| Recommendation | Strength of recommendation* | Level of evidence† |
|---|---|---|
| Prenatal diagnosis | ||
| Ultrasound measurement of O/E LHR should be used between 22 and 32 w of gestational age to predict the severity of pulmonary hypoplasia in isolated CDH. | B-NR | |
| In left-sided CDH, an O/E LHR < 25% predicts poor outcome. In right-sided CDH, an O/E LHR < 45% may predict poor outcome. | B-NR | |
| Fetal magnetic resonance imaging should be used (where available) for the assessment of lung volume and liver herniation in moderate and severe CDH. | B-NR | |
| Ventilation | ||
| Newborns with CDH and immediate respiratory distress should be preferentially intubated at birth. Bag-valve-mask ventilation should be avoided. | C-EO | |
| Sedation should be provided to all mechanically ventilated newborns with CDH. Deep sedation and neuromuscular blockade should be provided selectively to those with greater ventilation or oxygen requirements. | B-NR | |
| A T-piece should be used with the ventilator to avoid a peak inspiratory pressure > 25 cm H2O. | B-NR | |
| An arterial pCO2 between 45 and 60 mm Hg and a pH between 7.25 and 7.40 should be targeted in all newborns with CDH. | B-NR | |
| Supplemental oxygen should be titrated to achieve a preductal saturation of at least 85%, but not > 95%. | B-EO | |
| Gentle, intermittent mandatory ventilation should be the initial ventilation mode for newborns with CDH who require respiratory support. High-frequency oscillatory ventilation or high-frequency jet ventilation should be used when the peak inspiratory pressure required to control hypercapnia using intermittent mandatory ventilation exceeds 25 cm H2O. | B-NR | |
| Hemodynamic support | ||
Treatment of poor perfusion (capillary refill > 3 s, lactate > 3 mmol/L, urine output < 1 mL/kg/h) and blood pressure below norms for age should include:
|
B-NR | |
| Echocardiography | ||
| Two standardized echocardiograms, one within 48 h of birth and one at 2–3 w of life, are needed to assess pulmonary vascular resistance, as well as left ventricular and right ventricular function. Additional studies may be conducted as clinically indicated. | C-LD | |
| Management of pulmonary hypertension | ||
| iNO is indicated for confirmed suprasystemic pulmonary arterial hypertension without left ventricular dysfunction, provided lung recruitment is adequate. In the absence of clinical or echocardiographic response, iNO should be stopped. | C-EO | |
| Sildenafil should be considered in patients with refractory pulmonary hypertension (i.e., unresponsive to iNO) or as an adjunct when weaning iNO. | B-R | |
| Milrinone should be used to treat cardiac dysfunction, particularly if it is associated with pulmonary hypertension. | B-NR | |
| Prostaglandin E1 can be used to maintain ductus arteriosus patency and reduce right ventricular afterload in patients with pulmonary hypertension with right ventricular failure, or in the presence of a closing ductus. | C-LD | |
| Extracorporeal life support | ||
| The possibility of extracorporeal life support should be discussed during prenatal counselling for CDH, and should disclose that available evidence does not suggest a survival benefit to its use. | B-R | |
Diagnosis and Management of Congenital Diaphragmatic Hernia (CDH): A Clinical Practice Guideline
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