Emma Magavern

Latent Obstruction and Left Atrial Size Are Predictors of Clinical Deterioration Leading to Septal Reduction in Hypertrophic Cardiomyopathy

BACKGROUND Exercise echocardiography is a reliable tool to assess left ventricular (LV) dynamic obstruction in hypertrophic cardiomyopathy (HCM). The aim of this study was to determine the role of exercise echocardiography in the evaluation of latent obstruction and in predicting clinical deterioration in HCM patients. METHODS AND RESULTS We considered 283 HCM patients studied with exercise echocardiography. The end point was clinical deterioration leading to septal reduction (myectomy or alcohol septal ablation). LV latent obstruction was present at enrollment in 67 patients (24%). During a mean follow-up of 42 ± 31 months, 42 patients had clinical deterioration leading to septal reduction therapy: in 12/67 (22%) patients with a latent obstruction at enrollment, in 28/84 (33%) patients with obstruction at rest, and in 2/132 (1.5%) with obstruction neither at rest or during stress. Multivariate analysis identified the following variables as independently associated with the end point: LV gradient >30 mm Hg at rest (hazard ratio [HR] 2.56, 95% CI 1.27-5.14; P = .009), LV gradient >30 mm Hg during stress (HR 4.96, 95% CI 1.81-13.61; P = .002), and indexed left atrial volume (LAVi ) >40 mL/m(2) (HR 2.86, 95% CI 1.47-5.55; P = .002). In patients with a latent obstruction, the strongest independent predictor of outcome was LAVi >40 mL/m(2) (HR 3.75, 95% CI 1.12-12.51; P = .032). CONCLUSIONS Assessment of LV gradient during stress with exercise echocardiography is an important tool for the evaluation of latent obstruction in HCM and may have a role in risk stratification of these patients.

Impact of Septal Reduction on Left Atrial Size and Diastole in Hypertrophic Cardiomyopathy.

Both myectomy and alcohol septal ablation (ASA) can substantially reduce left ventricular (LV) outflow obstruction, relieve symptoms, and improve outcomes in hypertrophic cardiomyopathy (HCM). It is unclear whether septal reduction decreases left atrial (LA) size and improves diastolic function. The aim of this study was to analyze the consequences of septal reduction on LA size and diastolic function in a cohort of patients with HCM.

How does morphology impact on diastolic function in hypertrophic cardiomyopathy? A single centre experience

Objectives It is unclear if morphology impacts on diastole in hypertrophic cardiomyopathy (HCM). We sought to determine the relationship between various parameters of diastolic function and morphology in a large HCM cohort. Setting Tertiary referral centre from Stanford, California, USA. Partecipants 383 patients with HCM and normal systolic function between 1999 and 2011. A group of 100 prospectively recruited age-matched and sex-matched healthy participants were used as controls. Primary and secondary outcome measures Echocardiograms were assessed by two blinded board-certified cardiologists. HCM morphology was classified as described in the literature (reverse, sigmoid, symmetric, apical and undefined). Results Reverse curvature morphology was most commonly observed (218 (57%). Lateral mitral annular E′<12 cm/s was present in 86% of reverse, 88% of sigmoid, 79% of symmetric, 86% of apical and 81% of undefined morphology, p=0.65. E/E′ was similarly elevated (E/E′: 12.3±7.9 in reverse curvature, 12.1±6.1 in sigmoid, 12.7±9.5 in symmetric, 9.4±4.0 in apical, 12.7±7.9 in undefined morphology, p=0.71) and indexed left atrial volume (LAVi)>40 mL/m2 was present in 47% in reverse curvature, 33% in sigmoid, 32% in symmetric, 37% in apical and 32% in undefined, p=0.09. Each morphology showed altered parameters of diastolic function when compared with the control population. Left ventricular (LV) obstruction was independently associated with all three diastolic parameters considered, in particular with LAVi>40 mL/m2 (OR 2.04 (95% CI 1.23 to 3.39), p=0.005), E/E′>15 (OR 4.66 (95% CI 2.51 to 8.64), p<0.001) and E′<8 (OR 2.55 (95% CI 1.42 to 4.53), p=0.001). Other correlates of diastolic dysfunction were age, LV wall thickness and moderate-to-severe mitral regurgitation. Conclusions In HCM, diastolic dysfunction is present to similar degrees independently from the morphological pattern. The main correlates of diastolic dysfunction are LV obstruction, age, degree of hypertrophy and degree of mitral regurgitation.

Making a case for controlled organ donation after cardiac death: the story of Italy's first experience

Donation after circulatory death (DCD) is a valuable option for the procurement of organs for transplantation. In Italy, organ procurement after controlled DCD is legally and ethically conceivable within the current legislative framework. However, although formal impediments do not exist, the health care team is faced with many obstacles that may hinder the implementation of such programs. We report the case of Italys first controlled DCD, specifically discussing the role of the patients family in the shared decision-making process. In our case, the death of the patient subsequent to the withdrawal of life-sustaining therapies was consistent with the patients wishes, showing respect for his dignity and honoring his autonomy, as expressed to his family previously. By making donation possible, the medical team was able to fulfill the familys last request on behalf of the patient. This case should stimulate deliberation regarding the potential to shorten the 20-minute no-touch period currently in place in Italy. Such an action would not have injured this patient and would certainly have increased the quality of the procured organs.

Impact of Demographic Features, Lifestyle, and Comorbidities on the Clinical Expression of Hypertrophic Cardiomyopathy

Felix qui potuit rerum cognoscere causas Happy is he who has been able to learn the causes of things––Virgil, Georgiche, II, 489 Hypertrophic cardiomyopathy (HCM) is a common inherited cardiac disease defined clinically by the presence of unexplained left ventricular (LV) hypertrophy (LVH).

Ethical considerations for genetic testing in the context of mandated cardiac screening before athletic participation

Ethical considerations for genetic testing in the context of mandated cardiac screening before athletic participation