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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.

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]

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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]

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]

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]

Aims Methods and results Conclusion 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).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.The H2FPEF score is independently associated with HF outcome in patients with HCM and may be considered for risk stratification. [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]

This research letter discusses the different phenotypes of ABL1 deficiency syndromes caused by mutations in different isoforms of the ABL1 gene. Somatic mutations in ABL1 are associated with cancer, while germline gain-of-function variants are linked to Congenital Heart Defects and Skeletal Malformations Syndrome (CHDSKM). Biallelic loss-of-function variants in ABL1 are associated with Human ABL1 Deficiency Syndrome (HADS). The letter presents a case study of a Lebanese family with a history of cardiac abnormalities, including a child with a stenotic subaortic membrane. The study highlights the various types of heart defects and genetic mutations in the ABL1 gene, suggesting that different mutations can lead to distinct phenotypes. Further research is needed to fully understand ABL1-associated disorders. The study was funded by the Lebanese American University and the authors have no conflicts of interest. [Extracted from the article]

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]

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.

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. 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]

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]

Transcatheter mitral valve-in-valve is an alternative to high-risk reoperation on a failing bioprosthesis. It entails specific challenges such as left ventricular outflow tract obstruction. We propose a patient-specific augmented imaging based on preoperative planning to assist the procedure. Valve-in-valve simulation was performed to represent the optimal level of implantation and the neo-left ventricular outflow tract. These data were combined with intraoperative images through a real-time 3D/2D registration tool. All data were collected retrospectively on one case (pre and per-procedure imaging). We present for the first time an intraoperative guidance tool in transcatheter mitral valve-in-valve procedure. [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]

Background: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) worldwide. It accounts for 7% of CHD cases in Uganda and leads to fatal outcomes in the long term without surgery. Surgery is often delayed in developing countries like Uganda due to limited resources. Objective: This study aimed to determine the early surgical outcomes of patients with TOF who underwent primary intracardiac repair at the Uganda Heart Institute (UHI) and to identify factors associated. Methodology: This retrospective chart review evaluated outcomes of primary TOF repair patients at UHI from February 2012 to October 2022. Patient outcomes were assessed from surgery until 30 days post-operation. Results: Out of the 104 patients who underwent primary TOF repair at UHI, records of 88 patients (84.6%) were available for review. Males accounted for 48.9% (n = 43). The median age at the time of operation was 4 years (with an interquartile range of 2.5-8.0 years), ranging from 9 months to 16 years. Genetic syndromes were present in 5/88 (5.7%). These included 2 patients with trisomy 21, 2 with Noonan's, and 1 with 22q11.2 deletion syndrome. Early postoperative outcomes for patients included: residual ventricular septal defects in 35/88 (39.8%), right ventricular dysfunction in 33/88 (37.5%), residual pulmonary regurgitation in 27/88 (30.7%), residual right ventricular outflow tract obstruction in 27/88 (30.0%), pleural effusion in 24/88 (27.3%), arrhythmias in 24/88(27.3%), post-operative infections in 23/88(26.1%) and left ventricular systolic dysfunction in 9/88 (10.2%). Out of the children who underwent surgery after one year of age, 8% (7 children) died within the first 30 days. There was a correlation between mortality and post-operative ventilation time, cardiopulmonary bypass (CPB) time, aortic cross-clamp time, preoperative oxygen saturations, RV and LV dysfunction and the operating team. Conclusion: The most frequent outcomes after surgery were residual ventricular septal defects and right ventricular failure. In our study, the 30-day mortality rate following TOF repair was 8%. Deceased patients had lower pre-operative oxygen levels, longer CPB and cross-clamp times, longer post-operative ventilation, RV/LV dysfunction, and were more likely operated by the local team. [ABSTRACT FROM AUTHOR]

The "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy. A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate. Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy" have been updated with new evidence to guide clinicians. [ABSTRACT FROM AUTHOR]

AIM: The "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy. METHODS: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate. STRUCTURE: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy" have been updated with new evidence to guide clinicians. [ABSTRACT FROM AUTHOR]

Purpose: Assessment of aortic stenosis (AS) is based on aortic valve (AV) gradients and calculation of aortic valve area (AVA). These parameters are influenced by flow and dependent on geometric assumptions. The dimensionless index (DI), the ratio of the LVOT time‐velocity integral to that of the AV jet, is simple to perform, and is less susceptible to error but has only been examined in small selected groups of AS patients. The objective of this study was to assess the DI and prognosis in a large cohort. Methods: All subjects who underwent echocardiography with an assessment of the AV that included DI were included. Association between AV parameters including mean gradient, AVA, DI and AV resistance and mortality and cardiovascular hospitalizations was examined. Results: A total of 9393 patients (mean age 71 ± 16 years; 53% male) were included. 731 (7.7%) patients had DI less than.25. Increasing age and a diagnosis of heart failure were significantly associated with lower DI. Subjects with low DI had significantly lower ventricular function, a higher incidence of mitral and tricuspid regurgitation, worse diastolic function and more elevated pulmonary pressures. Decreasing DI was associated with significantly decreased survival and event‐free survival which remained highly significant on multivariate analysis. Conclusions: In a large population of patients with AV disease, decreased DI, was associated with increased mortality and decreased event‐free survival. The easily obtained DI identifies a broad range of AS subjects with worse prognosis and should be integrated into the assessment of these complex patients. [ABSTRACT FROM AUTHOR]

OBJECTIVES Left ventricular outflow tract obstruction (LVOTO) is a major cause of morbidity and mortality in infants with interrupted aortic arch (IAA). Left Ventricular Outflow Tract (LVOT) development may be flow-mediated, thus IAA morphology may influence LVOT diameter and subsequent reintervention. We investigated the association of IAA morphology [type and presence of aortic arch aberrancy (AAb)] with LVOT diameter and reintervention. METHODS All surgical patients with IAA (2001–2022) were reviewed at a single institution. We compared IAA-A versus IAA-B; IAA with aortic AAb versus none; IAA-B with aberrant subclavian (AAbS) artery versus others. Primary outcomes included LVOT diameter (mm), LVOTO at discharge (≥50 mmHg), and LVOT reintervention. RESULTS Seventy-seven infants (mean age 10 ± 19 days) were followed for 7.6 (5.5–9.7) years. Perioperative mortality was 3.9% (3/77) and long-term mortality was 5.2% (4/77). Out of 51 IAA-B (66%) and 22 IAA-A (31%) patients, 30% (n  = 22) had AAb. Smaller LVOT diameter was associated with IAA-B [IAA-A: 5.40 (4.68–5.80), IAA-B: 4.60 (3.92–5.50), P  = 0.007], AAb [AAb: 4.00 (3.70–5.04) versus none: 5.15 (4.30–5.68), P  = 0.006], and combined IAA-B + AAbS [IAA-B + AAbS: 4.00 (3.70–5.02) versus other: 5.00 (4.30–5.68), P  = 0.002]. The likelihood of LVOTO was higher among AAb [ N  = 6 (25%) vs N  = 1 (2%), P  = 0.004] and IAA-B + AAbS [ N  = 1 (2%) vs N  = 6 (30%), P  = 0.002]. Time-to-event analysis showed a signal towards increased LVOT reintervention in IAA-B + AAbS (P  = 0.11). CONCLUSIONS IAA-B and AAb are associated with small LVOT diameter and early LVOTO, especially in combination. This may reflect lower flow in the proximal arch during development. Most reinterventions occur in IAA-B + AAbS, hence these patients should be carefully considered for LVOT intervention at the time of initial repair. [ABSTRACT FROM AUTHOR]

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006–2021 were retrospectively reviewed, excluding patients with associated cardiac lesions. Two-dimensional measurements, LVOT gradients, and 4-chamber (4C) and short-axis (SAXM) strain were obtained from preoperative and predischarge echocardiograms. Univariate risk analysis for LVOTO reoperation was performed using unpaired t-test. Thirty patients were included with 21 (70%) IAA subtype B and mean weight at surgery 3.0 kg. Repair included aortic arch patch augmentation in 20 patients and subaortic obstruction intervention in three patients. Seven (23%) required reoperations for LVOTO. Patient characteristics were similar between patients who required LVOT reoperation and those who did not. Patch augmentation was not associated with LVOTO reintervention. Patients requiring reintervention had significantly smaller LVOT AP diameter preoperatively and at discharge, and higher LVOT velocity, smaller AV annular diameter, and ascending aortic diameter at discharge. There was an association between LVOT-indexed cross-sectional area (CSAcm2/BSAm2) ≤ 0.7 and reintervention. There was no significant difference in 4C or SAXM strain in patients requiring reintervention. LVOTO reoperation was not associated with preoperative clinical or surgical variables but was associated with smaller LVOT on preoperative echo and smaller LVOT, smaller AV annular diameter, and increased LVOT velocity at discharge. [ABSTRACT FROM AUTHOR]

A 13-year-old, Maltese dog presented with syncope and lethargy. Abdominal ultrasonography demonstrated anechoic peritoneal effusion and hepatic congestion. A focal echogenic round mass compressing the right ventricle and atrium was observed on echocardiography. Cardiac tamponade and right ventricular outflow tract obstruction occurred. On computed tomography, a homogeneous soft-tissue structure compressing the right chamber without contrast enhancement, suspected to be loculated pericardial effusion. During pericardiocentesis, cardiac tamponade was resolved, and irregular pericardial thickening was noted. Pericardial effusion was exudate and gram-positive bacterial colonies were observed on cytology. A diagnosis of fibrinous pericarditis secondary to bacterial infection was established. [ABSTRACT FROM AUTHOR]

Objective: To identify anatomical features, association and surgical results of patient with double chambered right ventricle. Study Design: Prospective longitudinal study. Place and Duration of Study: The Children Hospital and Institute of Child Health, Lahore Pakistan, from Jan 2013 to Dec 2018. Methodology: Al patients presenting with mid cavity right ventricular outflow tract obstruction were included. Al patients' demographic data, clinical profile, diagnostic reports, associated anomalies, and surgical data were recorded. Results: Fifty-two patients with mid-cavity right ventricular outflow tract obstruction of various ages (from 6 months to 31 years) were included. Ten patients (19%) presented in infancy. Forty-six patients (89%) had an associated ventricular septal defect, ten (15%) had aortic valve right coronary cusp prolapse with varying degrees of aortic regurgitation. Al patients had a right ventricular mid-cavity and infundibular muscle resection via the tricuspid valve. There was one hospital death due to an intraoperative global neurologic catastrophe. The median folow-up after surgery was 37.5 months. There was no late death. Conclusion: Doubly commited Ventricular septal defect with aortic valve right coronary cusp prolapse with varying degree of aortic regurgitation and absence of subaortic stenosis is a new finding in our study. Early and Medium-term surgical results of repair are excelent. [ABSTRACT FROM AUTHOR]

This article discusses a case study involving the prenatal detection and neonatal surgical resection of a giant aneurysm in the left atrial appendage of a fetus. The study highlights the feasibility of prenatal diagnosis and emphasizes the importance of timely assessment and treatment in the early postpartum period. The use of multimodal imaging is also emphasized for accurate characterization of the appendage and optimal surgical planning. The article concludes by noting that giant aneurysms in the left atrial appendage are rare and potentially associated with thromboembolism. [Extracted from the article]

The article focuses on introducing a new transcatheter myotomy technique, SESAME, aimed at addressing left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy (HCM) and facilitating transcatheter valve replacement (TVR). It is reported that this innovative approach utilizes intramyocardial guidewires and electrosurgery to widen the LVOT (Left Ventricular Outflow Tract), improving symptoms in HCM and TVR patients.

Background: Variants in the MYBPC3 gene are a frequent cause of hypertrophic cardiomyopathy (HCM) but display a large phenotypic heterogeneity. Founder mutations are often believed to be more benign as they prevailed despite potential negative selection pressure. We detected a pathogenic variant in MYBPC3 (del exon 23-26) in several probands. We aimed to assess the presence of a common haplotype and to describe the cardiac characteristics, disease severity and long-term outcome of mutation carriers. Methods: Probands with HCM caused by a pathogenic deletion of exon 23-26 of MYBPC3 were identified through genetic screening using a gene panel encompassing 59 genes associated with cardiomyopathies in a single genetic center in Belgium. Cascade screening of first-degree relatives was performed, and genotype positive relatives were further phenotyped. Clinical characteristics were collected from probands and relatives. Cardiac outcomes included death, heart transplantation, life-threatening arrhythmia, heart failure hospitalization or septal reduction therapy. Haplotype analysis, using microsatellite markers surrounding MYBPC3, was performed in all index patients to identify a common haplotype. The age of the founder variant was estimated based on the size of the shared haplotype using a linkage-disequilibrium based approach. Results: We identified 24 probands with HCM harbouring the MYBPC3 exon 23- 26 deletion. Probands were on average 51 ± 16 years old at time of clinical HCM diagnosis and 62 ± 10 years old at time of genetic diagnosis. A common haplotype of 1.19 Mb was identified in all 24 probands, with 19 of the probands sharing a 13.8 Mb haplotype. The founder event was estimated to have happened five generations, or 175–200 years ago, around the year 1830 in central Flanders. Through cascade screening, 59 first-degree relatives were genetically tested, of whom 37 (62.7%) were genotype positive (G+) and 22 (37.3%) genotype negative (G-). They were on average 38 ± 19 years old at time of genetic testing. Subsequent clinical assessment revealed a HCM phenotype in 19 (51.4%) G+ relatives. Probands were older (63 ± 10 vs. 42 ± 21 years; p < 0.001) and had more severe phenotypes than G+ family members, presenting with more symptoms (50% vs. 13.5%; p = 0.002), arrhythmia (41.7% vs. 12.9%, p = 0.014), more overt hypertrophy and left ventricular outflow tract obstruction (43.5% vs. 3.0%; p < 0.001). Male G+ relatives more often had a HCM phenotype (78.6% vs. 34.8%; p = 0.010) and were more severely affected than females. At the age of 50, a penetrance of 78.6% was observed, defined as the presence of HCM in 11 of 14 G+ relatives with age ≥50 years. Overall, 20.3% of all variant carriers developed one of the predefined cardiac outcomes after a median follow-up of 5.5 years with an average age of 50 (±21) years. Conclusion: A Belgian founder variant, an exon 23-26 deletion in MYBPC3, was identified in 24 probands and 37 family members. The variant is characterized by a high penetrance of 78.6% at the age of 50 years but has variable phenotypic expression. Adverse outcomes were observed in 20.3% of patients during follow-up. [ABSTRACT FROM AUTHOR]

Hypertrophic cardiomyopathy(HCM) is a genetic cardiomyopathy with a prevalence of 1/500 and causes adverse outcomes, usually due to left ventricular outflow tract obstruction. In this study, we aimed to determine the possible differences and predictors of obstructive hypertrophic cardiomyopathy HCM (Obs-HCM) and nonobstructive HCM (Nonobs-HCM) by electrocardiographic (ECG) and echocardiographic (ECHO) evaluations with clinical, demographic, and biochemical characteristics. This study is a subgroup analysis of a multicentre, national, and observational 'LVH-TR study' that included 886 left ventricular hypertrophy(LVH) patients in 22 centres between February 2020 and August 2021. After excluding six patients with atrial fibrillation, pace rhythm, bundle branch blocks, and second, and third-degree atrioventricular(AV) block, 60 HCM patients were included, 23 of whom were obstructive, and 37 were nonobstructive. Body surface area(BSA) (2.01 ± 0.17, 1.89 ± 0.19; p =.01), ST-segment depression (%82.6, %54.1; p =.02), QT and QTc durations (436.3 ± 58.3, 398.0 ± 65.5; p =.02/470.6 ± 58.7, 432.8 ± 74.7; p =.04), left ventricular mass index(LVMI) (176.4 ± 47.0, 152.7 ± 10.2; p =.004), and systolic anterior motion(SAM) rates (%82.6, %18.9; p < 0.001) were significantly higher in the obstructive HCM compared to nonobstructive HCM. Furthermore, the significance of ST-segment depression, QT duration, LVMI, and SAM continued in the univariate analyses to assess obstruction prediction (all p values <.05). In multivariate and correlation analyses, ST segment depression (rho = 0.29), QT prolongation (rho = 0.34), and SAM (rho = 0.62) are found as predictors for obstruction (all p values <.05). Our study will guide future studies since it has detailed ECG and ECHO comparisons of Obs-HCM and Nonobs-HCM patients over 18 are made. [ABSTRACT FROM AUTHOR]