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.

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.

Background: Creation of an extracardiac cavopulmonary connection has been proposed as a superior alternative to the lateral intracardiac tunnel for the completion of total cavopulmonary connection. Methods and results: We made a retrospective review of our experience with 125 patients undergoing a total cavopulmonary connection between June 1994 and January 2003. Our experience with the extracardiac connection for completion began in 1999. Since 1994, we have constructed an intracardiac tunnel in 50 patients, and an extracardiac connection in 75. Of the total number, 83 had undergone an earlier partial cavopulmonary connection. Additional intracardiac procedures were performed in 43 patients at time of completion, in 25 of those undergoing extracardiac completion, and in 18 of the patients having an intracardiac procedure. The mean size of the tube used for completion was 19 mm. The mean cross-clamp time for placement of the intracardiac tunnel was 77 min, with a median of 80.5 min, and a mean cardiopulmonary bypass time of 139 min, with a median of 131 min. For construction of the extracardiac connection, a mean cross-clamp time in 24 of the 75 patients was 54 min, with a median of 54 min. Mean cardiopulmonary bypass time for all the patients with an extracardiac connection was 100 min, with a median of 88 min. Reoperations were needed in 10 patients, 6 having intracardiac and 4 extracardiac procedures. Of these, 5 were early and 5 late, including one take down. None of the patients died after these interventions. Taken overall, 8 patients died, with 5 early deaths. In the multivariable analysis, cardiopulmonary bypass time of more than 120 min, atrioventricular valvar replacement, and banding of the pulmonary trunk prior to the total cavopulmonary connection, all reached statistical significance for early death, whereas only heterotaxy syndrome remained as the sole risk factor for late death. There was no significant difference in survival between the modifications used. Discussion: Whereas we could not identify any clinical superiority for the extracardiac approach in the short-term, the concept of extracardiac completion has helped to simplify the overall procedure. Longer follow-up will be required to elucidate any potential advantages.

We report three cases of an abnormal finding of duplicated left pulmonary artery: two of these occurring in children with Kabuki syndrome and configuring the setting of a pseudo-pulmonary sling without any clinical or cardiac cross-sectional evidence of tracheal compression. The other case instead represents duplicated left pulmonary artery with pulmonary sling caused by the retro-tracheal course of the lower left pulmonary artery associated with “Christmas Tree” arrangement of the tracheo-bronchial system.

In both patients with pseudo-pulmonary sling and Kabuki syndrome, the abnormal finding was incidental during echocardiographic examination and neither of the patients required surgical repair for the condition. To the best of our knowledge, they represent the third and fourth cases in which such an anomaly of the pulmonary artery branches not forming a sling is seen in association with Kabuki syndrome. Another case represents our second experience and the second case reported in literature with duplicated left pulmonary artery in the setting of a complex tracheal anatomy. In this symptomatic patient, surgical repair of atrial septal defect and relief of the vascular ring were indicated, and the surgical repair was performed successfully at the age of 3 years.

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.

Primary care cardiology, also known as ambulatory or outpatient cardiology, for the longitudinal management of patients with common arterial trunk or with transposition of the great arteries is both poorly described and has limited evidence to justify its basis. This article discusses some of the various complications that these patients can develop, reviews the medical literature, and describes a framework for care of these complex patients from infancy to transition to adult congenital cardiac specialist care.

An absent ductus venosus is a rare anomaly which results aberrant umbilical venous return. The fetus which is defined here referred to our clinic at 30th gestational week because of cardiomegaly. The diagnosis of ductus venosus agenesis and anomalous umbilical venous return was done by fetal echocardiography. The fetus has two unique features. The drainage of anomalous umbilical vein into the superior vena cava and associated persistent truncus arteriosus have not been reported yet in fetuses with absent ductus venosus.

Postural tachycardia syndrome is defined by a heart rate increment of 40 beats/minute (bpm) (or a heart rate that exceeds 125 bpm) within 10 minutes of change from the supine position to an upright position in the absence of obvious orthostatic hypotension. There are multiple pathophysiological mechanisms that underlie postural tachycardia syndrome, including peripheral denervation, β-receptor supersensitivity, hypovolaemia, and impaired muscle pump. Some children afflicted with postural orthostatic tachycardia syndrome and hypovolaemic dysregulation have been found to have perturbed renin–angiotensin–aldosterone profile, disturbed vascular endothelial function, and abnormal vasodilation. The hyperadrenergic state in some postural tachycardia syndrome patients is likely a driver for orthostatic tachycardia. Other mechanisms include the presence of treatable autonomic neuropathies. An understanding of these pathophysiological mechanisms might be helpful for the effective treatment of postural tachycardia syndrome.

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.

Different treatment options for pulmonary hypertension have emerged in recent years, and evidence-based management strategies have improved quality of life and survival in adults. In children with pulmonary vascular disease, therapeutic algorithms are not so clearly defined; this study determined current treatment initiation in children with pulmonary hypertension in participating centres of a registry.

Through the multinational Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry, patient demographics, diagnosis, and treatment as judged and executed by the local physician were collected. Inclusion criteria were >3 months and <18 years of age and diagnostic cardiac catheterisation consistent with pulmonary hypertension (mean pulmonary arterial pressure ⩾25 mmHg, pulmonary vascular resistance index ⩾3 Wood units×m2, and mean pulmonary capillary wedge pressure ⩽12 mmHg).

At diagnostic catheterisation, 217/244 patients (88.9%) were treatment naïve for pulmonary hypertension-targeted therapy. Targeted therapy was initiated after catheterisation in 170 (78.3%) treatment-naïve patients. A total of 19 patients received supportive therapy, 28 patients were not started on therapy, and 26 patients (10.7%) were on targeted treatment before catheterisation. Among treatment-naïve subjects, treatment was initiated with one targeted drug (n=112, 51.6%), dual therapy (n=39, 18%) or triple-therapy (n=5, 2.3%), and calcium channel blockers with one targeted medication in one patient (0.5%). Phosphodiesterase inhibitors type 5 were used frequently; some patients with pulmonary hypertension related to lung disease received targeted therapy.

There is a diverse therapeutic approach for children with pulmonary hypertension with a need of better-defined treatment algorithms based on paediatric consensus for different aetiologies including the best possible diagnostic workup.

This study describes single-unit experience and explores risk stratification, with protocolised inter-stage cardiac magnetic resonance imaging for infants with hypoplastic left heart syndrome. Survival was retrospectively analysed among the cohort of locally followed survivors of Norwood Stage I procedure, who underwent magnetic resonance imaging under general anaesthesia from 2003 to 2008. This included 32 patients: 17 with Sano conduit and 15 with arterio-pulmonary shunt. The median (inter-quartile range) age and weight at scan were 3.1 (2.6–4.6) months and 5.0 (4.5–5.3) kilograms, respectively. Using morphologic definitions, the median coarctation index was 0.71 (0.57–0.83). The degree of proximal right and left pulmonary artery narrowing was 25% (14–44%) and 25% (11–50%), respectively. The median right ventricular ejection fraction was 54% (48–59%). The ejection fraction was not related to the coarctation index or to pulmonary artery narrowing. Patients were followed up for a median of 19.2 (10.8–46.0) months, during which 13 (41%) had an intervention in addition to routine Norwood Stage II surgery and seven died. Risk of death was related to reduced right ventricular ejection fraction, with a hazard ratio of 0.91 (95% confidence interval 0.85–0.98, p = 0.02), and the cumulative number of focal stenoses of neo-aortic arch and pulmonary arteries (hazard ratio 2.71, 95% confidence interval 1.14–6.44, p = 0.02). Conclusions: In addition to comprehensive three-dimensional morphologic imaging, inter-stage cardiac magnetic resonance imaging provides a ventricular functional index that may predict outcome in patients with hypoplastic left heart syndrome. Measures to preserve right ventricular systolic function and relieve stenoses are paramount within the complex management strategies for these patients.

Despite tremendous advances in surgical treatment of tetralogy of Fallot, augmenting the small right ventricular outflow tract remains a challenge. Transannular patch augmentation revolutionised surgical management, but did so at the expense of rendering patients with pulmonary insufficiency and the resulting problems associated therewith. Recent surgical efforts have focused on pulmonary valve preservation at initial correction and pulmonary valve restoration after transannular patching, with favourable results. In this manuscript, we review methods of pulmonary valve preservation and restoration.