Transcatheter recanalisation for absent intra-pericardial pulmonary arteries.

Unilateral absence of intra-pericardial pulmonary artery is a rare congenital malformation. If untreated, it can lead to morbidity and mortality in adulthood. Early intervention and restoration of physiologic pulmonary blood flow is necessary.

Case report: hepatobronchial lymphatic communications in single ventricle patients as a pathophysiological mechanism of plastic bronchitis: diagnosis and treatment.

Background: Plastic bronchitis is a rare but devastating complication in single ventricle patients after Fontan completion. Recent advances in dynamic contrast-enhanced magnetic resonance lymphangiogram demonstrate the typical pathophysiological mechanism of the thoracic duct leaking lymphatic fluid towards the bronchi resulting in intraluminal casts. This has been termed abnormal pulmonary lymphatic perfusion and has been successfully treated in 94% of patients with thoracic duct occlusion.

Prominent Eustachian Valve and Atrial Septal Defect Presenting With Chronic Hypoxemia in a Teenager.

Prominent Eustachian valves, with obligate right-to-left shunts, have been reported as a cause of neonatal hypoxemia. This anomaly can present as an obstructive structure that inhibits antegrade flow through the tricuspid valve and furthermore contributes to right-to-left atrial shunting in the presence of a patent foramen ovale or atrial septal defect. This case highlights the evaluation and diagnostic workup for chronic hypoxemia in an adolescent female patient and considerations for percutaneous atrial septal defect closure.

Balloon angioplasty of bidirectional Glenn anastomosis.

Objective: We aim to assess the safety and efficacy of the transcatheter balloon dilation of superior cavopulmonary anastomosis (SCPA).

Background: SCPA stenosis can lead to impaired pulmonary blood flow, hypoxemia and development of veno-venous collaterals with right-to-left shunt. Balloon dilation of SCPA has been rarely reported and follow-up information is lacking.

Early Surgical Closure of Atrial Septal Defect Improves Clinical Status of Symptomatic Young Children with Underlying Pulmonary Abnormalities.

Elective closure of atrial septal defect (ASD) is usually recommended during preschool ages. However, ASD may contribute to deteriorating health in the presence of significant comorbidity and, thus, may need earlier closure. There is a lack of clarity regarding the indications for and outcomes after ASD closure in infancy and early childhood.

Lymphatic Management in Single-Ventricle Patients.

Lymphatic complications in patients with single ventricle include plastic bronchitis, protein-losing enteropathy, and chylous pleural effusion are a source of significant morbidity and mortality with historically limited therapeutic options. Novel lymphatic imaging techniques such as intranodal lymphangiography, dynamic contrast enhanced magnetic resonance lymphangiography and liver lymphangiography have allowed visualization of the lymphatic system and discovery of the pathophysiological mechanism of these conditions. This mechanism includes the combination of 2 factors: increased lymphatic flow in patients with elevated central venous pressure and presence of the lymphatic anatomical variant that allows the lymph to flow in close proximity to the serous (pleural space in chylothorax) or mucosal (plastic bronchitis and protein losing enteropathy) surfaces.

Identifying Subclinical Coronary Abnormalities and Silent Myocardial Ischemia After Arterial Switch Operation.

The incidence of late coronary complications is reported around 8% after arterial switch operation (ASO) for d-transposition of the great arteries, but the affected patients are usually asymptomatic. Exercise stress test (EST) and myocardial perfusion imaging (MPI) are common non-invasive modalities to screen for silent myocardial ischemia, but their diagnostic reliability in children after ASO is unclear. We retrospectively reviewed asymptomatic patients following ASO with EST, MPI, and coronary imaging studies (CIS) and examined the reliability of each test in identifying abnormal coronary lesions responsible for myocardial ischemia.

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.

The Medtronic Micro Vascular Plug™ for Vascular Embolization in Children With Congenital Heart Diseases.

Objectives: To describe the early multi-center, clinical experience with the Medtronic Micro Vascular Plug™ (MVP) in children with congenital heart disease (CHD) undergoing vascular embolization.

Background: The MVP is a large diameter vascular occlusion device that can be delivered through a microcatheter for embolization of abnormal blood vessels.

Methods: A retrospective review of embolization procedures using the MVP in children with CHD was performed in 3-centers.

Initial clinical experience with the Medtronic Micro Vascular Plug™ in transcatheter occlusion of PDAs in extremely premature infants.

Objectives: To describe the early multicenter, clinical experience with the Medtronic Micro Vascular Plug™ (MVP) for the occlusion of patent ductus arteriosus (PDA) in premature infants.

Background: The MVP is a large diameter plug that can be delivered through a microcatheter for occlusion of abnormal blood vessels.

Methods: A Retrospective review of PDA embolization procedures performed in two centers using the MVP was performed.

Maladaptive aortic properties after the Norwood procedure: An angiographic analysis of the Pediatric Heart Network Single Ventricle Reconstruction Trial.

Objectives: Aortic arch reconstruction in children with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe neoaortic anatomy and physiology, risk factors for abnormalities, and impact on right ventricular function in patients with single right ventricle lesions after arch reconstruction.

Methods: Prestage II aortic angiograms from the Pediatric Heart Network Single Ventricle Reconstruction trial were analyzed to define arch geometry (Romanesque [normal], crenel [elongated], or gothic [angular]), indexed neoaortic dimensions, and distensibility.

Coronary artery problems late after arterial switch operation for transposition of the great arteries.

Background: The incidence of late coronary artery abnormalities after arterial switch operation (ASO) for d-loop transposition of the great arteries may be underestimated.

Methods and results: We retrospectively reviewed coronary artery morphology in 40 of 97 patients who survived the first year after ASO. Seven asymptomatic patients developed significant late coronary artery abnormalities.

Improving early outcomes following hybrid procedure for patients with single ventricle and systemic outflow obstruction: defining risk factors†.

Objectives: The hybrid procedure has become an accepted management strategy for patients with single ventricle and systemic outflow obstruction (SVSOO), particularly those considered at high risk for mortality following Stage I Norwood. We sought to refine patient selection by identifying clinical variables associated with early mortality.

Methods: Single-centre review of all patients with SVSOO undergoing a hybrid procedure between January 2003 and December 2012.

Hybrid palliation for critical systemic outflow obstruction: neither rapid stage 1 Norwood nor comprehensive stage 2 mitigate consequences of early risk factors.

Objective: Hybrid palliation with branch pulmonary artery banding (bPAB) has become increasingly common in the early management of patients with critical left ventricular outflow obstruction. Optimal subsequent surgical palliation remains undefined.

Methods: We retrospectively reviewed patients undergoing initial bPAB for single ventricle physiology with systemic outflow obstruction (2001-2013, n = 37).

Acquired discrete subaortic stenosis in solid organ transplant recipients.

DSS is a rare acquired abnormality of the LV outflow tract in children and adults. DSS can be seen with or without associated congenital heart disease, but the mechanism of its emergence has been poorly understood. Here, we report two cases of DSS that developed after solid organ transplant in childhood.

Device closure of secundum atrial septal defects in infants weighing less than 8 kg.

This study aimed to assess the technical aspects of atrial septal defect (ASD) closure using the Amplatzer septal occluder (ASO) and the Gore Helex septal occluder (GHSO) for infants weighing less than 8 kg and to determine the safety, effectiveness, and near-to-intermediate-term outcome of the closure. The Mid-Atlantic Group of Interventional Cardiology Registry of percutaneous, transcatheter ASD closure procedures was reviewed for this analysis. Patients from 10 hospitals in the United States were included.

Impact of pre-stage II hemodynamics and pulmonary artery anatomy on 12-month outcomes in the Pediatric Heart Network Single Ventricle Reconstruction trial.

Objective: To compare the interstage cardiac catheterization hemodynamic and angiographic findings between shunt types for the Pediatric Heart Network Single Ventricle Reconstruction trial. The trial, which randomized subjects to a modified Blalock-Taussig shunt (MBTS) or right ventricle-to-pulmonary artery shunt (RVPAS) for the Norwood procedure, demonstrated the RVPAS was associated with a smaller pulmonary artery diameter but superior 12-month transplant-free survival.

Methods: We analyzed the pre-stage II catheterization data for the trial subjects.

Bilateral pulmonary arterial banding results in an increased need for subsequent pulmonary artery interventions.

Objective: Despite increasing use of bilateral branch pulmonary artery banding (bPAB), both as a temporary stabilizing treatment and as part of comprehensive hybrid management of hypoplastic left heart syndrome, little is known about the long-term outcomes of the pulmonary arteries (PAs) in banded patients.

Patients And Methods: We conducted a retrospective review of all patients with ductal-dependent systemic circulation (2001-2013) undergoing bPAB placement at a single institution (bPAB, n = 50); patients who underwent a stage I Norwood procedure (Norwood, n = 53) were used for comparison. The need for PA interventions (surgical arterioplasty, balloon angioplasty, and stent implantation) and PA growth were assessed.

Intervention for recoarctation in the single ventricle reconstruction trial: incidence, risk, and outcomes.

Background: Recoarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt or a right ventricle-pulmonary artery shunt. We sought to determine the incidence of recoarctation, risk factors, and outcomes in the SVR trial.

Hard choices for high-risk patients with critical left ventricular outflow obstruction: contemporary comparison of hybrid versus surgical strategy.

Surgical management of high-risk newborns with critical left ventricular outflow tract obstruction (LVOTO) involves difficult decision making and complex procedures associated with significant morbidity and mortality. We sought to compare the outcomes of the hybrid and surgical strategies for the management of neonates with critical LVOTO considered at high risk in a contemporary nonrandomized cohort. This is a retrospective review of all patients undergoing management of critical LVOTO between January 2001 and December 2008.

Complex atrial septal defect: percutaneous repair guided by three-dimensional echocardiography.

Three-dimensional transesophageal echocardiography (3D TEE) has been used to guide the percutaneous repair of simple atrial septal defects (ASDs). There has been limited experience in using this imaging modality to guide complex ASD repair. In this report, we describe how 3D TEE was used to guide the repair of a complex, multifenestrated ASD.

Bailout after failed biventricular management of critical aortic stenosis: another application of the hybrid approach.

The decision between a single-ventricle and biventricular strategy can be particularly difficult in patients with critical left ventricular outflow obstruction who have marginal left ventricular size or function. Overzealous pursuit of a biventricular strategy in borderline cases can lead to death or require conversion to a single-ventricle physiology at increased risk. We describe novel use of the hybrid approach as a bailout option for a patient with critical aortic stenosis who, despite balloon valvuloplasty, exhibited persistent severe left ventricular dysfunction.

Stage II reconstruction after hybrid palliation for high-risk patients with a single ventricle.

Background: The hybrid approach to palliate high-risk neonates with a single ventricle and systemic outflow obstruction continues to gain interest. Despite early success, few data exist regarding the physiologic adequacy of this palliation and the outcome of the stage II reconstruction.

Methods: We reviewed our experience with stage II reconstruction after hybrid palliation in high-risk newborns with hypoplastic left heart syndrome and variants, focusing on the hemodynamic, reintervention, and operative data.

Improving the outcome of high-risk neonates with hypoplastic left heart syndrome: hybrid procedure or conventional surgical palliation?

Objectives: Despite significant progress, surgical outcome for high-risk patients with hypoplastic left heart syndrome (HLHS) remain suboptimal. The hybrid palliation lessens the initial operative insult and is expected to improve overall survival; however the outcome of this management sequence is unknown.

Methods: Retrospective review of all high-risk neonates (prematurity, low birth weight, associated genetic or co-morbid conditions) undergoing initial palliation for HLHS either by hybrid or Stage I Norwood procedure at a single institution between January 2001 and December 2006.

Right ventricular outflow tract stent as a bridge to surgery in a premature infant with tetralogy of Fallot.

Despite dramatic progress in neonatal cardiac surgery, prematurity and low birth weight remain risk factors for poor outcome. Attempts to delay intervention with supportive therapy have been shown to increase morbidity and mortality. We present a case of an 840 gram, 28-week gestation newborn with tetralogy of Fallot, in whom palliation was achieved with a right ventricular outflow tract stent.