Pulmonary Hypertension – Symptoms and Causes

Overview

Pulmonary hypertension happens when blood pressure rises abnormally in the lung arteries. This condition affects both the lungs and the right side of the heart.

Pulmonary arterial hypertension (PAH) is one specific type where lung blood vessels become narrow, blocked, or damaged.

As these blood vessels change, blood moves more slowly through the lungs. This forces the heart to work harder, eventually weakening the heart muscle and potentially leading to heart failure.

The disease typically progresses gradually and can become serious enough to threaten life. While there is no cure available, several treatment options can:

• Improve symptoms
• Extend lifespan
• Enhance quality of life

Most patients benefit from medical management that slows progression and maintains heart function for as long as possible.

Signs to Watch For

Pulmonary hypertension often develops gradually, with symptoms that may go unnoticed for months or even years. As the condition progresses, these signs typically become more noticeable and severe.

Common symptoms include:

• Breathing difficulties – initially during physical activity, eventually occurring at rest.
• Skin discoloration – bluish or grayish tint (may appear differently depending on skin tone).
• Chest discomfort – pressure or pain in the chest area.
• Lightheadedness – including dizziness or fainting episodes.
• Heart irregularities – rapid heartbeat or pounding sensation.
• Extreme tiredness – persistent fatigue.
• Fluid retention – swelling in the ankles, legs, and abdominal region.

While shortness of breath is the most frequent symptom, it can also indicate other conditions such as asthma. A healthcare provider should evaluate your symptoms for proper diagnosis.

Causes

Group 1: Pulmonary Blood Vessel Disease

In this group, changes affect the actual blood vessels in the lungs. Causes include:

• Unknown origin (idiopathic) – doctors cannot identify why the condition developed.
• Genetic factors – inherited through family lines.
• Chemical triggers – certain medications or illegal substances like methamphetamine.
• Birth heart defects – problems with heart structure present from birth.
• Autoimmune conditions – including scleroderma and lupus.
• Liver diseases – such as cirrhosis that affect blood flow patterns.

Group 2: Left Heart Problems

This is the most common type of pulmonary hypertension. It happens when:

• The left side of the heart becomes weak and cannot pump effectively
• Heart valves on the left side (mitral or aortic) become damaged or diseased
• Blood backs up into the lungs because it can’t move forward properly

Group 3: Lung Disease-Related Hypertension

When the lungs themselves are damaged, it can lead to high blood pressure in the pulmonary arteries. Common causes include:

• Lung scarring (pulmonary fibrosis) that stiffens the lung tissue.
• Chronic obstructive pulmonary disease (COPD) narrows airways.
• Sleep apnea – breathing stops and starts during sleep.
• High-altitude exposure, especially in people already at risk.

Group 4: Blood Clots and Blockages

This type happens when something blocks the pulmonary arteries, such as:

• Long-term blood clots that get stuck in lung vessels
• Growths or tumors that develop within the pulmonary arteries
• Scar tissue from previous clots that narrows blood vessels

Group 5: Other Health Conditions

Various medical problems can trigger pulmonary hypertension, including:

• Blood disorders, like polycythemia vera (too many red blood cells).
• Inflammatory diseases, such as sarcoidosis
• Metabolic problems, including glycogen storage disease
• Kidney failure or chronic kidney disease

Eisenmenger Syndrome

This condition develops when heart defects aren’t repaired early in life. Key features include:

1. A hole exists between heart chambers (like a ventricular septal defect)
2. Blood flows incorrectly through this opening
3. Oxygen-rich and oxygen-poor blood mix
4. Extra blood gets sent to the lungs instead of the body
5. The increased flow damages lung vessels over time

This process creates a cycle where lung pressure rises and heart function worsens. The body cannot get enough oxygen, leading to serious complications.

Risk Factors

Several factors can increase your chances of developing pulmonary hypertension:

1. Age: Most diagnoses occur between ages 30-60, though unknown-cause PAH affects younger adults more frequently.
2. Health and Lifestyle Factors:
   • Family history of pulmonary hypertension
   • Excess weight
   • Tobacco use
   • Blood clotting disorders or family history of lung blood clots
   • Asbestos exposure
3. Other Risk Factors:
   • Congenital heart defects (heart problems present at birth)
   • High-altitude living
   • Certain substances, including some weight-loss medications and illegal drugs like cocaine or methamphetamine

Complications

Pulmonary hypertension can lead to several serious health problems. These complications can affect quality of life and may become life-threatening if not properly managed.

Right Heart Enlargement and Failure

When pulmonary hypertension persists, the right ventricle (lower heart chamber) must work harder to pump blood through narrowed lung arteries. This extra strain causes:

• Thickening of heart walls
• Stretching of the chamber to hold more blood
• Progressive weakening of heart function

Blood Clot Formation

People with pulmonary hypertension face a higher risk of developing blood clots in their small lung arteries, which can worsen breathing problems.

Heart Rhythm Disturbances

The disease can trigger dangerous irregular heartbeats that may threaten life if not promptly treated.

Pulmonary Bleeding

In some cases, this condition damages blood vessels enough to cause bleeding into the lungs. Patients might cough up blood, which requires immediate medical attention.

Risks During Pregnancy

Pregnancy can be extremely dangerous for women with pulmonary hypertension. It poses serious threats to both the mother and the baby’s health and survival.