Single stage repair for aortic root aneurysmin a patient with coexisting coarctationincorporating the Cabrol technique: acase reportYassir Iqbal1, Omar A. Jarral2,4*, Pantelis Tsipas3, Ilias Samiotis3, Theodoros Kratimenos3, John Kokotsakis3
and Thanos Athanasiou2
Abstract
Background: A 44 year old man who presented with a history of chest pain and dyspnoea was found to havean aneurysm of the aortic root, aortic valve insufficiency, and coarctation of the aorta.
Case presentation: The patient underwent a single stage procedure to treat the aortic root, valve and coarctationwith a composite valved conduit and extra-anatomic bypass of the coarctation. The modified Cabrol technique wasnecessary to attach the coronary buttons due to grossly abnormal anatomy. The patient made a remarkablerecovery and was discharged on the 8th post-operative day.
Conclusion: This case report highlights the feasibility and efficacy of performing a single stage procedure oncomplex coarctation with associated cardiac defects. To the best of our knowledge, this is the first report of themodified Cabrol technique being used in this particular setting.
BackgroundAortic coarctation is a relatively common congenitalabnormality. The prevalence accounts for 6–8% of alllive births with congenital heart defects. It occurs at theisthmus of the aorta (distal aortic arch between the leftsubclavian and the site of the ligamentum arteriosus)where there is a discrete narrowing [1].Patients with associated arch hypoplasia are at increased
risk of developing hypertension. They have an increasedincidence of acute cardiac events and delayed aneurysmand dissection formation even following correction [2].There has been much debate as to the optimum surgicalstrategy to treat this condition. Meticulous preoperativeplanning is required with thorough multidisciplinarydiscussion.
We report a case of a successful single stage repair ofa complex aortic coarctation with concomitant rootaneurysm, and aortic valve insufficiency.
Case presentationA 44-year old male was referred following investigationfor chest pain and dyspnoea. He had no pre-existingco-morbidities. Physical examination revealed feeble fem-oral pulses and he was found to be hypertensive withmarked differences between the upper and lower limbs(systolic blood pressure upper limb 190mmmHg, lowerlimb 75 mmHg, with an ankle brachial index (ABI) of0.39). Electrocardiogram revealed evidence of severe leftventricular hypertrophy. This was confirmed with echo-cardiography which also demonstrated a tricuspid aorticvalve with significant aortic regurgitation in the presenceof an aortic root aneurysm of approximately 9 cm. Leftventricular function was preserved. Computerised tomog-raphy angiography (CTA) was performed to evaluate theaortic pathology in further detail (Fig. 1). The scan noted
* Correspondence: [email protected] College, London, UK4Department of Surgery and Cancer, Imperial College London, London W21NY, UKFull list of author information is available at the end of the article
an aortic root aneurysm (8.8 cm), in addition to thepresence of severe aortic coarctation, with subtotal oc-clusion and a lumen less than 6 mm in size. The coarc-tation was just distal to the left subclavian artery, at theaortic isthmus. There was clear evidence of collateralcirculation to the descending thoracic aorta via the sub-clavian and intercostal arteries. Coronary angiographyconfirmed a right dominant coronary system with nosignificant coronary disease.A multidisciplinary team meeting took place and a
consensus was agreed to proceed with a two stagedhybrid approach, with the first phase involving an endo-vascular approach to stent the coarctation, followed by asecond stage to perform the surgical repair of the aorticroot aneurysm. The first stage to stent the coarctationwas unsuccessful via the femoral approach, as the guide-wire could not cross the coarctation. Assessment throughangiography via the left brachial artery showed completeobstruction at the aortic isthmus. The decision was thenmade to proceed to a single stage surgical approach totreat both lesions.After induction of anaesthesia, arterial lines were
placed in the left radial and left femoral artery. A rightinfraclavicular incision and a right groin incision wasmade this was to establish peripheral arterial cannulationaccess to the right axillary and right femoral artery. An8 mm dacron graft was anastomosed to each vessel forindirect cannulation. Median sternotomy was performedto access the mediastinum and expose the heart andaorta. Following heparinisation cardiopulmonary bypass(CPB) was established with venous return from bi-cavalcannulation. The body temperature was cooled to 25degrees Celsius. The right superior pulmonary vein wasused for venting. Once the cross clamp was applied,
complete cardiac arrest was achieved using Custodiol25 ml/kg crystalloid cardioplegia via a retrograde cannulathrough the coronary sinus. A further top up of cardiople-gia was given once the aorta was opened through directcannulation of the coronary ostia.The aortic root, valve and ascending aorta were excised.
The coronary ostia were fashioned as buttons from thenative aortic root. The coronary ostia were noted to besignificantly displaced, with distorted anatomy due to thepatient’s disease process. Therefore, 8 mm dacron graftswere attached end-to-end to each ostia, with view toperforming the modified Cabrol technique later followingreplacement of the root. The heart was then retracted in acephalad position to access the posterior pericardium. Avertical incision was made to expose the descendingthoracic aorta (DTA). An end to side anastomosis wasformed with a 20 mm dacron graft to the DTA (Fig. 2).This graft was then routed posterior to the inferior venacava (IVC) and anterior to the right inferior pulmonaryvein (RIPV), adjacent to the right atrium (RA). Root re-placement was then performed with a 25 mm biologicalvalved-conduit, as this was favoured by the patient overa mechanical prosthesis, despite the risk of a difficultredo procedure in the future. The 8 mm dacron graftsattached to the coronary ostia were anastmosed to theroot conduit as neo coronary ostia. The distal part ofthe valved-conduit was anastomosed to the proximalarch under selective antegrade cerebral perfusion (SACP).Finally, an end to side anastomosis was fashioned betweenthe 20 mm extra-cardiac graft (attached to the descendingthoracic aorta) and the ascending portion of thevalved-conduit. Valve-sparing root replacement was not
Fig. 1 Computer generated 3D reconstruction of aorta. Demonstratingextensive collateral network
Fig. 2 Surgeons view; longitudinal incision in posterior pericardiumto anastomose extra-anatomical bypass to DTA
Iqbal et al. Journal of Cardiothoracic Surgery (2018) 13:75 Page 2 of 5
considered in this patient due to the grossly abnormal aor-tic anatomy.Following rewarming and deairing the patient was
successfully weaned off CPB. The bypass time was160 min, the cross-clamp time was 120 min, and theSACP time was 40 min. Haemostasis was achieved andthereafter a routine closure of all incision sites. The pa-tient remained in ICU for less than 48 h, and made excel-lent progress on the ward. Minimal anti-hypertensiveswere required and the patient was discharged on 8th daypost operatively neurologically intact and independent.At 3 months follow up the patient underwent a re-peat CTA scan which showed complete patency inthe extra-anatomical graft and resolution of the col-lateral arterial network (Fig. 3).
DiscussionAortic coarctation is a common congenital cardiac de-fect, with narrowing of the aorta at the isthmus. Thecondition is generally identified and treated during in-fancy and childhood. However presentation of untreatedaortic coarctation in adults with associated cardiac de-fects are scarcely reported [3, 4].In this complex case we treated three distinct lesions
(aortic root aneurysm, aortic valve insufficiency and aor-tic coarctation) through a single staged approach. Anadditional challenge encountered was the reimplantationof the coronary buttons. The aortic root aneurysm wasof a significant size, hence reimplantation of the coron-ary ostia directly on to the neo aorta was not possible asthe coronary arteries would be overstretched. The use ofthe modified Cabrol technique was incorporated intothis single staged operation to provide safe implantationof the coronary buttons with a tension free anastomosis
[5]. We believe this is the first reported case where themodified Cabrol technique has been used in this setting.There remains a wide range of treatment options for
aortic coarctation which can be broadly divided into surgi-cal and non-surgical strategies. Non-surgical interven-tional techniques are becoming increasingly popular,either with balloon angioplasty and or insertion of a stent.These approaches can be used both in native and recur-rent disease. However, there is insufficient long-term dataavailable in comparing these interventions with surgicalrepair. Although these approaches are often associatedwith lower morbidity compared to surgery, there is anincreased rate of re-stenosis 11% versus 2% [6, 7] hence ahigher need for reintervention. Reintervention may alsobe required due to stent fracture, migration and possibleaneurysm formation. Careful patient selection is requiredas not all cases are amenable to percutaneuous interven-tion. Our patient underwent an interventional approachinitially, however the guidewire was unable to cross thelesion.Surgical repair can include the option of primary re-
pair of the coarctation, either through a left thoracot-omy (2-stage repair when in the presence of othercardiac defects) or median sternotomy. However, this isassociated with many challenges especially in the ado-lescent and adult patient population. Extensive mobil-isation of the aorta is required, in addition to control ofthe extensive collateral blood vessels this can contributeto increased risk of bleeding. Other complications caninclude possibility of lung parenchymal injury, damageto the recurrent laryngeal and or phrenic nerves, chy-lothorax and the possibility of spinal cord ischaemia[8]. Due to these many risks primary anatomic repair ofthe coarctation has been avoided in adult patients.
Fig. 3 Computer generated 3D reconstruction demonstrating surgical repair
Iqbal et al. Journal of Cardiothoracic Surgery (2018) 13:75 Page 3 of 5
In attempt to reduce complexity of open surgicalrepair, variations of extra-anatomic bypass have beendeveloped. The single stage procedure was first de-scribed by Vijayanagar and colleagues in 1980 [9]. Theprocedure incorporated an extra-anatomic bypass fromthe ascending aorta to the DTA through the posteriorpericardium. This operation allowed bypass of the coarc-tation in addition being able to correct other concomitantcardiac defects, i.e. valve replacement, in a single stagedapproach [8]. This strategy is also preferred in cases wherethere is a complex coarctation or re-coarctation where aprimary repair would be associated with major complica-tions as described above. In addition to the major benefitof being able to treat multiple cardiovascular disorders atthe same time in one sternotomy, there is no need forlaparotomy to reach the DTA, and the graft is routed in ashort course around the right margin of the heart withoutthe risk of compressing the right atrium or ventricle [8].The initial procedure by Vijayangar described routing
the extra anatomic bypass around the left lateral borderof the heart. Powell et al. modified this procedure by po-sitioning the graft along the right sided margin of theheart, the benefit being that the graft remains in a moreposterior position hence allowing safer re-entry into thechest if reoperation is required [8]. In addition position-ing the graft anterior to the RIPV and behind the IVCprevents compression to the right atrium and ventricle[10]. The possible difficulties in this procedure are thataccessing the DTA through the posterior pericardium isdifficult and exposure maybe more challenging in patientswho are obese or have a barrel shaped thorax. The pro-cedure may not be suited to patients in the adolescentgroup due to their potential somatic growth that couldlead to anastomotic dehiscence. Potential long term com-plications that have been described with this procedureinclude narrowing of the graft, neointimal thrombus for-mation, pseudoaneurysm formation or anastomotic dehis-cence in patients with who have had somatic growth sincetheir initial procedure. However, in a paper by Connolly etal. [8] with a mean follow up of 3.7 years there were nocomplications in patients who underwent single stage sur-gical treatment.
ConclusionWhen coarctation or re-coarctation is associated withcardiac defects that require repair, a single stage approachusing cardiopulmonary bypass and coarctation bypassgrafting through the posterior pericardium is a safe surgi-cal alternative, as demonstrated by this case. We under-stand this is also the first reported case where themodified Cabrol technique has been incorporated withthis single staged procedure to treat aortic root aneurysmand aortic coarctation. The surgical management of pa-tients with complex coarctation or recoarctation with or
without associated cardiac disorders must be individua-lised and treatment options should be discussed in anopen multidisciplinary platform.Extra-anatomic coarctation bypass is a safe alternative
method when endovascular interventions are not feas-ible. Particular attention must be paid to ensuring theextra-anatomic bypass conduit is well positioned and ofa significant calibre. Kinking of this conduit can lead toturbulent blood flow and abnormal wall shear stresswhich could impact on long term patency. We felt thatrouting the extra-anatomic bypass posterior to the IVCachieved the best haemodynamic result.Our strategy has proved to be particularly useful in an
adult patient where simultaneous intra cardiac repair isrequired.
Authors contributionsTA conceived the report. YI and OJ wrote the first draft with input from allauthors. All authors approved the final manuscript.
Ethics approval and consent to participateNot applicable.
Consent for publicationConsent for publication of this case report in its entirety was obtained fromthe patient.
Competing interestsThe authors declare that they have no competing interests.
Publisher’s NoteSpringer Nature remains neutral with regard to jurisdictional claims inpublished maps and institutional affiliations.
Author details1Birmingham Children’s Hospital, Birmingham, UK. 2Imperial College, London,UK. 3Evangelismos Hospital, Athens, Greece. 4Department of Surgery andCancer, Imperial College London, London W2 1NY, UK.
Received: 20 February 2018 Accepted: 15 June 2018
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