Optic Perineuritis Presenting as the Initial Manifestation of Central Nervous System Involvement in Rai Stage 0 Chronic Lymphocytic Leukemia.

BACKGROUND: To describe the first case of optic perineuritis because of meningeal involvement of early stage chronic lymphocytic leukemia (CLL). METHODS: A case report and review of the literature. RESULTS: A case of unilateral optic neuropathy associated with enhancement of the optic nerve sheath is described in a patient with a prior 2-year history of Rai Stage 0 CLL. Lumbar puncture revealed a lymphocytic pleocytosis. Cerebrospinal fluid flow cytometry revealed a monoclonal expansion of CD5+ B cells compatible with CLL, matching the flow cytometry characteristics of his peripheral blood. CONCLUSIONS: Optic perineuritis is often initially diagnosed as optic neuritis, yet the 2 have different etiologies and follow a different clinical course. Orbital MRI with contrast structurally separates the 2, revealing a characteristic pattern of peripheral optic nerve sheath rather than primary optic nerve enhancement. Etiologies of optic perineuritis are varied and include inflammatory, infectious, neoplastic, and toxic entities. Central nervous system (CNS) involvement by chronic lymphocytic leukemia is unusual, but cranial nerve and meningeal involvement have been reported. This case adds central nervous system chronic lymphocytic leukemia to the list of differential diagnostic possibilities for optic perineuritis. It also alerts clinicians to consider optic perineuritis as a potential presenting feature of CNS involvement in otherwise asymptomatic and stable CLL.

Heart failure with recovered ejection fraction: a distinct clinical entity.

BACKGROUND: A subset of patients with heart failure (HF) and preserved left ventricular ejection fraction (EF) previously had EF <40%. We postulated that such "recovered" EF patients would be prevalent in a referral HF population and clinically distinct from those with persistently preserved or reduced EF. METHODS AND RESULTS: We identified all subjects with a clinical diagnosis of HF seen in the advanced heart disease practice at our center from March to October 2008. Patients were classified into 1 of 3 groups based on retrospective review of the medical record: EF persistently ≥40% (HF-PEF), EF recovered to ≥40% (HF-REF) and low EF, <40% (HF-LEF). Clinical and echocardiographic characteristics were compared across groups using standard chi-square and analysis of variance tests. A total of 358 heart failure patients were identified, including 56 with HF-PEF, 121 with HF-REF, and 181 with HF-LEF. Compared with HF-PEF, HF-REF patients were younger with less atrial fibrillation, hypertension, and diabetes. Also, they tended to have lower systolic blood pressure, better renal function, and larger left ventricular diameter at end diastole. HF-REF patients were more similar to HF-LEF, but were younger and had lower rates of coronary artery disease. Of the 3 groups, HF-REF patients had the mildest reported HF symptoms and fewest previous HF hospitalizations. CONCLUSIONS: Patients with HF-REF comprise a substantial proportion of those with HF and EF ≥40% followed in an ambulatory referral practice. These patients appear to be clinically distinct from the residual HF population and should be specifically targeted for further research.

Patient expectations from implantable defibrillators to prevent death in heart failure.

BACKGROUND: Indications for implantable cardioverter-defibrillators (ICDs) in heart failure (HF) are expanding and may include more than 1 million patients. This study examined patient expectations from ICDs for primary prevention of sudden death in HF. METHODS AND RESULTS: Study participants (n = 105) had an EF <35% and symptomatic HF, without history of ventricular tachycardia/fibrillation or syncope. Subjects completed a written survey about perceived ICD benefits, survival expectations, and circumstances under which they might deactivate defibrillation. Mean age was 58, LVEF 21%, 40% were New York Heart Association Class III-IV, and 65% already had a primary prevention ICD. Most patients anticipated more than10 years survival despite symptomatic HF. Nearly 54% expected an ICD to save >or=50 lives per 100 during 5 years. ICD recipients expressed more confidence that the device would save their own lives compared with those without an ICD (P < .001). Despite understanding the ease of deactivation, 70% of ICD recipients indicated they would keep the ICD on even if dying of cancer, 55% even if having daily shocks, and none would inactivate defibrillation even if suffering constant dyspnea at rest. CONCLUSIONS: HF patients anticipate long survival, overestimate survival benefits conferred by ICDs, and express reluctance to deactivate their devices even for end-stage disease.

Thresholds of physical activity and life expectancy for patients considering destination ventricular assist devices.

BACKGROUND: Current implantable left ventricular assist devices (LVAD) improve survival and function for patients with very late stage heart failure (HF) but may also offer benefit before inotrope dependence. Debate continues about selection of HF patients for LVAD therapy. We sought to determine what level of personal risk and disability HF patients thought would warrant LVAD therapy. METHODS: The study included 105 patients with symptomatic HF and an LV ejection fraction (EF) < 35% who were given a written paragraph about LVADs and asked about circumstances under which they would consider such a device. New York Heart Association (NYHA) functional class, time trade-off utility, and patient-assessed functional score were determined. RESULTS: Participants (mean age, 58 years) had an LVEF of 21%. The median duration of HF was 5 years, and 65% had a primary prevention implantable cardioverter defibrillator. Presented with a scenario of bed-ridden HF, 81% stated they would definitely or probably want an LVAD; 50% would consider LVAD to prolong survival if HF survival were predicted to be < 1 year and 75% if < 6 months. Meanwhile, 44% would consider LVAD if they could only walk < 1 block and 64% if they could not dress without stopping. Anticipated thresholds did not differ by NYHA class, time trade-off, or functional score. CONCLUSIONS: Patient thresholds for LVAD insertion parallel objective survival and functional data. HF patients would be receptive to referral for discussion of LVAD by the time expected mortality is within 6 to 12 months and activity remains limited to less than 1 block.