Pulmonary hypertension history and symptoms

The hallmark of pulmonary hypertension is progressive dyspnea. The most common symptoms of pulmonary hypertension include dyspnea, fatigue, chest pain and syncope or presyncope. Ortner syndrome may also be seen (characterized by hoarseness due to compression of the left laryngeal nerve caused by enlargement of the pulmonary artery).

The history should be focused on the areas including:^[1]

• The average time from the onset of the initial symptoms of PH to the diagnosis is approximately 2 years.^[2] This can be attributed primarily due to the non-specificity of symptoms and the considerable overlap with symptoms of other pulmonary and cardiovascular diseases. Therefore, a detailed clinical history must be obtained.
• Pulmonary arterial hypertension (PAH) does not typically present with orthopnea or paroxysmal nocturnal dyspnea, while pulmonary venous hypertension typically does.
• Also, a history of exposure to cocaine, methamphetamine, alcohol leading to cirrhosis, and smoking leading to emphysema are considered significant.

• Many conditions are associated with PH and symptoms suggestive of hepatic disease, congenital heart disease, thyroid diseases, and diseases that cause hypoxia must be considered in the clinical history.
• If the patient complains of snoring and daytime sleepiness, then obstructive sleep apnea (OSA) under group 3 hypoxic PH may be a likely culprit.
• A cluster of associated symptoms such as skin changes, Raynaud’s phenomenon and joint pain may point towards a connective tissue disorder under group 1 PAH as the underlying cause.
• A history of deep vein thrombosis or pulmonary embolism may lead one to consider group 4 chronic thromboembolic PH.^[3]
• It is important to gather a comprehensive medication history including use of over-the-counter medications and herbal supplements as well as illicit drug use as many substances are associated with group 1 PAH.
• It is also wise to discern if the patient is high risk for HIV exposure as it has been shown that PH disease course is accelerated in HIV-affected patients.
• Finally, a family history should be examined to see if there is a hereditary component at play.

Symptoms of pulmonary hypertension include:^[1]

• Progressive [dyspnea]] (~85%)
• Fatigue (~26%)
• Chest pain (~22%)
• Lower extremity edema (~20%)
  • As systemic venous hypertension develops secondary to a failing right ventricle, leg swelling may be a feature of the condition in addition to upper right abdominal discomfort (from hepatic congestion) and abdominal swelling (ascites).^[3]
• Syncope or presyncope(~17%)
• Raynaud’s phenomenon (~10%)
  • Anginal chest pain is thought to be due to increased myocardial oxygen demand in a strained right heart that is either hypertrophied or dilated.
  • However, there have also been reports of angina due to decreased myocardial oxygen supply from compression of the left main coronary artery by a dilated pulmonary artery.^[4]
• Near Syncope (~5%)
  • Syncope can occur through either reduced cardiac output, arrhythmias or ventricular ischemia and indicates pulmonary hypertension is severe.
• Palpitations (~5%)
• Cough (rare)
• Hemoptysis (rare)
• Hoarseness (rare)^[2]
  • Hoarseness (Ortner’s syndrome) is thought to be due to compression of the left recurrent laryngeal nerve between a dilated pulmonary artery and the aorta.^[1]

Clinically, a patient may be categorized based on the severity of symptoms into a particular class using the WHO modified functional classification system for pulmonary hypertension (modified from NYHA functional classification system for heart failure). The baseline WHO functional classification is used for the assessment of the severity of PH in order to tailor the choice of therapy. Shown below is a table summarizing the different functional classes.^[5]

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