Prognosis of Adults With Isolated Left Ventricular Non-Compaction: Results of a Prospective Multicentric Study.

Background: Whether left ventricular non-compaction (LVNC) bears a different prognosis than dilated cardiomyopathy (DCM) is still a matter of debate.

Methods: From a multicenter French prospective registry, we compared the outcomes of 98 patients with LVNC and 65 with DCM. The primary endpoint combined cardiovascular death, heart transplantation, and hospitalization for cardiovascular events.

Phenotype/Genotype Relationship in Left Ventricular Noncompaction: Ion Channel Gene Mutations Are Associated With Preserved Left Ventricular Systolic Function and Biventricular Noncompaction: Phenotype/Genotype of Noncompaction.

Background: Few data exist concerning genotype-phenotype relationships in left ventricular noncompaction (LVNC).

Methods And Results: From a multicenter French Registry, we report the genetic and clinical spectrum of 95 patients with LVNC, and their genotype-phenotype relationship. Among the 95 LVNC, 45 had at least 1 mutation, including 14 cases of mutation in ion channel genes.

Right ventricular longitudinal strain: a tool for diagnosis and prognosis in light-chain amyloidosis.

Objectives: Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis.

Acquired Systemic-to-pulmonary Venous Shunt or Persistent Left Superior Vena Cava? A Rare Right-to-left Shunt Case-based Discussion.

Cardiac shunts are often described in congenital or pediatric populations, but systemic-to-pulmonary venous shunts in adult patients are reported in literature in isolated case reports. We present the case of a 70-year-old female with a left superior vena cava (SVC) draining into the left atrium by the left superior pulmonary vein, with a complete right-to-left shunt of the superior venous circulation caused by a former catheter thrombosis in the right SVC. Diagnosis was suspected after a contrast echocardiography showing an exclusive perfusion of left heart after intravenous injection and confirmed by helical computed tomography with three-dimensional reconstruction.