Franco Casazza

The varying evolution of friedreich's ataxia cardiomyopathy

During a mean follow-up period of 8 years, 17% of 66 patients with Friedreichs ataxia developed hypokinetic-dilated cardiomyopathy; most patients originally had a hypertrophic left ventricle. The presence of pathologic Q waves identifies a subgroup of patients with wall motion abnormalities; these patients are more likely to develop a hypokinetic left ventricle, and the prognosis is ostensibly poorer.

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.

Acute Pulmonary Embolism: External Validation of an Integrated Risk Stratification Model

BACKGROUND In hemodynamically stable patients with acute pulmonary embolism, risk stratification is essential to drive clinical management. In these patients, risk stratification for in-hospital adverse outcomes based on markers of right ventricular dysfunction and injury has been proposed. METHODS The aim of this study was to validate a model based on the incremental prognostic value of right ventricular dysfunction and injury in hemodynamically stable patients with acute pulmonary embolism. Patients from the prospective Italian Pulmonary Embolism Registry were included in the study. Study outcomes were in-hospital death and the composite of in-hospital death or clinical deterioration. RESULTS Among 1,515 hemodynamically stable patients, 869 had both echocardiography and troponin assessments. The risk for in-hospital death or clinical deterioration was higher in patients with right ventricular dysfunction and elevated troponin level (8.8%; hazard ratio [HR], 14.2 [95% CI, 1.94-104.16]; P < .01) and with either right ventricular dysfunction or elevated troponin level (4.7%; HR, 7.9 [95% CI, 1.1-59.9]; P < .05) compared with patients without dysfunction and normal troponin levels. The negative predictive value of the model was 100% for in-hospital death and 99% for death or clinical deterioration. C statistics showed an improvement of the discriminatory power for in-hospital death or clinical deterioration by using the overall model (0.66; 95% CI, 0.60-0.73) over either echocardiography (0.59; 95% CI, 0.53-0.67) or troponin level (0.61; 95% CI, 0.53-0.69) alone. CONCLUSIONS A model that includes both dysfunction and injury of the right ventricle has an incremental prognostic value for risk stratification in hemodynamically stable patients with acute pulmonary embolism. Patients with no dysfunction or injury have a favorable outcome. TRIAL REGISTRY ClinicalTrials.gov; No.: NCT01604538; URL: www.clinicaltrials.gov.

Prognostic significance of free-floating right heart thromboemboli in acute pulmonary embolism: results from the Italian Pulmonary Embolism Registry.

The exact prevalence of mobile right heart thromboemboli (RHTh) in patients with pulmonary embolism (PE) is unknown, depending upon PE severity and the use of early echocardiography. Similarly, the mortality rate is variable, though RHTh detection appears to substantially increase the risk of death in patients with PE. The aim of this study was to assess the prevalence of RHTh in different risk categories in a wide series of patients with PE, and to analyse the effect of RHTh on in-hospital mortality. Among 1,716 patients enrolled in the Italian Pulmonary Embolism Registry, 1,275 (13.3% at high risk, 59.3% at intermediate risk and 27.4% at low risk) had echocardiography within 48 hours from hospital admission and entered the study. Overall, RHTh were detected in 57 patients (4.5%, at admission echocardiography in 88%): in 27/169 (16%) high-risk, in 29/756 (3.8%) intermediate-risk and 1/350 (0.3%) low-risk patients, respectively. At multivariate analysis, only advanced age (odds ratio [OR] 1.61, 95% confidence [CI] 1.27-2.03, p<0.0001), high-risk category (OR vs low-risk category 37.82, 95% CI 11.26-127.06, p<0.0001) and recurrent PE (OR 45.92, 95%CI 15.19-139.96, p<0.0001) showed a statistically significant effect on mortality. The presence of RHTh significantly increased the risk of dying (OR 3.89, 95%CI 1.98-7.67, p=0.0001) at univariate analysis, but this result was not mantained in the multivariate model (OR 1.64, 95%CI 0.75-3.60, p=0.216). In conclusion, though patients with RHTh had a more severe presentation of PE, this study did not detect an association between RHTh and prognosis.

An international survey on isolated subsegmental pulmonary embolism.

The large and growing number of radiological examinations performed for several thoracic diseases with multidetector-row CT (MDCT) scanners are leading to a marked increase in newly diagnosed isolated (i.e. without a concomitant diagnosis of deep venous thrombosis) subsegmental PE (I-SSPE), often incidentally identified. A diagnosis of SSPE may account for up to 12% of positive CT scans performed for suspected PE, whereas the proportion of incidental PE in patients undergoing chest CT varies from 1.5% to over 4% [1–3]. Whether MDCT findings of SSPE show true emboli or something else (artifacts?in situ thrombosis?) is still an unresolved issue and studies directly comparing the findings of the most recent MDCT scanners with those of pulmonary angiography are lacking. However, a number of indirect criteria could help radiologists to exclude false positive diagnoses (e.g. lack of concomitant atelectasis, pleural effusion or interstitial disease). The prognostic value of these findings is still undefined, especially in asymptomatic patients, and evenmore so it is unclear whether affected patients should be treated, with which drugs, at what dosage and how long. As the achievement of high-quality randomized clinical trials on I-SSPE is likely to be unrealistic, we decided to explore this topic through an inquiry on the current perception of the problem among physicians practicing in several European countries.We submitted a questionnaire to attendees of medical conferences and members of both Italian thrombosis research program networks and of the Registro Informatizado de Pacientes con Enfermedad Tromboembolica (RIETE), an international registry on venous thromboembolism (VTE) collecting data from 14 countries. The questionnaire was a list of multiple-choice questions with additional freely fillable lines of text if more detailed comments were needed, without any presentation of clinical cases and inquiring whether an I-SSPE requires treatment, which factors should influence the clinical decisions, which drugs should be used, at what dosage and how long, whether the identification of I-SSPE should prompt the search for a thrombophilic defect and/or an underlying malignancy. The definition adopted of ISPPE was that of a diagnosis, by MDCT scanning and confirmed by the radiologist, of a single ormultiple pulmonary sub-segmental emboli not associated with the concomitant presence of both segmental emboli and thrombosis in the peripheral venous circulation, identified primarily but not exclusively by an ultrasound examination. The survey focused on the symptomatic or asymptomatic clinical presentations of I-SPPE episodes and the incidental nature of some episodes could have been considered by physicians among the factors that influenced their clinical decisions. The survey was conducted either on-line, on a dedicated internet portal of our Department or manually on paper forms; several invitations to complete the on-line questionnaire were sent out via e-mail and paper copies of the questionnaire were distributed among attendees of major scientific

Trattamento percutaneo dell'embolia polmonare: diffusione e utilizzo della metodica in centri cardiologici interventistici italiani. Risultati dell'indagine conoscitiva PETER

BACKGROUND Reperfusion in acute pulmonary embolism (PE) by percutaneous techniques is a valid therapeutic option when there is a formal contraindication to or failure of thrombolysis. In the last years, an increasing number of patients with acute PE have been treated with these techniques. METHODS In order to obtain a map of current availability and use of percutaneous techniques in PE, on behalf of the ANMCO Pulmonary Circulation Area, 56 Italian interventional cardiology and radiology departments, equipped with technology for percutaneous embolectomy, were invited to participate in a national survey. Questionnaires were e-mailed to each department from April to May 2015. RESULTS Thirty-one out of 56 centers (54.8% in the North, 9.7% in the Center and 35.5% in the South of Italy) answered to the questionnaire. Percutaneous techniques were available in 90% of the cardiology departments involved, reporting also a good experience with their use in PE (77.4%). Only two responders were interventional radiology departments. AngioJet(®) and EkoSonic Endovascular System(®) (64.7% and 19.4%, respectively) were the most common devices used. Overall, in 2014, 62 patients were treated with percutaneous techniques, mainly in the North of the country. With regard to local diagnostic and therapeutic protocols, 61.3% of respondents reported owning one. Great interest was provided by participants in adhering to this national multicenter registry. CONCLUSIONS Our results show the interest of Italian cardiology departments about percutaneous techniques as a therapeutic option for acute PE. Percutaneous techniques are largely available but still underused in routine clinical practice.

Fulminant Pulmonary Embolism Successfully Treated with Thrombolysis

Fulminant Pulmonary Embolism Successfully Treated with Thrombolysis A 70 year-old man, with a history of major depression and arterial hypertension, was admitted to the emergency department for severe hypotension and suspected abuse of anti anxiety drugs. Due to the rapid deterioration of his clinical conditions a bed-side echocardiography was rapidly performed,that was compatible with acute pulmonary embolism. During echocardiography, the patient developed a pulseless electrical activity with residual cardiac contractility (pseudo PEA) that evolved into pulseless electrical activity without cardiac contractility (true PEA). After 20 minutes of cardiopulmonary resuscitation (CPR), empirical alteplase 50 mg + 50 mg was administered intravenously over 5 minutes, with restoration of spontaneous circulation. PE was confirmed by computed tomographic angiography. His hospital stay was complicated by non-hemorrhagic neurological impairment that resulted in quadriplegia.

Impact of syncope and pre-syncope on short-term mortality in patients with acute pulmonary embolism.

BACKGROUND Syncope and pre-syncope are well-known symptoms of acute pulmonary embolism (PE). However, data about their impact on short-term mortality are scant. We assess the short-term mortality (30-day) for all-causes in PE patients admitted with syncope or with pre-syncope, according their hemodynamic status at admission. METHODS Patients from the prospective Italian Pulmonary Embolism Registry (IPER) were included in the study. At admission, subjects were stratified according to 2008 ESC guidelines (as high- and non-high-risk patients). RESULTS Among the 1716 patients with confirmed acute PE, syncope or pre-syncope was the initial manifestation of the disease in 458 (26.6%) patients. Short-term mortality (30-day) for all causes were significantly higher in patients with syncope/presyncope (42.5% vs 6.2%, p < 0.0001) while PE patients with presyncope demonstrated a worst short-term outcome, in terms of mortality for all-causes, when compared to those subjects with syncope at admission (47.2% vs 37.4%, p = 0.03). A statistically significant difference in survival between pre-syncope and syncope was observed only in hemodynamically unstable patients [log rank p = 0.036]. Cox regression analysis confirmed that pre-syncope resulted an independent predictor of 30-day mortality in hemodynamically unstable patients at admission (HR 2.13, 95% CI 1.08-4.22, p = 0.029), independently from right ventricular dysfunction (RVD) (HR 6.23, 95% CI 3.05-12.71, p < 0.0001), age (HR 1.03, 95% CI 1.00-1.06, p = 0.023) and thrombolysis (HR 2.27, 95% CI 1.11-4.66, p = 0.025). CONCLUSIONS PE patients with syncope/presyncope had a higher 30-day mortality for all-causes as well as patients with presyncope had a worst short-term outcome when compared to PE patients with syncope. Moreover, hemodynamically unstable patients with presyncope had a worst prognosis independently from the presence of RVD, age, positive cTn and thrombolytic treatment.

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

Abstract 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 four-chamber in transit heart thrombus in a pregnant woman with acute pulmonary embolism

A four-chamber in transit heart thrombus in a pregnant woman with acute pulmonary embolism -