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Year : 2020  |  Volume : 8  |  Issue : 3  |  Page : 144-147

Adult interrupted aortic arch with aortic regurgitation: Single-stage repair

Departments of Cardiothoracic Surgery and Cardiac Anaesthesia and Critical Care, Narayana Multispeciality Hospital, Jaipur, Rajasthan, India

Date of Submission30-Aug-2020
Date of Decision19-Sep-2020
Date of Acceptance11-Oct-2020
Date of Web Publication26-Nov-2020

Correspondence Address:
Dr. Ranajit Beeranna Naik
Department of Cardiothoracic Surgery, Narayana Multispeciality Hospital, Jaipur, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/heartindia.heartindia_35_20

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Adult patients with interruption of the aorta are an extremely rare occurrence. It is commonly associated with additional cardiovascular anomalies. Surgery is the mainstay in the management of the patients. Interrupted aortic arch and associated cardiac disease is a complex situation that requires correction either simultaneously or in stages. We describe the case of a 32-year-old male patient planned for aortic valve replacement at another center, incidentally diagnosed with interrupted aortic arch Type A. He was managed with single-stage aortic valve replacement and extra-anatomical bypass.

Keywords: Aortic regurgitation, aortic valve replacement, bicuspid aortic valve, extra-anatomical repair, interrupted aortic arch, single-stage repair

How to cite this article:
Srivastava CP, Naik RB, Sharma S, Goyal PK. Adult interrupted aortic arch with aortic regurgitation: Single-stage repair. Heart India 2020;8:144-7

How to cite this URL:
Srivastava CP, Naik RB, Sharma S, Goyal PK. Adult interrupted aortic arch with aortic regurgitation: Single-stage repair. Heart India [serial online] 2020 [cited 2021 Jan 26];8:144-7. Available from: https://www.heartindia.net/text.asp?2020/8/3/144/301596

  Introduction Top

The interrupted aortic arch, first described in 1778 by Steidele and classified by Celoria and Patton in 1959, is a rare congenital malformation reported in 3 per million live births, in which there is loss of luminal continuity between the ascending and descending portions of the aorta. It is commonly associated with other intracardiac malformations such as bicuspid aortic valve, left ventricular outflow tract obstruction, ventricular septal defect, and patent ductus arteriosus.[1] As per our knowledge, only 35 cases are reported in adults till today, making it an extremely rare occurrence.[2]

  Case Report Top

A 32-year-old male patient presented to another hospital with dyspnea and palpitation for 4 years. He was diagnosed to have rheumatic severe aortic regurgitation and was planned for aortic valve replacement. On midline sternotomy, dilated bilateral internal thoracic arteries with tortuous extensive collaterals in the retrosternal area were noted. Aortic cannulation revealed increased pressure gradient between the ascending aorta and the femoral artery, leading to the suspicion of coarctation of aorta. The planned procedure was abandoned and the subsequent computed tomography-aortogram done was suggestive of Type A interrupted aortic arch with tortuous extensive collaterals, as shown in [Figure 1]. He was referred to our center for further management.
Figure 1: Preoperative, contrast-enhanced, three-dimensional, computed tomography aortogram revealing interruption of the aortic arch beyond the left subclavian artery (interrupted aortic arch, type A). Extensive collateral vessels to the descending aorta are evident from the subclavian and intercostal arteries

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On physical examination, the blood pressure in the right arm was 192/80 mmHg with systolic pressure gradient of 30 mmHg between the upper and lower extremities. Transthoracic echocardiography done at our center was suggestive of a bicuspid aortic valve with severe aortic regurgitation and mild aortic stenosis. The proximal ascending aorta and the aortic annulus measured 36 mm and 31 mm, respectively. The patient had left ventricular ejection fraction of 40%–45%. Diastolic spill was seen in the descending aorta, suggestive of coarctation of aorta.

A single-stage operation was performed one day after the initial sternotomy. The procedure consisted of a reopening of the standard median sternotomy, aortobicaval cannulations, and the establishment of extracorporeal circulation. We did not adopt any additional techniques of perfusion because the pressure in the right femoral artery (60 mmHg) measured by the arterial line was adequately maintained. After the establishment of cardiopulmonary bypass and aortic cross-clamp, under cold blood cardioplegia, the entire heart was retracted upward and right out of the pericardial sac, so the retrocardiac descending aorta could be easily seen using transposterior pericardial approach. The aorta was then side clamped, and an adequate aortotomy was done. An 18-mm polytetrafluoroethylene graft was anastomosed end to side with 4-0 prolene continuous suture. The graft was clamped and kept in the pericardium. The aortic valve was replaced with a 23-mm St. Jude Medical Masters Series (SJM) mechanical valve. The aortotomy incision site was closed with full precautions, and rewarming was started. On releasing the aortic cross-clamp, cardiac activity resumed.

Proximal anastomosis of the graft to the ascending aorta was done using a side-biting clamp in an end-to-side fashion, with a running 4-0 polypropylene suture. After completion of the anastomosis, an extra-anatomic bypass appeared, as shown in [Figure 2]. The patient was weaned from cardiopulmonary bypass smoothly and decannulation was done.
Figure 2: Intraoperative photograph showing the proximal and distal end-to-side anastomosis of the 18-mm graft to the ascending and descending aorta, respectively

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The cardiopulmonary bypass time was 320 min, while the aortic cross-clamp time was 171 min. The patient temperature was maintained at 26 degree centigrade. Ventilatory support was needed for 13 h. The patient was kept in the intensive care unit for 3 days. Postoperatively, the pressure gradient between the upper and lower extremities was <10 mmHg.

Postoperative echocardiography showed the aortic prosthetic valve in situ with peak and mean gradients of 19 and 11 mmHg, respectively. There was no valvular or paravalvular leak. The left ventricular ejection fraction was estimated as 35%.

There were no perioperative complications. The patient recovered well uneventfully and was discharged on the postoperative day 6.

Repeat computed tomography-contrast aortogram postoperatively showed a well-homogenously opacified patent graft with no obvious filling defect or luminal narrowing. Almost all the collaterals had disappeared by the end of 1-month follow-up, as shown in [Figure 3], when the repeat aortogram was done suggestive of no significant afterload post extra-anatomic bypass in adults.
Figure 3: Postoperative, contrast-enhanced, two-dimensional, computed tomography maximum intensity projection images showing patent flow in the 18-mm polytetrafluoroethylene graft anastomosed end to side to the mid-portion of the ascending aorta and to the distal portion of the descending thoracic aorta. There is no luminal narrowing or filling defect. The collaterals disappeared within a month postoperatively

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  Discussion Top

Interrupted aortic arch commonly manifests in infants, as congestive heart failure. In rare cases, its presentation in adults ranges from asymptomatic or incidental finding in association with other cardiac anomalies.[3],[4] The distal flow in such cases is maintained by the developing obligatory extensive collateral circulation. Most of the reported cases in adults have been repaired in a single stage using an extra-anatomic approach.[5] The selection of single- or two-stage repair has been debatable. However, the single-stage repair is gaining popularity in infants.[6]

Operating on the coexistent cardiac lesions without correcting the aortic interruption can result in significant underperfusion of distal organs, severe pressure load on the left ventricle because of hypertension, and congestive heart failure. Hence, simultaneous repair of both the interruption and the associated cardiac lesion is preferred. Single-stage repair with combined thoracotomy and sternotomy increases postoperative pain and complications. Thus, the approach by median sternotomy is mostly preferable.

We preferred extra-anatomic repair because anatomic repair needs extensive dissection and mobilization of aorta, which increases the risk of complication such as bleeding, lung injury, chylothorax, and laryngeal or phrenic nerve damage. The presence of extensive collaterals further increases the risk of complications. In extra-anatomic bypass, we need to be vigilant of the graft length and its routing to avoid the risks of graft kinking and compression. We preferred the descending thoracic aorta for distal anastomosis of extra-anatomic bypass as exposure of the abdominal aorta leads to excessive dissection and increases the risks of morbidity and mortality.

The extra-anatomic bypass ascending to the descending aorta graft can be routed along the left or right margin of the heart and also in front of or behind the inferior vena cava.[7],[8] The latter avoids damage to higher vessels and phrenic nerve. Antecaval approach avoids compression of the pulmonary veins and inferior vena cava. Retrocaval method results in bleeding control difficult but can be easily dissected during reoperation.

Additional arterial cannulation and perfusion of both the ascending aorta and femoral arteries may be employed to avoid underperfusion of distal organs. They provide better protection from spinal cord ischemia and other neurological complications. However, we preferred anastomosis of the graft to the descending aorta with a partial side-biting clamp and hence did not use any additional techniques of perfusion.

  Conclusion Top

Interruption of aorta with concomitant cardiac lesions in adults can be safely and efficaciously repaired with the help of single-stage procedure using median sternotomy.

Informed consent


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initial will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Ethical approval

Informed consent was obtained from the patient. Patient anonymity has been maintained.

Authors' Contributions

Chandra Prakash Srivastava performed the single stage repair and was involved in editing the case report. Ranajit Beeranna Naik was involved in the patient care and wrote the manuscript. Sunil Sharma and Pradeep Kumar Goyal were involved in patient care and made contributions for editing the manuscript.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

John AS, Schaff HV, Drew T, Warnes CA, Ammash N. Adult presentation of interrupted aortic arch: Case presentation and a review of the medical literature. Congenit Heart Dis 2011;6:269-75.  Back to cited text no. 1
Serraf A, Lacour-Gayet F, Robotin M, Bruniaux J, Sousa-Uva M, Roussin R, et al. Repair of interrupted aortic arch: A ten-year experience. J Thorac Cardiovasc Surg 1996;112:1150-60.  Back to cited text no. 2
Sai Krishna C, Bhan A, Sharma S, Kiran U, Venugopal P. Interruption of aortic arch in adults: Surgical experience with extra-anatomic bypass. Tex Heart Inst J 2005;32:147-50.  Back to cited text no. 3
Burton BJ, Kallis P, Bishop C, Swanton H, Pattison CW. Aortic root replacement and extra-anatomic bypass for interrupted aortic arch in an adult. Ann Thorac Surg 1995;60:1400-2.  Back to cited text no. 4
Messner G, Reul GJ, Flamm SD, Gregoric ID, Opfermann UT. Interrupted aortic arch in an adult-single-stage extra-anatomic repair. Tex Heart Inst J 2002;29:118-21.  Back to cited text no. 5
Said SM, Burkhart HM, Dearani JA, Connolly HM, Schaff HV. Ascending-to-descending aortic bypass: A simple solution to a complex problem. Ann Thorac Surg 2014;97:2041-7.  Back to cited text no. 6
Vijayanagar R, Natarajan P, Eckstein PF, Bognolo DA, Toole JC. Aortic valvular insufficiency and postductal aortic coarctation in the adult: Combined surgical management through medial sternotomy: A new surgical approach. J Thorac Cardiovasc Surg 1980;79:266-8.  Back to cited text no. 7
Powell WR, Adams PR, Cooley DA. Repair of coarctation of the aorta associated with intracardiac repair. Tex Heart Inst J 1983;10:409-13.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]


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