Interactive Cardiovascular and Thoracic Surgery 2:231-233(2003)
© 2003 European Association of Cardio-Thoracic Surgery
Segmental agenesia of the descending aorta treated by extra-anatomic bypass
Pascal Berdat,
Mladen Pavlovic,
Jean-Pierre Pfammatter and
Thierry Carrel*
Clinic for Cardiovascular Surgery and Division of Pediatric Cardiology, University Hospital Berne, CH-3010 Berne, Switzerland
* Corresponding author. Tel.: +41-31-632-23-75; fax: +41-31-632-44-43
thierry.carrel{at}insel.ch
Received November 7, 2002;
accepted February 12, 2003
 |
Abstract
|
|---|
We present a 12-year-old girl with clinical manifestation of restenosis after coarctation repair in the neonatal period. However, MR-angiography revealed hypoplasia of the distal arch, complete absence of the descending aorta and severe hypoplasia of the thoraco-abdominal aorta. Treatment consisted of an extra-anatomic ascending–descending PTFE graft via sternotomy with normalization of upper-body hypertension and disappearance of leg claudication. It demonstrates how this very rare pathology of true segmental aortic agenesia may successfully be treated in older children with coexisting aortic arch or isthmus anomalies. Long-term follow-up is needed to assess the potential benefit of this approach.
Key Words: Aortic agenesia; Aortic atresia; Coarctation; Extra-anatomic aortic bypass
|
1. Introduction
|
|---|
Atresia of the aorta is best known from arch anomalies while the descending thoracic aorta is rarely affected with only few cases of juxtaductal [1,2] or thoracoabdominal [3–5] aortic atresia reported. True agenesia with complete segmental absence of aortic tissue, however, is extremely rare. We present a case with juxtaductal coarctation combined with agenesia of the mid- and distal descending thoracic aorta.
|
2. Clinical summary
|
|---|
A 12-year-old girl was referred to the nephrologist because of severe refractory arterial hypertension. In the neonatal period a typical juxtaductal short coarctation had been diagnosed echocardiographically, confirmed by intravenous digital subtraction angiography, however, without adequate visualization of the remaining descending aorta and surgically repaired with resection and end-to-end anastomosis. At the age of 7, she underwent right postero-lateral thoracotomy and resection of a benign mesenchymatous tumor of the esophagus. Repetitive dilations of esophageal strictures were necessary during which increasing upper body blood pressure was diagnosed. Despite a combined anti-hypertensive treatment systolic blood pressure usually exceeded 160 mmHg. Echocardiographic re-evaluations revealed mild coarctation restenosis and hypoplasia of the distal arch not deemed to be sufficiently severe to warrant re-intervention for several years. Recently, exertional leg pain with a pain-free walking distance of only 20–30 m developed. At clinical examination femoral pulses were absent and leg muscles hypotrophic. MR-angiography showed the surprising finding of agenesia of the mid-portion of the descending and severe hypoplasia of the thoraco-abdominal aorta with a diameter of 5 mm (Fig. 1A,B) and duplex-ultrasound showed pathologic flow patterns in all visceral arteries.
View larger version (66K):
[in this window]
[in a new window]
|
Fig. 1 (A) MR angiography (transverse section) demonstrating complete absence of aortic tissue (arrow) and (B) (right anterior oblique section) severe hypoplasia of the distal aortic arch (white arrow) and descending aorta (black arrow). Note the enlarged internal thoracic arteries (small white arrow).
|
|
Surgical exploration and an extra-anatomic bypass using a 10-mm ring-reinforced PTFE vascular prosthesis was performed between the ascending and distal descending aorta through a median sternotomy and aortic side-clamping. The distal descending aorta was exposed elevating the apex of the heart using a deep pericardial stitch in analogy to OPCAB surgery. Immediately after the operation, upper body systolic blood pressure decreased to 120 mmHg and anti-hypertensive treatment could be reduced to propranolol and low-dose enalapril.
Postoperative MR-angiography demonstrated a perfect result (Fig. 2). At follow-up, the girl is doing well and all signs of visceral and lower body hypoperfusion had completely receded.
View larger version (118K):
[in this window]
[in a new window]
|
Fig. 2 MR angiography 4 months postoperatively showing an excellent result (black arrow: graft, white arrow: distal anastomosis).
|
|
|
3. Comment
|
|---|
Agenesia of the descending thoracic aorta is extremely rare and to our knowledge no such case has ever been reported. Agenesia rather than atresia is probably present in this case, since no rudimental aortic tissue at all could be demonstrated by MRI. Agenesia may mimic or coexist with coarctation and therefore, full assessment of the descending aorta is necessary in patients with significant gradients, upper body hypertension and absent femoral pulses. Surgical correction with restoration of aortic continuity is usually indicated and may be achieved by direct anastomosis, orthotopic graft placement or extra-anatomic aortic bypass. With agenesia of short segments resection and direct anastomosis with preserved growth potential and no graft material used may be a good option in very young children. Orthotopic graft placement may be considered in older children or adolescents, when a graft of adult size can be placed to avoid later outgrowth, in first operations, in patients with normal aortic arch and adequately developed proximal and distal anastomotic segments. Both procedures require thoracotomy or even thoraco-phreno-laparatomy with an increased risk of bleeding from extensive collaterals. Furthermore, construction of an anastomosis may be troublesome since the proximal and distal segments of the aorta may not be of good quality, and short- and long-term results of this procedure are currently unknown in children. With agenesia of longer segments, in reoperations, and in patients with anomalous aortic arch, extra-anatomic aortic bypass may be useful. It allows approach via sternotomy, avoiding previous operation fields and concomitant bypass of stenotic aortic arch segments without the need for CPB and possible DHCA [6–10]. It also avoids construction of an anastomosis with potentially abnormal aortic segments and growth-related problems are rarely reported [7,9,10]. The risk of paraplegia, always present in operations on the descending aorta, may be considered low due to the formation of an extensive collateral circulation in these cases.
This report presents a very rare case of true segmental agenesia of the descending aorta. It demonstrates that this pathology can successfully be treated with extra-anatomic aortic ascending-descending bypass, especially in older children and with coexisting aortic arch or isthmus anomalies. Long-term follow-up is needed to assess the potential benefit of this treatment approach.
doi:10.1016/S1569-9293(03)00040-9
|
References
|
|---|
- Baron O, Remadi J, Lefevre M, Duveau D. One-stage repair of aortic isthmus atresia, aortic arch hypoplasia and ascending aorta aneurysm via midline sternotomy and normothermic perfusion. Eur J Cardiothorac Surg. 2002;22:457[Abstract/Free Full Text]
- Onuzo O, Rigby M, Redington A. Juxtaductal aortic atresia masquerading as coarctation. Pediatr Cardiol. 1993;14:191–193[CrossRef][Medline]
- Connolly JE, Wilson SE, Lawrence PL, Fujitani RM. Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies. J Am Coll Surg. 2002;194:774–781[CrossRef][Medline]
- Amaral FT, Ribeiro PJ, Salgado HC. Congenital coarctation of the lower thoracic aorta. A rare surgically correctable cause of hypertension in the young–case report. Int J Cardiol. 1993;39:109–111[CrossRef][Medline]
- Neirotti RA, Malcolm DD, Sugiyama GT, Fosse GR. Unusual form of coarctation of the distal thoracic aorta. Ann Thorac Surg. 2001;72:1395–1396[Abstract/Free Full Text]
- Barron DJ, Lamb RK, Ogilvie BC, Monro JL. Technique for extraanatomic bypass in complex aortic coarctation. Ann Thorac Surg. 1996;61:241–244[Abstract/Free Full Text]
- Connolly HM, Schaff HV, Izhar U, Dearani JA, Warnes CA, Orszulak TA. Posterior pericardial ascending-to-descending aortic bypass: an alternative surgical approach for complex coarctation of the aorta. Circulation. 2001;104:I133–I137[Medline]
- Grinda JM, Mace L, Dervanian P, Folliguet TA, Neveux JY. Bypass graft for complex forms of isthmic aortic coarctation in adults. Ann Thorac Surg. 1995;60:1299–1302[Abstract/Free Full Text]
- Heinemann MK, Ziemer G, Wahlers T, Kohler A, Borst HG. Extraanatomic thoracic aortic bypass grafts: indications, techniques, and results. Eur J Cardiothorac Surg. 1997;11:169–175[Abstract]
- Kanter KR, Erez E, Williams WH, Tam VK. Extra-anatomic aortic bypass via sternotomy for complex aortic arch stenosis in children. J Thorac Cardiovasc Surg. 2000;120:885–890[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
A. Al-Khaldi, F. Alhabshan, O. Tamimi, and N. Jha
Repair of aortic arch atresia with diffuse hypoplasia of the descending thoracic aorta
Eur. J. Cardiothorac. Surg.,
April 1, 2008;
33(4):
751 - 753.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
Top
Abstract
1. Introduction