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Background Contemporary registries on atrial fibrillation (AF) are scare in North African countries. Hypothesis In the context of the epidemiological transition, prevalence of valvular AF in Tunisia has decreased and the quality of management is still suboptimal. Methods NATURE‐AF is a prospective Tunisian registry, involving consecutive patients with AF from March 1, 2017 to May 31, 2017, with a one‐year follow‐up period. All the patients with an Electrocardiogram‐documented AF, confirmed in the year prior to enrolment were eligible. The epidemiological characteristics and outcomes were described. Results A total of 915 patients were included in this study, with a mean age of 64.3 ± 22 years and a male/female sex ratio of 0.93. Valvular AF was identified in 22.4% of the patients. The mean CHA2DS2VASC score in nonvalvular AF was 2.4 ± 1.6. Monotherapy with antiplatelet agents was prescribed for 13.8% of the patients. However, 21.7% of the subjects did not receive any antithrombotic agent. Oral anticoagulants were prescribed for half of the patients with a low embolic risk score. In 341 patients, the mean time in therapeutic range was 48.87 ± 28.69%. Amiodarone was the most common antiarrhythmic agent used (52.6%). During a 12‐month follow‐up period, 15 patients (1.64%) had thromboembolism, 53 patients (5.8%) had major hemorrhage, and 52 patients (5.7%) died. Conclusions NATURE‐AF has provided systematic collection of contemporary data regarding the epidemiological and clinical characteristics as well as the management of AF by cardiologists in Tunisia. Valvular AF is still prevalent and the quality of anticoagulation was suboptimal.

Objectives The aim of this study was to evaluate 1-year clinical safety and efficacy of the dual-therapy COMBO stent in a large, all-comers patient-level pooled cohort. Background The COMBO stent (OrbusNeich Medical, Fort Lauderdale, Florida) is a novel stent with abluminal sirolimus elution from a biodegradable polymer and a luminal pro-healing anti-CD34+ antibody layer, which attracts circulating endothelial progenitor cells. These endothelial progenitor cells can quickly mature into normal endothelium, providing rapid endothelialization. Methods The MASCOT (Multinational Abluminal Sirolimus Coated biO-engineered stenT) (N = 2,614, 61 global sites) and REMEDEE (Randomized study to Evaluate the safety and effectiveness of an abluMinal sirolimus coatED bio-Engineered StEnt Post Market Registry) (N = 1,000, 9 European sites) registries are 2 prospective, multicenter studies evaluating clinical outcomes after attempted COMBO stent placement in all-comer patients undergoing percutaneous coronary intervention. In this patient-level pooled analysis we analyzed 1-year target lesion failure (TLF), a composite of cardiac death, target vessel myocardial infarction, or clinically driven target lesion revascularization. Furthermore, we determined predictors of 1-year TLF. Results A total of 3,614 patients (63.5 ± 11.2 years of age; 23.8% women) were included in this analysis. The prevalence of diabetes mellitus was 29.3%, and 54.3% patients presented with acute coronary syndrome. The primary endpoint of 1-year TLF occurred in 140 (3.9%) patients, with incidence of cardiac death in 1.6%, target vessel myocardial infarction in 1.2%, clinically driven target lesion revascularization in 2.2%, and definite stent thrombosis in 0.5% patients. Insulin-treated diabetes mellitus, chronic renal failure, and American College of Cardiology/American Heart Association lesion type B2/C were independent predictors of 1-year TLF. Conclusions In this large patient-level pooled analysis of patients treated with the dual-therapy COMBO stent excellent results at 1-year were observed. (MASCOT - Post Marketing Registry [MASCOT]; NCT02183454; Prospective Registry to Assess the Long-term Safety and Performance of the COMBO Stent [REMEDEE Reg]; NCT01874002)

We aimed to study potential variables involved in interindividual variability to acenocoumarol (AC) response in order to establish a pharmacogenetic algorithm (PA) that includes clinical and genetic factors to predict adequate AC dose to stabilize anticoagulation in a cohort of Tunisian patients. Genotyping of the CYP2C9, VKORC1, CYP4F2, and CALU polymorphisms was conducted on 246 patients using PCR-RFLP technique. AC normalized maintenance dose (NMD): ((mean maintenance dose/international normalized ratio (INR)) equilibrium) was calculated. The statistical study was carried out with SPSS V20. A significant correlation was found between age, BMI, and daily AC dose (r = − 0.397; p

In this study, we describe the case of a 5-year-old boy who presumably presented with Loeys–Dietz syndrome. A huge aneurysm of the ascending aorta and the aortic arch extended beyond the left subclavian artery and was accompanied by a slight narrowing of the aortic isthmus.

Congenitally corrected transposition of the great arteries is a rare heart defect that can be associated with systemic ventricular dysfunction and conduction disturbances. The use of cardiac resynchronization therapy in patients with congenital heart disease is not fully established, and achievement of successful pregnancies after implantation of transvenous, biventricular system has never been described, and which resulted in a significant clinical improvement. We describe a 33-year-old female with congenitally corrected transposition of the great arteries, who achieved six pregnancies and successful vaginal deliveries. The two last pregnancies were achieved after cardiac resynchronization therapy for systemic ventricular dysfunction and complete heart block. A congenital cardiac disease has been identified in only one offspring.

Cardiac hemangiomas are benign tumors with an unpredictable natural history. Surgical resection is the treatment of choice; however, conservative management can be an alternative in some patients. We report a case of a left-sided cardiac hemangioma that we managed conservatively for 11 years without obvious major complications in the patient, an adult woman.

Background Unprotected left main coronary artery (ULMCA) disease is seen approximately 4% of patients who undergo angiography. Percutaneous coronary intervention (PCI) is an accepted alternative to surgery for the treatment of this disease. Purpose Aim of our study is to evaluate the results of our experience in ULMCA PCI and assess prognostic factors associated with such procedures. Methods Between January 2005 and August 2016, 109 consecutive procedures of LMCA PCI were assigned retrospectively. Clinical variables, the reasons for cardiac catheterization, therapeutic decisions and clinical evolution in long-term follow-up were analyzed. Results The mean age of our population was 62.27 ± 10.68 years and predominantly male (72%). Diabetes mellitus was found in 63 patients (58%). Presentation on admission was mainly stable angina in 40 patients (37%). True bifurcation lesions (Medina 1-1-1 and 1-0-1) were present in 56 distal LMCA patients (51%). Triple vessel disease was associated in 21 patients (19%). The Syntax score was Conclusion The results of our growing experience in LMCA PCI are encouraging with high rate of procedural success and acceptable medium and long-term results.

Background The persistent ductus arteriosus remains a common congenital pathology. The percutaneous closure has become the main technique for the closure of patent ductus arteriosus. Purpose Evaluate the short-, medium- and long-term results of percutaneous closure of patent ductus arteriosus, performed in Cardiology department of Sahloul University Hospital. Methods Children and adults who underwent percutaneous closure of patent ductus arteriosus in Cardiology department of Sahloul University Hospital, during 13 years between September 2003 and June 2016 were retrospectively included. We studied procedure's Results success rate, type of device, residual shunting, and adverse effects. Results One hundred and fifty-three procedures were realized during the study period. Patient's mean age at the time of procedure was 73.68 months (3 months to 53 years) and the sex ratio was 1.8 female for 1 male. The main circumstance for discovery was dyspnea (56.86%). The average echocardiographic persistent ductus arteriosus diameter was 4.7 mm. The average angiographic persistent ductus arteriosus diameter was 8.7 mm. Ninety-seven patients (63.4%), had a channel A-type, 30 patients (19.6%) had C type and 22 patients (17%) had E type on the Krichenko classification. A device was implanted in 140 cases (91.5%): 8 coils, and 132 Amplatz® devices. The failure rate of the procedure was 2.77%. The complications occurred in 11 patients (7.18%): 3 cases of immediate embolization, 3 cases of pulmonary protrusion, one case of inguinal hematoma and 4 cases of aortic protrusion with risk of aortic subocclusion in one of them. The device was removed immediately in this case. After a device was installed, complete occlusion was obtained in 85.8% at day 1, 90% at a month and 100% at 12 months. The average duration of the procedure 54 min (30 to 121 min). Conclusion Patent ductus arteriosus percutaneous closure gives good results with a low rate of complication.

Background Coronary chronic total occlusion (CTO) are associated with poor prognosis and cardiac death. Successful chronic total occlusions (CTO) percutaneous coronary intervention (PCI) is associated with a clinical benefit. However, in a substantial portion of cases, PCI is not attempted, and patients are treated with optimal medical therapy (OMT). Purpose The aim of this study was to compare long-term outcomes of CTO after OMT versus PCI. Methods Between 2006 to 2017, 144 patients with CTO were included. Patients who underwent coronary artery bypass grafting were excluded. We classified patients into the OMT group (n = 65) or PCI group (n = 62) according to initial treatment strategy. Endpoint of the study was long-term cardiac death. Results The median follow-up duration was 44 (interquartile range: 19–74) months. Baselines characteristics did not significantly differ between the two groups: mean age was 62.16 ± 10 vs. 61.52 ± 9 years; Left-ventricular ejection fraction (LVEF) 48 ± 11% vs. 50 ± 13%; Diabetes 48% vs. 46%; Acute coronary syndrome 52% vs. 48%. In the PCI group, 46 patients (74.2%) underwent successful revascularization. Kaplan–Meier plot with log rank analysis shows cumulative death was significantly lower in the PCI group than in the OMT group (P = 0.023; average follow up duration; PCI vs. OMT group = 131.52 ± 6.8 vs. 96.33 ± 8.2 months). Conclusions As an initial treatment strategy, PCI reduce cardiac death compared with OMT for the treatment of CTO.

Background In patients with left main coronary artery (LMCA) stenosis, PCI with drug-eluting stents may be an acceptable alternative to CABG. However, data from African subcontinent are lacking. Purpose We sought to evaluate trends in treatment strategies of LMCA disease over time in Sahloul University Hospital and to compare early and long-term adverse outcomes of each therapeutic option. Methods From 2005 to 2016, 260 patients with unprotected LMCA were included. In total, 102 patients underwent Surgery, 109 patients underwent PCI and 49 patients were medically treated. Results Over time, the proportion of patients treated with PCI rather than CABG increased substantially. Patients treated with PCI had more anterior ST-segment elevation myocardial infarction (MI) and cardiogenic shock at presentation compared to CABG group. More patients treated with CABG had multivessel disease, more distal LMCA bifurcation and higher SYNTAX scores. All the other baseline variables were similar. After a follow-up of 39 ± 26 months in PCI group and 52 ± 38 months in CABG group, there were no differences between PCI and CABG, at the adjusted analysis, in the rate of myocardial infarction (MI) (HR: 1.75; 95%, CI: 0.55 to 5.50; P = 0.33), cerebrovascular accidents (CVA) (P = 0.69), and the composite of MACCE (HR: 1.04; 95% CI: 0.59 to 1.83; P = 0.88). Compared to PCI group, CABG group has a higher all-cause mortality (P = 0.017) driven exclusively by an elevated incidence of operative mortality (13.7% vs. 6.4%; HR: 0.08; 95% CI: 0.017 to 0.43; P = 0.003). Nevertheless, long-term advantage of CABG over PCI was the less need for repeated revascularization (HR: 3.1; 95% CI: 1.26 to 8.12; P = 0.014). Medically treated patients produced a four–year all cause death rate of 44%. Conclusion Revascularization therapy of LMCA stenosis have evolved remarkably over the last decade in our faculty. PCI and CABG show comparable safety. However, repeat revascularization is more common after PCI.

Background The persistent ductus arteriosus remains a common congenital pathology. Although percutaneous closure is an attractive alternative to the surgical treatment for infants weighing less than 6 kg. Purpose The objective was to evaluate the efficacy and safety of percutaneous closure in low-weight children. Methods Children weighing less than 6 kg who underwent percutaneous closure of patent ductus arteriosus in Cardiology department of Sahloul University Hospital during 13 years between September 2003 and June 2016 were retrospectively included. Results Eighteen children (mean weight: 5.49 kg [range: 4.2–6]; mean age: 10.9 months [range: 3–85]) were included. The main circumstance for discovery was difficulty in breathing (100%). The average angiographic persistent ductus arteriosus diameter was 3.08 mm [range: 1.12–5.68 mm]. Fifteen children (83.35%) had pulmonary hypertension of cases. Eleven patients (61.2%) had a channel A-type, 5 patients (27.8%) had C type, un patient (5.5%) had D type and un patient (5.5%) had E type on the Krichenko classification. A device was implanted in all cases. The prosthesis was implanted successfully in 94.44 of cases. The only failure was explained by immediate embolization of the device. The average duration of the procedure was 50 ± 10 min. After a device was installed, complete occlusion was obtained in 90% at day 1, 92% at a month and 100% at 12 months. Two complications occurred: pulmonary protrusion in one case and aortic protrusion in another case. No late embolization occurred after 37.5 months of median follow-up [range: 3–138]. During this monitoring, we noted a marked clinical improvement with normalization of pulmonary pressure. Conclusion The study shows the feasibility and the efficacy of percutaneous closure in persistent ductus arteriosus in infants weighing less than 6 kg. It confirms the effectiveness of the procedure with a relatively low prevalence of complications.

B-type natriuretic peptide (BNP) is a biomarker of cardiovascular disease that is common in adults with chronic kidney disease (CKD). However, in children with CKD, the range and predictive power of BNP concentrations are not known. We aimed to determine the effect of HD on BNP, as well as the prognostic impact of BNP, in end-stage renal disease (ESRD) children undergoing hemodialysis (HD). Thirty-five children with chronic renal failure (16 boys age 12.1 ± 3.7 years) on maintenance HD were included. BNP level was measured, and Doppler echocardiography was performed 30 min before (pre-HD BNP) and 30 min after (post-HD BNP) HD in each patient. An adverse event was defined as all-cause death and heart failure hospitalization. The median pre-HD BNP, the post-HD BNP, and the change in BNP were, respectively, 240 pg/ml (72 to 3346), 318 pg/ml (79 to 3788), and 9 pg/ml (-442 to 1889). Pre-HD BNP concentration was negatively correlated with left ventricular (LV) ejection fraction (r = -0.41, P = 0.018). During a mean follow-up of 39 ± 14 months, 6 patients died, and 3 were hospitalized for heart failure. Using univariate analysis, BNP before and after HD as well as Doppler tissue imaging velocities had a strong graded relationship with adverse events. Cox proportional hazards model demonstrated that pre-HD body weight (P = 0.008), pre-HD BNP (P = 0.011), and post-HD BNP (P = 0.038) remained independent predictors of adverse outcome. Even in case of ESRD, BNP still strongly correlated with LV systolic and diastolic dysfunction and was associated with mortality in HD children.

Background: Most clinical studies of the clinical profile of Brugada syndrome (BS) have been conducted in either Asia, Europe, or America and their applicability to North African populations is largely unknown. The aim of the study was to analyze the clinical profile of BS in Tunisian patients. Methods: The clinical and follow-up data of 24 patients (22 men, mean age: 40.8 ± 13.7 years) were collected since 2002. Baseline characteristics, morbidity, and mortality data were obtained from medical records. Results: One patient (4.16%) survived sudden cardiac death (SCD), four patients (16.3%) had syncope, and 19 patients (79.1%) were asymptomatic. Eleven patients (45.8%) had a family history of SCD. Twenty patients showed a spontaneous coved-type ST-segment elevation on electrocardiogram and after medical challenge on the four remnants. An electrophysiological study was performed in 15 of 24 patients (62.5%), during which ventricular fibrillation was induced in six patients (40%); three of the six patients were previously asymptomatic. An implantable cardioverter defibrillator (ICD) was implanted in 14 patients (58.3%). After a mean follow-up of 26 ± 21 months, one patient died from a noncardiac cause and one patient (with a history of aborted SCD) received an appropriate shock from his ICD. None of the asymptomatic and noninducible patients experienced a cardiac event. Conclusions: BS is present in the North African population and is probably under-recognized. Tunisian patients with BS share with their western and Asiatic counterparts similar clinical profile. (PACE 2011; 47–53)

Background: Allergic reaction to pacemaker compounds is a rare complication of cardiac pacing. Initial management is difficult because accurate diagnosis is often delayed. The tendency is to initially suspect a bacterial infection, rather than to quickly rule out an allergy to the pacemaker components. Management of this condition is difficult and not well established. Case presentation: A 75-year-old man underwent a dual chamber pacemaker implantation. The patient needed two generator re-implantations because of sterile skin necrosis. Pace maker allergic reaction was suspected despite non-conclusive skin patch testing. The patient underwent pacemaker system removal and re-implantation of poly-tetra-fluoro-ethylene sheet coated generator in a retropectoral position. Subsequently, there has been no externalization or recurrence in nine years of follow-up. Take-away lesson: Contact allergy to pacemakers is often unrecognized. Once infection has been excluded, allergy testing must be performed. The only valuable treatment is the removal of all the system components, followed by a replacement with hypoallergenic material. Polytetrafluoroethylene coated materials can be effective to prevent recurrence.

Background: Atrial septal defect (ASD) is often an isolated disease, but its association with other abnormalities can make diagnosis challenging. Careful analysis of simple complementary exams can help precise anatomical diagnosis ensuring suitable treatment. The aim of this article is to report, from a case report and literature review, diagnostic challenges and the contribution of simple complementary exams, such as chest X-ray, for the diagnostic orientation of an ASD associated with peripheral pulmonary artery stenosis, as well as therapeutic particularities. Case report: We report the case of a girl born in 2007, with history of dyspnoea and recurrent bronchitis in whom a loud systolic murmur was detected fortuitously at the age of 2 years. Her clinical examination was otherwise normal. The electrocardiogram recorded sinus rhythm, incomplete right bundle branch block, and right ventricular hypertrophy. Chest X-ray showed moderate cardiomegaly and hypervascularity of the left lung field contrasting with reduced blood flow to the right lung. Doppler echocardiography revealed a wide ostium secundum ASD, right chamber volume overload and right pulmonary artery stenosis. The latter was confirmed by CT angiography and right cardiac catheterization. The patient underwent percutaneous right pulmonary artery dilation with stent placement. Control chest X-ray noted bilateral hypervascularity of the lung. The ASD was closed percutaneously one year later. The outcome was uneventful. Conclusion: The combination of ASD with pulmonary artery stenosis limits pulmonary hyperflow. In our case, this stenosis was tight and sat on the right branch of the pulmonary artery reducing significantly blood flow to the ipsilateral lung. Careful chest X-ray analysis may suggest diagnosis, which can be confirmed by ultrasounds and if necessary, by further examination, allowing treatment adaptation. To our knowledge, this association is very rare and no similar case has been reported.

Cardiobacterium hominis, a member of the HACEK group of organisms, is a rare cause of endocarditis and is even rarer in pediatric population. In this report, we present a case of infective endocarditis caused by C. hominis in a 16-year-old Tunisian girl who had undergone right ventricular outflow tract reconstruction using a Hancock® heterograft for double outlet right ventricle with pulmonary stenosis. Two weeks before admission, the patient suffered from worsened shortness of breath and fever. Tranthoracic echocardiography revealed right ventricular outflow tract stenosis and vegetation attached to the leaflet conduit. The Subsequent blood cultures grew Cardiobacterium hominis and the patient was treated successfully with 6 weeks of intravenous ceftriaxone therapy. Conduit replacement was performed after appropriate antibiotic therapy with favourable course.

Introduction: Primary cardiac tumors are uncommon during infancy and childhood. Myxomas originating from the right ventricle are even less common in pediatric patients. Case presentation: Here we describe a case of an 11-year-old Tunisian boy who was referred for syncope. Transthoracic echocardiography revealed a large mobile mass attached to his right ventricle, obstructing his right ventricular outflow tract. Complete surgical excision of the mass with preservation of the pulmonary valve was performed. The diagnosis of myxoma was histologically confirmed. Conclusion: Cardiac myxomas located in the right ventricular outflow tract are rare and can present unusual diagnostic and therapeutic challenges.

Introduction A Fontan operation is performed to provide palliation for patients with many forms of highly complex congenital heart disease that cannot support a biventricular circulation. Increasing numbers of women who have undergone these connections in childhood are now reaching their childbearing years, and some are becoming pregnant. The low flow and fixed cardiac output of a Fontan circulation poses several problems during pregnancy. Case presentation We report the case of four successful pregnancies in a 31-year-old Tunisian woman with congenital tricuspid atresia after Fontan operation. Her pregnancies resulted in delivery of four healthy neonates. Her clinical status remained unchanged. Conclusions This case suggests that patients after adequate Fontan palliation could complete pregnancy without long-term cardiac sequelae. Intensive care should be provided with specialists, including a neonatologist, anesthesiologist and cardiologist.

Introduction Dyglycemia on admission is associated with increased mortality rates in patients with ST-elevation myocardial infarction (STEMI). Aim Investigate the relationship between dysglycemia and angiographic and clinical outcome after primary or rescue angioplasty for STEMI. Methods We prospectively included 188 patients who underwent revascularization for STEMI. We compared intrahospital outcomes between the groups of patients with HG (HG + ) and without HG (HG-). Plasma glucose was measured at hospital admission. HG was defined as plasma glucose > 11 mmol/L. Results Among the overall population, 60 (31.9 %) patients had HG. The mean age was 60.77 ± 11.79 years. They were more frequently men (84.4 %) with a more frequent history of diabetes and hypertension. A total of 19 cases (31.7 % of the study group) of newly diagnosed diabetes mellitus were registered. In subjects with higher admission glucose the maximal levels of troponin I were higher, as well as maximal values of creatine phosphokinase. Coronary status was more severe in HG+ (52.6 % vs. 47.4 %, p = 0.003). Procedural success was lower in the HG+ group (31.4 % vs. 68.6 %) with higher rates of per-procedural complications (51.6 % vs. 48.4 %, p = 0.019). Intrahospital outcomes were worse in the HG+ group as attested by a higher mortality (3.19 % vs. 4.25 %, p = 0.043). Those outcomes were similar in the HG+ group regardless to the diabetic status. Conclusion Hyperglycemia on admission is associated with greater myocardial injury and an increased risk of major adverse cardiovascular events at long-term follow-up.

Introduction Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly and one of the causes of myocardial ischemia. The usual clinical course is severe left-sided heart failure and mitral valve insufficiency presenting during the first months of life. Case presentation We report the case of a 6-month-old Tunisian girl who presented with dilated cardiomyopathy. Echocardiography suspected anomalous origin of the left coronary artery. The definitive diagnosis of anomalous origin of the left coronary artery from the pulmonary artery was reached by multislice computed tomography and coronary angiography. Conclusion In cases of dilated cardiomyopathy, anomalous origin of the left coronary artery from the pulmonary artery syndrome has to be kept in mind as a surgically correctable cause.

La cardiomyopathie hypertrophique neonatale est une entite rare, heterogene regroupant plusieurs formes cliniques et donc de diagnostic etiologique difficile. Nous rapportons l�observation d�un nouveau ne issu d�une grossesse gemellaire, ayant presente a la naissance un tableau d�insuffisance cardiaque, l�echocardiographie avait conclut a une cardiomyopathie hypertrophique obstructive. Le bilan etiologique etait negatif notamment une mere non diabetique. L�evolution etait favorable avec regression de l�hypertrophie 2 semaines apres la naissance. L�etiologie finalement suggeree etait une cardiomyopathie secondaire a l�injection antenatale de corticoides dans le but d�accelerer la maturation pulmonaire. L�etablissement par les societes savantes d�un consensus de bilan etiologique minimal standard selon une chronologie bien determinee serait d�un grand apport dans la prise en charge de cette anomalie.

Combination of ruptured sinus of Valsalva aneurysm (SVA), and a coexisting asymptomatic adult aortic isthmic coarctation is extremely rare. The timing and sequence of surgical and/or interventional repair of these two pathologies are controversial. We present a case of a 37-year-old male who was admitted to our department because of severe acute congestive heart failure and signs of ruptured aneurysm of the SV into the right ventricle. Transthoracic and transoesophageal echocardiography confirmed the communication between an important right coronary SVA and right ventricle, bicuspid aortic valve, mild aortic regurgitation, and revealed severe aortic coarctation. Because of the severe dilation of right sinus of Valsalva a surgical repair of the ruptured aneurysm was performed. Aortic coarctation was treated four weeks later by a percutaneous stent-graft implantation. This case report supports the concept that hybrid approach is feasible in patients with ruptured SVA and aortic coarctation in adulthood.

Background Despite successful repair of aortic coarctation (AoC), changes in the left ventricular (LV) regional myocardial function are reported. The aim of this study was to assess the left ventricular systolic function in patients after the successful repair of aortic coarctation using tissue Doppler imaging (TDI) according to the age of aortic coarctation repair. Methods 31 patients (mean age, 12.0 +/− 4.2 years) followed up after a successful chirurgical repair of aortic coarctation (mean follow up, 8,6 ± 7,6 years) were studied by echocardiography. The study population was divided in two groups: patients repaired at age 2 years (Group 1, n = 17) and patients repaired at age > 2 years (group 2, n = 14). The TDI parameters and the conventional echocardiographic indices of the left ventricular systolic function were analyzed and compared between the two groups. Results Standard echocardiographic studies revealed normal global left ventricular (LV) function. The LV dimensions, LV ejection fraction, LV shortening fraction, indexed LV mass did not differ between the examined groups. Among the studied LV filling parameters, peak early (E wave) transmitral flow velocity, as well as deceleration time DT, was similar between the two groups. The Tei index was also not affected by the age at repair of aortic coarctation. Regarding TDI measures, the early diastolic and the systolic velocities on the lateral side of the mitral annulus were significantly higher in group 1 than in group 2 (Sa: 12,7 ± 2,59 cm/s vs 10,6 ± 1,8 cm/s, p = 0,025; Ea : 20,7 ± 3,4 cm/s vs 17,3 ± 4,7 cm/s, p = 0,03). TDI velocities on the septal side of the mitral annulus and on the right ventricle (RV) free wall side of the tricuspid annulus were not different between early (before 2 years) and late (after 2 years) repair of the aortic coarctation. There were no differences of the TDI or conventional parameters between hypertensive and normotensive patients. Conclusion Left ventricular systolic performance in patients after the surgical repair of aortic coarctation reveals tendency to decrease at follow-up in patients repaired at age > 2 years despite a satisfactory results after surgery.

IntroductionCoarctation of the aorta (CoA) is a stenosis usually located in the isthmus of the descending aorta. Treatment consists of surgical or percutaneous removal of the obstruction and may present excellent immediate results. However, despite immediate good results, significant residual problems often persist. The aim of the study is to describe the presentation, treatment and long-term evolution of a population of 48 unselected consecutive patients with CoA in a single pediatric cardiology center.MethodsThis was a retrospective study of all patients with isolated CoA associated or not to either atrial or ventricular septal defects. RESULTS: The patients (n = 48, 56,3% male) were diagnosed at a mean age of 84 ± 109 months. The clinical presentation differed between patients aged less or more than two years, the former presenting with heart failure and the latter being asymptomatic with evidence of hypertension (p < 0.01). Treatment was surgical in all cases (32 end-to-end anastomosis). The mean age of patients was 94 ± 109 months. There was two late deaths, in a mean follow-up of 8,6 ± 7,7 years. Recoarctation occurred in 12 patients (25%). There are patients who currently have hypertension (17 at rest, 2 with effort), their mean age at diagnosis being older than the others (128 vs. 76 months; p < 0.05). Aortic aneurysms occurred in five patients (10,4%). Aneurysm was associated to bicuspid aortic valve in 3 cases.Conclusionsrepaired CoA has a significant incidence of long-term complications, and should thus no longer be seen as a simple obstruction in the descending aorta, but rather as a complex pathology that requires careful follow-up after treatment.

Summary Background There is a lack of Tunisian data on the frequency and clinical significance of different coronary artery anomalies. Methods All patients who underwent coronary angiography from March 1996 to December 2006 were considered. Only patients with congenital anomalous aortic origin of the coronary artery were included. Results Among 7330 adult patients who underwent diagnostic coronary angiography, 20 (0.27%) patients (13 men; mean age 53.3 years) had anomalies of the coronary artery origin. The right coronary artery was the vessel involved most frequently ( n = 10); it originated separately from the left sinus of Valsalva (SV) in three patients and from the posterior sinus of Valsalva in one patient. In the other patients, it arose from the left main coronary artery or its branches in a single coronary artery originating from the left sinus of Valsalva. Isolated anomalous left circumflex artery was the second most frequent anomaly ( n = 6). Isolated anomalous left anterior descending artery was seen in one patient. A single coronary artery arising from the right SV was seen in three patients. Atheroslerotic lesions were seen in eight cases. Four patients underwent coronary revascularization; the remainder received medical management. All 20 patients are alive and had an uneventful follow-up (mean 34.2 months). Conclusions In Tunisia, the incidence of congenital anomalous aortic origin of the coronary artery in adults is 0.27%. The right coronary artery is involved most frequently. Medical management seems promising.

Ellis-van Creveld syndrome (EvC) is an autosomal recessive inherited disease resulting from mutations in EVC1 or EVC2. Patients with this condition normally have chondrodysplasia, postaxial polydactyly, ectodermal dysplasia and congenital heart defects. We report the case of a 13-year-old Tunisian child who was admitted for cyanosis and acute heart failure. On clinical examination, he presented with typical features of EvC, cyanosis and dyspnea. EvC was confirmed by genetic tests, and echocardiography showed a partial atrioventricular canal defect with supra-systemic pulmonary artery pressure. The patient was treated; however, the evolution was fatal.

Introduction Right ventricular apical pacing-related heart failure is reported in some patients after long-term pacing. The exact mechanism is not yet clear but may be related to left ventricular dyssynchrony induced by right ventricular apical pacing. Right ventricular septal pacing is thought to deteriorate left ventricular function less frequently because of a more normal left ventricular activation pattern. Case presentation We report the case of a 55-year-old Tunisian woman with preserved ventricular function, implanted with a dual-chamber pacemaker for complete atrioventricular block. Right ventricular septal pacing induced a major ventricular dyssynchrony, severe left ventricular ejection fraction deterioration and symptoms of congestive heart failure. Upgrading to a biventricular device was associated with a decrease in the symptoms and the ventricular dyssynchrony, and an increase of left ventricular ejection fraction. Conclusion Right ventricular septal pacing can induce reversible left ventricular dysfunction and heart failure secondary to left ventricular dyssynchrony. This complication remains an unpredictable complication of right ventricular septal pacing.

Introduction Congenital coronary artery fistula is a rare anomaly that may cause angina, atrial fibrillation, endocarditis, aneurysmal dilation and myocardial infarction. Both spontaneous regression and life-threatening complications have been described. Treatment can be conservative, surgical or more recently through transcatheter closure. Case presentation We report the case of a 27-year-old Tunisian man with a large coronary artery fistula from the left anterior descending artery to the right ventricle associated with pulmonary stenosis. This patient underwent a successful transcatheter closure of his coronary artery fistula followed by pulmonary dilatation and had an uneventful recovery after treatment. Conclusions Transcatheter closure of a congenital coronary artery fistula is feasible and should be considered in carefully selected patients. Recanalization of the treated coronary fistula can occur, so follow-up angiography or other imaging modality should be performed in these patients.