Tetralogy of Fallot Repair in Patients 40 Years or Older

Abstract

OBJECTIVE: To report the outcomes of patients with tetralogy of Fallot (TOF) undergoing surgical repair at age 40 years or older. PATIENTS AND METHODS:We reviewed records of patients (age, ≥40 years) who underwent TOF repair from January 1, 1970, through December 31, 2007. Symptoms, palliative procedures, surgical reports, and long-term outcomes were analyzed. RESULTS: Fifty-two patients (30 men [58%]) had surgery at a mean ± SD age of 50±8 years; 27 (52%) had prior palliative surgery at a mean ± SD age of 17±11 years. Procedures for TOF repair included pulmonary valve replacement (n=10), transannular patch (n=10), and native pulmonary valve preservation (n=32). The 30-day mortality rate was 6% (stroke, n=2; ventricular fibrillation, n=1). A mean ± SD follow-up of 14.9±9.3 years was feasible in 48 of 49 survivors; improvement in functional class was observed in 42 patients. Reoperation was performed in 7 patients (4 for pulmonary regurgitation). Twenty-nine patients died (mean ± SD age, 65±12 years); causes of death were cardiac (n=7), noncardiac (n=4), and unknown (n=18). Mean ± SD age at death was younger in patients with previous palliation (59±11 years vs 70±12 years; P=.03). The 10-year survival rate was lower than expected compared with an age-and sex-matched population (73% vs 91%; P<.001).CONCLUSION: Complete repair of TOF in patients 40 years or older is feasible but carries increased operative risk. Surgical survivors have improvement in functional class; however, survival remains lower than expected. Reduced survival and need for reoperation emphasize the importance of pulmonary valve replacement at the time of initial repair and long-term follow-up.

Congenital Heart Disease in the Older Adult

“…40 Those who present after years of pulmonary overcirculation, with Eisenmenger physiology or chronic cyanosis, have unique issues covered in the section on pulmonary vascular disease in the older adult with CHD. Patients with pulmonary overcirculation caused by high multiple aortopulmonary collateral flow or by palliation with systemic pulmonary shunt operations…”

Unrepaired Tetralogy of Fallot with Major Aortopulmonary Collateral Arteries in an Adult Patient

“…Their 10-year survival rate was 73%, but half of them had prior palliative surgeries. 6 Tetralogy of Fallot with major aortopulmonary collateral arteries was described in 1985 in a series of seven pediatric patients and one adult (30 years old). In six, collaterals were ligated during the surgery.…”

Uncorrected Tetralogy of Fallot, Biventricular Dysfunction, and a Large Pericardial Effusion

“…Surgical and anesthetic management presented multiple challenges. First, the 30-day mortality in adult surgical correction of TOF is as high as 6%; 13 though in the setting of acute or chronic heart failure and biventricular dysfunction, the risk of TOF is unknown but likely is increased. The nature and timing of surgical intervention were debated at length at a multidisciplinary conference.…”