Apical Hypertrophic Cardiomyopathy: Preliminary Attempt at Palliation with Use of Subselective Alcohol Ablation

We report a case of severe apical hypertrophic cardiomyopathy in order to discuss the nature of this unusual condition and the possibility of using selective alcohol ablation to effectively treat symptomatic hypertrophic cardiomyopathy that presents with apical aneurysm. A 73-year-old woman with severe, progressive dyspnea and intermittent chest pain was found to have localized left ventricular apical dyskinesia distal to an obstructive mid-distal muscular ring. The ring caused total systolic obliteration of the apical left ventricular cavity. Apical cavity pressure was extremely high, up to 330 mmHg—200 mmHg above that in the main left ventricular cavity. Because of the danger of apical rupture and clot formation, we attempted the experimental use of alcohol ablation for effective palliation. We present our pilot experience, offer a novel interpretation of the nature of this obscure entity, and possibly justify a new catheter treatment. In addition, we discuss the developmental, pathophysiologic, and clinical implications of this unusual form of hypertrophic cardiomyopathy. To our knowledge, ours is the first reported use of subselective, modified-protocol alcohol septal ablation to treat an obstructive mid-apical muscular ring in a patient with apical hypertrophic cardiomyopathy.Key words: Cardiomyopathy, hypertrophic/complications/epidemiology/physiopathology/therapy; ethanol/administration & dosage/therapeutic use; heart septum/pathology; hypertrophy, left ventricular/diagnosis; myocardial ischemia/complications; treatment outcomeHypertrophic obstructive cardiomyopathy (HOCM) is a congenital heart condition characterized by ventricular hypertrophy and a progressive worsening of symptoms, as related to the worsening of the subaortic gradient.^1,2 Either traditional surgical septal myomectomy^3,4 or alcohol septal ablation (ASA)^2,4,5 can be used to treat HOCM by effectively reducing the subaortic gradient.^1,2 We describe the case of an elderly woman who had the rarer variant of hypertrophic cardiomyopathy (HCM)—that with apical aneurysm and mid-ventricular obstruction. We describe in detail the experimental use of subselective alcohol ablation to treat the obstructive mid-apical muscular ring in this patient, and we revisit the nature of apical HCM.     

Hematologic responses to deferasirox therapy in transfusion-dependent patients with myelodysplastic syndromes

Background

Reductions in transfusion requirements/improvements in hematologic parameters have been associated with iron chelation therapy in transfusion-dependent patients, including those with myelodysplastic syndromes; data on there reductions/improvements have been limited to case reports and small studies.

Design and Methods

To explore this observation in a large population of patients, we report a post-hoc analysis evaluating hematologic response to deferasirox in a cohort of iron-overloaded patients with myelodysplastic syndromes enrolled in the Evaluation of Patients’ Iron Chelation with Exjade^® (EPIC) study using International Working Group 2006 criteria.

Results

Two-hundred and forty-seven, 100 and 50 patients without concomitant medication for myelodysplastic syndromes were eligible for analysis of erythroid, platelet and neutrophil responses, respectively. Erythroid, platelet and neutrophil responses were observed in 21.5% (53/247), 13.0% (13/100) and 22.0% (11/50) of the patients after a median of 109, 169 and 226 days, respectively. Median serum ferritin reductions were greater in hematologic responders compared with non-responders at end of study, although these differences were not statistically significant. A reduction in labile plasma iron to less than 0.4 μmol/L was observed from week 12 onwards; this change did not differ between hematologic responders and non-responders.

Conclusions

This analysis suggests that deferasirox treatment for up to 1 year could lead to improvement in hematologic parameters in some patients with myelodysplastic syndromes.