Ventricular Outflow Tract Obstruction Research Articles

Case Series of First Microinvasive Fully Endoscopic Use of a New Mitral Prosthesis.

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.

One-Year Outcomes of Transseptal Mitral Valve-in-Valve in Intermediate Surgical Risk Patients.

Transcatheter mitral valve-in-valve replacement offers a less-invasive alternative for high-risk patients with bioprosthetic valve failure. Limited experience exists in intermediate-risk patients. We aim to evaluate 1-year outcomes of the PARTNER 3 mitral valve-in-valve study. This prospective, single-arm, multicenter study enrolled symptomatic patients with a failing mitral bioprosthesis demonstrating greater than or equal to moderate stenosis and regurgitation and Society of Thoracic Surgeons score ≥3% and <8%. A balloon-expandable transcatheter heart valve (SAPIEN 3, Edwards Lifesciences) was used via a transeptal approach. The primary end point was the composite of all-cause mortality and stroke at 1 year. A total of 50 patients from 12 sites underwent mitral valve-in-valve from 2018 to 2021. The mean age was 70.1±9.7 years, mean Society of Thoracic Surgeons score was 4.1%±1.6%, and 54% were female. There were no primary end point events (mortality or stroke) through 1 year, and no left-ventricular outflow tract obstruction, endocarditis, or mitral valve reintervention was reported. Six patients (12%) required rehospitalization, including heart failure (n=2), minor procedural side effects (n=2), and valve thrombosis (n=2; both resolved with anticoagulation). An additional valve thrombosis was associated with no significant clinical sequelae. From baseline to 1 year, all subjects with available data had none/trace or mild (grade 1+) mitral regurgitation and the New York Heart Association class improved in 87.2% (41/47) of patients. Mitral valve-in-valve with a balloon-expandable valve via transseptal approach in intermediate-risk patients was associated with improved symptoms and quality of life, adequate transcatheter valve performance, and no mortality or stroke at 1-year follow-up. URL: https://www.clinicaltrials.gov; Unique identifier: NCT03193801.

The challenges in diagnosing hypertrophic cardiomyopathy in the presence of arterial hypertension: a clinical case

Aim. To describe a clinical case and analyze the challenges in diagnosing hypertrophic cardiomyopathy (HCM) in a patient with concomitant arterial hypertension (AH). Materials and methods. The article presents the clinical case of HCM in the patient with concomitant AH that was observed in an ambulatory setting in the Municipal Enterprise “Dnipropetrovsk Regional Clinical Center for Diagnostics and Treatment” of Dnipropetrovsk Regional Council. Results. A 66-year-old woman N., diagnosed by her family physician with coronary artery disease (CAD): stable angina FC II, grade 2 AH, chronic heart failure FC II NYHA, was referred to a cardiologist because of experiencing exertional dyspnea, chest pain, and uncontrolled blood pressure despite treatment compliance. Echocardiography identified concentric left ventricular hypertrophy with the left ventricular outflow tract (LVOT) obstruction (a mean gradient of 35 mmHg as per catheterization). Cardiac MRI confirmed the diagnosis and coronary angiography ruled out CAD. Adjustments to the treatment regimen, taking into account HCM with LVOT obstruction, effectively alleviated the patient’s symptoms and stabilized her blood pressure. Conclusions. It is especially important to follow the guidelines of AH management and perform echocardiography in all patients with high blood pressure, so as not to miss the signs of concomitant HCM, particularly with LVOT obstruction. In addition, in the case of HCM, it is necessary to timely detect, provide prevention and manage patients at risk for sudden cardiac death. Since HCM encompasses various diagnoses with different pathogenesis and distinct management, cardiac MRI, enzymatic or genetic testing may be needed according to guidelines. As HCM is a relatively common inherited cardiac disease, general practitioners could often encounter such patients in everyday clinical practice. Hence, they should have a certain suspicion of this diagnosis in persons with AH whose left ventricular mass meets the criteria for HCM.

Discontinuation of afterload-reducing drugs decreases left ventricular outflow tract obstruction in hypertrophic obstructive cardiomyopathy.

Hypertrophic cardiomyopathy (HCM), the most common genetic heart disease, is classified into hypertrophic non-obstructive and hypertrophic obstructive cardiomyopathy (HOCM). Patients with HOCM and coexisting heart failure or arterial hypertension are often prescribed afterload-reducing drugs. Although recommended in current guidelines, data on the direct effect of discontinuing afterload-reducing medication are scarce. This study aims to demonstrate the benefit of discontinuing afterload-reducing medication in HOCM patients. This monocentric retrospective analysis included 24 patients with HOCM with afterload-reducing medication, including angiotensin-converting enzyme inhibitors, angiotensin-1 receptor blocker and dihydropyridine-calcium channel blocker, at their first outpatient visit. Effects of discontinuing this medication on LVOTO were examined compared to patients with persistent use despite medical advice. 16 patients discontinued their afterload-reducing drugs, resulting in a significant decrease in median LVOT gradient from 86.5 [60.5-109.3] mmHg to 61.5 [28.3-97.50] mmHg (p = 0.0004). In 6 patients, beta-blocker therapy was initiated simultaneously, or the dose was increased. Regardless, LVOT gradient reduction was also significant in the remaining 10 patients (p = 0.001). The gradient was not changed significantly in the 8 patients continuing their afterload-reducing medication. Discontinuation of afterload-reducing drugs significantly decreases LVOTO. Our study underscores the significance of abstaining from afterload-reducing drugs in HOCM patients, particularly in patients with concomitant hypertension or heart failure. According to recently published European guidelines, HOCM patients should preferably be treated with beta-blockers or non-dihydropyridine-calcium channel blockers.

Value of Echocardiographic Evaluation of Myocardial Performance Index in Predicting Major Adverse Cardiovascular Events Within 1 Year after Percutaneous Coronary Intervention in Patients with Coronary Heart Disease

Objective: This study aimed to evaluate the predictive efficacy of the echocardiography-derived Tei index for the occurrence of major adverse cardiovascular events (MACE) within 1 year post-percutaneous coronary intervention (PCI) in patients with coronary heart disease (CHD). Methods: A total of 98 patients diagnosed with CHD and admitted to our hospital between January 2021 and May 2023 were retrospectively selected for this study, and the two groups were divided into good prognosis group (n = 67) and poor prognosis group (n = 31) according to whether cardiovascular adverse events occurred within 1 year after PCI. Univariate and multivariate logistic regression analyses were conducted to identify the influencing factors of adverse cardiovascular events in patients with CHD following PCI, and receiver operating characteristic (ROC) analysis was employed to evaluate the efficacy of myocardial performance index measured by echocardiography in predicting adverse cardiovascular events within 1 year post-PCI in patients with CHD. The inflammatory factors of patients with different Tei indices were compared before and 24 h after PCI. Results: The differences in general data, including preoperative Tei index, left anterior descending (LAD) level, left ventricular diameter in diastole (LVDd) level, and the number of cases with left ventricular outflow tract obstruction, were not statistically significant between two groups (p &gt; 0.05). Left ventricular ejection fraction (LVEF), maximum left ventricular thickness, postoperative Tei index, plaque score, and carotid intima–media thickness (IMT) showed statistically significant differences (p &lt; 0.05). The findings from logistic regression analysis, considering multiple factors, indicated that Tei index, plaque score, and carotid IMT were independent predictors for adverse cardiovascular events following PCI in patients with CHD (p &lt; 0.05). ROC analysis demonstrated an impressive area under the curve of 0.967 for echocardiographic assessment of myocardial performance index as a predictor for adverse cardiovascular events within 1 year after PCI in patients with CHD. The standard error was 0.017, 95% confidence interval was 0.935–0.999, optimal cut-off value was 0.88, sensitivity was 95.0%, and specificity was 93.3%. The comparison of inflammatory factors among patients with different Tei index values before PCI did not yield any statistically significant differences (p &gt; 0.05), and the comparison of inflammatory factors in patients with different Tei index 24 h after PCI. The levels of inflammatory cytokines in patients with Tei ≤0.5 were lower than those in patients with Tei &gt;0.5 (p &lt; 0.05). Conclusion: Evaluating the myocardial performance index through echocardiography holds considerable value in predicting MACE within one year following PCI in patients diagnosed with CHD.

217 Outcomes of Reoperation for Residual or Recurrent Obstruction After Previous Myectomy in Patients with Hypertrophic Cardiomyopathy

Abstract Aim Describe the mechanism(s) of residual or recurrent obstruction in patients who have previously undergone septal myectomy for hypertrophic cardiomyopathy and assess early outcomes of reoperation. Method From January 1989 to March 2022, we performed 73 repeat myectomies at our institution. Patients’ demographics, operative details, echocardiographic parameters, and postoperative complications were retrieved from a prospectively maintained surgical database and electronic healthrecords. Results Median age at reoperation was 48.3 years (IQR 38.6-58.6); 51 patients were women (69.9%). 90.4% presented with NYHA class III/IV symptoms. Median interval between previous myectomy and reoperation was 2.7 years (IQR 1.2-4.5). Mechanism of obstruction was an inadequate length of previous septectomy in 41 patients (56.2%), a combination of inadequate length and midventricular obstruction in 30 (41.1%), and isolated midventricular obstruction in 2 patients (2.7%). There were no instances of recurrent LVOT obstruction due to muscle regrowth at the site of previous resection. All patients had an extended transaortic septal myectomy; 14 patients (19.2%) had concomitant transapical myectomy. Preoperatively, the median LVOT gradient was 83 mmHg (IQR 60-122), which reduced to 2 mmHg (IQR 0-7) following repeat myectomy. There were no perioperative deaths. Postoperatively, 4 patients (5.5%) developed a new CHB, necessitating PPM implantation. The 5-,10-, and 20-year survival after reoperation were 100%, 91%, and 76%, respectively. Conclusions Inadequate resection at the time of primary septal myectomy is the commonest cause of residual or recurrent obstruction in oHCM patients. However, repeat myectomy, including a combined transaortic and transapical approach has minimal risk with excellent hemodynamic and long-term survival outcomes.

Pharmacokinetics and safety of mavacamten in healthy Chinese participants with different CYP2C19 phenotypes.

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.

Congenital heart disease: types, pathophysiology, diagnosis, and treatment options.

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.

Hybrid palliation to promote growth of left ventricle and left ventricular outflow tract.

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. 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. 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. 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.

Clinical outcomes of the neonates with critical pulmonary stenosis: intrauterine versus postnatal transport.

Pulmonary stenosis (PS) is one rare congenital heart disease (CHD) featuring obstruction of right ventricular outflow tract. Critical pulmonary stenosis (CPS) is neonatal PS having cyanosis and evidence of patent ductus arteriosus (PDA) dependency. There is limited data on the clinical outcomes of CPS with different modes of transportation. This study aimed to investigate clinical features and outcomes of CPS through the intrauterine transport (IT) and postnatal transport (PT). Single-center retrospective research was performed. Neonates with CPS were grouped into the IT group and PT group. Clinical characteristics and outcomes of the neonates were compared between the two groups. Totally 110 neonates with PS were included in this study, 77 with CPS and 33 with non-CPS. In the infants with CPS, there were 53 and 24 in the IT and PT group respectively. Echocardiography showed that transvalvular pulmonary gradient (TVG) stayed lower in the IT group than that in the PT group {77.0 [interquartile range (IQR), 60.5-91.5] vs. 92.0 (IQR, 73.3-125.0) mmHg, P=0.006}. Levels of serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and troponin I also remained lower in the IT group than those in the PT group [2,256 (IQR, 1,054-4,527) vs. 3,708 (IQR, 2,138-6,789) pg/mL, P=0.02; 0.020 (IQR, 0.011-0.034) vs. 0.042 (IQR, 0.027-0.072) ng/mL, P<0.001, respectively]. All infants with CPS received percutaneous balloon pulmonary valvuloplasty (PBPV) therapy in neonatal period and were discharged from the hospital. Length of hospital stay remained shorter in the IT group than that in the PT group [13.0 (IQR, 11.0-15.0) vs. 15.5 (IQR, 10.8-22.8) days, P=0.03]. IT and early management after birth could effectively reduce the severity of CPS before PBPV treatment and shorten the length of hospital stay among neonates suffering from CPS.

Early Feasibility Study with the SATURN Transapical Mitral Valve Replacement Device

PurposeTo report the outcomes of the early feasibility study of transapical transcatheter mitral valve replacement (TMVR) with the SATURN System (InnovHeart, Milano, Italy) to treat patients with severe functional mitral regurgitation. DescriptionFive high surgical risk patients underwent transapical transcatheter mitral valve replacement with the SATURN System at a single center. One-year follow-up is complete for all patients. EvaluationThe valve was implanted successfully in all patients without any major adverse events. All patients were alive at the last follow-up. Kansas City Cardiomyopathy Questionnaire improved from a median of 63.5 (interquartile range, 19.6) at baseline to 99.0 (interquartile range, 21.6) at 1 year. Echocardiographic follow-up demonstrates stable valve function, no transvalvular or paravalvular mitral regurgitation, and absence of left ventricular outflow tract obstruction. ConclusionsAt 1 year after transapical SATURN transcatheter mitral valve replacement, all patients are alive with quality of life improvement and favorable device hemodynamics. These initial results are promising and larger scale studies with continued follow-up are required to further elucidate the efficacy and safety of this novel technology.

Primary surgical repair of tetralogy of fallot at the Uganda Heart Institute: a ten-year review of 30day mortality and morbidity

BackgroundTetralogy 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.ObjectiveThis 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.MethodologyThis 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.ResultsOut 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.ConclusionThe 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.

Mavacamten: a novel avenue towards hypertrophic obstructive cardiomyopathy

Hypertrophic obstructive cardiomyopathy (HCM) is the most common heterogeneous genetic cardiovascular disorder. Its pathophysiology involves left ventricular hypertrophy, increased fibrosis, hypercontractility, and reduced compliance. The symptomatic obstructive HCM presents as dyspnoea, syncope, chest pain, palpitations, arrhythmias, or sudden death, usually after provocative manoeuvres like exercise. Until April 2022, treatment options were disease non-specific like Beta blockers, Cardio-selective Calcium Channel Blockers, Dipyridamole, and Ranolazine. Mavacamten is a first-in-class, FDA-approved drug molecule for HCM. It works by selective and reversible inhibition of the cardiac myosin ATPase thereby decreasing the formation of actin- myosin cross- bridges in systole and diastole. The excessive actin-myosin cross-bridging is the hallmark of disease and is responsible for the compromised functioning of the heart in both the systole and diastole phase due to left ventricular outflow tract (LVOT) obstruction and increased ventricular filling pressure respectively. Mavacamten acts by producing super-relaxed state of heart which is then translated into decreased LVOT obstruction and improved cardiac filling pressures thereby improving the functional capacity and symptoms in patients with New York Heart Association (NYHA) stage II, and III symptomatic or obstructive heart failure. Mavacamten is administered orally 2.5-15 mg per day with titration guided by lab investigations and clinical symptoms. Its bioavailability is 85%, undergoes metabolism by CYP2C19 and CYP3A4 and is excreted mainly in urine. It is also an enzyme inducer and shows considerable drug interactions. Common adverse effects are dizziness and syncope. Sometimes the drug may worsen heart failure or completely block ventricular function. Mavacamten has raised hopes for the possibility of managing this potentially lethal intrigue disorder with medicines alone.

Left ventricular posterior wall hypertrophy leads to poor prognosis of hypertrophic obstructive cardiomyopathy in children - a cohort study.

The modified Morrow operation for hypertrophic obstructive cardiomyopathy (HOCM) in children has a favorable outcome, but some children still have a poor prognosis after the procedure. In this study, we aimed to investigate the application of cardiac computed tomography (CCT) to construct a three-dimensional(3D) model of the left ventricle (LV) and analyze the association between hypertrophy in different parts of the LV and poor prognosis. We retrospectively analyzed 57 children with HOCM from April 2015 to October 2022, among whom 16 underwent preoperative CCT examination. All children underwent the modified Morrow surgery in our center. We defined heart failure (HF), malignant ventricular arrhythmia, and recurrent left ventricular outflow tract obstruction (LVOTO) as adverse events. We performed a retrospective Cox analysis and conducted genetic testing. A 3D model of the LV was built through the standard 17-segment method and analyzing the high-risk factors. 17 (29.8%) had adverse events during follow-up. Multivariate Cox analysis revealed that genetic mutation (HR:5.634, 95%CI:1.663-19.086, P=0.005), Noonan syndrome (HR:3.770, 95%CI:1.245-11.419, P=0.019), preoperational systolic anterior motion (SAM)(HR:4.596, 95%CI:1.532-13.792, P=0.007)and mid-ventricular obstruction (HR:4.763, 95%CI:1.538-14.754, P=0.007) were high-risk factors, suggesting that the degree of hypertrophy in the left ventricle is associated with poor prognosis. By analyzing the CCT with 3D model, children with poor prognosis have more hypertrophy in basal-inferior (P=0.014), mid-inferoseptal(P=0.044), mid-inferior(P=0.017). It suggests that a more hypertrophied posterior left ventricular wall portends a worse prognosis. Even after modified Morrow surgery, the prognostic impact of genetic mutation remains significant. Moreover, the degree of hypertrophy of the posterior wall in the LV was also related to the postoperative prognosis through CCT combined with 3D technology. It provides surgeons guiding to evaluate the overall prognosis and the treatment plan before surgery.

Percutaneous coronary intervention in patients with hypertrophic cardiomyopathy and coronary artery disease

Background Coronary artery disease and left ventricular outflow tract obstruction may overlap in symptoms in patients with hypertrophic cardiomyopathy. It has not been clear if coronary revascularization relieves exertional symptoms in patients with hypertrophic cardiomyopathy. Methods We reviewed 156 patients with hypertrophic cardiomyopathy who underwent percutaneous coronary intervention at Anzhen Hospital from 2009 to 2019. Improvement in symptoms after the percutaneous coronary intervention was investigated. Results The mean age was 60.8 ± 9.8 years, and 116 (74.4%) were men. The main symptoms of the patients were chest tightness in 115 (73.7%), chest pain in 88 (56.4%), dyspnea in 50 (32.1%), palpitation in 22 (14.1%), and presyncope/syncope in 12 (7.7%). At rest, the systolic anterior motion of the mitral valve was observed in only 36 patients. Among the 156 patients, 64 had single-vessel disease, 46 had two-vessel diseases, and the other 46 had three-vessel diseases. There was no early mortality in the present cohort. After the coronary intervention, there were significant improvements in symptoms including chest tightness (6.4%), chest pain (1.3%), dyspnea (2.6%), and palpitation (8.3%). In 93 patients with follow-up data, the overall survival was 77% at 10 years and the survival free from intervention was 60% at 10 years. Conclusions In patients with hypertrophic cardiomyopathy, coronary artery disease can be an important cause of symptoms and should be screened. Percutaneous coronary intervention is safe and effective for coronary revascularization. Cardiac symptoms can be significantly relieved and long-term outcomes are favorable.

Midterm Outcomes: A Comprehensive Approach to Surgery for Hypertrophic Obstructive Cardiomyopathy

BackgroundLeft ventricular outflow tract (LVOT) obstruction in obstructive hypertrophic cardiomyopathy (HCM) is caused by a constellation of abnormalities. This study reviewed outcomes of a comprehensive approach to correct these abnormalities during surgery. MethodsThis was a single-institution study of patients with HCM who underwent septal myectomy from 2016 to 2023. Their New York Heart Association functional classification and most recent echocardiogram that estimated LVOT gradient and mitral valve function were tracked. ResultsThe study included 103 patients with a mean age of 54 years (interquartile range, 40-67 years) and common comorbidities: hypertension (50%) and atrial fibrillation (25%). On average, the preprocedure resting echocardiogram showed an LVOT gradient of 36.4 mm Hg and moderate or severe mitral regurgitation in 50.5% of patients. All patients underwent septal myectomy, and associated abnormalities contributing to LVOT obstruction were addressed. Elongation of the anterior leaflet of the mitral valve was typically treated with papillary muscle realignment (72%). Aberrant papillary muscle heads and elongated secondary chordae tendineae contributing to systolic anterior motion were resected (66%). Myocardial bands, including apicoseptal bands contributing to LVOT obstruction, were resected (68%). With an average follow-up of 4 years, 91% of patients were considered to be in New York Heart Association functional class I or II. Long-term echocardiographic follow-up showed a mean peak LVOT gradient of 11 mm Hg (interquartile range, 4-13 mm Hg). Only 1 patient had more than mild mitral regurgitation. ConclusionsA comprehensive surgical approach to HCM that addresses the entire constellation of abnormalities associated with HCM, including mitral valve anterior leaflet elongation, aberrant or displaced mitral valve subvalvular apparatus, and myocardial bands, leads to outstanding midterm outcomes.

An overview of the treatments for hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a very prevalent inherited disease with a wide global distribution and a prevalence rate of approximately 0.2% in the general population. Left ventricular hypertrophy (LVH) caused by sarcomere mutation is the primary reason of HCM. The histopathology feature is that cardiomyocyte hypertrophy, myocyte disorder and myocardial fibrosis lead to diminished diastolic function, left ventricular outflow tract obstruction (LVOTO) and arrhythmia, all of which result in serious cardiac complications. Previously, HCM was considered a malignant disease that was almost untreatable. With the improvement of medical standards and increasing awareness of HCM, it has become a highly treatable disease in contemporary times, with a significant decrease in mortality rates. However, there are still significant unmet requirements in the therapy of HCM. This paper draws on more than 100 references from the past four decades and summarizes current advances in the treatment of HCM. The article will review the pathogenesis and types, recent development in pharmacotherapy, invasive treatments and gene therapies, as well as dilemma and future development of HCM.

The Role of Alcohol Ablation Therapy in Hypertrophic Obstructive Cardiomyopathy Management

Objectives: The following study aims to investigate alcohol septal ablation’s (ASA) role in hypertrophic obstructive cardiomyopathy (HOCM) management, focusing on in-hospital outcomes, efficacy in reducing left ventricular outflow tract (LVOT) obstruction, and safety profiles. Methods: The study, conducted at the Institute of Cardiology, National Hospital of Sri Lanka, included a total of nine HOCM patients. Participants with exercise-induced symptoms, dyspnea/angina, and specific ventricular gradients were included; unidentified septal branches led to exclusion. Diagnostic assessments, including electrocardiogram, contrast echocardiography, and cardiac catheterization, confirmed HOCM. ASA procedures involved careful target septal branch selection, alcohol injection, and post-procedure care with temporary pacemaker monitoring. Results: The study population (N=9) comprised seven males and two females, with a mean age of 40. Pre- and post-ablation measurements showed 78% reduction in LVOT gradient. Immediate complications included chest pain (100%), while 22.2% developed complete heart block. Notably, no mortality occurred during the study. Conclusion: ASA proved safe and effective in managing HOCM, with significant mean reduction in post-ablation LVOT gradient (78%). Complications were generally minor, emphasizing ASA’s viability for symptomatic HOCM patients. While acknowledging the absence of randomized controlled trials comparing ASA with alternative techniques, this study contributes valuable insights to guide collaborative treatment decisions by specialists.

Changes in left ventricular-aortic angulation are associated with the development of obstruction in hypertrophic cardiomyopathy

ObjectiveTo characterize changes in ventricular morphology in patients with hypertrophic cardiomyopathy who develop left ventricular (LV) outflow tract obstruction. MethodsWe reviewed patients with hypertrophic cardiomyopathy with LV outflow tract obstruction who underwent septal myectomy from May 2012 to June 2023. Among 68 patients initially without obstruction documented up to 7.6 years (interquartile range, 6.3-9.4 years) before the operation, a comparison was made with 78 patients with nonobstructive hypertrophic cardiomyopathy over a similar period. Patients who did not develop obstruction were matched with those who did on sex, age, and maximum septal wall thickness during the initial echocardiography, identifying 41 matched pairs. Echocardiographic data, including 5 measures of angulation, were compared between the groups. ResultsThe median interval between echocardiographic assessments was 7.5 years (interquartile range, 6.3-8.1 years) among patients with obstruction versus 7.3 years (interquartile range, 6.2-9.0 years) in patients without nonobstruction. Patients with obstruction were more likely to have hypertension at both times. The maximum septal wall thickness increased within both groups (both P values < .001), but the magnitude of increase was not different between groups (P = .130). Patients with obstruction exhibited a greater increase in LV mass (P < .001) compared with patients without obstruction (P = .004). Aortic angulation significantly increased in 4 of the 5 measurements (all P values < .001) in patients with obstruction, whereas patients with no obstruction showed no change. Anterior and posterior mitral valve leaflet lengths and coaptation lengths remained similar in both groups over time. ConclusionsThe development of LV outflow tract obstruction in patients with hypertrophic cardiomyopathy was associated with progressive LV outflow tract angulation and increased LV hypertrophy, as reflected by LV mass. Progression to obstruction was not related to changes in the mitral valve leaflet morphology.

Genetic variations in PTPN11 lead to a recurrent Left Ventricular Outflow Tract Obstruction phenotype in childhood hypertrophic cardiomyopathy

ObjectiveLeft ventricular septal myotomy provides a favorable prognosis for children with hypertrophic obstructive cardiomyopathy (HOCM). However, some children still suffer from recurrent left ventricular outflow tract obstruction (LVOTO) after surgery. Poor prognosis exists for HOCM caused by PTPN11 mutation. Therefore, the aim of this study was to determine the clinical features of recurrent obstruction in children with HOCM caused by pathogenic mutations in the PTPN11 gene. MethodsA total of 56 children were diagnosed with HOCM underwent septal myectomies. Whole exome sequencing of 49 pediatric cardiomyopathies associated genes (including PTPN11) were performed. We performed hematoxylin-eosin(H&E), Masson, and wheat germ agglutinin (WGA)staining of tissues positive for PTPN11 and those negative for PTPN11 were conducted. ResultsWhole exome sequencing results showed 11 PTPN11 mutation (19.6%) children. In long-term follow-up (median 37 months, maximum 9 years), children with PTPN11 mutation had 6(54.5%) recurrent LVOTO compared with other groups (P=.015), but similar survival rates(P=.514). The mean postoperative time to recurrent obstruction was 22±27 months. Children with PTPN11 mutation were 9-fold more likely to experience the risk associated with recurrent obstruction (95% CI = 1.77-45.81, P<.001). H&E, Masson and WGA staining also revealed more cardiomyocyte hypertrophy in PTPN11 mutation tissues. See Figure 4 for a graphical abstract of the study. ConclusionChildren with PTPN11 mutation-associated hypertrophic cardiomyopathy have a higher risk of recurrent LVOTO.