LANCELOT’S DIAGNOSIS
When Lancelot was young my goal was to get him through the day into the
next, if any goodness came from that day it was a blessing, but actually it did
not matter as long as the next morning he was still with me.
..
2008 According to Lancelot’s Doctor’s there is no medical reason for
him to be alive. He should be having major strokes, organ failure,  and he should not be able to walk or talk.
Yet everyday Lancelot is determined to follow through his agenda.
He pays no mind to the fact that he has..
Severe Peripheral Pulmonary Stenosis
Severe Aortic Stenosis
Renal Stenosis
William’s Syndrome
Von Willebrandt’s II B
Chronic Hypertension
Pulmonary Hypertension
Allergic to almost everything
Scoliosis
Joint Problems
Sensory Integration Problems
Etc.
( As of September 2010 Lancelot
no longer has Von Williebrandt’s
Disease)
GOD IS GOOD

2 Corinthians 3:4-5
We have such trust through
Christ toward God.
Not that we are sufficient of
ourselves to think of
anything as being from
ourselves, but our
sufficiency is from God.
Lancelot has had many
wonderful doctors He has had a few that were not so great. Two of them, Dr.
Bridgett Freeman and William Marvin have been with me every step of the way
with Lancelot. I have their office phone numbers, their pager numbers, their
cell phone numbers, and their home phone numbers. We have been to both their
homes. They have heard my voice on the phone many times saying Lancelot’s blood
pressure is up, Lancelot is having some strange symptom, or have you heard of
this new test of treatment. Never once have they  not been there to answer my questions.

( Lancelot and Dr. Marvin)

(Lancelot with Dr. Freeman)

University of Florida Pediatric Cardiovascular Center
841 Prudential Drive, Suite 100
Jacksonville,FL 32207
*’iill6fi;’1,’-il’i”
January ll,2010
Krupavati Maramreddy, M.D.
303 Pine View Drive
Waycross, GA 31501
PATIENT NAME: HOLTON, LANCELOT
DATE OF BIRTH: 0910411992
MEDICAL RECORD NUMBER: 759081
Dear Dr. Maramreddy:
Thank you for requesting a repeat cardiovascular consultation on your patient, I7-Il2-year-old
Lancelot Holton, whom I had the pleasure of seeing at the Pediatric Cardiovascular Center today
with his mother.
SUMMARY OF FINDINGS AND MANAGEMENT RECOMMENDATIONS: Lancelot has
Williams and von Willebrand’s syndromes: He has been clinically stable. He continues to have
a degree of bilateral branch pulmonary artery stenoses with the right somewhat less than the left
which has responded nicely to his infantile bilateral balloon dilation angioplasty performed at
Boston Children’s Hospital by Dr. Lock. At that time and subsequent catheterization he was
noted to have tubular hypoplasia of his entire descending aorta. Please note that this was recently
again evaluated by Dr. Faris Al-Mousily in Gainesville by MRI (05/2008). This hypoplasia has
remained remarkably severe and unchanged in comparison to the patient’s clinical state that is
the size of his descending aorta just proximal to the diaphragm is approximately 7 mm and at the
iiiac bifurcation is 4-5 mm in diameter. Despite his anatomic substrate Lancelot continues to
have higher blood pressures in his legs than in his arms and to have no increase in his left
ventricular mass index. He continues beyond antihypertensives as directed and followed by Dr.
Tolaymat in that clinic. He continues to be on numerous supplements provided by Dr. Bridget
Freeman including adrenal thyroid supplements and lithium as part of her holistic care. Based
on today’s findings which continue remained rernarkably unchanged I have asked Lancelot to
retum p.r.n. or in 6-12 months for repeat cardiovascular physical examination, EKG, limited 2-D
echocardiog&ffi, and for extremity blood pressure recordings. He can be active to his level of
comfort. He does not require SBE precautions. There are no absolute cardiovascular
contraindications to medications, anesthesia, surgery, however the risk of sudden demise with
stressful interventions has to be recall by all. The mother is fully cognizant of our findings and
recofirmendations.
1of 3

Patient Name: LANCELOT A. HOLTON
MRN:759081
DOS: 0111112010
SUMMARY OF INTERVAL HISTORY, PHYSICAL EXAMINATION AND LABORATORY
DATA: Periodically Lancelot does complain of some lightheadedness and tiredness. These
have been noted around Thanksgiving and other times. Sometimes he has a headache and
weight gains at night. The mother is concerned that his blood pressure has been lower than in
the past. She keeps inaccurate diary and shared that with me today. When his blood pressure is
lower she noted that his blood pressures run from 100-120 and I reassured that he should do
well. At point in time, now almost 10 years ago he had a transient ischemic attack, but
subsequently has had no reculrence of any neurologic symptoms nor has he had any sequelae
from that event. He is known to have involvement of his great arteries at his transverse arch and
the beginning of his aortic hypoplasia at the aortic isthmus. He has no complaints of chest pain
or palpitations. He has had no syncope, cyanosis, or dyspnea. He is able to accomplish his
physical activities without any difficulty. He has had no hospitalizations, surgeries, or major
illnesses in the interval. She reports no new problems. Under the review of systems. There
have been no changes in the family medical or the family social history. Lancelot remains
allergic to Ceclor. His immunizations are up to date. He continues to take atenolol 50 mg by
mouth twice a day, and Vasotec 10 mg by mouth twice a day. He sometimes takes Benadryl at
night. He is not changed his holistic supplements.
On physical exam today, Lancelot had a weight of 42.6 kg and a height of 161 cm, both
symmetrically beneath the 3rd percentile. His heart rate was 62 and respiratory rate 19. His
blood pressures were right arm 116158,1eft arm 11616l, right leg ll3l73, and left legll0l72. He
has a phenotypic features of Williams syndrome. He was well-nourished, well-hydrated,
acyanotic, and in no distress. He had no cranial bruits. His chest was clear to auscultation. His
precordium was quiet to palpation. His rhythm was regular. His first heart sound was normal.
His second heart sound was narrowly split and closed. He had a Grade II/VI pulmonic systolic
ejection munnur at the left upper sternal border, which could be heard faintly and symmetrically
in all lung fields. Diastole was quiet. He had no clicks, rubs, 53 or 54. Abdominal examination
reveals situs solitus without hepatosplenomegaly. He had a prominent aortic bruit that in the
midline was lower than his precordial munnur, Grade II-IIIA/I. There was no thrill. It was
slightly louder throughout his left abdomen than his right abdomen. Peripheral pulses were 1*
throughout and perhaps even slightly greater in his legs than his arms. He had no delay. He had
a brisk capillary refill without peripheral acrocyanosis.
Lancelot continues to have a normal EKG. He had normal sinus rhythm, without conduction
delay, or dysrhythmia. He had normal QRS progression without hypertrophy. His 2-D
echocardiogram with Doppler interrogation if anything remains remarkable and somewhat
improved. He continues to have normal chamber dimensions, with normal left ventricular mass
index and a left ventricular ejection fraction of 73oh. There is a peak instantaneous gradient up
to 45 in his proximal descending aorta. However the signal in his descending aorta is absolutely
normal and consistent with his coarctation. He has an 18 mm gradient and a 38 mm gradient
(peak instantaneous) across his right and left branch pulmonary arteries respectively.
2of3

Patient Name: LANCELOT A. HOLTON
MRN:759081
DOS: 0lllll20t0
Thank you for referring Lancelot to us. If you have any questions regarding today,s evaluation,please do not hesitate to contact me at your convenience. If you request, copies of thecomprehensive history (including history of present illness, past medical hisiory, family medical
history, social history, and review of systems), compreh.nrirr” physical examination, and copiesof laboratory reports can be forwarded from our records to your ornce-Hopefully these
summaries are suffi cient.
Respectfully yours,
WILLIAM J. MARVIN, JR., MD
Associate Professor of pediafics
CC: Mrs. Rene Holton
210 South Wheeler Avenue
Douglas,Georgia 31533
CC: ASAD TOLAYMAT, MD
CC: BzuDGET FREEMAN, MD
CC: JAMES E. LOCK, MD
D: 2010011309920368 MT: 23204Dictator: ll7 D:01/t3l1015:35 T:01/14/10 03:22Confi rmation Number : 7 5 692 (l 17 .56.254. 7590g I )
Eleckonically signed by:william Marvin-Jr. M.D. Jan t4 2oto 3:35pM EST
3of3

UNIVERSITY MEDICAL CENTER
PEDIATRIC CARDIAC CATHETERIZATION
NAME: HOLT N, LANCELOT
UMC 759081-4
DATE: MARCH
PRE-CATHETERIZA DIAGNOSIS: PERIPHERAL PULMONARY ARTERY
STENOSIS; COARCTATION OF THE
1
AORTA;
j
POST CATHETERIZATION NOSIS: SEVEBE DIFFUSE BRANCH PULMONARY
,/
AIITERY STENOSES; COARCTATION OF
\ -“*{HE AORTA; SUPRAVALVULAR AORTIC
STENOSIS; PATENT FORAMEN OVALE;
BIVENTRICULAR HYPERTROPHY; LABILE
SYSTEMIC HYPERTENSION.
PROCEDURE: COIVTFIIVE RIGHT AND LEFT
HEART CATHETERIZATION WITH
SELECTIVE CI NEANGIOGRAPHY
CARDIOLOGIST: WILLIAM J. MARVIN, JR., MD
REFERRING ENTITY: NEMOURS CHILDRENS CLINIC
t. INDICATIONS:
This 18 month old male toddler was suspected of having underlying pulmonic
stenosis by an outside pediatric cardiologist in Savannah, Georgia. He had
been extensively followed at the Nemours Childrens Clinic for multiple
anomalies, He was referred to Dr. Ed Bayrre who felt the toddler had
peripheral pulmonary artery stenosis and possible significant coarctation of
the aorta. He scheduled the patient for elective cardiac catheterization at this
time to define his anatomy and hemodynamics. The patient was on no
cardiovascular medications.
HOLTON, LANCELOT
uMc #0759081-4
CATH DATE: 314194
PAGE TWO
il. PROCEDURE:
After sedation with 1 .2 cc. of CM3 intramuscularly, the patient was taken to
the Cardiac Catheterization Laboratory and prepped and draped in the usual
manner, Via the percutaneous technique, a #6 F wedge catheter was
introduced into the right femoral vein and subsequently replaced with a #6
F Berman catheter. A #6 F pigtail catheter was placed into the right femoral artery. Complete right and left heart catheterization with selective
cinearrgiography was performed without difficulty or complication. During
the catheterization, there was unsustained second degree AV block and brief
episodes of supraventricular tachycardia with catheter manipulation, These
unsustained episodes resulted in no hypotension. Following cineangiography,
all catheters were removed and the bleeding controlled by pressure.
Estimated blood loss: 1o cc. Fluids given: Dbw 1/4 NS with 20 mqs. of
potassium chloride/L at 35 cclhr. Contrast given: 53 cc. Omnipaque 35O.
Radiation time: 18.5 minutes. Medications given: o.25 mgs. of Atropine lV.
Measured oxygen consumption: 94.8 mls/min.
ilt. DATA AND CALCULATIONS:
There was marked elevation of both ventricular pressures. The rightventricular pressure was extremely elevated at 145l2O. There was no
gradient across the pulmonic valve. There was severe pulmonary
hypertension proximalto the branch pulmonary arteries. Both the distal right
and left pulmonary arteries were entered demonstrating a peak 120 mm.
systolic gradient. Left ventricular pressure was elevated with a 30 mm.
gradient across the supravalvular aortic area. Additional 35 mm. gradient
was accounted across the aortic isthmus. Despite the multiple levels of left
heart obstruction, intermittently, the patient’s pressures would be as high as
2oO systolic in the descending aorta. Based on these pressures, the
calculated pulmonary resistance, systemic resistance, pulmonary arteriolar
resistance, and the pulmonary to systemic resistance ratios were all elevated.
Cardiac output was calculated to be normal. There were no intracardiac
shunts found and systemic arterial saturation was full.
tv. CIN EANGIOGRAPHY:
Left ventricular angiogram was performed in the long axial obliquebiplane with 20 cc, of omnipaque 3bo at a flow rate of 30 cclsec. at450 PSr.
HOLTON, LANCELOT
uMc #0759081-4
CATH DATE: 314194
PAGE THREE
2. Ascending aortogram was performed in the AP and lateral biplane with
30 cc. of Omnipaque 350 at a flow rate of 40 cc/sec. at 9OO PSl.
3. Abdominal aortogram was performed in the AP plane with 8 cc. of
Omnipaque 35O at a flo,w rate of 30 cc/sec. at 75O PSl.
4. Right ventricular angiogram was performed in the AP and lateral biplane
with cranial angulation using 15 cc. of Omnipaque 350 at a flow rate of
30 cclsec. at 45O PSl.
5. KUB was performed.
V. INTERPRETATION:
1.
2.
3,
5.
Left ventricular angiogram revealed signif icant left ventricular
hypertrophy. There was no true mitral regurgitation. There was no
ventricular septal defect present. There was excellent left ventricular
f unction.
Ascending aortogram revealed a supravalvular attenuation of the aortic
root with deformity of the aortic valve. There was slight catheter-
induced aortic regurgitation, The coronary arteries appeared normal
without any focal narrowings. There was attenuation of the aortic
isthmus distal to the left subclavian artery which represented the point
of coarctation. There was no ductus arteriosus present. There were
small collateral present.
Descending aortogram revealed no obvious renal artery stenosis. The
kidneys were normal in size and appeared to function normally from a
rad iographic standpoint.
Right ventricular angiogram revealed severe right ventricular
hypertrophy. There was slight catheter-induced tricuspid regurgitation.
The pulmonic valve appeared normal. There was severe main branch
narrowing of both the right and left pulmonary arteries with severe distal
peripheral pulmonary artery stenoses throughout the lung fields
appearing to be greater on the left than on the right. The pulmonary
venous return was normal. There was a patent foramen ovale present.
The left atrium was dilated.
KUB was normal.
Y20
1I LANCELOT, HOLTON
105 UMC #0759081-4 T6/ 55 B0 DOB: 914192
16 MARCH 8, 1994
10l2
*67 145
20
10s
ss m
*
= Saturations
ARTERIAL BLOOD GAS:
Hgb: 13.1 Weight: 1 1.2 kgs.
Resp: 24 pH 7.40 Hct: 37.8 Height: 83.0 cm.
Spontan. pCO2 31 HR: 160 BSA: 0.52 M2
FlA2: 21o/” pO2 1 19
SAT. 97 %
CALCULATIONS:
OXYGEN CAPACIW: 176 ml/Liter
OXYGEN CONSUMPTION: 182 ml/min/m2(measured)
PULM FLOW: 3.1 L/min/m2 SYST FLOW: 3.1 L/min/M’z
EFF PULM FLOW: 3.1 Llminlm2
PULM RESIST: 25,8 mmHg/L/minlm’ ARTERIOLAR: 22.3 mmHg/L/min/m2
SYST RESIST: 30.6 mmHg/L/minlm’ ARTERIOLAR: 28.0 mmHg/L/minlM2
L -> R SHUNT: 0 L/min/m2
R -> L SHUNT: 0 L/min/M’z
QP / QS: 1.0 RP/RS: 0.84 PP/PS: 0.84
HOLTON, LANCELOT
uMc #0759081-4
CATH DATE: 314194
PAGE FOUR
vt. IMPRESSION S:
Anatomic Diagnoses:
l. Severe peripheral pulmonary artery stenosis.
2. Supravalvular aortic stenosis, mild.
3. Coarctation of the aorta.
4. Patent foramen ovale.
5. Biventricularhypertrophy.
6. Labile systemic hypertension.
Hemodynamic Diagnoses:
1. No intracardiac shunts.
2. Marked pulmonary hypertension secondary to peripheral artery stenoses.
3. Systemic hypertension, labile.
Electrophysiologic Dia gnoses :
Transient second degree AV Flock and supraventricular tachycardia
2. Basically normal sinus rhythm.
3. No conduction delay other than transient episodes mentioned above.
vil. DISCUSSION:
The patient was presented at the Combined Pediatric Cardiology and
Cardiothoracic Surgery Conference. lt was the impression of allthat surgical
approach with not be appropriate at this time. lt was felt that this patient’s
severe and unusual anatomy should be discussed with Dr. Lock at Boston
Children’s Hospital for interventional catheterization. Following those
discussiorrs with Dr. Locl< who agreed to treat the patient.
William J. lVldrvin, Jr./ MD
Division of Pediatric Cardiology
University of Florida Health Science Center/Jax

God
knew my limits. He knew Dr. Freeman’s and Dr. Marvin’s gifts. He worked it out,
so that two of the greatest Doctors in World were there when I was not
sufficient.

Lancelot’s Diet
O’s & M’s
Cheerios
4 to 5 bowels a day
(Served
dry with a few plain M&M’s mixed in with cereal)

Coke
Whenever
he is thirsty
Coke Ices
1 or 2 a week
Chicken
Mama’s
Fried Chicken Legs
Mama’s
Hawaiian Chicken
Quail
Turkey
Legs
Baked Ham
McDonald’s
Hamburger
Happy Meal, plain, fries, and Coke
No.
7Grilled chicken  plain, Fries, and of
course Coke to drink
Every
Friday
Pizza
Night
Little
Caesars- Hot-n-Ready Pepperoni- Scrap off all toppings and eat crust
Domino’s-
Supreme- Scrap off everything, and eat crust.
Out of
the blue he may eat
Fruit
Loops
Cinnamon
Toast Crunch
Low fat
Chip Ahoy Cookies and Doritos’s-  He
may eat one of these everyday for weeks or months then that’s it until the
mood strikes him again. I do not know why, but he will not again until the
mood strikes.
Strawberry
Ice Cream (I can not remember the last time!)
He cannot
take vitamins; because the smell of them makes him gag… actually the smell
of most foods make him gag, along with the smell of about everything. Looking
at this diet, I nominate myself for the world’s worst mother.
(Lancelot
has never had a vitamin deficiency.)

!