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(Circulation. 1962;26:266.)
© 1962 American Heart Association, Inc.

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Congenital Absence of the Pulmonary Valve

The Clinical Syndrome of Tetralogy of Fallot with Pulmonary Regurgitation

ROBERT A. MILLER M.D.¹; MAURICE LEV M.D.¹; MILTON H. PAUL M.D.¹

¹ From the Division of Cardiology, The Children's Memorial Hospital, The Department of Pediatrics, Northwestern University Medical School, and the Congenital Heart Disease Research and Training Center, Chicago, Illinois.

A group of cases of tetralogy of Fallot with congenital absence of the pulmonary valve are presented, and it is indicated that this abnormality presents a characteristic clinical, physiologic, and pathologic syndrome.

It is postulated that the aneurysmal dilatation of the pulmonary artery may result either from a congenital weakness at the base of the pulmonary artery or from the hemodynamic effects of a ventricular septal defect and infundibular stenosis in the presence of pulmonary regurgitation.

The absence of the pulmonary valve has not been a deterrent to successful corrective surgery aimed at the basic pathology of tetralogy of Fallot in three cases.

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