Nature reviews. Cardiology
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Pulmonary hypertension (PH) with left-sided heart disease is defined, according to the latest Venice classification, as a Group 2 PH, which includes left-sided ventricular or atrial disease, and left-sided valvular diseases. These conditions are all associated with increased left ventricular filling pressure. Although PH with left-sided heart disease is a common entity, and long-term follow-up trials have provided firm recognition that development of left-sided PH carries a poor outcome, available data on incidence, pathophysiology, and therapy are sparse. ⋯ In patients with these conditions, PH develops as a consequence of impaired left ventricular relaxation and distensibility. Chronic sustained elevation of cardiogenic blood pressure in pulmonary capillaries leads to a cascade of untoward retrograde anatomical and functional effects that represent specific targets for therapeutic intervention. The pathophysiological and clinical importance of the hemodynamic consequences of left-sided heart disease, starting with lung capillary injury and leading to right ventricular overload and failure, are discussed in this Review, focusing on PH as an evolving contributor to heart failure that may be amenable to novel interventions.
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The clinical presentation of pulmonary embolism (PE) varies widely, ranging from only limited symptoms to severe cardiogenic shock. Treatment of PE comprises initial therapy--with low-molecular-weight heparin (LMWH), fondaparinux, or unfractionated heparin--and long-term treatment, most commonly with vitamin-K antagonists (VKAs). Methods of risk stratification, to determine whether a patient will benefit from thrombolysis, are currently under investigation. ⋯ Several new oral anticoagulants, such as factor IIa and factor Xa inhibitors, are now being investigated. For prevention of recurrent PE in situations where anticoagulation is contraindicated, a temporary inferior vena cava filter might be useful. Some patients with PE can be safely treated at home, but few outcome studies in this setting have been published.
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The Fontan palliation was introduced in 1968 to treat cardiac malformations unsuitable for biventricular repair. This procedure has transformed the surgical management of congenital heart disease. In this Review, we reflect on the outcomes and clinical problems associated with this unique circulation after more than 40 years of experience. We also summarize the evolution of the Fontan procedure, highlight the long-term clinical issues and their management, and consider future expectations of a circulation driven by a single ventricle with the systemic and pulmonary blood flow in series rather than in parallel.