Getting to the heart of the matter: diagnostic tools and therapeutic approach to cardiac involvement in Behçet syndrome A Tunisian case series.

The aim was to investigate the frequency and spectrum of cardiac involvement (CI) in patients with Behçet syndrome (BS) in the Tunisian context, and to assess the clinical and imaging features, treatment, and outcomes. We retrospectively retrieved the medical records of patients with CI among 220 BS patients admitted to the hospital internal medicine department between February 2006 and April 2019, who fulfilled the International Study Group diagnostic criteria for BS. Ten patients (8 men, 2 women) were eligible for the study. Mean age was 37.3 years. Three patients had 2 isolated episodes of cardiac BS. The different types of CI were coronary artery disease (5/10), intracardiac thrombus (4/10), pericarditis (1/10), myocarditis (1/10), and myocardial fibrosis (1/10). Five patients had associated vascular involvement (50%). Medical treatment was based on corticosteroids and colchicine in all patients (100%), anticoagulants in 8 (80%), and cyclophosphamide followed by azathioprine in 9 (90%). The clinical course was favorable in 9 patients; 1 patient died. CI remains an important feature of BS because of its association with increased risk of mortality and morbidity. Therefore, early screening and detection with imaging methods are paramount. Also, better cooperation between rheumatologists and cardiologists could improve outcomes.

Wide QRS in a 2-month infant associated with recurrent ventricular tachycardia: A case of Brugada-like syndrome.

We report the case of a 2-month old infant who experienced recurrent sustained ventricular tachycardia (VT) in a structurally normal heart. Resting electrocardiogram (ECG) showed wide QRS with a complete right bundle branch bloc (RBBB) morphology. There was no family history of syncope or sudden death, but the ECGs of the father and the brother showed incomplete RBBB with negative T waves on V1 lead. This case seems to fit well with the newly defined entity of Brugada-like syndrome with a highly suspected genetic underlying disposition.

[Percutaneous closure of persistent ductus arteriosus in low-weight infants]. / La fermeture percutanée du canal artériel persistant chez les enfants de faible poids.

INTRODUCTION: The persistent ductus arteriosus remains a common congenital pathology. Although percutaneous closure of wide channels using an Amplatzer Duct Occluder is an attractive alternative to the surgical treatment, this prosthesis is not recommended for infants weighing less than 6kg. AIM OF THE STUDY: The objective was to evaluate the efficacy and safety of this prosthesis in low-weight children. PATIENTS AND METHODS: The records of children weighing less than 6kg who underwent closure with the Amplatzer Duct Occluder prosthesis between January 2010 and December 2014 were retrospectively analyzed. RESULTS: Fourteen patients (mean weight: 5.7kg [range: 4.8-6]; mean age: 6.5months [range: 3-12]) were included. The main circumstance for discovery was difficulty in breathing (93% of children). The average angiographic persistent ductus arteriosus diameter was 3.5mm (range: 3-6mm), correlating well with that found on ultrasound (r=0.68). The prosthesis was implanted successfully in 93% of cases. The only failure was explained by the increased risk of aortic subocclusion. The immediate angiographic occlusion rate was 71%. The average duration of the procedure was 46±12min. Three children had a channel C-type on the Krichenko classification. Two complications occurred in two patients: a case of cardiac tamponade drained during the procedure without incident and one case of partial protrusion of the Amplatzer disk into the aortic lumen. C-type (tubular) persistent ductus arteriosus and a ratio of the diameter of the persistent ductus arteriosus/weight greater than 0.95 were significantly associated with intervention failure and/or major complications during the percutaneous closure, while weight of less than 6kg was not retained as a predictor of procedure failure. No late embolization occurred after 11months of median follow-up. During this monitoring, we noted a marked clinical improvement with normalization of pulmonary pressure. CONCLUSION: This study includes the few records reported in the literature assessing the feasibility of percutaneous closure in persistent ductus arteriosus in infants weighing up to 6kg. It confirms the effectiveness of the procedure with a relatively low prevalence of complications.

[Is PON1 a factor of cardiovascular risk in type 2 diabetes?]. / La PON1 est-elle un facteur du risque cardiovasculaire chez les diabétiques de type 2 ?

OBJECTIVE: We evaluate the association between the decrease of serum paraxonase 1 activity and the risk of cardiovascular disease in type 2 diabetes. METHODS: One hundred and fourteen patients with type 2 diabetes were included in the present study. Seventy-one of them have significant coronary disease. The control group consisted of 53 healthy adults. RESULTS: PON1 activity was significantly reduced in diabetic patients compared to controls (P=0.021), especially in those with significant coronary disease (P=0.013). No significant variation in PON1 activity according to age was observed both in controls and in patients. When HDLc≥1.03mmol/L, the PON1 activity was significantly higher in patients without significant coronary disease compared to those with significant coronary disease (0.030). In case of significant coronary disease, a decrease of 12.23% in PON1 activity was observed in smokers compared with non-smokers, but without statistical significance. The PON1 activity did not very significantly according to the presence or absence of hypertension in patients with significant coronary disease. CONCLUSION: The implication of diabetes in the decrease of PON1 activity seems highly probable but PON1 activity seems not to be in itself a marker of cardiovascular disease.

Permanent cardiac pacing in a patient with persistent left superior vena cava and concomitant agenesis of the right-sided superior vena cava.

Persistent left superior vena cava (PLSVC) can be incidentally detected during pacemaker implantation from the left pectoral side. Optimal site pacing is technically difficult, and lead stability of the right ventricle (RV) can lead to such a situation. We describe a case of successful single-chamber pacemaker implantation in a 76-year-old woman with a PLSVC and concomitant agenesis of the right-sided superior vena cava, after failed attempts with the conventional procedure. The pacemaker had been working well after 12 months of follow-up.

[Familial hypercholesterolemia in Tunisia]. / L'hypercholestérolémie familiale en Tunisie.

Familial hypercholesterolemia or autosomal dominant hypercholesterolemia is characterized by raised serum LDL (low density lipoproteins)-cholesterol levels, which result in excess deposition of cholesterol in tissues, leading to accelerated atherosclerosis and increased risk of premature coronary heart disease. Familial hypercholesterolemia results from defects in the hepatic uptake and degradation of LDL via the LDL receptor pathway. Familial hypercholesterolemia is commonly caused by a loss of function in the LDL receptor gene, or by a mutation in the gene encoding apolipoprotein B (APOB) or PCSK9 gene. In Tunisia, the frequency of this disease is about one of 165 for heterozygote. It is a higher frequency compared to most European countries, which is about one of 500 for heterozygote. Only five mutations in the LDLR gene were reported in this population. No mutations in the APOB or PCSK9 gene were reported.

[The effect of apolipoprotein e polymorphism on plasma cholesteryl ester transfer protein activity in type 2 diabetic patients].

We studied the relationship between apo E polymorphism and cholesteryl ester transfer protein (CETP) activity in 127 type 2 diabetic patients who did not take lipid lowering drugs. Furthermore, we studied the relationship between apo E and CETP in modulating plasma triglyceride and HDL cholesterol. Apo E genotypes were determined by PCR-RFLP and CETP activity was measured using an exogenous way. Our results showed that the CETP activity increased significantly in E2 carrier group compared to E4 carriers and E3E3 homozygous (84.7 +/- 43.9 vs 62.5 +/- 35.9 vs 52.6 +/- 23.6 nmol CE/ml 2h respectively; p = 0.015). However, there was no association between apoE polymorphism and lipid parameter variations. Even after adjustment for CETP activity the results remained unchanged showing that CETP did not step in the relationship between apo E and lipid parameter variations. In conclusion there is an association between apo E polymorphism and CETP activity and this association did not affect the relationship between apo E polymorphism and triglyceride and HDL cholesterol concentrations.

Long term outcome of balloon mitral commissurotomy during pregnancy: a prospective physical and mental evaluation of babies.

AIM: The immediate and long-term results of balloon mitral commissurotomy (BMC) during pregnancy were evaluated in patients and in their babies looking for radiation side effects. METHODS AND RESULTS: Sixty one patients (mean age: 28.5+/-5.0 years) had BMC at a mean age of gestation of 26.8+/-5.5 weeks. The procedure was successful in all patients except in one who had a severe mitral regurgitation and subsequent mitral valve replacement (MVR). All patients delivered at term vaginally in 58 (95.1%) cases. There was only one death in a patient who delivered at home. At a mean follow-up of 66.8+/-36.0 months, 4 patients had MVR, the remaining were in NYHA class I/II. Restenosis was found in 4 (7.2%) patients. The 63 babies (two gemellar pregnancies) had a normal weight of birth except of one case of hypotrophy. At a mean follow-up of 64.5+/-32.5 months, two babies died, 1 had hypotrophy, 2 had microcephaly, 8 had an IQ < 70 but none had a severe mental retardation (IQ <34). None of these events were radiation related. CONCLUSION: BMC is the procedure of choice in pregnant patients with mitral stenosis. No late radiation side effects were observed in children but longer follow-up is required.

[Cardiac involvement in Steinert's myotonic dystrophy]. / Anomalies cardiaques au cours de la dystrophie myotonique de Steinert.

INTRODUCTION: Cardiac involvement is described as one of the most frequent multisystemic manifestations of Steinert myotonic dystrophy (DM1). This study was performed to determine the frequency of cardiac abnormalities in Steinert myotonic dystrophy and to decipher the correlation between the severity of cardiac involvement and the degree of neurologic deficit. PATIENTS AND RESULTS: Thirty-four DM1 patients 23 men and 11 women, aged 13-61 years (mean 37.3+/-13.2 years) underwent neurological and cardiac evaluations. According to the MDRS scale, 32.5 percent were classified in the second stage, 23 percent in stage 3; 32.5 percent in stage 4 and 12 percent in stage 5. There was a positive correlation between neurological symptoms duration and the MRDS scale. Cardiac involvement was detected in 77.4 percent of patients. Electrocardiographic conduction abnormalities were the most frequent, represented by first-degree atrioventicular block in 64 percent of patients and bundle-branch block in 32 percent. From 5 patients having an invasive electrophysiology testing, subhisien block was observed in 3 patients. We respectively found alterations in systolic and diastolic left ventricular function in 22 percent and 30 percent of patients and a cardiac pacemaker was implanted in 3 patients. The frequency of cardiac manifestations was correlated to the degree of the neurological involvement assisted by MDRS scale, but it seam that the severity of cardiac abnormalities is not correlated to the degree of neurological deficit. CONCLUSION: We recommend that patients with DM1 undergo 24-h electrocardiogram monitoring and echocardiography at least yearly. Long-term prospective follow-up is required to determine the prognostic value of the observed abnormalities.

[Coronary angioplasty in the acute phase of a myocardial infarction on a myocardial bridge]. / Angioplastie coronaire à la phase aiguë d'un infarctus du myocarde sur pont myocardique.

A myocardial bridge is usually asymptomatic but can cause myocardial ischemia, myocardial infarction or sudden death. Two occurrences of coronary angioplasty in the acute phase of an anterior myocardial infarction on a myocardial bridge are reported. The first case was first treated only with a balloon, and then with a stent 12 h later after a relapse of angina pectoris and the recurrence of a severe compression. The second case immediately benefited from a stent. A systematic control at six months has shown the absence of restenosis in the first case and an asymptomatic occlusion of the stent in the second case. Its deocclusion has revealed a myocardial bridge downstream of the stent. Myocardial stunning might have caused a decreased systolic compression by the bridge in the first case, and an underestimation of its actual length in the second case. Its regression is held responsible for these two relapses. A long active stent installed at high pressure could be used to treat myocardial bridges during myocardial infarctions.

[Recurrent pericarditis revealing a malignant mediastinal mesenchymoma]. / Péricardite récidivante révélatrice d'un mésenchymome malin du médiastin.

INTRODUCTION: The malignant mesenchymoma is a malignant tumour composed of two or more types of non-differentiated tissue, associated with fibrosarcomatous elements. Its mediastinal localisation is exceptional. OBSERVATION: In a 65 year-old woman, recurrent pericardial effusion revealed a malignant mesenchymoma measuring 11 x 9 x 4 cm, located in the mediastinum and extending towards the pericardium. The histological examination of the surgical piece showed the predominance of an osteo-sarcomatous component. Complete resection was performed with partial pericardectomy, followed by adjuvant radiotherapy. CONCLUSION: Malignant mediastinal mesenchymoma is an exceptional tumour. Its diagnosis is based on anatomopathological study of a mass of anarchic composition, and its poor prognosis is related to its localisation and its capacity to relapse locally.

Association of plasma cholesteryl ester transfer protein activity and polymorphism with coronary artery disease extent in Tunisian type II diabetic patients.

INTRODUCTION: Cholesteryl ester transfer protein (CETP), a key protein in reverse cholesterol transport, has a controversial role in atherosclerosis. OBJECTIVES: : We investigated CETP activity and polymorphism in Tunisian type II diabetes and its relationship with coronary artery disease (CAD). DESIGN AND METHODS: 173 type II diabetic patients with or without CAD were compared to 67 controls. RESULTS: The HDL cholesterol concentration was low in a Tunisian population. The B1 allele of the CETP gene was associated with a low concentration of HDL cholesterol and was more frequent in Tunisians than in other populations. In type II diabetic patients, the B1 allele was associated with increased prevalence of CAD only in men (OR=0.357, CI=0.161-0.791, P=0.01). The CETP activity increased in type II diabetic patients compared to controls (P=0.05). Furthermore, the CETP activity was increased in patients with double or triple vessel disease compared to those with single vessel disease (P=0.025). CONCLUSIONS: Our data are in favour of an association between CETP and developing CAD, as well as the extent of CAD.

[Rheumatoid nodule and complete heart block: diagnosis by transesophageal echocardiography]. / Nodule rhumatoïde responsable d'un bloc auriculoventriculaire complet: diagnostic par l'échocardiographie transoesophagienne.

We report a case of 48 years old women with a 8 years history of rheumatoid arthritis and severe articular deformation treated during the last 6 months by prednisone (5 mg daily) and chloroquine (200 mg daily), admitted in the emergency room because of syncope. The electrocardiogram showed a complete atrioventricular block. Transesophageal echocardiography was performed and revealed an hyperechogenic mass (6 x 2.5 mm) in the interventricular septum probably related to a fibrous rheumatoid nodule. This potentially explain the atrioventricular block by infiltration of the conduction pathways. A permanent double chamber pacemaker was inserted. The chloroquine, another factor of conduction disturbances was not incriminated in this case. The conduction disturbances should be systematically detected in case of severe rheumatoid arthritis. Therefore, every patient must be submitted to a transthoracic echocardiography. Transesophageal echocardiography may be helpful to detect rheumatoid nodule.

Balloon mitral commissurotomy in juvenile rheumatic mitral stenosis: a ten-year clinical and echocardiographic actuarial results.

AIMS: To evaluate the safety, efficacy and long-term actuarial results of balloon mitral commissurotomy in young patients with severe rheumatic mitral stenosis. METHODS AND RESULTS: Event-free survival and freedom from restenosis were analyzed in 110 patients 20 years old or younger (group 1) and compared with those of 554 adults (group 2). Young patients were less frequently in atrial fibrillation (6% vs 35%, P<0.001) and had less mitral valve deformities (echo score 5.9+/-2.1 vs 7.5+/-3.0, P<0.0001). Mitral valve area index by 2D-echo was of 0.66+/-0.1cm(2)/m(2)in group 1 and 0.67+/-0.1cm(2)/m(2)in group 2 (P=ns) and was larger in group 1 (1.5 vs 1.3 cm(2)/m(2)) after the procedure (P<0.0001). There were more complications in group 2 (8.4% vs 0%, P=0.01). Procedural success was obtained in 110 (100%) patients of group 1 vs 501 (92%) patients of group 2 (P<0.0001). At follow-up mitral valve area index was 1.34 cm(2)/m(2)in group 1 and 1.16 cm(2)/m(2)in group 2 (P<0.0001). At 10 years, freedom from restenosis was 61% in group 1 vs 71% in group 2 (P=0.35) and event-free survival was 74% and 69% respectively (P=0.15 CONCLUSION: Balloon mitral commissurotomy is safe and effective in young with rheumatic mitral stenosis and provides better immediate results than in adults. However long-term outcome was similar between the 2 groups: 2/3 of patients were alive and free from clinical events at 10 years.

[Lipoprotein (a) and ischemic heart diseases in patients with type 2 diabetes]. / Lipoprotéine (a) et cardiopathies ischémiques chez les patients diabétiques de type 2.

Lipoprotein (a) is a new independent coronary risk factor, but the role of lipoprotein (a) in type 2 diabetes remains controversial. The objective of this study was to demonstrate the relationship between the level of lipoprotein (a) and the coronary artery diseases (CAD) in type 2 diabetes. Recruitment was carried out in 3 groups of patients: Group 1: 110 control subjects, Group 2: 115 diabetics (D), Group 3: 105 diabetics with CAD (DC). The mean age was, 51 + 7; 52 + 6; 56 + 6 respectively. Total cholesterol, triglyceride, HDL-C, LDL-C, Apo A-I, Apo B and lipoprotein (a) were measured for the patients. The Lp (a) level was significantly higher in the diabetic groups as compared to the controls (p < 0.05), but this level was different between D and DC: 312 + 232 vs 347.8 + (NS). However, when the Lp (a) level is higher than 300 mg/ml, there is a significant difference between DC and D (53% vs 42% p = 0.05). There is no correlation between Lp level and total cholesterol; however, there is a significant variation of Lp (a) level with LDL-C (r = -0.14, P = 0.01). There is a negative correlation between Lp (a) and HDL-C (r = -0.13, p = 0.03), Lp (a) and ApoA-I (r = - 0.11, p = 0.05); but there is a positive correlation between Lp (a) and ApoB (r = 0.14, p = 0.02). Lp(a) level higher than 300 mg/L constitutes a coronary risk factor in type 2 diabetes. This contributes, with the other lipid disorders, to the increase of the coronary risk factors in diabetes.

[Prognosis of infectious endocarditis]. / Pronostic de l'endocardite infectieuse.

OBJECTIVE: To study clinical course and prognostic factors of infective endocarditis. PATIENT AND METHODS: Infective endocarditis was identified in 126 consecutive patients (criteria of DURACK). Of these, 73 were male, mean age was 29.9 + 15 years, 98 (77.7%) had past history of cardiac disease. The evolution has been marked by 38 deaths (30%) in spite of the recourse to surgery (69 patients). The mean follow-up period was 52 months, the event-free survival was 61% at 5 years. RESULTS: By univariate analysis the predictors of bad prognosis: Large vegetations > 10 mm, delay of apyrexia > 10 days presence of a neurological accident and the absence of surgical treatment. Multivariate analysis: Vegetation > 10 mm (OR 1.97, 1-4.1, p = 0.05), presence of a neurological accident (OR:2.76, 1.32-5.76, p = 0.007) and the absence of surgical treatment (OR: 5.03, 2-11.4, p < 0.001). CONCLUSION: Infective endocarditis remains a serious affection, identification of patients with poor prognosis should lead to early surgical referral: this attitude provides good immediate and long-term results.

Predictors of long-term event-free survival and of freedom from restenosis after percutaneous balloon mitral commissurotomy.

BACKGROUND: Most long-term studies after balloon mitral commissurotomy (BMC) were from industrialized countries. Less is known about the long-term results of BMC from developing countries where patients are younger with fewer mitral valve deformities. METHODS: Between December 1987 and December 1998, we performed BMC in 654 patients whose mean age was 33 +/- 13 years. Baseline and postprocedural variables were evaluated to identify predictors of event-free survival (survival without repeat BMC or mitral valve replacement) and of freedom from restenosis defined as a mitral valve area (MVA) >/=1.5 cm(2) after BMC and <1.5 cm(2) at follow-up. RESULTS: The actuarial survival rates were 98%, 98%, and 97% at 5, 7, and 10 years, respectively. The 5-, 7-, and 10-year event-free survival rates were 85%, 81%, and 72%. Multivariate predictors of a higher 10-year event-free survival rate were lower echocardiographic score (79% for a score /=12, P <.001) and cardiac sinus rhythm (P =.04) before BMC, lower mean left atrial pressure (P <.001), lower mitral valve gradient (P <.001), and less than or equal to grade 2 mitral regurgitation (P =.036) after BMC. Restenosis occurred in 16% of patients. The restenosis-free rates were 88%, 80%, and 66% at 5, 7, and 10 years, respectively. A higher freedom from restenosis at 10 years was associated with a lower score (77% for a score /=12, P =.03) and a larger MVA before BMC (P =.03), a larger MVA (P <.001), and a lower mitral valve gradient (P =.04) after BMC. CONCLUSIONS: BMC produces excellent 10-year results in patients with pliable mitral stenosis and good results in patients with semipliable or calcified mitral stenosis. BMC is the procedure of choice in patients with pliable valves and it is a reasonable treatment option in young patients with unfavorable mitral valve anatomy.

[Right ventricular dilatation and intraventricular septal motion after surgical closure of atrial septal defect]. / Dilatation ventriculaire droite et cinétique du septum interventriculaire après fermeture chirurgicale de la communication interauriculaire.

The persistence of right ventricular dilatation and paradoxical interventricular septal motion are two echocardiographic abnormalities rarely reported after surgical closure of atrial septal defects. The aim of this study was to identify the predictive factors of these abnormalities in the long-term and to study their functional consequences. One hundred and two patients aged 18 +/- 14 years (range 1-62 years) underwent closure of atrial septal defects. Thirty-five patients were under 10 years of age, 33 were 10 to 20 years of age and 34 were over 20. Fifty-six patients were female. The rhythm was sinus in the great majority of cases (97%). Three patients, all over 40 years of age, were in atrial fibrillation. Before surgery, right ventricular dilatation was observed in 95 patients (91.2%), paradoxical septal wall motion in 93 patients (91.2%), the ratio of pulmonary/systemic output was 2.7 +/- 0.6 (range 1.7 to 7.4) and over 2 in 90% of patients: pulmonary systolic pressure was 32.3 +/- 12 mmHg and over 40 mmHg in 18 patients (17.6%). Ninety-four patients were followed up regularly with a mean follow-up time of 5.5 +/- 3.6 years (1-14 years). The right ventricle remained dilated in 37 patients (39.4%) after surgery: the right ventricular dimension decreased from 36 +/- 1 to 27.8 +/- 6.2 mm (p = 0.001). The ratio of end diastolic right ventricular/left ventricular dimension also decreased from 1.07 +/- 0.31 to 0.56 +/- 0.12 (p = 0.0001). Multivariate analysis identified two predictive factors of persistent right ventricular dilatation: age > 40 years (p = 0.009) and a pulmonary/systemic flow ratio > 3 (p = 0.03). Interventricular septal wall motion remained paradoxical in 21 patients (22%). Multivariate analysis identified two predictive factors of persistent paradoxical septal motion: age > 40 years (p = 0.02) and systolic pulmonary pressures > 40 mmHg (p = 0.03). These abnormalities remained asymptomatic in all but two patients with persistent long-term hypertension and a residual atrial septal defect. The persistence of right ventricular dilatation and paradoxical septal motion was quite common, with older age at surgery, systolic pulmonary artery pressure > 40 mmHg and a ratio of pulmonary/systemic blood flow > 3, being predisposing factors. These abnormalities were clinically asymptomatic when isolated.

[Unstable angina: study of in-hospital and short-term prognosis]. / Angor instable: etude du pronostic intra-hospitalier et à court terme.

UNLABELLED: The aim of our study was to identify predictor factors of coronary ischemic events to stratify the risk. 367 patients, mean age 59 years (22-90), 288 men (88%), Coronary risk factors: Smoking (62%), diabetes (38%), hypertension (37%), hypercholesterolemia (18%). BRAUNWALD class III was predominant (60%). Electrocardiographic changes were present in 113 patients. Coronary angiography identified: 148 single-vessel disease, 92 double-vessel and 68 triple-vessel. In the hospital phase, 296 patients (80.5%) were stabilised. 65 had recurrent ischemia (17.5%), 6 myocardial infarction (1.5%) and 6 deaths (1.5%). After multivariate logistic regression the predictors factors of ischemic events were. Age > or = 65 years (p = 0.03), coronary artery bypass grafting (p = 0.05), left ventricular failure (p = 0.024), modified baseline electrocardiogram (p = 0.04), ST-segment depression (p = 0.05), without aspirin (p = 0.043) and heparin (p = 0.047). At 6 months, 181 patients were asymptomatic (59.1%), 101 had recurrent ischemia (33%), 14 myocardial infarction (4.6%) and 10 deaths (3.3%). After multivariate analysis, the predictor factors were: Age > or = 65 years (p = 0.026), previous unstable angina (p = 0.023), left main stenosis (p = 0.008) and without aspirin (p = 0.003). CONCLUSION: Our study identified a subgroup of high risk patients who would benefit most from either low-molecular-weight heparins and Glycoprotein IIb/IIIa receptor blockers with an early revascularisation strategy.