Clinical experience with pheochromocytoma in a single centre over 16 years.

BACKGROUND: Pheochromocytoma and paraganglioma are rare tumours of neuroectodermal origin. Pheochromocytoma occurs in 0.1-2% of people with hypertension, while the incidence rises to 4-5% in patients with incidental adrenal mass. AIM: To analyse the presentation, diagnosis and localization, pathology, molecular genetic aspects, surgical management and long-term outcome of a large series of patients with pheochromocytoma referred to a single centre. METHODS: From 1992 to 2008, we observed 91 patients with pheochromocytoma, 70 (77%) with the sporadic form and 21 (23%) with the hereditary form. In the group with the hereditary form, the mean age at diagnosis was significantly lower than the mean age of the group with the sporadic form (38 vs 48 years; p < 0.001). RESULTS: Eighty (88%) patients with pheochromocytoma were symptomatic and the classical triad of palpitations, headache and diaphoresis was present in 30% of patients. In 12% of patients, the pheochromocytoma was discovered during radiological images (adrenal incidentaloma). The unilateral adrenal localization was observed in 78% of patients, bilateral in 15% and extra-adrenal in 7% of patients. All pheochromocytoma patients underwent surgical procedure and the laparotomic approach was used in 49 (53%) patients, while the laparoscopic approach was performed in 42 (47%) patients. Pheochromocytoma was benign in 86 patients (93%) [mean size was 4.3 cm] and the malignant form was found in five patients (7%) [mean size was 10 cm]. CONCLUSIONS: These data from a large cohort of patients are consistent with those reported in the literature and show that pheochromocytoma is an in important challenge for clinicians.

Biventricular assessment of light-chain amyloidosis using 3D speckle tracking echocardiography: Differentiation from other forms of myocardial hypertrophy.

BACKGROUND: Given that in patients with cardiac amyloidosis (CA), deposition of amyloid protein is not restricted to the left ventricular (LV) myocardium, it can be hypothesized that the diagnostic value of deformation mechanics would be enhanced by considering right ventricular (RV) strain measures. The aim of the present study was to examine the potential utility of left ventricular (LV) and right ventricular (RV) deformation and rotational parameters derived from three-dimensional speckle-tracking echocardiograph (3DSTE) to diagnose cardiac amyloidosis and differentiate this disease from other forms of myocardial hypertrophy. METHODS: Twenty-three patients with biopsy-proven light-chain (AL) amyloidosis, 23 patients with systemic arterial hypertension (HTN), 23 patients with hypertrophic cardiomyopathy (HCM), 23 athletes and 23 normal controls were prospectively studied by conventional echocardiography and 3DSTE. LV longitudinal strain (LV LS), LV circumferential strain (LV CS), RV global longitudinal strain and RV free-wall longitudinal strain (RV FW LS) were obtained by 3DSTE, as well as LV rotation and rotational velocities. RESULTS: LV and RV longitudinal strains were reduced in cardiac amyloidosis (CA) patients compared to controls. By multivariate analysis, LV basal LS (p = 0.002), LV peak basal rotation (p = 0.003), and RV basal FW LS (p = 0.014) were independently associated with CA in the overall population. A significant improvement in global χ2 value was noted with RV 3D-strain parameters over only LV-3DSTE + conventional indices for detection of CA (p < 0.001). Comparison of ROC curves showed that the AUC using combined LV basal LS, LV basal rotation and RV basal FW LS had a higher discriminative value than the other echocardiographic parameters used for detecting CA (AUC 0.93, 95%CI 0.81-0.97). CONCLUSIONS: Three-dimensional speckle tracking echocardiography reveals regional and global biventricular dysfunction in CA. Assessment of RV ventricular dysfunction has an additive value in differentiating CA from other causes of myocardial wall thickening.

Dataset on the use of 3D speckle tracking echocardiography in light-chain amyloidosis.

The dataset presented in this article is related to the research article entitled "Biventricular assessment of light-chain amyloidosis using 3D speckle tracking echocardiography: Differentiation from other forms of myocardial hypertrophy" (Vitarelli et al., 2018) [1], which examined the potential utility of left ventricular (LV) and right ventricular (RV) deformation and rotational parameters derived from three-dimensional speckle-tracking echocardiography (3DSTE) to diagnose cardiac amyloidosis(CA) and differentiate this disease from other forms of myocardial hypertrophy. The combined assessment of LV basal longitudinal strain, LV basal rotation and RV basal longitudinal strain had a high discriminative power for detecting CA. The data of this study provides more understanding on the value of LV 3DSTE deformation parameters as well as RV parameters in this particular cardiomyopathy.

Monascus purpureus for statin and ezetimibe intolerant heterozygous familial hypercholesterolaemia patients: A clinical study.

BACKGROUND: Hypercholesterolaemia is a major risk factor for cardiovascular disease and requires effective therapy in affected patients. Statins, the mainstay of lipid-lowering therapy, can cause side effects, including myalgia, in some patients. Ezetimibe, is frequently used as an add-on therapy for statins, and is also used as a monotherapy in statin-intolerant patients, however elevations in liver transaminases can occur. We examined the lipid-lowering efficacy of the natural fungal product Monascus purpureus (MP), which contains the natural statin monacolin K. METHODS: Fifty-five patients with familial hypercholesterolaemia who had discontinued statins due to muscle symptoms. Patients were placed on a lipid-lowering diet cholesterol-lowering diet (1500-1800 kcal daily, 30% lipids, 19% proteins and 52% carbohydrates). MP was added to the diet at a dose of 300 mg (providing monacolin K 10 mg). Patients were followed for 12 months. Lipid profiles and adverse event data were collected in the normal course of patient care. RESULTS: After 6 months of treatment with MP and diet therapy, statistically significant changes in low-density lipoprotein cholesterol were evident (-17% for males, -16% for females; p < 0.005) Levels fell to -24% and -27% respectively at 12 months. No patients experienced elevated serum aminotransferases or C-reactive protein levels. CONCLUSIONS: MP is a viable option for lipid-lowering therapy in statin-intolerant patients with hypercholesterolaemia, with good efficacy and safety profiles.

Diagnostic accuracy of laser-evoked potentials in diabetic neuropathy.

Although the most widely agreed neurophysiological tool for investigating small fiber damage is laser-evoked potential (LEP) recording, no study has documented its diagnostic accuracy. In this clinical, neurophysiological, and skin biopsy study, we collected age-corrected LEP normative ranges, verified the association of LEPs with pinprick sensory disturbances in the typical diabetic mixed fiber polyneuropathy, and assessed the sensitivity and specificity of LEPs in diabetic small fiber neuropathy. From 288 LEP recordings from the face, hand, and foot in 73 healthy subjects, we collected age-corrected normative ranges for LEPs. We then selected 100 patients with mixed-fiber diabetic neuropathy and 25 patients with possible small-fiber diabetic neuropathy. In the 100 patients with mixed fiber neuropathy, we verified how LEP abnormalities were associated with clinically evident pinprick sensory disturbances. In the 25 patients with possible pure small fiber neuropathy, using the skin biopsy for assessing the intraepidermal nerve fiber density as a reference standard, we calculated LEP sensitivity and specificity. In healthy participants, age strongly influenced normative ranges for all LEP variables. By applying age-corrected normative ranges for LEPs, we found that LEPs were strongly associated with pinprick sensory disturbances. In relation to the skin biopsy findings, LEPs yielded 78% sensitivity and 81% specificity in the diagnosis of diabetic small fiber neuropathy. Our study, providing age-corrected normative ranges for the main LEP data and their diagnostic accuracy, helps to make LEPs more reliable as a clinical diagnostic tool, and proposes this technique as a less invasive alternative to skin biopsy for diagnosing diabetic small fiber neuropathy.

Assessment of Right Ventricular Function in Obstructive Sleep Apnea Syndrome and Effects of Continuous Positive Airway Pressure Therapy: A Pilot Study.

BACKGROUND: It is known that obstructive sleep apnea syndrome (OSAS) can affect right ventricular (RV) performance even in the absence of systemic hypertension and other known cardiac or obstructive pulmonary disease. The purpose of the present study was to assess RV function in OSAS using 3-D echocardiography and speckle tracking echocardiography (STE) and evaluate changes after continuous positive airway pressure (CPAP) treatment. METHODS: Thirty-seven patients with OSAS without comorbidities and thirty control subjects were studied using 3-D echocardiography and STE. Fifteen patients underwent CPAP therapy and were studied before and after treatment. RV 3-D ejection fraction was calculated. Peak systolic strain was determined. RV dyssynchrony was defined as SD of the 6 time to peak systolic strain values. RESULTS: 3-D RV ejection fraction was lower and RV dyssynchrony was greater in patients with moderate-severe OSAS compared with control subjects in the presence and absence of pulmonary hypertension. 3-D RV ejection fraction and RV dyssynchrony were independently associated with apnea-hypopnea index. Patients treated with CPAP had significant changes in RV parameters. CONCLUSIONS: 3-D RV ejection fraction and RV dyssynchrony were abnormal in OSAS patients compared with control subjects and associated with OSAS severity. RV 3-D STE abnormalities improved after chronic application of CPAP.

Comprehensive assessment of biventricular function and aortic stiffness in athletes with different forms of training by three-dimensional echocardiography and strain imaging.

AIMS: Previous studies have shown distinct models of cardiac adaptations to the training in master athletes and different effects of endurance and strength-training on cardiovascular function. We attempted to assess left-ventricular (LV) function, aortic (Ao) function, and right-ventricular (RV) function in athletes with different forms of training by using three-dimensional (3D) echocardiography, tissue Doppler imaging (TDI) and speckle-tracking imaging (STI). METHODS AND RESULTS: We examined 35 male marathon runners (endurance-trained athletes, ETA), 35 powerlifting athletes (strength-trained athletes, STA), 35 martial arts athletes (mixed-trained athletes, MTA), and 35 sedentary untrained healthy men (controls, CTR). Two-dimensional and three-dimensional echocardiography were performed for the assessment of LV and RV systolic/diastolic function. LV and RV longitudinal strain (LS) and LV torsion (LVtor) were determined using STI (EchoPAC BT11, GE-Ultrasound). Maximum velocity of systolic wall expansion peaks (AoSvel) was determined using TDI. ETA experienced LV eccentric hypertrophy with increased 3D LV end-diastolic volume and mass and significant increase in peak systolic apical rotation and LVtor. In all groups of athletes, RV-LS was reduced at rest and improved after exercise. AoSvel was significantly increased in ETA and MTA and significantly decreased in STA compared with CTR. There were good correlations between LV remodelling and aortic stiffness values. Multivariate analysis showed aortic wall velocities to be independently related to LV mass index. CONCLUSION: In strength-trained, endurance-trained, and mixed-trained athletes, ventricular and vascular response assessed by 3DE, TDI, and STI underlies different adaptations of LV, RV, and aortic indexes.

Adipokines and cardiometabolic profile in primary hyperaldosteronism.

CONTEXT: Primary aldosteronism (PA) has been recently associated with an unfavorable cardiometabolic profile. However, whether pro- and antiinflammatory adipokines levels can vary in PA is unknown. OBJECTIVE: We evaluated the circulating levels of resistin, leptin, and adiponectin, echocardiographic left ventricle (LV) parameters, and the prevalence of metabolic syndrome (SM) in subjects with PA. PATIENTS: Seventy-five subjects with established diagnosis of PA and 232 consecutive individuals with known or suspected hypertension were enrolled. MAIN OUTCOME MEASURES: Plasma adipokine levels and echocardiographic parameters were calculated. Prevalence of SM was also estimated. RESULTS: Among the 75 PA subjects, 37 patients were affected by aldosterone-producing adenoma and 38 by idiopathic hyperaldosteronism; 40 subjects were affected by essential hypertension (EH) and SM (EH SM+); 152 subjects were affected by EH without SM (EH SM-); and 40 subjects were normotensive (NT). Subjects with PA had the highest plasma resistin levels among the four groups (P < 0.01). Plasma resistin concentration was significantly higher in PA subjects when compared with EH SM+ individuals (P < 0.01) and EH SM- subjects (P < 0.01). PA subjects showed the higher LV mass and left atrium than EH individuals, irrespectively of the presence of SM (P < 0.01 for both). Plasma resistin levels was significantly correlated with ejection fraction and LV end-diastolic volume. The prevalence of SM was higher in PA subjects than in those with EH (25.4 vs. 20.3%). CONCLUSIONS: Our data suggest that elevated aldosterone levels is associated with elevated circulating resistin levels and cardiac morphological changes independently of the presence of SM.

Assessment of ascending aorta wall stiffness in hypertensive patients by tissue Doppler imaging and strain Doppler echocardiography.

BACKGROUND: Aortic stiffness may be associated with an increased incidence of cardiovascular events and has been reported to be related to arterial wall motion velocities as measured by tissue Doppler imaging. OBJECTIVE: To investigate the potential clinical application of tissue Doppler imaging (TDI) for assessment of aortic function parameters in healthy and hypertensive adults. METHODS: 110 hypertensive and 80 healthy adults were examined. Pulse wave velocity (PWV) and augmentation index (Aix) were measured as standard parameters of arterial stiffness by an oscillometric system. Aortic M-mode and TDI parameters were measured. Aortic distensibility (D) and aortic stiffness index (SI) were calculated using accepted formulae. Anterior wall aortic expansion velocity (S(Ao)), acceleration time (AT(Ao)), early (E(Ao)) and late (A(Ao)) diastolic retraction velocity and peak systolic radial strain (epsilon(Ao)) were determined. Comprehensive echocardiography was performed for the assessment of left ventricular (LV) systolic/diastolic function. RESULTS: S(Ao), E(Ao) and e(Ao) were significantly lower in hypertensive subjects (p<.001, p<.001, and p<.0001, respectively). Reduced D (p<.05 vs controls) and increased PWV (p<.05 vs controls) and SI (p<.01 vs controls) were consistent with evidence of increased aortic stiffness in both male and female hypertensive patients. PWV and Aix increased and D decreased with increasing age or systolic blood pressure. Multivariate analysis showed epsilon(Ao) to be independently related (R2 = 0.63) to pulse pressure, LV mass index and diastolic function. CONCLUSION: Ascending aorta TDI provides wall velocity and strain data differentiating hypertensive from healthy adults and reflecting aortic compliance changes related to age and sex and LV diastolic function.