Therapy-related myelodysplastic syndrome presenting as fulminant heart failure secondary to myeloid sarcoma.

Rapidly progressive heart failure is commonly caused by an extensive myocardial infarction, a mechanical complication of infarction, myocarditis, or acute valvular insufficiency. We present an unusual case that was caused by a diffuse infiltration of the myocardium with leukemic cells (myeloid sarcoma). The patient presented with episodic shortness of breath, he was anemic and thrombocytopenic, and his bone marrow biopsy revealed myelodysplastic syndrome from treatment for oligodendroglioma. His clinical course was characterized by a chronic leak of cardiac enzymes, a new right bundle branch block, and a large pericardial effusion causing tamponade and death from fulminant heart failure and ventricular arrhythmias within 2 weeks. At autopsy, the heart was massively infiltrated with myeloblasts and other immature myeloid cells. There was no evidence of acute leukemia in the bone marrow or peripheral blood. Cardiac infiltration in a patient with myelodysplastic syndrome is extremely rare, especially in the absence of bone marrow involvement by blasts. The recognition of this entity is becoming increasingly important as the incidence of cardiac myeloid sarcoma may be on the rise as the number of patients receiving chemotherapy increases.

Multiple myeloma presenting with high-output heart failure and improving with anti-angiogenesis therapy: two case reports and a review of the literature.

INTRODUCTION: Common manifestations of multiple myeloma include osteolytic lesions, cytopenias, hypercalcemia, and renal insufficiency. Patients may also exhibit heart failure which is often associated with either past therapy or cardiac amyloidosis. A less recognized mechanism is high-output heart failure. Diuretic therapy in this setting has little efficacy in treating the congested state. Furthermore, effective pharmacotherapy has not been established. We report two patients with multiple myeloma and high-output heart failure who failed diuretic therapy. The patients were given dexamethasone in conjunction with lenalidomide and thalidomide, respectively. Shortly thereafter, each patient demonstrated a significant improvement in symptoms. This is the first report of successful treatment of multiple myeloma-induced high-output failure via the utilization of these agents. CASE PRESENTATION: Two patients with multiple myeloma were evaluated for volume overload. The first was a 50-year-old man with refractory disease. Magnetic resonance imaging demonstrated diffuse marrow replacement throughout the pelvis. Cardiac catheterization conveyed elevated filling pressures and a cardiac output of 15 liters/minute. He quickly decompensated and required mechanical ventilation. The second patient was a 61-year-old man recently diagnosed with multiple myeloma and volume overload. Skeletal survey demonstrated numerous lytic lesions throughout the pelvis. His cardiac catheterization also conveyed elevated filling pressures and a cardiac output of 10 liters/minute. Neither patient responded to diuretic therapy and they were subsequently started on dexamethasone plus lenalidomide and thalidomide, respectively. The first patient's brisk diuresis allowed for extubation within 48 hours after the first dose. He had a net negative fluid balance of 15 liters over 10 days. The second patient also quickly diuresed and on repeat cardiac catheterization, his cardiac output had normalized to 4.7 liters/minute. CONCLUSION: Multiple myeloma can cause high-output failure. The mechanism is likely extensive bony involvement causing innumerable intramedullary arteriovenous fistulas. Diuretic therapy is not effective in treating this condition. Lenalidomide and thalidomide, both of which inhibit angiogenesis, seem to be viable treatment options. Based on the rapid and effective results seen in these two patients, a potential novel mechanism of 'pharmacologic fistula ligation' with these agents may be the most effective way to treat this presentation.

Accelerating restrictive cardiomyopathy after liver transplantation in a patient with familial amyloidotic polyneuropathy: a case report.

INTRODUCTION: Hereditary amyloidodis is a rare disease process with a propensity to cause polyneuropathies, autonomic dysfunction, and restrictive cardiomyopathy. It is transmitted in an autosomal dominant manner, with disease onset usually in the 20s-40s. The most common hereditary amyloidogenic protein, transthyretin, is synthesized in the liver and lies on Chromosome 18. Over 80 amyloidogenic transthyretin mutations have been described, the majority of which are neuropathic and hence the common name, Familial Amyloidotic Polyneuropathy. Until 1990, the disease was intractable with a 5-15 year survival after diagnosis. The prognosis changed after the implementation of orthotropic liver transplantation as a treatment strategy which halts the synthesis of amyloidogenic transthyretin. This has now has been performed over 1300 times in 67 centers. CASE PRESENTATION: We describe the case of a man of Irish ancestry with Familial Amyloidotic Polyneuropathy and no clinical history of cardiac involvement. Shortly after orthotropic liver transplantation, he developed congestive heart failure. He was subsequently diagnosed with an accelerating post-transplant restrictive cardiomyopathy due to amyloid infiltration. CONCLUSION: A liver transplant induced cardiomyopathy in Familial Amyloidotic Polyneuropathy can be observed in patients without any history of cardiac symptoms. All patients with Familial Amyloidotic Polyneuropathy should be followed after transplantation to assess for a deterioration in cardiac function.

Are the American College of Cardiology/Emergency Cardiac Care (ACC/ECC) guidelines useful in triaging patients to telemetry units?

PURPOSE: To determine if the ACC/ECC guidelines (1991) properly stratify patients according to risk of arrhythmia, defined as a single event on cardiac monitoring, and benefit, defined as a subsequent management change from a recorded telemetry event. SUBJECTS AND METHODS: In 2003, a prospective study of 217 consecutive patients admitted to a 24-bed telemetry unit was conducted for 25 days at a major academic hospital. Patients were categorized per ACC/ECC guidelines as appropriate (class I & II) or inappropriate (class III) based on a non-cardiologist admission diagnosis. A cardiologist-led group then reclassified patients at the time of admission using a brief interview. Continuous telemetry-recorded arrhythmias and resultant management changes were reviewed and recorded daily. Subgroup analysis of patients admitted with a chief complaint of chest pain was also performed. In 2004, after this trial was performed, the American Heart Association released a scientific statement updating practice standards for ECG monitor; however, this paper is based upon the original 1991 ACC/ECC guidelines. RESULTS: Reclassification significantly decreased the percentage of all class I & II patients from 91% to 71% (P<0.001) and the percentage of class I & II patients with chest pain from 100% to 58% (P<0.001) without increasing the percentage of arrhythmias occurring in class III patients. Class II patients had a statistically significant higher percentage of arrhythmias than class I and III patients before and after reclassification (P<0.001 and P<0.001, respectively). Management changes occurring as a direct result of telemetry events were higher in class II than class I or III patients before and after reclassification (P = 0.01 and P = 0.03). Life-threatening arrhythmias (sustained ventricular tachycardia or ventricular fibrillation) occurred in 1% of the 216 patients enrolled in this study. CONCLUSIONS: (1) Cardiology input using ACC/ECC guidelines and a brief interview at admission safely reduced total admissions primarily by identifying low risk chest pain admissions inappropriate for inpatient telemetry monitoring. (2) Life threatening arrhythmias occurring in patients admitted to telemetry are rare.

Renal dialysis as a risk factor for appropriate therapies and mortality in implantable cardioverter-defibrillator recipients.

BACKGROUND: Patients with end-stage renal disease are at increased risk for sudden cardiac death, although the utility of implantable cardioverter-defibrillators (ICDs) in these patients is unknown. OBJECTIVES: The purpose of this study was to evaluate whether end-stage renal disease is an independent risk factor for appropriate ICD therapy for ventricular tachycardia (VT) or ventricular fibrillation (VF) and to compare the long-term survival of ICD recipients with and without end-stage renal disease. METHODS: A retrospective cohort study was performed on ICD recipients at a single center. The primary endpoint was first appropriate ICD therapy for VT/VF. The secondary endpoint was survival. RESULTS: The study included 585 patients, 19 (3.2%) of whom had end-stage renal disease prior to device implantation. Average follow-up time was 2.2 +/- 2.4 years, during which time 156 patients (26.7%) received appropriate ICD therapy. End-stage renal disease was strongly associated with appropriate ICD therapy (hazard ratio 2.30, 95% confidence interval 1.17-4.54) and remained a significant predictor following adjustment for implant indication, ejection fraction, diabetes, hypertension, and beta-blocker use. Survival was significantly shorter in the end-stage renal disease patients, with a median survival time of 3.2 +/- 0.6 (SEM) years in the dialysis cohort and 7.4 +/- 0.5 (SEM) years in those without end-stage renal disease (log rank P = .009). The majority of deaths in the end-stage renal disease cohort were due to non-device-related infection. CONCLUSION: In this cohort, end-stage renal disease was the single greatest predictor of ICD therapies for VT/VF. The survival rate was significantly shorter than that of ICD recipients without end-stage renal disease, suggesting that comorbidities in end-stage renal disease patients meeting current implant indications may reduce the survival benefit of ICD placement in this population.

Effect of contrast enhancement on measurement of carotid artery intimal medial thickness.

Previous studies have used standard B-mode ultrasound to quantify the aggregate mean intimal medial thickness (IMT) of the near and far wall of the common carotid artery (CCA). Many investigators have had difficulty in accurately evaluating the near wall IMT secondary to difficulty in discerning the vessel lumen and intima. The purpose of this study is to determine the effect of contrast enhanced ultrasound on IMT measurement when compared with non-enhanced images. Twenty-six patients who had standard carotid ultrasounds completed over a 6-month period were evaluated, with 24 imaged by the same sonographer. Five to six measurements of the near and far walls were obtained over a 1 cm distance, beginning and ending 0.5 cm and 1.5 cm proximal to the carotid bifurcation. The measurements were made with and without the contrast agent Optison (perflutren protein type-A microspheres), which was given as an i.v. bolus (0.5-0.7 cc). Of those imaged by the same sonographer, 40 carotid arteries were examined and a total of 867 measurements were obtained. A total of 10% of the carotid ultrasounds were restudied approximately 1 month after the initial interpretation to assess observer accuracy. The near wall CCA mean (SD) IMT was 0.075 (0.019) cm for left with contrast versus 0.067 (0.023) cm for left without contrast and 0.089 (0.024) cm for right with versus 0.071 (0.022) cm for right without, p < or = 0.0001 both sides. For the far wall of the CCA, the mean (SD) IMT comparison was 0.075 (0.021) cm for left with versus 0.070 (0.016) cm for left without, p = 0.005, and 0.070 (0.023) cm for right with versus 0.070 (0.016) cm for right without, p = 0.68. In conclusion, contrast-enhanced IMT measurement showed a highly statistically significant difference in near carotid wall thickness determinations versus non-contrast values. The thicker measurement is in agreement with previously reported data showing that non-contrast images underestimated near wall common carotid IMT in histologic samples.