Risk stratification in hypertrophic cardiomyopathy.

Sudden cardiac death (SCD) is the most devastating complication of hypertrophic cardiomyopathy (HCM). The greatest challenge in the management of HCM is identifying those at increased risk, since an implantable cardioverter-defibrillator (ICD) is a potentially life-saving therapy. We sought to summarize the available data on SCD in HCM and provide a clinical perspective on the current differing and somewhat conflicting data on risk stratification, with balanced guidance regarding rational clinical decision-making. Additionally, we sought to determine the status of the current implementation of guidelines compiled by HCM experts worldwide. The HCM Risk-SCD model helps improve the risk stratification of HCM patients for primary prevention of SCD by calculating an individual risk estimate that contributes to the clinical decision-making process. Improved risk stratification is important for decision-making before ICD implantation for the primary prevention of SCD.

Inherited primary arrhythmia disorders: cardiac channelopathies and sports activity.

Sudden cardiac death (SCD) in an apparently healthy individual is a tragedy. It is important to identify the cause of death and to prevent SCD in potentially at-risk family members. Inherited primary arrhythmia disorders are associated with exercise-related SCD. Despite the well-known benefits of exercise, exercise restriction has been a historical mainstay of therapy for these conditions. However, since familiarity with inherited arrhythmia conditions has increased and patients are often children and young adults, it is necessary to reassess the treatment guidelines regarding exercise constraints. The aim of this review is to analyze the risk of exercise-induced SCD in patients with inherited cardiac conditions and explore the challenges faced when advising patients about exercise limitations. We searched for publications on cardiac channelopathies in PubMed with the following medical subject headings (MeSH): "long QT syndrome"; "short QT syndrome"; "Brugada syndrome"; and "catecholaminergic polymorphic ventricular tachycardia". The abstracts of these articles were scanned, and articles of relevance, along with pertinent references, were read in full. The analysis was restricted to reports published in English. The findings of this analysis suggest that exercise with low-to-moderate cardiovascular demand may be possible under regular clinical follow-up in inherited primary arrhythmia disorders. Recent data show that patients with inherited primary arrhythmia disorders are at low risk for events once a comprehensive treatment program has been established. Recreational activity is likely safe for these individuals, with personalized management based on individual patient preferences and priorities.

[Vitamin C+sodium bicarbonate versus sodium bicarbonate alone in preventing contrast-induced nephropathy]. / Vitamine C+sérum bicarbonaté versus sérum bicarbonaté seul dans la prévention de la néphropathie induite par les produits de contraste.

INTRODUCTION: Contrast-induced nephropathy (CIN) is a common and severe complication in interventional cardiology. OBJECTIVE: The aim of our study was to compare the incidence of contrast-induced nephropathy in two accelerated hydration protocols: the first one by the serum bicarbonate and the second combining the serum bicarbonate and oral vitamin C. METHODS: This is a multicenter prospective, randomized study conducted between October 2012 and May 2013, including 160 patients. RESULTS: The mean age of our study population was 60.8±9.3 years (36-83 years). The two study groups were comparable in terms of cardiovascular risk factors, concomitant medication, and baseline serum creatinine. The CIN incidence was 6.3% in the vitamin C group and 10% in the control group (P=0.38). No significant difference was observed in terms of CIN incidence between the different subgroups analyzed. CONCLUSION: According to our study, ascorbic acid administered orally as part of an accelerated hydration protocol does not reduce the incidence of CIN.

[Management of patients treated for acute ST-elevation myocardial infarction in Tunisia: Preliminary results of FAST-MI Tunisia Registry from Tunisian Society of Cardiology and Cardiovascular Surgery]. / Prise en charge de l'infarctus du myocarde en Tunisie : résultats préliminaires du registre FAST-MI Tunisie de la Société tunisienne de cardiologie et de chirurgie cardiovasculaire.

UNLABELLED: FAST-MI Tunisian registry was initiated by the Tunisian Society of Cardiology and Cardio-vascular Surgery to assess characteristics, management, and hospital outcomes in patients with ST-elevation myocardial infarction (STEMI). METHODS: We prospectively collected data from 203 consecutive patients (mean age 60.3 years, 79.8 % male) with STEMI who were treated in 15 public hospitals (representing 68.2 % of Tunisian public centres treating STEMI patients) during a 3-month period at the end of 2014. The most common risk factor was tobacco (64.9 %), hypertension (38.6 %), diabetes (36.9 %) and dyslipidemia (24.6 %). RESULTS: Among these patients, 66 % received reperfusion therapy, 35 % with primary percutaneous coronary interventions (PAMI), 31 % with thrombolysis (28.6 % of them by pre-hospital thrombolysis). The median time from symptom onset to thrombolysis was 185 and 358 min for PAMI, respectively. The in-hospital mortality was 7.0 %. Patients enrolled in interventional centers (n=156) were more likely to receive any reperfusion therapy (19.8 % vs 44.6 %; p<0.001) than at the regional system of care with less thrombolysis (26.9 % vs 44.6 %; p=0.008) and more PAMI (52.8 % vs 8.5 %; p<0.0001). Also the in-hospital mortality was lower (6.4 % vs 9.3 %) but not significant. CONCLUSIONS: Preliminary results from FAST-MI in Tunisia show that the pharmaco- invasive strategy should be promoted in non-interventional centers.

[Arrhythmia and sleep apnea syndrome]. / Syndrome d'apnée du sommeil et troubles du rythme cardiaque.

CONTEXT: Arrhythmia is a major cause of morbidity and mortality in Europe and in the United States. The aim of this review article was to assess the results of the prospective studies that evaluated the risk of arrhythmia in patients with sleep apnea syndrome and discuss the management of this arrhythmia. EVIDENCE ACQUISITION: Reports published with the following search terms were searched: sleep apnea syndrome, atrial flutter, supraventricular arrhythmia, ventricular arrhythmia, ventricular tachycardia, ventricular fibrillation, torsade de pointe, atrial fibrillation and sudden death. The investigation was restricted to reports published in English and French. EVIDENCE SYNTHESIS: The outcome of this analysis suggests that patients with untreated overt sleep apnea syndrome are at increased risk of arrhythmia. CONCLUSIONS: The timely recognition and effective treatment of sleep apnea syndrome in patients with arrhythmia are mandatory because the prognosis of arrhythmia may be improved with the appropriate treatment of sleep apnea syndrome.

[Utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy: when is it superior to echocardiography?]. / Imagerie par résonance magnétique dans la cardiomyopathie hypertrophique : qu'apporte-t-elle de plus par rapport à l'échocardiographie ?

The diagnosis of hypertrophic cardiomyopathy is usually established by echocardiography. Recently, there has been greatly increased use of cardiac magnetic resonance (CMR) because of its precise determination of myocardial anatomy and the depiction of myocardial fibrosis. In this review, we describe the role of echocardiography and magnetic resonance in the assessment of this complex disease. In conclusion, there is a complementarity between cardiovascular magnetic resonance imaging and echocardiography for the diagnosis and the management of HCM.

Arrhythmia and thyroid dysfunction.

CONTEXT: Arrhythmia is a major cause of morbidity and mortality in Europe and in the United States. The aim of this review article was to assess the results of the prospective studies that evaluated the risk of arrhythmia in patients with overt and subclinical thyroid disease and discuss the management of this arrhythmia. EVIDENCE ACQUISITION: A literature search was carried out for reports published with the following terms: thyroid, hypothyroidism, hyperthyroidism, subclinical hyperthyroidism, subclinical hypothyroidism, levothyroxine, triiodothyronine, antithyroid drugs, radioiodine, deiodinase, atrial flutter, supraventricular arrhythmia, ventricular arrhythmia, ventricular tachycardia, ventricular fibrillation, torsade de pointes, amiodarone and atrial fibrillation. The investigation was restricted to reports published in English. EVIDENCE ANALYSIS: The outcome of this analysis suggests that patients with untreated overt clinical or subclinical thyroid dysfunction are at increased risk of arrhythmia. Hyperthyroidism increased atrial arrhythmia; however, hypothyroidism increased ventricular arrhythmia. CONCLUSION: The early recognition and effective treatment of thyroid dysfunction in patients with arrhythmia is mandatory because the long-term prognosis of arrhythmia may be improved with the appropriate treatment of thyroid dysfunction.

Wolff-Parkinson-white syndrome mimics a conduction disease.

Background. It is important to recognise Wolff-Parkinson-White (WPW) syndrome in electrocardiograms (ECG), as it may mimic ischaemic heart disease, ventricular hypertrophy, and bundle branch block. Recognising WPW syndrome allows for risk stratification, the identification of associated conditions, and the institution of appropriate management. Objective. The present case showed that electrophysiological study is indicated in patients with abnormal ECG and syncope. Case Report. A 40-year-old man with Wolff-Parkinson-White syndrome was presented to emergency with syncope. A baseline ECG was a complete right branch block and posterior left hemiblock. He was admitted to the cardiac care unit for pacemaker implantation. The atypical figure of complete right branch block and posterior left hemiblock was thought to be a "false positive" of conduction abnormality. But the long anterograde refractory period of the both accessory pathway and atrioventricular conduction may cause difficulty in diagnosing Wolff-Parkinson-White syndrome, Conclusion. A Wolff-Parkinson-White Syndrome may mimic a conduction disease. No reliable algorithm exists for making an ECG diagnosis of a preexcitation syndrome with conduction disorders. This can lead to diagnostic and therapeutic dilemmas in the context of syncope.

Cardiac resynchronization therapy allows the optimization of medical treatment in heart failure patients.

AIM: Cardiac resynchronization therapy (CRT) is recommended for selected patients with advanced heart failure (HF) despite optimal medical treatment. However, the doses of pharmaceuticals in this population are often limited by adverse effects. We compared the drug regimens of 21 patients before and 6 months after they underwent the implantation CRT systems. METHODS: We studied 17 men and four women (mean age=63.4 ± 11 years) presenting in New York Heart Association HF classes III-IV, and with a left ventricular ejection fraction (LVEF) ≤ 35% and cardiac dyssynchrony, who underwent implantation of CRT systems. RESULTS: At baseline, 52% of patients were treated with ß-adrenergic blockers (ß-B), though in optimal doses in only 19%. The introduction of (ß-B) was complicated by cardiogenic shock in three patients. At baseline, all patients were treated with angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARB), of whom 76% received optimal doses. After 6 months of CRT, ß-B were administered to 76% of patients, in optimaklon ACE or ARB but 75% of them were receiving maximal doses. After 6 months of CRT, ß blockers have been introduced in 72% of patients and maximal doses have been achieved in 60% of them. Maximal doses of ACE or ARB were reached in 95% of the study population. We noticed that systolic blood pressure was higher after implantation. There was also a significant improvement in functional status and left ventricular ejection fraction compared to baseline. CONCLUSION: CRT is an efficacious adjunctive device therapy to standard medical therapy for patients with heart failure and cardiac dyssynchrony. Its benefits are in addition to those afforded by standard pharmacological therapy. Achieving maximal doses of medical treatment and the possibility of introducing ß blockers after CRT prove that CRT and pharmacological treatment are complementary strategies and should not be considered as competitive.

Identification of two novel variants in PRKAG2 gene in Tunisian type 2 diabetic patients with family history of cardiovascular disease.

We report the identification of two novel polymorphisms in the PRKAG2 gene and preliminary association study between 5'-UTR and exon 1 polymorphisms with susceptibility to type 2 diabetes. No association with type 2 diabetes was identified. However, one of these newly identified polymorphisms (p.Ser20Ile) is likely associated with cardiac disease.

[Electrical storm secondary to hypokaliemia in a patient with implantable cardioverter defibrillator]. / Orage rythmique secondaire à une hypokaliémie chez un patient porteur d'un défibrillateur automatique implantable.

Electrical storm in patients with implantable cardioverter defibrillator (ICDs) is a rhythmic emergency which can be immediately lethal. It occurs especially in patients with an advanced cardiomyopathy. Such arrhythmias predictor factors and triggers are rarely found. We report the case of a 73-year-old man with ischemic dilated cardiomyopathy, who underwent a complete surgical revascularisation six years ago. In 2003, this patient was hospitalised in cardiology because of a sustained ventricular tachycardia reduced by electrical shock. Because of the aggravation of the heart failure with a NYHA functional class III, electrical and echocardiographic criteria of ventricular dyssynchrony, a biventricular ICD was implanted. Three years after, the patient was hospitalized because of an electrical storm with 96 appropriate shocks. A severe hypokaliemia was the cause of this electrical storm, and the evolution was favourable after correction of the hypokaliemia. Hypokaliemia is rarely the trigger of such arrhythmias, it represents only 3 % of the causes. Its prevalence may be underestimated especially in patients with heart failure who receive high doses of diuretics.

Assessment of heart failure and left ventricular systolic dysfunction after cardiac pacing in patients with preserved left ventricular systolic function.

BACKGROUND: There is an accumulating data suggesting the deleterious effects of right ventricular pacing on left ventricular performance. Such pacing mimics left bundle branch block resulting in a prolonged QRS duration and causes ventricular asynchrony. AIMS: The purpose of this study is to assess heart failure and left ventricular systolic function after cardiac pacemaker implantation in patients with atrioventricular block and preserved systolic left ventricular function. Secondly, we sought to search for predictive factors of developing left ventricular dysfunction after pacing. METHODS: In this prospective study, we included patients who had been implanted for at least six months. They underwent medical history and examination, 12 leads electrocardiogram and echocardiography before pacemaker implantation and when attending to routine pacemaker follow up. RESULTS: Forty-three patients (22 men and 21 women, age 71+/-12 years) were included in this study. Twenty-nine patients had DDD pacing and 14 VVI pacing. The ventricular lead was implanted in the apex in all patients. After a median follow up of 18+/-11 months, 11 patients (25%) developed signs of congestive heart failure. NYHA was higher after implantation (1.64+/-0.7 versus 2.27+/-0.8, p>0.00001). Left ventricular ejection fraction decreased significantly during follow up (60+/-6% versus 51+/-13%, p=0.0002). Eleven (25%) patients developed left ventricular dysfunction. We compared patients who had left ventricular ejection fraction (LV EF) less or equal to 40% (group A) and patients having LV EF greater than 40% (group B) after implantation. Patients in group A had a paced QRS width significantly larger than group B (181+/-32 ms versus 151+/-26 ms, p=0.002), a significantly prolonged intra left ventricular electromechanical delay (115+/-59 ms versus 45+/-35 ms, p<0.0001) and interventricular delay (44+/-29 ms versus 27+/-18 ms, p=0.02). Age, sex, diabetes hypertension, pacing mode and percentage of ventricular pacing were similar in both groups. A paced QRS width of 180 ms had the best sensitivity and specificity for detecting left ventricular dysfunction: sensitivity=54% and specificity=93%, p=0.01, area under the curve=0.75. CONCLUSION: Patients with atrioventricular block and preserved left ventricular systolic function at baseline decrease significantly left ventricular ejection fraction after pacing. Induced ventricular asynchronism plays a major role in the deterioration of left ventricular function. Prolonged paced QRS width is a good predictor of left ventricular dysfunction after pacing. Larger prospective studies are needed to confirm these data.

[Left hemothorax caused by aortic dissection]. / Hémothorax gauche, mode de révélation d'une dissection aortique.

Hemothorax is generally secondary to trauma, invasive thoracic surgery or specific lung of pleural disease. Hemothorax secondary to aortic dissection is rare. We report a case in a 69-year-old man with a history of smoking and hypertension. The patient was referred for exploration of a left pleural opacity with mediastinal widening. Pleural puncture produced a hemorrhagic fluid. The thoracic scan led to the diagnosis of Stanford type B aortic dissection. The patient was given antihypertensive treatment and now has a sixteen month follow-up. Aortic dissection can give rise to hemothorax, especially in a hypertensive subject with an anomalous aortic arch or mediastinal widening on the chest X-ray. Computed tomography generally gives the diagnosis. Treatment may be medical or surgical, depending on the site of the lesion and the severity of the clinical presentation. Aortic dissection is a cause of hemothorax, particularly in patients with favorable conditions or a suggestive radiological anomaly.

[The role of B type natriuretic peptide in the assessment of post myocardial infarction prognosis]. / Place du BNP dans l'evaluation du pronostic au décours de l'infarctus du myocarde.

Recently cardiac peptides have received close attention as cardiovascular markers. Brain (B type) natriuretic peptide is a neurohormone synthesized predominantly in ventricular myocardium. Previous studies have shown that this hormone can provide prognostic information in patients with myocardial infarction. The aim of this review is to evaluate the impact of plasma levels of BNP on prediction of left ventricular ejection fraction and remodelling and major cardiac events after myocardial infarction.

[Syphilitic aortic aneurysm. A case report]. / Anévrysme aortique d'origine syphilitique. A propos d'un cas]

The incidence of tertiary syphilis has declined in recent years owing to the early recognition of the disease and use of antibiotics. As a result, syphilitic aortic aneurysms are rarely encountered nowadays. We report the case of a 65 years old man, who was admitted to our hospital in June 2004 for dyspnea, cough and chest discomfort. On physical examination, blood pressure was 130/80 mmHg with no significant laterality, pulse rate was 70 per minute and there was a decrease of breath sounds over the right lung. Laboratory findings revealed a slight elevation of the erythrocyte sedimentation rate. Serological studies for syphilis showed a positive venereal disease laboratory test (VDRL) at 1/32 and a positive Treponema pallidum hemagglutination test (TPHA) at 1/2560. The chest radiography showed a right para cardiac opacity measuring 16 x 12 cm. Fiber optic bronchoscopy showed an extrinsic compression of the right upper lobar bronchus. Gadolinium-enhanced magnetic resonance angiography and 16 multidetector-row spiral computed aortography showed a huge partially thrombosed saccular aneurysm of the ascending aorta measuring 132 mm in diameter. The circulating lumen measured 53 mm in its largest diameter. This aneurysm involved the innominate artery. There was no other arterial involvement. The patient was given a three week course of intravenous penicillin followed by a successful surgical procedure in September 2004 with ascending aortic replacement and innominate artery reimplantation. This case illustrates well a formerly common, but now extremely rare disease.

[The first Tunisian cardiovascular diseases register: processes and results]. / Le registre des maladies coronaires en Tunisie: organisation et premiers résultats.

BACKGROUND: In Tunisia, cardiovascular diseases are the leading causes of death (30%) and a few studies conducted in the population have demonstrated that the level of their risk factors is increasing. For policy makers, the health system impact of these diseases is currently a crucial issue. The National Public Health Institute has identified the implementation of a morbidity register as a priority. METHODS: A CVD morbidity register is implemented since 2001, in 3 different geographical populations having contrasted levels of health status (Tunis, Ariana and Ben Arous). The 3 regions are covering about 2 millions inhabitants which is the fifth of the overall Tunisian population. All coronary heart events occurring among adults 25 years old and above in the 3 populations are recorded. The diagnosis of events, case fatality and classification are defined according to MONICA criteria. The data are recorded from public and private hospitals, death certificates and autopsies. RESULTS: During the year 2001, the total number of myocardial infarction events was estimated at 942: in men, the age-standardized rates were 163.8/100000 in Tunis population vs. 161.9 in Ariana and 170.5 in Ben Arous. In women, the rates were respectively 43.4, 61.1 and 44.6. Medical causes of death registration was the most crucial problem in spite of the implementation of the death certificate designed according to WHO model. Specific surveys for clinical assessment and surveillance of risk factors were conducted in the register populations. CONCLUSION: It is the first time that data on coronary heart disease incidence and fatality are available in Tunisia through this experience which highlights the practical difficulties experienced in registering and coding coronary events in a developing country. The data source quality should be improved and the register should be integrated in the local health system.

[A dead and calcified right ventricular hydatic cyst. The limits of imaging]. / Kyste hydatique mort et calcifié du ventricule droit. Limites de l'imagerie.

INTRODUCTION: Cardiac echinococcosis is rare and only represents around 0.5% of all hydatic locations. Right ventricular location is exceptional. OBSERVATION: We report the case of a 72 year-old man having a chronic obstructive pulmonary disease. During an acute respiratory failure, cardiac ultrasonography showed a right ventricular tumour. After respiratory stabilisation, surgical intervention found the tumour, the histopathological study of which concluded in a dead and calcified hydatic cyst. DISCUSSION: This case report demonstrates the limits of modern imaging and the difficulties in diagnosing a calcified right intra-ventricular hydatidosis. It is important to emphasize the exceptional nature of this form and the scarcity of its localisation. Surgical treatment is crucial in the majority of cases and helps to confirm the diagnosis. CONCLUSION: In endemic areas, the discovery of an intra-cardiac tumour must evoke and eliminate echinococcosis. Calcification of a cardiac location remains exceptional.

[Brugada syndrome]. / Le syndrome de Brugada.

The Brugada syndrome is a clinical-electrocardiographic diagnosis characterised by syncopal or sudden death episodes in patients with a structurally normal heart with a characteristic electrocardiographic pattern consisting of ST segment elevation in the precordial leads V1 to V3 and a morphology of the QRS complex resembling a right bundle branch block. In many patients with the Brugada syndrome, the electrocardiographic manifestations transiently normalize; leading to underdiagnosis of the syndrome. The administration of sodium channel blockers such as ajmaline, flecainide or procainamide accentuate the ST segment elevation and can be used to unmask concealed and intermittent forms of the disease. The incidence of sudden death in this syndrome is very high and can only be prevented by implanting a cardioverter-defibrillator. Because of high incidence of familial occurrence, the extension of the testing to family members is important.

[Hypertrophic cardiomyopathy and pregnancy]. / Cardiomyopathie hypertophique et grossesse.

We report the case of a 32 years old patient, with a known diagnostic of hypertrophic cardiomyopathy; who has presented at the first trimester of pregnancy a ventricular fibrillation treated by electric shock with a favorable outcome. The risks for the mother and the foetus are discussed; The recommendations for the conduct of the delivery are reviewed.