SAPH – Saudi Thoracic Society

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Saudi Association for Pulmonary Hypertension (SAPH)

Objectives & Activities

The Saudi Association for Pulmonary Hypertension (SAPH) is a medical and research national group of many medical specialists, who shared a common interest in the field of pulmonary vascular disease. The main goal for SAPH is to increase the awareness and the knowledge of pulmonary vascular diseases in the society and between the medical professionals, and to start variety of research projects in the field of pulmonary vascular disease in the region, in order to improve the understanding, and quantify the size of the problem in this part of the world. SAPH is registered and operates under the Saudi Thoracic Society.

SAPH Objectives:

To investigate the incidence, prevalence, and pathobiology of PVD in underserved areas that, to date, have not been well characterized
To identify unique characteristics of the illness including its morbidity and mortality
To establish effective therapies, which will be accessible to the affected patients
To provide expertise to areas with healthcare disparities that will address issues related to the education and training of healthcare professionals in the area of PVD
To focus on improving the treatment of PVD, by promoting research, by educating physicians, and by assisting the development and conduct of clinical trials following the highest standards of clinical research
Pleural Diseases
To provide consultative services to health agencies and industry regarding advancing the treatment of PVD.

Activities:

To investigate the incidence, prevalence, and pathobiology of PVD in underserved areas that, to date, have not been well characterized
To identify unique characteristics of the illness including its morbidity and mortality
SAPH provides expertise to advise physicians, scientists, health authorities and the healthcare industry regarding medical technologies and pharmaceuticals relevant to PVD
SAPH is establishing a national database on patients with PVD to allow a meaningful understanding of the similarities and differences in the spectrum of the illness between different regions in the kingdom and other countries in the world
SAPH has developed a web-based, multilingual educational materials and guidelines on the management of pulmonary vascular disease accessible to people involved in healthcare delivery
SAPH has been conducting workshops on the modern methods to evaluate patients, determine accurate diagnoses, and monitor the efficacy of treatments

SAPH Executive Board

Chairman
Abdullah M. Aldalaan, MD
Chairman, Saudi Association for Pulmonary Hypertension
Consultant Pulmonologist & Intensivist
King Faisal Specialist Hospital and Research Centre
Riyadh, Saudi Arabia

Member
Majdy Idrees, MD, FRCPC, FPVRI
Adjunct Professor of Respiratory Medicine
University of British Columbia, Vancouver, Canada
Head, Pulmonary Vascular Unit
Prince Sultan Military Medical City
Riyadh, Saudi Arabia

Member
Saleh Al Dammas, MD
Consultant, Pulmonary and Sleep Medicine
Prince Sultan Military Medical City
Riyadh, Saudi Arabia

Member
Tarek Kashour, MD, FRCPC, FACC
Adult Cardiology
Head, CHD Taskforce, SAPH
King Fahd Medical City,
Prince Salman Heart Center
Riyadh, Saudi Arabia

Member
Sarfraz Saleemi, MD
Pulmonary Medicine
Riyadh, Saudi Arabia

Member
Hanaa Banjar, MD, FRCPC
Associate Professor of Pediatrics and
Pediatric Pulmonology, Alfaisal University
Consultant Pediatric Pulmonology,
Department of Pediatrics
King Faisal Specialist Hospital and Research Centre
Riyadh, Saudi Arabia

Member
Badr R. Al-Ghamdi, MD
Associate Professor and Consultant Pulmonologist
Chairman Department of Medicine
College of Medicine, King Khaled University
Director of Chest unit, Aseer Central hospital
Abha, Saudi Arabia

Member
Manal S. Al Hazmi, MD
Consultant, Pulmonary and Critical Care Medicine
King Fahad Specialist Hospital, Dammam
Dammam, Saudi Arabia

Member
M. Ali Al Azem, MD, MHA, FCCP
Chairman, Department of Critical Care
Consultant, Pulmonary, Critical Care & Sleep Medicine
King Fahad Specialist Hospital- Dammam
Dammam, Saudi Arabia

Member
Abdulmajeed Al-Otay, MD
Consultant Pediatric Cardiologist,
Head of Cardiac Imaging Unit,
Prince Sultan Cardiac Center
Riyadh, Saudi Arabia

Member
Khalid Al Najashi, MD, MBBS
Consultant, Interventional ACHD and Pediatric Cardiology
Prince Sultan Cardiac Center
Prince Sultan Military Medical City- Riyadh
Riyadh, Saudi Arabia

Member
Hussam Sakkijha, MD
Chairman, Scientific Committee
Consultant, Pulmonary, Critical Care and Sleep Medicine
King Fahad Medical City, Riyadh
Riyadh, Saudi Arabia

Member
Abdulghafour Gari, MD
Consultant Pulmonologist,
Head of Pulmonary hypertension Program
National Guard Hospital
Jeddah, Saudi Arabia

Member
Hanan Al Rayes, MD
Consultant Rheumatology,
President of Saudi Society for Rheumatology
Deputy Director of Medicine Department
Prince Sultan Military Medical City
Riyadh, Saudi Arabia

Member
Hassan S. Alorainy, BsRC, RRT, FAARC
Executive Director, Saudi Association for
Pulmonary Hypertension (SAPH)
Senior Clinical Respiratory Specialist
Riyadh, Saudi Arabia

Member
M Omar Galal, MD
Pediatric Cardiology
Riyadh, Saudi Arabia

What is PH?

Pulmonary Hypertension Overview

Pulmonary hypertension (PH) is a rare lung disease that refers to an increase in blood pressure in the pulmonary arteries. These are the arteries that carry deoxygenated blood from a person’s heart to the lungs to pick up oxygen. Pulmonary hypertension is a chronic disease that can lead to a poor quality of life. PH can also lead to right heart failure if left without medical intervention, a serious complication that can lead to death. PH is said to be present when blood pressure of the lungs becomes greater than 25 mm of mercury (Hg) when a person is resting, or 30 mm Hg when exercising. Normal pulmonary artery pressure runs between 8 and 20 mm Hg at rest.

For people who have been diagnosed with HP years ago, the classification system was divided up into 2 categories known as primary and secondary forms, based on whether a specific cause could be determined. Primary HP was diagnosed if the cause of PH was unknown, and Secondary PH was diagnosed if the increased pulmonary artery pressure was due to some medical condition. In the late nineties, the World Health Organization (WHO) suggested that PH be classified into 5 categories based on similarities of pathophysiology, clinical presentation and medical treatments. The classification system has been revised again in 2003 and 2008. Whether or not PH is primary or secondary or is classified according to the new nomenclature, the disease results in a thickening of the pulmonary arteries, which causes narrowing of these blood vessels. As a result, the right side of the heart has to work harder to pump blood through the highly resistant pulmonary arteries. This causes the heart to become enlarged. As time goes on, over working the right side may cause right-side heart failure, which may result in death.

Pulmonary Hypertension Heart Anatomy Understanding The Heart in PH

For patients who are not familiar with heart and lung anatomy and physiology, some basic information is helpful. The human heart has two sides that are separated by an inner wall, known as the septum. Each side is further divided into an upper and lower compartment. The upper right compartment is known as the right atrium and it receives deoxygenated blood coming back from the body by way of the vena cava. The right atrium passes blood to the right ventricle (lower compartment), and the right ventricle is responsible for pumping blood to the lungs by way of the pulmonary artery. The pulmonary artery branches into capillaries in lung tissue, and this is where carbon dioxide is exchanged for oxygen from the lungs. The capillaries in the lungs turn into the pulmonary veins and these veins carry oxygenated blood back to the heart. Oxygenated blood enters into the left atrium (upper left compartment). The left atrium pumps blood into the left ventricle (left bottom compartment) and the left ventricle pumps blood to the aorta. Oxygenated blood is now available for the whole body. Inflammation and changes in the cells that line the pulmonary arteries can bring about pulmonary hypertension. Inflammation can restrict blood flow due to a smaller blood vessel diameter and the altered cell lining can make the arterial wall stiff not allowing for expansion when needed as blood flow increases. Blood clots are yet again another issue. There are a number of factors that can cause PH and we’ll take a look at those shortly as the causes are related to PH classification. Nevertheless, these arterial changes make it difficult for the heart to pump blood to your lungs and the damaged blood vessels have high resistance to blood flow. This causes the blood pressure in the pulmonary arteries to rise making your heart work even harder and this worsens with time. Eventually the right ventricle becomes enlarged and weak. The right ventricle may become so weak that it can no longer pump blood to the lungs. This results in heart failure and this is the most common cause of death in individuals who have PH.

Pulmonary Hypertension Symptoms

Unfortunately, the symptoms of PH are generally not apparent early on in the onset of the disease, and this may be the case for years to come. However, as PH progresses, signs and symptoms of the disease will become apparent.

Listed below are some of the most common symptoms associated with PH. Patients need not necessarily present with most or all of these symptoms for the disease to present, however, these symptoms are generally observed in the PH patient population:

Fatigue which may be due to poorly oxygenated blood;
You may experience shortness of breath. In early phase this may occur when you are exercising but later will progress and become apparent when you are resting;
You may experience pain or perhaps pressure in the chest area;
Fast heart beat or skipped beats (palpitations or arrhythmias);
You may feel faint or dizzy which is known as syncope;
Your lips or skin may turn blue, this is referred to as cyanosis (lack of oxygen in the blood);
Swelling in your legs and ankles/feet also known as edema
Internal lung bleeding and coughing up blood (hemoptysis);

Pulmonary Hypertension Diagnosis

Find out what you need to know about the Pulmonary Hypertension Diagnosis process.

Like any other disease, a patient’s healthcare provider will diagnose PH by collecting information about medical history as well as family history. A physical exam will be administered along with a number of other tests and procedures. Symptoms of PH can develop over a long period of time and mimic other heart and lung conditions (i.e. bronchitis and asthma), making PH very difficult to diagnosis. Mis-diagnosis is not uncommon. However, initially, a doctor will try to confirm a PH diagnosis and look for an underlying cause. Remember, there can be many causes to PH. Tests to confirm PH There are a number of different diagnostics for Pulmonary Hypertension Diagnosis. Note that not all of the tests below will necessarily need to be conducted in order to determine PH in a patient. In addition to these tests, medical and family history along with symptoms will narrow down what tests are appropriate.

Echocardiogram – Sound waves generate a moving picture of the heart. This can measure pulmonary artery pressure. The doctor can also observe the thickness and size of the heart’s right ventricle. This is a safe, noninvasive procedure.
Chest X-ray – This can tell the doctor if the heart’s right ventricle and pulmonary arteries are enlarged, as well as general heart and lung anatomy. X-rays have been known to demonstrate signs of underlying lung disease making them very useful for PH diagnosis.
Electrocardiogram (EKG) – This device measures electrical impulses from the heart with external electrodes placed on the body. This test can see whether a person’s heartbeat is regular or irregular, and may indicate if the heart’s right ventricle is enlarged. This is a safe and noninvasive procedure.
Right heart catheterization – This test uses a flexible tube (catheter) which is inserted into a blood vessel in a patent’s groin or neck region. The tube is then threaded into the right side of the heart and into the pulmonary arteries. This test can measure pulmonary artery pressure and can demonstrate how efficiently the heart is pumping blood to the rest of the body. This system allows the physician to apply treatments to the heart as well.

Tests to Find the Underlying Cause of PH Pulmonary Hypertension MRI

Tests to confirm PH are important, however, beyond that, patients need to know what caused the onset of pulmonary hypertension. This is important, as it will determine an appropriate treatment(s). Remember: there are many causes for all of the PH forms, so the underlying cause must be determined.

Lung function tests – These tests measure how well a patient’s lungs work, and are used to determine the cause of breathing problems. These tests look at how much air a patient can take into their lungs, and how much they can blow out, etc. This helps the doctor determine whether a patient has asthma, COPD (chronic obstructive pulmonary disease), or something else.
CT scan (computerized tomography) – This scan is done on the chest to get a picture of the structures within the chest, such as blood vessels, lungs, and heart. Lesions observed may indicate an underlying cause for PH.
Magnetic resonance imaging (MRI) – An MRI can be done on the chest that can provide information about how the heart’s right ventricle is working. It can also demonstrate how blood flows through the lungs. MRI can help identify an underlying cause of PH.
Polysomnogram or (PSG) – This is an interesting test in that it is given to patients while they sleep. This system records information about blood pressure, heart rate, brain activity, and eye movements. It can measure oxygen levels in blood which is important because PH patients commonly have low oxygen levels when they are asleep.

Familial or Heritable Pulmonary Hypertension

As with many diseases, there can be genetic predispositions for certain disease due to the presence of mutations. Pulmonary hypertension is no exception and research has demonstrated that mutations in the BMPR2 gene causes pulmonary arterial hypertension. It is believed that a mutation in BMPR2 actually promotes cell division (proliferation) or you prevents cell death. The result of this is an overgrowth of cells in the small arteries of the lungs. This in turn causes a narrowing of the vessel lumen effectively decreasing its diameter which increases resistance to blood flow. A larger diameter blood vessel has less resistance to flow. Blood pressure in the heart (right ventricle) and pulmonary artery increases to accommodate the increased resistance to flow. Research is currently underway to determine if other genes and possibly environmental factors might be involved as well.

It is known that BMPR2 is inherited in an autosomal dominant pattern. This means that each cell that expresses this gene has one copy of a mutated BMPR2 gene meaning you still have one good allele. People have two copies of a gene: one you got from your mother and one you got from your father. With one bad copy, this presents with predisposition. In other words, people need to come in contact with something that causes the good allele to mutate. This is what leads to contracting the disease. It has been reported that many individuals with a mutated BMPR2 gene never present with PAH. Nevertheless, the mutated gene will be passed down from generation to generation.

Pulmonary Hypertension Prognosis

Looking for information on the Pulmonary Hypertension Prognosis process? Find out what you need to know here.

Once a person is diagnosed with Pulmonary Hypertension, it may take a considerable amount of time to determine the best therapy and course of action. Since there are a number of causes for HP, the therapies can be complex and require repeated follow-up care. Patients may have to have their therapy changed if and when it becomes ineffective. Unfortunately, at this time, there is no cure for PH, however, early diagnosis makes it easier to control and ensures a better quality of life. It was once thought that PH was a disease that primarily affected women of childbearing age, but it is now known that it can affect many ages and races. However, older women account for the majority of cases and decedents with this condition. Depending on when a person is diagnosed and the particular type of HP they have, the mortality rate can be as high as 90 percent within 3 to 5 years.

PH WHO Classification

Pulmonary Hypertension WHO Classification

Pulmonary hypertension (PH) is a severe, rare lung disease characterized by high blood pressure in the pulmonary arteries. Due to the condition, the pulmonary arteries, which are responsible for transporting the blood from the right heart ventricle to the lungs, become narrowed and thickened. In order to properly pump the blood, the heart needs to work harder, which can lead to enlargement and weakening of the organ, as well as potential right heart failure.

There is currently no cure for pulmonary hypertension, but there are treatments that can help patients cope with the symptoms, which include shortness of breath, tiredness, chest pain or pressure, irregular heartbeat, pain on the upper right side of the abdomen and decreased appetite. Symptoms, diagnosis and treatment of pulmonary hypertension are different according to the different subtypes of the disease. In order to produce, maintain and implement international health information standards, the World Health Organization (WHO) defined a PH classification.

How Is The Pulmonary Hypertension WHO Classification Determined

The International Classification of Diseases (ICD) is created by the organization and comprises a standard diagnosis tool for physicians, nurses, other providers, researchers, health information managers and coders, health information technology workers, policy-makers, insurers and patient organizations. To determine the classification of pulmonary hypertension, the WHO analyzed the general health situation of patients, as well as the disease’s incidence and prevalence.

The pulmonary hypertension WHO classification is used for numerous purposes, including records, death certificates, information storage, diagnosis retrieval, national mortality and morbidity statistics, reimbursement, resource allocation, and other clinical, epidemiological and quality purposes, and the organization expects it to help fight the disease.

Different Pulmonary Hypertension WHO Classification Groups

The pulmonary hypertension WHO classification was updated in 2009, placing a greater importance on treating the underlying cause of the disease. There are now five groups, while the previous classification was more focused on whether the underlying cause was known and included only two categories: primary or idiopathic pulmonary hypertension, and secondary pulmonary hypertension. In all of the different groups, the average pressure in the pulmonary arteries is 25 mmHg or more at rest or 30 mmHg during physical activity, the normal levels between 8 and 20 mmHg at rest.

Group 1 includes the subtypes of pulmonary hypertension associated with abnormalities in the arterioles, the small branches of the pulmonary arteries, such as pulmonary arterial hypertension (PAH), and idiopathic pulmonary hypertension (IPH), which is the type of PH diagnosed when the causes of the disease are not known. Also in this group is inherited pulmonary hypertension, as well as pulmonary hypertension caused by connective tissue diseases that affect the body’s structure or composition of the tissue like scleroderma, congenital heart problems, high blood pressure in liver (portal hypertension), HIV, thyroid gland disorder, sickle cell disease, glycogen storage disorders, and rare blood conditions like pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis.

Group 2 refers to pulmonary hypertension caused by left heart disease, including cardiomyopathy, diastolic dysfunction, mitral stenosis, mitral regurgitation, aortic stenosis, and aortic regurgitation. The left side of the heart pumps blood to the entire body but the lungs, but about 60% of patients with severe left ventricle dysfunction also develop pulmonary hypertension, since the blood flows through the entire heart, affecting the right side as well.

Group 3 includes pulmonary hypertension resulting from lung diseases or shortage of oxygen in the body (hypoxia), with chronic obstructive pulmonary disease (COPD); interstitial lung disease; and sleep-disordered breathing, a group of diseases that affect the breathing during the sleep like obstructive sleep apnoea (OSA) being the most common diseases that can result in pulmonary hypertension.

Group 4 refers to pulmonary hypertension secondary to blood clots, which are the body’s response to bleeding and injuries but can harm the heart and lungs when they occur without an apparent cause. Pulmonary embolus are blood clots that travel to the lungs and pulmonary thrombosis are clots that are formed in the lungs, which can block the pulmonary arteries.

Group 5 is the last category and it includes other less common causes that do not fit into any of the other four groups. Sarcoidosis, which is a condition that results in inflammation of different organs like the lungs and lymph nodes, histiocytosis X, a rare disorder that causes scarring, granulomas and air-filled cysts mostly in the lungs, as well as compression of the blood vessels in the lungs, which can occur for numerous reasons like tumors, are some of the causes for pulmonary hypertension in the fifth group.

Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) involves abnormally high blood pressure (hypertension) in the pulmonary artery. The pulmonary artery carries blood from the heart (right ventricle) to the lungs. When the smallest of these vessels become narrower in diameter (perhaps due to disease), this increases resistance to blood flow through these vessels. This increases the pressure inside these vessels as well as the right ventricle. Through time, the right ventricle has to pump harder and harder to move blood into the pulmonary artery, causing the heart to become enlarged and weakened. Eventually, the heart (right ventricle) becomes so weakened that it can no longer pump blood and heart failure occurs.

Other names for PAH include Ayerza syndrome, Familial (heritable) primary pulmonary hypertension (FPPH), Idiopathic pulmonary hypertension (arterial), Primary pulmonary hypertension and Sporadic primary pulmonary hypertension.

Pulmonary Arterial Hypertension Causes

As there are many causes of Pulmonary Arterial Hypertension, we’ll keep the list general:

Any number of heart diseases can generate PAH
Viral infections such as HIV
Genetic mutations (this is known as familial or heritable PAH)
Drugs such as cocaine and amphetamines and some prescription diet medications
Scleroderma (autoimmune disease) which is a connective tissue disease that causes fibrosis
Chronic liver diseases
Unknown cause (can’t find a cause) which is referred to as idiopathic PAH

Pulmonary Arterial Hypertension Symptoms

Symptoms of PAH present when the increased blood pressure can’t fully overcome the increased resistance of the pulmonary arteries. This not only has effects on the heart and lungs but to the entire body.

In early phase PAH, patients may experience shortness of breath known as dyspnea during exercise along with possible fainting. As the disease progresses, these symptoms will occur even when patients are at rest:

Dizziness
Swelling of the legs and ankles known as edema.
Pain in the chest region.
tachycardia (fast heart rate) and irregular heart beat (palpitations or arrhythmias).

Pulmonary Arterial Hypertension Diagnosis

A doctor will ask potential PAH patients about family and medical history, and perform a physical exam and determine symptoms. From there s/he will try to confirm PH with tests, such as an echocardiogram, chest X-ray, electrocardiogram (EKG) and/or right heart catheterization. To try and determine the underlying cause, a doctor may perform Lung function tests, chest CT and MRI scans, and/or Polysomnograms. Please refer to the introduction on PH for an explanation of these various procedures.

Pulmonary Arterial Hypertension Treatments

Currently, there is no cure for any of the pulmonary hypertension types, however there are treatments that can help mitigate with symptoms. What therapies or combination of therapies are used depend on the underlying cause(s). The goals of such treatments ares to:

Treat the underlying cause if it is known. It is reported that some therapies can correct some forms of PAH in individuals with secondary pulmonary arterial hypertension (SPAH).
Improve the quality of life by reducing symptoms.
Reduce the heart’s workload by increasing the supply of blood and oxygen.
Reduce the production of blood clots and slow down the proliferation of smooth muscle cells in the blood vessel walls.

Treatments that are available include prescription drugs, oxygen therapy, lung transplants and possibly stem cell therapy.

Pulmonary Arterial Hypertension Medications

Blood thinners are commonly used to decrease the production of blood clots.
Calcium channel blockers help to relax blood vessels. This allows for an increase in blood flow and oxygen to the heart.
Prostacyclin is administered to relax arteries in the lungs and prevents blood clots from forming. There are several forms of this medication. Newer forms of prostacyclin can be inhaled through a nebulizer for fast symptomatic relief.
Nitric oxide, which can be inhaled has the ability to relax pulmonary arteries.
Diuretics which help to remove excess fluid from the body reducing edema.
Phosphodiesterase 5 inhibitors (PDE5) allows blood vessels to relax in the lungs.

Oxygen Therapy

As PAH progresses, blood oxygen levels decline, so patients may need oxygen therapy. The oxygen is administered through a mask or nasal prongs.

Lung Transplantation

If medical treatments are no longer an option for a PAH patient, they may require surgery to replace the diseased lung(s). A donor is required. Patients will have to take medication for the rest of their life to reduce the risk of rejection.

Pulmonary Arterial Hypertension Prognosis

Progress is being made to bring about new diagnostic tools and more efficient medications, however PAH is a fatal disease. It is reported that the survival rate is around 50 percent after 5 years.

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

Pulmonary Venous Hypertension

Pulmonary venous hypertension (PVH) differs from PAH in that high blood pressure occurs when the heart can’t efficiently carry blood away from the lungs. This results in blood collecting in lung tissue. This is generally caused by diseases of the left side of the heart. One such disease is endomyocardial fibrosis. Fibrous connective tissue can accumulate in one or both ventricles and results in a constricted (narrowed) heart chamber. This may also involve other heart structures such as the heart valves causing them to open and close abnormally.

Pulmonary Venous Hypertension Symptoms

PVH presents with many of the same symptoms seen in other forms of HP, however, it differs from PAH in that PVH’s shortness of breath occurs when a patient is in a reclined position or when sleeping. Symptoms include:

Increased blood pressure
Fluid in the lungs and between the layers that surround the lungs and chest wall
Night breathing difficulty
Fatigue
Edema
Decrease in urine production
Coughing that may produce bloody mucus
Decrease in overall heart function

Pulmonary Venous Hypertension Diagnosis

Your healthcare provider will want to know about your medical and family history. A physical exam will be administered to look for known symptoms of PVH such as a loud S2 (valve closure), jugular vein distention (jugular vein protrudes from the side of the neck), foot swelling and abdominal swelling (ascites).

Pulmonary Venous Hypertension Treatment

The goal is to try and treat the underlying causes. To prevent congestive heart failure, medications such as beta blockers, ACE inhibitors and diuretics are used. If a patient has structural heart issues, they may need surgical intervention.

Beta blockers block the effects of the sympathetic innervation of the heart. This decreases the hearts workload, so it needs less blood and oxygen. This aids in lowering blood pressure and helps to control abnormal beat rhythms that are either too fast or irregular. ACE (angiotensin-converting enzyme) inhibitors block the enzyme that is needed to form a molecule that constricts blood vessels. This allows blood vessels to relax allowing for easier flow of blood while reducing blood pressure. Diuretics help to remove excess fluid from the body decreasing edema (swelling).

Pulmonary Venous Hypertension Prognosis

PVH survival rates are essentially no different from other forms of PH. Depending on early diagnosis (if possible) and various therapies, an individual may survive somewhere between 3 to 10 years.

Pulmonary Hypoxic Hypertension

Pulmonary hypoxic hypertension as the name suggests involves high blood pressure and low oxygen (hypoxic) levels. This results from constricted pulmonary arteries to certain areas of the lungs. This phenomenon may seem illogical in that the pulmonary arteries actually constrict in the presence of low oxygen levels and this occurs without high carbon dioxide levels. Many would think that the arteries would relax and allow for greater blood flow to get more oxygen exchange. Under normal conditions, this is a mechanism to redirect blood flow to alveoli that have higher oxygen content. This constriction changes the distribution of blood flow in the lungs to areas of the lung that have more air. This increases the total area available in gas exchange since blood is not flowing to poorly ventilated aveoli. However, in the case of long-term whole-body hypoxia such as in HP, this works against the system. This is often seen in COPD and heart failure. Going to high altitudes can bring about the same phenomenon.

Pulmonary hypoxic hypertension Symptoms

Pulmonary hypoxic hypertension shares many of the same symptoms as other forms of HP.
Asymptomatic initially
Pain in the chest
Fainting spells and dizziness
Shortness of breath
Fatigue
Edema of the feet and ankles (fluid retention and swelling)
Jugular vein distention
Ascites (swollen abdomen due to fluid buildup)
Clubbing of fingers
Increased blood pressure in the vessels leading to the lungs

Pulmonary Hypoxic Hypertension Diagnosis

For a PHH diagnosis, a doctor will explore medical and family history. A physical exam will be done to determine what symptoms a patient has. To confirm that a person has some form of PH, certain tests will be run, such as an echocardiogram, chest X-ray, electrocardiogram (EKG) and/or right heart catheterization. If it is determined that a person has pulmonary hypoxic hypertension, a doctor will try to determine the underlying cause. To do this s/he will run Lung function tests, chest CT and MRI scans, and/or Polysomnograms.

Pulmonary Hypoxic Hypertension Treatments

Treatments for Pulmonary hypoxic hypertension are pretty much the same as for other forms of HP which include:

Diuretics to reduce fluid buildup in the body (edema)
Blood thinners (anticoagulants) to prevent clot formation
Digoxin which is used to treat congestive heart failure. This slows down heart rate during atrial fibrillation (a rapid, irregular heart beat).
Oxygen therapy
Calcium channel blockers for vessel relaxation
Prostacylins for blood vessel relaxation and to prevent clot formation
Heart or lung transplant
Medications to treat the underlying cause if known

Pulmonary Hypoxic Hypertension Prognosis

Early diagnosis is important as well as finding the appropriate therapy(s), however there is no cure.

Thromboembolic Pulmonary Hypertension

When thromboembolic pulmonary hypertension becomes chronic, it causes severe pulmonary hypertension. Unfortunately, this form of HP goes under diagnosed. The lesions involve clots (thrombi) within the lumen of the blood vessels and fibrous stenosis (blood vessel constriction) or total destruction of the pulmonary arteries. As a result, the pulmonary vascular resistance increases generating pulmonary hypertension and progressive right heart (right ventricle) failure. Current research suggests that this disease is initiated by pulmonary embolism (as a single or recurrent events) and followed by progressive pulmonary vessel remodeling (angiogenesis).

Thromboembolic Pulmonary Hypertension Causes

Although there can many causes of thromboembolic pulmonary hypertension, patients are at high risk if they have experienced recurrent pulmonary thromboendartectomy (PTE), splenectomy (spleen removal) or lupus anticoagulant syndrome (antiphospholipid antibody syndrome). PTE is an operation that removes blood clots from the pulmonary arteries. Lupus anticoagulant syndrome causes blood clot formation in blood vessels and is an autoimmune disease that produces antibodies against a particular phospholipid known as cardiolipin and β2 glycoprotein.

Thromboembolic Pulmonary Hypertension Symptoms

The symptoms of thromboembolic pulmonary hypertension are essentially the same as other forms of pulmonary hypertension which include:

Progressive breathing difficulty upon exercise
Hemotysis which means coughing and expulsion of bloody mucus
Fatigue
Fainting spells and dizziness
Syncope (brief loss of consciousness due to lack of oxygen)
Edema which is fluid retention in the body
Embolism due to clot formation (causes blood vessel obstruction)
Systolic murmur of the tricuspid valve (regurgitation)
Extended neck veins
Ascites (swollen abdomen from fluid build up)
Cyanosis such as blue lips and skin due to lack of oxygen

Thromboembolic Pulmonary Hypertension Diagnosis

Treatments for thromboembolic pulmonary hypertension is considerable different from PAH so it is of the essence to determine that the patient has thromboembolic type. One of the difficulties is that many patients have no history of pulmonary embolism which makes it difficult to diagnose properly. Some of these patients will be misdiagnosed.

Tests to Confirm Thromboembolic Pulmonary Hypertension

Echocardiography – This is the first diagnostic tool used in any form of PH and there should be long-term follow-ups for the thromboembolic type of hypertension.
Ventilation-perfusion scanning uses medical isotopes to observe air and blood circulation in a patients lungs. It can also observe blood clots if they are present.
Computed tomography (CT) scans.
Magnetic Resonance Imaging (MRI).
Pulmonary angiography uses a dye and X-rays to observe how blood flows through the lungs. This is the standard tool for diagnosis of thromboembolic hypertension. This is often performed in conjunction with right heart catheterization.

Thromboembolic Pulmonary Hypertension Treatment

Some forms of thromboembolic pulmonary hypertension require surgery, medical treatment or both.
Endothelin receptor antagonists may be prescribed as they prevent blood vessel constriction. Endothelin is a powerful blood vessel constrictor.
Endarterectomy is a surgical procedure that unblocks blood vessels. This procedure is done with complete circulatory arrest while the patient is in deep hypothermia between 18°C and 20°C. This procedure is reported to have great success.
Phosphodiesterase inhibitors are used to allow blood vessels to relax.
Prostacyclins work well as they relax blood vessels and prevent clot formation.

Thromboembolic Pulmonary Hypertension Prognosis

The mortality rate with individuals diagnosed with thromboembolic pulmonary hypertension is reported to be 90 percent within 3 years after diagnosis.

Guidelines

Saudi Guidelines on the Diagnosis and Management of Pulmonary Hypertension (2014 Update)

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