[Isolated pulmonary stenosis. Radiological aspects 10 to 25 years after surgical treatment].
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The authors report the clinical history of a 15 years old young man with severe congenital pulmonary valvular stenosis who was treated, for the first time in Panama, with percutaneous balloon pulmonary valvuloplasty (PBPV). The patient presented with an episode of syncope at rest. The EKG showed right ventricular hypertrophy (RVH) and suggested the presence, with the echocardiogram, of pulmonary systolic pressures higher than the systolic systemic pressures. The diameter of the interior pulmonary ring was estimated to 20 mm. The chest X-ray suggested the presence of RVH. Multiple premature ventricular systoles were detected by the Holter monitoring. Cardiac catheterization showed pressures of 150/12 mm Hg in the right ventricle (RV) and 24/9 mm Hg in the pulmonary artery (PA) with a transvalvular gradient of 126 mm Hg. PBPV was successfully done on 19 june 1993. The pressures in the RV were then 38/5 mm Hg and in the PA 25/7 mm Hg with a residual gradient of 13 mm Hg. Twenty minutes after completion of the procedure the patient experienced a hypertensive crisis and pulmonary edema, a complication of PBPV not previously reported in the literature.
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Although Ebstein's anomaly occurs mostly in isolation, in 2 to 5% of cases it is combined with pulmonary artery stenosis. This combination noticeably aggravates the natural course of the disease and complicates the diagnosis of the pathology. On the basis of five observations the authors present the specific differential-diagnostic signs which help to make the correct diagnosis. The key role in the identification of combined forms of Ebstein's anomaly is played by the catheterization of the cardiac chambers and by angiocardiography.
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