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March 1980

Heart Failure in Cystic Fibrosis: Treatment and Prognosis of Cor Pulmonale With Failure of the Right Side of the Heart

Author Affiliations

From the Divisions of Pediatric Pulmonary Disease (Drs Stern, Boat, Matthews, and Doershuk) and Pediatric Cardiology (Drs Borkat, Hirschfeld, and Liebman), Case Western Reserve University, School of Medicine, and Rainbow Babies and Childrens Hospital (Drs Stern, Borkat, Hirschfeld, Boat, Matthews, Liebman, and Doershuk), Cleveland.

Am J Dis Child. 1980;134(3):267-272. doi:10.1001/archpedi.1980.02130150025007

• Failure of the right side of the heart with cardiac dilation and fluid retention occurred in 55 of 170 patients who died of cystic fibrosis; six patients survive. All had severe hypoxia, but 24% had normal Paco2. Cardiac catheterization showed high mean pulmonary artery pressure and resistance. Pulmonary artery wedge pressure was greater than 12 mm Hg in 40% of the patients. Mean survival was eight months. Male survival was significantly better than female survival. Digitalis treatment was of no clear benefit. Tolazoline hydrochloride was also ineffective. Recent medical advances have not substantially affected prognosis.

(Am J Dis Child 134:267-272, 1980)