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Number 08-17 Isolated Left Ventricular Apical Hypoplasia.
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Number 08-17 Isolated Left Ventricular Apical Hypoplasia.

Case from: Enrico Franzi’, Giuseppe Leonardi. Department of Cardiology “San Vincenzo” Hospital, Taormina, Italy. Department of Cardiac Surgery  “Ferrarotto”  Hospital, Catania , Italy .

Clinical History:  35yr male. Known previous DCM diagnosed via echocardiography. Currently stable, NYHA Class I, and well controlled. No significant arrhythmias noted. Referred for CMR.

CMR: The LV is spherical in shape on black blood axial imaging (top image, white arrow). On cine imaging an elongated RV is wrapped around the LV, forming the apex. There is one complex papillary muscle and an area of fatty infiltration/substitution in the infero-lateral aspect of the LV. LV volumes are in the normal range but LV systolic function is significantly impaired (LVEF=35%).




CMR perspective: These are all features of Isolated Left Ventricular Apical Hypoplasia (ILVAH). A rare congenital cardiomyopathy previously described in 2004 and in the newborn in 2007. The natural progression of this disease is currently not known.

Fernandez-Valls Heart 2004;90:552-555 (free full text)
Marin C et al. Pediatr. Radiol. 37:703-705

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