Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update

Abstract

In 2010, the congenital diaphragmatic hernia (CDH) EURO Consortium published a standardized neonatal treatment protocol. Five years later, the number of participating centers has been raised from 13 to 22. In this article the relevant literature is updated, and consensus has been reached between the members of the CDH EURO Consortium. Key updated recommendations are: (1) planned delivery after a gestational age of 39 weeks in a high-volume tertiary center; (2) neuromuscular blocking agents to be avoided during initial treatment in the delivery room; (3) adapt treatment to reach a preductal saturation of between 80 and 95% and postductal saturation >70%; (4) target PaCO₂ to be between 50 and 70 mm Hg; (5) conventional mechanical ventilation to be the optimal initial ventilation strategy, and (6) intravenous sildenafil to be considered in CDH patients with severe pulmonary hypertension. This article represents the current opinion of all consortium members in Europe for the optimal neonatal treatment of CDH.

Clinical Outcome for Congenital Diaphragmatic Hernia at the Age of 1 Year in the Era of Fetal Intervention

“…FETO ( n = 10) was performed at a median age of 29.5 (IQR: 27.5-30.3) weeks, followed by removal of the balloon after a median interval of 27.0 (IQR: 26.5-34.5) days. No patients received ECMO [15]. …”

“…The strengths of our data are the uniform neonatal care and standardised postnatal follow-up of this single-centre study [15]. Ideally, follow-up should also include a formal neurological developmental assessment, an infant lung-function test, and newer imaging techniques such as hyperpolarised gas lung MRI to evaluate lung volumes and the vasculature.…”

Ventricular Dysfunction, Interdependence, and Mechanical Dispersion in Newborn Infants with Congenital Diaphragmatic Hernia

“…CDH cases were managed by a multi-disciplinary team according to an institutional treatment protocol, in accordance with international guidelines [6]. This includes a “gentle” ventilation strategy, targeted use of pulmonary vasodilators (inhaled nitric oxide, sildenafil), cardiotropes (including dopamine, milrinone, and epinephrine), and selective use of ECMO.…”

Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline

“…In preparing the current guideline, the panel reviewed existing guidelines published by the CDH EURO Consortium (CDH EURO) 8,9 and the American Heart Association and American Thoracic Society. 10 We used pre-established consensus criteria to accept, modify or reject these baseline recommendations in the guideline-finalization process described below.…”

“…Two major guidelines were taken into consideration in creating these recommendations: the CDH EURO Consortium recommendations published in 2010 and updated in 2016, 8,9 and the American Heart Association/American Thoracic Society 10 guideline on pulmonary hypertension management. This guideline differs from previously published guidelines in several respects.…”