Research Article
One-Stage Correction of Complex Aortic Arch and Descending Aorta Dysplasia Combined Intracardiac Anomalies with a Median Incision
Mingwen Li, Yingbin Xia and Lin Chen*
Department of Cardiovascular Surgery, Xinqiao Hospital, China
*Corresponding author: Lin Chen, Department of Cardiovascular Surgery, Xinqiao Hospital, 7F, Second in-Patient Building, 183 Xinqiao St, Shapingba District, Chongqing 400037, China
Published: 22 Oct, 2018
Cite this article as: Li M, Xia Y, Chen L. One-Stage
Correction of Complex Aortic Arch and
Descending Aorta Dysplasia Combined
Intracardiac Anomalies with a Median
Incision. Clin Surg. 2018; 3: 2173.
Abstract
>Objectives: A congenital aortic arch and descending aorta dysplasia with intracardiac anomalies
are rare cardiovascular malformations, and there is some controversy regarding the ideal surgical
response. Our objective is to report our experiences regarding the one-stage correction for these
complex cardiovascular anomalies in adolescent and adults.
Methods: The clinical data were analyzed retrospectively from 49 patients who underwent surgery
for these complex anomalies. Surgical treatments for aortic malformations include widening of the
aortic arch and descending aorta in 31 patients (Widening group) and ascending aorta-Gore Tex
graft-descending aorta bypass in 18 patients (Bypass group).
Results: There was one early death each in the Bypass group and the Widening group. There were 2
patients with early hoarseness and 2 patients with chylothorax in the Widening group. The change
in the systolic pressure gradients between the upper and lower limbs was less than 10 mmHg in all
patients within one year of follow-up. Conclusions Median sternotomy and concomitant surgical
treatment for aortic arch and descending aorta dysplasia associated with intracardiac anomalies are
safe and feasible. Gore-Tex vascular graft bypass was a good choice for patients with interrupted
aortic arch, distal aortic arch dysplasia, or obvious vascular calcification in adolescents and adults.
Keywords: Aortic arch; Descending aorta dysplasia; Intracardiac abnormalities; Surgical
treatments
Introduction
Congenital complex aortic arch with descending aorta dysplasia is a rare cardiovascular malformation. It is mainly divided into the following three types: simple aorta coarctation, aorta coarctation with isthmus hypoplasia, and aorta coarctation with aortic arch dysplasia. Simple aorta coarctation has good surgical outcomes. Interventional balloon dilation and stents can be used [1-7]. The other two types of aorta coarctation are complicated and have poor surgical outcomes. Treatment for patients with aorta coarctation associated with intracardiac anomalies is still controversial [7-9]. In this study, we describe and discuss our experiences with median sternotomy and concomitant surgical treatment against complex aortic arch and descending aortic dysplasia associated with intracardiac anomalies in forty-nine patients within a single institute.
Materials and Methods
Patients
Forty-nine patients were included in this study, consisting of 28 boys and men and 21 girls
and women ranging in age from 12 to 33 years. The patients included 11 cases of interrupted aortic
arch and 38 cases of aortic arch and descending aorta dysplasia (with coarctation segments longer
than 4 cm). The intracardiac complications included 35 cases of ventricular septal defect, 5 cases
of bicuspid aortic valve with stenosis, 4 cases of right ventricular outflow tract obstruction or
double-chambered right ventricle, 2 cases of atrial septal defect, 1 case of pulmonary stenosis, 1
case of mitral stenosis, 1 case of aortic stenosis with mitral stenosis, and 30 cases of patent ductus
arteriosus. Clinical manifestations included exertional dyspnoea, shortness of breath, intermittent
lower limb weakness, occasional chest pain, lower blood pressure in the lower limbs than in the
upper limbs, thin and impalpable femoral artery and dorsalis pedis artery, systolic or continuous
murmur at the left sternal border between the 3rd and 4th intercostal spaces, and loud P2. Twenty
seven patients showed differential cyanosis. Chest X-ray examination
showed bilateral pulmonary congestion and prominent pulmonary
artery segmentation. ECG showed left ventricular hypertrophy.
Echocardiography showed corresponding heart malformations,
which were confirmed by surgery (Table 1), and invisible descending
aortas. CT aortic angiography clearly showed aortic arch and
descending aortic dysplasia (Figure 1-4) and rich collateral circulation.
Intraoperative exploration showed left ventricular dilation and
hypertrophy, right ventricular enlargement, pulmonary artery
thickening, diffusive coarctation at the distal aortic arch, descending
aortic dilation after coarctation, and thin and fragile tissue and vessel
walls. Each participant enrolled in our study was assigned to the
Widening Group or Bypass Group based on their standard treatment
directed by the surgeon. Our study was approved by the institutional
research ethics committee of Xinqiao Hospital. All the participants in
our study provided written informed consent. Additionally, we had
obtained written informed consent from the statutory guardian of the
minors enrolled in our study.
Surgical approaches
Surgery was performed under intravenous an aesthesia. Surgical
procedures included median sternotomy, longitudinal incision of
the pericardium, and preparation of a pericardial patch. The patients
were divided into two groups based on the surgical approaches
widening group (widening of the aortic arch and descending aorta
in 31 patients) and Bypass group (ascending aorta-Gore Tex graftdescending
aorta bypass in 18 patients).
(1) In the Widening group comprising 31 patients with long
aortic arches and descending aortic dysplasia, we carefully separated
the coarctation segment of the descending aorta along the aortic arch
from the normal sites, which were 2 cm to 3 cm above the coarctation
segment. Once the separation was complete, the conventional
cardiopulmonary bypass was set up. When extracorporeal circulation
was cooled down to 18°C, and the subject underwent circulatory
arrest, the coarctation segment was incised longitudinally, widened
with blood vessel grafts and lined externally with strips of autologous
pericardium to prevent blood vessel rupture. We also added one layer
of strips of autologous pericardium to the lumens of the blood vessels
to prevent post-operative bleeding. Next, we exhausted and restored
circulation, warmed the patient up slowly, perfused the heart with
cardiac arrest fluid, and completed the correction of the anomalies.
(2) In the Bypass group comprising 7 patients who had long distal
aortic arch dysplasia and rich collateral circulation in CTA and 11
patients who had interrupted aortic arches, we carefully separated the
descending aorta from the lower end through the extra pleura, which
was 3 cm to 4 cm above the normal area, set up the conventional
cardiopulmonary bypass, placed the aortic cannula on the right side
of the aorta and as close as possible to the aortic arch, reduced the
patient’s systemic temperature to 18ºC, and performed Gore-Tex
bypass graft replacement with circulatory arrest. First, we performed
end-to-side anastomosis between the Gore-Tex vascular grafts and
normal descending aorta distal from the coarctation. The anastomosis
was continuously sutured with a 5/0 prolene line and was lined and
tightened with stripped autologous pericardium. We next restored
the cardiopulmonary bypass, allowed perfusion, and warmed up the
blood. After expulsion of gas from the vascular graft and clamping of
the said graft, we clamped the ascending aorta as needed depending
on the disease status, allowed aortic root perfusion with cold cardiac
arrest solution, and corrected the intracardiac anomalies. Finally, we
performed end-to-side anastomosis between another end of the Gore-
Tex vascular graft and normal ascending aorta and restored aortic
flow. In five patients in this group, the coarctation segment was too
long for the normal part of the descending aorta to be fully exposed
through extra pleura; thus, we opened the pleura, bypassed through
the left lung using long Gortex vascular grafts, and performed endto-
side anastomosis with descending aorta above the diaphragm. The
same procedure was used for the proximal end.
Concomitant surgical treatments for intracardiac anomalies
included ventricular septal defect correction in 35 patients, aortic
valve replacement in 4 patients, dredging and widening of the right
ventricular outflow tract in 5 patients, atrial septal defect correction
in 2 patients, mitral valve replacement in 1 patient, multi-valve
replacement in 1 patient, Bent all procedure in 1 patient, and patent
ductus arteriosus closure in 30 patients. Before the restoration of
circulation and perfusion, we performed superior vena cava retrograde
perfusion with 500 ml of arrest solution. This was completed in 3 min
to 5 mins to allow gas to be expelled and prevent air embolism in the
brain and other vital organs. After surgery, all patients were given
warfarin for 3 months to prevent thrombosis.
Measurement of peripheral oxygen saturation
Changes in the systolic pressure gradients between upper and
lower limbs in 47 surviving patients before the operation, 1 day postoperation,
and 30 days post-operation, 6 months post-operation, and
1 year post-operation were analyzed.
Statistical analysis
The Statistical Package for the Social Sciences (SPSS) version 11.0
was utilized for to data analysis. The count data were assessed by χ2
test. All data were reported as the means ± standard deviation. P<0.05
was considered statistically significant.
Table 1
Figure 1
Table 2
Table 2
Changes in the systolic blood pressure of the upper and lower limbs within one year of surgery in 47 surviving patients (± s mmHg).
Table 3
Table 3
Changes in the systolic pressure gradients between the upper and lower limbs within one year of surgery in 47 surviving patients (± s mmHg).
Results
The surgical approaches are listed in (Table 1). One patient died during the early stages of surgery due to low cardiac output syndrome in the Bypass group, and one patient died during the early stages of surgery due to respiratory function failure in the Widening group. No deaths occurred late in the two groups. There were 12 cases (38.71%) with complications in the Widening group, including 4 patients with low cardiac output syndrome, 2 patients with respiratory function failure, 2 with multi-organ failure, 2 with early hoarseness, and 2 with chylothorax. One patient required reoperation because of bleeding. In the Bypass group, 5 patients (27.78%) with complications that were lower than those in the Widening group comprised 2 patients with low cardiac output syndrome, 1 patient with respiratory function failure and 1 patient required reoperation because of bleeding. All of them were cured and discharged after treatment. We performed follow-up for 3 months to 9 years, and all patients were satisfied with the outcome and had a good quality of life. The changes in the systolic pressure gradients between the upper and lower limbs were less than 10 mmHg in all patients within one year of surgery, and there was no significant difference in the Bypass group compared with that in the Widening group (Table 2,3). All adult patients showed high blood pressure, and CTA follow-up showed good graft flow, no anastomotic strictures, no aneurysms, and no thrombosis (Figure 1-4). Further study is required to confirm these long-term effects.
Discussion
Congenital complex aortic arch associated with descending aortic
dysplasia is a rare cardiovascular malformation. It is divided into the
following three types: simple aortic coarctation, aortic coarctation
with isthmus hypoplasia, and aortic coarctation with aortic arch
dysplasia. Surgical treatment for simple aortic coarctation is easy and
effective. Interventional balloon dilation and stents are commonly
used. The other two types of aortic coarctation are complicated, and
both surgical and interventional approaches are difficult to apply. It
is more difficult to treat when they are associated with intracardiac
anomalies, and treatment strategies are still controversial [7-11].
Selection of surgical approach
The conventional surgical approaches for simple aortic
coarctation include left posterior lateral incision, excision of the
coarctation segment through the fourth intercostal space, and end-toend
anastomosis. It can also be corrected with incision and widening
at the coarctation segment, which has good exposure. This technique
requires only that the surface of the body be cooled and does not
require cardiopulmonary bypass or deep hypothermic circulatory
arrest. It is simple, and the outcomes are good. Interventional balloon
dilation and stents also have good effects [1-7].
Due to the complicated pathophysiology and haemodynamics,
there is no standard surgical approach to aortic coarctation associated
with complications such as isthmus hypoplasia, aorta arch dysplasia,
and interrupted aorta arch or intracardiac anomalies [8]. Common
surgical strategies include staging surgery, single-stage surgery with
two incisions, and single-stage surgery with a median incision [11-
13]. Staging surgery includes left posterior lateral incision to correct
aortic anomalies and selective surgery for intracardiac anomalies.
Single-stage surgery with two incisions includes left posterior lateral
incision to correct aortic anomalies and concomitant correction of
intracardiac anomalies after changing the posture of the patient. Both
methods have good exposure in the surgical field. However, patients
must undergo these painful and risky procedures twice. Single-stage
surgery with median sternotomy has relatively poor exposure in the
surgical field and poorer parameters than single-stage surgery with two
incisions with respect to the cardiopulmonary bypass time, duration
of ICU stay, mortality rate, and re-coarctation rate. However, it is
the best choice for newborns and infants with aortic arch dysplasia
[11-13]. It remains controversial whether this method is appropriate
for adolescents and adults [12,13]. Our experiences suggest that, in
adolescents and adults, the median sternotomy approach can be used
to successfully complete the correction with fewer incisions and a low
re-operation rate. It may also be easier for patients to accept.
Surgical strategies
Surgical treatment is the best approach for the treatment of
descending aortic dysplasia associated with intracardiac anomalies.
It improves the natural prognosis. However, no surgical strategy is
universally accepted. In end-to-end anastomosis, there is usually
insufficient vascular anastomosis, and anastomotic stricture may
occur. Aneurysms may occur after patch widening [3,8,14].
In the present study, we chose surgical approaches in accordance
with patient-specific conditions. For patients with aortic coarctation
and large-area isthmus hypoplasia, patients with concomitant aortic
arch dysplasia but mild coarctation, and young patients, we selected
longitudinal incision and Gore-Tex vascular graft plus autologous
pericardium to widen the narrow section of the aortic arch. Patch
widening can reduce the risk of post-operative stenosis [8]. Lining
with autologous pericardium can prevent aortic wall rupture and
post-operative bleeding. Gore-Tex vascular grafts have good elasticity
and plasticity and are less prone to post-operative aneurysm [15].
For patients with interrupted aortic arches, distal aortic arch
dysplasia, long coarctation segments, or obvious vascular calcification
and older children and adults, we adopted a Gore-Tex vascular graft
bypass (Figure 1). First, we performed end-to-side anastomosis
between the Gore-Tex vascular graft and normal descending aorta
distal from the coarctation, and the anastomosis was continuously
sutured with 5/0 prolene line, tightened and lined with strips of
autologous pericardium. Next, we restored the cardiopulmonary
bypass, allowed perfusion, and warmed up the blood. After
expelling gas from the vascular graft and clamping the said graft, we
performed end-to-side anastomosis between the Gore-Tex vascular
graft and normal ascending aorta. We only performed end-to-side
anastomosis between the vascular graft and normal descending
aorta with circulatory arrest. This can decrease the total surgical time
and facilitate good anastomosis. Lining with stripped autologous
pericardium can prevent vessel rupture. Unlike widening between
the descending aorta and distal aortic arch, complete separation of
the lateral and posterior walls of the aorta was unnecessary. This
left a smaller wound area, a lower chance of chylothorax, and intraoperative
bleeding due to the rich collateral circulation and vessel
wall fragility. If the coarctation segment was too long for full exposure
of the normal part of the descending aorta through the extra pleura,
we opened the pleura, bypassed the left lung using long Gortex
vascular grafts and performed end-to-side anastomosis above the
diaphragm (Figure 2). In this way, we prevented deficits in the length
and tension of the graft vessels and prevented poor outcomes caused
by lung expansion. This procedure was used in all five patients in this
group, to good effect. We then performed end-to-side anastomosis
between the vascular graft and ascending aorta after restoring
cardiopulmonary bypass and perfusion, warming the blood, and
blocking the ascending aorta. This can reduce the circulatory arrest
time and cardiopulmonary bypass time and reduce their effects on
the body. End-to-side anastomosis allows the physician to choose
large vessel grafts, which reduce the rate of stenosis and avoid the
problem of matching. During cannulation of the ascending aorta, the
cannulation should be placed to the right of the blood vessel and as
close as possible to the aortic arch. This provides sufficient space to
establish anastomosis between the vascular graft and ascending aorta.
The correction of intracardiac anomalies can be performed after
the lesion vessels are separated and the cardiopulmonary bypass is
cooled down or after the aortic malformations are corrected and the
cardiopulmonary bypass is warmed up. The process works well under
either set of conditions. We performed this procedure in 18 patients,
and it was successful in all cases.
In the present study, the changes in the systolic pressure gradients
between the upper and lower limbs were less than 10 mmHg in all
patients within one year of surgery, and there was no significant
difference in the Gore-Tex vascular graft bypass group compared
with that in the Patch widening group. It showed that the Gore-Tex
vascular graft bypass was a good choice for patients with interrupted
aortic arches, distal aortic arch dysplasia, long coarctation segments,
or obvious vascular calcification for adolescents and adults.
Protection of nearby vital organs
In this study, chylothorax occurred in 3 patients, and early-onset
hoarseness occurred in 2 patients, indicating the importance of
protecting important neighboring tissues. The phrenic nerve, vagus
nerve, and recurrent laryngeal nerve pass around the aortic arch
and descending aorta; thus, close attention must be paid to prevent
damage to these nerves. Electrocoagulation-blunt dissection should
be used at low output intensity to prevent indirect thermal damage.
If necessary, the nerves should be traced with a rubber strip. There
are abundant amounts of lymphatic tissue around the aortic arch and
descending aorta, and ligation should be used to reduce the chance of
chylothorax [16-18].
Advantages of concomitant perfusion and cannulation of
the ascending aorta and femoral artery
In adult patients, we performed concomitant perfusion and
cannulation of the ascending aorta and femoral artery because of the
weak tissue of the vessel walls and because of the long surgical time
required for adults. We chose either upper body perfusion or lower
body perfusion under circulatory arrest to shorten the duration of
ischaemia of vital organs and reduce the damage to organ function.
Because this process involves selective perfusion, the surgery can take
as long as required for perfect correction of the deformity, reducing
the chances of post-operative bleeding and other complications. In
this case, we achieved perfect correction of anomalies and reduced
post-operative bleeding. In addition, concomitant perfusion of the
ascending aorta and femoral artery can evenly and quickly cool down
the whole body. This prevents uneven cooling by a single perfusion
and the adverse effects attributable to uneven cooling of the distal end
of the coarctation. It works well in clinical settings.
Significance of retrograde perfusion of the superior vena
cava
To completely expel gas, a superior vena cava retrograde perfusion
was applied in patients. This technique can completely exhaust
intravascular gas and prevent air embolism in the brain and other
vital organs. The perfusion pressure should not be too high. Normally,
it is less than 40 mmHg, and the perfusion volume is normally 500 ml.
The perfusion should be performed within 3 min to 5 mins. After
the retrograde perfusion, arterial perfusion was restored, and the
body was warmed up slowly. No brain complications occurred in this
group of patients.
Figure 2
Figure 3
Figure 4
Conclusion
In summary, a complex aorta arch and descending aorta dysplasia associated with intracardiac anomalies are complicated situations. Median sternotomy and concomitant surgical treatment are safe and feasible. A Gore-Tex vascular graft bypass was a good choice for patients with interrupted aortic arches, distal aortic arch dysplasia, long coarctation segments, or obvious vascular calcification for adolescents and adults. No recurrence was observed during followup, and no related complications occurred.
Acknowledgement
The authors would like to thank all the doctors and nurses in our
department.
Funding Statement Supported by National Natural Science
Foundation of China (NSFC, 81170216).
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