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AimThis study sought to evaluate the feasibility and effectiveness of TAVI for treatment of aortic stenosis in the ACHD population.Methods and resultsReview of 802 patients that underwent TAVI from January 2008 to November 2019. 13 ACHD patients with different underlying congenital anatomy were identified (isolated bicuspid aortic valve was excluded and not defined as ACHD). The main endpoints were: paravalvular aortic regurgitation> grade 2 post-procedure, and all-cause 30-day mortality. Periprocedural and post-procedural complications were defined according to the UK TAVI registry. Median age was 66.7 years (range 29–84 years). Eleven patients had severe aortic stenosis (AS), 1 had both severe AS and severe aortic regurgitation (AR) and 1 had severe AR. Median hospital stay was 9 days (range 6–28 days). One patient required a pacemaker post-TAVI. No mortality or paravalvular AR > grade 2 at 30-days. Twelve patients had a reduction in NYHA class on their first post TAVI follow-up.ConclusionTAVI is viable option as an alternative to surgery for ACHD patients. Further experience with the use of TAVI in the ACHD patients is required to assess long-term outcomes in this unique group of patients.

A Fontan circulation is a series of palliative surgical procedures, which result in the diversion of the systemic venous return into pulmonary arterial circulation without passing through a ventricle. It is one of the available surgical strategies for patients with cardiac defects that preclude a successful bi-ventricular repair, which encompass a range of complex anatomy. This surgical repair has gone through a series of modifications since the concept was introduced in 1971. Echocardiography remains a vital tool in assessing patients with Fontan circulations but its limitations are well recognised. Cross-sectional imaging modalities such as cardiac MRI and CT are essential components in the systematic clinical evaluation of these patients. The purpose of this review is to understand the information that can be obtained with each cross-sectional modality as well as highlight the challenges that each modality faces.

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.

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.

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.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.There were 502 infants with AWD - 336 gastroschisis, 166 omphalocele. Infants with gastroschisis required a longer duration of total parenteral nutrition (19 vs 4days, p0.05), longer hospitalization (28 vs 15days, p0.05) and had a higher rate of systemic infection [23.5% vs 13.3%, OR 1.77 (1.15-2.74), p0.05] compared to infants with omphalocele. Overall, omphalocele infants had higher mortality rate compared to gastroschisis infants [OR 2.77 (1.53, 5.04), p0.05]. Gastroschisis mortality rates increased from epoch 1 to epoch 3 (4.2% to 8.8%).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.

The imaging of pulmonary venous anatomy has traditionally been performed with echocardiography and catheter pulmonary angiography. Magnetic resonance imaging (MRI) and notably multi-detector row computed tomography (CT) have refined the usual imaging techniques. Anomalous pulmonary venous drainage (APVD) is the drainage of one or more pulmonary veins outside the left atrium. Its detection is critical due to the strong association with congenital heart disease as well as other cardiac and respiratory anomalies, which have significant implications for patient management. The pervasive application of CT combined with the relatively non-specific clinical presentation of APVD has resulted in the increased incidental detection of these anomalies. Knowledge is hence vital as the imaging specialist is now usually the first person to make such a diagnosis. Furthermore, pulmonary veins are an important site for arrhythmogenic foci and radiofrequency ablation of such sites is used in the treatment of refractory atrial fibrillation. Hence an imaging road map of these veins is crucial before any management can take place. This pictorial review will illustrate embryology, normal and variant pulmonary vein anatomy, and varied patterns of APVD. Finally, we discuss the implications in the treatment of atrial fibrillation.

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.

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