Transcatheter stent implantation in a child with severe stenosis of the inferior caval vein secondary to injury.

Stenosis of the Inferior Caval Vein is rarely encountered in the paediatric setting. A 5-year-old male sustained severe injuries secondary to a fall from a three story balcony and was subsequently found to have severe stenosis of the inferior caval vein resulting in extensive lymphatic drainage with chylothorax, chyloperitoneum, and severe abdominal ascites. This was successfully treated with transcatheter stent placement resulting in complete resolution of the stenosis and significant clinical improvement allowing for transfer to a rehabilitation centre and eventual discharge home.

Living at an altitude adversely affects exercise capacity in Fontan patients.

BACKGROUND: Data assessing the effect of altitude on Fontan haemodynamics are limited to experimental models and case reports. Both suggest a detrimental impact. This study describes exercise performance in patients with Fontan circulation and matched controls at a low altitude versus at sea level. We sought to assess the impact of increasing altitude on functional capacity in patients with Fontan palliation. METHODS: A retrospective review of 22 patients at low altitude (1602 metres) and 119 patients at sea level with Fontan circulation, as well as age-, gender-, and altitude-matched controls, underwent maximal cardiopulmonary exercise testing. Linear regression models were created to determine the influence of altitude on differences in exercise variables between Fontan patients and their matched controls. RESULTS: Peak oxygen consumption was 28.4 millilitres per kilogram per minute (72% predicted) for the sea-level cohort and 24.2 millilitres per kilogram per minute (63% predicted) for the moderate altitude cohort. The matched case-control differences for patients at moderate altitude were greater for peak oxygen consumption (-29% against -13%, p = 0.04), anaerobic threshold (-36% against -5%, p = 0.001), and oxygen pulse (-35% against -18%, p = 0.007) when compared with patients living at sea level. When compared to institution-matched controls, the same parameters fell by 3%, 8.9%, and 4.2%, respectively, for each increase of 1000 feet in residential altitude (p = 0.03, p = 0.001, and p = 0.05, respectively). CONCLUSIONS: Patients with Fontan circulation at a higher altitude have impairment in aerobic capacity when compared with patients at sea level. Reduction in exercise capacity is associated with a reduction in stroke volume, likely related to increased pulmonary vascular resistance.

Selective use of cardiac computed tomography angiography: an alternative diagnostic modality before second-stage single ventricle palliation.

OBJECTIVES: To assess the accuracy and risk of substituting cardiac computed tomography for cardiac catheterization in select patients for evaluation of anatomy before second-stage single ventricle palliation. METHODS: This is a retrospective review of consecutive diagnostic cardiac catheterization (n=16) and computed tomography studies (n=16) performed before second-stage single ventricle palliation from March 2010 to July 2012 at a single institution. Risk (anesthesia, vascular access, contrast, and radiation exposure), accuracy, and postoperative course were compared. Nonparametric analysis was used to compare differences in group medians. RESULTS: General anesthesia was used for 16 of 16 cardiac catheterization studies and 1 of 16 computed tomography studies. Vascular access was central venous and/or arterial for all cardiac catheterization studies and a peripheral intravenous line for all computed tomography studies. Median age- and size-adjusted radiation dose was 14.0 mSv for cardiac catheterization and 1.1 mSv for computed tomography. Contrast dose was 4.8 mL/kg for the cardiac catheterization group and 2 mL/kg for the computed tomography group. There were no computed tomography discrepancies and 1 discrepancy between cardiac catheterization and surgical findings. There were 8 adverse events in 6 patients in the cardiac catheterization group and 1 adverse event in the computed tomography group. There was no difference between groups in postoperative course or need for repeat intervention. CONCLUSIONS: Cardiac computed tomography and cardiac catheterization are equally accurate for evaluation of anatomy before second-stage single ventricle palliation when compared with surgical findings. Computed tomography may be the preferred test in select patients because of decreased vascular access and anesthesia risk, lower radiation and contrast exposure, and fewer adverse events.

Cardiovascular imaging trends in congenital heart disease: a single center experience.

BACKGROUND: Cardiac magnetic resonance imaging (MRI) and CT are available in the recent era at many pediatric cardiac centers. OBJECTIVE: The aim was to provide a contemporary description of diagnostic imaging trends for definition of congenital heart disease (CHD). METHODS: Echocardiography, cardiac catheterization, cardiac MRI, and cardiac CT use in patients with congenital heart disease at a single institution was retrospectively recorded (2005-2012). Surgical procedures were recorded. Total and modality-specific rates were estimated by Poisson regression and compared. The median age, studies in patients aged >17 years, and referral diagnosis were tabulated for the last year of review. RESULTS: An average of 11,940 cardiovascular diagnostic tests was performed annually. The number of total studies, echocardiograms, catheterizations, and surgical procedures, did not change significantly across time. Echocardiography comprised 95% to 97% of all studies performed during each year of review. The use of cardiac MRI (2%) and cardiac CT (1%) increased linearly (P < .001), and the use of diagnostic catheterization decreased (0.7%; P = .0005). The median age was 3 years for echocardiography, 15 years for MRI, 11 years for CT, and 3 years for catheterization. The percentage of patients aged >17 years was 9% for echocardiography, 33% for cardiac MRI, 29% for cardiac CT, and 8% for catheterization. Most patients undergoing CT, MRI, and diagnostic catheterization had moderate or complex CHD. CONCLUSION: Cardiac CT is used increasingly in the recent era for evaluation of CHD. The increased use of both cardiac CT and cardiac MRI are temporally associated with a decrease in diagnostic cardiac catheterization.

Percutaneous pulmonary valve implantation in the young 2-year follow-up.

OBJECTIVES: The aim of this study was to investigate physiological and clinical consequences of percutaneous pulmonary valve implantation (PPVI) in patients with chronic right ventricular outflow tract (RVOT) obstruction and volume overload. BACKGROUND: The PPVI is a nonsurgical technique to address RVOT conduit dysfunction. METHODS: Twenty-eight adolescents (median age 14.9 years; age range 10.9 to 19 years) underwent PPVI due to RVOT stenosis and/or pulmonary regurgitation (PR). Before and after PPVI echocardiographic and magnetic resonance imaging, cardiopulmonary exercise tests were obtained. RESULTS: The RVOT gradient (p < 0.001) and right ventricular (RV) systolic pressure decreased (p < 0.001), acutely. Magnetic resonance imaging (median 6 months) documented reduction in RV end-diastolic (149 +/- 49 ml/m(2) vs. 114 +/- 35 ml/m(2), p < 0.005) volume, increases in left ventricular (LV) end-diastolic (p < 0.007) volume and cardiac output (RV: p < 0.04 and LV: p < 0.02), and reduced PR fraction (24 +/- 10% to 7 +/- 7%, p < 0.0001). Symptoms, aerobic exercise performance (maximal oxygen consumption: p < 0.0001) and ventilatory response to carbon dioxide production (p < 0.003) improved. After 24 months, echocardiography demonstrated the RV/systemic-pressure ratio, and RVOT peak pressure gradient reductions persisted, and PR was absent in 93% (n = 12 of 13) of the cohort. Freedom from surgery was 91%, 83%, and 83%, and freedom from transcatheter reintervention was 91%, 80%, and 80%, at 12, 24, and 36 months, respectively. There were no acute device-related complications, with stent fractures noted in 10.8%. CONCLUSIONS: Percutaneous pulmonary valve implantation is feasible and safe in the young with dysfunctional RVOT conduits. An improvement in symptoms, hemodynamic status, and objective findings of exercise performance occurs. Early follow-up demonstrates persistent improvement in ventricular parameters, PR, and objective exercise capacity.

Corticotropin-independent cushing syndrome in a child with an ovarian tumor misdiagnosed as nonclassic congenital adrenal hyperplasia.

OBJECTIVE: To describe a patient with corticotropin-independent Cushing syndrome previously diagnosed and treated as congenital adrenal hyperplasia (CAH). METHODS: We describe the initial manifestations, clinical investigations, and postoperative follow-up of the patient and review similar cases in the literature. RESULTS: A 5 and 9/12-year-old girl who was initially diagnosed and treated as having CAH and was noncompliant with glucocorticoid therapy presented with weight gain, hypertension, and a mass in the lower abdomen. On physical examination, she was a cushingoid-appearing girl with proximal muscle weakness and notable facial acne. Laboratory findings included elevated serum testosterone, 17-hydroxyprogesterone, dehydroepiandrosterone sulfate, androstenedione, estradiol, and cortisol, as well as elevated urinary cortisol and cortisone. Serum corticotropin was undetectable. She had normal serum electrolytes and plasma renin activity. Computed tomography scan of the abdomen and pelvis showed a cystic mass with a focal enhancing solid component arising from the right ovary, which was subsequently determined to be a steroid cell tumor not otherwise specified. CONCLUSION: Although ovarian steroid cell tumors typically secrete gonadal steroids, the rare steroid cell tumors not otherwise specified can secrete both glucocorticoids and gonadal steroids and are an unusual cause of Cushing syndrome.

The effects of birth weight discordance and prematurity on twin neonatal outcome.

OBJECTIVE: The purpose of this study was to determine the effect of birth weight discordance (BWD) and prematurity on twin neonatal outcome by measuring Apgar scores and blood glucose concentrations at birth. METHODS: This was a retrospective cross-sectional study. All twins born at NY Methodist Hospital between January 1998 and December 2001 were eligible. Excluded were fetal deaths, maternal systemic disease, maternal infections, major neonatal malformations, neonates with birth weight < 500 g and multiple gestation greater than 2. BWD was defined as intrapair difference in birth weight expressed as percentage of the larger twin greater than 20% and prematurity as gestation of less than 37 weeks. Hypoglycemia was defined as blood glucose concentration < 40 mg/dl. RESULTS: There were 75 (48%) preterm and 80 (52%) term twin pairs. Nineteen (12%) of the preterm and 15 (9.6%) of the term showed BWD. Mean Apgar scores at 1 and 5 minutes were higher for the term twins as expected. Neither prematurity nor hypoglycemia was present predominantly in BWD twins. Twelve of the preterm and six of the term BWD twins showed evidence of hypoglycemia. CONCLUSION: Hypoglycemia in the early hours of postnatal life is relatively common in preterm BWD twins. Blood glucose measurement using reagent strips is an efficient way of monitoring blood glucose concentrations in these infants.

Mode of conception, placental morphology and perinatal outcome of twin gestations.

OBJECTIVE: To compare the perinatal outcome and placental morphology of twins conceived by assisted reproductive technologies (ART) or natural conception (NC). METHODS: The present retrospective study included 88 twin pairs. Methods of ART included in vitro fertilization, intrauterine insemination, embryo transfer and induced ovulation. Placental morphology was described by chorionicity, amnionicity, placental weight, umbilical cord insertion and the number of cord vessels. Perinatal outcomes included maternal age, gestational age, birth weight (BW), BW discordance and mode of delivery. RESULTS: The mothers of ART twins were 4 years older than NC mothers. ART twins delivered 2.2 weeks earlier than NC twins. The BW of ART twins A and B were less than NC twins A and B. There was no difference in the proportion of discordant twins in either group. Dichorionic diamniotic (DD) placentas accounted for the majority of placentas in both groups. There were no monochorionic-monoamniotic placentas in ART pairs, but there were 2.9% in NC pairs. Mode of conception had no effect on placental weight in DD or monochorionic-diamniotic twins in either group except for larger DD fused placentas in the ART group. CONCLUSION: ART twin pairs were delivered at an earlier gestational age at lower birth weights to older mothers predominantly by cesarean section. This perinatal outcome was not reflected by placental morphology.