HLA class II polymorphisms in Tunisian patients with dilated cardiomyopathy.

Cardiomyopathies (CMs) are primary disorders of cardiac muscle. They are a major cause of morbidity and mortality for all ages and, like acquired forms of cardiovascular disease, often result in heart failure. Molecular genetic studies have made remarkable progress in defining the pathogenesis of CM. The present study was the first report to evaluate the relationship between class II major histocompatibility complex (MHC) genes (HLA-DRB1 and HLA-DQB1) and the genetic susceptibility to primary dilated cardiomyopathy (DCM) in Tunisian patients. The human leukocyte antigen (HLA)-DRB1 and -DQB1 alleles were analyzed in 76 patients with primary DCM and 111 ethnically matched healthy controls using polymerase chain reaction-sequence specific primers technique. An increased frequencies of HLA-DRB1*0401 (OR = 2.67, P < 0.001), HLA-DQB1*0302 (OR = 3.28, P = 0.001) and HLA-DQB1*0401 (OR = 6.26, P = 0.005) alleles were found in the patients with primary DCM compared with healthy controls. Individuals with HLA-DRB1*1301 (OR = 0.24, P < 0.001) and HLA-DQB1*0201 (OR = 0.49, P = 0.002) alleles have a protective effect against primary DCM. Two haplotypes were associated with increased risk of primary DCM: DRB1*0401/DQB1*0302 (OR = 4.53, P = 0.002) and DRB1*0401/DQB1*0401 (OR = 9.42, P = 0.004). In conclusion, our data suggest that the variation in class II HLA alleles could be a genetic factor involved in the susceptibility to primary DCM in the Tunisian population.

[Management of pulmonary embolism in a cardiology department]. / Prise en charge de l'embolie pulmonaire en milieu cardiologique: expérience d'un hôpital tunisien.

OBJECTIVES: The objective of our work is to study the clinical features and the management of pulmonary embolism in a cardiology department. MATERIAL AND METHODS: It is a retrospective study including 43 patients between 1993 and 2003 in the cardiology department of a Tunisian hospital for acute pulmonary embolism. RESULTS: The most common clinical signs were dyspnea, pleural pain and deep venous thrombosis. The most common physical signs were tachypnea and tachycardia. Electrocardiographic abnormalities were found in 38 patients (88.4%). Chest X-ray was abnormal in 25 patients (60.5%). The arterial blood gas found hypoxia and\or hypocapnia in 95% of cases. D-dimer was high in almost totality of patients (96%). US doppler of deep leg veins was positive in more than 40% of patients. Transthoracic echocardiography was normal in more than 40% of the patients. Lung scan noted a high or very high probability in 87% of cases. Pulmonary helical CT was positive in majority of cases. Unfractionated heparin was administered to about 75% of patients and low-molecular-weight-heparin was prescribed at only 40% of patients. CONCLUSION: Management of pulmonary embolism in our cardiology department is characterized by the frequent use of non invasive techniques on the diagnostic view and appeal to heparine on the therapeutic view.

[Chemotherapy against polycythemia due to a cyanotic congenital heart disease in adults. One case report]. / Chimiotherapie anti-erythrocytaire dans les cardiopathies congenitales cyanogenes de l'adulte. A propos d'une observation.

BACKGROUND: Hydroxyurea has largely been utilized in the management of primary polycythemia. It is certainly efficient in the short and medium terms side effects are marked by a leukemogenic risk. AIM: In this study we report the case in which hydroxyrea was for the first time used in the reatment of secondary polycythemia due to cyanotic congenital heart disease. CASE: Our patient was a 22-years-old man suffering from pulmonary atresia with ventricular septal defect, and for whom ther was no available surgical option. Because of severe symptomatic and demanding polycythemia requiring frequent phlebotomies, we decided to use hydroxyurea at the dose of Igr a day. Eight months later, ther was obvious clinical improvement with stable hematocrit levels below 60% and with only 3 phlebotomies in 8 months. CONCLUSION: Hydroxyurea seems to be an eddicient there alternative therapy for seconday polycythemia caused by incurable cyantic congenital heart disease.

[Homocysteinemia and coronary artery disease: a case-control study in a Tunisian population]. / Homocystéinémie et maladie coronaire: etude cas-témoins dans une population tunisienne.

We have determined the prevalence of hyperhomocysteinemia and tested its relationship with coronary heart disease in Tunisian patients. The study included 70 angiogrphically proven coronary patients and 140 age- and sex-matched healthy subjects. Plasma homocysteine folate and vitamin B12 were analyzed by immunoenzymatic methods. Hyperhomocysteinemia was considered for plasma homocysteine concentration >17 micromol/L. Mean plasma homocysteine concentration and hyperhomocysteinemia prevalence were significantly (p<0.001) higher in patients (16.3 +/- 7.9 micromol/L and 29%) than controls (12.6 +/- 4.0 micromol/L and 10%). The association between hyperhomocysteinemia and coronary heart disease persisted after adjusting on main cardiovascular risk factors (multi adjusted odds ratio, 2.99; 95% CI, 1.18-7.59; p=0.02). No association was observed between hyperhomocysteinemia and coronary disease severity and extent. This study showed an independent association between hyperhomocysteinemia and coronary heart disease, suggesting a role of hyperhomocysteinemia in atherothrombogenesis. However, causal relationship is not yet established. Until results of homocysteine-lowering therapy trials become available, hyperhomocysteinemia should be researched and treated in coronary heart disease patients.

[Inhospital prognosis of acute myocardial infarction: comparison of two periods to ten years of interval]. / Pronostic hospitalier de l'infarctus du myocarde comparaison de deux périodes a dix ans d'intervalle.

Acute myocardial infarction prognosis depends on many factors relative to the patient, to the disease and to the quality and promptness of the treatment. The interventional revascularisation is superior to pharmacological revascularisation. The aim of the study is to compare acute myocardial infarction inhospital lethality in the coronary care unit before and after use of emergency angioplasty. The retrospective study compared inhospital lethality over two four years periods separated by an interval of ten years: [1991-1994] and [2001-2004]. Lethality had increased from 7.8% to 11.3%. During the second period emergency angioplasty (under 24 hours) primary (4.8%) or rescue (2.5%), not applied in the nineties, was not related to survival and did not allow an improvement of patients' hospital prognosis. The population of the second period was characterized by: a greater average age (60.8 vs. 58.6 years), more female subjects (20.1 vs. 14.6%), less nicotinic subjects (60.2 vs. 78.5%) and less patients receiving fibrinolytic treatment (43.0 vs. 50.4%). These parameters were as many factors related to the risk of hospital death in univariate analysis. The multivariate analysis showed that after adjustment on all the other factors the lack of thrombolysis remains unquestionably related to the hospital overlethality. The time delays for admission in the intensive care unit were relatively long and comparable over the two periods (8.2 vs. 7.5 hours). Within our hospital structure early angioplasty not being able to be realized in the hour, which follows the diagnosis on a large scale, and in a continual way, it would be preferable to always consider thrombolysis first in all cases apart from contraindications without excluding the possibility of a hybrid treatment.

[Cardiac involvement in Emery-Dreifuss muscular dystrophy: a case report]. / L'atteinte cardiaque dans la dystrophie musculaire d'Emery-Dreifuss. A propos d'un cas.

Emery Dreifuss muscular dystrophy (EDMD) is an uncommon hereditary myopathy characterized by 3 symptoms: slow progressive muscular atrophy, muscular contractures and cardiac disease which affect prognosis. We report a 22 year-old patient with EDMD which shows the typical features of the associated dilated cardiomyopathy, ventricular arrhythmia, atrio-ventricular block, atrial standstill then atrial paralysis.

Pro- and anti-inflammatory cytokines in post-infarction left ventricular remodeling.

OBJECTIVES: Acute myocardial infarction (MI) leads to molecular, structural, geometric and functional changes in the heart during a process known as ventricular remodeling. Myocardial infarction is followed by an inflammatory response in which pro- and anti-inflammatory cytokines play a crucial role, particularly in left ventricular remodeling. This study aimed at evaluating serum concentrations of interleukin-8 (IL8), tumor-necrosis-factor-alpha (TNFα) and interleukin-10 (IL10), pro- and anti-inflammatory cytokines, and at correlating them with left ventricular remodeling as assessed by echocardiographic parameters. METHODS: In a case-control study 30 MI patients were compared with 30 healthy controls. Serum concentrations of IL8, TNFα and IL10 were measured on day 2 and day 30 post-MI by chemiluminescence immunoassay and correlated with echocardiographic parameters. RESULTS: There was an increase of IL8, and TNFα together with a decrease of IL10 at both time points. IL8 was negatively correlated with the left ventricular end-diastolic diameter (LVEDD) and positively with left ventricular systolic volume. IL10 was negatively correlated with LVEDD and left atrial volume 30days post-MI. CONCLUSION: The increase of pro-inflammatory cytokines TNFα and IL8 was accompanied by decreased anti-inflammatory IL10. This imbalance between pro- and anti-inflammatory cytokines might contribute to the progression of left ventricular remodeling and may lead to heart failure.

Percutaneous transluminal balloon valvuloplasty of adult aortic stenosis: report of 92 cases.

Percutaneous transluminal balloon valvuloplasty was attempted in 92 adult patients with severe calcific aortic stenosis. The mean age was 75 +/- 11 years (range 38 to 91) and 35 patients were more than 80 years old. Most of the patients were severely disabled; 66 were in New York Heart Association functional class III or IV, 27 had syncopal attacks and 21 had severe angina pectoris. Because of unacceptably high surgical risk or contraindication to thoracic surgery, 42 patients could not be considered for valve replacement. Other patients either were in a category of high operative risk or refused the surgical intervention. Valvuloplasty was performed by way of the femoral route (82 patients) or the brachial route (10 patients). Catheters of size 15, 18 and 20 mm were successively placed across the aortic valve and three inflations were usually done with each of them, lasting 80 seconds on average, until a decrease in peak to peak systolic pressure gradient to 40 mm Hg or less was attained, a result considered satisfactory. The inflated balloons were not totally occlusive in most cases and clinical tolerance of inflation was good. Valvuloplasty resulted in a reduction of mean systolic gradient from 75 +/- 26 to 30 +/- 13 mm Hg (p less than 0.001); the final gradient was less than 40 mm Hg in 78 patients. Mean calculated aortic valve area increased from 0.49 +/- 0.17 to 0.93 +/- 0.36 cm2 (p less than 0.001). Immediately after the procedure, ejection fraction increased from 48 +/- 16 to 51 +/- 16% (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

[Effects of intravenous trinitrin infusion on systemic and coronary circulations during atrial electric stimulation]. / Effets de la perfusion intraveineuse de trinitrine sur les circulations systémique et coronaire au cours de la stimulation électrique auriculaire.

Intravenous infusion of trinitrin (0.38 +/- 0.25 mg/hour) during rapid atrial pacing reduced pulmonary capillary pressures, cardiac output, coronary blood flow and myocardial oxygen consumption. At these dosages trinitrin allows patients with ischaemic heart disease to undergo atrial pacing in the best conditions by maintaining the pulmonary capillary and systemic arterial pressures and myocardial lactate production within limits close to the basal values. The beneficial effects last during the recovery period after the termination of pacing and of the intravenous infusion.

[Effects on the systemic and coronary circulation of 2 nitrate derivatives, intravenous trinitrin and sublingual isosorbide dinitrate, administered during atrial pacing]. / Effets sur les circulations systémique et coronaire de deux dérivés nitrés, trinitrine intraveineuse et dinitrate d'isosorbide sublingual, administrés au cours de la stimulation électrique auriculaire.

The changes in the systemic and coronary circulations produced by intravenous trinitrin 0.38 +/- 0.125 mg/hour and sublingual isosorbide dinitrate 5 mg were studied in 24 patients with coronary artery disease. When given during rapid atrial pacing both drugs decreased pulmonary capillary pressures (p less than 0.001), cardiac and coronary output (p less than 0.001 and p less than 0.01) and myocardial oxygen consumption (p less than 0.01). At these dosages, intravenous trinitrin has no significant effect on average systemic blood pressure or left ventricular work index; the coronary arterial resistances increased (p less than 0.005). Isosorbide dinitrate significantly decreased average systemic blood pressure and the left ventricular work index (p less than 0.001); there was no significant difference in the coronary arterial resistances; the decrease in coronary blood flow observed after sublingual isosorbide dinitrate seems partly due to a decreased perfusion pressure. The beneficial effect of these nitrite derivatives seems to be mainly related to a reduced preload.

[Percutaneous valvotomy in the combination of mitral and tricuspid valve stenosis. Report of 3 cases]. / Valvulotomie percutanée dans l'association sténose mitrale--sténose tricuspide. A propos de trois observations.

The authors report the results of a series of 3 cases of double valvuloplasty with a balloon catheter in young patients with combined mitral and tricuspid stenosis. Haemodynamic and Doppler echocardiographic evaluation after the procedure showed comparable results to those of surgical commissurotomy without significant secondary valvular regurgitation. Clinical and echocardiographic follow-up showed that valvular opening remained satisfactory in the 2 cases examined. Percutaneous valvuloplasty would seem to be a valuable alternative to surgical commissurotomy in selected patients with combined mitral and tricuspid valve stenosis.

[Percutaneous transluminal aortic valvuloplasty using a balloon catheter. A new therapeutic option in aortic stenosis in the elderly]. / Valvuloplastie aortique percutanée transluminale par sonde à ballonnet. Une possibilité thérapeutique nouvelle dans le rétrécissement aortique du sujet âgé.

Aortic valvular dilatation with a balloon catheter was performed in 44 patients, 20 men and 24 women, most of whom were very elderly (average age 77 years). The indication for valvular replacement had not been retained in these cases because of surgical contra-indications or a very high operative risk and in 3 cases because of patient refusal. Twenty-nine patients were in functional classes III or IV of the NYHA classification; 12 had syncopal episodes and 18 had invalidating angina. The dilatation was performed by a femoral arterial approach in 34 cases, and by a brachial arterial approach in 10 cases. MEDI-TECH catheters with 15, 18 or 20 mm diameters when inflated were used in the majority of cases. Several inflations lasting 10 to 240 seconds were performed in each case with balloons of increasing size. This was well tolerated in all but one patients who had a sharp syncope. The immediate results confirmed valvular dilatation. The average transvalvular pressure gradient fell from 76 +/- 25 mmHg to 30 +/- 13 mmHg (p less than 0.001). The aortic valve surface area calculated by the Gorlin formula increased from 0.5 +/- 0.18 cm2 to 1 +/- 0.42 cm2 (p less than 0.01). After dilatation the gradient was less than or equal to 40 mmHg in 37 cases; aortic valve surface area was greater than or equal to 1 cm2 in 14 cases and less than or equal to 0.7 cm2 in only 5 cases. The left ventricular ejection fraction increased immediately after valvuloplasty from 44 +/- 16 p. 100 to 49 +/- 15 p. 100 (p less than 0.01). In the 18 cases in which it was less than 40 p. 100 before valvuloplasty, it increased from 30 +/- 6 p. 100 to 36 +/- 9 p. 100 (p less than 0.02). Residual aortic regurgitation was only observed in one case. Two patients died in the hospital period (4.6 p. 100). There were no other serious complications. During an average follow-up period of 60 days (3 weeks to 6 months) there was a big improvement in symptoms in the great majority of cases and, in particular, syncopal and anginal attacks disappeared. Only 4 patients remained in functional classes III or IV after valvuloplasty. Percutaneous aortic valvuloplasty is a new, relatively simple, low risk, economic and very effective therapeutic procedure in all cases in which aortic valve replacement is contra-indicated or refused by the patient.(ABSTRACT TRUNCATED AT 400 WORDS)

[Coronary arteriovenous fistula. Apropos of a case with thallium myocardial scintigraphy and study of pre- and postoperative myocardial metabolism]. / Fistule artérioveineuse coronaire. A propos d'un cas avec scintigraphie myocardique au thallium et étude du métabolisme myocardique pré et post-opératoires.

Coronary angiography performed in a 62 year old man with spontaneous chest pain revealed a congenital fistula in the form of a vascular network arising from the proximal part of the left anterior descending artery and draining into the main pulmonary artery. A single severe atheromatous stenosis of the right coronary artery was observed. Thallium 201 myocardial scintigraphy at rest showed a large area of hypofixation in the artero-septo-apical zone. Myocardial and aorto-right coronary bypass, myocardial scintigraphy became almost normal, leaving a small antero-septal and apical deficit: myocardial metabolism returned to normal. The fact that lactate metabolism is a particularly sensitive index of myocardial hypoxia in the territory of the left coronary artery suggests a probable coronary steal syndrome induced by the fistula; this is probably also the cause of the isotopic hypofixation in the territory of the left anterior descending artery which emphasizes the value of Thallium myocardial scintigraphy in the study of congenital malformations of the coronary artery.

[Extensive aortic dissection and aneurysm of the left coronary trunk. 20-year spontaneous development]. / Dissection aortique étendue et anévrisme du tronc corconaire gauche. Vingt ans d'évolution spontanée.

A case is reported of dissection of the aorta in a lady of 35. The condition was complicated first by myocardial infarction and secondly by severe aortic incompetence which led, 20 years after the onset of the dissection, to the death of the patient from intractable heart failure. At post-mortem, an extensive dissection was found to involve the whole of the aorta and several of its branches, but did not involve the coronary arteries, which were normal except for the trunk of the left coronary; this was dilated. The natural history of dissections of the aorta is reviewed. The incidence, course and etiology of coronary aneurysms are also discussed.

[Distribution of cardiovascular risk factors in a Tunisian cohort of 6901 coronary patients]. / Distribution des facteurs de risque cardiovasculaire chez une cohorte tunisienne de 6901 coronariens.

The Tunisian epidemiological data on cardiovascular disease in the hospital environment are scarce. The aim of this study was to evaluate the frequency of cardiovascular risk factors and their association in patients hospitalised for coronary disease in coronary care units at Rabta, Charles Nicolle, Habib Thameur and Military hospitals, Tunis, over the period 1994-1998. The clinical features of 6901 patients (75.7% men, 3760 myocardial infarction, 3141 unstable angina) on hospital admission were analysed. The prevalence of smoking, dyslipidemia, hypertension, diabetes and obesity was 86; 49.8; 33.9; 40.7 and 15.2% respectively in the men and 12.9; 52.4; 64.6; 53.4 and 29.8% respectively in women. With this risk factor profile Tunisia has to implement a national strategy of primary prevention and heart health promotion in addition to the efforts recently made in secondary prevention of some chronic disease such as hypertension, diabetes and smoking.

[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.

[Treatment of chronic refractory cardiac insufficiency with enalapril]. / Traitement de l'insuffisance cardiaque chronique réfractaire par l'enalapril.

The objective is here to study the long and intermediate term clinical and haemodynamic effects of enalapril during chronic heart failure resistant to the classic digitalis-diuretics treatment. The study involves 16 patients (12 males and 4 females), with a mean age of 50 years. Before being given enalapril, 12 patients were at stage IV and 4 patients at stage II of the NYHA; the mean capillary pressure was quite elevated (30 +/- 6.3 mmHg), the cardiac index has collapsed (2.12 +/- 0.38 l.min.m2) and the stroke fraction (SF) is 0.28 +/- 0.08. At the 1st month control, there is a definite functional and haemodynamic improvement of the pre-charge as well as the post-charge. This improvement is still present at 6 months. The ventricular function is improved (SF = 0.38 +/- 0.13; p less than 0.001). The clinical tolerance of enalapril is excellent and the only adverse reaction is a transient deterioration of the renal function in a patient with diabetic glomerulopathy.

[Percutaneous mitral valvuloplasty using balloon catheterization. Immediate results in 80 cases]. / Valvuloplastia percutânea mitral por balão. Resultados imediatos de 80 casos.

In our first 80 attempts of percutaneous double balloon mitral valvuloplasty (BMV) 77 procedures have been successful. The mean patients age was 44 +/- 17 years, there were 16 men; 12 patients had previously been operated, 29 patients had important valvular deterioration (calcifications, thickening or unpliability) or of subvalvar system. For technical failure BMV was performed with only one balloon in 8 patients. BMV resulted in significant improvement in haemodynamic values: the mean capillary pressure fell from 22 +/- 6 to 12 +/- 5 mmHg (p less than 0.001), the mean mitral gradient from 15 +/- 6 to 5 +/- 3 mmHg (p less than 0.001). Cardiac index remained unchanged. The hemodynamic valve area, by Gorlin formula, increased from 1.09 +/- 0.29 to 2.19 +/- 0.72 cm2 (p less than 0.001). Doppler and echocardiography data were similar to haemodynamic data. Mitral valve area obtained with BMV was equivalent to the area usually obtained in closed mitral commissurotomy. There were 3 tamponades. The first, in a patient to whom BMV was not successful. For the 2 others, surgically evacuated, intracavitary pressures were measured after surgical pericardial drain. BMV was of little efficacy in one patient who died 3 days thoracotomy. The three tamponades were caused by straight tip balloon left ventricle perforation. There was no tamponade with pigtail tip catheter balloon. Mitral valve regurgitation was never increased more than 1 + Interatrial septal defect with QP/QS greater than or equal to 1.5 and less than 2 was present in 5 patients. BMV may be a useful alternative to surgery with low incidence of complication. Mitral valve area increase is similar with both treatment.

[Coronary artery disease in Takayasu's disease. Report of an operative case]. / L'atteinte coronarienne dans la maladie de Takayasu. A propos d'un cas opéré.

A 39-years-old woman with Takayasu's arteritis confirmed by histological examination was admitted in our hospital because of angina pectoris. Selective coronary arteriograms reveled a 90% isolated stenosis in the right coronary ostium. The patient underwent a right coronary artery bypass grafting using the saphenious vein. The post operative clinical course was uncomplicated. On follow-up over 14 months the patient has been completely free from anginal chest-pain.

[Congenital Valsalva sinus aneurysm opening into the right ventricle. Two operated cases]. / L'anévrisme congénital du sinus de Valsalva rompu dans le ventricule droit. A propos de deux cas opérés.

Congenital sinus of valsalva aneurysm is a rare cardiac lesion. Once the aneurysm has ruptured, a large left-to-right shunt is created. Prognosis is grave unless operative intervention is undertaken. The authors present 2 cases (2 male) of congenital aneurysm of the right sinus of valsalva ruptured into the right ventricle. The first patient (52 years) have a history of strenuous exertion followed by acute precordial pain and dyspnea. The second patient (25 years) was asymptomatic. The diagnostic was made by 2D and color doppler echocardiography. The defect was closed with a patch with good postoperative course. The authors emphasise the possible rupture of the congenital aneurysm of the sinus of valsalva after 50 years of age. Echocardiography is certainly the best method for diagnosing the condition and for following up these patients. The prognosis after surgical repair has usually been satisfactory.