T Cavalle-Garrido1, A Bernasconi1, D Perrin2 and R H Anderson3
1) Division of Cardiology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
1) Division of Cardiology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada
2) Division of Pathology, Department of Laboratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada
3) Cardiac Unit, Institute of Child Health, University College, London, UK
Objectives: To determine the characteristic morphological features of hearts with concordant ventriculoarterial connections and parallel arterial trunks, and to provide unequivocally a method to describe their anatomy.
Design, methods and patients: The entire cardiac database and cardiac pathological archive at the Hospital for Sick Children, Toronto, Ontario, Canada, was interrogated to identify all patients with concordant ventriculoarterial connections and parallel arterial trunks. The clinical records, autopsy reports and actual cardiac specimens of those who underwent autopsy, were reviewed.
Results: 8 cases meeting our criteria were identified. The infundibular anatomy was variable, including four hearts with bilateral infundibulums, three with subpulmonary infundibulums and one with bilaterally absent infundibulums. Considerable variability was also found in the type of atrial arrangement, along with the morphology of the atrioventricular junctions. The most common findings were the usual atrial arrangement (n = 5), left juxtaposition of the right atrial appendages (n = 3), an atrial septal defect (n = 6), univentricular atrioventricular connection (n = 5), ventricular septal defect (n = 8) and pulmonary obstruction (n = 4). In addition, five specimens had either a single coronary artery or two coronary arteries arising from the anticipated right coronary aortic sinus.
Conclusions: Concordant ventriculoarterial connections with parallel arterial trunks can be found in a variety of segmental combinations. An accurate diagnosis of these rare hearts can be achieved by detailed analysis of not only the ventriculoarterial connections but also the infundibular anatomy and the spatial relationship of the arterial trunks. Particular attention to the coronary arteries is warranted.
Heart 2007;93:100-106
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