Show
Pulmonary hypertension (PH) is a life-limiting disease affecting circulation to the lungs.[1] Pulmonary hypertension (PH): an increase of blood pressure in the pulmonary circulation, characterized by remodeling of the pulmonary vasculature, with mean pulmonary arterial pressure (mPAP) > 25 mmHg. In PH the arteries carrying blood from the right side of the heart to the lungs are constricted, When the arteries between the heart and lungs become narrowed and flow is constricted, the heart has to work extra hard to pump blood to the lungs. Over time, the heart can grow weak and proper circulation can diminish throughout the body[2]. This leads to eg, shortness of breath, exercise intolerance. In addition to cases of idiopathic pulmonary arterial hypertension, there are numerous known causes, and these can be subdivided in many ways.[3] It is a rare condition with an estimated prevalence in the range of 10–52 cases per million.
Classification[edit | edit source]It can arise from a variety of causes and is classified (Nice classification) into Five groups:
Anatomy - Pulmonary Circuit[edit | edit source]The pulmonary circuit begins in the right atrium, where practically all the venous blood in the body reaches, passes to the right ventricle and from there it is driven to the alveolar territory through the pulmonary artery that ends in an extensive capillary network that surrounds the alveoli. The blood is separated from the alveolar air by a membrane half to one micron thick. Once arterialized, the blood is carried by the pulmonary veins to the left atrium, where it joins the greater circuit. Epidemiology[edit | edit source]Epidemiology varies with the underlying cause and risk factors. Overall, there is a female predilection. Risk factors include: Pathology[edit | edit source]Pulmonary arterial hypertension is defined as a mean pulmonary arterial pressure >25 mmHg at rest 11 or >30 mmHg with exercise and pulmonary capillary wedge pressure ≤15 mmHg measured by cardiac catheterisation. [5] Image: A simple figure describing pulmonary artery catheter. Construction of a pulmonary artery catheter (Swan-Ganz catheter). The catheter has four lumens. These are the proximal canal (1), which comes to lie in the right atrium and u. a. serves to measure the central venous pressure, the channel that leads to the temperature probe and enables a cold dilution measurement (2), the channel for inflating the balloon (3) and the distal channel with which the pressure in the pulmonary artery (PAP) and the pulmonary artery occlusion pressure ( PCWP, wedge pressure) is measured (4). In this schematic diagram, the catheter is in the wedge position in which the balloon closes the pulmonary artery. PH can result from either increased pulmonary venous resistance (most common) or increased pulmonary arterial flow, such as with a left-to-right shunt. The main factor in generating severe pulmonary hypertension is an arteriopathy, which has four main components:
NB Plexogenic arteriopathy is a term used to describe a constellation of vascular changes occurring in those with pulmonary arterial hypertension. The earliest change is muscular hypertrophy in muscular arteries which with time result in changes in the more proximal arteries. Eventually, fibrosis of the wall occurs, at which point the process is irreversible.[3] Clinical presentation[edit | edit source]Classical clinical presentation of pulmonary arterial hypertension is the combination of
Treatment and Prognosis[edit | edit source]
Pulmonary arterial hypertension (PAH) carries a poor prognosis if not promptly diagnosed and appropriately treated. The development and approval of 14 medications over the last several decades have led to a rapidly evolving approach to therapy, and have necessitated periodic updating of evidence-based treatment guidelines[6]. Despite extensive research and recent advances in medical management prognosis remains poor, with a mean survival of only three years in untreated patients. Patients typically succumb to right heart failure or sudden death[3]. Physiotherapy[edit | edit source]A range of common interventions that contributed to acute patient recovery and discharge by physiotherapists may include:
Pulmonary rehabilitation is a widely researched intervention for different respiratory conditions and referral for such intervention is of benefit. Give Activity Guidelines:
[edit | edit source]
References[edit | edit source]
|