iloprost and Heart-Septal-Defects--Ventricular

A 38-year-old male was diagnosed with unrepaired ventricular septal defect associated with severe pulmonary arterial hypertension, cyanosis, and significant exercise intolerance. His echocardiogram showed right ventricular dysfunction and moderate pericardial effusion with no signs of cardiac tamponade. He was treated with an intensive course of inhaled iloprost and sildenafil. He showed a dramatic clinical response; his saturation went up from 60% on admission to 90% on minimal oxygen with significant improvement in his symptoms and signs of heart failure and total resolution of pericardial effusion. On follow up 3 and 6 weeks later, he was stable and could walk 360 meters in a 6 minutes walk test with disappearance of pericardial effusion. With unavailability of intravenous prostacyclin, we have shown in this case that intensive administration of inhaled iloprost could be used intensively as a rescue therapy in severe cases of pulmonary arterial hypertension with excellent results. 

Topics: Administration, Inhalation; Adult; Exercise Test; Heart Septal Defects, Ventricular; Humans; Hypertension, Pulmonary; Iloprost; Male; Pericardial Effusion; Pericarditis; Piperazines; Purines; Sildenafil Citrate; Sulfonamides; Treatment Outcome; Vasodilator Agents

The treatment of choice for congenital heart disease (CHD) with pulmonary arterial hypertension (PAH) is still controversial. We assessed the efficacy and safety of perioperative inhaled iloprost therapy in CHD with PAH.. Among 45 patients with a ventricular septal defect and/or an atrial septal defect with PAH, 28 patients were treated with inhaled iloprost before and after surgery. Perioperative clinical parameters and plasma B-type natriuretic peptide (BNP) were evaluated.. No statistical difference in the estimated right ventricular systolic pressure (e-RVP), the e-RVP-to-systemic pressure ratio, and preoperative BNP levels between the iloprost group and the control group were found. Among the iloprost group, oxygen saturation was increased significantly after iloprost inhalation therapy (p = 0.0052). The iloprost group was also significantly correlated with less use of inhaled nitric oxide in the immediate postoperative period compared to the control group (p = 0.021). The durations of mechanical ventilation (p = 0.018), ICU stay (p = 0.005), and chest tube use (p = 0.039) were significantly shorter in the iloprost group compared to the control group. The plasma BNP, checked on 7th day of postoperatively, was lower in the iloprost group than in the control group (p = 0.008).. Perioperative inhaled iloprost therapy showed the benefit of cardiac functional improvement and early weaning of postoperative supportive care in the management of CHD with PAH.

Topics: Administration, Inhalation; Adolescent; Adult; Child; Child, Preschool; Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Humans; Hypertension, Pulmonary; Iloprost; Infant; Perioperative Care; Retrospective Studies; Vasodilator Agents; Young Adult

Topics: Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Humans; Hypertension, Pulmonary; Iloprost; Vasodilator Agents

We report a case of a 6-year-old boy with fixed severe pulmonary artery hypertension secondary to a ventricular septal defect (VSD) together with a patent ductus arteriosus (PDA). As a preliminary step, PDA embolization was performed following therapy with inhaled prostacyclin over a period of 6 months. Further, the patient underwent successful surgical VSD closure. We postulate that a staged procedure with long-term prostaglandin therapy might be capable of reducing pulmonary artery resistance and permitting total correction in a patient once considered to have inoperable pulmonary arteriopathy.

Topics: Administration, Inhalation; Child; Combined Modality Therapy; Ductus Arteriosus, Patent; Embolization, Therapeutic; Heart Septal Defects, Ventricular; Humans; Hypertension, Pulmonary; Iloprost; Male; Septal Occluder Device; Vasodilator Agents