Surgical Approach to Left Ventricular Inflow Obstruction due to Dilated Coronary Sinus by Florentino J Vargas

Florentino J. Vargas, MD, Jorge Rozenbaum, MD, Ricardo Lopez, MD,
Miguel Granja, MD, Ana De Dios, MD, Beatriz Zarlenga, MD, Enrique Flores, MD,
Enrique Fischman, MD, and Eduardo Kreutzer, MD
Divisions of Cardiovascular Surgery and Cardiology, Hospital de Niños Pedro Elizalde, Buenos Aires, Argentina
Background. Left superior vena cava draining to a
dilated coronary sinus can cause left ventricular inflow
obstruction. Our purpose is to report 4 severely ill
patients with this malformation who were operated upon
and in whom repair was accomplished using an original
surgical approach.
Methods. An operative procedure was designed, which
included complete resection of the wall of the coronary
sinus along its entire extension in the left atrium; division of the left superior vena cava; and establishment of
the left superior vena cava–right atrial continuity by a
wide left superior vena cava–right atrial appendage anastomosis. The series included 1 patient with interrupted
inferior vena cava– hemiazygous continuation to left superior vena cava.
Results. There were no deaths. Absence of residual left
ventricular inflow obstruction was demonstrated at follow-up in all cases, together with an unobstructed left
superior vena cava–right atrial appendage–right atrial
connection.
Conclusions. A predictable relief of the left ventricular
inflow obstruction, together with preservation of an
adequate drainage for the systemic venous return, were
both achieved with this repair.
(Ann Thorac Surg 2006;82:191– 6)
© 2006 by The Society of Thoracic Surgeons
T
diac anatomy and hemodynamic assessment in all patients. All preoperative imaging studies were reviewed.
All operative, hospital, and follow-up records were obtained and analyzed. The hospital Institutional Review
Board granted an exemption to perform this study retrospectively on December 2005. All patients in the series
gave informed consent on the procedure. The Institutional Review Board granted authorization for the use of
this technique in future patients.
Since October 2000, 4 patients were admitted for surgery with the diagnosis of LSVC-DCS, left ventricular
inflow obstruction, and absence of the innominate vein.
One had been initially referred with the diagnosis of core
triatriatum. Ages ranged from 7 days to 7 months (median, 70 days). Chest roentgenograms of the series
showed moderate cardiomegaly and variable degrees of
passive pulmonary venous congestion. Electrocardiograms displayed patterns of right ventricular hypertrophy and increased right ventricular pressure. Preoperative TTE demonstrated LSVC-DCS in all patients. The
DCS was displayed as a large, thin-walled oval structure
occupying the external wall of the left atrium (LA) and
bulging interposed between an upper LA chamber
(which contained the pulmonary veins) and a lower LA
chamber (in continuity with the mitral valve), narrowing
the inflow path to the left ventricle (Fig 1A and B). In all
patients, a turbulent flow was present at this level,
together with an increased flow velocity by pulsed Doppler (median value, 1.7 m/s; range, 1.5 to 1.9 m/s), this
being interpreted as a pressure gradient caused by the
DCS. In all patients, the mitral valve annulus was smaller
he presence of left superior vena cava (LSVC) draining into a dilated coronary sinus (DCS) and obstructing the left ventricular inflow has been reported in
the literature as an infrequent anomaly [1– 6]. We are
reporting 4 patients with left ventricular inflow obstruction caused by DCS, in whom an original surgical technique was used for repair. In the first patient of this series
operated on, the attempted reduction plasty of the wall of
the DCS was insufficient to provide a satisfactory and
complete anatomic relief of the obstruction. As a consequence, resection of the entire wall of the DCS was then
mandatory for this purpose. This was followed by LSVC
division and the performance of a large, unobstructed
LSVC–right atrial appendage (RAA) anastomosis. This
procedure was subsequently performed in the rest of the
series.
Patients and Methods
A review of our surgical database was performed to
include patients with LSVC-DCS with left ventricular
inflow obstruction (in absence of innominate vein) who
underwent cardiac surgery. All patients underwent twodimensional transthoracic echocardiography (TTE) with
color flow Doppler analysis preoperatively. Cardiac catheterization was performed for further evaluation of carAccepted for publication Feb 27, 2006.
Address correspondence to Dr Vargas, Pediatric Cardiovascular Surgery
and Cardiopumonary Transplantation, Hospital de Niños Pedro Elizalde,
San Martin 1353, Banfield, Buenos Aires 1828, Argentina; e-mail:
florentino_jose@yahoo.com.
© 2006 by The Society of Thoracic Surgeons
Published by Elsevier Inc
0003-4975/06/$32.00
doi:10.1016/j.athoracsur.2006.02.062
CARDIOVASCULAR
Surgical Approach to Left Ventricular Inflow
Obstruction Due to Dilated Coronary Sinus
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Abbreviations and Acronyms
ASD ⫽ atrial septal defect
DCS ⫽ dilated coronary sinus
LA
⫽ left atrium
LSVC ⫽ left superior vena cava
RA
⫽ right atrium
RAA ⫽ right atrial appendage
RSVC ⫽ right superior vena cava
TTE ⫽ two-dimensional Doppler color flow
transthoracic echocardiography
than the tricuspid valve annulus (median mitral valve/
tricuspid valve annulus ratio, 0.6 cm; range, 0.5 to 0.8 cm).
The upper LA chamber communicated with the right
atrium (RA) by an atrial septal defect (ASD), which was
restrictive (median pulsed Doppler velocity, 1.7 m/s;
range, 1.5 to 2.1 m/s) in all patients. In 2 patients, bulging
of the superior portion of the atrial septum from LA to
RA was observed, indirectly indicating higher pressure in
the upper LA chamber, attributed to the obstruction from
DCS. Inversely, the inferior portion of the atrial septum
bulged from RA to LA, displaying an S-shaped septal
orientation (Fig 1A). The latter was interpreted as being
caused by increased RA pressure (due to pulmonary
hypertension and tricuspid regurgitation) when confronted with a decreased pressure in the lower LA
chamber, distally to the obstruction. In the remaining 2
patients, the entire atrial septum bulged moderately from
LA to RA. Right ventricular systolic and pulmonary
artery pressures determined from the tricuspid regurgitation gradient were found severely elevated in all cases
(75% to 100% systemic pressure). Other sources of left to
right shunt were not demonstrated either at the ventricular or aortic level from TTE. Additional intracardiac or
aortic lesions causing left-sided obstruction were also
ruled out.
Cardiac catheterization was performed in all patients.
A right superior vena cava (RSVC) angiogram showed
Ann Thorac Surg
2006;82:191– 6
this vein to be smaller than the LSVC in 3 cases, and
severely hypoplastic in 1. The innominate vein was
absent in all. The LSVC-DCS, filled from an interrupted
inferior vena cava with hemiazygous continuation, was
demonstrated from an inferior cavogram in 1 patient (Fig
2). LA filling after the pulmonary angiography displayed
the obstruction at supravalvular mitral level as a welldefined filling defect. Left ventriculograms were not
performed. In all patients, a pressure gradient was obtained (median, 9 mm Hg; range, 7 to 15 mm Hg),
between the upper LA chamber and the RA (restrictive
ASD). Tall “a” waves (median, 27 mm Hg; range, 20 to 35
mm Hg) were registered at the upper LA chamber in all
patients. Left ventricular pressures were obtained in 2
patients. A 14 mm Hg and a 16 mm Hg gradient between
the increased “a” waves and the end diastolic pressure of
the left ventricle were respectively registered in both,
confirming left ventricular inflow obstruction. Studies in
the remaining 2 patients were prematurely suspended
owing to an unstable hemodynamic situation, precluding
obtaining left ventricular pressures. There was severe
pulmonary hypertension, (median, 65 mm Hg; range, 100
to 55 mm Hg). Oxygen saturation difference registered
between RSVC and the pulmonary artery in all patients
confirmed the presence of the left to right shunt at atrial
level (median oxygen saturation difference, 7%; range,
5% to 8%).
At operation, it was confirmed that the innominate
vein was absent (Fig 3A).The LSVC was larger than the
RSVC in all patients and was receiving a dilated hemiazygous vein in 1 (patient with diagnosis of interrupted
inferior vena cava– hemiazygous continuation to LSVCDCS). Cardiopulmonary by pass was started, the aorta
was cross clamped, and the RA opened transversally
from below the base of the RAA. The orifice of the DCS
in the RA was large. The atrial septum was widely
excised, enlarging a restrictive ASD. Once into the LA,
the pulmonary veins were recognized. The mitral valve
could not be exposed through this approach yet; it was
hidden behind a severely enlarged DCS whose wall was
Fig 1. Preoperative echocardiogram. (A) Apical four-chamber view (systole). The interatrial septum bulges toward the right atrium in its superior portion, and toward the left atrium in the inferior part (arrows) adopting an S-shaped configuration. (B) Apical four-chamber view (diastole). The dilated coronary sinus is occupying a major part of the external border of the left atrium and protrudes, causing obstruction to the
left atrial emptying at a supravalvular mitral level (arrow). Both the mitral valve and left ventricle are smaller than the tricuspid valve (mitral
valve /tricuspid valve annulus ratio: 0.6 cm) and the right ventricular chamber. (CS ⫽ coronary sinus; LA ⫽ left atrium; LV ⫽ left ventricle;
RA ⫽ right atrium; RV ⫽ right ventricle.)
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the circular cuff of LA wall tissue previously isolated
together with the distal end of LSVC, establishing
LSVC-RA continuity (Fig 3F). This redundant portion of
LA wall in this way obtained, surrounding the base of the
LSVC, provided an adequate amount of tissue for the
anastomosis to be sufficiently large and without tension.
The right atriotomy was closed, and weaning from bypass was performed uneventfully.
Results
Fig 2. Preoperative angiogram performed in the inferior vena cava
displays interruption of the inferior vena cava, with hemiazygous
continuation to left superior vena cava and to a dilated coronary
sinus. Washout from the left superior vena cava is observed at the
hemiazygous–left superior vena cava junction level. (AZ ⫽ interrupted inferior vena cava– hemiazygous continuation; CS ⫽ coronary sinus; LSVC ⫽ left superior vena cava.)
redundant and protruded into the LA chamber (Fig 3A).
In the first patient of this series, reduction plasty of the
DCS was initially attempted and subsequently abandoned owing to a residual obstruction of the mitral
valve area (part of the valve area remained concealed
after the reduction plasty was performed). The roof of
the DCS was then excised longitudinally along its
course in the LA until the point of entrance of LSVC
into the heart, and the entire remaining wall tissue of
the DCS was completely removed. After this, the mitral
valve and, therefore, the left ventricular inlet were now
uncovered and exposed to be free of obstruction for the
first time (Fig 3B).
To perform a satisfactory relief of the obstruction in 1
case (a 7-day-old patient), the roof of the DCS was
opened from the RA toward the LA, across the previously
opened atrial septum (as in the technique for repair of
total anomalous pulmonary venous return to coronary
sinus). The previously enlarged ASD was then closed
with a pericardial patch (Fig 3C). At this point, the LSVC,
together with a wide cuff of additional LA wall tissue
around its distal orifice, was excised and separated from
the LA (Fig 3D). The large orifice created in the roof of the
LA was then closed by suturing a flap created with the LA
appendage longitudinally opened, thereby enlarging the
LA chamber (Fig 3E). The tip of the RAA was then
opened wide, and the entire muscular trabeculae carefully removed from inside. This was then anastomosed to
There was no hospital mortality. One patient showed
increased pulmonary blood flow and increasing signs of
postoperative cardiac failure. He was reoperated on to
close a perimembranous restrictive ventricular septal
defect that had not been evident in the TTE at admission.
In this case, a prolonged period of mechanical ventilation
was required (20 days). In the remaining patients, the
average period of hospitalization was 12 days. They were
all discharged asymptomatic, on vasodilator therapy.
Aspirin therapy was maintained for 3 months to prevent
thrombogenic complications at the level of the LSVCRAA anastomosis.
Mean follow-up duration is 34 months (range, 7 to 54).
No clinical signs of either systemic or pulmonary venous
return obstruction were detected. The TTE evaluation
demonstrated the absence of the previous left ventricular
inflow obstruction. There were no additional left-sided
obstructions. The LSVC-RAA-RA connection was widely
patent and unobstructed in all patients. In 1 of these,
peripheral venous contrast echocardiography performed
through the left arm displayed the LSVC draining freely
into the RA. Postoperative cardiac catheterization performed in 1 patient with interrupted inferior vena cava–
hemiazygous continuation to LSVC demonstrated an
absence of left ventricular inflow obstruction. Pulmonary
artery pressure was normal. An unobstructed systemic
venous return entering the RA following its way from the
interrupted inferior vena cava through the hemiazygous
vein–LSVC-RAA connection was demonstrated (Fig 4A
and B) simultaneously with a widely patent LSVC-RAA
anastomosis. There were no additional obstructions demonstrable at LA or at the mitral valve–left ventricular
level.
Comment
Coexistence of persistent LSVC draining to coronary
sinus and congenital heart disease has been reported in
the literature with a prevalence variable from 2.8% to
11% [7, 8]. Although generally regarded as a benign
anatomic association, it could impose technical differences during surgical procedures, including (a) the creation of bilateral cavopulmonary anastomosis (during
partial right ventricular bypass operation procedures);
(b) a different suture line during septation for atrioventricular canal defects; (c) the need to close a potentially
residual right to left shunt in patients with unroofed
coronary sinus; and (d) the need to create an adequate
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Fig 3. Surgical technique. (A) Interrupted inferior vena cava– hemiazygous continuation to left superior vena cava (dotted line) and to the dilated coronary sinus. The innominate vein is absent. The dilated coronary sinus causes left ventricular inflow obstruction. The mitral valve is
hidden underneath the dilated coronary sinus. (B) Through a wide excision of the atrial septum, the wall of the coronary sinus was entirely
removed (arrow) from inside the left atrium. (C) The previously enlarged atrial septal defect is closed with a pericardial patch. (D) The left
superior vena cava together with a wide circular cuff of left atrial wall is separated from the left atrium. A large orifice was created in the roof
of the left atrium. The left atrial appendage will be opened longitudinally following the dotted line (arrow) to create a flap. The pulmonary
artery is retracted. (E) A wide flap was tailored with the opened left atrial appendage and used to close the large defect created in the roof of
the left atrium. (F) The tip of the right atrial appendage is widely opened, its inner surface liberated from residual muscular trabeculae to create an unobstructed tubular structure, and a large anastomosis is performed with the wide circular cuff of left atrial tissue previously isolated
together with the left superior vena cava, thereby establishing left superior vena cava–right atrium continuity. The right atriotomy was closed.
(Ao ⫽ aorta; azg ⫽ hemiazygous [interrupted inferior vena cava– hemiazygous continuation to left superior vena cava]; cs ⫽ coronary sinus;
laa ⫽ left atrial appendage; laf ⫽ left atrial flap; lsvc ⫽ left superior vena cava; m ⫽ mitral valve; p ⫽ atrial septal patch; pa ⫽ pulmonary
artery; raa ⫽ right atrial appendage; rsvc ⫽ right superior vena cava.)
LSVC-RA connection while performing heart or heartlung transplantation [9, 10].
Left superior vena cava–DCS has been reported as an
infrequent cause for left ventricular inflow tract obstruction [1– 6]. It has also been suggested that it could lead to
a higher incidence of associated left heart obstructive
lesions [6]. It is generally associated with an absent
innominate vein. The anomaly can be severely symptomatic early in life and even mimic the features of core
triatrium, as had occurred in our series and in previous
reports [5]. It may present alone or associated with other
cardiac malformations [1, 2, 4, 6]. During repair of associated defects, the left ventricular inflow obstruction may
not be detected (it may only be assessed by direct LA
exposure through a wide atrial septal opening), unless a
preoperative diagnosis has been made. Death from residual obstruction due to undiagnosed obstructive DCS
after correction of partial atrioventricular canal, interpreted as precipitated by the closure of the ASD, has
been reported [4]. Pulmonary hypertension was a common finding in previous reports, as it was found in our
series [5, 6].
In our reoperated patient, an associated restrictive
ventricular septal defect had certainly contributed to the
severe pulmonary hypertension presented preoperatively. In this case, left to right shunt at the ventricular
level was not initially displayed in the TTE, precluding
diagnosis of the defect. We hypothesize that surgical
release of the left ventricular inflow obstruction and the
subsequent free emptying of the LA produced a drop in
the pulmonary vascular resistances postoperatively (and
the consequent development of a larger pulmonary
blood flow), thereby allowing the defect to become symptomatic and recognizable in the postoperative TTE. Based
upon the severe anatomic obstruction caused by the DCS
found at surgery in this patient (which concealed most of
the mitral valve area), we realize that DCS played a chief
role in the systemic pulmonary hypertension demonstrated at admission from both the TTE and hemodynamic study. A restrictive ASD was always present in the
series. As mentioned in previous reports, is it difficult to
determine the role of ASD in the hemodynamics of this
malformation [6]. Coincidently with a series of patients
with left ventricular inflow obstruction and DCS reported
by DiBardino and colleagues [6], in whom tall “a” waves
in the LA were constantly observed, a similar finding was
present in our experience.
From a surgical viewpoint, the goal would be to obtain
Fig 4. Postoperative catheterization. (A) Anteroposterior. (B) Lateral.
The cavogram performed in the interrupted inferior vena cava displayed the systemic venous return as it enters into the right atrium
following its way from the interrupted inferior vena cava through
the hemiazygous vein–left superior vena cava to right atrial appendage anastomosis. A widely patent left superior vena cava–right atrial
appendage anastomosis was demonstrated. Washout from the upper
left superior vena cava is present. (AZ ⫽ interrupted inferior vena
cava– hemiazygous continuation; LSVC ⫽ left superior vena cava;
RA ⫽ right atrium; RAA ⫽ right atrial appendage.)
a complete elimination of the left ventricular inflow
obstruction, providing that an adequate systemic venous
drainage of the LSVC to the RA remains. Repair by
performing reduction plasty of the DCS (tailoring around
a probe) has been reported previously with satisfactory
results [2, 3, 6]. This was attempted in our first operated
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195
case, in whom we found that residual supravalvular
mitral obstruction after reduction plasty was already
present at surgery. The presence of interrupted inferior
vena cava with hemiazyguos continuation to the LSVCDCS, which required the preservation of a nonobstructed
path for the major part of the systemic venous return,
could have played a role in this case by limiting the
extent of reduction in the size of the DCS. Both surgical
goals (left ventricular inflow obstruction relief and adequate systemic venous drainage) may be difficult to be
simultaneously achieved for these patients in a predictable manner.
The ideal procedure for absolute elimination of the obstruction would be the complete removal of the wall of the
DCS along its course within the LA. That must be followed
by the division of the LSVC, with subsequent creation of a
suitable path for the systemic venous return. This concept
has been proposed in the past, with the removal of the wall
of the DCS and interposing a prosthetic tube between the
divided LSVC and RSVC [4]. However, this technique has
the obvious disadvantages (no growth, thrombosis) imposed by a prosthetic tube inserted within a low pressure
venous system (mainly if performed with small vessels,
early in life). The importance of obtaining a predictable
drainage of the systemic venous return becomes crucial in
patients in whom an interrupted inferior vena cava with
hemiazygous continuation to LSVC carries the major part
of the systemic venous return. Palacios-Macedo and associates [11] reported a different procedure to divert the
systemic venous return from the LSVC to RSVC in 1 patient
with heterotaxy syndrome and interrupted inferior vena
cava. They transferred the LSVC in continuity with a long
left atrial appendage tailored and sutured longitudinally as
a tube, thereby creating a conduit that crossed transversally
in front of the aorta and was finally anastomosed to the
RSVC posteriorly.
The procedure herein reported allows for both a complete resection of the entire wall of DCS (ensuring a total
relief of the obstruction) and, subsequently, for the creation of an unobstructed drainage of the systemic venous
return to RA through the enlarged LSVC-RAA anastomosis. The wide cuff of LA wall tissue isolated together
with the distal LSVC allowed for a large, unrestricted
LSVC-RAA anastomosis to be performed without tension. The large orifice created in the roof of the LA was
adequately reconstructed with a wide flap obtained from
the opened LA appendage. As an interesting alternative
to be explored in the future, perhaps a portion of the
resected wall of the DCS might be employed as a flap for
closure of the wide LA roof opening.
The use of the herein described LSVC-RAA anastomosis technique may be expanded to other situations (in
absence of the innominate vein). This is the case of both
heart and heart-lung transplantation in the presence of
LSVC in the recipient. We have successfully used this
LSVC-RAA anastomosis technique during orthotopic
heart transplantation in one patient. The cuff of LA tissue
in these cases can be unusually large (as it is provided by
the recipient). This enlarged LSVC-RAA anastomosis can
also be considered as a part of repair in patients with
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common atrioventricular canal and LSVC. Division of the
LSVC in these cases should allow for the coronary sinus
to stay to the left of the patch if this becomes necessary,
avoiding a tortuous suture line during atrial septation.
Finally, division of the LSVC followed by LSVC-RAA
anastomosis may be an alternative for repair in some
cases with unroofed coronary sinus.
After the completion of this procedure, the distal LSVCRAA connection runs lateral to the aorta and, proximally,
occupies the same place and space that belonged to the
original RAA. Therefore, additional risk for anterior compression from the chest wall should not be expected.
However, long-term follow-up would be crucial to confirm
that an adequate LSVC-RA drainage is maintained. The
suitability of the herein reported LSVC-RAA connection to
establish a path for the systemic venous return is indirectly
supported by the previous experience [12] with the use of
RSVC-RAA connections as a part of the repair of some
forms of anomalous venous return.
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ED, Kung G. Left ventricular inflow obstruction associated with
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8. Buirsky G, Jordan SC, Joffe HS, Wilde P. Superior vena caval
abnormalities: their occurrence rate, associated cardiac abnormalities and angiographic classification in a paediatric population with congenital heart disease. Clin Radiol 1986;37:131– 8.
9. Menkis A, McKenzie FN, Novick RJ, et al, and the Paediatric
Heart Transplant Group. Special considerations for heart
transplantation in congenital heart disease. J Heart Transplant 1990;9:602–17.
10. von Oppell UO, Odell JA, Reichenspurner H, Reichart B,
Zilla P, Fasol R. Anomalous left superior vena cava in
combined heart-lung transplantation. J Heart Transplant
1988;7:444 –7.
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INVITED COMMENTARY
Nowadays in congenital cardiac surgery it is unusual to
discover new pathologies. However, a sum of “minor”
circumstances could create new entities that may need to
be treated. In my opinion this is the case when a
persistent left superior vena cava (LSVC) appears to be
draining in a significantly dilated coronary sinus. This
association has been proven to be partly responsible for
significant left atrioventricular supravalvar obstruction.
An excessive flow along the posterior inferior left atrial
wall in itself may markedly enlarge even a “normal”
LSVC with a normal coronary sinus up to a point of
partial obstruction of left ventricular inflow.
Vargas and associates [1] present their experience in this
unusual anatomic entity previously described by Cochrane
and associates in 1994. The classical technique has been
proposed to manage the problem by an intracardiac approach through transatrial septum with unroofing, segmental resection, and reconstruction of the dilated coronary
sinus wall. The present report combines the previous experience with an extracardiac method that consists in the
translocation of the LSVC to the right appendage.
It is clear that the final objective to repair this entity
should be to eliminate the left atrial outlet obstruction
and provide an adequate systemic venous drainage. To
solve the first problem, an intracardiac approach is compulsory. It is impossible to be sure that a dilated coronary
sinus with a proper flow will evolve to a normal situation
or lead to a significant symptomatic improvement. A
© 2006 by The Society of Thoracic Surgeons
Published by Elsevier Inc
plasty with reduction and reconstruction of the dilated
coronary sinus is the safe and necessary procedure.
Different options have been described to fix the second
component. These range from the no touch technique to
simple ligation of the LSVC (in presence of a left innominate vein connection with a larger right superior vena
cava [RSVC] than the LSVC) or diverse reimplantations.
Anastomosis of the LSVC to the RSVC, to the left pulmonary artery, or to the right atrium have been described
and used. The right atrial appendage technique has been
proven accessible and reproducible in different abnormal
and complex situations. It is probably the adequate
complement to an insufficient intracardiac approach.
Juan V. Comas, MD, PhD
Paediatric Heart Institute
Hospital Universitario “12 de Octubre”
Carretera de Andalucı́a km 5,400
Edificio Materno-Infantil
Madrid, 28041 Spain
e-mail: jvc@mi.madritel.es
Reference
1. Vargas FJ, Rozenbaum J, Lopez R, et al. Surgical approach to
left ventricular inflow obstruction due to dilated coronary
sinus. Ann Thorac Surg 2006;82:191– 6.
0003-4975/06/$32.00
doi:10.1016/j.athoracsur.2006.04.041