Surgical Treatment of Neonate With Congenital Left Main Coronary ...

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CASE REPORT D’SOUZA ET AL CORONARY ARTERY ATRESIA

FEATURE ARTICLES

adverse events of psoas abscesses [1, 2], spondylodiscitis [1], aortoenteric fistulas [2], and rupture [3]. Other reported arterial sites include carotid [4], popliteal [5], femoral [3], and aortic arch [6]. To our knowledge, this is the first report of a BCGrelated mycotic aneurysm in the descending thoracic aorta and the first report of mycotic aneurysm development in a patient receiving therapy for M. bovis. In reports, mycobacterial infection is often described as indolent, with delayed diagnosis being common. When the diagnosis is missed initially and open or endovascular grafts are placed without systemic antibiotic therapy, the course is usually an indolent graft infection or pseudoaneurysm and good outcome after reoperation, once systemic therapy is initiated [1, 3]. Another unusual feature of this case was the rapid rate of progression. In only one published case has this been described, with a 4.5-cm mycotic infrarenal aortic aneurysm compared with normal aorta on imaging 1 month earlier [7]. A recent review suggested that current practice patterns underuse BCG for bladder cancer and that future applications of BCG may include prostate and renal cancer [8]. Thus, given that the use of this therapy may grow, so should awareness of the adverse events. It is concluded that although rare, mycotic aneurysms complicating intravesical BCG treatment present challenges in recognition and treatment. Previous reports describe good outcomes despite delayed diagnosis, once appropriate antibiotic therapy is instituted. In a patient with respiratory symptoms and a history of bladder cancer, suspicion for dissemination, pulmonary tuberculosis, or mycotic aneurysm should be suspected and treated promptly.

References 1. LaBerge JM, Kerlan RK Jr, Reilly LM, Chuter TA. Diagnosis please. Case 9: mycotic pseudoaneurysm of the abdominal aorta in association with mycobacterial psoas abscess: a complication of BCG therapy. Radiology 1999;211:81–5. 2. Roylance A, Mosley J, Jameel M, Sylvan A, Walker V. Aortoenteric fistula development secondary to mycotic abdominal aortic aneurysm following intravesical bacillus CalmetteGuerin (BCG) treatment for transitional cell carcinoma of the bladder. Int J Surg Case Rep 2013;4:88–90. 3. Costiniuk CT, Sharapov AA, Rose GW, et al. Mycobacterium bovis abdominal aortic and femoral artery aneurysms following intravesical bacillus Calmette-Guerin therapy for bladder cancer. Cardiovasc Pathol 2010;19:e29–32. 4. Coscas R, Arlet JB, Belhomme D, Fabiani JN, Pouchot J. Multiple mycotic aneurysms due to Mycobacterium bovis after intravesical bacillus Calmette-Guerin therapy. J Vasc Surg 2009;50:1185–90. 5. Witjes JA, Vriesema JL, Brinkman K, Bootsma G, Barentsz JO. Mycotic aneurysm of the popliteal artery as a complication of intravesical BCG therapy for superficial bladder cancer. Case report and literature review. Urol Int 2003;71:430–2. 6. Izes JK, Bihrle W 3rd, Thomas CB. Corticosteroid-associated fatal mycobacterial sepsis occurring 3 years after instillation of intravesical bacillus Calmette-Guerin. J Urol 1993;150: 1498–500. 7. Psoinos CM, Simons JP, Baril DT, Robinson WP, Schanzer A. A Mycobacterium bovis mycotic abdominal aortic aneurysm resulting from bladder cancer treatment, resection, and Ó 2016 by The Society of Thoracic Surgeons Published by Elsevier

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reconstruction with a cryopreserved aortic graft. Vasc Endovascular Surg 2013;47:61–4. 8. Gandhi NM, Morales A, Lamm DL. Bacillus Calmette-Guerin immunotherapy for genitourinary cancer. BJU Int 2013;112: 288–97.

Surgical Treatment of Neonate With Congenital Left Main Coronary Artery Atresia Travis F. D’Souza, BS, Bennett P. Samuel, MHA, Joseph J. Vettukattil, MD, and Marcus P. Haw, MD College of Human Medicine, Michigan State University, Grand Rapids; and Congenital Heart Center, Helen DeVos Children’s Hospital, Grand Rapids, Michigan

Left main coronary artery atresia (LMCAA) is a rare congenital malformation with a nonspecific and varied clinical presentation. Ventricular dysfunction and mitral insufficiency are expected ischemic consequences in the neonatal period. Left internal mammary artery (LIMA) bypass grafting (CABG) is uncommon because of the technical difficulties in performing this procedure in neonates. We describe LMCAA revascularization with a LIMA graft and mitral valve repair in a 7-week-old neonate with successful outcome 1 year postoperatively. (Ann Thorac Surg 2016;101:352–5) Ó 2016 by The Society of Thoracic Surgeons

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ongenital left main coronary artery atresia (LMCAA) is an extremely rare abnormality in which the proximal main stem artery ends blindly and blood flows from the right coronary artery (RCA) to the left coronary artery through collaterals. We report successful LMCAA revascularization in a neonate using a left internal mammary artery (LIMA) graft 1 year after coronary artery bypass grafting (CABG). A 7-week-old male neonate presented to our emergency department with acute exacerbation of difficulty breathing and feeding; he was previously evaluated by his pediatrician at 4 weeks of life. Born at 41 weeks of gestation by normal spontaneous vaginal delivery without postnatal complications, he was referred to the emergency department because of increasing symptoms of cyanosis while crying, as well as diaphoresis, orthopnea, cool extremities, and a new murmur.

Accepted for publication Dec 23, 2014. Address correspondence to Dr Haw, Pediatric Cardiothoracic Surgery, Helen DeVos Children’s Hospital, 100 Michigan NE (MC273), Grand Rapids, MI 49503; email: [email protected].

The Videos can be viewed in the online version of this article [http://dx.doi.org/10.1016/j.athoracsur.2014.12. 104] on http://www.annalsthoracicsurgery.org.

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Abbreviations and Acronyms CABG LAD LIMA LMCA LMCAA MR MV RCA

= = = = = = = =

coronary artery bypass graft left anterior descending left internal mammary artery left main coronary artery left main coronary artery atresia mitral regurgitation mitral valve right coronary artery

FEATURE ARTICLES

The patient had normal oxygen saturation, tachypnea with retractions, symmetrical pulses, and harsh grade III/ VI murmur in the precordium with radiation to the axilla consistent with mitral regurgitation (MR). A chest roentgenogram showed cardiomegaly and pulmonary venous congestion. A transthoracic echocardiogram showed an atrial septal defect, left atrial enlargement with preserved left ventricular function, and a freely prolapsing posterior mitral leaflet with severe regurgitation. There was an echogenic intracardiac focus consistent with a ruptured cord secondary to an ischemic infarct of the papillary muscle. Considering the history, echogenic intracardiac focus, and progressive symptoms after 4 to 6 weeks of life, the neonate was scheduled for cardiac catheterization to exclude congenital coronary artery anomalies. Angiograms were significant for absence of filling of the left main coronary artery (LMCA) from the aortic root and late-phase delayed filling of the left anterior descending (LAD) and circumflex arteries (Figs 1A, 1B). The LMCA appeared to point toward the aorta with no connection between the coronary and pulmonary systems, confirming the diagnosis of LMCAA. Cardiopulmonary bypass was initiated in a bicaval fashion for revascularization and mitral valve (MV) repair. Moderate hypothermia (28 C) was induced with cold cardioplegic solution infused antegradely. The LIMA was harvested as a pedicle graft and wrapped in dilute papaverine solution. It was prepared and anastomosed to the LAD in its midportion using interrupted 8-0 polypropylene sutures. On release of the soft clip on the LIMA, a good flush was observed in the LMCA and bulging vasculature of the LAD. Severe regurgitation was visualized through the atrial septum between the anterior leaflets and cleft in the posterior leaflet between the P2 and P3 elements on inspection of the MV. The valve was not stenotic, and the anterior leaflet was of adequate size but with thickened edges. The tips of both papillary muscles were infarcted and partially replaced by fibrous tissue. The chords were thin and mobile. The repair consisted of closing the cleft between P2 and P3 with 6-0 polypropylene sutures and supporting the posterior commissure with a 5-0 polypropylene 2-layered annuloplasty suture. On static testing, the valve was competent. Initially the MV was intact, but gradually symptomatic MR returned. At reoperation 4 weeks later, the MV was repaired without complication. The graft was controlled

Fig 1. (A) Preoperative aortic root angiogram showing absent left main coronary artery (LMCA) and (B) late retrograde filling of the left anterior descending (LAD) with red arrows indicating the course of the remaining segments of the atretic LMCA and circumflex arteries with blue arrow indicating the origin of the right coronary artery (RCA).

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FEATURE ARTICLES

Fig 2. Postoperative angiogram showing flow into the patent left internal mammary artery (LIMA) graft.

during cardioplegia with a soft vascular clip. Bypass details were the same as the in the first operation. The previous repair was intact; however, further distortion had occurred in the intervening time because of fibrosis with thickened and rolled edges of both anterior and posterior leaflets. There was chordal elongation of the P2 segment of the posterior leaflet with infarcted papillary muscles beyond this and chordal elongation of the A2 segment of the anterior leaflet with billowing in the midportion. The chord supporting the posterior leaflet was shortened and the P2 segment was plicated, creating a shortened leaflet with no prolapse. The previous annuloplasty sutures were removed, and both commissures were supported with 5-0 polypropylene annuloplasty sutures. Left atrial pressure after the procedure was 2 to 4 mm Hg, and transesophageal echocardiography revealed only trace MR. Our estimation, in view of maintained ventricular function, was that deterioration would continue if LMCAA revascularization was not undertaken. One year after the initial procedure, elective cardiac catheterization angiograms showed a patent LIMA graft, complete filling of the LAD artery, and retrograde filling into the LMCA and circumflex arteries (Fig 2; Videos 1 and 2). The pulmonary artery bifurcation had no abnormalities. The Qp:Qs ratio was 1:1 with no step up in pulmonary artery saturation. Left ventricular function was excellent, with only mild MR.

Comment In congenital LMCAA, the left coronary ostium and left main coronary artery trunk are not patent. The RCA provides complete coronary circulation, with left-sided


vessels receiving perfusion through collaterals. In a similar condition called single-coronary anomaly, the LMCA is a branch of the RCA. The pattern of distribution and direction of flow differentiates the angiographic appearance of LMCAA and single-coronary circulation. In LMCAA, anterograde and retrograde flow is noted. Unlike similar conditions, LMCAA is exceedingly rare, with only 53 cases in the literature as of 2013 [1]. The clinical presentation varies according to the degree of collateral circulation and metabolic demands of the heart [2]. Some patients present early in life, whereas others are asymptomatic until the fourth or fifth decade. Infants and children commonly present with syncope. Other common symptoms include heart failure, failure to thrive, angina, decreased exercise tolerance, and sudden cardiac death [1, 3]. Commonly seen as an isolated defect, LMCAA has been reported with other malformations, including supravalvular aortic stenosis, right coronary ostial stenosis, ventricular septal defect, pulmonary stenosis, and MV prolapse secondary to ischemia [1]. A high index of suspicion is needed to diagnose LMCAA because of its rarity and nonspecific clinical presentation. Coronary angiography is necessary but is potentially dangerous, with risks such as coronary spasm, which can lead to death [4]. A recent case report showed cardiac computed tomography can be diagnostic, providing 3-dimensional visualization [5]. However, the efficacy of computed tomography must be tested in small children and neonates. In the pediatric population, the symptomatic nature of LMCAA and the risk of sudden cardiac death makes surgical correction the treatment of choice. Medical management of 1 pediatric case and 1 adult case was reported, but both resulted in death [3]. The predominant option is CABG using either the LIMA or saphenous vein [1]. Because of the rarity of this disease, long-term postoperative outcomes have not been reported [1, 2, 6]. Although data are limited, 2 other cases had optimal outcomes after CABG with a LIMA graft. A child with papillary muscle infarction from LMCAA had angiograms showing normal ventricular function, minimal MR, and a patent graft 6 months postoperatively [2]. Another child was described with MR secondary to ischemia resulting from LMCAA who underwent revascularization at 12 months. A 30-month follow-up showed excellent results with a patent graft [6]. A LIMA graft for LMCAA seems to be a reasonable early interventional approach with excellent outcomes 1 year postoperatively.

References 1. Tanawuttiwat T, O’Neill B, Schob A, Alfonso C. Left main coronary atresia. J Card Surg 2013;28:37–46. 2. Ahmad Z, Haw M, Vettukattil J. Congenital mitral regurgitation due to papillary muscle infarction: a case report and approach to evaluation. Eur J Pediatr 2010;169:621–3. 3. Musiani A, Cernigliaro C, Sansa M, Maselli D, Gasperis C. Left main coronary artery atresia: literature review and


therapeutical considerations. Eur J Cardiothorac Surg 1997;11: 505–14. 4. Sohn S, Jang G, Choi B. Congenital atresia of the left main coronary artery in an infant. J Zhejiang Univ Sci B 2010;11: 539–54. 5. Levisman J, Budoff M, Karlsberg R. Congenital atresia of the left main coronary artery: cardiac CT. Catheter Cardiovasc Interv 2009;74:465–7. 6. Sunagawa M, Shimabukuro T. Congenital atresia of the left main coronary artery: successful surgical treatment (myocardial revascularisation and mitral valve repair) in a 1-year-old boy. J Zhejiang Univ Sci B 2010;11:539–41.

Late Endovascular Pulmonary Artery Band Migration Giovanni Battista Luciani, MD, Gianluca Lucchese, MD, PhD, Stiljan Hoxha, MD, Salvatore Torre, MD, Oscar Treviso, MD, and Giuseppe Faggian, MD Divisions of Cardiac Surgery and Anesthesiology, University of Verona, Verona, Italy

Here reported is an unusual case of pulmonary artery band migration with serendipitous clinical presentation late after neonatal palliation of single ventricle with aortic arch hypoplasia. The diagnostic and therapeutic implications are discussed. (Ann Thorac Surg 2016;101:355–7) Ó 2016 by The Society of Thoracic Surgeons

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A 5.3-kg male infant presented with rising peripheral arterial oxygen saturation, diffuse pulmonary rales, and increasing diuretic requirements 2 months after neonatal palliation of double-inlet left ventricle, 1-transposition of the great arteries, and aortic arch hypoplasia by transverse arch repair and pulmonary artery banding, using cardiopulmonary bypass with selective cerebromyocardial perfusion [1]. A chest roentgenogram showed increased pulmonary vascular markings and bilateral pleural effusion, and a transthoracic echocardiogram suggested decreased pressure gradient across the pulmonary banding site and severe pulmonary artery hypertension. An image describing an endoluminal position of the band was deemed artifactual. Cardiac catheterization performed to refine the diagnosis demonstrated equalized aortic and pulmonary artery pressure, thereby confirming loss of band efficacy. In

Accepted for publication March 23, 2015. Address correspondence to Dr Luciani, Division of Cardiac Surgery, University of Verona, O.C.M. Piazzale Stefani 1, 37126 Verona, Italy; email: [email protected].

Ó 2016 by The Society of Thoracic Surgeons Published by Elsevier

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addition, a large recess communicating with the pulmonary trunk and extending ventrally towards the sternum was seen at angiography (Video 1). An urgent chest computed tomography scan confirmed a large (2.5-cm 2.0-cm) pulmonary artery pseudoaneurysm adhered to the sternal table, with dislocation of pulmonary band titration titanium clips within the vessel lumen (Fig 1). Images suggestive of localized pulmonary artery trunk dissection were also recorded (Fig 1B and C). Emergency surgical treatment was scheduled. Sternotomy reentry proved uneventful thanks to a polytetrafluoroethylene surgical membrane left in place at the first-stage operation. However, contained rupture of the pseudoaneurysmal sac occurred shortly thereafter, during dissection of the great arteries, which was controlled by pulmonary artery side-clamping. After cardiopulmonary bypass was established, the pulmonary artery was explored during a period of cardioplegic arrest. The umbilical tape used for banding had migrated within the vessel lumen, thereby transecting the pulmonary artery trunk and causing false aneurysm formation (Fig 2). There was no sign of dissection. Thus, the computed tomography image (Fig 1B and C) was likely due to the endoluminal position of the band itself. The hypothesis of slow migration of the band was supported by evidence of adhesion between the band material and the pulmonary artery neointima (Fig 2, inset). The pulmonary artery trunk was replaced with a polytetrafluoroethylene vascular graft, which was banded again. The infant made an uneventful recovery. Histologic examination of the pseudoaneurysm showed severe elastic fiber fragmentation and adventitial fibrosis with moderate medial layer necrosis and fibrosis, whereas the native pulmonary artery presented only mild elastic fiber fragmentation and fibrosis. Samples of pulmonary artery tissue were also sent for cultures but proved sterile.

Comment Pulmonary artery transection is an extremely rare and generally early complication after pulmonary artery banding, which has been associated with late timing of surgery, endocarditis, or adjustable-flow banding devices [2–5]. The present case suggests delayed migration may also occur with gradual pseudoaneurysm formation. In the patient reported here, none of the recognized factors associated with pulmonary artery transection could be identified. Therefore, the pathogenetic hypothesis made included a combination of severe great arteries size mismatch, typical of tricuspid atresia with transposition, where a severely hypoplastic ascending aorta coexists with a greatly enlarged pulmonary artery, and possibly congenital structural deficiency of the main pulmonary artery media layer, which has since been described in The Video can be viewed in the online version of this article [http://dx.doi.org/10.1016/j.athoracsur.2015.03. 074] on http://www.annalsthoracicsurgery.org.

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FEATURE ARTICLES

ransection of the pulmonary artery is an extremely rare event after pulmonary artery banding in infants. The complication is generally early after the operation and commonly associated with endocarditis or adjustable-flow mechanical devices.

CASE REPORT LUCIANI ET AL LATE PULMONARY ARTERY BAND MIGRATION