Cardiomyopathy in limb girdle muscular dystrophy R9, FKRP related.

Introduction: Reported frequencies of cardiomyopathy in limb girdle muscular dystrophy R9 (LGMDR9) vary. We describe the frequency and age at onset of cardiomyopathy in an LDMDR9 cohort.

Methods: Echocardiograms from 56 subjects (157 echocardiograms) with LGMDR9 were retrospectively reviewed.

Intervention for Supravalvar Pulmonary Stenosis After the Arterial Switch Operation.

Background: The arterial switch operation is standard of care for infants born with dextrotransposition of the great arteries. Supravalvar pulmonary stenosis is a common complication that may require reintervention-balloon angioplasty, stenting, or surgical augmentation. A subset of patients requires more than one reintervention.

Hypoplastic left heart with intact atrial septum and levoatriocardinal vein: a challenge in identifying aortic arch branches.

The levoatriocardinal vein provides alternative egress from the left atrium to the systemic veins in left-sided obstructive lesions. Although rare, it has been described in association with hypoplastic left heart syndrome. We report a case of hypoplastic left heart syndrome with levoatriocardinal vein and aberrant right subclavian artery where cardiac magnetic resonance imaging/angiography proved to be a valuable imaging modality for pre-operative evaluation.