Late noninvasive evaluation of cardiac performance in mildly symptomatic older patients with Ebstein's anomaly of tricuspid valve: Role of radionuclide imaging

AbstractTen patients 8 to 54 years of age with isolated Ebstein's anomaly of the tricuspid valve were evaluated by electrocardiography, maximal exercise treadmill testing, 24 h electrocardiographs (ECG) monitoring, echocardiography and rest radionuclide imaging of the left ventricle. The patients presented after the 1st year of life and had not undergone surgical intervention. All except one were in functional class II. No patient had preexcitation on the surface ECG, but abnormal tachyarrhythmias or bradyarrhythmias were seen in five patients on 24 h ECG monitoring. Subnormal exercise performance was observed in five patients.Echocardiography demonstrated typical variable tricuspid valve displacement and paradoxic interventricular septal motion. Left ventricular end-diastolic dimensions were normal in all patients, but posterior wall motion was reduced in two. Moderate to severe tricuspid regurgitation with a Doppler jet velocity <2.5 m/s was demonstrated in eight patients. Left ventricular radionuclide scintigraphy revealed a subnormal ejection fraction (<50%) in 5 of 10 patients; these 5 had previously shown suboptimal exercise performance.The two youngest patients (<15 years) had no arrhythmia, normal exercise performance and normal left ventricular ejection fraction. There was no correlation between the degree of tricuspid valve displacement or régurgitation and the presence of rhythm disturbance, exercise performance or radionuclide left ventricular function.Late evaluation of patients with Ebstein's anomaly may demonstrate significant unsuspected abnormalities in cardiac rhythm, exercise performance and left ventricular function. Radionuclide scintigraphy is a useful noninvasive technique for assessing left ventricular dysfunction in these patients

Detecting Pheochromocytoma: Defining the Most Sensitive Test

OBJECTIVE: To define the most sensitive biochemical test to establish the diagnosis of pheochromocytoma and also to assess the potential role of iodine 131-labeled metaiodobenzylguanidine scintigraphy ((131)I-MIBG) in the diagnosis of this tumor. SUMMARY BACKGROUND DATA: Pheochromocytoma is a rare, catecholamine-producing tumor with preferential localization in the adrenal gland. Despite its importance, the most sensitive test to establish the diagnosis remains to be defined. METHODS: Prospective data collection was done on patients with pheochromocytoma treated at the Duke University Medical Center and the Durham Veterans Affairs Medical Center, Durham, NC. All urinary, plasma, and platelet analyses were highly standardized and supervised by one investigator (J.M.F.). (131)I-MIBG scans were independently reviewed by 2 nuclear medicine physicians. RESULTS: A total of 152 patients (55.3% female) were enrolled in the present analysis. Patients were predominantly white (73.7%). Spells (defined as profuse sweating, tachycardia, and headache) and hypertension at diagnosis were present in 51.4% and 66.6%, respectively. Bilateral disease was found in 12.5%, malignant pheochromocytoma in 29.6%, and hereditary forms in 23.0%. The most sensitive tests were total urinary normetanephrine (96.9%), platelet norepinephrine (93.8%), and (131)I-MIBG scintigraphy (83.7%). In combination with (131)I-MIBG scintigraphy, platelet norepinephrine had a sensitivity of 100%, plasma norepinephrine/MIBG of 97.1%, total urine normetanephrine/MIBG of 96.6%, and urine norepinephrine/MIBG of 95.3%. CONCLUSIONS: The tests of choice to establish the diagnosis of pheochromocytoma are urinary normetanephrine and platelet norepinephrine. A combination of (131)I-MIBG scintigraphy and diagnostic tests in urine, blood, or platelets does further improve the sensitivity. We thus advocate performing an MIBG scan if the diagnosis of pheochromocytoma is clinically suspected and catecholamine measurements are within the normal range