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A well-organized, systematic classification scheme is important in any disease process; this is especially true in pulmonary hypertension. Classification serves as a framework for clinical studies, as well as patient care. It provides structure for inclusion and exclusion criteria for clinical trials, as well as the development of screening, diagnostic, and treatment algorithms. There are several...
The past two decades has seen remarkable advances in the understanding of the basic biology of various forms of pulmonary hypertension as well as the development of much more effective therapies. These advances have also impacted the prognosis of many patients with pulmonary hypertension; whereas pursuing an evaluation to determine the presence and cause of pulmonary hypertension was once considered...
Particulate air pollution is associated with mortality of many cardiorespiratory disease. This implausible association, is now well supported by epidemiology studies, as is discussed in this chapter. There is now additional evidence for morbidity from asthma, COPD, ischemic heart disease, and congestive heart failure. We now know that ultrafine particles escape detection by the macrophage defense...
Idiopathic pulmonary arterial hypertension (IPAH), formerly referred to as primary pulmonary hypertension, is a disease in which there is a persistent elevation of pulmonary artery pressure without demonstrable cause. There is expert agreement that the definition of pulmonary hypertension, as was required for entry into the Primary Pulmonary Hypertension Registry of the US National Institutes of Health...
Pulmonary arterial hypertension (PAH), formerly known as primary pulmonary hypertension (PPH), is further classified into a sporadic or idiopathic form (IPAH) and a familial or heritable form (HPAH). Multiple individual reports described 13 different families in the USA with PAH among family members. In 1984, we conducted a follow-up analysis of these original 13 families, including interval description...
Pulmonary arterial hypertension (PAH), defined as a mean pulmonary arterial pressure of greater than 25 mmHg in the absence of elevation of the pulmonary capillary wedge pressure, is a cause of significant morbidity and mortality. PAH is characterized by remodeling and occlusion of the small pulmonary arterioles leading to a progressive increase in pulmonary vascular resistance. Left untreated, this...
Portopulmonary hypertension (POPH) is the well-recognized relationship of pulmonary artery hypertension that evolves as a consequence of portal hypertension. POPH was first reported by Mantz and Craige in a 53-year-old female patient who presented with sudden hoarseness due to a large spontaneous portocaval shunt (1.5–3 cm in diameter) that lay in intimate relationship with the left recurrent laryngeal...
Among HIV-infected people, the primary pulmonary complications among adults remain infectious. However, for those HIV-infected persons who receive highly active antiviral therapy, the spectrum of pulmonary disease is changing as a decrease in the incidence of opportunistic infections of the lung yields an increase of noninfectious complications. HIV-associated pulmonary arterial hypertension (HIV-PAH)...
Several drugs and toxins have been shown to be associated with the development of pulmonary vascular hypertension. Pulmonary hypertension (PH) related to these factors has been classified as primary PH (PPH), currently referred to as idiopathic pulmonary arterial hypertension, because its morphological findings, clinical manifestations, hemodynamic measures, and pathological changes were reported...
The disease process underlying pulmonary arterial hypertension (PAH) involves a classic pattern of pulmonary vascular remodeling, consisting of muscularization (medial hypertrophy) of small and medium-sized pulmonary arteries, with endothelial abnormalities, intimal fibrosis, and occasionally thrombus or plexiform lesions. The current classification divides PAH into five major categories: IPAH, familial...
Much of current knowledge and momentum in the field of pulmonary hypertension (PH) has not penetrated the increasingly heterogeneous pediatric population managed by pediatric subspecialists worldwide. It is critical to prospectively test specific agents in children. Yet, the broad spectrum of pediatric pulmonary vascular diseases and the lack of clinically meaningful end points appropriate for the...
Some infants fail to achieve or sustain the normal decrease in pulmonary vascular resistance (PVR) at birth, leading to severe respiratory distress and hypoxemia, which is referred to as persistent pulmonary hypertension of the newborn (PPHN). PPHN is a major clinical problem, affecting up to 10% of full-term and preterm infants in neonatal intensive care, and contributing significantly to high morbidity...
The right-to-left shunting in patients with a large ventricular septal defect (VSD) is caused by increased pulmonary vascular resistance (PVR) secondary to pulmonary vascular disease. The pulmonary circulation hugely impacts the clinical phenotype of many congenital heart lesions. For example, PVR is a major determinant of pulmonary blood flow (PBF; hence left ventricular output and therefore the...
Pulmonary arterial hypertension associated with congenital left-to-right shunts remains a matter of concern not only in underserved areas, but also in developed countries, in both un-operated on and operated on patients. The risk of developing advanced pulmonary vascular disease generally but not invariably depends on the size and location of the defect. Patients with restrictive ventricular septal...
Congenital heart disease (CHD) is the most common birth defect, with an incidence of approximately 0.8% of live births. Improvements in surgery have altered the impact of CHD enormously, not only improving mortality in many conditions but also allowing some infants to survive into adulthood when previously they would have died within the first few days after birth. This increased survival has led...
Pulmonary veno-occlusive disease (PVOD) is a rare and highly lethal disorder of the pulmonary vasculature. In contrast to the insights into pathophysiology and management that have characterized the diagnosis and treatment of idiopathic pulmonary arterial hypertension (IPAH; formerly known as primary pulmonary hypertension) over the past decade, the pathophysiologic mechanisms underlying PVOD are...
Pulmonary venous hypertension is now one of the most common causes of pulmonary hypertension (PH) diagnosed by both pulmonologists and cardiologists. Patients with pulmonary venous hypertension will typically have elevated pulmonary venous pressure (as reflected in the pulmonary capillary wedge pressure), most frequently as a reflection of increased left ventricular end-diastolic pressure. Although...
Chronic obstructive lung diseases and so-called cor pulmonale have traditionally been connected by the link of pulmonary hypertension (PH). The concept that right ventricular hypertrophy is attributed to the afterload of the right ventricle, i.e., attributable to PH, has not been challenged and clinicians continue to attempt to explain cor pulmonale as a consequence of chronic PH. Both pulmonary parenchyma...
Interstitial lung diseases (ILDs) are a distinct type of chronic respiratory disorder that can result in pulmonary hypertension. There are numerous causes of ILD but all are characterized by dyspnea and abnormal lung function, with arterial oxygen desaturation occurring as the disease advances. Patients with idiopathic pulmonary fibrosis (IPF), a relentlessly progressive form of ILD, are particularly...
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