Idiopathic pulmonary arterial hypertension (previous primary pulmonary hypertension) was a progressive disease with high mortality. Many patients with idiopathic pulmonary arterial hypertension did not show vasoreactivity, rapidly resulted in marked disability, right heart failure and death. Recent advances of therapeutic modalities have revolutionized the treatment of idiopathic pulmonary arterial hypertension. Irrespective of pulmonary arterial vasoreactivity, new vasodilatng agents, such as epoprostenol, treprostinil, iloprost, bosentan, and sildenafil, significantly improved hemodynamics, symptoms, exercise capacities, quality of life, and survival. The median survival of patients with idiopathic pulmonary arterial hypertension has been prolonged from 2.8 years to more than 5 years. In a near future, pulmonary arterial hypertension could be easily controlled like a systemic arterial hypertension.
Diagnosis* ; Epoprostenol ; Heart Failure ; Hemodynamics ; Humans ; Hypertension* ; Iloprost ; Mortality ; Quality of Life ; Sildenafil Citrate

Chronic obstructive pulmonary disease (COPD) is one of the causes of cor pulmonale. Cor pulmonale patients with pulmonary hypertension have a significant lower survival rate than patients without. However, there is no conclusive treatment options in cor pulmonale and pulmonary hypertension associated with COPD until now. We report a patient with cor pulmonale and pulmonary hypertension associated with severe form of COPD and tuberculous destroyed lung who achieved marked clinical, functional and echocardiographic hemodynamic improvements with inhaled iloprost for six months.
Echocardiography ; Hemodynamics ; Humans ; Hypertension, Pulmonary* ; Iloprost* ; Lung* ; Pulmonary Disease, Chronic Obstructive ; Pulmonary Heart Disease* ; Survival Rate

Inhaled iloprost, a stable carbacyline derivative of prostacyclin, has been used recently for the treatment of adults with pulmonary hypertension but only few reports are available about its use in neonatal critical care. We report therapeutic trial of inhaled iloprost in newborn infants with persistent pulmonary hypertension of the newborn (PPHN) who did not respond to inhaled nitric oxide (iNO). Inhaled iloprost (Ventavis(R), Bayer Shering Pharma, Germany) was effective in neonates with severe PPHN who showed inadequate response to iNO. We suggest that inhaled iloprost could be considered as an additional therapeutic option in PPHN refractory to iNO.
Adult ; Critical Care ; Epoprostenol ; Humans ; Hypertension, Pulmonary ; Iloprost ; Infant, Newborn ; Nitric Oxide

OBJECTIVETo investigate the efficacy and safety of inhaled iloprost on top of other pulmonary hypertension (PH) specific therapies for patients with PH and severe right heart failure.

METHODSWe consecutively enrolled WHO functional class IV patients with PH and chronic thromboembolic pulmonary hypertension (CTEPH) in Shanghai Pulmonary Hospital from January 2011 to January 2013. Inhaled iloprost was administrated to all enrolled patients, oral endothelin antagonist receptors (ERAs) and/or type 5 phosphodiasterase inhibitors (PDE5-I) were also used as basis therapies. The in-hospital outcomes and the changes of right heart functional parameters were observed.

RESULTSTwenty-four patients with PH and 5 patients with CTEPH were enrolled. After a mean treatment duration of (23 ± 13) days, 3 patients dead and significant improvement was observed in the remaining 26 patients. Compared with the baseline, heart rate decreased from (99 ± 14) to (91 ± 12) bpm (P = 0.001), plasma NT-proBNP level decreased from 5 823 (3 029-13 248) to 3 220 (1 678-6 720) ng/L (P < 0.001), tricuspid annular plane systolic excursion (TAPSE) increased from (1.3 ± 0.4) to (1.4 ± 0.3) cm (P = 0.018), right ventricular diameter decreased (left-to-right diameter from (57 ± 11) to (53 ± 10) mm, P = 0.040, and superoinferior diameter from (69 ± 11) to (64 ± 16) mm, P = 0.027), Tbil also decreased from (41 ± 34) to (26 ± 17) µmol/L (P < 0.001). No severe side effects were observed.

CONCLUSIONThe strategy of inhaled iloprost on top of other PAH-specific target therapy medications is effective and safe for PH patients with severe right heart failure.

Heart Failure ; Humans ; Hypertension, Pulmonary ; Iloprost ; Natriuretic Peptide, Brain ; Peptide Fragments ; Vasodilator Agents ; Ventricular Dysfunction, Right

BACKGROUND: Various pharmacological treatments have been suggested to treat osteonecrosis of the femoral head. However, their practicability remains a controversial issue. METHODS: We systemically reviewed articles published during last 20 years to assess the efficacy and safety of the pharmacological treatments. RESULTS: To date, enoxaparin, statins, bisphosphonates, iloprost and acetylsalicylic acid have been practiced for the treatment of osteonecrosis. However, none of them were proven to be effective by high level studies, and most of them have adverse reactions. CONCLUSIONS: No pharmacological prevention or treatment of osteonecrosis is recommendable at this moment.
Aspirin ; Bone Remodeling ; Diphosphonates ; Drug Therapy ; Enoxaparin ; Head ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Iloprost ; Osteonecrosis

We report a case of a full-term neonate with persistent pulmonary hypertension who developed asphyxia after birth and was treated with iloprost. The neonate had persistent hypoxia and did not respond to supportive treatment. Because inhaled nitric oxide (iNO) was not available in our hospital, inhaled iloprost was administered via an endotracheal tube. This resulted in an immediate elevation of oxygen saturation. Echocardiography revealed the conversion of the right-to-left ductal shunt to the left-to-right one and a decrease of the right ventricular pressure. The use of inhaled iloprost did not cause any significant side effects. Here, we describe our experience where iloprost was used in a neonate with persistent pulmonary hypertension because the standard therapy with inhaled nitric oxide was not available.
Anoxia ; Asphyxia ; Echocardiography ; Female ; Humans ; Hypertension, Pulmonary ; Iloprost ; Infant, Newborn ; Nitric Oxide ; Oxygen ; Parturition ; Persistent Fetal Circulation Syndrome ; Ventricular Pressure

BACKGROUNDIloprost has been used to test acute pulmonary vasoreactivity in idiopathic pulmonary arterial hypertension (PAH). We aimed to investigate the acute hemodynamic and oxygenation responses and tolerability to 20 µg aerosolized Iloprost in Chinese patients with pulmonary hypertension.

METHODSBetween March 2005 and May 2010, 212 pulmonary hypertension patients inhaled a single dose of 20 µg Iloprost over 10 - 15 minutes for vasoreactivity testing. The acute hemodynamic and oxygenation responses and adverse events were recorded.

RESULTSIloprost decreased total pulmonary resistance ((1747 ± 918) dyn×s×cm(-5) vs. (1581 ± 937) dyn×s×cm(-5), P < 0.001), increased stroke volume ((45.0 ± 22.1) ml vs. (47.0 ± 24.2) ml, P = 0.002), and cardiac output ((3.7 ± 1.7) L/ml vs. (3.9 ± 1.9) L/min, P = 0.009). Heart rate and systemic vascular resistance remained stable during inhalation. However, systemic arterial blood oxygen saturation fell slightly ((91.0 ± 6.8)% vs. (90.3 ± 6.7)%, P = 0.002). Pulmonary and systemic arterial blood pressures declined within 1 - 3 minutes after inhalation initiation and reached their lowest levels within 10 - 15 minutes. Idiopathic PAH responded more favorably than pulmonary hypertension due to other causes (P £0.001) and patients with less severe pulmonary hypertension have better responses to Iloprost. No adverse events requiring medical care or leading to termination of inhalation occurred.

CONCLUSIONSInhalation of 20 µg Iloprost showed potent and selective pulmonary hemodynamic effects and was well tolerated in the Chinese pulmonary hypertension patients. Patients with idiopathic PAH and less severe pulmonary hypertension responded more favorably to inhalation of Iloprost.

Administration, Inhalation ; China ; Hemodynamics ; drug effects ; Humans ; Hypertension, Pulmonary ; drug therapy ; Iloprost ; administration & dosage ; adverse effects ; therapeutic use

Pulmonary arterial hypertension is a critical manifestation of systemic sclerosis (SSc) and is a main cause of death. Several treatment modalities for SSc have been identified, with effects that improve quality of life and mortality rates. However, whether these drugs can also normalize pulmonary arterial pressure, remains unclear. Here, we report the case of a woman with diffuse SSc with pulmonary arterial hypertension, who had a functional status equivalent to the New York Heart Association class III. The patient was treated with inhaled iloprost. After six years of inhaled iloprost therapy, echocardiography showed that pulmonary arterial pressure normalized, accompanied by improvement in functional capacity. Inhaled iloprost might not only normalize pulmonary arterial pressure, but also improve the functional status of patients with SSc with pulmonary arterial hypertension.
Arterial Pressure ; Cause of Death ; Echocardiography ; Female ; Heart ; Humans ; Hypertension* ; Hypertension, Pulmonary ; Iloprost* ; Mortality ; Quality of Life ; Scleroderma, Systemic*

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.
Adolescent ; Cardiac Output ; Double Outlet Right Ventricle ; Epoprostenol ; Female ; Fontan Procedure ; Humans ; Iloprost* ; Male ; Pulmonary Atresia ; Respiratory Therapy ; Vascular Resistance ; Vasodilation ; Ventricular Septum ; Young Adult

Moderate to severe pulmonary hypertension, mean pulmonary arterial pressure (mPAP) > 35 mmHg, in cirrhotic patients is usually considered an absolute contraindication to orthotopic liver transplantation (OLT) because of unacceptably high mortality. We present the case of successful OLT in a cirrhotic patient with a mPAP of 42 mmHg and a pulmonary vascular resistance (PVR) of 298 dyne . sec . cm(-5) preoperatively. He was treated with oral sildenafil (Viagra(R)) and inhaled iloprost (Ventavis(R)) for 45 days and then his mPAP and PVR were reduced to 33 mmHg and 206 dyne . sec . cm(-5) at the time of transplantation. During OLT, his mPAP was stable of 28?38 mmHg with the combined use of sildenafil via a nasogastric tube and iloprost via a nebulizer. His hemodynamic parameters were stable and significant postoperative bleeding was not noticed throughout his stay in the intensive care unit. Thereafter, he was transferred to general ward without any cardio-respiratory problems on 7th postoperative days.
Arterial Pressure ; Hemodynamics ; Hemorrhage ; Humans ; Hypertension ; Hypertension, Pulmonary ; Iloprost ; Intensive Care Units ; Liver ; Liver Transplantation ; Nebulizers and Vaporizers ; Patients' Rooms ; Piperazines ; Purines ; Sulfones ; Transplants ; Vascular Resistance ; Sildenafil Citrate