Florentino Vargas et al. A surgical technique for correction of total anomalous pulmonary venous drainage

J THoRAc CARDIOVASC SURG 90:410-413, 1985
A surgical technique for correction of total
anomalous pulmonary venous drainage
A tecbnique was employed successfully for correctionof total anomalous pulmonary venous drainageinto
the upper right superior vena cava. A J-sbaped right atriotomy was pedormed; the posterior flap was
sutured to the anterior border of a previously enlargedatrial septal defect The right superior venacava
was divided above the site of drainage of the pulmonary veim, and its proximal opening closed with a
suture. The pulmonary venous return was directed to the left atrium in this way. The right atrial-right
superior vena caval continuity was then reestabUshed by an anastomosis between the previously opened
right atrial appendage and the distal end of the right superiorvenacava. FmaUy the remaining atriotomy
was closed. The azygos vein must be Hgated to avoid systemic IDJS8turation. For correctionof anomalous
pulmonary venous drainage into the azygos vein with this technique, Hgature of the azygos vein must be
placed distally to the site of anomalous drainage. Three patients, aged 2 months, 7 years, and 16 years,
respectively, with different anatomic types of the anomaly, were successfully operated on with this
procedure. Fmdings displayed from the postoperative hemodynamic, echocardiographic, and clinical
evaluation are encouraging, after a foUow-up period that ranges from 4 months to 4 years. The
advantages of the repair are discussed.
Florentino J. Vargas, M.D.,· and Guillermo O. Kreutzer, M.D., Buenos Aires, Argentina
Results for correction of total anomalous pulmonary
venous drainage (APYD) have followed a difficult
evolution from a high mortality rate in the past to
reasonable survival at the present time. I However, even
with this remarkable progress in the treatment of the
disease, prognosis is not optimistic when considering
some particular anatomic variants of the anomaly.s '
Total APYD to the right superior vena cava (SYC) or
azygos vein is an infrequent entity, and its correction is a
surgical challenge when the common pulmonary vein
and the posterior left atrial wall are not in close
proximity.' Furthermore, if a common pulmonary
venous collector does not exist and the pulmonary veins
drain directly into the upper right SYC or azygos vein,4,5
surgical repair cannot be attempted with the techniques
described at present.'
From the Unit of Cardiovascular Surgery, Hospital de Nifios and
Clinica Bazterrica, Buenos Aires, Argentina,
Received for publication Nov. 13, 1984,
Accepted for publication Dec. 12, 1984,
Address for reprints: Florentino J, Vargas, M,D" Cirugia Cardiovascular, Clinica Bazterrica, Juncal 3002, Buenos Aires, Argentina,
"Currently Evarts A. Graham Memorial Traveling Fellow (The
American Association for Thoracic Surgery), Department of
Cardiovascular Surgery, Children's Hospital of Boston, Harvard
Medical School, Boston, Mass.
410
The purpose of this report is to describe an operative
technique designed for correction of this condition,
which was successfully performed in three consecutive
patients with different anatomic variants of the malformation.
Operative technique (Fig. 1)
Through a midline incision, a 'singlevenous cannula is
placed into the tip of the right atrial appendage when
total circulatory arrest is going to be used. Both the
innominate vein and the inferior vena cava are cannulated for conventional bypass in older patients.
A J-shaped incision is made in the right atrial wall, its
vertical limb starting at the base of the right atrial
appendage, near the SYC. The horizontal limb of the
incision runs equidistantly from each vena cava, until a
point is reached just a few millimeters to the right of the
interatrial groove (Fig. 1, B).
Through the right atriotomy, the lamina of the fossa
ovalis is resected, and the atrial septal defect is
enlarged upward and posteriorly (Fig. 1, E). The
posterior flap of the right atrial incision is then sutured
to the atrial septum from the base of the right atrial
appendage, following along the anterior and inferior free
edge of the atrial septal defect, to fmally reach the
posterior wall of the right atrium (Fig. 1, F). The right
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Fig. 1. Operative technique: A, Anomalous right and left
pulmonary veins (APV) drain separately into upper right
superior vena cava (RSVC) (see Case 3 in text). Common
pulmonary vein is absent. B, Broken lines illustrate both the
RSVC incision and the J-shaped atriotomy. C, RSVC has
been divided just above the site of drainage of the pulmonary
veins. D, The proximal end of RSVC is closed with a
continuous suture. RAA, Right atrial appendage. RA, Right
atrium.
SVC is transversely divided just above the site of
drainage of the pulmonary veins, Such a level should be
identified from the external aspect at the point where
the size of the right SVC increases markedly. The
proximal end of the right SVC is then closed with a
continuous 6-0 polypropylene monofilament suture, and
the azygos vein is ligated. In this way, the APVD has
been directed into the left atrium via the proximal SVC
and the enlarged atrial comunication. The right atrialright SVC continuity is then reestablished by an endto-end anastomosis between the distal end of the right
SVC, which has been widened with a vertical split, and
the tip of the right atrial appendage, which was previously opened (Fig. 1, F and G). The right atrium is
fmally closed by suturing the remaining anterior edge of
the atriotomy along the suture line of the posterior flap
(Fig. I, G).
If the orifice of pulmonary venous drainage is restrictive, it can be enlarged with a longitudinal split through
the walls of both the right SVC and the pulmonary vein,
which in tum is sutured transversely.
When the APVD is directed into the azygos vein, a
Total anomalous pulmonary venous drainage
4I1
Fig. 1. Cont'd. E, Through the right atriotomy, the laminae
of the fossa ovalis is removed, and the atrial defect is enlarged
upward and posteriorly. F, The posterior flap of the atriotomy
is sutured to the atrial septum from the base of the right atrial
appendage, following the anterior and inferior edge of the
atrial septal defect, to the posterior wall of the right atrium.
The pulmonary venous return has been directed into the left
atrium in this way. The tip of the right atrial appendage has
been opened wide (arrow). G, The right atrial-RSVC continuity has been reestablished by an anastomosis between the distal
end of RSVC and the previously opened right atrial appendage. The right atrium is finally closed by suturing the
remaining anterior edge of the atriotomy along the suture line
of the posterior flap. Azygos vein must be ligated as described
(see text) to avoid systemic insaturation. Arrow points the
channel of drainage of the pulmonary venous return.
similar procedure can be used. In such cases, the azygos
vein must be ligated distally to the site of drainage of the
pulmonary veins.
Case reports
CASE 1. A 7-year-old girl was admitted for operation in
October, 1980. The preoperative diagnosis of APVD of the
entire right lung into the upper right SVC and of the left upper
lobe into a persistent left SVC had been displayed from the
clinical and hemodynamic evaluation. After conventional
bypass was established, it was seen that the superior and
inferior right pulmonary veins drained separately into the
upper right SVC just below the innominate vein, and the left
upper pulmonary vein drained via the left SVC into the
innominate vein. The anomalous drainage into the upper right
SVC was repaired as described earlier, whereas the left upper
pulmonary veins were anastomosed with the left atrial append-
412
The Journal of
Thoracic and Cardiovascular
Surgery
Vargas and Kreutzer
Fig. 2. Case 3, postoperative catheterization. A, Superior cavogram displayed an unobstructive right SVC-right
atrial appendage connection. Arrow points to the approximate site of the anastomosis. B, The diagram shows the
posteriorly located channel for the pulmonary venous return. A, Right atrial appendage. VD, Right ventricle. P,
Pulmonary artery.
age and the left SVC was ligated. After an uneventful
postoperative period the patient was discharged and has
remained symptom free without medication since then. Systemic or pulmonary venous obstruction has been ruled out
from subsequent clinical, radiologic, and echocardiographic
evaluations. The family refused recatheterization.
CASE 2. A l6-year-old girl with a preoperative diagnosis of
APVD of the entire right lung into the upper right SVC
without atrial septal defect was-admitted for operation in
September, 1982. At operation it was shown that the right
pulmonary veins drained separately into the upper right SVC.
With conventional bypass the same surgical technique was
again employed, and the patient's recovery was uneventful.
She is doing well 2 years after the operation, without
medication and with normal activity. She also refused cardiac
recatheterization.
CASE 3. A 2-month-old boy was admitted for examination
in August, 1984, in markedly deteriorated general condition.
He weighed 3.2 kg and was severelycyanotic. A harsh systolic
murmur was heard along the sternal border, and the liver was
palpable 3 em below the right midcostal margin. The electrocardiogram showed sinus rhythm and a pattern of right
ventricular hypertrophy. The chest roentgenogram displayed
increased pulmonary vascular markings and a widened upper
mediastinal shadow on the right. Catheterization of the right
side of the heart revealed a left-to-right shunt at the atrial level
and systemic values for the right ventricular and pulmonary
arterial pressures. Injection into the main pulmonary artery
showed that all the pulmonary veins drained separately into
the right SVC just below the innominate vein. No COmmon
anomalous pulmonary vein was demonstrated.
Operation was performed on Aug. 16, 1984, with cardiopulmonary bypass and total circulatory arrest. The veins from
both the left and right lungs were found to drain separately
into the right SVC below the innominate vein (Fig. 1). There
was no common pulmonary vein. An operative procedure (Fig.
1) was carried out. The patient's postoperative recovery was
uneventful, and he was discharged from the hospital a week
later.
He is now asymptomatic, free of cyanosis, without medication, and gaining weight. Evidence of systemic or pulmonary
venous obstruction has not been demonstrated from clinical,
radiologic, hemodynamic, and echocardiographic evaluations.
Normal pressure was registered during recatheterization into
the right SVc. Superior cavogram showed no evidence of
obstruction at the site of the anastomosis with the right atrial
appendage (Fig. 2). A late phase showed free drainage of the
pulmonary veins into the left atrium through a venouschannel
placed above the atrial level.
Discussion
Theoretically, the spectrum of techniques used for
repair of the supracardiac type of total APVD should be
applicable when these veins are draining to the right
SVC or to the azygos system.?" A primary condition for
feasibility of all those techniques is the presence of a
common pulmonary vein of an appropriate size behind
the left atrium to allow an anastomosis to be performed
with ease. Such a favorable anatomic disposition is not
always present. The left pulmonary veins usually run
separately behind the left atrium, and furthermore, the
posterior venous collector can be absent.t' In either of
these situations, no common pulmonary vein is expected
to be present in an anatomic disposition suitable for an
adequate anastomosis.'
To preclude such anatomic limitations, in 1973
Kawashima and associates" described a different
approach. They made an anastomotic orifice between
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the anterior wall of the common pulmonary vein and
both the right SVC and superior wall of the left atrium.
The posterior wall of the right SVC was then reconstructed with a Teflon patch, directing the pulmonary
blood flow toward the left atrium. The anterior walls of
the right SVC and right atrium were finally closed after
they were enlarged with a patch of pericardium. This
complex procedure has some disadvantages. First,
reconstruction of the right SVC with a Teflon patch for
the posterior wall and a pericardial patch for the
anterior aspect of the vein has the potential hazard of
creating an obstruction later in life, since almost the
entire circumference of the vein will be of Dacron or
pericardial tissue. It is also quite possible to injure the
sinus node or its artery with this technique. Finally and
most important, even when a common pulmonary vein
just behind the left atrium is not necessary, the approach
of Kawashima and colleagues' requires the presence of
such a venous collector in a close relationship with the
superior wall of the left atrium and with the right SVC
itself. Because of this anatomic limitation, the authors
themselves stated in the original report that they were
not convinced that their approach was always applicable, as noted in our experience.'
The technique herein reported compares favorably
with the above-mentioned alternatives for repair of the
malformation. It also can be employed in partial APVD
into the upper right SVC, as it was used in two of our
cases. Anastomosis between the right atrial appendage
and the SVC has recently been reported by Williams
and colleagues" for repair of partial APVD.
The wide proximal right SVC, the posterior flap of
the right atriotomy, and the enlarged atrial communication will afford an appropriate drainage chamber for the
oxygenated blood to the left atrium without the use of
prosthetic material. The reestablishment of right atrialright SVC continuity by an anastomosis between the
distal right SVC and the right atrial appendage is
always feasible, since the right atrium and its appendage
are larger than usual in hearts with this anomaly.
Preservation of the sinus node and its pathways can also
be expected from this procedure." Finally, this technique can be indicated for all cases with APVD into the
upper right SVC or azygos system, regardless of age,
weight, or any of the known unfavorable anatomic
conditions.
Total anomalous pulmonary venous drainage 413
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