Dyspnea in a 63-Year-Old Who Had Heart Surgery as a Child

Sarah Blissett, MD, MHPE; Punag Divanji, MD; Harsh Agrawal, MD; Vaikom S. Mahadevan, MD; Elyse Foster, MD


December 09, 2019


In this case, a 63-year-old man with repaired tetralogy of Fallot presented with dyspnea upon exertion. Tetralogy of Fallot consists of four cardiac abnormalities: narrowing of the RV outflow tract, ventricular septal defect, RV hypertrophy, and overriding aorta. Most patients undergo surgical repair to relieve the RV outflow tract narrowing and to repair the ventricular septal defect during childhood. Contemporary data demonstrate that more than 90% of patients with repaired tetralogy of Fallot survive into adulthood.[1] Repaired tetralogy of Fallot is currently the most prevalent complex congenital cardiac lesion in adults, with approximately 72,000 adults in the United States affected.[2,3]

The differential diagnosis of dyspnea in a patient with repaired tetralogy of Fallot is broad and includes causes related to congenital heart disease, acquired heart disease, and noncardiac etiologies. Congenital issues include pulmonary stenosis, pulmonary regurgitation, tricuspid regurgitation, RV dysfunction, LV dysfunction, atrial arrhythmias, and ventricular arrhythmias. Acquired heart diseases that may be present include coronary artery disease, diabetic cardiomyopathy, alcohol-related cardiomyopathy, and endocarditis.

Although right-sided valvular dysfunction and RV dysfunction are more common than left-sided lesions, approximately 20%-30% of adult patients with repaired tetralogy of Fallot have LV dysfunction, defined as LVEF < 55%.[4,5,6] Of note, the presence of LV systolic dysfunction confers a worse prognosis, with higher rates of cardiac events (death or hospitalization) seen in patients with LVEF < 55% than in patients with normal EFs.[5]


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