Tricuspid Annuloplasty and Ventricular Plication for Ebstein's Malformation- Florentino J. Vargas et al.

Tricuspid Annuloplasty and Ventricular Plication
for Ebstein’s Malformation
Florentino J. Vargas, MD, Gustavo Mengo, MD, Miguel A. Granja, MD,
Jorge A. Gentile, MD, Maria E. Rannzini, MD, and Juan C. Vazquez, MD
Unit of Pediatric Cardiovascular Surgery, Hospital Italiano, Buenos Aires, Argentina
Background. Seven patients with the diagnosis of Ebstein’s malformation of the tricuspid valve were operated
on. Mean age was 12 years (range, 7 to 16 years). All were
cyanotic, with severe tricuspid regurgitation. Thromboembolism was not present. No associated cardiac malformations were present.
Methods. Surgical repair included tricuspid annuloplasty associated with longitudinal plication of the atrialized portion of the right ventricle. This was attained by
approximating the anterior-posterior commissure with
either the posterior-septal commissure or the septal leaflet remnant. The thin atrialized ventricular wall thus
excluded remained as a cul du sac and was plicated by
suturing along the longitudinal axis of the heart. When
present, the dysplastic posterior leaflet was included in
the plication. In essence, a monocuspid right atrioventricular valve was fashioned out of the anterior leaflet.
The remaining septal leaflet played a minimal functional
role. No additional procedures for treatment of arrhythmia were associated with the technique described.
Results. The postoperative course was uneventful in all
patients. Mean follow-up is 4.3 years (range, 1 to 10
years). Doppler echocardiographic studies reveal satisfactory monocusp valve function in all patients, with
adequate coaptation of the anterior leaflet and the septal
structures.
Conclusions. This technique seems applicable to most
forms of Ebstein’s malformation and is reproducible. The
technique relies on the adequate mobilization of the
anterior leaflet. Occasionally it is necessary to free fibrous
adhesions of the leaflet to the underlying ventricular
surface.
(Ann Thorac Surg 1998;65:1755–7)
© 1998 by The Society of Thoracic Surgeons
C
treatment of arrhythmia were not indicated in this series.
Preoperative echocardiographic assessment displayed an
adequate excursion of the anterior leaflet in all but 2
patients.
onservative procedures should prevail, when possible, for repair of Ebstein’s malformation of the
tricuspid valve, especially for patients in the pediatric age
group. Annuloplasty techniques associated with either
transverse or longitudinal plication of the atrialized right
ventricle have been used [1–5]. Prosthetic rings have also
been used when necessary [4]. Regardless of the method
used, feasibility for repair would depend on the presence
of an anterior leaflet of adequate size. Septal and posterior leaflets have no importance for repair, and are
frequently absent or severely dysplastic. We here report
on seven patients in whom successful tricuspid annuloplasty, associated with a longitudinal plication of the
atrialized portion of the right ventricle, was performed.
Material and Methods
Since 1987, 7 patients with the diagnosis of Ebstein’s
malformation of the tricuspid valve have been admitted
for surgical repair. Mean age was 12 years (range, 7 to 16
years). All were cyanotic, in New York Heart Association
functional class II to III, with severe tricuspid regurgitation. None had previous episodes of thromboembolism.
Two patients had histories of episodes of a nonspecific
type of supraventricular tachycardia. Procedures for
Accepted for publication Feb 6, 1998.
Address reprint requests to Dr Vargas, Pediatric Cardiac Surgery, Hospital Italiano, San Martin 1353, 1828 Banfield, Buenos Aires, Argentina.
© 1998 by The Society of Thoracic Surgeons
Published by Elsevier Science Inc
Surgical Technique
On cardiopulmonary bypass temperature was lowered to
28°C and the aorta cross-clamped after cardioplegia was
administered (Fig 1). A previous external inspection of
the atrialized portion of the right ventricle was made to
rule out the presence of a coronary artery branch.
Through a right atriotomy running parallel to the atrioventricular sulcus, the degree of mobility of the anterior
leaflet was assessed by injecting cold saline solution
under pressure into the ventricular cavity with a bulb
syringe. If leaflet excursion seemed adequate, a 4-0
polypropylene pledgeted suture was placed at the anterior-posterior commissure. When the posterior leaflet
was absent, and therefore no such commissure was
identified, this suture was passed through the corresponding part of the anterior leaflet only. From here, this
suture was passed through the septal leaflet tissue (or its
remnants if hypoplastic) at the level of the posteriorseptal commissure. Another piece of Dacron pledget was
then placed at this point for reinforcement, and the
suture was tied up. An almost monocuspid valve provided by the anterior leaflet was created. The resulting
annulus size was calibrated with the valvular probe that
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VARGAS ET AL
EBSTEIN’S MALFORMATION
Ann Thorac Surg
1998;65:1755–7
Fig 1. Surgical technique. (A) Through a right atriotomy, the abnormally implanted tricuspid valve, together with a zone of thin atrialized
ventricular wall (shaded area), is exposed. (B) A pledgeted suture that runs from the anterior-posterior commissure to the posterior-septal
commissure (or a septal leaflet remnant) produces the annuloplasty by approximating both points. A monocusp valve is fashioned out of the
anterior leaflet. (C) A cul-de-sac of thin atrialized ventricular wall is created. It is then plicated with a continuous double running suture. A
single suture is used for both annuloplasty and plication. (D) Both monocusp valve and plicature of the atrialized ventricle are seen. Plication
runs longitudinally from the initial annuloplasty site (pledget) to the true tricuspid annulus. Conduction system lies away, anterior to the coronary sinus, in its normal position. (A 5 anterior leaflet; B 5 conduction system; CS 5 coronary sinus; P 5 posterior leaflet; S 5 septal leaflet.)
corresponded to the predicted tricuspid valve annulus
for the patient’s age and height [6] to minimize the risk of
creating iatrogenic stenosis. Valve competence was then
tested by saline solution injection into the right ventricle
under pressure. At this point, most of the atrialized
noncontractile portion of the right ventricle became a
cul-de-sac, longitudinally oriented between the previously mentioned suture and the true tricuspid annulus.
This area was then plicated by using both ends of the
suture, with a double continuous running suture ending
at the level of the tricuspid annulus. Care was taken to
maintain this plication suture within the intramural tissue of this thin-walled chamber. After the valve was
tested again, the atrial septal defect was closed, the right
atriotomy was closed, and the aorta was unclamped.
At operation, the posterior tricuspid valve leaflet was
found absent in 4 patients, and the septal leaflet was
underdeveloped in 3 and was represented only by a
fibrous remnant. Operation was performed in all, using
the technique described. To obtain adequate mobilization of the anterior leaflet, resection of several fibrous
bands in its ventricular surface was necessary in 2 patients.
Results
The postoperative course was uneventful. Mean follow-up for the series is 4.3 years (range 1 to 10 years). All
patients are in New York Heart Association functional
class I, free of medication and have no clinical evidence
of tricuspid incompetence or arrhythmias. Twodimensional echocardiographic and color Doppler echocardiographic studies have shown absence of either valve
incompetence or stenosis. Adequate coaptation of the
anterior leaflet with the septal leaflet or its remnant was
the common finding for all. It was always easy to identify
the refringent area of plicature of the atrialized ventricular wall opposite to the septum. (Fig 2).
Comment
Most patients with Ebstein’s malformation of the tricuspid valve can now be treated with reconstructive proce-
Ann Thorac Surg
1998;65:1755–7
Fig 2. Postoperative two-dimensional echocardiogram. A monocusp
valve provided by the anterior leaflet is shown both opened (A) and
in closed position (B). Adequate leaflet coaptation with the septum
(septal leaflet remnant) is displayed. The area of plicature is seen
opposite to the septum (arrow). (AP 5 area of plicature; LV 5 left
ventricle; RV 5 right ventricle.)
dures [1–5]. Valve replacement should probably be confined to a small group of patients in whom the anterior
tricuspid valve leaflet cannot be mobilized enough to
meet the septal structures.
The largest experience in this regard has been reported
by the Mayo Clinic. Danielson and colleagues [2, 3] have
standardized the use of a combined annuloplasty with
transverse plication of the atrialized chamber, reporting
large series and excellent results. Carpentier and associates [4] reported successful repair even for patients in
whom the functional right ventricle was minute and the
VARGAS ET AL
EBSTEIN’S MALFORMATION
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tricuspid valve extremely dysmorphic. In their experience, plication of the atrialized portion of the right
ventricle was performed along the longitudinal axis of
the heart. Both anterior and posterior leaflets were detached and repositioned at the level of the tricuspid
annulus, leaving the area of plication included within the
right ventricular chamber. The creation of a larger ventricular chamber was proposed as an advantage of this
repair [4]. A valve ring annuloplasty was an additional
variant included in the procedure.
This repair has also been advocated by Quaegebeur
and colleagues [5], but without a valvuloplasty valve ring
as part of the repair.
In our view, no definitive rationale supports a clear
advantage of using a longitudinal versus a transverse
plicature of the atrialized ventricle. In both, a part of the
thin-walled chamber is excluded. It is also unclear
whether attempts to restore the shape of the right ventricle by including this plicated area beyond the translocated valve (ie, within the right ventricle), as proposed by
Carpentier and associates [4], would be of fundamental
importance. We believe it is doubtful that this plicated
area of thin muscle could be of significance in right
ventricular performance postoperatively. Perhaps a successful result will rely mainly on an effective treatment of
tricuspid valve regurgitation rather than on the type of
plicature used for repair.
The technique we have used in our patients seems to
fulfill both requirements by performing a successful
tricuspid annuloplasty together with the exclusion of a
part of the atrialized ventricular wall with a plicature. We
found it very reproducible with predictable results and
without postoperative arrhythmias. Both annuloplasty
and plication can be performed by using a single suture.
The long-term results obtained have encouraged us to
continue using this procedure for repair of Ebstein’s
malformation of the tricuspid valve.
References
1. Hardy KL, May IA, Webster CA, Kimball KG. Ebstein’s
anomaly: a functional concept and successful definitive repair. J Thorac Cardiovasc Surg 1964;48:927– 40.
2. Danielson GK, Maloney JD, Devloo RAE. Surgical repair of
Ebstein’s anomaly. Mayo Clin Proc 1979;54:185–92.
3. Danielson GK, Fuster V. Surgical repair of Ebstein’s anomaly.
Ann Surg 1982;196:499 –504.
4. Carpentier A, Chauvaud S, Mace L, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve.
J Thorac Cardiovasc Surg 1988;96:92–101.
5. Quaegebeur JM, Sreeram N, Fraser AG, et al. Surgery for
Ebstein’s anomaly: the clinical and echocardiographic evaluation of a new technique. J Am Coll Cardiol 1991;17:722– 8.
6. Shultz DM, Giordano DA. Hearts of infants and children.
Arch Pathol 1962;74:464–71.