Congenital Heart Disease最新文献

Background: Patients with Fontan physiology are predisposed to congestive hepatopathy, progressive liver fibrosis, and end-stage liver disease. Ultrasound-based shear wave elastography (SWE) is a non-invasive tool to diagnose and monitor liver fibrosis. We sought to determine whether the degree of hemodynamic derangement prior to and after the Fontan operation is associated with increased liver stiffness measured by SWE.

Methods: A single-center retrospective study of patients with Fontan circulation who underwent ultrasound elastography between 2008 and 2024 was conducted. Liver stiffness was measured by SWE and reported as velocity in m/s. Hemodynamic assessment with cardiac catheterization and echocardiograms were collected before and after the Fontan operation. Data was analyzed as a continuous (linear regression model) and a dichotomous variable (t-test).

Results: 78 post-Fontan ultrasound elastography studies performed in 56 patients were analyzed. Pre-Fontan hemodynamics included median effective pulmonary flow of 2.45 L/min/m² [2.21, 3.16] (p = 0.46), ventricular end-diastolic pressure of 10 mmHg [8, 10.5] (p = 0.77), and median Glenn pressure of 12 mmHg [13, 15] (p = 0.83). Post-Fontan median systemic cardiac index was 2.80 L/min/m² [2.4, 3.34] (p = 0.93), median ventricular end-diastolic pressure of 12 mmHg [13.5, 14] (p = 0.99), median systemic saturation of 93% [87, 96] (p = 0.77), median indexed pulmonary vascular resistance of 1.80 WU·m² [1.49, 2.37] (p = 0.93), and median Fontan pressure of 18 mmHg [16, 21] (p = 0.86). No correlation was found between SWE and hemodynamics. On echocardiography, no correlation was found between SWE and systemic ventricular systolic function (p = 0.35) or degree of systemic atrioventricular valve regurgitation (p = 0.35).

Conclusions: The degree of liver stiffness by SWE in this cohort did not correlate with pre- and post-Fontan hemodynamics on cardiac catheterization, degree of ventricular dysfunction, or severity of atrioventricular valve regurgitation by echocardiography.

Background: Given the pervasive issues of obesity and diabetes both in Puerto Rico and the broader United States, there is a compelling need to investigate the intricate interplay among BMI, pregestational, and gestational maternal diabetes, and their potential impact on the occurrence of congenital heart defects (CHD) during neonatal development.

Methods: Using the comprehensive System of Vigilance and Surveillance of Congenital Defects in Puerto Rico, we conducted a focused analysis on neonates diagnosed with CHD between 2016 and 2020. Our assessment encompassed a range of variables, including maternal age, gestational age, BMI, pregestational diabetes, gestational diabetes, hypertension, history of abortion, and presence of preeclampsia.

Results: A cohort of 673 patients was included in our study. The average maternal age was 26 years, within a range of 22 to 32 years. The mean gestational age measured 39 weeks, with a median span of 38 to 39 weeks. Of the 673 patients, 274 (41%) mothers gave birth to neonates diagnosed with CHD. Within this group, 22 cases were linked to pre-gestational diabetes, while 202 were not; 20 instances were associated with gestational diabetes, compared to 200 without; and 148 cases exhibited an overweight or obese BMI, whereas 126 displayed a normal BMI.

Conclusion: We identified a statistically significant correlation between pre-gestational diabetes mellitus and the occurrence of CHD. However, our analysis did not show a statistically significant association between maternal BMI and the likelihood of CHD. These results may aid in developing effective strategies to prevent and manage CHD in neonates.