Outcome following augmentation of superior cavopulmonary blood flow with an arteriovenous fistula.

BACKGROUND: Axillary or brachial arteriovenous fistulae are occasionally created to improve systemic oxygen saturation in patients with functionally univentricular circulation after superior cavopulmonary anastomosis. Different techniques of fistula construction may account for the conflicting results. We sought to determine the impact of axillary arteriovenous fistula on systemic oxygen saturation and survival before subsequent palliation or heart transplantation. METHODS: Medical records of patients who underwent a side-to-side anastomosis between an adjacent axillary artery and vein for cyanosis after superior cavopulmonary anastomosis between 1991 and 2010 were reviewed. RESULTS: The median age (n = 23) at the time of fistula creation was 54 months (4-278 months). There was no operative mortality. The median duration of fistula patency was 48 months (1-174 months). Oxygen saturation improved from 71% ± 10% preoperatively to 84% ± 4% early (P < .05) and 81% ± 4% long term after the arteriovenous fistula (P < .05). Twelve patients underwent additional surgery (total cavopulmonary anastomosis 6, partial biventricular repair 2, and heart transplantation 4) after an interval of 10 to 141 months. Six patients are alive with a patent fistula without subsequent interventions after an interval of 48 to 174 months. Two patients with spontaneous closure or device closure of the fistula are alive after an interval of 76 to 80 months without subsequent interventions. Three deaths occurred with a patent fistula without subsequent interventions after an interval of 1 to 84 months. CONCLUSIONS: Systemic oxygen saturation improved with creation of an axillary arteriovenous fistula after superior cavopulmonary anastomosis. The fistula extended the duration of this stage of palliation without precluding the option for additional palliation or heart transplantation.

Single ventricle palliation: greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone.

BACKGROUND: This study was performed to evaluate and compare the early, intermediate, and long-term outcomes of the bidirectional Glenn procedure and Fontan procedure in patients who live at moderately high altitude. METHODS: The outcome of each method of palliation for patients with a functionally single ventricle was retrospectively evaluated from a review of medical records. RESULTS: The bidirectional Glenn procedure was performed in 177 patients from October 1984 to June 2004. The Fontan procedure was performed in 149 patients from June 1978 to June 2004. Cardiovascular death or heart transplantation occurred in 8% of patients after the bidirectional Glenn procedure and 17% of patients after the Fontan procedure. Complications of systemic thromboembolic events, bleeding associated with anticoagulation therapy, protein losing enteropathy, and arrhythmias requiring implantation of a pacemaker, cardioversion, or radiofrequency ablation occurred in 7% of patients after the bidirectional Glenn procedure and 47% of patients after the Fontan procedure. Cardiovascular deaths and heart transplantation occurred less frequently when the Fontan procedure was performed in patients with a previous bidirectional Glenn procedure. However, the actuarial transplant-free survival and freedom from complications was not superior for a subgroup of patients who had a Fontan procedure after a bidirectional Glenn procedure in comparison to a subgroup of patients who had a bidirectional Glenn procedure alone. CONCLUSIONS: The bidirectional Glenn procedure can be used for long-term palliation of patients with a functionally single ventricle. Additional palliation with a Fontan procedure may increase the risk of stroke, protein losing enteropathy and arrhythmias without improving survival.

Current status of surgery for rheumatic carditis in children.

BACKGROUND: The incidence of rheumatic heart disease (RHD) has increased recently in the western United States. We reviewed our 18-year surgical experience with RHD in children to examine current surgical techniques and results. METHODS: From 1985 until 2003, 596 children (<21 years) with rheumatic fever were seen at Primary Children's Medical Center. Rheumatic carditis was diagnosed in 366 patients (61.4%). Twenty-six with carditis (26/366, 7.1%) required operation for rheumatic valve disease including 8 for mitral regurgitation, 7 for mitral and aortic regurgitation, 4 for aortic regurgitation, 4 for mitral regurgitation and stenosis, 2 for combined mitral stenosis and regurgitation with aortic insufficiency, and 1 for mitral and tricuspid regurgitation. RESULTS: Mean age at operation was 13.5 +/- 4 years. Three patients required operation during the acute phase of rheumatic fever (< 6 weeks), 2 during the subacute phase (< 6 months), and 21 during the chronic phase after the episode of rheumatic fever (6.7 +/- 3 years). Mitral valve repair was possible in 19 of 22 patients who required mitral operation. Aortic valve repair was possible in 4 patients whereas replacement was necessary in 9, including 2 Ross procedures. No operative deaths were recorded and 2 late deaths occurred at 4.6 and 10 years. Actuarial survival was 94% at 5 years and 78% at 10 years. Six patients required reoperation; actuarial freedom from reoperation was 78% at 5 years, 65% at 10 years, and 49% at 15 years. All survivors are in New York Heart Association class I or II. CONCLUSIONS: Children with RHD in the United States uncommonly require valve operation. Mitral repair with a technique that allows annular growth is possible in most children with good long-term functional results. Long-term surveillance of children with RHD is necessary because of the possible need for late valve operation.

Current results with intraaortic balloon pumping in infants and children.

BACKGROUND: Intraaortic balloon pumping (IABP) is useful for support in patients with moderate left ventricular dysfunction. IABP is usually timed with the R wave of the electrocardiogram. We have utilized M-mode echocardiography timed IABP in children with left-side heart failure since 1994. Electrocardiogram timing seems inappropriate for children, who have much higher heart rates. We describe our experience with children who underwent IABP therapy before and after 1994, when echocardiographic timing was instituted. METHODS: We reviewed records of 29 children who underwent IABP for all indications at Primary Children's Medical Center since 1988. RESULTS: Overall survival was 62.1% (18 of 29) in this series. Survival was similar for infants (odds ratio = 2.0, 95% confidence interval = 0.29 to 14.31, p = 0.43) and older children. Survival was similar in the echocardiography era when compared with the electrocardiogram era (odds ratio = 2.4, 95% confidence interval = 0.56 to 10.4, p = 0.44). CONCLUSIONS: IABP is a useful means of support in children with left ventricular dysfunction. M-mode echocardiography is effective in triggering IABP. The sample size in this study is too small to detect a mortality rate difference.