Technetium pyrophosphate nuclear scintigraphy for cardiac amyloidosis: Imaging at 1 vs 3 hours and planar vs SPECT/CT.

BACKGROUND: Multi-societal consensus recommendations endorse both planar and single photon emission tomographic (SPECT) image acquisitions for the evaluation of cardiac amyloidosis. However, the correlation between planar and SPECT findings and the optimal timing of image acquisitions remain uncertain. METHODS: This is an analysis of 109 consecutive patients who underwent technetium pyrophosphate nuclear scintigraphy for the evaluation of cardiac amyloidosis. Patients were imaged at 1 and 3 hours after radiotracer injection using both planar and SPECT/CT, and the correlations between imaging protocols were compared. RESULTS: In the overall cohort (median age 77 years, 75% male), 33 patients had radiotracer localized to the myocardium on SPECT/CT images. There was strong correlation between 1- and 3-hour planar heart-to-contralateral lung ratios (mean difference 0.07, r = 0.94). However, there was discordance between planar image interpretation (based upon semiquantitative score and H/CL ratio) and myocardial localization of radiotracer on SPECT/CT in 17 patients (16%). The pattern of SPECT/CT uptake was identical at 1 and 3 hours in all cases (32 diffuse, 1 focal). CONCLUSION: These data support the recommendation that SPECT/CT should be obtained in addition to planar images when performing nuclear scintigraphy for the detection of cardiac amyloidosis. A 1-hour planar and SPECT/CT protocol appears optimal.

Increasing Rate of Hospital Admissions in Patients With Amyloidosis (from the National Inpatient Sample).

Cardiac amyloidosis is an increasingly recognized cause of heart failure with preserved or mildly reduced ejection fraction with emerging treatment options. We sought to analyze the temporal trends and impact of hospital admissions in patients with amyloidosis. The National Inpatient Sample was queried to identify patients from 2005 to 2014 who were hospitalized with a diagnosis of amyloidosis using ICD9 codes. Trends over time of prevalence, demographics, co-morbidities, and outcomes were described. Propensity-matching was used to assess the impact of amyloidosis on in-hospital outcomes, including mortality. A total of 156,914 admissions in patients with amyloidosis (age 69.86 +/- 12.33 years, 45.7% female, 68.5% Caucasian) were identified. Hospitalizations more than doubled with a peak of 21,740 per year and 62 per 100,000 admissions in 2014. Over time, patients admitted with amyloidosis were older and more likely to have co-morbid medical conditions. A diagnosis of heart failure was present in 34.7% of patients, increased over time (p = 0.001), and was associated with further morbidity and mortality. In a propensity-matched analysis, patients admitted with amyloidosis had a longer length of stay (7.5 vs 6.2 days), were less likely to be discharged home (43.6% vs 48.7%), and were more likely to die during the hospitalization (7.4% vs 4.9%, p <0.001 for all). In conclusion, inpatient hospitalizations in the United States in patients with amyloidosis have increased over time and are associated with high morbidity and mortality, particularly when there is concomitant heart failure.

Stabilization of Early Duchenne Cardiomyopathy With Aldosterone Inhibition: Results of the Multicenter AIDMD Trial.

Background Duchenne muscular dystrophy incurs nearly universal dilated cardiomyopathy by the third decade of life, preceded by myocardial damage and impaired left ventricular strain by cardiac magnetic resonance. It has been shown that (1) mineralocorticoid receptor antagonist therapy with spironolactone attenuated damage while maintaining function when given early in a mouse model and (2) low-dose eplerenone stabilized left ventricular strain in boys with Duchenne muscular dystrophy and evident myocardial damage but preserved ejection fraction. We hypothesized that moderate-dose spironolactone versus eplerenone would provide similar cardioprotection in this first head-to-head randomized trial of available mineralocorticoid receptor antagonists, the AIDMD (Aldosterone Inhibition in Duchenne Muscular Dystrophy) trial. Methods and Results This was a multicenter, double-blind, randomized, noninferiority trial. Subjects were randomized to eplerenone, 50 mg, or spironolactone, 50 mg, orally once daily for 12 months. The primary outcome was change in left ventricular systolic strain at 12 months. Among 52 enrolled male subjects, aged 14 (interquartile range, 12-18) years, spironolactone was noninferior to eplerenone (∆strain, 0.4 [interquartile range, -0.4 to 0.6] versus 0.2 [interquartile range, -0.2 to 0.7]; P=0.542). Renal and pulmonary function remained stable in both groups, and no subjects experienced serious hyperkalemia. Infrequent adverse events included gynecomastia in one subject in the spironolactone arm and facial rash in one subject in the eplerenone arm. Conclusions In boys with Duchenne muscular dystrophy and preserved left ventricular ejection fraction, spironolactone added to background therapy is noninferior to eplerenone in preserving contractile function. These findings support early mineralocorticoid receptor antagonist therapy as effective and safe in a genetic disease with high cardiomyopathy risk. Clinical Trial Registration URL: http://www.clinicaltrials.gov. Unique identifier: NCT02354352.

Treatment of recurrent isolated right atrial metastatic cavitary mass from breast cancer with radiation therapy: A case report and review of literature.

The heart is a destination for metastases of various types of primary tumors, with lung, breast, melanoma, and mesothelioma being the most common types. Recurrent isolated cardiac metastasis presenting as a cavitary mass is rare. We present a case of recurrent isolated cavitary metastatic mass to the right atrium in a patient with the history of breast cancer who presented with new-onset atrial fibrillation. The patient successfully received radiation therapy with the resolution of the mass confirmed on repeat echocardiography.

Left Ventricular Thrombus in the Setting of Normal Left Ventricular Function in Patients with Crohn's Disease.

Crohn's disease results in a hypercoagulable state increasing the risk of venous or arterial thromboembolism. Cardiac involvement has not been routinely identified. Two cases are presented to illustrate that patients with Crohn's disease may represent an exception to the rule that left ventricular apical thrombus should be associated with an underlying wall motion abnormality and reduction in left ventricular ejection fraction.

Assessing cardiac CT--advantages & current limitations. Part II: Clinical utility.

The media's frenzy towards the imaging capabilities of 64-slice cardiac computed tomography (CCT) is not without cause. CCT allows non-invasive visualization of the coronary arteries, coronary artery bypass grafts, and the presence of atherosclerosis. CCT is not limited to the assessment of coronary vasculature, but can yield information about other causes of chest pain such as aortic dissection, or pericardial disease. Other applications for the high resolution scanner include anatomical assessment of congenital heart disease, post myocardial infarction complications, pulmonary embolism and aortic and peripheral arterial atherosclerosis and aneurysm evaluation. Finally, non-contrast CCT allows for quantification of coronary calcification which provides important prognostic information. As CT technology evolves, it is paramount for clinicians to understand when it is clinically appropriate to use CT coronary angiography (CTCA).

Approach to the athlete with thoracic aortic disease.

Sports medicine specialists have a unique opportunity to identify individuals who may be at risk for thoracic aortic disease. These patients may have physical features such as tall stature and long limbs (which typify Marfan syndrome), but also lend themselves to participation in competitive athletics. These individuals are at increased risk of sudden death from aortic dissection. Avoiding or delaying aortic complications involves recognition and screening, medications, and prophylactic surgery when the aorta reaches a size threshold. A key feature in management is recognition of the aortic disease and avoiding those sports or exercises that place excess strain or stress on the aorta. Care involves multiple disciplines including primary care physicians, cardiologists, geneticists, ophthalmologists, and orthopedic and cardiac surgeons; however, the sports medicine specialist is often instrumental in guiding the athlete toward proper diagnosis and safe activities in an effort to avoid catastrophic complications.