Familial himalayan p wave and left ventricular Hypertrabeculation/Noncompaction

Abstract

Background "Himalayan P waves," are reported in congenital heart disease and cardiomyopathies. Methods We report a family with hypertrophic cardiomyopathy, Himalayan P waves, extensive focal right atrial wall thickening and left ventricular hypertrabeculation/noncompaction (LVHT). Results The father received a pacemaker and underwent heart transplantation because of hypertrophic cardiomyopathy. His daughters showed Himalayan P waves and right atrial wall thickening. LVHT was diagnosed in sister A at age 23 years and developed in sister B between 42 and 46 years. In sister A the heart rate continuously declined. She refused implantation of a pacemaker and died with 49 years. Sister B, suffers from bradycardia. Conclusions Himalayan P waves are due to focal right atrial wall thickening, may be familially and associated with LVHT.