Evidence based therapy of pulmonary hypertension in congenital diaphragmatic hernia - A prospective observational study

Conditions: 10028971
high pulmonary resistance
pulmonary hypertension
10019280
10018018

Registration Number: NL-OMON35811

Lead Sponsor: Erasmus MC, Universitair Medisch Centrum Rotterdam

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Pending

Sex: Not specified

Target Recruitment: 25

Inclusion Criteria

All patients with congenital diaphragmatic hernia admitted to the ICU
Written parental informed consent

Exclusion Criteria

Out born patients with congenital diaphragmatic hernia
Recurrent congenital diaphragmatic hernia
Lung pathology mimicking diagnostic or clinical signs of congenital diaphragmatic hernia
Severe chromosomal anomaly which imply abstinence of therapy
Severe congenital cardiac anomaly with the exception of cardiac deformations associated with congenital diaphragmatic hernia (patent ductus arteriosus, patent foramen ovale, atrioseptal defect)
Cardiopulmonary resuscitation and subsequent therapeutic hypothermia

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>The main study parameters are echocardiographic signs of pulmonary hypertension<br /><br>in combination with plasma biomarkers (ICAM, VCAM, sP-selectin, VEGF, von<br /><br>Willebrand factor, thrombomodulin, activated factor VIII:C, nitrate, nitrite,<br /><br>ADMA, NT.pro BNP).</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>Microcirculatory perfusion (defined by the parameters PVD and MFI) at day 1-7<br /><br>and at 2 hours after start of iNO, sildenafil and bosentan<br /><br>Correlation with lactate, pH, MAP, pre- and postductal saturation, HF, Hb, Ht,<br /><br>oxygen demand, need of ventilation<br /><br>Drug and metabolite concentrations of sildenafil and bosentan<br /><br>Population PK analysis<br /><br>Pharmacodynamic analysis</p><br>