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HPI
18 year old white male presents to the ED after
passing out during his last basketball game as a high
school senior. Patient recalls that the event occurred
shortly after a steal and a fast break down the court.
He remembers that after scoring he began to feel
light headed and weak. The next thing he remembers
is waking up on the court surrounded by all of his
teammates, and the team nurse. 911 was called and
he was immediately brought to you. He claims to feel
fine now and appears relaxed. His mother is crying
and keeps saying “this can’t be happening again.”
What questions would you like to ask the patient and his family?
• Pmhx: none, other then the occasional cold he reports
being a healthy athletic kid
• Allergies: none
• Medications: none
• Hospitalizations/Surgeries: Tonsillectomy at age 6.
• Social: lives at home with parents and 2 sisters. Does
not smoke, admits to occasional alcohol use on the
weekends with 2 best friends. No illicit illegal drug use.
• Family Hx: Mom with DM2, Dad with HTN controlled
on HCTZ.
– Mom crying because her brother at 16 died suddenly after
collapsing on the football field. She said it had something
to do with his heart, but no other information is known.
• ROS: all negative.
What is in your differential diagnosis at this time?
• Syncope
DDx:
– Neuro: Stroke, embolus, ischemia, vasovagal
– CV: arrhythmia, cardiomyopathy, ischemia,
tamponade, aortic dissection, hemorrhage,
valvular disease
– Pulm: PE
– Meds: think of those affecting conduction, long QT
syndrome, anti-depressants
– Misc: dehydrated, illicit drug use (something he is
not telling you)
• And many many more
What is your next step to assess this patient?
• Vitals
Physical Exam
– HR 68, BP 124/75 (sitting) 118/69 (standing), RR 18, T 37.3
• General: appears in no acute distress, sitting comfortably
• CV: regular rate and rhythm, no rubs or gallops
appreciated. Do appreciate a 2/4 systolic ejection murmur
best heard over the left sternal border. When you have the
patient stand up the murmur seems to worsen, when the
patient is laying down though, the murmur decreases.
• Pulm: CTA
• Abd: wnl
• Neuro: CN II-XII grossly intact, 5/5 strength bilaterally UE
and LE. No numbness, tingling, loss of sensation
• Psych: normal affect
What to do next? Imaging? Labs? Send home?
Labs/Tests
• CBC: WBC 6000, Hgb 15.6, Hct 45%, Plt 200,000
• Chemistry Panel
– Na 140, K 3.8, Cl 101, Bicarb 24, BUN 12, Cr 1.0,
Glucose 94
•
•
•
•
•
TSH: 2.7uU/mL (normal)
Troponins : Negative
Urine Drug Screen: Negative
Head CT: Negative
CXR: Normal
Now ready to send home?
Two things need to be done:
1) EKG
2) Echo- used if still unsure of diagnosis, or if
need to visualize the heart
Any thoughts now?
Cardiomyopathy
Three Main Types
• Dilated Cardiomyopathy
• Restrictive Cardiomyopathy
• Hypertrophic Cardiomyopathy
Hypertrophic Cardiomyopathy
• Most common cause of Sudden Death in young
individuals.
• Sporadic and Familial forms exist
• Sporadic Seen in the elderly while familial seen in
young individuals
• Familial noted to be autosomal dominant with
nearly full penetrance.
Use of ECG and Echo in HCM
-
Findings on 12-lead ECG are abnormal in 75-95% of HCM patients.
Common abnormalities are LVH and widespread, deep, Q waves,
which suggest an old MI. Many patients will have arrhythmias, both
atrial and ventricular. ECG’s are useful principally for suggesting the
possibility of HCM in relatives of HCM patients and in athletes
undergoing pre-participation screening.
(is used to screen all athletes in Italy for HCM, not yet used as a screening tool in the US)
- Two-dimensional echocardiography is the usual method of diagnosis.
Echocardiography can be used to confirm the size of the heart, the
pattern of ventricular hypertrophy, the contractile function of the heart,
and the severity of the outflow gradient. It has the advantages of high
resolution and no known risk.
Echocardiography has been used extensively both as an aid to diagnosis and for
research into the pathophysiology of hypertrophic cardiomyopathy (HOCM). It is
characterized by an abnormal arrangement of the myocardial cells, which
instead of lying in parallel rows, form whorl-like patterns. It most commonly
affects the interventricular septum, but may also involve the entire myocardium
or occur in isolated areas undetectable except by detailed histopathologic
examination. As shown in the above echo, the interventricular septum is
enlarged, thus creating an outflow tract obstruction.
Pathophysiology
• Hypertrophy of the myocardium
– Disproportionately greater hypertrophy of the
interventricular septum then the LV free wall.
– This may lead to an obstruction of the LV outflow tract
below the aortic valve.
– Leaflet of the mitral valve is drawn against the IVS which
can lead to regurgitation
– This hypertrophy of the LV leads to a restricted filling and
therefore a DIASTOLIC dysfunction.
• Syncope caused by the outflow obstruction
• Sudden death due to ventricular
tachycardia/fibrillation
Enlarged interventricular septum leading to outflow tract
obstruction
This is a histological section of the conduction system in the
septum which shows the aberrant myofibers
Disarray and disorganization of myocardial fibers, showing abnormal branching,
overlapping and hypertrophy, with interstitial plexiform fibrosis
Common Clinical Findings
• Harsh Systolic Ejection Murmur
• Murmur intensity increases with a decreased
preload (obstruction worsens).
– Such as standing up, valsalva etc.
• Murmur will decrease with an increased preload
– The increased preload essentially opens the outflow
tract
– Therefore our treatment will be aimed at increasing
the preload. And avoiding activities that increase the
HR thereby reducing filling time.
• Angina or Syncope are common due to the
outflow obstruction
Treatment
• Avoid Strenuous Activity to avoid shortening
filling time.
• Avoid drugs that decrease the pre-load (diuretics)
or increase the hearts force of contraction
(digitalis).
• Beta Blockers remain the mainstay of treatment.
– By decreasing the HR you prolong diastole increase
filling time and therefore increase preload
– Also affective and decreasing myocardial contractility.
• Defibrillator implantation
– This may be a necessary procedure to prevent sudden
cardiac death.
What Next?
• Screen All First Degree Relatives!
• Stop all sports.
Board Review Questions
• Young teenager playing sports drops dead?
Think Hypertrophic Cardiomyopathy
• What part is most thickened? Interventricular
Septum, this leads to an inwardly drawn mitral
valve leaflet
• Dysfunction? Mainly diastolic as the left
ventricle is non compliant and therefore
restricts filling.
• Main treatment? Beta Blockers
• http://www.ncbi.nlm.nih.gov/pubmedhealth/
PMH0001243/
• http://www.4hcm.org/
• http://www.heart.org/HEARTORG/Conditions/
More/Cardiomyopathy/HypertrophicCardiomyopathy_UCM_444317_Article.jsp
• Goljan, Edward. Rapid Review Pathology,
Elsevier 2009 Edition