Sunday, December 27, 2015

A Congenital Heart Valve

I picked up the medical chart outside the patient exam room and walked into introduce myself. 
“Hi, I’m Sharon, I’m a PA and am working in this clinic temporarily covering for another PA who is on maternity leave.  How can I help you?”
The male Hispanic patient was leaning forward on the exam table obviously short of breath as well as showing facial grimaces from pain.  He was only 24, way too young to be having chest pain from a MI. 
“I’m having chest pain and can hardly catch my breath.  I was here being seen about a month ago with almost the same symptoms but the PA who saw me didn’t think anything about it.  Now my symptoms are worse and I can hardly breathe.  I’m also very tired, I have no energy.”
“Okay, well do you have any history of asthma, breathing diseases, heart disease, for instance?”
“No, I’ve never been sick.  I’ve had the flu a few times but that’s it.  I rarely saw a doctor when I was younger.  I think I only went in when I needed a vaccination for school.”
“Does anyone in your family have any lung problems such as asthma, or COPD for instance?”
“No, not that I know of.”
“How about any cardiac history, anyone in your family with a history of heart attacks, or the like?”
“No.  Not in my mother’s relatives.  I don’t know anything about my dad’s side of the family, seeing that he left when I was a toddler.”
As he answered my questions he continued to give me facial grimaces from his chest discomfort. 
“Okay, well let me check you over quickly.”  After checking his vital signs which showed a somewhat high blood pressure as well as a pulse oximetry (blood oxygen levels) which showed his oxygen level at 90% (a little low) I started doing my physical exam. 
I quickly went over the head and neck exam, and moved my stethoscope over his lungs to listen to them.  His lung fields were clear, he didn’t have any wheezes or abnormal sounds.  I then listened to his heart sounds.  I put my stethoscope over his anterior chest wall to find out he had a very loud systolic murmur.  The murmur was over his aortic valve.  It was at least a 4/6, possible a 5/6 in intensity.  He didn’t have a thrill.  The remaining three areas that we clinicians listen to for heart sounds were normal. 
I felt his radial pulses bilaterally and they were equal.  The murmur radiated up to his carotid pulses in his neck.  I could also hear the murmur over his posterior back.  I had the patient lay down and finished doing my abdominal exam as well as feeling his pedal pulses (pulses in the feet). 

When working up a patient who has chest pain you have to acquire a full medical history on them.  This will then guide you on what potential medical diagnosis’  the patient maybe having.  This patient was 24, he was too young to be having a heart attack (these generally occur in men > 40 years of age).  He denied having any problems with asthma or other lung conditions.  So I am left with a cardiac (heart) problem. 
After I listened to his chest I heard the typical sound of a stenotic (hardened) aortic valve.  When we listen to the heart we grade heart murmurs from 1 to 6.  A one means that most people even with a stethoscope listening to the chest wall will not be able to hear it.  A murmur graded as a two is a soft murmur, barely perceptible, you have to really listen to hear these.  A murmur graded as a three is louder, definitely there.  A four is quite loud.  A five is louder yet, it sometimes has a thrill associated with it, meaning that you can feel with your hand on the chest wall the vibration of the underlying cardiac murmur.  A six is very loud, sometimes it can even be heard without your stethoscope even on the chest wall.  Thrills are always felt with a murmur determined to be a six. 
Then there are systolic murmurs vs diastolic murmurs.  This just means at what point in the cardiac (heart) cycle is the murmur heard.  Is it during systole (when the heart is pumping) or in diastole (when the heart is at rest)?
Heart murmurs can also radiate their sounds to other areas of the body.  Many of them will radiate to the back or neck for instance. 

I advised the patient, “I want you to stay laying down, I’m going to send the medical assistant back in here to do an EKG on you and then I’ll come back in and explain what needs to be done, okay?”
“Ok.”
I left the exam room and advised my medical assistant to get an EKG on him.  While she was in doing this, I picked up the phone and called the local cardiologist’s office.  When his office receptionist picked up the phone I advised her that I had a patient who needed an urgent echocardiogram and an appointment to be seen by the cardiologist within the next few days. 
The receptionist made the necessary appointments and I wrote them down on a piece of paper which I would give to the patient when I went back into see him. 
By that time the medical assistant came out of the patient’s room with the EKG in her hands which she gave to me.  The EKG showed he had an enlarged left atrium, a slightly enlarged left ventricle, with no tall R waves or ST changes. 

An EKG (electrocardiogram) is useful in patients who have a cardiac diagnosis.  It can tell you whether any of the chambers are enlarged, whether the nerve conduction from the upper to the lower chambers has been affected, or slowed down due to damage, such as in a patient who has a history of having a heart attack. 
In a patient who has aortic valve involvement you are looking for changes on the left side of the heart, strain on the left ventricle (ST changes) as well as changes in the R wave. 

I went back into to see the patient and began explaining everything I had found out.  He was still laying down. 
“You can sit up if you’d like,” I advised him.
“No, it’s more comfortable for me if I lay instead.”
“Okay, well then let me explain to you what I found out and what needs to be done.  You have a defective heart valve, with you it’s your aortic valve.  The heart has four chambers from which it pushes blood into your lungs and then into your whole system.  Between each of these chambers is a heart valve which helps to keep the blood from back flowing when the heart muscle contracts.  In your case one of your valves which we call the aortic valve has decided it doesn’t want to work anymore and hence it has become hardened and probably almost unmoving.  The aortic valve is the valve that blood flows through when it is leaving the heart and going to the rest of the body.”
“When I listened to your chest just a few minutes ago I heard a very loud heart murmur which told me that your aortic valve was damaged.  Each part of your chest wall represents a certain location where heart sounds are referred to.  The aortic valve sounds are referred to your upper chest on the left side.” 
“Do you understand everything I’ve said so far?”
“I think so.”
“Okay, well when a heart valve decides to begin not working like it should, it gives you a lot of symptoms, such as your chest pain and your having a hard time breathing.  You also mentioned you were tired and had no tolerance for walking for any long distances.  Your elevated blood pressure is also a symptom of your aortic valve not working the way it should.”
“Do you understand what I just said?”
“Yeah, I was going to ask you about my chest pain and not being able to breath, but you just explained it.”

Patients who have heart problems can have a myriad of signs and/or symptoms.  These can include:  (not a complete list, just some of the more common ones)
--high blood pressure
--fatigue, low exercise tolerance
--chest pain
--shortness of breath
--passing out (syncope)
--palpitations (a feeling or knowledge that your heart is beating)
--dizziness

“Okay, well I have written down on this paper a referral for you to get an echocardiogram, which is a study of your heart where they bounce sound waves off of your chest wall to ascertain your heart function and the function of your valves.  It won’t hurt at all.  Below your echocardiogram appointment is your appointment to see the cardiologist whose office you’ll be visiting to get your echocardiogram done at.  Your appointment for both of these is in two days.  Keep these appointments, it’s very important.  The cardiologist will get a lot of information off of the echocardiogram.  You can expect him to see you and then immediately refer you over to the cardiovascular surgeon to have your valve replaced.  Due to your age the valve replacement will be a pig valve, this will be used so that you don’t have to be on blood thinners, which you would have to be on, if they put in a St. Jude metallic valve for instance.  Once your valve is replaced all of your symptoms should disappear.  You can expect the cardiologist to tell you he has to see you every 6 to 12 months and that you will have to have an echocardiogram done every year to assess the valve replacement’s function.  Any questions will this information?”
“No, I think you covered it.  So I just have to deal with this chest discomfort and feeling as though I can’t catch my breath until I see the cardiologist?  
“That’s right.  Because I think you’re going to be in the operating table before the week is out, I don’t want to be giving you anything for your blood pressure or pain because that will mask your symptoms, and the cardiologist needs to see how symptomatic you are.  He will use this information when he talks to the surgeon and this will be a deciding factor in how soon you get your valve replacement.”
“Do you understand?”
“Yeah, I think so.”
“Okay, well I wish you well.  You can let your employer know to expect you out of work for at least six weeks, if you need me to sign the paperwork for this, please bring it in.  Otherwise the cardiologist can do it.”
“Thanks.”

A few days later I received the echocardiogram results with the cardiologist’s encounter note.  The echocardiogram showed a bicuspid aortic valve (people have a tricuspid, or 3 leaflet aortic valve normally).  His mean pressure across the valve was 45, with an aortic valve area of 1 cm2.  His left atrium was mildly enlarged, his left ventricle was slightly enlarged but his ejection fraction was 60%. 

The cardiologist note showed the same physical exam findings as I found and his plan was to have the patient seen by the cardiac surgeon within 2 days to be scheduled for an urgent aortic valve replacement.

There are accepted guidelines put out by the American Heart Association/American College of Cardiology regarding when to replace a heart valve.  The latest guidelines were put together in 2014.  This patient was symptomatic, had little exercise tolerance, his echocardiogram showed severe aortic stenosis, he was determined to be a D1 (based on the guidelines) which necessitated his having his valve replaced. 

Due to his age (24) he will be facing several valve replacements over his lifetime.  With this in mind they don’t like having to put in a metallic valve replacement, they would rather use a pig valve initially so that the patient doesn’t have to be on daily blood thinners. 

Bicuspid valves are acquired while in utero, they are generally associated with a genetic mutation on chromosome 9.  They can be associated with other medical conditions such as Turner Syndrome, coarctation of the aorta, etc.  Patients typically are told to have their family members screened for the presence of their having a bicuspid valve.  Patients are also given instructions on prevention of endocarditis (infected heart valve). 

I didn’t hear anything else until two months later when the patient showed up on my clinic schedule.  I was happy to see him back. 

I knocked on the door and went in. 
“How do you feel?” I asked excitedly. 
“Oh, you don’t know how great it feels to be back to normal again.  I can do anything I want, all of my symptoms are gone.  The cardiologist doesn’t want to see me back for a year he said.  I went back to work two weeks ago.  I now have this ‘zipper’ as they say on my chest to remind me of what happened.” 
“Well, yeah for you.  I’m so glad you’re back to normal.”
“I just need you to check this on my surgical scar, I get just a speck of yellow drainage every day or so on the band aid I have over it.  It doesn’t hurt, it doesn’t bother me otherwise.”
“Well, these are generally called a ‘seroma’ which just means yellow colored fluid drainage.   Let me put some exam gloves on and I’ll look at it.”
After inspecting the very minimal ¼” opening over his surgical scar and ascertaining that the fluid was coming from just below the skin epidermis and didn’t extend down to his sternal wires as well as there not being any skin erythema (redness), nor any increased heat to the area, I knew it would continue to heal just fine. 
“What you have is what we call a seroma.  It’s not infected, it will continue to close up on its own.  Just keep it clean with soap and water every day when you take a shower and if you want to keep a band aid on it to absorb the one or two drops of yellow fluid it expresses daily that’s fine.  Just come back into clinic if it starts getting larger, or you start having a fever, or the skin around it starts becoming red and/or painful.  Otherwise you’re doing great.  I’m so happy for you.  Can I listen to your new valve?”
“Sure, go ahead.”
I put my stethoscope up to his anterior chest wall and listened.  His heart sounds were all totally normal, as if he had never had an abnormal congenitally acquired bicuspid valve. 

  

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