Mohamedou Ly

Results of the double switch operation for congenitally corrected transposition of the great arteries

BACKGROUND Congenitally corrected TGA (CC-TGA) is characterized by discordant atrioventricular and ventriculo arterial connections. In absence of right ventricular outflow tract obstruction (RVOTO), repair by atrial and arterial switches remains a challenging procedure for which long term follow-up is uncertain. METHODS From 1995 to 2007, 20 patients (median age: 26 months) with CC-TGA had double switch procedure. Segmental anatomy was {SLL} in all patients, dextrocardia in two patients, mesocardia in two patients. Ventricular septal defect was present in 17 patients, aortic coarctation in 2 patients and interrupted aortic arch (IAoA) in 1 patient. Five patients had tricuspid valve regurgitation. Six patients had AV blocks, 4 patients had pacemaker implantation prior to repair. Pulmonary artery banding was performed in 17 patients, for congestive heart failure (14 patients) or left ventricular retraining (3 patients). Three patients, including one patient with IAoA had primary repair. After LV retraining, repair was performed when indexed LV mass to LV volume ratio was above 1.5. A median follow-up of 60 months was achieved in all. RESULTS There were no deaths. Postoperative pacemaker implantation was required in four patients. Reoperation for Senning obstruction was necessary in one patient, and pacemaker battery replacement in another patient. One patient had mild neoaortic insufficiency, two had mild tricuspid regurgitation and two had mild mitral regurgitation. All were in NYHA I-II. Actuarial survival at 10 years was 100% and freedom from reoperation at 5 and 10 years were 93% and 77.4%, respectively. CONCLUSION Double switch for CC-TGA without RVOTO can be performed with no mortality and low morbidity. Since these results seem to last for several years, it should be considered as the optimal procedure.

A standardized repair-oriented strategy for mitral insufficiency in infants and children: Midterm functional outcomes and predictors of adverse events

OBJECTIVE Surgical management of mitral regurgitation (MR) in children remains a challenge because of the heterogeneity of the anatomy, growth potential, and necessity to avoid valve replacement. Our objective was to assess the functional outcomes and prognostic factors of a standardized strategy of mitral valve (MV) repair for children with MR. METHODS Consecutive patients aged <18 years who had undergone surgery for severe MR from 2001 to 2012 were studied retrospectively. The standardized repair strategy mainly included leaflet debridement, annuloplasty, and leaflet augmentation. Multivariable risk analyses for recurrent MR (grade>II), transmitral mean echocardiographic gradient>5 mm Hg, MV reoperation, replacement, and mortality were performed. RESULTS A total of 106 patients were included (median age, 5.1 years; range, 11 days to 18 years). The mean follow-up period was 3.9±3.2 years (range, 2 months to 11 years). The proportion of congenital and left heart obstruction-related (left ventricular outflow tract obstruction) etiology was 49% (n=52) and 11% (n=12), respectively. MV repair was performed primarily in 97% of the patients. The mortality, reoperation, replacement, and MR rate at the last follow-up visit was 4.5% (n=5), 23% (n=24), 5.5% (n=6), and 17% (n=18), respectively. Actuarial survival was 93%±2% at 10 years. Freedom from MV replacement was 95%±2% and 86%±7% at 5 and 15 years, respectively. Native valve preservation was obtained in 85% of the infants and 94% beyond infancy. Independent predictors of recurrent MR, MV reoperation, and replacement included left ventricular outflow tract obstruction etiology (hazard ratio, 45; P=.004), associated preoperative mitral stenosis (hazard ratio, 21; P=.03), and young age (hazard ratio, 1.2; P=.04). CONCLUSIONS A standardized and reproducible MV repair strategy can achieve satisfactory functional results in infants and children with severe MR, allowing native valve preservation. The left ventricular outflow tract obstruction-related etiology was the main independent predictor of recurrent MR, MV reoperation, and MV replacement.

Reoperations for left atrioventricular valve dysfunction after repair of atrioventricular septal defect

OBJECTIVES Postoperative left atrioventricular valve (LAVV) dysfunction is known to be the principal risk factor influencing outcome after repair of all types of atrioventricular septal defect (AVSD). The purpose of the present study was to identify the risk factors for reoperation and to assess the outcomes after reoperation for LAVV dysfunction. METHODS Records of 412 patients who underwent anatomical repair for different types of AVSD from January 2000 to July 2012 were reviewed. The study group (n = 60) included 13 additional patients for whom repair ± LAVV reoperation was performed in a primary institution. Outcomes, independent risk factors, reoperation and death were analysed. RESULTS There were 7 early, (1.7%) and 1 late death. Forty-seven (11.4%) required 64 reoperations for LAVV dysfunction. The median delay for the first LAVV reoperation was 3.5 months (range: 5 days to 10.0 years). Unbalanced ventricles with small left ventricle [odds ratio (OR) = 4.06, 95% confidence interval (CI): 1.58-10.44, P = 0.004], double-orifice LAVV (OR = 5.04, 95% CI: 1.39-18.27, P = 0.014), prior palliative surgery (OR = 3.5, 95% CI: 1.14-10.8, P = 0.029) and discharge echocardiography documenting LAVV regurgitation grade >2 (OR = 21.96, 95% CI: 8.91-54.09, P < 0.001) were found to be independent risk factors for LAVV reoperation. Twelve-year survival and freedom from LAVV reoperation rates were, respectively, 96.1% (95% CI: 94.1-98.1) and 85.8% (95% CI: 81.3-90.3). Survival was significantly worse in patients who underwent LAVV reoperation (P < 0.001) and in those who underwent valve replacement vs valve repair (P = 0.020). CONCLUSION After AVSD repair, LAVV dysfunction appears to be the principal factor that influences outcome. It can usually be managed by repair. Need for multiple reoperations is not uncommon. Long-term outcome in patients with repaired LAVV is favourable.

Transcatheter Closure of Large Atrial Septal Defects Feasibility and Safety in a Large Adult and Pediatric Population

Background—Data are needed on the safety and efficacy of device closure of large atrial septal defects. Methods and Results—Between 1998 and 2013, 336 patients (161 children <15 years) with large, isolated, secundum atrial septal defects (balloon-stretched diameter ≥34 mm in adults or echocardiographic diameter >15 mm/m2 in children) were managed using the Amplatzer device, at the Marie Lannelongue Hospital. Transthoracic echocardiographic guidance was used starting in 2005 (n=219; 65.2%). Balloon-stretched diameter was >40 mm in 36 adults; mean values were 37.6±3.3 mm in other adults and 26.3±6.3 mm/m2 in children. Amplatzer closure was successful in 311 (92.6%; 95% confidence interval, 89%–95%) patients. Superior and posterior rim deficiencies were more common in failed than in successful procedures (superior, 24.0% versus 4.8%; P=0.002; and posterior, 32.0% versus 4.2%; P<0.001). Device migration occurred in 4 adults (2 cases each of surgical and transcatheter retrieval); in the 21 remaining failures, the device was unreleased and withdrawn. After a median follow-up of 10.0 years (2.5–17 years), all patients were alive with no history of late complications. Conclusions—Closure of large atrial septal defects using the Amplatzer device is safe and effective in both adults and children. Superior and posterior rim deficiencies are associated with procedural failure. Closure can be performed under transthoracic echocardiographic guidance in experienced centers. Early device migration is rare and can be safely managed by device extraction. Long-term follow-up showed no deaths or major late complications in our population of 311 patients.

Arterial switch for transposition with left outflow tract obstruction: outcomes and risk analysis.

BACKGROUND The long-term results and indications of the arterial switch operation (ASO) for transposition of the great arteries (TGA) and anatomic left ventricular outflow tract obstruction (LVOTO) remain undetermined. The aims of this study were to determine long-term outcomes and prognostic factors in this specific population. METHODS Between 1986 and 2011, 55 patients with TGA and anatomic LVOTO underwent ASO. Anatomic LVOTO was defined as an echocardiographic peak LVOT gradient exceeding 20 mm Hg associated with an anatomic narrowing. Forty-three patients had a ventricular septal defect. Median follow-up was 7.9 ± 6.5 years (maximum, 25 years). Univariate and multivariate risk analyses for late LVOTO, aortic regurgitation, LVOT reintervention, and death were performed. RESULTS The early mortality rate was 11% (n = 6); 2 deaths were LVOTO-related. At the latest follow-up, 3 patients (5%) had a LVOTO, 7 (13%) had moderate aortic regurgitation, and 4 (7%) had LVOT reoperation. Actuarial freedom from LVOT reoperation was 90% ± 5% at 10 and 15 years. The mean LVOT peak gradient was 3 ± 9 mm Hg at the latest follow-up. A preoperative pulmonary valve z-score of less than -1.7 (odds ratio, 19; p = 0.02) and an atrioventricular valve-related LVOTO (odds ratio, 15; p = 0.02) are independent predictors of recurrent LVOTO. A preoperative pulmonary valve z-score of less than -1.8 is an independent predictor of LVOT reoperation (odds ratio, 17; p = 0.03). The LVOT gradient per se and the presence of ventricular septal defect or a bicuspid valve do not influence outcomes. CONCLUSIONS Long-term outcomes of ASO for patients with TGA and anatomic LVOTO are satisfactory in selected patients. A lower preoperative pulmonary valve z-score and complex multilevel atrioventricular valve-related LVOTO are independent predictors of recurrent LVOTO and LVOT reoperation. TGA/LVOTO patients with pulmonary valve z-score exceeding -1.8 and resectable valvular or subvalvular LVOTO, or both, should be candidates for ASO, regardless of the severity of the LVOT peak gradient.

Giant Aortic Aneurysm in an Infant With Arterial Tortuosity Syndrome

AA 1-month-old boy presented with malignant arterial hypertension, cardiac failure, and respiratory symptoms. Chest computed tomographic scan revealed diffuse arterial tortuosity, an aneurysm of the descending aorta (30 25 mm) with left airway compression, and a coarctation. Arterial tortuosity syndrome (ATS) was confirmed by two mutations in the SLC2A10 gene (20q13.12). Symptoms and hypertension were improved by an optimal medical treatment, allowing the growth of the infant. At 1 year, chest computed tomographic scan revealed a giant aneurysm of the descending aorta (98 71 mm), a coarctation proximal to the aneurysm (6 mm), and a tortuous abdominal aorta (6 mm; Fig 1). Because of the fast aneurysm expansion, surgery was performed. Through a left posterolateral thoracotomy (fifth space), a moderately hypothermic beating heart cardiopulmonary bypass was established between the right atrial appendage and the ascending aorta. The aneurysm was resected, and an end-to-end aortic anastomosis was performed (clamping time, 32 minutes). No complications occurred. The histologic description was consistent with ATS. At 9 months after surgery, chest computed tomographic scan shows an excellent anatomic result (Fig 2) in a patient free of symptoms.

Unrepaired Incomplete Pentalogy of Cantrell in a 3-Year-Old Girl

A 3-year-old girl was referred for evaluation of an epigastric pulsatile mass covered by a thin layer of skin. A class II pentalogy of Cantrell was diagnosed with the following features: an absent anterior pericardium, a dehiscent anterior diaphragm, a supraumbilical omphalocele (Figure 1A), a restrictive membranous ventricular septal defect with an aneurysm (Figure 1B), and a left ventricle diverticulum (Figure 1C1E). There was no sternal malformation. The central perimembranous ventricular septal defect was restrictive without 1 Pôle de cardiopathies congénitales de l’enfant et de l’adulte, Centre de Référence Malformations Cardiaques Congénitales Complexes M3C, Hôpital Marie Lannelongue, Le Plessis-Robinson, France 2 Faculty of Medicine and Biomedical Sciences, University of Yaoundé, Yaoundé, Cameroon