Pulmonary Hypertension FAQ

A new diag­no­sis can leave you with many ques­tions and con­cerns. To help you from feel­ing lost, our pul­monary hyper­ten­sion spe­cial­ist, Dr. Priyan­ka Rajaram, answers some of the most fre­quent­ly asked ques­tions when receiv­ing this diagnosis.

What is it?

Pul­monary hyper­ten­sion (PH) refers to a type of high blood pres­sure that affects the arter­ies in your lungs and the right side of your heart. This occurs when the tiny arter­ies in your lungs, called pul­monary arte­ri­oles, become nar­rowed, blocked or destroyed, mak­ing it chal­leng­ing for prop­er blood flow.

PH can be clas­si­fied in one of five ways, based on vary­ing causes.

Pul­monary arte­r­i­al hyper­ten­sion (PAH)

The cause of PAH is typ­i­cal­ly unknown, but in some cas­es, it has been linked to con­gen­i­tal heart dis­ease, con­nec­tive tis­sue dis­ease, HIV or liv­er disease.

PAH is a rare con­di­tion that is dif­fer­ent from the oth­er groups of PH, which are caused by under­ly­ing con­di­tions of the heart, lungs or oth­er organs. Symp­toms are dif­fi­cult to diag­nose as it usu­al­ly begins with sub­tle changes in breath­ing, chest pain­or fatigue.

PH caused by left-sided heart disease

If you’ve been diag­nosed with left ven­tric­u­lar heart fail­ure, then your risk of devel­op­ing PH may increase.

PH caused by left-sided heart dis­ease is the most com­mon group of PH diag­nosed in patients.

PH caused by lung disease

Sim­i­lar to PH caused by left-sided heart dis­ease, you can devel­op PH if you have been diag­nosed with inter­sti­tial lung dis­ease (ILD) or chron­ic obstruc­tive pul­monary dis­ease (COPD).

With the devel­op­ment of PH, your COPD and ILD symp­toms includ­ing cough and dif­fi­cul­ty breath­ing can wors­en, along with the emer­gence of dizzi­ness and swelling of the ankles and legs.

PH caused by blood clots

About three to four per­cent of patients with large pul­monary embolism (PE) or recur­rent PEs can devel­op pul­monary hyper­ten­sion. This type of diag­no­sis is called chron­ic throm­boem­bol­ic PH. Anoth­er cause of this type of PH is tumors grow­ing in the pul­monary arter­ies. In many cas­es, this type of PH has a cure.

PH asso­ci­at­ed with oth­er conditions

While less com­mon, there are still a hand­ful of oth­er con­di­tions that can increase your risk of devel­op­ing PH. These include:

• End-stage renal dis­ease (the last phase of kid­ney dis­ease), sar­coido­sis (a dis­or­der affect­ing sev­er­al dif­fer­ent organs), sick­le cell dis­ease (a genet­ic blood dis­ease affect­ing red blood cells) to name a few.

What are the symp­toms of pul­monary hypertension?

Symp­toms that are com­mon to PH include:

• Chest pres­sure or pain
• Short­ness of breath (dys­p­nea) with exertion
• Dif­fi­cul­ty breath­ing while lay­ing flat
• Swelling in your ankles, legs and occa­sion­al­ly abdomen
• Heart pal­pi­ta­tions
• Fatigue
• Dizzi­ness or faint­ing (syn­cope)

What puts me at risk of pul­monary hypertension?

There are many fac­tors to keep in mind when eval­u­at­ing the devel­op­ment of PH, including:

• Age — when the cause of PAH is typ­i­cal­ly unknown, is more com­mon in young adults
• Pre-exist­ing ill­ness which pre­dis­pose patients to PH (scle­ro­der­ma, liv­er dis­ease, HIV, heart dis­ease, ILD, sar­coido­sis, end-stage renal dis­ease, pul­monary embolism)
• Con­sum­ing cer­tain drugs such as metham­phet­a­mine, cocaine or appetite-suppressants
• Fam­i­ly his­to­ry of PH
• Liv­ing at high altitudes

How is pul­monary hyper­ten­sion diagnosed?

An echocar­dio­gram is used as a screen­ing tool to detect the pres­ence of PH. A diag­no­sis is con­firmed by per­form­ing a heart catheter­i­za­tion by a PH specialist.

Can pul­monary hyper­ten­sion be prevented?

Many types of PH stem from oth­er dis­eases, genet­ics or unknown caus­es. Avoid­ing some of the con­trol­lable risk fac­tors and receiv­ing treat­ment for the under­ly­ing ill­ness­es list­ed above, can help reduce your risk of devel­op­ing this condition.

How is pul­monary hyper­ten­sion treated?

Depend­ing on your type of pul­monary hyper­ten­sion, your spe­cial­ist will pro­vide med­ica­tions and treat­ment options that are right for your con­di­tion. The med­ica­tions can be admin­is­tered in a num­ber of ways includ­ing oral­ly, through inhala­tion, beneath the skin or intravenously.

To learn more about PH and our PH spe­cial­ist, please vis­it Dr. Priyan­ka Rajaram’s pro­file or sched­ule an appoint­ment by call­ing 630−871−6699.