Cardiac involvement is common in adult patients with the presence of HLA B27 with or without the HLA B27-associated spondyloarthropathy ankylosing spondylitis. Most patients with juvenile spondyloarthropathy, which begins at age 16 or younger, do not have spinal involvement and there are only few reports of cardiac involvement. This study sought to assess the prevalence of carditis in patients with HLA B27-associated juvenile arthritis (B27-JA). In a controlled study, 40 patients with B27-JA, among them only 1 with ankylosing spondylitis, were examined by electrocardiogram and echocardiography with pulsed and color-flow Doppler at rest and at the termination of a bicycle exercise and compared to an age- and sex-matched control group negative for HLA B27. Four patients with B27-JA, and none in the control group, had inflammatory aortic regurgitation. Late diastolic flow velocity was significantly increased in patients with B27-JA at the termination of exercise. HLA B27 is a risk factor for endo-/myocardial damage in patients with B27-JA, even in the presence of only short and mild articular disease. Patients with B27-JA should be screened for the presence of aortic regurgitation.

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In a 10-year-old boy with pseudohypoparathyroidism type Ia (PHP-Ia) echocardiography detected calcifications within the ventricular septum. This is thought to be the first report of intracardiac calcifications in PHP. Echocardiography should be done routinely in all patients with this rare disorder.

Balloon valvuloplasty (BVP) of pulmonary stenosis (PST) was performed in 305 infants and children in 20 heart centres of the Federal Republic of Germany. 273 patients suffered from isolated PST. Their age ranged from 3 days to 18 years (mean = 5.85 +/- 4.82 years), their weight from 2.9 to 60 kg. The pressure gradient was measured invasively in the sedated or anaesthetized child, partly by CW Doppler. BVP was performed by mono- or trefoil catheters, mostly oversized. As a result, mean pressure gradient fell from 71.7 +/- 32 mmHg before to 31.8 +/- 25 mmHg immediately after BVP (P less than 0.0001). The gradient was reduced by less than 30% in 13% of the patients, by 30-49% in 23%, and by greater than or equal to 50% in 64%. Among 33 patients with a gradient reduction of less than 30%, nine were aged less than 1 year, nine had dysplastic valves, and nine had a short-term infundibular reaction and good late results. Gradient reduction was not improved with oversized balloons and was independent of the initial gradient. Follow-up for a mean 11.2 +/- 8 months showed a further slight improvement, probably due to regression of infundibular hypertrophy. In the whole group we observed infundibular reaction in 12%, serious complications with one late death in 4%. From the start, BVP proved to be a secure and effective tool for elimination of pulmonary stenosis.