Pulmonary Vascular Disorder

Pulmonary vascular disorder is a broad term for any condition that affects the blood vessels between your heart and lungs, causing symptoms like shortness of breath and potentially leading to serious heart problems if left untreated.

Table of contents

• What Is Pulmonary Vascular Disorder?
• Causes and Types
• Symptoms
• Diagnosis
• Treatment Options
• Living with Pulmonary Vascular Disorder

What Is Pulmonary Vascular Disorder?

Pulmonary vascular disorder, also called pulmonary vascular disease (PVD), includes any condition that affects the blood vessels within the lungs^[2]. To understand this condition, it helps to know how blood flows through your body.

Your blood travels in a continuous circuit. Oxygen-poor blood returns from your body’s tissues through veins back to the right side of your heart. The right heart then pumps this oxygen-poor blood through the pulmonary arteries (blood vessels that carry blood from the heart to the lungs) into your lungs. In the lungs, this blood picks up oxygen. The oxygen-rich blood then returns from the lungs through the pulmonary veins (blood vessels that carry blood from the lungs to the heart) back to the left side of your heart. Finally, the left heart pumps the oxygen-rich blood out to your entire body through the aorta and many other arteries^[1].

Any part of this heart-lung blood circuit can become damaged or blocked, leading to pulmonary vascular disorder^[1]. When these blood vessels become affected, it disrupts the normal flow of blood through your heart and lungs. This can cause the blood pressure in your pulmonary arteries to rise, making your heart work much harder to pump blood to your lungs^[2].

Pulmonary vascular disorders are a group of conditions that commonly cause shortness of breath and decrease your ability to exercise^[3]. A pulmonary vascular disorder can lead to cardiovascular problems as well as impairing your quality of life^[2].

Causes and Types

The causes of pulmonary vascular disease vary according to which of the lungs’ blood vessels are affected^[1][2]. Pulmonary vascular disease is divided into several categories.

Pulmonary arterial hypertension (PAH) refers to increased blood pressure in the pulmonary arteries that carry blood away from the heart to the lungs. Pulmonary arterial hypertension can be caused by lung disease, autoimmune disease (conditions where the body’s immune system attacks its own tissues), or heart failure. When there is no clear cause, it’s called idiopathic pulmonary arterial hypertension^[1].

Pulmonary venous hypertension is increased blood pressure in the pulmonary veins that carry blood away from the lungs to the heart. Pulmonary venous hypertension is most often caused by congestive heart failure (a condition where the heart cannot pump blood effectively). A damaged mitral valve in the heart may also contribute to pulmonary venous hypertension^[1].

Pulmonary embolism occurs when a blood clot breaks off from a deep vein, usually in the leg, travels into the right heart, and is pumped into the lungs. Rarely, the blockage can be a large bubble of air or ball of fat rather than a blood clot^[1].

Chronic thromboembolic disease is a rare condition where a blood clot in the lungs is never reabsorbed by the body. Instead, multiple small blood vessels in the lungs also develop blood clots. The process occurs slowly and gradually affects a large part of the pulmonary arterial system^[1].

Other forms of pulmonary vascular disorder include those caused by lung diseases like COPD or interstitial lung disease, conditions associated with low oxygen levels, and various other medical conditions^[3].

Symptoms

Most forms of pulmonary vascular disease cause shortness of breath^[1]. The symptoms of pulmonary vascular disease vary according to several factors: how suddenly the process affecting the blood vessels occurs, which pulmonary blood vessels are affected, and how much of the pulmonary vascular system is involved^[1].

For example, a sudden, large pulmonary embolism blocking a large pulmonary artery can cause severe shortness of breath and chest pain. But a very small pulmonary embolism blocking only a small blood vessel may cause no symptoms at all^[1].

Symptoms can develop slowly and may initially be mild. As the condition progresses, symptoms often get worse. Common symptoms include increasing fatigue, shortness of breath while exercising or at rest in more advanced stages, dizziness or fainting, blue lips or fingers, swelling in your feet, legs, and eventually belly and neck, an irregular or very fast heartbeat, and chest pain^[16].

It’s vital to monitor your symptoms and be on the lookout for any new ones. Make sure you call your health care provider if any symptoms get worse, as it might mean that your condition is changing^[16].

Diagnosis

In addition to taking a full medical history, your physician will conduct a variety of tests to diagnose pulmonary vascular disease, as well as determine the specific disorder^[2].

Diagnostic tests may include:

• Blood tests to help find the cause or complications of pulmonary vascular disease
• Chest X-ray, which may be used to check for lung conditions^[2]
• Electrocardiogram (ECG or EKG), a simple test that records the electrical activity of the heart
• Echocardiogram, which uses sound waves to create pictures of the beating heart and shows how blood flows through the heart and heart valves^[2]
• CT scan, an imaging test that provides detailed pictures of the body^[2]
• Right heart catheterization, a procedure where a doctor places a thin, flexible tube into a blood vessel and guides it into the heart to measure pressure^[2]
• Pulmonary angiogram, an imaging test to examine the blood vessels in the lungs^[2]

These tests help your healthcare team understand the location and extent of the disease and allow assessment of how your heart and lungs are functioning.

Treatment Options

In treating pulmonary vascular disease, your physician aims to lessen the severity of symptoms, which will help improve your quality of life. Treatment will also lessen the strain on your heart and decrease your risk for heart failure^[2].

You and your healthcare team will develop a personalized plan based on your specific condition, age, and other factors. Treatment may include a variety of medications and surgery^[2]. Depending on the cause, specific treatments are given^[3].

If pulmonary vascular disease is caused by another condition, such as a heart or lung problem, treatments will focus on the underlying condition^[1].

Treatment options may include:

• Medications such as anticoagulant medicines to help prevent blood clots, diuretics to remove excess fluid from the body, and various specialized drugs that help relax the arteries in the lungs and reduce blood pressure^[1]
• Home oxygen therapy, which involves inhaling air that contains a higher concentration of oxygen^[1]
• Surgical procedures such as pulmonary endarterectomy (an operation to remove old blood clots from the pulmonary arteries) or balloon pulmonary angioplasty (where a tiny balloon is guided into the arteries and inflated to push the blockage aside)^[1]
• Lung transplantation, which is the only definitive treatment in severe cases^[2]

Living with Pulmonary Vascular Disorder

Living with pulmonary vascular disorder presents unique challenges that extend beyond routine medical care. Understanding how to navigate everyday life with this condition is crucial^[23].

Managing pulmonary vascular disease on a daily basis is key to minimizing its impact on your life and maintaining your well-being. One of the cornerstones of effectively managing the condition is adhering strictly to your prescribed medication regimen. Medications can only help if taken consistently^[23].

Fatigue and shortness of breath frequently accompany a pulmonary vascular disorder diagnosis. Setting reasonable priorities, breaking up tiring tasks into smaller segments, and streamlining household chores whenever possible can help conserve energy^[22].

It’s important to:

• Prioritize your daily tasks and distinguish absolute needs from discretionary wants
• Schedule mandatory breaks and work slowly in short, manageable intervals
• Organize your home to minimize unnecessary bending, lifting, or climbing
• Ask others for help with meals, cleaning, laundry, and yard work
• Consider using mobility aids when needed to conserve energy^[22]

While stringent bed rest was once the standard protocol, recent research confirms that remaining mildly active when tolerated provides significant quality-of-life benefits, both physically and emotionally. As long as you carefully avoid overexertion, gentle exercise may help strengthen muscles and boost overall mental well-being^[22].

It’s essential to consult your healthcare team about safe exercise guidelines customized to your individual case, as overly strenuous physical activity can worsen symptoms. Focus on low-impact activities like water walking, recumbent cycling, and mild yoga^[22].

Living with pulmonary vascular disorder can bring anxiety, sadness, or frustration. Talk to professionals like psychologists or counsellors who understand chronic illness. Join support groups to connect with others who understand your experience. Practice coping strategies like mindfulness, meditation, and relaxation exercises^[23]. Caring for your mental health makes you stronger, calmer, and better able to handle challenges.

When you become a patient, your team of experts develops a personalized care plan based on your specific condition. Many programs provide long-term care and support to help patients manage their disease, emphasizing patient education and offering support groups for patients and families to share personal experiences^[2].