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James J Joyce

Publications and source records attributed to James J Joyce.

7 recordsLinked to original sources
PubMed2006

Complications of non-compaction of the left ventricular myocardium in a paediatric population: a prospective study.

AIMS: Non-compaction of the left ventricular myocardium (NCVM) is reportedly exceedingly rare and associated with a high morbidity and mortality. A different genetic background has been suggested for NCVM with [non-isolated NCVM (ni-NCVM)] and without [isolated NCVM (i-NCVM)] other congenital heart defects. We prospectively evaluated both the NCVM subgroups regarding frequency of occurrence and cardiovascular complications in a paediatric population. Results In a prospective, single-centre study, 66/5220 consecutive patients (1.26%) were diagnosed (25 i-NCVM, 41 ni-NCVM). The median age was 4 years (range 0-21), the median follow-up 12 months (range 0-51). The occurrence of congestive heart failure (CHF) at follow-up was 68.0%. CHF was as frequently seen in i-NCVM and ni-NCVM patients (77.5 vs. 62.1%, P = 0.322). The occurrence of arrhythmias (20.0%) and thrombo-embolic events (13.9%) was not different between subgroups. The cardiomyopathy related mortality was 7.1%, with three and one deaths in the i-NCVM and ni-NCVM groups, respectively (P = 0.126). CONCLUSION: When prospectively evaluated, NCVM appears to have been previously under-diagnosed. Whereas arrhythmias and thrombo-embolic events were rare, CHF was frequently found. An equally aggressive anticongestive treatment regimen would seem indicated for both the NCVM subgroups.

Adolescent↗
PubMed2006

Right ventricular abnormalities in sickle cell anemia: evidence of a progressive increase in pulmonary vascular resistance.

OBJECTIVE: To assess the effects of sickle cell anemia (SCA) on the right ventricle (RV). STUDY DESIGN: Echocardiograms of 32 children with SCA were compared with age-matched healthy controls. RV measurements included diastolic area index, fractional area change, free-wall mass index, ejection time corrected for heart rate (ET(c)), and tricuspid regurgitation (TR) gradient. RESULTS: SCA subjects had elevated RV ETc (mean +/- standard deviation, 0.369 +/- 0.030 sec vs 0.351 +/- 0.022 sec; P < .01), diastolic area index (19.9 +/- 2.4 cm(2)/m(2) vs 13.2 +/- 2.1 cm(2)/m(2); P < .01) and free-wall mass index (33.2 +/- 4.4 g/m(2) vs 23.9 +/- 4.3 g/m(2); P < .01), whereas RV fractional area change (37 +/- 8% vs 36 +/- 4%) was not different from controls. Although RV diastolic area index in SCA paralleled the normal range over time, RV free-wall mass index continued to gradually rise throughout childhood (r = .42; P < .05). TR gradients > 2.5 m/sec, consistent with pulmonary hypertension, were found in 5 (16%) of SCA subjects, all older than 9 years. CONCLUSIONS: RV preload and systolic function do not worsen during childhood in SCA; however, RV mass index and the prevalence of pulmonary hypertension increase consistent with rising pulmonary vascular resistance.

Adolescent↗
PubMed2004

Normal right and left ventricular mass development during early infancy.

The average newborn's right ventricular mass, indexed to body surface area, is approximately 20% above the adult level, consistent with in utero physiologic hypertrophy. In contrast, the left ventricular mass index is underdeveloped by about 30% at birth, resulting in a ratio of right to left ventricular mass that is nearly 75% higher than at maturity. Left ventricular mass index increases rapidly during the first 2 weeks of life and then more slowly thereafter, whereas the right ventricular mass index decreases steadily during the first 4 months of life.

Adult↗
PubMed2004

Echocardiographic assessment of the right ventricular stress-velocity relationship under normal and chronic overload conditions.

UNLABELLED: The effects of chronic volume or pressure overload on the velocity of right ventricular ejection have not been previously well defined. We hypothesized that, as formerly shown for the left ventricle, there would be a direct relationship between the velocity of ejection and an estimate of systolic wall stress. METHODS: Echocardiograms of asymptomatic patients, not on cardiac medications, with either an isolated secundum atrial septal defect > or = 5 mm in diameter or isolated pulmonic stenosis with a peak instantaneous pressure gradient > or = 20 mmHg, were reviewed. Forty-one patients with an atrial septal defect and 34 with pulmonary stenosis met criteria, and were compared to age-matched normal controls. Total subjects were 127 with ages ranging from 1 day to 54 years. Right ventricular monoplane ejection fraction, ejection time corrected for heart rate (ETc), mean normalized systolic ejection rate (MNSERc) and meridianal peak-systolic wall stress (WSps) were measured. RESULTS: Compared to controls, ejection fractions were not significantly different, but WSps averaged 81% and 110% higher, ETc 8% and 9% longer, and MNSERc 5% and 9% slower in the atrial septal defect and pulmonary stenosis groups, respectively. Among all subjects WSps had a significant linear correlation with ETc (r = 0.61, P < 0.01), MNSERc (r =-0.46, P < 0.01), and ejection fraction (r =-0.19, P < 0.05). CONCLUSIONS: Increases in WSps cause an incremental slowing of MNSERc in the right ventricle, with a relationship that is linear over a wide range of normal and abnormal loading conditions.

Adolescent↗
PubMed2004

Parental preference regarding hospitals for children undergoing surgery: a trade-off between travel distance and potential outcome improvement.

OBJECTIVE: To explore parental preference in the choice between a local and a referral hospital for children undergoing heart surgery. METHODS: One hundred three parents or adult primary caregivers of children referred to a pediatric cardiology clinic were interviewed. Participants were presented with hypothetical scenarios in which they or their children had a heart condition requiring elective surgery. The surgery could be performed at either a local hospital or a regional referral hospital. The travel time to the referral hospital was initially presented as 2 h, and the mortality rate was set at 3% for both the local and the referral hospitals. The parents were then presented with scenarios that sequentially increased the mortality of the local hospital and the distance to the referral hospital, and were asked to choose between the local and regional referral hospitals. RESULTS: When the regional referral hospital was 2 h away and the mortality rates for the referral hospital and the local hospital were equal at 3%, 82.5% of participants chose the local hospital for their children. The percentage of participants choosing the local hospital decreased progressively as the mortality rate of the local hospital increased (to 9.7% at 18% mortality). Between 5% and 10% more participants chose the local hospital when the distance to the referral hospital was increased from 2 h to 4 h. There was no difference in age, sex, ethnicity, language, type of insurance, level of education and availability of personal transportation between participants who chose the regional referral hospital and those who chose the local hospital. Participants who lived closer to the hospital at which the survey was conducted were more likely to choose the local hospital. CONCLUSION: The present study defines a relation between potential outcome improvement and increasing travel distance from a patient or parent's perspective. This trade-off is an important consideration when planning for regionalization.

Adolescent↗
PubMed2003

Parental preference for one-stage versus two-stage surgical repair for children with congenital heart disease.

BACKGROUND: Little is known regarding parental preference for a one-stage complete repair versus a two-staged approach with initial palliation, followed by repair, of the congenital cardiac malformation. METHODS: We interviewed 103 parents of healthy children referred to a clinic for pediatric cardiology. Participants were presented with a hypothetical scenario in which their children had a cardiac lesion requiring surgery. The surgery could be performed either by means of one-stage complete repair, or using a two-stage approach, with palliation first followed by complete repair a year later. The mortality rate for the one-stage repair was set at 5%. Participants were asked to choose between the one- and two-stage approaches, with differing mortality rates for the two-stage approach. The scenarios included options when the two-stage combined mortality rate was lower than the one-stage mortality, and the first stage mortality rate was at 1% and 3%, and when the two-stage combined mortality rate was the same as that for one-stage mortality, these being set at 1% and 3%. RESULTS: When the two-stage combined mortality rate was lower than that of the one-stage repair, participants were more likely to choose the two-stage approach if the first stage mortality rate was 1% as compared to 3% (57% and 44%, respectively, p = 0.04). When the two-stage combined mortality rate was the same as the one-stage approach, participants choosing the two-stage approach when the mortality rate was set at 1%, and when it was raised to 3%, were not significantly different (42% and 34%, respectively, p = 0.24). When the combined two-stage mortality was the same as that set for one-stage repair, participants with no insurance were less likely to choose the two-stage approach than those covered by insurance (p = 0.03). CONCLUSIONS: In the chosen scenarios, when the mortality for a two-stage combined approach is the same as that for one-staged repair, more parents choose the one-staged repair. If the two-stage combined mortality is lower than that for one-staged repair, parents are more likely to choose the two-stage repair if the mortality for the first stage is lower. When the mortality rates for the one-stage and two-stage approaches are the same, people without insurance are more likely to choose one-staged repair.

Adult↗