176 ª 2022 by The Society of Thoracic Surgeons 0003-4975/$36.

00
Published by Elsevier Inc. https://doi.org/10.1016/j.athoracsur.2021.04.058

One and One-Half Ventricle Repair: Role for

Restricting Antegrade Pulmonary Blood Flow
CONGENITAL HEART

Anagha Prasanna, AB, Corinne W. Tan, MD, Alexandra Anastasopulos, MD,

Rebecca S. Beroukhim, MD, and Sitaram M. Emani, MD

Harvard Medical School, Boston, Massachusetts; Department of Cardiovascular Surgery, Boston Children’s Hospital,
Harvard Medical School, Boston, Massachusetts; SIMPeds, Boston Children’s Hospital, Boston, Massachusetts;
Department of Global Health and Population, Harvard T.H. Chan School of Public Health, Boston, Massachusetts; and
Department of Cardiology, Boston Children’s Hospital, Boston, Massachusetts

ABSTRACT

BACKGROUND In patients with hypoplastic subpulmonary ventricles, the one and one-half ventricle (1.5V) repair is an
alternative to the Fontan procedure. However, in 1.5V-treated patients with pulsatile pulmonary blood flow, superior
vena cava (SVC) hypertension or right atrial hypertension may develop. This study aimed to (1) describe patient out-
comes after 1.5V repair and (2) determine whether pulmonary artery septation at 1.5V repair confers a lower risk of SVC
or right atrial hypertension.

METHODS This study retrospectively reviewed patients who underwent a 1.5V repair between 1989 and 2020. The
primary outcome was transplant-free survival. Secondary outcomes were postoperative SVC hypertension (defined by
mean Glenn pressures greater than 17 mm Hg, SVC flow reversal or pulsatility, venovenous collateral vessels, or SVC
syndrome) and right atrial hypertension (defined as mean right atrial pressures greater than 10 mm Hg with inferior vena
cava and hepatic vein dilation or flow reversal).

RESULTS A total of 74 patients underwent 1.5V repair at a median age of 29.6 months (interquartile range, 8.9 to 45.5
months). Median follow-up time was 39.9 months (interquartile range, 11.4 to 178.1 months). Transplant-free survival at
10 years was 92.4%. Among survivors, 12% (8 of 69) had right atrial hypertension and 39% (27 of 69) had SVC hy-
pertension on follow-up. Survivors with unseptated pulmonary arteries had a greater risk of SVC hypertension compared
with patients with septated pulmonary arteries (44% vs 10%; P [ .04). No difference was found in right atrial hyper-
tension between the 2 groups.

CONCLUSIONS Patients with 1.5V repair avoid Fontan-associated complications with favorable transplant-free sur-
vival. However, SVC hypertension remains a significant long-term complication. Pulmonary artery septation at 1.5V
repair may reduce the risk of SVC hypertension.
(Ann Thorac Surg 2022;114:176-83)
ª 2022 by The Society of Thoracic Surgeons

I n patients with hypoplastic or dysfunctional sub-

pulmonary ventricles, the one and one-half
ventricle (1.5V) repair is an alternative to Fontan
palliation. Although blood flow enters the pulmonary
successfully converted into the
ventricle in patients after failed Fontan palliation.2
Although pulmonary atresia with intact ventricular
septum and Ebstein anomaly are the most common in-
subpulmonary

circulation passively through a superior cavopulmo-
nary anastomosis (Glenn), inferior vena cava (IVC)
inflow is actively pumped by the subpulmonary
ventricle to provide pulsatile antegrade pulmonary
1
blood flow (APBF). The right ventricle (RV) has tradi-
tionally served as the “one-half” ventricle in 1.5V circu-
lation; however, recently the left ventricle has been
dications for 1.5V repair, this approach has been safely
demonstrated in patients with more complex cardiac
anatomy, including unbalanced complete atrioventric-
ular canal and double-outlet right ventricle.3-5 Previous
The Video can be viewed in the online version of this article [10.1016/j.
athoracsur.2021.04.058] on http://www.annalsthoracicsurgery.org.

Accepted for publication Apr 14, 2021.

Presented at the Fifty-seventh Annual Meeting of The Society of Thoracic Surgeons, Virtual Meeting, Jan 29-31, 2021.
Address correspondence to Dr Emani, Department of Cardiovascular Surgery, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA, 02115; email:
sitaram.emani@cardio.chboston.org.
Ann Thorac Surg PRASANNA ET AL 177
2022;114:176-83 ROLE FOR PULMONARY ARTERY SEPTATION

dilation or flow reversal. Time to SVC or RA

Abbreviations and Acronyms
hypertension was defined as the time between

APBF [ antegrade pulmonary blood flow
AVM [ arteriovenous malformation
BDG [ bidirectional Glenn
IQR [ interquartile range
IVC [ inferior vena cava
PA [ pulmonary artery
RA [ right atrial
RV [ right ventricle
SVC [ superior vena cava
VVC [ venovenous collateral vessel
1.5V [ one-and-one-half ventricle
literature on outcomes in patients who underwent 1.5V
repair reported 10-year transplant-free
ranging from mid-70% to 90%.6-8
Patients with pulsatile APBF may be at risk for supe-
rior vena cava (SVC) hypertension, SVC aneurysms,
pleural effusions, and chylothorax.9,10 With an inade-
quate subpulmonary ventricle, there is risk for right
atrial (RA) hypertension, arrhythmias, and hepatic
congestion. Restricting APBF between the SVC and APBF
insertion sites by either partial or complete separation in
1.5V circulation (PA septation) may reduce SVC pulsa-
tility and thereby prevent SVC hypertension. The pur-
pose of this study was to describe a single-institution
experience with patients who underwent 1.5V repair and
initial experience with PA septation.
PATIENTS AND METHODS
STUDY DESIGN. This was a retrospective review of pa-
tients followed at Boston Children’s Hospital (Boston,
MA) after 1.5V repair. The study was approved by the
Boston Children’s Hospital Institutional Review Board,
and informed consent was waived. Hospital records of
patients with a 1.5V repair diagnostic code were
reviewed for demographic data, diagnosis, previous
surgical interventions, 1.5V repair details, and post-
operative outcomes. Primary outcomes were death and
cardiac transplantation. Secondary outcomes included
complications such as complete heart block, need for
extracorporeal membrane oxygenation support, perma-
nent pacemaker implantation, renal failure, and long-
term outcomes such as reoperation, SVC hypertension,
RA hypertension, atrial arrhythmias, and vascular
collateral formation. SVC hypertension
composite end point defined by mean Glenn pressures
greater than 17 mm Hg by catheterization, presence of
SVC flow reversal or pulsatility, large decompressing
venovenous collateral vessels (VVCs), or evidence of
SVC syndrome (head and arm swelling, headaches, and
chest wall venous distention). RA hypertension was
defined as mean RA pressures greater than 10 mm Hg
by catheterization with either IVC and hepatic vein
CONGENITAL HEART
completion of 1.5V repair and the first evidence of SVC
or RA hypertension on follow-up. Salvage 1.5V repair
was defined as surgical intervention within 30 days of
the last known operation. Clinical follow-up was
calculated as the time between 1.5V repair and the
latest recorded clinical encounter in the electronic
medical record.
INCLUSION CRITERIA. Patients included in this study
underwent 1.5V repair between August 1989 and
February 2020. The 1.5V repair was defined by a superior
cavopulmonary anastomosis, atrial and ventricular sep-
survival tation, preservation of APBF, and takedown of systemic-
to-pulmonary shunts. Date of 1.5V repair was
determined as the date of APBF establishment or, in
patients with a ventricular septal defect, ventricular
septation. A subset of patients had bilateral SVCs
without a bridging vein and only 1 superior
cavopulmonary anastomosis. Atrial fenestration was
present in some patients to reduce sequelae of RA
hypertension.
PULMONARY ARTERY SEPTATION PROCEDURE. Pulmonary
artery septation (PA septation) was defined as restriction
of pulmonary artery externally (PA band), intraluminally
(fenestrated membrane), or by division, or preservation
of native pulmonary artery stenosis between the inser-
tion sites of the Glenn procedure and the source of APBF
(Figure 1, Video 1). All patients undergoing 1.5V repair
were considered candidates for PA septation. No standard
criteria were used to determine the need for PA septation,
and this was left to the surgeon’s discretion, with 1 of the
authors (S.M.E.) predominantly using this technique.
Pulmonary artery septation was performed through a
median sternotomy with cardiopulmonary bypass and
moderate hypothermia (28 to 32
C). After opening the
central PA anteriorly, a bovine pericardial patch was sewn
in place between the Glenn anastomosis and the contra-
lateral branch PA receiving APBF. For a fenestrated
membrane, a 2- to 3-mm fenestration was created within
the central portion of the patch to allow communication
between the branch PAs while protecting the Glenn
from excessive pulsatile pulmonary blood flow.
Alternatively, PA banding was performed by externally
restricting the central PA between the Glenn and APBF
with a heavy 2-0 nonabsorbable suture tie over a 3-mm
was a
Hegar dilator.
ECHOCARDIOGRAPHY, CARDIAC MAGNETIC RESONANCE
IMAGING, CARDIAC COMPUTED TOMOGRAPHY, AND
CARDIAC CATHETERIZATION DATA. Preoperative data
were collected from the most recent study before 1.5V
repair. Postoperative data were collected from the most
recent follow-up studies in patients who remained with
1.5V circulation or the most recent study before death,
 178 PRASANNA ET AL Ann Thorac Surg
ROLE FOR PULMONARY ARTERY SEPTATION 2022;114:176-83

transplantation, or biventricular conversion. Imaging data

included atrioventricular valve dimensions and Z-score,
CONGENITAL HEART

atrioventricular valve regurgitation and stenosis,

pulmonary valve regurgitation and stenosis, subpulmo-
nary end-diastolic volume, biventricular function, and
ejection fractions. Catheterization data included SVC or
Glenn pressures, RA pressures, subpulmonic end
diastolic pressure, and evidence of ABPF.

STATISTICAL ANALYSIS. Statistical analysis was per-

formed using R Studio version 1.3.959 software (R
version 4.0.2, R Foundation, Vienna, Austria). Categoric
variables were reported as numbers and percentages.
Continuous variable were reported as median and
interquartile range (IQR). Comparison of median values
FIGURE 1 Magnetic resonance imaging demonstrating pulmonary artery was performed by the Mood median test. Kaplan-Meier
septation (arrow) between bidirectional Glenn (A) and the left pulmonary artery
methodology was used to estimate transplant-free
(B).
survival and distribution of time to adverse events.
The log-rank test was used to compare end points
between septated and unseptated PA cohorts and to
identify variables associated with adverse events. P
values <.05 were considered significant.

RESULTS
TABLE 1 Characteristics of Patients Undergoing One
and One-half Ventricle Repair (n ¼ 74) PREOPERATIVE PATIENT CHARACTERISTICS. Between
August 1989 and February 2020, 74 patients underwent
Variable Value
1.5V repair at a median age of 29.6 months (IQR, 8.9 to
Age at 1.5V repair, mo 29.6 (8.9-45.5)
Sex
45.5 months). Pulmonary atresia with intact ventricular
Male 38 (51) septum was the most common anatomic diagnosis
Female 36 (49) among patients (31 of 74; 42%). A total of 72 patients
Weight at 1.5V repair, kg 11.7 (8.6-15.5) (97%) had the RV as the subpulmonary ventricle. Addi-
Diagnoses tional characteristics are described in Table 1. A total of
Pulmonary atresia with IVS 31 (42)
73 patients (99%) underwent previous surgical in-
DORV 13 (18)
terventions, including modified Blalock-Taussig shunt
Unbalanced CAVC 12 (16)
(38 of 74; 51%), bidirectional Glenn (BDG) (29 of
Ebstein anomaly 4 (5)
D-TGA 4 (5) 74; 39%), right ventricular outflow tract patch (18
L-TGA 4 (5) of 74; 24%), PA band (10 of 74; 14%), central shunt (6
Tetralogy of Fallot 3 (4) of 74; 8%), RV-PA conduit (6/74, 8%), and Fontan
HLHS 3 (4) repair (4 of 74; 5%). Two patients (3%) underwent 1.5V
DILV 3 (4) repair with the left ventricle as the subpulmonary
Shone complex 1 (1)
ventricle after Fontan circulation failed. On imaging,
Critical pulmonary stenosis 1 (1)
46% (34 of 74) of patients had mild, 12% (9 of 74) had
Genetic conditions
Heterotaxy 14 (19)
moderate, and 4% (3 of 74) had severe subpulmonary
Trisomy 21 1 (1) atrioventricular valve regurgitation. Median subpulmo-
Other 2 (3) nary atrioventricular valve size was 1.14 cm (IQR, 0.79
Subpulmonary ventricle to 1.78 cm) (n ¼ 38), and median subpulmonary
Right ventricle 72 (97) atrioventricular valve Z-score was 2.50 (IQR, 3.41
Left ventricle 2 (3)
to 1.30) (n ¼ 30). Median subpulmonary ventricle size
Bilateral superior vena cava 7 (10)
was 48.45 mL/m2 (IQR, 38.23 to 71.05 mL/m2) (n ¼ 30).
Outside hospital 9 (12)
Median SVC pressure was 12 mm Hg (IQR, 9.25 to 15 mm
Values are median (IQR) or n (%). CAVC, complete atrioventricular canal; DILV, Hg) (n ¼ 50). Median subpulmonary atrial pressure was
double-inlet left ventricle; D-TGA, D-transposition of the great arteries; DORV,
8 mm Hg (IQR, 7 to 11 mm Hg) (n ¼ 49).
double-outlet right ventricle; HLHS, hypoplastic left heart syndrome; IQR,
interquartile range; IVS, intact ventricular septum; L-TGA, L-transposition of the
great arteries; 1.5V, one and one-half ventricle. SURGICAL PROCEDURES. A total of 69 patients (93%)
underwent additional procedures other than BDG at the
Ann Thorac Surg PRASANNA ET AL 179
2022;114:176-83 ROLE FOR PULMONARY ARTERY SEPTATION

TABLE 2 Procedural Details of One and One-half

the conduction system, considering that all but 1
Ventricle Repair (n ¼ 74) patient underwent closure of a ventricular septal

CONGENITAL HEART
defect. Eight patients (11%) had permanent complete
Procedural Details Value
heart block and received permanent pacemakers. Renal
Concomitant procedures
failure was observed in all 5 deaths.
ASD fenestration 30 (41)
Among 69 survivors, 81% (n ¼ 56) maintained 1.5V
VSD closure 21 (28)
RV-PA conduit 19 (26) circulation on follow-up, 17% (n ¼ 12) underwent BDG
TV repair or replacement 19 (26) takedown, and 1.4% (n ¼ 1) underwent transplantation.
RVOT patch 11 (15) A total of 41 survivors (59%) underwent reoperation,
RV myectomy 8 (11) with the most common procedure being BDG takedown
LV-Ao baffle 8 (11) to biventricular circulation at a median time of 122
ASD closure 7 (9)
months (IQR, 53 to 186 months) from 1.5V repair
IVC-RA baffle 4 (5)
(Figure 3). Median Glenn pressure among survivors was
Maze procedure 4 (5)
PA band 3 (4)
15.5 mm Hg (IQR, 13 to 19.25 mm Hg) (n ¼ 37). The pa-
PV repair or replacement 3 (4) tient who received a transplant was born with an un-
Pacemaker 3 (4) balanced complete atrioventricular canal defect and a
IVC-RV baffle 2 (3) hypoplastic RV. Given progressive biventricular failure,
LV-PA conduit 2 (3) this patient underwent cardiac transplantation 11 years
MV repair or replacement 2 (3) after 1.5V repair. Thirteen survivors had a fenestrated
Atrial baffle 1 (1)
atrial septum on follow-up, with right-to-left flow
PFO closure 1 (1)
documented in 7 patients, left-to-right flow in 3 patients,
Intraoperative times, min
Cross-clamp time 82 (44-149.5)
and bidirectional flow in 3 patients.
Cardiopulmonary bypass time 170 (113-240) On follow-up, 27 survivors (39%) had new develop-
Hospital course, d ment of SVC hypertension (Figure 4A, Table 3). The most
ICU LOS 5 (2.5-9) common findings related to SVC hypertension included
Hospital LOS 10 (7-17.25) SVC flow reversal in 14 patients, elevated Glenn pres-
Mechanical ventilation duration 2 (1-5)
sures in 11 patients, and development of large decom-
Values are n (%) or median (IQR). Ao, aorta; ASD, atrial septal defect; ICU,
pressing VVCs in 10 patients. Five patients underwent
intensive care unit; IQR, interquartile range; IVC, inferior vena cava; LOS, length BDG takedown for SVC hypertension, and 3 had resolu-
of stay; LV, left ventricle; MV, mitral valve; PA, pulmonary artery; PFO, patent
foramen ovale; PV, pulmonary valve; RA, right atrium; RV, right ventricle; RVOT, tion of SVC hypertension postoperatively.
right ventricular outflow tract; TV, tricuspid valve; VSD, ventricular septal defect. RA hypertension was noted in 8 survivors (12%) on
follow-up (Table 3). One patient received a cardiac
transplant as described earlier, and 1 patient underwent
time of 1.5V repair (Table 2). Ten patients (14%) under- a biventricular repair. Three patients who had RA hy-
went PA septation with 1.5V repair. Nineteen (26%) pa- pertension development had documented atrial fenes-
tients underwent 1.5V repairs as salvage operations for trations, and on follow-up, 1 patient remained with a
desaturation or subpulmonary ventricular failure in the fenestrated atrial septum with right-to-left flow. Three
setting of biventricular circulation. patients had venous liver congestion noted on ultra-
sound, and 1 patient had portal hypertension.
POSTOPERATIVE OUTCOMES. Median follow-up time was There was no difference in transplant-free survival
39.9 months (IQR, 11.4 to 178.1 months). Transplant-free between septated PA and unseptated PA cohorts (1% vs
survival at 10 years was 92.4% (95% confidence interval, 8%; P ¼0.8); however, risk for SVC hypertension was
86.0% to 99.0%), with 5 early deaths (Figure 2). Four higher in patients with unseptated PAs (10% vs 44%;
deaths occurred in patients who underwent 1.5V repair P ¼ .04) (Figure 4B). There were no differences in pre-
as a salvage procedure, were undergoing operative SVC or Glenn pressures between septated and
extracorporeal membrane oxygenation postoperatively unseptated PA groups (P ¼ .23). Unseptated patients
with multiorgan system failure, and ultimately had were at a greater risk for reoperation (30% vs 64%;
life-sustaining treatment withheld after it was deemed P ¼ .03). No differences were found in RA hypertension
futile. One death occurred after discharge, and the between the 2 cohorts (P ¼ .4) (Table 3).
cause of death was not available. Patient-related and Indications for Glenn takedown included failure of
operative characteristics associated with early mortality 1.5V circulation secondary to circular shunt, SVC hy-
by log-rank analysis included heterotaxy diagnoses pertension, or exercise intolerance in 7 patients. Other
(P < .001) and salvage operations (P ¼ .01). Twelve patients demonstrated adequate growth of the RV for
patients (16%) had complete heart block immediately biventricular conversion. On follow-up, 3 patients had
postoperatively, likely because of iatrogenic damage to decreased exercise tolerance, 3 patients had improved
 180 PRASANNA ET AL Ann Thorac Surg
ROLE FOR PULMONARY ARTERY SEPTATION 2022;114:176-83
CONGENITAL HEART

FIGURE 2 Kaplan-Meier curve of transplant-free survival in 74 patients who underwent one-and-one half ventricle repair.
(CI, confidence interval.)

exercise tolerance, and 1 patient had recurrent symp- hypertension. The modified 1.5V repair with PA septa-
tomatic SVC occlusion. tion was associated with a lower risk of SVC hyperten-
sion compared with traditional 1.5V repair.

COMMENT
This study describes outcomes in patients who under-
went 1.5V repair and had a focus on adverse events
related to SVC and RA hypertension, as well as a po-
tential role for PA septation in avoiding SVC-related
complications. We describe our experience with a
modification to the 1.5V repair involving restriction of
the PA between the BDG and APBF by use of a fenes-
trated membrane with the goal of avoiding SVC
The 1.5V repair has advantages over the Fontan pro-
cedure through the preservation of pulsatile APBF,
which provides hepatic venous blood flow to both lungs,
maintains a low-pressure circulation in the IVC, and
minimizes pulmonary hypertension.11,12 At a median
follow-up time of 39.9 months, our cohort of 1.5V-
treated patients did not have the postoperative protein-
losing enteropathy, chylothorax, or plastic bronchitis
seen in Fontan-treated patients.6,13-18 The 1.5V repair can
be a successful salvage procedure, as seen in our

FIGURE 3 Kaplan-Meier curve of freedom from reoperation among 69 survivors. (CI, confidence interval.)
Ann Thorac Surg
2022;114:176-83
FIGURE 4 Kaplan-Meier curve of (A) freedom from superior vena cava hypertension (SVC HTN) among 69 survivors and (B)
freedom from superior vena cava hypertension in patients with a septated vs unseptated pulmonary artery (PA) (P [ .04).
(CI, confidence interval.)
patients with failed Fontan circulation.2,19 Among
limited criteria for 1.5V candidacy, atrioventricular valve
size and subpulmonary ventricle volume and morpho-
logic features may influence the pathway for surgical
palliation.6 One study reported a tricuspid valve Z-score
between 1.5 and 4.8 and a bipartite RV appropriate
for 1.5V repair.6,16
SVC hypertension was a notable outcome in 1.5V pa-
tients. Current literature has focused on serious com-
plications such as facial swelling and SVC aneurysm.9
Although elevated Glenn pressures do not pose
an immediate risk to patients, longstanding SVC
hypertension can progress to more clinically significant
presentations. For some patients, SVC hypertension
resolved after BDG takedown to biventricular circula-
tion. As in Fontan-treated patients, RA hypertension
with IVC or hepatic venous congestion remains a
PRASANNA ET AL 181
ROLE FOR PULMONARY ARTERY SEPTATION
CONGENITAL HEART
concern in 1.5V patients.13,14 Further investigation is
necessary to see whether long-term 1.5V circulation can
prevent Fontan-related liver complications.
PA division and banding have been advocated to
prevent excessive SVC pulsatility and aneurysm; how-
ever, few investigators have reported its use since the
initial publications.17,20,21 PA banding has been used in
patients with super-Glenn physiology (BDG along with
an aortopulmonary shunt and central pulmonary artery
banding to augment pulmonary blood flow) where SVC
hypertension was a recognized complication.22 Addition
of PA septation during super-Glenn procedures has
reduced the incidence of SVC hypertension, thus
prompting the use of PA septation in 1.5V repair.22 Spe-
cifically, use of the fenestrated membrane began in 2018.
Currently, almost all patients who undergo 1.5V repair
also undergo PA septation.
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TABLE 3 Long-term Outcomes in Survivors of One and One-half Ventricle

volume loading, and PA septation may reduce the pul-
Repair (n ¼ 69) monary regurgitant fraction by limiting flow from SVC to
CONGENITAL HEART

RV during diastole.
Survivors With Survivors With
Outcomes All Survivors Septated PA Unseptated PA P
VVC formation is another concern in patients under-
Total number of patients 69 10 59 .
going 1.5V repair .24 Previously reported rates of VVC
Median follow-up time, mo 39.9 (11.4- 66.9 (14.3- 38.8 (10.1- . formation in these patients range from 0% to 33%.6,20,25-
178.1) 320.8) 157.6) 27
None of our cohort with septated PA had VVC devel-
Clinical outcomes
opment, a finding supporting our hypothesis that PA
Reoperation 41 (59) 3 (30) 38 (64) .03a
septation reduces the risk of elevated Glenn pressure
SVC hypertension 27 (39) 1 (10) 26 (44) .04a
and its sequalae. Over time, PA septation may increase
SVC flow reversal 14 (20) 0 (0) 14 (24) .
Mean Glenn pressure >17 mm 11 (16) 1 (10) 10 (17) . the risk for AVM formation secondary to reduced IVC
Hg flow to the lung perfused by the SVC; however, none of
VVC formation 10 (14) 0 (0) 10 (17) . our patients who had a septated PA had documented
Glenn pulsatility 5 (7) 0 (0) 5 (8) .
postoperative pulmonary AVMs at a median follow-up of
SVC syndrome 2 (3) 0 (0) 2 (3) .
66.9 months.28,29
Right atrial hypertension 8 (12) 2 (20) 6 (10) .4
This study has several limitations in addition to those
Atrial arrhythmias 4 (6) 1 (10) 3 (2) .9
Transplantation 1 (1) 1 (10) 0 (0) .05 inherent in a retrospective analysis. The study popula-
Arteriovenous malformations 1 (1) 0 (0) 1 (1) .7 tion was a heterogenous group with a variety of cardiac
anatomic diagnoses biased toward pulmonary atresia
a
P value for difference between patients with and without septation <.05 using 2-tailed log-rank test. Values
are n, median (IQR), or n (%). IQR, interquartile range; PA, pulmonary artery; SVC, superior vena cava; VVC,
with intact ventricular septum. Moreover, our septated
venovenous collateral vessel. PA subgroup was small. Longer-term follow-up is
necessary for a more complete understanding of the
complications of 1.5V physiology repair, such as AVM
formation. Not all patients had catherization data at
Here we describe outcomes in patients who under- most recent follow-up, and that limits our ability to
went a band, fenestrated membrane, or division pro- appreciate postoperative changes in hemodynamics.
cedure or had native stenosis preserved to restrict Further, asymptomatic patients with SVC or RA hyper-
pulsatile flow from the subpulmonary ventricle. One tension may be missed or delayed in diagnosis. A sig-
patient who underwent a PA band procedure had a loose nificant limitation of this study is the lack of a
or absent band on follow-up, thus raising concerns over propensity-matched cohort of Fontan-treated patients
its longevity.23 The fenestrated membrane in 1.5V- for comparing outcomes such as liver disease and SVC
treated patients may be associated with less PA distor- syndrome.
tion with more potential for subsequent transcatheter In conclusion, in patients with inadequate sub-
intervention. Compared with complete septation, this pulmonary ventricles, 1.5V repair is associated with
approach allows for a hepatic factor into both lungs to 92.4% transplant-free survival at 10 years. RA hyper-
prevent pulmonary arteriovenous malformation (AVM) tension and SVC hypertension remain significant long-
formation, but it does pose the risk of spontaneous term complications in patients undergoing 1.5V repair.
fenestration closure. Modification of the 1.5V repair with PA septation may
There are several potential benefits of PA septation in reduce the risk of this adverse outcome. However,
1.5V in addition to reduced risk of SVC hypertension. In longer-term studies are necessary to confirm the dura-
patients who have significant VVC development bility of this strategy and ensure the lack of other com-
decompressing the hypertensive SVC, retrograde flow in plications such as AVMs.
the SVC recirculates into the IVC. This circular shunt
volume loads the RV and created inefficient antegrade The authors wish to thank Breanna L. Piekarski (Boston Children’s Hospital)
pulmonary blood flow. PA septation reduces this risk. In for assistance in patient query and Dr. Kimberlee Gauvreau and Isabella
Nogues (Harvard T.H. Chan School of Public Health) for their advice on
patients with pulmonary and tricuspid regurgitation,
statistical analysis.
regurgitation of SVC flow into the RV may contribute to

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