Ann Thorac Surg 2015;99:1803–5

1803

2. Silveira PG, Franklin RN, Cunha JR, Neves TT, Nascimento GG, Bortoluzzi CT. Total endovascular repair of aberrant left subclavian artery with kommerell’s diverticulum using a customized branched device. J Vasc Surg 2013;57: 1123–5. 3. Knepper J, Criado E. Surgical treatment of kommerell’s diverticulum and other saccular arch aneurysms. J Vasc Surg 2013;57:951–4. 4. Kim JB, Yang DH, Kang JW. Right aortic arch and an aberrant left subclavian artery arising from a Kommerell diverticulum complicated by acute aortic dissection. J Thorac Cardiovasc Surg 2012;144:978–9. 5. Motoki M, Hattori K, Kato Y, et al. Endovascular repair of ruptured aberrant left subclavian artery with right aortic arch. Ann Thorac Surg 2013;95:699–701. 6. Wong RH, Chow SC, Lok JK, et al. Hybrid treatment for ruptured diverticulum of Kommerell: a minimally invasive option. Ann Thorac Surg 2013;95:e95–6. 7. Frigatti P, Grego F, Deriu GP, Lepidi S. Hybrid endovascular treatment of aneurysm degeneration in a rare right-aortic arch anomaly with Kommerell diverticulum. J Vasc Surg 2009;50: 903–6. 8. Cooper DG, Walsh SR, Sadat U, Noorani A, Hayes PD, Boyle JR. Neurological complications after left subclavian artery coverage during thoracic endovascular aortic repair: a systematic review and meta-analysis. J Vasc Surg 2009;49: 1594–601.

Rare Case of Aortopulmonary Window With Anomalous Origin of Right Coronary Artery Brojendra N. Agarwala, MD, Peter Varga, MD, Ziyad M. Hijazi, MD, and Gerhard Ziemer, MD Department of Pediatric Cardiology, University of Chicago, Chicago, Illinois; Departments of Pediatrics and Medicine, Rush University, Chicago, Illinois; Department of Pediatrics, Sidra Medical & Research Center, Doha, Qatar; and Departments of Pediatrics and Surgery, University of Chicago, Chicago, Illinois

A 5-month-old infant presented with a rare, congenital heart disease: aortopulmonary window with an anomalous origin of the right coronary artery from the aortopulmonary window. Using echocardiography and computed tomography, the exact diagnosis could only be ascertained retrospectively; however, cardiac catheterization and angiography confirmed the diagnosis, which led to elective open-heart surgery. The infant made a full recovery. (Ann Thorac Surg 2015;99:1803–5) Ó 2015 by The Society of Thoracic Surgeons

A

This study was supported by State Key Clinical Specialty Major cardiovascular surgery (2011).

ortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary artery. It is a rare defect accounting for 0.15% of all congenital cardiac defects. There are four types of

References

Accepted for publication June 24, 2014.

1. Cina CS, Althani H, Pasenau J, Abouzahr L. Kommerell’s diverticulum and right-sided aortic arch: a cohort study and review of the literature. J Vasc Surg 2004;39:131–9.

Address correspondence to Dr Agarwala, University of Chicago Children’s Hospital, 5721 S Maryland Ave, Rm C140, Chicago, IL 60637-1470; e-mail: [email protected].

Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier

0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.06.116

FEATURE ARTICLES

are symptomatic [1, 5]. The size of the aneurysm in this patient was approximately 40 mm in diameter; thus, early intervention was strongly recommended. Open surgical repair tended to be utilized more frequently for its documented long-term durability before the advent of endovascular repair. The standard operation usually includes removal of the KD, division of the ligamentum arteriosum to relieve the vascular ring structure, and reconstruction of the aberrant LSA [1]. However, the conventional approach has been reported to be associated with high morbidity and mortality rates because of the need for thoracotomy or sternotomy, cross-clamping of the aorta, and deep hypothermic circulatory arrest [1, 2]. As a result, surgical treatment might now be necessary only for patients who suffer any symptoms from the compression of mediastinal structures because of the presumed vascular ring. Endovascular repair has been a feasible and safe alternative to conventional surgical repair [5–7]. The minimally invasive treatment requires coverage of the origin of the aberrant subclavian artery with artificial devices, with or without simultaneous or staged extra-anatomic carotidsubclavian or axillary artery bypass or transposition [6, 7]. In this case, endovascular therapy was preferable to the conventional surgical treatment based on the patient’s physical condition and the anatomic features of the KD. As shown in this case, the stent-graft placement combined with the LSA occlusion permitted the complete exclusion of this type of aneurysmal lesion without endoleaks. The necessity for revascularization of the LSA, however, remains controversial during thoracic endovascular repair. The circle of Willis, a ring-like cerebral arterial structure, is the main collateral pathway in the cerebral circulation. The coverage of the origin of the LSA without revascularization might be a tolerable procedure if the circle of Willis is complete and the contralateral vertebral artery is dominant and patent [8]. No symptoms of left upper arm claudication or neurologic complications were observed at the follow-up examination in this case. Nonetheless, careful radiographic assessment of the circle of Willis and the vertebral arteries should be routinely required before performing the endovascular treatment. In conclusion, we have described a case of an aberrant LSA arising from a KD in a right-side aortic arch that was successfully treated with an endovascular approach involving thoracic endovascular stenting and Amplatzer vascular plug embolization of the aberrant LSA. This endovascular treatment can provide a feasible and safe alternative to conventional open surgery for KD in a right-side aortic arch with an aberrant LSA and offers minimal invasiveness and quicker recovery.

CASE REPORT AGARWALA ET AL AP WINDOW ANOMALOUS ORIGIN OF RCA

1804

CASE REPORT AGARWALA ET AL AP WINDOW ANOMALOUS ORIGIN OF RCA

FEATURE ARTICLES

APW. Type I is a proximal communication between the lateral wall of the ascending aorta and the pulmonary trunk; a type II defect is at the origin of the right pulmonary artery; a type III defect is a large (total) defect combination of the above 2 defects; and a type IV defect is an intermediate type where the defect is intermediate in size and location between types I and II [1]. APW associated with anomalous origin of the right coronary artery (RCA) from the pulmonary artery is an extremely rare congenital cardiac anomaly. In the few cases published [2–4], the RCA arose from the APW itself. The purpose of this report is to describe a 5-monthold infant born with APW (type I) and anomalous origin of RCA from the window with successful surgical outcome. The diagnosis of APW with anomalous RCA origin was suspected but not confirmed initially by echocardiography and non-reformatted cardiac computed tomographic (CT) angiography. However, after proper multiplanar reformatting and threedimensional reconstruction, the anomalous RCA was visualized retrospectively after cardiac catheterization and coronary angiography. The majority of cases of APW with anomalous origin of RCA reported in the literature was diagnosed by angiography or was recognized incidentally during surgical repair of the other cardiac defects [5, 6].

Ann Thorac Surg 2015;99:1803–5

not clearly demonstrate the origin of the RCA before multiplanar reformatting. Retrospective review after multiplanar and three-dimensional reformatting confirmed the diagnosis (Fig 1). She remained asymptomatic, and her growth and development remained normal. At cardiac catheterization, the pulmonary artery pressure was normal (30/13 mm Hg), suggesting the APW communication to be small. Both APW and anomalous origin of the RCA from the pulmonary artery were confirmed by angiography (Fig 2). At surgery, a type I APW was identified with some length, from which the anomalous RCA originated clearly. The origin of the RCA was distinctly separate from the great vessels, and it coursed in between the great vessels to the heart (Fig 3). The aortic end of the APW seemed to be somewhat smaller than the pulmonary end. The RCA was reimplanted into the anterior aspect of the ascending aorta, while the main pulmonary artery (MPA) was reconstructed with a curved polytetrafluoroethylene patch cut from a tube prosthesis. The aortic defect of the APW was closed with direct suture. After the patient came off bypass, the proximal, extra-epicardial RCA distended somewhat because of first-time exposure to high pressure

A 5-month-old female infant, who was born at 34 weeks’ gestation weighing 2.15 kg, was noted to have continuous heart murmur at an outside hospital. The infant remained asymptomatic. Echocardiography was performed, and a patent ductus arteriosus was diagnosed. She was transferred to our hospital for further management of the PDA. Follow-up echocardiography revealed the presence of an APW and an abnormal origin of right coronary artery; however, the exact origin of the RCA could not be ascertained. Cardiac CT angiography was performed, but it did

Fig 1. CT angiography with multiplanar reconstruction showing small, tortuous APW giving rise to right coronary artery (RCA). The RCA has a normal course. (Ao ¼ aorta; APW ¼ aortopulmonary window; MPA ¼ main pulmonary artery.)

Fig 2. Selective left coronary artery (LCA) angiogram showing retrograde filling of the right coronary artery (RCA) that it ends just before the main pulmonary artery (MPA), suggesting an origin from the aorotopulmonary window and not from the pulmonary artery.

Ann Thorac Surg 2015;99:1803–5

Fig 3. Surgical image (surgeon’s view) before bypass. Cannulation stitches for aorta (to the left) and the right atrial auricle (to the right) are placed with red rubber tourniquets. The forceps are around the pulmonary end of the aortopulmonary window. In a right angle in front of the forceps, the main right coronary artery (RCA) is running parallel to the ascending aorta toward the further subepicardially bifurcating course of the RCA.

1805

Fig 4. Surgical image (surgeon’s view) after repair. White ventricular and blue atrial temporary pacemaker wires are in place. At the top right, the white polytetrafluoroethylene patch with a blue dotted line is visible, restoring the MPA/RPA-takeoff anatomy. Note the dilated, thin-walled, proximal right coronary artery. (MPA ¼ main pulmonary artery; RPA ¼ right pulmonary artery.)

patients with APW. To our knowledge, our report is the first to document this rare anomaly demonstrated by CT angiography. Appropriate CT reconstruction is essential for recognizing this anomaly.

Comment Anomalous origin of the left and right coronary arteries from the pulmonary artery are usually isolated congenital cardiac defects; however, on occasion, they could be associated with other congenital heart defects [5, 6]. Therefore, it is extremely important to define the coronary artery origin before cardiac surgery. In this combination of anomalies (APW and anomalous RCA), pulmonary hypertension may actually be protective of myocardial perfusion. In our patient, the APW opening to the aorta was small and the pulmonary artery pressure was therefore normal. This fact explained the retrograde flow from the left coronary artery into the RCA and into the APW and finally into the MPA. Hisorshi et al. [7] described a 20-month-old male child with a large ventricular septal defect, patent ductus arteriosus, pulmonary hypertension, and right aortic arch. At operation, the patent ductus arteriosus was not seen, but an APW was found associated with anomalous origin of the RCA from the APW. The origins of coronary arteries need to be outlined in all patients with congenital heart defects before beginning surgical repair. Even though an anomalous origin of coronary arteries is very rare, this is also important in

References 1. Jacobs JP, Quintessenza JA, Gaynor JW, Burke RP, Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: aortopulmonary window. Ann Thorac Surg 2000;69(4 Suppl):S44–9. 2. Grunenfelder J, Zund G, Vogt PR, Turina MI. Aortopulmonary window with anomalous origin of right Coronary artery. Ann Thorac Surg 1999;67:233–5. 3. Izumoto H, Ishihara K, Fuiii Y, Oyama K, Lawazoe K. AP window and anomalous origin of right Coronary artery from the AP window. Ann Thorac Surg 1999;68:557–9. 4. Greenway SC, Bradley TJ, Caldarone CA, Silverman NH, Hanley FL, Smallhorn JF. Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery: two cases highlighting the importance of complete pre-operative echocardiographic evaluation of the coronary arteries in all conotruncal anomalies. Eur J Echocardiogr 2006;7:379–82. 5. McMahon CJ, DiBardino DJ, Undar A, Faser CD Jr. Anomalous origin of left Coronary artery from the right Pulmonary artery in association with type III aortopulmonary window and interrupted aortic arch. Ann Thorac Surg 2002;74:919–21. 6. Morell VO, Feccia M, Cullen S, Elliott MJ. Anomalous Coronary artery with Tetralogy of Fallot and Aortopulmonary window. Ann Thorac Surg 1998;66L:1403–5. 7. Hiroshi I, Kazuaki I, Yutaka F, Kotaro O, Kohei K. AP window and anomalous origin of right coronary artery from the window. Ann Thorac Surg 1999;68:557–9.

FEATURE ARTICLES

(Fig 4) and a potential wall abnormality of this anomalous proximal RCA. Her postoperative course was uneventful, and she was discharged home on postoperative day 5.

CASE REPORT AGARWALA ET AL AP WINDOW ANOMALOUS ORIGIN OF RCA

Rare Case of Aortopulmonary Window With Anomalous Origin of Right Coronary Artery.

A 5-month-old infant presented with a rare, congenital heart disease: aortopulmonary window with an anomalous origin of the right coronary artery from...
643KB Sizes 1 Downloads 9 Views