Introduction
Before the diagnosis of pulmonary hypertension (PH) may be made, a clinician must first rule out other diseases that present in a manner similar to PH. Left heart failure, COPD, asthma, and other diseases may manifest with dyspnea or exertional dyspnea in a patient, making their overt presentation look similar to a case of PH. For this reason, it is important as part of the diagnostic process for PH to carefully review the patient’s physical signs and have a full understanding of their medical and medication history.

Since patients in the early phases of PH may be asymptomatic or may have mild to moderate exertional dyspnea, fatigue, or chest pain, understanding root causes of these symptoms can help create a high index of suspicion that PH may underlie the symptoms. Pulmonary hypertension should be considered in any patient who presents with dyspnea and who shows no signs or minimal signs of any specific heart or lung disease. To aid in the elucidation of PH in a patient complaining of dyspnea, a detailed history and thorough physical examination are essential as part of the battery of diagnostic tests needed to pinpoint PH as the root disease state.

Classifying the Patient
The New York Heart Association has created classification guidelines to aid the health care provider in determining severity of disease. These criteria are based on simple functional and lifestyle components and can place the patient into one of 4 possible categories.

As part of thorough patient assessment, there are several key questions that must be addressed, including the patient’s history of symptoms, medication history, and the health care providers with whom they have consulted. The more detail gained, the greater the efficiency of the diagnostic process. Here are some fundamental questions to ask the patient:

• Have they been diagnosed with a connective tissue disease, such as scleroderma?
• Have they had liver disease, hepatitis, or congenital heart disease?
• In the patient’s description of their dyspnea, it is important to understand the severity of chest pain, heart palpitations, dizziness or fainting.
• Have they noticed any swelling in their lower extremities?
• Does the patient have asthma?
• Does the patient have HIV, sickle cell anemia, or systemic sclerosis?
• Have they ever had a pulmonary embolism?

The answers to these basic questions will help determine the presence or absence of underlying diseases, medical treatments, or drug use. These could impact a patient’s exercise tolerance, lung function (e.g. pulmonary embolism), or heart function (e.g. congenital heart defects, arrhythmias or use of weight loss drugs such as fenfluramine).

Once a detailed patient history is taken, the patient should be physically examined.

Physical Examination of the PH Patient
Since pulmonary hypertension inevitably results in dysfunction and failure of the right heart, many of the physical examination procedures for PH are the same as those for heart failure. In the physical examination, the health care provider will be looking for potential signs in the head, ears, eyes, nose and throat (HEENT ), lungs, heart, abdomen and extremities that could possibly suggest underlying disease.

• Heart Failure
• Obstructive or fibrotic lung disease
• Disorders of the liver, spleen
• Joint disease
• Thyroid disease

• Palpate the thyroid gland: hyperthyroidism can influence development of pulmonary hypertension.
• Recline the patient to 45 degrees and evaluate them for jugular venous distension: elevated jugular venous pressure is symptomatic of heart failure
• A combination of jugular venous distension, hepatojugular reflex, pulmonary rales, and peripheral edema is an indication of volume overload and increases the probability of diagnosing heart failure.

Next, the patient’s lungs will be examined. Patients with PAH generally have clear lungs.

• The presence of rales or crackles may indicate concomitant lung disease such as COPD or interstitial lung disease (ILD).
• Pulmonary rales may also be present in patients with volume overload and developing left heart failure.

• On physical examination, a patient with PAH may evidence a right parasternal lift, an accentuation of pulmonary second heart sound (P2), a parasystolic murmur (indicating triscupid regurgitation), a third heart sound and a diastolic murmur (indicating pulmonary valve unsufficiency).
• Patients with decompensated right heart failure due to PH may show evidence of right ventricular “heave” or and S3 “gallop”.

The next phase of the physical examination is the abdominal and lower extremity evaluation.

• Palpate the abdomen to evaluate for splenomegaly or hepatomegaly, which may indicate presence of hepatopulmonary hypertension.
• Abdominal ascites may also be revealed through palpation.
• Examine the lower extremities for signs of edema.
• Evaluate skin temperature of the lower extremities. Cool legs can frequently indicate a reduced cardiac output.

Some of the features of physical exam that are relevant in the diagnosis of pulmonary hypertension are listed here.

Adapted from McLaughlin VV, Archer SL, Badesch DB, et al , ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension, JACC, 2009; 53(17):1573-1619)

Summary
A detailed patient history and physical examination are essential components of the overall diagnostic process in determining if symptoms such as dyspnea with or without exertion are due to pulmonary hypertension. While no single examination result should definitively suggest the presence of PH, the data derived from the physical exam and history can contribute strongly to an index of suspicion that PH is present. Physical and history results can lead the healthcare provider to order the more definitive diagnostic tests, such as right heart catheterization, which is considered the “gold standard” in the diagnosis of this disease.

References

Stringham R, Shah NR. Am Fam Physician 2010; 82(4):370-377.
Rubin LJ. Chest. 2004;126:7S-10S.
McLaughlin VV, Archer SL, Badesch DB, et al. JACC 2009;53(17):1573-1619. King M, Kingery J, Casey B. Am Family Physician 2012; 85(12):1161-1168.