We report a male child with haemophagocytic lymphohistiocytosis who developed myocardial infarction after receiving etoposide. He recovered well with supportive measures and after discontinuation of etoposide. We discuss the possible mechanisms and differential diagnoses of myocardial infarction in our patient.

The use of intraoperative echocardiography with Doppler color flow imaging has acquired increased popularity for early assessment of efficacy of repair of congenital heart defects soon after discontinuance of cardiopulmonary bypass. This technology has been found useful also for evaluating the anatomy of the lesion prior to repair. Furthermore, with the use of color flow mapping, additional anomalies, such as patent arterial duct or multiple ventricular septal defects, which might have been overlooked during the initial examination, can be easily diagnosed. Moreover, intraoperative echocardiography provides the quickest and most sensitive method for a surgeon to look for residual atrioventricular valves regurgitation and/or residual atrial or ventricular septal defects as well as for depressed ventricular contractility. Previous studies1–4 have discussed specifically the applicability of intraoperative color flow imaging during the repair of atrioventricular septal defect with a common atrioventricular orifice using both epicardial and transesophageal methodology. Routine intraoperative echocardiography with Doppler color flow imaging has been used since March 1987 at Duke University Medical Center during repair of congenital heart defects. Our aim was to confirm the preoperative dignosis and to assess the quality of repair soon after discontinuance of cardiopulmonary bypass. Our overall experience now includes 612 patients of whom 239 or 39% were less than one year of age at the time of repair. This indicates our tendency to undertake early correction regardless of the age of patients and of the severity of the lesion.

A young female athlete is described with anomalous origin of the main stem of the left coronary artery from the non-facing sinus of Valsalva who sustained myocardial infarction and died suddenly after physical exertion. Autopsy findings illustrated the mechanistic importance of acute angle take-off of the left main coronary artery and a slit-like orifice, which was likely compressed and obstructed by acute expansion of the aortic wall. This rare type of coronary anomaly has been regarded as having little clinical significance, but it can lead to sudden cardiac death under physical exertion.

A 3-year-old patient is described with an unusual form of co-arctation due to hypoplasia of the transverse arch and fibromuscular dysplasia involving a long segment of the thoracic aorta. Surgical repair required resection of the aorta from the distal transverse arch to the mid-descending thoracic aorta, and replacement with a 16-mm Dacron interposition graft. This case demonstrates the importance of preoperative evaluation of the entire aorta in the presence of co-arctation due to fibromuscular dysplasia.

Sinus venosus defects are relatively uncommon forms of interatrial communication in which one of the caval veins has a biatrial connection, and partial anomalous pulmonary venous connections are the rule. The variety related to the inferior caval vein is much the rarer of the two forms, and here the defect is posterior and inferiorly placed. To determine whether an inferior sinus venosus defect could be identified preoperatively, we reviewed retrospectively the available echocardiograms and cineangiograms of the five patients with an inferior sinus venosus defect who underwent surgical repair between January 1983 and December 1991. A cross-sectional echocardiogram had been performed preoperatively in four of these patients, but the correct diagnosis had been made in only one case. Retrospective study, however, showed that the extent of the true atrial septum and the nature of the junction of the inferior caval vein with the atriums could be evaluated from the subcostal position in three of these four patients. Each of the three had an interatrial communication located posteriorly and inferiorly adjacent to the atrial connection of the inferior caval vein, along with abnormally connected right pulmonary veins. The oval fossa was intact and the coronary sinus was normal in all. Cardiac catheterization had been performed in three of the five patients. Anomalous return of right pulmonary veins was noted in each, and the posterior and inferior location of the interatrial communication was evident in two. An interatrial communication adjacent to the atrial connection of the inferior caval vein, along with anomalous connection of the right-sided pulmonary veins, are the diagnostic features of the inferior variety of sinus venosus defects. These features can be demonstrated by cross-sectional echocardiography from the subcostal position.

For forty years, heparin has been used to temporarily inhibit normal coagulation so as to permit the use of extracorporeal circulation. It has proved effective in preventing gross thrombosis and has not been compromised by changes in adjunctive practices (such as profound hypothermia; circulatory arrest), new pharmacological therapies (such as anesthetics or antibiotics), devices (such as membrane oxygenators), or during use in patients at the extremes of age. Unfortunately, heparin has been and continues to be inefficient in preserving hemostatic function during cardiopulmonary bypass resulting in undesirable bleeding after surgery.

As we described in the previous review,1 double inlet ventricle is usually found with the atrial chambers connected to a dominant left ventricle, less frequently to a dominant right ventricle, and rarely to a solitary and indeterminate ventricle. As we have also discussed in this supplement,2 double inlet to the left ventricle was, for many years, considered the exemplar of so-called “single ventricle”, despite the fact that such patients unequivocally possess one big and one small ventricle. Echocardiographic interrogation has served to resolve this controversy, showing that such patients make up a significant proportion of those having functionally univentricular hearts. Such echocardiographic investigation has also served to resolve similar controversies regarding patients having tricuspid atresia. For some time, it was argued that patients with tricuspid atresia also had “univentricular hearts”,3 but the logic used to underscore this approach was just as flawed as that used to justify the use of “single ventricle” in patients with double inlet atrioventricular connection.4,5 The increasing use of the Fontan procedure has served to demonstrate that these patients, along with many having mitral atresia in the setting of hypoplastic left heart syndrome, also have functionally univentricular arrangements. As we will show in this review, however, the anatomical substrates found in patients with atrioventricular valvar atresia are much more complex than those seen in the setting of double inlet ventricle. This is because atrioventricular valvar atresia can be produced either by absence of one atrioventricular connection, or by presence of an imperforate valvar membranes closing completely one or other of the two normal atrioventricular junctions. This important difference, combined with multiple segmental combinations, produces a bewildering array of potential anatomical substrates, with the complications magnified by the fact that, when one atrioventricular connection is absent, the other atrioventricular junction can be shared between the two ventricles, the so-called uniatrial and biventricular arrangement.6 In our review, we will first describe the anatomical options, before concentrating our attention on the more frequent patterns seen in clinical practice.

Tetralogy of Fallot is the most common cyanotic heart defect seen in children beyond infancy. There is considerable variation in surgical management today ranging from differences in timing of complete repair versus initial use of a shunt for palliation, and methods for right ventricular outflow tract reconstruction. This article will examine some of the current surgical management techniques for patients with Tetralogy of Fallot.

Improvements in diagnosis and surgical technique for correction have led to an increasing number of women with congenital heart disease reaching the child-bearing age. Pregnancy places considerable strain on the heart and circulation and necessitates marked cardiorespiratory adaptation. Today, with the exception of the Eisenmenger syndrome, there is no increased mortality associated with pregnancy in congenital heart disease. In contrast, there is still considerable morbidity, due to congestive heart failure, thromboembolic complications and disturbances of rhythm.

Fetal outcome is complicated by a high rate of spontaneous abortions (20–25%), retardation of fetal growth, and premature delivery (almost 100% in cyanotic mothers). Based on an extensive review of the literature, we discuss the specific risks in pregnancy depending on the hemodynamic situations produced by different heart defects. We also discuss the risks and advantages of different regimens for anticoagulation.

Counselling concerning contraception is frequently inadequate. The most important problems are thromboembolic complications with the use of hormonal contraception, and hyper- and dysmenorrhea in those using intrauterine devices. Finally, the genetic risks must be considered, differentiating between single gene defects and the sex of the parents suffering from congenital heart diseases.

The need for population-based studies of adults with CHD has motivated the growing use of secondary analyses of administrative health data in a variety of jurisdictions worldwide. We aimed at systematically reviewing all studies using administrative health data sources for adult CHD research from 2006 to 2016. Using PubMed and Embase (1 January, 2006 to 1 January, 2016), we identified 2217 abstracts, from which 59 studies were included in this review. These comprised 12 different data sources from six countries. Of these, 55% originated in the United States of America, 28% in Canada, and 17% in Europe and Asia. No study was published before 2007, after which the number of publications grew exponentially. In all, 41% of the studies were cross-sectional and 25% were retrospective cohort studies with a wide variation in the availability of patient-level compared with hospitalisation-level episodes of care; 58% of studies from eight different data sources linked administrative data at a patient level; and 37% of studies reported validation procedures. Assessing resource utilisation and temporal trends of relevant epidemiological and outcome end points were the most reported objectives. The median impact factor of publication journals was 4.04, with an interquartile range of 3.15, 7.44. Although not designed for research purposes, administrative health databases have become powerful data sources for studying adult CHD populations because of their large sample sizes, comprehensive records, and long observation periods, providing a useful tool to further develop quality of care improvement programmes. Data linkage with electronic records will become important in obtaining more granular life-long adult CHD data. The health services nature of the data optimises the impact on policy and public health.

We report the very unusual perforation of the mitral valve in the setting of Sweet's syndrome, or acute febrile neutrophilic dermatosis, in a boy aged 5 years. Surgical repair was uneventful, and follow-up showed no residual anomalies. Acute or delayed valvitis, with damage to either the mitral or aortic valves, should be screened for in this rare disease.

Introduction: An association between mitral valvar prolapse and low body mass index has been proposed. The goal of this study was to evaluate this suggested association using two independent and large databases. For comparison, we evaluated the association, if any, between bicuspid aortic valve and low body mass index. Methods: We retrospectively analyzed, using uni- and multivariate analysis, 1742 echocardiograms that were performed as a part of a cardiac screening of teenage athletes and 24,265 echocardiograms performed between 1984 and 1998 for various clinical indications. Results: The first database included a total of 12,926 (53%) female and 11,339 (47%) male patients. The second database included a total of 1172 male (67%) and 570 female (33%) high school athletes. Mitral valvar prolapse was independently associated with low body mass index in addition to mitral regurgitation in both data bases. An index less than 30 occurred in 78 of 13,874 (0.6%) as opposed to 7 of 3236 (0.2%) in the echo data base, p equal to 0.03, odds ratio: 2.4 confidence intervals: 1.1–5.2, and an index less than 20 occurred in 7 of 354 (2%) as opposed to 6 of 944 (0.6%) in the athletic data base, p equal to 0.03, odds ratio: 3.2 confidence intervals: 1.05–9.5. The finding of a bicuspid aortic valve did not have any association with low body mass index. Conclusion: In our two independent databases, mitral valvar prolapse was independently associated with low body mass index in addition to mitral and tricuspid regurgitaton. The cause of this association remains unknown.

This study aimed at examining three tilt angle-based positive responses and the time to positive response in a head-up tilt test for children with orthostatic intolerance, and the psychological fear experienced at the three angles during head-up tilt test. A total of 174 children, including 76 boys and 98 girls, aged from 4 to 18 years old (mean 11.3±2.8 years old), with unexplained syncope, were randomly divided into three groups, to undergo head-up tilt test at the angles of 60°, 70° and 80°, respectively. The diagnostic rates and times were analysed, and Wong–Baker face pain rating scale was used to access the children’s psychological fear. There were no significant differences in diagnostic rates of postural orthostatic tachycardia syndrome and vasovagal syncope at different tilt angles during the head-up tilt test (p>0.05). There was a significant difference, however, in the psychological fear at different tilt angles utilising the Kruskal–Wallis test (χ2=36.398, p<0.01). It was mildest at tilt angle 60° utilising the Kolmogorov–Smirnov test (p<0.01). A positive rank correlation was found between the psychological fear and the degree of tilt angle (rs=0.445, p<0.01). Positive response appearance time was 15.1±14.0 minutes at 60° for vasovagal syncope children. There was no significant difference in the time to positive response, at different tilt angles during the head-up tilt test for vasovagal syncope or for postural orthostatic tachycardia syndrome. Hence, it is suggested that a tilt angle of 60° and head-up tilt test time of 45 minutes should be suitable for children with vasovagal syncope.

Among populations of patients with the congenital heart disease, there is considerable diversity in the anatomy of the coronary arteries. Understanding these anatomical differences is vitally important in directing interventions and surgical repair. In this report, the authors describe the echocardiographic evaluation of the variants of coronary artery anatomy in the following lesions: transposition of the great arteries, congenitally corrected transposition of the great arteries, double-inlet left ventricle, common arterial trunk, tetralogy of Fallot, and double-outlet right ventricle.

The aim of the study was to determine the optimal size and technique for construction of the systemic-to-pulmonary arterial shunt which will provide suitable pulmonary blood flow in first-stage Norwood palliation for hypoplastic left heart syndrome in neonates. Our clinical experience suggested that an arterial oxygen tension of about 30 mm Hg immediately after cardiopulmonary bypass, with the patients being ventilated at the lowest possible mean airway pressure with an FiO2 of 1.0, provided a suitable pulmonary-to-systemic flow ratio. We also aimed to clarify the characteristics of pulmonary blood flow in accordance with the size of the shunt and the change in the pulmonary vascular resistance in a simplified rigid model of the Norwood procedure. A hole of2.0 mm diameter proved adequate to provide a suitable pulmonary blood flow of 200−300 mlx002F;min in the presence of a pressure gradient of 20−40 mm Hg between the systemic and pulmonary circulations in neonates weighing 3 kg. A short central shunt with a prosthesis of4 mm in diameter produced an excessive flow of pulmonary blood. Our data suggest that using a smaller shunt than that commonly used is necessary to decrease the early and intermediate postoperative mortality. A prosthesis of 3.0 or 3.5 mm in diameter arising from the brachiocephalic artery would be acceptable and can be recommended for first-stage Norwood palliation in small infants, especially in view of the operative difficulties encountered in taking down the shunt at the time of subsequent operations.