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      Familial left ventricular hypertrabeculation in two blind brothers.

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          Abstract

          So far, left ventricular hypertrabeculation (LVHT) has been described to occur only sporadically. In a 49-year-old man with Leber's hereditary optic neuropathy (LHON) due to the primary LHON mutation G3460A, arterial hypertension was reported since 2000 and palpitations since 1995. ECG revealed Wolff-Parkinson-White syndrome. Transthoracic echocardiography and cardiac MRI showed myocardial thickening and LVHT. Pindolol markedly improved the cardiac abnormalities. Surprisingly, LVHT was also found in the 50-year-old brother of the index patient who also had LHON and also carried the G3460A mtDNA mutation. This brother also had Wolff-Parkinson-White syndrome and myocardial thickening, but without hypertension. It is concluded that LVHT, previously described to occur only sporadically, may be hereditary in single cases.

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          Author and article information

          Journal
          Cardiovasc. Pathol.
          Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology
          1054-8807
          1054-8807
          May 29 2002
          : 11
          : 3
          Affiliations
          [1 ] Ludwig Boltzmann Institute for Research in Epilepsy and Neuromuscular Disorders, Vienna, Austria. josef.finsterer@nkr.magwien.gv.at
          Article
          S1054880701000874
          12031765
          e75bd3da-c882-424e-8db3-4626ed2c5f11
          History

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