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Background Chronic hemodynamically relevant pulmonary regurgitation (PR) resulting in important right ventricular dilation and ventricular dysfunction is commonly seen after tetralogy of Fallot (TOF) repair. Late adverse clinical outcomes, including exercise intolerance, arrhythmias, heart failure and/or death accelerate in the third decade of life and are cause for considerable concern. Timing of pulmonary valve replacement (PVR) to address chronic PR is controversial, particularly in asymptomatic individuals, and effect of PVR on clinical measures has not been determined. Methods Canadian Outcomes Registry Late After Tetralogy of Fallot Repair (CORRELATE) is a prospective, multicentre, Canada-wide cohort study. Candidates will be included if they are ≥ 12 years of age, have had surgically repaired TOF resulting in moderate or severe PR, and are able to undergo cardiovascular magnetic resonance imaging. Enrollment of > 1000 individuals from 15 participating centres (Toronto, Montreal, Quebec City, Sherbrooke, Halifax, Calgary, Edmonton, and Vancouver) is anticipated. Clinical data, health-related quality of life metrics, and adverse outcomes will be entered into a web-based database. A central core lab will analyze all cardiovascular magnetic resonance studies (PR severity, right ventricular volumes, and ventricular function). Major adverse outcomes (sustained ventricular tachycardia and cardiovascular cause of death) will be centrally adjudicated. Results To the best of our knowledge, CORRELATE will be the first prospective pan-Canadian cohort study of congenital heart disease in children and adults. Conclusions CORRELATE will uniquely link clinical, imaging, and functional data in those with repaired TOF and important PR, thereby enabling critical evaluation of clinically relevant outcomes in those managed conservatively compared with those referred for PVR.

BACKGROUND: In older children with severe aortic insufficiency (AI), a strategy of aortic valve REPAIR is often pursued in order to delay ROSS procedure or aortic valve replacement with a mechanical/bioprosthetic device (AVR). We aimed to test this strategy of valve REPAIR by evaluating its durability. METHODS: From 2001-2012, 90 children with severe AI underwent: REPAIR (N1⁄446, 51%), ROSS (N1⁄421, 23%) or AVR (N1⁄423, 26%). Repeated measures (N1⁄41081 echos) mixed model analysis were used to evaluate haemodynamic outcomes. RESULTS: Mean age at operation was identical for REPAIR and ROSS (11 years), but slightly older for AVR (13 years). Annular dimensions were largest for REPAIR versus either AVR or ROSS (P1⁄4.01, figure). Cardiopulmonary bypass times were significantly shorter for REPAIRS versus either ROSS or AVR (figure, P

BACKGROUND: 51 years ago, the first Mustard operation was performed at our institution and was successful; the patient is still alive today. The procedure’s success was such that many thought they were “cured”. They were not; “failing Mustards” with severe RV failure, TR and often pulmonary hypertension present to us with increasing frequency. We reviewed our experience with an emphasis on this “Mustard dilemma”. METHODS: In 2010-2013, we undertook multiple iterations of cross-sectional follow-up of all 546. Mustard procedures as part of a “double-switch” operation were not included. All points of last known contact were pursued via as many avenues (primary cardiologists, surgeons, institutions, family physicians). RESULTS: Institutional data protection policies were extremely difficult obstacles, as were breaks in follow-up continuity caused by physician retirement or death. The 546 Mustards (1963 2007) were performed at mean age 2.1 years for diagnoses including predominantly variants of arterial transposition or DORV. Most had undergone prior palliation via atrial septectomy (249), systemic-pulmonary shunt (19), PA band (44) or cavopulmonary connection (3). DHCA was used in w60% (mean 55 minutes). In-hospital mortality was 9%. Following discharge, late survival was excellent, with very slow rising late hazard for death over the subsequent decades (instantaneous risk w1%/ year after 40 years). Estimated survival 50 years after surgery is w46% overall (but 58% for isolated TGA). There have been 184 known deaths (33%). Of the 372 presumed survivors, the median age today would be 36 years (IQ range 30 40). More than 20% of adult Mustards we follow have decompensated heart failure by age 36, and this proportion rises disproportionately thereafter (figure). At last follow-up, prevalence of moderate TR was 20% and RV dysfunction 34%. Nevertheless, follow-up with a cardiologist is known for only 50% (182/372) of presumed survivors since 2010, and 60% (224/372) since 2000. The remaining 148 (40% of survivors) only had last known points of cardiology contact in the 1980s or 1990s. CONCLUSION: The bulk of adult Mustard survivors are approaching high-risk periods for onset of heart failure. The insidious onset of RV dysfunction, TR and pulmonary hypertension emphasizes the need for national tracking registry for this and other complex CHD patients, similar to those for cancer patients, to prevent patients re-presenting with excessive risk for any intervention, including transplantation. Canadian Cardiovascular Society (CCS) Poster HEART FAILURE: IMAGING, TREATMENT AND OUTCOMES Sunday, October 26, 2014

tion with response to heparin. METHODS: Pediatric patients had a comprehensive coagulation panel performed prior to cardiac surgery (antithrombin, protein C/S, coagulation factors II (FII), FVII, FVIII, FIX, FX, FXI, FXIII, fibrinogen, prothrombin time/international normalized ratio (PT/INR) and activated partial thromboplastin time). Previously published, age-appropriate values obtained from normal children were used as reference values across age groups. We also examined the effect of preoperative oxygen saturation and C-reactive protein on blood levels of coagulation factors. The Hemostatic Management System (Medtronic) was used to measure response to the initial heparin dose. RESULTS: Of the 157 enrolled patients, 8%were 10 years old; 31% had a cyanotic condition and 11% had recent heparin exposure. Patients had lower coagulation circulating level activity than expected for age, ranging from 61% to 92% of age-appropriate values (p

BACKGROUND: In tetralogy of Fallot (TOF), the risk of cyanotic spells can lead to emergency surgery like palliative systemicpulmonary shunt before the ideal time to do complete repair. During corrective surgery in case of insufficient development of the pulmonary valve, the use of a trans-annular patch is required which, in the long-term, causes excessive dilatation of the right ventricle, arrhythmia and sudden death. The development of the pulmonary branch and the onset of cyanotic spells are conditioned by the volume of the trans-valvular flow. METHODS: We conducted a retrospective study to determine whether elective primary pulmonary valvuloplasty (EPPV) in the neonatal period 1) allows a better development of the pulmonary valve and artery; 2) reduces the risk of cyanotic spells and the use of emergency surgery; 3) increase the possibilities of conservative surgery of the pulmonary valve. Patients with simple TOF who received an EPPV within the first month of live between 2000 and 2010were compared to an observational group (OBS).Main parameters included progress of O2-saturation, pulmonary valve and artery growth, need for urgent aorto-pulmonary shunt, need for trans-annular patch reconstruction. RESULTS: Of a total of 203 patients identified, 42 were analyzed (23 EPPV and 19 OBS). At birth O2-saturation was 91.52 7.12 respectively vs 94.16% 3.5%; p1⁄4 0.043; The Z score of the pulmonary valve was -3.01 2.12 vs 0.508 1.71 (p1⁄40.66) and Nakata index 115.12 39.67 vs 156.3 43.06 mm2/m2 (p1⁄40.001). At the time surgery the growth of pulmonary annulus was z-score: -3.47 2.09 (p1⁄40.73) vs -3.11 2.43 (p1⁄40.214). Nakata index increased to 174.51 93.74 (p1⁄4 0.57) in EPPV and to 151.19 74.14 (p1⁄40.14) in OBS, this increase was not significant between the 2 groups however (39.59 91.39 vs 4.85 78.34, p1⁄40.214). The need for pulmonary re-intervention after corrective surgery was lower in EPPV 4/22(18%) vs. OBS 6/20(30 %); p1⁄40.04. CONCLUSION: Neonatal EPPV in simple TOF allows appropriate growth of the pulmonary arteries and reduces the rate of re-intervention on the pulmonary arteries after corrective surgery. In this small series, there was no significant difference in the use of a transannular patch or on cyanotic spells.