[Long-term repaired tetralogy of Fallot : Echocardiographic parameters in correlation with cardiac MRI]. / Tétralogie de Fallot réparée au long terme : paramètres échographiques en corrélation avec l'IRM cardiaque.

INTRODUCTION: Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease. After surgical repair, the excellent survival rate has led to long-term complications dominated by pulmonary regurgitation (PR). Our study aimed to identify echocardiographic criteria for assessment of right ventricular (RV) function and quantification of PR correlated with cardiac MRI indices. METHODS: We conducted a descriptive study between June 2021 and March 2022 including patients followed for repaired ToF since 2016 or earlier. All patients were seen for clinical, electrocardiographic, and ultrasound evaluation. Cardiac MRI was performed with a maximum delay of 1 month from Doppler echocardiography. RESULTS: 38 patients were included, with a mean age at imaging of 18 ± 9 years. Our study showed that the echocardiographic parameters of PR quantification correlated with a pulmonary regurgitation fraction on MRI greater than 40% were: the PHT value (p = 0.005) and the ratio of PR jet width to pulmonary valve annulus diameter (p = 0.032). The only ultrasound parameter estimating RV systolic function correlated with RVEF on cardiac MRI was FAC, with p = 0.007. CONCLUSION: Quantitative assessment of RV parameters in patients followed for repaired ToF, as well as the assessment of the severity of PR, is feasible with a standardized ultrasound examination, with good correlation with cardiac MRI.

Assessment of ventricular function after total cavo-pulmonary derivation in adult patients: Interest of global longitudinal strain.

Ventricular dysfunction is the most frequent complication in adult patients post-Fontan completion. Through this work, we aim to evaluate ventricular systolic function by conventional echographic parameters and by global longitudinal strain (GLS) to determine the prediction of early ventricular systolic dysfunction. This is a prospective monocentric study enrolling 15 clinically stable adult Fontan patients with preserved ejection fraction (EF). Myocardial deformation study by GLS with speckle tracking technique in addition to a standard Doppler transthoracic echocardiography (TTE) was performed. Cardiac magnetic resonance imaging (CMR) was also performed. A comparison of echocardiographic and CMR parameters was made. In comparison to CMR-derived EF, we found a significant correlation with GLS and TTE-derived EF (P=0.003 and 0.014). We divided our population into two groups based on the cut-off value of 50% of CMR derived EF. Comparison of GLS in both groups showed a significant correlation (P=0.003). A cut-off value of -13.3% showed sensitivity of 67% and specificity of 100%. GLS has a moderate diagnostic value for systolic myocardial dysfunction in the population of adult patients with Fontan circulation.

Electocardiographic features in Ebstein's disease.

INTRODUCTION: Ebstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the electrocardiographic features of this disease. AIM: To describe the electrocardiographic features observed in Ebstein's disease. METHODS: We conducted a retrospective descriptive study that enrolled 26 patients followed for ED. RESULTS: The mean age of discovery of the ME was 103.5±99 months [0-31 years]. The diagnosis of ME is most often made between 5 and 10 years. We noted right atrial hypertrophy in 11 patients (42%), right ventricular hypertrophy in half of the patients. Right axial deviation was noted in 11 patients (42%). Eight patients (30%) had wide QRS≥ 120 ms. Seven of these 8 patients (27%) had a fragmented QRS appearance. A right bandle block was noted in 22 patients (84%), it was a complete block in 7 cases (27%). A preexcitation was found in 6 patients (23%). The localization of accessory pathway was right postero-septal in all cases. Rhythmic disorders were noted in 9 patients (34%). It was a junctional tachycardia in 3 patients (11%), atrial flutter in 4 patients (15%) and atrial fibrillation in 2 patients (7%). A second degree atriventricular block was observed in one patient, it was Mobitz I type. Two cases of postoperative rhythm disturbances were recorded: paroxysmal atrial fibrillation and junctional tachycardia related to Wolf Parkinson White (WPW) syndrome. CONCLUSION: Surface ECG in the ED is often pathological with prevalence of rhythm disturbances related to WPW syndrome.

Giant aortic arch aneurysm complicating Kawasaki disease: an original case report.

Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented, related reports on peripheral arterial and aortic aneurysms are scarce. We report an original case of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28 months- old patient diagnosed with Kawasaki Disease. This complication was managed by steroids therapy in the beginning than surgery was indicated because of mechanical complication and risk of rupture.

Cardiac anomalies in Cantrell's pentalogy: From ventricular diverticulum to complete thoracic ectopia cordis.

Cantrell's pentalogy is a very rare syndrome associating varying degrees of midline wall defects and congenital cardiac anomalies. It is characterized by a combination of five anomalies that are: a midline supra umbilical abdominal wall defect, a sternal defect, an anterior diaphragmatic defect, a diaphragmatic pericardial defect and a congenital intra cardiac defect. Ectopia cordis, defined as a developmental defect in which the heart is abnormally located partially or totally outside the thorax, is in some cases a part of this syndrome. We report two cases of Cantrell's pentalogy in which cardiac ectopia was complete in one case and limited to left ventricular diverticulum in the other case. Both cases had a common intracardiac defect which is a double outlet right ventricle. The first case underwent surgical repair of the intracardiac lesions with resection of the diverticulum associated to repair of the midline defects with good outcome. The second case that presented with complete extra thoracic ectopia cordis died because of sepsis. We review through this article the main characteristics of Cantrell's pentalogy, we highlight the diversity of anatomic lesions and study the prognosis of this syndrome.