Objectives: To detail the US multi-institutional experience with the Occlutech(c) (Occlutech International AB, Helsingborg, Sweden) atrial flow regulator (AFR) in children and adults with acquired or congenital heart disease. Background: The creation of a long-term atrial communication is desirable in several cardiovascular disease phenotypes, most notably pulmonary arterial hypertension, disorders of increased left ventricular filling and increased cavopulmonary pressures in patients with a Fontan type circulation. Methods: Patients were identified for inclusion from the AFR device manufacturer database. Data was collected using a RedCap database following IRB approval. 8 weeks of follow up data was sought for each patient based on available data. Data was analyzed and summarized using SPSS. Results: We report the experience of 6 US centers in the implantation of AFR devices in 15 patients, across a wide age range, with different disease phenotypes and a variety of indications. Implantation was technically successful in all patients and improvement was noted in both clinical and hemodynamic parameters. There were no immediate or intermediate term complications reported. 3 patients died remote from implantation. Their deaths were not felt to be related to the AFR device or related procedural complications. Conclusion: Compassionate use of the AFR device in children and adults with congenital & acquired heart disease is technically feasible and produces beneficial short term hemodynamic and symptomatic improvement. Widespread uptake of this technique and treatment at specialist centers has the potential to provide significant benefits to a variety of complex patients with currently limited treatment options and indeterminate prognosis.

Background: Balloon aortic valvuloplasty and open surgical valvotomy are procedures to treat neonatal aortic stenosis, and there is controversy as to which method has superior outcomes. Methods: We reviewed the records of patients at our institution since 2000 who had a balloon aortic valvuloplasty or surgical valvotomy via an open commissurotomy prior to 2 months of age. Results: Forty patients had balloon aortic valvuloplasty and 15 patients had surgical valvotomy via an open commissurotomy. There was no difference in post-procedure mean gradient by transthoracic echocardiogram, which were 25.8 mmHg for balloon aortic valvuloplasty and 26.2 mmHg for surgical valvotomy, p = 0.87. Post-procedure, 15% of balloon aortic valvuloplasty patients had moderate aortic insufficiency and 2.5% of patients had severe aortic insufficiency, while no surgical valvotomy patients had moderate or severe aortic insufficiency. The average number of post-procedure hospital days was 14.2 for balloon aortic valvuloplasty and 19.8 for surgical valvotomy (p = 0.52). Freedom from re-intervention was 69% for balloon aortic valvuloplasty and 67% for surgical valvotomy at 1 year, and 43% for balloon aortic valvuloplasty and 67% for surgical valvotomy at 5 years (p = 0.60). Conclusions: Balloon aortic valvuloplasty and surgical valvotomy provide similar short-term reduction in valve gradient. Balloon aortic valvuloplasty has a slightly shorter but not statistically significant hospital stay. Freedom from re-intervention is similar at 1 year. At 5 years, it is slightly higher in surgical valvotomy, though not statistically different. Balloon aortic valvuloplasty had a higher incidence of significant aortic insufficiency. Long-term comparisons cannot be made given the lack of long-term follow-up with surgical valvotomy.

Objective To compare the incidence of post-PDA ligation syndrome after surgical vs. percutaneous closure of PDAs in very low birth weight (VLBW) infants. Study design Cohort study comparing 59 infants who underwent surgical ligation with 25 infants who underwent percutaneous closure. Comparisons between groups were made using two-sample t-tests and a Fisher's exact test. Comparisons of pre vs. post variables within each group were made using paired t-tests. Results Patients who underwent surgical ligation had a higher need for initiation of post-procedure hemodynamic support (12/59 vs. 0/24, p = 0.016), a higher post-procedure peak FiO(2) (0.64 vs. 0.43, p = 0.004), and a larger absolute change in peak FiO(2) (0.23 vs. 0.09, p = 0.008). Conclusion VLBW infants who had percutaneous closure of their PDA did not experience post ligation syndrome and had less escalation of respiratory support compared with infants who underwent surgical ligation.

Williams syndrome is a rare genetic disease that affects elastin production, leading to medium and large vessel stenoses and other abnormalities. Cardiac manifestations of Williams syndrome are the most life-threatening, occurring in 80% of children. Children with Williams syndrome are known to be at risk for sudden cardiac death. These tragic events are often precipitated by diagnostic or therapeutic procedures requiring anaesthesia or sedation, such as cardiac catheterisation. We present the case of a 3-month-old infant with Williams syndrome who suffered sudden cardiac arrest during cardiac catheterisation and subsequent arrest approximately 48 hours after the procedure. We also review the current literature focused on children with Williams syndrome who have suffered sudden cardiac arrest during or after cardiac catheterisation procedures.

Patent ductus arteriosus is the most common cardiovascular abnormality in premature infants. With newly available percutaneous devices, centres are reporting high rates of success and favourable safety profiles with percutaneous closure of haemodynamically significant ductus arteriosi in infants under 1000 g. We report the case of a 5-week-old, previous 25-week gestation, 1200-g infant who underwent successful percutaneous closure of a ductus arteriosus with a Medtronic Microvascular Plug but who developed late-term coarctation from the device. This case should prompt practitioners to consider the need and timing of follow-up echocardiograms in this population and sheds light on a newly reported long-term complication of device closure in premature infants.