Les myocardites aiguës compliquant les maladies inflammatoires chroniques de l’intestin : à propos de deux observations

Introduction

Acute myocarditis is one of the less common extra-intestinal manifestations of inflammatory bowel diseases (IBD). It may reveal IBD or occur during the follow-up.

Case reports

We report two patients who presented an acute myocarditis associated with ulcerative colitis that reveal the disease in one of them. Clinical features at presentation were non specific and both patients presented with chest pain. Diagnosis was obtained with cardiac MRI. Outcome was favourable in the two patients.

Conclusion

Etiologies of myocarditis are various and the diagnosis of extra-intestinal manifestations related to IBD remains a diagnosis of exclusion. Cardiac MRI is an important diagnostic tool. Colchicine and acétylsalicylic acid are commonly prescribed in a first therapeutic approach.     

Une cause réversible de la cardiomyopathie dilatée : l’hypocalcémie

Background

Hypocalcemia is very rare reversible cause of dilated cardiomyopathy (DCMP) witch can concern one or both ventricules. We here presented two cases of DCMP that caused by hypocalcemia and recovered totally after oral calcium and vitamin D supplementation.

Case presentation 1

A 29-year-old Caucasian female was admitted in our hospital due to congestive heart failure with dyspnea (NYHA class IV) and generalized edema for 2 days. She had a history of total thyroidectomy one year a go. She had taken synthyroid as a daily medication associated to calcium supplementation and vitamin D because of hypoparathyroidism. Patient was not compliant to treatment. Trans thoracic echocardiography (TTE) showed a dilated left ventricule (LV) with global hypokinesia with 28% of left ventricule ejection and moderate mitral regurgitation. Laboratory test showed a severe hypocalcemia. After correction of hypocalcemia, there was a clear clinical improvement and four months later a total recovery was found.

Case presentation 2

A 44-year-old Caucasian male was referred to the hospital for rebel congestive heart failure with dyspnea and edema of lower limbs despite optimal treatment. Patient had no medical past history. TEE showed dilated cardiomyopathy with severe alteration of left ventricule systolic function (25%). Biological tests showed a hypocalcemia and primary hypoparathyroidism. An improvement of symptoms after correction of metabolic disorder was found. LV gradually recovered its performance.

Conclusion

Patients outcome in end stage heart failure is different from the classical outcomes in patients with hypocalcemia induced heart disease. DCMP induced by hypocalcemia should be considered in patients with heart failure associated with medical conditions leading to hypocalcemia. It requires a specific treatment.     

Une cause de cardiomyopathie dilatée chez l’enfant : le déficit primaire en carnitine

Aim

The aim of this case report was to show the importance to research metabolic etiology, especially a carnitine deficiency in dilated cardiomyopathy of children.

Case report

A three years old Togolese child presented muscular hypotonia, dyspnea. Examination showed left galop murmur and systolic murmur 2/6. Chest X-ray showed cardiomegaly (CTI: 0.66), electrocardiogram, a sinusal rythm, left ventricle hypertrophy and T wave abnormalities. Echocardiogram showed a markedly dilated left ventricle with reduced systolic function (EF: 0.43; reference range 0.55–0.80) and moderate mitral regurgitation. The inflammatory signs where negatives. Magnetic resonance imaging don’t show signs of ischemic or myocarditis. The levels of free and total plasmatic carnitine decreased: 3 μmol/L (N: 18–48 μmol/L) and 5 μmol/l (N: 29–70 μmol/L) respectively. Mutation analysis of the gene SLC22A5 confirms the diagnosis of primary systemic carnitine deficiency. Treatment with oral carnitine was started at 200 mg/kg per day. Within three weeks of treatment, we observed the decrease of all symptoms and the left ventricular size and function normalized (EF: 0.62). He has now been on oral carnitine for live.

Conclusion

Primary carnitine deficiency is a cause of dilated cardiomyopathy in child. It must systematically be suspected when a child presents a primitive cardiomyopathy. The treatment with oral carnitine for live is simple, with excellent prognosis.     

Cardiovascular magnetic resonance imaging in asymptomatic patients with connective tissue disease and recent onset left bundle branch block

Background–aim

Recent LBBB in connective tissue diseases (CTDs) is challenging, due to high incidence of underlying pathology that may remain undetected, due to limitations of imaging tests. We hypothesized that cardiovascular magnetic resonance (CMR) may be of diagnostic value in CTDs with recent LBBB and normal echocardiogram.

Patients–methods

26 CTDs, aged 32 ± 7 yrs (19 F) and 26 controls without CTDs, aged 60 ± 4 yrs (10 F) with recent LBBB and normal echo were evaluated by CMR. The CTDs included 6 sarcoidosis (SRC), 4 systemic sclerosis (SSc), 6 systemic lupus erythematosus (SLE), 6 rheumatoid arthritis (RA) and 4 inflammatory myopathies (IM). CMR was performed by 1.5 T. LVEF, T2 ratio (oedema imaging) and late gadolinium enhancement (LGE) (fibrosis imaging) were evaluated. Acute and chronic lesions were characterised by T2 > 2 and positive LGE and T2 < 2 and positive LGE, respectively. According to LGE, lesions were characterised as diffuse subendo-, subepicardial/intramural not following and subendocardial/transmural following the distribution of coronaries, indicative of vasculitis, myocarditis and myocardial infarction, respectively.

Results

CTDs were younger (p < 0.001), with higher incidence of abnormal CMR (42.31 vs 30.77%, p = NS), including dilated cardiomyopathy (11.54%), diffuse subendocardial fibrosis (11.54%), myocardial infarction (7.69%) and acute myocarditis (11.54%) vs dilated cardiomyopathy (19.23%), myocardial infarction (7.69%) and acute myocarditis (3.85%), detected in non-CTDs.

Conclusions

In CTDs with recent LBBB, CMR documented acute and chronic cardiac pathology, particularly myocarditis. CMR should be considered as an adjunct to conventional diagnostic workup in both patient groups, more so in CTDs.     

Agreement between ST elevation and late enhancement evaluated by MRI in patients with acute myocarditis

Background

ECG is widely used in the evaluation of patients with acute myocarditis. Magnetic resonance imaging (MRI) has emerged as the most important imaging tool in the diagnosis of myocarditis. The objective of this study is to determine the agreement between ECG and MRI findings in patients with acute myocarditis.

Methods

This is a retrospective cohort that includes 32 consecutive patients with acute myocarditis. ST elevation (STE) in mm was registered in every ECG lead. In every myocardial segment the presence of late enhancement (LE) was registered.

Results

STE was found in 75% of the patients, with the inferolateral region being the most frequently affected (46.9%). LE was found in most of the patients (87.5%); the inferolateral wall was also the most frequently affected (50%). There was a moderate agreement between the inferolateral localization of STE and LE in patients with acute myocarditis, k = 0.43, p = 0.01. There was no agreement for the other localizations.

Conclusion

There was a moderate agreement between the localization of STE and LE only in the inferolateral localization. LE localization based on the STE localization cannot be inferred, neither vice versa in another localization different from the inferolateral.     

Manifestations cardiaques au cours la granulomatose de Wegener : à propos de quatre observations et revue de la littérature

Introduction

Discordance exists between the results of post-mortem studies and the low number of clinical reported cases of cardiac involvements in Wegener's granulomatosis.

Case reports

Data from four patients were studied retrospectively. Three patients had associated airway localization and three had kidney involvement. All patients had positive test for anti-PR3 antineutrophil antibodies. Two patients presented with dilated cardiomyopathy (one with terminal cardiac failure), another patient with complete atrioventricular block and pericarditis, and the remaining one with myopericarditis. One patient was asymptomatic. For three of these patients, the cardiac manifestations were contemporary of the diagnosis of Wegener's granulomatosis and had a severe disease course.

Conclusion

Cardiac events in Wegener's granulomatosis are probably underestimated, given the various type of heart damage and the clinical presentation. Cardiac involvement seems to be associated with a poor prognosis. Thus, we recommend systematic and regular cardiac assessment in the follow-up of patients with Wegener's granulomatosis.     

Prevalence of myocarditis and cardiotropic virus infection in Africans with HIV-associated cardiomyopathy,idiopathic dilated cardiomyopathy and heart transplant recipients: a pilot study

Background:

The prevalence of myocarditis and cardiotropic viral infection in human immunodeficiency virus (HIV)-associated cardiomyopathy is unknown in Africa.

Methods

Between April 2002 and December 2007, we compared the prevalence of myocarditis and cardiotropic viral genomes in HIV-associated cardiomyopathy cases with HIV-negative idiopathic dilated cardiomyopathy patients (i.e. negative controls for immunodeficiency) and heart transplant recipients (i.e. positive controls for immunodeficiency) who were seen at Groote Schuur Hospital, Cape Town, South Africa. Myocarditis was sought on endomyocardial biopsy using the imunohistological criteria of the World Heart Federation in 33 patients, 14 of whom had HIV-associated cardiomyopathy, eight with idiopathic dilated cardiomyopathy and 11 heart transplant recipients.

Results

Myocarditis was present in 44% of HIV-associated cardiomyopathy cases, 36% of heart transplant recipients, and 25% of participants with idiopathic dilated cardiomyopathy. While myocarditis was acute in 50% of HIV- and heart transplant-associated myocarditis, it was chronic in all those with idiopathic dilated cardiomyopathy. Cardiotropic viral infection was present in all HIV-associated cardiomyopathy and idiopathic dilated cardiomyopathy cases, and in 90% of heart transplant recipients. Multiple viruses were identified in the majority of cases, with HIV-associated cardiomyopathy, heart transplant recipients and idiopathic dilated cardiomyopathy patients having an average of 2.5, 2.2 and 1.1 viruses per individual, respectively.

Conclusions

Acute myocarditis was present in 21% of cases of HIV-associated cardiomyopathy, compared to none of those with idiopathic dilated cardiomyopathy. Infection with multiple cardiotropic viruses may be ubiquitous in Africans, with a greater burden of infection in acquired immunodeficiency states.     

Importancia de los hallazgos de la resonancia magnética cardiaca en el diagnóstico de la miocardiopatía arritmogénica del ventrículo izquierdo

Introduction and objectivesLeft dominant arrhythmogenic cardiomyopathy (LDAC) has recently been recognized as falling on the spectrum of arrhythmogenic cardiomyopathy. It is characterized by fibroadipose replacement of the left ventricle. The aim of this study was to describe the most frequent forms of clinical presentation of LDAC, imaging findings, and events at follow-up, highlighting the importance of cardiac magnetic resonance (CMR).MethodsProspective registry of patients with findings compatible with LDAC. CMR image analysis and clinical follow-up was performed. The primary endpoint was the appearance of major adverse cardiovascular events (MACE) during follow-up, defined as sudden cardiac death, sustained ventricular arrhythmias, and heart transplant.ResultsWe included 74 consecutive patients (mean age, 48.6 years; 50 men [67.6%]). The most frequent CMR indications were chest pain with normal coronary angiography, ventricular arrhythmias, and suspicion of cardiomyopathies. The main CMR findings were midwall and/or subepicardial pattern of late gadolinium enhancement (91.9%), fatty epicardial infiltration (83.8%), and left ventricle segmental contractility abnormalities (47.9%). At a mean follow-up of 3.74 years, 24 patients (32.4%) had a MACE (sudden cardiac death 8.1%, sustained ventricular arrhythmias 21.6%, and heart transplant 4.1%). Independent predictors for the appearance for MACE were a CMR study showing severe late gadolinium enhancement, male sex, and practicing sports.ConclusionsCMR is a key tool for diagnosing LDAC. Characteristic findings are subepicardial fatty infiltration and midwall-subepicardial late gadolinium enhancement. The prognosis of this population is poor with a high incidence of sudden cardiac death and ventricular arrhythmias.     

Intérêt de l’imagerie par résonance magnétique nucléaire au cours de l’atteinte cardiaque du syndrome de Churg-Strauss. Trois observations et revue de la littérature

Purpose

The reported prevalence of cardiac complications is variable in patients with Churg-Strauss syndrome (15–92%) and depends on diagnostic tools. Diagnosis at early stage of heart involvement is crucial, resulting in appropriate management.

Methods

We report three patients who developed cardiac manifestations, revealing Churg-Strauss syndrome. The diagnosis of cardiac involvement was obtained using cardiac magnetic resonance imaging (MRI).

Results

Two patients were males and the remaining one was a female. Presenting clinical manifestations were: cardiac failure (n = 1) and retrosternal pain (n = 2). Laboratory findings disclosed: high blood count of eosinophils (range: 6000–11 000/mm³); antineutrophil cytoplasmic antibodies were positive in a single patient. Cardiac MRI demonstrated: (1) late gadolinium enhancement (n = 3), involving mainly the apical and mid-cavity left ventricular segments; (2) impaired left ventricular function (n = 2), mean left ventricular ejection fraction being: 51%; and (3) pericardial effusion (n = 3). Outcome was favourable after institution of combined therapy with prednisone and cyclophosphamide (n = 2); one patient also underwent plasma exchanges.

Conclusion

Our case series underlines that MRI is a helpful tool in the diagnosis of Churg-Strauss syndrome-related cardiac complications. We further suggest that clinical assessment of patients with Churg-Strauss syndrome should include cardiac MRI, in order to detect cardiac involvement at an early stage; indeed, because cardiac manifestations are predictive factors of poor prognosis, diagnosis at early stages of cardiac involvement may result in improvement of patients management.     

Insuffisances cardiaques du sujet jeune : aspects épidémiologiques,cliniques et étiologiques au CHU Sylvanus Olympio de Lomé

Aims

The aim of the study was to analyze the epidemiologic, clinical and etiologic aspects of heart failures of the young age to 18 to 45 years.

Patients and method

It consisted of a cross-sectional study realized in hospitalization in the service of cardiologic of Teaching Hospital Sylvanus Olympio of Lomé on 35 months (January 2009 to November 2012). The completion of a Doppler echocardiography was necessary to include the patients in the study.

Results

The prevalence heart failure in the 45 years old young people to more was of 28.6%. The median age of the patients was of 36.5 ± 3 years with a sex ratio of 10.7. Heart failure was total among 268 patients (71.3%). The electrocardiogram found 88 patients (23.4%) in complete arrhythmia by auricular fibrillation. Cardiac echography found a dilation of the left ventricle among 271 patients (72.1%), a systolic dysfunction of left ventricle among 213 patients (56.6%) and an intracavitary thrombus among 37 patients (9.8%). The etiologies were: hypertension 161 cases (42.8%), heart valve diseases 68 cases (18.1%), the peripartum cardiomyopathy 58 cases (15.4%), dilated cardiomyopathy 22 cases (5.8%), the alcoholism 12 cases (3.1%), ischaemic heart diseases 10 cases (2.7%), congenital heart diseases 10 cases (2.7%), the chronic pulmonary heart 8 cases (2.1%), the cardiothyreosis 7 cases (1.8%), the pericardial tamponnade 4 cases (1.1%) and myocarditis with VIH 4 cases (1.1%). Hospital lethality was of 16.4% (62 patients).

Conclusion

Heart failure is a serious and frequent pathology in Africa. It affects young and active subjects. The causes are dominated by hypertension.     

Arrhythmogenic cardiomyopathy. Patterns of ventricular involvement using cardiac magnetic resonance

Introduction and objectives

Biventricular arrhythmogenic cardiomyopathy and left dominant arrhythmogenic cardiomyopathy forms had recently been included in the spectrum of arrhythmogenic cardiomyopathy. The aim of the study was to describe, using cardiovascular magnetic resonance, the patterns of ventricular involvement as well as late gadolinium enhancement in these conditions.

Methods

Medical databases and records from the cardiology units of 3 hospitals were reviewed to obtain data from patients with arrhythmogenic cardiomyopathy.

Results

Twenty-six consecutive patients were included (40 [16] years, 16 males). Right ventricle involvement was present in 19 patients (73%). Among them, 13 patients (50%) had volumes over the upper limit of normality, 11 (42%) patients had late gadolinium enhancement in right ventricle and 6 patients (23%) had just mild involvement with wall motion abnormalities or microaneurysms. Left ventricle involvement was present in 24 patients (92%), all of them with late gadolinium enhancement. In 15 patients (57%) left ventricular systolic dysfunction was observed, and dilatation in 3 patients (11%). Late gadolinium enhancement was more frequent in the inferior, lateral, and inferolateral walls (65%, 57%, and 61% of patients, respectively) while septum was seldom affected (26% of cases). The pattern of late gadolinium enhancement was mainly subepicardial (46% of patients) or transmural (19%), and was intramyocardial in only 12% of the cases.

Conclusions

In this sample, left ventricle involvement is very common. The most frequent finding was left ventricular late gadolinium enhancement, while the least frequent was dilatation. The pattern of late gadolinium enhancement was more frequently subepicardial and located in the inferior and inferolateral walls.Full English text available from:www.revespcardiol.org     

Manifestations cliniques et biologiques de la primo-infection à parvovirus B19 chez l’adulte immunocompétent : étude rétrospective de 26 observations

Purpose

Parvovirus B19 causes erythema infectiosum in children, transient aplastic anemia in patients with hemoglobinopathies, pur red cell aplasia in immunocompromised persons and hydrops fetalis in pregnancy. The spectrum of clinical and biological manifestations in immunocompetent adult continues to grow up.

Methods

We report on a case series of 26 patients with primary parvovirus B19 infection in immunocompetent adults. This is a retrospective study over the period 2000 to 2010 in two departments of internal medecine. The diagnostic was clinical, serological or molecular.

Results

There was a female predominance (sex-ratio 3.33/1). Median patient age at diagnostic was 38.8 years (range: 18–68). The predominant symptoms were fever (65%), peripheral and symmetrical polyarthralgia (62%) and skin rash (58%). Two patients had neurological manifestations (sixth cranial nerve palsy, distal paresthesia) and one patient had myocarditis. Abnormal laboratory values included increased acute phase reactants (73%), thrombocytopenia (43%), lymphopenia (38%) and elevated liver enzymes (37%). Antinuclear (19%), anti-DNA (28%) and anti-phospholipids antibodies (14%), and hypocomplementemia (32%) were observed. False reaction with anti-CMV and anti-EBV IgM positivity was documented in 27% of cases. Two patients had persistent parvovirus B19 infection.

Conclusion

The diversity of the clinical manifestations of parvovirus B19 infection may be misleading for the clinician. However, the diagnosis should be suspected in immunocompetent adults to limit the risk of transmission to the patients who could develop a severe infection such as pregnant women or immunocompromised patients.     

Troubles du rythme et intolérance musculaire à l’effort chez un adulte jeune révélant une laminopathie

Introduction

Arrhythmic disorders are infrequent in young adult and should evoke myopathy associated cardiomyopathy, even though muscular symptoms are moderate or absent.

Case report

We report a 25-year-old woman who developed severe supraventricular rhythm disturbances with exercise intolerance and elevated serum creatine kinase level. Initially the echocardiography showed normal ventricular function. Mutation in the lamin gene (LMNA) was identified. During the disease course, arrhythmia and ventricular function worsened and required cardioverter defibrillator implantation.

Conclusion

Laminopathies are genetic disorders among which dilated cardiomyopathy associated with skeletal muscular involvement is the most frequent phenotype, usually like Emery-Dreifuss muscular dystrophy. Other phenotypes are progeria, lipodystrophic syndromes and peripheral neuropathy. Cardiac involvement is responsible for syncope, thromboembolic events and sudden death and often requires early cardioverter defibrillator implantation.     

Nécrose aiguë de l’œsophage

Introduction

Acute necrosis of the esophagus, frequently referred to as black esophagus is a rare clinical entity.

Case report

We here report a case of an acute necrosis of the esophagus secondary to hemodynamic compromise after total hip replacement. Past medical history of our 72-year-old patient was remarkable for coronary heart disease, obstructive arteriopathy of the lower limbs, diabetes mellitus and hypertension. He was referred for hematemesis and epigastric pain one day after the surgical intervention was performed. Gastric endoscopy showed necrosis of the esophagus. Treatment consisted on intravenous proton pump inhibitor, parenteral renutrition, and red blood cell transfusion. Fours days later, endoscopy found complete disappearance of necrosis.

Conclusion

Black esophagus develops in debilitated patients during hypoperfusion and stress. The outcome is usually favourable in the absence of comorbidities.     

Intérêt du coroscanner en urgence pour l’exclusion de la maladie coronaire chez les patients souffrant de douleur thoracique à risque faible ou intermédiaire

Purpose

To demonstrate the usefulness of the multislice computer tomography coronary angiography (CCTA) in patients with suspected acute chest pain without electrical changes or enzyme rise, and with low cardiovascular risk.

Patients and methods

Fifty-three patients at low or intermediate risk for coronary artery disease, who were admitted in the emergency department for an acute chest pain, and who underwent a CCTA, were included in the study. Results of the CCTA were classified as normal, non-obstructive stenosis (≤ 50% stenosis in diameter), obstructive stenosis (> 50% stenosis in diameter). The mortality was assessed during a 4-years follow-up period.

Results

Mean age was 61 years (36–86), 43% of patients were women. The CCTA was normal in 35 patients (66%), seven patients (13%) had non-obstructive stenosis and 11 (21%) had obstructive stenosis. In the group of normal CCTA, 8.5% of patients were admitted in cardiac intensive care unit, 57.1% in the non-obstructive stenosis and 90.9% in the group of obstructive stenosis. No deaths occurred during the 4-year follow up in the group of patients with normal CCTA.

Conclusion

This study confirms the negative predictive value of CCTA for the diagnosis of coronary artery disease and for further clinical events in patients at low or intermediate risk referred to emergency department for an acute chest pain.     

Rupture aiguë post-traumatique de l’aorte thoracique en double foyer

Purpose

Traumatic thoracic aortic rupture are commonly localised in one site essentially in the aortic isthmus but multiple localisation are not uncommon. The authors reported the case of a young man who had a double localisation of RTA after a violent car accident.

Case report

A 23-year-old man had a violent car crush involving sudden deceleration. He had multiple injuries essentially: a traumatic thoracic injury with acute posttraumatic aortic rupture in double localization, in the isthmus and in the descending thoracic aorta. He underwent thoracic endovascular aortic repair (TEVAR) with the use of stent graft three weeks after his car accident. The endovascular treatment was successful and no case of perigraft leakage has been detected during a meaning follow-up of five months.

Conclusion

The systematic analysis of the whole thoracic aortic vessel is crucial to not misdiagnose eventual multiple aortic rupture.     

Valor pronóstico de la isquemia miocárdica y la necrosis en pacientes con la función ventricular izquierda deprimida: un registro multicéntrico con resonancia magnética cardiaca de estrés

Introduction and objectives

The incremental prognostic value of inducible myocardial ischemia over necrosis derived by stress cardiac magnetic resonance in depressed left ventricular function is unknown. We determined the prognostic value of necrosis and ischemia in patients with depressed left ventricular function referred for dipyridamole stress perfusion magnetic resonance.

Methods

In a multicenter registry using stress magnetic resonance, the presence (≥ 2 segments) of late enhancement and perfusion defects and their association with major events (cardiac death and nonfatal infarction) was determined.

Results

In 391 patients, perfusion defect or late enhancement were present in 224 (57%) and 237 (61%). During follow-up (median, 96 weeks), 47 major events (12%) occurred: 25 cardiac deaths and 22 myocardial infarctions. Patients with major events displayed a larger extent of perfusion defects (6 segments vs 3 segments; P <.001) but not late enhancement (5 segments vs 3 segments; P =.1). Major event rate was significantly higher in the presence of perfusion defects (17% vs 5%; P =.0005) but not of late enhancement (14% vs 9%; P =.1). Patients were categorized into 4 groups: absence of perfusion defect and absence of late enhancement (n = 124), presence of late enhancement and absence of perfusion defect (n = 43), presence of perfusion defect and presence of late enhancement (n = 195), absence of late enhancement and presence of perfusion defect (n = 29). Event rate was 5%, 7%, 16%, and 24%, respectively (P for trend = .003). In a multivariate regression model, only perfusion defect (hazard ratio = 2.86; 95% confidence interval, 1.37-5.95]; P = .002) but not late enhancement (hazard ratio = 1.70; 95% confidence interval, 0.90–3.22; P =.105) predicted events.

Conclusions

In depressed left ventricular function, the presence of inducible ischemia is the strongest predictor of major events.Full English text available from:www.revespcardiol.org/en     

TRAPS : influence du génotype sur l’expression de la maladie. À propos de deux observations

Introduction

Tumor necrosis factor receptor associated periodic fever syndrome (TRAPS) is defined as recurrent attacks of generalized inflammation for which no infectious or auto-immune cause can be identified; it is caused by dominantly inherited mutations in the gene encoding the first TNF receptor. We report two additional cases of patients with TRAPS, suggesting that mutation pattern of TNFRSF 1A gene may influence the TRAPS phenotype.

Case reports

The first patient, with a C30S mutation, exhibited severe digestive clinical manifestations; because the patient required high-dose corticosteroids regimen to improve TRAPS manifestations, he was further given successfully etanercept. The second patient, with a R92Q mutation of TNFRSF 1A gene, presented with moderate symptoms; TRAPS outcome was favourable after corticosteroid therapy initiation.

Conclusion

Therefore, R92Q may be associated with a mild disease phenotype. On the other hand, C30S mutation appears to be associated with a severe phenotype, leading to an increased risk of amyloidosis. These findings suggest that these latter patients may require a closer follow-up.     

Seguimiento de 3 años de pacientes con lesiones de bifurcación tratados con stents liberadores de sirolimus o everolimus: estudio de colaboración de SEAside y CORpal

Introduction and objectives

To compare the 3-year incidence of major events in patients with bifurcation lesions treated with provisional sirolimus-eluting stents vs everolimus-eluting stents.

Methods

A pooled analysis of 2 prospective randomized trials with similar methodology (SEAside and CORpal) was performed. In these trials, 443 patients with bifurcation lesions were randomly assigned to treatment with either sirolimus-eluting stents or everolimus-eluting stents. The clinical follow-up was extended up to 3 years to assess major adverse cardiovascular events (death or acute myocardial infarction or target vessel revascularization).

Results

At 3 years, survival free of major adverse cardiovascular events was 93.2% vs 91.3% in the everolimus-eluting stent group vs the sirolimus-eluting stent group (P = .16). Exploratory land-mark analysis for late events (occurring after 12 months) showed significantly fewer major adverse cardiovascular events in the everolimus-eluting stent group: 1.4% vs 5.4% in the sirolimus-eluting stent group (P = .02).

Conclusions

Provisional stenting with either sirolimus-eluting stents or everolimus-eluting stents in bifurcation lesions is associated with low rates of major adverse events at 3-years’ follow-up. The results of a subanalysis of events beyond 1 year, showing a lower event rate with everolimus-eluting stents than with sirolimus-eluting stents, suggest that studies exploring the long-term clinical benefit of the latest generation of drug-eluting stents are warranted.Full English text available from: www.revespcardiol.org/en