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Year : 2018  |  Volume : 4  |  Issue : 3  |  Page : 137-141

Coarctation of Aorta With Valvular Heart Disease: A Hybrid Approach

Department of Cardiothoracic and Vascular Surgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, Delhi University, New Delhi, India

Date of Web Publication31-Dec-2018

Correspondence Address:
Dr. Ankit Jain
B/126 Surajmal Vihar, Delhi 110092
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mamcjms.mamcjms_41_18

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We describe the management of three patients with coarctation of aorta (COA) associated with valvular heart disease. All the three patients underwent initial balloon dilatation following which they underwent surgical correction of the valvular pathology. Staged approach in such diseases has better outcome in terms of morbidity and mortality. Both single-stage and two stage operations can be used. As surgical correction of COA is frequently associated with potential complications, we adopted the staged approach for better results.
Background COA with valvular heart disease is generally a rare combination. Both these entities require correction which can be done simultaneously or as staged procedure.
Objective To study the outcome of staged procedures in such patients.
Materials and Methods We managed 3 patients with COA associated with valvular heart disease.
Results All the 3 patients had uneventful post operative recovery. At the time of discharge gradients were <20 mm Hg across the coarctated segment. Post operative echocardiography (echo) after 3 months on follow up were normal.
Conclusion Simultaneous surgical correction of COA along with valvular heart disease is associated with potential surgical and anesthetic complications. Hence staged procedures are preferred if the anatomy of coarctation is feasible for endovascular correction.

Keywords: Balloon angioplasty, coarctation of aorta, valvular heart disease

How to cite this article:
Jain A, Agarwal P, Satyarthy S, Sharma K, Geelani MA. Coarctation of Aorta With Valvular Heart Disease: A Hybrid Approach. MAMC J Med Sci 2018;4:137-41

How to cite this URL:
Jain A, Agarwal P, Satyarthy S, Sharma K, Geelani MA. Coarctation of Aorta With Valvular Heart Disease: A Hybrid Approach. MAMC J Med Sci [serial online] 2018 [cited 2020 Nov 24];4:137-41. Available from: https://www.mamcjms.in/text.asp?2018/4/3/137/249029

  Introduction Top

Coarctation of aorta (COA) is defined as a narrowed aortic segment, most commonly located near the ligamentum arteriosum adjacent to the left subclavian artery.[1] COA accompanied by an intracardiac lesion is a major clinical challenge as the pathology lies at two separate anatomical locations. To date there is no consensus about how to approach such cases.[2] COA in association with left-sided valvular lesions is a well-recognized entity, but because of the multiple pathologic processes, presentation normally occurs in early childhood. Presentation in adults is rare.[3] In some reports it has been suggested that COA should be treated first whereas others suggested that cardiac lesion be treated first.[4] Since two different surgical procedure increases peri-operative morbidity, mortality use of minimal invasive procedure followed by surgical correction improves patient outcome. This advancement has also been implemented successfully in infants.[5]

  Materials and Methods Top

We hereby report reterospective evaluation of three patients operated for COA with valvular heart disease during the period of january 2015 to november 2017.

All the patients underwent initial balloon dilation followed by surgical correction via median sternotomy. The approach for balloon dilatation was similar in all the three cases. Using percutaneous retrograde catheterization technique, end hole catheter was passed to the site of coarctation to the aortic isthmus, and pull-back pressure were recorded. Anatomy of COA was noted and balloon dilatation performed. Post-procedural gradient came down to 10, 15, 12 mmHg for the cases, respectively. Preprocedural angiographic images for three cases 1, 2, and 3 are [Figure 2]a, [Figure 3]a, and [Figure 4]a, and postprocedural images are [Figure 2]b, [Figure 3]b, and [Figure 4]b, respectively.
Figure 1 (a) CT image of case 1 showing fusiform ascending aortic aneurysm with concomitant coarctation of aorta. (b) CT image of case 2 showing coarctation of aorta distal to subclavian artery

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Figure 2 (a) Preprocedural angiographic image of case 1 showing segment of coarctation. (b) Post balloon dilatation angiographic image of case 1

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Figure 3 (a) Preprocedural angiographic image of case 2 showing segment of coarctation. (b) Post balloon dilatation angiographic image of case 2

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Figure 4 (a) Preprocedural angiographic image of case 3 showing segment of coarctation. (b) Post balloon dilatation angiographic image of case 3

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In all these cases, after balloon dilatation, the patient was planned for surgical correction of the valvular disease, 5 days postprocedure. Median sternotomy was performed and cardiopulmonary bypass (CPB) was established via aortic and bicaval cannulation.

Case 1

26 year old male presented to the emergency department with dyspnea on exertion (DOE) NYHA class III. On detailed examination he was clinically diagnosed with features of Marfan’s Syndrome with difference in upper and lower limb pressure. On detailed workup his echo revealed ascending aorta aneurysm of 7.2 cm with severe aortic regurgitation. COA was present with gradient of 80 mmHg. Computed Tomographic Angiography (CTA) revealed fusiform aneurysmal dilatation of ascending aorta [[Figure 1]a]. There was no evidence of dissection.[6]

Case 2

A 22-year-old female presented in our outpatient department (OPD) with DOE NYHA class III. Her 2D echo revealed rheumatic heart disease with severe mitral regurgitation and mild aortic regurgitation. Left ventricular functions were normal. COA was present with a gradient of 65 mmHg. CTA revealed severe stenosis of a short segment of descending thoracic aorta distal to the origin of left subclavian artery with collaterals [[Figure 1]b].

Case 3

A 19-year-old male presented the OPD with DOE NYHA class III. His 2D echo findings revealed severe subvalvular aortic stenosis with mild aortic regurgitation with subaortic membrane. COA with a peak gradient of 50 mmHg was present. CTA of heart and great vessels showed subvalvular aortic stenosis with juxtaductal COA. This patient had already undergone balloon dilatation for COA 6 years back.

In the first case, Bentall’s operation was performed using 27 Saint Jude Medical aortic valve conduit under low–moderate hypothermia. As the aneurysm was extending up to the right brachiocephalic artery, a brief period of deep hypothermic circulatory arrest of 9 min was required for the distal anastomosis. The anatomic suture line was strengthened with Teflon felts due to poor tissue strength in this patient.

In the second case, mitral valve replacement was performed under moderate hypothermia with 25 mitral sorin carbomedics valve. In the third case, resection of the subaortic membrane was performed and aortic valve replaced with 21 aortic sorin carbomedics valve. The patients were gradually weaned off CPB.

  Results Top

The mean CPB time was 107.3 min, and the mean hospital stay was 6.3 days [[Table 1]]. Postoperative blood pressure measurements revealed no significant pressure differences between upper and lower extremities [[Table 2]]. All of them had good peripheral pulses at the time of discharge. They are in regular follow-up and presently in NYHA class I.
Table 1 Intraoperative and postoperative outcome variables

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Table 2 Comparison of coarctation gradients using 2D echocardiogram

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  Discussion Top

Liberthon et al in his study of 234 patients of COA showed 80 patients had associated cardiac lesions.[7] The incidence of other cardiac disorders such as valvular heart disease, ischemic heart disease and aneurysmal disease, is as high as 69% in patient surviving more than 20 years.[4]

Although COA is primarily a disease of childhood, it can also present in later stages of the life along with cardiac problem, which imposes severe surgical challenge. COA with valvular heart disease can be managed in several ways; the options include single-stage, two-stage, and hybrid procedure. Every procedure has its own advantages and disadvantages.

Mulay et al. in their study of repair of COA with valvular heart disease reported that treating the valvular lesion primarily enables myocardium and coronary flow redistribution. They also reported coarctation repair 6 to 12 weeks after the valvular surgery. He added that treatment of COA in the second stage avoids ischemia of the left ventricle due to sudden increase of afterload and related hemodynamic instability. In another school of thought, it was suggested that increased afterload can be a cause of bleeding and clinical deteoriation in those whose cardiac lesions were operated in the first stage.[3] In experimental studies it has been shown that coronary perfusion is decreased in patients with aortic regurgitation therefore aortic valve surgery is performed in first stage and COA repair in second stage.[8],[9],[10],[11],[12],[13],[14],[15] Also atrial fibrillation and ischemia might occur in dilated ventricle due to increase in afterload after cross clamping.[16]

Due to low perfusion along the distal segment of coarctation, features of low cardiac output syndrome can ensue. Performing the cardiac operation primarily can cause malperfusion of visceral organs.[16] Therefore, coarctation associated with valve disease but without signs of congestive heart failure should be repaired in the first stage.[17]

In patient with concomitant aneurysm and coarctation, repair of COA primarily helps in minimizing the blood pressure which decreases the tension on the aortic valve and facilitates easier aortic cannulation during the second-stage procedure. This also decreases the left ventricle outflow gradient and minimizes the risk of rupture and dissection of the aortic aneurysm during its repair. If the aneurysm is repaired in the first stage due to increase in afterload caused by COA, troublesome bleeding can occur from the suture line.[18] On the contrary, although the surgical treatment of coarctation in the first stage decreases the afterload, risk of rupture remains the same in the two procedures.[3] Therefore, it is preferred that a single-stage procedure should be performed for combination of COA associated with the aneurysm of aorta.

Vijayanagar et al. described an ascending-to descending bypass in COA.[19] Though this technique is safer and easier, especially in patients with an ascending aortic aneurysm, coronary artery or valve disease associated with coarctation or recoarctation, [20],[14],[21] it is not appropriate before adulthood, because of anastomotic dehiscence which might occur during the growth period[14] as well as it increases the risk of spinal cord ischemia, neurological complication, kinking, stenosis and thrombosis of the graft. Hence in all 3 patients we initially resorted to balloon dilatation followed by surgical correction.

Although Intimal/transmural injury, dissection and aneurysm are the reported complications of balloon dilatation of coarctation[22],[23],[24],[25], but with the advancements and improvements in techniques of interventional cardiology the surgical repair of both these combined pathologies can be safely performed, minimizing the post operative pain and lung atelectasis which can cause severe morbidity due to separate incision. The potential complications such as chylothorax, bleeding from fragile arteries, laryngeal and phrenic nerve injuries, prolonged exposure to anesthetic agents, and unnecessary cannulation of femoral arteries are also prevented.

  Conclusion Top

Hybrid approach is more beneficial as it is less invasive and decreases the morbidity with overall improvement in patient outcome. Moreover, it reduces the hospital stay and cost. Furthermore, long-term studies are required with stringent follow-up.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Sampath R, O’Connor WN, Noonan JA, Todd EP. Management of ascending aortic aneurysm complicating coarctation of the aorta. Ann Thorac Surg 1982;34:125-31.  Back to cited text no. 2
Musumeci F, Penny WJ. Aortic coarctation associated with aortic or mitral valve disease: Which lesion to correct first? Ann Thorac Surg 1998;66:603-4.  Back to cited text no. 3
Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 2008;23:e143-263.  Back to cited text no. 4
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Satyarthy S, Roy A, Gupta U, Geelani MA, Tyagi S, Datt V. Incidental finding of coarctation of the aorta in a Marfan patient: A case report. Indian J Thorac Cardiovasc Surg 2018;34:157-60.  Back to cited text no. 9
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Buckberg GD, Fixler DE, Archie JP, Hoffman JIE. Experimental subendocardial ischemia in dogs with normal coronary arteries. Circ Res 1972;30:67-81.  Back to cited text no. 11
Karp RB, Roe BB. Effect of aortic insufficiency on phasic flow patterns in the coronary artery. Ann Surg 1966;164:959-66.  Back to cited text no. 12
Folts JD, Rowe GG. Coronary and hemodynamic effect of temporary acute aortic insufficiency in intact anesthetized dogs. Circ Res 1974;35:238-46.  Back to cited text no. 13
Sweeney MS, Walker WE, Duncan JM, Hallman GL, Livesay JJ, Cooley DA. Reoperation for aortic coarctation: Techniques, results, indications for various approaches. Ann Thorac Surg 1985;40:46-9.  Back to cited text no. 14
Vijayanagar R, Natarajan P, Eckstein PF, Bognolo DA, Toole JC. Aortic valvular insufficiency and postductal aortic coarctation in the adult. Combined surgical management through median sternotomy: A new surgical approach. J Thorac Cardiovasc Surg 1980;79:266-8.  Back to cited text no. 15
Thomka I, Szedo F, Arvay A. Repair of coarctation of the aorta in the adults with simultaneous aortic valve replacement and coronary artery bypass grafting. Thorac Cardiovasc Surg 1997;45:93-6.  Back to cited text no. 16
Connolly HM, Schaff HV, Izhar U, Dearani JA, Warnes CA, Orszulak TA. An alternative surgical approach for complex coarctation of the aorta. Circulation 2001;104:133-7.  Back to cited text no. 17
Ovaert C, Benson LN, Nykanen D, Freedom RM. Transcatheter treatment of coarctation of the aorta: A review. Pediatr Cardiol 1998;19:27-44.  Back to cited text no. 18
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Morris RJ, Samuels LE, Brockman SK. Total simultaneous repair of coarctation and intracardiac pathology in adult patients. Ann Thorac Surg 1998;65:1698-702.  Back to cited text no. 20
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Magee AG, Blauth CI, Qureshi SA. Interventional and surgical management of aortic stenosis and coarctation. Ann Thorac Surg 2001;71:713-5.  Back to cited text no. 24
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1], [Table 2]


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