MMCTS
(March 28, 2008). doi:10.1510/mmcts.2006.002451
Copyright © 2008 European Association for Cardio-thoracic Surgery
Procedure
Surgical repair of truncus arteriosus associated with interrupted aortic arch
François Lacour-Gayet* and
Steven Goldberg
The Children's Hospital, Denver, Colorado, USA
* Corresponding author: Tel: +1-303-8616624; fax: +1-303-7648022. lacour-gayet.francois{at}tchden.org
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Summary
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The surgical repair of truncus arteriosus associated with an interrupted aortic arch (TAC-IAA) requires performing two major procedures at the same time. Due to the small number of patients, there is nearly no surgical learning curve. The surgical technique has greatly improved since the introduction of a homograft patch enlargement of the small ascending aorta. The association with a severe truncal regurgitation is a major risk factor as well as the presence of preoperative multiple organs failure. The series published by single centers are <10 patients, which make statistical analysis troublesome. The mortality varies from 0% to 50%. The multicentric study published in 2006 by the Congenital Heart Surgeons Society (CHSS) reports a 68% mortality (34/50). Nevertheless, the results can be excellent in experienced centers using a modern one stage surgical technique, undertaken in the first week of life.
Key Words: Congenital heart surgery • Interrupted aortic arch • Truncus arteriosus
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Introduction
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Truncus arteriosus communis (TAC) with interrupted aortic arch (TAC-IAA) is a very uncommon congenital heart disease (1/1000) needing a challenging surgical procedure. An association with a significant truncal valve regurgitation as well as any delay in the surgical repair can worsen the clinical picture with a patient presenting with severe multiple organs failure.
Surgical anatomy, physio-pathology and indications
The Van Praagh [1] classification (Schematic 1) is the only one identifying TAC-IAA [2]. The truncus types I and II are the most usual (frequent ‘one and half’ aspect). Type III is exceptional. The type IV is the association with an IAA, which accounts for 10% of common arterial trunks. There are two forms of TAC-IAA: IV with IAA type A and IV with IAA type B (Schematic 1). These two forms may vary according to the take off of the PA branches that may either arise from a single trunk or take off separately with frequent anterior location of the left PA branch ostium. Pulmonary branches stenosis or hypoplasia can be seen. The ascending aorta is hypoplastic as in all IAA; it arises as an ascending branch of the common trunk. A dextroposition of the common arterial trunk is possible. Truncus without VSD (type B of Van Praagh) seems not viable and seen essentially by pathologists. The presence of an arterial duct is a singular characteristic of TAC-IAA, as common arterial trunks I and II usually have no ductus arteriosus. TAC-IAA share equally with other truncus the risk of an associated truncal valve regurgitation. When severe, the truncal regurgitation is a major risk factor. Truncal valve stenosis is possible. The association with a DiGeorge syndrome is constant when the IAA is of type B.
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Schematic 1 Richard Van Praagh Classification. The TAC-IAA are of type A or B according to the left subclavian artery location. Notice the small size of the ascending aorta in TAC-IAA and the presence of an arterial duct.
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The closure of the arterial duct (PDA) is a catastrophic event. Fetal diagnosis and early diagnosis are crucial to start Prostaglandin infusion immediately after birth. Patients seen after PDA closure may be moribund with major multiple organs failure (renal failure, hepatic failure and necrotizing enterocolitis) and severe heart failure with massive increase of the pulmonary flow and worsening of the truncal regurgitation. Small birth weight <2.5 kg is not unusual.
The surgical repair of TAC-IAA is undertaken in the first week of life. Even with an arterial duct maintained patent, these patients remain very fragile with a poor coronary perfusion. In presence of multiple organs failure, the dilemma is between waiting for improvement or going on and repair. This is a sort of ‘loose-loose’ situation that should essentially be prevented. Palliation may be an option.
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Surgical technique
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Palliation of TAC-IAA is exceptionally performed; it may be considered in cases of multiple organs failure and very small weight. It is achieved with bilateral PA branch banding with maintaining the duct opened (hybrid I type).
It is Dwight C. McGoon who first published a TAC-IAA repair [3]. The modern one stage surgical repair, undertaken in the first days of life, includes a double repair. Even if usually considered as a major technical difficulty, the surgical technique is quite well defined since the mid-1990s [4, 5, 6, 7]. The truncus arteriosus and the IAA repair follow basically the usual techniques. The particularity of the repair is the need to reconstruct a good size ascending aorta to control the mismatch between the ascending aorta and the large common trunk. This is achieved by an enlargement patch of the ascending aorta and of the transverse arch, using homograft tissue (a technique very similar to that of a TGA-VSD-coarctation repair) [7].
The anesthesia time should be minimized. Due to the low diastolic pressure and the poor coronary perfusion, the delay between the induction of anesthesia and the incision should be minimal. A right arterial pressure line is preferable. The blood must be irradiated because of the presence of a DiGeorge syndrome. Hypocalcemia should be permanently corrected.
In order to improve the coronary perfusion and decrease the pulmonary blood flow, the right pulmonary artery is snared immediately after opening the pericardium (check first that the tracheal tube is not mistakenly placed in the right bronchus). This allows a more stable hemodynamic before cannulation.
A rapid arterial cannulation of the ductus arteriosus (Video 1) and a single venous cannulation of the right appendage allow a fast start in cardio-pulmonary bypass. The ductus arteriosus is ligated and the right PA unclamped. The repair can be performed under circulatory arrest or brain perfusion according to ‘individual beliefs’ [8].
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Video 1 The anatomy of truncus arteriosus with interrupted aortic arch is shown. Two arterial cannulas are inserted (one via a 3.5 mm Gore-Tex tube implanted on the brachiocephalic artery, one into the ductus arteriosus). Deep hypothermia is induced and the ascending aorta is cross-clamped.
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We have personally chosen to favor brain perfusion in using an arterial cannulation of the brachiocephalic artery [9] through a Gore-Tex conduit #3.5 (Schematic 2). The operation is undertaken in hypothermia at 20°C rectal temperature. The brain perfusion flow is between 50 and 80 ml/kg/min according to the right radial artery pressure, which should not exceed 50 mmHg. A left atrium vent is placed.
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Schematic 2 TAC-IAA type B. The ascending aorta arises from the common trunk as an ascending branch. A Gore-Tex #3.5 is implanted on the brachiocephalic artery to allow brain perfusion.
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The cross-clamp is placed at the terminal end of the ascending aorta. The myocardial protection is insured using the Bretschneider crystalloid cardioplegia (Custodiol® Solution, Essential Pharmaceuticals, Newtown, PA, USA) that is repeated every 40 min. In case of significant truncal insufficiency, direct coronary perfusion is needed.
First, the common arterial trunk is transected (Schematic 3) at the origin of the ascending aorta and a large pulmonary button containing both PA ostia is harvested (Videos 2 and 3). Care is taken to remain well distant from the left coronary ostium that could be very close.
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Schematic 3 The common trunk is transected, taking a large pulmonary ostia button. The ductus is resected and the ascending aorta incised.
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Video 2 The ascending aorta is transected.
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Video 3 The pulmonary bifurcation is harvested.
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Second, the aortic arch and ascending aorta are reconstructed (Schematics 3 and 4). The descending aorta is largely mobilized as well as the neck vessels. The brain perfusion is instituted. The brachiocephalic artery is snared ahead of the Gore-Tex arterial cannulation; the left carotid and left subclavian are snared. The descending aorta is cross-clamped as low as possible, using a deep femoral clamp. The arterial duct is largely resected until no more ductal tissue can be seen. The ascending aorta is incised all the way (Video 4). The descending aorta is anastomosed to the transverse arch on half of its posterior circumference (Video 5). A patch of pulmonary branch homograft is used to enlarge both the transverse arch and the ascending aorta. This patch should be large enough to meet the diameter of the truncal root (Videos 6, 7, Schematic 4). The cross-clamp is placed back on the reconstructed end of the ascending aorta and full perfusion is resumed. The temperature is brought to 25°C. The brain perfusion time is usually around 30 min.
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Schematic 4 The descending aorta is anastomosed to the ascending aorta on half of its circumference. A large homograft patch is used to enlarge the reconstructed aortic arch. The ventricular septal defect is closed through a low infundibulotomy.
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Video 4 The ductal tissue is resected. The descending aorta and the ascending aorta are opened longitudinally.
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Video 5 The descending aorta is anastomosed to the ascending aorta on half of its circumference.
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Video 6 A patch of pulmonary homograft is prepared.
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Video 7 The homograft patch is sutured to the descending aorta and to the ascending aorta, in order to enlarge the reconstructed aortic arch.
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Third, the distal anastomosis of the valved conduit to the pulmonary bifurcation is performed using a bovine jugular valved conduit (Contegra®, Medtronic Inc, Minneapolis, MN) conduit #12 (Schematic 5). The Contegra distal end should be trimmed as short as possible. Care is taken to respect the potential antero-posterior anatomy of the pulmonary artery (PA) branch ostia.
Fourth, the truncal root is anastomosed to the reconstructed ascending aorta (Video 8, Schematic 5).
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Video 8 The truncal root is anastomosed to the reconstructed aortic arch.
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Fifth, the ventricular septal defect (VSD) is closed. The infundibulotomy is carried out low on the infundibulum for two reasons: the Contegra leaflets are long and the truncal valve can be located low in the right ventricle. The VSD is closed using a Gore-Tex patch, adapting the continuous suture line to the possible dextroposition of the truncus.
Sixth, still under cross-clamping the Contegra proximal anastomosis is performed (Schematic 5). The Contegra should be trimmed short. In case of a large patent foramen ovale (PFO); it is reduced to a 4 mm fenestration. The cross-clamp is removed.
Management of a severe truncal valve insufficiency
The truncal valve is always malformed. It can be made of four or even five leaflets. Only severe truncal valve regurgitation should be treated. It is possible to reduce the regurgitation in closing a commissure, knowing that the truncal tissue is immature and friable. The real issue is to take the decision to replace the truncal valve and add another hour of cross-clamp time. The valve is replaced with a homograft; the positive aspect is the large diameter of the truncal annulus, allowing placing a 12 mm or larger homograft. It has been our experience that the truncal regurgitation improves with time as the valvar tissue matures [6, 7]. Nevertheless, severe regurgitation requires replacement; the long cross-clamp time potentially leading to post op extra corporeal membrane oxygenation (ECMO).
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Results
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The single center reports [5, 6, 10, 11, 12, 13, 14, 15, 16] do not exceed ten patients. The mortality varies from 0% to 50% (Table 1), making difficult the identification of significant risk factors. The multicentric study from the Congenital Heart Surgeons Society (CHSS) [17] is the largest series published; it reports a mortality of 68% (34/50). This is in contrast with the 0% mortality reported by three centers [9, 11, 16]. Most reports indicate that the association with a severe truncal regurgitation and the presence of pre-operative multiple organ failure are major risk factors. The technique of homograft enlargement of the ascending aorta was published in the mid-1990s; since then mortality has clearly improved in experienced centers. The results are excellent when the TAC-IAA is not associated with major risk factors [15, 18, 19, 20].
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Comments
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The recent large multicentric study published by the CHSS [17] introduces a note of pessimism with a 68% mortality. In fact, this series closes in 1997 at a time when the homograft patch enlargement of the ascending aorta was just introduced. The authors acknowledge that many of the patients in their cohort have not benefited from an optimal technique. In our opinion [21], the mortality is far less in the current era and the results can be optimal provided that the patient is operated in good condition: in the first week of life, eventually waiting a few days to treat extra cardiac disorders, in a one stage repair using a homograft patch enlargement technique as described here.
The presence of a severe truncal regurgitation is the most concerning issue. Truncal valve repairs have been successfully undertaken at the time of the neonatal repair [10, 22]. Nevertheless, the truncal valve leaflets are immature and friable. A replacement with an aortic homograft in TAC-IAA was used three times in the entire literature. A heart transplantation has been proposed [17, 23]; the long delay usually needed to obtain an infant donor makes this option quite exceptional.
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References
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Summary
Introduction