ObstructedPulmonaryVenousDrainage
with Total AnomalousPulmonaryVenous
Connectionto the CoronarySinus
RICHARD A. JONAS, MD, ARAM SMOLINSKY, MD, JOHN E. MAYER, MD,
and ALDO R. CASTANEDA, MD
Obstructed pulmonary venous drainage is generally
considered extremely rare with total anomalous pul-
monary venous connection (TAPVC) to the coro-
nary sinus (CS). A retrospective review of 27 oper-
ated patients with TAPVC to CS revealed 6 cases of
obstruction (22% ). Two of 6 patients who died ear-
ly had evidence of obstruction at the pulmonary vein
confluence at autopsy. Among the 21 hospital survi-
vors, obstruction proximal to the point of CS unroof-
ing developed in 4 (19% ), necessitating reopera-
tion and resulting in death in 3. One other patient
died late. Mean follow-up of the 17 long-term survi-
vors, who are generally doing well, is 65 months
(range 2 to 2 12). There have been no cases of late
obstruction at the site of unroofing among 10 pa-
tients who underwent the fenestration procedure
P ulmonary venous obstruction is considered rare
with total anomalous pulmonary venous connection
(TAPVC) to the coronary sinus (CS).*-5 In the only re-
port in the last 10 years in which more than 10 operated
patients with this condition were described,‘j a high
frequency of late obstruction was found. Two recent
cases of postoperative obstruction after repair of
TAPVC to the CS prompted us to review our surgical
experience with this anomaly.
From the Department of Cardiovascular Surgery, Children’s
Hospital, and the Department of Surgery, Harvard Medical
School, Boston, Massachusetts. Manuscript received June 30,
1986; revised manuscript received and accepted September 2,
1986.
Address for reprints: Richard A. Jonas, MD, Department of
Cardiac Surgery, Children’s Hospital, 300 Longwood Avenue,
Boston, Massachusetts 02115.
431
suggested by Van Praagh. The incidence of early
bradyarrhythmias (60 % ) does not appear to be de-
creased by this procedure. Revlew of 13 autopsy
specimens qgests that If the right and left pulmo-
nary veins dld not drain directly to the CS but con-
verged to form a short common vertical vein (4
cases), obstruction was likely. When pulmonary ar-
tery pressure approaches systemic levels preopera-
tively, careful echocardiiraphic and intraoperative
assessment of the junction of the pulmonary vein
confluence with the CS should be made. If there is
evidence of obstruction, consideration should be
given to anastomosing the horizontal rtght and left
pulmonary veins directly to the left atrium rather
than performing a simple unroofing procedure.
(Am J Cardiol 1967;59:431-435)
Methods
Patients: Between 1960 and 1985,27 patients under-
went surgery for TAPVC to the CS at our institution.
The hearts of 7 patients who had undergone surgery
and 6 nonoperated autopsy specimens were available
for review, yielding a total of 33 patients. Patients with
heterotaxy (asplenia/polysplenia) and TAPVC to the
CS were excluded. Twenty-nine patients had both
right and left pulmonary veins draining directly into
the CS. One patient had drainage of the left upper lobe
to the innominate vein, 1 had drainage of the left lower
lobe directly to the left atrium and 1 had a left superior
vena cava that connected to the innominate vein and
CS and also drained the left upper lobe. One patient
had a left superior vena cava that joined the CS with
the confluence of the right and left pulmonary veins.
One patient had an absent right lung.
There were 17 male and 16 female patients. Medi-
an age at operation was 2 months [mean 8.6, range 2
432 TOTAL ANOMALOUS PULMONARY
/
/
/ tients] or isoproterenol (1 patient). Two patients had
I I I I
0
0 20 40 60
SYSTEMIC ARTERY
(mmHg)
FIGURE 1. Preoperative or premortem catheterization
pulmonary artery pressure to systemic arterial
SAP).
days to 5.5 years). Median weight at operation was 3.5
kg (mean 5.3, range 1.9 to 161. Most children were
cyanotic and in some respiratory distress in early
infancy.
All but 1 of the 27 operated patients underwent
preoperative cardiac catheterization. Four of the 6
nonoperated autopsy patients had undergone cardiac
catheterization. Figure 1 shows the ratio of pulmonary
artery pressure to systemic arterial pressure in the 28
patients in whom these data were available. Pulmo-
nary vein to right atria1 pullback pressure measure-
ments were available in 5 patients and pulmonary
artery wedge/right atria1 gradients in 13. Two-dimen-
sional echocardiographic reports were available in 9
of the 27 operated patients.
Surgical technique: Ten patients underwent the
fenestration procedure described by Van Praagh in
1972.7 Briefly, in the hope of minimizing damage to
atria1 conduction pathways, the roof of the CS within
the left atrium is unroofed without incising between
the CS and foramen ovale. The CS ostium and fora-
men ovale are closed by direct suture. The 10 Van
Praagh procedures were performed between 1971 and
1979.
Thirteen patients underwent a procedure in which
the CS was unroofed by incising between the CS osti-
urn and foramen ovale. The resulting atria1 septal de-
fect was closed with a pericardial patch in 4 patients
and with a synthetic patch in 7. In 2 patients the patch
material was not stated. Eight of these procedures
were performed between 1961 and 1971 and 5 be-
tween 1981 and 1985.
Four other procedures were performed. Two pa-
tients in whom obstruction was found preoperatively
had an unroofing procedure combined with anastomo-
sis of fileted pulmonary veins to the left atrium. The
first 2 procedures in the series in 1960 to 1961 amount-
ed to little more than exploratory procedures, with
VENOUS CONNECTION TO CORONARY SINUS
major difficulties encountered in conducting cardio-
pulmonary bypass.
Results
Clinical study: Six patients died early after surgery.
All died before 1971, for an early mortality rate to that
time of 6 of 11. The early mortality rate since 1971 has
been 0 of 16. Two deaths were intraoperative, related
to difficulties establishing cardiopulmonary bypass.
Two deaths were due to postoperative bleeding and
tamponade and 2 were due to low cardiac output.
Six of the 10 patients who underwent the Van
Praagh procedure were noted to have bradyarrhyth-
mias in the intensive care unit postoperatively. Four of
the 6 had slow nodal rhythm treated by pacing (3 pa-
I I
80 100 120
sinus bradycardia, which was treated by pacing in 1.
PRESSURE Of the 13 patients who underwent a more traditional
procedure, 6 had bradycardic rhythms. Three patients,
data: ratio of all of whom underwent operation before 1971, had
pressure (PAP/ complete heart block, 2 of whom died, and 3 had nodal
rhythm requiring intervention. Late pacemaker im-
plantation has not been required in any of the long-
term survivors, although 1 patient who underwent a
Van Praagh procedure is receiving long-term p-block-
ade therapy for recurrent paroxysmal atria1 tachycar-
dia. Late systematic Holter monitoring, however, has
not been performed. No bradyarrhythmias were noted
among the 4 patients who underwent miscellaneous
procedures, although 2 died early. Other complica-
tions included bleeding (4 patients), neurologic events
(2 patients] and low cardiac output (2 patients].
Among the hospital survivors, the mean duration of
intubation was 34 hours (range 10 to 72), excluding 1
patient who underwent ventilation for 18 days before ’
reoperation for pulmonary venous obstruction and 2
patients who required tracheostomy, 1 for Pierre-Rob-
in syndrome and 1 for a prolonged low output state.
The mean duration of hospitalization was 16 days (me-
dian 11, range 6 to 77).
Four of the 21 hospital survivors died late. The first
hospital survivor of the series, who originally under-
went operation at age 5 years in 1961, was noted to
have a residual left-to-right shunt at the atria1 level.
Reoperation in 1969 was complicated by air embolus
and neurologic injury, which resulted in death.
Three other patients died late, at 4,6 and 10 months,
because of recurrent pulmonary venous obstruction.
One child had signs of pulmonary venous obstruction
on preoperative catheterization with suprasystemic
(120%) pulmonary artery pressure and a g-mm Hg gra-
dient between pulmonary artery wedge pressure and
right atria1 pressure. Obstruction was believed to be
due to a small CS. At surgery at 10 days of age, the CS
was unroofed and a patch of polytetrafluoroethylene
was used to close the atria1 septal defect, created by the
CS ostium and patent foramen ovale. She did well
initially and was extubated on the second postopera-
tive day and discharged on the sixth postoperative day.
Two months later she returned in pulmonary edema
and was found to have pulmonary venous obstruc-
tion. The confluence of pulmonary veins connected
 February 15, 1987 THE AMERICAN JOURNAL OF CARDIOLOGY Volume 59 433

FIGURE 3. Preoperative 2-dimensional echocardiogram of a patient

with obstructed total anomalous pulmonary venous connection.
Subxiphoid long-axis cut shows narrowed connection (arrow) be-
tween the pulmonary venous confluence and the mildly dilated
FIGURE 2. Preoperative l-dimensional echocardiogram from a pa- coronary sinus. Abbreviations as in Figure 2.
tient with unobstructed total anomalous pulmonary venous connec-
tion. Subxiphoid long-axis cut shows wide connection (arrow) be-
tween the pulmonary venous confluence and the dilated coronary
sinus (CS). * = eustachian valve; LPV = lefl pulmonary vein; p/s = tient, already described, had episodes of paroxysmal
posterior and superior; r = rightward; RA = right atrium; RPV = right atria1 tachycardia, which are now controlled by a fi-
pulmonary vein. blocking drug. All other patients are asymptomatic.
Eight of the 17 long-term survivors have had postoper-
ative catheterizations at approximately 1 year after
through a narrow vertical vein, 4 mm long to the roof of surgery. Except for the patient in whom pulmonary
the left atrium. A polytetrafluoroethylene patch plasty venous obstruction was found and reoperation per-
of the connecting vein was performed and once again formed, all patients had normal pulmonary artery
she did well. She was extubated on the second postop- pressures with no shunts or gradients. One patient has
erative day and discharged on the eighth day, with mild left ventricular dysfunction and mitral valve
no &dimensional echocardiographic evidence of ob- prolapse.
struction. However, obstruction recurred within 2 Autopsy study: In 9 of the 13 autopsy specimens,
months, At the third operation, a profuse fibrous pro- the right and left pulmonary veins drained directly to
liferative process was extending into both pulmonary the CS (Fig. 2). There was no anatomic evidence for
veins. She died several weeks after this third pro- obstruction in any of these specimens. The mean ratio
cedure. Both of the other 2 children who died late of pulmonary artery pressure to systemic artery pres-
from pulmonary venous obstruction followed similar sure in 6 of these 9 patients in whom this was measured
courses. during life was 0.78 (0.5 to 1.22). Mean maximal diame-
The course of 1 other patient was complicated by ter of the CS was 2.3 times the mean sum of the maxi-
pulmonary venous obstruction. A 4-month-old boy mal diameters of the right and left pulmonary veins in
had a preoperative gradient of 18 mm Hg between the this group.
pulmonary artery wedge pressure and right atria1 pres- In 4 specimens, a short common vertical vein
sure. At first operation, the child was found to have a emerged from the junction of the right and left pulmo-
stenosis at the junction of the pulmonary veins and CS. nary veins and drained inferiorly to the CS (Fig. 3). The
The CS was unroofed and the right and left pulmonary mean diameter of the vertical vein was 0.7 times the
veins were fileted. The pulmonary veins were anasto- mean sum of the diameters of the right and left pulmo-
mosed to the posterior wall of the left atrium. Three nary veins. The mean ratio of pulmonary artery pres-
months postoperatively, the child returned with left- sure to systemic artery pressure in these 4 patients was
sided pulmonary venous obstruction, which was suc- 1.02 (range 0.7 to 1.23). Two of these patients had died
cessfully relieved at reoperation. This child remains in low cardiac output early after surgery. One patient
asymptomatic 4 years postoperatively. died at 2 months of age without operation and the other
The mean follow-up of the 17 long-term survivors is at 11 years without operation.
85 months (range 2 to 212). Two patients, who are both
more than 13 years after surgery, report symptoms af- Discussion
ter exercise: 1 chest pain and 1 dyspnea. Both have The impression that TAPVC to CS is rarely if ever
normal chest x-ray, exercise electrocardiographic and complicated by pulmonary venous obstruction has
2-dimensional echocardiographic findings. One pa- largely developed from autopsy series containing few
434 TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION TO CORONARY SINUS

surgical patients and almost no patients who died late had undergone catheterization before death had sys-
after surgery. Burroughs and Edwards, in their 1960 temic or suprasystemic pulmonary artery pressure. In
review,8 focused on the length of the anomalous path- that series of 93 cases, anatomic obstruction was pres-
way and size of atria1 septal defect as important deter- ent in 74% of patients with suprasystemic pulmonary
minants of patient survival before surgery. Gathman artery pressure.
and Nadas, in 197O,g in a predominantly autopsy study, The current review of our surgical experience with
found pulmontiry venous obstruction in 1 of 14 pa- TAPVC to the CS over 25 years suggests that pulmo-
tients with drainage to the CS. Delisle et al,1° in a 1976 nary venous obstruction may be more common with
autopsy study, found 17 cases of TAPVC to the CS but this anomaly than previously suspected. This concept
could not define anatomic obstruction in any. How- is supported by careful analysis of the surgical litera-
ever, 3 of 8 patients from the series of Delisle et al who ture, particularly from centers with high volumes of
infant surgery. In this series of 27 surgical patients, 6
(22%) were identified whose course was complicated
by pulmonary venous obstruction. Two patients who
died early had anatomic evidence of obstruction
at postmortem examination. These patients were 3
months and 1 month old at surgery. Four other patients
had obstruction necessitating reoperation within 1
year of their primary surgery; 3 of them died. The 3
patients who died were all less than 10 days of age at
primary surgery and the other patient was 4 months
old at first operation. This is compared with a mean
age for the entire group of 8.6 months.
In 1978, Whight et al6 reported the long-term results
of surgery for 11 infants with TAPVC to the CS. There
were no early deaths. However, 4 patients (36%) had
late pulmonary venous obstruction, resulting in 3 late
deaths. The site of the obstruction was localized to the
point of unroofing [after a Van Praagh procedure] in
only 1 patient. In the other 3 patients the point of ob-
struction appeared to be at the junction of the pulmo-
nary veins and CS.
In 1980, Turley et all’ reviewed 22 infants who had
undergone correction of TAPVC in infancy. In 5 pa-
tients drainage was to the CS. Two of these patients
had important pulmonary artery wedge to right atria1
gradients of 12 and 6 mm Hg preoperatively, and 1 of
these 2 patients died early. One other patient was not-
ed to have a markedly “narrowed orifice” despite a
low pulmonary venous pressure 9 days previously.
There were no late deaths in this group, although the
duration of long-term follow-up is not stated. Thus, the
early incidence alone of obstruction in this small surgi-
cal series is 60%.
In 1982, Dickinson et all2 reported 44 infants who
had undergone repair of TAPVC. The early mortality
rate was highest (40%) in the group of 10 patients with
drainage to the CS. One of 6 late survivors died due to
pulmonary venous obstruction. In this patient, the 4
pulmonary ireins were described as entering “the apex
of the CS via a single stenotic orifice of less than 4 mm
diameter.”
All 6 postoperative cases of obstruction in the cur-
rent series were localized to a very short, narrow seg-
ment of vertical vein connecting the horizontal right
and left pulmonary veins to the CS. A similar appear-
FIGURE 4. Top, the left horn ot the sinus venosus, precursor of the ance was found in 2 other autopsy specimens, 1 from a
coronary sinus, drains the anterior and posterior cardinal veins patient known to have suprasystemic pulmonary ar-
through the left common cardinal vein in the 10 mm embryo. f30t tery pressure before death. The other specimen was
tom, normal regression of the left common cardinal vein leaves the from a child who died at 2 months of age. We have not
ligament of left superior vena cava (LSVC) as its only remnant at seen obstruction develop at the level of the CS ostium
term. as reported by Arcinieagas et a1.13
 February 15, 1987 THE AMERICAN JOURNAL OF CARDIOLOGY Volume 59 435

The CS develops from the left horn of the sinus Foregut

Covered by
venosus,which early in intrauterine life drains the left , Splanchmc Plexus

common cardinal vein (Fig. 4, top). The left common Luno Bud -./
cardinal vein gradually regresses,with its only rem-
nant being the ligament of Marshall (Fig. 4, bottom).14 L. Anterior
TAPVC occurswhen the pulmonary vein evagination Cardinal Van
from the posteriorsurfaceof the left atrium fails to fuse
with the pulmonary venous plexus surrounding the
developing lung (Fig. 5). In this case,communication
betweenthe pulmonary venousplexus and splanchic
venousplexus persists.If the point of communication
happensto be in the left common cardinal vein rather
than the left horn of the sinus venosus,a restrictive
communication between the pulmonary veins and CS
could result.
In 1972,Van Praaghet al7describeda revisedtech-
Common ’
nique for surgicalmanagementof TAPVC to the CS. It Cardmal Vem
was hoped that this fenestration technique would
avoid damageto the “atria1internodal pathways.”Our
review, however,has revealed a continuing high inci- FIGURE 5. Total anomalous pulmonary venous connectlon results
dence of early bradyarrhythmias(60%)despiteuse of when the primordial pulmonary veln falls to unite wlth the plexus of
this methodand 1 late caseof recurrentsupraventricu- veins derlved from the cardinal veln system and surrounding the
lar arrhythmia necessitatinglong-term prophylactic lung buds.
medication. We have not seenobstruction develop at
the site of the fenestration, although others have.6
However, we have also not seenobstruction develop References
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