scholarly journals Long-Term Outcome in High-Risk Patients With Hypertrophic Cardiomyopathy After Primary Prevention Defibrillator Implants

2020 ◽  
Vol 13 (10) ◽  
Author(s):  
Ethan J. Rowin ◽  
Austin Burrows ◽  
Christopher Madias ◽  
N.A. Mark Estes ◽  
Mark S. Link ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
Sudden Death ◽  
Initial Therapy ◽  
Study Cohort ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Long Time ◽  
Device Complications

Background: The implantable cardioverter-defibrillator (ICD) is effective for preventing sudden death in patients with hypertrophic cardiomyopathy. However, data on performance and complications of implanted ICDs over particularly long time periods to inform clinical practice is presently incomplete. Methods: The study cohort comprises 217 consecutive hypertrophic cardiomyopathy patients with primary prevention ICDs implanted before 2008 and followed for ≥10 years (mean 12±4; range to 31). Results: Patients were 38±17 years at implant and 45 (21%) experienced appropriate interventions terminating ventricular tachycardia/ventricular fibrillation. The majority of ICD discharges occurred ≥5 years after implant (29 patients; 64%), including ≥10 years in 16 patients (36%). Initial device therapy increased in frequency from 2.3% of patients at <1 year to 8.5% of patients at ≥10-years after implant ( P =0.005). Inappropriate ICD shocks in 39 patients occurred most commonly <5 years after implant (54%) and decreased in frequency with increasing time from implant (from 9.7% of patients at <5 years to 3.8% at ≥10 years, P =0.02). Other major device complications including infection and lead fractures and dislodgement occurred in 27 patients (12%) but did not increase in frequency over follow-up after implant ( P =0.47). There were no arrhythmic sudden death events among the 217 patients with ICD. Conclusions: In hypertrophic cardiomyopathy, after a primary prevention implant, ICD therapy often followed prolonged periods of device dormancy and increased progressively in frequency over time, including one-third of patients with initial therapy after 5 to 9 years, and an additional one-third of patients at ≥10 years. Frequency of inappropriate shocks decreased over follow-up, likely reflecting standard changes in device programming, while occurrence of device complications, such as lead fractures/infection, did not increase during follow-up.

scholarly journals Persistent disease after surgery for primary hyperparathyroidism: the long-term outcome

2004 ◽  
pp. 19-25 ◽  
Author(s):  
G Hedback ◽  
A Oden
Keyword(s):  
Primary Hyperparathyroidism ◽  
Serum Calcium ◽  
Bone Fractures ◽  
State Of Health ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Persistent Disease ◽  
Long Time ◽  
Extensive Surgery

OBJECTIVE: Nineteen patients with persistent primary hyperparathyroidism were investigated to evaluate their clinical and laboratory status a long time after treatment was ended. The risk of persistent disease and need for extensive surgery for cure, i.e. more than two neck operations, or mediastinal exploration, was also evaluated. DESIGN: The medical records of 896 consecutive patients operated on for primary hyperparathyroidism were scrutinised at follow-up, a mean of 10.3 Years after surgery. Data on state of health, medication, bone fractures and other diagnoses were collected by use of a questionnaire. There were 600 patients still alive, among whom 13 had persistent disease and they were compared with 509 patients who were cured and without any suspicion of recurrent disease, according to laboratory examination. RESULTS: Serum calcium and creatinine values had with few exceptions remained stable over the Years. In five patients, serum calcium levels were within the normal range at follow-up. Still, all 19 patients were considered hyperparathyroid. They had substantial cardiovascular morbidity, and their state of health was not as good as that of the patients who were surgically cured. After one operation, 5.5% (95% confidence interval (CI) 4.0-7.2%) had persistent disease, and 2.1% after reoperation. Extensive surgery for cure was performed in 2% of the patients (95% CI 1.1-3.2%). CONCLUSIONS: We found that the state of health was significantly better for patients with cured primary hyperparathyroidism than for patients with persistent disease, but serious deterioration of laboratory values was uncommon. The result of the present study supports surgical treatment.

scholarly journals Long-term outcome with lenalidomide and dexamethasone therapy for newly diagnosed multiple myeloma.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 8096-8096 ◽  
Author(s):  
Geetika Srivastava ◽  
Vishal Rana ◽  
Martha Lacy ◽  
Morie A. Gertz ◽  
Angela Dispenzieri ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Initial Therapy ◽  
Cell Transplant ◽  
Newly Diagnosed ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Entire Cohort ◽  
Dexamethasone Therapy

8096 Background: The combination of lenalidomide and dexamethasone (Len-Dex) is a commonly used initial therapy for newly diagnosed multiple myeloma. While the short-term outcomes with respect to response and toxicity is well-known, long-term outcome with this combination as initial therapy is not well described. Methods: We studied 286 consecutive patients with newly diagnosed MM seen at our institution, who received initial therapy with Len-Dex, and who had complete follow up records. Data regarding the clinical course was obtained from medical records. Results: The median (range) age at diagnosis was 63 (28-92) yrs; 166 (58% were ≤ 65 yrs and175 (61%) were male. The median estimated follow-up was 3.9 yrs (95% CI, 3.4, 4.2) and 203 (71%) pts were alive at the time of last follow up. The median estimated duration on Len-Dex was 5.3 mos (95% CI, 4.6, 6.4). The best overall response (≥PR) was 72%, including 26% with VGPR or better and 14 (5%) not being evaluable for a response. At last follow up, 41 (14%) patients were continuing on therapy. There were 93 pts (32%) who stayed on therapy for 12 months or more. Among these patients, the ORR was 86%, including 45% with VGPR or better. The median overall survival (OS) for the entire cohort from diagnosis was 7.4 yrs (95% CI; 5.8, NR) and the estimated 5-yr survival was 67%. There were 16 (5.5%) pts who died within a year of diagnosis. The median time to first disease progression, irrespective of transplant status, was 30.2 mos (95% CI, 25, 42). Overall, 143 (50%) of the patients have gone to stem cell transplant. Censoring those patients who proceeded to SCT prior to relapse at the time of BMT, the median TTP was 25.5 mos (95% CI, 22, 29). The median OS was 7.4 yrs for those ≤65 yrs, compared with 6.2 yrs for the older patients (P=0.01). The 5-yr OS estimate for patients in ISS stage 1, 2 and 3 were 82, 65, and 44 months respectively. Conclusions: The current study provides long-term estimates of responses and survival in a series of patients treated initially with lenalidomide and dexamethasone. The median survival of nearly 8 years reflects the efficacy of the novel agents both at diagnosis and at relapse and confirms the survival improvements seen in MM in the last decade.

Long-Term Outcome of Autologous Stem Cell Transplantation (ASCT) for Hodgkin Lymphoma (HL) in the ABVD Therapeutic Era.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1907-1907
Author(s):  
Jonathan W. Friedberg ◽  
Elizabeth Sensenig ◽  
Jennifer Kelly ◽  
Jamie Oliva ◽  
Louis Constine ◽  
...  
Keyword(s):  
Survival Curve ◽  
Allogeneic Transplantation ◽  
Initial Therapy ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Free Survival ◽  
Prognostic Features ◽  
The Impact

Abstract ASCT is the standard therapy for relapsed HL. However, the majority of published studies suggesting benefit of ASCT for relapsed HL include relatively young patients (pts) with favorable prognostic features, and have relatively short follow-up. Moreover, very little data exists on the outcome of pts who experience progression of HL after ASCT. To determine the long-term outcome of ASCT in the modern, “ABVD-era”, and the impact of allogeneic transplantation in pts who fail ASCT, we reviewed all pts with HL who were treated with ASCT between 1990 and 2005 at the University of Rochester. 117 pts (44% female; 89% Caucasian) with documented HL were treated with ASCT for relapsed or refractory HL. At ASCT, median age was 34 years (range 19–66). 75% of these pts were treated initially with ABVD or MOPP-ABVD hybrid therapy. Histology was: NS (n=82), mixed cellularity (n=20), LP (n=6), LD (n=3), and classical NOS (n=6). 32% of pts had relapsed within 1 year of initial therapy, and 25% were refractory to therapy prior to ASCT. Conditioning regimens at ASCT were BEAC (n=80); BEAM (n=28); CBV (n=1); Cy/TBI (n=8). 49% of pts received XRT following ASCT. Median follow-up of entire cohort exceeded 5 years. At 5 years, overall survival (OS) for entire population was 50%, and event-free survival (EFS) was 38%. As expected, pts older than 45 yrs of age (n=21, p=0.06) and pts who relapsed within 1 year of initial therapy (p=0.0004) had an inferior OS after ASCT. In total, 49 pts have died; of these, 30 pts died of HL. 19 deaths occurred in remission: 9 were conditioning-related within the first 100 days of ASCT; 3 were from secondary malignancies (2 AML, 1 NHL), 1 from colitis, 1 from pulmonary fibrosis, and 5 with unknown cause. 9 of the deaths occurred more than 5 yrs after ASCT. 54 pts had progression of HL post ASCT; median survival for this group was 2.1 years after ASCT. 14 of these pts (26% of pts who failed ASCT) underwent allogeneic transplantation. 8 of these patients have died: 5 from progression of HL, 2 from pulmonary toxicity, and 1 from GVHD. Of the 5 remaining pts, 1 has progression of HL after allogeneic transplantation, and 3 have chronic GVHD. We conclude that ASCT remains a curative option for patients relapsing after ABVD, as there is a clear plateau on the relapse free survival curve after 5 years. Primary refractory disease and older age at ASCT dramatically affects outcome. In this modern era, deaths in remission after ASCT remain a significant problem due to both short-term and long-term toxicities of therapy. The risk of progression of HL persists until 5 years after ASCT, emphasizing the importance of prolonged follow-up. Although some pts who progress with HL after ASCT may live for years with a seemingly indolent form of HL, there is no plateau on the survival curve, and the majority of pts who progress after ASCT are not alive 2 years post ASCT. Allogeneic transplantation has not yet had a significant impact on OS for this group of pts. Novel approaches are clearly needed for the majority of pts who progress after ASCT, particularly older patients at ASCT and patients with primary induction failure.

scholarly journals Long-Term Clinical Outcome in Familial and Sporadic Papillary Thyroid Carcinoma

2020 ◽  
Vol 9 (4) ◽  
pp. 213-220 ◽  
Author(s):  
Marco Capezzone ◽  
Noemi Fralassi ◽  
Chiara Secchi ◽  
Silvia Cantara ◽  
Lucia Brilli ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Clinical Presentation ◽  
Initial Therapy ◽  
Clinicopathological Features ◽  
Significant Trend ◽  
Papillary Thyroid ◽  
Term Outcome ◽  
Long Term Outcome

Background: The definition and the behaviour of familial papillary thyroid cancer (FPTC) compared to the sporadic form (SPTC) are still debated. Some authors believe that only families with 3 or more affected members represent an actual example of familial diseases. Objectives: The objective of the study was to analyse the clinicopathological features and the outcome of sporadic and familial PTC patients also according to the number of affected members. Methods: Among 731 patients, we identified 101 (13.8%) with familial diseases, 79 with 2 affected members (FPTC-2) and 22 with 3 or more affected members (FPTC-3) followed for a mean period of 10 years. Results: FPTC patients had more frequently bilateral tumour (p = 0.007). No difference was found between the 2 groups for the other evaluated variables. At the time of the first follow-up (1–2 years after initial therapy), FPTC patients had a higher rate of persistent disease. However, at the last follow-up, the clinical outcome was not different between sporadic and familial patients. When the comparison between SPTC and FPTC was performed, according to the number of affected members, a significant trend between the 3 groups was observed for tumour diameter (p = 0.002) and bilaterality (p = 0.003), while we did not observe a significant trend for both response to initial therapy (p = 0.15) and last clinical outcome (p = 0.22). Conclusions: Our results suggest that, although the clinicopathological features of FPTC may be more aggressive, the long-term outcome is similar between FPTC and SPTC. A possible explanation is that PTC has a favourable prognosis, even when clinical presentation is more aggressive.

Stable Blood Group Chimerism in Recipients of ABO Incompatible Allogeneic Stem Cell Transplantation

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1553-1553
Author(s):  
Ami Glik ◽  
Lilach Bonstein ◽  
Nardeen Atwah ◽  
Eti Ganon-Elazar ◽  
Israel Henig ◽  
...  
Keyword(s):  
Blood Group ◽  
Conflicts Of Interest ◽  
Blood Type ◽  
Secretory Cells ◽  
Study Cohort ◽  
Type Conversion ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Abo Blood Type

Abstract Background: The ability to express the ABH antigens in oral mucosa and saliva is present in about 80% of the general population (hence called secretors) and is regulated by the Sec gene on chromosome 19q13.3, which encodes for the enzyme fut2 (α(1,2)Fucosyltransferase), resulting in the expression of blood types in body fluids, including saliva. The gene encoding for the ABH antigens that determines ABO blood type, is located on chromosome 9q34, and transcribes to the enzyme fut1. While fut1 is mainly expressed in erythroid tissue, fut2is expressed in secretory cells. The aim of the present study was to evaluate the ABO type in saliva and red blood cells of patients undergoing allogeneic SCT from ABO mismatched donors Methods: Secretion status and ABO type in saliva and blood were analyzed in patients with different hematological malignancies undergoing alloSCT from ABO incompatible donors and from healthy donors serving as controls. All study participants signed informed consent. For the determination of ABO type in saliva, following mouth rinse, 5 cc of saliva were collected from each participant into fresh tube and immediately frozen at -800C. Saliva ABH antigens were extracted and enzymes were inactivated. Secretor status and ABO type in patients' saliva were determined by inhibition test. Agglutination of diluted A, B or O typed donor red cells was tested macroscopically in the presence or absent of the extracted ABH antigens pre-incubated with anti-A, anti-B or anti-H, respectively. ABO blood type was routinely determined in patients at least every 2 weeks and time to ABO type conversion was recorded as along with all transplant-related clinical data Results: The study cohort included 30 patients (16 males and 14 females; median age 54.2, 18.8-68.5), who underwent alloSCT between Dec 2009 and Feb 2014 from an ABO incompatible donor and were available for routine follow-up. Median follow-up time from transplant to last ABO determination in saliva was 613 days (153-2789).Donors were matched related in 11, matched unrelated in 16 and mismatched unrelated in 3 cases. All grafts were from mobilized peripheral blood. Transplant from major, minor and bidirectional ABO incompatible donors was present in 9, 16 and 5 recipients, respectively. All patients engrafted. Chimerism analysis at day 30 and 100 post transplant by PCR for STR was 100% in 24/25 and 23/25 of tested patients, respectively. Median days to ABO type conversion were 64 (21-290). Of 30 patients, 26 were found to be secretors (87%).In the secretor group, 29/30(96.6%) retained original blood group in the saliva, while one patient originally typed as AB and transplanted from an A type donor, did not retain his original AB blood type in the saliva. It is not clear whether the lack of B-antigen is attributed to the acute mucosal GvHD or to a true conversion of the ABH antigen expression in the saliva Conclusion: To the best of our knowledge, this is the first report of stable chimerism of the ABO blood groups post ABO-incompatible allogeneic transplantation, such that the majority of recipients (96.6%) retained the recipient ABO group in the saliva, while expressing the donor ABO group in the blood. The significance of these findings and correlation with long-term outcome need to be further studied in larger patient cohort Disclosures No relevant conflicts of interest to declare.

Comparison of Long-Term Outcome between Apical and Asymmetric Septal Hypertrophic Cardiomyopathy

Cardiology ◽  
2016 ◽  
Vol 136 (2) ◽  
pp. 108-114 ◽  
Author(s):  
Shuoyan An ◽  
Chaomei Fan ◽  
Lirong Yan ◽  
Chi Cai ◽  
Yinjian Yang ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Cardiovascular Mortality ◽  
Cardiovascular Death ◽  
Outflow Tract ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Unexplained Syncope ◽  
Diagnostic Age

Objectives: As reported, diagnostic age, gender and presence of outflow tract obstruction have an impact on prognosis in patients with hypertrophic cardiomyopathy. The aim of this study was to compare the long-term outcome between apical hypertrophic cardiomyopathy (ApHCM) and asymmetric septal hypertrophic cardiomyopathy (ASHCM) after the exclusion of these factors. Methods: A total of 540 patients (270 with ApHCM and 270 with ASHCM) identified in a consecutive single-center cohort were retrospectively studied. The two groups were matched by diagnostic age, gender and the presence of outflow tract obstruction. Clinical characteristics and long-term outcomes were compared. Results: The mean follow-up duration in ASHCM and ApHCM were 6.6 ± 5.5 and 7.6 ± 4.1 years, respectively. During follow-up, 16 patients experienced cardiovascular death in the ASHCM group, while 2 patients experienced cardiovascular death in the ApHCM group (6.3 vs. 0.7%, p < 0.01). Cardiovascular morbidity in the ASHCM and ApHCM groups were 39.9 and 18.5% (p < 0.01). In the multivariate Cox regression analysis late gadolinium enhancement (LGE; HR 4.81, 95% CI 1.28-78.0, p = 0.03) and unexplained syncope (HR 9.68, 95% CI 1.9-17.2, p < 0.01) were independent predictors for cardiovascular mortality. Unexplained syncope was independently associated with a higher risk for sudden cardiac death (HR 4.3, 95% CI 1.2-15.3, p = 0.02). Conclusions: After eliminating the interference of diagnostic age, gender and outflow tract obstruction, ASHCM represented a worse prognosis with a higher incidence of cardiovascular mortality and morbidity than ApHCM. LGE was a strong predictor for cardiovascular death.

Klinische Langzeitergebnisse der perkutanen transluminalen Angioplastie bei Patienten mit peripherer arterieller Verschlusskrankheit

VASA ◽  
2002 ◽  
Vol 31 (1) ◽  
pp. 36-42 ◽  
Author(s):  
. Bucek ◽  
Hudak ◽  
Schnürer ◽  
Ahmadi ◽  
Wolfram ◽  
...  
Keyword(s):  
Success Rate ◽  
Clinical Stage ◽  
Ankle Brachial Index ◽  
Clinical Situation ◽  
Clinical Results ◽  
Transluminal Angioplasty ◽  
Term Outcome ◽  
Long Term Outcome

Background: We investigated the long-term clinical results of percutaneous transluminal angioplasty (PTA) in patients with peripheral arterial occlusive disease (PAOD) and the influence of different parameters on the primary success rate, the rate of complications and the long-term outcome. Patients and methods: We reviewed clinical and hemodynamic follow-up data of 166 consecutive patients treated with PTA in 1987 in our department. Results: PTA improved the clinical situation in 79.4% of patients with iliac lesions and in 88.3% of patients with femoro-popliteal lesions. The clinical stage and ankle brachial index (ABI) post-interventional could be improved significantly (each P < 0,001), the same results were observed at the end of follow-up (each P < 0,001). Major complications occurred in 11 patients (6.6%). The rate of primary clinical long-term success for suprainguinal lesions was 55% and 38% after 5 and 10 years (femoro-popliteal 44% and 33%), respectively, the corresponding data for secondary clinical long-term success were 63% and 56% (60% and 55%). Older age (P = 0,017) and lower ABI pre-interventional (P = 0,019) significantly deteriorated primary clinical long-term success for suprainguinal lesions, while no factor could be identified influencing the outcome of femoro-popliteal lesions significantly. Conclusion: Besides an acceptable success rate with a low rate of severe complications, our results demonstrate favourable long-term clinical results of PTA in patients with PAOD.

Psychopathology 8½ Years Post Parasuicide

Crisis ◽  
1999 ◽  
Vol 20 (3) ◽  
pp. 115-120 ◽  
Author(s):  
Stephen Curran ◽  
Michael Fitzgerald ◽  
Vincent T Greene
Keyword(s):  
Rating Scales ◽  
Psychiatric Morbidity ◽  
Parental Bonding ◽  
Social Maladjustment ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Face To Face ◽  
Long Term Follow Up

There are few long-term follow-up studies of parasuicides incorporating face-to-face interviews. To date no study has evaluated the prevalence of psychiatric morbidity at long-term follow-up of parasuicides using diagnostic rating scales, nor has any study examined parental bonding issues in this population. We attempted a prospective follow-up of 85 parasuicide cases an average of 8½ years later. Psychiatric morbidity, social functioning, and recollections of the parenting style of their parents were assessed using the Clinical Interview Schedule, the Social Maladjustment Scale, and the Parental Bonding Instrument, respectively. Thirty-nine persons in total were interviewed, 19 of whom were well and 20 of whom had psychiatric morbidity. Five had died during the follow-up period, 3 by suicide. Migration, refusals, and untraceability were common. Parasuicide was associated with parental overprotection during childhood. Long-term outcome is poor, especially among those who engaged in repeated parasuicides.

Muenke syndrome: long-term outcome of a syndrome-specific treatment protocol

2019 ◽  
Vol 24 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Bianca K. den Ottelander ◽  
Robbin de Goederen ◽  
Marie-Lise C. van Veelen ◽  
Stephanie D. C. van de Beeten ◽  
Maarten H. Lequin ◽  
...  
Keyword(s):  
Treatment Protocol ◽  
First Year ◽  
Ventricular Size ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Skull Growth ◽  
Muenke Syndrome ◽  
First Year Of Life

OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.

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