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What Age Does Pulmonary Hypertension Start?

by Amy

Pulmonary hypertension (PH) is a serious condition that affects the blood vessels in the lungs. It causes high blood pressure in the pulmonary arteries. This condition can affect people of all ages, from newborns to the elderly. However, the age at which pulmonary hypertension starts depends on its type and underlying cause. This article explains when PH usually begins, how it presents at different stages of life, and what to expect during diagnosis and treatment.

What Is Pulmonary Hypertension?

Basic Definition

Pulmonary hypertension is a form of high blood pressure that specifically affects the arteries in the lungs and the right side of the heart. It occurs when these blood vessels become narrowed, blocked, or damaged. This raises pressure inside the arteries, making it harder for the heart to pump blood through the lungs.

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Classification of Pulmonary Hypertension

The World Health Organization (WHO) classifies pulmonary hypertension into five groups based on its causes:

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  • Group 1: Pulmonary arterial hypertension (PAH)
  • Group 2: PH due to left heart disease
  • Group 3: PH due to lung diseases or low oxygen levels
  • Group 4: PH due to chronic blood clots
  • Group 5: PH with unclear or multiple causes

At What Age Does Pulmonary Hypertension Start?

PH in Infants and Children

Pulmonary hypertension can begin at birth or in early infancy. This is often due to congenital heart defects or genetic disorders. Some infants are born with structural heart issues that disrupt normal blood flow. Others may have persistent pulmonary hypertension of the newborn (PPHN), which prevents normal adaptation of the lungs after birth.

Common Causes in Infants:

  • Congenital heart defects (e.g., ventricular septal defect)
  • Bronchopulmonary dysplasia in premature babies
  • Meconium aspiration syndrome
  • Persistent pulmonary hypertension of the newborn (PPHN)

PH in Children and Adolescents

In older children and teens, PH may develop due to underlying conditions or as part of rare syndromes. Pediatric PH is often linked to congenital heart disease, chronic lung problems, or connective tissue disorders.

Typical Onset Ages:

  • PAH in genetic forms: usually appears before age 20
  • Chronic lung disease-related PH: can develop during childhood
  • Connective tissue diseases: may cause PH in adolescence

PH in Adults

Most cases of pulmonary hypertension occur in adults. It is more common in people aged 30 to 60, depending on the cause. Adult-onset PH is usually related to left heart disease, chronic lung conditions like COPD, or idiopathic pulmonary arterial hypertension (IPAH), which has no known cause.

Age-Related Causes:

  • Group 1 PAH: usually appears in 30s to 50s
  • Group 2 PH (left heart disease): typically after age 50
  • Group 3 PH (lung diseases): commonly in 50s or 60s
  • Group 4 PH (chronic clots): more frequent in middle-aged adults

PH in the Elderly

Older adults may develop pulmonary hypertension due to age-related heart and lung changes. PH in the elderly is often underdiagnosed. It is frequently linked to heart failure with preserved ejection fraction (HFpEF), a common issue in older patients.

How Does the Cause Affect the Onset Age?

Genetic and Inherited Causes

Some forms of PH run in families. Genetic mutations, such as BMPR2 mutations, can cause hereditary pulmonary arterial hypertension (HPAH). These cases often present earlier in life, sometimes during the teenage years or early adulthood.

Congenital Heart Disease

Babies born with heart defects are at high risk of developing PH. If not corrected early, the abnormal heart function can cause irreversible damage to the pulmonary vessels over time.

Chronic Lung Diseases

Conditions like interstitial lung disease or chronic obstructive pulmonary disease (COPD) can lead to PH. These diseases usually develop after years of damage, so PH from this source appears later in life, often in the 50s or 60s.

Left Heart Disease

Heart failure, especially left-sided, is the most common cause of PH in older adults. As people age, conditions like hypertension and coronary artery disease become more common. These can lead to PH through backward pressure into the lungs.

Symptoms by Age Group

Symptoms in Infants

  • Poor feeding
  • Rapid breathing
  • Failure to thrive
  • Cyanosis (blue lips or skin)

Symptoms in Children

Symptoms in Adults and Elderly

  • Exertional dyspnea
  • Fatigue and weakness
  • Swelling in ankles or legs
  • Lightheadedness
  • Palpitations

How Is Pulmonary Hypertension Diagnosed?

Initial Evaluation

Diagnosis begins with a clinical history and physical exam. Physicians look for signs of right heart strain and fluid buildup.

Key Diagnostic Tests

  • Echocardiogram: Estimates pulmonary artery pressure
  • Chest X-ray and CT scan: Checks for lung disease
  • Pulmonary function tests: Measures lung capacity
  • Right heart catheterization: Confirms PH diagnosis by measuring pressures directly

Treatment Options by Age Group

In Infants and Children

Treatment often focuses on correcting the underlying cause, such as congenital heart repair or oxygen therapy. Medications like sildenafil may be used in severe cases.

In Adults

Adults may require a combination of treatments:

  • Diuretics for fluid overload
  • Blood thinners if blood clots are present
  • Vasodilators (e.g., endothelin receptor antagonists, phosphodiesterase-5 inhibitors)
  • Oxygen therapy
  • Lung transplant in extreme cases

In the Elderly

Management in older adults focuses on treating heart failure and minimizing symptoms. Careful medication use is important to avoid side effects.

Prognosis and Life Expectancy

Varies with Cause and Age

The outlook for pulmonary hypertension depends on the age of onset and the underlying cause. Infants with early diagnosis and treatment may live normal lives. Adults with PAH may live 7–10 years after diagnosis, but newer drugs are improving outcomes. Elderly patients often have other conditions that complicate treatment, but symptom control is possible.

Conclusion

Pulmonary hypertension can start at any age. In newborns and infants, it is often due to congenital problems. In children and adolescents, genetic or systemic diseases may be involved. Adults commonly develop PH in their 30s to 60s due to heart or lung diseases. Older adults face PH mainly as a result of age-related heart issues. Understanding when and why PH starts is crucial for early detection and better outcomes.

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