Heart Failure in Cystic Fibrosis

Abstract

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.

Gas Exchange in Disease: Asthma, Chronic Obstructive Pulmonary Disease, Cystic Fibrosis, and Interstitial Lung Disease

“…Not unexpectedly, mortality was significantly higher over a 5-year follow-up period in those patients with pulmonary hypertension (P = 0.0129). This is in agreement with earlier data (211) indicating that the median survival of children with CF after the development of right heart failure was 4 months.…”

Complications of Cystic Fibrosis Pulmonary Disease

“…Although chronic cor pulmonale may be present and very slowly progressive over many years in a group of CF patients, the prognosis for such patients is dismal once overt cardiac failure develops. Stern and associates 57 found mean survival time to be 8 months in 55 such patients, and Moss and associates 62 reported 87% of 36 children with overt failure had died at 30 months after initial symptoms. At best, therapeutic measures for cor pulmonale "can be viewed as delaying actions."…”

CFTR deficiency causes cardiac dysplasia during zebrafish embryogenesis and is associated with dilated cardiomyopathy

“…Ryland et al reported that post-mortem autopsies of 37 patients uncovered right ventricular hypertrophy in all patients, and right ventricular dilatation in 51% of patients[38]. In children with CF, right ventricular hypertrophy was also evidenced in over 70% of cases in post-mortem studies[44]. Here, we used a zebrafish model without lung tissue to demonstrate that cftr deficiency impairs cardiac development at early embryonic stages, supporting the argument that the CF-related heart disease is an intrinsic primary defect in the CF heart.Dilated cardiomyopathy (DCM) is a primary cardiomyopathy characterized by unilateral or bilateral cardiac expansion, myocardial contractile dysfunction, and thinning of the ventricular wall.…”