Simon Jong-Koo Lee

Temporal Variation of Birth Prevalence of Congenital Heart Disease in the United States

BACKGROUND This is a longitudinal analysis of the largest and most comprehensive inpatient care database in the United States to determine temporal variation of birth prevalence of congenital heart disease (CHD) diagnosis, adjusting for potentially confounding factors. METHODS We compared all entries of CHD diagnoses in the Nationwide Inpatient Sample (NIS) database in 1998 and 2008 to determine differences in birth prevalence of overall CHD and specific CHD phenotypes stratified by race, gender, socioeconomic status, and geographical location. RESULTS CHD prevalence was 10.2/1000 in 1998 and 10.8/1000 live births in 2008, without significant changes in prevalence (P = .09). Prevalence of isolated patent ductus arteriosus increased from 1.9 to 2.8 per 1000 (P < .001), and this temporal increase remained statistically significant after stratification by race, income status, and geographic location. Prevalence of mild CHD increased from 8.0 to 9.1 per 1000 (P = .01), with most of this increase occurring among Caucasians and the upper socioeconomic class. There was a decrease in prevalence of severe CHD from 1.5 to 0.9 (P = .03), while prevalence of moderate CHD and all other specific CHD phenotypes remained unchanged in both cohorts. CONCLUSIONS We report increased prevalence of isolated patent ductus arteriosus and mild CHD; decreased prevalence of severe CHD; and unchanged prevalence of overall CHD and all other specific CHD phenotype. We speculate that increased prevalence of mild CHD was due to increased case detection because of improvement in echocardiography. Decrease in diagnosis of severe CHD could be due to the impact of pregnancy termination.

Prevalence of congenital anomalies in newborns with congenital heart disease diagnosis.

Background: There is a known association between congenital heart disease (CHD) and other congenital anomalies (CA). These associations have been altered by changes in prenatal factors in recent time. We reviewed the largest database of inpatient hospitalization information and analyzed the current association between common CHD diagnoses and other congenital anomalies. Materials and Methods: Case-control study design. We reviewed the Nationwide Inpatient Sample (NIS) database from 1998 to 2008 and identified all live births with CHD diagnosis (case) and live births without CHD diagnosis (control). We compared prevalence of associated congenital anomalies between the case and control groups. Results: Our cohort consisted of 97,154 and 12,078,482 subjects in the case and control groups, respectively. In the CHD population, prevalence of non-syndromic congenital anomaly (NSCA), genetic syndrome (GS), and overall extra-cardiac congenital anomaly (CA) were 11.4, 2.2, and 13.6%, respectively. In the control group, prevalence of NSCA, GS, and CA were 6.7, 0.3, and 7.0%, respectively. NSCA (odds ratio (OR): 1.88, confidence interval (CI): 1.73-1.94), GS (OR 2.52, CI 2.44-2.61), and overall CA (OR: 2.01, CI: 1.97-2.14) were strongly associated with CHD. Prevalence of GS and multiple organ-system CA decreased significantly over the study period. Conclusions: This is the largest and most comprehensive population-based study evaluating association between CHD and extra-cardiac malformation (ECM) in newborns. There was significant decrease in prevalence of GS and multiple CA over the study period.

Congenital malformations in the newborn population: A population study and analysis of the effect of sex and prematurity

BACKGROUND Congenital malformation (CM) is a leading cause of infant mortality. We hypothesized that the current estimates of the prevalence of CM are obsolete because of the increased rate of terminating fetuses with severe CMs and the widespread use of prenatal vitamins. METHODS This population-based cross-sectional study analyzed the effect of sex and prematurity on CM prevalence. All data were derived from birth entries in the 2008 Nationwide Inpatient Sample (NIS) database. Our objectives were to determine the prevalence of CM diagnoses among all birth hospitalizations in 2008 and to analyze the effect of sex and gestational maturity on CM prevalence. RESULTS We identified 29,312 patients with CMs from among 1,014,261 live births, which yielded a CM prevalence of 28.9 per 1000 live births. Associated genetic syndromes were present in 1172 (4%) patients. Among newborns with nonsyndromic CM, 91% of newborns had an isolated CM and 9% of newborns had multiple CMs. The cardiovascular system was the most commonly involved organ system. The risk of CM was significantly higher in preterm newborns for an isolated CM [odds ratio (OR), 1.5; confidence interval (CI), 1.4-1.5]; multiple CMs (OR, 2.1; CI, 2.0-2.3); and overall CMs (OR, 1.4; CI, 1.3-1.5). Males had higher risk of isolated CMs (OR, 1.3; CI, 1.2-1.5). However, there was no sex difference in the risk of overall CM. CONCLUSION We reported up-to-date national estimates of the prevalence of CM, which is important for monitoring trends, determining service planning, and assessing disease burden because of congenital malformations in the United States of America. We also showed a strong association between CM and prematurity. Further study of this association is needed to provide insight into the etiology of these relatively common public health problems.

Infective Endocarditis and Phlebotomies May Have Killed Mozart

Thirty-five year-old Amadeus Mozart died in Vienna after an acute illness that lasted only 15 days but no consensus has been reached on the cause of his death. From many letters written by his farther it is almost certain that he experienced at least three episodes of acute rheumatic fever attack in his childhood, and a relapse of rheumatic fever was suggested to have killed Mozart, although death from acute rheumatic fever is very rare in adults. His last illness was characterized by high fever, massive edema, vomiting and skin rash. His last illness can be explained by infectious endocarditis and heart failure. During his last hours, he was given phlebotomy, possibly for the third time in two weeks, and soon after he became unconscious and died. As such, phlebotomy performed on a man dehydrated by high fever and vomiting may have caused systemic shock. In summary, Mozart probably died from chronic rheumatic heart disease complicated by infective endocarditis and heart failure, and repeated phlebotomy-induced hypovolemic shock.

Endoperoxides and Thromboxane A2 in Porcine Coronary Arteries with Regenerated Endothelium

AIM To determine the role of endothelium-derived contracting factor (EDCF) in the response to endothelin-1 in arteries with regenerated endothelium. METHODS Rings of porcine coronary arteries, with and without endothelium of previously deendothelialized left anterior descending coronary arteries and native left circumflex coronary arteries, were suspended in conventional organ chambers for the measurement of isometric force. RESULTS In quiescent rings of the previously deendothelialized left anterior descending coronary artery treated with the NO-synthase inhibitor nitro-L-arginine, endothelin-1 caused contractions which were larger in rings with than that in those without endothelium. Under the same experimental conditions, in the left circumflex coronary artery, the contractions to endothelin-1 were augmented markedly by the removal of the endothelium. In rings with endothelium of the previously deendothelialized left anterior descending coronary artery, indometacin (inhibitor of cyclooxygenase) and ridogrel (thromboxane A2 receptor antagonist and inhibitor of thromboxane synthase) inhibited contractions to endothelin-1. Dazoxiben (inhibitor of thromboxane synthase) inhibited, to the same extent as indometacin and ridogel, the response to higher concentrations of endothelin-1. The endothelium-dependent component of the response to lower concentrations of endothelin-1 was inhibited by indometacin and ridogrel, but not by dazoxiben. In rings without endothelium of both previously deendothelialized left anterior descending and native left circumflex coronary arteries, indometacin and ridogrel did not affect the contractions to endothelin-1. CONCLUSION These findings suggest that in regenerated endothelium, high concentrations of endothelin-1 stimulate the release of thromboxane A2. Endoperoxides generated by activation of endothelial cyclooxygenase may be the endothelium-derived contracting factor(s) released in regenerated endothelium by lower concentrations of the peptide.