Aims After tetralogy of Fallot (TOF) repair patients have right ventricular (RV) dysfunction and reduced exercise tolerance. Diastolic dysfunction may be important but is as yet poorly characterized. The early diastolic strain rate (SR) is a measure of ventricular relaxation, and may be useful to assess diastolic mechanics in TOF. We hypothesized that children after TOF repair have diastolic dysfunction and dyssynchrony by this measure, and sought to determine their relationship with pulmonary regurgitation (PR), RV enlargement, and aerobic exercise capacity. Methods and results We prospectively recruited asymptomatic children after TOF repair. RV and PR volumes were measured by magnetic resonance imaging; Doppler and tissue Doppler indices by echocardiography and RV and left ventricular (LV) early diastolic SR by two-dimensional speckle tracking. Exercise peak oxygen consumption (VO₂) was determined using bicycle ergometry. Results were compared with healthy controls. We studied 53 TOF patients and 49 age-matched controls. TOF patients had significant PR (2.05 ± 1 L/m²) with moderate RV dilatation (157 ± 39 mL/m²), low-normal RV ejection fraction (49 ± 8.8%), and moderate QRS prolongation (141 ± 23 ms). The RV outflow gradient was 21.7 ± 16.0 mmHg. Patients had RV diastolic dysfunction vs. controls [reduced tricuspid valve (TV) E/A ratio, E′ velocity, and longitudinal diastolic SR; increased right atrial volume and TV E/E′ ratio]. LV early diastolic radial and circumferential SR were lower in TOF patients in association with more PR [parameter estimate (PE) 0.177 standard error (SE) (0.08) mL/m², P = 0.02] and higher RV volumes [(PE) 0.005 (0.002)mL/m², P = 0.01]. Diastolic dyssynchrony was not different in TOF patients vs. controls. Conclusion TOF patients have RV and LV diastolic dysfunction associated with RV enlargement and reduced early filling. SR imaging may be useful to quantify early myocardial diastolic dysfunction in these children.

Although ABO blood group incompatible cardiac transplantation in neonates and infants reduces waiting list mortality without compromising outcome, the technique has not been adopted by all centers, and to date Toronto remains the only center to have published results from a large case series. We present a review of ABO-incompatible heart transplantation in the United Kingdom (UK) where current recipient selection criteria differ somewhat from those used in the United States (US) and Canada. Between February 2000 and November 2006, 21 ABO-incompatible cardiac transplants were performed in children aged 2-40 months (median 10.0). Immunosuppression followed standard regimens. Pretransplant donor-specific isohemagglutinins of >1:4, (the UNOS cutoff), were present in five patients and reduced by plasma exchange. After transplantation, 19/21 recipients demonstrated persisting deficiency of donor-specific isohemagglutinins. Significant donor-specific isohemagglutinins levels were detected repeatedly in 2/21 recipients who have shown no clinical or biopsy evidence of rejection. All recipients survive without retransplantation and there have been no episodes of humoral rejection. We conclude it is possible for other centers to replicate the excellent results achieved in Toronto and that ABO-incompatible transplantation may be performed successfully in some patients beyond infancy with established isohemagglutinin production providing preoperative antibody removing strategies are used.