Cardiac amyloidosis screening using a relative apical sparing pattern in patients with left ventricular hypertrophy.

BACKGROUND: Cardiac amyloidosis (CA) mimics left ventricular hypertrophy (LVH). It is treatable, but its prognosis is poor. A simple screening tool for CA would be valuable. CA is more precisely diagnosed with echocardiographic deformation parameters (e.g., relative apical sparing pattern [RASP]) than with conventional parameters. We aimed to 1) investigate incremental benefits of echocardiographic deformation parameters over established parameters for CA screening; 2) determine the resultant risk score for CA screening; and 3) externally validate the score in LVH patients. METHODS: We retrospectively studied 295 consecutive non-ischemic LVH patients who underwent detailed diagnostic tests. CA was diagnosed with biopsy or 99mTc-PYP scintigraphy. The base model comprised age (≥65 years [men], ≥70 years [women]), low voltage on the electrocardiogram, and posterior wall thickness ≥ 14 mm in reference to the literature. The incremental benefit of each binarized echocardiographic parameter over the base model was assessed using receiver operating characteristic curve analysis and comparisons of the area under the curve (AUC). RESULTS: Fifty-four (18%) patients had CA. RASP showed the most incremental benefit for CA screening over the base model. After conducting multiple logistic regression analysis for CA screening using four variables (RASP and base model components), a score was determined (range, 0-4 points). The score demonstrated adequate discrimination ability for CA (AUC = 0.86). This result was confirmed in another validation cohort (178 patients, AUC = 0.88). CONCLUSIONS: We developed a score incorporating RASP for CA screening. This score is potentially useful in the risk stratification and management of LVH patients.

Semiquantitative assessment of the relative apical sparing pattern of longitudinal strain for cardiac amyloidosis identification.

BACKGROUNDS: The relative apical sparing pattern (RASP) of left ventricular (LV) longitudinal strain (LS) is frequently associated with cardiac amyloidosis (CA). However, the visual assessment of RASP is inconsistent, and the quantitative assessment of RASP is time-consuming. This study aimed to compare assessments of RASP for the identification of CA with conventional assessments and investigate their reproducibility and relevance on the assessments. METHODS: Forty patients with biopsy-proven CA were compared with 80 hypertrophied patients matched for mean LV wall thickness. We compared the discriminative abilities of three assessments of RASP to identify CA (visual, quantitative, and semiquantitative). Nine patterns of semiquantitative RASP were investigated; finally, it was defined as "reduction of LS" (≥ -10%) in ≥5 (of 6) basal segments, relative to "preserved LS" (< -15%) in at least one apical segment. RESULTS: The concordance between the two observers for visual RASP was modest (κ = 0.65). On the other hand, the consistency for semiquantitative RASP was perfect (κ = 1.00). The discriminative ability of semiquantitative RASP (area under the curve [AUC]  = 0.74) was significantly better than that of visual RASP (AUC = 0.65) and equivalent to that of binary quantitative RASP. CONCLUSION: Semiquantitative RASP assessment is reproducible and accurately discriminates CA. This simple assessment may help readily refine the risk stratification of patients with diffuse LV hypertrophy.

Methylephedrine-induced heart failure in a habitual user of paediatric cough syrup: a case report.

BACKGROUND: For relief of cold symptoms, methylephedrine is considered to be safer than ephedrine, particularly when used at the predetermined dose. It is often present in various over-the-counter (OTC) drugs for cold, including paediatric cough syrups. CASE SUMMARY: A 52-year-old man presented with worsening dyspnoea and anorexia for 2 weeks. He was a night shift worker and had been habitually taking large doses of methylephedrine-containing paediatric cough syrup for 20 years for sleep averting. On admission, his chest X-ray revealed pulmonary congestion and electrocardiogram showed sinus tachycardia with left-axis deviation. Echocardiography revealed diffuse hypokinesis with a reduced ejection fraction (EF) of 25%. The B-type natriuretic peptide level was elevated to 1092 ng/L. Even after treatment with low-dose dobutamine and furosemide in intensive care unit, right-heart catheterization demonstrated a 'wet and cold' profile. Coronary angiography revealed normal coronary arteries. Pathological examination by endomyocardial biopsy revealed cardiomyocyte hypertrophy with moderate interstitial and replacement fibrosis. In addition, cardiac magnetic resonance imaging revealed diffuse hypokinesis with mid-wall late gadolinium enhancement, which suggested fibrosis. Discontinuation of the cough syrup and optimal medical treatment with an angiotensin-converting enzyme inhibitor and a ß blocker resulted in improvement in the heart failure symptoms to New York Class Association Class II. The EF also improved to 50% at 4 months after discharge. DISCUSSION: Methylephedrine is considered to have adrenergic effects; it has milder side effects on the cardiovascular system than ephedrine. However, the long-lasting excessive intake of methylephedrine, even through OTC paediatric cough syrups, has the potential to cause heart failure.

Clinical Utility of Diffusion-weighted Whole-body Magnetic Resonance Imaging with Background Body Signal Suppression for Assessing and Monitoring IgG4-related Disease.

A 77-year-old man with symptoms of chest pain was diagnosed with immunoglobulin G4 (IgG4)-related disease. Fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) revealed an intense uptake in the submandibular gland, lymph nodes and abdominal aortic wall. Diffusion-weighted imaging with background body signal suppression (DWIBS) revealed signal enhancements at the same location as those of the FDG-PET/CT findings. The DWIBS signal intensity decreased after steroid treatment, so we decreased the steroid dosage. Relapse did not occur. DWIBS makes it possible to adjust the medicine dosage while confirming the therapeutic effects and will likely be a useful method for monitoring IgG4-related disease.