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Ann Thorac Surg 2002;73:657-659
© 2002 The Society of Thoracic Surgeons

Case report

Isolated left ventricular ischemia after the Norwood procedure

Joseph J. DeRose, Jr, MDa, Rozelle Corda, NPa, M. Renate Dische, MD, PhDa, Jennifer Eleazar, MDa, Ralph S. Mosca, MD*a

a Division of Pediatric Cardiac Surgery and Department of Pathology, Columbia University College of Physicians and Surgeons, New York, New York, USA

Accepted for publication June 22, 2001.

* Address reprint requests to Dr Mosca, Pediatric Cardiac Surgery, Columbia-Presbyterian Medical Center, 3959 Broadway, BHN 274, New York, NY 10032, USA
e-mail: rm891{at}columbia.edu


   Abstract
Top
 Abstract
Introduction
 Comment
 References
 
Aortic atresia is the most severe variant of hypoplastic left heart syndrome (HLHS), and has been associated with significant mortality after stage I palliation. Coronary artery abnormalities are more prominent in this group of patients, especially in the presence of a patent mitral valve. Herein, we describe a case of isolated left ventricular ischemia after the Norwood procedure in a neonate with hypoplastic left heart syndrome, left ventricular hypertrophy, mitral stenosis, aortic atresia, and anomalous left coronary artery.


   Introduction
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 Abstract
 Introduction
Comment
 References
 
Despite improvements in surgical technique and postoperative care, in-hospital mortality after stage I palliation for hypoplastic left heart syndrome (HLHS) remains high in most large series (15% to 25%) [1, 2]. Aortic atresia has been identified as a risk factor by some surgeons for decreased early and late survival after the Norwood procedure [3]. Coronary insufficiency can result from technical challenges associated with the anastomosis of the small ascending aorta, but intrinsic coronary abnormalities can further compromise myocardial perfusion [4–7]. We describe a case of intractable left ventricular ischemia after the Norwood procedure in a patient with profound left ventricular hypertrophy and left coronary artery abnormalities.

A 2.4 kg male infant born at 30 weeks gestation presented with cyanosis in the newborn nursery of a referring hospital. Transthoracic echocardiogram established the diagnosis of HLHS. The infant was begun on prostaglandin E and transferred to our institution for definitive treatment. Upon arrival, continued respiratory distress led to endotracheal intubation. A repeat echocardiogram revealed HLHS with marked left ventricular hypertrophy, mitral stenosis, aortic atresia, and a mildly restrictive atrial septal defect (Fig 1).



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  		Fig 1. Echocardiogram demonstrating small, hypoplastic left ventricular cavity with marked left ventricular muscular hypertrophy. (Asc Ao = ascending aorta; LA = left atrium; LV = left ventricle; RV = right ventricle.)

 
The infant was taken to the operating room on day of life 8 without evidence of end-organ hypoperfusion. Cardiopulmonary was initiated through right atrial and pulmonary artery cannulation. The branch pulmonary arteries were snared and the infant was cooled to 18°C. The descending aorta was cross-clamped and cardioplegia was administered (15 mL/kg) with prompt arrest and cooling of the heart. The Norwood reconstruction was then performed under circulatory arrest as previously described. A 3.0-mm shunt was fashioned from the innominate artery to the pulmonary bifurcation during rewarming.

After complete rewarming and an attempt to wean from cardiopulmonary bypass, it became evident that despite excellent right ventricular function and perfusion, the left ventricle was markedly ischemic. Although the left coronary artery did not appear compromised, the patient was again cooled to 20°C and the circulation arrested. The proximal anastomosis was taken down and the coronary arteries inspected from the inside. There was no evidence of kinking or intraluminal obstruction. The patient was rewarmed but isolated left ventricular hypoperfusion persisted. Despite good right ventricular perfusion and function, the patient could not be weaned from cardiopulmonary bypass with maximal inotropic support and died in the operating room. An autopsy limited to the heart and thoracic great vessels revealed severe left ventricular hypertrophy with aortic atresia, mitral valve stenosis, and mild focal endocardial sclerosis. The thick ventricular septum bulged markedly into the right ventricle encroaching on its lumen. The increased left ventricular wall thickness appeared secondary to an increase in myocardial cell number without evidence of myofiber enlargement. The coronary anastomosis was widely patent, but the coronary arteries feeding the left ventricle were quite abnormal and atretic. The left coronary artery branched abnormally resulting in a significant steal of blood from the left ventricle. It gave rise to a small anterior descending artery, the circumflex artery, and an aberrant branch that coursed below the right pulmonary artery and behind the right atrium toward the right pulmonary hilum (Fig 2). The intramyocardial vessels demonstrated significant tortuosity and ventriculocoronary communications were not identified.



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  		Fig 2. Postmortem schematic drawing demonstrating aberrant coronary artery coursing posteriorly from the circumflex coronary artery to the right pulmonary hilum. (LA = left atrium; LAD = left anterior descending coronary artery; LV = left ventricle; OD = outer dimension; RA = right atrium; RCA = right coronary artery; RV = right ventricle.)

 

   Comment
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 Abstract
 Introduction
 Comment
References
 
Coronary abnormalities have previously been described in patients with HLHS [4–7]. Analogous to the patent inflow and obstructed outflow of pulmonary atresia with intact ventricular septum, HLHS characterized by mitral stenosis and aortic atresia carries the highest incidence of coronary abnormalities [5, 7]. Ventriculocoronary communications of the luminal subtype are the most common and are observed in approximately 10% to 20% of such patients [7]. These direct arterioluminal connections have intimal abnormalities ranging from mild thickening to severe stenosis or obliteration of the lumen. Epicardial coronary abnormalities are rare and include aberrant origin from the pulmonary artery [9], diffuse hypoplasia [6, 9], and tortuosity [5–7].

In the present case, two causes of potential coronary hypoperfusion exist: (1) left coronary hypoplasia in both the anterior descending and circumflex distribution, and (2) an abnormal coronary artery branch stealing blood flow from the myocardial circulation. Marked left ventricular wall thickness undoubtedly placed increased oxygen demands on the hypoplastic left coronary circulation. It is presumed that upon the institution of cardiopulmonary bypass a decrease in coronary perfusion pressure together with an increase in coronary steal from the aberrant coronary branch resulted in critical left ventricular ischemia. Baffa and colleagues [7] have documented that left ventricular coronary abnormalities in HLHS have little effect on both right ventricular perfusion and histology. However, the severe left ventricular and septal hypertrophy in this infant, coupled with ischemic dysfunction may have resulted in the catastrophic consequences of septal dyscoordination and right ventricular obstruction.

In autopsy report 122 patients who died after the Norwood procedure, Bartram and associates [10] found impairment in coronary perfusion to be the cause of death in 27% of the cases. However, the cause of stenosis in the vast majority of these patients (31 of 33) was secondary to either intraluminal stenosis at the anastomosis or external kinking from the homograft. Only 1 patient exhibited coronary arterial hypoplasia. These findings support the fact that ischemic episodes after the Norwood operation are most frequently the result of technical errors, and a rigorous evaluation of the proximal coronary anastomosis including reinstitution of circulatory arrest is nearly always warranted.

Nonetheless, HLHS characterized by aortic atresia/mitral stenosis and profound left ventricular hypertrophy may represent a subset of patients who should undergo detailed angiography of the coronary circulation before embarking on Norwood palliation. If coronary abnormalities in such patients are found, serious consideration should be given to transplantation as an alternative therapeutic option [8].


   References
Top
 Abstract
 Introduction
 Comment
 References
 

  1. Mosca R.S., Bove E.L., Crowley D.C., Sandhu S.K., Schork M.A., Kulik T.J. Hemodynamic characteristics of neonates following first stage palliation for hypoplastic left heart syndrome. Circulation 1995;92(Suppl 2):267-271.[Abstract/Free Full Text]
  2. Kern J.H., Hayes C.J., Michler R.E., Gersony W.M., Quaegebeur J.M. Survival and risk analysis for the Norwood procedure for hypoplastic left heart syndrome. Am J Cardiol 1997;80:170-174.[Medline]
  3. Jonas R.A., Hanson D.D., Cook N., Wessel D. Anatomic subtype and survival after reconstructive operation for hypoplastic left heart syndrome. J Thoracic Cardiovasc Surg 1994;107:1121-1128.[Abstract/Free Full Text]
  4. Beckman C.B., Moller J.H., Edwards J.E. Alternate pathways to pulmonary venous flow in left-sided obstructive anomalies. Circulation 1975;52:509-516.[Abstract/Free Full Text]
  5. O’Connor W.N., Cash J.B., Cottrill C.M., Johnson G.L., Noonan J.A. Ventriculo-coronary connections in hypoplastic left hearts: an autopsy microscopic study. Circulation 1982;66:1078-1086.[Free Full Text]
  6. Sauer U., Gittenberger-de Groot A.C., Geishauser M., Babic R., Buhlmeyer K. Coronary arteries in the hypoplastic left heart syndrome: Histopathologic and histometrical studies and implications for surgery. Circulation 1989;80(Suppl 1):I168-I176.
  7. Baffa J.M., Chen S.L., Guttenberg M.E., Norwood W.I., Weinberg P.M. Coronary artery abnormalities and right ventricular histology in hypoplastic left heart syndrome. J Am Coll Cardiol 1992;20:350-358.[Abstract]
  8. Freedom R.M., Culham J.A.C., Moes C.A.F., Harrington D.P. Selective aortic root angiography in the hypoplastic left heart syndrome. Eur J Cardiol 1976;4:25-29.[Medline]
  9. Sarris G.E., Drummond-Webb J.J., Ebdeid M.R., Latson L.A., Mee R.B. Anomalous origin of left coronary artery from the right pulmonary artery in hypoplastic left heart syndrome. Ann Thorac Surg 1997;64:836-838.[Abstract/Free Full Text]
  10. Bartram U., Grunenfelder J., van Praagh R. Causes of death after the modified Norwood procedure: a study of 122 postmortem cases. Ann Thorac Surg 1997;64:1795-1802.[Abstract/Free Full Text]



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