Prediction of incident atrial fibrillation in hypertrophic cardiomyopathy.

BACKGROUND AND AIM: Atrial fibrillation (AF) is the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM) with significant effects on outcome. We aim to compare the left atrial (LA) diameter measurement with HCM-AF Score in predicting atrial fibrillation (AF) development in HCM. METHODS: From the regional cohort of the Campania Region, Italy, 519 HCM patients (38% women, age45 ± 17 years) without history of AF, were enrolled in the study. The primary clinical endpoint was the development of AF, defined as at least 1 episode documented by ECG. RESULTS: During the follow-up (mean 8 ± 6, IQ range 2.5-11.2 years), 99 patients (19%) developed AF. Patients who developed AF were more symptomatic, had higher prevalence of ICD implantation, had larger LA diameter, greater left ventricular (LV) maximal wall thickness and LV outflow tract obstruction (p < 0.01). Both LA diameter and HCM-AF score were higher in patients who developed AF versus those who did not (LA diameter 49 ± 7 versus 43 ± 6 mm; HCM-AF score 22 ± 4 versus 19 ± 4; p < 0.0001); however, ROC curve analysis demonstrated that LA diameter had a significant greater area under the curve than HCM-AF Score (p < 0.0001). At 5 years follow-up, a LA diameter > 46 mm, showed a similar accuracy in predicting AF development of HCM-AF score ≥ 22, which identifies patients at high risk to develop AF. CONCLUSION: Our analysis shows that LA diameter, a worldwide and simple echocardiographic measure, is capable alone to predict AF development in HCM patients.

Mechano-energetic efficiency in patients with hypertrophic cardiomyopathy with and without sarcomeric mutations.

Hypertrophic cardiomyopathy (HCM) is mainly caused by sarcomeric mutations which may affect myocardial mechano-energetic efficiency (MEE). We investigated the effects of sarcomeric mutations on MEE. A non-invasive pressure/volume (P/V) analysis was performed. We included 49 genetically screened HCM patients. MEEi was calculated as the ratio between stroke volume and heart rate normalized by LV mass. Fifty-seven percent (57%) HCM patients carried a sarcomeric mutation. Patients with and without sarcomeric mutations had similar LV ejection fraction, heart rate, LV mass, and LV outflow gradient. Younger age at diagnosis, family history of HCM, and lower MEEi were associated with presence of sarcomeric mutation (p = 0.017; p = 0.001 and p = 0.0001, respectively). Lower MEEi in HCM with sarcomeric mutation is not related to significant differences on filling pressure as shown on P/V analysis. Sarcomeric mutations determine a reduction of the LV pump performance as estimated by MEEi in HCM. Lower MEEi may predict a positive genetic analysis.

Pitfalls and Tips in the Assessment of Aortic Stenosis by Transthoracic Echocardiography.

Aortic stenosis (AS) is a valvular heart disease that significantly contributes to cardiovascular morbidity and mortality worldwide. The condition is characterized by calcification and thickening of the aortic valve leaflets, resulting in a narrowed orifice and increased pressure gradient across the valve. AS typically progresses from a subclinical phase known as aortic sclerosis, where valve calcification occurs without a transvalvular gradient, to a more advanced stage marked by a triad of symptoms: heart failure, syncope, and angina. Echocardiography plays a crucial role in the diagnosis and evaluation of AS, serving as the primary non-invasive imaging modality. However, to minimize misdiagnoses, it is crucial to adhere to a standardized protocol for acquiring echocardiographic images. This is because, despite continuous advances in echocardiographic technology, diagnostic errors still occur during the evaluation of AS, particularly in classifying its severity and hemodynamic characteristics. This review focuses on providing guidance for the imager during the echocardiographic assessment of AS. Firstly, the review will report on how the echo machine should be set to improve image quality and reduce noise and artifacts. Thereafter, the review will report specific emphasis on accurate measurements of left ventricular outflow tract diameter, aortic valve morphology and movement, as well as aortic and left ventricular outflow tract velocities. By considering these key factors, clinicians can ensure consistency and accuracy in the evaluation of AS using echocardiography.

Diagnosis and Clinical Implication of Left Ventricular Aneurysm in Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a genetic disease with heterogeneous clinical presentation and prognosis. Within the broad phenotypic expression of HCM, there is a subgroup of patients with a left ventricular (LV) apical aneurysm, which has an estimated prevalence between 2% and 5%. LV apical aneurysm is characterized by an area of apical dyskinesis or akinesis, often associated with regional scarring. To date, the most accepted pathomechanism of this complication is, in absence of coronary artery disease, the high systolic intra-aneurysmal pressure, which, combined with impaired diastolic perfusion from lower stroke volume, results in supply-demand ischemia and myocardial injury. Apical aneurysm is increasingly recognized as a poor prognostic marker; however, the efficacy of prophylactic anticoagulation and/or intracardiac cardioverted defibrillator (ICD) in improving morbidity and mortality is not yet clearly demonstrated. This review aims to elucidate the mechanism, diagnosis and clinical implication of LV aneurysm in patients with HCM.

Rapid Evolution of an Aortic Endocarditis.

Cardiac surgery is necessary in almost 50% of patients with endocarditis. Early surgery, i.e., the surgery performed during the first hospitalization, is required in the following cases: heart failure secondary to valve regurgitation; S. aureus, fungal organism, or other highly resistant organism infection; heart block, annular or aortic abscess, or destructive penetrating lesions; evidence of persistent infection as manifested by persistent bacteremia or fevers lasting >5 days after onset of appropriate antimicrobial therapy. A 62-year-old man developed a fever (38 °C) 3 days after a transaortic electrophysiological study; blood cultures were positive for S. aureus, and were sensitive to vancomycin and ceftaroline. Antibiotic therapy was started, controlling the fever and the patient's infective and inflammatory profiles well; however, 3 days later, acute aortic regurgitation developed. At transesophageal echocardiography (TEE), a rare condition was revealed-vegetation was attached to the aortic wall, impeding correct aortic valve closure. Cardiac operation was carried out and the time for surgery was discussed; based on the patient's clinically stable condition, and on the infection, which was controlled well by antibiotics therapy, surgery was not performed in emergency circumstance (within 24-48 h)-rather, it was programmed during the hospitalization. A TEE surveillance was initiated, and after 7 days, TEE revealed a new picture, with images of an aortic abscess with small perforation in the right atrium, requiring emergency surgery, carried out 20 h later. In our case, the rapid evolution of the vegetation attached to the aortic wall suggested the following: (1) that the time for the surgery cannot be guided only by clinical procedure but must also be guided by imaging pictures; (2) that strictly TEE surveillance is mandatory in patients with aortic endocarditis not initially referred for emergency surgery.