Pulmonary hypertension history and symptoms

Editor(s)-in-Chief: C. Michael Gibson, M.S., M.D. [mailto:mgibson@perfuse.org] Phone:617-632-7753; Assistant Editor(s)-in-Chief: Lisa Prior

Overview

 * When approaching pulmonary hypertension, it is important to commence with a detailed clinical history.


 * The presenting symptoms are important but a comprehensive past medical history, medication history, family and social history and review of systems may reveal further clues as to the etiology of the condition.


 * A history usually reveals gradual onset of shortness of breath, fatigue, non-productive cough, angina pectoris, fainting or syncope, peripheral edema (swelling of the limbs, especially around the ankles and feet), and rarely hemoptysis (coughing up blood).
 * To note, pulmonary arterial hypertension (PAH) typically does not 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.

This will be explored in more detail in the sections below.

Symptoms
The average time of onset of initial symptoms in pulmonary hypertension (PH) to diagnosis is 2 years. This is perhaps primarily due to non-specificity of symptoms and considerable overlap with symptoms of other pulmonary and cardiovascular diseases. It for this reason, a detailed clinical history must be obtained. Presenting symptoms suggestive of pulmonary hypertension on initial presentation are as follows in order of likelihood (National Registry):


 * Dyspnea - 60%
 * Fatigue - 19%
 * Raynaud's phenomenon - 10%
 * Syncope - 8%
 * Chest pain - 7%
 * Near Syncope - 5%
 * Palpitations - 5%
 * Leg swelling - 3%
 * Cough- rare
 * Hemoptysis - rare
 * Hoarseness - rare

Dyspnea emerges as by far the most common symptom followed by fatigue. These symptoms represent the inability of the heart to increase its output on exertion. Anginal chest pain is purported 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. Syncope can occur through either reduced cardiac output, arrhythmias or ventricular ischemia and indicates pulmonary hypertension is severe. As systemic venous hypertension develops secondary to a failing right ventricle, leg swelling may be a feature of the condition as well as upper right abdominal discomfort (from hepatic congestion) and abdominal swelling (ascites). Cough, hemoptysis and hoarseness have been described but are relatively rare. 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.

WHO Functional Classification
Clinically, a patient may be categorised into a particular class using the WHO modified functional classification system for pulmonary hypertension (modified from NYHA functional classification system for heart failure). The benefit of this model is that it provides a baseline for monitoring; it is a prognostic predictor and it guides therapy selection. See table below for WHO functional classification:

Other aspects of clinical history
Many conditions are associated with PH and symptoms suggestive of hepatic disease, congenital heart disease, thyroid diseases and diseases that cause hypoxia or increased left atrial pressures etc. must be considered in the clinical history. For example, 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 rash, Raynaud's phenomenon and joint pain may point towards a connective tissue disorder under Group 1 Pulmonary Arterial Hypertension as the underlying cause. A history of DVT/PE (deep venous thrombosis/pulmonary embolism) may lead one to consider Group 4 Chronic Thromboembolic PH. 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 Pulmonary Arterial Hypertension. 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 sought to see if there is a hereditary component at play.