Study of Inhaled Iloprost in Pediatric Pulmonary Hypertension (PH) After Surgery

Randomized Double-blind Study of Inhaled Iloprost for the Treatment of Pulmonary Hypertension (PH) and Pulmonary Hypertensive Crisis (PHC) After Repair of Congenital Heart Disease (CHD)

The aim of this study is to determine whether inhaled iloprost can be used to prevent and treat PH and PHC while in children after operation of CHD

Pulmonary hypertension (PH) is a significant contributor to the postoperative morbidity and mortality of congenital heart disease. Inhaled iloprost has been approved for the treatment of adults with PH, but little is known about the effects in children with PH.

Iloprost is a prostacyclin analogue. When applied by inhalation, it selectively dilates pulmonary vessels without side affecting the systemic circulation. There is no RCTs of iloprost have previously been performed in this indication.

Inclusion Criteria:

Before corrective procedure for CHD, two of bellow ten criteria should be met

1. Pulse SaO2 smaller than 93% in left-right shunt CHD case (in room air)
2. EKG: right ventricular hypertrophy, right atrial dilatation
3. Chest X-ray: enhanced vascular signs in trans-hilar, loss of blood vessal in bilateral lung fields, pulmonary arterial trunk dilatation, right ventricular enlargement
4. Cardiac echocardiography: fast tricuspid or pulmonary valve regurgitant velocity, ventricular and aortic level bidirectional shunt, or even right-to-left shunt
5. Under-filling of pulmonary capillary, 'pruning' of the peripheral blood vessels
6. Pp/Ps greater than 0.75
7. Qp/Qs smaller than 1.5
8. PVR grater than 9 Wood Unit/m2
9. Rp/Rs graeter than 0.5

Exclusion Criteria:

After corrective procedure for CHD:

1. Deficient anatomy associated with remained intracardiac shunts and severe artrio-ventricular regurgitation
2. Severe arrhythmia led to low cardiac output
3. PLT smaller than 50,000*109/L and obvious bleeding