Leadless pacemaker implantation in a patient with complex congenital heart disease and limited vascular access.

Management of rhythm related issues might be particularly challenging in patients with congenital heart disease due to complex anatomy and restricted vascular access. The leadless technology appears a suitable and attractive alternative for this population. We describe a patient with single ventricle physiology who successfully underwent implantation of a leadless pacemaker.

Mitral valve geometry in paediatric rheumatic mitral regurgitation.

Rheumatic mitral valve disease is associated with valvulitis and valvular regurgitation; however, the effect of the rheumatic process on the mitral valve geometry and function is not well understood. To assess mitral valve annulus remodelling in rheumatic mitral valve disease, 16 subjects aged 6-15 years with rheumatic mitral valve regurgitation [MR] [6 mild or moderate (Group 1), 10 severe (Group 2)] and 7 age- and body size-matched normal controls with adequate trans-thoracic echocardiograms were recruited. None of the patients had undergone surgical intervention and none had more than mild aortic regurgitation. None of the patients had mitral stenosis. 3D mitral valve geometry was assessed using a Tomtec system. The non-planar angle was increased in all subjects during early (control 147° ± 10, Group 1 168° ± 9, Group 2 166° ± 10; p < 0.05) and late systole (control 149° ± 12, Group 1 162° ± 10, Group 2 164° ± 6; p < 0.05), indicating loss of saddle shape. 2D annular area increased in Group 2 (control 397 ± 48 mm(2)/m(2) vs Group 2 739 ± 207, p < 0.05) with no significant change in annular perimeter indicating leaflet effacement. Mitral valve area correlated with left ventricular size (p < 0.001, r (2) = 0.74). There was no significant change in valve area and perimeter between early and late systole. Remodelling of the mitral valve apparatus in childhood rheumatic heart disease results in abnormal annular geometry. The mitral valve loses its saddle shape regardless of the severity of MR. This may be a consequence of inflammation on the fibrous trigones and surrounding annulus. In contrast, annular area enlargement occurs as a result of leaflet effacement and relates to MR severity and LV size.

Emergency cardiac resynchronisation in a 4kg infant post surgical closure of ventricular septal defect.

Cardiac resynchronisation therapy (CRT) is an established treatment for adult patients with cardiac failure due to mechanical and electrical dyssynchrony. Data on CRT in infants are scarce. We report the remarkable success of emergent CRT by epicardial pacing of the left ventricular apex in a 4kg infant with left ventricular failure due to LV dyssynchrony from left bundle branch block.

Successful Reconstruction of the Right Ventricular Outflow Tract by Implantation of Thymus Stem Cell Engineered Graft in Growing Swine.

Graft cellularization holds great promise in overcoming the limitations associated with prosthetic materials currently used in corrective cardiac surgery. In this study, the authors evaluated the advantages of graft cellularization for right ventricular outflow tract reconstruction in a novel porcine model. After 4.5 months from implantation, improved myocardial strain, better endothelialization and cardiomyocyte incorporation, and reduced fibrosis were observed in the cellularized grafts compared with the acellular grafts. To the authors' knowledge, this is the first demonstration of successful right ventricular outflow tract correction using bioengineered grafts in a large animal model.

Use of 3D Models in the Surgical Decision-Making Process in a Case of Double-Outlet Right Ventricle With Multiple Ventricular Septal Defects.

3D printing has recently become an affordable means of producing bespoke models and parts. This has now been extended to models produced from medical imaging, such as computed tomography (CT). Here we report the production of a selection of 3D models to compliment the available imaging data for a 12-month-old child with double-outlet right ventricle and two ventricular septal defects. The models were produced to assist with case management and surgical planning. We used both stereolithography and polyjet techniques to produce white rigid and flexible color models, respectively. The models were discussed both at the joint multidisciplinary meeting and between surgeon and cardiologist. From the blood pool model the clinicians were able to determine that the position of the coronary arteries meant an arterial switch operation was unlikely to be feasible. The soft myocardium model allowed the clinicians to assess the VSD anatomy and relationship with the aorta. The models, therefore, were of benefit in the development of the surgical plan. It was felt that the clinical situation was stable enough that an immediate intervention was not required, but the timing of any intervention would be dictated by decreasing oxygen saturation. Subsequently, the oxygen saturation of the patient did decrease and the decision was made to intervene. A further model was created to demonstrate the tricuspid apparatus. An arterial switch was ultimately performed without the LeCompte maneuver, the muscular VSD enlarged and baffled into the neo aortic root and the perimembranous VSD closed. At 1 month follow up SO2 was 100%, there was no breathlessness and no echocardiogram changes.

Computed tomography in congenital heart disease: how generic principles can be applied to create bespoke protocols in the Fontan circuit.

Cardiac computed tomography (CCT) has become an invaluable cross-sectional imaging modality in congenital heart disease (CHD) patients. However, altered anatomical connections and cardiovascular physiology makes CHD arguably the most challenging area in CCT imaging, which remains a complimentary modality to cardiac magnetic resonance and echocardiography. A bespoke CT protocol is often required to achieve a diagnostic examination; this can be achieved through careful consideration of the basic principles of image acquisition and contrast administration. This article reviews these principles and demonstrates how they can be applied to CCT in CHD using the Fontan circulation as an example.

Outcomes of infants with abdominal wall defects over 18years.

BACKGROUND/PURPOSE: Infants with abdominal wall defects (AWD) are at risk of poor outcomes including prolonged hospitalization, infections and mortality. Our objective was to describe and compare the outcomes of infants admitted with gastroschisis and omphalocele over 18years. METHODS: Population-based study of clinical data and outcomes of live-born infants with AWD admitted to all tertiary-level neonatal intensive care units in New South Wales and Australian Capital Territory from 1992 to 2009. RESULT: There were 502 infants with AWD - 336 gastroschisis, 166 omphalocele. Infants with gastroschisis required a longer duration of total parenteral nutrition (19 vs 4days, p<0.05), longer hospitalization (28 vs 15days, p<0.05) and had a higher rate of systemic infection [23.5% vs 13.3%, OR 1.77 (1.15-2.74), p<0.05] compared to infants with omphalocele. Overall, omphalocele infants had higher mortality rate compared to gastroschisis infants [OR 2.77 (1.53, 5.04), p<0.05]. Gastroschisis mortality rates increased from epoch 1 to epoch 3 (4.2% to 8.8%). CONCLUSION: Compared to infants with omphalocele, infants with gastroschisis required significantly longer hospitalization and parenteral nutrition with higher rates of infection. Infants with omphalocele had higher overall mortality rates. However, there has been an increase in the gastroschisis mortality rates but the cause for this is unclear.

Syndrome of inappropriate antidiuretic hormone associated with lopinavir therapy.

We report a case of the syndrome of inappropriate antidiuretic hormone associated with use of lopinavir in an HIV-infected child. This rare phenomenon has not previously been reported in children. Clinicians should be alert to the possibility of the syndrome of inappropriate antidiuretic hormone when prescribing lopinavir in children.