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The infant with single ventricle and excessive pulmonary blood flow: results of a
strategy of pulmonary artery division and shunt
Scott M. Bradley, Janet M. Simsic, Andrew M. Atz and B. Hugh Dorman
Ann Thorac Surg 2002;74:805-810
The online version of this article, along with updated information and services, is located
on the World Wide Web at:
http://ats.ctsnetjournals.org/cgi/content/full/74/3/805
The Annals of Thoracic Surgery is the official journal of The Society of Thoracic Surgeons and the
Southern Thoracic Surgical Association. Copyright 2002 by The Society of Thoracic Surgeons.
Print ISSN: 0003-4975; eISSN: 1552-6259.
0003-4975/02/$22.00
PII S0003-4975(02)03836-5
806
BRADLEY ET AL
PULMONARY ARTERY DIVISION AND SHUNT
Table 1. Patients
Patient
Age
(days)
Diagnosis
Initial Palliation
PA division, shunt
PA division, shunt
PA division, shunt
PA ligation, shunt
PA division, shunt
PA ligation, shunt
DKS
DKS
DKS
Mod Norwood
Mod Norwood
1
2
3
4
5
6
7
8
9
2
22
65
105
140
180
22
41
210
10
11
12
13
14
15
16
17
18
19
20
4
6
7
8
8
8
9
13
13
13
30
21
22
60
90
Mod
Mod
Mod
Mod
Mod
Mod
Mod
Mod
Mod
Mod
Mod
Norwood
Norwood
Norwood
Norwood
Norwood
Norwood
Norwood
Norwood
Norwood
Norwood
Norwood
Associated Procedures
Fontan Stage
Repair TAPVC
Atrial sept
Atrial sept
BSCC
Fontan
BSCC
Atrial sept
Repair TAPVC
BSCC
BSCC
BSCC
Fontan
Fontan
Atrial sept
Atrial sept
Atrial
Atrial
Atrial
Atrial
sept
sept
sept
sept
Atrial sept
Fontan
BSCC
BSCC
BSCC
BSCC
Fontan
Fontan
BSCC
BSCC
Died 1 y after BSCC
BSCC
BSCC
BSCC
Surgical Techniques
All patients underwent interruption of flow through the
pulmonary artery and placement of a systemic-topulmonary artery shunt. The proximal pulmonary artery
was handled by three different approaches, depending
on the potential for systemic outflow tract obstruction. (1)
Six patients were judged to have no potential for development of systemic outflow obstruction. In these 6 patients, the proximal pulmonary artery was divided and
oversewn (4 patients), ligated (1 patient), or ligated by
progressive constriction of an adjustable pulmonary ar-
BRADLEY ET AL
PULMONARY ARTERY DIVISION AND SHUNT
807
Results
Early Outcomes
Fig 1. Actuarial survival in 22 patients with a functional single ventricle and excessive pulmonary flow.
nitely. Associated procedures performed were atrial septectomy in 12 patients, repair of total anomalous pulmonary venous connection in 2, and resection of accessory
atrioventricular valve tissue in 1 (Table 1). Cardiopulmonary bypass, cardioplegic myocardial arrest, and periods
of circulatory arrest were used in 21 of the 22 operations.
The average cardiopulmonary bypass time (mean SD)
was 164 52 minutes, cross-clamp time 46 17 minutes,
and circulatory arrest time 32 21 minutes.
Subsequent bidirectional superior cavopulmonary
connection (BSCC) consisted of a bidirectional Glenn
shunt in 16 patients, hemi-Fontan procedure in 4, and
Kawashima procedure in 1. Additional procedures at the
time of BSCC were connection of a left superior vena
cava to the left pulmonary artery (left bidirectional Glenn
shunt) in 5 patients, atrioventricular valve repair in 2,
reimplantation of an anomalously draining right upper
pulmonary vein in 1, and patch closure of an insufficient
left atrioventricular valve in 1. Completion Fontan procedures were fenestrated intra-atrial lateral tunnel connections in 4 patients, and extracardiac conduits in 2.
Data Collection/Follow-Up
All records were retrospectively reviewed. Cardiac catheterization was performed before each BSCC, and again
before each Fontan procedure. Subaortic stenosis or
aortic arch obstruction during follow-up were defined as
a gradient of 20 mmHg or greater by echocardiogram or
catheterization. Neoaortic insufficiency and atrioventricular valve regurgitation were qualitatively graded by
echocardiogram on a scale from 0 (none) to 4 (severe).
Ventricular function was qualitatively graded by echocardiogram and angiography. Actuarial survival curve
Fontan Pathway
Twenty-one of the 22 patients have undergone a subsequent BSCC at a median age of 8 months (range 4 to 15
months). Six patients have undergone a completion Fontan procedure at a median age of 29 months (range 23 to
44 months). There were no deaths or operative failures at
either stage.
Variable
Patients
Pulmonary Artery
Pressure (mmHg)
Transpulmonary
Gradient
(mmHg)
Pulmonary Vascular
Resistance (Wood units)
22
9
15 (10 32)
10 (6 17)
7 (4 18)
4 (3 4)
Pre-BSCC
Pre-Fontan
a
808
BRADLEY ET AL
PULMONARY ARTERY DIVISION AND SHUNT
Comment
Patients with a functional single ventricle and excessive
pulmonary blood flow continue to present a surgical
challenge. The traditional approach to such patients has
been a pulmonary artery band. Associated coarctation
has been treated by repair via a left thoracotomy. This
approach has the advantages of avoiding cardiopulmonary bypass and circulatory arrest. However, the early
results of this approach were not optimal, with significant
operative and late mortality, and poor candidacy for a
Fontan operation [1, 4 6, 13]. For example, in patients
with double-inlet ventricle, Franklin and colleagues
found that among 35 patients treated with a pulmonary
band, survival was 77% at 1 year and 45% at 5 years [5].
Suitability for a Fontan operation was 14 of 33 patients
(42%) [6]. Among 18 patients treated with a pulmonary
band and coarctation repair, survival was 44% at 1 year
and 22% at 5 years [5]. Fontan suitability was 1 of 12
patients (8%) [6].
The use of a pulmonary band in patients with a
functional single ventricle and excessive pulmonary flow
can be complicated by the occurrence of subaortic stenosis. Freedom and coworkers observed the development
of subaortic stenosis in 72% of patients with a univentricular heart treated with a pulmonary band [2]. Subaortic stenosis can result in ventricular hypertrophy, which
is a risk factor for a Fontan operation [9, 10]. The factors
leading to subaortic stenosis have been extensively and
expertly discussed [3, 8]. Morphologic factors include
origin of the aorta from an outlet chamber which is
supplied by a bulboventricular foramen or ventricular
septal defect. Matitiau and associates further refined this
observation by determining that a bulboventricular foramen size of less than 2 cm2/m2 is associated with the later
development of subaortic stenosis [14]. The presence of
aortic arch obstruction is also associated with both a
small bulboventricular foramen and the subsequent development of subaortic stenosis [14]. Several groups have
reported that subaortic stenosis developed in 100% of
BRADLEY ET AL
PULMONARY ARTERY DIVISION AND SHUNT
809
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surgery of the neonate and infant. Philadelphia: WB Saunders, 1994:25571.
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810
BRADLEY ET AL
PULMONARY ARTERY DIVISION AND SHUNT
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DISCUSSION
DR JOHN H. CALHOON (San Antonio, TX): Dr Bradley, what a
beautifully illustrated series and nice presentation. I have one
question: How do you decide what anatomic substrate should go
to each of your three operative strategies, whether you oversew
the pulmonary artery, perform a Damus-Kaye-Stansel, or utilize
a Norwood? What anatomic things do you see that have given
you the ability to predict which infants will do best with those
and to have avoided the problems of subaortic obstruction or
arch obstruction with a Damus-Kaye?
Thank you again for a nice series and this information.
DR BRADLEY: Thank you very much for your comments. We
basically start by looking at the aortic arch. Any patient who has
coarctation of the aorta, with or without hypoplasia of the aortic
arch, undergoes a modified Norwood procedure. In the remaining patients, we look at the internal anatomy of the heart. Any
patient who has origination of the aorta from an outlet chamber,
in other words, a chamber served not by an atrioventricular
valve, but instead by a bulboventricular foramen or a ventricular
septal defect, undergoes a Damus-Kaye-Stansel procedure. One
other patient in our series, with double-outlet right-ventricle
and mitral atresia, had subaortic conal tissue that was judged to
be potentially restrictive, and also underwent a Damus-KayeStansel procedure. The remaining patients have been shown in
a number of series to be at low risk for the development of
subaortic stenosis, and they undergo simple division of the main
pulmonary artery and shunt placement.
DR ROSS M. UNGERLEIDER (Portland, OR): Scott, thanks for
sharing that. I think you have presented three distinctly different
groups of patients, and I would have no argument personally
with the strategy you have employed for the groups that need
either aortic arch reconstruction with a modified Norwood or a
Damus-Kaye-Stansel for the prevention of subsequent subaortic
obstruction.
The infant with single ventricle and excessive pulmonary blood flow: results of a
strategy of pulmonary artery division and shunt
Scott M. Bradley, Janet M. Simsic, Andrew M. Atz and B. Hugh Dorman
Ann Thorac Surg 2002;74:805-810
Updated Information
& Services
References
This article cites 28 articles, 18 of which you can access for free at:
http://ats.ctsnetjournals.org/cgi/content/full/74/3/805#BIBL
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