Cardiovascular Magnetic Resonance of Isolated Left Ventricular Apical Hypoplasia
A 37-year-old white peripartum female was admitted to intensive care with pulmonary edema. She had no past medical history of note. Left bundle-branch block, P mitrale, and left-axis deviation were identified on her ECG, and cardiomegaly on her chest x-ray (Figures 1 and 2⇓). She was referred for cardiovascular magnetic resonance (CMR) after diagnostic coronary angiography revealed normal coronary arteries.
CMR (Figure 3) demonstrated several apparent aneurysms of the left ventricle (LV). However, these areas demonstrated contraction and so are perhaps better termed “out-pouchings” or “diverticulae” (see online-only Data Supplement Movies I to III). Contrast imaging showed no thrombus and no late enhancement of these areas. The LV was shortened with apical fatty replacement (arrow) and the right ventricle wrapped around it. The left atrium was very dilated with evidence of raised pressure.
After initial antifailure therapy, she recovered well and was subsequently maintained on a β-blocker, angiotensin-converting enzyme inhibitor, aldosterone antagonist, amiodarone (for nonsustained ventricular tachycardia on Holter monitoring) and coumarin. Two years later, she is in New York Heart Association class II.
The CMR appearances are unusual and not typical for postpartum cardiomyopathy. The lack of scarring excludes several acquired conditions (eg, sarcoid, Chagas). A pregnancy-precipitated decompensation of an existing congenital condition appears likely. In fact, the images are typical for the unclassified cardiomyopathy, “isolated left ventricular apical hypoplasia,” previously described in 3 Cleveland Clinic patients.1 These patients (aged 22 to 46) presented with nonspecific symptoms (eg, fatigue and atypical chest pain). Cardiac computed tomography and CMR demonstrated similar findings of a truncated, spherical, and impaired LV, apical LV fatty material, papillary muscle/trabecular abnormalities, and right ventricular elongation wrapping around the deficient LV apex.
Like our patient, the Cleveland Clinic patients responded well to treatment. With just 4 cases described, however, the pathogenesis and prognosis of isolated LV apical hypoplasia remains at this stage unclear.
The online-only Data Supplement, which includes movies and figures, is available with this article at http://circ.ahajournals.org/cgi/content/full/117/ e504/DC1.