Familial ligand-defective apolipoprotein B-100: simultaneous detection of the ARG3500-->GLN and ARG3531-->CYS mutations in a French population

BACKGROUND: Flask pulmonary edema (FPE) may be a manifestation of renovascular hypertension (RVHTN) and unresponsive to antihypertensive therapy. METHODS: Response to antihypertensive therapy and perioperative outcomes were determined in 5 consecutive patients with FPE. RESULTS: A mean of 2.3 admissions for the treatment of FPE were observed despite a mean cardiac ejection fraction of 60%. Preoperative treatment was attempted for 12 days and included ventilatory support (n = 3) and hemodialysis (n = 2). Total decreased renal perfusion was demonstrated by arteriography and radionuclide scans, no patient having a functional, contralateral kidney. Renal revascularizations were not associated with mortalities; 1 patient experienced atalectasis requiring bronchoscopy. All patients were extubated within 48 hours of surgery. A significant reduction in blood pressure (BP, 46%) and serum creatinine (Cr, 53%, P < or = 0.05) was observed. A mean of 1 antihypertensive medication was required at discharge compared with 3.4 on admission. At follow-up (mean 57 months) all patients remain cured of FPE. CONCLUSIONS: Medical management was unsuccessful in the treatment of FPE. Renal revascularization was associated with low morbidity and mortality, control of BP, restoration of renal function, and cure of FPE. These data suggest surgical intervention is the optimal mode of treatment of RVHTN associated with FPE.