scholarly journals 201 Clinical presentation profiles and natural history of cardiac amyloidosis: long-term monocentric analysis (1990–2020)

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Aldostefano Porcari ◽  
Valentina Allegro ◽  
Linda Pagura ◽  
Francesca Longo ◽  
Maddalena Rossi ◽  
...  
Keyword(s):  
Natural History ◽  
Cardiac Death ◽  
Cardiac Amyloidosis ◽  
Outcome Measure ◽  
Favourable Outcome ◽  
Secondary Outcome ◽  
Historical Cohort ◽  
All Cause Mortality ◽  
History Of

Abstract Aims Cardiac Amyloidosis (CA) is considered a rare condition comprising different entities. Epidemiological data are limited and the natural history of disease is largely unknown. Understanding the clinical profiles at presentation, the impact of novel diagnostic strategies and the prognostic predictors at baseline will improve patients’ clinical management. We aimed to examine the epidemiology and natural history of CA in the last 30 years at a tertiary referral centre for amyloidosis. Methods and results Data of patients included in the prospective ‘Cardiac Amyloidosis Registry’ of Trieste from January 1990 to December 2020 were extracted from an electronical database and analysed. The diagnosis of CA was made in presence of (a) amyloid deposition found at endomyocardial biopsy (EMB), or (b) high grade cardiac uptake at bisphosphonate scintigraphy in absence of monoclonal components. Cardiological data of patients included (i) clinical examination, (ii) electrocardiogram (ECG), (iii) echocardiography and (iv) medications. The primary outcome measure was all-cause mortality. The secondary outcome measure was cardiac death. Of the 143 patients with CA included in this analysis, 77 (54%) were diagnosed before 2016 (historical cohort) and 66 (46%) ≥2016 (contemporary cohort). Light chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis accounted for 49% and 38%, respectively, of all CA patients. CA of unknown aetiology accounted for 13% of cases. CA was diagnosed by EMB in 98 (69%) patients and by cardiac scintigraphy with bone tracers in 45 (31%) patients. Patients in the contemporary cohort (67% ATTR-CA) were diagnosed more frequently by non-invasive approach compared to those in the historical cohort. At a median global Follow-up of 36 months, a more favourable outcome was found in a) patients from the contemporary cohort compared to those from the historical cohort (P < 0.001), b) ATTR- compared to AL-CA (at 18 months of Follow-up 42 (85%) ATTR patients and 32 (60%) AAL patients were alive, P = 0.013), and, (c) patients diagnosed non-invasively by scintigraphy rather than by histology (at 18 months of Follow-up 36 (80%) of patients diagnosed by cardiac scintigraphy and 57 (60%) of those diagnosed by histology were alive, P = 0.001). Of note, while no difference in outcome was found among AL- and ATTR-CA in the historical cohort, ATTR-CA patients had lower all-cause mortality and cardiac death than AL-CA patients in the contemporary cohort. Overall, death for end stage HF was more prevalent in patients with AL- than ATTR-CA (58% vs. 25%, P = 0.002). At univariable analysis, ACE-i and beta blockers (BBs) therapy were associated with a more favorable outcome [HR: 0.38, (0.26–0.60, P < 0.001) and HR: 0.53 (0.33–0.85, P = 0.008), respectively], while experiencing a previous syncope and having low QRS voltages at surface ECG portended a worse overall survival [HR: 2.42 (1.3–4.5, P = 0.006) and HR: 1.94 (1.3–3.0, P = 0.002), respectively]. At multivariate analysis, treatment with ACE-i, BBs, and syncope had independent prognostic value [HR 0.41 (0.23–0.71, P = 0.002); HR 0.50 (0.31–0.82, P = 0.007), and HR: 2.1 (1.0–4.1, P = 0.040); respectively]. Conclusions The epidemiology and natural history of CA has been changing. Over years, ATTR-CA had the most favourable outcome. NYHA functional class, syncope, BBs and ACE-i therapy were useful parameters for prognostic stratification. Further research is needed to investigate if they could be integrated in multiparametric scores for more accurate outcome prediction.

Natural History of Disseminated Coccidioidomycosis: Examination of the Veterans Affairs–Armed Forces Database

2020 ◽  
Author(s):  
Derek J Bays ◽  
George R Thompson ◽  
Susan Reef ◽  
Linda Snyder ◽  
Alana J Freifeld ◽  
...  
Keyword(s):  
Natural History ◽  
Pulmonary Nodules ◽  
Armed Forces ◽  
Veterans Affairs ◽  
Initial Infection ◽  
All Cause Mortality ◽  
History Of ◽  
Insight Into

Abstract Background The natural history of non–central nervous system (non-CNS) disseminated coccidioidomycosis (DCM) has not been previously characterized. The historical Veterans Affairs (VA)–Armed Forces coccidioidomycosis patient group provides a unique cohort of patients not treated with standard antifungal therapy, allowing for characterization of the natural history of coccidioidomycosis. Methods We conducted a retrospective study of 531 VA–Armed Forces coccidioidomycosis patients diagnosed between 1955–1958 and followed to 1966. Groups were identified as non-DCM (462 patients), DCM (44 patients), and CNS (25 patients). The duration of the initial infection, fate of the primary infection, all-cause mortality, and mortality secondary to coccidioidomycosis were assessed and compared between groups. Results Mortality due to coccidioidomycosis at the last known follow-up was significantly different across the groups: 0.65% in the non-DCM group, 25% in the DCM group, and 88% in the CNS group (P < .001). The primary fate of pulmonary infection demonstrated key differences, with pulmonary nodules observed in 39.61% of the non-DCM group, 13.64% of the DCM group, and 20% of the CNS group (P < .001). There were differences in cavity formation, with 34.20% in the non-DCM group, 9.09% in the DCM group, and 8% in the CNS group (P < .001). Dissemination was the presenting manifestation or was concurrent with the initial infection in 41% and 56% of patients in the non-CNS DCM and CNS groups, respectively. Conclusions This large, retrospective cohort study helps characterize the natural history of DCM, provides insight into the host immunologic response, and has direct clinical implications for the management and follow-up of patients.

Natural history of brain capillary vascular malformations in hereditary hemorrhagic telangiectasia patients

2016 ◽  
Vol 9 (1) ◽  
pp. 26-28 ◽  
Author(s):  
Waleed Brinjikji ◽  
Vivek N Iyer ◽  
Giuseppe Lanzino ◽  
Kent R Thielen ◽  
Christopher P Wood
Keyword(s):  
Natural History ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Vascular Malformations ◽  
Neurological Deficits ◽  
Secondary Outcome ◽  
Consecutive Series ◽  
Focal Neurological Deficit ◽  
Brain Capillary ◽  
History Of

Background and purposeBrain capillary vascular malformations (CVMs) are known to occur with relatively high frequency in hereditary hemorrhagic telangiectasia (HHT) patients. These lesions are thought to have a benign natural history but this has not been systematically studied. The purpose of our study was to examine the natural history of CVMs in a consecutive series of HHT patients.Materials and methodsConsecutive patients with untreated CVMs receiving serial imaging were included. Baseline data including demographics, HHT gene mutations, and Curacao diagnostic criteria were collected. The primary outcome was rupture on follow-up. A secondary outcome was new focal neurological deficit or seizure related to the lesion.Results22 patients with 42 CVMs were included. Mean age was 45.9±16.9 years. 18 patients (81.8%) were women and 4 (18.2%) were men. 19 patients (86.4%) had definite HHT and 3 patients (13.6%) had probable HHT. Mean follow-up was 4.6±3.7 years. There were a total of 100.2 patient years of follow-up and 222.5 lesion years. No lesions ruptured on follow-up and no patient had focal neurological deficits or seizures related to the lesions.ConclusionsOur study found that CVMs in HHT patients have a benign natural history as no patients had hemorrhage or other symptoms related to these lesions. These findings should be confirmed in additional multicenter longitudinal studies.

The prevalence and natural history of mitral annulus calcification

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N Kato ◽  
M Guerrero ◽  
C.G Scott ◽  
A.T Lee ◽  
M.F Eleid ◽  
...  
Keyword(s):  
Natural History ◽  
Mitral Annulus ◽  
Left Ventricular ◽  
Large Cohort ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Echocardiographic Evaluation ◽  
All Cause Mortality ◽  
History Of

Abstract Background There is a paucity of data about the prevalence and natural history of mitral annular calcification (MAC). Purposes In a large cohort of patients undergoing clinically-indicated echocardiography, we aimed to evaluate the prevalence and the natural history of MAC, and the frequency of associated mitral valve dysfunction (MVD). Methods Retrospective review of all patients who underwent echocardiographic evaluation at our institution between January 1st and December 31st 2015. The patients were stratified into 3 groups: MAC with MVD, MAC without MVD, no MAC. MVD was defined as moderate or greater mitral regurgitation (MR) or stenosis (MS). The 3 groups were compared for baseline clinical and echocardiographic characteristics and all-cause mortality during follow-up. Results Of 24,415 evaluated patients, 5478 (22%) had MAC. Patients with MAC were older (75±10 vs. 60±16 years, p<0.01) and more often female (46% vs. 43%, p<0.01). MVD was more frequently observed in patients with MAC (16% vs. 7%, p<0.01); moderate or greater MR was found in 10% of patients with MAC vs. 6% without MAC (p<0.01) and MS in 7% vs. 0.5% (p<0.01). In patients with MAC, those with MVD were more often female and more frequently had creatinine ≥2mg/dl and moderate or greater aortic stenosis (AS) than those without MVD (Fig. A). Kaplan-Meier survivals at 1 year were 75% in patients with MAC and MVD, 87% in those with MAC without MVD, and 92% in those without MAC (Fig B). Adjusted for age, sex, diabetes, creatinine ≥2 mg/dl, coronary artery disease, left ventricular ejection fraction <50%, and AS, MAC was associated with higher all-cause mortality (adjusted hazard ratio1.4, 95% confidence interval: 1.3–1.5, p<0.01); combined MAC and MVD was associated with the highest mortality during follow-up of 2.5±1.8 years. Conclusion In a large cohort of patients undergoing echocardiographic evaluation, the prevalence of MAC was 22%. MV dysfunction was more than twice as prevalent in patients with MAC as without. Patients with MAC had higher all-cause mortality than patients without MAC. Among patients with MAC and mitral valvular dysfunction, adjusted mortality was two-fold higher compared to those without MAC. Funding Acknowledgement Type of funding source: None

Natural history of helicobacter pylori infection from infancy to adulthood: A 21-year follow-up cohort study

2001 ◽  
Vol 120 (5) ◽  
pp. A128-A128 ◽  
Author(s):  
H MALATY ◽  
D GRAHAM ◽  
A ELKASABANY ◽  
S REDDY ◽  
S SRINIVASAN ◽  
...  
Keyword(s):  
Helicobacter Pylori ◽  
Cohort Study ◽  
Natural History ◽  
Helicobacter Pylori Infection ◽  
History Of

The natural history of pituitary apoplexy: long term follow-up study

2019 ◽  
Author(s):  
Ayesha Shaikh ◽  
Natasha Shrikrishnapalasuriyar ◽  
Giselle Sharaf ◽  
David Price ◽  
Maneesh Udiawar ◽  
...  
Keyword(s):  
Natural History ◽  
Pituitary Apoplexy ◽  
Follow Up Study ◽  
Long Term Follow Up ◽  
History Of

The natural history of benign thyroid nodules: 10 years follow-up

2020 ◽  
Author(s):  
Valeria Ramundo ◽  
Giorgio Grani ◽  
Rocco Bruno ◽  
Giuseppe Costante ◽  
Domenico Meringolo ◽  
...  
Keyword(s):  
Natural History ◽  
Thyroid Nodules ◽  
History Of ◽  
Benign Thyroid Nodules ◽  
Benign Thyroid

Unruptured intracranial aneurysms in children: 18 years’ experience in a tertiary care pediatric institution

2019 ◽  
Vol 24 (2) ◽  
pp. 184-189 ◽  
Author(s):  
Daniel-Alexandre Bisson ◽  
Peter Dirks ◽  
Afsaneh Amirabadi ◽  
Manohar M. Shroff ◽  
Timo Krings ◽  
...  
Keyword(s):  
Carotid Artery ◽  
Natural History ◽  
Internal Carotid Artery ◽  
Intracranial Aneurysms ◽  
Internal Carotid ◽  
Tertiary Care ◽  
Unruptured Intracranial Aneurysms ◽  
History Of ◽  
Mean Size

OBJECTIVEThere are little data in the literature on the characteristics and natural history of unruptured intracranial aneurysms in children. The authors analyzed their experience with unruptured intracranial aneurysms in the pediatric population at their tertiary care pediatric institution over the last 18 years. The first objective was to assess the imaging characteristics and natural history of these aneurysms in order to help guide management strategies in the future. A second objective was to evaluate the frequency of an underlying condition when an incidental intracranial aneurysm was detected in a child.METHODSThe authors conducted a Research Ethics Board–approved retrospective review of incidental intracranial aneurysms in patients younger than 18 years of age who had been treated at their institution in the period from 1998 to 2016. Clinical (age, sex, syndrome) and radiological (aneurysm location, type, size, thrombus, mass effect) data were recorded. Follow-up imaging was assessed for temporal changes.RESULTSSixty intracranial aneurysms occurred in 51 patients (36 males, 15 females) with a mean age of 10.5 ± 0.5 years (range 9 months–17 years). Forty-five patients (88.2%) had a single aneurysm, while 2 and 3 aneurysms were found in 3 patients each (5.8%). Syndromic association was found in 22 patients (43.1%), most frequently sickle cell disease (10/22 [45.5%]). Aneurysms were saccular in 43 cases (71.7%; mean size 5.0 ± 5.7 mm) and fusiform in the remaining 17 (28.3%; mean size 6.5 ± 2.7 mm). Thirty-one aneurysms (51.7%) arose from the internal carotid artery (right/left 1.4), most commonly in the cavernous segment (10/31 [32.3%]). Mean size change over the entire follow-up of 109 patient-years was a decrease of 0.6 ± 4.2 mm (range −30.0 to +4.0 mm, rate −0.12 ± 9.9 mm/yr). Interval growth (2.0 ± 1.0 mm) was seen in 8 aneurysms (13.3%; 4 saccular, 4 fusiform). An interval decrease in size (8.3 ± 10.7 mm) was seen in 6 aneurysms (10%). There was an inverse relationship between aneurysm size and growth rate (r = −0.82, p < 0.00001). One aneurysm was treated endovascularly with internal carotid artery sacrifice.CONCLUSIONSUnruptured pediatric intracranial aneurysms are most frequently single but can occur in multiples in a syndromic setting. None of the cases from the study period showed clinical or imaging signs of rupture. Growth over time, although unusual and slow, can occur in a proportion of these patients, who should be identified for short-term imaging surveillance.

Long-term systolic blood pressure and all-cause mortality in heart failure with preserved ejection fraction: an analysis of the TOPCAT trial

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
P Huang ◽  
C Liu
Keyword(s):  
Heart Failure ◽  
Blood Pressure ◽  
Ejection Fraction ◽  
Funding Source ◽  
Preserved Ejection Fraction ◽  
Risk Of Mortality ◽  
All Cause Mortality ◽  
History Of

Abstract Background Lower systolic blood pressure (SBP) at admission or discharge was associated with poor outcomes in patients with heart failure and preserved ejection fraction (HFpEF). However, the optimal long-term SBP for HFpEF was less clear. Purpose To examine the association of long-term SBP and all-cause mortality among patients with HFpEF. Methods We analyzed participants from the Treatment of Preserved Cardiac Function Heart Failure with an Aldosterone Antagonist (TOPCAT) study. Participants had at least two SBP measurements of different times during the follow-up were included. Long-term SBP was defined as the average of all SBP measurements during the follow-up. We stratified participants into four groups according to long-term SBP: &lt;120mmHg, ≥120mmHg and &lt;130mmHg, ≥130mmHg and &lt;140mmHg, ≥140mmHg. Multivariable adjusted Cox proportional hazards models were used to estimate hazard ratios (HRs) and 95% confidence intervals (CI) for all-cause mortality associated with SBP level. To assess for nonlinearity, we fitted restricted cubic spline models of long-term SBP. Sensitivity analyses were conducted by confining participants with history of hypertension or those with left ventricular ejection fraction≥50%. Results The 3338 participants had a mean (SD) age of 68.5 (9.6) years; 51.4% were women, and 89.3% were White. The median long-term SBP was 127.3 mmHg (IQR 121–134.2, range 77–180.7). Patients in the SBP of &lt;120mmHg group were older age, less often female, less often current smoker, had higher estimated glomerular filtration rate, less often had history of hypertension, and more often had chronic obstructive pulmonary disease and atrial fibrillation. After multivariable adjustment, long-term SBP of 120–130mmHg and 130–140mmHg was associated with a lower risk of mortality during a mean follow-up of 3.3 years (HR 0.65, 95% CI: 0.49–0.85, P=0.001; HR 0.66, 95% CI 0.50–0.88, P=0.004, respectively); long-term SBP of &lt;120mmHg had similar risk of mortality (HR 1.03, 95% CI: 0.78–1.36, P=0.836), compared with long-term SBP of ≥140mmHg. Findings from restricted cubic spline analysis demonstrate that there was J-shaped association between long-term SBP and all-cause mortality (P=0.02). These association was essentially unchanged in sensitivity analysis. Conclusions Among patients with HFpEF, long-term SBP showed a J-shaped pattern with all-cause mortality and a range of 120–140 mmHg was significantly associated with better outcomes. Future randomized controlled trials need to evaluate optimal long-term SBP goal in patients with HFpEF. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): China Postdoctoral Science Foundation Grant (2019M660229 and 2019TQ0380)

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