Ann Thorac Surg 2006;81:341-343
© 2006 The Society of Thoracic Surgeons
Case report
Hypoplastic Left Heart Syndrome With Anomalous Origin of the Right Coronary Artery
Julie Cleuziou, MD
a
,
*
,
Felix Haas, MD
c
,
Christian Schreiber, MD
a
,
Hans-Jörg Mössinger, MD
b
,
Rüdiger Lange, MD
a
a Department of Cardiovascular Surgery, German Heart Center Munich at the Technical University, Munich, Germany
b Department of Anesthesia, German Heart Center Munich at the Technical University, Munich, Germany
c Department of Pediatric Cardiothoracic Surgery, Wilhelmina Children's Hospital, UMC Utrecht, the Netherlands
Accepted for publication September 24, 2004.
* Address correspondence to Dr Cleuziou, Department of Cardiovascular Surgery, German Heart Center Munich, Lazarettstrasse 36, D-80636 Munich, Germany (Email: cleuziou{at}dhm.mhn.de).
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Abstract
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Hypoplastic left heart syndrome in association with an anomalous origin of a coronary artery from the pulmonary artery is a very rare congenital malformation. In the few reported cases, the left coronary artery or the circumflex artery arises from the right pulmonary artery. We describe a newborn who presented with hypoplastic left heart syndrome, and at the time of operation had an anomalous origin of the right coronary artery from the right pulmonary artery that was detected. The patient underwent a successful modified Norwood procedure with direct reimplantation of the right coronary artery to the neo-aorta.
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Introduction
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Anomalous origin of the right coronary artery (RCA) from the pulmonary artery is a rare congenital malformation that can be found as a sole entity or in association with other cardiac defects [1]. We believe that the anomalous origin of the RCA from the right pulmonary artery (RPA) in association with hypoplastic left heart syndrome (HLHS) has never been described before. In the HLHS, the right coronary artery supplies the systemic ventricle; therefore adequate coronary perfusion is mandatory. We report the case of a newborn with HLHS and an aberrant right coronary artery originating from the RPA and its surgical management.
A 3,250 g full-term newborn was delivered by caesarian section with Apgar scores of 9,10,10 to a 38-year-old mother after an uneventful pregnancy. Prenatal diagnosis of HLHS had been made by fetal echocardiography at 22 weeks of gestation. Preoperatively, the newborn was in good clinical condition breathing room air with saturations of 89%. Echocardiography showed HLHS with mitral atresia, severe aortic stenosis, hypoplastic ascending aorta, and an aortic arch of 4 mm with severe coarctation. Antegrade flow through the aortic valve was still present. The duct was large and widely open. Right atrial enlargement was present with a restrictive patent foramen ovale and a rudimentary left ventricle with a small perimembranous ventricular septal defect. On day 6 of life, we performed a modified Norwood operation [2]. Through a standard median sternotomy, the chest was opened, the thymus gland was excised, and the pericardium was opened. On dissection of the RPA, an aberrant vessel of about 2 mm in diameter arising from its middle part at its inferior aspect was found (Fig 1). On further inspection, we could not visualize the origin of the RCA. Cardiopulmonary bypass was instituted with cannulation of the main pulmonary artery and single venous cannulation of the right atrium. The right and left pulmonary arteries were snared distally to the aberrant vessel. The patient was cooled down to 16°C core temperature and circulatory arrest was commenced. To clarify the course of the anomalous vessel, blood was selectively injected into the aberrant artery through a syringe. It became clear that the RCA and all its right ventricular branches were supplied by it. Thus, the RPA was transected just proximal and distal to the aberrant vessel so that enough tissue would remain to allow the reimplantation of the RCA. Then the modified Norwood procedure was performed in the usual manner using circulatory arrest and a period of selective brain perfusion. The hypoplastic aortic arch was augmented with a patch of cryopreserved pulmonary homograft. The RCA was reimplanted to the posterior wall of the native aorta using a 7-0 resorbable suture (Fig 2). During rewarming, a right ventriculotomy was performed and a 5-mm Gore-Tex tube (W. L. Gore & Assoc, Flagstaff, AZ) was anastomosed. The cross clamp was removed, the coronaries were well filled, and spontaneous sinus rhythm had occurred. The RPA was reconstructed with a homograft patch into which the distal end of the Gore-Tex tube (W. L. Gore & Assoc) was inserted to complete the right ventricle to the pulmonary artery conduit. After full rewarming, the patient was weaned from extracorporeal circulation with moderate inotropic support (adrenaline, 0.05 µg/kg/min; milrinone, 0.5 µg/kg/min). Postoperative recovery was uneventful without any arrhythmias or electrocardiographic changes.
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Fig 1. Intraoperative view of the anomalous origin of the right coronary artery (arrow) from the right pulmonary artery (RPA). (Ao = aorta; RA = right atrium; RV = right ventricle.)
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Comment
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Coronary anomalies are a rare feature in association with HLHS and most likely affect the left coronary artery. Most abnormalities in HLHS concern the coronary morphology, such as wall thickness and tortuosity rather than the origin [3]. In a postmortem study of 151 hearts with HLHS, Baffa and colleagues [4] found 51 hearts with coronary anomalies. They included coronary–ventricular communications, single left and single right coronary arteries, as well as tortuosity. Anomalous origin of coronaries in HLHS is very rare. Aberrant origin of the left coronary artery from the right pulmonary artery has only recently been described in the literature [5, 6]. In a study of 15 patients with HLHS, Pizarro and colleagues [7] found 1 patient with the circumflex artery arising from the left pulmonary artery. We believe that there has been no publication of anomalous origin of the RCA from the RPA. In our patient, the anomalous coronary was found only by chance. Obviously, the anomalous origin of the RCA could have been diagnosed with preoperative angiography; however, echocardiography alone is our routine preoperative diagnostic tool in HLHS. Although this congenital malformation is very rare, one should be aware of the possibility of aberrant origin of coronaries in HLHS when difficulties are encountered while weaning off bypass [6]. Intraoperative inspection of the coronaries should be performed routinely in patients undergoing the Norwood procedure. Reimplantation of the coronary artery into the reconstructed aorta has proven to be the best option for repair [5, 6]. The relatively large ascending aorta of our patient made reimplantation of the aberrant vessel into the native aorta possible, which is favorable in view of the given potential for growth. In this case, a careful anastomosis without any kinking or torsion was mandatory to maintain adequate coronary perfusion to the systemic right ventricle. Experience with reimplantation of coronary arteries has grown through the arterial switch operation and has proven to be a safe and reliable method. We can advocate direct reimplantation of an anomalous coronary artery in HLHS as the best option for repair.
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References
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- Maher KO, Pizarro C, Gidding SS, et al. Hemodynamic profile after the Norwood procedure with right ventricle to pulmonary artery conduit Circulation 2003;108:782.[Abstract/Free Full Text]
- Sauer U, Gittenberger-de Groot AC, Geishauer M, Babic R, Bühlmeyer K. Coronary arteries in the hypoplastic left heart syndrome. Histopathologic and histometrical studies and implications for surgery Circulation 1989;80(Suppl 1):I168-I176.
- Baffa JM, Chen SL, Guttenberg ME, Norwood WI, Weinberg PM. Coronary artery abnormalities and right ventricular histology in hypoplastic left heart syndrome J Am Coll Cardiol 1992;20(2):350-358.[Abstract]
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Abstract
Introduction
