Physiological and Phenotypic Characteristics of Late Survivors of Tetralogy of Fallot Repair Who Are Free From Pulmonary Valve Replacement

• Published in: Circulation (New York, N.Y.) Vol. 128; no. 17; pp. 1861 - 1868
• Main Authors: Frigiola, Alessandra, Hughes, Marina, Turner, Mark, Taylor, Andrew, Marek, Jan, Giardini, Alessandro, Hsia, Tain-Yen, Bull, Kate
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD by the American College of Cardiology Foundation and the American Heart Association, Inc 22-10-2013; Lippincott Williams & Wilkins
• Subjects: Adolescent; Adult; Biological and medical sciences; Blood and lymphatic vessels; Cardiac Surgical Procedures - mortality; Cardiology. Vascular system; Child; Child, Preschool; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Exercise Test; Exercise Tolerance; Female; Follow-Up Studies; Heart; Humans; Male; Medical sciences; Postoperative Complications - diagnosis; Postoperative Complications - mortality; Postoperative Complications - physiopathology; Pulmonary Valve - physiology; Pulmonary Valve - surgery; Pulmonary Valve Insufficiency - diagnosis; Pulmonary Valve Insufficiency - mortality; Pulmonary Valve Insufficiency - physiopathology; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the heart; Survivors - statistics & numerical data; Tetralogy of Fallot - mortality; Tetralogy of Fallot - physiopathology; Tetralogy of Fallot - surgery; Ventricular Outflow Obstruction - diagnosis; Ventricular Outflow Obstruction - mortality; Ventricular Outflow Obstruction - physiopathology; Young Adult; Physical exercise; magnetic resonance imaging; Cardiovascular disease; Cardiac surgery; Heart valve; tetralogy of Fallot; Heart disease; Fallot tetralogy; Exercise tolerance test; Cardiology; Repair; Human; Corrective surgery; Late; Survivor; Characteristic; Tolerance; Congenital disease; Nuclear magnetic resonance imaging; Pulmonary valve; Phenotype; Treatment; Characteristics; Medical imagery; exercise tolerance; Circulatory system; cardiac surgery
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/CIRCULATIONAHA.113.001600

BACKGROUND—Pulmonary valve replacement (PVR) after repair of tetralogy of Fallot is commonly required and is burdensome. Detailed anatomic and physiologic characteristics of survivors free from late PVR and with good exercise capacity are not well described in a literature focusing on the indications for PVR.
METHODS AND RESULTS—Survival and freedom from PVR were tracked in 1085 consecutive patients receiving standard tetralogy of Fallot repair in a single institution from 1964 to 2009. Of 152 total deaths, 100 occurred within the first postoperative year. Surviving patients between 10 and 50 years of age had an annual risk of death of 4 (confidence limit, 2.8–5.4) times that of normal contemporaries. To date, 189 patients have undergone secondary PVR at mean age of 20±13 years (36% of those alive at 40 years of age). A random sample of 50 survivors (age, 4–57 years) free from PVR underwent cardiovascular magnetic resonance, echocardiography, and exercise testing. These patients had mildly dilated right ventricles (right ventricular end-diastolic volume=101±26 mL/m) with good systolic function (right ventricular ejection fraction=59±7%). Most had exercise capacity within normal range (z peak (Equation is included in full-text article.)O2=−0.91±1.3; z (Equation is included in full-text article.)E/(Equation is included in full-text article.)CO2=0.20±1.5). In patients >35 years of age with normal exercise capacity, there was mild residual right ventricular outflow tract obstruction (mean gradient, 24±13 mm Hg), pulmonary annulus diameters <0.5z, and unobstructed branch pulmonary arteries.
CONCLUSIONS—An important proportion of patients require PVR late after tetralogy of Fallot repair. Patients surviving to 35 years of age without PVR and with a normal exercise capacity may have had a definitive primary repair; their right ventricular outflow tracts are characterized by mild residual obstruction and pulmonary annulus diameter <0.5z.