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...
him to be alive. He should be having major strokes, organ failure, and he should not be able to walk or talk.
Christ toward God.
ourselves to think of
ourselves, but our
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 Holton, whom I had the pleasure of seeing at the Pediatric Cardiovascular Center today
with his mother.
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
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
Patient Name: LANCELOT A. HOLTON
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.
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
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.
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.
Patient Name: LANCELOT A. HOLTON
Associate Professor of pediafics
210 South Wheeler Avenue
CC: ASAD TOLAYMAT, MD
CC: BzuDGET FREEMAN, MD
CC: JAMES E. LOCK, MD
Eleckonically signed by:william Marvin-Jr. M.D. Jan t4 2oto 3:35pM EST
STENOSIS; COARCTATION OF THE
STENOSIS; PATENT FORAMEN OVALE;
BIVENTRICULAR HYPERTROPHY; LABILE
HEART CATHETERIZATION WITH
SELECTIVE CI NEANGIOGRAPHY
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
CATH DATE: 314194
the Cardiac Catheterization Laboratory and prepped and draped in the usual
manner, Via the percutaneous technique, a #6 F wedge catheter was
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
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.
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
CATH DATE: 314194
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.
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
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.
105 UMC #0759081-4 T6/ 55 B0 DOB: 914192
16 MARCH 8, 1994
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
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
CATH DATE: 314194
2. Supravalvular aortic stenosis, mild.
3. Coarctation of the aorta.
4. Patent foramen ovale.
6. Labile systemic hypertension.
2. Marked pulmonary hypertension secondary to peripheral artery stenoses.
3. Systemic hypertension, labile.
Electrophysiologic Dia gnoses :
3. No conduction delay other than transient episodes mentioned above.
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.
Division of Pediatric Cardiology
University of Florida Health Science Center/Jax
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
O’s & M’s
4 to 5 bowels a day
dry with a few plain M&M’s mixed in with cereal)
he is thirsty
1 or 2 a week
Fried Chicken Legs
Happy Meal, plain, fries, and Coke
7Grilled chicken plain, Fries, and of
course Coke to drink
Caesars- Hot-n-Ready Pepperoni- Scrap off all toppings and eat crust
Supreme- Scrap off everything, and eat crust.
the blue he may eat
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
Ice Cream (I can not remember the last time!)
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.
has never had a vitamin deficiency.)