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1.
Intern Emerg Med ; 2024 Mar 29.
Article in English | MEDLINE | ID: mdl-38551753

ABSTRACT

Recurrent pericarditis (RP) complicates approximately 30% of acute pericarditis (AP) cases. We sought to compare the prevalence and severity of objective findings seen in patients with RP. A retrospective single-center study during 2010-2019, including 765 patients diagnosed with AP. Clinical, electrocardiographic, echocardiographic, and laboratory findings were extracted from the local electronic health records. Recurrence during follow-up was documented in 134 patients (17.5%), with a median time to recurrence of 101 (± 59-251) days. The median age was 60 years (IQR 45-72), 68% were male. Most patients were defined as having idiopathic\viral pericarditis (64%). The clinical manifestation during the recurrent event of pericarditis was less prominent or attenuated when compared to the initial event-ECG signs (ST elevation 12% vs. 26%; p = 0.006, Knuckle sign 13% vs. 33%; p < 0.001, ST larger in lead L2 than L3 4% vs. 19%; p < 0.001), pericardial effusion moderate and above (11% vs. 30%; p = 0.02), and inflammatory markers (mean peak CRP levels 66 mg/l vs. 97 mg/l; p < 0.001). Similar results were seen in the subgroup of patients defined as having idiopathic\viral pericarditis. Up to 20% of patients who did not have ECG signs or a significant pericardial effusion in their 1st event demonstrated these findings during the recurrence, though still to a lesser extent compared with those who had these signs in their 1st event. The objective findings of AP are less pronounced during recurrent events. Future studies should focus on the role of advanced biomarkers and imaging in defining true RP events.

2.
J Am Heart Assoc ; 13(6): e032516, 2024 Mar 19.
Article in English | MEDLINE | ID: mdl-38471825

ABSTRACT

BACKGROUND: Rilonacept, a once-weekly interleukin-1 alpha and beta cytokine trap, reduced pericarditis recurrence in the phase 3 study, RHAPSODY (Rilonacept Inhibition of Interleukin-1 Alpha and Beta for Recurrent Pericarditis: A Pivotal Symptomatology and Outcomes Study). The RHAPSODY long-term extension further explored recurrent pericarditis natural history and treatment duration decision-making during 24 additional months of open-label rilonacept treatment. METHODS AND RESULTS: Seventy-four patients commenced the long-term extension, with a median (maximum) total rilonacept duration of 22 (35) months. Individually, 18 months after the most proximal pericarditis recurrence, investigators decided to continue rilonacept on study, suspend rilonacept for off-treatment observation (rescue allowed), or discontinue the study. The annualized incidence of pericarditis recurrence on rilonacept up to the 18-month decision milestone was 0.04 events/patient-year versus 4.4 events/patient-year prestudy while on oral therapies. At the 18-month decision milestone, 64% (33/52) continued rilonacept, 15% (8/52) suspended rilonacept for observation, and 21% (11/52) discontinued the study. Among the 33 patients (1/33; 3.0%) continuing rilonacept (median time to recurrence could not be estimated due to too few events), a single recurrence occurred 4 weeks after a treatment interruption. Among patients suspending rilonacept, 75% (6/8) experienced recurrence (median time to recurrence, 11.8 weeks [95% CI, 3.7 weeks to not estimable]). There was a 98% reduction in risk of pericarditis recurrence among patients continuing rilonacept treatment after the 18-month decision milestone versus those suspending treatment for observation (hazard ratio, 0.02; P<0.0001). CONCLUSIONS: In the RHAPSODY long-term extension, continued rilonacept treatment resulted in continued response; treatment suspension at the 18-month decision milestone was associated with pericarditis recurrence. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03737110.


Subject(s)
Interleukin-1alpha , Pericarditis , Humans , Pericarditis/drug therapy , Pericarditis/epidemiology , Recombinant Fusion Proteins/adverse effects , Recurrence , Risk Reduction Behavior , Treatment Outcome
3.
Hellenic J Cardiol ; 2024 Jan 25.
Article in English | MEDLINE | ID: mdl-38280633

ABSTRACT

BACKGROUND: The ratio between early mitral flow wave to early diastolic mitral annulus velocity (E/e' ratio) varies according to age and sex and is associated with mortality in heart failure. We sought to describe the association between E/e' and mortality in patients with no apparent structural or functional cardiac abnormality and explore possible modifiers of this association. METHODS: A retrospective study of 104,315 patients who underwent echocardiographic evaluation during 2009-2021 in the largest tertiary center in Israel. Patients with cancer, ventricular dysfunction, significant valvular or structural heart disease, or evidence of pulmonary hypertension were excluded. RESULTS: The final analysis included 32,836 patients with a median age of 56 (43-66) years, and 13,547 (41%) were female. The median E/e' was 8.3 (6.8-10.3), and 9,306 (28%) had an E/e' >10. During a median follow-up of 5.7 (3.3-8.5) years, 2,396 (7.3%) individuals died. E/e' >10 was associated with mortality (adjusted hazard ratio [HR] 1.16, 95% confidence interval [CI] 1.07-1.27, p<0.001). The mortality risk associated with E/e' >10 was significantly higher in those aged ≤70 (HR 1.26, 95% CI 1.12-1.42, p<0.001), males (HR 1.34, 95% CI 1.19-1.49, p<0.001), a normal left ventricular mass (HR 1.13, 95% CI 1.02-1.24, p = 0.017), and pulmonary artery pressure <30 mmHg (HR 1.18, 95% CI 1.06-1.30, p = 0.003). CONCLUSION: An elevated E/e' is associated with mortality, specifically in younger individuals, males, and those with a normal left ventricular mass and lower pulmonary artery pressure. This suggests that an elevated E/e' might be a marker of subclinical risk in these subgroups. Further studies are needed to identify whether an elevated E/e' is useful in shared decision-making regarding the management of cardiovascular risk factors.

4.
Catheter Cardiovasc Interv ; 103(1): 160-168, 2024 01.
Article in English | MEDLINE | ID: mdl-38059295

ABSTRACT

BACKGROUND: New vascular closure devices (VCD) are being introduced for achieving hemostasis after transcatheter aortic valve implantation (TAVI). However, no safety or efficacy data have been published compared to other contemporary VCD. AIM: To compare the safety and efficacy of suture-based Perclose Prostyle as compared to plug-based MANTA device. METHODS: A total of 408 consecutive TAVI patients from two high volume TAVI centers were included in the present study. Patients were grouped according to VCD: Prostyle versus MANTA. Propensity score matching (PSM) and multivariable analysis were utilized to compare clinical endpoints between the two groups. The primary endpoint was any vascular complication (VC) according to VARC-3 criteria. RESULTS: After PSM, a total of 264 patients were analyzed, of them 132 in each group. Overall baseline characteristics of the two groups were comparable. Primary end-point was similar between MANTA as compared to Prostyle (16.7% vs. 15.3% respectively, p = 0.888). The main driver for VC among MANTA group were minor vascular complications (15.2%). Conversely, minor and major VC contributed equally to the primary endpoint among Prostyle group (7.6%) (p = 0.013). No outcome predictors were identified in multivariate analysis. CONCLUSIONS: VCD for transfemoral TAVI using the new-generation Prostyle device or the MANTA device achieved comparable VARC-3 VC rates.


Subject(s)
Aortic Valve Stenosis , Catheterization, Peripheral , Transcatheter Aortic Valve Replacement , Vascular Closure Devices , Humans , Transcatheter Aortic Valve Replacement/adverse effects , Treatment Outcome , Femoral Artery/diagnostic imaging , Femoral Artery/surgery , Catheterization, Peripheral/adverse effects , Aortic Valve Stenosis/diagnostic imaging , Aortic Valve Stenosis/surgery , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Hemostatic Techniques/adverse effects
5.
Heart Rhythm ; 20(10): 1385-1392, 2023 10.
Article in English | MEDLINE | ID: mdl-37385464

ABSTRACT

BACKGROUND: Hypertrophic cardiomyopathy (HCM) carries an increased risk of sudden cardiac death. Ventricular fibrillation (VF) is thought to be the common culprit arrhythmia. OBJECTIVE: The purpose of this study was to describe the incidence and predictors of sustained ventricular arrhythmias (VTAs) in HCM patients. METHODS: We retrospectively analyzed all patients with HCM and an implantable cardioverter-defibrillator (ICD) from a prospectively derived registry in 2 tertiary medical centers. Clinical, electrocardiographic, echocardiographic, ICD interrogation, and genetic data were collected and compared, first between patients with and without VTAs and then between patients with only VF and those with ventricular tachycardia (VT) with or without VF. RESULTS: Of the 1328 HCM patients, 207 (145 [70%] male; mean age 33 ± 16 years) were implanted with ICDs. Over a mean follow-up of 10 ± 6 years, 37 patients with ICDs (18%) developed sustained VTAs. These were associated with a family history of sudden cardiac death and a personal history of VTAs (P = .036 and P = .001, respectively). Sustained monomorphic VT was the most common arrhythmia (n = 26, 70%) and was linked to decreased left ventricular (LV) ejection fraction and increased LV end-systolic and end-diastolic diameters. Antitachycardia pacing (ATP) successfully terminated 258 (79%) of the 326 VT events. Mortality rates were comparable between patients with and without VTAs (4 [11%] vs 29 [17%]; P = .42) and between those with and without ICDs (24 [16%] vs 85 [20%]; P = .367). CONCLUSION: VT rather than VF is the most common arrhythmia in patients with HCM; it is amenable to ATP and is associated with lower LV ejection fraction and higher LV diameters. Therefore, ATP-capable devices may be considered in HCM patients with these LV features.


Subject(s)
Cardiomyopathy, Hypertrophic , Defibrillators, Implantable , Tachycardia, Ventricular , Humans , Male , Adolescent , Young Adult , Adult , Middle Aged , Female , Retrospective Studies , Prevalence , Tachycardia, Ventricular/epidemiology , Tachycardia, Ventricular/etiology , Tachycardia, Ventricular/therapy , Ventricular Fibrillation/epidemiology , Ventricular Fibrillation/etiology , Ventricular Fibrillation/therapy , Defibrillators, Implantable/adverse effects , Death, Sudden, Cardiac/epidemiology , Death, Sudden, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/epidemiology , Adenosine Triphosphate
7.
Ann Clin Transl Neurol ; 10(4): 553-567, 2023 04.
Article in English | MEDLINE | ID: mdl-36772971

ABSTRACT

OBJECTIVE: Amyloidosis due to the transthyretin Ser77Tyr mutation (ATTRS77Y) is a rare autosomal-dominant disorder, characterized by carpal-tunnel syndrome, poly- and autonomic-neuropathy, and cardiomyopathy. However, related symptoms and signs are often nonspecific and confirmatory tests are required. We describe the age and frequency of early symptoms and diagnostic features among individuals of Jewish Yemenite descent in Israel. METHODS: Records of mutation carriers were retrospectively reviewed. ATTRS77Y diagnosis was defined by the presence of amyloid in tissue and/or amyloid-related cardiomyopathy. RESULTS: We identified the Ser77Tyr mutation at the heterozygous state in 19 amyloidosis patients (mean age at diagnosis: 62 ± 5.7 years, range 49-70) and 30 amyloid-negative carriers. The probability for disease diagnosis increased from 4.4% at age 49 to 100% at 70 and occurred earlier in males. Initial symptoms preceded diagnosis by 5 ± 3.8 years (range 0-12) and were commonly sensory changes in the extremities. Erectile dysfunction predated these in 8/13 (62%) males. In two patients cardiac preceded neurological symptoms. Two patients declined symptoms. Electrophysiological studies near the time of diagnosis indicated a median neuropathy at the wrist in 18/19 (95%) and polyneuropathy in 13/19 (68%). Skin biopsy revealed epidermal denervation in 15/16 (94%) patients. Cardiomyopathy was identified in 16/19 (84%). Sensory complaints or epidermal denervations were present in 17/30 (57%) of amyloid-negative carriers and co-occurred in 10/30 (33%). INTERPRETATION: ATTRS77Y symptoms commonly occur after age 50, but may begin earlier. Median neuropathy, skin denervation and cardiomyopathy are frequently identified. Symptoms may be absent in patients and common in amyloid-negative carriers.


Subject(s)
Amyloid Neuropathies, Familial , Cardiomyopathies , Carpal Tunnel Syndrome , Aged , Female , Humans , Male , Middle Aged , Amyloid , Amyloid Neuropathies, Familial/diagnosis , Amyloid Neuropathies, Familial/genetics , Amyloid Neuropathies, Familial/pathology , Israel , Retrospective Studies , Prealbumin/metabolism
8.
Int J Cardiol Heart Vasc ; 42: 101084, 2022 Oct.
Article in English | MEDLINE | ID: mdl-35873860

ABSTRACT

Background: Vaccines against SARS-COV2 have been crucial in efforts against COVID19, yet there have been reports of pericarditis following vaccination with mRNA-based vaccines. Methods: We questioned consecutive patients with a history of acute pericarditis (AP) evaluated in the pericardial disease clinic during 3-11/2020 in a single tertiary center. Patients with significant myocardial involvement or pericarditis secondary to another systemic disease were excluded. Results: We included 64 patients in the final analysis. Mean age was 53.1 (±18), and 26 (41%) were female. At least 1 recurrence of AP was documented in 47 (73%) cases, 32 (50%) had ≥ 3 recurrences prior to vaccination. AP was considered to be idiopathic\viral in 45 (70%) cases, 20 (31%) cases were post-injury. All patients received at least 2 doses of the vaccine, and 48 patients (75%) received a 3rd dose. Two cases of breakthrough COVID19 infections were documented. Overall, 12 patients (19%) reported any adverse events. Of which, 2 had recurrent pericarditis. There was a trend for a younger age in those patients who had adverse events (median age 45 [IQR 36-61] vs. 60 [38-71], p = 0.08). no other significant difference was seen. Conclusion: In patients with a history of acute\recurrent pericarditis, the use of BNT162b2 was mostly uneventful, but some mild disease recurrences did occur.

9.
ESC Heart Fail ; 9(4): 2301-2312, 2022 08.
Article in English | MEDLINE | ID: mdl-35488723

ABSTRACT

AIMS: To describe the phenotype, genetics, and events associated with the development of hypertrophic cardiomyopathy (HCM) with reduced ventricular function (HCMr). Heart failure in HCM is usually associated with preserved ejection fraction, yet some HCM patients develop impaired systolic function that is associated with worse outcomes. METHODS AND RESULTS: Our registry included 1328 HCM patients from two centres in Spain and Israel. Patients with normal baseline ventricular function were matched, and a competing-risk analysis was performed to find factors associated with HCMr development. Patient records were reviewed to recognize clinically significant events that occurred closely before the development of HCMr. Genetic data were collected in patients with HCMr. A composite of all-cause mortality or ventricular assist device (VAD)/heart transplantation was assessed according to ventricular function. Median age was 56, and 34% were female patients. HCMr at evaluation was seen in 37 (2.8%) patients, and 46 (3.5%) developed HCMr during median follow up of 9 years. HCMr was associated with younger age of diagnosis, poor functional class, and ventricular arrhythmia. Atrial fibrillation, pacemaker implantation, and baseline left ventricular ejection fraction (LVEF) of ≤55% were significant predictors of future HCMr development, while LV obstruction predicted a lower risk. Genetic testing performed in 53 HCMr patients, identifying one or more pathogenic variant in 38 (72%): most commonly in myosin binding protein C (n = 20). Six of these patients had an additional pathogenic variant in one of the sarcomere genes. Patients with baseline HCMr had a higher risk (hazard ratio 6.4, 4.1-10.1) for the composite outcome and for the individual components. Patients who developed HCMr in the course of the study had similar mortality but a higher rate of VAD/heart transplantation compared with HCM with normal LVEF. CONCLUSIONS: Hypertrophic cardiomyopathy with reduced ejection fraction is associated with heart failure and poor outcome. Arrhythmia, cardiac surgery, and device implantation were commonly documented prior to HCMr development, suggesting they may be either a trigger or the result of adverse remodelling. Future studies should focus on prediction and prevention of HCMr.


Subject(s)
Cardiomyopathy, Hypertrophic , Heart Failure , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/genetics , Female , Humans , Male , Phenotype , Prognosis , Risk Factors , Stroke Volume , Ventricular Function, Left
10.
ESC Heart Fail ; 9(3): 1682-1688, 2022 06.
Article in English | MEDLINE | ID: mdl-35178886

ABSTRACT

AIMS: To assess the effect of angiotensin receptor blockers/neprilysin inhibitors (ARNI) on left ventricular (LV) ejection fraction (LVEF) and LV dimensions in a real-life cohort of heart failure and reduced ejection fraction (HFrEF) patients, while analysing patient characteristics that may predict reverse LV remodelling. METHODS AND RESULTS: The ARNI-treated HFrEF patients followed at a single tertiary medical centre HF-outpatient clinic were included in the study. Clinical and echocardiographic parameters were evaluated prior to ARNI initiation, and while on ARNI therapy, assessing patient characteristics associated with reverse LV remodelling. The cohort included 91 patients (mean age 60.5 years, 90% male) and 47 (52%) patients exhibited ARNI responsiveness, defined as an increase in LVEF during therapy. Overall, LVEF increased by 19% post-ARNI (23.8 to 28.4%, P < 0.001). Subgroup analysis revealed several parameters associated with significant LVEF improvement, including baseline LVEF <30%, non-ischaemic HF aetiology, lack of cardiac resynchronization therapy (CRT), better initial functional class and ARNI initiation within 3 years from HF diagnosis (P ≤ 0.001 for all). Significant reduction in LV dimensions was noted in patients with lower initial LVEF, non-ischaemic HF and no CRT. Further combined subgrouping of the study population demonstrated that patients with both LVEF <30% and a non-ischaemic HF gained most benefit from ARNI with an average of 51% improvement in LVEF (19.9 to 30%, P < 0.001). CONCLUSIONS: The ARNI treatment response is not uniform among HFrEF patient subgroups. More pronounce reverse LV remodelling is associated with early ARNI treatment initiation in the course of HFrEF, and in those with LVEF <30%, non-ischaemic HF and no CRT.


Subject(s)
Heart Failure , Ventricular Remodeling , Angiotensin Receptor Antagonists/therapeutic use , Female , Heart Failure/drug therapy , Humans , Male , Middle Aged , Neprilysin , Stroke Volume
11.
Eur Heart J ; 43(7): 641-650, 2022 Feb 12.
Article in English | MEDLINE | ID: mdl-34463727

ABSTRACT

AIMS: Severe mitral regurgitation (MR) following acute myocardial infarction (MI) is associated with high mortality rates and has inconclusive recommendations in clinical guidelines. We aimed to report the international experience of patients with secondary MR following acute MI and compare the outcomes of those treated conservatively, surgically, and percutaneously. METHODS AND RESULTS: Retrospective international registry of consecutive patients with at least moderate-to-severe MR following MI treated in 21 centres in North America, Europe, and the Middle East. The registry included patients treated conservatively and those having surgical mitral valve repair or replacement (SMVR) or percutaneous mitral valve repair (PMVR) using edge-to-edge repair. The primary endpoint was in-hospital mortality. A total of 471 patients were included (43% female, age 73 ± 11 years): 205 underwent interventions, of whom 106 were SMVR and 99 PMVR. Patients who underwent mitral valve intervention were in a worse clinical state (Killip class ≥3 in 60% vs. 43%, P < 0.01), but yet had lower in-hospital and 1-year mortality compared with those treated conservatively [11% vs. 27%, P < 0.01 and 16% vs. 35%, P < 0.01; adjusted hazard ratio (HR) 0.28, 95% confidence interval (CI) 0.18-0.46, P < 0.01]. Surgical mitral valve repair or replacement was performed earlier than PMVR [median of 12 days from MI date (interquartile range 5-19) vs. 19 days (10-40), P < 0.01]. The immediate procedural success did not differ between SMVR and PMVR (92% vs. 93%, P = 0.53). However, in-hospital and 1-year mortality rates were significantly higher in SMVR than in PMVR (16% vs. 6%, P = 0.03 and 31% vs. 17%, P = 0.04; adjusted HR 3.75, 95% CI 1.55-9.07, P < 0.01). CONCLUSIONS: Early intervention may mitigate the poor prognosis associated with conservative therapy in patients with post-MI MR. Percutaneous mitral valve repair can serve as an alternative for surgery in reducing MR for high-risk patients.


Subject(s)
Heart Valve Prosthesis Implantation , Mitral Valve Insufficiency , Myocardial Infarction , Aged , Aged, 80 and over , Female , Heart Valve Prosthesis Implantation/adverse effects , Humans , Male , Middle Aged , Mitral Valve Insufficiency/complications , Mitral Valve Insufficiency/surgery , Myocardial Infarction/complications , Myocardial Infarction/therapy , Retrospective Studies , Treatment Outcome
12.
Cardiol J ; 29(2): 245-251, 2022.
Article in English | MEDLINE | ID: mdl-34708864

ABSTRACT

BACKGROUND: The treatment of choice for severe rheumatic mitral stenosis (MS) is balloon mitral valvuloplasty (BMV). Assessment of MS severity is usually performed by echocardiography. Before performing BMV, invasive hemodynamic assessment is also performed. The effect of anesthesia on the invasive assessment of MS severity has not been studied. The purpose of the present study was to assess changes in invasive hemodynamic measurement of MS severity before and after induction of general anesthesia. METHODS: The medical files of 22 patients who underwent BMV between 2014 and 2020 were reviewed. Medical history, laboratory, echocardiographic and invasive measurements were collected. Anesthesia induction was performed with etomidate or propofol. Pre-procedural echocardiographic measurements of valve area using pressure half time, and continuity correlated well with invasive measurements using the Gorlin formula. RESULTS: After induction of anesthesia the mean mitral valve gradient dropped by 2.4 mmHg (p = 0.153) and calculated mitral valve area (MVA) increased by 0.2 cm2 (p = 0.011). A wide variability in individual response was observed. While a drop in gradient was noted in 14 patients, it increased in 7. Gorlin derived MVA rose in most patients but dropped in 4. Assuming a calculated MVA of 1.5 cm2 and below to define clinically significant MS, 4 patients with pre-induction MVA of 1.5 cm2 or below had calculated MVA above 1.5 cm2 after induction. CONCLUSIONS: The impact of general anesthesia on the hemodynamic assessment of MS is heterogeneous and may lead to misclassification of MS severity.


Subject(s)
Anesthesia , Balloon Valvuloplasty , Mitral Valve Stenosis , Hemodynamics , Humans , Mitral Valve/diagnostic imaging , Mitral Valve/surgery , Mitral Valve Stenosis/diagnosis
13.
Eur Heart J Case Rep ; 5(8): ytab304, 2021 Aug.
Article in English | MEDLINE | ID: mdl-34476337

ABSTRACT

BACKGROUND: Venous thromboembolism (VTE) is a common condition that may manifest as intermediate or high-risk pulmonary embolism (PE), requiring either primary or subsequent fibrinolytic therapy. In these cases, catheter-directed thrombolysis (CDT) has been shown to be beneficial. CASE SUMMARY: We present the case of a borderline obese but otherwise healthy 43-year-old male individual, who was admitted with acute intermediate- to high-risk PE requiring treatment with intravenous unfractionated heparin. After initial therapy failure, the patient received CDT, with subsequent clinical worsening, and a mixed result of imaging studies suggesting partial central worsening and partial peripheral improvement of the thrombotic burden and right ventricular (RV) function. After a multidisciplinary PE response team (PERT) consultation, the diagnosis of heparin-induced thrombocytopenia (HIT) with normal platelet levels was made. Therapy was changed to intravenous bivalirudin, with an excellent clinical response and complete recovery of RV function. The patient was discharged with oral rivaroxaban therapy, and on follow-up was otherwise well. DISCUSSION: Apparent failure of thrombolytic therapy for VTE warrants a clinical investigation into possible causes of a pro-thrombotic state. In this case, the diagnosis of HIT was surprising, especially due to only a mild decline in platelet levels that were well within normal range. We also acknowledge the significance of our PERT in the key diagnosis made in this case.

14.
Circ Genom Precis Med ; 13(6): e003117, 2020 12.
Article in English | MEDLINE | ID: mdl-33151750

ABSTRACT

BACKGROUND: The X-linked Danon disease manifests by severe cardiomyopathy, myopathy, and neuropsychiatric problems. We designed this registry to generate a comprehensive picture of clinical presentations and outcome of patients with Danon disease in cardiomyopathy centers throughout Europe. METHODS: Clinical and genetic data were collected in 16 cardiology centers from 8 European countries. RESULTS: The cohort comprised 30 male and 27 female patients. The age at diagnosis was birth to 42 years in men and 2 to 65 in women. Cardiac involvement was observed in 96%. Extracardiac manifestations were prominent in men but not in women. Left ventricular (LV) hypertrophy was reported in 73% of male and 74% of female patients. LV systolic dysfunction was reported in 40% of men (who had LV ejection fraction, 34±11%) and 59% of women (LV ejection fraction, 28±13%). The risk of arrhythmia and heart failure was comparable among sexes. The age of first heart failure hospitalization was lower in men (18±6 versus 28±17 years; P<0.003). Heart failure was the leading cause of death (10 of 17; 59%), and LV systolic dysfunction predicted an adverse outcome. Eight men and 8 women (28%) underwent heart transplantation or received an LV assist device. Our cohort suggests better prognosis of female compared with male heart transplant recipients. CONCLUSIONS: Danon disease presents earlier in men than in women and runs a malignant course in both sexes, due to cardiac complications. Cardiomyopathy features, heart failure and arrhythmia, are similar among the sexes. Clinical diagnosis and management is extremely challenging in women due to phenotypic diversity and the absence of extracardiac manifestations.


Subject(s)
Glycogen Storage Disease Type IIb/pathology , Myocardium/pathology , Adolescent , Adult , Age Factors , Aged , Cause of Death , Child , Child, Preschool , Europe , Female , Heart Failure/diagnosis , Heart Failure/epidemiology , Humans , Infant , Male , Middle Aged , Registries , Survival Analysis , Treatment Outcome , Young Adult
15.
J Clin Med ; 9(10)2020 Sep 30.
Article in English | MEDLINE | ID: mdl-33008125

ABSTRACT

Sarcopenia and frailty are causes for morbidity and mortality amongst heart failure (HF) patients. Low alanine transaminase (ALT) is a marker for these syndromes and, therefore, could serve as a biomarker for the prognostication of HF patients. We performed a retrospective analysis of all consecutive hospitalized HF patients in our institute in order to find out whether low ALT values would be a biomarker for poor outcomes. Our cohort included 11,102 patients, 35.6% categorized as heart failure with reduced ejection fraction. We excluded patients with ALT > 40 IU/L and cirrhosis. 8700 patients were followed for a median duration of 22 months and included in a univariate analysis. Patients with ALT < 10 IU/L were older (mean age 78.6 vs. 81.8, p < 0.001), had past stroke (24.6% vs. 19.6%, p < 0.001), dementia (7.7% vs. 4.6%, p < 0.001), and malignancy (13.4% vs. 10.2%, p = 0.003). Hospitalization length was longer in the low-ALT group (4 vs. 3 days, p < 0.001), and the rate of acute kidney injury during hospitalization was higher (19.1% vs. 15.6%; p = 0.006). The in-hospital mortality rate was higher in the low-ALT group (6.5% vs. 3.9%; p < 0.001). Long-term mortality was also higher (73.3% vs. 61.5%; p < 0.001). In a multivariate regression analysis, ALT < 10 IU/L had a 1.22 hazard ratio for mortality throughout the follow-up period (CI = 1.09-1.36; p < 0.001). Low ALT plasma level, a biomarker for sarcopenia and frailty, can assist clinicians in prognostic stratification of heart failure patients.

16.
J Clin Med ; 9(8)2020 Aug 17.
Article in English | MEDLINE | ID: mdl-32824484

ABSTRACT

BACKGROUND: Frailty and sarcopenia are associated with frequent hospitalizations and poor clinical outcomes in geriatric patients. Ascertaining this association for younger patients hospitalized in internal medicine departments could help better prognosticate patients in the realm of internal medicine. METHODS: During a 1-year prospective study in an internal medicine department, we evaluated patients upon admission for sarcopenia and frailty. We used the FRAIL questionnaire, blood alanine-amino transferase (ALT) activity, and mid-arm muscle circumference (MAMC) measurements. RESULTS: We recruited 980 consecutive patients upon hospital admission (median age 72 years (IQR 65-79); 56.8% males). According to the FRAIL questionnaire, 106 (10.8%) patients were robust, 368 (37.5%) pre-frail, and 506 (51.7%) were frail. The median ALT value was 19IU/L (IQR 14-28). The median MAMC value was 27.8 (IQR 25.7-30.2). Patients with low ALT activity level (<17IU/L) were frailer according to their FRAIL score (3 (IQR 2-4) vs. 2 (IQR 1-3); p < 0.001). Higher MAMC values were associated with higher ALT activity, both representing robustness. The rate of 30 days readmission in the whole cohort was 17.4%. Frail patients, according to the FRAIL score (FS), had a higher risk for 30 days readmission (for FS > 2, HR = 1.99; 95CI = 1.29-3.08; p = 0.002). Frail patients, according to low ALT activity, also had a significantly higher risk for 30 days readmission (HR = 2.22; 95CI = 1.26-3.91; p = 0.006). After excluding patients whose length of stay (LOS) was ≥10 days, 252 (27.5%) stayed in-hospital for 4 days or longer. Frail patients according to FS had a higher risk for LOS ≥4 days (for FS > 2, HR = 1.87; 95CI = 1.39-2.52; p < 0.001). Frail patients, according to low ALT activity, were also at higher risk for LOS ≥4 days (HR = 1.87; 95CI = 1.39-2.52; p < 0.001). MAMC values were not correlated with patients' LOS or risk for re-admission. CONCLUSION: Frailty and sarcopenia upon admission to internal medicine departments are associated with longer hospitalization and increased risk for re-admission.

17.
Intern Med J ; 50(8): 997-1000, 2020 08.
Article in English | MEDLINE | ID: mdl-32697030

ABSTRACT

We hereby present two case reports of moderate coronavirus disease patients, suffering from profound hypoxaemia, further deteriorating later on. A schedule pre-planned awake prone position manoeuvres were executed during their hospital stay. Following this, the patients' saturation improved, later to be weaned from oxygen support. Paucity of evidence and data regarding this topic led us to review the concept of awake prone position.


Subject(s)
COVID-19/complications , COVID-19/etiology , Hypoxia/therapy , Prone Position , Wakefulness , Adult , Female , Humans , Male , Middle Aged , Pandemics , Patient Positioning
18.
Isr Med Assoc J ; 22(5): 271-274, 2020 May.
Article in English | MEDLINE | ID: mdl-32378815

ABSTRACT

BACKGROUND: In February 2020, the World Health Organisation designated the name COVID-19 for a clinical condition caused by a virus identified as a cause for a cluster of pneumonia cases in Wuhan, China. The virus subsequently spread worldwide, causing havoc to medical systems and paralyzing global economies. The first COVID-19 patient in Israel was diagnosed on 27 February 2020. OBJECTIVES: To present our findings and experiences as the first and largest center for COVID-19 patients in Israel. METHODS: The current analysis included all COVID-19 patients treated in Sheba Medical Center from February 2020 to April 2020. Clinical, laboratory, and epidemiological data gathered during their hospitalization are presented. RESULTS: Our 162 patient cohort included mostly adult (mean age of 52 ± 20 years) males (65%). Patients classified as severe COVID-19 were significantly older and had higher prevalence of arterial hypertension and diabetes. They also had significantly higher white blood cell counts, absolute neutrophil counts, and lactate dehydrogenase. Low folic acid blood levels were more common amongst severe patients (18.2 vs. 12.9 vs. 9.8, P = 0.014). The rate of immune compromised patients (12%) in our cohort was also higher than in the general population. The rate of deterioration from moderate to severe disease was high: 9% necessitated non-invasive oxygenation and 15% were intubated and mechanically ventilated. The mortality rate was 3.1. CONCLUSIONS: COVID-19 patients present a challenge for healthcare professionals and the whole medical system. We hope our findings will assist other providers and institutions in their care for these patients.


Subject(s)
Coronavirus Infections/diagnosis , Pneumonia, Viral/diagnosis , Adult , Aged , Betacoronavirus , COVID-19 , Cohort Studies , Coronavirus Infections/complications , Coronavirus Infections/therapy , Diabetes Mellitus/virology , Disease Outbreaks , Female , Hospitalization , Humans , Hypertension/complications , Israel , Male , Middle Aged , Pandemics , Pneumonia, Viral/complications , Pneumonia, Viral/therapy , SARS-CoV-2 , Tertiary Care Centers
19.
Int J Health Plann Manage ; 34(4): e1854-e1861, 2019 Oct.
Article in English | MEDLINE | ID: mdl-31523844

ABSTRACT

BACKGROUND: While glycemic control of hospitalized diabetic patients is straightforward, personalization of management at discharge is challenging. Treatment guidelines base recommendations on the clinical profile of patients. We checked the feasibility of implementing discharge recommendations, based on the clinical profile in the patients' electronic health records (EHR). METHODS: A decision-making algorithm was devised according to current guidelines. It was incorporated into the EHR. A prospective follow-up of eligible diabetes patients was done. RESULTS: During 15 months, 835 patients (HbA1c was 6.9% [6.2%-7.8%]) met our inclusion criteria. The rate of HbA1c acquisition increased from 55% during Q1 to 85%, 86%, 88%, and 87% thereafter. Also, the rate of incorporating personalized management recommendations to discharge letters increased: from 14.9% during Q1 to 42.9%, 43.0%, 47.2%, and 53.4% thereafter. Fifty-eight (17.3%) of patients who got personalized recommendations upon discharge were found to have HbA1c values that were over 1% deviating from suggested target HbA1c. They got the most stringent recommendations. Twenty-nine (50%) of them had available follow-up HbA1c values showing a significant drop in HbA1c: from 9.1% (8.4%-10.2%) to 8.5% (7.4%-9.5%), P = .03. CONCLUSIONS: Personalized, EHR algorithm-based, management recommendations for diabetes upon discharge from hospitalization are feasible and beneficial.


Subject(s)
Diabetes Mellitus, Type 2/therapy , Electronic Health Records , Patient Discharge Summaries , Precision Medicine/methods , Aged , Algorithms , Electronic Health Records/statistics & numerical data , Female , Glycated Hemoglobin/analysis , Humans , Male , Patient Discharge , Prospective Studies
20.
J Cardiol ; 74(5): 466-472, 2019 Nov.
Article in English | MEDLINE | ID: mdl-31235420

ABSTRACT

BACKGROUND: While physical rehabilitation has been shown to be beneficial and safe for patients suffering from heart failure, data on rehabilitation for hypertrophic cardiomyopathy (HCM) patients are limited. METHODS: Forty-five HCM patients participated in an exercise rehabilitation program. Exercise capacity was measured in metabolic equivalent of task (METs) units and functional status was defined according to the New York Heart Association (NYHA). Self-reported measurements addressed the quality of life and daily life function. RESULTS: Of the 45 participants, 32 completed at least 3 months of rehabilitation and had data from two sequential exercise tests. A significant increase in exercise capacity (from mean 5.3 to 6.7 METs, p=0.01), was achieved at higher peak heart rates. Eighteen patients (56%) who showed improvement in exercise capacity did not differ in their NYHA class, clinical, electrocardiographic, or echo-Doppler parameters compared to those who did not improve. The benefit from training was associated with a lower exercise capacity at baseline and was most pronounced in those capable of less than 6.8 METs (p=0.008). No significant arrhythmias or adverse events were recorded in HCM patients during participation. In ∼40% of participants, training improved the subjective perception of functional capacity and quality of life; only 4 patients (9%) discontinued their participation due to discomfort during or following training. The improvement in exercise capacity was comparable between HCM and a reference group of dilated cardiomyopathy patients. CONCLUSIONS: Exercise rehabilitation appears to be applicable and safe in HCM. It mainly benefits patients suffering from significant functional limitation. Larger prospective studies are needed to validate these findings and better characterize patients expected to benefit from these programs.


Subject(s)
Cardiac Rehabilitation/methods , Cardiomyopathy, Hypertrophic/rehabilitation , Exercise Therapy/methods , Adult , Exercise/physiology , Exercise Test , Female , Heart Rate/physiology , Humans , Male , Middle Aged , Prospective Studies , Quality of Life , Treatment Outcome
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