Novel use of covered stents to treat profound cyanosis in a hepatic vein exclusion Fontan.

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.

Particle embolization of systemic-to-pulmonary collateral artery networks in congenital heart disease: Technique and special considerations.

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.

Staged Palliation of Cyanotic Obstructive Lesions With a Modified Right Ventricular Outflow Procedure.

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.

Transcatheter closure of patent ductus arteriosus using the AMPLATZER™ duct occluder II (ADO II).

Objectives: The study purpose is to evaluate the safety and efficacy of the ADO II device for closure of patent ductus arteriosus (PDA) in children.

Background: Transcatheter 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.

"Not-so-identical" twins with trisomy 21 and perimembranous ventricular septal defects.

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.

Gain-of-function mutations in SMAD4 cause a distinctive repertoire of cardiovascular phenotypes in patients with Myhre syndrome.

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.

Percutaneous Closure of Perimembranous Ventricular Septal Defects Using the Second-Generation Amplatzer Vascular Occluders.

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.

3D harmonic phase tracking with anatomical regularization.

This paper presents a novel algorithm that extends HARP to handle 3D tagged MRI images. HARP results were regularized by an original regularization framework defined in an anatomical space of coordinates. In the meantime, myocardium incompressibility was integrated in order to correct the radial strain which is reported to be more challenging to recover.

Achieving Benchmark Results for Neonatal Palliation of Hypoplastic Left Heart Syndrome and Related Anomalies in an Emerging Program.

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.

Systemic Semilunar Valve Replacement in Pediatric Patients Using a Porcine, Full-Root Bioprosthesis.

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.

"How to do it": hybrid stent placement for pulmonary vein stenosis.

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.

3D strain assessment in ultrasound (Straus): a synthetic comparison of five tracking methodologies.

This paper evaluates five 3D ultrasound tracking algorithms regarding their ability to quantify abnormal deformation in timing or amplitude. A synthetic database of B-mode image sequences modeling healthy, ischemic and dyssynchrony cases was generated for that purpose. This database is made publicly available to the community.

Catheter conversion of classic Glenn to bidirectional Glenn with closure of left central shunt years after surgical attempts.

Bidirectional Glenn shunt is usually performed in patients with single ventricle in preparation for a total cavo-pulmonary connection. We present a patient born with complex congenital heart disease consisting of single ventricle, pulmonary atresia, non confluent pulmonary arteries, and anomalous pulmonary venous return in whom surgical bidirectional Glenn was attempted. After multiple surgical attempts she was converted to a Classic Glenn and a central ascending aorta to left pulmonary artery shunt.

Transhepatic approach for rehabilitation of stenosed pulmonary arteries.

Transhepatic cardiac catheterization and intervention is used in selected cases in our institution. A retrospective review of transcatheter interventions for the pulmonary artery was conducted. Forty-five transhepatic procedures were performed.

Preclosure pressure gradients predict patent ductus arteriosus patients at risk for later left pulmonary artery stenosis.

The objective of this study was to evaluate the incidence of pre-existing catheterization left pulmonary artery (LPA) gradients and correlation of these gradients with later LPA stenosis after successful patent ductus arteriosus (PDA) occlusion. We performed a single-center review of 130 patients with PDA closure from October 1993 to February 2005. We analyzed the pre-PDA closure LPA pressure gradients at catheterization to determine if these were predictive of late LPA stenosis.

Closure of external tunnel Fontan fenestration: a novel use of the Amplatzer vascular plug.

Background: Newer modifications of the Fontan operation include the external tunnel conduit with placement of an elongated type of fenestration. Atrial septal defect closure devices used traditionally to close fenestrations have short connecting waists with retention discs on each side. These may not be suitable for this type of Fontan fenestration.

Balloon dilation of the pulmonary valve in premature infants with tetralogy of Fallot.

Infants with tetralogy of Fallot (ToF) presenting with desaturation may require augmentation of the pulmonary blood flow, usually in the form of a Blalock-Taussig shunt. Shunts may result in a preferential increase in blood flow to one lung. They also may be associated with significant morbidity and possibly mortality of premature infants.

Intermediate follow-up following intravascular stenting for treatment of coarctation of the aorta.

Background: We report a multiinstitutional study on intermediate-term outcome of intravascular stenting for treatment of coarctation of the aorta using integrated arch imaging (IAI) techniques.

Methods And Results: Medical records of 578 patients from 17 institutions were reviewed. A total of 588 procedures were performed between May 1989 and Aug 2005.

Procedural results and acute complications in stenting native and recurrent coarctation of the aorta in patients over 4 years of age: a multi-institutional study.

Background: We report a multi-institutional experience with intravascular stenting (IS) for treatment of coarctation of the aorta.

Methods And Results: Data was collected retrospectively by review of medical records from 17 institutions. The data was broken down to prior to 2002 and after 2002 for further analysis.

Transhepatic vascular access for diagnostic and interventional procedures: techniques, outcome, and complications.

Early or multiple cardiac catheterizations may result in occlusion of the femoral veins prohibiting their use. The internal jugular or sub-clavian approach may be an appropriate alternative. However, these approaches may not be suitable in patients with surgical interruption of the superior vena cava.

Transhepatic closure of atrial septal defect and assisted closure of modified Blalock/Taussig shunt.

The details of transhepatic closure of moderate - large size atrial septal defect as well as assisted closure of a modified Blalock Taussig shunt in a 2 1/2 year old patient with occluded femoral vessels are discussed. The technical variations as well as the potential advantages of this route are outlined.

Catheter management of occluded superior baffle after atrial switch procedures for transposition of great vessels.

We present successful catheter intervention in 4 patients with total occlusion of the superior baffle after an atrial switch procedure. A transseptal needle (in addition to the transhepatic route in 1 patient with known occluded femoral veins) was used to open the occluded segments and place stents. Additionally, we report the regression of multiple systemic-to-pulmonary venous fistulas that developed as a result of the occluded baffle.