Myocardial integrated ultrasonic backscatter for early detection of cardiac involvement in patients with Wilson disease.

BACKGROUND/AIMS: Videodensitometry is a feasible noninvasive ultrasound tissue characterization method allowing early detection of myocardial changes. This study aimed to investigate ultrasonic backscatter properties of the myocardium in Wilson disease patients. MATERIALS AND METHODS: We compared cardiologically asymptomatic Wilson disease patients (W group) (n=18) with age-matched (26.7±9.6 years) healthy controls (C group) (n=15). Diagnosis of Wilson disease was made on the basis of clinical manifestations, family history, and laboratory findings and confirmed by liver biopsy. Transthoracic echocardiographic quantitative texture analysis was performed on data from the septum and left ventricular posterior wall, and mean gray level (MGL) histograms at end-diastole (d) and end-systole (s) were obtained after background correction (c). Cyclic variation index (CVI) was calculated using the formula [(cMGLd - cMGLs) / cMGLd] ×100. RESULTS: There were no significant differences in sex, age, body mass index, heart rate or blood pressure, and conventional echocardiographic parameters between the 2 groups. The cMGLs value of the posterior wall was higher in the W group than in the C group (30.9±2.6 vs. 22.2±2.7, p=0.033). The W group had a significantly lower CVI of the septum than did the C group (-22±4.4% vs. 43.4 ±12.9%, p<0.001), and there was no significant difference in the CVI of the posterior wall (-67.0±15.9% vs. 41.7±18.6%, p=0.32). CONCLUSION: Abnormalities in two-dimensional echocardiographic grey-level distributions were present in Wilson disease patients. These videodensitometric myocardial alterations were significantly lower in Wilson disease patients than in the controls, and this probably represents an early stage of cardiac involvement.

Right ventricular and septal function in patients with pulmonary hypertension.

OBJECTIVE: Pulmonary hypertension (PHT) exacerbates the functions of both ventricles. This prospective, randomised study was planned to investigate the effects of PHT on kinetics of both ventricles and the septum. METHODS: Twenty-five patients were randomly selected among the patients who had been planned to undergo mitral valve replacement (MVR) because of isolated mitral stenosis and divided into two groups according to their preoperative pulmonary artery pressure (PAP) values. Blood pool gated single photon emission tomography (BPGS) and transthoracic echocardiography were performed. Ventricles' regional, global and functional parameters were also assessed by using pulsed wave Doppler tissue imaging (DTI). RESULTS: Preoperative and postoperative PAP of the group 1 (PAP < 50 mmHg) were 40.0 ± 2.8 and 30.0 ± 2.6 mmHg (p = 0.03), group 2 (PAP ≥ 50 mmHg) were 71.9 ± 4.7 and 50.6 ± 3.5 mmHg (p < 0.05). The global right and left ventricle scores were decreased after the operation. The decrement was only significant in group 2. Considering the septal kinetics, right ventricle septal score was decreased from 7.6 to 3.3 (p < 0.05) in group 1, from 3.8 to 1.6 (p < 0.05) in group 2 postoperatively. CONCLUSION: Following MVR, a decrement in PAP values, and an improvement in ventricular function, especially in the right ventricular and septal kinetics were achieved. Furthermore, it was found that both DTI and BPGS techniques are beneficial to investigate the functional changes postoperatively and in the follow-up period of the patients who undergo mitral valve surgery.

A case of simultaneous anterior, inferior, and right ventricular ST-segment elevation myocardial infarction due to occlusion of the wrapped left anterior descending coronary artery.

Simultaneous anterior, inferior, and right ventricular ST-segment elevation myocardial infarction is an unusual condition. A 50-year-old male patient presented with severe, squeezing chest pain of an hour onset. ST-segment elevations were detected in all precordial derivations, DII, DIII, aVF and V3R, V4R. Coronary angiography showed a significant lesion in the left anterior descending (LAD) coronary artery, just below the second diagonal branch. Balloon predilatation was performed after 40 minutes of admission, followed by bare metal stent implantation, which resulted in ST-segment resolution in all leads and relief of chest pain. Echocardiography showed dyskinesia of the left ventricular apical wall, and hypokinesia of the interventricular septum and inferior wall. Left ventricular ejection fraction was 40%. Coronary angiography and cardiac CT angiography demonstrated a wrapped LAD. The patient was discharged five days after percutaneous coronary intervention (PCI) with stabilization of his clinical status. Ten days after PCI, he presented with chest pain associated with left ventricular anterior and inferior reinfarction. Successful balloon dilatation was performed for thrombotic in-stent restenosis. His clinical condition improved and he was asymptomatic for a month, during which no signs of deterioration were observed in electrocardiographic and echocardiographic findings.

P wave dispersion is prolonged in patients with Wilson's disease.

AIM: To investigate the P wave dispersion as a non-invasive marker of intra-atrial conduction disturbances in patients with Wilson's disease. METHODS: We compared Wilsonos disease patients (n = 18) with age matched healthy subjects (n = 15) as controls. The diagnosis was based on clinical symptoms, laboratory tests (ceruloplasmin, urinary and hepatic copper concentrations). P wave dispersion, a measurement of the heterogeneity of atrial depolarization, was measured as the difference between the duration of the longest and the shortest P-waves in 12 lead electrocardiography. RESULTS: All the patients were asymptomatic on cardiological examination and have sinusal rhythm in electrocardiography. Left ventricular and left atrial diameters, left ventricular ejection fraction and left ventricular mass index were similar in both groups. The Wilson's disease patients had a significantly higher P wave dispersion compared with the controls (44.7 +/- 5.8 vs 25.7 +/- 2.5, P < 0.01). CONCLUSION: There was an increase in P wave dispersion in cardiologically asymptomatic Wilson's disease patients which probably represents an early stage of cardiac involvement.

Complete regression of massive cardiac involvement associated with acute T cell leukemia following chemotheraphy.

Adult T cell leukemia/lymphomas are aggressive disorders, which infiltrate not only the bone marrow but extensively the visceral organs as well. A case with left ventricular systolic dysfunction with myocardial infiltration and massive pericardial effusion which was demonstrated with echocardiography is discussed. The patient responded well to pericardial drainage and subsequent chemotherapy. The dramatic improvement in echocardiographic findings after chemotherapy gave a clue to investigate suspected patients with aggressive leukemia and lymphomas for exclusion of leukemic infiltration of myocardium.

Comparison of traditional risk factors, natural history and angiographic findings between coronary heart disease patients with age <40 and >or=40 years old.

OBJECTIVE: In this study we aimed to examine the angiographic findings, traditional risk factors and natural history of Turkish patients <40 and >or=40 years old with coronary heart disease (CHD). METHODS: The records of 491 patients with stable angina pectoris or acute coronary syndrome (ACS), who had undergone coronary angiography (CAG) were reviewed. The patients <40 years (group 1) and >or=40 years (group 2) were compared. RESULTS: The study population was classified as group 1 with 240 patients (mean age 35.7+/-3.4 years) and group 2 with 251 patients (mean age 61.0+/-9.7 years). Smoking, family history, hypercholesterolemia, hypertriglyceridemia and low levels of high-density lipoprotein cholesterol were more prevalent in group 1 while diabetes mellitus, hypertension was higher in group 2. The common presentation among <40 years patients was ACS whereas stable angina was the most common presentation in patients >or=40 years old. Patients in group 1 showed a preponderance of single-vessel disease whereas patients of group 2 showed dominance of multivessel disease. Early clinical course of patients with ACS in group 1 was better than in group 2. CONCLUSION: Our study shows a significantly different clinical, angiographic and biochemical profile in <40 years patients with CHD compared with >or=40 years patients. Dominance of smoking and dyslipidemias that are the preventable risk factors in premature CHD patients is an important threat for our community health. Healthy life styles should be encouraged beginning from young ages and new precautions about smoking must be taken.

A rare cause of sudden vision loss in a young male: papillary fibroelastoma of the aortic valve.

Cardiac papillary fibroelastoma (CPF) is a rare cardiac neoplasm predominantly affecting the cardiac valves. Although it is most commonly an incidental finding, it can result in life-threatening complications, such as coronary and cerebral embolism, acute valvular dysfunction, and sudden death. In this report, we describe a case of a patient with aortic valve papillary fibroelastoma, which presented with sudden vision loss due to branch retinal artery occlusion. To the best of our knowledge, this is the third case of CPF-related retinal artery embolism in English literature.