Post-natal prognostic factors in CDH: experience of 11 years in a referral center in Brazil

Objective: To describe post-natal risk factors associated with death in Newborns (NB) with Congenital Diaphragmatic Hernia (CDH) in a Brazilian reference center. Methods: In this retrospective cohort study, post-natal clinical factors of all NB diagnosed with CDH were reviewed in an 11-year period (2007‒2018). The primary outcome was death. Secondary outcomes included clinical features, prognostic indexes, type of mechanical ventilation, complications during hospitalization and surgical repair. Results: After applying the exclusion criteria, the authors analyzed 137 charts. Overall mortality was 59% (81/137), and the highest rates were observed for low-birth-weight NB (87%), syndromic phenotype (92%), and those with major malformations (100%). Prognostic indexes such as Apgar, SNAPPE-II and 24hOI (best oxygenation index in 24 hours) were all associated with poor evolution. In a multivariate analysis, only birth weight and 24hOI were statistically significant risk factors for mortality, with a reduction in mortality risk of 17.1% (OR = 0.829, 95% IC 0.72‒0.955, p = 0.009) for each additional 100g at birth and an increase by 26.5% (OR = 1.265, 95% IC 1.113‒1.436, p = 0.0003) for each unitary increase at the 24hOI. Conclusion: Prognostic indexes are an important tool for predicting outcomes and improving resource allocation. Post-natal risk factors may be more suitable for settings where antenatal diagnosis is not universal. Classical risk factors, such as prematurity, low birth weight, higher need for supportive care, and poorer prognostic indexes were associated with mortality in our CDH population

Congenital diaphragmatic hernia: postnatal factors associated with prognosis

Apesar dos avanços terapêuticos obtidos nas últimas décadas, a Hérnia Diafragmática Congênita (HDC) ainda é uma doença com elevada mortalidade. A ampla variedade de apresentações fenotípicas e os diferentes graus de comprometimento cardiopulmonar estão associados a diferentes desfechos. Realizamos o presente estudo com o objetivo de descrever e analisar fatores pósnatais relacionados ao mau prognóstico destes recém-nascidos (RN). Efetuamos estudo retrospectivo baseado em dados registrados de 146 RN com HDC internados na Instituição num período de 11 anos. Incluímos 137 pacientes. Três análises foram executadas: a primeira, que avaliou o perfil destes RN quanto a variáveis clínicas e sua associação ao óbito; a segunda, que buscou destacar o grupo de pacientes com cardiopatia congênita, frequente tipo de malformação (MF) associada à doença, avaliando suas características e comparando-as ao grupo semtal condição; e a terceira, que objetivou avaliar o subgrupo de pacientes sem alteração anatômica cardíaca, que haviam realizado ao menos um ecocardiograma nas primeiras 72 horas de vida, buscando descrever os dados encontrados ecorrelacioná-los a variáveis clínicas frente o desfecho de morte. Como principais resultados, encontramos maior associação da prematuridade, do baixo peso ao nascer e da coexistência de outras MF ao óbito. O grupo de cardiopatas apresentou ainda maiores taxas de mortalidade, com tendência a menores idades gestacionais (IG) e pesos de nascimento (PN), assim como maior associação a outras MF e fenótipo sindrômico. Dentre as cardiopatias encontradas, asrelacionadas à obstrução da via de saída do coração esquerdo estiveram presentesem metade dos casos. Quando avaliados os pacientes sem cardiopatia e com ecocardiograma, reduções funcionais e diametrais ventriculares esquerdas bem como a maior gravidade da hipertensão pulmonar foram fatores contribuintes aopior desfecho. Em relação aos índices prognósticos estudados, piores pontuações no índice de oxigenação das primeiras 24 horas de vida (IO 24h) tiveram relevante associação à mortalidade, inclusive na análise multivariada. Concluímos assim que determinados fatores pós-natais, índices prognósticos comumente utilizados nos cuidados intensivos neonatais e exames como o ecocardiograma podem fornecer importantes bases para nortear as perspectivas de vida destes RN, fornecendo maior substrato para antepor metas e limites de propostas terapêuticasDespite the therapeutic advances obtained in recent decades, Congenital Diaphragmatic Hernia (CDH) is still a disease with high mortality. The wide variety of phenotypic presentations and different degrees of cardiopulmonary involvement are associated with distinct outcomes. We carried out the present study with the aim of describing and analyzing postnatal factors related to the grim prognosis of these newborns (NB). We performed a retrospective study based on recorded data from 146 NB with CDH, who were admitted to the Institution over an 11-year period. 137 patients were included. Three analyses were performed: the first one assessed the profile of these NB regarding clinical variables and their association with death; the second one sought to highlight the group of patients with congenital heart disease, a frequent type of malformation (MF) associated with the disease, evaluating its characteristics and comparing them to the group without such condition; and thethird one aimed at evaluating the subgroup of patients without cardiac anatomical changes, who had undergone at least one echocardiogram in the first 72 hours of life, seeking to describe the data collected, and to correlate them with clinicalvariables regarding death as an outcome. As main results, we found a greater association between prematurity, low birth weight, the coexistence of other MF, and death. The group of patients with heart disease also had higher mortality rates, witha tendency to lower gestational ages and birth weights, as well as a greater association with other MF and syndromic phenotype. Among the observed heart diseases, those related to obstruction of the left heart outflow tract were present in half of the cases. When patients without heart disease and with echocardiogram were evaluated, functional and left ventricular diameter reductions, as well as the greater severity of pulmonary hypertension, were factors that contributed to a worse outcome. Regarding the prognostic indexes assessed, worse oxygenation index scores in the first 24 hours of life (24h OI) had a relevant association with mortality, even in the multivariate analysis. Therefore, we conclude that certain postnatal factors, prognostic indexes commonly used in neonatal intensive care, and tests such as echocardiography can provide important bases to guide the life perspectives of these NB, providing greater substrate for setting goals and limits for therapeutic proposal