Recurrent atrial flutter after radiofrequency ablation for atrial fibrillation in a patient with hypertrophic obstructive cardiomyopathy undergoing repetitive transcoronary ablation of septal hypertrophy.

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.

Resolution of dynamic left ventricular outflow tract obstruction and reversible left ventricular hypertrophy in 4 cats.

This case series describes spontaneous resolution of systolic anterior motion of the mitral valve, cessation of a dynamic left ventricular outflow tract obstruction, and reverse cardiac remodeling in 4 young cats. Following initial presentation with or without congestive heart failure, subsequent rechecks documented resolution of systolic anterior motion of the mitral valve and normalization of left heart dimensions. Those cats originally presented with congestive heart failure were successfully weaned off diuretic medications. Atenolol was prescribed to all 4 cats, and all remained on oral atenolol through the final recheck. There was no documented recurrence of progressive heart disease and heart failure in any of the cats. Consideration is given to transient myocardial thickening, spontaneous resolution of mitral valve dysplasia, and response to beta-1 adrenergic antagonism as possible underlying mechanisms. Key clinical message: When presented with young cats with hypertrophic obstructive cardiomyopathy, veterinarians should consider multiple differential diagnoses, as lifespan in these cases may be longer than typically expected for cats with primary hypertrophic cardiomyopathy, even with concurrent congestive heart failure.

Résolution d'une obstruction dynamique de la voie d'éjection du ventricule gauche et d'une hypertrophie réversible du ventricule gauche chez 4 chatsCette série de cas décrit la résolution spontanée du mouvement antérieur systolique de la valve mitrale, la cessation d'une obstruction dynamique de la voie d'éjection du ventricule gauche et le remodelage cardiaque inverse chez 4 jeunes chats. Après une présentation initiale avec ou sans insuffisance cardiaque congestive, des vérifications ultérieures ont documenté la résolution du mouvement antérieur systolique de la valve mitrale et la normalisation des dimensions du cœur gauche. Les chats initialement présentés avec une insuffisance cardiaque congestive ont été sevrés avec succès des médicaments diurétiques. De l'aténolol a été prescrit aux 4 chats, et tous sont restés sous aténolol oral jusqu'au dernier contrôle. Aucune récidive de maladie cardiaque progressive et d'insuffisance cardiaque n'a été documentée chez aucun des chats. L'épaississement transitoire du myocarde, la résolution spontanée de la dysplasie de la valve mitrale et la réponse à l'antagonisme bêta-1 adrénergique sont pris en compte comme mécanismes sous-jacents possibles.Message clinique clé :Lorsqu'ils sont confrontés à de jeunes chats atteints de cardiomyopathie hypertrophique obstructive, les vétérinaires doivent envisager plusieurs diagnostics différentiels, car la durée de vie dans ces cas peut être plus longue que celle généralement attendue pour les chats atteints de cardiomyopathie hypertrophique primaire, même en cas d'insuffisance cardiaque congestive concomitante.(Traduit par Dr Serge Messier).

Biventricular Hypertrophic Cardiomyopathy in a 26-year-old Nigerian Woman with Noonan Syndrome.

BACKGROUND: Cardiac disorders are found in about half of cases of Noonan syndrome (NS). The most common congenital heart diseases in this syndrome include pulmonary valvular stenosis obstructive or nonobstructive hypertrophic cardiomyopathy (17%). Biventricular hypertrophic cardiomyopathy (HCM) is very rare in this condition. OBJECTIVE: The objective is to report a case of biventricular hypertrophic cardiomyopathy in a 26-year-old Nigerian female with the phenotype. METHODS: This is a descriptive case report. RESULTS: The patient presented with dyspnoea on exertion which started at the age of 7 years and has progressively worsened. There was associated precordial chest pain and palpitation. Clinical examination revealed a young woman, who is small for her age. She had some dysmorphic features such as a webbed neck, lowset ears, low posterior hairline, crowded teeth, high arched palate, a small and asymmetric chin and a high carrying angle at the elbows. The pulses were synchronous and there was no radio-radial or radiofemoral delay and her blood pressures were within normal limits. Cardiac auscultation was unremarkable. The 12-lead ECG showed biventricular hypertrophy with a strain pattern. The echocardiogram showed features in keeping with biventricular hypertrophic cardiomyopathy. CONCLUSION: Biventricular HCM is relatively uncommon in Noonan syndrome. Patients with typical dysmorphia should have a full cardiac evaluation to look for these anomalies.

CONTEXTE: Des troubles cardiaques sont présents chez environ la moitié des patients atteints du syndrome de Noonan. Les cardiopathies congénitales les plus fréquentes dans ce syndrome incluent la sténose valvulaire pulmonaire (60 %), la communication interauriculaire (25 %) et la cardiomyopathie hypertrophique obstructive ou non obstructive (17 %). La cardiomyopathie hypertrophique (HCM) biventriculaire est très rare dans ces conditions. OBJECTIF: L'objectif est de rapporter un cas de cardiomyopathie hypertrophique biventriculaire chez une femme nigériane de 26 ans présentant le phénotype. MÉTHODES: Il s'agit d'un rapport de cas descriptif. RÉSULTATS: La patiente s'est présentée avec une dyspnée à l'effort, débutant à l'âge de 7 ans et s'aggravant progressivement. Il y avait des douleurs précordiales associées ainsi que des palpitations. L'examen clinique a révélé une jeune femme, petite pour son âge, avec quelques traits dysmorphiques tels qu'un cou palmé, des oreilles basses, une implantation basse des cheveux à l'arrière, des dents serrées, un palais ogival, un menton petit et asymétrique et un angle de port élevé au niveau des coudes. Les pouls étaient synchrones, sans retard radio-radial ou radiofémoral, et la tension artérielle était dans les limites normales. L'auscultation cardiaque n'a révélé aucune anomalie. L'électrocardiogramme à 12 dérivations a montré une hypertrophie biventriculaire avec un pattern de strain. L'échocardiogramme a révélé des caractéristiques compatibles avec une cardiomyopathie hypertrophique biventriculaire. CONCLUSION: La cardiomyopathie hypertrophique biventriculaire est relativement rare dans le syndrome de Noonan. Les patients présentant une dysmorphie typique devraient subir une évaluation cardiaque complète afin de détecter ces anomalies. MOTS CLÉS: Syndrome de Noonan, Dysmor phie, Cardiomyopathie hypertrophique.

Distinct Phenotypic Groups and Related Clinical Outcomes in Patients With Hypertrophic Cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a heterogeneous disorder with varying risks of clinical outcomes, including sudden cardiac death (SCD). We aimed to identify distinct phenotypes among patients with HCM in relation to SCD risk factors, interpret their clinical characteristics, and examine their outcomes. METHODS AND RESULTS: This retrospective study analyzed 1231 consecutive patients with HCM from 2 tertiary hospitals. We performed latent class analysis to categorize patients into phenotypic groups. Three distinct phenotypic groups were identified using latent class analysis. Group 1 (n=554) consisted of young patients with HCM with minimal SCD risk factors and favorable cardiac remodeling. Group 2 (n=114) comprised young patients with HCM and a high prevalence of SCD risk factors, whereas Group 3 (n=563) included older patients (median age, 68 years). Over a median 6.5-year follow-up, 34 SCD-related events, 131 cardiovascular events, 133 all-cause mortality events, and 70 noncardiovascular mortality events were observed. Group 2 exhibited the highest rate of SCD-related events (5-year SCD rate: Group 1 versus 2 versus 3: 0.8% versus 8.2% versus 4.0%, respectively, P<0.001), and cardiovascular events were more frequent in Groups 2 and 3 compared with Group 1. All-cause and noncardiovascular mortality were the most frequent in Group 3. A simplified decision tree was developed for the straightforward assignment of phenotypic group membership, demonstrating fair concordance. CONCLUSIONS: This study identified 3 distinct clinical phenotypes in patients with HCM, each associated with different SCD risks and outcomes. Data-driven phenotyping of patients with HCM offers effective risk stratification and may optimize patient management.

Cardiopulmonary Fitness and Personalized Exercise Prescription in Patients With Hypertrophic Cardiomyopathy.

BACKGROUND: Patients with hypertrophic cardiomyopathy (HCM) are generally restricted regarding participation in competitive sports based on the potential risk of sudden cardiac death and malignant arrhythmias. As a result, they are often inactive and experience the negative consequences of a sedentary lifestyle. Hence, the need arises to strike the right balance between these 2 extremes through personalized exercise prescription. The aims of this study were (1) to assess the characteristics of patients with HCM practicing regular aerobic physical activity compared with sedentary patients; (2) to perform a personalized moderate-intensity exercise prescription and evaluate its effects. METHODS AND RESULTS: Patients with HCM were evaluated through clinical assessment, ECG, ambulatory ECG monitoring, echocardiography, and cardiopulmonary testing. A personalized moderate-intensity exercise prescription was performed, and the same investigations were repeated. Physically active patients with HCM demonstrated better cardiopulmonary functional capacity than sedentary patients (oxygen consumptionpeak/kg 32.9±7.4 versus 25.2±7.4 mL/min per kg P≤0.0001, ventilation/carbon dioxide production slope 26.7±4.3 versus 29.9±5.2 P=0.004), with similar prevalence of ventricular arrhythmias (P=0.43). Sedentary subjects showed a borderline higher prevalence of obesity (P=0.07) than physically active subjects. Moderate-intensity exercise prescription led to improved cardiopulmonary fitness without occurrence of adverse events. CONCLUSIONS: Patients with HCM practicing regular aerobic exercise have a better functional capacity in the absence of relevant events than sedentary patients. Conversely, a sedentary lifestyle led to a deterioration of cardiopulmonary functional capacity and fitness. The tailored moderate-intensity personalized exercise prescription appears to be a feasible approach in carefully selected patients with HCM to counterbalance the negative effects of sedentary behavior without significant major events.

Hybrid versus catheter ablation for Hypertrophic CardioMyopathy with Atrial Fibrillation (HCM-AF): study protocol for a randomised controlled trial.

INTRODUCTION: Atrial fibrillation (AF) is an independent predictor of adverse outcomes in patients with hypertrophic cardiomyopathy (HCM). Although catheter ablation is highly recommended for general AF populations, it is less effective in maintaining sinus rhythm in patients with HCM associated with AF. Hybrid ablation, combining a cosmetic approach with a lower rate of AF relapse, lacks comparative studies to verify its efficacy against CA in HCM. This study aims to assess the rhythm control effectiveness of hybrid versus CA in non-obstructive HCM (non-oHCM) patients with AF. METHODS/ANALYSIS: This prospective, multicentre, randomised trial involves a blinded assessment of outcomes in non-oHCM patients with non-paroxysmal AF. Sixty-six candidates from three centres will be randomised 1:1 to either hybrid or CA, including isthmus addressed lesion sets. Participants will be stratified by left atrial (LA) size (LA diameter ≤50 mm or >50 mm). Follow-ups at the 3rd, 6th and 12th months will evaluate the primary endpoint of freedom from documented atrial tachycardia lasting over 30 s within 12 months post-procedure without antiarrhythmic drugs, along with secondary endpoints of all-cause mortality, cardiovascular-related mortality, cerebral stroke, peripheral vascular embolism, heart failure-related rehospitalisation, all-cause rehospitalisation and quality of life assessments. ETHICS AND DISSEMINATIONAPPROVAL: The central ethics committee at Fuwai Hospital has approved the Hypertrophic CardioMyopathy with Atrial Fibrillation trial (approval number: 2022-1736). Results will be disseminated through publications in peer-reviewed journals and presentations at conferences. TRIAL REGISTRATION NUMBER: NCT05610215.

Presence of Apical Aneurysm and Its Impact on Left Ventricular Mechanics and Mechano-Energetic Coupling in Patients With Apical Hypertrophic Cardiomyopathy.

BACKGROUND: Left ventricular (LV) apical aneurysms (ApAn+) occur in 10%-15% of apical hypertrophic cardiomyopathy (ApHCM) patients and confer considerable morbidity. We hypothesized that ApAn+ adversely impact ventricular mechanics and mechano-energetic coupling in ApHCM. METHODS: Ninety-eight ApHCM patients were identified, of which nine (9%) had ApAn+ and were compared with 89 (91%) who did not (ApAn-). 2D speckle-tracking echocardiography assessed ventricular mechanics using LV global longitudinal strain (GLS) and torsion, and mechano-energetic coupling as myocardial work indices. Clinical events over follow-up were adjudicated. RESULTS: Mean age was 64 ± 15 years, 46% were female, and 3% had an HCM family history, with similar clinical risk factors between groups. Of the nine ApAn+ patients, there were six small (<2 cm) and three moderate-sized (2-4 cm) aneurysms. There was no difference in LV ejection fraction (65 ± 15 vs. 67 ± 11%, p = 0.51) or GLS (-9.6 ± 3.3 vs. -11.9 ± 3.9%, p = 0.09) between ApAn+ versus ApAn-. ApAn+ patients had greater myocardial global wasted work (347 ± 112 vs. 221 ± 165 mmHg%, p = 0.03) and lower global work efficiency (GWE, 75 ± 5 vs. 82 ± 8%, p = 0.006). LV GLS (ß = -0.67, p < 0.001), ApAn+ (ß = -0.15, p = 0.04), and twist rate (ß = -0.14, p = 0.04) were independently associated with GWE. At 3.9-year follow-up, cardiovascular mortality (4%) and heart failure hospitalization (14%) events were similar between groups. CONCLUSION: ApHCM patients with ApAn+ are characterized by more impaired LV mechano-energetic coupling when compared with ApAn-. ApAn+ is independently associated with worse GWE.

Clinical Characteristics and Outcomes in Patients With Apical and Nonapical Hypertrophic Cardiomyopathy.

BACKGROUND: Apical hypertrophic cardiomyopathy (ApHCM) is a variant of hypertrophic cardiomyopathy, with distinct clinical characteristics and outcomes. We aimed to clarify the natural history of patients with ApHCM and identify the risk of end-stage heart failure incidence. METHODS AND RESULTS: This retrospective study was conducted on patients with hypertrophic cardiomyopathy in China between January 2009 and February 2024. Patients were stratified into ApHCM and non-ApHCM groups. The primary outcome was a composite of major adverse cardiovascular events, including all-cause deaths, heart failure hospitalization, sudden cardiac death, and ventricular tachycardia. The secondary outcome was the incidence of end-stage heart failure, defined as left ventricular ejection fraction <50%. Kaplan-Meier and univariable and multivariable Cox proportional analyses were applied. Adjustment variables were included for important baseline characteristics, comorbidities, and medication use. Of 5653 patients enrolled with hypertrophic cardiomyopathy, 584 (10.3%) had ApHCM and 5069 (89.7%) had non-ApHCM. During the median follow-up period of 4.6 years (1.6-8.0 years), major adverse cardiovascular events occurred in 32.2% (n=1808), with a lower incidence in patients with ApHCM than non-ApHCM (20.4% versus 33.3%, P<0.001). Non-ApHCM was an independent predictor of major adverse cardiovascular events (hazard ratio [HR], 1.65 [95% CI, 1.36-1.99]; P<0.001). In the serial cohort, patients with ApHCM exhibited a lower incidence of end-stage heart failure than those with non-ApHCM (12.4% versus 2.7%, P<0.001). Non-ApHCM was associated with a higher risk of end-stage heart failure development (HR, 2.31 [95% CI, 1.28-4.15]; P<0.001). In subgroup and sensitivity analysis, the results were consistent for our main and secondary outcomes. CONCLUSIONS: ApHCM is relatively common in hypertrophic cardiomyopathy and shows lower rates of all-cause mortality and heart failure hospitalizations than non-ApHCM.

Return to professional football after ICD implantation in athlete with apical hypertrophic cardiomyopathy.

The risk of sports participation in elite athletes with cardiac disease with an indication for implantable cardioverter-defibrillator (ICD) therapy is largely unknown. Currently, international guidelines provide restrictive sports advice for such athletes. This case report presents a professional football player who after an episode of syncope and diagnosis of apical hypertrophic cardiomyopathy expressed a strong wish to explore the feasibility of returning to elite-level sports. After a shared decision-making and monitored stepwise graduated rehabilitation, the athlete made a full return to professional football. Our case indicates that individualised sports advice in elite athletes with cardiac disease and an ICD may be warranted.

A Cautionary Tale of Hypertrophic Cardiomyopathy-From "Benign" Left Ventricular Hypertrophy to Stroke, Atrial Fibrillation, and Molecular Genetic Diagnostics: A Case Report and Review of Literature.

This case report concerns a 48-year-old man with a history of ischemic stroke at the age of 41 who reported cardiac hypertrophy, registered in his twenties when explained by increased physical activity. Family history was positive for a mother with permanent atrial fibrillation from her mid-thirties. At the age of 44, he had a first episode of persistent atrial fibrillation, accompanied by left atrial thrombosis while on a direct oral anticoagulant. He presented at our clinic at the age of 45 with another episode of persistent atrial fibrillation and decompensated heart failure. Echocardiography revealed a dilated left atrium, reduced left ventricular ejection fraction, and an asymmetric left ventricular hypertrophy. Cardiac magnetic resonance was positive for a cardiomyopathy with diffuse fibrosis, while slow-flow phenomenon was present on coronary angiography. Genetic testing by whole-exome sequencing revealed three variants in the patient, c.309C > A, p.His103Gln in the ACTC1 gene, c.116T > G, p.Leu39Ter in the PLN gene, and c.5827C > T, p.His1943Tyr in the SCN5A gene, the first two associated with hypertrophic cardiomyopathy and the latter possibly with familial atrial fibrillation. This case illustrates the need for advanced diagnostics in unexplained left ventricular hypertrophy, as hypertrophic cardiomyopathy is often overlooked, leading to potentially debilitating health consequences.

Qualitative interview study of patient-reported symptoms, impacts and treatment goals of patients with obstructive hypertrophic cardiomyopathy.

OBJECTIVE: Hypertrophic cardiomyopathy (HCM), including obstructive HCM (oHCM), is the most common inherited cardiomyopathy causing lifestyle-limiting symptoms. Data are lacking about patients' perspectives on the daily impact of their symptoms. This qualitative interview study was conducted to better understand patients' experiences with oHCM. METHODS: In October 2019, telephone interviews were conducted with 20 US adults with oHCM identified by the Hypertrophic Cardiomyopathy Association. Using a semi-structured interview guide, key symptoms, impacts of oHCM and oHCM treatment goals were discussed. RESULTS: Median age was 54 years (range 29-78), 55% were women, 85% were white and 15% were Hispanic or Latino. Median time since diagnosis was 3 years. Symptoms included shortness of breath, dizziness/light-headedness, heart palpitations/fluttering (all 95%), fatigue (90%) and chest pain/pressure (80%). All participants reported limitations in physical functioning/activities; most reported additional impacts (emotional stress (80%), fear of dying (55%)). Shortness of breath and fatigue were among their most bothersome symptoms; an effective oHCM treatment would need to improve ≥1 of these symptoms (allowing increased physical/social activity). CONCLUSIONS: Patients with oHCM experience a high symptom burden and psychosocial impacts, affecting health status. Improved shortness of breath, fatigue and physical functioning are highly valued by patients and represent important treatment goals.

Electric storm triggered by short-coupled premature ventricular complexes in a young patient with non-obstructive hypertrophic cardiomyopathy.

Ventricular arrhythmias are commonly associated with hypertrophic cardiomyopathy with and without midventricular obstruction. Although the overall prognosis is relatively good with an annual mortality rate <1%, the propensity to potentially fatal ventricular arrhythmias (ventricular tachycardia) is the most feared complication. Electrical storms are a severe manifestation of ventricular arrhythmias, with poor outcomes. In this report, we present a case of a young patient with non-obstructive hypertrophic cardiomyopathy who presents after a syncopal episode and is found to have an electric storm that is refractory to medical therapy.

The impact of diabetes mellitus on cardiac function assessed by magnetic resonance imaging in patients with hypertrophic cardiomyopathy.

BACKGROUND: The adverse prognostic impact of diabetes on hypertrophic cardiomyopathy (HCM) is poorly understood. We sought to explore the underlying mechanisms in terms of structural and functional remodelling in HCM patients with coexisting diabetes (HCM-DM). METHODS: A total of 45 HCM-DM patients were retrospectively included. Isolated HCM controls (HCM patients without diabetes) were matched to HCM-DM patients in terms of maximal wall thickness, age, and gender distribution. Left ventricular (LV) and atrial (LA) performance were evaluated using cardiac magnetic resonance feature tracking strain analyses. The associations between diabetes and LV/LA impairment were investigated by univariable and multivariable linear regression. RESULTS: Compared with the isolated HCM controls, the HCM-DM patients had smaller end-diastolic volume and stroke volume, lower ejection fraction, larger mass/volume ratio and impaired strains in all three directions (all P < 0.05). In terms of the LA parameters, HCM-DM patients presented impaired LA reservoir and conduit strain/strain rate (all P < 0.05). Among all HCM patients, comorbidity with diabetes was independently associated with a low LV ejection fraction (ß = - 6.05, P < 0.001) and impaired global longitudinal strain (ß = 1.40, P = 0.007). Moreover, compared with the isolated HCM controls, HCM-DM patients presented with more myocardial fibrosis according to late gadolinium enhancement, which was an independent predictor of impaired LV global radial strain (ß = - 45.81, P = 0.008), LV global circumferential strain (ß = 18.25, P = 0.003), LA reservoir strain (ß = - 59.20, P < 0.001) and strain rate (ß = - 2.90, P = 0.002). CONCLUSIONS: Diabetes has adverse effects on LV and LA function in HCM patients, which may be important contributors to severe manifestations and outcomes in those patients. The present study strengthened the evidence of the prevention and management of diabetes in HCM patients.

Modern Perspectives on Hypertrophic Cardiomyopathy-No One Size Fits All.

Despite substantial advances in the management of hypertrophic cardiomyopathy, advanced heart failure remains a major cause of morbidity in this patient population. This narrative review presents the case of a patient with hypertrophic obstructive cardiomyopathy who underwent alcohol septal ablation to frame a discussion of modern therapies for hypertrophic cardiomyopathy. The current treatment landscape includes medications, both old and new, and surgical and procedural interventions to relieve mechanical obstruction. Several promising new modalities for relieving obstruction are in the nascent stages of development.