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Ann Thorac Surg 1998;65:1698-1702
© 1998 The Society of Thoracic Surgeons

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Original articles: cardiovascular

Total Simultaneous Repair of Coarctation and Intracardiac Pathology in Adult Patients

^a Department of Cardiothoracic Surgery, Allegheny University of the Health Sciences-Hahnemann, Philadelphia, Pennsylvania, USA

Accepted for publication February 4, 1998.

Address reprint requests to Dr Morris, Department of Cardiothoracic Surgery, Allegheny University of the Health Sciences-Hahnemann, Broad & Vine Sts, Mail Stop 111, Philadelphia, PA 19102-1192

	Abstract

Top Abstract Introduction Patients and methods Comment References

 
Background. Thoracic aortic coarctation accompanied by a second surgically reparable lesion is a rare combination in the adult patient. The simultaneous operative management of both lesions is desirable because of the higher morbidity and mortality that would occur with staged procedures.

Methods. We describe the simultaneous operative management in three adult patients with coarctation and a second cardiac lesion. All 3 patients had intrapericardial ascending aorta–descending aorta bypass and concomitant repair of a cardiac lesion. The attendant repairs in the 3 patients, respectively, were aortic valve replacement, orthotopic heart transplantation, and coronary artery bypass grafting.

Results. Double arterial cannulation, retrograde cardioplegia, large-bore aorto–aortic bypass grafts, and early use of -agonists to stabilize systemic pressure were all key to ensuring safe conduct of the operation. Each patient had an essentially uneventful postoperative course.

Conclusions. Thoracic coarctation and concomitant cardiac pathology can be safely and readily managed with a single-stage approach involving cardiac repair and extraanatomic ascending aorta–descending aorta bypass grafting. A review of the English-language literature of patients managed similarly is included.

	Introduction

Top Abstract Introduction Patients and methods Comment References

 
Coarctation of the thoracic aorta is a common congenital anomaly, which was first surgically corrected more than 50 years ago by Crafoord and Nylin [1]. They performed complete resection and end-to-end anastomosis on 2 young patients, a procedure later popularized in the United States by Gross [2]. Other approaches have been described, including subclavian flap angioplasty, patch aortoplasty, and interposition grafting. Recent nonsurgical methods of management have included intraluminal balloon angioplasty and stent placement. However, coarctation occasionally presents in adulthood, when direct repair may be contraindicated for serious medical problems. Recurrent coarctation may be problematic because of scarring at the site of previous repair and because it can also present with intracardiac lesions in adulthood. We describe here 3 patients with thoracic aortic coarctation and concomitant cardiac pathology, and present a review of our current surgical method to treat this difficult combination.

	Patients and methods

Top Abstract Introduction Patients and methods Comment References

 
Patient 1
A 72-year-old man with a history of hypertension and congestive heart failure was admitted for increasing shortness of breath and dizziness. Critical aortic stenosis, with an aortic valve area of 0.7 cm², was diagnosed by echocardiography. At the time of cardiac catheterization, a 60-mm gradient was noted across a classic coarctation in the descending thoracic aorta. There were normal coronary arteries, global left ventricular dysfunction, an ejection fraction of 0.25, and a 30-mm Hg gradient across the aortic valve. The aortic valve stenosis was deemed to be critical, and with severe left ventricular dysfunction we believed a staged approach was not feasible. The use of cardiopulmonary bypass during valve replacement would allow us to dislocate the heart and expose the descending thoracic aorta through the sternotomy incision only. We decided, therefore, that he should undergo single-stage operation involving aortic valve replacement and transpericardial ascending aorta-descending aorta bypass.

Cardiopulmonary bypass was established with double arterial cannulation in the ascending aorta and right femoral artery, to ensure adequate perfusion proximal and distal to the coarctation. Bicaval cannulation through the right atrium was employed for venous return. The ascending aorta was cross-clamped, and antegrade blood cardioplegia was used to arrest the heart. Additional cardioplegic delivery was given in a retrograde manner. The heart was retracted superiorly, and the descending thoracic aorta exposed just above the diaphragm by incising the posterior pericardium. A side-biting vascular clamp was applied and a 16-mm Hemashield (Meadox Medicals, Oakland, NJ) graft was sutured in an end-to-side manner to the descending aorta. The graft was allowed to fill retrograde, and a clamp was placed on its proximal portion. A standard aortic valve replacement was then performed with the insertion of a 25-mm St. Jude Medical (St. Paul, MN) valve. The ascending aortic cross-clamp was removed after deairing of the heart. An adequate rhythm and contractions were rapidly obtained. The graft was then placed in a curvilinear fashion around the right atrium and anastomosed in an end-to-side manner to the ascending aorta. The patient was weaned from cardiopulmonary bypass in standard fashion on intravenous drips of dobutamine and norepinephrine. The latter drug was required for a short time because of very low peripheral vascular resistance. Measurement in the upper and lower extremities showed the previous pressure gradients to be essentially abolished.

The patient’s postoperative course was marked only by transient atrial dysrhythmias, which resolved on digoxin therapy. He was discharged on postoperative day 8, with bounding femoral pulses. There was no gradient on upper arm and calf blood pressures, and the patient was well 1 year after discharge.

Patient 2
A 59-year-old man with known cardiomyopathy and hypertension, in New York Heart Association class IV, underwent cardiac catheterization to rule out ischemic causes. There was a 30-mm Hg gradient across his coarctation in the descending thoracic aorta. Left ventricular dilatation with a left ventricular end-diastolic diameter of 7.7 cm was shown on echocardiography. There were normal coronary arteries, an ejection fraction of less than 0.15, and a cardiac index of 1.6 L · min · ^-1 · m² on catheterization. The patient required hospital admission for intravenous inotropic therapy and was listed for cardiac transplantation. Repair of the coarctation alone would not have been tolerated because of the poor left ventricular function. We therefore decided to perform both orthotopic heart transplantation and transpericardial ascending aorta–descending aorta bypass in a single stage. After the patient was sustained on intravenous dobutamine and milrinone for 110 days, a donor heart from a 35-year-old man was obtained.

Cardiopulmonary bypass was established in the standard fashion. The ascending aorta was cross-clamped and a recipient cardiectomy was performed, leaving right and left atrial cuffs and long segments of aorta and pulmonary artery. The posterior pericardium was incised, exposing the descending aorta. A side-biting vascular clamp was used to suture a 16-mm Hemashield graft in an end-to-side manner to the descending aorta. The graft was filled in a retrograde manner and a clamp placed on its proximal portion. Orthotopic heart transplantation was performed by the standard method of Lower and Shumway.

The ascending aortic cross-clamp was removed after deairing of the heart and adequate cardiac contractions were obtained. The graft from the descending aorta was placed in a curvilinear fashion around the right atrium and anastomosed in an end-to-side manner to the recipient ascending aorta. The patient was weaned from cardiopulmonary bypass in standard fashion on intravenous drips of isoproterenol and norepinephrine. A pressure tracing was once again obtained to compare upper and lower extremity pressures. The previous pressure gradient was totally abolished.

The patient’s postoperative course was uneventful and he was discharged from the hospital on postoperative day 8 with bounding femoral pulses. There was no difference in upper arm and calf blood pressures 6 months after discharge, and the patient was doing very well.

Patient 3
A 63-year-old man with a history of hypertension, diabetes mellitus, and prior repair of coarctation 30 years earlier was admitted with acute pulmonary edema after suffering an anterior wall myocardial infarction. After stabilization, the patient underwent cardiac catheterization. Critical stenoses were found in the left anterior descending and ramus intermedius coronary arteries. A 45-mm Hg gradient was noted from a recurrent coarctation in the descending thoracic aorta. Selective arteriography also revealed an old partially obstructed bypass graft from the left subclavian artery to the descending thoracic aorta just beyond the coarctation.

Cardiopulmonary bypass was established in standard fashion along with arterial inflow into the ascending aorta and femoral artery. Antegrade and retrograde cardioplegia were administered for myocardial protection. After elevating the heart, the posterior pericardial space was opened and the descending thoracic aorta was isolated. A 14-mm Hemashield graft was sutured to the descending thoracic aorta in an end-to-side manner. The graft was positioned in a curvilinear fashion around the right atrium, and filled by back bleeding. Coronary artery grafts were then placed to the left anterior descending with the left internal thoracic artery, and to the ramus intermedius, with the saphenous vein. After the aortic cross-clamp was removed and cardiac contractions obtained, the proximal portion of the 14-mm graft was anastomosed to the ascending aorta. The patient was weaned from cardiopulmonary bypass in a standard manner with the use of epinephrine and norepinephrine. There was no pressure gradient between the upper and lower extremities postoperatively. The patient made an uneventful recovery, was discharged home on postoperative day 7, and was well 9 months later.

	Comment

Top Abstract Introduction Patients and methods Comment References

 
Coarctation of the descending thoracic aorta generally presents in childhood. However, a significant number of patients will present later in life, with primary or occasionally with recurrent coarctation. Liberthson and co-workers [3] noted that 24 of 234 (10.3%) patients presented with thoracic coarctation after the age of 40. The rates of recurrent coarctation, when repaired in early infancy, have ranged from 5% to 10% [4]. A direct approach to repair of de novo or recurrent thoracic coarctation may entail enormous difficulties in the adult population. Severe lung disease, large collateral formation, and lung dysfunction from thoracotomy all present technical challenges. Dense scarring and vessel friability caused by previous surgical repair all increase the incidence of surgical complications. Interrupted collateral flow from previous dissection may also complicate a direct approach to coarctation repair. Total aortic cross-clamping, with diminished collaterals, has been implicated as a causal factor for paraplegia. For these reasons, extraanatomic bypass grafting using a number of different methods has been employed.

The first nonanatomic bypass graft for coarctation of the thoracic aorta was reported by Siderys and associates [5] in 1974 in a patient with fibrothorax. After abandoning an approach through the left chest because of dense adhesions, they performed an extraanatomical 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 [4, 6–9]. Moreover, by avoiding total aortic cross-clamping, the dreaded complication of paraplegia may be minimized. Rates of spinal complication after direct coarctation repair approximate 0.5% to 5.0% and are thought to increase with older age.

The incidence of other cardiovascular disorders (aortic aneurysm, valvular disease, and ischemic heart disease) is as high as 69% in patients surviving more than 20 years after simple repair of coarctation [10]. When presenting de novo in adulthood, there are often associated serious medical problems, including concurrent cardiac pathology. The incidence of associated cardiac anomalies was greater in patients presenting after the age of 30 years (19/47 [40%] patients) than in those presenting before the age of 30 years (51/87 [27%] patients) [3]. Operation to repair only coarctation, when presenting in adulthood, is associated with significantly higher hospital and late cardiovascular mortality [11]. The majority of these deaths are caused by myocardial infarction, pointing out the significant factor that myocardial disease plays in these patients. A multitude of variant cardiac diseases can be associated with coarctation. Our 3 patients exhibited quite variable pathologies, including coronary artery disease, aortic stenosis, and dilated cardiomyopathy. These were in combination with both primary and recurrent coarctation. Patients who have had previous repair of simple coarctation also have been shown to have an incidence of late cardiovascular morbidity, with ischemic heart disease being the most common cause of death [12]. These patients presenting with thoracic aortic coarctation, recurrent or primary, with significant simultaneous cardiac pathology, thus present a vexing clinical problem.

The avoidance of a second direct operative approach to recurrent coarctation of the thoracic aorta is desirable. A double-incision approach has been espoused by Brewer and associates [13], who reported performing ascending-descending aortic bypass grafts by constructing the distal anastomosis through a left thoracotomy and the proximal anastomosis through a median sternotomy. Surgical management of patients with coarctation of the descending thoracic aorta and concurrent cardiac defects has usually been managed with a staged approach. These approaches have been espoused to minimize the morbidity of two or more simultaneous incisions. However, concurrent repair of the coarctation and intracardiac pathology avoids the hemodynamic derangement of the second lesion when operating on only one lesion at a time. We believed that in our patients the cardiac derangements, whether aortic stenosis, cardiomyopathy, or ischemic heart disease, were all of a significant enough degree that a staged approach with primary repair of the coarctation would have produced marked hemodynamic instability and likely mortality. Operating on the cardiac defect without addressing the significant coarctation may have led to significant underperfusion of organs distal to the coarctation and to leaving the left ventricle with a severe pressure load because of late hypertension, as well as congestive heart failure [14, 15]. It was our opinion that simultaneous repair of both problems would be the only curative procedure. DeLeon and associates [16] reported on 20 infants and children who underwent transsternal approach for direct repair of the coarctation and associated cardiac defects. Isolation of the descending thoracic aorta just above the diaphragm is much simpler, and avoids the difficult dissection in cases with previous surgical repairs. In patients being fully heparinized for cardiopulmonary bypass, isolation of the aorta in the chest minimizes the incision and the attendant morbidity from dissecting in the abdomen in a heparinized patient. While on cardiopulmonary bypass, the heart can be easily dislocated upward for free access to the descending thoracic aorta beneath the pericardium and above the diaphragm.

The use of cardiopulmonary bypass also adds safety for patients with unstable hemodynamics. Arterial cannulation and perfusion of both the ascending aorta and femoral arteries should be used to avoid underperfusion distal to the coarctation. Hypothermic cardiopulmonary bypass techniques lend a margin of safety for spinal cord ischemia, especially if collateral circulation is not fully developed [17]. Double venous cannulation is necessary for unimpeded drainage. In patients with dense adhesions of the posterior pericardium, full cardiopulmonary bypass is necessary to approach the descending thoracic aorta through the posterior wall of the left atrium, as described by Barron and coworkers [18]. Use of a side-biting clamp on the descending aorta just above the diaphragm, with the heart dislocated superiorly, delivers adequate control to suture a large graft. We have sized the graft by the diameter of the descending aorta, although various authors have reported sizes ranging from 12 to 18 mm (Table 1), as there is still flow through the coarctation. Length was measured to adequately reach around the right atrium to the ascending aorta without undue compression. Passage through the oblique sinus may cause greater impingement on cardiac structures, but Barron and colleagues found no difficulties in using this approach in children [18]. If extensive adhesions prevent adequate exposure of the descending aorta above the diaphragm for the distal anastomosis, the midline incision can be extended to the umbilicus to allow access to the supraceliac aorta [21, 22]. Removal of air from the graft is performed in a retrograde manner, and the proximal anastomosis to the ascending aorta can be constructed with a side-biting clamp without cardiopulmonary bypass. Any number of intracardiac problems can then be managed, including valvular and ischemic heart disease. In our patient who required transplantation, care was taken to ensure adequate length of aorta on both the donor and recipient. This allowed a safe spacing between the suture lines of the donor–recipient anastomosis and the conduit–aortic anastomosis. If the ascending aorta is diseased, an ascending aortic graft is easily interposed, as described by Svensson [23] in the management of a patient with aortic dissection as well as coarctation. In patients requiring coronary artery bypass grafting in combination with coarctation repair, care must be taken to ensure adequate mammary artery flow before its use, because of its greater susceptibility for atherosclerotic narrowing. Chen and coworkers [24] described severe atherosclerosis and calcification in the mammary arteries of 2 patients with prior coarctation repair who required coronary artery bypass grafting 36 and 38 years after their initial corrective coarctation operation.

Listed in Table 1 is a review of 9 adult patients, including ours, in the English-language literature who had a simultaneous transpericardial ascending aorta–descending thoracic aortic graft and correction of an intracardiac lesion. The ages ranged from 19 to 72 years, and the majority, 7, were men. Six patients had aortic valve disease, 1 had mitral pathology, 2 had coronary artery disease, and 1 had an ischemic cardiomyopathy. Two of the 9 patients also had previous coarctation repair. The grafts used ranged in size from 12 to 18 mm. All patients had good survival.

We conclude that coarctation of the descending thoracic aorta with concomitant cardiac lesions can be safely and efficaciously repaired simultaneously when patients present in adulthood. As shown in previously reported series, as well as in our 3 patients, a number of various cardiac pathologies may be treated with a single-stage repair. We believe that the best approach, minimizing morbidity and mortality, is with median sternotomy only, allowing safe access for repair of cardiac lesion on cardiopulmonary bypass, with concurrent transpericardial ascending aorta to descending thoracic aortic bypass.

	References

Top Abstract Introduction Patients and methods Comment References

 

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Rohinton J. Morris Louis E. Samuels Stanley K. Brockman Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Morris, R. J. Articles by Brockman, S. K. Search for Related Content PubMed PubMed Citation Articles by Morris, R. J. Articles by Brockman, S. K.