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Ann Thorac Surg 1995;60:462-463
© 1995 The Society of Thoracic Surgeons

Case Reports

Organized Thrombus in Left Main Coronary Artery in Hypoplastic Left Heart Syndrome

Jacques A. M. van Son, MD, PhD, Michael D. Black, MD, Kim Devoe, MD, Gary S. Haas, MD

Divisions of Cardiothoracic Surgery and Anatomic Pathology, University of California, San Francisco, San Francisco, California

Accepted for publication January 30, 1995.


   Abstract
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A neonate is reported in whom, during Norwood stage I correction for hypoplastic left heart syndrome with mitral stenosis, an organized thrombus was found to protrude from the left coronary ostium into the aortic root. With ventricular assist device support the patient survived despite severe left ventricular ischemia. The presumed origin of the thrombus is from left ventriculocoronary arterial connections that serve to decompress the blind left ventricular cavity.


   Introduction
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We present the case of a neonate in whom severe left ventricular myocardial ischemia occurred secondary to occlusion of the left coronary artery during Norwood stage I correction for hypoplastic left heart syndrome.

A 9-day-old girl with the diagnosis of aortic atresia, mitral stenosis, and hypoplastic left heart syndrome was transferred to UCSF Medical Center after an uncomplicated pregnancy. Two-dimensional echocardiography demonstrated aortic atresia, severe mitral stenosis, and a hypoplastic left ventricle with a slitlike cavity. In addition, a large right coronary artery and an underdeveloped left coronary artery (without evidence of intracoronary thrombus) were seen; the contractility of the ventricular septum was normal. Cardiac catheterization was not performed.

A Norwood stage I correction was performed in routine fashion with single venous cannulation, deep hypothermia, and circulatory arrest. A 3.5-mm polytetrafluoroethylene tube graft (Gore-Tex; W.L. Gore & Assoc, Flagstaff, AZ) was inserted between the innominate artery and the right pulmonary artery.

Before the circulation was arrested, a single dose of hand-injected blood cardioplegia was administered into the aortic root via the ascending aorta-innominate artery junction. The myocardium appeared well perfused. Upon incision of the ascending aorta for patch augmentation with a pulmonary homograft, a 4-mm-long and 1-mm-wide, gray-brown, organized thrombus was found protruding from the left coronary ostium into the ascending aorta. Upon removal it was felt to be adherent to the intima of the left coronary ostium. Examination of the remaining aorta revealed no further thrombus.

With the aortic reconstruction completed and the aortic cross-clamp removed, both ventricles contracted poorly, appeared ischemic, and were lividly discolored with no perfusion noted in the left epicardial coronary vessels. Through a limited lateral aortotomy we ascertained that the aortopulmonary anastomosis was well aligned and that there was no residual thrombus in the ostium of the left main coronary artery. With support of a Medtronic Biomedicus ventricular assist device (Medtronic, Inc, Minneapolis, MN) (with cannulation of the right atrium and the neo-ascending aorta) at an initial flow rate of 200 mL • kg-1 • min-1 we were able to wean the patient off cardiopulmonary bypass. The activated clotting time, as a parameter for the degree of heparinization, was kept between 160 and 200 seconds.

Serial echocardiographic examinations demonstrated a gradual improvement of right ventricular and septal function, so that after 48 hours ventricular assist device support could be withdrawn successfully. Ventilatory and inotropic support were discontinued during the next 7 days. Histologic examination of the removed thrombus showed areas of organization with presence of polymorphonuclear leukocytes and mild to moderate degenerative changes (Fig 1Go); bacterial and fungal stains were negative. Seven months postoperatively, the infant is thriving well and echocardiographically has excellent right ventricular and septal function.



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  		Fig 1.. Photomicrographs of removed thrombus. (A) The distinct layering with bands of fibrin alternating with bands of erythrocytes and polymorphonuclear leukocytes is indicative of thromboembolus, not fresh blood clot. (B) High-power view of thromboembolus: the polymorphonuclear leukocytes show mild to moderate degenerative changes. (Hematoxylin and eosin stain; original magnification: A, x100; B, x400.)

 

   Comment
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In 95% of cases with aortic atresia the left ventricle is severely hypoplastic, with the ventricular septum always being intact [1– 4]. The mitral valve is either atretic (approximately one third of cases) or severely hypoplastic (approximately two thirds of cases) [2]. In the latter situation, similar to the situation in the right ventricle in pulmonary atresia with intact ventricular septum [5], there may be ventriculocoronary arterial connections of the luminal, sinusoidal, and capillary subtypes, which are postulated to decompress the blind left ventricular chamber [6, 7]. The luminal subtype connects the left ventricular cavity directly to the vertically penetrating branches of the left anterior descending coronary artery, obtuse marginal branches of the circumflex coronary artery, and the posterior descending coronary artery [6]. Alternating reversal of the direction of coronary blood flow from antegrade (during diastole) to retrograde (during systole) creates turbulence with the potential for stasis and thrombus formation. In addition, capillary congestion, myocardial edema, and extravasation of red blood cells may occur [6]. The tendency toward regional distribution of ventriculocoronary connections, accompanied by a variable degree of endocardial fibroelastosis, focal subendocardial scarring, and calcification [2, 6, 8], suggests that regional differences in flow, pressure, and oxygenation may exist in the hypoplastic left ventricle.

Although intracoronary histologic evidence is lacking, it is conceivable that the thrombus in the left coronary artery as observed in the presented case was formed in ventriculocoronary connections and propelled in a retrograde fashion into the left coronary arterial system during the systolic phase of the cardiac cycle. The presence of a histologically proven organized thrombus in the left main coronary artery with adherence to the coronary ostium pleads in favor of such pathogenesis, as opposed to selective migration of the thrombus from the aorta into the left coronary ostium. We hypothesize that, after administration of cardioplegia into the aortic root and removal of thrombus from the left coronary ostium, dislodgement or embolization of residual thrombus in the left main coronary artery may have led to partial or complete obstruction of the left coronary circulation, as evidenced by the poor contractility and livid discoloration of the left ventricle after discontinuation of cardiopulmonary bypass.

Although the left coronary arterial system may be of limited value in hypoplastic left heart syndrome because the left ventricle is physiologically nonfunctional, its obstruction after Norwood stage I correction may lead to decreased septal contractility with adverse effects on right ventricular function and a potentially fatal outcome. Although the presented patient survived with aid of a ventricular assist device, if confronted with a similar case in the future, we probably would administer cardioplegic solution selectively into the coronary sinus, hoping that this technique is efficacious in propagating migration of intracoronary thrombus toward the coronary ostium and into the aortic root.


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Address reprint requests to Dr van Son, Division of Cardiothoracic Surgery, University of California, San Francisco, San Francisco, CA 94143-0118.


   References
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 Abstract
 Introduction
 Comment
 References
 

  1. Sinha SN, Rusnak SL, Sommers HM, Cole RB, Muster AJ, Paul MH. Hypoplastic left ventricle syndrome. Analysis of thirty autopsy cases in infants with surgical considerations. Am J Cardiol 1968;21:166–73.[Medline]
  2. Roberts WC, Perry LW, Chandra RS, Myers GE, Shapiro SR, Scott LP. Aortic valve atresia: a new classification based on necropsy study of 73 cases. Am J Cardiol 1976;37:753–6.[Medline]
  3. Murdison KA, Baffa JM, Farrell PE Jr, et al. Hypoplastic left heart syndrome. Outcome after initial reconstruction and before modified Fontan procedure. Circulation 1990;82(Suppl 4):199–207.
  4. Chang AC, Farrell PE Jr, Murdison KA, et al. Hypoplastic left heart syndrome: hemodynamic and angiographic assessment after initial reconstructive surgery and relevance to modified Fontan procedure. J Am Coll Cardiol 1991;17:1143–9.[Abstract]
  5. Gittenberger-de Groot AC, Sauer U, Bindl L, Babic R, Essed CE, Bühlmeyer K. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol 1988;18:243–58.[Medline]
  6. O'Connor WN, Cash JB, Cottrill CM, Johnson GL, Noonan JA. Ventriculocoronary connections in hypoplastic left hearts: an autopsy microscopic study. Circulation 1982;66:1078–86.[Free Full Text]
  7. Beckman CB, Moller JH, Edwards JE. Alternate pathways to pulmonary venous flow in left-sided obstructive anomalies. Circulation 1975;52:509–16.[Abstract/Free Full Text]
  8. Bulkley BH, D'Amico B, Taylor AL. Extensive myocardial fiber disarray in aortic and pulmonary atresia. Relevance to hypertrophic cardiomyopathy. Circulation 1983;67:191–8.[Abstract/Free Full Text]



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This Article
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Right arrow Articles by Haas, G. S.

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