Neoaortic Dilation and Polyvalvar Dysfunction: The Coexistence of Marfan Syndrome with Hypoplastic Left Heart Syndrome.

We present a unique case of progressive neoaortic root and neoascending aortic dilatation, with poly-valvar regurgitation in a patient with Marfan syndrome and hypoplastic left heart syndrome palliated to Fontan procedure. This progressive dilation and valvar dysfunction necessitated a modified Bentall procedure and tricuspid valve repair. Our case highlights the impact of both a primary and secondary aortopathy complicating the Fontan procedure and the need for mechanical aortic valve replacement.

Imaging-Guided Therapies for Pericardial Diseases.

Frequently, multimodality imaging is indispensable in the care of patients with pericardial disease. With cardiac magnetic resonance imaging, pericardial inflammation can be characterized as acute, subacute, or chronic. This spectrum of inflammation is variably associated with reduced compliance of the pericardium, which may result in constrictive pathophysiology, typically well-defined with echocardiography.

Catheter ablation of ventricular fibrillation storm in patients with infiltrative amyloidosis of the heart.

Recent studies have demonstrated that premature ventricular contractions (PVCs) originating from the Purkinje system are responsible for initiation of ventricular fibrillation (VF) in patients with and without structural heart disease. Ablation of the PVCs has been shown to be feasible. We report 2 patients with repetitive VF associated with cardiac amyloidosis.