Perinatal outcome after prenatal diagnosis of single-ventricle cardiac defects.

OBJECTIVES: To investigate the perinatal outcome of cases with a prenatal diagnosis of single-ventricle cardiac defects, single ventricle being defined as a dominant right ventricle (RV) or left ventricle (LV), in which biventricular circulation was not possible. METHODS: We reviewed patients with a prenatal diagnosis of single-ventricle cardiac defects, made at one institution between 1995 and 2008. Cases diagnosed with double-inlet LV, tricuspid atresia, pulmonary atresia with intact ventricular septum and severe RV hypoplasia and those with hypoplastic left heart syndrome (HLHS) were included in the study population. Patients with HLHS were identified prenatally as being standard risk or high risk (HLHS with highly restrictive or intact atrial septum, mitral stenosis with aortic atresia and/or LV coronary artery sinusoids). Patients with an address over 200 miles from the hospital, diagnosed with heterotaxy syndrome or referred for fetal intervention, were excluded. RESULTS: We identified 312 cases of single-ventricle cardiac defect (208 dominant RV; 104 dominant LV) that were diagnosed prenatally. Most (96%) patients with a dominant RV had HLHS. Among the total 312 cases there were 98 (31%) elective terminations of pregnancy (TOP), 12 (4%) cases of spontaneous fetal demise, 12 (4%) cases lost to prenatal follow-up and 190 (61%) live births. Among the 199 patients that underwent fetal echocardiography before 24 weeks' gestation, there were 97 (49%) cases of elective TOP. There was no difference in prenatal outcome between those with a dominant RV and those with a dominant LV (P = 0.98). Of the 190 live births, five received comfort care. With an average of 7 years' follow-up (to obtain data on the Fontan procedure), transplantation-free survival was lower in those with a dominant RV than in those with a dominant LV (standard-risk HLHS odds ratio (OR), 3.0 (P = 0.01); high-risk HLHS OR, 8.8 (P < 0.001)). CONCLUSIONS: The prenatal outcome of cases with single-ventricle cardiac defects was similar between those with a dominant RV and those with a dominant LV, however postnatal intermediate-term survival favored those with a dominant LV. High-risk HLHS identified prenatally was associated with the lowest transplantation-free survival.

The signs and symptoms of Ernest Shackleton.

Ernest Shackleton, an accomplished Antarctic explorer, developed a life-threatening illness during the Discovery Antarctic expedition of 1901-4. His documented signs and symptoms included inflamed gums attributed to scurvy, severe dyspnea, and exercise intolerance, presenting in a setting of nutritional deficiency. Physical examinations at a later date, also following a prolonged diet of limited fresh food, revealed a pulmonary systolic murmur. Thiamine deficiency with cardiomyopathy, either alone or subsequently exacerbated by advanced scurvy, may have been a prominent cause of Shackleton's condition.

Racial and ethnic disparities in mortality following congenital heart surgery.

Our objective was to assess risk-adjusted racial and ethnic disparities in mortality following congenital heart surgery. We studied 8483 congenital heart surgical cases from the Kids' Inpatient Database 2000. Black sub-analysis was performed using predetermined regional categories. For our Hispanic sub-analyses, we categorized Hispanics into state groups according to a state's predominant Hispanic group: West (Mexican-American), Southeast (Cuban-American), Northeast (Puerto Rican), and Mixed/Heterogeneous. Risk adjustment was performed using the Risk Adjustment for Congenital Heart Surgery method. Multivariate analyses assessed the effect of race/ethnicity and Hispanic state group on mortality and explored the effects of gender, income, insurance type, and region. Black children had a higher risk for death than Whites odds ratio (OR), [1.65; p = 0.003]. Hispanics and the Cuban-American state group showed a trend toward a higher death risk (Hispanic: OR, 1.24; p = 0.16; Southeast Cuban-American: OR 1.55; p = 0.08). Disparities were not influenced by insurance. Among Blacks, disparities were greatest in the Northeast region (OR, 2.25; p = 0.007). After adjusting for gender, income, and region, Blacks (OR, 1.76; p = 0.002) and Hispanics (OR, 1.34; p = 0.05) had a higher death risk. Racial and ethnic disparities in risk-adjusted mortality following congenital heart disease exist for Blacks and Hispanics. These disparities are not due to insurance but are partially explained by gender and region.

Mice lacking E-selectin show normal numbers of rolling leukocytes but reduced leukocyte stable arrest on cytokine-activated microvascular endothelium.

OBJECTIVE: Previous work indicated that E-selectin mediates transient interactions between leukocytes and cytokine-activated endothelium in vitro. Here we examine the role of E-selectin in blood leukocyte interactions with microvascular endothelium in vivo. METHODS: E-selectin-deficient (E-/-) mice were produced by gene targeting. The effect of this null mutation on leukocyte-endothelial interactions was determined by intravital microscopy before and 4 to 5 hours after local administration of the proinflammatory cytokine tumor necrosis factor alpha (TNF alpha) in dermal microvessels with low blood flow (dorsal skin-fold chambers, intact ear skin), and after endotoxin activation in exteriorized mesenteric microvessels with higher blood flow. RESULTS: E-/- mice were viable, fertile with normal circulating leukocyte and platelet profiles. Approximately 60% of circulating leukocytes rolled in dermal microvessels of both normal (E+/+) and E-/- mice without inflammatory stimulation. After local administration of TNF alpha, rolling increased modestly and equivalently in both genotypes. The main effect of TNF alpha was a dramatic increase in leukocyte stable adhesion and, unlike rolling, this manifestation of endothelial activation was significantly reduced in E-/- animals. This reflected fewer dermal microvessels supporting higher adhesion densities in E-/- mice, and a similar trend was observed in mesenteric microvessels. CONCLUSIONS: E-selectin plays a previously unappreciated role in facilitating and/or mediating stable adhesion of leukocytes to inflamed microvascular endothelium.