LPCH 13 - Outpatient Letter

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February 05, 2009
Robert Achtel, M.D.
5301 F Street, Ste 316
Sacramento, CA 95819
Jill Stocker, D.O.
874 Plumas St
Yuba City, CA 95991
RE: Last, First
MEDICAL RECORD #: xxx
DATE OF BIRTH: October 24, 1991
Dear Doctors:
Dr. Anne Dubin and I had the pleasure of seeing XXXXX XXXXX and her family in the
Pediatric Arrhythmia Clinic at Lucile Packard Children's Hospital on February 5, 2009.
As you know, she is a now 17-year-old young lady who was previously seen in the Pediatric
Cardiology Outpatient Clinic on November 4, 2008, by Dr. David Rosenthal for a family
history of sudden death, as well as a questionable left ventricular noncompaction. Her
evaluation at that time showed an echocardiogram with a normal left ventricular size and
function. They did not believe she had the diagnosis of left ventricular noncompaction
at this time but did recommend continued followup and screening for left ventricular
noncompaction. She was referred to the Pediatric Arrhythmia Clinic due to the strong
family history of sudden death and multiple episodes of dizziness with exercise.
XXXXX reports that these episodes of dizziness occur when she has been doing strenuous
exercise, such as sprinting. She just feels dizzy and lightheaded and, if she stops
exercising and rests, this resolves by itself. She denies any palpitations at that time.
She does not have any pounding in her chest or chest pain or feelings of rapid heart rate
at that time. She has never passed out. These episodes also only occur with strenuous
exercise and do not occur if she is just walking or jogging.
REVIEW OF SYSTEMS: On review of systems, she denies any other symptoms referable to the
cardiovascular system. More specifically, she denies any chest pain, tachypnea,
palpitations, syncope. She has had 1 episode previously where she was on her menstrual
period had had just gotten up from lying down. The mother saw her and noticed that she
was appearing lightheaded and became weak. She never actually passed out during that
time. The mother was able to catch her and take her back to bed. After lying down and
resting, she felt much better.
Other than the episodes of dizziness with exercise, XXXXX has been relatively healthy.
She has not had any major medical illnesses. She has no other chronic illnesses.
FAMILY HISTORY: Her family history does have multiple family members who have died from
sudden death. The father has 7 family members who have died from sudden death and 1
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family member who has an ICD. The father's father died from sudden death. The father's
3 brothers died from sudden death at ages 24, 31 and 32. The father also has 1 sister
who died at age 26. There is also a nephew who died at age of 15. All of these deaths
were during sleep, with the exception of 1 brother that died while playing softball or
baseball and the nephew who died while at a school dance. There is another living nephew
who is 28 years old at this time. Back in 2000 we put in an ICD and presumed his
diagnosis to be Brugada syndrome. The father currently has only 2 remaining siblings who
are still alive. Those are his twin sisters, who are now 59 years old. They both have
multiple children who are healthy and have not had any events. The father reports that
he and his other remaining family members have undergone multiple tests and have not been
told of any diagnosis linking all of their family deaths. The father himself has not had
any syncopal events. He has 1 other son from a previous marriage who is healthy and
asymptomatic. The patient also has a sister who is 13 years old and is healthy.
On the mother's side, there are 2 family members who have died of myocardial infarctions.
These were thought to be related to hypertension and hypercholesterolemia. These were
the mother's brother and sister, who both died around the age of 44. The mother's father
has also had heart attacks and has also had a valve replaced. There are also multiple
other family members with hypercholesterolemia, hypertension and hyperlipidemia.
MEDICATIONS:
The patient is not currently taking any medications.
ALLERGIES:
She has no known drug allergies.
PHYSICAL EXAMINATION: On physical examination today, her height is 155 cm, and her
weight is 60.5 kg. Her heart rate is 74 beats per minute, and her respiratory rate is 22
breaths per minute. Her blood pressure is 112/72 measured in the right arm. Her oxygen
saturation is 97% on room air. In general, she is a well-developed, well-nourished young
lady, in no apparent distress. Her mucous membranes are moist and pink. Her lungs are
clear to auscultation bilaterally. Her precordium is quiet, with a normal S1 and S2.
She has no murmurs, rubs or gallops. Her abdomen is soft, nontender, nondistended. She
has no hepatosplenomegaly. Her pulses are 2+ in all 4 extremities. Her capillary refill
is less than 2 seconds.
ELECTROCARDIOGRAM: An electrocardiogram was performed today, which showed a normal sinus
rhythm, with a heart rate 66 beats per minute. Her intervals are normal with a QTc of
approximately 400 milliseconds.
Review of her previous study, on an exercise test, which showed a normal sinus rhythm at
baseline and during exercise, with no ectopy or ischemic changes. Her functional
capacity was normal. The study results were read as normal. That study was performed on
November 3, 2008. She also had a 24-hour Holter monitor done on November 3, 2008, which
showed a rare premature atrial and ventricular contractions. It was otherwise within
normal limits.
An event monitor also showed multiple episodes where the patient documented feeling dizzy
and the rhythms at that time were sinus tachycardia. There was no evidence of any
arrhythmias during her recorded events. The patient reports that, during those recorded
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events, she was feeling her full symptoms which she had been describing of dizziness.
IMPRESSION: In summary, XXXXX is a now 17-year-old young lady with a history of
dizziness with exercise but no palpitations, as well as a strong family history of sudden
death. She has had an electrocardiogram, a 24-hour Holter monitor, exercise study and
event monitor recording, which all are within normal limits and no evidence of
arrhythmias. These episodes of dizziness during exercise did not appear to be related to
any arrhythmia, as the recordings on event monitor where she documented feeling her
symptoms of dizziness show sinus tachycardia.
However, given the strong family history of sudden death with multiple family members in
multiple generations who have died from sudden cardiac deaths, we are concerned for the
possible diagnosis of Brugada syndrome in XXXXX, as well as her other family members. We
would therefore like to do a procainamide challenge test, which can diagnose Brugada
syndrome. This would involve bringing XXXXX to the catheterization lab and giving
procainamide via IV to see if we can induce the EKG changes which are diagnostic of
Brugada syndrome. We have also discussed the possibility of genetic testing for the
entire family, and we are trying to arrange for them to be seen by one of the adult
cardiologists at the Stanford Hospital.
We have discussed the above with the family, and we have discussed the risks and the
benefits for the procainamide challenge test. They have agreed to proceed with the
study, and we have gone ahead and scheduled them for February 20, 2009, at 2 p.m.
Consent was obtained and has been placed in the chart. They have been given the
instructions for preparation for coming to the catheterization laboratory.
It was a pleasure to see XXXXX and her family in the Pediatric Arrhythmia Clinic today.
Should you have any questions or concerns, please do not hesitate to contact us.
Sincerely yours,
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