Ebstein's Anomaly: From Fetus to Adult-Literature Review and Pathway for Patient Care.

Ebstein's anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve abnormalities. Ebstein's anomaly affects the development of the tricuspid valve with widely varying morphology and, therefore, clinical presentation. Associated congenital cardiac lesions tend to be found more often in younger patients and may even be the reason for presentation. Presentation can vary from the most extreme form in fetal life, to asymptomatic diagnosis late in adult life. The most symptomatic patients need intensive care support in the neonatal period. This article summarizes and analyzes the literature on Ebstein's anomaly and provides a framework for the investigation, management, and follow-up of these patients, whether they present via fetal detection or late in adult life. For each age group, the clinical presentation, required diagnostic investigations, natural history, and management are described. The surgical options available for patients with Ebstein's anomaly are detailed and analyzed, starting from the initial mono-leaflet repairs to the most recent cone repair and its modifications. The review also assesses the effects of pregnancy on the Ebstein's circulation, and vice versa, the effects of Ebstein's on pregnancy outcomes. Finally, two attached appendices are provided for a structured echocardiogram protocol and key information useful for comprehensive Multi-Disciplinary Team discussion.

Trifecta St. Jude medical® aortic valve in pulmonary position.

Introduction: To evaluate an aortic pericardial valve for pulmonary valve (PV) regurgitation after repair of congenital heart defects. Methods: From July 2012 to June 2016 71 patients, mean age 24 ± 13 years (four to years) underwent PV implantation of aortic pericardial valve, mean interval after previous repair = 21 ± 10 years (two to 47 years). Previous surgery at mean age 3.2 ± 7.2 years (one day to 49 years): tetralogy of Fallot repair in 83% (59/71), pulmonary valvotomy in 11% (8/71), relief of right ventricular outflow tract (RVOT) obstruction in 6% (4/71). Pre-operative echocardiography and MRI showed severe PV regurgitation in 97% (69/71), moderate in 3% (2/71) with associated RVOT obstruction. MRI and knowledge-based reconstruction 3D volumetry (KBR-3D-volumetry) showed mean PV regurgitation = 42 ± 9% (20-58%), mean indexed RV end-diastolic volume = 169 ± 33 (130-265) ml m-2 BSA and mean ejection fraction (EF) = 46 ± 8% (33-61%). Cardio-pulmonary exercise showed mean peak O2/uptake = 24 ± 8 ml kg-1 min-1 (14-45 ml kg-1 min-1), predicted max O2/uptake 66 ± 17% (26-97%). Pre-operative NYHA class was I in 17% (12/71) patients, II in 70% (50/71) and III in 13% (9/71). Results: Mean cardio-pulmonary bypass duration was 95 ± 30' (38-190'), mean aortic cross-clamp in 23% (16/71) 46 ± 31' (8-95'), with 77% (55/71) implantations without aortic cross-clamp. Size of implanted PV: 21 mm in seven patients, 23 mm in 33, 25 mm in 23, and 27 mm in eight. The z-score of the implanted PV was -0.16 ± 0.80 (-1.6 to 2.5), effective orifice area indexed (for BSA) of native PV was 1.5 ± 0.2 (1.2 to -2.1) vs. implanted PV 1.2 ± 0.3 (0.76 to -2.5) (p = ns). In 76% (54/71) patients surgical RV modelling was associated. Mean duration of mechanical ventilation was 6 ± 5 h (0-26 h), mean ICU stay 21 ± 11 h (12-64 h), mean hospital stay 6 ± 3 days (three to 19 days). In mean follow-up = 25 ± 14 months (six to 53 months) there were no early/late deaths, no need for cardiac intervention/re-operation, no valve-related complications, thrombosis or endocarditis. Last echocardiography showed absent PV regurgitation in 87.3% (62/71) patients, trivial/mild degree in 11.3% (8/71), moderate degree in 1.45% (1/71), mean max peak velocity through RVOT 1.6 ± 0.4 (1.0-2.4) m s-1. Mean indexed RV end-diastolic volume at MRI/KBR-3D-volumetry was 96 ± 20 (63-151) ml m-2 BSA, lower than pre-operatively (p < 0.001), and mean EF = 55 ± 4% (49-61%), higher than pre-operatively (p < 0.05). Almost all patients (99% = 70/71) remain in NYHA class I, 1.45% = 1/71 in class II. Conclusion: (a) Aortic pericardial valve is implantable in PV position with an easy and reproducible surgical technique; (b) valve size adequate for patient BSA can be implanted with simultaneous RV remodelling; (c) medium-term outcomes are good with maintained PV function, RV dimensions significantly reduced and EF significantly improved; (d) adequate valve size will allow later percutaneous valve-in-valve implantation.

Techniques for transcatheter retrieval of the occlutech ASD device United Kingdom-European multicenter report.

AIMS: To gather current experience in Occlutech ASD device retrieval, to determine whether snaring is an effective technique and to highlight alternative retrieval techniques; METHODS AND RESULTS: United Kingdom and European Occlutech ASD device implanters reported their experience in dealing with device embolization and retrieval. Six operators reported 12 retrieval cases. Retrieval was successful in 92% (11/12), although in most cases it was not straightforward and required multiple attempts using different techniques and equipment. When each different technique or equipment combination was considered separately, there were a total of 23 retrieval attempts. Fifteen attempts involved snaring the ball on the right atrial disc of the device ("the RA pin"). In 12/15 of these attempts the snare slipped off the RA pin. In 8/15 attempts snaring eventually failed. In two cases retrieval was facilitated by elongating the device in a blood vessel. In three cases retrieval was achieved by grasping the RA pin with the jaws of the Occlutech Flex II delivery cable; CONCLUSIONS: Snares do not grip the RA pin sufficiently to reliably retrieve the device. Funnelling the device into a blood vessel or grasping the RA pin with the jaws of the delivery cable may be successful alternatives. © 2016 Wiley Periodicals, Inc.

Radiofrequency ablation on veno-arterial extracorporeal life support in treatment of very sick infants with incessant tachymyopathy.

AIMS: To evaluate the use of extracorporeal membrane oxygenation (ECMO) in supporting infants who require radiofrequency ablation (RFA) for incessant tachyarrhythmias, with particular emphasis on modifications required to standard ablation techniques. METHODS AND RESULTS: Three cases of RFA carried out in infancy on ECMO support were reviewed retrospectively. Two infants with permanent junctional reciprocating tachycardia (PJRT) and one with ventricular tachycardia (VT) presented in a low cardiac output state, owing to cardiomyopathy caused by incessant tachycardia. In each case antiarrhythmic drug therapy caused haemodynamic collapse, requiring emergency ECMO support. Drug therapy on ECMO was not successful. In one patient, the tachycardia was controlled on ECMO with antiarrhythmic drugs, but recurred following ECMO decannulation. Each patient had a successful RFA on ECMO support. Power delivery was low during ablation lesions. In the PJRT cases power as low as 3-5 Watts was effective. In the VT ablation, an irrigated tip RFA catheter was required when cooling remained poor even after temporarily stopping ECMO flow. CONCLUSION: Extracorporeal membrane oxygenation provides a haemodynamically stable and safe platform for antiarrhythmic drug therapy and RFA in infants with incessant tachyarrhythmias. Once ECMO has been commenced, if the tachyarrhythmia remains difficult to control with antiarrhythmic drugs, RFA should be strongly considered, to avoid the risk of tachycardia recurrence following ECMO decannulation. Power delivery during ablation lesions may be low because of inadequate cooling of the catheter tip. Reducing or stopping flow in the ECMO circuit may not provide adequate cooling and an irrigated tip catheter may be required.

A congenital fistula between the descending aorta and the right pulmonary vein in a neonate presenting with heart failure.

We report a large congenital fistula connecting the descending thoracic aorta to the right upper pulmonary vein in a newborn baby presenting on the seventh day of life with cardiac failure and a continuous murmur heard posteriorly. The fistula was detected echocardiographically, and shown at cardiac catheterisation not to be suitable for percutaneous occlusion. The anatomy of the fistula was confirmed at surgery, when it was ligated successfully.

Awareness of the risk of endocarditis associated with tattooing and body piercing among patients with congenital heart disease and paediatric cardiologists in the United Kingdom.

Body art in the form of tattoos and piercing has become increasingly popular amongst children and teenagers, and is nowadays more socially acceptable despite media reports citing tissue destruction and death. Our study explored the awareness and experience of patients with congenital heart disease, and of cardiologists and professionals responsible for their care.

Right ventricular outflow tract reconstruction using Contegra valved conduit: natural history and conduit performance under pressure.

OBJECTIVE: To assess the performance of the bovine Contegra valved conduit used for right ventricular (RV) outflow tract reconstruction, particularly in relation to post-operative RV pressure. METHODS: Follow-up study of 64 consecutive right ventricular to pulmonary artery-conduit implants in 62 patients between January 2000 and April 2003. The majority of cases were forms of pulmonary atresia/VSD (n=24, 39%) or Fallot's tetralogy (n=13, 21%). Thirteen cases (21%) had aortic atresia, truncus arteriosus or discordant connections with pulmonary atresia/VSD. Twelve cases (19%) were conduit replacements. Echocardiography was performed for a median follow-up of 14 months (range 0-38 months). RESULTS: Median age at implantation was 13.8 months (range 0.1-244 months) and median weight was 8.9 kg (range 2.1-84.1 kg). Thirty-eight patients (59.4%) were <10 kg at the time of surgery. Early mortality was 6.4% (n=4). During follow-up there were four explantations (one for endocarditis and three for conduit dilatation) and 16 (28.6%) catheter interventions. Overall freedom from intervention at 1 and 3 years was 71+/-6% and 53+/-11%, respectively. Freedom from conduit-specific reintervention was 66+/-11% at the end of the study period. Reintervention was associated with small conduits (p=0.04), age <1 year (p=0.04) and with high RV/LV pressure ratio in the immediate post-operative period (p=0.0003). On multivariate analysis, the RV/LV pressure ratio was the strongest single factor predicting the overall reintervention (OR 5.45). Acquired distal conduit stenosis at suture line was the commonest indication for conduit-specific reintervention and was associated with the smaller conduits. The conduits explanted for dilatation showed neointimal proliferation, thrombosis, calcification and chronic inflammation. CONCLUSIONS: The Contegra conduit is widely applicable to RVOT reconstruction with satisfactory mid-term results. However, there is a significant incidence of conduit-related complications, particularly with the smaller conduits. Adverse performance was strongly associated with high RV/LV pressure ratio at completion of surgery. We would recommend cautious use of the conduits in patients with predicted high RV/LV pressure ratios, where careful monitoring of conduit performance is crucial. There is some element of unpredictability, which adds to the importance of close follow-up. Further studies are needed to explore the issues of thrombogenicity, degeneration, possible 'rejection', and the potential role of anti-platelet and anti-inflammatory modulation.