Transcatheter Fontan completion: Creation of an extracardiac Fontan.

Patients with functionally univentricular hearts are usually palliated surgically. There have been several reports of successful attempts to complete the Fontan procedure without surgery. The pathways created at the time of the preconditioning were largely reminiscent of the lateral tunnel Fontan. However, this approach is still confidentially limited to a small number of centers. In 2013, we designed a circuit that mimics the actual surgical technique of extracardiac total cavopulmonary connection to allow for transcatheter completion in an animal study. A polytetrafluoroethylene conduit was connected between the pulmonary artery and the inferior vena cava (IVC). The superior anastomosis was occluded to avoid flow between IVC and superior vena cava (SVC). The conduit was connected to the right atrium (RA) and a large fenestration was created to allow free flow from the IVC to the RA. Extrapolating our approach, a center reported the successful transcatheter completion of an extracardiac Fontan in a 6-year-old child. However, this technique is not directly transposable to our population of patients who require preconditioning in infancy. We report here an innovative extension of this technique that may allow preparing patients in infancy, ideally at the time of the Glenn in the future, to receive an extracardiac Fontan at 2 years/11 kg without additional surgery.

Severe Left Ventricular Outflow Tract Obstruction Immediately After Surgical Repair of Ebstein's Anomaly.

A 52-year-old man following surgery for Ebstein's anomaly after repair developed acute hemodynamically significant left ventricular outflow tract obstruction with systolic anterior motion of the mitral valve and severe mitral regurgitation. Fluid resuscitation and weaning of inotropes were unsuccessful. Left ventricular outflow tract obstruction and mitral regurgitation resolved by using esmolol. (Level of Difficulty: Intermediate.).

The Ozaki Procedure With CardioCel Patch for Children and Young Adults With Aortic Valve Disease: Preliminary Experience - a Word of Caution.

BACKGROUND: The Ozaki procedure is a surgical technique for patients with significant aortic stenosis or regurgitation or both where valve repair cannot be performed. Individual cusps are cut from glutaraldehyde-treated autologous pericardium or bovine pericardium and implanted into the aortic valve position. Encouraging results have been reported within the adult population. There are limited published data on success of this procedure in younger patients. METHODS: We present a series of five children and young adults who underwent the Ozaki procedure with neoaortic valve cusps made from CardioCel, a decellularized bovine pericardial patch treated with a monomeric glutaraldehyde. RESULTS: There were no complications in the initial postoperative period and short inpatient stay. At a mean follow-up of 29.6 months (range: 22-36 months), 4 patients had no evidence of stenosis and 3 patients had trivial or no regurgitation from the neoaortic valve. Overall, two patients had complications related to the valve and underwent reintervention during the follow-up period with a Ross procedure. One of these patients who was not taking long-term anticoagulation experienced a transient ischemic attack. CONCLUSIONS: Our experience demonstrates that the Ozaki procedure with CardioCel in pediatric and young adult patients should be approached with caution. Further research with larger groups of pediatric patients, comparison of different graft materials, and longer follow-up is required to ascertain long-term success in children.

Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly.

BACKGROUND: Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. METHODS: Fifty consecutive EA patients (1 day-18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ±â€¯41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease. RESULTS: Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m2 (IQR 27.5-83) vs 28.4 mm/m2 (IQR 17.5-47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ±â€¯7.7% vs 36.7 ±â€¯9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7-0.98) vs 0.55 (IQR 0.4-0.7); p = 0.000], a lower functional RV-longitudinal strain (-10.2 ±â€¯6.2% vs -16.2 ±â€¯7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ±â€¯13.5% vs 36.3 ±â€¯12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis. CONCLUSION: Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children.

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.

Accuracy of computed tomography in detection of great vessel stenosis or hypoplasia before superior bidirectional cavopulmonary connection: Comparison with cardiac catheterization and surgical findings.

BACKGROUND: Cardiac catheterization is the gold-standard modality for investigation of cardiovascular morphology before bidirectional cavopulmonary connection, but requires general anaesthesia and is associated with procedural risk. AIMS: To assess the diagnostic accuracy and safety of computed tomography in diagnosing great vessel stenosis/hypoplasia compared with cardiac catheterization and surgical findings. METHODS: Twenty-seven patients (10 after Norwood stage I) underwent computed tomography before surgery between January 2010 and June 2016; 16 of these patients also underwent cardiac catheterization. Proximal and distal pulmonary artery, aortic isthmus and descending aorta measurements, radiation dose and complications were compared via Bland-Altman analyses and correlation coefficients. RESULTS: The accuracy of computed tomography in detecting stenosis/hypoplasia of either pulmonary artery was 96.1% compared with surgical findings. For absolute vessel measurements and Z-scores, there was high correlation between computed tomography and angiography at catheterization (r=0.98 for both) and a low mean bias (0.71mm and 0.48; respectively). The magnitude of intertechnique differences observed for individual patients was low (95% of the values ranged between -0.9 and 2.3mm and between -0.7 and 1.7, respectively). Four patients (25%) experienced minor complications from cardiac catheterization, whereas there were no complications from computed tomography. Patients tended to receive a higher radiation dose with cardiac catheterization than with computed tomography, even after exclusion of interventional catheterization procedures (median 2.5 mSv [interquartile range 1.3 to 3.4 mSv] versus median 1.3 mSv [interquartile range 0.9 to 2.6 mSv], respectively; P=0.13). All computed tomography scans were performed without sedation. CONCLUSIONS: Computed tomography may replace cardiac catheterization in identification of great vessel stenosis/hypoplasia before bidirectional cavopulmonary connection when no intervention before surgery is required. Computed tomography carries lower morbidity, can be performed without sedation and may be associated with less radiation.

Symptomatic partial and transitional atrioventricular septal defect repaired in infancy.

OBJECTIVES: Infants with symptomatic partial and transitional atrioventricular septal defect undergoing early surgical repair are thought to be at greater risk. However, the outcome and risk profile of this cohort of patients are poorly defined. The aim of this study was to investigate the outcome of symptomatic infants undergoing early repair and to identify risk factors which may predict mortality and reoperation. METHODS: This multicentre study recruited 51 patients (24 female) in three tertiary centres between 2000 and 2015. The inclusion criteria were as follows: (1) partial and transitional atrioventricular septal defect, (2) heart failure unresponsive to treatment, (3) biventricular repair during the first year of life. RESULTS: Median age at definitive surgery was 179 (range 0-357) days. Sixteen patients (31%) had unfavourable anatomy of the left atrioventricular valve: dysplastic (n=7), double orifice (n=3), severely deficient valve leaflets (n=1), hypoplastic left atrioventricular orifice and/or mural leaflet (n=3), short/poorly defined chords (n=2). There were three inhospital deaths (5.9%) after primary repair. Eleven patients (22%) were reoperated at a median interval of 40 days (4 days to 5.1 years) for severe left atrioventricular valve regurgitation and/or stenosis. One patient required mechanical replacement of the left atrioventricular valve. After median follow-up of 3.8 years (0.1-11.4 years), all patients were in New York Heart Association (NYHA) class I. In multivariable analysis, unfavourable anatomy of the left atrioventricular valve was the only risk factor associated with left atrioventricular valve reoperation. CONCLUSIONS: Although surgical repair is successful in the majority of the cases, patients with partial and transitional atrioventricular septal defect undergoing surgical repair during infancy experience significant morbidity and mortality. The reoperation rate is high with unfavourable left atrioventricular valve anatomy.

Initial 2-year results of CardioCel® patch implantation in children.

OBJECTIVES: We present the initial 2-year results of CardioCel® patch (Admedus Regen Pty Ltd, Perth, WA, Australia) implantation in paediatric patients with congenital heart diseases. METHODS: This was a single-centre retrospective study with prospectively collected data of all patients aged 18 years and under operated for congenital heart disease. The patch was introduced in 2014, with clinical practice committee approval and a special consent in case of an Ozaki procedure. Standard follow-up was performed with systematic clinical exams and echocardiograms. In case of reoperation or graft failure, the patch was removed and sent for a histological examination. RESULTS: Between March 2014 and April 2016, 101 patients had surgical repair using a CardioCel patch. The mean age was 22 (±36.3) months, and the mean weight was 9.7 (±10.3) kg. No infections and no intraoperative implantation difficulties were associated with the patch. The median follow-up period was 212 (range 4-726) days. The overall 30-day postoperative mortality was 3.8% (n = 4), none of which were related to graft failure. Five children were reoperated because of graft failure, 4 of whom had the patch implanted for aortic and were aged less than 10 days. The indications for patch implantation in the aortic position were aortopulmonary window, truncus arteriosus, coarctation and aortic arch hypoplasia repair. The median time between the first and the second operation for graft failure was 245 (range 5-480) days. CONCLUSIONS: Our experience shows that the patch is well tolerated in the septal, valvar and pulmonary artery positions. However, we experienced graft failures in infants in the aortic position.

Surgical repair of a complete atrioventricular septal defect with tetralogy of Fallot using a classical one-patch technique and pulmonary valve preservation.

The association of complete atrial ventricular septal defect (cAVSD) with tetralogy of Fallot remains a complex malformation that involves both inlet and outlet components of the ventricles. The surgical treatment used to be, in the 1980s, a staged repair strategy due to the high mortality rate of first primary repairs (PRs). However, nowadays, PRs have better outcomes. Although double-patch procedures are widely performed, this article describes the single-patch approach for cAVSD repair with transatrial-transpulmonary repair of the Fallot component with preservation of the pulmonary valve.

Arterial Switch Operation in Single Coronary Ostium With Intramural Course: Subclavian Artery Patch Angioplasty.

This report describes a neonatal arterial switch operation in a transposition of the great arteries with single ostium and intramural course coronary pattern. The technique proposed is based on the creation of two coronary neoostia by incising the left coronary intramural course and splitting the common button followed by a patch enlargement of left coronary button using the left subclavian artery.

Impact of an open-chest extracorporeal membrane oxygenation model for in situ simulated team training: a pilot study.

OBJECTIVES: To develop an affordable realistic open-chest extracorporeal membrane oxygenation (ECMO) model for embedded in situ interprofessional crisis resource management training in emergency management of a post-cardiac surgery child. METHODS: An innovative attachment to a high-fidelity mannequin (Laerdal Simbaby) was used to enable a cardiac tamponade/ECMO standstill scenario. Two saline bags with blood dye were placed over the mannequin's chest. A 'heart' bag with venous and arterial outlets was connected to the corresponding tubes of the ECMO circuit. The bag was divided into arterial and venous parts by loosely wrapping silicon tubing around its centre. A 'pericardial' bag was placed above it. Both were then covered by a chest skin that had a sutured silicone membrane window. False blood injected into the 'pericardial' bag caused expansion leading to (i) bulging of silastic membrane, simulating tamponade, and (ii) compression of tubing around the 'heart' bag, creating negative venous pressures and cessation of ECMO flow. In situ Simulation Paediatric Resuscitation Team Training (SPRinT) was performed on paediatric intensive care unit; the course included a formal team training/scenario of an open-chest ECMO child with acute cardiac tamponade due to blocked chest drains/debriefing by trained facilitators. RESULTS: Cardiac tamponade was reproducible, and ECMO flow/circuit pressure changes were effective and appropriate. There were eight participants: one cardiac surgeon, two intensivists, one cardiologist, one perfusionist and three nurses. Five of the eight reported the realism of the model and 6/8 the realism of the clinical scenario as highly effective. Eight of eight reported a highly effective impact on (i) their practice and (ii) teamwork. Six of eight reported a highly effective impact on communication skills and increased confidence in attending future real events. CONCLUSIONS: Innovative adaptation of a high-fidelity mannequin for open-chest ECMO simulation can achieve a realistic and reproducible training model. The impact on interprofessional team training is promising but needs to be validated further.

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.

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.

Isolated cleft of the mitral valve: distinctive features and surgical management.

BACKGROUND: Controversy remains as to whether isolated cleft of the mitral valve and cleft of the atrioventricular septal defect are different entities. Our objectives were to provide a precise description of isolated cleft of the mitral valve and to clarify its surgical management and outcome. METHODS: Patients with surgical repair of isolated cleft of the mitral valve were included. RESULTS: Ten patients (9 female) underwent repair at a mean age of 12.1 +/- 10.5 years and mean weight of 32.1 +/- 17.8 kg. Preoperative echocardiography showed mild or less than mild mitral regurgitation in 6 cases and moderate to severe regurgitation in 4. Intraoperative examination confirmed in all cases a cleft dividing the anterior leaflet of an otherwise normal mitral valve. Attachment of the cleft to the ventricular septum by accessory chordae was found in 3 cases whereas preoperative echocardiography found such attachments in 5. Direct suture of the cleft was performed in 9 cases, associated with repair of tricuspid valve straddling (n = 1), subaortic stenosis (n = 1), and ventricular septal defect (n = 1). One patient with thickened cleft's edges required an Alfieri-type repair. After a mean follow-up of 4.9 years (range, 1.3 to 11.9), all patients are asymptomatic without significant mitral regurgitation. CONCLUSIONS: Echocardiographic description of isolated cleft of the mitral valve is not always as accurate as intraoperative analysis. This is a distinct morphologic entity from the cleft of the left-sided valve of atrioventricular septal defect, and seems associated with a strong female predominance, with various cardiac and extracardiac features. Surgical repair is successful with excellent midterm results.

Transcatheter perforation followed by pulmonary valvuloplasty in neonates with pulmonary atresia and ventricular septal defect.

BACKGROUND: The classic management of neonates with pulmonary atresia with ventricular septal defect (PAVSD) and moderately hypoplastic pulmonary arteries is usually a systemic to pulmonary artery shunt or ductus arteriosus stenting. We report our experience of transcatheter treatment of PAVSD by perforation followed by balloon dilation of the valve, as it is performed in pulmonary atresia - intact ventricular septum. PATIENTS AND METHOD: Three patients were treated at a mean age and weight of 7.5 days (range 7-8) and 2.9kg (range 2.5-3.3), respectively. Two newborns were prenatally diagnosed, with micro deletion 22q11 in one case. The three patients had no other pulmonary blood flow support than the ductus arteriosus and were on prostaglandin E1 infusion. The pulmonary atresia was predominantly valvular without significant muscular obstruction. By echocardiography, the mean size of the pulmonary annulus was 6.5mm (range 6-7). In all cases, the valvular perforation was performed with a 0.014 coronary guidewire, followed by balloon dilation when successfully. RESULTS: The procedure succeeded in two cases but failed in the third newborn in whom a long subvalvar muscular stenosis was found at surgery. In the two successful cases, the mean postprocedural transpulmonary doppler gradient was 33.5mmHg (range 17-50). One patient experienced a femoral venous thrombosis that was successfully treated by heparin and a transient right bundle branch block occurred in another one. In the two successful cases, the prostaglandin E1 infusion was weaned and the surgical repair was performed at 4 and 12 months, respectively. CONCLUSION: In selected cases with PAVSD, perforation followed by balloon dilation of the pulmonary valve offers an interesting alternative to other surgical or transcatheter palliative therapies.

Ross and Ross-Konno procedures in infants, children and adolescents: a 13-year experience.

BACKGROUND AND AIM OF THE STUDY: Due to hemodynamic performance and potential for growth of the pulmonary autograft, the Ross operation is considered to be the surgery of choice for irreparable aortic valve disease in pediatric patients. The study aim was to analyze the long-term clinical and echocardiographic results of the Ross operation. METHODS: Between February 1993 and July 2006, 55 consecutive patients (mean age 10.0 +/- 6.2 years; range: 3 months to 18 years) underwent eithera Ross operation (n=46) or a Ross-Konno procedure (n=9). The underlying left ventricular outflow tract pathology was mainly congenital (n=47). Among patients, 23 (42%) had undergone a previous aortic valve procedure. Concomitant procedures were performed in 16 patients (29%). The Ross operation was performed as a root replacement in all cases; the mean cross-clamp time was 132 min (range: 100-188 min). The autograft diameter was indexed to the body surface area and compared to normal values. The mean follow up was 5.5 +/- 3.8 years, and was 100% complete. RESULTS: There was one early death (2%) and two late deaths (4%). The actuarial patient survival was 93% at 10 years. None of the patients developed moderate or severe autograft regurgitation. All measured maximal root diameters were above the 90th percentile of normal aortic diameter, without correlation to autograft regurgitation. Five patients (9%) had a mean homograft gradient > or = 40 mmHg, and two (4%) were reoperated on. The freedom from reoperation for homograft degeneration was 91% at 10 years. CONCLUSION: Autograft regurgitation after the Ross and Ross-Konno procedures is uncommon, and the risk of homograft degeneration appears low. Autograft dilatation is common but does not correlate with autograft regurgitation. When considering long-term freedom from autograft and homograft degeneration, the results of the present study confirm the Ross operation as the surgery of choice for irreparable aortic valve disease in infants, children and young adults.

Complete atrioventricular canal repair under 1 year: Rastelli one-patch procedure yields excellent long-term results.

BACKGROUND: Considering more recently proposed techniques, we have evaluated our midterm and long-term results of Rastelli one-patch repair in complete atrioventricular canal. METHODS: Between 1984 and 2005, 107 patients with a complete atrioventricular canal underwent a Rastelli one-patch procedure. Two groups were identified: 1984 to 1995 and 1995 to 2005 (respectively, 56 and 51 patients). Mean age at surgery was 5.3 +/- 3.4 months; mean weight was 5.5 +/- 3 kg; trisomy 21 was present in 81 patients; complete atrioventricular canal type A was found in 67 patients, type C in 40 patients. There were 12 cases of potentially parachute mitral valve and 14 associated anomalies treated simultaneously (pulmonary obstruction 11, coarctation 3). The coronary sinus was always left on the right side. After functional and anatomic evaluation, the cleft was closed completely in 8 and partially in 29, and was left intact in 70 cases. RESULTS: Early survival was 86% +/- 3%. Five patients underwent early reoperation for residual ventricular septal defect (n = 2) and mitral valve repair (n = 3). Nine patients underwent late reoperations with successful repair: subaortic stenosis (n = 4) and mitral valve repair (n = 5). Late survival at 10 and 15 years was 84% +/- 3%. Freedom from reoperation for mitral regurgitation was 94% +/- 3% at 10 years, and 91% +/- 3% at 15 and 20 years. At last follow-up 30 patients had mild and 3 had moderate mitral regurgitation. CONCLUSIONS: Rastelli single-patch repair in complete atrioventricular canal is a safe and reproducible technique. Among survivors, freedom from late reoperation for mitral regurgitation is very satisfactory. A properly taught, learned, and transmitted Rastelli one-patch technique compares very well with any other proposed technique.

Atresia of the aortic arch, with a collateral artery from the right subclavian artery supplying the descending aorta.

An infant of 2 months presented with absence of the femoral pulses, albeit with no signs of cardiac failure. The mother was known to have ingested Valproate during pregnancy. Echocardiography showed the aortic arch to be interrupted between the left common carotid and left subclavian arteries, so-called type B interruption, in the setting of an intact ventricular septum. Angiography, and multislice computed tomography, revealed the descending aorta to be supplied by a collateral artery originating from the right subclavian artery. Corrective surgery was successfully performed, but revealed an atretic segment of the arch at the site of interruption of flow.