Ann Thorac Surg 1998;65:1151-1153
© 1998 The Society of Thoracic Surgeons
Case Reports
Two-Stage Repair for Aortic Regurgitation With Interrupted Aortic Arch
Hitoshi Ogino, MDa,
Shigehito Miki, MDa,
Keiji Matsubayashi, MDa,
Yuichi Ueda, MDa,
Takuya Nomoto, MDa
a Department of Cardiovascular Surgery, Tenri Hospital, Tenri, Nara, Japan
Accepted for publication November 11, 1997.
Address reprint requests to Dr Ogino, Cardiovascular Surgery, Tenri Hospital, 200 Mishima-cho, Tenri, Nara, 632 Japan
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Abstract
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We performed two-stage repair for a rare adult case of interrupted aortic arch with aortic regurgitation and sinus of Valsalva aneurysm. A lateroisthmic bypass was established with minimal thoracotomy and partial clamping of the descending aorta to preserve collateral circulation. This was followed by aortic root reconstruction with a prosthetic graft and valve for aortic regurgitation with sinus of Valsalva aneurysm. This less invasive two-stage repair for such a rare pathology may facilitate smooth recovery of the patient.
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Introduction
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We encountered a rare adult case of interrupted aortic arch (IAA) with aortic regurgitation (AR) and sinus of Valsalva aneurysm. A less invasive two-stage repair successfully performed with lower morbidity facilitated the patient’s smooth recovery. We report on our surgical strategies for this rare pathology.
A 32-year-old man with AR was referred for further examination. Blood pressure was 164/20 mm Hg in both arms with bounding pulses, whereas femoral pulses were weak. Chest radiography showed cardiomegaly, absence of the aortic knob, and rib notching. Echocardiography revealed AR with bicuspid aortic valve and sinus of Valsalva aneurysm (50 mm in diameter). Aortography showed IAA (type A of Cerolia and Patton’s categories [1]), with abundant collaterals (Fig 1); this also was seen on magnetic resonance imaging (Fig 2). Pulmonary arterial pressure of 50/11 mm Hg, pulmonary capillary wedge pressure of 15 mm Hg, left ventricular pressure of 154/
17 mm Hg, ascending aortic pressure of 156/59 mm Hg, descending aortic pressure of 94/52 mm Hg, and a cardiac index of 3.0 L · min-1 · m-2 were demonstrated by cardiac catheterization.
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Fig 1. Preoperative aortography revealed moderate to severe aortic regurgitation, aneurysm of the sinus of Valsalva, and interrupted aortic arch (type A of Cerolia and Patton’s categories) with well-developed collaterals to the hypoplastic descending aorta.
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Fig 2. Preoperative magnetic resonance imaging also demonstrated (A) the hypoplastic descending aorta (black arrow) and (B) the well-developed collateral circulation (black arrows).
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At the first operation, the IAA was repaired with intrathoracic lateroisthmic bypass grafting. Pressure in the right radial artery and the left femoral artery, pulmonary artery pressure, and somatosensory evoked potentials were monitored and the degree of AR was assessed by transesophageal echocardiography. A relatively small left posterolateral thoracotomy with a 20-cm incision was made to preserve collateral circulation. The left subclavian artery was dilated to a diameter of 15 mm, whereas the descending aorta was hypoplastic with a diameter of 17 mm with lots of collaterals. A 16-mm Hemashield woven Dacron graft (Meadox Medical, Inc, Boston, MA) was anastomosed to the left subclavian artery with a thin wall. Thereafter, partial clamping of the descending aorta with preservation of collateral circulation induced no remarkable changes in hemodynamics, somatosensory evoked potentials, or AR, thus allowing us to perform distal anastomosis to the descending aorta without temporary bypass. The pressure gradient between the radial artery and the femoral artery decreased from 50 mm Hg to 12 mm Hg. The patient recovered without deterioration of cardiac function and with a short stay.
Two months later, the second intervention was performed through a median sternotomy. The coronary orifices were anomalously positioned above the rim of the sinus of Valsalva with an aneurysm. After excision of the aortic valve with dilated bicuspid leaflets, a composite graft made of a 25-mm Carbomedics mechanical valve (Carbomedics, Inc, Austin, TX) and 26-mm Hemashield woven Dacron graft was inserted into the aortic root. Coronary arteries were reimplanted to the graft by the "button technique." Finally, the distal graft end was anastomosed to the ascending aorta. The aortic cross-clamping time was 111 minutes and the cardiopulmonary bypass time was 151 minutes. This second procedure was also performed without allogeneic transfusion. The patient made an uneventful recovery with a short hospital stay (Fig 3).
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Fig 3. Postoperative digital subtraction angiography showed satisfactory results of the two interventions.
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Comment
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Interrupted aortic arch in adulthood is a rare complex condition with coexisting or secondary lesions on the aortic valve or the ascending aorta caused by long-standing proximal hypertension or its own pathology. The patient had substantial AR and a sinus of Valsalva aneurysm. Initially, we scheduled a one-stage repair for both lesions, which has been reported [2, 3]. However, there were concerns about challenging aspects of this rare condition in adults, differing from that in children. The patient might hemorrhage because of the well-developed collaterals in a one-stage repair in which there could be difficulty in reaching the hypoplastic descending aorta via a sternotomy. Principally, bypass grafts for IAA have been extraanatomic between the ascending aorta and the abdominal aorta [2, 3] except in one report [4] in which a bypass graft was anastomosed to the retrocardiac descending aorta for aortic coarctation. However, the extraanatomic bypass grafts might have technical difficulties and induce potential abdominal disorders. On the other hand, intrathoracic lateroisthmic bypass [5] was preferable physiologically and easier to establish with minimal invasion.
There has been a shortage of detailed descriptions of two-stage repair of this rare complex. Herein, we describe our two-stage intervention. The first stage involves temporary bypass during the lateroisthmic bypass. For IAA with sufficient collaterals, temporary occlusion of the descending aorta may be considered safe without temporary bypass. However, the extremely rare condition of adult IAA gave us apprehension about the safety without temporary bypass. Therefore, we managed to preserve collateral circulation by minimally invasive thoracotomy and by partial side-clamping on the descending aorta without occlusion of the intercostal arteries. The preservation of collateral circulation was confirmed by no remarkable change in the left femoral artery pressure or somatosensory evoked potentials. Likewise, pressure overload on the heart with AR induced by this clamp was estimated to be tolerable by monitoring pulmonary capillary wedge pressure and AR grade, which did not worsen during clamping. Neither neurologic disorders nor cardiac dysfunction developed postoperatively.
Another aspect of our procedure was avoidance of allogeneic transfusion. With predonation of 1,200 mL of his own blood during the interval between the two interventions, the patient did not require transfusion at either procedure, although he weighed 40 kg and had a preoperative hemoglobin level of 12 mg/dL. Concerning the proper interval between the two interventions, there was a concern that the patient would bleed from the lateroisthmic bypass graft under full heparinization, with too early a second procedure. It has been reported that the sealing effect of the Hemashield graft gradually disappears after implantation and it takes 6 weeks for tissue ingrowth to be completed [6]. On this basis, we estimated 2 months as an adequate interval for the patient, who did not bleed from the graft during the second procedure.
Finally, a two-stage repair consisting of a lateroisthmic bypass and aortic root reconstruction was considered favorable as a less invasive intervention for this rare complex pathology and produced a smooth recovery with lower morbidity.
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References
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- Cerolia G.C., Patton R.B. Congenital absence of the aortic arch. Am Heart J 1959;58:407-413.[Medline]
- Burton B.J.L., Kallis P., Bishop C., Swanton R.H., Pattison C.W. Aortic root replacement and extraanatomic bypass for interrupted aortic arch in an adult. Ann Thorac Surg 1995;60:1400-1402.[Abstract/Free Full Text]
- Ohuchi H., Kawazoe K., Kosakai Y., Kitoh Y., Kawashima Y. One-stage repair for coarctation of the aorta and annuloaortic ectasia with severe aortic regurgitation in a patient with Turner syndrome. Nihon Kyobugeka Gakkaishi 1992;40(12):123-127.
- Vijayanagar R., Natarajan P., Eckstein P.F., Bognolo D.A., Toole J.C. Aortic valvular insufficiency and postductal aortic coarctation in the adult. J Thorac Cardiovasc Surg 1980;79:266-268.[Abstract]
- Grinda J.M., Mace L., Dervanian P., Folliguet T.A., Neveux J.Y. Bypass graft for complex forms of isthmic aortic coarctation in adults. Ann Thorac Surg 1995;60:1299-1302.[Abstract/Free Full Text]
- Hirt S.W., Aoki M., Demenrtzis S., Siclari F., Haverich A., Borst H.G. Comparative in vivo study on the healing qualities of four different presealed vascular prostheses. J Vasc Surg 1993;17:538-545.[Medline]
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Abstract
Introduction
