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Aortic Root Involvement in Congenital Heart Defects
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anterior leaflet of the tricuspid valve (infundibular subarterial defect), so that risk of complete
heart block at the time of surgical closure is small. The other 20% of patients have a
perimembranous infundibular defect, the posterior margin of which extends back to the
anterior leaflet of the tricuspid valve. In rare cases, when the ventricular septal defect is very
small, closure can create left ventricular outflow tract obstruction [3, 4].
Often the origin of the coronary arteries is abnormal, there is wide variability of coronary
arterial ostial location, number, angle of takeoff and degree of patency.
Lenox et al. published a high incidence of coronary ostial and arterial abnormalities in a
study of 30 pathologic specimens of truncus arteriosus. They found following types of
abnormalities: left coronary ostium in a posterior and high position; close relation of the left
coronary ostium to the pulmonary artery segment in three-leaflet truncal valves; stenosis of
the coronary ostium or the location of the ostium above or in a commissure; the acute angle
takeoff of the coronary artery; the position of the left anterior descending artery as it courses
posteriorly and close to the truncal wall; the size and course of the conal and diagonal arteries
from the right coronary artery across the right ventricular outflow area; a single coronary
artery or ostium with branches crossing the right ventricle below the truncus, the circumflex
arising from the right coronary artery and coursing behind the truncus, and the right coronary
artery originating from the left anterior descending artery. The ostial location of the coronary
arteries can be located very close to the truncal valve commissures and to the origins of the
right and left pulmonary arteries [5]. There are usually two coronary arteries, but a single
coronary artery is not uncommon. Sometimes the left coronary artery originates high from the
posterior wall of the truncus [3]. Although the distal branching of the coronary arteries is
usually normal. The anomalous anterior descending coronary artery can originate from the
right coronary artery and run across the infundibulum of the right ventricle, like in tetralogy
of Fallot [6, 7, 8, 11]. Surgeons should keep in mind that the coronary arteries can be located
essentially anywhere around the truncal root.
The aortic arch is left-sided in about 60%, right-sided in about 25-30% and interrupted in
about 10%-15% of cases of persistent truncus arteriosus. The interruption is usually of type B
(interruption distal to the origin of the left carotid artery). In up to 90% of patients with
truncus arteriosus, a DiGeorge syndrome can be found [9, 10].
Truncal Valve
The truncal valve has a variable number of leaflets and variable morphology. The number
of cusps varies from two to six. Rarely there are more than four individual cusps [2]. The
truncal valve is tricuspid in approximately 50%, bicuspid in about 30% and quadricuspid in
about 15 – 20% of cases of persistent truncus arteriosus. In exceptional cases, the valve is
unicuspid. Various degrees of truncal valve dysplasia with abnormal leaflets and inadequate
commissural support are often present, resulting in important truncal valve regurgitation. It is
rare for a truncal valve to be structurally stenotic. There is always continuity between the
truncal valve and the mitral valve. The truncal valve overrides the ventricular septal defect
and relates equally to the two ventricles in about 50% of cases. In the other cases, the truncal
valve originates predominantly from the right ventricle but in some instances from the left
ventricle [1, 3].
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Methods
Surgical Repair of Truncal Valve Regurgitation
Truncal valve regurgitation remains a challenging problem and estimated to occur in 25–
50% of patients with truncus arteriosus.
The presence of truncal valve regurgitation usually evaluate by preoperative transthoracic
echocardiography and colour Doppler imaging using standard echocardiographic criteria,
including M-mode assessment of left ventricular function, diastolic flow reversal in the
descending aorta (mild = brief diastolic reversal flow; moderate = intermediate diastolic
reversal flow; severe = holodiastolic reversal flow), colour-flow regurgitant jet size (mild =
small central jet <25% of left outflow tract; moderate = intermediate jet greater than mild but
no signs of severe; severe = large central jet >65% of left outflow tract) and measurements of
the truncal regurgitant jet width: annulus ratio (mild < 25; moderate = 25–64;
severe ≥65).[15]. Traditional strategies for the operative management of these patients have
included temporization of this problem or attempts at valve replacement with a homograft
valve or a mechanical prosthesis. However, none of these options have been shown to be very
successful or desirable. Thus, repair of the moderate or severe regurgitant truncal valve
constitutes the optimal surgical strategy, particularly in the neonatal period [12, 13].
The significant regurgitant truncal valve is almost always amenable to various repair
techniques. The reported techniques can be summarized as approximation, resection, or
extension of the leaflets, remodeling of the valvar support, and external annuloplasty and etc.
In this chapter we try to describe the techniques of surgical repair of the truncal valve
insufficiency currently used by pediatric cardiac surgeons.
In case when regurgitation of truncal valve seemed to be peripheral at the commissures
can be used a commissuroplasty, as described by Trusler, to suspend a prolapsed cusp (Figure
2) [14].
Naimo et al. describe a subcommissural annuloplasty in patients with a large truncal
valve annulus and relatively normal leaflets by placement of 5-0 Ti-Cron (Medtronic,
Minneapolis, MN, USA) sutures pledgeted with autologous pericardium to the
subcommissural region of the truncal valve. If the sino-tubular junction and annulus were
dilated, they were also plicated with a full-thickness pledgeted suture. (Figure 3) [15].
Another technique can be used is the approximation technique, based on the fact that many
patients will have a quadricuspide valve. The valve can be converted to a trileaflet or bileaflet
structure by approximating one or two of the cusps (Figure 4) [16].
Figure 2. Commissuroplasty and commissural suspension performed for prolapsing leaflet.
Aortic Root Involvement in Congenital Heart Defects
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Figure 3. Subcommissural annuloplasty.
Figure 4. Approximation of the leaflets for correction of truncal valvar insufficiency. (A) Quadricuspid
truncal valve with a deficient leaflet. (B) Repair is accomplished using leaflet union to create either a
tricuspid or bicuspid valve. (C) Additional commissuroplasty sutures are utilized as needed to provide
additional support at the commissural posts.
Figure 5. Cusp resection and annular reduction. (A) The quadrileaflet truncal valve with one prolapsed
leaflet. (B) Resection and remove of the smaller of the four leaflets (C) Resection of the truncal valve
sinus. (D) Remodelling of the annular support with pledget-based sutures to reduce the diameter of the
outflow tract. The truncal wall is closed with running polypropylene suture in two layers.
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Imamura et al. described other technique to resect the leaflets with tricuspidisation of a
quadricuspid truncal valve. The smallest/incompetent leaflet is excised, along with the
corresponding segment of the annulus. The remaining annular edges are sutured together to
approximate the size of the ascending aorta (Figure 5) [17].
The same procedure performed in a patient in whom the prolapsing small leaflet arises
from a truncal sinus that gives rise to a coronary artery (Figure 6).
Those working at the Cleveland Clinic have also reported a valvoplasty technique for
insufficient aortic valves but this type of repair is suitable even in case of truncal valve
insufficiency with presence of one prolapsed cuspid. They performed surgery on 28 patients,
threequarters with bifoliate valves. They carried out triangular resection of the free edge of
the prolapsing leaflet, coupled with plication of the peripheral attachments of the zones of
apposition between the leaflets at the sinutubular junction (Figure 7) [18].
Figure 6. (a) A coronary arterial button has been excised, and the leaflet being removed. (b) The
coronary arterial button has been reimplanted into the adjacent sinus of the truncal root. The annular
support of the leaflets has been remodeled with pledget-supported sutures, and the truncal wall is being
closed. (c) The completed result.
Figure 7. Aortic valvoplasty technique. Upper panel, a triangular shaped wedge of the prolapsed leaflet
is excised (dashed lines). The leaflet is then reapproximated with interrupted polypropylene suture. The
left lower panel shows the prolapsed leaflet, causing aortic insufficiency, which is then corrected by the
wedge resection, resulting in a competent valve (right lower panel).
In some cases when insufficiency of the truncal valve with normal anatomy of the cusps
caused only by dilation of the annulus, to improve central coaptation a circumferential
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annuloplasty can be used. The subvalvar suture is tied over an appropriate-sized probe to aid
in reduction annuloplasty (Figure 8) [16].
Figure 8. (A) Subvalvar annuloplasty technique. (B) Lateral view of repaired valve.
Figure 9. Surgical technique of extending aortic leaflets with fresh autologous pericardium.
Another technique of repair of the truncal valve is the extension of leaflets with
pericardium was described. The principle is to utilize glutaraldehyde-preserved autologous
pericardium to augment the valvar leaflets. Pericardial strips are sutured to the leading edge
of the leaflets, and anchored to the aortic wall. In general, the strips are tailored so as to be
slightly redundant, thus allowing approximation of the leaflets (Figure 9) [19, 20, 21].
In cases of inability to preserve the truncal valve, only unique solution is its replacement
with a mechanical prosthesis or with a homograft [22, 23].
Other surgical techniques such as leaflet-base-preserving truncal valve repair with
ethanol-treated autologous pericardium or aortic valve neocuspidization (Ozaki) procedure,
recently been published. Until midterm results are available, however, these procedures
cannot be recommended for the truncal valve repair [24, 25].
Discussion
Outcomes of Truncal Valve Surgery and Current Perspestives
Truncal valve regurgitation remains a risk factor for early and late morbidity and
mortality of patients with truncus arteriosus. Previous studies have reported initial moderate
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or severe truncal valve insufficiency as a risk factor for truncal valve reoperation [26, 27]. A
review of the Society of Thoracic Surgeons Congenital Heart Surgery Database results,
reporting 572 truncus arteriosus repairs from 2000 to 2009, included 27 patients with truncal
valve surgeries (23 at truncus arteriosus repair, 4 later). The mortality of truncus repair with a
concomitant truncal valve procedure (30%) was significantly increased compared with
controls without truncal valve surgery at truncus arteriosus repair (10%, p = 0.0002), while all
4 patients who had delayed truncal valve procedures died. The association of truncus
arteriosus repair, IAA repair and TVR had a mortality of 60%. Postoperative mechanical
circulatory support was utilized in 45 patients (7.8%) in the overall cohort, and was
significantly more common in the truncal valve surgery group versus the isolated common
arterial trunk repair group (18% versus 7%, p = 0.002). Postoperative length of stay was also
longer in the common arterial trunk plus truncal valve surgery group (Table 1) [28].
The association of truncal valve insufficiency with mortality risk has been noted by
multiple authors. The report by Ebert and colleagues in 1984 of 100 patients with common
arterial trunk undergoing repair in the first 6 months of life revealed a mortality rate of 11%.
In that series, 8 of the 11 deaths had evidence of preoperative truncal valve insufficiency; 1
patient underwent truncal valve replacement [29]. Hanley and colleagues published a series of
63 patients undergoing repair of common arterial trunk at Children’s Hospital Boston. They
found that severe truncal valve insufficiency before surgery was a significant risk factor for
early death in both univariate and multi- variate analyses [30]. Rajasinghe and colleagues
reported the long-term follow-up of patients undergoing repair of common arterial trunk in
infancy and found severe truncal insufficiency to be a risk factor for late death among initial
hospital survivors (n = 27, 30% mortality) [31]. Di Donato and colleagues from the Mayo
Clinic found moderate or severe truncal valve regurgitation to be associated with poor long
term survival in a study of 167 patients over a 17-year period (n = 62, 50% mortality) [32].
Pearl and associates at the University of California, Los Angeles, reviewed their experience
of 32 patients who underwent common arterial trunk repair in infancy and similarly
concluded that truncal valve insufficiency is an incremental risk factor for early and late
mortality [33].
Table 1. Outcomes (CAT- common arterial trunk; IAA- interrupted aortic arch;
TVS - truncal valve surgery)
In contrast to these reports, some recent single-center series have not shown truncal valve
regurgitation to be associated with increased mortality at the time of primary repair. Bove and
associates reported a series of 46 neonates undergoing repair of common arterial trunk at the
University of Michigan. Five patients required truncal valve replacement, but neither truncal
valve regurgitation nor truncal valve replacement was related to death. A recent report by
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Hawkins and associates showed that in their institution’s experience (n = 42), complex cases
with truncal valve insufficiency, interrupted aortic arch, or both, were associated with greater
utilization of resources but not with a higher rate of operative mortality [34].
Myers and colleagues published a series of 36 patients underwent truncal valve repair
during correction of common arterial trunk at Children’s Hospital Boston from 1997 to 2012.
Valve repair improved regurgitation in 31 of 36 repairs (86%) and was less than moderate in
27 patients (75%) after repair. There were 3 early deaths (8%), all of which were in neonates.
During a mean follow-up of 38.3 ± 44.9 months (range 1 month—15 years), there was 1 late
death, 16 patients required reoperation on the truncal valve and 1 required a second
reoperation. Freedom from reoperation for truncal valve insufficiency was 91.4 ± 4.8% at 1
year, 87.2 ± 6.1% at 2 years, 55.0 ± 10.4% at 5 years and 22.9 ± 12.2% at 10 years (Figure
10) [27].
Figure 10. Kaplan–Meier estimates of freedom from truncal valve reoperation after truncal valve repair.
(A-Entire cohort).
Figure 11. Kaplan–Meier estimates of freedom from truncal valve reoperation after truncal valve repair.
(B-Analysis stratified by age).
In that series, 22 patients had a quadricuspid, 13 a tricuspid and 1 a bicuspid truncal valve
before repair. The Kaplan–Meier survival analysis stratified by age category showed that
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neonates and adults had significantly more reoperations than children (P = 0.039). (Figure 11)
A quadricuspid anatomy after repair tended to have worse freedom from reoperation,
however, not to a significant level (P = 0.15), and tricuspidization also tended towards better
freedom from reoperation (P = 0.19) (Figure 12) [27].
Similarly, Ivanov et al. found that overall freedom from truncal valve reoperation at 10
years was 83.9 ± 5.9 (95% CI 72.2–95.5). Freedom from truncal valve reoperation at 10 years
in patients with initial tricuspid valve morphology was 93.8 ± 6.1 (95% CI 81.9–100.0); with
a quadricuspid valve, it was 66.8 ± 11.6 (95% CI 44.1–89.5) and none of the patients with a
bicuspid valve required reoperation at 8 years. Initial significant truncal valve regurgitation
was associated with a later truncal valve reoperation (hazard ratio 17.2, 95% CI 2.1–145.0;
P = 0.008) (Table 2) [37].
Kaza et al. reviewed their single-centre experience in truncal valve repair in 17 patients
from 1995 to 2008. This study, although on a more limited number of patients, has the
advantage of including 3 non-neonatal patients and follow-up data. Three patients had 1 rerepair, and 1 had 2 re-repairs before undergoing a prosthetic valve replacement at age 13
years. Freedom from reintervention on the truncal valve is 70% at 5 years and 50% at 7 years
after the initial valve repair. Freedom from truncal valve replacement is 100% at 10 years
(Figure 13) [35].
Table. 2. Analysis of variables for truncal valve reoperation
Figure 12. Kaplan–Meier estimates of freedom from truncal valve reoperation after truncal valve repair.
(C- Analysis stratified by truncal valve anatomy).
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Figure 13. Kaplan-Meier graph demonstrating freedom from repeat valvuloplasty.
Henaine and colleagues reported a contemporary review of 153 patients who underwent
repair of truncus arteriosus at Marie-Lannelongue Hospital. This series included 9 patients
who underwent truncal valve intervention at the time of the primary repair (valvuloplasty in
6, valve replacement in 3). There were 4 early deaths in this series, and 2 patients were noted
to have a competent native truncal valve at follow-up. Authors found that freedom from
truncal valve reoperation was 96%, 82% and 63% at 1, 10 and 18 years respectively (Figure
14) [36].
Figure 14. Freedom from truncal valve reintervention after correction of truncus arteriosus.
Naimo et al. focused on 80 patients with truncus arteriosus and truncal valve
regurgitation. Sixty-one (76%) had mild, 17 (21%) had moderate and 2 (2.5%) patients had
severe truncal valve regurgitation in the preoperative echocardiography. Patients with
moderate or severe regurgitation showed more frequently quadricuspid valves. Sixty-three
percent of patients with moderate or severe regurgitation underwent concomitant truncal
valve surgery with a 25% early mortality and 81% of patients required truncal valve
reparation at a median follow-up time of 20 years [15]. Truncal valve insufficiency has been
reported in approximately 25% of truncus arteriosus patients. The optimal surgical approach
to management of truncal valve insufficiency remains controversial.
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The following procedures and guidelines to be helpful:
Trivial or mild truncal valve regurgitation has in general a good outcome without any
concomitant valve surgery.
For moderate to severe truncal valve insufficiency, truncal valve repair is the most
desirable and first choice. Severe regurgitation should be addressed at the primary
valve repair in any age.
Multiple leaflets (more than three) require careful attention. In cases when a
quadricuspid truncal valve is found in truncus arteriosus patients with moderate
truncal valve regurgitation the repair is especially recommended.
The truncal valve replacement for treatment of truncal valve insufficiency remains as
a second option especially at the initial operation.
Anomalies associated with truncus arteriosus such as significant truncal valve
regurgitation, interrupted aortic arch and/or coronary anomalies remain a risk factor
for morbidity and mortality.
The truncal valve repair could be lifesaving and can also be performed in the
neonates without increasing operative mortality but it has limited durability.
Initial moderate or severe truncal valve insufficiency is a risk factor for late truncal
valve reoperation. Truncal valve reintervention procedures can be performed with
good early-term and mid-term results.
The main advantages of truncal valve repair include the natural postoperative valve
hemodynamics and the avoidance of oral anticoagulation. Although they do not exist on the
best truncal valve repair surgery, understanding the mechanisms of truncal valve insufficiency
is believed to be very important for the choice of individualized surgical approach for each
patient.
VENTRICULAR SEPTAL DEFECT AND AORTIC
VALVE REGURGITATION
Elisa Barberi
Introduction
Description
The International Society for Nomenclature of Paediatric and Congenital Heart Disease
(ISNPCHD) has defined ventricular septal defect (VSD) as a congenital cardiac malformation
in which there is a hole or pathway between the ventricular chambers [38]. VSD is a common
congenital heart disease with an incidence of approximately 1.5–6.0 per 1000 newborns.
The scheme proposed by the ISNPCHD classifies VSDs as central perimembranous,
inlet, trabecular muscular, and outlet defects, using a geographic approach as the starting
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