Francesco Negri

Persistent Recovery of Normal Left Ventricular Function and Dimension in Idiopathic Dilated Cardiomyopathy During Long‐Term Follow‐up: Does Real Healing Exist?

Background An important number of patients with idiopathic dilated cardiomyopathy have dramatically improved left ventricular function with optimal treatment; however, little is known about the evolution and long‐term outcome of this subgroup, which shows apparent healing. This study assesses whether real healing actually exists in dilated cardiomyopathy . Methods and Results Persistent apparent healing was evaluated among 408 patients with dilated cardiomyopathy receiving tailored medical treatment and followed over the very long‐term. Persistent apparent healing was defined as left ventricular ejection fraction ≥50% and indexed left ventricular end‐diastolic diameter ≤33 mm/m2 at both mid‐term (19±4 months) and long‐term (103±9 months) follow‐up. At mid‐term, 63 of 408 patients (15%) were apparently healed; 38 (60%; 9% of the whole population) showed persistent apparent healing at long‐term evaluation. No predictors of persistent apparent healing were found. Patients with persistent apparent healing showed better heart transplant–free survival at very long‐term follow‐up (95% versus 71%; P=0.014) compared with nonpersistently normalized patients. Nevertheless, in the very long term, 37% of this subgroup experienced deterioration of left ventricular systolic function, and 5% died or had heart transplantation. Conclusions Persistent long‐term apparent healing was evident in a remarkable proportion of dilated cardiomyopathy patients receiving optimal medical treatment and was associated with stable normalization of main clinical and laboratory features. This condition can be characterized by a decline of left ventricular function over the very long term, highlighting the relevance of serial and individualized follow‐up in all patients with dilated cardiomyopathy, especially considering the absence of predictors for long‐term apparent healing.

RESTRICTIVE CARDIOMYOPATHY: CLINICAL ASSESSMENT AND IMAGING IN DIAGNOSIS AND PATIENT MANAGEMENT.

The main difference between restrictive cardiomyopathy (RCM) and the other cardiomyopathies (CMP) is that its diagnosis and definition depend on functional rather than morphologic criteria. All causes of diastolic dysfunction are included in the differential diagnosis of RCM, in particular, infiltrative/storage CMP and constrictive pericarditis (CP). The recognition of CP is clinically important, as it is potentially curable with surgery, whereas RCM has no specific therapy. Rare forms characterized by endomyocardial involvement with severe fibrosis, with or without eosinophilic infiltration, are included within the spectrum of RCM disease. It must be noted that, although RCM is the least common CMP and extremely rarely encountered in clinical practice, its diagnosis must be an exclusion diagnosis, and it is important to exclude all the other causes of restrictive filling in order to manage these patients with the most appropriate and possibly specific therapy.

Role of right ventricular involvement in acute myocarditis, assessed by cardiac magnetic resonance

OBJECTIVES Right ventricular (RV) myocarditis (MY) is unrecognized, and its prevalence is unknown. We evaluated the prevalence of RV involvement in acute MY and its association with cardiac events (cardiac death, cardiac arrest, ventricular assist device, transplantation, and appropriate ICD intervention). METHODS We enrolled 151 patients who underwent cardiac magnetic resonance for clinical suspicion of acute MY. The CMR protocol included T2-STIR images for edema detection, post-contrast cine-SSFP for hyperemia detection and late gadolinium enhancement (LGE) images. RESULTS Signs of RV MY were found in 27 patients (17.8%): RV edema at T2-STIR in all of these 27 patients; RV LGE was detected in 11 patients (7.3%). The median RV myocardial segment involved was 2 (1-3). In 13 patients, RV edema was in direct continuity with LV edema of septum and inferior wall or with anterior septum and anterior wall. In 2 patients RV myocarditis was found without any signs of LV involvement. Patients with RV MY had higher RV end-diastolic volume index (p = 0.04) and RV mass index (p = 0.03), and lower RV ejection fraction (p < 0.001) than others. At Kaplan-Meier survival curve patients with RV MY had more cardiac events than those without RV involvement (p = 0.015). RV involvement, anteroseptal LGE and RV LGE were associated with cardiac events. CONCLUSION RV involvement in acute MY is more frequent than previously hypothesized. RV MY was associated with cardiac events.

A Large Ostium Secundum Atrial Septal Defect in an 88-Year-Old Asymptomatic Woman

version and poster were presented at the 98th Annual Meeting of the Endocrine Society, April 3, 2016, Boston, Massachusetts. Conflict of Interest: None. Author Contributions: Wong, Wang: study concept and design, acquisition of subjects and data, analysis and interpretation of data, preparation of manuscript. Sponsor’s Role: N/A.

The eight-shaped heart: an incidental giant left ventricular pseudoaneurysm

A 77-year-old man was admitted to the ER for sepsis. Four years before he had an anterior myocardial infarction. At the chest radiography, a new large opacity was recognizable in the basal field of …

Dilated Cardiomyopathy: Usefulness of Imaging in Prognostic Stratification and Choice of Treatment

Prognosis of idiopathic dilated cardiomyopathy (DCM) was once considered ominous. However, in the last 30 years, advancements in knowledge regarding the pathophysiology of heart failure (HF), earlier diagnosis, systematic long-term follow-up, and—mostly—the use of effective pharmacological and nonpharmacologic treatments have contributed to substantially improved survival rates. Evaluating left ventricular (LV) dimensions, systolic and diastolic function, and contractile reserve with echocardiography and/or cardiac magnetic resonance (CMR) can help in prognostic stratification. In the near future, the possibility of genotyping all patients could have a prognostic importance, allowing identification of some malignant mutations with higher mortality risk. A critical and still unresolved issue is early stratification of risk of sudden death (SD) and indication to implantable device therapy. SD can also occur in some apparently stable DCM patients. In this sense, some technologies such as T-wave alternans analysis and QT dynamicity are emerging but require further evidence before being routinely applied in clinical practice.

Other Cardiomyopathies: Clinical Assessment and Imaging in Diagnosis and Patient Management

In this chapter describes clinical and imaging assessment in diagnosis and patient management of other cardiomyopathies (CMP) not included among the previously defined main groups of CMP. Most of these unclassified CMP are characterized by frequent reversibility of myocardial dysfunction after adequate treatment. The peculiar form called left ventricular noncompaction is also addressed.