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. Intercostal neuralgia is a painful disorder linked to nerve irritation or injury from trauma, infection or pressure. Although chronic postsurgical pain after cardiac surgery is prevalent, rarely the aetiology relates to valve irritation on a single intercostal nerve. After failing pharmacological therapy for 8 months, the neuralgia completely resolved after an ultrasound-guided neurolytic block with long-term effectiveness and improvement in patient satisfaction.

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). The patient population was divided into four age groups for the purpose of comparisons. The Acute Kidney Injury Network's (AKIN's) validated, three-tiered staging system for acute kidney injury was used to categorize the degree of worsening renal function. Data on patient demographics, baseline characteristics, renal function parameters, dialysis, ultrafiltration, duration of mechanical cardiac support, and mortality were collected. Comparisons of baseline characteristics, duration of mechanical cardiac support, and renal function were made between the four age groups. During the study period, 311 patients qualified for inclusion, of whom 289 patients (94%) received venoarterial (VA) ECMO, 12 (4%) received venovenous (VV) ECMO, and 8 (3%) received both VV and VA ECMO. A total of 109 patients (36%) received ultrafiltration on ECMO, 58 (19%) received hemodialysis, and 51 (16%) received peritoneal dialysis. There was a steady and sustained improvement in renal function in all age groups during the ECMO run, with the maximum and longest-sustained improvement occurring in the oldest age group. Proportions of patients in different AKIN stages remained similar in the first 7 days after ECMO initiation. We demonstrate that renal dysfunction improves early after ECMO support. Irrespective of the underlying disease process or patient age, renal function improves in children with pulmonary or cardiac failure who are placed on ECMO.

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. The outcome variables evaluated in this report included: hospital length of stay (LOS), survival to hospital discharge, and current survival. Outcome data were compared among critically ill children with and without syndromes. During the study period, there were 377 ECMO runs in 336 children with heart disease. Of these, 43 ECMO runs occurred in children with genetic syndromes whereas 334 ECMO runs occurred in children with no genetic abnormality. Children in the group with underlying genetic syndrome were older at the time of ECMO cannulation than the group with no syndrome. During the ECMO run, hospital LOS and mortality were similar in children with and without underlying genetic abnormality. Among genetically abnormal patients, renal insufficiency and need for dialysis were associated with mortality. In this group, 24 patients (56%) were discharged alive. However, only 10 patients are living to date in this cohort. ECMO can be used in children with heart disease and genetic syndromes with good results. The survival rate is high and the complication rate is low.

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. The fistula was sealed with an Amplatzer Vascular Occluder II device without complications.

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). There was a strong correlation between flank NIRS values (rSO(2)) and renal vein saturation (r = 0.821, p = 0.002) and IVC saturation (r = 0.638, p = 0.004) in children weighing ≤ 10 kg. In children weighing > 10 kg, there was no correlation between rSO(2) and renal vein saturation (r = 0.158, p = 0.57) or IVC saturation (r = -0.107, p = 0.67). Regional tissue oxygenation as measured by flank NIRS correlates well with both renal vein and IVC oxygen saturations in children weighing <10 kg undergoing cardiac catheterization, but not in larger children.

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. Selective hypothermia is used as a neuroprotective therapy for neonates with moderate-to-severe hypoxic-ischemic encephalopathy. We report a case of an asphyxiated term newborn with severe neonatal encephalopathy following neonatal resuscitation and subsequently found to have transposition of the great arteries with a relatively intact atrial septum, who underwent CoolCap(®) therapy following emergent balloon atrial septostomy. Brain injury observed on magnetic resonance imaging preoperatively after cooling was focal and did not extend following neonatal arterial switch operation. The patient's neurologic outcome appeared to be favorable at hospital discharge and at age 6 months. This case presents a therapeutic intervention which may represent a valuable neuroprotective strategy to limit brain injury, and therefore, improve neurodevelopmental outcomes in neonates with CCHD with perinatal asphyxia.

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. SETTING: Quaternary care children's hospital. PATIENTS: All patients undergoing interhospital extracorporeal membrane oxygenation transport by the Arkansas Children's Hospital extracorporeal membrane oxygenation team. INTERVENTIONS: Data (age, weight, diagnosis, extracorporeal membrane oxygenation course, hospital course, mode of transport, and outcome) were obtained and compared with the most recent Extracorporeal Life Support Organization Registry report. RESULTS: Interhospital extracorporeal membrane oxygenation transport was provided to 112 patients from 1990 to 2008. Eight were transferred between outside facilities (TAXI group); 104 were transported to our hospital (RETURN group). Transport was by helicopter (75%), ground (12.5%), and fixed wing (12.5%). No patient died during transport. Indications for extracorporeal membrane oxygenation in RETURN patients were cardiac failure in 46% (48 of 104), neonatal respiratory failure in 34% (35 of 104), and other respiratory failure in 20% (21 of 104). Overall survival from extracorporeal membrane oxygenation for the RETURN group was 71% (74 of 104); overall survival to discharge was 58% (61 of 104). Patients with cardiac failure had a 46% (22 of 48) rate of survival to discharge. Neonates with respiratory failure had an 80% (28 of 35) rate of survival to discharge. Other patients with respiratory failure had a 62% (13 of 21) rate of survival to discharge. None of these survival rates were statistically different from survival rates for in-house extracorporeal membrane oxygenation patients or for survival rates reported in the international Extracorporeal Life Support Organization Registry (p > .1 for all comparisons). CONCLUSIONS: Outcomes of patients transported by an experienced extracorporeal membrane oxygenation team to a busy extracorporeal membrane oxygenation center are very comparable to outcomes of nontransported extracorporeal membrane oxygenation patients as reported in the Extracorporeal Life Support Organization registry. As has been previously reported, interhospital extracorporeal membrane oxygenation transport is feasible and can be accomplished safely. Other experienced extracorporeal membrane oxygenation centers may want to consider developing interhospital extracorporeal membrane oxygenation transport capabilities to better serve patients in different geographic regions.

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. Data retrieved retrospectively included cardiac diagnosis, age, and weight at transplant, surgical technique of transplant (bicaval vs. biatrial anastomosis), previous cardiovascular surgeries, presenting symptoms, date of SVC stent placement, and need for reintervention. RESULTS: From March 1990 to June 2006, 5.1% (7/138) pediatric heart transplant recipients who were followed at our institution had SVC obstruction requiring stent placement. Median age and weight at transplant was 9 months and 8.7 kg, respectively. Four patients previously had a cavopulmonary anastomosis. Transplant surgery involved bicaval anastomosis in 6 and biatrial in 1. Of the 7 patients included in the study, 2 were asymptomatic, 2 were symptomatic (1 with chylothorax, 1 with headache), and 3 were identified at the time of transplant surgery. Median time from transplant surgery to SVC stent placement was 2 months (0-14 months). Three patients required reintervention as redilation of SVC stent (n = 1) or additional SVC stent (n = 2). In one patient the stent migrated to the pulmonary artery but was retrieved. CONCLUSION: SVC obstruction can be an important complication following heart transplantation, especially in infants with previous cavopulmonary anastomosis, undergoing heart transplant using bicaval technique. SVC obstruction can be safely and effectively treated using endovascular stents.

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 = 0.67, p = 0.0002 on room air; r = 0.44, p = 0.02 on NRB), RA saturation (r = 0.56, p = 0.002; r = 0.56, p = 0.002), and PA saturation (r = 0.67, p < 0.001; r = 0.4, p = 0.03). A significant correlation also existed between rSO(2) and measured cardiac index (r = 0.45, p = 0.01) and hemoglobin levels (r = 0.41, p = 0.02). The concordance correlations were fair to moderate. Bias and precision of rSO(2) compared to PA saturations on room air were -0.8 and 13.9%, and they were 2.1 and 15.6% on NRB. A stepwise linear regression analysis showed that rSO(2) saturations were the best predictor of PA saturations on both room air (p = 0.0001) and NRB (p = 0.012). In children with biventricular anatomy, rSO(2) readings do correlate with mixed venous saturation.

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. Median dialysis creatinine clearance was 74.25 L/week/1.73m(2) (range 28.28-96.63 L/week/1.73m(2)). Residual renal function provided additional solute clearance as total creatinine clearance was 215.97 L/week/1.73m(2) (range 108.04-323.25 L/week/1.73m(2)). Dialysis Kt/V of >2.1 (median 4.84 [range 2.12-5.59]) was achieved in all patients. No dialysis-associated complications were observed. We conclude that peritoneal dialysis is a safe, simple method of providing adequate clearance in children who develop acute renal failure following exposure to cardiopulmonary bypass.