ABSTRACT
OBJECTIVES: To describe the scope of left ventricular (LV) dysfunction and left heart hypoplasia (LHH) in infants with congenital diaphragmatic hernia (CDH), to determine associations with CDH severity, and to evaluate the odds of extracorporeal membrane oxygenation (ECMO) and death with categories of left heart disease. STUDY DESIGN: Demographic and clinical variables were collected from a single-center, retrospective cohort of patients with CDH from January 2017 through May 2022. Quantitative measures of LV function and LHH were prospectively performed on initial echocardiograms. LHH was defined as ≥2 of the following: z score ≤ -2 of any left heart structure or LV end-diastolic volume <3 mL. LV dysfunction was defined as shortening fraction <28%, ejection fraction <60%, or global longitudinal strain <20%. The exposure was operationalized as a 4-group categorical variable (LV dysfunction +/-, LHH +/-). Logistic regression models evaluated associations with ECMO and death, adjusting for CDH severity. RESULTS: One hundred eight-two patients (80.8% left CDH, 63.2% liver herniation, 23.6% ECMO, 12.1% mortality) were included. Twenty percent demonstrated normal LV function and no LHH (LV dysfunction-/LHH-), 37% normal LV function with LHH (LV dysfunction-/LHH+), 14% LV dysfunction without LHH (LV dysfunction+/LHH-), and 28% both LV dysfunction and LHH (LV dysfunction+/LHH+). There was a dose-response effect between increasing severity of left heart disease, ECMO use, and mortality. LV dysfunction+/LHH + infants had the highest odds of ECMO use and death, after adjustment for CDH severity [OR (95% CI); 1.76 (1.20, 2.62) for ECMO, 2.76 (1.63, 5.17) for death]. CONCLUSIONS: In our large single-center cohort, patients with CDH with LV dysfunction+/LHH + had the highest risk of ECMO use and death.
Subject(s)
Extracorporeal Membrane Oxygenation , Hernias, Diaphragmatic, Congenital , Ventricular Dysfunction, Left , Humans , Hernias, Diaphragmatic, Congenital/complications , Hernias, Diaphragmatic, Congenital/mortality , Hernias, Diaphragmatic, Congenital/therapy , Male , Female , Retrospective Studies , Ventricular Dysfunction, Left/mortality , Infant, Newborn , Infant , Echocardiography , Severity of Illness IndexABSTRACT
Se presenta el caso clínico de una gestante de 30 años de edad con antecedentes de captación tardía a las 20 semanas de gravidez, por lo que no se le realizaron la alfa-fetoproteína y la ecografía del primer trimestre. Posteriormente, se le efectuó la pesquisa ultrasonográfica del segundo trimestre en el Hospital General "Alberto Fernández Montes de Oca" del municipio de San Luis en Santiago de Cuba y se observó un embarazo gemelar bicoriónico biamniótico y la presencia de una anomalía en uno de los fetos: hipoplasia de las cavidades cardíacas izquierdas, cuyo diagnóstico fue confirmado a través de una ecocardiografía fetal en el Cardiocentro de esta provincia.
The case of a 30 year-old pregnant woman with history of first prenatal visit at 20 weeks of pregnancy is reported, reason why alpha fetoprotein test and ultrasound of the first trimester were not made. Subsequently, ultrasound scan of the second trimester was performed at "Alberto Fernández Montes de Oca" General Hospital of San Luis municipality in Santiago de Cuba, and a bichorionic biamniotic twin pregnancy and the presence of an anomaly in one fetus were found: hypoplasia of the left heart, diagnosis of which was confirmed through a fetal ultrasound at the Heart Center of this province.