Endarterectomy of the pulmonary artery in patients with myeloproliferative neoplasms

A Pulmonary Thromboendarterectomy (PTE), also known as a Pulmonary Endarterectomy (PEA), is a thoracic surgical procedure that removes clots of blood from the pulmonary arteries, which feed blood to the lungs. Patients with surgically accessible pulmonary artery emboli should have surgery. Recurrent/chronic pulmonary emboli, and hence Chronic Thromboembolic Pulmonary Hypertension (CTEPH), are mainly caused by thrombi. PTE is the only therapy option for CTEPH that has been proven to be effective. Patients with major hemodynamic or breathing issues or impairments may be unable to undergo PTE due to the nature of the technique.

As of 2008, Stuart W. Jamieson of the UCSD Medical Center's cardiothoracic surgery department was widely regarded as a pioneer in the relatively new surgery, having performed more PTEs than the rest of the world combined (over 3000 since 1970 out of a total of 4500 worldwide) with the lowest mortality rate. PTE is exclusively available in the United Kingdom at Royal Papworth Hospital, which is directed by surgeon Mr. David Jenkins. He is one of only four surgeons in the UK qualified to perform pulmonary endarterectomy surgery, and they are all based at Royal Papworth Hospital, which is one of the busiest centers in the world for the procedure, with about 190 operations per year and a total caseload of more than 2,000 since 1996. Myeloproliferative Neoplasms (MPNs) are a type of rare blood malignancies in which the bone marrow produces too many red blood cells, white blood cells, or platelets. Myelo refers to the bone marrow, proliferative to the rapid proliferation of blood cells, and neoplasm to the abnormal and uncontrolled growth of blood cells. Overproduction of blood cells is frequently linked to a somatic mutation, such as those found in the JAK2, CALR, TET2, and MPL genes. Some MPNs, such as primary myelofibrosis, can accelerate and progress to acute myeloid leukemia in rare situation.