Classification Of Pulmonary Hypertension
There are many different reasons a person may develop pulmonary hypertension. The World Health Organisation classifies pulmonary hypertension by these underlying causes. These classifications include:-Group 1: Pulmonary hypertension from genetic or unknown causes, drug-induced, or related to connective tissue diseases-Group 2: Pulmonary hypertension from failure of the left side of the heart-Group 3: Pulmonary hypertension from lung diseases and chronic low levels of oxygen-Group 4: Pulmonary hypertension from chronic blood clots on the lungs-Group 5: Pulmonary hypertension from mixed or miscellaneous causes.
Pulmonary hypertension is also classified by its severity and the impact it has on a persons activities. These classifications include:
–Functional Class I: symptoms do not limit the persons physical ability-Functional Class II: symptoms result in slight limitation of physical ability, but the person is comfortable at rest. Ordinary physical activity may cause shortness of breath-Functional Class III: symptoms result in marked disability, with less than ordinary physical activity bringing on breathlessness. Comfortable at rest-Functional Class IV: symptoms at rest. Severely limited by shortness of breath.
Pulmonary Hypertension: Prognosis And Life Expectancy
What is pulmonary hypertension?
Pulmonary hypertension is a serious health condition that results when the arteries carrying blood from the right side of the heart to the lungs are constricted, disrupting blood flow. Blood must travel through the lungs for air exchange in order to pick up oxygen that it delivers to all the organs, muscles, and tissue in the body.
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.
There are five types, or groups, of PH:
Prognosis Of Pulmonary Hypertension
Whether your pulmonary hypertension gets better or worse, and how quickly, depends largely on whats causing it and other individual factors.
Theres no direct cure for pulmonary hypertension, but in some people, the condition may be improved or resolved if its caused by a treatable underlying health condition.
But in other people, pulmonary hypertension gets worse over time and may lead to life-threatening complications. You may be able to improve your symptoms, and prevent or delay the worsening of pulmonary hypertension, by taking drugs or following other treatments as prescribed and making recommended lifestyle changes.
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Talk With Others Who Understand
By joining myPHteam, the social network for people with pulmonary hypertension and their loved ones, you can ask questions and share advice with more than 46,000 members who understand life with pulmonary hypertension.
What diet and nutrition tips do you have for living with PH? Share your experience in the comments below, or start a conversation by posting on myPHteam.
How Does Pulmonary Hypertension Affect My Body
Pulmonary hypertension can cause serious problems in your body, including:
PH is dangerous for people who are pregnant. It can cause complications for both the birthing parent and fetus.
Without treatment, pulmonary hypertension can overtax your heart and eventually be fatal. High blood pressure in your pulmonary arteries forces your heart to work harder to send oxygen-poor blood to your lungs. Your right ventricle is responsible for pumping this blood to your lungs. So, over time, PH causes your right ventricle to get bigger due to the extra work. This condition can lead to right-sided heart failure.
Right-sided heart failure has a ripple effect throughout your body. It can disrupt the normal workings of many organs and systems.
Because pulmonary hypertension can affect your entire body, its essential that youre diagnosed and treated as early as possible. Your provider will prescribe treatment based on whats causing your PH. No matter the cause, untreated PH is life-threatening.
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Investigation Into Pulmonary Hypertension
The series of tests required to confirm a diagnosis of pulmonary hypertension can be quite extensive. Your first appointment with your specialist might just be spent organising these tests, which may include:
If your doctor asks you to have any tests before the appointment, please do so because it will speed up the process of determining what the nature of your pulmonary hypertension may be, and how to treat it.
Pah Associated With Other Conditions
PAH can be associated conditions that affect your blood vessels, such as:
- pulmonary veno-occlusive disease a rare condition that causes high blood pressure in the lungs
- pulmonary capillary hemangiomatosis when capillaries grow within the lungs, causing blockages
Other conditions or treatments associated with PAH include:
- a condition affecting the structure or composition of your body tissue, such as scleroderma
- surgery to artificially connect the aorta to the pulmonary artery this is often done to treat congenital heart disease and increase the blood supply to the lungs
- portal hypertension a condition where the blood pressure inside the liver is abnormally high, leading to the development of swollen veins
- HIV one in 200 people with HIV will develop PAH
- certain medications or drugs such as fenfluramine, which is now no longer available, and amphetamines, a type of illegal drug
- disorders of the thyroid gland
- glycogen storage disorder
- splenectomy when you have had your spleen surgically removed
- sickle cell anaemia
Sickle-cell anaemia is an inherited disorder where your red blood cells are abnormal and die prematurely, leading to a shortage of red blood cells. The lack of red blood cells means that haemoglobin, a protein normally found in these cells, is released into the blood.
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Nitric Oxide In Cardiac Surgery
Pulmonary hypertension can occur even after cardiopulmonary bypass because of microembroli, ischemia, sedimentation of blood cells or stress reaction. Existing methods of reducing pulmonary resistance before and after surgery include systemic alkalization, O2 therapy, increased cardiac contractility, stress reaction reduction, anticoagulant drugs and vascular relaxants. The most commonly used drugs are alpha-adrenal receptor antagonists, beta-lactam antibiotics, nitrates, Ca++ channel blockers and prostaglandins. However, these drugs seriously reduce systemic vascular resistance and cause unstable vital signs . When PHT cannot be corrected with drugs, extracorporeal circulation is conducted to induce oxygenation however, there are no special methods of treatment besides symptomatic therapy, and often needed long-term hospitalization.
Schematic representation of the cGMP pathway that mediates vasodilation and inhibition of cellular proliferation of drugs.
Pathophysiology And Pathology Of Pulmonary Hypertension
According to Ohms law, factors affecting pulmonary pressure are pulmonary blood flow and the pulmonary vascular resistance. Accordingly, changes in right cardiac output , setting of pulmonary vascular resistance and venous pressure of pulmonary system are the major factors of pulmonary hypertension . This equation represents that pulmonary arterial hypertension depends on three factors:
First: right cardiac output
Second: pulmonary vascular resistance
Third: mean pulmonary artery pressure .
Accordingly, diseases such as atrial septal failure, ventricular septal failure and many congenital heart diseases such with the increase of blood flow , diseases like pulmonary embolism, rheumatic diseases and pulmonary interstitial diseases through the overall decrease of vascular setting and increase of the pulmonary hypertension resistance and diseases such as miteral stenosis, pulmonary venous occlusive and heart failure through the increase of venous pressure , all cause pulmonary hypertension . It shall be noted that in many cases, there are a couple of factors involved in PH.
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Research And Statistics: Who Has Pulmonary Hypertension
There are few reliable estimates of how common adult pulmonary hypertension is in the United States, in part because its often related to a variety of other health conditions. Group 1 PAH is considered a rare disease, with 500 to 1,000 new cases diagnosed each year, according to the American Lung Association.
While pulmonary hypertension is more common with older age, it affects people of all ages. Its more common in women, non-Hispanic black people, and people over age 75 than in other groups in the United States.
Worldwide, the incidence of pulmonary hypertension appears to be growing, according to a study published in February 2018 in the journal Circulation: Cardiovascular Quality and Outcomes. From 1993 to 2012, the number of people living with condition each year went from 99.8 to 127.3 per 100,000 people. The most common type of pulmonary hypertension is group 2 , making up 34.2 percent of cases, with an overlap of group 3 that also meets criteria for group 2 making up another 29.3 percent.
Research Into Pulmonary Hypertension
Pulmonary hypertension is an area of intense research, particularly with respect to:
- the correct type of medication to use in the different groups of pulmonary hypertension, and in which combinations
- management of right heart failure, and possible new treatment targets or therapies
- treatments at the molecular level, such as monoclonal antibodies, which may be useful tools to slow disease progression
- further genetic markers that may identify people at high-risk of developing pulmonary hypertension.
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What Are The Different Types Of Pulmonary Hypertension
The World Health Organization divides pulmonary hypertension into five groups based on its cause.
- Group 1 PH due to pulmonary arterial hypertension . PAH has many different causes, ranging from underlying diseases to certain drugs. PAH makes your pulmonary arteries become narrow, thick or stiff. Less blood can flow through, which raises the pressure in your pulmonary arteries.
- Group 2 PH due to left-sided heart disease. The left side of your heart pumps out blood to your entire body. If theres a problem on this side of your heart, it affects the right side of your heart and your entire pulmonary circuit. Blood backs up in your heart, raising the pressure in your pulmonary arteries.
- Group 3 PH due to lung disease or hypoxia. Certain lung problems cause the arteries in your lungs to tighten. Less blood can flow through your lungs, raising the pressure in your pulmonary arteries.
- Group 4 PH due to blockages in your lungs. Blood clots or scars from blood clots prevent your blood from flowing normally through your lungs. This puts more stress on the right side of your heart and raises pulmonary blood pressure.
- Group 5 PH due to other disorders. PH occurs along with other conditions like blood disorders and metabolic disorders. The exact mechanisms for how the condition triggers PH arent always clear.
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B History Part : Prevalence:
These are updated risk factors for PAH as outlined from the Dana Point World Symposium in 2008. Risk factors for PAH include any factor or condition that is suspected to play a predisposing or facilitating role in the development of the disease.
Definite association defined as an epidemic or from a large, multicenter epidemiologic study
aminorex, fenfluramine, dexfenfluramine, toxic rapeseed oil
Possible association defined as drugs with similar mechanisms of action as those in the definite or likely categories but which have not been studied
cocaine, phenylpropanolamine, St. Johns Wort, chemotherapeutic agents, SSRI
Likely association defined as a single-center, case-control study demonstrating an association or a multiple-case series
amphetamines, L-tryptophan, methamphetamines
Recent observational studies have reported methamphetamine abuse significantly increases the risk of developing PAH
Unlikely association defined as one in which a drug has been studied in epidemiologic studies and an association with PAH has not been demonstrated
Oral contraceptives, estrogen, cigarette smoking
A. Idiopathic PAH corresponds to sporadic disease in which there is neither a family history of PAH nor an identified risk factor.
B. Heritable PAH when PAH occurs with a family history. Mutations in the BMPR2 gene can be detected in approximately 70% of the cases that are transmitted in autosomal dominant pattern.
B. First degree relative of a patient with BMPR2 mutation
What Tests Diagnose Pulmonary Hypertension
Your provider may use several different tests for different purposes.
These tests measure the blood pressure in your pulmonary arteries:
- Right heart catheterization: This test is also called pulmonary artery catheterization. It measures the pressure inside your pulmonary arteries and checks how much blood your heart can pump per minute.
- Doppler echocardiogram: A Doppler echo uses sound waves to show how your right ventricle is working. It also measures blood flow through your heart valves. It allows your provider to calculate your systolic pulmonary artery pressure.
These tests look for the underlying cause of pulmonary hypertension:
- Blood tests: Check for a range of issues related to organ function, hormone levels and infections. Specific blood tests include a complete metabolic panel and a complete blood count.
- Chest CT scan: Looks for blood clots and other lung conditions that may be causing your pulmonary hypertension or making it worse.
- Chest X-ray: Shows if your right ventricle or pulmonary arteries are bigger than they should be.
- Polysomnogram : This overnight sleep test checks if you have sleep apnea.
- Pulmonary ventilation/perfusion scan: Looks for blood clots in your lungs.
Your provider may also perform a six-minute walk test. This test shows how much exercise you can handle and how much oxygen is circulating in your blood as you exercise. The results indicate if your pulmonary hypertension is mild or severe.
Ii Diagnostic Confirmation: Are You Sure Your Patient Has Pulmonary Arterial Hypertension
If PH is suspected , an echocardiogram is the next step. The Doppler echocardiogram provides the following:
Echocardiographic Findings to Differentiate PAH vs. PVH
Right Heart Catheterization: Necessary To Diagnose PAH
Right heart catheterization is needed in PAH to:
Establish the diagnosis of PAH by confirming that pulmonary artery pressures are elevated with normal left sided filling pressure.
Perform acute vasodilator testing for patients with a new diagnosis of PAH of idiopathic, hereditary, or anorexigen-associated conditions.
Assess prognosis of PAH based on RAP and cardiac output measurements.
Guide treatment of the patient
Range of Normal Pressure Obtained with Invasive Hemodynamic Assessment
Pulmonary Artery Pressure Measurements in PAH
The PA diastolic pressure is close to both the mean PCWP and left ventricular end-diastolic pressure in normal individuals . The PA diastolic pressure does not correlate well with the mean PCWP in the presence of pulmonary vascular disease where the PA diastolic pressure overestimates the mean PCWP. The lack of correlation is also seen in mitral regurgitation with large v wave where the PA diastolic pressure underestimates the mean PCWP.
Common Pitfalls in Obtaining Accurate PCWP: Underwedging or Hybrid Waveform
Common Pitfalls in Obtaining Accurate PCWP: Overwedged Waveform
Common Pitfalls in Obtaining Accurate PCWP: The V Wave
Characteristics of a Physiologically Reliable PCWP
What Is The Life Expectancy For People With Pulmonary Hypertension
The life expectancy varies from person to person. It depends how quickly youre diagnosed and what other medical conditions you have. Talk with your provider about what you can expect in your individual situation.
Pulmonary hypertension is a progressive disease. That means it gets worse over time. It progresses more quickly in some people than in others. Treatment can improve your chances of surviving pulmonary hypertension for many years.
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What Is Pulmonary Arterial Hypertension
Pulmonary hypertension is a life-threatening condition that gets worse over time, but treatments can help your symptoms so you can live better with the disease. It may take some planning, but plenty of people who have it find ways to do all the things they love, just as they did before they were diagnosed.
With PAH, the tiny arteries in your lungs become narrow or blocked. It’s harder for blood to flow through them, and that raises the blood pressure in your lungs. Your heart has to work harder to pump blood through those arteries, and after a while the heart muscle gets weak. Eventually, it can lead to heart failure.
Diet And Nutrition Tips For Pulmonary Hypertension
Everyone can benefit from eating a healthy diet, but for people living with pulmonary hypertension , proper nutrition is a powerful tool for managing the condition. Pulmonary hypertension is a type of high blood pressure in the blood vessels of the lungs. It can lead to symptoms, such as shortness of breath, and serious complications, such as heart failure. Specific dietary practices can help people with PH control symptoms and prevent or repair damage caused by disease.
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