Search

Although septal myectomy is the preferred treatment for medication-refractory hypertrophic obstructive cardiomyopathy (HOCM), the procedure remains subjective. A preoperative planning procedure is proposed using computational fluid dynamics simulations and shape optimization to assist in the objective assessment of the adequacy of the resection. 3 patients with HOCM were chosen for the application of the proposed procedure. The geometries of the preoperative left ventricular outflow tract (LVOT) of patients in the systolic phase were reconstructed from medical images. Computaional fluid dynamics (CFD) simulations were performed to assess hemodynamics within LVOT. Sensitivity analysis was performed to determine the resection extent on the septal wall, and the depth of the resection was optimized to relieve LVOT obstruction while minimizing damage to the septum. The optimized resection was then transferred from systole to diastole to provide surgeons with instructive guidance for septal myectomy. Comparison between preoperative and postoperative hemodynamics showed an evident improvement with respect to the pressure gradient throughout the LVOT. The resected myocardium in the diastolic phase is more extended and thinner than its state in the systolic phase. The proposed preoperative planning procedure may be a viable addition to the current preoperative assessment of patients with HOCM. [ABSTRACT FROM AUTHOR]

Background: Implantable Cardioverter Defibrillator (ICD) implantation has significantly modified the natural history of patients at high risk of sudden cardiac death (SCD) in various types of heart diseases. However there is a high rate of psychological distress and reduced quality of life in patients with an ICD, more evident in younger individuals. The ICD removal upon patient request is a very rare event and causes many clinical and ethical issues. Methods: The article discusses the case of a young patient affected by hypertrophic obstructive cardiomyopathy, who underwent implantable cardioverter defibrillator (ICD) implantation as a primary prevention of sudden cardiac death (SCD). Two years after the implantation, the patient repeatedly requested removal of the ICD due to of a significant and untreatable psychological device intolerance. Results: Intervention became possible only after extensive psychological evaluation, which excluded specific pathology, and the ratification of Italian law 219/2017 on informed consent and advance directives, which guarantees the patient's independent decisions on current and future medical treatment. The explantation was performed 7 years after the implant. The patient is alive and in good health. Conclusions: The paper debates the issues related to establishing a patient–physician relationship based on respect for the patient's autonomy and experience of illness, in reference to principles such as beneficence and non‐maleficence, and the conflicts that sometimes arise between them. If a paternalistic approach in the patient–physician relationship evolves into a patient‐centered model, it is more certain that the patient's choice is realistically known and shared, and that it is consistent with the patient's values and life goals. The shared decision making (SDM) process and the use of pathology‐specific decision aids are able to transform the informed consent tool, usually related to medical‐legal issues, into an aid for true partnership between the patient and the medical care team. [ABSTRACT FROM AUTHOR]

Introduction: We described the clinical characteristics of a patient with hypertrophic obstructive cardiomyopathy (HOCM) who had undergone transcoronary ablation of septal hypertrophy (TASH) twice and developed atrial flutter after radiofrequency ablation for atrial fibrillation (AF) due to pulmonary vein reconnection. This case of HOCM is unique because of its complex complications and multiple complex atrial arrhythmias. The treatment of HOCM was successful and the postoperative follow-up results was good. Methods and results: A 71-year-oldfemale, developed exertional dyspnea with palpitations 12 years ago, with a valid diagnosis of HOCM according to the echocardiography which showed an absolute increase in the interventricular septum thickness (22.8 mm). She underwent two rounds of TASH and only the second round was successful. During a visit due to recurrent palpitations, the patient was diagnosed with AF based on electrocardiographic examination. Circumferential pulmonary vein isolation (CPVI) was performed to treat AF. However, the recurrence of atrial flutter was detected on her electrocardiograms (ECGs) three years after the operation. Since the patient had an interstitial lung injury, there were relative contraindications for antiarrhythmic drugs. Due to restrictive use of antiarrhythmic drugs and continuous palpitation, the patient agreed to receive a second radiofrequency ablation. Left-sided macroreentrant circuits were identified via high-density mapping and successful ablation was performed at the isthmus. Conclusions: Performing catheter ablation and TASH respectively in patients with HOCM associated with AF would be tricky. But taking such a comprehensive and respective clinical treatment would be beneficial to patients in the long term. [ABSTRACT FROM AUTHOR]

This retrospective cohort study aimed to assess whether basal septal wall thickness (BSWT), anterior (AML) and posterior (PML) mitral leaflet length, or sex were associated with remaining left ventricular outflow tract obstruction (LVOTO) in patients with hypertrophic obstructive cardiomyopathy (HOCM) undergoing alcohol septal ablation (ASA). One hundred fifty-four patients who underwent ASA at the Karolinska University Hospital in Stockholm, Sweden, between 2009 and 2021, were included retrospectively. Anatomical and hemodynamic parameters were collected from invasive catheterization before and during ASA, and from echocardiography (ECHO) examinations before, during, and at 1-year follow-up after ASA. Linear and logistic regression models were used to assess the association between sex, BSWT, AML, PML, and outcome, which was defined as the remaining LVOTO (≥ 30 mmHg) after ASA. The median follow-up was 364 days (interquartile range 334–385 days). BSWT ≥ 23 mm (n = 13, 12%) was associated with remaining LVOTO at follow-up (p = 0.004). Elongated mitral valve leaflet length (either AML or PML) was present in 125 (90%) patients. Elongated AML (> 24 mm) was present in 67 (44%) patients, although AML length was not associated with remaining LVOTO at follow-up. Elongated PML (> 14 mm) was present in 114 (74%) patients and was not associated with remaining LVOTO at follow-up. No significant sex differences were observed regarding the remaining LVOTO. ECHO measurement of BSWT can be effectively used to select patients for successful ASA and identify those patients with a risk of incomplete resolution of LVOTO after ASA. [ABSTRACT FROM AUTHOR]

Abstract Although septal myectomy is the preferred treatment for medication-refractory hypertrophic obstructive cardiomyopathy (HOCM), the procedure remains subjective. A preoperative planning procedure is proposed using computational fluid dynamics simulations and shape optimization to assist in the objective assessment of the adequacy of the resection. 3 patients with HOCM were chosen for the application of the proposed procedure. The geometries of the preoperative left ventricular outflow tract (LVOT) of patients in the systolic phase were reconstructed from medical images. Computaional fluid dynamics (CFD) simulations were performed to assess hemodynamics within LVOT. Sensitivity analysis was performed to determine the resection extent on the septal wall, and the depth of the resection was optimized to relieve LVOT obstruction while minimizing damage to the septum. The optimized resection was then transferred from systole to diastole to provide surgeons with instructive guidance for septal myectomy. Comparison between preoperative and postoperative hemodynamics showed an evident improvement with respect to the pressure gradient throughout the LVOT. The resected myocardium in the diastolic phase is more extended and thinner than its state in the systolic phase. The proposed preoperative planning procedure may be a viable addition to the current preoperative assessment of patients with HOCM.

Abstract Introduction We described the clinical characteristics of a patient with hypertrophic obstructive cardiomyopathy (HOCM) who had undergone transcoronary ablation of septal hypertrophy (TASH) twice and developed atrial flutter after radiofrequency ablation for atrial fibrillation (AF) due to pulmonary vein reconnection. This case of HOCM is unique because of its complex complications and multiple complex atrial arrhythmias. The treatment of HOCM was successful and the postoperative follow-up results was good. Methods and results A 71-year-oldfemale, developed exertional dyspnea with palpitations 12 years ago, with a valid diagnosis of HOCM according to the echocardiography which showed an absolute increase in the interventricular septum thickness (22.8 mm). She underwent two rounds of TASH and only the second round was successful. During a visit due to recurrent palpitations, the patient was diagnosed with AF based on electrocardiographic examination. Circumferential pulmonary vein isolation (CPVI) was performed to treat AF. However, the recurrence of atrial flutter was detected on her electrocardiograms (ECGs) three years after the operation. Since the patient had an interstitial lung injury, there were relative contraindications for antiarrhythmic drugs. Due to restrictive use of antiarrhythmic drugs and continuous palpitation, the patient agreed to receive a second radiofrequency ablation. Left-sided macroreentrant circuits were identified via high-density mapping and successful ablation was performed at the isthmus. Conclusions Performing catheter ablation and TASH respectively in patients with HOCM associated with AF would be tricky. But taking such a comprehensive and respective clinical treatment would be beneficial to patients in the long term.

Atrial fibrillation (AF) in patients with hypertrophic obstructive cardiomyopathy (HOCM) may be caused by a primary atrial myopathy. Whether HOCM-related atrial myopathy affects mainly electrophysiological properties of the left atrium (LA) or also the right atrium (RA) has never been investigated. The purpose of this study was to characterize atrial conduction and explore differences in the prevalence of conduction disorders, potential fractionation, and low-voltage areas (LVAs) between the RA and LA during sinus rhythm (SR) as indicators of potential arrhythmogenic areas. Intraoperative epicardial mapping of both atria during SR was performed in 15 HOCM patients (age 50 ± 12 years). Conduction delay (CD) and conductin block (CB), unipolar potential characteristics (voltages, fractionation), and LVA were quantified. Conduction disorders and LVA were found scattered throughout both atria in all patients and did not differ between the RA and LA (CD: 2.9% [1.9%–3.6%] vs 2.6% [2.1%–6.4%], P =.541; CB: 1.7% [0.9%–3.1%] vs 1.5% [0.5%–2.8%], P =.600; LVA: 4.7% [1.6%–7.7%] vs 2.9% [2.1%–7.1%], P =.793). Compared to the RA, unipolar voltages of single potentials (SPs) and fractionated potentials (FPs) were higher in the LA (SP: P75 7.3 mV vs 10.9 mV; FP: P75 2.0 mV vs 3.7 mV). FP contained low-voltage components in only 18% of all LA sites compared to 36% of all RA sites. In patients with HOCM, conduction disorders, LVA, and FP are equally present in both atria, supporting the hypothesis of a primary atrial myopathy. Conceptually, the presence of a biatrial substrate and high-voltage FP may contribute to failure of ablative therapy of atrial tachyarrhythmias in this population. [Display omitted] [ABSTRACT FROM AUTHOR]

A 56-year-old female diagnosed with hypertrophic obstructive cardiomyopathy and myocardial bridge (MB) of the left anterior descending (LAD) coronary artery underwent septal myectomy with resolution of her left ventricular outflow tract gradient. She had ongoing refractory symptoms of exertional angina and fatigue for over a decade and finally presented to our clinic to be re-evaluated for treatment. Provocative angiographic testing confirmed significant ischemia secondary to LAD MB. She underwent robotic totally endoscopic off pump unroofing of the LAD MB with complete relief of her symptoms and return to full activity. We conclude that patients undergoing septal myectomy for hypertrophic obstructive cardiomyopathy should be evaluated for MB and undergo unroofing of the bridge at the time of surgery. A myocardial bridge (MB) is a condition in which a coronary artery, most often the left anterior descending, takes an intramuscular route and is covered by the myocardium leading to compression and potential ischemia. This case report adds to the growing body of evidence supporting the significance of considering MB in the overall management of hypertrophic obstructive cardiomyopathy, and the symptomatic relief that a patient can obtain from an unroofing procedure. [ABSTRACT FROM AUTHOR]

Background Myocardial fibrosis is a key pathological factor for the occurrence of ventricular arrhythmias in hypertrophic obstructive cardiomyopathy (HOCM). Case summary This case series reports on two patients diagnosed with HOCM who underwent 18F-fibroblast activation protein inhibitor (FAPI) positron-emission tomography/computed tomography imaging and Morrow myotomy procedure. The collected myocardial tissue was examined histopathologically. Both patients exhibited intense and heterogeneous 18F-FAPI uptake in the septum, with significant number of activated fibroblasts. Discussion Enhanced 18F-FAPI uptake was observed before irreversible fibrosis, and the degree of 18F-FAPI uptake was higher in tissue with greater fibrosis. 18F-FAPI imaging may provide a promising tool for guiding surgical strategy in HOCM, and further research is needed to fully explore its potential in clinical practice. [ABSTRACT FROM AUTHOR]

Background: Left ventricular outflow tract obstruction (LVOTO) impairs survival and diminishes quality of life in patients with hypertrophic obstructive cardiomyopathy (HOCM). In this study, we aimed to investigate the safety and the efficacy of earlier alcohol septal ablation (ASA) in patients with HOCM. Methods: A total of 47 patients with mildly symptomatic HOCM (NYHA II) and having poor functional capacity despite maximal tolerated medical therapy were included. Results: The mean age of the patients was 55 ± 14, and 57% of the patients were male. All clinical endpoint targets including 30 d mortality (1% vs. 0% p < 0.01), 30 d adverse complications (10% vs. 0% p < 0.01), 30 d complete heart block resulting in need for permanent pacemaker (10% vs. 4.2% p < 0.01), more than moderate residual mitral regurgitation (5% vs. 2.1% p < 0.01), repeat procedure rate (10% vs. 4.2% p < 0.01), improvement of (NYHA) class (90% vs. 95.7% p < 0.01), rest and provoked (LVOT) gradient < 50 mmHg (90% vs. 97.8% p < 0.01) were significantly reached. Conclusions: In patients with mildly symptomatic HOCM (NYHA II), earlier ASA may be performed as an effective and safe procedure in experienced centers. [ABSTRACT FROM AUTHOR]

Pulsed-field ablation (PFA) is a nonthermal method for achieving selective cell death with little inflammation response. However, there are no reports of PFA for septal reduction therapy (SRT). The purpose of this study was to investigate the effectiveness and safety of PFA for SRT. A novel transvenous intraseptal PFA method with 3-dimensional (3D) guidance was introduced in Yorkshire pigs. Electrocardiographic parameters, transthoracic echocardiography, and histopathology were used to evaluated. The maximum injury diameter of intramyocardial PFA increased with electric field intensity. After PFA, bipolar electrogram amplitude and pacing threshold measured by the PFA electrodes significantly decreased (F = 6.945, P =.007) or increased (F = 5.842, P =.024), respectively. In the ablated septal region, motion amplitude and systolic wall thickening rate significantly decreased and remained at low levels (motion amplitude: F = 20.793, P =.000; systolic wall thickening rate: F = 14.343, P =.000); however, septal thickness did not significantly change after PFA (F = 1.503, P =.248). Histologic examination showed specific cardiomyocyte death with gradually increased hyperchromatic cytoplasm and nuclear pyknosis, without obvious inflammatory cell infiltration in acute phase. TUNEL stain for fragmented DNA showed extensively positive in the ablation region 24 hours after PFA. During PFA, no sustained ventricular arrhythmia or atrioventricular conduction block occurred. A novel intraseptal PFA method with 3D guidance was described. Intraseptal PFA resulted in effective myocardial injury and local hypokinesis without significant acute edema. Histologic examination showed widely programmed cardiomyocyte death with little inflammatory cell infiltration. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: Radiofrequency ablation has been applied for the treatment of hypertrophic obstructive cardiomyopathy (HOCM). The two known procedures are percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) and endocardial radiofrequency septal ablation (ERSA). Methods: This study presents a retrospective analysis of the PIMSRA and ERSA procedures in patients with drug‐refractory HOCM. A total of 28 patients participated in the study, with 12 receiving PIMSRA and 16 receiving ERSA. The objective of our study was to compare the short‐term effects of these two radiofrequency ablation procedures. Results: At the 30‐day follow‐up, the PIMSRA group demonstrated a greater reduction in left ventricular outflow tract peak gradient at rest compared to the ERSA group (22.25 [16.72] mmHg versus 47.75 [21.94] mmHg) (p <.01). The values for the PIMSRA group decreased from 99.33 (32.00) mmHg to 22.25 (16.72) mmHg (p <.01), while the ERSA group decreased from 97.75 (30.24) mmHg to 47.75 (21.94) mmHg (p <.01). Only the PIMSRA group exhibited a decrease in mitral regurgitation (MR). The area of MR decreased from 10.13 (4.12) mm2 to 3.65 (2.80) mm2 in the PIMSRA group (p <.01). Additionally, the PIMSRA group experienced reductions in left atrial diameter (LAD) and left ventricular ejection fraction (LVEF)%. The values for LAD changed from 43.58 (7.53) mm to 37.08 (6.92) mm (p =.03), and the values for LVEF% decreased from 65.75 (6.12) pg/mL to 60.83 (4.06) pg/mL (p =.03). Conclusion: In terms of the two types of radiofrequency ablation methods used in HOCM, it has been observed that PIMSRA demonstrates a more favorable early treatment effect compared to ERSA. [ABSTRACT FROM AUTHOR]

Septal Myectomy (SM) and Alcohol Septal Ablation (ASA) improve symptoms in patients with Hypertrophic Cardiomyopathy with outflow tract obstruction (oHCM). However, outcomes data in this population is predominantly from specialized centers. The National Inpatient Database was queried from 2011 to 2019 for relevant international classification of diseases (ICD)-9 and -10 diagnostic and procedural codes. We compared baseline characteristics and in-hospital outcomes of patients with oHCM who underwent SM vs ASA. A p-value < 0.001 was considered statistically significant. We identified 15,119 patients with oHCM who underwent septal reduction therapies, of whom 57.4% underwent SM, and 42.6% underwent ASA. Patients who underwent SM had higher all-cause mortality (OR: 1.8 (1.3–2.5)), post-procedure ischemic stroke (OR: 2.3 (1.7–3.2)), acute kidney injury (OR: 1.4 (1.2–1.7)), vascular complications (OR: 3.6 (2.3–5.3)), ventricular septal defect (OR: 4.4 (3.2–6.1)), cardiogenic shock (OR: 1.7 (1.3–2.3)), sepsis (OR: 3.2 (1.9–5.4)), and left bundle branch block (OR: 3.5 (3–4)), compared to ASA. Patients who underwent ASA had higher post-procedure complete heart block (OR: 1.3 (1.1–1.4)), right bundle branch block (OR: 6.3 (5–7.7)), ventricular tachycardia (OR: 2.2 (1.9–2.6)), supraventricular tachycardia (OR: 1.6 (1.4–2)), and more commonly required pacemaker insertion (OR: 1.4 (1.3–1.7)) (p < 0.001 for all) compared to SM. This nationwide analysis evidenced that patients undergoing SM had higher in-hospital mortality and periprocedural complications than ASA; however, those undergoing ASA had more post-procedure conduction abnormalities and pacemaker implantation. The implications of these findings warrant further investigation regarding patient selection strategies for these therapies. [ABSTRACT FROM AUTHOR]

Severe left ventricular outflow tract obstruction (LVOTO) of hypertrophic cardiomyopathy is an acutely life-threatening, must-not miss, cardiology emergency that infrequently presents to the emergency department (ED). Patients with this condition usually manifest chest pain, syncope, cardiogenic shock, and severe ischemia. LVOTO is easy misdiagnosed as acute coronary syndrome. In our patient, the ECG showed a significant ST-segment depression and a 0/0 mmHg blood pressure when the peak left ventricular outflow tract gradient was abruptly increased by provocable activities. However, the patient had normal coronaries on cardiac catheterization, and, upon being immediately treated with intravenous esmolol, his symptoms were relieved and blood pressure was normal after 30 minutes. This case highlights, not only that early and exact diagnosis of LVOTO is crucial, but also the importance of the therapeutic strategies used.

Background Percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) has been reported to be safe and effective at midterm follow‐up to treat drug‐refractory hypertrophic obstructive cardiomyopathy in a single center. However, data from other centers are lacking. This retrospective cohort study aimed to investigate the efficacy and safety of PIMSRA from another independent center. Methods and Results PIMSRA was performed in 76 patients with hypertrophic obstructive cardiomyopathy in our center from April 2020 to June 2023. The primary outcome was the reduction of left ventricular outflow tract gradient after 6 months or more post‐PIMSRA. Secondary outcomes were periprocedural major adverse clinical events. Sixty‐one patients returned to the hospital for follow‐up 6 to 30 (median, 14) months after the procedure. At the last follow‐up of the 61 patients, the maximum septal thickness decreased from a median of 23.6 (interquartile range, 20.5–26.4) to 19.1 (interquartile range, 16.0–22.1) mm (P

Septal reduction therapy (SRT) is indicated for drug-refractory hypertrophic obstructive cardiomyopathy (HOCM). SRT includes surgical myectomy and alcohol septal ablation (ASA). The outcome between SRTs are similar except complete atrioventricular (AV) block. Intracoronary myocardial contrast echocardiography is used to minimize myocardial damage by ASA. We report a case of 40-year-old male who was diagnosed of HOCM with progressed symptoms under optimal medication. Echocardiography revealed peak velocity cross left ventricular outflow tract (LVOT) 5.3 m/s, systolic anterior motion (SAM) of mitral valve with eccentric mitral regurgitation (MR) and interventricular septal thickness 16 mm. Alcohol (99.5%) 1.5 mL was injected into the first small branch of the first septal artery, under precise localization by intracoronary myocardial contrast echocardiography. The pressure gradient of apex-LVOT-aorta reduced from 90 to 20 mmHg after ASA. No AV block was noted after the procedure and echocardiography revealed improved peak velocity cross LVOT and interventricular septal thickness. No more SAM or eccentric MR was observed. Previous studies recommended ASA reserved for patients with higher surgical risk and severe comorbidities. However, a recent study showed that young adults had better long-term survival and only one-half pacemaker implantation rate than older group following ASA. Under the guidance of intracoronary myocardial contrast, target vessel could be precisely localized to small branch from a septal artery to decrease myocardial damage. Therefore, ASA may be considered as the first-line SRT for symptomatic HOCM due to minimal invasiveness and effective outcome.

Hypertrophic cardiomyopathy (HCM) is characterized by excessive growth of myocardial tissue, most commonly due to genetic mutations in sarcomere proteins. This can lead to complications such as heart failure, mitral regurgitation, syncope, arrhythmias, sudden cardiac death, and myocardial ischemia. While we have come a long way in our understanding of the pathophysiology, genetics, and epidemiology of HCM, the past 10 years have seen significant advancements in diagnosis and treatment. As the body of evidence on hypertrophic cardiomyopathy continues to grow, a comprehensive review of the current literature is an invaluable resource in organizing this knowledge. By doing so, the vast progress that has been made thus far will be widely available to all experts in the field. This review provides a comprehensive analysis of the scientific literature, exploring both well-established and cutting-edge diagnostic and therapeutic options. It also presents a unique perspective by incorporating topics such as exercise testing, genetic testing, radiofrequency ablation, risk stratification, and symptomatic management in non-obstructive HCM. Lastly, this review highlights areas where current and future research is at the forefront of innovation in hypertrophic cardiomyopathy.

Background: Percutaneous radiofrequency catheter ablation (RFA) in hypertrophic obstructive cardiomyopathy (HOCM) with intracardiac echocardiography (ICE) guidance is a novel method that has been proven to be safe and effective in a small sample size study. RFA of the interventricular septum through a trans-atrial septal approach in HOCM patients with a longer follow-up has not been reported. Methods: 62 consecutive patients from March 2019 to February 2022 were included in this study. The area between the hypertrophied septum and anterior mitral valve (MV) leaflet was established using the three-dimensional system (CARTO 3 system), and all patients received atrial septal puncture under the guidance of intracardiac echocardiography (ICE). Point-by-point ablation was performed to cover the contact area. After ablation, the patients were followed up for 1, 3, 6, and 12 months. Transthoracic echocardiography was performed at 1, 3, 6, and 12 months, and resting and exercise-provoked left ventricular outflow tract (LVOT) gradients were obtained. Results: During the 1-year follow-up, most patients' symptoms improved. The NYHA grading of the patient decreased from 2 (2, 3) at baseline to 2 (1, 2) (p < 0.001). LVOT peak gradient at rest was decreased from 59 (±27) mmHg to 30 (±24) mmHg (p < 0.001), and the provoked peak gradient was decreased from 99 (±33) mmHg to 59 (±34) mmHg (p < 0.001). The average maximum septal thickness was reduced from 21 (±4) mm to 19 (±4) mm (p < 0.001). Conclusions: After a 1-year follow-up, ice-guided radiofrequency ablation for HOCM might be a safe, accurate, and effective method. The catheter might be reliably attached to the ablation target area via trans-atrial septal access. [ABSTRACT FROM AUTHOR]

Aims: In the EXPLORER‐HCM trial, mavacamten reduced left ventricular outflow tract obstruction (LVOTO) and improved functional capacity of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) patients. We sought to define the potential use of mavacamten by comparing real‐world HOCM patients with those enrolled in EXPLORER‐HCM and assessing their eligibility to treatment. Methods and results: We collected information on HOCM patients followed up at 25 Italian HCM outpatient clinics and with significant LVOTO (i.e. gradient ≥30 mmHg at rest or ≥50 mmHg after Valsalva manoeuvre or exercise) despite pharmacological or non‐pharmacological therapy. Pharmacological or non‐pharmacological therapy resolved LVOTO in 1044 (61.2%) of the 1706 HOCM patients under active follow‐up, whereas 662 patients (38.8%) had persistent LVOTO. Compared to the EXPLORER‐HCM trial population, these real‐world HOCM patients were older (62.1 ± 14.3 vs. 58.5 ± 12.2 years, p = 0.02), had a lower body mass index (26.8 ± 5.3 vs. 29.7 ± 4.9 kg/m2, p < 0.0001) and a more frequent history of atrial fibrillation (21.5% vs. 9.8%, p = 0.027). At echocardiography, they had lower left ventricular ejection fraction (LVEF, 66 ± 7% vs. 74 ± 6%, p < 0.0001), higher left ventricular outflow tract gradients at rest (60 ± 27 vs. 52 ± 29 mmHg, p = 0.003), and larger left atrial volume index (49 ± 16 vs. 40 ± 12 ml/m2, p < 0.0001). Overall, 324 (48.9%) would have been eligible for enrolment in the EXPLORER‐HCM trial and 339 (51.2%) for treatment with mavacamten according to European guidelines. Conclusions: Real‐world HOCM patients differ from the EXPLORER‐HCM population for their older age, lower LVEF and larger atrial volume, potentially reflecting a more advanced stage of the disease. About half of real‐world HOCM patients were found eligible to mavacamten. [ABSTRACT FROM AUTHOR]

Accessory mitral valve tissue (AMVT) is a rare congenital anomaly that sometimes causes left ventricular outflow tract (LVOT) obstruction. We report the case of a 72-year-old woman with hypertrophic obstructive cardiomyopathy (HOCM) complicated by AMVT. The patient presented at our hospital with palpitations and shortness of breath. Transthoracic echocardiography revealed a diagnosis of HOCM and an abnormal structure inside the LVOT. Transesophageal echocardiography revealed an AMVT. We initially treated the patient with oral medication, but due to side effects, the patient could not take the target dose and her symptoms did not improve. We suggested surgical treatment, but the patient refused. By evaluating the relationship between AMVT and the surrounding tissues using three-dimensional transesophageal echocardiography, we determined that percutaneous septal myocardial ablation (PTSMA) might be successful. The first PTSMA was not effective, but the second procedure showed significant improvement in the pressure gradient and symptoms. The patient with HOCM and concomitant AMVT had a severe LVOT pressure gradient, and PTSMA was performed with excellent results. Since we experienced a rare case and were able to treat it percutaneously, we report our findings in relation to the literature. This case study highlights successful use of percutaneous septal myocardial ablation (PTSMA) in treating a patient with hypertrophic obstructive cardiomyopathy (HOCM) and accessory mitral valve tissue (AMVT). The key objective is to understand PTSMA can be an effective treatment option for HOCM with Type IIa AMVT, characterized by the attachment only to the mitral leaflets, when surgical intervention is not preferred, enhancing management of this rare condition. [ABSTRACT FROM AUTHOR]

Background: Right ventricular involvement in hypertrophic cardiomyopathy is uncommon. This study aimed to evaluate clinical outcomes of the modified septal myectomy in patients diagnosed with biventricular hypertrophic cardiomyopathy (BHCM), a subject seldom explored in the literature. Methods: We conducted a retrospective cohort study from January 2019 to January 2023, enrolling 12 patients with BHCM. Each patient underwent a modified septal myectomy and was followed postoperatively. Clinical data and echocardiographic parameters, including the ventricular outflow tract peak pressure gradient and maximum interventricular septum thickness, were collected and analyzed. Results: The study cohort had a median age of 43.0 (interquartile range 14.5-63.0) years at surgery, with four patients (33.3%) being children. Two patients (16.7%) previously underwent percutaneous transluminal septal myocardial ablation. Surgical relief of biventricular outflow tract obstruction (BVOTO) was achieved in five patients (41.7%), aside from those managed solely for left ventricular outflow tract obstruction. In five instances, three-dimensional (3D) printing technology assisted in surgical planning. The postoperative interventricular septum thickness was significantly reduced (21.0 mm preoperative vs. 14.5 mm postoperative, p < 0.001), effectively eliminating residual ventricular outflow tract obstruction. There were no severe complications, such as septal perforation or third-degree atrioventricular block. During a mean follow up of 21.2 ± 15.3 months, no sudden deaths, residual outflow tract obstruction, permanent pacemaker implantation, recurrent systolic anterior motion, or reoperations were reported. Conclusions: Our findings affirm that the modified septal myectomy remains the gold standard treatment for BHCM, improving patient symptoms and quality of life. BVOTO relief can be safely and effectively achieved through septal myectomy via transaortic and pulmonary valve approaches in selected patients. For intricate cases, the application of 3D printing technology as a preoperative planning tool is advised to optimize surgical precision and safety. [ABSTRACT FROM AUTHOR]

Introduction: Hypertrophies of the cardiac septum are caused either by aortic valve stenosis (AVS) or by congenital hypertrophic obstructive cardiomyopathy (HOCM). As they induce cardiac remodeling, these cardiac pathologies may promote an arrhythmogenic substrate with associated malignant ventricular arrhythmias and may lead to heart failure. While altered calcium (Ca2+) handling seems to be a key player in the pathogenesis, the role of mitochondrial calcium handling was not investigated in these patients to date. Methods: To investigate this issue, cardiac septal samples were collected from patients undergoing myectomy during cardiac surgery for excessive septal hypertrophy and/or aortic valve replacement, caused by AVS and HOCM. Septal specimens were matched with cardiac tissue obtained from postmortem controls without cardiac diseases (Ctrl). Results and discussion: Patient characteristics and most of the echocardiographic parameters did not differ between AVS and HOCM. Most notably, the interventricular septum thickness, diastolic (IVSd), was the greatest in HOCM patients. Histological and molecular analyses showed a trend towards higher fibrotic burden in both pathologies, when compared to Ctrl. Most notably, the mitochondrial Ca2+ uniporter (MCU) complex associated proteins were altered in both pathologies of left ventricular hypertrophy (LVH). On the one hand, the expression pattern of the MCU complex subunits MCU and MICU1 were shown to be markedly increased, especially in AVS. On the other hand, PRMT-1, UCP-2, and UCP-3 declined with hypertrophy. These conditions were associated with an increase in the expression patterns of the Ca2+ uptaking ion channel SERCA2a in AVS (p = 0.0013), though not in HOCM, compared to healthy tissue. Our data obtained from human specimen from AVS or HOCM indicates major alterations in the expression of the mitochondrial calcium uniporter complex and associated proteins. Thus, in cardiac septal hypertrophies, besides modifications of cytosolic calcium handling, impaired mitochondrial uptake might be a key player in disease progression. [ABSTRACT FROM AUTHOR]

Abstract Midventricular hypertrophic obstructive cardiomyopathy (HOCM) is characterized by hypertrophy of the interventricular septum (IVS) and - in rare cases - of the papillary muscles (PM), which subsequently can cause dynamic left ventricular outflow tract obstruction (LVOTO) and severe heart failure symptoms. We report on a rare case of a 44-year-old patient suffering from midventricular HOCM with hypertrophic anterolateral PM and an additional chorda between the PM and the IVS. We describe a new surgical approach via right anterolateral thoracotomy in 3-dimensional (3D) video-assisted minimal-invasive technique with resection of hypertrophic PMs as well as the entire mitral valve-apparatus and valve replacement avoiding septal myectomy and potentially associated complications. After an uneventful procedure clinical symptoms improved from NYHA III-IV at baseline to NYHA 0-I postoperatively and remained stable over a follow-up period of 24 months. Therefore, the presented technique may be considered as a new and alternative approach in patients with hypertrophic PMs and hypertrophic IVS as subtype of midventricular HOCM.

BackgroundThe pulsed-electron avalanche knife (PEAK) PlasmaBlade provides an atraumatic, scalpel-like cutting precision and electrocautery-like hemostasis. PlasmaBlade operates near body temperature, and its long, thin, and malleable tip can overcome the limitations of a surgical knife. In this study, we aimed to evaluate our clinical experience and histopathological outcomes of septal myectomy using PlasmaBlade.MethodsElectronic medical records were reviewed for preoperative, operative, and follow-up data of the patients who underwent septal myectomy using PEAK PlasmaBlade at our institute between January 2019 and December 2022. Histopathology of the myectomy specimens was reviewed for the depth of muscle necrosis and compared with the left atrial appendage (LAA) specimen.ResultsTwenty-nine patients underwent septal myectomy using the PEAK PlasmaBlade. No mortality was reported. The mean age was 60.6 ± 12.5 years, and 58.6% of patients were male. Peak left ventricular outflow tract (LVOT) gradients were 40.5 ± 34.9 mmHg at rest and 56.5 ± 34.9 mmHg after provocation. Concomitant procedures performed were LAA ligation in 20 (69.0%), aortic valve replacement in 5 (17.2%), and coronary artery bypass grafting in 3 (10.3%) patients. Postoperative complications were complete heart block in one (3.4%) and ventricular septal defect in two (6.9%) patients. Both the ventricular septal defects were identified intraoperatively and repaired. Histopathology of myectomy specimens demonstrated cautery artifact limited to 1,000 µm with conventional electrocautery. At a mean follow-up of 8.4 ± 10.3 months, the mean LVOT gradient was 4.4 ± 5.8 mmHg at rest and 9.5 ± 3.3 mmHg after provocation. All patients were alive and in New York Heart Association class I/II. No patient developed complications or required reintervention or reoperation.ConclusionAdequate septal myectomy can be precisely and safely performed using the PEAK PlasmaBlade with minimal collateral damage.

BackgroundAnomalous papillary muscle (APM) insertion into the mitral valve leaflet is rare but clinically important in hypertrophic obstructive cardiomyopathy (HOCM). In this study, we report the detection rate of APM insertion into the mitral valve using preoperative imaging modalities and the surgical outcomes of the patients.MethodsBy retrospectively reviewing the clinical records of patients with HOCM who underwent surgical treatment by a single operation group at our center from January 2020 to June 2023, patients with APM insertion into the mitral valve leaflet were identified. Baseline data, image characteristics, and surgical outcomes were analyzed.ResultsThe incidence of APM insertion into the mitral valve leaflet was 5.1% (8/157). The insertion site was located at A3 in six cases, which was more common than at A2 (n = 2). Preoperative echocardiography was used to identify two patients (25%) with APM insertion. We observed a particular echocardiographic feature for APM in HOCM patients, which was noted as a “lip nevus sign”, with a higher detection rate (62.5%). All patients successfully underwent septal myectomy with concomitant APM excision or mitral valve replacement via the transaortic (n = 5) or transmitral (n = 3) approach. The mean age was 49.0 ± 17.4 years and seven patients (87.5%) were female. Interventricular septum thickness (17.0 mm vs. 13.3 mm, P = 0.012) and left ventricular outflow gradient (117.5 mmHg vs. 7.5 mmHg, P = 0.012) were significantly decreased after surgery. Residual outflow obstruction, systolic anterior motion, and ≥3+ mitral regurgitation were negative. During the follow-up of 26.2 ± 12.2 months, there were no reported operations, adverse events, mitral regurgitation aggravations, recurrences of outflow obstruction, or instances of SAM.ConclusionsPapillary muscles inserted into the mitral valve leaflet are a subtype of subvalvular malformation in HOCM that requires surgical correction. The lip nevus sign on echocardiography is a characteristic of APM insertion in HOCM and may improve the preoperative detection rate. Adequate myectomy with anomalous papillary muscle excision has achieved good results in reducing the outflow gradient and eliminating mitral regurgitation, with good outcomes at short-to-intermediate follow-up.

Purpose: This study aimed to evaluate the early morphology and function of the left heart in hypertrophic obstructive cardiomyopathy (HOCM) after transapical beating-heart septal myectomy (TA-BSM) using cardiovascular magnetic resonance (CMR). Materials and methods: Between April 2022 and January 2023, HOCM patients who underwent CMR before and 3 months after TA-BSM were prospectively and consecutively enrolled in the study. Preoperative and postoperative cardiac morphological and functional parameters, including those for the left atrium (LA) and left ventricle (LV), were compared. The left ventricular remodeling index (LVRI) was defined as the ratio between left ventricular mass (LVM) and left ventricular end-diastolic volume (LVEDV). Healthy participants with a similar age and sex distribution were enrolled for comparison. Pearson or Spearman correlation analysis was used to investigate the relationships between the parameters and LVRI. Last, univariate and multivariate linear regression identified variables associated with the LVM index (LVMI) and LVRI. Results: Forty-one patients (mean age ± standard deviation, 46 ± 2 years; 27 males) and 41 healthy control participants were evaluated. Eighteen (44%) HOCM patients were classified as having a sigmoid septum, and 23 patients had a reverse septal curvature. LA volume, diameter and function were significantly improved postoperatively, but still worse than healthy controls (all p < 0.001). Compared to before the operation, left ventricular wall thickness, left ventricular ejection fraction (LVEF), LVMI, and LVRI decreased after TA-BSM (all p < 0.001). The left ventricular end-diastolic volume index (LVEDVI) and left ventricular end-diastolic diameter (LVEDD) decreased in patients with a sigmoid septum. However, LVEDVI and LVEDD increased in those with a reverse septal curvature (both p < 0.001). In addition, both preoperative and postoperative LVRI was positively correlated with LVMI (r = 0.734 and 0.853, both p < 0.001) and maximum wall thickness (r = 0.679 and 0.676, both p < 0.001), respectively. In the multivariable analysis, the weight of the resected myocardium (adjusted β = 0.476, p = 0.005) and △mitral regurgitation degree (adjusted β = − 0.245, p = 0.040) were associated with △LVRI. Last, the △LVOTG (adjusted β = 0.436, p = 0.018) and baseline LVMI (adjusted β = 0.323, p = 0.040) were independently associated with greater left ventricular mass regression after TA-BSM. Conclusion: CMR confirmed early reverse remodeling of left heart morphology and function in HOCM patients following TA-BSM. [ABSTRACT FROM AUTHOR]

Current pharmacological options for hypertrophic cardiomyopathy (HCM) are not disease-specific; while it treats symptoms, mavacamten targets the underlying pathology. We aim to assess the efficacy and safety of mavacamten, a first-in-class cardiac myosin inhibitor, in symptomatic obstructive HCM. This systematic review of the literature followed the PRISMA guidelines. Title/abstract and topics were searched using the following term: "mavacamten". The electronic research literature databases included the Cochrane Library, MedLine, and clinicaltrials.gov from July to August 2022. Primary efficacy endpoint was to assess clinical response at the end of treatment compared with baseline, defined as, at least one New York Heart Association (NYHA) class reduction. Two secondary endpoints from baseline were determined. The first was defined as improvement in mixed venous oxygen pressure (pVO 2). The second was defined as reduction of the post-exercise left ventricular outflow tract (LVOT) gradient. We included in our analyses data from four studies that met our review eligibility criteria. There were three randomised placebo-controlled clinical trials and one non-randomised open-label clinical trial. All four studies showed a reduction in NYHA class from mavacamten use. Three out of four studies demonstrated >1 NYHA functional class improvement ranging from 34% to 80%, while only one study showed a smaller percentage of patients remaining at class 3. Three out of four studies measured pVO 2 as an outcome, and all three studies noticed an increase in peak oxygen consumption after mavacamten treatment. Additionally, three out of four studies measured post-exercise LVOT gradient reduction as an outcome and all three found significant reduction in the post-exercise LVOT gradient after treatment. The most commonly observed adverse side effects were atrial fibrillation and decreased left ventricular ejection fraction, but all participants recovered without long-term sequelae and only one patient dropped out of the trial. Mavacamten has a greater efficacy than placebo in the treatment of HCM. It also showed promising tolerability and efficacy profiles in the treatment of HCM in adults. The three endpoints used in the evaluation of studies were reduction in NYHA class, increase in pVO 2 , and post-exercise LVOT gradient reduction. Mavacamten showed greater reduction in NYHA, larger effects on increase of pVO 2 , and significant reduction of the LVOT gradient. Mavacamten was also found to be well tolerated, like the placebo. The side effect profile was limited for the majority of individuals taking mavacamten. In the future, authors recommended dose-optimisation studies, and studies that evaluate mavacamten both in comparison to, and in conjunction with other current treatments. [ABSTRACT FROM AUTHOR]

Left ventricular apical aneurysm (LVAA) is a rare complication of hypertrophic obstructive cardiomyopathy (HOCM). This study aimed to illustrate the clinical characteristics and surgical outcomes of these patients. Patients with HOCM and LVAA who underwent modified extended Morrow myectomy and surgical left ventricular reconstruction (SLVR) between October 2012 and March 2021 were retrospectively recruited. Patients with coronary artery disease were excluded. Clinical characteristics were summarised. Time-to-event outcomes were calculated using the Kaplan-Meier method and compared by a log-rank test. Fifteen eligible patients were identified; the mean age was 39.9±17.2 years and 40.0% of them were female. All patients had dyspnoea, 46.7% presented with syncope and/or chest pain, and 13.3% had a family history of hypertrophic cardiomyopathy. The mean LVAA size was 36.9±12.3 mm in length and 28.5±11.3 mm in width. Echocardiography showed LV outflow tract obstruction in seven (46.7%) patients, mid-cavity LV obstruction in 12 (80.0%), while systolic anterior motion (SAM) was seen in seven (46.7%). The symptoms were resolved in all patients postoperatively. During a median follow-up of 22.0 months, one (6.7%) patient had sudden cardiac death, one (6.7%) had a haemorrhagic stroke, and the LVAA recurrence was 40.0%. Subgroup analysis showed that signs of SAM and larger LVAA (≥30 mm) were associated with an increased tendency for a longer hospital stay. Patients with HOCM and LVAA present with high-risk profiles. Modified extended Morrow myectomy combined with SLVR is useful in relieving the symptoms and improving the prognosis, although there might be recurrent LVAA. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Anomalous insertion of chordae is a rare disease that could be associated with hypertrophic obstructive cardiomyopathy (HOCM), but clinical and echocardiographic diagnoses tend to be delayed. Alcohol septal ablation has emerged as an alternative to surgical myomectomy in HOCM. When a patient showed an anomalous insertion of chordae, physicians generally opt for surgery and not alcohol septal ablation. In this report, we present the case of a lady, with symptomatic HOCM associated with a chord inserted on the left ventricular outflow tract. We succeeded to relieve obstruction by alcohol septal ablation without the need for surgery. [ABSTRACT FROM AUTHOR]

Background We present an uncommon case of a patient with hypertrophic obstructive cardiomyopathy and idiopathic pulmonary fibrosis. The case demonstrates the importance of pre-transplant cardiology workup and the need of interdisciplinary approach in diagnosing the cause of dyspnoea. Case summary The 52-year-old male patient was diagnosed with idiopathic pulmonary fibrosis in 2019 and gradually became oxygen dependent due to progression of dyspnoea. Bilateral lung transplantation was recommended in 2021. During pre-transplant cardiology workup, the patient was diagnosed with hypertrophic cardiomyopathy with left ventricular outflow tract (LVOT) obstruction. Considering the high surgical risk of the patient, alcohol septal ablation was performed with subsequent decrease of LVOT gradient. Bilateral lung transplantation was successfully performed afterwards. The patient's symptoms improved to NYHA class II at one year follow-up. Discussion We present a rare case of combined cause of dyspnoea—coexistence of hypertrophic obstructive cardiomyopathy and idiopathic pulmonary fibrosis in one patient. Due to high surgical risk, the patient underwent alcohol septal ablation with successful elimination of LVOT gradient and subsequently bilateral lung transplantation. [ABSTRACT FROM AUTHOR]

Introduction: Hypertrophies of the cardiac septum are caused either by aortic valve stenosis (AVS) or by congenital hypertrophic obstructive cardiomyopathy (HOCM). As they induce cardiac remodeling, these cardiac pathologies may promote an arrhythmogenic substrate with associated malignant ventricular arrhythmias and may lead to heart failure. While altered calcium (Ca2+) handling seems to be a key player in the pathogenesis, the role of mitochondrial calcium handling was not investigated in these patients to date.Methods: To investigate this issue, cardiac septal samples were collected from patients undergoing myectomy during cardiac surgery for excessive septal hypertrophy and/or aortic valve replacement, caused by AVS and HOCM. Septal specimens were matched with cardiac tissue obtained from post-mortem controls without cardiac diseases (Ctrl).Results and discussion: Patient characteristics and most of the echocardiographic parameters did not differ between AVS and HOCM. Most notably, the interventricular septum thickness, diastolic (IVSd), was the greatest in HOCM patients. Histological and molecular analyses showed a trend towards higher fibrotic burden in both pathologies, when compared to Ctrl. Most notably, the mitochondrial Ca2+ uniporter (MCU) complex associated proteins were altered in both pathologies of left ventricular hypertrophy (LVH). On the one hand, the expression pattern of the MCU complex subunits MCU and MICU1 were shown to be markedly increased, especially in AVS. On the other hand, PRMT-1, UCP-2, and UCP-3 declined with hypertrophy. These conditions were associated with an increase in the expression patterns of the Ca2+ uptaking ion channel SERCA2a in AVS (p = 0.0013), though not in HOCM, compared to healthy tissue. Our data obtained from human specimen from AVS or HOCM indicates major alterations in the expression of the mitochondrial calcium uniporter complex and associated proteins. Thus, in cardiac septal hypertrophies, besides modifications of cytosolic calcium handling, impaired mitochondrial uptake might be a key player in disease progression.

Hypertrophic cardiomyopathy (HCM) is a genetic myocardial disease, with an estimated incidence of 0.2%–6%, and is the main cause of sudden cardiac death (SCD) in young athletes. Left ventricular apical aneurysm (LVAA) is a rare subtype of HCM, accounting for about 5% of HCM patients, and has a higher incidence of cardiovascular adverse events. In cases of hypertrophic obstructive cardiomyopathy with LVAA (HOCM-LVAA) that do not respond adequately to optimized medical therapy, the echocardiography-guided percutaneous intra-myocardial septal radiofrequency ablation (PIMSRA, Liwen procedure) emerges as a promising and effective novel therapeutic approach. In this case report, we present for the first time a comprehensive application of echocardiographic techniques, including TTE, 2-D STE, and contrast enhancement, in the diagnosis, treatment, surgical guidance, and assessment of therapeutic outcomes in a case of HOCM-LVAA.

Key Clinical Message Anomalous insertion of chordae is a rare disease that could be associated with hypertrophic obstructive cardiomyopathy (HOCM), but clinical and echocardiographic diagnoses tend to be delayed. Alcohol septal ablation has emerged as an alternative to surgical myomectomy in HOCM. When a patient showed an anomalous insertion of chordae, physicians generally opt for surgery and not alcohol septal ablation. In this report, we present the case of a lady, with symptomatic HOCM associated with a chord inserted on the left ventricular outflow tract. We succeeded to relieve obstruction by alcohol septal ablation without the need for surgery.

To simplify surgical septal reduction therapy for hypertrophic obstructive cardiomyopathy (HOCM), we developed a novel transapical beating-heart septal myectomy (TA-BSM) procedure. In this study, we sought to evaluate the clinical utility of TA-BSM in a first-in-human trial. Patients with HOCM were enrolled if they presented with drug-refractory disabling symptoms. TA-BSM was performed via minithoracotomy with the use of our beating-heart myectomy device under echocardiographic guidance, without the use of cardiopulmonary bypass. Repeated resections were performed to tailor the extent of the septal myectomy for sufficient abolishment of left ventricular outflow tract (LVOT) obstruction and mitral regurgitation (MR). The primary outcome measure was procedural success, defined by resting/provoked LVOT gradient <30/50 mm Hg and residual MR grade ≤1+ (of 4+) at 3-month follow-up. A total of 47 patients aged 12 to 77 years were enrolled. Of the 46 patients who were followed for 3 months, 42 achieved procedural success. The maximal LVOT gradient decreased from 86 mm Hg (IQR: 67-114 mm Hg) at baseline to 19 mm Hg (IQR: 14-28 mm Hg) at 3 months. MR grade was ≤1+ in 3 patients at baseline and in 45 patients at 3 months. One patient died on postoperative day 10 owing to device-unrelated reasons. Other major adverse events included 1 delayed ventricular septal perforation and 1 intraoperative left ventricular apical tear. TA-BSM is a safe and efficient minimally invasive procedure for septal reduction of heterogeneous HOCM. Compared with conventional septal myectomy, TA-BSM provides real-time evaluation to guide resection while reducing surgical trauma. (Transapical Beating-Heart Septal Myectomy in Patients With Hypertrophic Obstructive Cardiomyopathy; NCT05332691) [Display omitted] [ABSTRACT FROM AUTHOR]

Hypertrophic obstructive cardiomyopathy (HOCM), although a worldwide prevalent cardiac disease, it imposes a greater task in the patient management. The association of coronary artery disease with HOCM poses an immense perioperative challenge and it demands an expert transesophageal echocardiography (TEE) examination for guiding the surgery and detailed assessment after surgical correction. We report a case of HOCM with coronary artery disease where the post-cardiopulmonary bypass three-dimensional TEE played a crucial role in exact identification of the mechanism of fixed left ventricular outflow tract obstruction, when the two-dimensional TEE failed to provide adequate information. [ABSTRACT FROM AUTHOR]

Background: The outcomes after septal myectomy in young children and infants with hypertrophic obstructive cardiomyopathy (HOCM) are not clear. The study sought to report the outcomes after septal myectomy in young children and infants and identify the mechanisms of residual or recurrent obstruction after surgery. Methods: The authors performed an observational cohort study of children and infants under the age of 14 who underwent septal myectomy for HCOM from January 2013 to December 2020. Mean follow-up among 94.3% (n=50) of hospital survivors was 42.09 ± 24.38 months. Results: In total, 56 children and infants [mean (SD) age, 5.38 (3.78) years; 29 (58.1%) were male] underwent septal myectomy for HOCM. Cumulative survival was 100, 96.6, 93.0, and 81.4% at 1, 3, 5, and 7 years, respectively, among hospital survivors. The incidence of residual and recurrent obstruction was 14.3% (8/56) and 13.0% (6/46), respectively. The mechanisms of residual obstruction were identified as subaortic obstruction caused by inadequacy of previous septal excision in two patients, midventricular obstruction caused by inadequacy of septal excision in five patients, and untreated abnormal papillary muscles in one patient. Recurrent obstruction was caused by isolated midventricular obstruction (n= 4) and newly emerged systolic anterior motion (SAM)- related subaortic obstruction combining abnormal mitral valve apparatus (n =2). Residual or recurrent obstruction was associated with age less than 2 years at surgery (OR= 6.157, 95% CI: 1.487-25.487, P =0.012) and biventricular outflow obstruction (OR =6.139, 95% CI: 1.292-29.172, P= 0.022). Recurrent obstruction was associated with age less than 2 years at surgery (OR =6.976, 95% CI: 1.233-39.466, P= 0.028). Conclusions: Septal myectomy is still effective and safe in young children and infants. The rate of residual or recurrent obstruction with diverse causes is relatively high, which is more likely to occur in children aged less than 2 years at surgery and those with biventricular obstruction. [ABSTRACT FROM AUTHOR]

Aims Echocardiographic diagnosis of left ventricular outflow tract obstruction (LVOTO) in hypertrophic cardiomyopathy (HCM) often requires extensive provocative manoeuvers. We investigated, whether echocardiography-derived parameters obtained at rest can aid to determine the presence of LVOTO in persons with HCM. Methods and results Consecutive patients with HCM admitted to a referral centre underwent standardized transthoracic echocardiographic examination including provocative manoeuvers. Under resting conditions, the length of mitral leaflets and distances between mitral valve coordinates and ventricular walls were blindly measured in parasternal long axis (PLAX) and apical three-chamber (3ch) views, both at early and late systole. Among 142 patients (mean age 59 ± 13 years, 42% women), 68 (42%) had resting or provocable LVOTO with maximal LVOT gradients ≥30 mmHg. Late-systolic distance between mitral leaflet tip and ventricular septum (TIS) was measurable in 137 participants (96%) in 3ch view and independently associated with LVOTO in multivariable logistic regression analysis. The area under the ROC curve of TIS for the identification of LVOTO was 0.91 [95% confidence interval (CI) 0.87–0.96]. TIS ≤ 14 mm yielded 97% sensitivity and 57% specificity regarding LVOTO. TIS >14 mm ruled out LVOTO with a negative predictive value of 95%. TIS ≤9 mm ruled in LVOTO with a positive predictive value of 92% (sensitivity 73%, specificity 95%). Among 43 patients with TIS between 10 and 14 mm, 35% had LVOTO. Conclusion In our study, the novel echocardiographic parameter TIS showed high negative and positive predictive values for LVOTO in HCM. These exploratory results await confirmation in larger collectives and prospective investigations. [ABSTRACT FROM AUTHOR]

We report an unusual case of a patient with hypertrophic obstructive cardiomyopathy, anomalous aortic origin of a coronary artery, obesity hypoventilation syndrome, and acquired long QT syndrome who was able to defy the odds of sudden cardiac death in the rarest of circumstances. (Level of Difficulty: Advanced.).

Abstract Background Assessing the structure and function of left atrium (LA) is crucial in hypertrophic obstructive cardiomyopathy (HOCM) because LA remodeling correlates with atrial fibrillation. However, few studies have investigated the potential effect of myomectomy on LA phasic remodeling in HOCM after myectomy using cardiovascular magnetic resonance (CMR) feature tracking (FT). This study aims to evaluate the LA structural and functional remodeling with HOCM after myectomy by CMR-FT and to further investigate the determinants of LA reverse remodeling. Methods In this single-center study, we retrospectively studied 88 patients with HOCM who received CMR before and after myectomy between January 2011 and June 2021. Preoperative and postoperative LA parameters derived from CMR-FT were compared, including LA reservoir function (total ejection fraction [EF], total strain [εs], peak positive strain rate [SRs]), conduit function (passive EF, passive strain [εe], peak early negative strain rate [SRe]) and booster function (booster EF, active strain [εa], late peak negative strain rate [SRa]). Eighty-six healthy participants were collected for comparison. Univariate and multivariate linear regression identified variables associated with the rate of change of εa. Results Compared with preoperative parameters, LA reservoir function (total EF, εs, SRs), booster function (booster EF, εa, SRa), and SRe were significantly improved after myectomy (all P

Hypertrophic obstructive cardiomyopathy (HOCM), although a worldwide prevalent cardiac disease, it imposes a greater task in the patient management. The association of coronary artery disease with HOCM poses an immense perioperative challenge and it demands an expert transesophageal echocardiography (TEE) examination for guiding the surgery and detailed assessment after surgical correction. We report a case of HOCM with coronary artery disease where the post-cardiopulmonary bypass three-dimensional TEE played a crucial role in exact identification of the mechanism of fixed left ventricular outflow tract obstruction, when the two-dimensional TEE failed to provide adequate information.