Perioperative Anesthetic Management of the Pregnant Patient With Congenital Heart Disease Undergoing Cardiac Intervention: A Systematic Review.

Maternal congenital heart disease is increasingly prevalent, and has been associated with a significantly increased risk of maternal, obstetric, and neonatal complications. For patients with CHD who require cardiac interventions during pregnancy, there is little evidence-based guidance with regard to optimal perioperative management. The periprocedural management of pregnant patients with congenital heart disease requires extensive planning and a multidisciplinary teams-based approach. Anesthesia providers must not only be facile in the management of adult congenital heart disease, but cognizant of the normal, but significant, physiologic changes of pregnancy.

Intraperitoneal corticosteroids for recurrent ascites in patients with Fontan circulation: Initial clinical experience.

Individuals with single-ventricle congenital heart disease who are palliated to a Fontan circulation are at risk for heart failure and liver disease, with recurrent ascites being a potentially debilitating cause of late morbidity. Although ascites associated with heart failure or liver failure is usually characterized by a high serum-ascites albumin gradient (SAAG), we have observed multiple instances of ascites in Fontan patients with low SAAG, suggesting an inflammatory process. We present three cases in which recalcitrant ascites severely and adversely impacted the quality of life and describe our initial experience with intraperitoneal corticosteroids in this setting.

Transient elastography may identify Fontan patients with unfavorable hemodynamics and advanced hepatic fibrosis.

BACKGROUND: Transient elastography (TE) offers a noninvasive correlate with the degree of hepatic fibrosis. However, factors other than fibrosis affect liver stiffness. We sought to determine whether hepatic congestion related to hemodynamics in Fontan circulation influences liver stiffness measurement (LSM) assessed by TE. METHODS: We studied 45 subjects with Fontan circulation undergoing cardiac catheterization with or without simultaneous liver biopsy. Subjects underwent TE within 5 days before catheterization. Clinical history, hemodynamic and biopsy data, and hepatic biomarkers were collected. Five subjects who had previously undergone liver biopsy and TE were also included. RESULTS: Median age was 13.1 years (range 2.4-57.8); median time since Fontan was 9.9 years (range 0.1-32.5). No subject had known hepatitis C. Mean LSM for the entire cohort was 21.4 ± 10.8 kPa. Univariate regression analysis using LSM as a continuous outcome variable shows significant correlations with age (R = 0.35, P = .01), time since Fontan (R = 0.41, P = .003), Fontan pressure (R = 0.31, P = .04), cardiac index (R = 0.33, P = .03), pulmonary vascular resistance (R = 0.34, P = .03), systemic arterial oxygen saturation (R = 0.31, P = .04), and platelet count (R = 0.29, P = .05). On multiple regression analysis, Fontan pressure (ß = 0.901, P = .03) and cardiac index (ß = 2.703, P = .02) were significant predictors of LSM with overall model R(2) = 0.206. Univariate analysis shows LSM to be associated with more severe centrilobular fibrosis (P = .05). CONCLUSIONS: Higher LSM is associated with unfavorable Fontan hemodynamics and advanced centrilobular hepatic fibrosis. TE may be a useful tool for identifying Fontan patients who warrant invasive testing.

Acute dyspnea in a young man with an old sternotomy.

Described is a 42-year-old man with complex congenital heart disease with single-ventricle physiology who underwent a Fontan operation at 27 years of age. He presented with sudden-onset chest pain and dyspnea and was initially misdiagnosed with a pulmonary embolism. This case is described in order to highlight the challenges in the evaluation of common presentations in adults with congenital heart disease.