Tall stature is a common feature of both Marfan syndrome and XYY syndrome. Differential diagnosis between these entities has important prognostic implications. We report the case of a 21-year-old young man with a previously known diagnosis of XYY syndrome, in whom the identification of a fibrilin-1 mutation was determinant to establish an appropriate diagnosis, medical follow-up, and genetic counselling.

Gastrointestinal bleeding complicated with protein-losing enteropathy after the Fontan procedure has been often reported in recent years, but there is no established therapy for it.

We report the case of an 18-year-old boy who suffered from abdominal pain, melena, and anaemia due to intractable haemorrhagic protein-losing enteropathy after the Fontan procedure. He was successfully treated with octreotide therapy.

Branch pulmonary artery valve implantation has been reported in larger patients with dysfunctional right ventricular outflow tracts via routine femoral access. Here, we report treatment of severe pulmonary insufficiency with bilateral branch pulmonary artery Melody valve implantation (Medtronic, Minneapolis, Minnesota). To the best of our knowledge, this is the first report of bilateral valve implantation utilising the hybrid approach in a small-size patient.

Objectives: This study was designed to identify predictors of residual defects following deployment of the Amplatzer device to close septal defects within the oval fossa. Methods: Between February 1997 and February 2000, we used the Amplatzer device to close defects in the oval fossa in 89 patients. Of these patients, 18 (20%) had residual defects. At 6 or 12 months following placement of the device, 13 defects (14.6%) had persisted. We evaluated several variables derived from clinical features, transesophageal echocardiography and catheterization to establish predictors for residual shunting. Results: Multivariate analysis identified a shorter superior rim of less than 8 mm (Odds ratio = 10.1; 95% confidence intervals = 2.64–38.72; p = 0.001), and a smaller interatrial septum in the 30-degree transesophageal echocardiographic plane of less than 30 mm (Odds ratio = 5.5; 95% confidence intervals = 1.17–26.14; p = 0.03) as independent predictors of residual defects. When the analysis was repeated defining only those 13 patients with persisting residual defects at 6 or 12 months as failures, a short superior rim (p = 0.004) remained a predictor for residual shunting. Conclusions: Defects with a short superior rim and smaller interatrial septum in the 30-degree transesophageal echocardiographic plane independently and additively predict an increased probability of residual shunting following closure of defects in the oval fossa using the Amplatzer device.

A 4-year-old boy presented to our institution with pancytopenia, consumptive coagulopathy, hepatosplenomegaly and recurrent complex pericardial effusion secondary to kaposiform lymphagiomatosis. Due to extensive loculation, conventional drainage was minimally effective. As an adjunct to medical therapy, the Indigo™ aspiration system was used to remove thrombus within the pericardial space. Our patient had good medium-term results with complete resolution of his pericardial effusion at 4 months.

Anomalous circumflex coronary artery origin from pulmonary artery is a very rare congenital heart disease. Misdiagnosis of this condition may lead to inadequate myocardial protection during cardiopulmonary bypass surgery.

The large outbreak of coronavirus disease 2019 (COVID-19) is spreading all over the world rapidly. There have recently been publications in the literature regarding the relationship between COVID-19 and Kawasaki disease, but there is no sufficient knowledge about the treatment and follow-up.

Plasma leakage in polytetrafluoroethylene grafts arises through complex interactions between the prosthetic physical/chemical microstructure and patient environment. While many of the precipitating factors can be avoided by careful graft-handling and meticulous surgical technique, this unpredictable complication still contributes significant morbidity to shunt procedures. Protein sealing of the graft offers a possible solution to the problem.

Background: Little is known regarding parental preference for a one-stage complete repair versus a two-staged approach with initial palliation, followed by repair, of the congenital cardiac malformation. Methods: We interviewed 103 parents of healthy children referred to a clinic for pediatric cardiology. Participants were presented with a hypothetical scenario in which their children had a cardiac lesion requiring surgery. The surgery could be performed either by means of one-stage complete repair, or using a two-stage approach, with palliation first followed by complete repair a year later. The mortality rate for the one-stage repair was set at 5%. Participants were asked to choose between the one- and two-stage approaches, with differing mortality rates for the two-stage approach. The scenarios included options when the two-stage combined mortality rate was lower than the one-stage mortality, and the first stage mortality rate was at 1% and 3%, and when the two-stage combined mortality rate was the same as that for one-stage mortality, these being set at 1% and 3%. Results: When the two-stage combined mortality rate was lower than that of the one-stage repair, participants were more likely to choose the two-stage approach if the first stage mortality rate was 1% as compared to 3% (57% and 44%, respectively, p = 0.04). When the two-stage combined mortality rate was the same as the one-stage approach, participants choosing the two-stage approach when the mortality rate was set at 1%, and when it was raised to 3%, were not significantly different (42% and 34%, respectively, p = 0.24). When the combined two-stage mortality was the same as that set for one-stage repair, participants with no insurance were less likely to choose the two-stage approach than those covered by insurance (p = 0.03). Conclusions: In the chosen scenarios, when the mortality for a two-stage combined approach is the same as that for one-staged repair, more parents choose the one-staged repair. If the two-stage combined mortality is lower than that for one-staged repair, parents are more likely to choose the two-stage repair if the mortality for the first stage is lower. When the mortality rates for the one-stage and two-stage approaches are the same, people without insurance are more likely to choose one-staged repair.

Haemoptysis may occur in patients with tetralogy of Fallot and major aorto-pulmonary collateral arteries. We describe such a patient in whom bleeding from a major aorto-pulmonary collateral artery produced severe pulmonary haemorrhage. Interventional closure of the artery could not be performed because it perfused the native pulmonary arteries. Instead, we inserted a conduit between the right ventricle and the native pulmonary arteries, followed by percutaneous closure of the collateral artery. Our patient demonstrates the increasing necessity for combined surgical and interventional procedures.

This case report presents an infant patient with the association of trisomy 7p and tetralogy of fallot (TEF). Patients diagnosed with trisomy 7p should certainly be scheduled for an echocardiographic exam and be scanned for any CHDs that may accompany it. The CHDs that most frequently accompany this syndrome include atrial septal defect, ventricular septal defect, and patent ductus arteriosis. Yet, it should be known that TEF may also be present, albeit rarely.