Tetralogy of Fallot: T-shaped infundibulotomy for pulmonary valve-sparing procedure.

This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment. After myocardial resection and ventricular septal defect closure, an extensive commissurotomy is achieved. Finally, the right ventricular outflow tract remodelling is completed by a shield-shaped bovine patch with an oversized square superior edge, attached directly on the pulmonary valve annulus, with an effect of systolic traction. Sixty patients (89.5%) had a Tetralogy of Fallot repair with preservation of the pulmonary valve. To date, with a median follow-up of 38.2 [14-64] months, no patient has needed a surgical or interventional procedure for pulmonary valve stenosis or regurgitation, with low residual gradients. This procedure could provide a significant increase in native pulmonary valve preservation. Long-term studies are needed to assess pulmonary valve growth and the consequent reduction in surgical or interventional reoperations.

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. BACKGROUND: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. METHODS: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018­2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. CONCLUSIONS: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.

Wilms tumor, pleuropulmonary blastoma, and DICER1: case report and literature review.

BACKGROUND: Pleuroblastoma (PPB) is a rare pediatric tumor which, in 30% of cases, is associated with cystic nephroma. It has been recently linked to the DICER1 mutation as part of a predisposition syndrome for various tumors. However, if DICER 1 anomalies have been reported in patients with Wilms tumor (WT), to date, no cases of PPB, WT, and DICER1 mutations have been reported in the same patient. CASE PRESENTATION: We report the case of a 3-year-old patient, initially managed for metastatic WT. During his clinical course, the diagnosis of a PPB was made after detecting the DICER1 mutation and subsequent management was therefore modified. CONCLUSION: This case highlights that in case of simultaneous discovery of a renal tumor and a pulmonary lesion in a child, the DICER 1 mutations should be looked for as these could help adapt management and schedule the surgical procedures.

Tetralogy of Fallot with critical biventricular dysfunction: is surgical correction achievable?

We describe the case of a 32-month-old patient from a developing country with tetralogy of Fallot associated with a severe biventricular dysfunction. This association is rare but makes the surgical strategy complex and potentially contraindicated. An acute severe hypoxic episode led us to perform palliative rescue intervention involving the placement of an undersized systemic-to-pulmonary shunt. This surgery was well tolerated and allowed a fast and impressive recovery of the ventricular function, making complete repair possible.

Thymic-Specific Serine Protease Limits Central Tolerance and Exacerbates Experimental Autoimmune Encephalomyelitis.

The genetic predisposition to multiple sclerosis (MS) is most strongly conveyed by MHC class II haplotypes, possibly by shaping the autoimmune CD4 T cell repertoire. Whether Ag-processing enzymes contribute to MS susceptibility by editing the peptide repertoire presented by these MHC haplotypes is unclear. Thymus-specific serine protease (TSSP) is expressed by thymic epithelial cells and thymic dendritic cells (DCs) and, in these two stromal compartments, TSSP edits the peptide repertoire presented by class II molecules. We show in this article that TSSP increases experimental autoimmune encephalomyelitis severity by limiting central tolerance to myelin oligodendrocyte glycoprotein. The effect on experimental autoimmune encephalomyelitis severity was MHC class II allele dependent, because the lack of TSSP expression conferred protection in NOD mice but not in C57BL/6 mice. Importantly, although human thymic DCs express TSSP, individuals segregate into two groups having a high or 10-fold lower level of expression. Therefore, the level of TSSP expression by thymic DCs may modify the risk factors for MS conferred by some MHC class II haplotypes.

Image of a left atrial mass after a cardiopulmonary bypass in a child.

Invagination of an appendage into the left atrium is a rare complication. It occurs spontaneously or after open-heart surgery. In our case, a postoperative transesophageal echocardiogram, after closure of a ventricular septal defect in a 5-month-old infant, revealed a large mass in the left atrium. A diagnosis of a left appendage inversion was confirmed after external examination of the heart. Herein, we provide echocardiographic images before, during, and after manual reversion of the left appendage. Misdiagnosis of this complication could have led to an additional unnecessary surgical procedure that could have impacted on the patient's morbidity.

Isolated severe leftward displacement of the septum primum: anatomic and 3D echocardiographic findings and surgical repair.

OBJECTIVES: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage. METHODS: Three patients presenting with situs solitus and extreme leftward displacement of the septum primum were included. All of the pulmonary veins drained anomalously into the anatomical right atrium, and the distance between the mitral valve and the abnormal septum primum was greatly reduced, compromising the size of the left atrial chamber, but with normal left ventricle diameters. Preoperative 3D echocardiographic findings are reported. We achieved a biventricular surgical repair in all cases. The atrial septation was accomplished using an autologous pericardial patch after removing the abnormal septal membrane. RESULTS: The postoperative course was free from any cardiovascular complications. Echocardiographic scans showed a harmonious reconstruction without pulmonary venous obstructions or stenosis. CONCLUSIONS: This article reports the severe leftward displacement of the septum primum presented as an isolated cardiac malformation; 3D transthoracic echocardiography allowed an accurate diagnosis of this malformation and helped in choosing the best surgical strategy.

Two-dimensional right ventricular strain by speckle tracking for assessment of longitudinal right ventricular function after paediatric congenital heart disease surgery.

BACKGROUND: Right ventricular (RV) function is a prognostic marker of cardiac disease in children. Speckle tracking has been developed to assess RV longitudinal shortening, the dominant deformation during systole; little is known about its feasibility in children with congenital heart disease (CHD). AIMS: To evaluate the feasibility and reproducibility of RV two-dimensional (2D) strain assessed by speckle tracking in infants undergoing CHD surgery compared with conventional markers. METHODS: In this prospective single-centre study, RV peak systolic strain (RV-PSS) was measured using 2D speckle tracking in 37 consecutive children undergoing CHD surgery. Examinations were performed the day before surgery, a few hours after surgery and before discharge. Relationships with the z score of tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular systolic velocity (TA Sa) were assessed. RESULTS: Median (interquartile range) age was 19 months (5-63); median weight was 9.2 kg (5.3-18.0). RV-PSS analysis was feasible in 92.9% (95% confidence interval [CI]: 86.0-97.1) of examinations. The coefficient of variation was 9.7% (95% CI: 7.4-11.9) for intraobserver variability and 15.1% (95% CI: 12.7-17.6) for interobserver variability. Correlations between RV-PSS and z score of TAPSE and TA Sa were strong (r=0.71, P<0.0001 and r=0.70, P<0.0001, respectively). RV-PSS was significantly reduced after surgery compared with baseline (-10.5±2.9% vs. -19.5±4.8%; P<0.0001) and at discharge (-13.5±4.0% vs. -19.5±4.8%; P<0.0001). Similar evolutions were observed with TAPSE and TA Sa (both P<0.0001). CONCLUSION: RV longitudinal strain by speckle tracking is a feasible and reproducible method of assessing perioperative evolution of RV function in children with CHD.

Assessment of Ventricular Septal Defect Size and Morphology by Three-Dimensional Transthoracic Echocardiography.

BACKGROUND: Morphologic description of ventricular septal defect (VSD) is mandatory before performing the newly developed transcatheter closure procedure. Inaccurate estimation of defect size has been reported using conventional two-dimensional (2D) transthoracic echocardiography (TTE). The aim of this study was to assess VSD morphology and size using three-dimensional (3D) TTE compared with 2D TTE and surgery. METHODS: Forty-eight children aged 21.4 ± 29.3 months with isolated muscular (n = 11 [22.9%]) and membranous (n = 37 [77.1%]) VSDs were prospectively included. Three-dimensional images were acquired using full-volume single-beat mode. Minimal diameter, maximal diameter, and systolic and diastolic VSD areas were measured from 3D data sets using multiplanar reconstruction mode (QLAB 9). Maximal-to-minimal VSD diameter ratio was used to assess VSD geometry. Linear regression analysis and the Bland-Altman method were used to compare 3D measurements with 2D and surgical measurements in a subgroup of 15 patients who underwent surgical VSD closure. RESULTS: VSD 3D diameters and areas were measured in all patients (100%; 95% CI, 92.6%-100%). Maximal diameter was lower on 2D TTE compared with 3D TTE (7.3 vs 11.3 mm, P < .0001). Mean bias was 4 mm, with 95% of values ranging from -1.76 to 9.75 mm. Correlation between 3D maximal diameter and surgical diameter was strong (r(2) = 0.97, P < .0001), while correlation between maximal 2D diameter and surgical diameter was moderate (r(2) = 0.63, P < .0001). VSDs had an oval shape when assessed by 3D TTE. Maximal-to-minimal diameter ratio assessed by 3D TTE was significantly higher in muscular VSDs compared with membranous VSDs (3.20 ± 1.51 vs 2.13 ± 1.28, respectively, P = .01). VSD area variation throughout the cardiac cycle was 32% and was higher in muscular compared with membranous VSDs (49% vs 26%, P = .0001). CONCLUSIONS: Three-dimensional TTE allows better VSD morphologic and maximal diameter assessment compared with 2D TTE. VSD shape and its changes during the cardiac cycle can be visually and quantitatively displayed. Three-dimensional echocardiography may thus be particularly useful before and during percutaneous VSD closure.

Aortic arch aneurysm: short- and mid-term results comparing open arch surgery and the hybrid procedure†.

OBJECTIVES: Open arch surgery for aortic arch aneurysm was historically associated with a high risk of postoperative morbi-mortality. Improved operative techniques have now lowered the incidence of these complications but in parallel, hybrid arch procedures have emerged. Nowadays, very little data are available about their mid-term results compared with open surgery. METHODS: From January 2002 to January 2014, 46 patients had treatment for an exclusive aortic arch aneurysm including 25 open arch surgeries and 21 type I hybrid arch procedures in our institution. All cases involved arch aneurysms involving at least one carotid artery (Zone 0 and Zone 1). Aneurysms of the distal arch and descending aorta were excluded (Zone 2 and beyond). Results from a retrospective database are reported. There were no patients lost to the follow-up. RESULTS: There was no significant difference in preoperative comorbidities between the two groups. The incidence of in-hospital mortality was similar at 20% (5/25) for open surgery and 19% (4/21) for hybrid procedure (P = 0.830). The incidence of permanent cerebral neurological deficit was comparable at 17.4% (4/23) for open surgery and 21.1% (4/19) for hybrid procedure (P = 1). Median survival was 109.5 months for open surgery and 56.3 months for hybrid procedure. Freedom from all-cause mortality was 78, 63, 63 and 57% at 1, 3, 5 and 7 years, respectively in the open surgical group. Freedom from all-cause mortality was 74, 55, 46 and 28% at 1, 3, 5 and 7 years, respectively in the hybrid group. Survival rates and incidence of major adverse cardiac and cerebro-vascular event between open surgery and hybrid procedure were not statistically different (P = 0.530 and P = 0.325, respectively). However, incidence of reintervention was in favour of open surgery [14.5 vs 44.8% at 7 years, P = 0.045; 95% confidence interval: (0.06-0.97)]. CONCLUSIONS: The type I hybrid arch procedure fails to demonstrate better results compared with open surgery, regarding morbi-mortality at the short- and mid-term follow-up. Moreover, it increases the risk of reintervention. Patients treated by this technique must undergo a closer follow-up because of this risk. Larger randomized studies are needed to better define the exact indications of this therapy.

The medical history of adults with complex congenital heart disease affects their social development and professional activity.

BACKGROUND: In recent decades, advances in surgery and therapeutic catheterization have steadily increased the life expectancy and prevalence of adults with congenital heart disease (CHD). AIMS: We assessed medical and psychosocial variables of adults with CHD, according to the disease complexity. METHODS: We included, from a single-centre observational cohort study, 135 consecutive adults with CHD (median age of 40 years, interquartile range: 28.0-51.0) followed in our cardiology unit, who answered a questionnaire assessing daily activity and psychosocial functioning. Disease complexity was classified according to the Bethesda conference. RESULTS: Cardiac malformation complexity was simple in 61 (45.2%), moderate in 50 (37.0%) and complex in 24 (17.8%) patients. Cardiac surgery had been performed in 86.5% of moderate and complex patients. Complications (such as heart failure, arrhythmia and pulmonary hypertension) were mainly observed in the complex group (P=0.003). Physical activity was lower in the complex group (no activity in 58.8%, but sport previously contraindicated in 50% of these; P=0.03). Education level tended to be lower in the complex and moderate groups than in the simple group (respectively, 31.2% and 33.3% vs. 45.7% had passed the Baccalaureate; P=0.47). The pass rate was lower in patients with complications (P=0.037) or more than one cardiac surgery (P=0.03). In the complex group, 56.3% of patients were unemployed (P=0.048). CONCLUSIONS: Complexity of heart disease and medical history affect education level and employment of adults with CHD. Academic education of children with a complex defect and career counselling are important to prevent unemployment among adults with CHD.

Safety and efficiency of the new micro-multiplane transoesophageal probe in paediatric cardiology.

BACKGROUND: Transoesophageal echocardiography (TOE) is feasible in neonates using a miniaturized probe, but is not widely used because of low imaging quality. AIMS: To assess handling and imaging quality of a new release of a micro-TOE probe in children. METHODS: Thirty-eight consecutive children, enrolled during February and May 2013, underwent TOE with the Philips S8-3t probe. Insertion, handling and image quality were assessed. RESULTS: The 38 children (aged 7days to 12years; weight 3.1-27kg) underwent 75 TOE (30 [40.0%] before cardiac surgery, 31 [41.3%] after cardiac surgery, 4 [5.3%] during a percutaneous procedure, 10 [13.3%] in the intensive care unit). Insertion of the micro-TOE probe was 'very easy' in 37/38 patients (97.4%). Handling was better in the lightest children (P=0.001). Image quality was mainly 'good' or 'very good', with no significant changes between preoperative and postoperative examinations or over time. Total scores (insertion, handling, image quality) were significantly better in the lightest children (P=0.02). Preoperative TOE did not provide additional information over transthoracic echocardiography. Postoperative TOE was useful to assess surgical results, but no residual lesions required extracorporeal circulation return. Micro-TOE was useful during the postoperative care of neonatal surgery with open breastbone to assess the surgical result and ventricular function. It was also useful to guide extracorporeal membrane oxygenation (ECMO) indication and withdrawal; and was a useful guide for percutaneous procedures. CONCLUSION: Micro-multiplane TOE is safe and efficient for use in neonates and children. This minimally invasive tool increases the impact of TOE in paediatric cardiology.

Phase I trial: the use of autologous cultured adipose-derived stroma/stem cells to treat patients with non-revascularizable critical limb ischemia.

BACKGROUND AIMS: Non-revascularizable critical limb ischemia (CLI) is the most severe stage of peripheral arterial disease, with no therapeutic option. Extensive preclinical studies have demonstrated that adipose-derived stroma cell (ASC) transplantation strongly improves revascularization and tissue perfusion in ischemic limbs. This study, named ACellDREAM, is the first phase I trial to evaluate the feasibility and safety of intramuscular injections of autologous ASC in non-revascularizable CLI patients. METHODS: Seven patients were consecutively enrolled, on the basis of the following criteria: (i) lower-limb rest pain or ulcer; (ii) ankle systolic oxygen pressure <50 or 70 mm Hg for non-diabetic and diabetic patients, respectively, or first-toe systolic oxygen pressure <30 mm Hg or 50 mm Hg for non-diabetic and diabetic patients, respectively; (iii) not suitable for revascularization. ASCs from abdominal fat were grown for 2 weeks and were then characterized. RESULTS: More than 200 million cells were obtained, with almost total homogeneity and no karyotype abnormality. The expressions of stemness markers Oct4 and Nanog were very low, whereas expression of telomerase was undetectable in human ASCs compared with human embryonic stem cells. ASCs (10(8)) were then intramuscularly injected into the ischemic leg of patients, with no complication, as judged by an independent committee. Trans-cutaneous oxygen pressure tended to increase in most patients. Ulcer evolution and wound healing showed improvement. CONCLUSIONS: These data demonstrate the feasibility and safety of autologous ASC transplantation in patients with objectively proven CLI not suitable for revascularization. The improved wound healing also supports a putative functional efficiency.

Benefits of extracorporeal membrane oxygenation for major blunt tracheobronchial trauma in the paediatric age group.

Tracheobronchial rupture due to blunt chest trauma is a rare but life-threatening injury among children. The severity of this condition ranges from death before hospital admission to clinical stability resulting in delayed management. Diagnosis is difficult because there is sometimes no evidence of external trauma, in spite of severe chest crush injury and consecutive rupture of airways. Here, we report the case of a 32-month-old girl whose torso was crushed by a van, resulting in bilateral bronchi disconnection. She was admitted to our hospital with cardiac and respiratory arrest. After prompt resuscitation, flexible bronchoscopy permitted the accurate visualization of the rupture and its extent. The life-saving procedure consisted of surgical repair using extracorporeal membrane oxygenation (ECMO) as ventilatory support. This provided rapid relief from the injury, which was previously expected to result in death. These data suggest that ECMO could be beneficial as supportive therapy for selected paediatric patients with major tracheobronchial traumas.

Three-dimensional transthoracic echocardiographic assessment of supramitral ring in a young child.

Three-dimensional echocardiography is an increasingly used method to accurately depict congenital heart disease in children. We report the case of a 2-month-old girl with supramitral ring. Three-dimensional echocardiography allowed perfect visualisation of the fibrous shelf before surgery.

Mechanical occlusion of the inferior vena cava: an early complication after repair of pectus excavatum using the Nuss procedure.

The Nuss procedure is the most widely used surgical procedure to correct pectus excavatum. Although it is a minimally invasive approach, a number of major early complications, such as heart perforation, have been reported. We describe a 15-year-old boy in whom acute occlusion of the inferior vena cava developed after a Nuss repair. The diagnosis was confirmed by emergency postoperative CT examination, and treatment consisted of immediate removal of the Nuss bar.

Wrapping procedure for stanford type a acute aortic dissection: is there an indication for surgery without a cardiopulmonary bypass?

A wrapping procedure for acute type A aortic dissection was performed on six elderly patients at high risk for conventional surgery. Aortic valve insufficiency was mild, with no malperfusion syndrome. A Teflon plaque or Dacron vascular prosthesis was passed around the aorta and tightened from the coronary ostia to the innominate artery. No severe neurologic complications or deaths occurred in the postoperative period. Computed tomography and magnetic resonance imaging imaging during follow-up showed aortic diameters had stabilized in all patients.

The surgical management and outcome of congenital mediastinal malformations.

We reviewed our institutional experience with congenital mediastinal masses and compared the postnatal management and outcome of patients with or without prenatal diagnosis. Between January 1997 and August 2011, 24 patients underwent surgical procedures for congenital mediastinal mass. For eight patients, the mass was detected by prenatal ultrasonography at 27 weeks of gestation (range 22-35). Postnatal management consisted in open surgery for seven patients at a mean age of 9 months (range 1 day-20 months) and sclerotherapy for one lymphangioma at 5 months of life. Sixteen patients had postnatal diagnosis at 137 months (±194) of median age. Eight bronchogenic cysts, seven bronchopulmonary foregut malformations, five teratomas, three lymphangiomas and one haemangioma were operated on. The median age at resection was 28 months (1 day-15 years). There were four emergency procedures and no surgical mortality. The median follow-up was 45 months (3-144). The duration of mechanical ventilation and hospital stay was, respectively, 4.6 h and 7.5 days for antenatal patients and 24.3 h and 14.3 days for postnatal diagnosed patients. Prenatal diagnosis allows early management of congenital mediastinal malformations. Early resection can be performed prior to the occurrence of symptoms ∼1 year of life and is associated with an excellent outcome and less morbidity.