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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.

Despite clinical importance of ventricular repolarisation, it remains difficult to analyse. Conventionally, quantification of the electrocardiographic ventricular repolarization is usually performed with reference to axis of the T wave and QT interval duration. A variety of factors can prolong the QT interval, such as drug effects, electrolyte imbalances, and myocardial ischemia. The biggest risk with prolongation of the QT interval is the development of torsades de pointes. Commonly accepted reference ranges for the electrocardiogram (ECG) have been in use, with little change, for many years. Populations throughout the world present several differences: age, ethnic compositions, and are exposed to different environmental factors. Recent studies have reported reference data for QT interval in healthy population and have evaluated the influence of age, gender, QRS duration and heart rate on this interval. In this review, we address several issues relative to the measurement, and interpretation of QT interval and its adjustment for rate, age, gender and QRS duration.

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.

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.

Takayasu arteritis is an inflammatory condition that involves the large cardiac vessels, predominantly the aorta and its main branches. It typically affects young women (age, ≤40 yr), most often Asians and Latin Americans. Herein, we describe a rare manifestation of Takayasu arteritis in a 19-year-old black Tunisian man who presented with acute inferior myocardial infarction and complete atrioventricular block after occlusion from a giant aneurysm in the right coronary artery. The coronary artery disease was associated with aneurysmal dilations in the carotid, vertebral, and right renal arteries. Medical therapy improved Thrombolysis in Myocardial Infarction flow in the area of the giant aneurysm from grade 1 to grade 3. Upon the diagnosis of Takayasu arteritis, intravenous methylprednisolone and oral prednisone therapy was started. After 10 days of hospitalization, the patient was discharged on a medical regimen. Renovascular hypertension due to renal artery stenosis was suspected, so he underwent successful percutaneous transluminal angioplasty of the inferior segmental artery of the right renal artery. During 12 months of close postprocedural monitoring, he experienced lower blood pressure, no chest pain, and no cardiovascular complications.This association of conditions has not been previously reported. Besides presenting this very rare combination of findings, we discuss the differential diagnosis of Takayasu arteritis in our patient.

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.

Dual-chamber pacing is believed to have an advantage over single-chamber ventricular pacing. The aim of the study was to determine whether elderly patients with implanted pacemaker for complete atrioventricular block gain significant benefit from dual-chamber (DDD) compared with single-chamber ventricular demand (VVIR).The study was designed as a double-blind randomized two-period crossover study-each pacing mode was maintained for 3 months. Thirty patients (eight men, mean age 76.5 +/- 4.3 years) with implanted PM were submitted to a standard protocol, which included an interview, functional class assessment, quality of life (QoL) questionnaires, 6-minute walk test, and transthoracic echocardiographic examinations. QoL was measured by the SF-36. All these parameters were obtained on DDD mode pacing and VVIR mode pacing. Paired data were compared.QoL was significantly different between the two groups and showed the best values in DDD. Overall, no patient preferred VVIR mode, 18 preferred DDD mode, and 12 expressed no preference. No differences in mean walking distances were observed between patients with single-chamber and dual-chamber pacing. VVI pacing elicited marked decrease in left ventricle ejection fraction and significant enlargement of the left atrium. DDD pacing resulted in significant increase of the peak systolic velocities in lateral mitral annulus and septal mitral annulus. Early diastolic velocities on both sides of mitral annulus did not change.In active elderly patients with complete heart block, DDD pacing is associated with improved quality of life and systolic ventricular function compared with VVI pacing.

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.

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.