Intermediate-effect size p.Arg637Gln in FHOD3 increases risk of HCM and is associated with an aggressive phenotype in homozygous carriers.

Formin homology 2 domain-containing 3 (FHOD3) gene has emerged as one of the main non-sarcomeric genes associated with hypertrophic cardiomyopathy (HCM), but no cases of biallelic variants associated with disease have been described to date. From 2014 until 2021, FHOD3 was evaluated in our center by next-generation sequencing in 22 806 consecutive unrelated probands. The p.Arg637Gln variant in FHOD3 was enriched in our HCM cohort (284 of 9668 probands; 2.94%) compared with internal controls (64 of 11 480; 0.59%) and gnomAD controls (373 of 64 409; 0.58%), with ORs of 5.40 (95% CI: 4.11 to 7.09) and 5.19 (95% CI: 4.44 to 6.07). The variant affects a highly conserved residue localised in a supercoiled alpha helix considered a clustering site for HCM variants, and in heterozygosis can act as a predisposing factor (intermediate-effect variant) for HCM, with an estimated penetrance of around 1%. Additionally, seven homozygous carriers of p.Arg637Gln in FHOD3 were identified. All but one (unaffected) showed an early presentation and a severe HCM phenotype. All this information suggest that p.Arg637Gln variant in FHOD3 is a low-penetrant variant, with an intermediate effect, that contributes to the development of HCM in simple heterozygosis, being associated with a more severe phenotype in homozygous carriers.

Usefulness of risk scores and predictors of atrial fibrillation recurrence after elective electrical cardioversion.

INTRODUCTION: Electrical cardioversion (ECV) is a frequently used procedure for restoring sinus rhythm in atrial fibrillation (AF); however, the rate of recurrence is high. The identification of patients at high risk of recurrence could influence the decision-making process. The present study evaluates the predictive value of risk scores in atrial fibrillation recurrence after elective electrical cardioversion. METHODS: Unicentric, observational, and prospective study of adult patients who have undergone an elective ECV as rhythm control strategy between July 2017 and September 2022. RESULTS: From the 283 analyzed patients (mean age 63.95 ± 10.76212, 74.9% male); 99 had paroxysmal AF (35%) and 159 (59%) presented AF recurrence during a follow-up of 6 months. In patients with post-ECV AF recurrence, the period of time from diagnosis until the performance of the procedure was longer (393 ± 891 vs. 195 ± 527, p = .02). No paroxysmal AF (71.3% vs. 57.8%, p = .02) and LA dilatation with >40 mL/m2 (35.9% vs. 23.3%, p = .02) volumes were more frequent within these patients. AF recurrence was more frequent in patients who had previous ECV (HR = 1.32; 95% CI: 1.12-2.35; p = .01) and more than 1 shock to recover sinus rhythm (HR = 1.62; 95% CI: 1.07-1.63; p = .01). The SLAC, ALARMEc, ATLAS, and CAAP-AF scores were statistically significant, although with a moderate predictive capacity for post-ECV recurrence. CONCLUSIONS: Risk scores analyzed showed a modest value predicting AF recurrence after ECV. Previous ECV, and greater difficulty in restoring SR were independent predictors of recurrence.

Alpha-protein kinase 3 (ALPK3) truncating variants are a cause of autosomal dominant hypertrophic cardiomyopathy.

AIMS: The aim of this study was to determine the frequency of heterozygous truncating ALPK3 variants (ALPK3tv) in patients with hypertrophic cardiomyopathy (HCM) and confirm their pathogenicity using burden testing in independent cohorts and family co-segregation studies. METHODS AND RESULTS: In a discovery cohort of 770 index patients with HCM, 12 (1.56%) were heterozygous for ALPK3tv [odds ratio(OR) 16.11, 95% confidence interval (CI) 7.94-30.02, P = 8.05e-11] compared to the Genome Aggregation Database (gnomAD) population. In a validation cohort of 2047 HCM probands, 32 (1.56%) carried heterozygous ALPK3tv (OR 16.17, 95% CI 10.31-24.87, P < 2.2e-16, compared to gnomAD). Combined logarithm of odds score in seven families with ALPK3tv was 2.99. In comparison with a cohort of genotyped patients with HCM (n = 1679) with and without pathogenic sarcomere gene variants (SP+ and SP-), ALPK3tv carriers had a higher prevalence of apical/concentric patterns of hypertrophy (60%, P < 0.001) and of a short PR interval (10%, P = 0.009). Age at diagnosis and maximum left ventricular wall thickness were similar to SP- and left ventricular systolic impairment (6%) and non-sustained ventricular tachycardia (31%) at baseline similar to SP+. After 5.3 ± 5.7 years, 4 (9%) patients with ALPK3tv died of heart failure or had cardiac transplantation (log-rank P = 0.012 vs. SP- and P = 0.425 vs. SP+). Imaging and histopathology showed extensive myocardial fibrosis and myocyte vacuolation. CONCLUSIONS: Heterozygous ALPK3tv are pathogenic and segregate with a characteristic HCM phenotype.

Fluoroscopy-guided axillary vein access vs cephalic vein access in pacemaker and defibrillator implantation: Randomized clinical trial of efficacy and safety.

INTRODUCTION: The most widespread venous sites of access for implantation intravenous implantable cardiac electronic device (CIED) are the cephalic and subclavian vein. Fluoroscopy-guided axillary venous access is an alternative, but efficacy and safety have not been studied under equal conditions. The aim of the present study is to compare the efficacy and safety of fluoroscopy-guided axillary vs cephalic vein access in CIED implant. METHODS AND RESULTS: Two hundred and forty patients were randomized to receive CIED implantation by the fluoroscopy-guided axillary vein access vs cephalic vein access. The implantation success, the procedure times and the complications were recorded. A comparison of the results of operators was made. The success rate of the randomized venous access was superior in the axillary group than in cephalic (98.3% vs 76.7%, P < .001). Time to access (6.8 ± 3.1 minute vs 13.1 ± 5.8 minutes, P < .001) and implantation duration was significantly shorter in the axillary group than in the cephalic group (42.3 ± 11.6 minutes vs 50.5 ± 13.3 minutes, P < .001). There was no difference in the incidence of complication and inter-operator success rate, complications rate and time to access. CONCLUSION: The fluoroscopy-guided axillary venous access is safe and has a better success rate and faster execution time compared with the cephalic vein access. When the results were compared among the study operators, neither in the axillary nor in the cephalic group there were differences in the success rate, the complication rate, and the time to access. TRIAL REGISTRATION: www.clinicaltrials.gov, NCT03860090.

Efficacy and Safety of Loading Doses With P2Y12-Receptor Antagonists in Patients Without Dual Antiplatelet Therapy Undergoing Elective Coronary Intervention.

BACKGROUND: European Guidelines on Myocardial Revascularization recommend clopidogrel loading dose added to acetylsalicylic acid in elective percutaneous coronary interventions (PCIs). However, there is few evidence supporting this recommendation and other P2Y12 inhibitors have not been tested in these patients. PURPOSE: To evaluate the effectiveness and safety of different loading doses of clopidogrel and ticagrelor in patients without double antiplatelet therapy and stable coronary artery disease (SCAD) undergoing elective PCI. METHODS: Retrospective study of 147 consecutive patients with SCAD undergoing elective PCI. Loading P2Y12 inhibitor doses evaluated were: clopidogrel 600 mg, clopidogrel 300 mg, clopidogrel 150 mg, and ticagrelor 180 mg. We analyzed the occurrence of major adverse cardiovascular events and periprocedural myocardial infarction. RESULTS: One hundred twenty-five patients were treated with clopidogrel (16 with clopidogrel 150 mg, 7 with clopidogrel 300 mg, and 93 with clopidogrel 600 mg) and 21 with ticagrelor 180 mg at the catheterization laboratory. The ticagrelor group had a significantly lower postprocedural peak of troponin-I (0.7 ± 3.4 vs. 0.3 ± 0.7 ng/mL; P = 0.02). There were no differences between groups in terms of major bleeding and hemoglobin drop after PCI (0.6 ± 0.8 vs. 4 ± 0.6; P = 0.8). The median of follow-up was 17 months (interquartile range 9-32.7). At the end of follow-up, major adverse cardiovascular event rate was not different between groups. CONCLUSIONS: In patients without dual antiplatelet therapy undergoing elective PCI, the use of ticagrelor showed lower postprocedural myocardial injury without more bleeding complications.

Cardiac amyloidosis and red flags: natural history and its impact in morbimortality. / Amiloidosis cardiaca y red flags: historia natural e impacto en la morbimortalidad.

INTRODUCTION AND OBJECTIVES: Red flags (RF) are typical cardiac and extracardiac manifestations that may precede the definitive diagnosis of cardiac amiloidosis (CA) by several years, playing a pivotal role in the early diagnosis of the disease. The principal aim of the research was to determine the chronology of onset of RF throughout the natural history of CA as well as its prognostic influence. PATIENTS AND METHODS: Observational, retrospective inquiry of consecutive patients with a definitive diagnosis of CA in a terciary hospital centre in Ciudad Real (Spain) between February 2016 and December 2023. We defined 21 RF and 3 adverse clinical events, establishing the date of occurrence of each of them. RESULTS: 102 patients (81.6±7.7 years; 84,3% males) with a diagnosis of CA (89.2% TTR; 10,8% AL) were included. The prevalence of RF was very high (8.4±2.3). In the natural history, the first to appear were integumentary, with the most specific cardiological ones being the closest to diagnosis. The 2-year mortality was 49%, with biomarker RFs and the presence of ≥10 RFs being associated with higher mortality. CONCLUSIONS: RFs proved highly prevalent among patients with CA and substantially preceded disease diagnosis. RF burden was associated with prognosis in the follow-up of ATTR patients.

[Red flags of cardiac amyloidosis in patients with spinal stenosis and yellow ligament hypertrophy]. / «Banderas rojas¼ de amiloidosis cardiaca en pacientes con estenosis de canal lumbar e hipertrofia de ligamento amarillo.

INTRODUCTION: Studies addressing the prevalence of cardiac amyloidosis (CA) among patients with spinal stenosis (SS) are lacking. The identification of the red flags (RF) of CA could lead to early detection of cases of CA. The primary objective of this study was to address the prevalence of RF of CA among patients with SS. METHODS: Transversal study including consecutive cases with SS and yellow ligament hypertrophy (YLH). A clinical assessment that included electrocardiogram, echocardiogram and urine and blood test was performed. A clinical suspicion of CA was defined by the presence of left ventricular hypertrophy plus any RF. RESULTS: One hundred and three patients with SS and YLH were assessed. The prevalence of RF was high: heart failure: 18.4%; aortic stenosis: 1.9%; carpal tunnel syndrome: 7.8%; bicipital tendon rupture: 1.9%; arterial hypotension: 17.4%; polyneuropathy symptoms: 51.5%; pseudoinfarction pattern: 3.9%; low voltages: 15.5%; conduction abnormalities: 15.5%; decreased longitudinal strain: 25.3%; apical sparing pattern: 3.9%. The 57.3% of the cohort met the CA suspicion criteria. CONCLUSION: The prevalence of RF of CA is high among patients with SS and YLH. A high proportion of patients met the CA suspicion criteria.

Penetrance of Dilated Cardiomyopathy in Genotype-Positive Relatives.

BACKGROUND: Disease penetrance in genotype-positive (G+) relatives of families with dilated cardiomyopathy (DCM) and the characteristics associated with DCM onset in these individuals are unknown. OBJECTIVES: This study sought to determine the penetrance of new DCM diagnosis in G+ relatives and to identify factors associated with DCM development. METHODS: The authors evaluated 779 G+ patients (age 35.8 ± 17.3 years; 459 [59%] females; 367 [47%] with variants in TTN) without DCM followed at 25 Spanish centers. RESULTS: After a median follow-up of 37.1 months (Q1-Q3: 16.3-63.8 months), 85 individuals (10.9%) developed DCM (incidence rate of 2.9 per 100 person-years; 95% CI: 2.3-3.5 per 100 person-years). DCM penetrance and age at DCM onset was different according to underlying gene group (log-rank P = 0.015 and P <0.01, respectively). In a multivariable model excluding CMR parameters, independent predictors of DCM development were: older age (HR per 1-year increase: 1.02; 95% CI: 1.0-1.04), an abnormal electrocardiogram (HR: 2.13; 95% CI: 1.38-3.29); presence of variants in motor sarcomeric genes (HR: 1.92; 95% CI: 1.05-3.50); lower left ventricular ejection fraction (HR per 1% increase: 0.86; 95% CI: 0.82-0.90) and larger left ventricular end-diastolic diameter (HR per 1-mm increase: 1.10; 95% CI: 1.06-1.13). Multivariable analysis in individuals with cardiac magnetic resonance and late gadolinium enhancement assessment (n = 360, 45%) identified late gadolinium enhancement as an additional independent predictor of DCM development (HR: 2.52; 95% CI: 1.43-4.45). CONCLUSIONS: Following a first negative screening, approximately 11% of G+ relatives developed DCM during a median follow-up of 3 years. Older age, an abnormal electrocardiogram, lower left ventricular ejection fraction, increased left ventricular end-diastolic diameter, motor sarcomeric genetic variants, and late gadolinium enhancement are associated with a higher risk of developing DCM.

Cardiac amyloidosis in patients with spinal stenosis and yellow ligament hypertrophy.

BACKGROUND: Spinal stenosis (SS) is a manifestation associated with cardiac amyloidosis (CA). However, there is a lack of studies assessing the prevalence of CA among patients with SS. We aimed to address the prevalence of CA among patients with SS and YLH. METHODS: We performed a cross-sectional study of consecutive patients older than 65 years with SS and yellow ligament hypertrophy (YLH). All the patients were assessed with an electrocardiogram, echocardiogram and biohumoral evaluation. Patients with CA red flags was further studied with cardiac magnetic resonance and 99mTc-DPD scintigraphy. A cohort of patients with confirmed CA and SS was used to assess clinical features associated with CA. RESULTS: 105 patients (75.0 ± 6.6 years old; 45.7% males) with SS and YLH [5.5 [5-7] mm] were screened. Prevalence of red flags of CA was high and 58 patients presented clinical suspicion of CA. One patient (0.95%) was finally diagnosed of CA. Patients with confirmed CA presented a more expressive phenotype than the screened population. Patients with suspected CA had greater YLH than patients without suspicion of CA (6.4 ± 1.3 vs. 5.0 ± 0.8 mm; p < 0.001) and patients with confirmed CA presented greater YLH than the screening population (6.7 ± 1.8 vs. 5.7 ± 1.2 mm; p = 0.018). CONCLUSION: Despite red flags of CA are common among patients with SS, the prevalence of confirmed CA was low in our sample of screened patients.

ROD2 domain filamin C missense mutations exhibit a distinctive cardiac phenotype with restrictive/hypertrophic cardiomyopathy and saw-tooth myocardium.

INTRODUCTION AND OBJECTIVES: Missense mutations in the filamin C (FLNC) gene have been reported as cause of inherited cardiomyopathy. Knowledge of the pathogenicity and genotype-phenotype correlation remains scarce. Our aim was to describe a distinctive cardiac phenotype related to rare missense FLNC variants in the ROD2 domain. METHODS: We recruited 21 unrelated families genetically evaluated because of hypertrophic cardiomyopathy (HCM)/restrictive cardiomyopathy (RCM) phenotype carrying rare missense variants in the ROD2 domain of FLNC (FLNC-mRod2). Carriers underwent advanced cardiac imaging and genetic cascade screening. Myocardial tissue from 3 explanted hearts of a missense FLNC carrier was histologically analyzed and compared with an FLNC-truncating variant heart sample and a healthy control. Plasmids independently containing 3 FLNC missense variants were transfected and analyzed using confocal microscopy. RESULTS: Eleven families (52%) with 20 assessed individuals (37 [23.7-52.7]) years showed 15 cases with a cardiac phenotype consisting of an overlap of HCM-RCM and left ventricular hypertrabeculation (saw-tooth appearance). During a median follow-up of 6.49 years, they presented with advanced heart failure: 16 (80%) diastolic dysfunction, 3 heart transplants, 3 heart failure deaths) and absence of cardiac conduction disturbances or skeletal myopathy. A total of 6 families had moderate genotype-phenotype segregation, and the remaining were de novo variants. Differential extracellular matrix remodeling and FLNC distribution among cardiomyocytes were confirmed on histology. HT1080 and H9c2 cells did not reveal cytoplasmic aggregation of mutant FLNC. CONCLUSIONS: FLNC-mRod2 variants show a high prevalence of an overlapped phenotype comprising RCM, HCM and deep hypertrabeculation with saw-tooth appearance and distinctive cardiac histopathological remodeling.

Development and Validation of a Prediction Model and Score for Transthyretin Cardiac Amyloidosis Diagnosis: T-Amylo.

BACKGROUND: Although transthyretin cardiac amyloidosis (ATTR-CA) is often underdiagnosed, clinical suspicion is essential for early diagnosis. OBJECTIVES: The aim of this study was to develop and validate a feasible prediction model and score to facilitate the diagnosis of ATTR-CA. METHODS: This retrospective multicenter study enrolled consecutive patients who underwent 99mTc-DPD scintigraphy for suspected ATTR-CA. ATTR-CA was diagnosed if Grade 2 or 3 cardiac uptake was evidenced on 99mTc-DPD scintigraphy in the absence of a detectable monoclonal component or by demonstration of amyloid by biopsy. A prediction model for ATTR-CA diagnosis was developed in a derivation sample of 227 patients from 2 centers using multivariable logistic regression with clinical, electrocardiography, analytical, and transthoracic echocardiography variables. A simplified score was also created. Both of them were validated in an external cohort (n = 895) from 11 centers. RESULTS: The obtained prediction model combined age, gender, carpal tunnel syndrome, interventricular septum in diastole thickness, and low QRS interval voltages, with an area under the curve (AUC) of 0.92. The score had an AUC of 0.86. Both the T-Amylo prediction model and the score showed a good performance in the validation sample (ie, AUC: 0.84 and 0.82, respectively). They were tested in 3 clinical scenarios of the validation cohort: 1) hypertensive cardiomyopathy (n = 327); 2) severe aortic stenosis (n = 105); and 3) heart failure with preserved ejection fraction (n = 604), all with good diagnostic accuracy. CONCLUSIONS: The T-Amylo is a simple prediction model that improves the prediction of ATTR-CA diagnosis in patients with suspected ATTR-CA.

[Cardiovascular disease after COVID-19 infection in elderly patients. Results of annual follow-up of a cohort of survivors]. / Enfermedad cardiovascular tras infección por SARS-CoV-2 en pacientes ancianos. Resultados del seguimiento anual de una cohorte de supervivientes.

INTRODUCTION: Although the effects of SARS-CoV-2 infection on the cardiovascular system is well known in the acute phase, the cardiovascular impact of the elderly population surviving COVID-19 respiratory infection after 1 year of follow-up has not been sufficiently studied. METHODS: Observational registry of 240 elderly patients (75 years or older), consecutively admitted for COVID-19 respiratory infection and survivors of the same, between March 1 and April 30, 2020, at the Hospital General Universitario de Ciudad Real. The incidence of major cardiovascular events [MACE] (cardiovascular death [CD], acute coronary syndrome [ACS], cerebrovascular disease [CVD], venous thromboembolic disease [VTE] and heart failure [HF]) was prospectively analysed. RESULTS: The mean age was 83.75±5.75 years. After a mean follow-up of 352.2±70.4 days, 13.8% of patients died and 9.6% had MACE, the most frequent being heart failure, with no differences in severity or overall course of acute illness. In the multivariate Cox regression model, the risk of developing MACE was higher in patients with chronic obstructive pulmonary disease and (HR 4.29; 95%CI 1.62-11.39; P=.003) and loop diuretic (HR 2.99; 95%CI 1.27-7.07; P=.01). CONCLUSIONS: In elderly COVID-19 survivors, the incidence of MACE after one year of follow-up is high, the main manifestation being heart failure.

[Differences among renin-angiotensin system inhibitor drugs in prognosis of hypertense patients with COVID-19]. / Diferencias entre fármacos inhibidores del sistema renina-angiotensina en pacientes hipertensos ingresados por COVID-19.

Introduction: Concerns have been raised about Renin-angiotensin system inhibitors (RASi) in patients with COVID-19. Although recent trials have proved its security, evidence regarding intrinsic differences between RASi is lacking, especially in patients with arterial hypertension. Our objective was to analyse the prognosis of hypertense patients who received angiotensin converting enzyme inhibitors (ACEi) or angiotensin-2 receptor blockers (ARBs) and were hospitalized due to COVID-19. Materials and methods: 392 consecutive patients with hypertension and COVID-19 were analyse. Incidence of the combined event (death or mechanical ventilation need) was the primary endpoint. Secondary, incidence of each event and time to event were analysed. Results: 155 received ACEi and 237 ARBs. During the hospitalization, the combined event was observed in the 31,6 % of patients. No differences were observed between those previously treated with ACEi and ARBs (33.5 vs. 30.9%; p = 0.51). In the survival analysis, no differences were observed regarding time to combined event (p = 0.91). In-hospital mortality was similar in both groups (32.3 vs. 29.1%; p = 0.51), as well as the need of mechanical ventilation (3.2 vs. 5.9%; p = 0.23). Conclusions: The type of RASi was not associated with in-hospital major events in patients with arterial hypertension hospitalized due to COVID-19.

Introducción: Han surgido dudas sobre la seguridad de los fármacos inhibidores del sistema renina-angiotensina (SRA) en pacientes con enfermedad por coronavirus 2019 (COVID-19). Aunque estudios recientes han demostrado la seguridad de este grupo de fármacos, la evidencia sobre la comparativa de los diferentes fármacos inhibidores del SRA es escasa, sobre todo en pacientes hipertensos. Nuestro objetivo fue analizar el pronóstico de los pacientes hipertensos tratados con inhibidores de la enzima convertidora de angiotensina (IECA) o antagonistas del receptor de angiotensina II (ARA II) que presentaron COVID-19. Materiales y métodos: Se analizaron 582 pacientes hipertensos con COVID-19. Se registró la incidencia del evento combinado de muerte o necesidad de ventilación mecánica invasiva (VMI) durante la hospitalización. De forma secundaria, se analizó la incidencia de eventos de manera independiente y se realizó un análisis de supervivencia para analizar el tiempo hasta los eventos. Resultados: 155 pacientes recibían tratamiento previo con IECA y 237 con ARA II. Durante la hospitalización por COVID-19, se observó una incidencia del evento combinado del 31.6%. No se detectaron diferencias entre los pacientes que recibían tratamiento con IECA y los tratados con ARA II (33.5 vs. 30.9%; p = 0.51). En el análisis de supervivencia, no se hallaron diferencias en el tiempo hasta el evento combinado (p = 0.91). La mortalidad intrahospitalaria fue similar en ambos grupos (32.3 vs. 29.1%; p = 0.51), así como la necesidad de VMI (3.2 vs. 5.9%; p = 0.23). Conclusiones: El tipo de inhibidor del SRA no se asoció a diferencias pronósticas significativas entre los pacientes hipertensos ingresados con COVID-19.

Impact of SARS-Cov-2 infection in patients with hypertrophic cardiomyopathy: results of an international multicentre registry.

AIMS: To describe the natural history of SARS-CoV-2 infection in patients with hypertrophic cardiomyopathy (HCM) compared with a control group and to identify predictors of adverse events. METHODS AND RESULTS: Three hundred and five patients [age 56.6 ± 16.9 years old, 191 (62.6%) male patients] with HCM and SARS-Cov-2 infection were enrolled. The control group consisted of 91 131 infected individuals. Endpoints were (i) SARS-CoV-2 related mortality and (ii) severe clinical course [death or intensive care unit (ICU) admission]. New onset of atrial fibrillation, ventricular arrhythmias, shock, stroke, and cardiac arrest were also recorded. Sixty-nine (22.9%) HCM patients were hospitalized for non-ICU level care, and 21 (7.0%) required ICU care. Seventeen (5.6%) died: eight (2.6%) of respiratory failure, four (1.3%) of heart failure, two (0.7%) suddenly, and three (1.0%) due to other SARS-CoV-2-related complications. Covariates associated with mortality in the multivariable were age {odds ratio (OR) per 10 year increase 2.25 [95% confidence interval (CI): 1.12-4.51], P = 0.0229}, baseline New York Heart Association class [OR per one-unit increase 4.01 (95%CI: 1.75-9.20), P = 0.0011], presence of left ventricular outflow tract obstruction [OR 5.59 (95%CI: 1.16-26.92), P = 0.0317], and left ventricular systolic impairment [OR 7.72 (95%CI: 1.20-49.79), P = 0.0316]. Controlling for age and sex and comparing HCM patients with a community-based SARS-CoV-2 cohort, the presence of HCM was associated with a borderline significant increased risk of mortality OR 1.70 (95%CI: 0.98-2.91, P = 0.0600). CONCLUSIONS: Over one-fourth of HCM patients infected with SARS-Cov-2 required hospitalization, including 6% in an ICU setting. Age and cardiac features related to HCM, including baseline functional class, left ventricular outflow tract obstruction, and systolic impairment, conveyed increased risk of mortality.

Impact of the creation of specialized units for patients with hypertrophic cardiomyopathy.

INTRODUCTION AND OBJECTIVES: According to current international guidelines, hypertrophic cardiomyopathy (HCM) patients should be managed in specialized units. However, there is lack of data on the impact of the creation of these units in the management of HCM patients. Our goal was to assess the impact of the creation of an Inherited Inherited Diseases Cardiac Unit (ICDU) in the current management of patients with HCM. METHODS: We analyzed 114 consecutive patients (62.6±8 years old, 70.2% males) with HCM. Variables related to optimal management of HCM patients and their family study were recorded, as well as guidance on the risk of sudden death. We analyzed whether patients were assessed by the ICDU or at a general cardiology consultation (GCC). RESULTS: 50 patients were assessed in the IDCU and 64 in the GCC. Familial screening was more frequent in patients assessed by the IDCU (45.3% vs. 4%; p<0.01), requesting more genetic studies of the index case (70.3% vs. 14%; p<0.01) and cardiac magnetic resonance (53.1% vs. 18%; p<0.01). Sudden death risk score was performed more frequently in patients after the creation of an IDCU (67.2% vs. 28%; p<0.01). Treatment with beta-blockers was similar in both groups (72% vs. 78.1%; p=0.24). An implantable cardiac defibrillator was indicated similarly in both groups (12.5% in ICDU and 6% in GC; p=0.24). CONCLUSIONS: The implementation of an IDCU improved the quality of the medical care for HCM patients by performing a better study of the patients and their families.

[Mortality and associated prognostic factors in elderly and very elderly hospitalized patients with respiratory disease COVID-19]. / Mortalidad y factores pronósticos asociados en pacientes ancianos y muy ancianos hospitalizados con infección respiratoria COVID-19.

INTRODUCTION: Elderly patients with COVID-19 has a worse clinical evolution, being more susceptible to develop serious manifestations. The differences between the elderly and very elderly population, mortality and associated prognostic factors of SARS-CoV-2 infection have not been enough studied yet. METHODS: An observational study of 416 elderly patients admitted consecutively to Hospital General Universitario de Ciudad Real for COVID-19 respiratory infection from March 1st to April 30th, 2020. Data were collected including patient demographic information, medical history, clinical characteristics, laboratory data, therapeutic interventions and clinical outcomes during the hospitalization and after discharge, until June 15, 2020 with the aim of analyzing mortality, and associated prognostic factors. RESULTS: The mean age was 84.43±5.74 years old; elderly patients (75-84 years) were 50.2% of the sample and very elderly (≥85 years) the remaining 49.8%. In Cox regression model, mortality rate was higher in very elderly group (HR = 2.58; 95% CI: 1.23-5.38; P = .01), hypertensive (HR = 3, 45; 95% CI: 1.13-10.5; P = .03) and chronic kidney disease patients (HR = 3.86; 95% CI: 1.3-11.43; P = .02). In contrast, calcium antagonists (HR = 0.27; 95% CI: 0.12-0.62; P = .002) and anticoagulant therapy during hospitalization (HR = 0.26; 95% CI: 0.08 0, 83; P = .02) were associated with a longer time free of mortality. CONCLUSIONS: Mortality rate was higher in very eldery patients compared with eldery; and in hypertensive and chronic kidney disease patients. Anticoagulation therapy and calcium chanel bloquers treatment during hospitalization were associated with a higher survival in the short-term follow-up in patients hospitalized with COVID-19.

Long-Term Follow-Up of Percutaneous Coronary Intervention With Paclitaxel-Eluting Balloon Catheter.

Drug-eluting balloons currently constitute a therapeutic tool used in percutaneous coronary interventions (PCI). Long-term results remain unknown. We evaluated the prognosis of PCI using a second generation paclitaxel-eluting balloon (PEB) in real-world patients. We included all PCI with PEB in de novo or in-stent restenosis coronary lesions performed in our unit from March 2009 to March 2019. We assessed the composite of major adverse cardiovascular events (MACE) rate after a median follow-up of 42 months. Consecutive patients (n = 320) with 386 lesions were included; 46.9% presented with stable angina and 53.1% acute coronary syndromes; 52.6% of the lesions were in-stent restenosis and 47.3% de novo lesions with a mean diameter of 2.4 ± 0.5 mm. A bare metal stent was implanted in 6.7% and a drug-eluting stent in 8.5% of patients. The MACE rate was 8%: 10 (2.6%) cardiovascular deaths, 13 (3.4%) myocardial infarctions, and 16 (4.1%) target lesion revascularization. The all-cause death rate was 5.2%. No cases of thrombosis were recorded. In conclusion, PEB was a safe and effective tool to treat in-stent restenosis and de novo coronary lesions, especially small vessel disease, during long-term follow-up.

Comparative analysis between the use of renin-angiotensin system antagonists and clinical outcomes of hospitalized patients with COVID-19 respiratory infection.

INTRODUCTION: Hypertension has been associated with worse outcomes in patients with COVID-19 infection, so concerns have been raised about the possibility that inhibitors of the renin-angiotensin system (RAS) could influence the prognosis of these patients. METHODS: This is an observational study of 921 consecutive patients admitted with COVID-19 respiratory infection to Hospital General Universitario Ciudad Real from March 1 to April 30, 2020. Following data were collected including patient demographic information, medical history, clinical characteristics, laboratory data, therapeutic interventions during the hospitalization and clinical outcomes. RESULTS: The mean age was 78 years, and 59.2% of patients had a history of hypertension. Patients with previous treatment with RAS inhibitor (42.4%) showed lower risk of the primary composite endpoint (mortality or need for invasive mechanical ventilation). Treatment with RAS inhibitor (both outpatient treatment and during hospitalization) had neither effect on mortality nor need for invasive ventilation. There were no differences in time-to-event analysis between groups. CONCLUSIONS: RAS inhibitor treatment prior to admission in patients with COVID-19 respiratory infection was associated with lower risk of the primary composite endpoint and did not show neither impact on mortality nor need for invasive mechanical ventilation, even if these drugs were prescribed during hospitalization.

INTRODUCCIÓN: La presencia de hipertensión arterial se asocia con peor pronóstico en pacientes con COVID-19, y se ha sugerido que el uso de inhibidores del eje renina-angiotensina puede influir en el pronóstico de los pacientes. MÉTODOS: Registro observacional de 921 pacientes consecutivos ingresados por infección respiratoria COVID-19 entre el 1 de marzo y el 30 abril de 2020 en el Hospital General Universitario de Ciudad Real. Se registraron datos clínicos y analíticos, intervenciones terapéuticas y desarrollo de eventos durante el ingreso hospitalario. RESULTADOS: La mediana de edad fue de 78 años y el 59,2% tenían hipertensión arterial. Aunque el perfil clínico fue más desfavorable en el grupo de pacientes con prescripción previa de IECA o ARA2 respecto al resto, los primeros presentaron menor riesgo de desarrollo del evento primario combinado (mortalidad total o necesidad de soporte ventilatorio invasivo). Asimismo, el empleo previo al ingreso o durante el mismo de estos fármacos mostró un efecto neutro sobre la mortalidad total y sobre la necesidad de ventilación mecánica invasiva. En el análisis de supervivencia no se observó mayor riesgo de presentar más precozmente ninguno de los eventos registrados. CONCLUSIONES: La prescripción previa al ingreso por infección respiratoria COVID-19 de inhibidores del eje renina-angiotensina se asoció a un menor riesgo de desarrollo del evento primario combinado y a un efecto neutro sobre la mortalidad total y sobre la necesidad de ventilación mecánica invasiva.

Impact of Treatment with Renin-Angiotensin System Inhibitors on Clinical Outcomes in Hypertensive Patients Hospitalized with COVID-19.

INTRODUCTION: Concerns have been raised about the possible harmfulness of angiotensin-converter enzyme inhibitors (ACEi) and aldosterone receptor blockers (ARB) in patients with COVID-19. However, few data from a European population have been published, especially from hypertensive patients. AIM: To study the association between ACEi or ARB treatments and major adverse outcomes during hospitalisation in COVID-19 patients. METHODS: We studied 545 consecutive hypertensive patients admitted to our institution due to COVID-19 with respiratory involvement. We analysed the incidence of combined event (death or mechanical ventilatory support) during hospitalisation, as well as the time to independent events. RESULTS: 188 (34.5%) patients presented the combined endpoint. 182 (33.4%) patients died, and 21 (3.9%) needed mechanical ventilatory support. Patients with previous treatment with ACEi or ARB presented similar incidence of the combined endpoint during hospitalisation (31.6% vs. 41.8%; p = 0.08), with a lower all-cause mortality rate (30.4% vs. 41.2%; p = 0.03) compared with those without prior treatment. Use of ACEi or ARB was not independently associated with lower incidence of the combined endpoint [Adjusted OR 0.675 (95% CI 0.298-1.528; p = 0.146)], but it was associated with lower mortality [Adjusted OR 0.550 (95% CI 0.304-0.930; p = 0.047)]. CONCLUSIONS: The use of ACEi or ARB was associated with less incidence of all-cause death during hospitalisation among hypertensive patients admitted with COVID-19 respiratory infection.