Congenital heart disease never goes away, even when it has been 'treated': the adult with congenital heart disease

PURPOSE OF REVIEW: As the specialties of pediatrics and pediatric cardiology continue to forge ahead with better diagnoses, medical care, and surgical results, an expanding population of patients with congenital heart disease (CHD) outgrows the pediatric age group, yet does not quite graduate to routine adult cardiology or general medicine. The adult with congenital heart disease (ACHD) faces medical, surgical, and psychosocial issues that are unique to this population and must be addressed as such. This review attempts to discuss and highlight some of the important advances and controversies brought up in the past year, in the care and management of these patients. RECENT FINDINGS: The past five to 10 years have seen dynamic interest in understanding sequelae of corrected, uncorrected, or palliated congenital heart disease. The search for the ideal surgery, optimal prosthesis, and a smooth transition to adult care continues and is reflected in the vast amount of academic work and publications in this field. Of particular interest, conduit reoperations and single ventricle pathway modifications are still an art and a science in evolution. SUMMARY: While all are agreed that there is a pressing need to focus on the delivery of care to the adult with congenital heart disease, this essentially requires a clearer understanding of late sequelae of CHD. The sheer heterogeneity of anatomy, age, surgery, and institutional management protocols can make it difficult to develop clear guidelines. This review attempts to give an up-to-date perspective on some of the new findings related to the more common lesions and problems faced in this group.     

Trends in state-specific prevalence of overweight and underweight in 2- through 4-year-old children from low-income families from 1989 through 2000

OBJECTIVES: To document overweight and underweight state-specific prevalence and examine trends among 2- through 4-year-old children from low-income families. METHODS: State-specific and overall overweight and underweight prevalence for 1989, 1994, and 2000 and trend analyses during the study period are documented. Overweight was defined as a sex-specific body mass index (BMI) for age in the 95th percentile or higher and underweight as a sex-specific BMI for age in less than the fifth percentile on the 2000 Centers for Disease Control and Prevention (CDC) growth charts. These analyses are based on one randomly selected record per child per year for 30 states consistently participating in the CDC Pediatric Nutrition Surveillance System in 1989, 1994, and 2000. Prevalence in 1989 and 1994 is adjusted to state-specific age and race/ethnicity distribution of the population in 2000. Overweight and underweight prevalence were categorized as 5% or less, more than 5% to 10%, more than 10% to 15%, more than 15% to 20%, and more than 20%. RESULTS: The number of states that reported overweight prevalence of more than 10% increased from 11 in 1989 to 28 in 2000. Underweight decreased during the study period: 9 states in 1989 and 23 states in 2000 had a prevalence of 5% or less. No geographic predominance was apparent. Trend analyses showed significant increases in overweight in 30 states (P < .01) and decreases in underweight in 26 states (P < .05). CONCLUSIONS: Overweight is increasing and underweight is decreasing in our study population. We need to expand prevention and intervention efforts to reverse the rising trend of overweight in the United States.     

Notfallmäßiger freier mikrovaskulärer Lappentransfer zum Verschluss eines Pharynxdefektes nach akuter Arrosionsblutung der A. carotis

We report on a case of an emergency free flap cover of a pharyngeal defect which was made necessary by aggressive digestive salivatory and radiation effects 3 weeks after neck-dissection and laser ablation of an epidermoid left tonsil carcinoma. Life threatening intraoral bleeding resulted from the erosion of branches of the external carotid artery. After management of the bleeding, massive blood transfusions and restoration of the patient's general condition, a sandwich patch cover of the transmural pharyngeal defect was achieved using a microvascular lateral arm flap. This aimed at preventing further digestive effects and bacterial colonisation of the neurovascular structures at the carotid triangle. Unfortunately, due to external mechanical forces, the flap became avascular and thus necrotic at the eighth postoperative day; however, until the successful replacement by a myocutaneous latissimus dorsi flap it remained water-tight and fulfilled its sealing task.     

Quantitative analysis of fungal DNA in chronic rhinosinusitis

OBJECTIVES/HYPOTHESIS: Fungi have been recognized as important pathogens in sinusitis; however, they are equally present in patients with and without sinusitis. The authors postulated that the quantity of fungal DNA in the nose is determinant of disease, is greater in patients with chronic rhinosinusitis, and is directly correlated to their quality of life. STUDY DESIGN: Prospective recruitment of patients with chronic rhinosinusitis. METHODS: Objective quality of life data were collected using three validated questionnaires: the Sinonasal Outcomes Test (SNOT-20), Medical Outcomes Short-Form 36 Survey (SF-36), and Guy Marks Asthma Questionnaire (GMAQ). Endoscopically guided middle meatus mucosal samples were collected from patients with chronic rhinosinusitis and normal control subjects. Fungal-specific polymerase chain reaction was performed on each sample. Every fungal-positive sample underwent fungal-specific quantitative polymerase chain reaction analysis. Statistical analysis was used to correlate fungal DNA quantities with outcomes indices between groups. RESULTS: Patients with chronic rhinosinusitis had a mean SNOT-20 index of 32.0 as compared with a SNOT-20 index of 17.3 (P <.01) in the normal control subjects. There were no statistical differences between the groups' indices for the SF-36 or GMAQ outcomes questionnaires. Four of 19 (21.1%) patients with chronic rhinosinusitis and 7 of 19 (36.8%) normal control subjects had positive findings for fungal DNA using polymerase chain reaction. The median relative quantity of fungal DNA to human DNA for chronic rhinosinusitis and control samples was identical (0.13) using quantitative polymerase chain reaction. CONCLUSION: The quantity of fungal DNA in the middle meatus did not differ in patients with and without chronic rhinosinusitis and was not correlated with quality of life outcomes. Therefore, the quantity of fungi does not explain pathogenicity in patients with chronic rhinosinusitis. However, because of small sample size, the study must be replicated in a larger patient population.     

Choice and dose of corticosteroid for antenatal treatments

Although antenatal glucocorticoids are standard of care for women at risk of preterm delivery before 32 to 34 weeks' gestation, the choice and dosing of the corticosteroid has not been standardized. An analysis of the trial data demonstrates that the risk of neonatal death is decreased with betamethasone, but not dexamethasone. Other clinical data also indicate that betamethasone is the drug of choice for antenatal treatment. The pharmacology of the corticosteroids suggests that a lower total glucocorticoid dose per treatment may be as effective as the current treatment recommendations. However, a change from current practice will require further randomized controlled trials.     

Prospective echocardiographic evaluation of atrioventricular conduction in fetuses with maternal Sjögren's antibodies

OBJECTIVE: Fetal complete atrioventricular block (AVB) occurs in 2-5% of Sj?gren's antibodies (SSA/SSB)-positive pregnancies with substantial morbidity and mortality. We evaluated the incidence of 1 degrees and 2 degrees AVB by measuring Doppler-derived mechanical PR intervals. STUDY DESIGN: We compared mechanical PR intervals of fetuses of SSA/SSB positive mothers referred to a single cardiology center between 1997 and 2003 with control fetuses and fetuses with 1 degrees or 2 degrees AVB confirmed by magnetocardiography or postnatal electrocardiogram. RESULTS: One hundred thirty-nine fetal echocardiograms performed on 59 SSA/SSB-positive pregnant women at 24.3 +/- 5.0 weeks gestation were compared with 150 controls. Mechanical PR intervals of the study group (120.5 +/- 9.8 milliseconds) and controls (120.6 +/- 8.7 milliseconds) were the same but differed significantly from fetuses with 1 degrees and 2 degrees AVB (191.5 +/- 29.6 msec). CONCLUSION: The incidence of 1 degrees and degrees 2 fetal AVB in SSA/SSB-positive pregnancies is low and can be identified by abnormal mechanical PR interval.     

In vivo feasibility of image-guided transvaginal focused ultrasound therapy for the treatment of intracavitary fibroids

OBJECTIVE: To determine the feasibility of uterine tissue ablation in vivo using a transvaginal focused ultrasound applicator guided by ultrasound imaging. DESIGN: Randomized in vivo animal study. SETTING: Academic research environment. ANIMAL(S): Healthy anesthetized sheep. INTERVENTION(S): Uterine treatment location was determined using a computerized targeting system. Five sonications 10 seconds in duration and averaging 2,000 W/cm(2) of focal ultrasound intensity were applied in each animal's uterus. Animals were euthanized either immediately or 2, 7, or 30 days post-treatment. MAIN OUTCOME MEASURE(S): Gross and microscopic analysis of the dissected uterus was used to quantitatively and qualitatively determine the ablated region and treatment side effects. RESULT(S): Treatments resulted in coagulative necrosis. Histopathological analysis showed that over 7 days, inflammatory cells appeared and smooth muscle bundles regenerated. By day 30, treated tissues healed and scar tissue formed. None of the animals showed abnormal behavior or medical problems. Complications in three animals were damage to the vaginal wall and colon, possibly due to inadequate applicator cooling and an empty bladder during treatment. CONCLUSION(S): Transvaginal image-guided high-intensity focused ultrasound has potential for treating uterine fibroids. Further safety testing of this treatment will prepare it for human use.     

The rising incidence of childhood type 1 diabetes and reduced contribution of high-risk HLA haplotypes

The incidence of childhood type 1 diabetes has risen over the past 50 years. We compared the frequency of HLA class II haplotypes in 194 patients diagnosed more than 50 years ago and 582 age-matched and sex-matched individuals diagnosed between 1985 and 2002. The proportion of high-risk susceptibility genotypes was increased in the earlier cohort (p=0.003), especially in those diagnosed at age 5 years or younger, which is consistent with the hypothesis that the rise of type 1 diabetes is due to a major environmental effect.