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.

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-ß signaling with immunohistochemistry for pSMAD2 and CTGF. RESULTS: Stenoses, tortuosity, and aneurysm formation are widespread occurrences. Severe but rare vascular complications include early and aggressive aortic root aneurysms, neonatal intracranial bleeding, ischemic stroke, and gastric perforation. Thus far, no reports unequivocally document vascular dissections or ruptures. Of note, diaphragmatic hernia and infant respiratory distress syndrome (IRDS) are frequently observed. Skin and vascular biopsies show fragmented elastic fibers (EF) and increased collagen deposition. EM of skin EF shows a fragmented elastin core and a peripheral mantle of microfibrils of random directionality. Skin and end-stage diseased vascular tissue do not indicate increased TGF-ß signaling. CONCLUSION: Our findings warrant attention for IRDS and diaphragmatic hernia, close monitoring of the aortic root early in life, and extensive vascular imaging afterwards. EM on skin biopsies shows disease-specific abnormalities.

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.

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.