Chronic intercostal neuralgia after placement of right ventricle to pulmonary artery conduit with prosthetic valve.

Adults with congenital heart disease often have complex medical issues requiring individualised multidisciplinary care for optimising outcomes and quality of life. Chronic pain is an example. We report a rare case of intercostal neuralgia seemingly caused by irritation from a prosthetic valve in a right ventricle to pulmonary artery conduit in a patient with tetralogy of Fallot.

Correction: Arterial tortuosity syndrome: 40 new families and literature review.

In the published version of this paper the author Neus Baena's name was incorrectly given as Neus Baena Diez. This has now been corrected in both the HTML and PDF versions of the paper.

Arterial tortuosity syndrome: 40 new families and literature review.

Purpose: We delineate the clinical spectrum and describe the histology in arterial tortuosity syndrome (ATS), a rare connective tissue disorder characterized by tortuosity of the large and medium-sized arteries, caused by mutations in SLC2A10.

Methods: We retrospectively characterized 40 novel ATS families (50 patients) and reviewed the 52 previously reported patients. We performed histology and electron microscopy (EM) on skin and vascular biopsies and evaluated TGF-β signaling with immunohistochemistry for pSMAD2 and CTGF.

Removal of a retained intracardiac radiolucent guidewire fragment using an Atrieve™ vascular snare using combined fluoroscopy and transesophageal echocardiography guidance in an infant.

Retained intravascular foreign body is a well-known complication of central venous access placement in children as well as adults. Most of these foreign bodies are radio-opaque and hence are removed under fluoroscopy guidance. In our case, we describe the removal of an intracardiac radiolucent foreign body in an infant utilizing a combination technique - transesophageal echocardiogram and fluoroscopy.

Relationship between renal function and extracorporeal membrane oxygenation use: a single-center experience.

The effects of extracorporeal membrane oxygenation (ECMO) support on renal function in children with critical illness are unknown. The objective of this study was to investigate the impact of ECMO on renal function among children in different age groups. We performed a single-center retrospective observational study in critically ill children ≤ 18 years supported on ECMO for refractory cardiac or pulmonary failure (2006-2012).

Extracorporeal membrane oxygenation in children with heart disease and genetic syndromes.

Our objective was to evaluate morbidity and mortality associated with extracorporeal membrane oxygenation (ECMO) in children with genetic syndromes and heart disease. We conducted a retrospective review of all children with heart disease and genetic syndromes receiving ECMO during the period January 2000 and March 2012 at Arkansas Children's Hospital, Little Rock. The medical charts were reviewed to obtain the following variables: demographic information, medical and surgical history, laboratory and microbiological, information on organ dysfunction, and outcome characteristics.

Novel use of an Amplatzer Vascular Occluder II to close an acquired fistula between a reconstructed aortic arch and the left atrial appendage in a patient with hypoplastic left heart syndrome.

An 8-month-old female with hypoplastic left heart syndrome had undergone bidirectional cavopulmonary anastomosis at the age of 4.5 months and presented with a new continuous flow murmur on routine follow-up. Diagnostic catheterization demonstrated a fistula between the left atrial appendage and the neo-aortic arch.

Extracorporeal membrane oxygenation support for intractable primary arrhythmias and complete congenital heart block in newborns and infants: short-term and medium-term outcomes.

Objectives: To describe the experience with extracorporeal membrane oxygenation support for intractable primary arrhythmias in newborns and infants.

Design: Retrospective study.

Setting: A tertiary care pediatric hospital.

Interhospital transport of children requiring extracorporeal membrane oxygenation support for cardiac dysfunction.

Objective: Many centers are able to emergently deploy extracorporeal membrane oxygenation (ECMO) as support in children with refractory hemodynamic instability, but may be limited in their ability to provide prolonged circulatory support or cardiac transplantation. Such patients may require interhospital transport while on ECMO (cardiac mobile [CM]-ECMO) for additional hemodynamic support or therapy. There are only three centers in the United States that routinely perform CM-ECMO.

Use of near-infrared spectroscopy for estimation of renal oxygenation in children with heart disease.

We evaluated whether near-infrared spectroscopy (NIRS) measurement from the flank correlates with renal vein saturation in children undergoing cardiac catheterization. Thirty-seven patients <18 years of age were studied. A NIRS sensor was placed on the flank, and venous oxygen saturations were measured from the renal vein and the inferior vena cava (IVC).

Therapeutic hypothermia in a neonate with perinatal asphyxia and transposition of the great arteries.

Despite advances in surgical techniques and perioperative management, many newborns with complex congenital heart disease (CCHD) continue to have adverse neurodevelopmental outcomes. With increasing survival of these infants, neuroprotective therapies at various time points, should be considered as an important area of investigation. As some brain injury has been shown to precede surgery, the preoperative period may be a key time to direct protective therapy.

Long-term outcomes of intraoperative pulmonary artery stent placement for congenital heart disease.

Objective: Our objective was to examine long-term outcomes of intraoperative pulmonary artery stents and determine risk factors for reintervention

Background: Short-term outcomes of intraoperative pulmonary artery stents have been reported previously. However, long-term results are unknown.

Methods: We conducted a retrospective review of patients who underwent intraoperative pulmonary artery stent placement for branch pulmonary artery stenosis.

Single-institution experience with interhospital extracorporeal membrane oxygenation transport: A descriptive study.

Objective: Patients with refractory cardiopulmonary failure may benefit from extracorporeal membrane oxygenation, but extracorporeal membrane oxygenation is not available in all medical centers. We report our institution's nearly 20-yr experience with interhospital extracorporeal membrane oxygenation transport.

Design: Retrospective review.

Intrahospital transport of children on extracorporeal membrane oxygenation: indications, process, interventions, and effectiveness.

Objective: To evaluate indications, process, interventions, and effectiveness of patients undergoing intrahospital transport. Critically ill patients supported with extracorporeal membrane oxygenation are transported within the hospital to the radiology suite, cardiac catheterization suite, operating room, and from one intensive care unit to another. No studies to date have systematically evaluated intrahospital transport for patients on extracorporeal membrane oxygenation.

Pathologic aneurysmal dilation of the ascending aorta and dilation of the main pulmonary artery in patients with Kabuki syndrome: valve-sparing aortic root replacement.

We report the aneurysmal dilation of the ascending aorta and the main pulmonary artery in 2 children with Kabuki syndrome. In 1 patient, there was progressive aneurysmal dilation of the ascending aorta necessitating aortoplasty. Histologic examination of the resected aorta revealed disrupted and fragmented elastic fibers in the medial layer, along with mucinous degeneration of the aortic wall.

Stenting for superior vena cava obstruction in pediatric heart transplant recipients.

Background: Superior vena cava (SVC) obstruction can be a complication in heart transplant recipients. We reviewed our experience with relief of SVC obstruction using endovascular stents in pediatric heart transplant recipients.

Methods: Study cohort included pediatric heart transplant recipients, followed at our institution, who required endovascular stent placement for SVC obstruction.

Noninvasive cerebral oximeter as a surrogate for mixed venous saturation in children.

We evaluated the relationship between regional cerebral oxygen saturation (rSO(2)) measured by near-infrared spectroscopy (NIRS) cerebral oximeter with superior vena cava (SVC), inferior vena cava (IVC), right atrium (RA), and pulmonary artery (PA) saturation measured on room air and 100% inspired oxygen administered via a non-rebreather mask (NRB) in children. Twenty nine pediatric post-orthotopic heart transplant patients undergoing an annual myocardial biopsy were studied. We found a statistically significant correlation between rSO(2) and SVC saturations at room air and 100% inspired oxygen concentration via NRB (r = 0.

Adequacy of peritoneal dialysis in children following cardiopulmonary bypass surgery.

Acute renal failure requiring renal replacement therapy can complicate cardiopulmonary bypass in children. Peritoneal dialysis has been shown to stabilize electrolytes and improve fluid status in these patients. To assess dialysis adequacy in this setting, we prospectively measured Kt/V and creatinine clearance in five patients (6-839 days of age) requiring renal replacement therapy at our institution.