Seminars in respiratory and critical care medicine
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Semin Respir Crit Care Med · Aug 2009
ReviewPulmonary arterial hypertension in congenital heart diseases.
Pulmonary hypertension complicates the course of many children and adults with congenital heart diseases (CHDs). The increase in pulmonary pressure associated with CHD is secondary to either increased pulmonary blood flow or increased postcapillary pressures. ⋯ The recent introduction of targeted therapies in other forms of pulmonary arterial hypertension has led to a renewed interest in pulmonary hypertension associated with CHD and this particularly for the most advanced form, the so-called Eisenmenger syndrome (ES). This review summarizes the current knowledge on pulmonary hypertension associated with CHD, focusing on the pathophysiology and treatment of ES.
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Semin Respir Crit Care Med · Aug 2009
ReviewPulmonary arterial hypertension complicating connective tissue diseases.
Pulmonary arterial hypertension (PAH) may complicate diverse connective tissue diseases (CTDs) such as systemic sclerosis (SSc), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and mixed CTD (MCTD) and is an important cause of morbidity and mortality in this context. From a histological standpoint, the pulmonary vascular lesions in PAH complicating CTD are similar to those observed in idiopathic PAH (IPAH). However, prognosis and responsiveness to therapy are distinctly worse in PAH associated with CTD. ⋯ Although PAH may involve other CTDs, data are more limited (primarily small, uncontrolled series and case reports). Treatment strategies for PAH associated with CTD are similar to but are distinctly less effective than those for IPAH. Lung transplantation may be offered for a subset of patients with CTD-associated PAH, but results are less favorable than for IPAH due to comorbidities and specific complications associated with CTD (e.g., esophageal involvement, aspiration with SSc).
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Semin Respir Crit Care Med · Aug 2009
Definition, classification, and epidemiology of pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is a distinct subgroup of pulmonary hypertension that comprises idiopathic PAH, familial/heritable forms, and PAH associated with connective tissue disease, congenital heart disease, portal hypertension, human immunodeficiency virus (HIV) infection, and some other conditions. The hemodynamic definition of PAH was recently revised: PAH is now defined by a mean pulmonary artery pressure at rest > or =25 mm Hg in the presence of a pulmonary capillary wedge pressure < or =15 mm Hg. ⋯ The revised classification of pulmonary hypertension still consists of five major groups: (1) PAH, (2) pulmonary hypertension due to left heart disease, (3) pulmonary hypertension due to chronic lung disease and/or hypoxia, (4) chronic thromboembolic pulmonary hypertension, and (5) miscellaneous forms. Modifications have been made in some of these groups, such as the addition of schistosomiasis-related pulmonary hypertension and pulmonary hypertension in patients with chronic hemolytic anemia to group 1.
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Hypoventilation in neuromuscular disease is attributed to both respiratory muscle weakness and reduced chemoreceptor sensitivity essential in ventilatory drive. Acute or chronic respiratory failure is seen in a spectrum of neuromuscular disease; whereas some are treatable others are progressive and devastating. ⋯ The timing of respiratory failure is variable, but knowledge of the clinical aspects, pathogenesis, and treatment of respiratory failure and hypoventilation may be helpful in evaluating the patient with neuromuscular disease. For those with progressive and terminal disease, additional factors such as end of life care, especially ventilation and cough, may be useful for the patient, caregivers, and treating medical personnel.
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Semin Respir Crit Care Med · Jun 2009
ReviewCentral congenital hypoventilation syndrome: changing face of a less mysterious but more complex genetic disorder.
Central congenital hypoventilation syndrome (CCHS) is a disorder in which affected individuals fail to breathe during sleep despite progressive hypercapnia and hypoxia. Discovery of the genetic link between PHOX2B gene mutations and CCHS represents a breakthrough in the diagnosis of CCHS, identification of patients with late-onset central hyperventilation syndrome (LO-CHS), association of mutated alleles with disease severity, and clues to the pathophysiology responsible for the disorder. ⋯ Management of CCHS and LO-CHS is primarily directed at optimizing respiratory status, most often with nighttime mechanical ventilatory support and monitoring with polysomnography. Multidisciplinary care is also necessary to promote better outcomes and long-term survival.