Author + information
- Received February 7, 2011
- Accepted February 15, 2011
- Published online November 22, 2011.
A 63-year-old man presented with exertional dyspnea and poorly controlled atrial fibrillation. Transthoracic echocardiography revealed a severely impaired truncated left ventricle and an elongated right ventricle (A, Online Videos 1, 2, 3, and 4). Adequate ventricular rate control could not be achieved, but successful direct-current cardioversion led to significant reduction in symptoms. Cardiovascular magnetic resonance demonstrated all 4 of the phenotypical features of isolated left ventricular apical hypoplasia (1): 1) a spherical truncated left ventricle with impaired function (Online Videos 5, 6, and 7); 2) replacement of the left ventricular apex with fatty material contiguous with epicardial fat (C); 3) anteroapical origin of the papillary muscle network (D); and 4) an elongated right ventricle (E). We also demonstrate apical thrombus on early gadolinium enhancement imaging (F, arrow) and the novel finding of midwall fibrosis in the basal-anterior septum identified on late gadolinium enhancement imaging (G, H, arrows) that persisted after swapping the phase-encoding direction (I, arrow), distinguishing it from artifact.
This is the first description of midwall fibrosis in isolated left ventricular apical hypoplasia and entertains the possibility of an acquired etiology such as in utero infection for what is presumed to be a rare congenital developmental defect. Our case also demonstrates the degree to which such patients are highly dependent on atrial contractile function because of altered left ventricular geometry.
Drs. Plein and Greenwood have received a research grant from Philips Healthcare
- Received February 7, 2011.
- Accepted February 15, 2011.
- American College of Cardiology Foundation