Diagnosis and management of heart failure from hospital admission to discharge: A practical expert guidance.

Heart failure (HF) has high event rates, mortality, and is challenging to manage in clinical practice. Clinical management is complicated by complex therapeutic strategies in a population with a high prevalence of comorbidity and general frailty. In the last four years, an abundance of research has become available to support multidisciplinary management of heart failure from within the hospital through to discharge and primary care as well as supporting diagnosis and comorbidity management. Within the hospital setting, recent evidence supports sacubitril-valsartan combination in frail, deteriorating or de novo patients with LVEF≤40%. Furthermore, new strategies such as SGLT2 inhibitors and vericiguat provide further benefit for patients with decompensating HF. Studies with tafamidis report major clinical benefits specifically for patients with ATTR cardiac amyloidosis, a remaining underdiagnosed and undertreated disease. New evidence for medical interventions supports his bundle pacing to reduce QRS width and improve haemodynamics as well as ICD defibrillation for non-ischemic cardiomyopathy. The Mitraclip reduces hospitalisations and mortality in patients with symptomatic, secondary mitral regurgitation and ablation reduces mortality and hospitalisations in patients with paroxysmal and persistent atrial fibrillation. In end-stage HF, the 2018 French Heart Allocation policy should improve access to heart transplants for stable, ambulatory patients and, mechanical circulatory support should be considered to avoid deteriorating on the waiting list. In the community, new evidence supports that improving discharge education, treatment and patient support improves outcomes. The authors believe that this review fills the gap between the guidelines and clinical practice and provides practical recommendations to improve HF management.

Prognostic value of longitudinal strain and ejection fraction in Friedreich's ataxia.

BACKGROUND: Friedreich's ataxia (FA) is a rare autosomal recessive mitochondrial disease most commonly due to a triplet repeat expansion guanine-adenine-adenine (GAA) in the FXN gene. Cardiac disease is the major cause of death, patients with reduced left ventricular ejection fraction (LVEF) having the worse prognosis. Longitudinal strain (LS) appeared to be a better predictor of outcome than LVEF in different diseases. We compared the prognostic value of LS measured from the 4 chambers view to LVEF. METHODS: From 2003 to 2017 consecutive patients with FA were included and LS analysis was retrospectively performed. RESULTS: We studied 140 patients, with a median age of 34 (26-41) years (Q1-Q3) with age at onset of 14 (11-19) years and GAA repeats on the shorter allele of 600 (467-783) pb. Mean LS was 19.9 ± 5.0% and LVEF 64 ± 8%. After a mean follow-up of 7.4 ± 3.9 years, 14 patients died. In univariate Cox analysis, all-cause mortality was associated with: LS (HR 0.83; 95%CI, 0.75-0.91, p = 0.0002), LVEF (HR 0.30; 95%CI, 0.19-0.49, p < 0.0001), GAA repeats on the shorter allele (HR 1.29; 95%CI, 1.10-1.51, p = 0.002), age at onset (HR 0.87; 95%CI, 0.77-0.98, p = 0.018), LVSystolic Diameter (HR 1.17; 95%CI, 1.09-1.26, p < 0.0001), LVMass index (HR 1.02; 95%CI, 1.00-1.04, p = 0.027), and LVDiastolic Diameter (HR1.12; 95%CI, 1.01-1.23, p = 0.028). In multivariate analysis, LVEF was the only independent predictor of mortality (HR 0.41; 95%CI, 0.23-0.74, p = 0.0029). CONCLUSION: In FA, LS was not an independent predictor of mortality, LVEF remained the only independent predictor in the present study.

[Venous embolization--treatment of choice in varicoceles]. / La embolización venosa, tratamiento de elección en los varicoceles.

Varicocele consists of the varicose expansion of the spermatic vein. It's more frequently in the left side (10:1) as consequence of an evil functioning or absence of the valves of the spermatic left vein. Between March 1999 and December 2009 there have been gathered a total of 37 cases diagnosed of varicocele in the pediatric population of our center. After a local anesthetic we created a femoral aproach. We advance into the left renal vein using a catheter Simmons type I and then we position the top of the catheter beyond the ostium of the spermatic vein to fulfil the renal left vein by means of the administration of contrast iodized and gonadal vein could be visualized using retrograde phlebography. Transcatheter embolization will carry out across the introduction of coils (approximately from 6 to 8) of 0.038 using a hidrofilic catheter of 4 or 5F. Of the total of the 37 cases, we obtained correctly embolization in 33 patients (89.2%), being necessary a reembolization in three cases (10.8%) of which one finished in surgery. Only in a case (2.7%) surgery was practised for embolization incomplete.

A randomized study comparing isotope and echocardiography stress testing in the screening of silent myocardial ischaemia in type 2 diabetic patients.

AIMS: This study aimed to compare the positive predictive value (PPV) of stress myocardial scintigraphy (SPECT) and of dobutamine echocardiography (DE) in the diagnosis of significant coronary artery stenosis (CAD) in asymptomatic type 2 diabetic patients, and to assess long-term clinical outcomes according to silent myocardial ischaemia (SMI) screening. METHODS: A total of 204 asymptomatic type 2 diabetic patients at high cardiovascular (CV) risk were prospectively randomized to undergo either SPECT (n=104) or DE (n=100). Coronary angiography was proposed in cases of SMI, with revascularization of suitable lesions. Intensive treatment of CV risk factors was prescribed for all patients. Death and myocardial infarction (MI) were recorded during the 3-year follow-up. RESULTS: Clinical characteristics were similar in the two testing groups. The prevalence of SMI and significant CAD were 13% and 4%, respectively, in the SPECT group vs 11% and 5%, respectively, in the DE group (not significant [NS]). The PPV for the detection of significant CAD was 29% for SPECT and 45% for DE (NS). Seven patients (3%) underwent initial revascularization. The 3-year rate of CV death and MI was 2.5%, and similar in both groups. CONCLUSION: Rates of SMI and significant CAD in asymptomatic high-risk type 2 diabetic patients receiving intensive care of risk factors are low, and SPECT and DE are similar in the detection of SMI and CAD. Coronary revascularization and intensive CV risk-factor therapy are associated with a low rate of adverse CV events at 3 years, whichever stress test was used.

Is the gap between guidelines and clinical practice in heart failure treatment being filled? Insights from the IMPACT RECO survey.

BACKGROUND: Recent registries have shown that recommended drugs for the treatment of chronic heart failure (CHF) are under-prescribed in daily practice. AIMS: To determine prescription rates of CHF drugs, and to assess predictive factors for drug prescription using data from a large panel of French cardiologists. METHODS AND RESULTS: We included 1919 outpatients, with NYHA class II-IV heart failure and a left ventricular ejection fraction <40%. The most frequently prescribed drugs were diuretics (83%), angiotensin converting enzyme inhibitors (ACE-I) (71%), beta-blockers (65%), spironolactone (35%) and angiotensin receptor blockers (ARB) (21%); 61% of patients received a combination of a beta-blocker and an ACE-I or ARB. Target doses were reached in 49% of the patients for ACE-I, but in only 18% for beta-blockers and in 9% for ARBs. Multivariate analyses showed that age >75 years was an independent factor associated with under-prescription of ACE-I-ARBs, beta-blockers or spironolactone. Renal failure was associated with a lower prescription of ACE-I-ARB and spironolactone, and asthma was a predictor of under-prescription of beta-blockers. CONCLUSIONS: In this contemporary survey, prescription rates of CHF drugs were higher than previously reported. However, dosages were lower than those recommended in guidelines. Age remained an independent predictor of under-prescription of CHF drugs.

Low negative predictive value of dobutamine stress echocardiography before abdominal aortic surgery.

BACKGROUND: According to previous studies, a negative dobutamine stress echocardiography (DSE) test before major vascular surgery indicates that postoperative myocardial necrosis is very unlikely. We believe that the use of new cardiac troponin assays which can detect small amounts of myocardial necrosis results in a lower DSE negative predictive value for myocardial necrosis. METHODS: A total of 418 consecutive patients were screened using the ACC/AHA Guideline for Perioperative Cardiovascular Evaluation for Noncardiac Surgery before scheduled abdominal aortic surgery. Of these 143 met ACC/AHA criteria for non-invasive testing and underwent DSE. Patients with a negative DSE were deemed to be fit for surgery. A positive DSE led to a coronary angiogram. DSE was negative in 110 (77%) and positive in 33 (23%) patients. Myocardial necrosis was monitored up to the third postoperative day by daily cardiac troponin I (cTnI) measurement and a daily 12-lead ECG. RESULTS: Coronary angiography showed artery stenosis in 27 (84%) of 32 patients with a positive DSE. The negative predictive value of DSE for cTnI elevation was 92.7% (95% CI 86.2-96.8%). This was significantly lower than the lowest value of negative predictive value for myocardial necrosis assessed in previous studies. CONCLUSION: A negative DSE prescribed before scheduled aortic surgery according to ACC/AHA guidelines does not rule out postoperative myocardial necrosis.

[Treatment of obstructive aortic atheroma in homozygotic familial hypercholesterolemia]. / Traitement de l'athérome aortique obstructif au cours de l'hypercholestérolémie familiale homozygote.

The object of this report is to describe the surgical treatment of a rare clinical form of homozygotic familial hypercholesterolaemia (HFH) associating valvular and supravalvular stenosis with coronary ostial stenosis. Three patients, two male and one female, aged 15, 23 and 41 respectively, suffering from HFH diagnosed in early childhood, presented with obstacles to left ventricular ejection and myocardial ischaemia due to coronary ostial stenosis. Surgery consisted of corrections in a single procedure of all abnormalities by aortic valve replacement, ascending aortic replacement and widening of the coronary artery ostia which were reimplanted on the aortic tube. The postoperative course of all three patients was favourable. Postoperative echocardiography showed the normal position of the valvular prosthesis, normalisation of the left ventricular ejection fraction with no significant residual obstruction. Angioscan of the coronary arteries showed a good result of coronary ostial widening. The authors conclude that HFH is a rare condition and that disease of the ascending aorta is common in this variety with involvement of the aortic valve, the ascending aorta and the coronary ostia. The surgical procedure described by the authors allows correction of all the abnormalities with the hope of a good long-term result.

[Preoperative cardiac assessment using dobutamine stress echocardiography]. / Evaluation du risque cardiaque avant chirurgie programmée par l'échocardiographie dobutamine.

Postoperative myocardial ischaemia is the leading cause of life expectancy impairment after high cardiac risk surgical procedures. Preoperative identification of patients at high risk for such complication helps reducing its postoperative incidence through therapeutic adjustments. The former relies upon preoperative selection of patients who are candidates for cardiac testing using dobutamine stress echocardiography, according to ACC/AHA guidelines. This exam evaluates echographic myocardial response to a pharmacological stress induced by dobutamine infusion. Its aim is to reproduce part of the stress the myocardium will undergo during surgical procedure. A stress induced myocardial ischaemia suggests such a complication could occur postoperatively. A positive dobutamine stress echocardiography justifies to prescribe preoperative anti-ischaemic treatment in order to reduce the cardiac risk of the further surgical procedure. Moreover, it justifies clear definition of perioperative haemodynamic objectives. Whatever the result of the dobutamine stress echocardiography, cardiac ischaemia should be monitored up to the third postoperative day on the basis of a daily 12-lead electrocardiogram recording and daily plasmatic troponin Ic measurement.

[Laparoscopy in chronic abdominal pain in children]. / La laparoscopia en el dolor abdominal crónico en la infancia.

UNLABELLED: The recurrent chronic abdominal pain (RAP) is one of the most usual pathologies in pediatrics. It may appear in the form of periodical or continuous sharp crisis. In order to be accepted as chronic, the presence of this pain must last at least 3 months. In many of the reference publications, only between 5 and 1% of the cases are considered to have an organic ethiology and the rest are labelled as functional processes of psychological origin. The reason for our communication is to show our experience on 304 RAP patients from 1995 to 2001. We also want to highlight the usefulness of laparoscopy in order to diagnose and treat 14.45% (44) of cases of this group of patients. MATERIAL AND METHODOLOGY: 304 patients between 5 and 14 years old were studied from 1995 to 2001. We followed a protocol of: medical and personal medical record, specific anamnesis for RAP, detailed description of the diet and intestinal habits, physical exploration. Laboratory: 1) blood analysis, hepatic profile, glucose, cholesterol, creatinine, amylase, 2) urine sediment and may be urine culture, 3) faeces analysis and 4) vaginal flow analysis. Image diagnosis is: simple abdomen radiography, ultrasonography and intestinal Rx. Guided and specific determinations: spired urea test, gastric chemism, lactose test, gastroscopy and biopsy, colonoscopy and biopsy, laparoscopy. RESULTS: In short, we can say: 74 patients (23.4%) come with inappropiate feeding diets, 31 (10.1%) with helicobacter pilori, 20 (6.5%) with adenoids, pharynx and pharyngeal processes, the same figure from gynaecologic origin, 18 (5.9%) from psychological origin, and in 44 cases (14.45%) laparoscopy was indicated. CONCLUSIONS: The anatomopathological study in the cases in which we carried out laparoscopic appendicectomy does not allow us to accept the symptoms of chronic appendicitis. The exploratory laparoscopy and the appendectomy have meant benefits for the solution to this pathology in 14.45% of patients. It is essential to carry out a long-term follow-up, at least 12 months, to be sure RAP is cured. We must carry on looking for aetiological cause of RAP.

[Surgical treatment of a pheocromocytoma bilateral in a 5 year old patient with the von Hippel-Lindau disease]. / Tratamiento quirúrgico de un feocromocitoma bilateral en un paciente de 5 años con enfermedad de von Hippel-Lindau.

INTRODUCTION: The disease of Von Hippel Lindau (VHL) is hereditary and causes a predisposition to the development of tumours. Organs such as the cerebellum, the pancreas, the kidney, the suprarenal glands and the retina are more usually affected by this disease. CLINICAL CASE: We present the case of a 5-year-old patient who suffers from asiymptomatic high blood pressure. In the family antecedents, it is relevant the case of the father, with pheocromocytoma bilateral, which led us to carry out a genetic study of his two sons. Our patient, the younger; presented a mutation of the VHL gene in the short arm of the chromosome 3. In one of the periodic controls, it could be detected high blood pressure of 160/100 mm. Hg, clinically asymptomatic. The other child did not present a genetic mutation and has no disease. The presence of high catecholamines, the detection of a 3 cm left suprarenal mass through the ecography, the TAC that did not show a right suprarenal pathology and the MBIG scintigraphy confirmed the diagnostic of pheocromocytoma. The RNM showed another 0.8-cm mass which confirmed a pheocromocytoma bilateral. We started the treatment against high blood pressure with fenoxibenzamine and diltiazem, and we controlled this problem. We also prepared the pre-and-post operation anesthetic strategy, which is so important for the surgical success. The operation started by a laparoscopic, we made left adrenalectomy and we had to reconvert to laparotomy to make partial right adrenalectomy. Six months after the operation, the patient is free from symptomatology and follows a treatment with glucocorticoides with smaller and smaller doses. COMMENTS: The case is exceptional because it embodies the following characteristics: early diagnostic age, family affectation and discovery of asymptomatic high blood pressure. It needed an appropriate preanesthetic and anesthetic preparation, which gave way to an operation without complications. The postoperation was also stable and presented no complications.

Clinical applications of B-type natriuretic peptide (BNP) testing.

Many claims have been made in recent years regarding the utility of plasma B-type natriuretic peptide (BNP) concentration measurements in the diagnosis, risk stratification and monitoring of patients with heart failure. This paper summarizes the current evidence and provides guidance for practising clinicians. Overall, plasma BNP testing appears to be of most value in the diagnostic arena, where it is likely to improve the performance of non-specialist physicians in diagnosing heart failure. In clinical practice, BNP testing is best used as a 'rule out' test for suspected cases of new heart failure in breathless patients presenting to either the outpatient or emergency care settings; it is not a replacement for echocardiography and full cardiological assessment, which will be required for patients with an elevated BNP concentration. Although work is ongoing in establishing the 'normal' values of BNP, heart failure appears to be highly unlikely below a plasma concentration of 100 pg/ml. However, as BNP levels rise with age and are affected by gender, comorbidity and drug therapy, the plasma BNP measurement should not be used in isolation from the clinical context.

[Intestinal myopathy in Steinert's disease]. / Miopatía intestinal en la enfermedad de Steinert.

The myothonic dystrophy or Steinert's disease is a congenital, autosomal, dominant disorder which seriously affects the striated muscle and also to a certain extent, several organs and systems and on rare occasions, the intestinal smooth muscle. In the case, we treated a four years old girl in whom Steinert's disease was diagnosed when she was born and who developed the characteristics of severe constipation after a few months of life. The ano-rectal manometry showed a paradoxical reaction of external Sphincter with a normal inhibitory reflex. The rectal biopsy revelated a miophathy which affected the muscularis propia with the normal neuronal innervation. The X-ray studies showed that motility disorder was stopped in the distal area of the left colon. A colostomy in the healthy zone worked extremely well. Six months later, the normal colon was brought down retro-rectal. Only a partial incontinence remained in the external sphincter caused by Steinert's disease. The interest of this case lies in the pathological association, striade muscle and smooth muscle, in such an early age of life. It is a new contribution to the complex chapter of the intestinal pseudo-obstruction.

[Usefulness of transrectal ultrasonography in the diagnosis of anomalies of intersexual conditions]. / La utilidad de la ecografía transrectal en el diagnóstico de las anomalías de los estados intersexuales.

INTRODUCTION: Between the external genital exploration and the internal genital direct vision, through laparoscopia, there is a black point which is the pelvic floor; we have to know whether there is a vagina or not, what it is like and where it arrives at. The reason for this research is to present our experience with the transrectal ecography, which allows us a very good exploration of the pelvic floor. MATERIAL AND METHODS: We present 6 patients, the youngest is 16 months old and the oldest is 19 years old, who have a diagnostic of 3 congenital adrenal hyperplasia (HSC), 2 gonadal dysgenesis and 1 vaginal agenesis (S. Rokitanski). Under sedation, we carried out a transrectal ecography with Aloka SSD650 ecograph and 7.5 MHz vaginal scanner. Ultrasone. RESULTS: In case of HSC (16 months old), the transrectal ecography showed the vaginal arrival at the urethra and we were able to measure the distance from the external sphincter. In the other two HSC, which had been surgically corrected, the transrectal ecography clearly showed the vagina (length and calibre). In the two gonadal dysgenesis (two 11-and-19-year-old sisters, the first of whom had undergone vulvo-vaginoplasty, we appreciated the length of the vagina and, in the case of the sister with a relatively normal vagina, we confirmed the presence of the vagina connected to the uterus. In the vaginal agenesis, in which a neovagina with amnion membrane had been carried out, which, in its turn, ended in a situation of hematometra due to a stenosis, the transrectal ecography was really helpful to obtain vaginal dilatations. CONCLUSIONS: Ecography is an easily available technique and provides both through and detailed information of the genital structures going through the pelvic floor, a zone which is otherwise difficult to explore. It was done under sedation for the patient's age and idiosyncrasy. The experience has just started but we are sure that in the future it will replace the genitograma.

Accuracy of European diagnostic criteria for familial hypertrophic cardiomyopathy in a genotyped population.

BACKGROUND: Since the sensitivity of conventional diagnostic criteria for familial hypertrophic cardiomyopathy (HCM) is low, new diagnostic criteria were proposed by a European collaboration. However, their diagnostic value remains unknown. The aim of the study was to evaluate the accuracy of these new criteria, using the genetic status as the criterion of reference. METHODS: We studied 109 genotyped adults (54 genetically affected, 55 unaffected) from 7 families (mutations in 3 genes). Major European echographic criteria were a maximal wall thickness >or=13 mm or >or=15 mm according to the segment involved, or the presence of SAM. Major European ECG criteria were abnormal Q waves, left ventricular hypertrophy, or marked ST-T changes. Combined major/minor European criteria were also evaluated. RESULTS: Sensitivity and specificity of major European criteria (72 and 92%, respectively) were similar to those of major conventional criteria (70 and 94%) and were not improved by combined major/minor European criteria (72 and 90%). When all the minor European criteria were considered, sensitivity increased to 87% but specificity dramatically decreased to 51%. However, one of these minor ECG criteria, deep S V2, was of interest and when added to major European criteria, sensitivity increased to 76% and specificity remained good (90%). CONCLUSIONS: The diagnostic value of new European criteria for HCM was evaluated for the first time. We found that it was not different from that of conventional criteria, with a good specificity but a low sensitivity. Additional criteria should be studied to improve the early identification of HCM.

[Prognostic value of neurohormones in heart failure in clinical practice]. / Valeur pronostique des neuro-hormones dans l'insuffisance cardiaque en pratique clinique.

The decisive therapeutic advances achieved in cardiac insufficiency in recent years have been thanks to drugs affecting the different neurohormonal systems in operation. Neurohormonal activation plays a major role in cardiac insufficiency. Several neuro-endocrine mechanisms exert vasoconstrictor effects: the sympathetic system, the renin-angiotensin-aldosterone system and the endothelins. In cardiac insufficiency these effects are counterbalanced, but insufficiently, by vasodilatory agents, mainly the natiuretic peptides, EDRF (endothelium derived relaxing factor), vasodilatory prostaglandins, bradykinin and adrenomedulin. Neurohormonal activation is an excellent marker of not only the severity but also the prognosis of cardiac insufficiency. Standardisation of dosage is desirable in order to allow the use of neurohormonal drugs in a very large number of centres.