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(Circulation Research. 2008;103:337.)
© 2008 American Heart Association, Inc.

Editorials

Pulmonary Atresia or Persistent Truncus Arteriosus

Is It Important to Make the Distinction and How Do We Do It?

Margaret L. Kirby

From the Department of Pediatrics (Neonatology), Neonatal-Perinatal Research Institute, Duke University Medical Center, Durham, NC.

Correspondence to Margaret L. Kirby, Department of Pediatrics (Neonatology), Neonatal-Perinatal Research Institute, Bell Building, Rm 157, Box 3179, Duke University Medical Center, Durham, NC 27712. E-mail mlkirby@duke.edu

See related article, Circ Res 2008:103:142–148

Key Words: arterial pole development • conotruncal malformations • pulmonary atresia • persistent truncus arteriosus

An extract of the first 250 words of the full text is provided, because this article has no abstract.

The congenital cardiac anomaly known as tetralogy of Fallot (TOF) is characterized by right ventricular outflow tract obstruction caused by subpulmonary stenosis, dextroposition (overriding) of the aorta, a ventricular septal defect, and right ventricular hypertrophy. The right ventricular hypertrophy is secondary to the presence of right ventricular outflow obstruction (pulmonic valvar or subvalvar stenosis or in the most severe case, atresia). Cyanosis in these patients is attributable to the passage of systemic venous blood into the aorta, bypassing the lungs, with the degree of cyanosis dependent on the severity of the outflow tract obstruction. The malformations that are classified as persistent truncus arteriosus (PTA) are characterized by a single multicuspid semilunar valve with a single vessel, the truncus, arising from the ventricles and giving rise to systemic, pulmonary, and coronary circulations. Thus, both PTA and TOF with pulmonary atresia are characterized by a single vessel emanating from the heart. In PTA, the septation that would divide the common arterial trunk into an aorta and pulmonary trunk is missing, whereas in TOF with pulmonary atresia, it is unclear whether this septation is missing or misplaced. Diagnosis of TOF with pulmonary atresia relies on the presence of a pulmonary valve remnant by clinical imaging. This criterion permits differentiation of TOF with pulmonary atresia and PTA. However, if the pulmonary atresia develops embryonically before a valve is formed, then this criterion would not distinguish PTA from TOF with pulmonary atresia. New findings in experimental mouse models by Théveniau-Ruissy et al reported recently in . . . [Full Text of this Article]