Background Recently there has been increased interest in a possible association between mast cell activation (MCA) disorder and postural orthostatic tachycardia syndrome (POTS). This study examined the frequency with which symptoms and laboratory findings suggesting MCA disorder occurred in patients diagnosed with POTS. Methods and Results Data were obtained from patients in whom symptoms and orthostatic testing were consistent with a POTS diagnosis. Individuals with <4 months symptom duration, evident ongoing inflammatory disease, suspected volume depletion, or declined consent were excluded. All patients had typical POTS symptoms; some, however, had additional nonorthostatic complaints not usually associated with POTS. The latter patients underwent additional testing for known MCA biochemical mediators including prostaglandins, histamine, methylhistamine, and plasma tryptase. The study comprised 69 patients who met POTS diagnostic criteria. In 44 patients (44/69, 64%) additional nonorthostatic symptoms included migraine, allergic complaints, skin rash, or gastrointestinal symptoms. Of these 44 patients, 29 (66%) exhibited at least 1 laboratory abnormality suggesting MCA disorder, and 11/29 patients had 2 or more such abnormalities. Elevated prostaglandins (n=16) or plasma histamine markers (n=23) were the most frequent findings. Thus, 42% (29/69) of patients initially diagnosed with POTS exhibited both additional symptoms and at least 1 elevated biochemical marker suggesting MCA disorder. Conclusions Laboratory findings suggesting MCA disorder were relatively common in patients diagnosed with POTS and who present with additional nonorthostatic gastrointestinal, cutaneous, and allergic symptoms. While solitary abnormal laboratory findings are not definitive, they favor MCA disorder being considered in such cases.
Amyloid infiltration of the atrium is described in patients with valvular heart disease and is associated with an increased risk for atrial fibrillation(AF) while amyloid deposits in the ventricles is increasingly being diagnosed in patients with HFpEF. The role of amyloid deposits in patients with AF without valvular heart disease, which represents the most common form of AF globally, is undefined. In this study, we sought to assess the prevalence of sub-clinical isolated cardiac amyloidosis (ICA) at autopsy and the odds of AF in these patients. A total of 1083 patients were included in the study and 3.1% of patients were found to have asymptomatic ICA. Patients with ICA were older and had a higher odds of AF independent of age and CHA2DS2VASc score. Amongst patients with AF, those with ICA were more likely to have persistent forms of AF and had a lower sinus rhythm P-wave amplitude. Further studies are required to further define this entity, identify imaging modalities to aid in antemortem diagnosis of ICA and to establish the optimal management strategies in these patients.
Objective Delayed presentation after ST-elevation myocardial infarction (STEMI) and complicated by cardiogenic shock (CS-STEMI) is commonly encountered in developing countries and is a challenging scenario because of a delay in revascularization resulting in infarction of a large amount of the myocardium. We aimed to assess the clinical characteristics, angiographic profile, and predictors of outcome in patients with a delayed presentation after CS-STEMI. Methods A total of 147 patients with CS-STEMI with time to appropriate medical care ≥12 h after symptom onset were prospectively recruited at a tertiary referral center. Results The median time to appropriate care was 24 h (interquartile range 18–48 h). The mean age was 58.7 ± 11.1 years. Left ventricular pump failure was the leading cause of shock (67.3%), whereas mechanical complications accounted for 14.9% and right ventricular infarction for 13.6% of cases. The overall in-hospital mortality was 42.9%. Acute kidney injury [Odds ratio (OR) 8.04; 95% confidence intervals (CI) 3.08–20.92], ventricular tachycardia (OR 7.04; CI 2.09–23.63), mechanical complications (OR 6.46; CI 1.80–23.13), and anterior infarction (OR 3.18; CI 1.01–9.97) were independently associated with an increased risk of mortality. Coronary angiogram (56.5%) revealed single-vessel disease (45.8%) as the most common finding. Percutaneous coronary intervention was performed in 53 patients (36%), at a median of 36 h (interquartile range 30–72) after symptom onset. Conclusion Patients with a delayed presentation after CS-STEMI were younger and more likely to have single-vessel disease. We found a high in-hospital mortality of 42.9%. Appropriate randomized studies are required to evaluate the optimal treatment strategies in these patients.
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