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

Abstract

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 2 ) with good systolic function (right ventricular ejection fraction=59±7%). Most had exercise capacity within normal range (z peak V i o 2 =−0.91±1.3; z V i e/V i co 2 =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.

miR-21 is associated with fibrosis and right ventricular failure

“…This is a critical issue, as more children with complex CHD are surviving into adulthood, many of whom are at risk for chronic complications, such as arrhythmias and heart failure (9, 26-28). Although pulmonary valve replacement can ameliorate the combined volume and pressure loads, reduce RV dimension, and improve New York Heart Association functional class, surgical intervention has not necessarily improved exercise capacity, arrhythmia burden, ejection fraction, or survival, demonstrating the large gaps in our knowledge about how best to manage this rapidly growing group of patients (2,10,26,28). RV fibrosis in this patient population is associated with worsening symptoms, exercise intolerance, and ventricular dysfunction (29).…”

“…However, over decades, the resultant combination of moderate-to-severe PI plus mild-to-moderate PS results in progressive RV dilation and early RV diastolic dysfunction. Progression to RV systolic failure may take years to develop, but once RV failure ensues it may persist even after pulmonary valve replacement (2,3). Persistence of RV dysfunction may reflect irreversible adverse remodeling associated with chronic volume and pressure overload and may be a precursor to late arrhythmias and sudden death (8).…”

Almanac 2014: congenital heart disease

“…In a study from the UK, long-term outcome of patients with ToF was characterised with a duration of follow-up up to 45 years. 33 The authors demonstrate that the annual probability of death remains fourfold higher than that of the general population throughout the first five decades of life. In a large multinational study (International Multicentre TOF Registry; INdiCaTOR), 873 adults with ToF were screened for risk factors that are predictive of death or sustained ventricular tachycardia.…”

Improving risk adjustment in the PRAiS (Partial Risk Adjustment in Surgery) model for mortality after paediatric cardiac surgery and improving public understanding of its use in monitoring outcomes

“…pulmonary regurgitation) or when a code highly specific to a given primary diagnosis that was already a factor in the risk model (intramural coronary in transposition). [108][109][110] Both PRAiS 1 and PRAiS 2 include the risk factors of primary diagnosis and univentricular status, and hence the expert panel elected not to consider prior cardiac surgery as a risk factor given its overlap with these features.…”