aortic flow assessment by left ventricular outflow tract ‐ velocity time integral and cardiogenic shock, were retrieved from PubMed®. Results: Left ventricular outflow tract ‐ velocity time integral is an easily sampled and reproducible parameter that has already been shown to have prognostic value in various cardiovascular pathologies, including myocardial infarction and heart failure. Although there are still few data available in the literature, the LVOT‐VTI also seems to have an important role in CS from prognosis to guidance in the escalation/de‐escalation of vasoactive therapy and to support devices by allowing an estimate of patient's probability of response to fluid administration. Conclusion: Aortic flow assessment can become a very useful invasive parameter in the management of cardiogenic shock. [ABSTRACT FROM AUTHOR]

Background/Aim: Left ventricular outflow tract obstruction related to systolic anterior motion (SAM) of the mitral valve is a common complication of dobutamine stress echocardiography (DSE). However, the mechanisms underlying SAM have not been fully characterized. The objective of the present study was to use three‐dimensional echocardiography to identify anatomic features of the mitral valve that predispose to SAM during DSE. Methods: We retrospectively evaluated consecutive patients included prospectively in our database and who had undergone 3D echocardiography (including an assessment of the mitral valve) before DSE. Patients who had developed SAM during DSE (the SAM+ group) were matched 2:3 with patients who did not (the SAM− group). Results: One hundred patients were included (mean age: 67 ± 10). Compared with SAM− patients (n = 60), SAM+ patients (n = 40) had a lower mitral annular area, a smaller perimeter, and a smaller diameter (p <.01 for all, except the anteroposterior diameter). The SAM+ group had also a narrower mitral‐aortic angle (126 ± 12° vs. 139 ± 11° in the SAM− group; p <.01) and a higher posterior mitral leaflet length (1.4 ±.27 cm vs. 1.25 ±.29, respectively; p <.01). Furthermore, the mitral annulus was more spherical, more flexible, and more dynamic in SAM+ patients than in SAM− patients (p <.05 for all). In a multivariate analysis of anatomic variables, the mitral‐aortic angle, the mitral annular area, and posterior leaflet length were independent predictors of SAM (p ≤.01 for all). In a multivariate analysis of standard echo and hemodynamic variables, the presence of wall motion abnormalities at rest (p <.01) was an independent predictor of SAM. Conclusion: SAM during DSE is multifactorial. In addition to the pharmacologic effects of dobutamine on the myocardium, 3D echocardiographic features of the mitral valve (a smaller mitral annulus, a narrower mitral‐aortic angle, and a longer posterior leaflet) appear to predispose to SAM. [ABSTRACT FROM AUTHOR]

The use of bioprostheses is increasing in younger patients, but it is associated with the risk of later valve deterioration, especially in the mitral position. A new bioprosthesis for mitral valve replacement offers possible longer-term durability and improved hemodynamics. Objectives: Here, we report the implantation of the novel Edwards MITRIS RESILIA mitral valve (Edwards Lifesciences Inc., Irvine, CA, USA) through microinvasive fully endoscopic access as an innovative surgical approach based on a series of twelve patients. Methods: Contrast-based ECG gated CT was preoperatively performed in all patients to determine the intravascular calcifications and vascular parameters, as well as to assess noticeable problems during the operation. CT software for cardiac interventions (3Mensio Medical Imaging BV) was used to simulate surgical prostheses digitally inside the native annulus. With this, a digital LVOT and neo LVOT was created, and the difference between the valve prostheses was measured. Implantation of the MITRIS RESILIA valve was performed in 12 patients according to the instructions for use through microinvasive access in a fully endoscopic fashion using 3D visualization. Results: The mean patient age was 56.50 years, and 7/12 (58.33%) were redo procedures. All patients survived the first 30 days after the procedure, the mean aortic cross-clamp time was 40.17 ± 13.72 min. and mean postoperative transvalvular gradient was 4.45 ± 1.74 mmHg. The neo LVOT in the CT-based simulation was measured with an average area of 414.98 ± 88.69 mm2. The average difference between the LVOT and neo LVOT area was 65.35 ± 34.99 mm2. There was no case of paravalvular leakage or obstruction of the left ventricular outflow tract. Conclusions: The novel MITRIS RESILIA valve is a promising new bioprosthesis for mitral valve replacement that offers improved features as compared to other prostheses. The ease of implantation is increased by this prosthesis by the improved pliability of the sewing cuff and the inward folding of the struts, which was confirmed by short operative times in our series. [ABSTRACT FROM AUTHOR]

Introduction: We aimed to investigate the incidence, prenatal factors and outcomes of twin‐to‐twin transfusion (TTTS) with right ventricular outflow tract obstruction (RVOTO). Material and methods: A systematic search was conducted to identify relevant studies published until February 2023 in English using the databases PubMed, Scopus and Web of Science. Studies reporting on pregnancies with TTTS and RVOTO were included. The random‐effect model pooled the mean differences or odds ratios (OR) and the corresponding 95% confidence intervals. Heterogeneity was assessed using the I2 value. Results: A total of 17 studies encompassing 4332 TTTS pregnancies, of which 225 cases had RVOTO, were included. Incidence of RVOTO at time of TTTS diagnosis was 6%. In all, 134/197 (68%) had functional pulmonary stenosis and 62/197 (32%) had functional pulmonary atresia. Of these, 27% resolved following laser and 55% persisted after birth. Of those persisting, 27% required cardiac valve procedures. Prenatal associations were TTTS stage III (53% vs 39% in no‐RVOTO), stage IV TTTS (28% in RVOTO vs 12% in no‐RVOTO) and ductus venosus reversed a‐wave (60% in RVOTO vs 19% in no‐RVOTO). Gestational age at laser and gestational age at delivery were comparable between groups. Survival outcomes were also comparable between groups, including fetal demise of 26%, neonatal death of 12% and 6‐month survival of 82% in RVOTO group. Findings were similar when subgroup analysis was done for studies including head‐to‐head analysis. Conclusions: RVOT occurs in about 6% of the recipient twins with TTTS, especially in stages III and IV and those with reversed ductus venosus a‐wave. The findings from this systematic review support the need for a thorough cardiac assessment of pregnancies complicated by TTTS, both before and after laser, to maximize perinatal outcome, and the importance of early diagnosis of TTTS and timely management. [ABSTRACT FROM AUTHOR]

Background Although Takotsubo syndrome (TTS) is generally considered a benign disease, recent reports showed the incidence of cardiogenic shock due to left ventricular outflow tract obstruction (LVOTO), mitral regurgitation (MR), and primary pump failure was estimated to be 6–20%. Case summary A 78-year-old woman presented with chest pain and cold sweats 2 days after surgery for lung cancer. Acute coronary syndrome was suspected based on her symptoms, electrocardiography, transthoracic echocardiography (TTE), and laboratory data; thus, emergency catheterization was performed. Normal coronaries were observed, with hyperkinesis at the base of the left ventricle and akinesis at its apex, leading to the diagnosis of the apical ballooning type of TTS. Pressure differences between the apex of the left ventricle (168/8/28 mmHg) and aorta (94/50/64 mmHg) indicated the presence of LVOTO. Two days after TTS onset, she developed cardiogenic shock (blood pressure was 54/38 mmHg). Transthoracic echocardiography showed acute MR due to systolic anterior motion of the mitral valve caused by LVOTO, which was further exacerbated by paroxysmal atrial fibrillation. Fluid resuscitation, intravenous β-blockers, and amiodarone were administered for reduction of the pressure gradient in the left ventricular outflow, rate control, and sinus rhythm maintenance. Her condition improved along with the MR, thereby improving LVOTO and maintaining sinus rhythm. Discussion Takotsubo syndrome should be kept in mind as a potential cause of acute MR due to LVOTO. Catheterization and multiple follow-up TTE play a major role in early detection for this condition. [ABSTRACT FROM AUTHOR]

Background We report endocardial radiofrequency (RF) ablation as an alternative treatment approach for a symptomatic patient with obstructive hypertrophic cardiomyopathy (oHCM), who is not suitable for surgical septal myectomy or alcohol septal ablation. Endocardial RF ablation, with detailed 3D mapping of the intrinsic conducting system, offers the possibility of reducing the risk of complete heart block rates and of effectively relieving symptoms. Case summary We present a symptomatic 51-year-old female patient with oHCM and a maximum left ventricular outflow tract (LVOT) gradient of 148 mmHg. Because of alcohol septal ablation failure, endocardial RF ablation in combination with detailed mapping of the intrinsic conduction system and intraprocedural imaging was performed. The 6-month follow-up showed a significant improvement in exercise tolerance, no relevant dynamic LVOT obstruction with a gradient of 22 mmHg under Valsalva. Discussion In the current case report, endocardial RF ablation persistently reduced LVOT gradients in a patient with oHCM. Pre-interventional imaging, detailed 3D mapping of the conduction system, and correlation to intracardiac and transthoracic echocardiography were key for an effective and safe ablation of a small target zone. [ABSTRACT FROM AUTHOR]

Background. Takotsubo cardiomyopathy (TC) is a reversible left ventricular systolic dysfunction with apical ballooning. Left ventricular outflow tract (LVOT) obstruction may develop in these cases due to hyperdynamic state of the left ventricle. Limited data are available on the prevalence of LVOT gradient in TC and its association with patient outcomes and mortality. Methods. Data were collected retrospectively for patients diagnosed with TC in a single tertiary center, demographic information, blood analysis results, and imaging finding including ejection fraction, and LVOT gradient was obtained from medical records. Additionally, medical treatment and outcome during hospitalization were extracted. Follow‐up was conducted through cardiology clinic or phone contact. Result. A total of 59 patients diagnosed with TC were reviewed during hospitalization, and 40 patients were followed up after discharge by phone contact and cardiology clinic. Most patients were female (91.5%), and nonsignificant coronary artery disease was present in 16.9% of patients. Approximately two‐third of the patients had a reduced ejection fraction on admission, and only two patients (5.4%) continued to have reduced ejection fraction on echocardiography follow‐up within a period of 2–14 days. LVOT gradient was present in 17 patients (28.5%); patients with preserved ejection fraction had a higher probability of having an LVOT gradient. However, there was no association between LVOT gradient and shock or mortality. Four patients (6.7%) experienced 30‐day mortality, while all‐cause mortality was reported in eight patients (13.5%) over the follow‐up period (mean (±SD) 20.8 months ± 16.2). Conclusion. LVOT obstruction may occur in TC patients; it has no correlation with shock or mortality. However, determining whether there is a gradient is important for deciding on specific treatment approach. [ABSTRACT FROM AUTHOR]

Neonates with congenital heart disease (CHD) have smaller brain volume at birth. High rates of placental vascular malperfusion lesions may play a role in disrupted brain development. This is a single-center retrospective cohort study of infants born between 2010 and 2019 who were diagnosed with a major cardiac defect requiring surgery in the first year of life. Doppler ultrasound RI of the middle cerebral artery (MCA) and anterior cerebral artery were calculated within the first 72 hours of life. Placentas were evaluated using a standardized approach. Over the study period, there were 52 patients with hypoplastic left heart syndrome (HLHS), 22 with single-ventricle right ventricular outflow tract obstruction (SV-RVOTO), 75 with a two-ventricle cardiac defect (2V), and 25 with transposition of the great arteries (TGA). MCA Doppler RI were significantly higher for all subgroups of CHD compared with control subjects (0.68 ± 0.11 in control subjects compared with 0.78 ± 0.13 in HLHS, P = 0.03; 0.77 ± 0.10 in SV-RVOTO, P = 0.002; 0.78 ± 0.13 in 2V, P = 0.03; and 0.80 ± 0.14 in TGA; P = 0.001) with the highest average MCA RI in the TGA group. In subgroup analyses, placental fetal vascular malperfusion in the 2V group was associated with higher MCA RI, but this relationship was not present in other subgroups, nor in regards to maternal vascular malperfusion. Major forms of CHD are associated with significantly higher cerebral artery RI postnatally, but placental vascular malperfusion lesions may not contribute to this hemodynamic adaptation. [ABSTRACT FROM AUTHOR]

OBJECTIVES In patients with borderline left hearts or a severe left ventricular outflow tract obstruction, hybrid palliation can be used to stabilize the patient and postpone biventricular repair (BVR). In this study, we analysed growth of left-sided structures and outcomes of these patients. METHODS We conducted a retrospective cohort study including patients who received hybrid palliation between January 2010 and September 2023. Echo measurements were collected at hybrid palliation, BVR and last follow-up. Growth of left ventricular structures were analysed. RESULTS In 38 patients, hybrid palliation was used to promote growth of left ventricular structures. In total, 15 patients received a Ross–Konno/Yasui procedure, while 23 patients received conventional BVR. In patients with a conventional BVR, a significant increase was found in left ventricular volume indexed by body surface area, Z-score of aortic valve and left ventricular outflow tract between hybrid palliation and BVR. Mitral valve Z-score did not increase significantly. After BVR until follow-up, only increase of the aortic valve Z-scores and left ventricular volume indexed by body surface area was found significant. Of all included patients (n  = 38), additional surgical procedures were necessary in 8 patients during the interstage period and 15 patients after BVR. Additional catheter interventions were needed in 14 patients in the interstage period and 15 after BVR. Six patients died, with no mortality in the conventional BVR group. CONCLUSIONS Hybrid palliation as part of a staged BVR is a safe and effective initial step and promotes the growth of left ventricular structures in patients with small left-sided heart structures. Close follow-up is mandatory because extra catheter or surgical interventions are frequently needed. [ABSTRACT FROM AUTHOR]

Background: Friedreich ataxia is a rare genetic disorder associated with progressive mitochondrial dysfunction leading to widespread sequelae including ataxia, muscle weakness, hypertrophic cardiomyopathy, diabetes mellitus, and neuromuscular scoliosis. Children with Friedreich ataxia are at high risk for periprocedural complications during posterior spinal fusion due to their comorbidities. Aim: To describe our single‐center perioperative management of patients with Friedreich ataxia undergoing posterior spinal fusion. Methods: Adolescent patients with Friedreich ataxia presenting for spinal deformity surgery between 2007 and 2023 were included in this retrospective case series performed at the Children's Hospital of Philadelphia. Perioperative outcomes were reviewed along with preoperative characteristics, intraoperative anesthetic management, and postoperative medical management. Results: Seventeen patients were included in the final analysis. The mean age was 15 ± 2 years old and 47% were female. Preoperatively, 35% were wheelchair dependent, 100% had mild‐to‐moderate hypertrophic cardiomyopathy with preserved systolic function and no left ventricular outflow tract obstruction, 29% were on cardiac medications, and 29% were on pain medications. Intraoperatively, 53% had transesophageal echocardiography monitoring; 12% had changes in volume status on echo but no changes in function. Numerous combinations of total intravenous anesthetic agents were used, most commonly propofol, remifentanil, and ketamine. Baseline neuromonitoring signals were poor in four patients and one patient lost signals, resulting in 4 (24%) wake‐up tests. The majority (75%) were extubated in the operating room. Postoperative complications were high (88%) and ranged from minor complications like nausea/vomiting (18%) to major complications like hypotension/tachycardia (29%) and need for extracorporeal membrane oxygenation support in one patient (6%). Conclusions: Patients with Friedreich ataxia are at high risk for perioperative complications when undergoing posterior spinal fusion and coordinated multidisciplinary care is required at each stage. Future research should focus on the utility of intraoperative echocardiography, optimal anesthetic agent selection, and targeted fluid management to reduce postoperative cardiac complications. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Ruptured sinus of Valsalva (RSOV) upon physical exertion is rare but should be considered in differential in young adults. Symptoms of acute heart failure, hemodynamic instability, and continuous heart murmur raises suspicion for RSOV and requires emergent surgical repair of right ventricular outflow tract. [ABSTRACT FROM AUTHOR]

Transcatheter mitral valve replacement (TMVR) faces anatomical challenges, currently limiting widespread adoption. To describe the natural history and prognosis of patients ineligible for various TMVR devices. During a 4-year period (2019–2023) 3 TMVR devices (SAPIEN M3, Intrepid and Alta Valve) became available at a single institution (The Christ Hospital, Cincinnati, OH) in the setting of pivotal clinical trials or early feasibility study. Consenting patients who were deemed ineligible ≥1 of these trials were prospectively studied to capture anatomical reasons for ineligibility, cross-over to alternative mitral valve therapies (surgery or high-risk mitral transcatheter edge to edge repair [M-TEER]), and clinical events. A total of 61 patients (out of 71 consenting patients or 85.9 %) were deemed ineligible for TMVR during the study period. The mean age was 79.2 ± 8.8 years, 65.6 % were female, with elevated surgical risk (median STS 4.3, IQR: 2.7–7.3). The 2 most common anatomical reasons for ineligibility were increased risk of left ventricular outflow tract obstruction (LVOTO) (n = 24, 39.3 %) and annular size (n = 29, 47.5 %). During follow-up (median 277 [162–555] days) there were 7 deaths (11.5 %) and 12 (19.7 %) hospitalizations for heart failure. Management strategies included high-risk M-TEER in 11 patients (1 death [9.0 %], 0 HF hospitalizations [0 %]), surgery in 9 patients (0 deaths, 1 HF hospitalizations [11.1 %]), and medical management in 41 patients (6 deaths [14.6 %], 11 HF hospitalizations [26.8 %]) (p = 0.715 for mortality and p = 0.093 for HF hospitalizations). Residual MR ≥ moderate was 0 %, 50 %, and 100 % for surgery, M-TEER and medical treatment, respectively (p < 0.001). One third of patients deemed ineligible for TMVR are candidates for high-risk M-TEER or surgery with acceptable morbidity and mortality. Our results have practical implications for patient management. • Depiction of the natural history of patients with mitral regurgitation who screened failed for transeptal TMVR technologies • 1/3 of patients ineligible for TMVR are candidates for high risk M-TEER or surgery with acceptable morbidity and mortality. • This study highlights anatomical challenges of existing TMVR devices. [ABSTRACT FROM AUTHOR]

Obstructive hypertrophic cardiomyopathy (oHCM) is a subtype of HCM characterized by left ventricular outflow tract obstruction resulting from cardiac muscle hypertrophy and anatomic alterations in the mitral valve and apparatus. Mavacamten, a cardiac myosin inhibitor metabolized primarily by CYP2C19 in the liver, is the first and only targeted medication approved for the treatment of symptomatic New York Heart Association (NYHA) class II–III oHCM. Previous pharmacokinetic (PK) results of mavacamten in healthy Caucasian, Japanese, and Asian participants demonstrated that mavacamten exposure was affected by CYP2C19 metabolism status. This open‐label, parallel‐group, phase I trial aimed to determine the PK and safety of mavacamten in healthy Chinese participants with different CYP2C19 genotypes. The primary outcome was to define the PK of mavacamten in healthy Chinese participants; the secondary outcome was to examine safety and tolerability. After a single oral dose of 15 or 25 mg mavacamten in fasted healthy adult Chinese individuals, Cmax was reached within a median Tmax of 0.6–1.5 h, indicating rapid absorption. Inter‐individual variability was moderate, and individuals carrying non‐functional CYP2C19 alleles (*2/*2, *3/*3, or *2/*3) exhibited longer half‐life and increased total exposure. After stratification of CYP2C19 genotypes, total mavacamten exposures were similar among different ethnic groups when compared with prior PK studies. No significant adverse events were observed in this study. Single oral administration of mavacamten at 15 mg was well tolerated across all CYP2C19 genotypes, and 25 mg dose was well tolerated in healthy participants with CYP2C19 genotypes UM/RM/NM. The PK profile of mavacamten in the healthy Chinese population was consistent with that in other healthy populations. [ABSTRACT FROM AUTHOR]

Pediatric hypertrophic cardiomyopathy (HCM) has a wide range of clinical manifestations. Left ventricular outflow tract obstruction (LVOTO) at rest is present in up to one-third of children with HCM, with a further 50-60% of symptomatic children developing a gradient under exertion. Treatment options are limited, and there is a relative lack of data on the pediatric population. Disopyramide is a sodium channel blocker with negative inotropic properties. This therapy effectively reduces LVOTO in adults with HCM and delays surgical interventions, but it is not licensed for use in children. We aimed to review and analyze the influence of disopyramide over the pathophysiological, clinical, electrocardiographic, and echocardiographic characteristics of patients with HCM in infancy, childhood, adolescence, and adult age. While disopyramide remains a cornerstone in the management of pediatric HCM, the advent of mavacamten and aficamten heralds a new era of potential advancements. These emerging therapies could significantly improve the quality of life and prognosis for young patients with HCM. [ABSTRACT FROM AUTHOR]

Background Löffler's endocarditis (LE) is a frequently encountered condition in hypereosinophilic syndrome and is associated with a significant morbidity and mortality rate. Case Summary A 22-year-old man presented with acute dyspnoea, recurring wheezing, and cough, leading to his hospital admission. Multimodal diagnostic imaging revealed a manifestation of eosinophil-mediated cardiac injury in the thrombus formation stage. Moreover, a mural thrombus and thickened endocardium had caused severe obstruction of the right ventricular outflow tract (RVOT) and complete obliteration of the right ventricular apex, resulting in a significant reduction in right ventricular cardiac output. The patient received a diagnosis of LE and was treated with high-dose corticosteroids and anticoagulants. To alleviate the RVOT obstruction, an emergency surgical intervention was conducted through median sternotomy to the removal of the mural thrombus and resection of the thickened endocardium. Subsequently, eosinophil counts normalized within 1 month. Follow-up imaging examination demonstrated the existence of a residual section of thickened endocardium within the right ventricular free wall. Importantly, no mural thrombus was detected with complete relief of the RVOT obstruction. Notably, a transthoracic echocardiography examination at the 3-month postoperative unveiled a significant regression in right ventricular endomyocardial fibrosis. The patient's condition exhibited tangible improvement, with no adverse events observed. Discussion Multimodal imaging is essential for the early diagnosis and accurate staging of LE. Timely surgical intervention, combined with corticosteroid therapy, is an effective therapeutic approach in selected patients with LE. This approach is crucial to achieve remission of acute phase symptoms and improve prognosis. [ABSTRACT FROM AUTHOR]

Congenital heart disease (CHD) is a structural abnormality of the heart and/or great vessels and patients with CHD are at an increased risks of various morbidities throughout their lives and reduced long‐term survival. Eventually, CHD may result in various complications including heart failure, arrhythmias, stroke, pneumonia, and sudden death. Unfortunately, the exact etiology and pathophysiology of some CHD remain unclear. Although the quality of life and prognosis of patients with CHD have significantly improved following technological advancement, the influence of CHD is lifelong, especially in patients with complicated CHD. Thus, the management of CHD remains a challenge due to its high prevalence. Finally, there are some disagreements on CHD among international guidelines. In this review, we provide an update of the pathophysiology, diagnosis, and treatment in most common type of CHD, including patent foramen ovale, atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, coarctation of the aorta, transposition of the great arteries, congenitally corrected transposition of the great arteries, coronary anomalies, left and right ventricular outflow tract obstruction, tetralogy of Fallot and Ebstein anomaly. In particular, we focus on what is known and what is unknown in these areas, aiming to improve the current understanding of various types of CHD. [ABSTRACT FROM AUTHOR]

Hypertrophic cardiomyopathy is a prevalent cause of sudden cardiac death among young people. This distinctive genetic condition can manifest at any age from infancy to adulthood. The primary features include unexplained left ventricular hypertrophy coupled with dynamic left ventricular outflow tract (LVOT) obstruction and a variable degree of mitral valve regurgitation. In certain cases, patients may also experience biventricular outflow tract obstruction. Surgical septal myectomy is the gold standard treatment for symptomatic children experiencing severe LVOT obstruction. This review focuses on surgical techniques for septal myectomy in pediatric patients and other adjunct procedures and summarizes the current outcomes. Keywords:Hypertrophic cardiomyopathy, sudden cardiac death, left ventricular outflow tract obstruction, asymmetrical septal hypertrophy, left ventricular myectomy: [ABSTRACT FROM AUTHOR]