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.

Fontan completion in patients with complex cardiac anatomy, and specifically heterotaxy syndrome, can present unique physiologic considerations. For example, existing venous connections may be "unmasked" after a cavopulmonary anastomosis operation. We present the case of a child with heterotaxy, dextrocardia, single-ventricle physiology, and anomalous hepatic venous drainage that resulted in profound shunting and cyanosis. We addressed the problem utilizing a novel strategy with a "fenestrated" covered stent.

Background: Traditional palliation for biventricular cyanotic congenital heart lesions often involves staging with systemic-to-pulmonary arterial shunts to secure pulmonary blood flow (PBF) in the newborn period prior to complete repair. However, shunts may lead to life-threatening events secondary to shunt occlusion or acute coronary steal. They may be associated with morbidity secondary to diastolic runoff, systemic steal and volume loading, and do not provide pulsatile flow which has the potential to promote pulmonary artery (PA) growth. We have alternatively performed modified right ventricular outflow (mRVO) procedures by establishing antegrade right ventricle-to-PA flow. Methods: Retrospective review of data on all patients who underwent the mRVO procedure from 2013 to 2016, including anatomy, number of interstage catheterizations, reoperations, intensive care unit admissions, hypercyanotic episodes, interval to complete repair, and mortality. Results: Seventeen nonconsecutive patients included tetralogy of Fallot (n = 14), pulmonary valve stenosis (n = 2), and 1 with pulmonary atresia-intact septum; 14 had significant branch PA stenosis. Median age of first mRVO procedure was 14 days (range 5-193), and median duration of follow-up was 15.3 months (range 4-47 months). No patients had post-palliation acute hypercyanotic episodes. Nine were admitted to the ICU for persistent interstage hypoxemia, 7 of whom required reintervention prior to complete repair, which was achieved in 11 patients. Two late deaths unrelated to mRVO occurred. Conclusions: The mRVO procedure is a potential option with satisfactory results. It avoids potential shunt-related sudden death. The physiology of the mRVO palliation may provide unique benefits by providing antegrade pulsatile PBF, facilitates catheter interventions, and avoids branch PA distortion and stenosis.

Systemic-to-pulmonary artery collateral networks commonly develop in patients with single-ventricle physiology and chronic hypoxemia. Although these networks augment pulmonary blood flow, much of the flow is ineffective and contributes to cardiac volume loading. This volume loading can have detrimental effects, especially for single-ventricle patients. Some data suggest that occluding collaterals may improve outcomes after subsequent operations, especially when the volume of collateral flow is significant. Traditional practice has been to coil occlude the feeding vessel. We perform particle embolization of these collateral networks for two primary reasons. First, access to the feeding vessel is not blocked as collaterals may redevelop. Second, particles occlude the most distal connections. Thus, embolization with particles should be considered as an alternative to coil occluding the proximal feeding vessel.

ObjectivesThe study purpose is to evaluate the safety and efficacy of the ADO II device for closure of patent ductus arteriosus (PDA) in children.BackgroundTranscatheter treatment of PDA has been evolving for 40+ years and is the treatment of choice. The AMPLATZER™ Duct Occluder (ADO) device was developed for larger diameter ducts and is not ideal in all PDAs. ADO II was developed for small to moderate‐sized ducts.MethodsThis is a single‐arm, multicenter study evaluating safety and efficacy of the ADO II device. Patients <18 years were screened for a PDA ≤5.5 mm in diameter and 3‐12 mm in length. Right and left heart catheterization was performed, and hemodynamic data were obtained at the time of implant. The diameter of the left pulmonary artery (LPA) and descending aorta, and the presence of any pre‐existing pressure gradients across the LPA or aortic arch were assessed at baseline and 6 months post‐implant.ResultsA total of 192 patients were enrolled. The median implant time was 74 min. Median fluoroscopy time was 12 min. A retrograde (aortic) approach was used in 33% of procedures and demonstrated a statistically significant reduction in fluoroscopy time (P value = 0.0018) compared to an antegrade approach. The device was successfully implanted in 93% of patients, with complete closure in 98% of successful implantations.ConclusionsIn this prospective study, the ADO II was safe and effective for closure of small to moderate PDAs. Implantation is simple and the ability for retrograde aortic delivery reduces procedure‐related radiation exposure. © 2017 Wiley Periodicals, Inc.

While trisomy 21 is a common genetic disorder in singletons, the incidence among identical twins is very rare, occurring in approximately 1-2 per 1000 twin gestations. Trisomy 21 is associated with high incidence of congenital heart defects, and commonly occurs with ventricular septal defects (VSDs). Physiologic burden of VSDs depends on prevalence of anatomic and other circulatory factors. A case of identical twins with trisomy 21 and large VSDs is described in the present report. Though genetically identical, phenotypes varied significantly. One twin was managed medically, while the other developed more significant heart failure, requiring operative repair.

Myhre syndrome is a rare, distinctive syndrome due to specific gain‐of‐function mutations in SMAD4. The characteristic phenotype includes short stature, dysmorphic facial features, hearing loss, laryngotracheal anomalies, arthropathy, radiographic defects, intellectual disability, and a more recently appreciated spectrum of cardiovascular defects with a striking fibroproliferative response to surgical intervention. We report four newly described patients with typical features of Myhre syndrome who had (i) a mildly narrow descending aorta and restrictive cardiomyopathy; (ii) recurrent pericardial and pleural effusions; (iii) a large persistent ductus arteriosus with juxtaductal aortic coarctation; and (iv) restrictive pericardial disease requiring pericardiectomy. Additional information is provided about a fifth previously reported patient with fatal pericardial disease. A literature review of the cardiovascular features of Myhre syndrome was performed on 54 total patients, all with a SMAD4 mutation. Seventy percent had a cardiovascular abnormality including congenital heart defects (63%), pericardial disease (17%), restrictive cardiomyopathy (9%), and systemic hypertension (15%). Pericarditis and restrictive cardiomyopathy are associated with high mortality (three patients each among 10 deaths); one patient with restrictive cardiomyopathy also had epicarditis. Cardiomyopathy and pericardial abnormalities distinguish Myhre syndrome from other disorders caused by mutations in the TGF‐β signaling cascade (Marfan, Loeys–Dietz, or Shprintzen–Goldberg syndromes). We hypothesize that the expanded spectrum of cardiovascular abnormalities relates to the ability of the SMAD4 protein to integrate diverse signaling pathways, including canonical TGF‐β, BMP, and Activin signaling. The co‐occurrence of congenital and acquired phenotypes demonstrates that the gene product of SMAD4 is required for both developmental and postnatal cardiovascular homeostasis. © 2016 Wiley Periodicals, Inc.

Pulmonary vein stenosis (PVS) is often progressive and severe. Surgical and percutaneous angioplasty are acutely successful; however, restenosis is common and many patients require multiple reinterventions. We perform intraoperative “hybrid” stent placement to deliver larger, stronger stents. Hybrid stent placement is well described for pulmonary arterial stenosis (PAS). The PAS data demonstrate that smaller stents are associated with rapid in-stent restenosis. Data from PVS in adults demonstrate superior outcomes with larger stents. Hybrid stent placement requires a strong collaborative effort between congenital heart surgeons and interventional cardiologists.

Background. Management of systemic semilunar valve disease in growing, young patients is challenging. When replacement is necessary, use of a pulmonary autograft is sometimes not possible for anatomic, pathologic, or technical reasons or due to parental or patient preference. We employed a stentless, porcine, full-root bioprosthesis in this setting and report our outcomes.Methods. Over 9 years (2005 to 2013), 24 patients of mean age 13.1 years (range, 3 months to 20.3 years) underwent operation for mixed stenosis and insufficiency in 16 of 24 (67%), pure insufficiency in 7 of 24 (29%), and pure stenosis in 1 of 24 (4%). Twenty patients had previous interventions of repair or replacement, valvuloplasty, or multiple operations. Survival, follow-up echocardiographic findings, and outcomes were documented. All patients were maintained on daily aspirin.Results. There were no hospital deaths and no early or late deaths over a mean follow-up for 23 patients of 46.1 months (range, 14 months to 9.2 years). One patient moved abroad and was lost to follow-up. Echocardiographic follow-up (mean 34.0 months) demonstrated that no patient developed more than mild insufficiency or moderate stenosis. In total, 20 of 24 (83%) showed no insufficiency and 11 of 24 patients (46%) showed no stenosis. Near or complete normalization of left ventricular mass and dimension was demonstrated. There were no explants and no thromboembolic or bleeding events.Conclusions. When use of a pulmonary autograft is not an option, the porcine full-root bioprosthesis appears favorable for systemic semilunar valve replacement in the pediatric and young adult population. Of note, when prosthetic degeneration does occur, stenosis predominates rather than insufficiency. Longer term studies are warranted. (C) 2015 by The Society of Thoracic Surgeons

BACKGROUND:Results of surgical management of hypoplastic left heart syndrome (HLHS) and related anomalies are often compared to published benchmark data which reflect the use of a variety of surgical and hybrid protocols. We report encouraging results achieved in an emerging program, despite a learning curve at all care levels. Rather than relying on a single preferred protocol, surgical management was based on matching surgical strategy to individual patient factors.METHODS:From 2010 to 2014, a total of 47 consecutive patients with HLHS or related anomalies with ductal-dependent systemic circulation underwent initial surgical palliation, including 30 Norwood stage I, 8 hybrid stage I, and 9 salvage-to-Norwood procedures. True hybrid procedures entailed bilateral pulmonary artery banding and ductal stenting. In the salvage-to-Norwood strategy, ductal stenting was withheld in favor of continued prostaglandin infusion in anticipation of a deferred Norwood procedure. Cardiac comorbidities (obstructed pulmonary venous return, poor ventricular function, and atrioventricular valve regurgitation) and noncardiac comorbidities influenced the choice of treatment strategies and were analyzed as potential risk factors for extracorporeal membrane oxygenation (ECMO) support or in-hospital mortality.RESULTS:Overall hospital survival was 81% (Norwood 83.3%, hybrid 88%, "salvage" 67%; P = .4942). Extracorporeal membrane oxygenation support was used for eight (17%) patients with two survivors. For cases with obstructed pulmonary venous return (n = 10, 21%), management choices favored a hybrid or salvage strategy (P = .0026). Aortic atresia (n = 22, 47%) was treated by a Norwood or salvage-to-Norwood. No cardiac, noncardiac, or genetic comorbidities were identified as independent risk factors for ECMO or discharge mortality in a multivariable analysis.CONCLUSIONS:Our emerging program achieved outcomes that compare favorably to published benchmark data with respect to hospital survival. These results reflect rigorous interdisciplinary teamwork and a flexible approach to surgical palliation based on matching surgical strategy to patient factors. With major associated cardiac/noncardiac comorbidity and antegrade coronary flow, a true hybrid with ductal stenting was our preferred strategy. For high-risk situations such as aortic atresia with obstructed pulmonary venous return, the salvage hybrid-bridge-to-Norwood strategy may help achieve survival albeit with increased resource utilization.

Pulmonary vein stenosis (PVS) is often progressive and severe. Surgical and percutaneous angioplasty are acutely successful; however, restenosis is common and many patients require multiple reinterventions. We perform intraoperative "hybrid" stent placement to deliver larger, stronger stents. Hybrid stent placement is well described for pulmonary arterial stenosis (PAS). The PAS data demonstrate that smaller stents are associated with rapid in-stent restenosis. Data from PVS in adults demonstrate superior outcomes with larger stents. Hybrid stent placement requires a strong collaborative effort between congenital heart surgeons and interventional cardiologists.

Earlier attempts at percutaneous closure of perimembranous ventricular septal defects (Pm VSDs) were abandoned because of incidence of heart block likely as a result of device rigidity and/or oversizing. This is retrospective review and data reporting of patients who underwent percutaneous closure using the softer second-generation Amplatzer vascular occluders; namely the Amplatzer vascular plug, second generation, (AVP II) and the Amplatzer duct occluder, second generation (ADO II) in our institution. A total of 20 patients were identified; AVP II was used in 9 patients and ADO II in 11 patients. Median weight was 13.45 kg (range 6.5 to 76); age 28.5 months (range 11 to 352). After procedure, 4 were noted to have aortic insufficiency; trivial in 3 and mild in 1 (unrelated to the device). Mild tricuspid regurgitation possibly device or procedure related was seen in 4. Residual flow through the device was common after procedure and disappeared in all but 3, graded as trivial in 1, small in 2. Average follow-up period was 7.54 months ± 7.5 (1 day to 25 months). There was no incidence of heart block, bacterial endocarditis, hemolysis, device embolization, or fracture. The aortic insufficiency resolved in 1 patient and was estimated to be trivial in the remaining 3 patients. In conclusion, percutaneous closure of Pm VSDs using the softer new generation devices as the AVP II and the ADO II is feasible and safe. Longer follow-up and larger series are needed.