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A 25-year-old man presented with a history of bicuspid aortic valve (BAV), moderate aortic valve stenosis, moderate aortic valve insufficiency, dilated aortic root, and left ventricular noncompaction cardiomyopathy.

He was born with BAV and severe aortic valve stenosis and underwent surgical aortic valvotomy at 2 days of age. There was recurrence of the aortic valve stenosis requiring percutaneous balloon valvuloplasty at age 1 year and again at age 2 years. He ultimately required repeat surgical valvotomy at age 4 years which also included supravalvular aortoplasty with a Gore-Tex patch. He was also noted to have short stature and developmental delay. Genetic evaluation was pursued with no definitive diagnosis being established. He has been seen in scheduled follow-up since and had done reasonably well without report of cardiovascular related symptoms until 2 years ago when he began experiencing 2 pillow orthopnea and increasing fatigue with activity. Up until this time, he was living alone and working part time in a factory.

Assessment of his symptoms at that time included an echocardiogram which revealed moderate-to-severe aortic insufficiency with preserved left ventricular ejection fraction (LVEF). However, detailed assessment of the left ventricular apex showed abnormal myocardium consistent with left ventricular noncompaction (LVNC). Diuretic therapy was instituted to manage his orthopnea. He was referred to the care of the Adult Congenital Heart Disease (ACHD) and Cardiomyopathy services for further management. He continued to have heart failure (HF) symptoms and had worsening aortic insufficiency on serial imaging studies with a decline in his LVEF. Treatment with appropriate heart failure therapies was instituted and aspirin was given for his diagnosis of LVNC. Based on his severe aortic insufficiency and worsening LV systolic function, he was referred for elective replacement of his aortic valve.

He underwent successful aortic valve replacement with a 21-mm Carpentier Edwards prosthetic valve. He did well postoperatively and was discharged to home on postoperative day 6 on his preoperative medical regimen with the substitution of Coumadin for aspirin therapy. Outpatient evaluation 1 week after discharge was significant for complete resolution of his heart failure symptoms. An echocardiogram at that time revealed improvement in his LVEF with mild systolic dysfunction (LVEF 48%) and no evidence of significant prosthetic aortic valve stenosis or insufficiency.


  • Many, if not all, ACHD patients are at risk of HF whether unrepaired, repaired, or palliated.

  • Several of these patients may have underlying myocardial dysfunction, valvular heart disease along with exercise intolerance. Some progress to have heart failure.

  • Myocardial dysfunction (right, left, or biventricular) is a common final pathway for these patients, which underscore the need for surveillance of systolic and diastolic function as well as resting and provocable HF symptoms.

  • Recognition of dysfunction prior to symptoms allows for institution of appropriate medical therapies and more regimented evaluations in an attempt to avoid progression to more advanced stages of HF.

  • The above ...

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