Isolated absence of the right pulmonary artery in a neonate: a rare but critical incidental diagnosis.

Pulmonary Hypertension: From an Orphan Disease to a Public Health Problem

Systemic to pulmonary collateral arteries.

40-Year-Old Woman With Breathlessness and Fatigue

The role of calcium channel blockers for the treatment of pulmonary arterial hypertension: How much do we actually know and how could they be positioned today?

NT-proBNP as a tool to stratify disease severity in pulmonary arterial hypertension

A New Classification of Pulmonary Hypertension

World Health Organization Pulmonary Hypertension Group 2: Pulmonary hypertension due to left heart disease in the adult—a summary statement from the Pulmonary Hypertension Council of the International Society for Heart and Lung Transplantation

See related article, pages 1109–1114 Idiopathic pulmonary artery hypertension (IPH) is a rare illness with a poor prognosis. Whereas chronic intravenous prostacyclin relieves some of the symptoms of progressive dyspnea and prolongs survival, most patients ultimately require a lung transplant.1 Newer therapies such as nonintravenously administered prostacyclin derivatives,2,3,4 endothelin receptor blockers,5,6 and, to some extent, phosphodiesterase inhibitors,7 hold some promise as alternatives for intravenous prostacyclin, but current expectation is that, like prostacyclin, they will, at best, retard disease progression, serving as a bridge to transplant rather than as an alternative. The pathological features of IPH are loss of small distal precapillary pulmonary arteries, obliterative changes (plexogenic lesions) in more proximal pulmonary arteries associated with migration and proliferation of smooth muscle cells, and increased extracellular matrix deposition. There is also dysregulation of endothelial cells associated with increased proliferation.8 The mechanism underlying the evolution of these changes is unknown, so there was great interest when 2 groups independently identified a mutation in bone morphogenetic protein receptor 11 (BMP-RII) in 60% of families with IPH.9,10 A BMP-RII mutation also occurs in 20% of sporadic cases of IPH,11 but the biological connection between the mutation and the pathobiology of IPH has been relatively elusive. Recent studies using pulmonary artery smooth muscle cells from patients with IPH, including those with and without a BMP-RII mutation, showed similar abnormal proliferation in response to agents such as transforming growth factor-β (TGF-β) or BMP-2.12 In other studies, pulmonary artery smooth muscle cells were transfected with constructs encoding different mutant forms of BMP-RII expressing aberrant kinase or cytoplasmic domains, and impaired signaling was observed related to alterations in the induction of Smads and p38.13 Specifically, suppression of Smad1/5 and activation of p38 were related to smooth muscle cell proliferation. It …

Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France.

Descriptive patterns of severe chronic pulmonary hypertension by chest radiography

With more than 25 centuries of efforts to resolve its mysteries, the pulmonary circulation continues to inspire and humble researchers, frustrate clinicians, and scare patients with pulmonary arterial hypertension (PAH). Its mysteries lie in lost manuscripts written in the 12th-century Syria; within the pages of one of the rarest books in history written in 1531 by an author burned alive with his books, condemned by the Inquisition; within a publication from a young urologist self-experimenting 30 years before he received the Nobel prize…What kind of disease is this one that in the age of planning missions to Mars, no medical therapy can save its victims? It is certainly a disease that deserves our respect. The objective of this compendium is to present the state of the art in PAH, but mostly emphasize its future, discussing several evolving theories on its pathogenesis. Its objective is also to comprehensively summarize our current clinical approach to the disease, with patients in mind. In addition to researchers, clinicians, health professionals, students, and residents, its target audience includes patients: the patients who suffer, desperate for answers, and the patients who, one way or another, not only participate in but also fund our research through their taxes and donations. In the age of instant and easy information dissemination, many patients are already quite informed when they visit a specialist. How do we describe the cutting-edge developments in this complex disease to the informed patient? It is not easy, but most would agree that only the expert who really understands his/her subject is able to describe it in lay terms and pass its main messages. Humbled by the complexity of PAH and its challenges, we need to focus on what drives all of us fighting this disease. In addition to our patients, it is scientific curiosity: PAH …

The Cardio-Obstetrics Patient and the Cardiothoracic Anesthesiologist

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.

Evidence for the use of combination targeted therapeutic approaches for the management of pulmonary arterial hypertension

Mechanisms responsible for pulmonary hypertension