Memos
Editor's Memo
One of the many challenges in managing pulmonary hypertension patients encompasses the broad array of associated conditions. While advancement has occurred with clinical description and course as well as pathobiological relationships, there is much to be learned. Often, the best source of information is a detailed review of the current state of knowledge. For that very reason, I am extraordinarily proud of the current issue prepared under the insightful direction of Drs. Kelly Chin and Sonja Bartolome, guest editors. They have marshalled expert resources to generate informative discussions on topics ranging from sarcoidosis to pulmonary veno-occlusive disease. It is my firm belief that the reader will enjoy a refinement of the understanding of these varied disease states that manifest with pulmonary vascular disease.
Guest Editors' Memo
Diagnosing and treating pulmonary hypertension (PH) is frequently challenging. This is even truer for the 4 PH subtypes discussed in this issue of Advances. Challenges include the widely varying pathophysiology, the lack of definitive clinical trials, and in many cases, the seriousness of the underlying medical conditions. A recurring theme throughout this issue is the importance of asking. “What is the pathophysiology of this patient's PH,” even when the associated condition is already known. This is needed due to the varied subtypes of PH that can occur with each condition. For example, PH in the setting of hereditary hemorrhagic telangiectasia most commonly occurs in the setting of high output heart failure, but a heritable form classified as Group 1 pulmonary arterial hypertension also occurs. Similarly, PH in the setting of chronic myeloproliferative disorders includes both a small vessel pulmonary arteriopathy and chronic thromboembolic pulmonary hypertension, with significant differences in prognosis and treatment options for the 2 conditions. Finally, sarcoidosis-associated PH has even more varied pathophysiology, including granulomatous angiitis, pulmonary fibrosis and hypoxia, direct myocardial involvement and cirrhosis, among others. Pulmonary veno-occlusive disease, the fourth PH subtype included in this issue, was chosen because it too often presents both a significant diagnostic and therapeutic challenge. This issue of Advances focuses on providing a broad clinical overview of each condition, the typical hemodynamic findings, and treatment recommendations based on clinical studies and expert recommendation.