Preoperative Venovenous ECMO in an Infant With Late Diagnosis of Hypoplastic Left Heart Syndrome.

Late diagnosis and late referral for the Norwood stage 1 procedure in patients with hypoplastic left heart syndrome is rare and associated with a higher mortality. We present a case of a cyanotic almost five-week-old infant with hypoplastic left heart syndrome, highly restrictive foramen ovale, and patent ductus arteriosus, who was bridged with venovenous extracorporeal membrane oxygenation to the Norwood stage 1 procedure.

Early postoperative extubation of unstable patients following total cavopulmonary connection: impact on circulation and outcome.

OBJECTIVES: We aimed to investigate whether early postoperative extubation following the Fontan operation is universally feasible and can be used as a management tool in unstable patients. METHODS: All patients undergoing the Fontan operation in our centre between 2004 and 2013 (n=253) were analysed. Until 2008, patients were extubated according to standard criteria and comprised group 1. Group 2 included all patients presenting after 2009, when early extubation was always aimed regardless of the haemodynamic status. Patients who exceeded the 75th percentiles for volume requirements and inotrope scores for the respective group were defined as unstable. Comparisons of outcomes between groups and subgroups and analysis of the changes in haemodynamic and treatment parameters with extubation in unstable patients after 2009 were performed. RESULTS: Compared with group 1, patients from group 2 were ventilated for shorter duration (p<0.001), had similar re-intubation rates (p=0.50), and needed less volume (p=0.01). In group 2, the unstable patients were not ventilated for longer durations (p=0.19), but had higher re-intubation rates (p=0.03) than the stable patients. Compared with the unstable patients from group 1, the unstable patients from group 2 were ventilated for shorter duration (p<0.001), had similar re-intubation rates (p=0.66), and needed less volume (p=0.006). There was a significant acute and sustained increase in mean arterial pressure with extubation and a parallel reduction in volume requirements and inotrope scores in the unstable patients from group 2. CONCLUSIONS: Timely extubation is universally applicable following the Fontan operation. Early postoperative extubation can be valuable for improving Fontan haemodynamics.

A Rare Anomaly of a Single Coronary Artery Arising From the Right Pulmonary Artery in a Neonate.

An anomalous origin of the entire coronary circulation from the right pulmonary artery is a very rare congenital malformation. We report on a male newborn presenting with hypoplastic aortic arch and coarctation of the aorta in whom a previously undetected single coronary artery originating from the right pulmonary artery was detected at the time of corrective surgery. The patient underwent a resection of the coarctation with a patch enlargement of the aortic arch and a successful reimplantation of the coronary artery into the aorta.

Huge coronary fistula from the left anterior descending coronary artery to the right ventricle in a newborn: a rare congenital anomaly.

We present a case of a neonate with a huge congenital fistula from the left anterior descending coronary artery to the right ventricle and additional heart defects. The circumflex coronary artery was supplied by the right coronary artery. The fistula was closed surgically at both ends.

Replacement of the systemic atrioventricular valve with a mechanical prosthesis in children aged less than 6 years: late clinical results of survival and subsequent replacement.

OBJECTIVE: We analyzed the survival, clinical course, and role of prosthesis-patient mismatch after systemic atrioventricular valve replacement in children. METHODS: From 1974 to 2006, 69 patients underwent systemic atrioventricular valve replacement (median age 1.2 years, range 1.1 months to 5.4 years), with 17 patients requiring re-replacement of the systemic atrioventricular valve. Prosthesis-patient relationship was analyzed by comparing (1) the prosthetic valve diameter and the predicted annulus diameter based on the body surface area and (2) the prosthetic valve diameter and the measured annulus diameter. RESULTS: Survival was 73% at 1 year and 65% at 5, 10, and 15 years. Age, weight, body surface area, predicted annulus diameter, prior surgery, underlying disease, and ratio of prosthetic valve diameter to body weight were significant predictors of death. Variables associated with re-replacement of the systemic atrioventricular valve were body surface area, prosthetic valve diameter, predicted annulus diameter, and presence of multiple left-sided obstructive lesions. The majority of patients received a prosthesis larger than the predicted annulus diameter. There was good correlation between the prosthetic valve diameter and the measured annulus diameter (r = 0.85). Mismatch, as described by the difference in z scores of prosthetic valve diameter and measured annulus diameter, was not a significant predictor of death or re-replacement of the systemic atrioventricular valve. CONCLUSIONS: Although valve replacement is considered the last therapeutic option after failed attempts of valvuloplasty, long-term outcome is favorable. Selection of the prosthesis is made on the basis of the measured annulus diameter. An elevated ratio of prosthetic valve diameter to body weight is associated with patients with low body weight or a large native annulus in dilated ventricles.