Prevalence and management of familial hypercholesterolemia in patients with coronary artery disease: The heredity survey.

BACKGROUND AND AIMS: Familial hypercholesterolemia (FH) is a genetic disorder characterized by high levels of low density lipoprotein cholesterol (LDL-C) predisposing to premature cardiovascular disease. Its prevalence varies and has been estimated around 1 in 200-500. The Heredity survey evaluated the prevalence of potential FH and the therapeutic approaches among patients with established coronary artery disease (CAD) or peripheral artery disease (PAD) in which it is less well documented. METHODS: Data were collected in patients admitted to programs of rehabilitation and secondary prevention in Italy. Potential FH was estimated using Dutch Lipid Clinic Network (DLCN) criteria. Potential FH was defined as having a total score≥6. RESULTS: Among the 1438 consecutive patients evaluated, the prevalence of potential FH was 3.7%. The prevalence was inversely related to age, with a putative prevalence of 1:10 in those with <55yrs of age (male) and <60yrs (female). Definite FH (DLCN score>8) had the highest percentages of patients after an ACS (75% vs 52.5% in the whole study population). At discharge, most patients were on high intensity statin therapy, but despite this, potential FH group still had a higher percentage of patients with LDL-C levels not at target and having a distance from the target higher than 50%. CONCLUSIONS: Among patients with established coronary heart disease, the prevalence of potential FH is higher than in the general population; the results suggest that a correct identification of potential FH, especially in younger patients, may help to better manage their high cardiovascular risk.

ANMCO Position Paper: long-term follow-up of patients with pulmonary thromboembolism.

Venous thromboembolism (VTE), including pulmonary embolism and deep venous thrombosis, is the third most common cause of cardiovascular death. The management of the acute phase of VTE has already been described in several guidelines. However, the management of the follow-up (FU) of these patients has been poorly defined. This consensus document, created by the Italian cardiologists, wants to clarify this issue using the currently available evidence in VTE. Clinical and instrumental data acquired during the acute phase of the disease are the cornerstone for planning the FU. Acquired or congenital thrombophilic disorders could be identified in apparently unprovoked VTE during the FU. In other cases, an occult cancer could be discovered after a VTE. The main targets of the post-acute management are to prevent recurrence of VTE and to identify the patients who can develop a chronic thromboembolic pulmonary hypertension. Knowledge of pathophysiology and therapeutic approaches is fundamental to decide the most appropriate long-term treatment. Moreover, prognostic stratification during the FU should be constantly updated on the basis of the new evidence acquired. Currently, the cornerstone of VTE treatment is represented by both the oral and the parenteral anticoagulation. Novel oral anticoagulants should be an interesting alternative in the long-term treatment.

[A rare cardiomegaly]. / Una rara cardiomegalia.

We present the case of a patient who came to our attention for enlargement of the cardiac silhouette on chest X-ray. Echocardiography showed moderate diastolic overload of both ventricles with enhanced cardiac output without valvular disease or cardiac shunt that could account for this cardiomegaly. A subsequent abdominal echocardiographic exploration showed an angiomatous transformation of the liver due to diffuse lacunar enlargement of hepatic portal vein branches and arterial-venous shunts. Computed tomography and magnetic resonance imaging confirmed the echocardiographic findings. The imaging findings coupled with cutaneous and nasopharyngeal lesions were suggestive of hereditary hemorrhagic telangiectasia (HHT) and the diagnosis was confirmed after the identification of a mutation in the ACVRL1 gene on chromosome 12. HHT is a rare but underestimated vascular disease that can affect different organs, in particular the liver, leading to organ failure requiring transplantation as occurred in our patient. Echocardiography is a useful imaging tool to exclude cardiac abnormalities as a cause of cardiomegaly and to guide the correct diagnosis of a peripheral origin of high cardiac output.

[ANMCO Position paper: Recommendations for the follow-up of patients with pulmonary thromboembolism]. / Position paper ANMCO: Raccomandazioni per il follow-up del paziente con tromboembolia polmonare.

Venous thromboembolism (VTE), including deep venous thrombosis and pulmonary embolism, is the third most common cause of cardiovascular death. The management of the acute phase of VTE is well described in several papers and guidelines, whereas the management of the follow-up of the patients affected from VTE is less defined. This position paper of the Italian Association of Hospital Cardiologists (ANMCO) tries to fill the gap using currently available evidence and the opinion of the experts to suggest the most useful way to manage patients in the chronic phase.The clinical and laboratory tests acquired during the acute phase of the disease drives the decision of the following period. Acquired or congenital thrombophilic factors may be identified to explain an apparently not provoked VTE. In some patients, a not yet clinically evident cancer could be the trigger of VTE and this could lead to a different strategy. The main target of the post-acute management is to prevent relapse of the disease and to identify those patients who could worsen or develop chronic thromboembolic pulmonary hypertension. The knowledge of the etiopathogenetic ground is important to address the therapeutic approach, choosing the best antithrombotic strategy and deciding how long therapy should last. During the follow-up period, prognostic stratification should be updated on the basis of new evidences eventually acquired.Treatment of VTE is mainly based on oral or parenteral anticoagulation. Oral direct inhibitors of coagulation represent an interesting new therapy for the acute and extended period of treatment.

Depression is associated with increased vagal withdrawal during unpleasant emotional imagery after cardiac surgery.

BACKGROUND: The aim of this study was to examine the influence of depression on heart rate and heart rate variability (HRV) during emotional imagery in patients after cardiac surgery. METHODS: Based on the scores of the Center for Epidemiological Studies of Depression (CES-D) scale, 28 patients after cardiac surgery were assigned either to the group with depression (CES-D scores ≥ 16; N = 14) or the one without depression (CES-D scores<16; N = 14). Each patient completed a rest period and an emotional imagery including pleasant, neutral and unpleasant scripts. Inter-beat intervals (IBIs) and HRV were measured during the entire protocol. RESULTS: Compared to nondepressed patients, those with depression had greater reductions in high frequency expressed in normalized units (HF n.u.) during the imaging of the unpleasant script (p = .003, Cohen's d = 1.34). Moreover, HF n.u. were lower during the imaging of the unpleasant script than the pleasant one in depressed patients only (p = .020, Cohen's d = 0.55). CES-D scores were also inversely correlated with residualized changes in IBIs (r = -.38, p = .045) and HF n.u. (r = -.49, p = .008) from rest to the imaging of the unpleasant script. CONCLUSIONS: The relationship between depression and increased vagal withdrawal during unpleasant emotional imagery extends to patients after cardiac surgery. The present study suggests that increased vagal withdrawal to negative emotions in patients after cardiac surgery may mediate the conferral of cardiac risk by depression.

[Diagnosis and therapy of pulmonary arterial hypertension in Italy: results of the INCIPIT2 survey]. / Diagnosi e terapia dell'ipertensione arteriosa polmonare nelle Cardiologie italiane: risultati dell'indagine conoscitiva INCIPIT2.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare clinical condition characterized by increased pulmonary vascular resistance and premature death. It is necessary to activate a pathway from the screening of pulmonary hypertension to the diagnosis of PAH, so as to use the drugs able to improve the outcome. Nowadays, in Italy, there are no data about the management of PAH in peripheral centers and about the integration of peripheral centers with those of excellence. METHODS: In order to have a map of the actual Italian pathway for diagnosis and therapy of PAH, on behalf of the ANMCO Pulmonary Circulation Area, 923 Italian cardiology departments were asked to reply, on a special electronic file, to a few simple questions about their organization, from December 2012 to May 2013. RESULTS: 101/923 centers (48 in the North, 18 in the Middle, 35 in the South) answered correctly. 32% has no organization for PAH, 68% has a pathway for PAH diagnosis and management, and two thirds of them collaborate with excellence centers. 36 centers perform right heart catheterization with vascular reactivity (21 with nitric oxide, 8 with adenosine, 5 with epoprostenol, 2 with nitric oxide or epoprostenol). 61/101 are prescriber centers: 33 perform right heart catheterization with vascular reactivity test, 23 send their patients to the reference center for right heart catheterization, 5 perform no right heart catheterization before the prescription of specific drugs for PAH, and only 14 prescribe intravenous prostanoids. In 2011, the participating centers followed 561 patients with PAH, of whom 126 (23%) were in independent centers. With regard to the network organization of the groups, the participating centers are partly independent of the diagnostic pathway, partly refer to outside regions; in others there is a structured regional network and there are 3 Italian regions with Hub & Spoke networks that receive patients coming from other regions. CONCLUSIONS: Our results show the interest of Italian Cardiology to find a pathway for the diagnosis of PAH and a heterogeneity suggesting the need for a shareable pathway, thus improving the collaboration between peripheral cardiology departments and the excellence centers for PAH in a functional Hub & Spoke network.

Depression and reduced heart rate variability after cardiac surgery: the mediating role of emotion regulation.

BACKGROUND: Heart rate variability (HRV), as an index of autonomic nervous system (ANS) functioning, is reduced by depression after cardiac surgery, but the underlying mechanisms of this relationship are poorly understood. Poor emotion regulation as a core symptom of depression has also been associated with altered ANS functioning. The present study aimed to examine whether emotion dysregulation could be a mediator of the depression-reduced HRV relationship observed after cardiac surgery. METHODS: Self-reported emotion regulation and four-minute HRV were measured in 25 depressed and 43 nondepressed patients after cardiac surgery. Mediation analysis was conducted to evaluate emotion regulation as a mediator of the depression-reduced HRV relationship. RESULTS: Compared to nondepressed patients, those with depression showed lower standard deviation of normal-to-normal (NN) intervals (p<.05), root mean square successive difference of NN intervals (p<.004), and number of interval differences of successive NN intervals greater than 50ms (NN50) (p<.05). Increased low frequency (LF) in normalized units (n.u.) and reduced high frequency (HF) n.u. were also found in depressed compared to nondepressed patients (p's<.01). Mediation analysis revealed that suppression of emotion-expressive behavior partially mediated the effect of depression on LF n.u. and HF n.u. CONCLUSIONS: Results confirmed previous findings showing that depression is associated with reduced HRV, especially a reduced vagal tone and a sympathovagal imbalance, after cardiac surgery. This study also provides preliminary evidence that increased trait levels of suppression of emotion-expressive behavior may mediate the depression-related sympathovagal imbalance after cardiac surgery.

Role of Cardiovascular Ultrasound in the Evaluation of Obese Subjects.

Obesity represents a worldwide increasing health problem. Obesity, through complex and not fully understood pathogenetic mechanisms, induces different structural and functional changes of left heart chambers, right heart chambers, and arteries. Ultrasound techniques are the first choice for a comprehensive assessment of the cardiovascular adaptation to obesity. This review summarizes the up-to-date literature on the topic, with particular focus on the main clinical studies, which range over different cardiovascular adaptations to obesity, namely left ventricular mass, diastolic function, right ventricle structure and function, arterial stiffness, and intima-media thickness. Also, the importance of epicardial fat and of the degree of obesity is described. Finally, the role of weight loss and bariatric surgery and the study of cardiovascular obesity-induced abnormalities in children and adolescent are discussed.

Lower extremities peripheral arterial disease among patients admitted to cardiac rehabilitation: the THINKPAD registry.

BACKGROUND/OBJECTIVES: Lower extremities peripheral arterial disease (LE-PAD) across the wide range of conditions for Cardiac Rehabilitation (CR) is poorly understood. The "ATHerosclerosis of the lower extremIties as a liNKed comorbidity in Patients Admitted for carDiac rehabilitation" (THINKPAD) registry explored LE-PAD in CR patients in terms of prevalence and interventions delivered. METHODS: Multicenter, consecutive case series of 1506 patients discharged from 16 CR Units in Italy from May 1 to June 30, 2012. RESULTS: LE-PAD constituted a primary indication for CR and a comorbidity on admission in 2.6% and 9.3% of patients respectively. LE-PAD patients were significantly older (72 ± 9 vs. 67 ± 12 years, p<0.001) and displayed a worse cardiovascular risk profile (diabetes 38% vs. 23%, hypertension 86% vs. 63%, hypercholesterolemia 74% vs. 52%, smoking 72% vs. 50%, low level of physical activity 84% vs. 69%, impaired diet habits 69% vs. 55%, p<0.01 for all). COPD (17% vs. 11%, p<0.05), CKD (20% vs. 10%, p<0.01), and past history of coronary revascularization (29% vs. 14%, p<0.001) were also more represented in the LE-PAD group. Half of LE-PAD patients received a formal staging, with low provision of ABI (18%) and color Doppler (48%) investigation. Secondary prevention targets at the end of CR for blood pressure and lipid control were accomplished in 83% and 46% of patients respectively (strongly correlated with the presence of CAD), while other guideline-recommended drugs for LE-PAD were prescribed in less than 4% of cases. CONCLUSION: LE-PAD represents an uncommon referral indication for CR. Our data confirm its systematic underassessment and undertreatment.

Biofeedback assisted control of respiratory sinus arrhythmia as a biobehavioral intervention for depressive symptoms in patients after cardiac surgery: a preliminary study.

The current study investigated whether biofeedback training aimed at increasing respiratory sinus arrhythmia (RSA), a measure of cardiac vagal modulation, can reduce depressive symptoms in patients after cardiac surgery. This randomized controlled study enrolled 26 patients after first-time cardiac surgery. The patients were randomly assigned to an RSA-biofeedback group (N = 13) or to a treatment as usual group (N = 13). The biofeedback training consisted of five 45 min sessions designed to increase RSA. The outcome was assessed as changes in RSA and in the Centre for Epidemiologic Studies of Depression (CES-D) values from pre- to post-training. Both groups were comparable for demographic and biomedical characteristics. RSA increased significantly in patients who underwent RSA-biofeedback compared to controls. Moreover, the CES-D scores were reduced significantly from pre- to post-training in the RSA-biofeedback group compared to the controls. Changes in RSA were inversely related to changes in CES-D scores from pre- to post-training. These findings extend the effectiveness of RSA-biofeedback for increasing vagal modulation as well as for reducing depressive symptoms in post-surgical patients. Overall, the current study also suggests that this biobehavioral intervention may add to the efficacy of postoperative risk reduction programs and rehabilitation protocols in cardiac surgery patients.

Clinical features and short term outcomes of patients with acute pulmonary embolism. The Italian Pulmonary Embolism Registry (IPER).

BACKGROUND: Registries are essential to obtain information on the whole spectrum of patients with pulmonary embolism (PE). The aim of the Italian Pulmonary Embolism Registry (IPER) is to report on demographics, clinical features, management, and outcomes of patients diagnosed with PE in everyday clinical practice. METHODS: Patients with confirmed acute PE were enrolled in a web-based registry, in Cardiology, Emergency or Internal Medicine Departments in 47 hospitals in Italy. RESULTS: Overall, 1716 patients were included, mean age 70 ± 15 years, (14% of the patients were <50 and 43% >75 year old); 57% of female gender and 11.7% hemodynamically unstable at presentation/diagnosis. D-dimer was performed in 1358 patients (80%). Computerized tomographic pulmonary angiogram (CT) was used for diagnosis in the majority of the patients (82.1%), followed by perfusion lung scan (8.6%). Thrombolytic agents were used in 185 (10.8%) patients, percutaneous thrombectomy in 14 (0.8%) and surgery in 2 (0.1%). One hundred sixteen patients died while in-hospital (6.7%), 68 (3.9%) due to PE. Death or clinical deterioration occurred in 138 patients (8.0%). All-cause mortality was 31.8% in hemodynamically unstable patients and 3.4% in hemodynamically stable patients; the corresponding PE-related deaths were 23.3% and 1.4% respectively. Age >75 (HR 1.50, 95% CI 1.01-2.25), immobilization > 3 days before diagnosis of PE (HR 2.54, 95% CI 1.72-3.77) and hemodynamic impairment (HR 6.38, 95% CI 4.26-9.57) were independent predictors for in-hospital death. CONCLUSIONS: Patients with PE have a considerable risk of death during the hospital stay, PE being the most common cause of early mortality.

Association between depression and heart rate variability in patients after cardiac surgery: a pilot study.

OBJECTIVE: Depression is a risk factor for cardiovascular diseases. Reduced heart rate variability (HRV), which reflects altered autonomic nervous system activity, has been suggested as one of the mechanisms linking depression to cardiovascular diseases. However, the relationship between depression and HRV has not yet been investigated in patients undergone cardiac surgery. Therefore, the main aim of this study was to examine whether postoperative depression could be related to reduced HRV. METHODS: Eleven patients with depression and 22 patients without depression, who had undergone cardiac surgery, were enrolled postoperatively. In all patients, HRV was derived from a four-minute blood volume pulse recording at rest. Analyses of covariance and partial correlations, while controlling for anxiety, were used to examine the associations between postoperative depression and each HRV parameter. RESULTS: Compared to non-depressed patients, patients with depression showed significantly lower standard deviation of N-to-N intervals (SDNN) (p=.02), root mean square successive difference of N-to-N intervals (rMSSD) (p=.001), and high-frequency power (p=.002). Partial correlation analyses showed that depression was inversely related to SDNN (r=-.49, p=.005), rMSSD (r=-.58, p=.001), and high-frequency power (r=-.41, p=.02), whereas it was unrelated to other HRV parameters (p's>.09). CONCLUSIONS: The current findings extend the depression-reduced HRV relationship to the patients after cardiac surgery. Also, our study suggests that postoperative depression is more likely to be associated with reduced vagal modulation on the heart than with excessive sympathetic activity.

Gender differences in cardiac rehabilitation programs from the Italian survey on cardiac rehabilitation (ISYDE-2008).

PURPOSE: In recent years epidemiological and clinical evidence has shown gender disparities in several aspects of cardiovascular disease. Aim of this study was to identify gender differences in the clinical profile and management of patients admitted to cardiac rehabilitation (CR) programs. POPULATION: Patients enrolled in the ISYDE-2008 survey were considered. RESULTS: The ISYDE-2008 survey enrolled 2281 patients; 604 (26.5%) were women. Compared to men, women were older (mean age 70.8 ± 11.5 versus men's 65.6 ± 11.5 years), had less traditional risk factors (low cardiovascular risk profile in 45.3% of women and 38.0% of men, p=0.003), were more frequently admitted after valvular surgery and heart failure, but less for post-acute myocardial infarction and post-by-pass procedure. Women were more frequently admitted to an in-hospital rehabilitation program. Women showed a more complicated acute and rehabilitative course, with 63.2% of them having at least one complication during acute-phase, compared to 52.5% of men, and 48.3% during rehabilitation, compared to 35.0% of men (p<0.0001). During rehabilitation, women underwent exercise tests less frequently, except for the 6-minute walking test. At discharge, women received ACE-inhibitors/ARBs, ß-blockers, statins, omega-3 fatty acids, antiplatelet agents less frequently, but more frequently digoxin, amiodarone, diuretics, oral anticoagulants, insulin and anti-depressive drugs. The duration of the rehabilitation program was longer for women. Mortality was very low in the entire population. CONCLUSIONS: Women are less frequently admitted to CR than men. They are older and show a greater cardiovascular burden. Women are more likely to be enrolled in CR after valvular surgery and heart failure than men.

[Chronic thromboembolic pulmonary hypertension]. / Ipertensione polmonare cronica tromboembolica.

Chronic thromboembolic pulmonary hypertension is due to unresolved or recurrent pulmonary embolism. In the United States the estimated prevalence is 0.1-0.5% among survived patients with pulmonary embolism. The survival rate at 5 years was 30% among patients with a mean pulmonary artery pressure > 40 mmHg at the time of diagnosis and only 10% among those with a value > 50 mmHg. The interval between the onset of disturbances and the diagnosis may be as long as 3 years. Doppler echocardiography permits to establish the diagnosis of pulmonary hypertension. Radionuclide scanning determines whether pulmonary hypertension has a thromboembolic basis. Right heart catheterization and pulmonary angiography are performed in order to establish the extension and the accessibility to surgery of thrombi and to rule out other causes. The surgical treatment is thromboendarterectomy. A dramatic reduction in the pulmonary vascular resistance can be achieved; corresponding improvements in the NYHA class--from class III or IV before surgery to class I-II after surgery--are usually observed. Patients who are not considered candidates for thromboendarterectomy may be considered candidates for lung transplantation.