The aim was to determine whether the clinical features of tachycardias originating from the right ventricular outflow tract in children with an apparently normal heart could predict the presence and the severity of the histopathological substrate. Thirteen children (median age 6 years; range 6 months–12 years) with tachycardia originating from the right ventricular outflow tract of apparently normal hearts, were assessed by echocardiography, heart catheterization with angiography, endomyocardial biopsy (13 patients) and magnetic resonance imaging (MRJ) (nine patients). Tachycardia was symptomatic in six and sustained in nine. Endomyocardial biopsy and MRI revealed acute myocarditis in five patients (38%), fatty infiltration of the right ventricle in two (15%), and minor histologic abnormalities in three (23%). Myocarditis was diagnosed in three of nine patients with sustained ventricular tachycardia, as opposed to two of four with non-sustained tachycardia (p = NS); in three of six symptomatic versus two of seven asymptomatic patients (p = NS); and in two of eight patients in whom ventricular tachycardia was induced during exercise testing as opposed to one of three in which it was not inducible (p = NS). A histopathological substrate was found in six of nine patients with sustained ventricular tachycardia, and in all four with non-sustained tachycardia (p = NS); in five of six patients with symptoms versus five of seven asymptomatic patients (p = NS); and in five of eight with inducible ventricular tachycardia during exercise testing versus all three in whom it was not inducible (p = NS). The mean rate of tachycardia was 184 ± 39 beats min−1 in patients with myocarditis, as opposed to 171 ± 48 in patients without myocarditis (p = NS); and 163 ± 33 in patients with a histopathological substrate compared with 210 ± 65 in patients without a histopathological substrate (p = NS). It is concluded that a histopathological substrate is present in the greater majority of children affected by the so-called right ventricular outflow tract tachycardia, but that the clinical features of the tachycardia do not predict the presence and the severity of this histopathological substrate.

Between January, 2009 and December, 2013, 84 patients were identified who underwent isolated mitral valve surgery in Jamaica at The University Hospital of the West Indies and The Bustamante Hospital for Children. The most common pathology requiring surgery was rheumatic heart disease, accounting for 84% of the procedures performed. The majority of patients had regurgitation of the mitral valve (67%), stenosis of the mitral valve (22%), and mixed mitral valve disease (11%). The most common procedure performed was replacement of the mitral valve (69%), followed by mitral valve repair (29%). Among the patients, one underwent closed mitral commissurotomy. The choice of procedure differed between age groups. In the paediatric population (<18 years of age), the majority of patients underwent repair of the mitral valve (89%). In the adult population (18 years and above), the majority of patients underwent mitral valve replacement (93%). Overall, of all the patients undergoing replacement of the mitral valve, 89% received a mechanical valve prosthesis, whereas 11% received a bioprosthetic valve prosthesis. Of the group of patients who underwent mitral valve repair for rheumatic heart disease, 19% required re-operation. The average time between initial surgery and re-operation was 1.2 years. Rheumatic fever and rheumatic heart disease remain significant public health challenges in Jamaica and other developing countries. Focus must remain on primary and secondary prevention strategies in order to limit the burden of rheumatic valvulopathies. Attention should also be directed towards improving access to surgical treatment for young adults.

Objective: Acute kidney injury is common in neonates following surgery for congenital heart disease. We conducted a retrospective analysis to determine whether neonates with smaller pre-operative renal volume were more likely to develop post-operative acute kidney injury. Design/Setting: We conducted a retrospective review of 72 neonates who underwent congenital heart surgery for any lesion other than patent ductus arteriosus at our institution from January 2007 to December 2011. Renal volume was calculated by ultrasound using the prolate ellipsoid formula. The presence and severity of post-operative acute kidney injury was determined both by measuring the peak serum creatinine in the first 7 days post-operatively and by using the Acute Kidney Injury Network scoring system. Results: Using a linear change point model, a threshold renal volume of 17 cm3 was identified. Below this threshold, there was an inverse linear relationship between renal volume and peak post-operative creatinine for all patients (p = 0.036) and the subgroup with a single morphologic right ventricle (p = 0.046). There was a non-significant trend towards more acute kidney injury using Acute Kidney Injury Network criteria in all neonates with renal volume ≤17 cm3 (p = 0.11) and in the subgroup with a single morphologic right ventricle (p = 0.17). Conclusions: Pre-operative renal volume ≤17 cm3 is associated with a higher peak post-operative creatinine and potentially greater risk for post-operative acute kidney injury for neonates undergoing congenital heart surgery. Neonates with a single right ventricle may be at higher risk.

Background: Altered mesenteric perfusion may be a contributor to the development of necrotising enterocolitis in patients with hypoplastic left heart syndrome. The goal of this study was to document mesenteric flow patterns in patients with hypoplastic left heart syndrome pre- and post-hybrid procedure. Methods: A prospective study on all patients with hypoplatic left heart syndrome undergoing the hybrid procedure was conducted. Doppler ultrasound analysis of the coeliac and superior mesenteric artery was performed. Results: A total of 13 patients were evaluated. There was a significant difference in the coeliac artery effective velocity-time intergral pre- and post-hybrid procedure (8.69±3.84 versus 12.51±4.95 cm, respectively). There were significant differences in the superior mesenteric artery antegrade velocity-time integral pre- and post-hybrid procedure (6.86±2.45 versus 10.52±2.64 cm, respectively) and superior mesenteric artery effective velocity-time integral pre- and post-hybrid procedure (6.22±2.68 versus 9.73±2.73 cm, respectively). There were no significant differences between the coeliac and superior mesenteric artery Doppler indices in the pre-hybrid procedure; there were, however, significant differences in the post-hybrid procedure between coeliac and superior mesenteric artery antegrade velocity-time integral (13.8 2±5.60 versus 10.52±2.64 cm, respectively) and effective velocity-time integral (13.04±4.71 versus 9.73±2.73 cm, respectively). Conclusion: Doppler mesenteric indices of perfusion improve in patients with hypoplastic left heart syndrome after the hybrid procedure; however, there appears to be preferential flow to the coeliac artery versus the superior mesenteric artery in these patients post-procedure.

We present a case of severe aortic coarctation in a donor of twin–twin transfusion syndrome. Patients underwent two angioplasty procedures at age 7 and 47 days, weighing 900 and 1500 g, respectively. Umbilical artery approach was used in the first procedure, and femoral artery approach was used in the second. Follow-up at the age of 13 months showed no recurrence of coarctation.

Percutaneous closure of muscular ventricular septal defects has been well described and has not attracted the same controversy or scrutiny as perimembranous defect closure. Therefore, the development of specifically designed devices has been limited. We report the first use of the Occlutech® muscular ventricular septal defect device. Does its design add any significant benefit?

We present a 3.5-year-old patient with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals, after repeat implantation of a central shunt, in whom we successfully re-purposed previous imaging scans with a novel image fusion software to guide diagnostic heart catheterisation. The pre-registered CT scan was segmented before the procedure and subsequently manually fused with two-dimensional fluoroscopy images. The overlaid roadmap enhanced selective cannulation of all major vessels originating from the aorta, making aortography unnecessary.

the bidirectional cavopulmonary anastomosis is commonly used in the palliation of patients with a functionally univentricular physiology. the management of alternative sources of flow of blood to the lungs, as well as the magnitude of acceptable accessory blood flow at the time of surgery, nonetheless, remains controversial. these issues are particularly significant when a cavopulmonary anastomosis is performed in infants who may become candidates for a fontan procedure. indeed, a long-standing volume overload, which is invariably associated with the maintenance of accessory sources of pulmonary blood flow, may result in systemic ventricular dysfunction. these observations prompted us to review the influence of antegrade pulmonary blood flow in the management of infants undergoing a cavopulmonary anastomosis.

We describe a case of a baby girl born with hypoplastic left heart syndrome consisting of mitral atresia, aortic atresia, hypoplastic ascending aorta, and left ventricle. The pulmonary arteries were hypoplastic, measuring 3 mm. Fluorescence in situ hybridisation analysis demonstrated a microduplication of chromosome 22q11.2. Subsequent array comparative genomic hybridisation showed a gain of 2.3 Mb in one copy of chromosome 22q at band 22q11.21. The proband underwent a successful Norwood procedure with Sano shunt and subsequently underwent bi-directional Glenn shunt and Fontan procedure. This report highlights the association between hypoplastic left heart syndrome with hypoplastic pulmonary arteries and chromosome 22q11.21 microduplication.

Aortico-left ventricular tunnel and left ventricular non-compaction are rare congenital cardiac anomalies with varied clinical presentations and sparsely described co-existence in the medical literature. Owing to the limited information about these diagnoses in tandem, we present four cases of aortico-left ventricular tunnel and left ventricular non-compaction from our institution and discuss the clinical presentation, management, and follow-up.

We described previously that the degree of deficiency of the ventricular septum (scooping) affects electrocardiographic findings in atrioventricular septal defect with separate right and left atrioventricular orifices. The aim of this study was to clarify the clinicomorphological relationships of the degree of scooping, the precise morphology of the atrioventricular valve, and electrocardiographic findings in hearts having a common atrioventricular orifice. In 63 patients with usual atrial arrangement, the outlet, scoop and inlet dimensions of the ventricular septum were measured by biplanar cineangiography and were compared with the body surface area. The scoop/outlet ratio and inlet/outlet ratio were also compared with the body surface area, atrioventricular valvar morphology, electrocardiographic findings, and severity of regurgitation of the atrioventricular valve. The results showed that the three individual dimensions of the septum correlated significantly with the body surface area, even though the scoop/outlet ratio and inlet/outlet ratio did not. A correlation was found between the scoop/outlet ratio and the degree of left axis deviation on the electrocardiogram (r=0.74, p<0.001). In 16 patients with division of the inferior bridging leaflet, there was a lower scoop/outlet ratio than in the remaining 37 with an undivided bridging leaflet (p<0.01). Of 24 patients with counterclockwise rotation in the horizontal loop of the vectorcardiogram, 22 had an undivided inferior bridging leaflet (p<0.01). The morphology of the superior bridging leaflet, in contrast, was not associated with the scoop/outlet ratio, and neither was the severity of valvar regurgitation. We conclude that the degree of scooping of the ventricular septum is related not only to electrocardiographic findings, but also to the morphology of the inferior bridging leaflet in atrioventricular septal defect with common atrioventricular orifice.

Sudden cardiac death is an uncommon but yet catastrophic event, which can occur in neonates and young children. Although extensive research has been carried out assessing the underlying causes, there still remains a degree of uncertainty around this area. Congenital heart disease (CHD) is one known cause of sudden cardiac death in children, the aetiology of which embraces virally induced mechanisms, genetic susceptibility, drug-induced, and maternal factors. Screening tools and investigations including electrocardiograms and echocardiograms alongside a concise history taking and physical examination can be used to identify the potential cardiovascular risk factors of sudden death. This review has comprehensively studied the causes and risk factors for sudden cardiac death in children with CHD and provides a collation and summary of the evidence available so far underpinning the complex link between the two. Moreover, current screening and prevention methods are discussed in detail in order to increase awareness and understanding of how we can improve patient outcomes.