How can my doctor be sure whether I have PH?


PH is difficult to diagnose clinically, based on your symptoms and physical examination of your body. As a result, PH is frequently misdiagnosed as another medical illness. The correct diagnosis of PH can be delayed for many months. This is because of several reasons:

  • A patient may have early or mild PH, but may not have any symptoms for months or even years
  • A patient may have early or mild PH, but there may not be any signs of PH on physical examination
  • In a patient with symptoms of PH, the signs of PH on physical examination are subtle and hard to detect in the early stages of PH
  • The symptoms and signs of PH on physical examination are very similar to many other diseases of the heart and lungs

As PH progresses, symptoms and physical examination signs become more obvious. As a result, PH has often progressed to more severe stages by the time it is accurately diagnosed. More severe PH can be diagnosed by findings or signs present on physical examination of the heart:

  • The pulmonic valve of the right side of the heart closes more sharply, which is heard as a louder 2nd heart sound (also known as loud P2)
  • The RV of the heart is enlarged, which is felt as a lift (also known as RV heave) along the left border of the breastbone (also known as sternum)
  • The pulmonary artery is enlarged, which can sometimes be felt
  • The RV of the heart can become weak (also known as RV failure), which can be seen by increased pressure in the veins of the neck (also known as higher jugular venous pressure = JVP), swelling in the feet and ankles (also known as edema), and swelling of the belly (also known as ascites).
  • The tricuspid valve on the right side of the heart (between the RA and the RV) can open up and leak blood, which is known as tricuspid regurgitation or tricuspid insufficiency. This can be heard as a murmur along the left border of the breastbone
  • The pulmonic valve of the right side of the heart can open up and leak blood, which is known as pulmonic regurgitation or pulmonic insufficiency. This can be heard as a murmur along the upper left border of the breastbone

To confirm a diagnosis of PH requires measurement of the abnormally high blood pressure in the pulmonary arteries (also known as pulmonary artery pressure = PAP). This can be done in 2 ways:

(1) Ultrasound of the heart (also known as echocardiogram = ECHO):

  • This is a noninvasive test that is safe and generally painless
  • An ECHO machine is a medical instrument that uses harmless sound waves to form pictures of your heart. This is similar to an obstetrical ultrasound, which is used to look at the fetus in a pregnant woman
  • ECHO allows your doctor to see your heart, to measure the size and thickness of your heart muscle, to assess the size of the chambers of the heart (RA, RV, LA, LV), to see and measure the flow of blood through the heart, and to see how well your heart is functioning
  • Sometimes, ECHO is performed during exercise, to assess how well your heart works under stress
  • ECHO can see the leaking of blood across the tricuspid valve (also known as tricuspid regurgitation), measure the speed of this leaking blood, which is related to the blood pressure in the RV (also known as RV systolic pressure = RVSP)
  • The RVSP is approximately the same as the systolic blood pressure in the pulmonary artery (systolic PAP)
  • Normally, the speed of tricuspid regurgitation is no more than 2.7 m/sec, and the RVSP is
  • PH is defined as TR speed >2.7 m/sec and RVSP >40 mmHg
  • It not always possible to get a measurement of the RVSP on ECHO, in these cases your doctor may suggest alternative testing

In PH, ECHO can also see:

  • Enlargement of the RV and RA
  • Thickening of the muscle of the RV (also known as hypertrophy)
  • The presence of poor function of the RV (also known as RV failure)
  • Pericardial effusion (fluid in the pericardial sac around the heart)

Another way to measure pulmonary artery pressure (PAP) is:

Pulmonary artery catheterization (also known as right heart catheterization = Swan-Ganz catheterization). This is a very important test for many reasons: to directly confirm the presence of PH, to assess the severity of PH, to assess how well your heart is working and how much blood it is pumping (also known as cardiac output), and assessment of whether your PH responds to different vasodilator medications (which dilate or relax pulmonary arteries).

  • Right heart catheterization is the most reliable way of diagnosing PH. It is performed on an outpatient basis using local anesthesia
  • A doctor (usually a cardiologist or heart specialist) will insert a thin, flexible, plastic or silicone tube (also known as catheter) into a large vein (in your neck, groin or arm), carefully slide it up through the heart, and into the pulmonary artery
  • This is an invasive test that has risks. Risks include trauma to the vein in which the catheter is inserted leading to bleeding, infection of the blood, the formation of blood clots at the tip of the catheter, irritation of the heart by the catheter, leading to abnormal electricity of the heart and missed or abnormal beats, which can lead to low blood pressure and the patient fainting
  • Right heart catheterization can directly measure the blood pressure in the pulmonary arteries (also known as pulmonary artery pressure = PAP)
  • Normally, PAP is around 25/10 mmHg (25 mmHg systolic over 10 mmHg diastolic), and the average (also known as mean) PAP is 15-20 mmHg

PH is defined as mean PAP equal to or greater than 25 mmHg in a patient at rest

Echocardiogram (ECHO)

Pulmonary Artery Catheterization