The use of imaging that employs ionising radiation is increasing in the setting of paediatric cardiology. Children's high radiosensitivity and the lack of contemporary radiation data warrant a review ...of the radiation doses from the latest "state-of-the-art" angiography and computed tomography systems.
In children aged less than 16 years with congenital cardiac disease, we aimed to report: recent trends in the use of diagnostic angiography and cardiac dual-source computed tomography; the characteristics, lesions, and imaging histories of patients undergoing these procedures; and the average radiation doses imparted by each modality.
Retrospective review of consecutive cases undergoing cardiac computed tomography or diagnostic angiography in a teaching hospital between January, 2008 and December, 2009. Radiation doses were converted to effective doses (millisievert) using published conversion factors.
Angiography was performed 3.7 times more often than computed tomography. Computed tomography examinations increased by 92.5%, whereas angiography decreased by 26.4% in 2009 compared with 2008. Patients undergoing computed tomography were younger and weighed less than those undergoing angiography, but lesions were similar between the 2 groups. Multiple lifetime angiography was more prevalent than multiple lifetime computed tomography (p < 0.001). The median procedural dose - range - from angiography and computed tomography was 5 (0.2-27.8) and 1.7 (0.5-9.5) millisieverts, respectively (p < 0.001).
Despite not being completely analogous investigations, computed tomography should be considered prior to angiography and not withheld on radiation dose concerns, given that it imparts lower and more consistent doses than conventional angiography.
Iron overload and hemochromatosis are common, treatable conditions. HFE genotypes, levels of serum ferritin, transferrin saturation values, and self-reported medical history were studied in a ...multiethnic primary care population.
Participants were recruited from primary care practices and blood-drawing laboratories. Blood samples were tested for transferrin saturation, serum ferritin, and C282Y and H63D mutations of the HFE gene. Before genetic screening, participants were asked whether they had a history of medical conditions related to iron overload.
Of the 99,711 participants, 299 were homozygous for the C282Y mutation. The estimated prevalence of C282Y homozygotes was higher in non-Hispanic whites (0.44 percent) than in Native Americans (0.11 percent), Hispanics (0.027 percent), blacks (0.014 percent), Pacific Islanders (0.012 percent), or Asians (0.000039 percent). Among participants who were homozygous for the C282Y mutation but in whom iron overload had not been diagnosed (227 participants), serum ferritin levels were greater than 300 mug per liter in 78 of 89 men (88 percent) and greater than 200 microg per liter in 79 of 138 women (57 percent). Pacific Islanders and Asians had the highest geometric mean levels of serum ferritin and mean transferrin saturation despite having the lowest prevalence of C282Y homozygotes. There were 364 participants in whom iron overload had not been diagnosed (29 C282Y homozygotes) who had a serum ferritin level greater than 1000 microg per liter. Among men, C282Y homozygotes and compound heterozygotes were more likely to report a history of liver disease than were participants without HFE mutations.
The C282Y mutation is most common in whites, and most C282Y homozygotes have elevations in serum ferritin levels and transferrin saturation. The C282Y mutation does not account for high mean serum ferritin levels and transferrin saturation values in nonwhites.
Pulmonary arterial stenoses commonly occur in patients with congenital heart disease. Indications for treatment are based around reduction of right ventricular hypertension and equalization of flow ...to each lung and its constituent segments. There are many treatment approaches for these lesions depending on the anatomy, location and the age of the patient. Although surgical reconstruction remains an option, it is technically challenging and in many cases, the results are disappointing. In the modern era, the majority of these patients are treated with transcatheter therapy, including balloon angioplasty, and increasingly, the use of intravascular stents. In this article, we discuss these issues and deal with both the technical approach to transcatheter therapy and up-to-date results.
Objectives: To assess survival and long term arch patency rates in a consecutive group of children after extended arch repair for coarctation of the aorta. Methods: Review of 191 consecutive children ...(154 (81%) under 1 year of age) operated on between 1990 and 2002 by a single surgeon using extended arch reconstructive techniques. For assessment of survival patients were divided into three groups: 1, coarctation alone, n = 104; 2, coarctation and ventricular septal defect, n = 38; and 3, coarctation in association with complex intracardiac anomalies, n = 49. A prospective and systematic clinical and echocardiographic evaluation of the aortic arch was undertaken. Results: Median time to follow up was 4.2 years (range 1–10.6 years). Overall actuarial survival was 92%, 88%, and 88% at two, five, and 10 years. Mortality was significantly higher in those patients with complex intracardiac anatomy. Arch obstruction recurred in seven of 165 (4.2%) patients: four of 139 (2.9%) term and three of 10 (30%) premature infants (p < 0.001). Conclusions: Survival after extended arch reconstruction for coarctation is excellent. At long follow up recurrent arch obstruction is rare, with prematurity the only risk factor.
Total parenteral nutrition (TPN) causes intrahepatic cholestasis and membrane phospholipid changes. Fatty acid (FA) composition of bile and hepatocyte phospholipid is influenced by dietary FA ...composition. We hypothesized that altering FA composition of i.v. lipid emulsions modifies 1) severity of TPN-induced cholestasis; 2) hepatocyte membrane composition and function; 3) bile flow and composition. Newborn piglets received either sow's milk, TPN with i.v. soybean oil or TPN with i.v. fish oil (FO). After 3 wk, basal and stimulated bile flow were measured after bolus injections of 20, 50, and 100 micromol/kg of taurocholate (TCA). Bile was analyzed for bile acids, cholesterol, phospholipids, and phospholipid-FA. Sinusoidal and canalicular membrane PL-FA, fluidity, and Na+/K+-ATPase were measured. Although the soybean oil-fed animals developed cholestasis, the FO and milk group had similar liver and serum bilirubin. Basal and stimulated bile flow rates were impaired in the soybean oil but not in the FO group. Hepatocyte membrane FA composition reflected dietary FA. Changes in sinusoidal and canalicular membrane fluidity and sinusoidal Na+/K+-ATPase activity did not explain the effect of FO on TPN-induced cholestasis. Intravenous FO reduces TPN-induced cholestasis by unknown mechanisms.
We report on pediatric epicardial pacing activity, patient and lead survival for more than two decades in a single center.
The data cover 96 pacing leads implanted in 59 patients. Median age at ...implantation was 1.9 years (1 day to 18.2 years). Forty-four percent had structural cardiac disease. Most frequent indications for pacing were postoperative (42%) and congenital complete heart block (42%).
Median activity was 3 pacing leads per year; 326 patient pacing years were observed (median 11.9 years; range, 1.1 to 22 years). Death due to pacemaker failure occurred in a single patient. Lead failure occurred in 33 of 96 leads (median of 28 months postimplantation) with lead fracture the commonest cause (47%). Risk factors for lead failure were decade of implant and nonsteroid eluting leads. Acute implant energy thresholds were significantly lower for steroid than nonsteroid eluting leads but did not predict subsequent lead failure.
The epicardial approach has offered an effective solution to pacing problems in the pediatric age range. Lead survival has improved with more than 75% of modern steroid eluting leads surviving to 5 years.
To evaluate the safety and efficacy of transcatheter occlusion of the arterial duct without femoral arterial catheterization.
Patent arterial ducts have been closed percutaneously since the 1960s. It ...remains standard practice to use arterial access for aortography before, during, and after implantation of the device. Femoral arterial catheterisation has well recognised complications, and should be avoided unless absolutely necessary.
We reviewed prospectively collected data relating to 389 occlusions of the arterial duct performed consecutively between 1994 and 2004. We inserted Cook detachable coils in 288 instances using the Amplatzer duct occluder in the remaining 101. Information was obtained regarding procedural success, displacement of the device, and re-intervention. We have followed out patients for a median of 1.15 years in those closed with the Amplatzer device, and 1.09 years in those closed with a coil.
In the patients in whom we used coils, occlusion was possible in 75% using venous access alone. We reintervened in 25 patients, because of embolisation of the device in 6, haemolysis in 5, and residual shunting in 14. On follow-up, complete occlusion had been achieved in 98%. We found trivial stenosis of the left pulmonary artery in 3 patients. When using the Amplatzer device, closure using venous access alone was achieved in 82%, and 2 patients required reintervention because of embolisation of the device. Complete occlusion had been achieved in all patients as judged by follow-up at 1 year, and 2 patients had trivial stenosis of the left pulmonary artery.
Arterial catheterisation is unnecessary in the great majority of patients undergoing occlusion of the arterial duct. Use of venous catheterisation alone is safe, and does not appear to increase the risk of device-related complications.
During the Atlanta Supersite Project, four particle mass spectrometers were operated together for the first time: NOAA's Particle Analysis by Laser Mass Spectrometer (PALMS), University of California ...at Riverside's Aerosol Time‐of‐Flight Mass Spectrometer (ATOFMS), University of Delaware's Rapid Single‐Particle Mass Spectrometer II (RSMS‐II), and Aerodyne's Aerosol Mass Spectrometer (AMS). Although these mass spectrometers are generally classified as similar instruments, they clearly have different characteristics due to their unique designs. One primary difference is related to the volatilization/ionization method: PALMS, ATOFMS, and RSMS‐II utilize laser desorption/ionization, whereas particles in the AMS instrument are volatilized by impaction onto a heated surface with the resulting components ionized by electron impact. Thus mass spectral data from the AMS are representative of the ensemble of particles sampled, and those from the laser‐based instruments are representative of individual particles. In addition, the AMS instrument cannot analyze refractory material such as soot, sodium chloride, and crustal elements, and some sulfate or water‐rich particles may not always be analyzed with every laser‐based instrument. A main difference among the laser‐based mass spectrometers is that the RSMS‐II instrument can obtain size‐resolved single particle composition information for particles with aerodynamic diameters as small as 15 nm. The minimum sizes analyzed by ATOFMS and PALMS are 0.2 and about 0.35 μm, respectively, in aerodynamic diameter. Furthermore, PALMS, ATOFMS, and RSMS‐II use different laser ionization conditions. Despite these differences the laser‐based instruments found similar individual particle classifications, and their relative fractions among comparable sized particles from Atlanta were broadly consistent. Finally, the AMS measurements of the nitrate/sulfate mole ratio were highly correlated with composite measurements (r2 = 0.93). In contrast, the PALMS nitrate/sulfate ion ratios were only moderately correlated (r2 ∼ 0.7).
The bone morphogenetic receptor type II gene is the major genetic determinant for the inherited form of pulmonary arterial hypertension. However, deleterious mutations of this gene are not observed ...in the majority of subjects who develop the condition spontaneously and familial disease displays age- and sex-dependent penetrance, indicating the requirement for additional environmental and/or genetic modifiers for disease development.
We investigated polymorphic variation of the serotonin transporter gene, a biological candidate for predisposition to this vascular disorder.
No significant evidence of association between alleles of the serotonin transporter gene and pulmonary hypertension was detected, nor did we observe a relationship with age of onset in familial and idiopathic disease.
Variation of the serotonin transporter gene appears unlikely to confer significant susceptibility to pulmonary arterial hypertension. This study emphasizes the need for adequately powered cohorts for association analyses to identify not only genetic determinants of disease susceptibility but also inherited modifiers for disease development.
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