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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.

BackgroundThe FAST-MI Tunisia registry was set up by the Tunisian Society of Cardiology and Cardiovascular Surgery to assess the demographic and clinical characteristics, management and hospital outcome of patients with ST-elevation myocardial infarction (STEMI).MethodsData for 459 consecutive patients (mean age 60.8 years; 88.5% male) with STEMI, treated in 16 public hospitals (representing 72.2% of public hospitals in Tunisia treating STEMI patients), were collected prospectively.The most common risk factors were smoking (63.6%), hypertension (39.7%), diabetes (32%) and dyslipidaemia (18.2%).ResultsAmong the 459 patients, 61.8% received reperfusion therapy: 30% with primary percutaneous coronary intervention (PPCI) and 31.8% with intravenous fibrinolysis (IF) (28.6% with pre-hospital thrombolysis). The median time from symptom onset to thrombolysis was 185 min and to PPCI was 358 min. In-hospital mortality was 5.3%. Compared with those managed at regional hospitals, patients managed at interventional university hospitals (n = 357) were more likely to receive reperfusion therapy (52.9% vs. 34.1%; pConclusionsData from the FAST-MI Tunisia registry show that a pharmaco-invasive strategy of management for STEMI should be promoted in non-interventional regional hospitals.

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 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.

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: 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.

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 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.

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.