Pulmonary hypertension (PH). The phrase alone is enough to make the hair on the back of my neck stand up — and I work with it every day. As nurses, we know this diagnosis means a lifetime of medication dependence and monitoring the right heart as the arteries slowly close on the left.
Recently, I personally encountered a PH situation so complex and unusual that I wanted to share it with other nurses who might benefit from my learning. While preparing for my morning rounds one day, I noticed a 36-year-old patient on my list, whom we’ll call “Kay.” Kay’s admitting diagnosis included chemosis. Chemosis? Now, I know I see a lot of different patients, but why am I, the PH nurse practitioner, being consulted for chemosis?
What is chemosis? For those of you, like me, who don’t know, chemosis is swelling (or edema) of the conjunctiva.
It turns out that this young patient and mother of two boys had chemosis for the second time in her life. Kay presented to her local emergency department (ED) — as many people in her situation do — assuming she would be treated with diuretics and eye drops just like the first time. However, this time was different.
This time, there was more going on. The ED staff checked Kay’s oxygen level via pulse-ox, and her SpO2 was 83%. She denied any shortness of breath (SOB), so the staff tried a different pulse-ox device. The reading was the same, which obviously was concerning. At this point, her work-up that started with chemosis was quickly turning to hypoxia. In a seemingly healthy young woman, what could possibly be causing such profound, asymptomatic hypoxia and chemosis?
Trying to Find the Root Cause
Kay was asked if, in the last 30 days, she had experienced:
1Any trouble working due to SOB
2Any trouble completing activities of daily living (ADLs) due to SOB
3Needing to stop and rest with either of the above activities
She replied “yes” to all of these questions, and then began rationalizing the problem, especially why she didn’t report these symptoms or do anything about them: “I own my own cleaning business and I’m trying to keep it going; it’s my and my children’s livelihood. I’ve gained some weight and I’m too busy to exercise. I try to eat right. I have two children at home. My oldest is 14 years old and he’s rejecting the bilateral lung transplant he had at 10 years old related to pulmonary hypertension!”
Well, there it was … the sometimes idiopathic, but highly inheritable, condition of PH. This was not weight gain; it was fluid building up because the right side of Kay’s heart was unable to get the blood to the lungs for oxygenation due to constricted pulmonary arteries. This fluid build up and artery constriction caused further backflow of blood and enlargement of the right side of Kay’s heart, ultimately causing her to become volume overloaded — quite literally “up to her eyeballs.”
A Heart so Big, Yet Incredibly Still Functioning
Kay’s workup began with an echocardiogram and chest x-ray (CXR). As a reminder, a normal echo should consist of a large left atrium (LA) and left ventricle (LV), as the left side is responsible for systemic circulation throughout the body, and a modest right atrium (RA) and right ventricle (RV) — definitely much smaller than the left side — since the right is responsible for pulmonic circulation only.
Kay’s echo and CXR both showed a severely enlarged RA and RV, and small underfilled LA and LV. On her CXR, the cardiac silhouette was so large, it covered most of the film. In addition, her echo revealed that she had a hole in her atrial septum, most likely a patent foramen ovale (PFO) that had popped open due to the high pressures on the right side of her heart. Luckily, the PFO was shunting right-to-left. All of these findings were consistent with long-standing severe PH.
Making the Diagnosis
As a result of the findings and her presentation, Kay was admitted to an inpatient unit and the PH team I work with was consulted. This was the first time I met her and, upon my assessment, I immediately noticed that her lips were blue and her fingers were clubbed. These symptoms are significant findings related to her pending disease diagnosis. Clubbed fingers can signal that the body is compensating for prolonged hypoxia. Kay confirmed that she had never used oxygen a day in her life, but she was now on 8L NC to maintain an SpO2 >92%.
I now saw fear in her eyes, not only for herself, but for her children — especially her ailing 14-year-old son.
The PH team proceeded to do a right-heart catheterization (RHC) to assess Kay’s pulmonary artery pressure (PAP), her fluid level as measured by her pulmonary capillary wedge pressure (PCWP) and her pulmonary vascular resistance (PVR). We suspected that she had pulmonary arterial hypertension (PAH). The World Health Organization (WHO) defines the diagnosis of PAH via RHC as a mean PAP >20mmHg, a PVR >3 wu, and a PCWP <15mmHg, via RHC.
|Measurement||Normal Right-Heart Hemodynamics||Kay’s Right-Heart Hemodynamics|
|PVR||>3 wood units (wu)||23wu|
As you can see, Kay’s pressures were severely elevated. Her PVR was so high that, essentially, her pulmonary arteries were clamped down 20 times more than a typical patient’s heart. Ultimately, she was diagnosed with Group 1 PAH and immediately started on IV Remodulin.
Clinical Knowledge: Understanding and Treating PAH
After a patient completes an RHC, the next step is to figure out where they fit within WHO’s five groups of PH, since there are different treatments for each. It’s important to note that although Kay was initially diagnosed with PH, her diagnosis was then narrowed. We ruled out PAH groups 2-5 through testing and assessment and, per WHO guidelines, diagnosed her with Group 1 PAH. Group 1 is considered the sickest group, as it is idiopathic and incurable, and thus is the only group called pulmonary arterial hypertension. Fortunately, there are a variety of pulmonary vasodilators that come in pill, inhaler and injection form that can treat PAH — but not cure it.
The 5 Groups of PH
Group 1: Idiopathic — Fairly rare, typically resulting from:
- Hereditary PAH Drug-induced PAH Connective tissue disease associated with PAH (e.g., scleroderma) Congenital heart disease associated with PAH
Group 2: Related to Left-Heart Disease — Risk factors include:
- Coronary artery disease
- High cholesterol
- Obstructive sleep apnea
- Atrial arrhythmias
- Heart failure
Group 3: Lung Disease/Hypoxia — Caused by advanced lung disease, including:
- Chronic obstructive pulmonary disease (COPD)
- Interstitial lung disease (ILD)
- Restrictive lung disease
- Obstructive sleep apnea
- Smoking, inhalants, dusts, asbestosis, coal mining, etc.
Group 4: Chronic Thromboembolic PH — Most common:
- Deep vein thrombosis
- Pulmonary embolism
- Hypercoagulable blood disorders
- Heart/lung tumors
Group 5: Miscellaneous — Causes are unclear and multifactorial:
- Chronic renal failure on hemodialysis
- Sickle cell anemia
Typically, the patients with PAH that we treat fall into groups 1 and 4. However, the FDA recently approved a drug for Group 3 ILD, and while it’s not yet approved for COPD, we have started treating Group 3 patients as well.
Per the FDA, PAH medications are generically called pulmonary vasodilators. They cause vasodilation via one of three pathways: nitric oxide, endothelin or prostacyclin. These medications can be delivered in pill form, by inhalation or, most commonly, through a continuous infusion using a subcutaneous site or intravenous catheter and ambulatory pump. Common adverse effects of vasodilators include headache, jaw discomfort, flushing, rashes and upset stomach. We make sure to educate patients and their families about the potential adverse effects and thoroughly teach patients how to take care of their medication and pump upon discharge. It’s important that patients understand they will be on this medication for the rest of their lives.
|Pulmonary Vasodilators and Associated Medications|
|Nitric Oxide (PDE-5i)
Endothelin Receptor Antagonists
|Sildenafil (Revatio)||Bosentan (Tracleer)||Treprostinil (comes in 3 forms)
|Tadalafil (Circa)||Ambrisentan (Letaris)||Selexipag (Uptravi) - pill form|
|Riociguat (Adempas)||Macitentan (Opsumit)||Epoprostenol (Flolan or Veletri) - IV forms|
No Storybook Ending, Only Hope
Sadly, I received a call from Kay recently, on the day her 14-year-old son died. Needless to say, she was crying, distraught and very angry that this horrible disease took her son at such a young age. She wanted to stop all care for herself. She begged to come off her Remodulin pump and cease all medications. In her grief, she just wanted to be done with it. With tears rolling down my cheeks, I stayed on the phone and listened to her talk and cry. When she was done, I told her I would support whatever decision she made, but I did propose that she use her anger in a positive way to fight PAH.
By the end of the conversation, Kay chose to stay on her IV pump and medications, both for herself and for her 10-year-old son. Since then, she’s called a couple of times to talk and to tell me that she and her son are planning some trips. She’s also made the decision to join our hospital’s PAH support group. Kay wants — and needs — the support of others who are struggling with this awful, incurable disease, and she wants to help others, too. We both know that she will have many bumps on the road ahead. She will have difficult decisions to make and, as her nurse practitioner, I will be there to encourage and care for her every step of the way.
I wanted to tell you this story and share my experience for two important reasons, which I hope you will remember:
- The first reason is to provide you with education and knowledge about the very rare and often misdiagnosed disease process known as PAH. As I noted previously, Kay was misdiagnosed with chemosis, not once but twice. Unfortunately, many patients with PAH are often misdiagnosed with asthma or obesity and placed on inhalers or diets. When they fail to thrive on the prescribed treatment, they are labeled noncompliant. As critical care nurses, we spend the most time with each patient and are often the ones completing the patient’s initial assessment. You can help facilitate an accurate diagnosis and avoid delays in treatment by noticing cyanotic lips, clubbed extremities and/or lower extremity edema, and presenting these findings and your concerns about PAH to the provider.
- The second reason is to highlight the importance of self-care. Remember Kay‘s echocardiogram and how severely enlarged the right side of the heart was vs. how small the left side was? These changes in the heart do not occur quickly. In fact, they take a long time to occur, which means Kay has had PAH for a very long time. Most likely, she was born with it. Recall the excuses that she made for being SOB … she works full-time and has two sons, one of whom is very ill. Many of you work full-time, work overtime and are also mothers, fathers and/or caregivers to aging parents. Caring for yourself is probably last on your list. Squeezing in regular exercise and eating well become especially difficult when you’re so busy. Busyness can easily overrun your life, resulting in an unintended, unhealthy pattern. Please know that self-care is not selfish! It’s actually the opposite: Caring for yourself enables you to provide excellent care for your family, loved ones and patients.
Have you ever cared for a patient with suspected or confirmed pulmonary hypertension?