Ann Thorac Surg 2008;86:284-287. doi:10.1016/j.athoracsur.2007.12.048
© 2008 The Society of Thoracic Surgeons
Case Reports
Single-Stage Repair of Acute Type A Aortic Dissection Associated With Aortic Coarctation, Perimembranous Ventricular Septal Defect, and Bicuspid Aortic Valve
Cemal Levent Birincioglu, MD,
Levent Mavioglu, MD*,
Soner Yavas, MD,
Ertan Demirtas, MD,
Garip Alt
ntas, MD,
Hakki Zafer Iscan, MD
Departmant of Cardiovascular Surgery, Turkiye Yuksek Ihtisas Hospital, Ankara, Turkey
Accepted for publication December 17, 2007.
* Address correspondence to Dr Mavioglu, Teras Evler Sitesi D Blok No. 49, 06550 Oran, Ankara, Turkey (Email: leventmavioglucvs{at}gmail.com).
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Abstract
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A 17-year-old man with coarctation of the thoracic aorta, bicuspid aortic valve, perimembranous ventricular septal defect, and acute type A dissection of the ascending aorta underwent emergency repair for dissection by means of single-stage replacement of not only the aortic valve and ascending aorta, but also simultaneous repair of the coarctation by graft interposition. These combined conditions comprised some difficulties that included decisions on the optimal timing and sequence of surgical repair, technique, and circulatory support.
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Introduction
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The combination of acute aortic dissection associated with significant coarctation of the aorta and the other cardiac abnormalities is a rare condition that has only been documented in a few reports [1, 2]. In the literature review, these combined disorders are usually treated by multi-staged surgery [3, 4]. The presented case of acute dissection of the ascending aorta associated with aortic coarctation, perimembranous ventricular septal defect, and bicuspid aortic valve resulted in limited aortic rupture into the pericardium, which we were able to successfully treat with a single-stage operative approach.
A 17-year-old man was admitted into an outlying hospital with sudden onset of severe back pain and he was referred to our hospital with a diagnosis of acute dissection of the ascending aorta based on clinical and echocardiographic findings. On clinical examination a systolic murmur was noted in the precordium, the blood pressure in his upper extremities was 150/78 mm Hg, and his femoral pulses could not be palpated. The chest roentgenogram showed evidence of coarctation of the aorta with notching of the ribs and a reversed-three sign at the aortic arch. Transthoracic echocardiography revealed bicuspid aortic valve with a peak systolic gradient of 75 mm Hg, a perimembranous ventricular septal defect with a left-to-right shunt (Qp/Qs = 1.8), an aneurysmal dilatation with intimal flap in the ascending aorta, and minimal pericardial effusion. The diameter of the ascending aorta was 6.4 cm. Because the clinical findings were highly suggestive of coarctation of the thoracic aorta, a computed tomographic angiography was performed. An intimal flap was visualized above the aortic valve with an aneurysmal dilatation of the ascending aorta was again demonstrated by computed tomographic angiography, and a coarctation of the thoracic aorta was established. There was a luminal stenosis of greater than 70% at the coarctated aortic segment (Fig 1).
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Fig 1. Preoperative computed tomographic angiography study of the aorta revealed the coarctation of the thoracic aorta, type A dissection and the ascending aortic aneurysm.
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The patient was immediately taken to the operating room. Cardiopulmonary bypass was established with double arterial cannulation in the right axillary artery and right common femoral artery to ensure adequate perfusion proximal and distal to the coarctation. A median sternotomy was performed. The pericardium was then opened and an 800 mL serous hemorrhagic fluid was drained from the pericardial cavity. Bicaval cannulation through the right atrium was used for venous return. The patient was slowly cooled to 26°C. The ascending aorta was cross-clamped (just proximal to the innominate artery), antegrade and retrograde crystalloid cardioplegia was used to arrest the heart. Additional cardioplegic (cold blood) delivery was given in a retrograde manner. An aortotomy was performed, which confirmed a type A dissection, situated 2 cm distal to the coronary ostia, confined to the ascending aorta, but not extending into the aortic arch. The bicuspid aortic valve with partially fused, thickened, and calcified leaflets was resected and replaced with a mechanical Sorin prosthesis (Sorin Biomedica, Saluggia, Italy) using interrupted sutures reinforced with pledgets. A perimembranous ventricular septal defect was repaired with pledgets sutured through the right atriotomy. The aneurysmatic aortic tissue was removed so that both of the two coronary ostium stayed as one single penninsula, which was not dilated and seemed to be normal. These two coronary ostia were anatomically close to each other, and both were too short for an anastomosis without an approach as Cabrol. A 30-mm Dacron graft (Hemashield; Meadox Medicals Inc, Oakland, NJ) was used to replace the ascending aorta above the coronary artery ostia and mechanical prosthesis to the aortic cross clamp. The clamp was removed, and during the rewarming period, an 18-mm Dacron graft (Hemashield; Meadox Medicals Inc) was anastomosed to the descending thoracic aorta (below the coarctation segment) after the process of passing through the pericardium over the phrenic nerve, in the area superior to the pulmonary hilus, with a side-biting clamp. The proximal part of the graft was anastomosed to the ascending aortic graft (Fig 2).
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Fig 2. Operative photograph showing ascending aorta repair and ascending-to-descending aortic bypass grafting.
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Cardiopulmonary bypass flow was maintained at 4.5 L/min/m2 throughout the entire procedure. The total cardiopulmonary bypass time was 215 minutes. The total aortic cross-clamp time was 111 minutes.
The postoperative course was uneventful, and the patient was discharged on postoperative day 7 in good condition. One month after the repair, the patient has continued to do well, and a follow-up computed tomographic angiogram revealed a satisfactory repair (Fig 3).
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Comment
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The combination of acute dissection associated with coarctation of the aorta is a rare but significant problem. Bicuspid aortic valve is common and occurs in 1% to 2% of the population [5]. In addition, up to 40% of patients with coarctation have an associated bicuspid aortic valve that may also become stenotic or incompetent, or both [6]. The association between coarctation of the aorta and aortic dissection has been known for a long time, as having been described in early studies of the natural history of the coarctation of the aorta. These studies showed that the aortic rupture was the cause of death in 19% to 23% of patients with coarctation of the aorta; in 75% of instances, rupture occured in the ascending aorta [7].
The incidence of aortic dissection in patients with coarctation of the aorta is well known. However, the reported cases of surgical correction of these conditions are very few [8]. The repair of aortic dissection in the presence of coarctation comprise some difficulties; these include decisions on the optimal timing and sequence of the surgical repair, the best surgical exposure on the technique, and circulatory support. Lawson and Fenn [9] reported the first successful repair of this combination using a staged procedure. The main idea in this staged procedure is initial repair of coarctation to relieve proximal hypertension, thereby decreasing the risk of progressive dissection on rupture. Therefore, cardiopulmonary support during correction of the aortic dissection can be safely used through the femoral artery or the aortic arch. However, this approach is only applicable to presence of an aortic aneursym or chronic dissection. In addtion, the major risk of the staged procedure is rupture while waiting for the second stage of the replacement. Because of these drawbacks, immediate repair of the dissection was mandatory for survival.
Nevertheless, replacement of the ascending aorta without coarctation repair has also yielded some specific problems such as cardiac decompensation and increased risk of perioperative hemorrhage from aortic suture lines caused by the high afterload affecting cardiac performance. It seems that both techniques have some disadvantages. For this reason, simultaneous repair of the dissection and coarctation seems a better procedure for these complex conditions.
In 1928, Abbott [7] summarized the association of coarctation of the aorta and the development of proximal dissection of the ascending aorta or aortic arch. The first single-stage generation of coarctation of the thoracic aorta and ascending aorta aneurysm was reported by Svensson and colleagues [1] in 1994.
When a patient presents with a combination of acute dissection and coarctation of the aorta, establishing adequate blood flow to the upper and lower torso during cardiopulmonary bypass is the major concern of this condition. Therefore, we used the right axillary artery and femoral artery for arterial cannulation for the maintenance of adequate flow to both areas of the body. However, in some patients with coarctation, increasing numbers of collateral vessels and collateral circulation develop around the coarctated segment and therefore, single arterial cannulation may be adequate for the maintenance of the blood flow during cardiopulmonary bypass.
Another important point of the single-stage procedure is extra-anatomical bypass grafting from the ascending aorta during the rewarming period. This technique was suitable, not only as the safest technique for this anastomosis with this particular exposure, but also for shortening the ischemic period. The first extra-anatomical bypass graft for coarctation of the thoracic aorta was reported by Siderys and associates [10] in 1974. They performed an extra-anatomical bypass from the ascending aorta to the abdominal aorta, distal to the renal arteries, with a 22-mm Dacron graft. Since that time, various bypass grafting methods from the ascending aorta or subclavian or axillary arteries to the descending thoracic aorta, abdominal aorta, or femoral artery have been described with good, long-term patencies [11–15].
The technical comfort and well, hemodynamic performance results of the ascending-to-descending aorta bypass grafting was performed as an extra-anatomical bypass grafting.
The extra-anatomical bypass grafting areas to the descending aorta are shown in Figure 4. The superior approach (superior of the left pulmonary hilum): passing lateral pericardium through (over the phrenic nerve) (Fig 4A) and passing lateral pericardium through (under the phrenic nevre) (Fig 4B). The inferior approach (nferior of the left pulmonary hilum): along the left side of the heart (Fig 4C) and along the right side of the heart (Fig 4D).
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Fig 4. Extra-anatomical bypass grafting areas to the descending aorta. The superior approach (superior of the left pulmonary hilum): (A) passing lateral pericardium through (over the phrenic nerve) and (B) passing lateral pericardium through (under the phrenic nerve). The inferior approach (nferior of the left pulmonary hilum): (C) along the left side of the heart and (D) along the right side of the heart.
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We performed the extra-anatomical bypass with the superior approach passing through the lateral pericardium and over the phrenic nerve. Concerning the superior approach (under the phrenic nerve), some important points should be considered, such as phrenic and vagus nerve injuries and lymphatic drainage caused by the damages of the ductus Botalli lymph nodes. In cases concerning the inferior approach, that long graft has been better used, and in such cases, the possibilty of causing injuries of the coronary artery graft and atrium should be taken under consideration.
In conclusion, coarctation of the descending thoracic aorta with concomitant cardiac lesions, such as perimembranous ventricular septal defect, bicuspid aortic valve, and type A aortic dissection, can be safely and efficaciously repaired with a single-stage approach involving cardiac repair and extra-anatomic ascending-to-descending aorta bypass grafting, thereby avoiding the additional morbidity and mortality risks of staged operations. For this purpose, hybrid procedures through endovascular stent grafts are also growing in interest. The clinical conditions of the patient, the optimal sequence and timing of repair, the best surgical exposure, and the adequacy of blood perfusion should be considered preoperatively.
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References
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- Svensson LG. Management of acute aortic dissection associated with coarctation by a single operation Ann Thorac Surg 1994;58:241-243.[Abstract]
- Buket S, Yagdi T, Cikirikcioglu M, Alayunt EA. Single-stage transpericardial repair of acute aortic dissection associated with recoarctation J Thorac Cardiovasc Surg 2001;121:987-989.[Free Full Text]
- Bricker DL, Parker TM, Mistrot JJ, Dalton Jr ML. Repair of acute dissection of the ascending aorta, associated with coarctation of the thoracic aorta in a Jehovah's Witness J Cardiovasc Surg (Torino) 1980;21:374-378.[Medline]
- Hovaguimian H, Aru GM, Floten HS. Acute type I aortic dissection with coarctation of the aorta: discussion of management and the report of a successful brain perfusion across an aortic coarctation J Thorac Cardiovasc Surg 1990;100:152-153.[Medline]
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- Sabet HY, Edwards WD, Tazelaar HD, Daly RC. Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases Mayo Clin Proc 1999;74:14-26.[Abstract]
- Abbott ME. Coarctation of the aorta of the adult type. II. A statistical study and historical retrospect of 200 recorded cases with autopsy, of stenosis or obliteration of the descending arch in subjects above the age of two years. Am Heart J 1928;3;:574-618.
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- Siderys H, Graffs R, Hallbrook H, et al. A technique for management of inaccessible coarctation of the aorta J Thorac Cardiovasc Surg 1974;67:568-570.[Medline]
- Sweeney MS, Walker WE, Duncan JM, Hallman GL, Livesay JJ, Cooley DA. Reoperation for aortic coarctation: techniques, results, and indications for various approaches Ann Thorac Surg 1985;40:46-49.[Abstract]
- Edie RN, Janani J, Attai LA, Malm JR, Robinson G. Bypass grafts for recurrent or complex coarctations of the aorta Ann Thorac Surg 1975;20:558-566.[Abstract]
- Gelfand ET, Callaghan JC, Sterns LP. Extended aortic bypass J Thorac Cardiovasc Surg 1980;79:381-387.[Abstract]
- Connery CP, DeWeese JA, Eisenberg BK, Moss AJ. Treatment of aortic coarctation by axillofemoral bypass grafting in the high-risk patient Ann Thorac Surg 1991;52:1281-1284.[Abstract]
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Abstract
Introduction
