Anticoagulant regimens in Fontan patients varied widely with a significant trend for warfarin use in patients with impaired haemodynamics. Low arterial oxygenation may predict haemostatic events. The relatively high prevalence of haemorrhagic complications indicates the need for individualized anticoagulant administration throughout the follow-up.
Circulation Journal Official Journal of the Japanese Circulation Society http://www. j-circ.or.jp our decades have passed since the first Fontan operation was performed in a patient with tricuspid atresia 1 and since then a lot of patients with congenital heart disease who were not suitable for a biventricular repair have enjoyed the benefits of this procedure. The lack of a pulmonary ventricle forced us to rely on an elevated central venous pressure (CVP) and the sucking property of the systemic ventricle to maintain the pulmonary circulation. However, these inadequate compensatory adaptations lead to a diminished preload for the systemic ventricle, resulting in a low cardiac output (CO) and, therefore, chronically elevated CVP and low CO characterize the Fontan circulation. 2 In general, we categorize Fontan patients with a low CVP and high cardiac index (CI) as "good" in terms of the hemodynamics. However, the definition of a low or high CI remains unknown. The other important clinical characteristic of the Fontan patients are a high prevalence of postoperative complications, 3 such as arrhythmias, 4 re-intervention, and non-cardiac lesions that include protein-losing enteropathy. 5-7 Thus, long-term Fontan survivors without those complications can also be categorized as those with a good clinical status. We thought that our ongoing management strategy of seeking a better long-term outcome based on serial comprehensive assessments, including cardiac catheterization and cardiopulmonary exercise testing (CPX), would enable us to clarify clinically and hemodynamically the long-term good Fontan survivors. 8 Accordingly, our purpose for the present study was to identify the long-term clinically (ie, no clinical events) and hemodynamically good Fontan survivors, to characterize the hemodynamics, and finally to determine the clinical predictors of long-term good Fontan survivors.
A 2-month-old infant with congestive heart failure was referred to the authors' hospital. Echocardiography exhibited a dilated left ventricle (LV), poor LV systolic function, and intraventricular thrombus. Laboratory data showed a normal creatinine phosphokinase level and negative troponin T test results. The congestive heart failure was managed using a beta-blocker, an angiotensin receptor blocker, and diuretics. Head computed tomography performed during the treatment course showed periventricular calcifications. Congenital cytomegalovirus infection was subsequently diagnosed. Fetal echocardiography performed during pregnancy showed impaired LV function, suggesting that the cardiomyopathy was associated with cytomegalovirus infection in utero.
To assess left (LV) and nght ventnmlar (R V) systolic and diastolic function in small animab (mouse and rat) we developed a field-by-field angiogaphic analysk progam. Interlaced video images were acquired at constant radiopaphic technique and digitized at 512x512, 8 bit, 30 frames/s resolution. Fields were separated to improve the temporal resolution. Field-byfield video-density curves for L V and R V were derived.Beat averaging was performed after wrrecting for vaqing contrast between beats. The following parameters were measured: 1) heart rate, 2) ejection fraction, 3) end diastolic volumes 4) stroke volume, 5) cardiac output, 6) mean systolic ejection rate, 7) peak ejection rate, 8) peak filLig rate, 9) time to peak f"llling and 10) time to peak ejection rate.
Aims
Left ventricular apical pacing (LVAP) has been reported to preserve left ventricular (LV) function in chronically paced children with complete atrioventricular block (CAVB). We sought to evaluate long-term feasibility of LVAP and the effect on LV mechanics and exercise capacity as compared to normal controls.
Methods and results
Thirty-six consecutive paediatric patients with CAVB and LVAP in the absence (N = 22) or presence of repaired structural heart disease (N = 14, systemic LV in all) and 25 age-matched normal controls were cross-sectionally studied after a median of 3.9 (interquartile range 2.1–6.8) years of pacing using echocardiography and exercise stress testing. Pacemaker implantation was uneventful and there was no death. Probability of the absence of pacemaker-related surgical revision (elective generator replacement excluded) was 89.0% at 5 years after implantation. Left ventricular apical pacing patients had lower maximum oxygen uptake (P = 0.009), no septal to lateral but significant apical to basal LV mechanical delay (P < 0.001) which correlated with decreased LV contraction efficiency (P = 0.001). Left ventricular ejection fraction and global longitudinal LV strain were, however, not different from controls. Results were similar in both the presence and absence of structural heart disease.
Conclusion
Left ventricular apical pacing is technically feasible with a low reintervention rate. Mechanical synchrony between LV septum and free wall is maintained at the price of an apical to basal mechanical delay associated with LV contraction inefficiency as compared to healthy controls. Global LV systolic function is, however, not negatively affected by LVAP.
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