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CONGENITAL ABSENCE OF THE PULMONARY VALVE ASSOCIATED WITH CONGENITAL APLASIA OF THE THYMUS (DIGEORGE’S SYNDROME)

JUDITH S. ROSE M.D., DAVID C. LEVIN M.D., STANLEY GOLDSTEIN M.D., and WAYNE LASTER M.D.

Two cases of DiGeorge’s syndrome associated with tetralogy of Fallot and absence of the pulmonic valve are reported. Both infants had complete absence of the thymus and parathyroids. This brings to a total of 4 the number of reported cases of absence of the pulmonic valve in this rare syndrome.

Some of the expected roentgen signs, such as a narrow mediastinal silhouette and marked enlargement of the central pulmonary arteries, may be masked by cardiomediastinal shift due to bronchial compression.

The angiographic findings are discussed.


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