Shortness of breath

[daedalus]

Here is a scenario meant to expose providers to a patient population that is given no coverage in most of EMS education.

You respond to the park in a poor area of town for a 20 year old Brazilian male with shortness of breath.

HPI: Patient was playing basketball with family while at a picnic, when he became so short of breath he had to immediately stop playing. Sitting down and relaxing did not palliate the sensation and family requested EMS. He tells you that he has experienced mild short of breath for the past 6 months or so and has found that sometimes he is breathing fast even after simply walking to his house from his car. He has been hoping that he had a chest cold or allergies and that it would go away by itself. However, today it is much worse and he is worried. Mother on scene says patient has never been athletic and always tired easily which has always been chocked up as just not athletic, however she has noticed a decline in his condition the past half year. He has not seen a physician because of socioeconomic reasons, and has never really had a primary care physician.

Prior medical history: None. Patient does not see a physician.

Prior surgical history: None

Allergies: None

Medications: no prescription or OTC/vitamins/supplements taken.

Family history: diabetes and hypertension on the father's side

Social history: Born in Brazil at home, and immigrated to the United States 8 years ago. He and his family are impoverished, and the patient works at a local car body repair shop at the front desk. He does not drink or smoke, and lives with family in a house.

Review of systems: General: no fever. decreased exercise tolerance past six months. Patient is easily fatigued. HEENT: gums bleed easily when brushing teeth. slight yellow discoloration of sclera. Otherwise unremarkable. Cardio: patient states that he has had to keep his shoelaces untied because his ankles have swelled recently. Pulmonary: shortness of breath on exertion and very occasionally at rest. no fever, night sweats, cough. no asthma or wheezing GI: occasional dull pain in the RUQ. otherwise unremarkable.

physical.

Vitals: radial pulse: 100 bpm and regular. BP 130/84. resp rate 24, adequate depth. temp 98.8 F.

HEENT: Jaundiced and slightly blood injected sclera is noted. bilateral jugular venous pressure is elevated to cause obvious distention as 45 degrees. some cyanosis noted around the lips.

Pulm: Lungs are clear to auscultation. Chest is symmetrical and atraumatic. No scars noted

CV: A palpable right ventricular heave and thrill are felt at the left sternal border. A grade II-III holosystolic murmur, slightly harsh, is heard at the left lower sternal border. All peripheral pulses are easily palpated and are adequate.

GI: liver span is consistent with hepatomegaly by percussion and scratch test.

Extremities: Finger clubbing is noted, along with slight peripheral cyanosis. some pitting edema is noted at the ankles. Patient has several bruises on his legs, says he is bruising easily.

Your EKG monitor shows sinus tachycardia with no ectopy in lead two. Finger oximetry is 92% on room air.

You can ask for whatever study you would like. I will even give you labs if you ask for specific ones.

What is the immediate field diagnosis? What is the underlying pathology and what is the pathogenesis of the recent decline in his condition? What is the field treatment, and what is the definitive treatment? Prognosis?

12 lead

Edited June 3, 2010 by daedalus

[DartmouthDave]

Hello,

Thank you for a well presented scenario.

This fellow has a long standing history of fatigue that has worsen over the past six months. The most notable recent changes are:

• Elevated JVD

• Icterus

• Edemous feet

• SOBOE

• Massive Mummur

• Bleeding of the gums

• Bruises

• Hepatojugular reflux

Add to this clubbing. This means this fellow has had a long standing tissue hypoxia.

Now, this is my weak area so bear with me here. I have very little experience with CV Surgical ICU type of stuff. To me is it sounds like a long standing valve issues that has worsened.

As opposed to liver failure causing the fluid over load that has stress his incompetent valves. I have seen a few non-alcoholic liver failure patients present like this (NASH). But, none had a long standing history of fatigue, clubbing and weakness. Most had decreased LOC due to ammonia levels.

So....time to make a Dx.

What is the immediate field diagnosis?

Since the physical finding lean towards righted sided diastolic failure I am going to go with an incompetent tricuspid or pulmonary valve.

What is the underlying pathology and what is the pathogenesis of the recent decline in his condition?

The pathogenesis could be worsening of a murmur he has had all his life. The right sided failure is causing hepatic congestion and the signs of liver failure.

What is the field treatment, and what is the definitive treatment?

Field treatment should be supportive care. IV, O2 and VS.

Definitive treatment will depend. In the ED this fellow would get CBC, INR, PTT, LFT to start. HIV and HEP serology. Ammonia level. ABG. A CXR and a quick look with an ultra sound. The EP would call Internal Medicine to sort it out.

Prognosis?

Good. I have seen bigger wrecks walk out the hospital. Also, he is young without a bad medical history or bad habits.

Cheers

Edited June 3, 2010 by DartmouthDave

[daedalus]

Hello,

Thank you for a well presented scenario.

This fellow has a long standing history of fatigue that has worsen over the past six months. The most notable recent changes are:

• Elevated JVD

• Icterus

• Edemous feet

• SOBOE

• Massive Mummur

• Bleeding of the gums

• Bruises

• Hepatojugular reflux

Add to this clubbing. This means this fellow has had a long standing tissue hypoxia.

Now, this is my weak area so bear with me here. I have very little experience with CV Surgical ICU type of stuff. To me is it sounds like a long standing valve issues that has worsened.

As opposed to liver failure causing the fluid over load that has stress his incompetent valves. I have seen a few non-alcoholic liver failure patients present like this (NASH). But, none had a long standing history of fatigue, clubbing and weakness. Most had decreased LOC due to ammonia levels.

So....time to make a Dx.

What is the immediate field diagnosis?

Since the physical finding lean towards righted sided diastolic failure I am going to go with an incompetent tricuspid or pulmonary valve.

What is the underlying pathology and what is the pathogenesis of the recent decline in his condition?

The pathogenesis could be worsening of a murmur he has had all his life. The right sided failure is causing hepatic congestion and the signs of liver failure.

What is the field treatment, and what is the definitive treatment?

Field treatment should be supportive care. IV, O2 and VS.

Definitive treatment will depend. In the ED this fellow would get CBC, INR, PTT, LFT to start. HIV and HEP serology. Ammonia level. ABG. A CXR and a quick look with an ultra sound. The EP would call Internal Medicine to sort it out.

Prognosis?

Good. I have seen bigger wrecks walk out the hospital. Also, he is young without a bad medical history or bad habits.

Cheers

Thanks for your thought out reply!

The CBC will show marked erythrocytosis. HIV and HEP are negative. I could not find the INR, PTT, and LFT, I am sorry. pCO2 is 34 and pO2 is 70. CXR shows cardiomegaly, and dilated main pulmonary artery, increased hilar vascular markings. Quick look on the ultrasound reveals no effusion.

The location of the murmur (LLSB), the cyanosis, the low room air O2 sats, the RV heave, the EKG findings, and the findings consistent with pulmonary hypertension on CXR should help refine your Dx.

[DartmouthDave]

Hello,

Hemochromatosis?

I saw a liver failure secondary to hemochromatosis once. I have never seen cardiac involvement in my clinical experience yet. However, a quick look online shows that hemochromatosis can cause:

* arthritis

* liver disease, including an enlarged liver, cirrhosis, cancer, and liver failure

* damage to the pancreas, possibly causing diabetes

* heart abnormalities, such as irregular heart rhythms or congestive heart failure

* impotence

* early menopause

* abnormal pigmentation of the skin, making it look gray or bronze

* thyroid deficiency

* damage to the adrenal glands

Also,

"Pulmonary hypertension is a problem that has been widely recognized only recently in patients with iron overload. A number of reports have involved patients with thalassemia major or thalassemia intermedia with iron overload (Aessopos, et al., 1995)"

Here is a link I read:

HEMOCHROMTOSIS

Also, my Grandmother had it as well. It wasn't acute like this. Rather a chronic condition. Chronic management to my understanding is bleeding. You go to the lab and they pull a pile of tubes off of you and toss them.

Cheers

PS.... I had thought about it earlier as a cause of liver failure. But, valve issues seemed more logical.

PPS... Give him Viagra!!! Just kidding. Not sure how hospital management would go. Need more time to think about it.

Edited June 4, 2010 by DartmouthDave

[Kiwiology]

* early menopause

*slap on forehead

Thats it!! :D

[DartmouthDave]

*slap on forehead

Thats it!! :D

Ooopppps....

LOL.....I think I need to take more care when I cut and paste stuff in the future.

[daedalus]

*slap on forehead

Thats it!! :D

Early menopause! Spot on my dear Watson. JK..

Our patient's present pathology is rare in the developed world, however the causative lesion is common but is usually corrected in childhood. If you were able to know somehow, you would find that our patient has had his heart murmur all of his life. In fact, the murmur used to be louder earlier in his life and has recently quieted a bit, with the thrill diminishing as well when his symptoms became worse. However, our patient was born at home and has never had proper medical care. Being in a third world environment in rural Brazil, he never had well child visits with a pediatrician and his pathology was never discovered when it could have been treated.

With a holosystolic murmur at the left lower sternal border, an RV heave, hepatic congestion with other signs of RV failure, and RV hypertrophy on EKG you can infer that the patient's underlying congenital heart defect is an isolated, moderately sized ventricular septal defect. VSD is the most common congenital heart defect, and about 1% of all live babies are born with congenital heart defects. You might also consider tetralogy of fallot, however you could rule that out based on his age (20), because he would probably have died before his second decade of life with untreated tetralogy. Further, our patient has been acyanotic most of his life. A moderately sized untreated VSD would account for the delay in full blown RV failure. With the recent cyanosis and low oxygen saturation, along with the marked right axis deviation on EKG and the dilated pulmonary trunk on CXR, you will find that the untreated VSD has progressed to eisenmenger's syndrome.

For most of his life, the hole in the intraventricular septum has caused a left to right shunt. The larger and more powerful left ventricle forced oxygenated blood through the defect and back into the right ventricle, where it was again pumped into the pulmonary circulation. Over time, the increased pulmonary blood flow caused restrictive changes to his pulmonary arteries which progressed to pulmonary hypertension. The right ventricle had to pump harder and harder to overcome the increased pulmonary vascular resistance, causing RV hypertrophy and eventually failure. The enlarged and now powerful RV reversed the shunt, and now deoxygenated blood was forced through the defect into the LV and pumped into regular circulation causing the cyanosis. Eisenmenger's syndrome is a late complication of untreated VSD where the shunt (left to right) is reversed to become right to left. The pulmonary hypertension is irreversible, and fatal. The erythrocytosis is a compensatory mechanism for the chronic hypoxemia. Jaundice and coagulopathy are secondary to liver dysfunction, caused from the chronic hepatic congestion from the RV failure. His prognosis at this point is actually quite poor, and death will probably occur within a few years even with treatment. Definitive treatment for eisenmengers is a heart-lung transplant, but survival past 5 years after transplant is quite rare.

Why did I write this scenario? You will probably never encounter eisenmenger's syndrome in your career. It is helpful however to understand the late sequelae of untreated VSD, because VSDs are other congenital heart defects are very common. But the point of this thread was to introduce you to a patient population you will start seeing of very soon. A patient population that is rapidly expanding, and one that is new to all of us. Because of the advancements of modern medicine, children with congenital heart defects are now living into adulthood, and this is a very new development. It will not be uncommon for you to care for young adults who had very complicated open heart surgery to cure or palliate congenital heart lesions.

JEMS wrote an absolutely useless, horrible article on congenital heart defects last year. The author went in with the idea that EMTs and paramedics were too stupid to be taught about the physiology and altered physiology of the heart, so instead attempted to broadly classify the many different defects into neat little "cardiodromes" dumbed down so that we could all understand. He royally butchered the whole thing and probably left most readers scratching their heads and turned off of CHD.