PVR Timing Is Tricky In Tetralogy of Fallot

TORONTO — There are three main reasons to send an adult with tetralogy of Fallot for pulmonary valve replacement: moderate to severe pulmonary regurgitation, evidence of right ventricular overload, and a clinical context suggesting the need for the procedure.

Pulmonary valve replacement (PVR) should only be undertaken in patients in whom all three reasons are present, Dr. Gary Webb said at the 18th International Symposium on Adult Congenital Heart Disease.

The clinical situations that might drive a decision to replace a leaky pulmonary valve include exercise intolerance attributable to the pulmonary regurgitation or congenital heart defect, sustained atrial flutter or fibrillation, sustained ventricular tachycardia or resuscitated sudden death, or an asymptomatic patient with “excessive” right ventricular dilation.

“Ten years ago, we replaced the pulmonary valve for exercise intolerance, sustained arrhythmias, and 'progressive' right ventricular dysfunction,” said Dr. Webb, the director of the Philadelphia Adult Congenital Heart Center. “However, using these criteria for surgery, we learned that we had waited too long for many of these patients, and in the end they had suboptimal results.”

The decision of when to intervene in pulmonary regurgitation has evolved and continues to evolve, said Dr. Webb. “Even mild left ventricular systolic dysfunction is an indication for surgery for aortic and mitral regurgitation, so ideally, if we could see that right ventricular systolic function was declining from normal, then we could apply the same criteria. But, of course, it doesn't work in tetralogy patients because the right ventricle is not normal.”

Efforts to risk-stratify these patients, therefore, have focused instead on right ventricular diastolic volumes.

Although there are risks to waiting too long before PVR, Dr. Webb does not think a low threshold for replacing a leaking pulmonary valve is wise. Indeed, 10-year and 30-year survival after repair of tetralogy of Fallot in one large study was 97% and 89% in patients surviving at least 1 year (J. Am. Coll. Cardiol 1997; 30:1374–83).

“If we have 11% mortality over 30 years in this cohort of survivors we don't want to be pulling the trigger too impulsively on these patients,” he said.

TORONTO — There are three main reasons to send an adult with tetralogy of Fallot for pulmonary valve replacement: moderate to severe pulmonary regurgitation, evidence of right ventricular overload, and a clinical context suggesting the need for the procedure.

Pulmonary valve replacement (PVR) should only be undertaken in patients in whom all three reasons are present, Dr. Gary Webb said at the 18th International Symposium on Adult Congenital Heart Disease.

The clinical situations that might drive a decision to replace a leaky pulmonary valve include exercise intolerance attributable to the pulmonary regurgitation or congenital heart defect, sustained atrial flutter or fibrillation, sustained ventricular tachycardia or resuscitated sudden death, or an asymptomatic patient with “excessive” right ventricular dilation.

“Ten years ago, we replaced the pulmonary valve for exercise intolerance, sustained arrhythmias, and 'progressive' right ventricular dysfunction,” said Dr. Webb, the director of the Philadelphia Adult Congenital Heart Center. “However, using these criteria for surgery, we learned that we had waited too long for many of these patients, and in the end they had suboptimal results.”

The decision of when to intervene in pulmonary regurgitation has evolved and continues to evolve, said Dr. Webb. “Even mild left ventricular systolic dysfunction is an indication for surgery for aortic and mitral regurgitation, so ideally, if we could see that right ventricular systolic function was declining from normal, then we could apply the same criteria. But, of course, it doesn't work in tetralogy patients because the right ventricle is not normal.”

Efforts to risk-stratify these patients, therefore, have focused instead on right ventricular diastolic volumes.

Although there are risks to waiting too long before PVR, Dr. Webb does not think a low threshold for replacing a leaking pulmonary valve is wise. Indeed, 10-year and 30-year survival after repair of tetralogy of Fallot in one large study was 97% and 89% in patients surviving at least 1 year (J. Am. Coll. Cardiol 1997; 30:1374–83).

“If we have 11% mortality over 30 years in this cohort of survivors we don't want to be pulling the trigger too impulsively on these patients,” he said.

TORONTO — There are three main reasons to send an adult with tetralogy of Fallot for pulmonary valve replacement: moderate to severe pulmonary regurgitation, evidence of right ventricular overload, and a clinical context suggesting the need for the procedure.

Pulmonary valve replacement (PVR) should only be undertaken in patients in whom all three reasons are present, Dr. Gary Webb said at the 18th International Symposium on Adult Congenital Heart Disease.

The clinical situations that might drive a decision to replace a leaky pulmonary valve include exercise intolerance attributable to the pulmonary regurgitation or congenital heart defect, sustained atrial flutter or fibrillation, sustained ventricular tachycardia or resuscitated sudden death, or an asymptomatic patient with “excessive” right ventricular dilation.

“Ten years ago, we replaced the pulmonary valve for exercise intolerance, sustained arrhythmias, and 'progressive' right ventricular dysfunction,” said Dr. Webb, the director of the Philadelphia Adult Congenital Heart Center. “However, using these criteria for surgery, we learned that we had waited too long for many of these patients, and in the end they had suboptimal results.”

The decision of when to intervene in pulmonary regurgitation has evolved and continues to evolve, said Dr. Webb. “Even mild left ventricular systolic dysfunction is an indication for surgery for aortic and mitral regurgitation, so ideally, if we could see that right ventricular systolic function was declining from normal, then we could apply the same criteria. But, of course, it doesn't work in tetralogy patients because the right ventricle is not normal.”

Efforts to risk-stratify these patients, therefore, have focused instead on right ventricular diastolic volumes.

Although there are risks to waiting too long before PVR, Dr. Webb does not think a low threshold for replacing a leaking pulmonary valve is wise. Indeed, 10-year and 30-year survival after repair of tetralogy of Fallot in one large study was 97% and 89% in patients surviving at least 1 year (J. Am. Coll. Cardiol 1997; 30:1374–83).

“If we have 11% mortality over 30 years in this cohort of survivors we don't want to be pulling the trigger too impulsively on these patients,” he said.