Ebstein's anomaly: a potential cause of misinterpretation of myocardial perfusion scintigraphy.

We present the case of a 43-year-old woman with a history of Ebstein's anomaly, who was referred for a myocardial perfusion single-photon emission computed tomography (SPECT) study due to angina-like symptoms. Dilatation of the right ventricle and right atrium, apical displacement of the septal tricuspid leaflet, small left ventricle with good left ventricular and a moderate degree of tricuspid regurgitation with mild pulmonary hypertension were found on echocardiography. Myocardial SPECT perfusion imaging with [99m Tc]tetrofosmin demonstrated a large defect in the inferior septal wall in the stress study with mild partial reversibility at rest. Coronary angiography revealed normal coronary arteries. Histological studies have shown that these patients develop increased fibrosis in the left ventricular wall and ventricular septum, especially in the basal and middle region of the septum, which adjoins with the atrialized component of the right ventricle. This is in concordance with the findings of the myocardial perfusion scan in the case described.

Discordance between echocardiographic and MRI findings in two cases of acute myocarditis mimicking myocardial infarction.

Two male adolescents were admitted due to chest pain influenced by the respiratory movements. A lobar pneumonia caused by Mycoplasma pneumoniae, was radiographically and serologically diagnosed in the first patient (a 15-year-old boy) and a febrile diarrhea of unidentified etiology, despite repetitive stool cultures and serological assessments in the second one (a 19-year-old male). Both patients combined the aforementioned infectious conditions with outstanding thoracic pain the previous hours before admission, markedly elevated cardiac enzymes and ST-segment elevation in the inferior and left precordial leads with a normal coronary angiogram, thus composing a clinical setting highly indicative of acute myocarditis. Surprisingly however, the echocardiogram performed in both patients failed to reveal any regional or global wall motion abnormalities or even diastolic dysfunction and remained absolutely normal throughout their 3-month follow-up period. Cardiac MRI within 7 days, using T2-weighted and gadolinium-enhanced T1-weighted images demonstrated extensive focal contrast enhancement, consistent with acute inflammatory myocardial involvement. It can therefore be concluded that contrast MRI is a more sensitive method than the echocardiogram for the diagnosis of acute focal myocarditis.

Clinical and prognostic value of elevated CA125 levels in patients with congestive heart failure.

BACKGROUND: Increased serum levels of carbohydrate antigen 125 (CA125), a tumor marker associated with ovarian cancer, have also been reported in other malignant and non-malignant diseases. We assessed the correlation of the CA125 serum levels with the severity of congestive heart failure (CHF) and investigated their potential prognostic value in relation to major cardiovascular events. METHODS: CA125 levels were measured in 95 male patients aged 70+/-10 years, admitted for decompensated CHF. The patients were divided into three groups, according to their New York Heart Association (NYHA) functional class. Group A contained 23 patients in NYHA IV, group B 34 patients in NYHA III, and group C 38 patients in NYHA I-II. The patients were also divided into two groups according to their CA125 value on admission. Group 1 included 45 patients with normal CA125 levels and group 2 50 patients with elevated CA125. All patients were followed for 15+/-8.5 months and the major cardiovascular events (death and re-hospitalizations due to CHF) were recorded. RESULTS: Serum levels of CA125 were higher in groups A and B than in group C (36.4 [19.8-82] U/ml and 34.6 [26-78] U/ml vs. 25.3 [9.1-29] U/ml, respectively, p<0.05). No correlation was detected between CA125 levels and left ventricular ejection fraction. However, patients with pulmonary congestion and peripheral edemas had higher levels of CA125 (p=0.002 and p<0.03, respectively). Nineteen patients died during the follow-up period, but the mortality rate was not significantly different between groups 1 and 2 (p=0.8). Nevertheless, the patients of group 1 reported fewer re-hospitalizations than patients of group 2 (p=0.003). The relative risk (RR) for re-hospitalization was calculated to be RR: 0.4, 95% CI: 0.215-0.76 (p<0.005), in patients with elevated levels of CA125. Cox regression analysis revealed that CA125 had independent prognostic value (OR: 1.007 [95% CI: 1.004-1.010], p<0.0001) for re-hospitalizations. CONCLUSION: Serum levels of CA125 are associated with the severity of CHF and are also independent predictive markers for re-hospitalizations. We therefore conclude that CA125 can be used as a prognostic marker of disease severity and increased morbidity in patients with decompensated CHF.

Atrioventricular conduction disturbances in a young patient with Fabry's disease without other signs of cardiac involvement.

A 30 year-old male patient with a history of Fabry's disease, was referred to hospital with symptoms of dizziness, hypotension and weakness. Fabry's disease had been diagnosed 2 years before, based on angiokeratoma and hypohidrosis on physical examination and complete lack of alpha-galactosidase A on laboratory examination. The ECG on admission demonstrated sinus bradycardia, with a poor response to atropine administration. Echocardiograms on admission and 2 years before were normal, as well as Holter ambulatory ECG recording. Subsequent electrophysiological study demonstrated mild AV conduction disturbances at a site proximal to His, and the patient was simply advised to be regularly followed up. It can therefore be concluded that even young patients with Fabry's disease and normal echocardiograms might develop cardiac symptoms due to AV conduction abnormalities.

The significance of CA125 levels in patients with chronic congestive heart failure. Correlation with clinical and echocardiographic parameters.

OBJECTIVE: To assess serum levels of carbohydrate antigen 125 (CA125) in patients with chronic congestive heart failure (CHF) and to assess any correlation with clinical symptoms and echocardiographic indices. PATIENTS AND METHODS: We enrolled 77 male patients (mean age: 73+/-10 years) admitted to the Cardiology Emergency Department (ED) with cardiac symptoms requiring hospitalization. Diagnosis of CHF was based upon medical history or initial echocardiographic evaluation on current admission. Serum CA125 was measured by an enzyme immunoradiometric assay, on admission and before discharge. RESULTS: The median overall CA125 value was 22.4 (11.5-48.9) U/ml. Serum CA125 levels were related to the severity of CHF [New York Heart Association (NYHA) class I: 19.2 (7.2-31) U/ml, NYHA class II: 17.6 (10-23) U/ml, NYHA class III: 32 (25-77) U/ml and NYHA class IV: 34.3 (18.6-77) U/ml (p<0.04)]. Patients in NYHA classes III and IV had significantly higher mean values of CA125, than patients in class II (p<0.005 and p<0.05, respectively). Moreover, patients with fluid congestion (pulmonary congestion, ankle edema) had higher levels of serum CA125 than patients without congestion (p=0.002 and p<0.03, respectively). Finally, levels of serum CA125 correlated weakly with right ventricular systolic pressure (RVSP) and renal function, while no significant correlation was found between CA125 and E wave deceleration time on Doppler echocardiography, left ventricular ejection fraction (LVEF), left ventricular end-diastolic diameter (LVEDD), liver function and the medical treatment prescribed. CONCLUSION: Serum CA125 is associated with the clinical severity of CHF and the symptoms and signs of fluid congestion and therefore may be a useful additional tool for the evaluation and clinical staging of these patients.