Research priorities in children and adults with congenital heart disease: a James Lind Alliance Priority Setting Partnership.

OBJECTIVE: To bring together patients, parents, charities and clinicians in a Priority Setting Partnership to establish national clinical priorities for research in children and adults with congenital heart disease. METHODS: The established James Lind Alliance methodology was used to identify and prioritise research on the management of congenital heart disease, focusing on diagnosis, treatment and outcomes. An initial open survey was used to gather potential uncertainties which were filtered, categorised, converted into summary questions and checked against current evidence. In a second survey, respondents identified the unanswered questions most important to them. At two final workshops, patients, parents, charities and healthcare professionals agreed the top 10 lists of priorities for child/antenatal and adult congenital heart disease research. RESULTS: 524 respondents submitted 1373 individual questions, from which 313 out of scope or duplicate questions were removed. The remaining 1060 questions were distilled into summary questions and checked against existing literature, with only three questions deemed entirely answered and removed. 250 respondents completed the child/antenatal survey (56 uncertainties) and 252 completed the adult survey (47 uncertainties). The questions ranked the highest by clinicians and non-clinicians were taken forward to consensus workshops, where two sets of top 10 research priorities were agreed. CONCLUSIONS: Through an established and equitable process, we determined national clinical priorities for congenital heart disease research. These will be taken forward by specific working groups, a national patient and public involvement group, and through the establishment of a UK and Ireland network for collaborative, multicentre clinical trials in congenital heart disease.

Reconstruction of left main coronary artery with subclavian artery free graft in an infant.

We report the case of a 3-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) with absent left main coronary artery (LMCA). She underwent repair by reimplantation technique with the construction of a short LMCA using two opposite flaps. Two months later she was re-admitted in cardiac failure. Occlusion of the reconstructed LMCA was found by angiogram. At reoperation the right subclavian artery was used as a free interposition graft to reconstruct the LMCA. The post-operative course was uneventful. At 8 months she was asymptomatic and LMCA patency was demonstrated by angiogram.

Effect of two different bypass techniques on the serum troponin-T levels in newborns and children: does pH-Stat provide better protection?

BACKGROUND: Cardiac troponin-T is a sensitive marker of myocardial damage. In a prospective study, the effect of 2 different pH strategies during cardiopulmonary bypass on ischemic myocardial injury and clinical outcome was measured in a pediatric population. METHODS AND RESULTS: One hundred one patients (31 neonates 13.2+/-8.3 days and 70 children 34.5+/-44.1 months of age) undergoing open-heart surgery were selected to either alpha-stat (n=51) or pH-stat (n=50) acid-based management protocol. Serum troponin-T levels were measured before and 30 minutes after bypass and then 4 and 24 hours postoperatively. Surgical procedure, bypass details, inotropic support requirement, and postoperative recovery were recorded. Baseline troponin-T level was higher in neonates than in children (0.18+/-0.22 versus 0.04+/-0.05 microg/L, P=0.02). Also, a higher baseline level was found in patients with pulmonary hypertension (0.13+/-0.21 versus 0.04+/-0.05 microg/L, P=0.04). Cyanotic children showed a higher peak troponin-T level (3.76+/-3.11 versus 1.67+/-1.33 microg/L, P=0.04). Peak troponin levels showed a correlation with the length of circulatory arrest and aortic cross-clamp time. Postoperative levels remained high at 24 hours in patients requiring inotropic support. Peak troponin-T levels were significantly lower in the pH-stat group in patients with pulmonary hypertension (P=0.03) and in cases where circulatory arrest (P=0.01) or inotropic support (P=0.01) was necessary during operation than in those with alpha-stat technique. Postoperative ventilation time and length of intensive care unit stay were also significantly longer with alpha-stat than with pH-stat technique (P=0.005 and P=0.006, respectively). CONCLUSIONS: Cardiac troponin-T sensitively reflects myocardial damage in children. Our results suggest that pH-stat acid-based management protocol may provide better protection against ischemic myocardial damage than alpha-stat technique.