Pulmonary Hypertension

Populations and Risk Factors

Primary (idiopathic pulmonary arterial hypertension): Rare. Sometimes linked to BMPR2 gene mutations. More common in young women
Secondary (most cases): Complication of COPD, interstitial lung disease, obstructive sleep apnea, chronic pulmonary embolism, left-sided heart disease (most common cause overall)
Autoimmune diseases: systemic lupus erythematosus, scleroderma
HIV infection, portal hypertension, certain drugs (appetite suppressants)

Vascular remodeling: Abnormal proliferation of pulmonary vascular smooth muscle with intimal fibrosis narrows and obstructs pulmonary arteries and arterioles
Right ventricular overload: The right ventricle hypertrophies to overcome increased pulmonary vascular resistance. Over time, the right ventricle dilates and fails (cor pulmonale)
Hypoxia-vasoconstriction cycle: Long-term hypoxia from lung disease triggers pulmonary vasoconstriction, which further increases pulmonary pressure in a vicious cycle
Thrombosis in situ: Endothelial dysfunction in pulmonary vessels promotes local thrombus formation, further reducing the vascular bed

Dyspnea (initially only during exertion, progressing to dyspnea at rest)
Profound fatigue and weakness
Angina-like chest pain from right heart strain
Syncope or near-syncope during exertion (cardiac output cannot increase to meet demand)
Distended neck veins (jugular venous distention)
Peripheral edema and ascites (advancing right heart failure)
Cyanosis of lips and nail beds. Finger clubbing (chronic hypoxia)
Red flags: Syncope during exertion indicates severe disease — do not provoke; progressive edema with dyspnea suggests advancing heart failure

Category A7 Systemic Conditions — Cardiovascular/Respiratory
Unexplained lower extremity edema + shortness of breath should raise suspicion for pulmonary hypertension
Many patients are asymptomatic until disease is advanced
Right heart catheterization (mean PAP > 25 mmHg) is the gold standard for diagnosis
Medications (vasodilators, anticoagulants, diuretics) may cause dizziness or easy bruising — modify treatment accordingly

Indications: Palliative relaxation techniques. Address accessory respiratory muscle tightness (scalenes, SCM, intercostals, pectoralis minor)
Contraindications: Mechanically pushing fluid (vigorous effleurage, lymphatic techniques) is contraindicated if progressed to heart failure. Do not impose heavy circulatory load
Techniques: Gentle relaxation work. Myofascial release of respiratory muscles. Avoid techniques that significantly increase venous return
Positioning: Semi-supine or seated positioning for orthopnea (inability to breathe when flat). Assist with table transitions — exertional syncope is a risk
Hydrotherapy: Mild warmth for comfort only. Avoid hot immersion (vasodilation may cause dangerous hypotension)
Medication awareness: Anticoagulants increase bruising risk (reduce pressure). Vasodilators may cause orthostatic hypotension (assist with position changes)

Pulmonary hypertension involves elevated pulmonary artery pressure leading to right ventricular hypertrophy and eventually cor pulmonale (right-sided heart failure)
Many patients remain asymptomatic until disease is advanced. Unexplained lower extremity edema plus shortness of breath should raise suspicion
Mechanically pushing fluid is contraindicated if the condition has progressed to heart failure
Position clients semi-supine or seated for orthopnea. Address accessory respiratory muscle tightness with gentle myofascial release
Medications (vasodilators, anticoagulants, diuretics) may cause dizziness or easy bruising — modify pressure and transition assistance