Anterior translocation of THE right pulmonary artery AS THE INITIAL
SURGICAL OPTION FOR A PREMATURE NEWBORN WITH severe bronchopulmonary
dysplasia
Okan Yurdakök MD1, Murat Çiçek MD1, Oktay Korun MD1, Serap Nur Ergor MD
2, Arif Selcuk MD3, Fırat Hüsnü Altın MD1, Filiz Ozyilmaz MD4, Emine
Hekim Yilmaz MD 5, Numan Ali Aydemir MD1, Ahmet Şaşmazel MD1.
- Dr Siyami Ersek Thoracic and Cardiovascular Surgery Training and
Research Hospital, Department of Pediatric Cardiac Surgery,
Istanbul, TR
- Bezmialem University Medical Faculty, Department of Pediatrics,
Newborn Pediatric Intensive Care Unit, Istanbul, TR
- Gaziantep Cengiz Gokcek Obstetrics, Gynecology and Children’s
Hospital, Department of Pediatric Cardiac Surgery,
Gaziantep, TR
- Dr Siyami Ersek Thoracic and Cardiovascular Surgery Training and
Research Hospital, Department of Anesthesiology,
Istanbul, TR
- Dr Siyami Ersek Thoracic and Cardiovascular Surgery Training and
Research Hospital, Department of Pediatric Cardiology,
Istanbul, TR
Short Title: Anterior Translocation of Right Pulmonary Artery for
Bronchopulmonary Dysplasia
Keywords: Prematurity, Bronchopulmonary Dysplasia
Corresponding Author: Okan Yurdakok
Siyami Ersek Hastanesi, Tıbbiye Caddesi,
Uskudar, Istanbul
Email:
okanyurdakok@gmail.com
Fax: +90 (216) 418 96 49
* The authors do not have any financial disclosures.
ABSTRACT
We hereby present a case report of an extremely preterm newborn with
bronchopulmonary dysplasia (BPD), spending more than 4 months of his
early life in the newborn intensive care unit (NICU). The uniqueness of
this case report is the difference in the algorithm used for the
treatment of BPD with regards to the family’s preferences and its
successful outcome.
CASE REPORT
A 37-year-old female, who was previously G2P1A1, had a pre-eclamptic
pregnancy and received steroids 2 days prior to delivery and gave birth
to a very low weight actively crying baby. Apgar score was 7-8 and the
baby was immediately put-on nasal CPAP with a FiO2 of 40%.
The 26 weeks premature newborn, which was weighing 760 grams at birth,
had a rough time in the newborn intensive care unit (NICU). NICU
specialist successfully managed the baby to survive and able to gain
weight up to 3,5 kilograms at the end of 4 months.
In the first 30 days, the baby was stable on full enteral feeding and
was followed on room air according to the BPD
protocol1 but was put on temporary non-invasive
ventilatory support during agitation episodes.
The first significant respiratory distress episode was on day # 70; the
patient was put on non-invasive ventilatory support after the right lung
showed pneumonic infiltration and treated accordingly. While improving
clinically, the patient was intubated after a severe bronchospasm on day
# 79. Blood gases were consistent with respiratory acidosis and the
X-Ray was showing signs of Acute Respiratory Distress Syndrome (ARDS).
The baby was extubated on Day # 88 but followed with non-invasive
respiratory support.
The repeated transthoracic echocardiograms‘ (TTE) did not show any
congenital heart disease or pulmonary hypertension. The baby was on
steroids according to the BPD protocol but was never able to breathe on
room air again. The patient was restless in general and more symptomatic
when crying or agitated.
On Day # 105, the patient suddenly deteriorated when both lungs
collapsed and reintubated. No pneumonic infiltration detected and was
extubated 10 days later but still on continuous respiratory support. The
echocardiogram showed enlarged right heart structures and pulmonary
arteries due to the newly developed pulmonary hypertension and
successfully treated with a maximum dose of selective type 5
phosphodiesterase inhibitor and a short course of milrinone infusion
after pediatric cardiology consultation, but the pulmonary arteries
remain dilated even after treatment.
The repeated worsening episodes without any source of infection or ARDS
make the team to consider the trachea-bronchomalasia (TBM) as a possible
diagnosis and a dynamic flexible bronchoscopy was performed. Two main
bronchuses were showing significant malasia but when the probe was
advanced distally, the left main bronchus was showing signs of
compression from outside.
A contrasted Computerized Tomograhy (CT) scan was planned to rule out
any vascular rings or mediastinal structures, causing compression and
obstruction. The results were consistent with generalized consolidated
and atelectatic areas on both lungs and clear of any mediastinal
abnormalities except the enlarged pulmonary arteries, especially the
right pulmonary artery compressing and almost blocking the left main
bronchus (Picture 1).
After 4 months in NICU with invasive and non-invasive ventilatory
support; the long-term ventilatory support and the tracheostomy options
were discussed exclusively with the team and the family. The family did
not agree with the chronic respiratory support therapy, including
tracheostomy and the home ventilator setting and their main argument was
predicting the difficulty in maintaining proper childcare at home and
they have concerns about handling the ventilator. So, they decided to
proceed with the surgical option if there were any available.
Pediatric cardiologists and surgeons in the same hospital suggested that
aortopexy operation might be a solution to relieve the compression on
the bronchial tree, which was resistant to all medical strategy; and
their suggestion was also supported with the
literature234. The patient was consulted to our
service by the newborn intensivist for a second opinion.
We initially requested to see the CT scan, in case if it was not
evaluated in the light of a pulmonary artery sling anomaly or other
vascular ring anomalies, which is quite common for missing the cause of
recurrent pulmonary infections and difficulty in feeding. But there were
no anomalies detected as stated earlier in the report; significantly
enlarged right pulmonary artery was almost completely obstructing the
left main bronchus soon after the bifurcation.
The options for surgical treatment of BPDs include open aortopexy, or
recently, thoracoscopic aortopexy, tracheal resection and reconstruction
or external stabilization and more recently direct anterior and/or
posterior tracheopexy5. During the investigations in
children with BPDs, it is very important to search for vascular causes
of tracheal collapse with a CT, because vascular compression can be
treated first or simultaneously as this may resolve or mitigate the
severity of the TM. The technique of aortopexy was first used by Filler
et al. in 1976, and it was derived from the operation described by Gross
in 1948 to treat the ”innominate artery compression
syndrome”5. Aortopexy is reported to be effective for
treating tracheomalacia of different origins and other pathological
conditions as well34. The main issue in our case was
basically the significantly enlarged pulmonary arteries and especially
the right pulmonary artery compressing the left main bronchus. Even if
we used the technique for a variety of different indications in the
past, we were not sure if aortopexy will be the right choice to create
enough space for the left main bronchus compressed by the enlarged
pulmonary arteries.
When we evaluate our options; we thought that performing either a
modified LeCompte maneuver or an anterior translocation of right
pulmonary artery will be a better choice to address the true compression
on the left main bronchus, since the main issue was the enlarged
pulmonary arteries due to pulmonary hypertension, accompanying the BPD.
In the literature search, we have reached a few reports of using similar
techniques, but they were all performed concomitant to surgeries for
repair of congenital heart defects67 or for the
bronchial compressions that were presented as a complication of
previously performed surgeries for intracardiac congenital heart or
other kind of defects and repaired afterwards8. There
were not any other reports available in literature search for
application of anterior translocation of the pulmonary as the initial
choice for treating the isolated BPD. And another aspect in the
decision-making process was whether to perform a LeCompte maneuver or an
anterior translocation of the right pulmonary artery for treating the
disease78. Performing a classic LeCompte maneuver will
necessitate a cross (X) clamp application and arresting the heart, which
might be necessary to perform, when there is an enlarged and elongated
aorta that needs to be shortened and reconstructed to create more free
space for the trachea bronchial tree in the mediastinum. Otherwise, when
there is no congenital heart disease in addition to BPD and no need to
open the heart chambers, then it seems like there is no need to put the
baby on unnecessary risk. We thought the anterior translocation of the
right pulmonary artery is a better choice, which can be applied on
cardiopulmonary bypass, without the need for arresting the heart. The
augmentation of the pulmonary artery with fresh pericardium during the
anterior translocation also prevents further tension on the vessel.
The case and the treatment options were discussed in our hospital’s
pediatric cardiology and pediatric heart surgery conference. There were
concerns as the surgical decompression by translocating the right
pulmonary artery that was compressing the left main bronchus from
outside may not be enough to relieve the obstruction on the probably
severe bronchomalacia. There were also concerns about putting the
premature newborn under the risks of cardiopulmonary bypass without any
congenital heart defects might surpass the potential benefits to relieve
the external pressure on the bronchus. But considering the choice of the
family, finally, the team agreed to proceed with anterior translocation
of right pulmonary artery.
SURGICAL TECHNIQUE
The terms were discussed with the family in detail and surgical consent
was obtained. Midline incision was followed by sternotomy, followed by
subtotal thymectomy. After heparinization, high aortic and bicaval
cannulation was made accordingly, in case of a need for a Cross clamp (X
clamp). The cardiopulmonary bypass was initiated, but the patient was
not cooled down. A patent Ductus Arteriosus was found and divided. The
pulmonary arteries were significantly enlarged and were dissected to
free up from all adhesions all the way distally close to the intra
pulmonary bifurcation level. The right pulmonary artery is transected
from the main pulmonary artery and the proximal site on the distal MPA
was over sawn as two layers; one being horizontal mattress and the other
as continuous over and over with 6/0 polypropylene suture. The right
pulmonary artery was brought anterior to the aorta and spatulated on the
posterior wall. Meanwhile a medially based trap door was created from
the remaining anterior wall of the main pulmonary artery and attached
and sawn to the backwall of the spatulated right pulmonary artery and
the rest of the anterior wall of the reconstructed pulmonary artery is
augmented with a generous sized patient‘s own fresh pericardium, to
prevent any possible upward compression of the ascending aorta to the
newly reconstructed anteriorly translocated pulmonary artery. The
patient is weaned from cardiopulmonary bypass gradually following the
optimization of the volume status and the ventilatory parameters and
closed back up in the routine fashion, transferred to the pediatric
cardiac ICU uneventfully.
On the 2nd postoperative day, the patient is transferred from the
pediatric cardiac ICU to a pediatric ICU in a different hospital, weaned
from ventilator successfully in the following week. Had a CT scan which
was showing the translocated pulmonary arteries, the unobstructed left
bronchus and the anteriorly translocated right pulmonary artery (Picture
2). The patient was discharged one month after surgery and was doing
well, gaining weight and no difficulty with breathing and feeding were
reported at the 11th month clinic visit.
COMMENT
Advances in perinatal care have dramatically improved the survival of
extremely preterm infants, but the incidence of bronchopulmonary
dysplasia (BPD) has not changed over the past few decades. BPD remains
the most common late morbidity of preterm birth, but many controversies
persist regarding how to best define BPD, grade its severity, prevent
and treat the disease9.
The newborns with BPDs exposed to intubation and mechanical ventilation
remains high along with a continued high incidence of chronic
respiratory failure and the subsequent development particularly severe
chronic respiratory disease and have related comorbidities that persist
throughout their NICU course and needing prolonged respiratory support,
including mechanical ventilation and high inspired oxygen concentrations
post-discharge.
The decision to commit an infant with sBPD to chronic ventilator support
with the placement of a tracheostomy tube can be complex and difficult
and must involve extensive discussions among care providers and family
members. Tracheostomy placement is only part of an organized strategy to
provide chronic ventilation for enhancement of better long-term
outcomes, if the optimized circumstances can be arranged properly.
But if there is resistance to recovery despite all medical management
measures and an indication for surgery is inevitable; we thought that
this case report might be helpful to shed light for keeping an open mind
to find solutions for problems by using and combining different field
practices. Another important aspect is as being the first case reported
to use this congenital heart surgery method as the first choice instead
of the last resort for an isolated BPD patient without any other
congenital cardiac anomaly. In our opinion, for selected cases, it may
be preferable to use the direct removal of the pressuring source method
like the translocation of pulmonary artery, which might help to create
more space for the bronchial tree to heal and grow, instead of the
collateral pressure relief approach like aortopexy.
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Picture 1: A-coronal and B-transverse CT images with white arrows
showing the narrowed and almost blocked left bronchus and the enlarged
right pulmonary artery compressing from outside.
Picture 2: A-coronal and B-transverse CT images with black arrows
showing the open left bronchus and the anteriorly translocated right
pulmonary artery.