Abstract Background Wild-type transthyretin cardiac amyloidosis (ATTRwt) is increasingly recognized as an important cause of heart failure. Objectives The purpose of this study was to determine the ...natural history of ATTRwt and the predictors of survival. Methods We retrospectively reviewed patients diagnosed with ATTRwt at the Mayo Clinic through 2013 and recorded clinical data and survival data. Factors affecting overall survival (OS) were identified, and a prognostic staging system was developed. Results The median age of the 360 patients diagnosed before death was 75 years (range: 47 to 94 years), and 91% were male. Presenting signs and symptoms included dyspnea or heart failure in 67% and atrial arrhythmias in 62%. Median OS from diagnosis was 3.6 years and did not change over time. Multivariate predictors of mortality included age, ejection fraction, pericardial effusion, N-terminal pro–B-type natriuretic peptide, and troponin T. A staging system was developed that used thresholds of troponin T (0.05 ng/ml) and N-terminal pro–B-type natriuretic peptide (3,000 pg/ml). The respective 4-year OS estimates were 57%, 42%, and 18% for stage I (both values below cutoff), stage II (one above), and stage III (both above), respectively. Stage III patients were at an increased risk of mortality after adjustment for age and sex compared with stage I patients (hazard ratio: 3.6; p < 0.001). Conclusions The natural history of ATTRwt is poor. We report a novel cardiac biomarker staging system that enables risk stratification in an era of emerging treatment strategies.
Abstract Background Papillary fibroelastomas (PFE) are benign neoplasms with little available outcome data. Objectives This study sought to describe the frequency and clinical course of patients with ...surgically removed PFE and echocardiographically suspected, but unoperated, PFE. Methods Mayo Clinic pathology and echocardiography databases (January 1, 1995, to December 31, 2010) were queried, resulting in 511 patients: group 1 (n = 185), including patients with surgically removed, histopathologically confirmed PFE; group 1a (n = 94; 51%) with PFE removed at primary surgery; and group 1b (n = 91; 49%) with PFE removal at time of another cardiac surgery. Group 2 (n = 326) patients had echocardiographic evidence of PFE but no cardiac surgery to remove PFE. Results Group 1 had mean age of 63 ± 14 years (116 women 63%). During the study period, we identified 112 cardiac myxomas in the pathology database and 142 in the echocardiographic database. Mean age in group 2 was 67 ± 14 years (162 women 50%). PFE occurred most commonly on cardiac valves (n = 400 78%). In group 1, transient ischemic attack or stroke was the presenting symptom in 58 patients (32%). With surgical removal of valvular PFE, the valve was preserved in 92 (98%). Recurrence was documented in 3 patients (1.6%). Follow-up stroke risk in groups 1, 1a, and 1b at 1 year was 2%, 0%, and 4%; at 5 years, 8%, 5%, and 11%, respectively. Cerebrovascular accident risk in group 2 at 1 and 5 years was 6% and 13%. Conclusions In patients with echocardiographically suspected PFE who do not undergo surgical removal, rates of cerebrovascular accident and mortality are increased.
The 2021 WHO Classification of Tumors of the Heart Maleszewski, Joseph J.; Basso, Cristina; Bois, Melanie C. ...
Journal of thoracic oncology,
April 2022, 2022-04-00, 20220401, Letnik:
17, Številka:
4
Journal Article
The intersection of oncological and cardiovascular diseases is an increasingly recognized phenomenon. This recognition has led to the emergence of cardio-oncology as a true subspecialty. This field ...is not simply limited to primary cardiac tumors or complications of chemotherapeutic medications. Rather, it also encompasses metastatic cardiovascular complications and secondary cardiovascular effects of the underlying neoplasia. This review will broadly cover primary and metastatic cardiac neoplasms, as well as secondary cardiovascular effects of extracardiac neoplasia (e.g., amyloidosis, carcinoid valvulopathy, and chemotherapeutic cardiotoxicities).
Recurrent pericarditis is a debilitating condition that can be recalcitrant to conventional therapy with nonsteroidal anti-inflammatory agents, colchicine, and glucocorticoids. The aim of this study ...was to evaluate the therapeutic role of the recombinant interleukin-1 receptor antagonist anakinra in a series of adult patients with recurrent pericarditis refractory to conventional therapy. We retrospectively reviewed the medical records of 13 consecutive patients with treatment-refractory recurrent pericarditis who received anakinra for management of their disease. None of the patients had an identified systemic inflammatory rheumatic disease. The primary end points were symptom resolution and glucocorticoid discontinuation. Thirteen patients (10 women) treated with anakinra were followed for a median (range) of 16.8 months (1.3 to 24). All patients had chest pain. Total duration of symptoms before initiation of anakinra was 3 years (1.1 to 6.0). Pericardial thickening was detected by echocardiography in 9 patients (69%). All 13 patients (100%) experienced at least a partial and, most, a complete resolution of symptoms. Response to therapy was rapid, within 2 to 5 days. At last follow-up, 11 patients (84%) had successfully discontinued concomitant nonsteroidal anti-inflammatory agent, colchicine, and glucocorticoid therapy; 11 patients remained on anakinra at the end of the follow-up period. The only side effect was transient injection site reaction in 4 patients (31%). In conclusion, anakinra may be an effective alternative agent for the management of glucocorticoid-dependent recurrent pericarditis. Side effects were minor. A formal clinical trial to evaluate the usefulness of this agent should be considered.
Primary amyloidosis has a poor prognosis as a result of frequent cardiac involvement. We recently reported a high prevalence of intracardiac thrombus in cardiac amyloid patients at autopsy. However, ...neither the prevalence nor the effect of anticoagulation on intracardiac thrombus has been evaluated antemortem.
We studied all transthoracic and transesophageal echocardiograms of cardiac amyloid patients at the Mayo Clinic. The prevalence of intracardiac thrombosis, clinical and transthoracic/transesophageal echocardiographic risks for intracardiac thrombosis, and effect of anticoagulation were investigated. We identified 156 patients with cardiac amyloidosis who underwent transesophageal echocardiograms. Amyloidosis was the primary type (AL) in 80; other types occurred in 76 patients, including 56 with the wild transthyretin type, 17 with the mutant transthyretin type, and 3 with the secondary type. Fifth-eight intracardiac thrombi were identified in 42 patients (27%). AL amyloid had more frequent intracardiac thrombus than the other types (35% versus 18%; P=0.02), although the AL patients were younger and had less atrial fibrillation. Multivariate analysis showed that atrial fibrillation, poor left ventricular diastolic function, and lower left atrial appendage emptying velocity were independently associated with increased risk for intracardiac thrombosis, whereas anticoagulation was associated with a significantly decreased risk (odds ratio, 0.09; 95% CI, 0.01 to 0.51; P<0.006).
Intracardiac thrombosis occurs frequently in cardiac amyloid patients, especially in the AL type and in those with atrial fibrillation. Risk for thrombosis increased if left ventricular diastolic dysfunction and atrial mechanical dysfunction were present. Anticoagulation therapy appears protective. Timely screening in high-risk patients may allow early detection of intracardiac thrombus. Anticoagulation should be carefully considered.
Apical hypertrophic cardiomyopathy (HC) has been considered a “benign” form of HC, with limited data on long-term outcome. We compared apical HC patients with a non-HC, age- and gender-matched ...Minnesota white population to identify outcomes and prognostic factors. Between 1976 and 2006, 193 patients (62% men) with apical HC were seen at our clinic. Their most recent echocardiographic examinations were reviewed. Mean ± SD age at first presentation was 58 ± 17 years. A family history of HC or sudden cardiac death (SCD) was reported by 43 patients (22%); coronary artery disease was known in 22 (11%). An apical pouch was present in 29 patients, including an apical aneurysm in 6 and apical dilatation with hypokinesis in 23. Median follow-up (187 patients 97%) was 78 months (range, 1–350). Death from all causes occurred in 55 patients (29%; 33 women) at a mean age of 72 years (range, 20–92). During follow-up, more women had heart failure (p = 0.001), atrial fibrillation (p = 0.009), or died (p <0.001) than men. Survival was worse than expected (p = 0.001); the observed versus expected 20-year survival was 47% versus 60%. SCD, resuscitated cardiac arrest, and/or defibrillator discharge was observed in 11 patients (6%) during follow-up. Multivariate predictors of decreased survival were higher age at baseline (p <0.001), female gender (p <0.001), and atrial fibrillation at baseline (p = 0.06). In conclusion, apical HC in this population was associated with increased mortality, especially in women. Because apical HC is less benign than previously suspected, careful longitudinal care is warranted.
Echocardiographic assessment of cancer-associated non-bacterial thrombotic endocarditis (Ca-NBTE) is limited to cases reports and small clinical series. Identifying heart valves abnormalities and its ...relation to embolic complications and cancer types.
Manual review of echocardiographic images and medical records of Mayo Clinic patients (03/31/2002-06/30/2022) was performed. Ca-NBTE in 111 patients (mean age 63.2±9.7 years, 66.7% female) predominantly affected mitral valves (MV) (69), 56 aortic (AV), 8 tricuspid (TV) and rarely pulmonic (PV) (1). In 18 patients 2 valves were involved, 3 and 4 valves involvement in only a single patient each. Embolic complications were prevalent (n=102, 91.9%). Ca-NBTE affected MV more frequently the on upstream (atrial) (90% vs 49.3%) and TV downstream (ventricular) side (75% vs 37.5%). NBTE size (cm) varied significantly among valves, with TV hosting the largest masses (0.63-2.40 x 0.39-1.77), compared to MV (0.11-1.81 x 0.11-1.62), (length p=0.001; width p=0.03), and to AV (0.20-2.70 x 0.11-1.51), (length p=0.001; width p=0.056); MV masses were borderline longer in systemic compared to cerebral emboli (p=0.057). Majority of MV (79.6%) and AV (69.6%) had thickened leaflets. NBTE lesions commonly affected closing margins (73.9%MV, 85.7% AV, and 62.5% of TV), but rarely commissures of MV (8.7%), yet fairly frequently of AV (41.1%). Five patients had severe regurgitation of MV and 5 AV.
Ca-NBTE manifests mainly as thrombotic mobile masses attached to thickened MV and AV, with distinct variations in size based on valve type. Embolic destination but not cancer type is associated with NBTE mass size, and location.
To describe the survival of a large nonobstructive hypertrophic cardiomyopathy (NO-HCM) cohort and to identify risk factors for increased mortality in this population.
Patients were identified from ...the Mayo Clinic HCM database from January 1, 1975, through November 30, 2006, for this retrospective observational study. Patients with resting or provocable left ventricular outflow tract gradients were excluded. Echocardiographic, clinical, and genetic data were compared between subgroups, and survival data were compared with expected population rates.
A total of 706 patients with NO-HCM were identified. During median follow-up of 5 years (mean, 7 years), there were 208 deaths. Overall survival was no different than expected compared with age- and sex-matched white US population mortality rates (P=.77). Independent predictors of death were age at diagnosis, "burned out" HCM, and history of transient ischemic attack or stroke; use of an implantable cardioverter defibrillator (ICD) was inversely related to death. After exclusion of patients with an ICD, there was no difference in survival compared with age- and sex- matched individuals (P=.39); age, previous transient ischemic attack/stroke, and burned out HCM were predictors of death.
In this cohort, patients with NO-HCM had similar survival rates as age- and sex-matched white US population mortality rates. Although use of an ICD was inversely related to death, no differences in overall survival were seen after those patients were excluded. Burned out HCM was independently associated with an increased risk of death, identifying a subset of patients who may benefit from more aggressive therapies.
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