Abstract
BackgroundCongenital diaphragmatic hernia (CDH) is a congenital malformation associated with life-threatening pulmonary dysfunction and high neonatal mortality. Outcomes are improved with protective ventilation, less severe pulmonary pathology, and the proximity of the treating center to the site of delivery. The major CDH treatment center in Croatia lacks a maternity ward, thus all CDH patients are transferred from local Zagreb hospitals or remote areas (outborns). In 2000 this center adopted protective ventilation for CDH management. In the present study we assess the roles of protective ventilation, transport distance, and severity of pulmonary pathology on survival of neonates with CDH.MethodsThe study was divided into Epoch I, (1990–1999, traditional ventilation to achieve normocapnia), and Epoch II, (2000–2014, protective ventilation with permissive hypercapnia). Patients were categorized by transfer distance (local hospital or remote locations) and by acuity of respiratory distress after delivery (early presentation-occurring at birth, or late presentation, ≥6 h after delivery). Survival between epochs, types of transfers, and acuity of presentation were assessed. An additional analysis was assessed for the potential association between survival and end-capillary blood CO2 (PcCO2), an indirect measure of pulmonary pathology.ResultsThere were 83 neonates, 26 in Epoch I, and 57 in Epoch II. In Epoch I 11 patients (42 %) survived, and in Epoch II 38 (67 %) (P = 0.039). Survival with early presentation (N = 63) was 48 % and with late presentation 95 % (P <0.001). Among early presentation, survival was higher in Epoch II vs. Epoch I (57 % vs. 26 %, P = 0.031). From multiple logistic regression analysis restricted to neonates with early presentation and adjusting for severity of disease, survival was improved in Epoch II (OR 4.8, 95%CI 1.3–18.0, P = 0.019). Survival was unrelated to distance of transfer but improved with lower partial pressure of PcCO2 on admission (OR 1.16, 95%CI 1.01–1.33 per 5 mmHg decrease, P = 0.031).ConclusionsThe introduction of protective ventilation was associated with improved survival in neonates with early presentation. Survival did not differ between local and remote transfers, but primarily depended on severity of pulmonary pathology as inferred from admission capillary PcCO2.
Highlights
Congenital diaphragmatic hernia (CDH) is a congenital malformation associated with life-threatening pulmonary dysfunction and high neonatal mortality
To explore the association between local vs. remote transfer on survival we focused on neonates with early presentation of symptoms during Epoch II
Survival was higher in Epoch II vs. Epoch I (25 of 44, 57 % vs. 5 of 19, 26 %, OR 3.7 95 % CI 1.1–12.0, P = 0.031)
Summary
Congenital diaphragmatic hernia (CDH) is a congenital malformation associated with life-threatening pulmonary dysfunction and high neonatal mortality. In 2000 this center adopted protective ventilation for CDH management. In the present study we assess the roles of protective ventilation, transport distance, and severity of pulmonary pathology on survival of neonates with CDH. In neonates with CDH the presence of respiratory distress after delivery indicates severe pulmonary involvement that requires medical management which includes mechanical ventilatory support prior to undergoing surgical correction [1]. The University Hospital Center (UHC) in Zagreb Croatia is the national treatment center for neonates with CDH. Before 2000, neonates with CDH cared for at UHC underwent intermittent mandatory ventilation (IMV) to achieve hyperventilation, a strategy based on the premise that respiratory alkalosis may help control degree of pulmonary hypertension [1,2,3]. In the year 2000 UHC adopted protective ventilation for neonates with CDH
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