surgery for hlhs with surgery for hlhs with coronary artery fistulas or ...

SURGERY FOR HLHS WITH
CORONARY ARTERY FISTULAS
OR SINUSOIDS
THOMAS L. SPRAY, M.D.
Chief, Cardiothoracic Surgery
Alice Langdon Warner Endowed Chair
The Children’s Hospital Of Philadelphia
Professor of Surgery
The University of Pennsylvania

CORONARY ARTERY
DEVELOPMENT
• Coronary arteries arise as solid angioplastic buds before
conotruncal partioning
• Extend through the epicardium to divide into usual
arrangement by 7 th week
• Smaller branches join capillary network already connected
to coronary veins and dCS and connected dto the LV cavitary
lumen
• Some coronary arteries connect to the trabecular spaces
and thereby to the lumen
• These natural ventriculocoronary connections shrink as the
outer compact myocardium develops

PATHOLOGY
• Congenital Obstruction Of LV Outflow
With Patent LV Inflow Associated With
Small Thick-Walled LV With EFE
• Sinusoids From Endocardial Surface May
Extend Into The Myocardium, And May
Connect To Coronary Veins, Arteries, Or
Capillaries

HLHS TYPES
• AA/MA
• AA/MS
• AA/MS/ Intact t Atrial Septum
• AA/MA/Intact Atrial Septum
•AS/MS
• AA/MS/VSD
• AA/VSD
• AS/MS/VSD

HLHS TYPES
• AA/MA
• AA/MS
• AA/MS/ Intact t Atrial Septum
• AA/MA/Intact Atrial Septum
•AS/MS
• AA/MS/VSD
• AA/VSD
• AS/MS/VSD

VENTRICULOCORONARY
CONNECTONS
• Arterioluminal : Connects LV directly to verticallypenetrating
branches of CAs (OM,LAD,PD –
regardless of dominance)
• Arteriosinusoidal: Connects LV chamber to small
ramifications of CAs, opening through primitive
sinusoids id with multiple l luminal l orifices extending to
the inner 2/3 of the myocardium behind the posterior
MV
• Arteriocapillary: Connects LV to network of
capillaries of CAs and also veins, mostly
subendocardial

VENTRICULOCORONARY CONNECTIONS
From: O’Connor et al. Circulation 1982;66:1078-86

VENTRICULOCORONARY CONNECTIONS
From: O’Connor et al. Circulation 1982;66:1078-86

VENTRICULOCORONARY
CONNECTIONS
• Present to some extent in all with AA/MS
• Epicardial and Intramural coronary branches have
intimal thickening in free wall and septum when
ventriculoarterial t i l connections present without t luminal
l
narrowing
• EFE present in all cases of AA/MS but not AA/MA
…less behind MV
• Myofiber disarray present in inner 2/3 of LV free
wall and septum (not RV or outer myocardium)
• Calcification and scarring in subendocardium of LV
From: O’Connor et al. Circulation 1982;66:1078-86

DOES IT MATTER?

SURGICAL RESULTS

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BOSTON DATA
From: Vida, VL et al. JTCVS 2008;135:339-46

The image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again.
BOSTON DATA
From: Vida, VL et al. JTCVS 2008;135:339-46

The image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again.
BOSTON DATA
From: Vida, VL et al. JTCVS 2008;135:339-46

BOSTON DATA
From: Vida, VL et al. JTCVS 2008;135:339-46

CHICAGO DATA
• 100 PTS. Echo dx of VCC
• 31 AA/MA; 42 AS/MS; 27 AA/MS (15
VCC -42%)
• 91% 30-day survival; 13% interstage
mortality; 72% 6-month survival (74%for
AA/MS)
• AA/MS with VCC 91% vs. 50% AA/MS
without VCC
From: Polimenakos, AC et al STS 2010

CHOP DATA
ANATOMY Freq. Percent Cum.
MA/AA 49 40.5 40.5
MS/AA 24 19.83 60.33
MS/AS 48 39.67 100
Total 121 100

CHOP DATA
ANATOMY Freq. Percent Cum.
MA/AA 49 40.5 40.5
MS/AA 24 19.83 60.33
MS/AS 48 39.67 100
Total 121 100

CHOP DATA

CHOP DATA

QUESTIONS
• Situation in HLHS is similar to that in PA/IVS
• Why would fistulae/sinusoids increase operative
risk? Decreased LV inflow would be same with
hybrid if ASD unrestrictive
• No difference in mortality with RV-PA so not
diastolic runoff
• Why is EFE present in LV in HLHS but not RV in
PA/IVS?
• Do the fistulae regress as in PA/IVS?
• ? Arrhythmias from LV hypertension, but
interstage mortality seems the same

CONCLUSION
• While there is intriguing goutcome data
from one center, other centers have not
identified AA/MS group as at higher
risk after Stage I
• Therefore at the present time it is hard
to justify an alternative surgical
approach for the AA/MS subgroup of
patients with HLHS