High blood pressure in the lungs in patients with congenital diaphragmatic hernia
Conditions
Interventions
Postnatally, all newborns will be treated according to the standard protocol
for patients with CDH, which is implemented in all participating centers
according to the revised CDH consortium guidelin
Sponsors
Erasmus MC, Universitair Medisch Centrum Rotterdam
Eligibility
Age
2 Years to 11 Years
Inclusion criteria
Inclusion criteria: • Diagnosis of CDH and pulmonary hypertension defined as 2 of the following 4 criteria: , I. PAP> 2/3 systemic pressure estimated by echocardiography , II. RV dilatation/septal displacement, RV dysfunction +/- LV dysfunction , III. Pre-post ductal SpO2 difference > 10% , IV. OI>20. , • Parental informed consent, • Children born at or after a gestational age of 34 weeks , • Newborns who received a fetal intervention may be included
Exclusion criteria
Exclusion criteria: • Severe chromosomal anomaly, like trisomy 18 or trisomy 13, which may imply a decision to stop or not to start life-saving medical treatment, • Severe cardiac anomaly, expected to need corrective surgery in the first 60 days of life (such as transposition of the great arteries, truncus arteriosus, coarctation aortae or double outlet right ventricle), • Renal anomalies associated with oligohydramnios, • Severe orthopaedic and skeletal deformities, which are likely to influence thoracic, and / or lung development (such as chest wall deformities and spine anomalies), • Severe anomalies of the central nervous system, • iNO already started for postnatal transport
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The primary objective of this study is to determine if there is a difference in OI after 12 hours of treatment between CDH patients treated with iNO versus those treated with intravenous sildenafil. The OI is a calculated value to estimate severity of hypoxemic respiratory failure and is often used as a derivative for the severity of PH in newborns | — |
Secondary
| Measure | Time frame |
|---|---|
| The presence/absence of pulmonary hypertension on day 14 without pulmonary vasodilator therapy and without treatment failure and/or death within the first 28 days of life, overall mortality, treatment failure, time on study drug, need for ECMO, number of ventilator free days at day 28, severity of pulmonary hypertension, laboratory markers and tracheal aspirates for proteomic, metabolomics and biochemical analysis, the use of other medication given for pulmonary hypertension during the hospital admission, the use of pulmonary and/or cardiac medication at discharge, long-term pulmonary hypertension on echocardiography, chronic lung disease. | — |
Countries
Netherlands
Outcome results
None listed