The frequency of hypertrophic osteoarthropathy in cyanotic congenital heart disease has previously been considered to be very low. Only a few isolated reports have described such an association. We studied 32 consecutive patients older than 6 years with various types of cyanotic congenital heart disease and examined each case for hypertrophic osteoarthropathy. We also assessed the role of the altered cardiopulmonary hemodynamics in the development of hypertrophic osteoarthropathy by means of cardiac catheterization. Our results showed that 31% of the patients had hypertrophic osteoarthropathy. When the hemodynamic parameters of the group of patients with hypertrophic osteoarthropathy were compared with those of the remaining patients, we found significant differences in the systemic blood flow (P I 0.05), right-to-left shunt (P I 0.05), and arterial oxygen unsaturation (P I 0.005). Thus, we found a much higher prevalence of hypertrophic osteoarthropathy in patients with cyanotic congenital heart disease than has previously been recognized. The development of hypertrophic osteoarthropathy is related to the degree of bypass of the lung. This is consistent with the concept that hypertrophic osteoarthropathy results from mediators in the systemic venous circulation that escape inactivation in the pulmonary capillary bed.Although digital clubbing is common in patients with cyanotic congenital heart disease (CCHD), hypertrophic osteoarthropathy (HOA) has rarely been reported in this condition. In two standard textbooks of internal medicine (1-3), the association of HOA and CCHD is given little attention. Harrison (1) mentions only in passing that HOA may occur in CCHD. Cecil (2) lists it with lung cancer, mesothelioma, and bronchiectasis, but omits mention of it with CCHD. The English language literature does contain a few isolated reports of HOA associated with CCHD (4-8). Trever (6) quoted Helen Taussig that only 3 cases of HOA were seen at the Harriet Lane Home in 3,000 patients with CCHD. It seems to us, however, that the true frequency of HOA in CCHD has never been properly assessed, since many cases may have been missed or ignored by physicians far more interested in the heart than the joints.In the present study, we investigated the prevalence of HOA in CCHD patients over 6 years old in our institution. We also assessed the role of the altered cardiopulmonary hemodynamics in the development of HOA by means of cardiac catheterization.
PATIENTS AND METHODSWe studied 32 consecutive CCHD patients older than 6 years who were admitted t o our institution for evaluation of their heart conditions. The age limit was set arbitrarily; all previously reported cases were older than 6 years (4) and also, with this age limit, the clinical and x-ray assessments are more accurate. Each patient had a complete history and