RESUMO
OBJECTIVE: To assess patterns of postnatal ventricular function and their relationship to prenatal and postnatal markers of disease severity in infants with congenital diaphragmatic hernia (CDH). STUDY DESIGN: In this observational case-control study of cardiac function in infants with CDH in the first 5 days of life, systolic and diastolic function in the right ventricle (RV) and left ventricle (LV) were assessed using speckle tracking echocardiography-derived global strain and tissue Doppler imaging. Correlation between cardiac function and prenatal observed:expected total fetal lung volume (TFLV), oxygenation index (OI), duration of intubation, and hospital length of stay were assessed. RESULTS: All measures of systolic and diastolic function were significantly reduced in the CDH group (n = 25) compared with controls (n = 20) at <48 hours, and were improved by 72-120 hours. LV global systolic longitudinal strain (GLS) correlated with prenatal TFLV (R2 = 0.32; P = .03), OI (R2 = 0.35; P < .001), duration of intubation (R2 = 0.24; P = .04), and length of stay (R2 = 0.4; P = .006). Mean (SD) LV GLS at <48 hours was significantly lower in infants with CDH who did not survive and/or required ECMO compared with those who did not: -11.5 (5.3)% vs -16.9 (5.3)% (P = .02). CONCLUSIONS: RV and LV function are impaired in the transitional period in infants with CDH. Early LV systolic function correlates with prenatal and postnatal markers of clinical disease severity and may be an important determinant of disease severity and therapeutic target in CDH. These findings support regular assessment of cardiac function in CDH and investigational trials of targeted cardiovascular therapies.
Assuntos
Hérnias Diafragmáticas Congênitas/complicações , Disfunção Ventricular Esquerda/complicações , Disfunção Ventricular Direita/complicações , Estudos de Casos e Controles , Diástole , Ecocardiografia , Atresia Esofágica/patologia , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Tempo de Internação , Pulmão/fisiologia , Masculino , Oxigênio/química , Artéria Pulmonar/fisiopatologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Sístole , Ultrassonografia Doppler , Função Ventricular EsquerdaRESUMO
OBJECTIVE: We evaluated the long-term outcome of neonates receiving extracorporeal membrane oxygenation (ECMO) for congenital diaphragmatic hernia (CDH). Study design A retrospective review of all 73 neonates with CDH supported with ECMO in the United Kingdom between 1991 and 2000, with follow-up to January 2003. Information was from hospital charts and from communication with family doctors and pediatricians. Median follow-up period for survivors was 67 months. RESULTS: 46 infants (63%) were weaned from ECMO, 42 (58%) survived to hospital discharge, and 27 (37%) survived to age 1 year or more. A higher birth weight, higher 5-minute Apgar score, and postnatal diagnosis were "pre-ECMO" predictors of long-term survival. Comorbidity was common in long-term survivors: 13 (48%) had respiratory symptoms, 16(59%) had gastrointestinal problems, and 6 (19%) had severe neurodevelopmental problems. Only 7 children were free of significant neurodevelopmental deficit and required no further medical or surgical intervention. CONCLUSION: Using the current referral criteria, ECMO can be used to support the sickest neonates with CDH. However, there is significant mortality in the first year of life, and long-term physical and neurodevelopmental morbidity remains in the majority of survivors.