Abdominal aortic coarctation: Surgical treatment of 53 patients with a thoracoabdominal bypass, patch aortoplasty, or interposition aortoaortic graft

Article Outline

• Abstract
• Methods
  • Patient characteristics
  • Etiology
  • Aortic disease
  • Aortic branch disease
  • Clinical manifestations
• Results
  • Surgical treatment
  • Operative outcomes
• Discussion
  • Aortic coarctation character
  • Associated renal and splanchnic arterial disease
  • Clinical manifestations
  • Pathogenesis
  • Treatment options
• Author contributions
• Acknowledgment
• References
• Copyright

Objective

Abdominal aortic coarctation is uncommon and often complicated with coexisting splanchnic and renal artery occlusive disease. This study was undertaken to define the clinical and anatomic characteristics of this entity, as well as the technical issues and outcomes of its operative treatment.

Methods

Fifty-three patients, 34 males and 19 females, underwent surgical treatment of abdominal aortic coarctations from 1963-2008 at the University of Michigan. Patient ages in years ranged from 2-4 (n = 4), 5-8 (n = 17), 9-14 (n = 16), 15-20 (n = 11) and 25-49 (n = 5). The mean age was 11.9 years. Developmental disease (n = 48), inflammatory aortitis (n = 4), and iatrogenic trauma (n = 1) were suspected etiologies. Aortic coarctations were suprarenal (n = 37), intrarenal (n = 12), or infrarenal (n = 4). Patients often had coexisting occlusive disease of the splanchnic (n = 33) and renal (n = 46) arteries.

Results

Major clinical manifestations included: aortic and renal artery-related secondary hypertension (n = 50), symptomatic lower extremity ischemia (n = 3), and intestinal angina (n = 3). Primary aortic reconstructive procedures included: thoracoabdominal bypass (n = 26), patch aortoplasty (n = 24), or an aortoaortic interposition graft (n = 3). Primary splanchnic (n = 19) or renal (n = 47) arterial reconstructions were performed as simultaneous (n = 45) or staged (n = 13) procedures in relation to the aortic surgery. Benefits existed regarding improved control of hypertension (n = 46), as well as elimination of extremity ischemia (n = 3) and mesenteric angina (n = 3). Secondary renal or splanchnic arterial reoperations (n = 8) were performed without mortality 5 days to 12 years postoperative for failed primary procedures. Secondary aortic procedures, 5 to 14 years postoperative, were performed for patch aortoplasties that became stenotic (n = 2) or aneurysmal (n = 1), and when thoracoabdominal bypasses developed an anastomotic narrowing (n = 1) or proved inadequate in size with patient growth (n = 1). No perioperative mortality accompanied either the primary or secondary aortic reconstructive procedures.

Conclusion

Abdominal aortic coarctation represents a complex vascular disease. Individualized treatment changed little over the period of study, remaining dependent on the pattern of anatomic lesions, patient age, and anticipated growth potential. This experience documented salutary outcomes exceeding 90% following carefully performed operative therapy.

Coarctation of the abdominal aorta is a rare disease encompassing many differing etiologies and diverse methods of treatment.¹, ², ³, ⁴, ⁵, ⁶, ⁷, ⁸, ⁹, ¹⁰, ¹¹, ¹², ¹³, ¹⁴, ¹⁵, ¹⁶, ¹⁷, ¹⁸, ¹⁹, ²⁰ The impetus for this report was to better define the anatomic and clinical character of this entity, as well as the technical issues and adequacy of conventional open vascular reconstructive procedures in treating a broad spectrum of abdominal aortic coarctation. Benefits of surgical repair were assessed with an emphasis on long-term follow-up.

Back to Article Outline

Methods 

Patient characteristics 

Fifty-three patients with abdominal aortic coarctation underwent operative treatment at the University of Michigan Medical Center from 1963 to 2008. The series included 34 male and 19 female patients, ranging in age from 2 to 49 years, with a mean age of 11.9 years. Patient ages in years were: 2-4 (n = 4), 5-8 (n = 17), 9-14 (n = 16), 15-20 (n = 11) and 25-49 (n = 5). Male patients outnumbered females 24:13 under age 15 years and 10:6 over 15 years.

Two earlier publications from this institution in 1979 and 1981 included 10 of the current series' patients.⁸, ¹⁶ Two additional publications, one on pediatric splanchnic arterial disease in 2002, and another on pediatric renovascular hypertension in 2006, briefly referred to this entity in children.²¹, ²² The current report evolved from a retrospective review of existing medical records, direct communications with the patients or their families, as well as referring physicians. Statistical analysis of anatomic differences, patient demographics, and treatment variations compared to outcomes were undertaken. This study was approved by the University of Michigan Medical School Institutional Review Board (HUM 00006223).

Etiology 

Developmental coarctations were suspected in the majority (n = 48) of this series' patients. A developmental etiology received support in the confirmed coexistence of neurofibromatosis-1 (NF-1) in 14 patients, an excessive number of multiple renal arteries in 16 of the 37 patients with suprarenal narrowings, Williams Syndrome in 1 patient, and Alagille syndrome in another patient. Certain developmental aortic narrowings may have evolved from a quiescent aorto-arteritis, yet no discernable evidence existed to confirm such a diagnosis. Nevertheless, others exhibited atypical abdominal aortic coarctations having the characteristics of an inflammatory aortitis (n = 4). Balloon angioplasty of a thoracic aortic coarctation in a newborn at another hospital, complicated by aortic thrombosis, was considered the cause of an intrarenal coarctation (n = 1) in the series remaining patient.

Aortic disease 

Extensive preoperative imaging of the aorta, the celiac artery (CA), superior mesenteric artery (SMA), and renal arteries was undertaken in all patients (Fig 1, Fig 2, Fig 3). Imaging during the earlier years of this experience involved catheter-based aortography. Magnetic resonance angiography (MRA) subsequently became a common diagnostic test, but conventional arteriography was often necessary to confirm the hemodynamic importance of aortic branch stenoses. Thin-cut computed tomographic arteriography (CTA) has improved the anatomic characterization of many complex lesions, and is currently the single most frequently used preoperative study.

• 
  • View Large Image
  • Download to PowerPoint

• Fig 1. 

  A, Suprarenal abdominal aortic coarctation (bracket) with superior mesenteric artery stenosis (arrow) and (B) bilateral renal artery ostial stenoses (arrows). Note common trunk of lower lumbar artery (arrow). Preoperative computed tomographic angiography (CTA) (anterior and posterior projections, respectively).

• 
  • View Large Image
  • Download to PowerPoint

• Fig 2. 

  Intrarenal abdominal aortic coarctation with bilateral artery stenoses (arrows). Preoperative aortogram.

• 
  • View Large Image
  • Download to PowerPoint

• Fig 3. 

  Infrarenal abdominal aortic coarctation with an associated spontaneous pseudoaneurysm (arrow). Preoperative aortogram.

Suprarenal abdominal aortic coarctations (n = 37), beginning above the celiac artery or superior mesenteric artery included 5 patients with diffuse aortic hypoplasia, extending three times from the mid thoracic aorta to the mid abdominal aorta, and twice from the upper-most abdominal aorta to below the inferior mesenteric artery. In the later cases, the narrowing was most severe in the upper abdominal aorta. Among suprarenal coarctations, all but four exhibited renal or splanchnic arterial stenoses or occlusions. Intrarenal coarctations (n = 12), beginning above the renal arteries, but below the SMA, were all associated with renal artery disease. Infrarenal coarctations (n = 4), beginning below the renal arteries were focal in one case, presented as long tubular narrowings twice, and were associated with a terminal aortic occlusion once. Two of the four infrarenal coarctations exhibited renal artery stenoses.

Aortic branch disease 

Renal artery stenoses affected 46 patients, being most often ostial (n = 44) and bilateral (n = 41). Aneurysms affected the proximal renal artery in 3 patients and the distal renal artery in 1 patient. Multiple renal arteries accompanied 43% of the suprarenal abdominal aortic coarctations. Splanchnic arterial disease included CA and SMA ostial stenoses or occlusions (n = 33), involving both vessels in all but 6 patients, as well as a CA aneurysm (n = 1) and common celiaco-mesenteric trunks (n = 4) of which one was aneurysmal.

Clinical manifestations 

Refractory hypertension was the major clinical manifestation, affecting 50 patients, whose mean preoperative blood pressures were 164/112 mm Hg while on antihypertensive medications. Appropriate published standards for normal blood pressure were used in the pediatric-aged patients.²³ Hypertension was classified as cured if the patient was taking no antihypertensive medications and they were normotensive for the preceding 6 months; improved if they were normotensive while on drug therapy exclusive of angiotensin-converting enzyme (ACE) inhibitors, or if their diastolic pressure was higher than normal but 15% lower than preoperative levels; and failures if the diastolic pressure was higher than the normal and not 15% lower than preoperative levels or if ACE inhibitors were required for blood pressure control.

Three patients experienced intestinal angina with weight loss. Three additional patients described ischemia-related lower extremity fatigue with ambulation and had abnormal ankle-brachial indices that declined following treadmill exercise.

Back to Article Outline

Results 

Surgical treatment 

Aortic reconstructions included thoracoabdominal bypasses, patch aortoplasties, and interposition aortoaortic grafts. No major therapeutic changes occurred over the decades of practice, with specific interventions being dependent on the patient's age, and the pattern of the aortic disease as well as the associated renal and splanchnic arterial disease (Table I). Statistical analyses did not find a correlation between the patient's age, aortic anatomy, and treatment type, to the outcome of survival or improvement in the patient's clinical status.

Table I. Patterns of aortic and aortic branch reconstructive procedures

	Aortic Coarctation
	Suprarenal (n = 37)	Intrarenal (n = 12)	Infrarenal (n = 4)
Patch aortoplasty (n = 24)
Concomitant renal procedure	14	7	1
Concomitant splanchnic procedure	10	1	0
Staged renal procedure	4*	4*, 1†	0
Staged splanchnic procedure	1	1†	0
No renal or splanchnic procedure	1	0	0
Thoracoabdominal bypass graft (n = 26)
Concomitant renal procedure	17	3	0
Concomitant splanchnic procedure	14	1	0
Staged renal procedure	1*, 2†	0	0
Staged splanchnic procedure	0	0	0
No renal or splanchnic procedure	4	0	1
Interposition aortic graft (n = 3)
Concomitant renal procedure	0	1	1
Concomitant splanchnic procedure	0	0	1
Staged renal procedure	0	1*	0
Staged splanchnic procedure	0	1†	0
No renal or splanchnic procedure	0	0	1

Renal Procedure – Number of primary unilateral or bilateral renal artery reconstructive procedures.

Splanchnic Procedure – Number of primary celiac, superior mesenteric, or combined celiac and superior mesenteric arterial reconstructive procedures.

Concomitant – Procedure performed at the same time of aortic reconstruction.

Staged – Procedure performed either before* or after^† aortic reconstruction.

Thoracoabdominal bypass grafts (n = 26) originated from the distal thoracic aorta above the diaphragm or from the supraceliac aorta at the diaphragmatic hiatus, being passed behind the left kidney to the distal aorta (Fig 4). In most older patients, aortic exposure was facilitated by a thoracoabdominal incision through the left sixth or seventh intercostal space extending from the posterior axillary line across the costal margin, onto the abdomen, in either an oblique fashion to the right of the umbilicus or as a midline incision to just above the pubis. In younger children and adolescents, a transverse supraumbilical abdominal incision was used most often, extending laterally to the posterior axillary lines, combined with medial rotation of the viscera, allowing access to the abdominal aorta from its supraceliac level at the aortic hiatus to the origin of the iliac arteries.

• 
  • View Large Image
  • Download to PowerPoint

• Fig 4. 

  A, Suprarenal coarctation (bracket) with superior mesenteric artery stenosis (arrow). Preoperative magnetic resonance angiography (MRA). B, Thoracoabdominal bypass (broad arrow) with aortic implantation of superior mesenteric artery (arrow). Postoperative computed tomographic angiography (CTA).

Dacron graft knitted or woven thoracoabdominal grafts (n = 9) were used in the earlier experience, with expanded Teflon grafts (n = 17) used more often in recent years because of their greater stability regarding postimplantation dilatation. Graft diameter was chosen to be as big as possible, short of being so large that excessive luminal thrombus would accumulate. In children, the intent was always to oversize grafts compared to the aorta, with anticipated growth otherwise resulting in a graft too small to maintain normal distal pressures and flow. In the ideal circumstance, one should use a graft whose size would not represent an energy-consuming constriction as the patient grows into maturity. This means having a conduit at least 60% or 70% the size of the adult aorta. This translates into using 8-12 mm grafts in young children, 12-16 mm grafts for early adolescents, and 14-20 mm grafts in late adolescents and adults. In the very young child, use of large conduits may not be possible. Graft length was a non-issue in older children and adolescents, with axial growth from the diaphragm to pelvis being minimal after age 9 or 10 in late childhood.

Patch aortoplasty (n = 24) was usually undertaken when the coarctation segment had a large enough diameter to allow completion of an anastomosis without an overlap of sutures from the opposing sides of the patch (Fig 5). Whenever possible, patches in children were made sufficiently large enough, similar to thoracoabdominal graft sizing, so as to not be constrictive with growth into adulthood, yet not so generous as to risk development of an extensive lining of unstable thrombus. Teflon graft material was again favored over Dacron graft, because of the latter's propensity for dilatation years after implantation.

• 
  • View Large Image
  • Download to PowerPoint

• Fig 5. 

  A, Suprarenal coarctation (bracket) with bilateral renal artery ostial stenoses. Preoperative MRA. B, Patch aortoplasty (broad arrow) with aortic diameter exceeding that of uninvolved proximal and distal aorta. Reimplantation of the renal arteries accompanied the aortic reconstruction. Postoperative computed tomographic angiography (CTA).

Interposition aortoaortic grafts (n = 3) were used in treating one suprarenal and two infrarenal coarctations. The latter were placed using surgical techniques similar to those used in aortic aneurysm surgery. Exposure was through the retroperitoneum at the root of the mesocolon and small bowel mesentery, from the level of the left renal vein to the aortic bifurcation.

Primary renal and splanchnic arterial reconstructions were performed as simultaneous (n = 45) or staged (n = 13) procedures in relation to the patient's aortic surgery. When simultaneous reconstructions were performed, they usually were done following completion of the aortoplasty or aortic bypass. Among the staged operations, nine were prior to the aortic reconstruction, and four occurred after the aortic procedure. These nonaortic reconstructions included direct aortic implantations of the normal renal or splanchnic artery beyond the resected stenotic segment, as well as internal iliac aorto-visceral bypasses. Implantation of these arteries into a synthetic conduit or patch is not recommended because of the greater potential for later anastomotic narrowing. Four patients underwent a nephrectomy for unreconstructable renal artery disease, twice before the aortic procedure and two times at the time of the aortic reconstruction. These procedures were the topic of earlier publications by the authors.²¹, ²², ²⁴

Operative outcomes 

No perioperative mortality followed the primary aortic or combined aortic and visceral arterial reconstructive procedures. Similarly, there were no ischemic intestinal complications or patients having postoperative renal insufficiency requiring dialysis. One patient with Moya Moya syndrome incurred a perioperative stroke and made a satisfactory recovery.

Early reoperations were performed without sequelae for nonaortic-related complications, including intra-abdominal bleeding (n = 2), and a renal artery anastomotic pseudoaneurysm (n = 1). Life table analysis documented excellent aortic graft patencies (Table II). Late reoperative aortic surgery occurred in 2 patients having thoracoabdominal bypasses. One outgrew the original graft and had a replacement graft 7 years postoperatively, and the other underwent revision of a proximal anastomotic stenosis 9 years postoperatively. Three patients with patch aortoplasties had late reoperations. Two with patches that proved inadequate in size with growth underwent thoracoabdominal bypasses, performed 5 and 10 years after their initial aortic procedure. The third patch failure, aneurysmal deterioration of the aorta at the site of the aortoplasty, occurred 14 years postoperatively and was treated with placement of an interposition aorto-aortic graft.

Table II. Life table analysis of aortic graft patency over time in 53 patients with mid abdominal aortic coarctation

Reoperations were anticipated in the 3 children who outgrew their original aortic reconstructions performed at ages 5, 6, and 8 years. The initial aortic procedure was considered essential for the child's health, could not be deferred to an older age, and could not safely have encompassed an initial aortic repair of greater dimensions. No perioperative mortality or major complications accompanied the aortic reoperations.

Thirty-nine of this series' patients had patent and satisfactory function of their visceral artery reconstructions (63 renal and 16 splanchnic individual revascularizations) at the latest time these vessels were imaged, averaging 5.4 years postoperative. Nine patients, including 4 of the former individuals and 5 additional patients experienced failed visceral artery reconstructions (10 renal and 3 splanchnic revascularizations). These reconstructive failures included aneurysmal deterioration of four aortorenal bypass grafts, which are not used in contemporary practice. Eight patients with failed visceral reconstructions underwent successful reoperations 5 days to 12 years postoperatively, without mortality. The remaining patient's failed visceral reconstruction did not require reoperation. Life table analysis of renal and splanchnic procedures revealed excellent patencies (Table III).

Table III. I. Life table analysis of reconstructive patency over time in 92 renal and splanchnic revascularization procedures in patients with mid abdominal aortic coarctation

Benefits of surgical therapy were reflected in cured (n = 28) or improved (n = 18) hypertension in 46 of the 53 patients. Their mean postoperative pressure was 121/72 mm Hg at the most recent time of follow-up. This represented a significant decrease compared to the mean preoperative pressure (P < .01). Four patients had no change in their hypertensive state. All were subjected to repeated operations, including two with secondary intraluminal stent placement (one aortic and one renal artery). The 3 patients with symptomatic lower extremity ischemia experienced complete relief following surgery. The 3 patients with intestinal angina had resolution of their postprandial abdominal pain and all had rapid weight gain.

Six patients died late in follow-up, with none directly related to the patient's aortic disease or surgery. These deaths in patients ranging in age from 23 to 70 years, occurred 9 to 26 years postoperatively, and were due to trauma (n = 2), cancer (n = 2), stroke (n = 1) and myocardial infarction (n = 1). Follow-up averaged 5.9 years. Contemporary follow-up was complete in all but 4 of the 47 surviving patients. The patients' clinical status in the case of those individuals residing at long distances from the authors' hospital was obtained by direct contact with the referring physician. Forty-one of the series' 53 patients have been treated since 1994.

Back to Article Outline

Discussion 

Abdominal aortic coarctation is uncommon and, as evident in the present series, is often associated with coexisting splanchnic and renal artery occlusive disease. Differences in the reported clinical relevance of abdominal aortic coarctations usually reflect differences in the etiology of coarctations being treated at a given institution.

Aortic coarctation character 

An earlier collective review of 119 cases identified suprarenal coarctations in 11%, intrarenal coarctations in 54%, infrarenal coarctations in 25%, and diffuse aortic hypoplasia in 12%.⁸ Differences in the current series, with 69% suprarenal, 23% intrarenal, and 8% infrarenal, reflect a more contemporary classification of aortic coarctation, based upon the most superior level of the narrowing. Indeed, it is the most cephalic extent of the disease that defines the complexity of the aortic reconstruction, with considerable differences if the CA and SMA are involved, compared to the renal arteries alone. Most aortic coarctations are diminutive vessels, often with an hour-glass narrowing representing a lack of growth in developmental lesions or contraction in cases of an inflammatory aortitis.

Associated renal and splanchnic arterial disease 

Nearly 80% of patients with abdominal aortic developmental lesions have been reported to have renal artery stenoses,⁸ a finding consistent with the present series in which 87% had renal artery narrowings or occlusions. Splanchnic arterial occlusive disease has been previously reported to affect 22% of patients with abdominal aortic coarctations.⁸ The true incidence of splanchnic arterial involvement may be much greater, in that lateral aortograms have not been routinely obtained in evaluating these patients. The more complete imaging in the present series revealed 62% to have CA or SMA stenoses and occlusions, with both vessels involved in 82% of these cases. Suprarenal or infrarenal coarctations, when distant from the CA and SMA, are less likely to be associated with stenotic branch disease, compared to more centrally located abdominal aortic coarctations.

Clinical manifestations 

Most patients, like those of the current series, present with uncontrolled hypertension due to suprarenal or intrarenal aortic coarctations, with coexisting renal artery stenoses commonly associated with developmental and inflammatory-related abdominal aortic narrowings. Changes in pulsatile flow and pressure across renal stenoses or aortic narrowings are responsible for renin-angiotensin system activation and subsequent blood pressure elevations. This form of renovascular hypertension is usually resistant to simple pharmacologic control. An occasional patient reports exercise-related lower extremity fatigue, but true claudication is rare. Associated splanchnic arterial occlusive disease affects a majority of those aortic narrowings, yet symptomatic intestinal ischemia is very uncommon. In the present series, more than half manifest splanchnic occlusive lesions, yet only 6% experienced intestinal angina.

Abdominal aortic coarctations usually cause signs or symptoms during the first or second decade of life, yet an earlier review noted that patients had reached a mean age of 22 years before the diagnosis was actually confirmed.⁸ Untreated, this entity has been associated with stroke, progressive left ventricular hypertrophy with congestive heart failure and flash pulmonary edema, and less often with renal insufficiency.²² In one review, 55% of untreated patients died at a mean age of 34 years.⁸

Pathogenesis 

Many abdominal aortic coarctations appear related to events occurring around day 25 of fetal development. At that time the two embryonic dorsal aortas fuse and lose their intervening wall to form a single vessel. Overfusion of the two embryonic dorsal aortas or their failure to fuse with subsequent obliteration of one of these vessels would predictably result in an aortic narrowing.²⁵ Developmental overfusion of the two primitive dorsal aortas receives support in patients with decreased aortic diameters who have single origins of the lumbar arteries.²⁶ Such a remarkable finding occurred frequently in our series (Fig 1, B).

Multiple renal arteries to one or both kidneys in nearly half of the patients exhibiting suprarenal and intrarenal abdominal aortic coarctations exceeds the 25 to 35% observed in the general population and also supports a developmental etiology of these narrowings.⁸, ¹⁶ Normal aortic development occurs at approximately the same embryonic time that the multiple metanephric arteries involute, leaving a single renal artery. Dominance of this single renal artery is alleged to result from its obligate hemodynamic advantage over adjacent metanephric vessels. It is likely that if aortic narrowings exist, flow disturbances will occur in the vicinity of this principle renal artery and diminish its hemodynamic advantage, allowing persistence of adjacent metanephric channels. The fact that aortic narrowings distant from the renal arteries are less likely to be associated with multiple renal arteries lends further credence to this developmental hypothesis.⁸, ¹⁶, ²⁴

Viral-mediated events may impede transition of fetal mesenchymal tissue to vascular smooth muscle or alter its organization and growth in utero, and also result in developmental aortic narrowings. Certain viruses, including rubella, are cytocidal and inhibitory to cell replication, with intimal fibroplasia and aortic hypoplasia as a consequence being well recognized.²⁷, ²⁸, ²⁹ In fact, fibroproliferative intimal disorders have been documented in the aorta and large elastic arteries of 16.5% of patients exhibiting the congenital rubella syndrome.³⁰

Patients with NF-1 exhibit an unusually high frequency of arterial abnormalities, including developmental abdominal aortic coarctations and renal artery stenoses.³¹ Because of the protean nature of NF-1 and infrequent genetic analyses of patients with abdominal aortic coarctation, the exact frequency of this disease among these individuals is unknown. Nevertheless, 29% of the current series' patients carried a diagnosis of NF-1. The primary vascular pathology in neurofibromatosis appears to be related to abnormal smooth muscle growth, not entrapment or invasion of the arterial wall by neural elements.³², ³³ Similar events may affect patients with the Alagille syndrome,³⁴ and Williams' syndrome.³⁵ The authors have performed renal revascularizations in two additional Williams' syndrome patients not in the present series, who had earlier treatment of their abdominal aortic coarctations performed elsewhere.

Panaortitis with adventitial or periadventitial fibrosis and associated inflammatory cell infiltrates, suggesting an active or chronic aortitis, is another well recognized cause of abdominal aortic coarctations. The proposition that most abdominal aortic coarctations are a variant of an inflammatory aortitis like Takayasu's disease is quite controversial and not supported by histological findings.²⁰, ³⁶ This cause of aortic narrowings, suspected in only 8% of the current series, is encountered much more often in the subcontinent populations of Asia and South America.

Treatment options 

The two procedures most commonly performed in treating aortic coarctations remain open interventions. Patch aortoplasty, when technically feasible has become the authors' preferred means of treating isolated abdominal aortic coarctations. In some cases, an autologous arterial patch, especially for short coarctations, may be appropriate.³⁷ Thoracoabdominal bypass may be favored in certain patients having too small of a coarctation segment to allow placement of a patch or in cases of complex disease affecting the renal and splanchnic arteries.

Reoperations are infrequent, but may be required for anastomotic narrowings or if a patient outgrows the adequacy of the primary procedure. The fact that nearly 10% of the currently reported cases required late secondary operations supports the importance of life-long follow-up of these patients. Aneurysmal aortic deterioration in the region of a patch in one of our patients, many years after the initial reconstruction and 2 years after she completed her only pregnancy, deserves note. The effect of gestational hormones and blood pressure increases during pregnancy may be relevant, in that pregnancy-related aortic diameter increases of 1.5 cm or more have been observed at the site of thoracic aortic coarctation repairs in nearly 10% of those undergoing such an intevervention.³⁸ Although this finding may not be directly extrapolated to abdominal aortic coarctation repairs, it does justify close surveillance of those patients who subsequently become pregnant.

Simultaneous or staged aortic and visceral artery reconstructions depend on the clinical relevance of the nonaortic disease as well as the proximity of the aortic reconstruction to the affected aortic branches. Certainly, renal artery stenoses and secondary renovascular hypertension justify an aggressive reconstructive approach. A mandate to reconstruct the CA or SMA applies only to symptomatic cases. Nevertheless, a relative indication to prophylactically reconstruct these vessels exists when performance of an aortoplasty or renal revascularization would make a subsequent CA or SMA revascularization exceedingly difficult. When the aortic reconstruction was distant from the CA or SMA, such as with thoracoabdominal bypass, a concomitant splanchnic revascularization is less likely to be performed.

Treatment of select abdominal aortic coarctations may involve endoluminal stenting. Although endovascular therapy for thoracic aortic coarctation has resulted in a higher incidence of restenosis and secondary procedures than open surgery,³⁹ a technical success rate exceeding 97% and peri-procedural mortality of 0.4% associated with stenting native and recurrent coarctations of the thoracic aorta make this an appealing option for treating abdominal coarctations.⁴⁰ Current endovascular technologies appear to allow the safe treatment of focal stenoses remote from the CA, SMA, and renal arteries. Percutaneous transluminal angioplasty of an abdominal aortic coarctation was first reported in 1983.¹³ Subsequent case reports described the transition in the use of stents in these cases.¹, ⁶, ¹³ In fact, early and late failures of balloon angioplasty alone suggest that stent placement is necessary to overcome the significant recoil of these often hypoplastic and highly fibrotic aortic narrowings.⁷, ¹⁰, ¹⁷, ⁴¹

The authors remain cautious at accepting the long-term benefits of endoluminal treatment of abdominal aortic coarctation in any patient, except adults with very focal narrowings distant from their renal arteries. Given the high frequency with which the renal and splanchnic arteries are affected in abdominal aortic coarctation, especially in the younger-growing patient, the number of lesions amenable to endovascular repair is likely to be limited.

Long-term follow-up of patients undergoing surgical treatment of their abdominal aortic coarctation is warranted. No patient in the present experience has developed an unacceptable failure of their aortic reconstruction. However, follow-up in this series has been relatively brief and has involved a generally younger group of patients with long life expectancies. Annual noninvasive assessments of lower extremity blood flow with exercise ankle-brachial indices are recommended. Imaging with MRA studies or CTA should be obtained if any evidence of diminished blood flow exists. Similar imaging is appropriate if blood pressure increases occur in those who have undergone concomitant renal artery reconstructions or whose renal blood flow is dependent upon their aortic reconstruction.

Back to Article Outline

Author contributions 

Back to Article Outline

The authors appreciate contributions to this work from those referring physicians from Pittsburgh, Pa, Philadelphia, Pa, and Sioux Falls, SD, whose patients' preoperative images (Fig 1, Fig 4, A and 5, A) are included in this publication.

Back to Article Outline

References 

1. Ballweg J, Liniger R, Rocchini A, Gajarski R. Use of Palmaz stents in a newborn with congenital aneurysms and coarctation of the abdominal aorta. Catheter Cardiovasc Interv. 2006;68:648–652
   • View In Article
   • MEDLINE
   • CrossRef
2. Bergamini TM, Bernard JD, Mavroudis C, Backer CL, Muster AJ, Richardson JD. Coarctation of the abdominal aorta. Ann Vasc Surg. 1995;9:352–356
   • View In Article
   • Abstract
   • Full-Text PDF (507 KB)
   • CrossRef
3. 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
   • View In Article
   • Abstract
   • Full Text
   • Full-Text PDF (298 KB)
   • CrossRef
4. DeBakey ME, Garrett HE, Howell JF, Morris GC. Coarctation of the abdominal aorta with renal arterial stenosis: surgical considerations. Ann Surg. 1967;165:830–843
   • View In Article
   • MEDLINE
   • CrossRef
5. Delis KT, Gloviczki P. Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Persp Vasc Surg Endovasc Ther. 2005;17:187–206(Editorial comment by JC Stanley included)
   • View In Article
6. Eliason JL, Passman MA, Guzman RJ, Naslund TC. Durability of percutaneous angioplasty and stent implantation for the treatment of abdominal aortic coarctation: a case report. Vasc Surg. 2001;35:397–401
   • View In Article
   • MEDLINE
7. Fava MP, Foradori GB, Garcia CB, Cruz FO, Aguilar JG, Kramer AS, et al. Percutaneous transluminal angioplasty in patients with Takayasu arteritis: five year experience. J Vasc Interv Radiol. 1993;4:649–652
   • View In Article
   • Abstract
   • Full-Text PDF (1900 KB)
   • CrossRef
8. Graham LM, Zelenock GB, Erlandson EE, Coran AG, Lindenaeur SM, Stanley JC. Abdominal aortic coarctation and segmental hypoplasia. Surgery. 1979;86:519–529
   • View In Article
   • MEDLINE
9. Hallett JW, Brewster DC, Darling RC, O'Hara PJ. Coarctation of the abdominal aorta: current options in surgical management. Ann Surg. 1980;191:430–437
   • View In Article
   • MEDLINE
   • CrossRef
10. Lin Y-J, Hwang B, Lee P-C, Yang L-Y, Meng CCL. Mid-aortic syndrome: a case report and review of the literature. Int J Cardiol. 2007;[In Press].
    • View In Article
11. Messina LM, Reilly LM, Goldstone J, Ehrenfeld WK, Ferrell LD, Stoney RJ. Middle aortic syndrome (Effectiveness and durability of complex arterial revascularization techniques). Ann Surg. 1986;204:331–339
    • View In Article
    • MEDLINE
    • CrossRef
12. Mickley V, Fleiter T. Coarctations of descending and abdominal aorta: long-term results of surgical therapy. J Vasc Surg. 1998;28:206–214
    • View In Article
    • Abstract
    • Full Text
    • Full-Text PDF (129 KB)
    • CrossRef
13. Nanni GS, Hawkins IF, Alexander JA. Percutaneous transluminal angioplasty of an abdominal aortic coarctation. Am J Roentgenol. 1983;140:1239–1241
    • View In Article
14. Schechter C, Angelini P, Treistman B. Percutaneous balloon catheter angioplasty of coarctation of the abdominal aorta: report of two cases. Cathet Cardiovasc Diagn. 1985;11:401–407
    • View In Article
    • MEDLINE
    • CrossRef
15. Stadlmaier E, Spary A, Tillich M, Pilger E. Midaortic syndrome and celiac disease: a case of local vasculitis. Clin Rheumatol. 2005;24:301–304
    • View In Article
    • MEDLINE
    • CrossRef
16. Stanley JC, Graham LM, Whitehouse WM, Zelenock GB, Erlandson EE, Cronenwett JL, et al. Developmental occlusive disease of the abdominal aorta and the splanchnic and renal arteries. Am J Surg. 1981;142:190–196
    • View In Article
    • MEDLINE
    • CrossRef
17. Stiller B, Weng Y, Berger F. Images in cardiology (Mid aortic syndrome: a rare cause of reversible cardiomyopathy). Heart. 2006;92:640
    • View In Article
18. Terramani TT, Salim A, Hood DB, Rowe VL, Weaver FA. Hypoplasia of the descending thoracic and abdominal aorta: a report of two cases and review of the literature. J Vasc Surg. 2002;36:844–848
    • View In Article
    • Abstract
    • Full-Text PDF (108 KB)
    • CrossRef
19. Vaccaro PS, Myers JC, Smead WL. Surgical correction of abdominal aortic coarctation and hypertension. J Vasc Surg. 1986;3:643–648
    • View In Article
    • Abstract
    • Full Text
    • Full-Text PDF (3082 KB)
    • CrossRef
20. Wada J, Kazui T. Long-term results of thoracoabdominal bypass graft for atypical coarctation of the aorta. World J Surg. 1978;2:891–896
    • View In Article
    • MEDLINE
    • CrossRef
21. Upchurch GR, Henke PK, Eagleton MJ, Grigoryants V, Sullivan VV, Wakefield TW, et al. Pediatric splanchnic arterial occlusive disease: clinical relevance and operative treatment. J Vasc Surg. 2002;35:860–867
    • View In Article
    • Abstract
    • Full Text
    • Full-Text PDF (668 KB)
    • CrossRef
22. Stanley JC, Criado E, Upchurch GR, Brophy PD, Cho KJ, Rectenwald JE Michigan Pediatric Renovascular Group. Pediatric renovascular hypertension: 132 primary and 30 secondary operations in 97 children. J Vasc Surg. 2006;44:1219–1228discussion 1228-9.
    • View In Article
    • Abstract
    • Full Text
    • Full-Text PDF (871 KB)
    • CrossRef
23. National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents (2004). The fourth report on the diagnostic, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics. 2004;114(2 Suppl 4th Report):555–576
    • View In Article
    • CrossRef
24. Stanley JC, Zelenock GB, Messina LM, Wakefield TW. Pediatric renovascular hypertension: a thirty-year experience of operative treatment. J Vasc Surg. 1995;21:212–227
    • View In Article
    • Abstract
    • Full Text
    • Full-Text PDF (5716 KB)
    • CrossRef
25. Maycock WD'A. Congenital stenosis of the abdominal aorta. Am Heart J. 1937;13:633–646
    • View In Article
    • CrossRef
26. Arnot RS, Louw JH. The anatomy of the posterior wall of the abdominal aorta (Its significance with regard to hypoplasia of the distal aorta). S Afr Med J. 1973;47:899–902
    • View In Article
    • MEDLINE
27. Siassi B, Glyman G, Emmanouilides GC. Hypoplasia of the abdominal aorta associated with the Rubella syndrome. Am J Dis Child. 1970;120:476–479
    • View In Article
    • MEDLINE
28. Esterly JR, Oppenheimer EM. Vascular lesions in infants with congenital rubella. Circulation. 1967;36:544–554
    • View In Article
    • MEDLINE
29. Stewart DR, Price RA, Nebesar R, Schuster SR. Progressive peripheral fibromuscular hyperplasia in an infant: a possible manifestation of the rubella syndrome. Surgery. 1973;73:374–380
    • View In Article
    • MEDLINE
30. Singer DB, Rudolph AJ, Rosenberg HS, Rawls WE, Boniuk M. Pathology of the congenital rubella syndrome. J Pediatr. 1967;71:665–675
    • View In Article
    • Abstract
    • Full-Text PDF (3372 KB)
    • CrossRef
31. Halperin M, Currarino G. Vascular lesions causing hypertension in neurofibromatosis. N Engl J Med. 1965;273:248–252
    • View In Article
    • CrossRef
32. Finley JL, Dabbs DJ. Renal vascular smooth muscle proliferation in neurofibromatosis. Human Path. 1988;19:107–110
    • View In Article
33. Greene JF, Fitzwater JE, Burgess J. Arterial lesions associated with neurofibromatosis. Am J Clin Pathol. 1974;62:481–487
    • View In Article
    • MEDLINE
34. Quek SC, Tan L, Quek ST, Yip W, Aw M, Quak SH. Abdominal coarctation and Alagille syndrome. Pediatrics. 2000;106:E9
    • View In Article
    • CrossRef
35. Radford DJ, Pohlner PG. The middle aortic syndrome: an important feature of Williams' syndrome. Cardiol Young. 2000;10:597–602
    • View In Article
    • MEDLINE
36. Lande A. Takayasu's arteritis and congenital coarctation of the descending thoracic and abdominal aorta: a critical review. Am J Roentgenol. 1976;127:227–233
    • View In Article
37. Neirotti RA, Malcolm DD, Sugiyama GT, Fosse GR. Unusual form of coarctation of the distal thoracic aorta. Ann Thorac Surg. 2001;72:1395–1396
    • View In Article
    • MEDLINE
    • CrossRef
38. Vriend JWJ, Drenthen W, Pieper PG, Roos-Hesselink JW, Zwinderman AH, van Veldhuisen DJ, et al. Outcome of pregnancy in patients after repair of aortic coarctation. Eur Heart J. 2005;26:2173–2178
    • View In Article
    • MEDLINE
    • CrossRef
39. Carr JA. The results of catheter-based therapy compared with surgical repair of adult aortic coarctation. J Am Coll Cardiol. 2006;47:1101–1107
    • View In Article
    • Abstract
    • Full Text
    • Full-Text PDF (123 KB)
    • CrossRef
40. Forbes TJ, Garekar S, Amin Z, Zahn EM, Nykanen D, Moore P, et al. Procedural results and acute complications in stenting native and recurrent coarctation of the aorta in patients over 4 years of age (A multi-institutional study). Catheter Cardiovasc Interv. 2007;70:276–285
    • View In Article
    • CrossRef
41. Siwik ES, Perry SB, Lock JE. Endovascular stent implantation in patients with stenotic aortoarteriopathies: early and medium-term results. Catheter Cardiovasc Interv. 2003;59:380–386
    • View In Article
    • MEDLINE
    • CrossRef