Monday, December 13, 2010

An Unexpected Pregnancy Complication

I was working in a rural health clinic when I picked up a new patient intake questionnaire form to read before I went into the exam room.  The form stated that the patient was an 18 year old female who had just been discharged from the hospital 6 days prior after giving birth to a baby boy.  The form stated that the patient was complaining of shortness of breath. 
I knocked on the exam door and then went in to introduce myself. 
“it says here on the form that you’re short of breath, tell me what’s going on?” I asked the young female. 
“Well shortly after I gave birth to my son.  I started having problems with my ankles swelling and feeling as though I couldn’t catch my breath.  I told the OB residents that this was going on and they just told me that was normal and it would resolve on its own.  My blood pressure was also up, but the OB residents told me that this would also resolve itself.  I was discharged two days after he was born still having some shortness of breath and with my blood pressure still high.  My ankle swelling hadn’t gotten any better either.”
“Okay, so then when you went home what happened?”
“Well, my shortness of breath got worse, my ankle swelling became worse and I started having a fever.  So I went into the community hospital ER across the street here two days ago and they diagnosed me with a urinary tract infection.  They gave me an antibiotic and sent me home.  But I know that’s not what’s wrong with me.  Something else is going on.  That’s why I came here.”
“Okay.  I’ll see what I can do for you.”
I proceeded to ask her some further questions about her personal medical history, family history, medical allergies, social history and what medications she was taking currently.  I then did the physical exam.
The patient, whom I’ll call Amy had some significant findings on her physical exam. Listening to her chest she had a S3 gallop (which means she had an extra heart sound characteristic of heart failure).  Her pedal edema was 4+ and pitting (meaning that her ankles held a large amount of fluid in them and when I pressed in with my fingers and then removed them you could still see where I had pressed in with my fingers for several seconds afterwards).  Her blood pressure was indeed elevated at 150/96.  In addition, Amy was uncomfortable lying down, she was able to breathe better when her head was elevated off of the exam table. 
I sent her off to the laboratory to give us a urine sample, so as to follow up on her presumed urinary tract infection.  While she was doing this, I walked out the back door of my clinic building, went next door, walked in the back door of my supervising physicians office (a family practice) and waited for one of the two family physicians to finish seeing a patient, so that I could run this patient by them, asking their opinion of what was going on. 
“Well,  Sharon the symptoms Amy is having is typical after just giving birth.  Why don’t you just give her a beta blocker for her hypertension (high blood pressure)  and have her come back in two days for a follow up appointment.”
Something in the back of my mind told me that this wasn’t the whole story, but I didn’t know what was wrong with Amy.  I had seen a similar case somewhere and my intuition told me that I had seen a case while I worked at St. Luke’s Hospital in Houston. 
Seeing that I didn’t know what else to do, I walked back over to the rural health clinic next door, walked into the exam room and advised Amy that I was going to start her on metoprolol for her blood pressure, she should continue with her antibiotic for her urinary tract infection and to come back in and see me in two days for a follow-up.

There are many similarities between pregnancy related changes and post-partum cardiomyopathy.  Pregnant women can have a S3 gallop present during their third trimester.  They can also have some mild pedal edema with exertional shortness of breath. Having hypertension is abnormal for pregnancy.  Having shortness of breath while sitting down or just merely walking short distances is abnormal.  Coughing up blood is abnormal. 

Due to their being many similar signs/symptoms between pregnancy and post-partum cardiomyopathy, it is understandable that these can confuse even the best of clinicians .  But for all clinicians they need to really be in tune with their intuition and listen to it when it says, “this doesn’t quite add up.”  We, as clinicians need to remember to always make sure that any patient we see fits the criteria for a particular condition.  If there is anything that doesn’t fit, then we need to re-adjust our lens and take another look to determine what is really going on. 
That evening after driving back to Houston where I lived I kept thinking about Amy and what was wrong with her.  My schedule allowed me to be off on Wednesdays so the following day, a Wednesday, I woke up the next morning and with clarity of mind I knew what was wrong with her.  I literally ran over to the medical school library to look up the diagnostic criteria for post-partum cardiomyopathy. 

Now it all made sense, I had indeed seen such a patient at St. Luke’s Hospital while I was working as an infectious diseases PA.  The majority of the patients our infectious disease service consulted on were cardiac patients or those who had received cardiac surgery.  And that is indeed where I had seen this disease in a patient.  It finally clicked with me. 

Returning to the rural health clinic the following morning I was ready to see Amy.  I was armed with two medical journal articles which described in detail how to work up a patient who potentially had post-partum cardiomyopathy.  She was my first appointment. 

I walked into the exam room to see Amy and asked her how she was doing. 
“Not well, “ Amy replied.  “I couldn’t sleep last night except by sitting upright in a chair, I was so short of breath.  Then this morning I started coughing up blood.”
Coughing up blood was one of the diagnostic signs for post-partum cardiomyopathy.  I immediately went to work. 
I sent her for a CXR across the street to the hospital, then off for a EKG reading.  When she came back I listened to her cardiac sounds, she still had the S3 gallop and now had some fine crackles in the base of her lungs.  Her ankle edema wasn’t any better.  I told her to stay put and went outside of the exam room.  I advised my medical assistant to put her on a nasal cannula with oxygen at four liters and check her pulse ox.  I took the two medical articles I had with me next door and advised my supervising physician of what was going on with Amy.
“She has what disease?”
“Post-partum cardiomyopathy.  Here read these articles, they write about how to diagnose it.  She fits all of the criteria for it.”
“I’ve never heard of it.  Are you sure about this?”
“As sure as I’ll ever be.  She needs to be admitted to a cardiology service right away.  I’m going to call the cardiologist up in Temple, Texas and get her admitted.”
“Okay, if the cardiologist agrees with you, go for it.”
I quickly walked back to the rural health clinic, picked up the phone and paged the cardiologist at Temple.

To correctly diagnose post-partum cardiomyopathy the patient must have the following signs/symptoms:  they must be in their last month of pregnancy or within the first six months after their delivery with new onset heart failure with absence of an identifiable cause, and left ventricular systolic dysfunction on echocardiogram.

Patients typically have symptoms of: dyspnea (shortness of breath), cough, orthopnea (needing to sleep with their head elevated), hemoptysis (coughing up blood), or paroxysmal nocturnal dyspnea (shortness of breath at night).

Risk factors include: age >30, mother who is multiparous (mothers who have had more than one child), African-American race, maternal history of hypertension,(increased blood pressure) pre-eclampsia.(ankle swelling, protein in the urine, high blood pressure and rapid weight gain) or eclampsia (pre-eclampsia signs plus seizures). 
“Dr. Ambrose, this is Sharon, I’m a PA at the rural health clinic.  I have a patient that needs to be admitted to the ICU under your care.  I believe she has post-partum cardiomyopathy.  She started coughing up blood this morning, she has a S3 gallop, I can hear some fine crackles over her lung bases, she has ankle edema of 4+ which is pitting, her EKG reading shows non-specific ST-T wave elevations, her CXR shows pleural effusions, a widened cardiac silhouette, a history of hypertension and she delivered her baby 8 days ago.”

I had to take the phone receiver away from my ear quickly as Dr. Ambrose started yelling at me to get her up to Temple immediately.  She needed his medical expertise immediately, if not sooner. 

I hung up the phone and went back in to see Amy.  I advised her that Dr. Ambrose had agreed with my diagnosis and that I was sending her up to Temple to be admitted to the hospital.  Amy had a look of relief on her face that finally someone had figured out what was wrong with her.  The ambulance with the EMTs came shortly to pick up Amy and take her 50 miles north to be admitted to the hospital in Temple where Dr. Ambrose was waiting for her. 

I didn’t hear again about Amy’s condition until 5 weeks later, when I picked up her patient chart outside of an exam room.  I was surprised to see her back, I thought she would just see the cardiologist in follow-up. 

I walked into the exam room, “Amy, what a pleasant surprise to see you.  I thought once you were discharged from the hospital, Dr. Ambrose would be doing your follow-up appointments.”
“Well, Dr. Ambrose said that I could alternate my follow-up appointments between him and you.  He told me I could only see you, no one else because you knew what was wrong with me and what to do, which no one else knew. “
“Wow.  So when were you discharged from the hospital?”
“I was in the hospital for ten days.  I saw Dr. Ambrose in clinic about two weeks ago, which was a little over a week after I was discharged.  He told me to come and see you in two weeks, so that’s why I’m here.  Dr. Ambrose gave me these to give to you.”
With that, Amy handed over copies of her hospital record.  I scanned through them to see that her echocardiogram has shown an EF of 30%.  She had been in the ICU for 1 week during which time Dr. Ambrose had given many doses of lasix, started her on catopril, and kept her on oxygen supplement.  Amy had been sicker than I originally thought.  She had lost almost 50% of her heart function, from a normal of 55%, it had decreased down to 30%. 
After I finished looking through the hospital records, I turned to Amy and asked, "So how are you doing now?”
“I feel so much better, I’m finally able to breathe lying down at night and sleep normally. Dr. Ambrose has me on catopril and lasix every day.  My blood pressure is staying down and look, my ankles are now normal!”
“I can see, how nice.  Well, let me listen to you with my stethoscope and then I’ll see what Dr. Ambrose wrote in his follow-up note for me.”
Amy’s physical exam was normal, her lung fields were clear, her heart sounds revealed a normal S1 and S2 with no S3 gallop.  Her pedal edema was totally gone as was her shortness of breath.  Her blood pressure readings were normal with her on an ace inhibitor and a diurectic. 
“Alright, I see here that Dr. Ambrose wants you to see him and then me alternatively, as you said.  I also see here in the records that he wants to repeat your echocardiogram in 2 months and then again at 6 months post discharge.  Has he talked to you about your not being able to have another child?”
“Yes, he brought that up.  I have a prescription for birth control pills and I’ll be starting those next week, six weeks after delivery of my son.”
“Okay, well then it looks as though your doing really well, considering what you’ve been though.  Seeing that Dr. Ambrose wants to repeat your echocardiogram at 3 months post discharge, why don’t you make an appointment to see him in 4 weeks, which will then be 3 weeks prior to when he wants to do the echocardiogram.  That will give him enough time to get your echocardiogram scheduled.”
“Sounds good.”
“Great, well then I’m glad to see you back.”

Treatment for post-partum cardiomyopathy depends upon when it is diagnosed.  If the patient is in her last month of pregnancy, she usually undergoes delivery of her child.  During this time period she is on beta-blockers and diurectics.  After she has delivered she is then switched over to an angiotension converter enzyme inhibitor which helps address ventricular afterload.  Diurectics are continued.  They are then followed by serial echocardiogram to assess for return of heart function.

Amy stayed on her birth control pills and at six months after discharge her echocardiogram showed her ejection fraction to be 45%, which was still 10% below normal.  I knew not to expect any more recovery of her heart function over this.  By this time, Amy was on 20 mgs of lasix a day and 25 mgs of captopril three times a day.  She had been very compliant with her medical regimen, which pleased me no end.

With the results of her last echocardiogram, Dr. Ambrose brought up the need for her to be permanently sterilized again.  Amy set up an appointment to be seen by the OB/Gyn service at the teaching hospital where she had delivered with the intentions of acquiring a tubal ligation.

Patients who have post-partum cardiomyopathy are at a high risk of having it occur again with any subsequent pregnancy.  This is especially true if patient’s echocardiogram at six months post diagnosis shows that their heart function has not returned to normal.  If a patient has a subsequent pregnancy and post-partum cardiomyopathy re-occurs their heart failure tends to be worse and they face increased chance of dying.  It is for this reason that in patients who do not re-acquire their normal heart function (based on their echocardiogram findings) they are counseled to receive permanent sterilization.

Her next alternating appointment follow-up after she had seen the OB/Gyn service was with me. 

“Amy, I’m so glad to see you.  How are you doing?”
“I have some questions.  I went down to the teaching hospital and saw the OB/Gyn service two weeks ago.  They did a repeat echocardiogram and they told me that I didn’t need to be permanently sterilized.  They told me that I could have another baby without a problem.  So what do I do?”
“Well,  Amy, do you remember my giving you that medical article to read on your disease many months back, when you came to see me the first time after your discharge?”
“Do you remember what it said about having another child after you have been diagnosed with post-partum cardiomyopathy?”          
“Yes.  That’s why I’m confused.  You and Dr. Ambrose have repeatedly told me that I can’t have another child.  Yet, I went down to see the OB/Gyn service and they told me I could.  So whom am I to believe?”
“Well, let me put it this way.  Who diagnosed you correctly and then treated you in the ICU?”
“You and Dr. Ambrose.”
“Who has treated you since your discharge for your post-partum cardiomyopathy, seen you at your follow-up appointments, monitored your medications, and given you information to read on your disease so that you could understand what has happened to you?”
“You and Dr. Ambrose.”
“And when you told the OB/Gyn service that you were having problems right after your delivery of your son, did they believe you, or just discharge you?”
“Discharged me.”
“So now, are you going to believe the OB/Gyn service regarding your capability of carrying another child to term, or are you going to believe the literature I gave you to read, Dr. Ambrose’s conversations with you about it, as well as my own?”
“Well, now that you put it that way.  I can only believe what you and Dr. Ambrose have told me.”
“Yeah.  So I can assume that you are going to make another appointment with the OB/Gyn service and be sterilized this time?”
“Yes, I’ll go home and make another appointment with them.  And this time, I’ll stick to my guns about it.”

At her next follow-up appointment with me, Amy had received her tubal ligation.  Her days of having children were over at the tender age of 20.  Since her high school graduation she had faced a lot.  She had married shortly after graduating at the age of 18, had a child 11 months later, faced a life-threatening disease, and now at the tender age of 20 would never conceive again.   But at least her 8 month old son would have his mother around to see him graduate from high school one day.  I could take pleasure in that.

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