What is Platypnea?

Platypnea is a term used to describe difficulty in breathing when in an upright position, which gets better when lying down, while Orthodeoxia refers to breathlessness associated with being upright. There’s a particular condition called Platypnea-Orthodeoxia Syndrome (POS) which is diagnosed when a person’s oxygen levels reduces by more than 5% and their arterial oxygen partial pressure drops by more than 4 mm Hg when they move from lying down to standing up.

POS is often linked to a condition where blood bypasses the lungs and is not oxygenated, as might occur in certain heart conditions like patent foramen ovale (PFO, a hole in the heart that didn’t close after birth), atrial septal defect (ASD, a hole in the wall between the heart’s upper chambers), atrial septal aneurysm (ASA, a rare but serious condition that involves the septum and can impact heart function), and congenital cardiomyopathy (heart muscle disease present from birth). This can also happen due to lung conditions like pulmonary arteriovenous malformation (PAVM, a rare lung blood vessel disorder), hepatopulmonary syndrome (HPS, a lung disorder associated with liver disease), and acute respiratory distress syndrome (ARDS, a severe lung condition causing shortness of breath). Conditions causing unequal distribution of airflow and blood flow in the lungs, prevalent in diseases like pneumonectomy (surgical removal of a lung), chronic obstructive pulmonary disease (COPD, a type of obstructive lung disease), interstitial lung disease (lung disease causing scarring to the lungs), and cryptogenic organizing fibrosis (a rare lung condition) can bring about the symptoms. PFO is the most frequently seen structural problem linked with POS and it often goes without symptoms for many years.

POS was first noted in 1949 and was initially termed “orthostatic cyanosis”. The terms “platypnea” and “orthodeoxia” were later coined in 1969 and 1976 respectively to describe a situation where breathlessness and a reduction in oxygen levels worsen when standing and get better when lying down.

What Causes Platypnea?

Platypnea-orthodeoxia syndrome, or POS, can be caused by problems within the heart (intracardiac) or outside the heart (extracardiac). Some common heart-related causes of POS include certain heart defects such as a hole in the upper chambers of the heart (Patent Foramen Ovale & Atrial Septal Defect) or a bundle of extra heart muscle (Atrial Septal Aneurysm).

Causes of POS that are outside the heart are split into two categories: those related to the lung (intrapulmonary) and those not related to the lung (extrapulmonary). Lung-related causes include abnormal blood vessels in the lung (Pulmonary Arteriovenous Malformation) and lung disease. Hepatopulmonary Syndrome (a liver condition) leads to non-lung-related causes which can result in POS.

Other possible causes of POS, as suggested by some studies, include a blockage in a blood vessel caused by fat (fat embolism) and Parkinson’s disease, although the details of how these conditions can cause POS is still being debated in the medical community.

Risk Factors and Frequency for Platypnea

Right now, we don’t have any concrete numbers about how often Positional Oxygenation Saturation (POS) occurs. Many instances might be missed unless doctors specifically look for the signs. Usually, they don’t check for changes in a patient’s breathlessness or oxygen saturation depending on their position during routine check-ups. As a result, changes in oxygen levels based on position could be easily missed.

Signs and Symptoms of Platypnea

Platypnea-Orthodeoxia Syndrome, or POS, isn’t a disease itself, but a symptom of another condition. It’s crucial to figure out what’s causing POS. Patients with POS often describe feeling short of breath when they sit or stand up. This breathlessness usually gets better when they’re lying down. They might also mention a long history of heart or lung issues and struggle with physical activity. Other symptoms they may report include chest pain, heart palpitations, feeling dizzy, and passing out.

The physical exam can show a drop in oxygen levels when the patient stands up, which improves when they lie down. The other findings on the exam depend on what’s causing the POS. Patients might have a heart murmur if they have a birth defect in their heart, but people with a specific heart condition called a patent foramen ovale (PFO) may have normal heart sounds. Oxygen levels may not improve even with extra oxygen if there’s a right-to-left shunt (a condition where blood bypasses the lungs and thereby a key step in the oxygenation process).

Shortness of breath that improves when lying down
History of heart or lung problems
Poor exercise tolerance
Chest pain
Palpitations
Dizziness
Fainting spells

If the POS is caused by issues with the heart or lungs, the patient may have abnormal breath sounds, bluish skin or lips (cyanosis), and clubbing (changes in the shape of the fingers and nails). If it’s due to a chronic liver disease, they may have spider nevi (small, spider-shaped blood vessels), red palms, fluid buildup in the abdomen (ascites), and widespread fluid buildup in the body (anasarca).

Testing for Platypnea

When a patient is suffering from Platypnea-Orthodeoxia Syndrome (POS), a condition where the body experiences trouble getting enough oxygen, a series of specific tests are required. These tests help doctors determine the source of the problem. Many patients with a defect called Patent Foramen Ovale (PFO), a hole in the heart that didn’t close properly after birth, may have normal results on common tests like ECGs, chest X-rays, blood counts, and metabolic panels. However, a special heart scan known as an echocardiogram can reveal the issue.

One type of echocardiogram, known as agitated saline bubble echocardiography, is extremely useful. This test uses a saline solution with tiny bubbles that can be tracked as they move through your heart chambers. If these tiny bubbles appear in the left side of your heart within the first three beats after they fill the right side, it indicates a possible hole in your heart. If the bubbles show up after three to six beats, it suggests the issue might be outside your heart. During this procedure, the Valsalva maneuver, which involves forcefully exhaling while your mouth and nose are closed, can be used to enhance the detection of the problem. Other important tests include contrast-enhanced echocardiography, macroaggregated-albumin scintigraphy (a nuclear medicine test), and invasive angiography (an X-ray image of the blood vessels).

Chest X-rays are often not sensitive enough to detect POS but can show if an underlying lung disease may be causing the issue. CT scans can also be helpful. A CT scan without contrast can sometimes pick up a PFO, while a CT scan with contrast can help to identify an arteriovenous malformation (AVM), an abnormal connection between arteries and veins.

Because POS can be related to liver disease, liver enzyme tests and coagulation tests, which measure how well your blood clots, will be important. Hepatopulmonary syndrome (HPS) is a severe complication related to POS. To diagnose HPS, doctors look for the following: existence of liver disease, high oxygen gradient levels in a room air sample, presence of dilated blood vessels in the lower parts of the lungs, and absence of other significant heart or lung diseases.

Depending on the patient’s test results, it may be necessary to involve different types of specialists, such as lung doctors (pulmonologists), heart doctors (cardiologists), liver doctors (hepatologists), and specialized radiologists to conduct more specific tests.

Treatment Options for Platypnea

When a person has low oxygen levels in their blood, or persistent hypoxia, it’s crucial to address the underlying illness causing this condition. How to handle each disease is complex and varies widely, but generally, those suffering from persistent hypoxia might need to receive supplemental oxygen therapy as part of the treatment.

Early consultations with specific medical experts such as heart doctors (cardiologists) or lung specialists (pulmonologists) are incredibly beneficial. They can provide a more in-depth assessment of the patient’s condition and suggest the most appropriate ways to manage it.

For example, people who are believed to have a heart-related condition may need to see a cardiologist. If they have a PFO – a hole in the heart that is present from birth – they usually don’t need any treatment, as this condition exists in about a quarter of the population. However, if this hole in the heart is causing reduced oxygen levels and there’s a risk of an embolic stroke – a blood clot that travels to the brain – a cardiovascular surgeon might be necessary. They can evaluate whether the person needs surgery to close the hole.

For conditions related to the lungs, appropriate and timely treatment is necessary. If someone has pneumonia, for instance, they may need to take antibiotics. If they have more complicated lung conditions like ILD – a group of diseases that cause progressive scarring of lung tissue – they should see a pulmonologist. Large PAVMs – abnormal blood vessels that let blood pass from arteries to veins without going through small vessels called capillaries – might require a specific procedure called embolotherapy, which can be performed by an interventional radiologist.

For patients with a severe liver disease called cirrhosis combined with HPS – a condition where low oxygen levels are caused by abnormal blood vessels in the lungs and liver – a liver transplant might be the best option. This type of surgery is performed by a liver transplant specialist. While patients are waiting for this surgery, they may receive supportive treatment to help manage their symptoms.

When a person experiences positional dyspnea, or trouble breathing based on their body position, there could be many potential causes. Doctors will typically consider these possible conditions:

Heart problems, such as intracardiac shunting, valvular heart disease, and cardiomyopathy
Blood flow issues in the lungs, like pulmonary vascular disorders and pulmonary hypertension
Lung issues, like chronic obstructive pulmonary disease (COPD), pneumonia, and interstitial lung disease (ILD)
Extrapulmonary conditions, such as issues with the diaphragm or liver failure
Other conditions, like extreme low blood pressure due to severe dehydration or blood loss, and sleep-related breathing disorders

Positional dyspnea is a rare condition that usually requires a team of professionals from different medical disciplines for a thorough assessment and to choose the right treatment plan.

What to expect with Platypnea

The outcome of POS largely depends on how serious the original condition is and how well it’s managed. If the root cause is quickly treated and the patient follows through with rehabilitation, the results are typically very good. However, factors that can lead to worse outcomes include delaying treatment, inadequate response to treatment, less than ideal heart function, and the occurrence of complications affecting the entire body.

Possible Complications When Diagnosed with Platypnea

The conditions that are underlying POS – not POS itself – can create serious health issues. These issues can include:

Continued low levels of oxygen in the blood, leading to chronic heart failure and multiple organ dysfunction
The risk of blood clots, which is common with intracardiac shunts
A lower quality of life due to breathing difficulties and associated problems such as tiredness and cognitive impairment
Surgical problems, like reactions to anesthesia, bleeding, and infection, when the conditions are treated surgically
The underlying diseases may worsen, potentially resulting in death, as seen in cases of liver and heart failure

An accurate diagnosis and quick treatment are key in preventing or reducing the possible complications of POS.

Preventing Platypnea

Managing the conditions that cause POS (Pneumoconiosis, a lung disease caused by breathing in certain dust particles) should be the main focus of strategies to avoid it. When doctors talk to patients about POS, they need to focus on several important things.

The first thing is making patients aware of what might cause this condition. Understanding health information can help patients take better care of their health. The second important aspect is having regular check-ups with your doctor. Regular health checks can spot conditions that might lead to POS before they become serious. Catching these conditions early means they can be treated in time, possibly preventing POS from developing.

The third key point is the role of a healthy lifestyle. POS can be caused by diseases that can be avoided, such as pneumonia, liver failure, and COPD (a lung disease that makes it hard to breathe). Good personal hygiene, clean surroundings, a balanced diet, exercise, and not smoking can help keep these diseases—and therefore POS—at bay.

The fourth crucial factor is effectively managing ongoing conditions that could trigger POS. Sticking to a long-term treatment plan can delay complications in people who have conditions that pose a risk of POS.

The fifth thing to consider is genetic counseling for patients whose POS could be inherited. Parents worried about passing on genetic disorders linked to POS might want to get advice to help them plan their families and explore prenatal testing. Finally, the sixth thing to remember is to seek medical help quickly if symptoms appear or get worse. Swift treatment of conditions that could lead to POS often leads to better health outcomes.

For most people, it’s hard to predict whether they will develop POS. However, knowing where the condition comes from and how to prevent it can help patients avoid getting it.