Clinical
Briefing: Multidisciplinary
Management of Adult
Congenital Heart Disease
May/June 2007
The Philadelphia Adult Congenital
Heart Center was founded
in 2005 as
a joint program between the University of Pennsylvania Health
System and the
Children's Hospital of Philadelphia. The Center's
primary mission is to provide
comprehensive, multidisciplinary care for the region's
rapidly growing population
of adults with congenital
heart disease (ACHD). To accomplish
its mission,
the Center has assembled a team of cardiovascular specialists
whose expertise
encompasses the full range of services necessary to provide
lifelong medical care
for patients with ACHD.
The spectrum of cardiovascular anomalies
that comprise congenital heart
disease includes cyanotic
defects, coarctation
of the aorta,
septal defects (atrial or ventricular), tetralogy
of Fallot, and a host of associated abnormalities of
ventricular and valvular
function. Although many of these defects are surgically corrected
in childhood,
successful repair does not confer a “normal” heart.
For many of these patients,
surgery can precipitate discreet secondary pathologies that
contribute to
cardiovascular deterioration in later life.
Thus, the Center
welcomes ACHD patients in need of revised surgeries,
as well as those with electrophysiological issues and other
conditions that may
further compromise their cardiovascular health. Moreover,
to reflect the spectrum
of coexisting needs in the ACHD population, the Center offers
family planning,
genetic counseling and obstetrical care.
“Many ACHD patients
believe that
they are cured, and are lost to the
cardiovascular health care system. It is
critically important to reach these
patients and to bring them into a
multidisciplinary setting where their
complex anatomical and physiological
needs can best be addressed.” *
– Gary D. Webb, MD
Director, The Philadelphia Adult Congenital Heart
Center
Hospital of the University of Pennsylvania
|
|
Case History
L. W., a 32-year-old man, was referred to the Philadelphia
Adult Congenital
Heart Center from the Penn Transplant Institute, where he
was evaluated for
heart/liver transplantation. Mr. W. had tricuspid
atresia palliated by a Bjork-style
RA-RV Fontan procedure at age five. His health was good until
age 30, when
he developed signs indicative of Fontan circulation failure,
including recurrent
tachyarrhythmia and occult cardiogenic cirrhosis.
After careful
evaluation,
surgeons at the Adult Congenital Heart Center concluded that
Mr. W would
benefit from Fontan revision and antiarrhythmic surgery.
A takedown of the
existing Fontan using a 24 mm synthetic extracardiac graft
and a biatrial maze
procedure were subsequently performed without complication.
Mr.
W.'s prospects for a healthy recovery are considered good.
In the months since his Fontan revision, his quality of life
has improved dramatically. He has
experienced a substantial enhancement in mobility, and has
returned to work.
Our Team of Faculty
The multidisciplinary team at the Philadelphia Adult Congenital
Heart Center
is led by Gary
D. Webb, MD, one of the world's leading
authorities in adult
congenital heart disease. To encompass the full array of
services necessary to
meet the demanding needs of patients with ACHD, the team
includes physicians
and surgeons from anesthesiology, cardiology, cardiovascular
surgery, cardiac
catheterization, electrophysiology, genetics, heart failure
and transplantation, imaging
services, pulmonary hypertension, and reproductive services.
Cardiology
Gary
D. Webb, MD, Director
Richard M. Donner, MD
Martin G. St. John Sutton, MBBS
Advanced Practitioners
Desiree Fleck, CRNP
Deborah Shellenberger, PA-C
Cardiovascular Surgery
Michael A. Acker, MD
Peter J. Gruber, MD, PhD
Alberto Pochettino, MD
Thomas L. Spray, MD
Cardiac Anesthesia
Jiri Horak, MD
David R. Jobes, MD
Susan Nicolson, MD
Joseph S. Savino, MD
Cardiac Catheterization
Matthew Gillespie, MD
Howard C. Herrmann, MD
John W. Hirshfeld, Jr., MD
Jonathan J. Rome, MD
Cardiac Electrophysiology
Joshua M. Cooper, MD
Edward P. Gerstenfeld, MD
Maully J. Shah, MBBS
Victoria Vetter, MD
Cardiac Imaging
Victor A. Ferrari, MD
Mark A. Fogel, MD
Susan E. Wiegers, MD
Cardiopulmonary Testing
Steven M. Paridon, MD
Paul Stephens, MD
Michael McBride, PhD
Heart Failure and Transplantation
Maryanne R.K. Chrisant, MD
Beth D. Kaufman, MD
Lee R. Goldberg, MD
Mariell L. Jessup, MD
Paul R. Forfia, MD
Genetic Counseling
Barbara Bernhardt, MS
Elizabeth Goldmuntz, MD
Paige B. Kaplan, MD
Reed E. Pyeritz, MD
Maternal/Fetal Medicine
Michal A. Elovitz, MD
Emmanuelle Pare, MD
Pulmonary Hypertension
Harold I. Palevsky, MD
Darren B. Taichman, MD
Radiology/Scanning
Sridhar R. Charagundla, MD
Harold I. Litt, MD
PACHC Research Program
Jonathan A. Epstein, MD
Peter J. Gruber, MD, PhD
Michael S. Parmacek, MD
Access
Patient appointments are available at
Hospital of the University of
Pennsylvania
6 Penn Tower
3400 Spruce Street
Philadelphia, PA 19104
The Children's Hospital of Philadelphia
Wood Building, 6th Floor
34th Street and Civic Center Boulevard
Philadelphia, PA 19104
To refer a patient and/or consult with a doctor:
|
|
Referring Physicians: To speak with a Penn physician
or refer a patient, contact PennHealth through the secure online
referral form or by calling
1-800-789-PENN
(7366). |
 |
|
|
*Reprinted from Cardiology Clinics, Volume
24, Guest Editor Gary D. Webb, MD, Consulting Editor Michael
H. Crawford, MD, Adult Congenital Heart Disease (515-667).
Copyright (2006), with permission from Elsevier Inc. |
