Antenatal Diagnosis of a Partial Atrioventricular Canal with Ebstein's Anomaly.

The simultaneous occurrence of an atrioventricular canal defect (AVCD) and Ebstein's anomaly is extremely rare, occurring in less than 0.5% of all patients with AVCD. Only 22 cases are described in the literature. This patient's antenatal diagnosis of both Ebstein's anomaly and partial AVCD was made at 25 weeks of gestation. The delivery was organized in a tertiary center. The initial neonatal course was difficult but with adequate treatment, a rapid improvement allowed for a gap of almost 2 years before a complete surgical repair including a cone tricuspid plasty. To our knowledge, this is the first case of antenatal diagnosis, with carefully tailored delivery, neonatal care and subsequent follow-up before indication for successful surgery.

Anomalous left coronary artery from the pulmonary artery: modified extra-anatomic reimplantation.

The anomalous origin of the left coronary artery from the pulmonary artery in the position of a non-facing coronary sinus is extremely rare. The anatomical position of the ectopic ostia will determine which is the appropriate operative approach to create a dual-coronary supply. This report describes a technique of modified extra-anatomical rerouting using autologous pericardium patch and pulmonary artery flap to create a neo-composite coronary trunk passing anterior to the right ventricular outflow tract.

Anatomic Repair of a Left Coronary Artery Main Stem Atresia.

Atresia of the main stem of the left coronary artery is the least observed congenital coronary anomaly; most patients tend to receive a coronary artery bypass graft, although some anatomical corrections have been described. A 17-year-old female patient with left coronary artery main stem atresia underwent a coronary trunk construction with an autologous pericardial patch in our department. At a 3-year follow-up, the patient was asymptomatic, with a normal cardiac stress test. The coronary computed tomography showed no stenosis between the aorta and coronary bifurcation. Long-term patency has yet to be determined.

Should transcatheter closure of atrial septal defects with inferior-posterior deficient rim still be attempted?

BACKGROUND: Transcatheter closure for atrial septal defect (ASD) with inferior-posterior rim deficiency has been scarcely reported with proper identification of the indications and limits. We aimed to assess the safety and feasibility of transcatheter closure of ASDs with deficient rims, paying particular attention to cases with inferior-posterior rim deficiency. METHODS: From January 2008 to January 2013, 241 patients underwent transcatheter ASD closure, including 50 cases (20.7%) with deficient rims, other than the anterior-superior one. Eighteen patients (12 females) presented inferior-posterior rim deficiency. Their median age was 8 (1.4-85) years and their median weight was 24 [9-97] kg. Transcatheter closure was performed in all cases under transesophageal echocardiography (TEE) guidance in children and intracardiac echocardiography (ICE) guidance in adults. RESULTS: Out of 18 patients with inferior-posterior rim deficiency, only 8 underwent successful immediate transcatheter closure. Four cases failed to be closed. Major complications occurred in 6 patients, including 4 device embolizations, 1 pericardial effusion and 1 complete atrioventricular block that resolved after surgical removal of the device. During a median follow up of 54±13 months, a residual right-to-left shunt was documented in 2 more cases, requiring surgery in one case because of cyanosis. Transcatheter closure was successfully performed in the rest of the 223 patients, including in the 32 cases with deficient rims other than inferior-posterior. CONCLUSIONS: Transcatheter closure of ASDs with inferior-posterior rim deficiency cannot be recommended.

Mobile extracorporeal membrane oxygenation for children: single-center 10 years' experience.

OBJECTIVES: Extracorporeal membrane oxygenation has become a gold standard in treatment of severe refractory circulatory and/or pulmonary failure. Those procedures require gathering of competences and material. Therefore, they are conducted in a limited number of reference centers. Emergent need for such treatments induces either hazardous transfers or a mobile pediatric extracorporeal membrane oxygenation team able to remote implantation and transportation. The aim of this work is not to focus on pediatric extracorporeal membrane oxygenation outcomes or indications, which have been extensively discussed in the literature. This study would like to detail the implementation, safety, and feasibility, even in a middle-size pediatric cardiac surgery reference center. PATIENTS: This is a retrospective analysis of a series of patients initiated on extracorporeal membrane oxygenation in a peripheral center and transferred to a reference center. The data were collected from 10 consecutive years: from 2006 to 2016. RESULTS: A total of 57 pediatric patients with a median weight of 6.00 (3.2-14.5) kg and median age of 2.89 (0.11-37.63) months were cannulated in peripheral center and transported on extracorporeal membrane oxygenation. We did not experience any adverse event during transport. The outcomes were comparable to our literature-reported on-site extracorporeal membrane oxygenation series with 42 patients (74%) weaned from extracorporeal membrane oxygenation and a 30-day survival of 60%. Neither patient's age nor weight, indication for extracorporeal membrane oxygenation or length of transport, was statistically significant in terms of outcomes. CONCLUSION: Offsite extracorporeal membrane oxygenation implantation and ground or air transport for pediatric patients on extracorporeal membrane oxygenation appeared to be safe when performed by a dedicated and experienced team, even within a mid-size center.

Hybrid Melody Valve Implantation in Mitral Position in a Child: Usefulness of a 3-Dimensional Printed Model for Preprocedural Planning.

We present the case of a 4-month-old child with atrioventricular canal associated with severe left atrioventricular valve dysfunction who previously underwent 3 surgical valve reconstructions without significant improvement. A Hybrid Melody valve (Medtronic, Minneapolis, MN) insertion was planned. Because of the low weight, the risk of left ventricular outflow tract obstruction was significant and therefore evaluated preprocedurally using a cardiac computed tomography-derived 3-dimensional printed model. In vitro tests showed good anchorage of the valve without subaortic obstruction and the procedure was then achieved with an excellent clinical result.

Early to mid-term results after total cavopulmonary connection performed in the second decade of life.

OBJECTIVES: Total cavopulmonary connection (TCPC) performed in the second decade of life has rarely been studied. Thus, we investigated (bicentric study) early and late morbidity and mortality following completion of TCPC in these patients. METHODS: From January 1999 to June 2014, 63 patients (14.5 ± 2.9 years) underwent TCPC (extracardiac conduit). Palliation before completion was an isolated bidirectional cavopulmonary shunt (BCPS) in 3 patients or BCPS associated with additional pulmonary blood flow (APBF) that was either antegrade (Group 1) in 38 (63%) or retrograde (Group 2) in 22 (37%). Preoperative and perioperative data were reviewed retrospectively. RESULTS: Mean pulmonary arterial and ventricular end-diastolic pressures were 12.2 and 9.2 mmHg, respectively. Mean Nakata index was 279 ± 123 and 228 ± 87 mm 2 /m 2 in Groups 1 and 2, respectively ( P = 0.01). Aortic cross-clamping was performed in 22 from Group 1 and 8 from Group 2 ( P = 0.04). Mean follow-up was 4.57 years [0.8-15]. Nine patients had prolonged stays in the intensive care unit (>6 days). There were 1 early and 2 late deaths (non-cardiac related). Actuarial survival was 96% at 4 years. At last follow-up, single-ventricle function remained normal or improved in all patients (Group 1) compared to 82% in Group 2 ( P = 0.02). New York Heart Association (NYHA) class had improved in both groups: 47 patients were NYHA class II and 16 class III preoperatively vs 50 class I and 10 class II postoperatively ( P < 0.001). CONCLUSIONS: Single-ventricle palliation with BCPS and APBF allowed completion of TCPC in the second decade of life, with encouraging mid-term results. However, BCPS with retrograde APBF was associated with single-ventricle dysfunction: thus, this technique needs to be used cautiously as long-lasting palliation.

Tricuspid valve repair and pulmonary valve replacement in adults with repaired tetralogy of Fallot.

BACKGROUND: Pulmonary valve replacement (PVR) often is performed in adults with repaired tetralogy of Fallot (TOF). For patients who have tricuspid regurgitation (TR), tricuspid valve (TV) repair associated to PVR is still debated. OBJECTIVE: We sought to evaluate perioperative factors related to TV repair when performed at the time of PVR in patients with repaired TOF. METHODS: We retrospectively reviewed 104 patients with repaired TOF (or its equivalent) who underwent PVR (2002-2014). RESULTS: Mean age at initial complete correction and at PVR was 20.1 ± 17.2 months and 26.3 ± 9.5 years, respectively. Forty-one patients had significant preoperative TR: 24 were moderate (group M) and 17 were severe (group S). A total of 16 TV repair were performed (8 for each group). Moderate and severe tricuspid regurgitation observed in the first year following the initial complete repair were significantly associated with severe TR at PVR (P < .001). In group M patients, TR was improved regardless of TV repair, whereas, in group S, residual significant TR was reported in 7 patients who did not have TV repair. No cases were observed for patients who underwent concomitant TV repair (P = .002). Among these patients with residual significant TR, 2 needed a tricuspid valve replacement. The functional status (New York Heart Association classification) of group S patients was significantly improved by concomitant TV repair. CONCLUSIONS: In adults with repaired TOF, TV repair is a safe procedure when performed at the time of PVR. If, at mid-term follow-up, there is probably no benefit of TV repair when preoperative TR is moderate, TV repair may improve both tricuspid valve function and functional status of the patients in case of severe preoperative TR.

Transposition of the great arteries: Rationale for tailored preoperative management.

As preoperative morbi-mortality remains significant, care of newborns with transposition of the great arteries is still challenging. In this review of the literature, we discuss the different treatments that could improve the patient's condition into the preoperative period. Instead of a standardized management, we advocate personalized care of these neonates. Considering the deleterious effects of hypoxia, special attention is given to the use of non-invasive technologies to assess oxygenation of the tissues. As a prolonged preoperative time with low cerebral oxygenation is associated with cerebral injuries, distinguishing neonates who should undergo early surgery from those who could wait longer is crucial and requires full expertise in the management of neonatal congenital heart disease. Finally, to treat these newborns as soon as possible, we support a planned delivery policy for foetuses with transposition of the great arteries.

Right Thoracotomy to Repair Scimitar Syndrome and an Accessory Hemidiaphragm.

The association of an accessory hemidiaphragm and scimitar syndrome is extremely rare and occurs exclusively in the right hemithorax. We present the case of a 27-year-old female, who underwent complete resection of the accessory hemidiaphragm and direct anastomosis of the scimitar vein to the left atrium through a lateral right thoracotomy. This surgical approach offered easy access to both anomalies and allowed single-stage concomitant repair; this approach avoided postoperative respiratory complications and possible torsion or stenosis of the scimitar vein.

Scimitar Syndrome Repair in Adults: Intermediate-Term Results Using an Extracardiac Conduit.

BACKGROUND: Scimitar syndrome may be corrected using different techniques. Repair using an extracardiac conduit has rarely been performed. This study assessed the intermediate-term outcomes of this technique in adults. METHODS: From January 2000 to June 2011, 7 adult patients underwent correction with a ringed polytetrafluoroethylene conduit used to connect the scimitar vein (SV) to the left atrium, posterior to the inferior vena cava (IVC). Preoperative and perioperative data were reviewed retrospectively. RESULTS: All patients (32 ± 10.6 years old) were symptomatic (3 patients were New York Heart Association [NYHA] functional class II, 4 had recurrent pneumonia), with a ratio of pulmonary to systemic blood flow greater than 2, without significant pulmonary hypertension. In all patients, the SV drained the entire right lung venous return to the IVC below the diaphragm. Surgical repair was performed by sternotomy, normothermic cardiopulmonary bypass, and aortic cross-clamping. Four patients required additional closure of an atrial septal defect. Mean conduit diameter was 14 mm (range, 12 to 16 mm). Patients received long-term platelet suppression therapy with aspirin. There were no deaths and no reoperations. Mean follow-up time was 9.1 ± 3.6 years. Postoperative morbidity was nil. No evidence of subclinical stroke or embolization was found in postoperative magnetic resonance imaging of the brain. No thrombi on the prosthesis or in the left atria were detected at the latest echocardiogram, with laminar flow from the SV to the left atrium. At last follow-up, all patients were in NYHA class I. CONCLUSIONS: Correction of scimitar syndrome with an extracardiac conduit can be easily and safely performed in adults, with excellent intermediate-term durability, without graft thrombi or stenosis. This technique avoids deep hypothermic circulatory arrest when the SV is short or enters the IVC in an unusually caudad location.

Kommerell Diverticulum Should Be Removed in Children With Vascular Ring and Aberrant Left Subclavian Artery.

BACKGROUND: Right aortic arch with aberrant left subclavian artery is the most frequent cause of vascular ring. Ligamentum arteriosus division opens the ring but leaves the Kommerell diverticulum in place, with a risk of residual compression, aneurysmal dilation, dissection, or even rupture. A procedure consisting of translocation of the aberrant left subclavian artery to the left carotid artery with removal of the Kommerell diverticulum and division of the ligamentum is currently advocated. METHODS: Between September /2009 and August 2011, 12 consecutive patients underwent the foregoing procedure. Clinical findings, surgical procedure, complications, histopathologic findings, and follow-up data were retrospectively analyzed. RESULTS: Mean age at the time of operation was 7.7 years (median, 4.3 years, range, 0.9 to 18.9 years), and mean weight was 26 kg (median, 18 kg; range, 8.4 to 59 kg). All patients had symptoms. Computed tomography scan or magnetic resonance imaging, or both, had confirmed the diagnosis in all patients. Postoperative events, all transient, included chylothorax (1 patient), phrenic palsy (1 patient), pneumonia (1 patient), and the need for prolonged chest drainage (3 patients). Mean follow-up reached 19 months (median, 13 months; range, 12 to 41 months). Mild residual respiratory symptoms were noted in 3 patients. Echo-Doppler analysis showed a patent left subclavian-to-carotid artery anastomosis. Histopathologic analysis of the resected diverticulum (n = 6) showed cystic medial necrosis in four diverticula (focal in one). CONCLUSIONS: Translocation of the aberrant left subclavian artery with diverticulum resection and ligamentum division is an efficient procedure for symptom relief. Profound wall abnormalities such as medial necrosis in at least 50% of the analyzed diverticula encourage us to maintain this strategy, to reduce the risk of aneurysm formation and dissection.

Surgical closure of persistent arterial duct with minimal invasive anterior thoracotomy: an alternative technique.

Surgical approach for persistent ductus arteriosus ligation is typically a left lateral thoracotomy opening the pleural-space with left lung retraction. We describe an alternative approach, with a minimally invasive anterior parasternal incision. This is particularly adapted to preterm infants weighing less than 1.5 kg. This approach ensures a good exposure of vessels. We believe that it is safe, reliable and reproducible. The learning curve should not be an issue for surgeons used to manage low weight patients.

Surgical repair of pulmonary artery branches.

Surgical repair of pulmonary artery (PA) branches encompasses many different clinical scenarios and technical challenges. The most common, such as bifurcation and central PA reconstruction, are described, as well as the challenges of complex and peripheral reconstruction.

Isolated left common carotid artery connected to the pulmonary artery: where was the arterial duct?

A three-year-old boy was referred for persistent arterial duct. Transthoracic echocardiography showed a right aortic arch and an unusual Doppler flow in the arch vessels and the pulmonary artery. The tomodensitometry showed a right-sided aortic arch, with successive origin of the right common carotid, the right subclavian artery, and an aberrant (lusoria) left subclavian artery. The left common carotid took origin from the pulmonary trunk. During surgery, a fibrous cord independent from the anomaly was identified. An end-to-side anastomosis between the left carotid and the ascending aorta was done and the fibrous cord was divided. Was this fibrous cord a ductal ligament?

Management of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals: Focus on the strategy of rehabilitation of the native pulmonary arteries.

Pulmonary atresia with ventricular septal defect (VSD), hypoplastic native pulmonary arteries (PAs) and major aorto-pulmonary collateral arteries (MAPCAs) is a rare and complex congenital cardiac disease. In broad outline, two surgical approaches are available for patients with this condition. The first is characterized by one or several stages of complete unifocalization of the supplying MAPCAs, with or without incorporation of the native pulmonary arteries (PAs), connection of the right ventricle to the 'neo-Pas' and, if possible, concomitant or delayed closure of the VSD. The second strategy is based on rehabilitation of the native pulmonary arteries. The first step is a direct right ventricle to native PA connection, to promote the growth of native PAs. The establishment of antegrade flow also allows an easier approach for interventional catheterization, enabling dilatation or stenting of the stenosis and then closure of the communicant collaterals. When the development of the native PAs is satisfactory, the complete repair is performed. If it is necessary to suture a MAPCA to the PA ('unifocalization'), this is accomplished by connecting the collateral artery to an already developed native branch. Our team developed this multidisciplinary strategy with good results. Based on this experience as well as on the published literature, we describe this strategy of management of patients with pulmonary atresia, VSD, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals (MAPCAs).

Pulmonary position cryopreserved homograft in non-Ross patients: how to improve the results?

OBJECTIVES: The outcomes of homografts (HGs) in the reconstruction of the right ventricular outflow tract (RVOT) in non-Ross patients are often considered disappointing, compared with Ross patients; and the risk factors for HG degeneration are still controversial. The objective of this study was to determine the durability and prognostic factors related to the HGs implanted in non-Ross patients and to propose potential ways to improve the results. METHODS: A retrospective study (1993-2010) included 115 consecutive non-Ross patients who received a HG for RVOT reconstruction. The median age at implantation was 2.8 years (4 days-58 years). The main heart defects were pulmonary atresia with ventricular septal defect (n = 40; 34%), truncus arteriosus (n = 28; 24%) and tetralogy of Fallot (n = 23; 20%). Thirty-eight percent had preoperative pulmonary hypertension. A low-dose corticosteroid therapy was used during the postoperative period in patients displaying a HG-related inflammatory response (no septic context) (n = 11). The median diameter of the implanted HG was 22 mm (range 9-30 mm). The median age of the HG donor was 14 years (range 0.5-65 years). ABO compatibility rules were not systematically respected for the HG implantation: 43% of the implanted HGs were ABO compatible with the recipient. The endpoints were HG stenosis (peak gradient ≥20 mmHg), regurgitation (moderate or severe), dysfunction (peak gradient ≥ 50 mmHg or regurgitation moderate or severe) and failure (explantation or balloon dilation). RESULTS: Freedom from HG explantation and failure were 89 and 80% at 5 years, and 76 and 69% at 10 years, respectively. HG donor age <30 years [hazard ratio (HR): 2; P = 0.012], preoperative pulmonary hypertension (HR: 3; P = 0.02) and HG mismatch (HR: 5; P = 0.04) were multivariate risk factors for HG stenosis, regurgitation and failure, respectively. HG diameter <22 mm was a multivariate risk factor for HG regurgitation (HR: 8; P < 0.001), dysfunction (HR: 9; P = 0.02) and failure (HR: 5; P = 0.03). ABO incompatibility increased the risk of HG stenosis (HR: 4; P = 0.009) and dysfunction (HR: 2; P = 0.04). The use of corticosteroids significantly protected against the risk of HG regurgitation (HR: 0.08; P = 0.04) in the multivariate analysis. CONCLUSIONS: The cryopreserved HG implanted to reconstruct the RVOT in non-Ross patients remains one of the most acceptable options in this specific non-Ross population. The outcomes of HGs in non-Ross patients might be improved by implanting an ABO-compatible HG with an adapted diameter, coming from a donor >30 years and by optimizing the perioperative afterload of the HG.

Associated double-orifice mitral and tricuspid valves without ostium primum defect.

The simultaneous existence of double orifice right and left atrioventricular valves in the absence of ostium primum defects is rare and scarcely reported. We present the case of a 20-month old boy diagnosed with tetralogy of Fallot with pulmonary atresia who was found to have associated double-orifice mitral and tricuspid valves.

An uncommon course of the right superior vena cava in a patient with heterotaxy syndrome.

We present the case of an infant with congenital heart disease which includes a partial atrioventricular canal defect with the absence of the atrial septum (common atrium) and an extremely uncommon course of the right-sided superior vena cava (SVC) including an intra-atrial segment coursing intramurally along the right posterolateral atrial wall, with an intracardiac orifice situated low within the right side of the atrium, close to the atrial orifice of the right hepatic veins. This feature of the anatomy was discovered intraoperatively at the time of surgical repair. Systemic venous anatomy also included interrupted inferior vena cava (IVC) with azygos continuation to a left-sided SVC draining directly into the left side of the atrium. The successful surgical procedure included tunneling of the left-sided SVC to the right side of the common atrium and atrial septation with a patch.