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.

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.

Scimitar syndrome and anomalous origin of the circumflex artery from the main pulmonary artery in infancy: a case report.

We present a neonate with an antenatal diagnosis of Scimitar syndrome and aortic arch hypoplasia. After delivery, computerised tomography scan additionally revealed an anomalous origin of the circumflex coronary artery from the main pulmonary artery. The management of this rare combination is discussed.

The del22q11.2 candidate gene Tbx1 controls regional outflow tract identity and coronary artery patterning.

TBX1, encoding a T-box containing transcription factor, is the major candidate gene for del22q11.2 or DiGeorge syndrome, characterized by craniofacial and cardiovascular defects including tetralogy of Fallot and common arterial trunk. Mice lacking Tbx1 have severe defects in the development of pharyngeal derivatives including cardiac progenitor cells of the second heart field that contribute to the arterial pole of the heart. The outflow tract of Tbx1 mutant embryos is short and narrow resulting in common arterial trunk. Here we show by a series of genetic crosses using transgene markers of second heart field derived myocardium and coronary endothelial cells that a subdomain of myocardium normally observed at the base of the pulmonary trunk is reduced and malpositioned in Tbx1 mutant hearts. This defect is associated with anomalous coronary artery patterning. Both right and left coronary ostia form predominantly at the right/ventral sinus in mutant hearts, proximal coronary arteries coursing across the normally coronary free ventral region of the heart. We have identified Semaphorin3c as a Tbx1-dependent gene expressed in subpulmonary myocardium. Our results implicate second heart field development in coronary artery patterning and provide new insights into the association between conotruncal defects and coronary artery anomalies.

First pediatric transatlantic air ambulance transportation on a Berlin Heart EXCOR left ventricular assist device as a bridge to transplantation.

INTRODUCTION: Mechanical circulatory devices are indicated in patients with refractory cardiac failure as a bridge to recovery or to transplantation. Whenever required, transportation while on mechanical support is a challenge and still limited by technical restrictions or distance. CASE REPORT: We report the first pediatric case of transatlantic air transportation on a Berlin Heart EXCOR ventricular assist device (Berlin Heart, Berlin, Germany) of a 13-yr-old American female who presented in cardiogenic shock with severe systolic dysfunction while vacationing in France. Rapid hemodynamic deterioration occurred despite maximal medical treatment, and she was supported initially with extracorporeal membrane oxygenation converted to a Berlin Heart EXCOR left ventricular assist device. Long-distance air transportation of the patient was accomplished 3 wks after implantation from Marseille, France, to Denver, Colorado. No adverse hemodynamic effects were encountered during the 13.5-hr flight (8770 km). The patient did not recover sufficient cardiac function and underwent successful orthotopic heart transplantation 3 months after the initial event. CONCLUSIONS: Our experience suggests that long-distance air transportation of pediatric patients using the Berlin Heart EXCOR mobile unit as a bridge to recovery or transplantation is feasible and appears safe.

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.).

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.

Paclitaxel drug-eluting stent placement for pulmonary vein stenosis as a bridge to heart-lung transplantation.

Congenital pulmonary vein stenosis (PVS) presents as an isolated lesion or in association with other congenital heart anomalies. The most extensive forms of the disease are uniformly fatal because neither surgical repair nor transcatheter therapy results in long-term relief of the stenosis. A case is presented involving single-ventricle physiology associated with extensive and recurrent congenital PVS despite multiple attempts with surgical therapy. Heart-lung transplantation was ultimately performed after drug-eluting stents were placed in pulmonary veins as a bridge to the transplantation.

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.

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.

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.

Mitral annuloplasty in dilated cardiomyopathy: eighteen-year follow up in a pediatric case.

Dilated cardiomyopathy is often complicated by severe mitral regurgitation (MR) caused by dilation of the annulus. Heart transplantation remains the ultimate treatment when heart failure fails to respond to medical treatment. Mitral annuloplasty for severe MR in dilated or ischemic cardiomyopathy may delay or replace the need for heart transplantation. The case is reported of a six-year-old child with dilated cardiomyopathy, severe MR and high pulmonary arterial pressure. Following beneficial mitral annuloplasty in 1989, the patient is in excellent functional condition eighteen years later.

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.

Percutaneous pulmonary valve implantation in small conduits: A multicenter experience.

BACKGROUND: Guidelines allow percutaneous pulmonary valve implantation (PPVI) in conduits above 16mm diameter. Balloon dilatation of a conduit to a diameter>110% of the original implant size is also not recommended. We analyzed patients undergoing PPVI in such conditions. METHODS AND RESULTS: Nine patients (May 2008-July 2016) from 8 institutions underwent PPVI in conduits <16mm diameter. Five patients with 16-18mm conduit diameter underwent PPVI after over-expansion of the conduit>110%. Mean age and weight of the 14 patients was 12.1 (7.7 to 16) years and 44.9 (19 to 83) kg. Median conduit diameter at PPVI was 12 (10 to 17) mm. Median systolic right ventricular pressure was 70 (40 to 94) mmHg. Procedure was successful in all cases. A confined conduit rupture occurred in 7 patients (50%) and was treated with covered stent in 6. One patient experienced dislocation of 2 pulmonary artery stents that were parked distally. The post-implantation median systolic right ventricular pressure was 36 (28 to 51) mmHg. A fistula between right-ventricle outflow and aorta was found in one patient, secondary to undiagnosed conduit rupture. This was closed surgically. After a median follow-up of 20.16 (6.95 to 103.61) months, all the patients are asymptomatic with no significant RVOT stenosis. CONCLUSIONS: PPVI is feasible in small conduits but rate of ruptures is high. Although such ruptures remain contained and can be managed with covered stents in our experience, careful selection of patients and high level of expertise are necessary. More studies are needed to better assess the risk of PPVI in this population.

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.

Surgical Atrioventricular Valve Replacement With Melody Valve in Infants and Children.

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes <15 mm. Based on successful experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, the prosthesis has been modified for surgical valve replacement in pediatric patients with atrioventricular dysfunction with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9-24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P<0.001) with no significant increase in grade of regurgitation. Twelve months after implantation, cumulative incidence analysis indicated that 55% of the patients would be expected to be free from death, heart transplantation, structural valve deterioration, or valve replacement. Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.

Secondary subaortic stenosis in heart defects without any initial subaortic obstruction: a multifactorial postoperative event.

BACKGROUND/OBJECTIVE: Secondary subaortic stenosis (SSS) can occur after surgery for various congenital heart defects with or without initial left ventricular outflow tract obstruction (LVOTO). The objective of this study was to highlight the anatomical lesions and surgical procedures associated with the development of SSS after surgery on defects without initial LVOTO. METHODS: A retrospective study of 4710 patients was performed (1984-2005). The criterion for inclusion was a fixed subaortic obstruction requiring surgery, after an open- or closed-heart operation. The criterion for exclusion was an LVOTO at the time of the first operation. RESULTS: Twenty-eight patients were studied. The mean age at initial surgery was 32 months (4 days-47 years; median: 2 months). SSS occurred after three main types of surgery: repair of coarctation of the aorta, repair of AVSD and LV-aorta rerouting for double outlet right ventricle or transposition of great arteries. The mean delay of occurrence was 4.4 years (2 months-19 years). Frequently associated initial anatomical conditions were coarctation of the aorta (40%), lesions of the mitral valve (32%), bicuspid aortic valve (21%) and left superior vena cava (LSVC) (14%). Preoperative anatomical lesions of the LVOT were present in 93% of the cases. After the initial operation, only one patient had a mean echo-Doppler pressure gradient across the LVOT>20 mmHg. SSS was most frequently a subaortic membrane (n=23). The mean pressure gradient across SSS at the time of reoperation was 47+/-29 mmHg. Five patients developed a second SSS after 7.4 years (mean). One patient developed a third SSS. No patient died. When compared with patients without SSS, significant risk factors for SSS were low age at surgery (32 vs 74.9 months, p<10(-4)), pre-existing coarctation of the aorta (40 vs 10%, p<10(-4)), bicuspid aortic valve (21 vs 6%, p=0.002) and LSVC (14 vs 4%, p=0.02). CONCLUSIONS: SSS development is multifactorial, depending on initial anatomical lesions and initial surgery. Low age at initial surgery, coarctation of the aorta, bicuspid aortic valve and LSVC significantly increase the risk of SSS. These elements warrant long-term follow-up for early detection of SSS.

Right ventricular outflow tract reconstruction for pulmonary regurgitation after repair of tetralogy of Fallot. Preliminary results.

BACKGROUND: Pulmonary regurgitation after tetralogy of Fallot (ToF) repair is associated with right ventricular dilatation, failure and arrhythmia. Timing and technique for re-intervention remain controversial. METHODS: Our recent approach is to reconstruct the dilated right ventricle outflow tract (RVOT) as a fibro-muscular sleeve to support a pulmonary homograft valve conduit in orthotopic position. Indication is based on clinical and magnetic resonance (MR) criteria. We reviewed all patients who underwent RVOT reconstruction between January 2004 and February 2005. There were seven children (mean age 14+/-2 years) operated 13+/-2 years after ToF repair, and 12 adults (mean age 30+/-15 years) operated 23+/-10 years after ToF repair. Exercise testing and MR evaluation prior to surgery and at 1 year postoperative follow-up were compared. RESULTS: There was no operative mortality. At 1 year, pulmonary regurgitation was mild or less in 16/19 patients. Right ventricular (RV) end-diastolic (158+/-51 to 103+/-36ml/m(2), p<0.001) and end-systolic volumes (85+/-42 to 49+/-24ml/m(2), p=0.001) fell significantly. Importantly, effective RV stroke volume (43+/-10 to 48+/-7ml/m(2), p=0.019) and left ventricular (LV) stroke volume (43+/-7 to 47+/-7ml/m(2), p=0.009) increased significantly. The mean RV/LV end-diastolic volume ratio fell markedly in both children and adults (2.22+/-0.62 to 1.38+/-0.52). However, no improvement in maximal VO(2) on exercise was noted in either group. CONCLUSIONS: RVOT reconstruction restored valve function, improved RV dimensions and left and right stroke volumes. Maximal exercise capacity did not improve in either children or adults.

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.

Mitral valve replacement in neonatal endocarditis: time to discuss prevention strategies for Group B streptococcus disease.

Group B streptococcus (GBS) is recognised as one of the leading organisms in early-onset neonatal sepsis but is also a cause of late-onset GBS septicaemia, meningitis and rarely, infective endocarditis (IE). We report a case of a healthy term neonate who developed GBS septicaemia and meningitis having presented with parental concern and poor feeding. Subsequent identification and treatment of GBS resulted in the requirement for long-line intravascular access in order to administer antibiotic therapy. One week later, after repeated parental concern and symptoms of shortness of breath, the neonate presented to Accident and Emergency and subsequently a Paediatric Cardiorespiratory Intensive Care Unit where emergency resuscitation procedures were required and diagnosis of severe IE affecting the mitral valve was made. Mitral valve replacement was complicated with significant morbidity and prolonged hospitalisation. An innovative procedure to insert a Melody valve was successful.