High Grade Atrioventricular Block in Von Recklinghausen Disease: A Rare Phenomenon

A case of a 73-year-old woman with a history of von Recklinghausen disease (neurofibromatosis type 1) who presented initially with a gradual onset of shortness of breath and lightheadedness with no associated fever, chills, angina, palpitations, cough, weight loss, night sweats, nausea, vomiting, or constipation. She was found to be severely bradycardic and in third degree atrioventricular block by her primary care physician. She was admitted in the hospital because of intermittent bouts of lightheadedness and progression of the shortness of breath. Twelve-lead electrocardiogram documented the high grade atrioventricular block. Chest radiograph showed subtle mild pulmonary congestion. Transthoracic echocardiogram revealed mild concentric hypertrophy and normal systolic function with no regional wall motion abnormalities or evidence of significant valvular disease. Pacemaker was inserted and her symptoms improved significantly.