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

As with patients with acquired heart disease, adult patients with repaired tetralogy of Fallot benefit from treatment of atherosclerotic coronary artery disease and aggressive management of cardiovascular factors. Risk factors should be screened for and treated as in patients without congenital heart disease.^[10,11]

The patient in this case had a low-density lipoprotein level of 65 mg/dL (target, < 70 mg/dL), high-density lipoprotein level of 32 mg/dL, and triglyceride level of 90 mg/dL. His most recent A1c level was 6.5%. His blood pressure was controlled, at 117/64 mm Hg.

Little evidence is available to direct management in patients with repaired tetralogy of Fallot and heart failure symptoms due to LV dysfunction. Clinicians often extrapolate the principles of management that have been extensively researched in patients without congenital heart disease, including the use of beta-blockers, angiotensin-converting enzyme (ACE) inhibitors/angiotensin II receptor blockers, aldosterone antagonists, and cardiac resynchronization therapy. However, the underlying pathophysiology in patients with repaired tetralogy of Fallot is distinct from that in patients with acquired heart disease and may limit the efficacy of these therapies.

Symptomatic management with diuretics may be initiated, with judicious use in patients with concomitant pulmonic stenosis or restrictive RV physiology who may be preload-dependent. The use of ACE inhibitors has been investigated, with subtle improvements in LV systolic and diastolic function noted in adult patients with repaired tetralogy of Fallot and at least moderate pulmonary regurgitation.^[12] Currently, no data are available to guide the use of beta-blockers, aldosterone antagonists, or neprilysin inhibitors for LV dysfunction in patients with repaired tetralogy of Fallot. Experience with cardiac resynchronization therapy in pediatric patients with repaired tetralogy of Fallot continues to evolve, with most devices implanted in the presence of chronic ventricular pacing and most patients experiencing improvements in symptoms and left ventricular function.^[13,14]

Given the established increased risk for sudden cardiac death in patients with repaired tetralogy of Fallot and LV dysfunction, considering whether an implantable cardiac defibrillator could be of benefit is prudent. Guidelines for management of patients with adult congenital heart disease highlight that implantable cardiac defibrillators can be considered in patients with LV or RV dysfunction, nonsustained ventricular tachycardia, QRS duration > 180 msec, extensive RV scarring, or inducible sustained ventricular tachycardia on electrophysiologic study.^[15] The potential benefit must be balanced with the risks, including inappropriate shocks, lead malfunction, and effect on quality of life.

In conclusion, the patient in this case was found to have LV dysfunction that was probably due to coronary artery disease and sequelae of repaired tetralogy of Fallot. His cardiovascular risk factors were managed according to guidelines. He was already taking medications typically used to treat heart failure, including bisoprolol, spironolactone, and lisinopril. He underwent percutaneous coronary intervention with a drug-eluting stent to relieve the stenosis in the ostium of the left anterior descending artery (Figure 2).

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Figure 2.

He was seen for follow-up 3 months after stenting. His dyspnea had improved to New York Heart Association class I, and his LVEF improved to 40%-45% on repeat transthoracic echocardiography.

This case illustrates two important take-home messages. First, as many as one third of patients with repaired tetralogy of Fallot develop LV dysfunction due to various pathophysiologic mechanisms in adulthood. Second, considering coronary artery disease as a contributor to LV dysfunction is important in patients with repaired tetralogy of Fallot because revascularization may improve symptoms and LV function.

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