A fenestration in the atrial septum may be temporarily beneficial in high–risk patients undergoing the modified Fontan operation. The venous system is decompressed into the left atrial cavities resulting in an improvement in the systemic output at the expense of a moderate arterial desaturation due to a right–to–left shunt. Two groups of patients undergoing the modified Fontan operation were compared. Those treated with the classic operation (non–fenestrated Fontan group, n=56) had lower prevalence of preoperative risk factors for the Fontan operation than those undergoing the Fontan operation with a fenestrated patch (n=91). Nevertheless, early results of mortality, repair take–down and morbidity (duration of pleuropericardial effusions) were comparable or better in the fenestrated Fontan group. By regression analysis the presence of a fenestrated patch was the major determinant in the reduction in the amount and duration of postoperative effusion. Of the early 73 survivors in the fenestrated Fontan group, fifty tolerated the closure of the fenestration, which occurred either spontaneously (five cases) or was achieved by a clamshell device during cardiac catheterization. Eleven patients failed the first attempt at fenestration closure, but this was successful at the second attempt a few months later. Coil embolization of arterial collaterals to the lung circulation and balloon dilatation of pulmonary artery stenoses were required in some cases. Other patients following the Fontan operation with a fenestrated patch showed an improvement of their ventricular function which was initially impaired. This favorable experience demonstrates the usefulness of the “fenestrated Fontan approach” in patients who are suboptimal candidates for the classic operation, provided that the preoperative risk factors are potentially reversible.

This review will outline the role of visiting cardiac surgical teams in low- and middle-income countries drawing on the collective experience of the authors in a wide range of locations. Requests for assistance can emerge from local programmes at a beginner or advanced stage. However, in all circumstances, careful pre-trip planning is necessary in conjunction with clinical and non-clinical local partners. The clinical evaluation, surgical procedures, and postoperative care all serve as a template for collaboration and education between the visiting and local teams in every aspect of care. Education focusses on both common and patient-specific issues. Case selection must appropriately balance the clinical priorities, safety, and educational objectives within the time constraints of trip duration. Considerable communication and practical challenges will present, and clinicians may need to make significant adjustments to their usual practice in order to function effectively in a resource-limited, unfamiliar, and multilingual environment. The effectiveness of visiting trips should be measured and constantly evaluated. Local and visiting teams should use data-driven evaluations of measurable outcomes and critical qualitative evaluation to repeatedly re-assess their interim goals. Progress invariably takes several years to achieve the final goal: an autonomous self-governing, self-financed, cardiac programme capable of providing care for children with complex CHD. This outcome is consistent with redundancy for the visiting trips model at the site, although fraternal, professional, and academic links will invariably remain for many years.

The abilities for both computer technology, and intra-operative video-imaging, are evolving rapidly. The merger of these two sciences can be very beneficial, both to congenital cardiac surgeons in general, and in facilitating the creation of a cardioscopic database in particular.

The association of left isomerism with interruption and azygos continuation of the inferior caval vein, anomalous connection of the right pulmonary veins to the right atrium, an interatrial commu nication of sinus venosus type, and tetralogy of Fallot with right aortic arch and anomalous coronary artery has been observed in a 6-month-old patient. As far as we are aware, the association of these congenital car diac malformations has not previously been reported in patients with isomeric left atrial appendages.

Congenital anomalies of the coronary arteries are an uncommon, but important, cause of pain in the chest, myocardial ischaemia and even sudden cardiac death, especially in young individuals. This paper focuses on the surgical treatment of congenital anomalies of the coronary arteries in adults; indications for surgery and the different surgical options will be reviewed.

Aim: To report results of transcatheter occlusion of moderate to large patent arterial ducts, having a minimum diameter above 2.5 mm, with the Amplatzer duct occluder, and to compare these with results achieved using Rashkind or Sideris devices and Cook detachable coils. Design and setting: Retrospective study conducted on intention-to-treat data from a tertiary referral centre. Patients: Since 1989, 116 consecutive patients, 77 females and 39 males, underwent percutaneous closure with several devices. We used the Rashkind double umbrella in 23 patients, the Sideris buttoned device in 39 patients, coils in 17 patients, and the Amplatzer duct occluder in 37 patients. The median age of the patients was 37 months, and the median weight 13 kg. The mean minimum diameter of the duct was 3.8 ± 1.22 mm, with a median of 3.5 mm, and range from 2.5 to 10 mm. Results: Implantation succeeded in all but 9 of the children (92%). The time of fluoroscopy was shorter, and full occlusion was better as judged on angiography, in patients closed using the Amplatzer device, despite closure of larger ducts, than in patients closed using other devices (p < 0.0001, p = 0.0003, and p = 0.0015 for the Rashkind, Sideris, and coils, respectively). Complications included embolisation in 2 patients, and haemolysis in 3 patients. In 12 patients, a second device was inserted because of residual shunting noted during follow-up. Complete occlusion was achieved earlier after implantation (p = 0.0002), and the rate of complete occlusion was better in patients receiving an Amplatzer device (97%, p = 0.024) than in patients undergoing closure with other devices. Conclusion: Transcatheter closure of moderate to large patent arterial ducts using the Amplatzer duct occluder is an effective and safe procedure, providing better results than those achieved using other occluders.

Since June 1998, we have used an Amplatzer device whenever considered appropriate in patients with isolated defects within the oval fossa. The aim of this study was to define the total cohort of patients with isolated defects in the oval fossa seen at this hospital, so as to assess the impact of this policy on contemporary management. In the two-year period commencing 1st June 1998, 116 patients older than 6 months were seen with an isolated septal defect within the oval fossa. Mean age at closure or last review was 5.8 years, with a range from 0.5 to 20 years. In total, 42 (36%) patients were assigned to surgical closure, 25 (22%) to closure using an Amplatzer device, and 49 (42%) remained under clinical follow up. Direct referral for surgical closure occurred in 24 (21%) patients, in whom transcatheter closure was considered not appropriate after transthoracic echocardiography. Transoesophageal echocardiography was performed in 45 (39%) patients to assess suitability for closure using the Amplatzer device. Of these, 20 (44% of the group undergoing transoesophageal echocardiography) were considered unsuitable for closure in this fashion. Of these, 8 were referred for surgery and 2 with small defects were considered not to require closure. Patients undergoing closure with the device were older than the group referred for surgical closure, having a median age of 7.8 versus 3.6 years, and stayed for a shorter period in hospital. Those closed using the device stayed for 2 days, as opposed to a median of 5 days, with a range from 4 to 10 days for those undergoing surgical closure. Closure was complete as assessed by echocardiography after follow up of 1–3 months in both groups. There were no recognised complications related to insertion of the device, whereas transient postoperative morbidity occurred in 38% of those closed surgically. Insertion of an Amplatzer device was considered to be appropriate in 37% of patients older than 6 months requiring closure of an atrial septal defect in the oval fossa.

We report a 10-month-old girl with KCNT1 (c1420C > T; p. Arg474Cys, R474C) mutation-associated epileptic encephalopathy, systemic-to-pulmonary artery “collateralopathy”, and intermittent QTc prolongation. Spontaneous regression of systemic-to-pulmonary artery collateral-mediated left heart dilation was noted in this patient, a finding which was ominous as it heralded the onset of severe pulmonary hypertension. The structural and electrical phenotypic features of KCNT1 mutation-associated heart disease, including the novel findings noted in our patient, are discussed in detail.

The heterotaxy syndromes are characterized by a high incidence of cardiac anomalies of extreme heterogeneity. Due to this, the surgical management is varied and challenging. Although a minority of patients can undergo biventricular repair, the complexity of the cardiac defects, and the high incidence of ventricular hypoplasia, mandate a surgical approach in the majority of patients depending on the creation of a functionally univentricular heart. Traditionally, the functionally univentricular approach was associated with a high mortality. More recently, the results have been improving as a result of better understanding of these malformations, and logical improvements in surgical technique. In this review, we will make brief comments on the nomenclature and classification of the heterotaxy syndromes, and the range of cardiac anomalies, before concentrating on the surgical treatment for those patients having functionally univentricular hearts in the setting of heterotaxy. More specifically, we will review initial palliation in early life, creation of the cavopulmonary anastomoses, including the Kawashima procedure and the Fontan circulation, and the role of transplantation.

Anomalous drainage of the right superior caval vein into the morphologically left atrium as an isolated cardiac malformation is a rare anomaly. Most patients present with cyanosis. Thus far, about 20 cases have been reported in the literature. We report a case of cyanosis due to this malformation in a male neonate, which was complicated by the meconium aspiration syndrome. The malformation was diagnosed by echocardiography and cardiac catheterization. Surgery resulted in complete recovery.

Introduction: A variety of patch materials have been used in the repair of intracardiac defects. We evaluated the short- and mid-term clinical and echocardiography results of glutaraldehyde-preserved bovine pericardium patches used to repair intracardiac defects in our clinic. Methods and results: This study examines the short- (up to 30 days post-operatively) and mid-term (up to 24 months post-operatively) results of 533 patients with intracardiac defects who underwent surgical correction with glutaraldehyde-preserved bovine pericardium patches between 2004 and 2010 at a university clinic. Short- and mid-term post-operative echocardiographic studies showed no evidence of calcification, thrombus, or aneurysmal dilatation on the patch. Vegetation developed in two (0.37%) of the 533 patients. Conclusion: These results that have been obtained from a group of large number of patients imply that the glutaraldehyde-preserved bovine pericardium patches may be preferable in the closure of cardiac defects because of their low complication rates and ease of use.

Constrictive pericarditis is a uncommom disease in children. We have now encountered pericardial thickening as the cause of severe constrictive physiology in two patients, one also having haemodynamic features of restrictive cardiomyopathy. Both patients, who had refractory ascites and evidence of increased systemic venous pressure, underwent Doppler echocardiography, cardiac catheterisation, and magnetic resonance imaging. Resonance imaging failed to show any thickning of the pericardium, but cardiac catheterisation revealed diastolic equalisation of pressures in all four chambers, with only mild elevation of pulmonary pressure in the first patient, but nearly equalisation of diastolic pressure, and a very high pulmonary arterial pressure with a difference of 7 mm Hg between the end diastolic pressures in the two ventricles in the second patient. Doppler revealed a restrictive pattern of mitral inflow, with high E and small A velocities and a short deceleration time. The clinical background did not suggest pericardial disease in either of the patients. We conclude that a careful search is needed to uncover constrictive pericarditis when there is no previous disease which may suggest late pericardial constriction. The haemodynamic features of restrictive cardiomyopathy can co-exist with pericardial restriction, and differentiation between the two entities is critical in view of the diverse management and prognosis of the two conditions.

Antiepileptic medications have been reported to cause disturbances in cardiac conduction. Lacosamide decreases seizure burden by modulating sodium channels. Although it has been demonstrated to have few side effects, there have been reports of clinically significant cardiac conduction disturbances. We report the case of a child with hypoplastic left-heart syndrome and well-controlled multifocal atrial tachycardia who developed haemodynamically significant atrial tachycardia after receiving two doses of lacosamide.

Coronary vasospasm can result from silent myocardial ischaemia to sudden death. There are many precipitant factors including different pharmacological agents. Kounis syndrome is defined by acute coronary syndromes associated with anaphylactic or anaphylactoid reactions. We report, to the best of our knowledge, the first paediatric case of Kounis syndrome due to intravenous atropine.

Although mortality rates for patients with d-transposition of the great arteries remain quite low, these patients have a unique circulation that requires careful management in the peri-operative period. Despite the improved mortality for patients with common arterial trunk, the course in the intensive care unit is remarkable for significant morbidity and utilisation of significant resources. Pre-operative patient management focuses on balancing competing circulations, pulmonary and systemic, which exist in parallel rather than in series, as in the normal circulation. Post-operative patient management in both lesions focuses on optimising systemic output, respiratory status, and mitigating the effects of cardiopulmonary bypass. In this article, we review pre- and post-operative intensive care management in neonates with d-transposition of the great arteries and common arterial trunk.

We undertook our study in order to evaluate right ventricular function and perfusion by conventional and contrast echocardiography in adults with congenitally corrected transposition who had not undergone cardiac surgery, comparing the echocardiographic findings with those obtained using equilibrium radionuclide ventriculography and gated single-photon emission computed tomography with Technetiumc-99 m sestamibi.

We discovered severe tricuspid regurgitation in 8 patients (61%). Right ventricular ejection fraction, as calculated by nuclear medicine, had a correlation of 0.67 (p = 0.059) with area fractional shortening and 0.84 (p = 0.01) with ejection fraction calculated by the method depending on descent of the tricuspid ring. All patients with severe tricuspid regurgitation also had right ventricular dysfunction. Of these 8 patients, 7 had persistent perfusion defects, while 6 also had ischemic perfusion defects.

Echo contrast had a high sensitivity, at 91%, and also specificity and positive predictive value, both at 100%, for persistent defects, and a negative predictive value of 66% compared to methods depending on nuclear medicine. The sensitivity of contrast echocardiography for detection of ischemic defects was 66%, the specificity 100%, the positive predictive value 100%, and the negative predictive value 77% compared to the methods involving nuclear medicine.

The method depending on descent of the tricuspid ring had the highest correlation with equilibrium radionuclide ventriculography in evaluation of right ventricular function in patients with congenitally corrected transposition.

We conclude that contrast echocardiography is extremely valuable when assessing right ventricular myocardial perfusion, having high sensitivity and specificity for detecting persistent defects, although sensitivity was less for detection of ischemic defects than that of gated single-photon emission computed tomography with Technetium-99 m Sestamibi.

Persistent and ischemic perfusion defects, together with chronic volume overload from tricuspid regurgitation, are the determining factors of right ventricular dysfunction.