Sufentanil, sold under the brand names Dsuvia and Sufenta, is a synthetic opioid analgesic drug approximately 5 to 10 times as potent as its parent drug, fentanyl, and 500 times as potent as morphine. Structurally, sufentanil differs from fentanyl through the addition of a methoxymethyl group on the piperidine ring (which increases potency but is believed to reduce duration of action), and the replacement of the phenyl ring by thiophene. Sufentanil first was synthesized at Janssen Pharmaceutica in 1974.Sufentanil is marketed for use by specialist centers under different trade names, such as Sufenta and Sufentil. Sufentanil with and without lidocaine or mepivacaine is available as a transdermal patch similar to Duragesic in Europe under trade names such as Chronogesic. It is available as a sublingual tablet under the trade name Dsuvia. Medical uses
The main use of this medication is in operating suites and critical care where pain relief is required for a short period of time. It also offers properties of sedation and this makes it a good analgesic component of anesthetic regimen during an operation.Because of its extremely high potency, it is often used in surgery and post-operative pain management for patients that are heavily opioid dependent/opioid tolerant because of long term opiate use for chronic pain or illicit opiate use. Currently sufentanil is the most potent opioid painkiller available for use in humans. Although more potent narcotic pain medications do exist, all medications stronger than sufentanil are approved for veterinary use only.
Pulmonary capillary hemangiomatosis (PCH) is a disease affecting the blood vessels of the lungs, where abnormal capillary proliferation and venous fibrous intimal thickening result in progressive increase in vascular resistance. It is a rare cause of pulmonary hypertension, and occurs predominantly in young adults. Together with pulmonary veno-occlusive disease, PCH comprises WHO Group I causes for pulmonary hypertension. Indeed, there is some evidence to suggest that PCH and pulmonary veno-occlusive disease are different forms of a similar disease process. Presentation
These are non specific. Typical symptoms include dyspnea, cough, chest pain and fatigue. Genetics
At least some cases appear to be due to mutations in the eukaryotic translation initiation factor 2-alpha kinase 4 (EIF2AK4) gene. Diagnosis
Lung biopsy is essential to make this diagnosis. This can be difficult if the pulmonary pressure is high. Investigations
Chest X ray may show enlargement of the heart and ill-defined patchy lesions in the lung fields. CT chest typically shows wide spread ill-defined centrilobular nodules of ground glass opacity. This is a nonspecific finding and may be seen in a number of pulmonary diseases. CT pulmonary angiography usually shows enlargement of the main pulmonary artery. When measured by echocardiography or pulmonary angiography, the pulmonary arterial pressure is typically elevated. Differential diagnosis
Pulmonary veno-occlusive disease

Associations
This condition has been reported in patients with Ehlers Danlos syndrome, CREST syndrome and scimitar syndrome. Treatment
The only definitive treatment for this condition currently is lung transplantation. Median survival without treatment is 3 years.Imatinib may be of use.Epoprostenol does not appear to be of use.