Pulmonary hypertension, or PH, is a serious condition where blood pressure becomes too high in the arteries of the lungs. The pressure makes it harder for blood to flow normally, forcing the heart to work overtime. But there's good news: medical advances are giving patients reasons for hope.

Understanding the warning signs

The challenge with catching PH early lies in its subtle beginnings. Ronald Zolty, MD, an advanced heart failure and transplant cardiologist, explains that PH is often missed in its early stages.

“In its earliest stages, patients often complain of shortness of breath,” he says. “The problem is that many diseases have shortness of breath, so it’s not a specific symptom.”

As the condition progresses, patients might experience dizziness or even fainting spells, known medically as syncope. The appearance of these symptoms may mark a turning point when patients and providers realize something more serious is happening.

In addition to shortness of breath and syncope, patients with PH may experience:

• Fatigue: Persistent tiredness and lack of energy are common symptoms.
• Chest pain: Atypical chest pain may occur due to the strain on the heart and inadequate blood supply.
• Swollen ankles and legs: Fluid retention can occur due to the increased pressure in the pulmonary arteries.

Getting diagnosed

Diagnosing PH is like putting together a complex puzzle – it requires multiple pieces to see the complete picture. 

"We perform a comprehensive evaluation to determine if the patient has pulmonary hypertension and to find the source or cause of the condition," says Dr. Zolty. "Knowing the cause of this condition is extremely important as this will guide us in how to appropriately treat the patient.

Dr. Zolty outlines several critical tests that work together to confirm the diagnosis and determine the specific type of PH a patient has.

• Echocardiogram: The primary screening tool for PH.
• Right heart catheterization: Measures pressure in the heart and lungs.
• VQ scan: Checks for blood clots in the lungs.
• Pulmonary function test: Assesses lung health.
• Blood tests: To check for conditions like lupus and rheumatoid arthritis that may be contributing to the problem.

The five types of pulmonary hypertension

Pulmonary hypertension is not just one condition, but five distinct groups. "It's important to differentiate the group, because the treatments are different," Dr. Zolty says. "If you treat for the wrong group, you may potentially hurt the patient."

1. Group 1 (arterial PH): Though rare (about 50 cases per million people), this type has the highest death rate if left untreated. The walls of the lung arteries become thicker over time.
2. Group 2 (left heart disease PH): This is the most common type, making up over 80% of cases. It's often linked to heart failure, valve disease or other heart conditions.
3. Group 3 (lung disease PH): Connected to lung diseases or low oxygen levels.
4. Group 4 (blood clot-related PH): Caused by old blood clots in the lungs. Currently the only type that can be cured by removing the clots.
5. Group 5 (unknown causes): May be related to various conditions like sarcoidosis, kidney disease or sickle cell anemia.

Risk factors and prevention

Risk factors for PH may include:

• Heart and lung conditions: Diseases such as congenital heart defects, chronic obstructive pulmonary disease, or COPD, interstitial lung disease and pulmonary embolism.
• Connective tissue diseases: Conditions like scleroderma, lupus and rheumatoid arthritis can increase your risk of PH.
• Obesity: Excess weight can put additional strain on your heart and lungs, potentially leading to PH.
• Sleep apnea: Obstructive sleep apnea can contribute to increased blood pressure in the lungs.
• Family history: A genetic predisposition to PH can increase your risk, particularly for Group 1 PH.
• Age: The risk of developing PH increases with age.

Maintaining a healthy weight through a balanced diet and aerobic exercise can help reduce the strain on the heart and lungs. Avoiding tobacco products is advised, as they can exacerbate respiratory and cardiovascular issues. 

Dr. Zolty also advises PH patients to avoid salt. “Eating salt will cause fluid retention, swelling in the lower extremities and abdominal distension. If the pressures become too high, pulmonary hypertension patients can develop congestive heart failure. So, avoiding salt is important.”

Treatment: Understanding your options

Treatment for PH has come a long way in the last two decades. Dr. Zolty explains that these treatments target four different pathways in the body:

Pathway 1: Nitric oxide

• The body may not produce enough nitric oxide naturally.
• Medications like Viagra or Cialis help enhance this pathway.
• These drugs are taken as pills.

Pathway 2: Endothelin

• People with PH often produce too much endothelin, a peptide that narrows blood vessels.
• Endothelin receptor antagonists help block this effect.
• Treatment involves one pill daily.

Pathway 3: Prostacyclin

• Some patients don't produce enough of this natural substance, which reduces blood pressure and helps blood flow normally.
• Prostacyclin medications are delivered through pumps (similar to insulin pumps), inhalers or pills that are taken two to three times daily.

Pathway 4: The newest treatment

• A medication called sotatercept, approved by the Food and Drug Administration in April 2024, works by blocking a protein called activin that contributes to PH.
• Sotatercept is injected by the patient every three weeks.
• Shows promise in possibly stopping or even reversing the disease.

"Today we have very good options," Dr. Zolty says, noting the significant progress in treatment options. "I cannot say we have a cure, but the prognosis is way better. So, there is hope, and there is a lot of ongoing research."