Complete Follow-Up Echocardiograms are Needed to Detect Stenosis of Normally Connecting Pulmonary Veins

Reimbursement for limited echocardiograms focusing on known pathology rather than complete studies has recently received widespread attention. Few data are available to determine if these limited examinations provide enough information to adequately evaluate many forms of congenital heart disease. Stenosis of normally connecting pulmonary veins is a congenital or acquired cardiac anomaly that is difficult to diagnose clinically and may be detectable only by echocardiography. To evaluate the yield of complete versus limited echocardiograms for detecting the presence and development of stenosis in pulmonary veins with anatomically normal connections, the cardiology database was searched for all patients with this diagnosis presenting between June 1990 and January 2000. Charts were reviewed for demographic data, associated defects, surgeries, and outcomes. Angiograms and echocardiograms were reviewed for location and severity of pulmonary vein stenosis. A pulsed-wave Doppler signal of > 1.6 mm/sec with loss of phasic flow was used to define stenosis. Eighteen patients were identified and ranged in age at first evaluation from 1 day to 17 years (median 15 days). All 18 patients had associated cardiac anomalies, and 4 (22%) of 18 had Trisomy 21. Pulmonary vein stenosis was detected on the initial evaluation in 5 patients, detected 8 +/- 5 months after the initial echocardiogram in 11 patients, and missed by echocardiography in 2 patients who were diagnosed by cardiac catheterization. The initial echocardiograms were complete, with pulsed-Doppler sampling of all four pulmonary veins in 17 of 18 patients. Of the 12 patients who had echocardiographic evidence of late stenosis, 10 had 17 limited interim studies prior to eventually having a complete diagnostic follow-up study. Of the two patients in whom the diagnosis was missed by echocardiography, one initial study was technically inadequate (17-year-old) and one had only limited interim studies after the initial echocardiogram. Of the 9 patients in whom repair of the pulmonary vein stenosis was attempted, 3 had no residual obstruction and 6 had progressive stenosis (three deaths). Of the remaining nine patients who had no intervention for their pulmonary venous stenosis, four have died from progressive pulmonary hypertension. Stenosis of normally connecting pulmonary veins is an uncommon lesion that has a significant impact on clinical outcome. The stenoses might be undetectable on limited echocardiograms that focus on evaluating only specified pathology. Complete follow-up examinations might be warranted to diagnose this lesion. This may be an important consideration when formulating reimbursement policies.