Membranous ventricular septal aneurysm is a known entity but rarely causes severe right ventricular outflow obstruction. We report a 40-year-old female with trisomy 18 who developed severe right ventricular outflow obstruction caused by an enormous membranous septal aneurysm associated with unrepaired inlet ventricular septal defect with perimembranous extension.

Ebstein anomaly is a rare CHD known for its wide spectrum of presentation with the age of diagnosis dependent on the malformation’s severity. Here, the authors describe a case of delayed diagnosis of Ebstein anomaly, secondary to lack of medical attention, which resulted in severe tricuspid regurgitation and pulmonary hypertension. Furthermore, the case was complicated by a unique pulmonary venous abnormality.

Coronary artery fistulas have been described in patients after heart transplantation more often than in the normal population. We reviewed our centre’s database between 2008 and 2020. Thirty patients had coronary angiography and 13 showed non-cameral coronary artery fistulas in their first coronary angiography. Distribution, degree and evolution of the fistulas and characteristics of transplant procedure, patient and immunosuppressive treatments were analysed.

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.

The portrayal of a beautiful youngster performing uninhibited acrobatics based on Fontan physiology, as presented by Marshall Jacobs, is a brilliant and beautiful thing for us to see. It is, perhaps, all about will over physiology. But it is, nevertheless, happening for that child. Marshall mentioned the need for re-transplantation, whether the beginning strategy was transplantation or reconstructive surgery. Indeed, a relatively small percentage of children transplanted will require re-transplantation because of severe graft coronary disease. Remarkably, in the Loma Linda experience, 10-year actuarial survival for 26 patients following elective re-transplantation is over 85%, exceeding overall actuarial survival at 10 years for children following primary transplantation. Many of the transplanted infants, however, seem to be realistically hopeful that one heart will last their entire lifetime. Of course, the hope is that their's will be a long and healthy lifetime. The requirement for late transplantation following Fontan procedures, however, seems almost inevitable. We'll simply have to keep these children with Fontan circulation under surveillance to see when, in the course of their lives, transplantation will become necessary. Unfortunately, operative and long-term survival among children who are transplanted for failing Fontan physiology have, as yet, been somewhat suboptimal.

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.

This study investigated the incidence and risk factors of perioperative clinical seizure and epilepsy in children after operation for CHD. We included 777 consecutive children who underwent operation from January 2013 to December 2016 at Kanagawa Children’s Medical Center, Kanagawa, Japan. Perinatal, perioperative, and follow-up medical data were collected. Elastic net regression and mediation analysis were performed to investigate risk factors of perioperative clinical seizure and epilepsy. Anatomic CHD classification was performed based on the preoperative echocardiograms; cardiac surgery was evaluated using Risk Adjustment in Congenital Heart Surgery 1. Twenty-three (3.0%) and 15 (1.9%) patients experienced perioperative clinical seizure and epilepsy, respectively. Partial regression coefficient with epilepsy as the objective variable for anatomical CHD classification, Risk Adjustment in Congenital Heart Surgery 1, and the number of surgeries was 0.367, 0.014, and 0.142, respectively. The proportion of indirect effects on epilepsy via perioperative clinical seizure was 22.0, 21.0, and 33.0%, respectively. The 15 patients with epilepsy included eight cases with cerebral infarction, two cases with cerebral haemorrhage, and three cases with hypoxic-ischaemic encephalopathy; white matter integrity was not found. Anatomical complexity of CHD, high-risk cardiac surgery, and multiple cardiac surgeries were identified as potential risk factors for developing epilepsy, with a low rate of indirect involvement via perioperative clinical seizure and a high rate of direct involvement independently of perioperative clinical seizure. Unlike white matter integrity, stroke and hypoxic-ischaemic encephalopathy were identified as potential factors for developing epilepsy.

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.

This study reviewed the short-term outcome of transcatheter closure of the defects within the oval fossa using an Amplatzer® Septal Occluder. From January 1997 to December 2000, 210 patients with defects within the oval fossa underwent successful transcatheter closure. We reviewed a total of 190 patients with left-to-right shunts, assessing the patients for possible complications and the presence of residual shunts using transthoracic echocardiogram at 24 h, 1 month, 3 months and one year. Their median age was 10 years, with a range from 2 to 64 years, and their median weight was 23.9 kg, with a range from 8.9 to 79 kg. In 5 patients, a patent arterial duct was closed, and in 2 pulmonary balloon valvoplasty performed, at the same sitting. The median size of the Amplatzer® device used was 20 mm, with a range from 9 to 36 mm. The median times for the procedure and fluoroscopy were 95 min, with a range from 30 to 210 min, and 18.4 min, with a range from 5 to 144 min, respectively. Mean follow-up was 20.8 ± 12.4 months. Complete occlusion was obtained in 168 of 190 (88%) patients at 24 h, 128 of 133 (96.2%) at 3 months, and 103 of 104 (99%) at one year. Complications occurred in 4 (2.1%) patients. In one, the device became detached, in the second the device embolized into the right ventricular outflow tract, the lower end of the device straddled in the third, and the final patient had significant bleeding from the site of venupuncture. There were no major complications noted on follow-up. We conclude that transcatheter closure of defects within the oval fossa using the Amplatzer® Septal Occluder is safe and effective. Long-term follow-up is required, nonetheless, before it is recommended as a standard procedure.

Inverted left atrial appendage is an unusual complication associated with congenital cardiac surgery. Inversion of the left atrial appendage may occur during the surgical procedure or afterwards. The left atrial appendage may invert iatrogenically or as a result of the negative pressure during placement or removal of the left atrial vent or during deairing manoeuvres. This event can be life-threatening because of the mass effect of the atrial appendage within the left atrial cavity.

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.