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Introduction: Extracorporeal membrane oxygenation (ECMO) is an adaptation of conventional cardiopulmonary bypass techniques used for long-term support of respiratory and/or cardiac function. It provides physiologic cardiopulmonary support for patients with acute, reversible cardiac or respiratory failure. The term “extracorporeal life support” (ECLS) was proposed to describe prolonged but temporary (1–30 days) support of heart or lung function using mechanical devices. Technically, ECMO terminology is used for modalities that provide pulmonary support system involving oxygenation and carbon dioxide removal, and ECLS is used for both cardiac and pulmonary support systems, but these terminologies are still used interchangeably. 1,2 Over the past 30 years, due to significant advances in the understanding of physiology, improvement in clinical care, innovation of novel therapies for primary diseases, and technological advances, there have been major changes in indications, cannulation, duration of treatment, ...

Arterial cannulation is routinely performed in children undergoing cardiac surgery to aid the intraoperative and intensive care management. Most commonly cannulated peripheral site in children is radial artery, and alternatives include posterior tibial, dorsalis pedis, and rarely superficial temporal artery (STA). Two specific situations in cardiac surgery where STA cannulation and monitoring was useful during the surgical procedure are reported. To our knowledge, such selective use of STA pressure monitoring has not been reported in the literature previously. Our experience suggests that STA monitoring can be useful and reliable during repair of coarctation of aorta or administration of anterograde cerebral perfusion in patients having associated aberrant origin of the right subclavian artery.

Objective:Theidealvalvesubstitutein childrendoesnot exist.Biologicandbioprosthetic valvesdo not requireanticoagulation, howevertheir use is complicated by accelerated degeneration and requirement for reoperation. We examine results following mitral (MVR) or aortic (AVR) replacement with biologic and bioprosthetic valves at our institution. Methods:Medical records of children who underwentAVRor MVR from 1986 to 2006 were reviewed. Median follow-up duration was 10.5 years. Competing-risks methodology determined time-related prevalence and associated factors for three mutually exclusive end states: death, valve reoperation, and survival without subsequent reoperation. Results: One hundred and ten children (age 15.6 2.6 years, 80% females) underwent 123 valve replacements with biologic and bioprosthetic substitutes including 87 MVR and 36 AVR (13 had both). Underlying pathology was mainly rheumatic fever (91%). Thirty-nine patients (35%) had undergone a previous cardiac surgery. Most common mitral substitute was Hancock (73%) and homograft (8%); most common aortic substitute was homograft (41%) and Carpentier—Edwards (39%). Competing-risks analysis showed that 15 years after valve replacement, 16% of patients had died without subsequent reoperation, 66% underwent valve reoperations, and only 18% remained alive without further reoperation. Factors associated with increased reoperation risk included younger age at surgery (p = 0.005), AVR (p = 0.005), male gender (p = 0.02) and homograft use (p = 0.007) especially in the mitral position (p = 0.002). Fifteen-year freedom from endocarditis was 97% while freedom from bleeding and thrombo-embolic complications was 100%. Majority of patients (95%) were in NYHA functional classes I/II at last follow-up. Conclusion: While valve reoperation is inevitable following AVR and MVR with biologic and bioprosthetic substitutes; favorable results such as low valve-related morbidity rate, good long-term survival and functional status encourage their consideration as valid replacement alternatives in selected children especially females. Valve durability is higher in the mitral position and longevity of bioprosthetic valves is greater than that of homografts especially in the mitral position.

Optimal repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) relies on the creation of a dual-coronary system. If the anomalous coronary arises at a long distance from the aorta, we use various coronary extension techniques to facilitate tension-free implantation.Thirty patients underwent ALCAPA operations using direct coronary transfer (n = 11) or coronary extension techniques (n = 19). Surgical outcomes were analyzed.Median age and weight were 5.7 months (range, 46 days to 5.45 years) and 5.35 kg (range, 3.3 to 15.9 kg). Five patients had concomitant mitral annuloplasty. Mean cardiopulmonary bypass and ischemic times were 108 +/- 38 and 57 +/- 25 minutes. Two patients required intraoperative revision of the implantation. There were three hospital deaths (10%) and no late deaths. Follow-up echocardiograms demonstrated significant improvement postoperatively vs preoperatively in shortening fraction (35% +/- 2% vs 16% +/- 2%, p0.00001), ejection fraction (64% +/- 3% vs 32% +/- 4%, p0.00001), and mitral regurgitation (11% moderate vs 70% moderate or severe, p = 0.0002). Left ventricular end-diastolic dimension Z-score decreased from 9.1 +/- 0.9 to 1.2 +/- 0.5 (p0.00001). Both techniques were equally effective. Two patients underwent reoperation 1 and 12 years postoperatively (coronary artery bypass grafting, 1; mitral repair with coronary angioplasty, 1). Surviving patients remain asymptomatic (p0.00001).Dual-coronary system can be established in patients with ALCAPA. Coronary extension implantation techniques have acceptable operative mortality and excellent cardiac recovery and late survival. Although the rate of late coronary occlusion is low, continual ventricular or mitral dysfunction should trigger evaluation of persistent coronary compromise.

The anesthetic implications of Joubert syndrome in an infant who required cardiac surgery using cardiopulmonary bypass (CPB) is presented. Children with Joubert syndrome present with central apnea due to malformations in the midbrain and cerebellum. These patients have a marked sensitivity to opioids. The use of dexmedetomidine along with remifentanil was effective in this case.

The management of an infant who underwent corrective surgery for a supracardiac total anomalous pulmonary venous drainage, which was complicated by the development of pulmonary hemorrhage while separating from cardiopulmonary bypass, is presented. Initiation of high-frequency oscillatory ventilation was effective in weaning the patient off cardiopulmonary bypass.

Objectives: We report results of left atrioventricular valve reoperations (LAVVR) following atri- oventricular septal defect (AVSD) repair and examine variables predictive of outcome. Methods: Multiple demographics and operative variables were analyzed to determine factors affecting survival and reopera- tion. Results: Forty patients following partial (n = 9) or complete (n = 31) AVSD repair underwent 47 LAVVR (1992-2005). Median age was 0.87 years (24 days-7.7 years) at initial AVSD repair and 3.15 years (84 days-13.6 years) at subsequent LAVVR with median interval between AVSD repair and LAVVR of 1.76 years (1 day- 12.9 years). First LAVVR included repair (n = 20) or replacement (n = 20). Operative mortality was 10% and five-year survival was 76 ± 6%. Significant risk factors were complete AVSD (p < 0.001), valve replacement (p < 0.001) for early death, and young age at time of LAVVR (p = 0.03) for late death. Five-year freedom from LAVV re-intervention was 100% for replacement versus 55 ± 13% for repair (p = 0.006). Overall, ejection fraction increased to 61 ± 3% versus 42 ± 2% preoperatively (p < 0.01), and left-ventricle end-diastolic dimension Z-score decreased to 0.05 ± 0.36 versus 3.1 ± 0.3 preoperatively (p < 0.01). Eighty-seven percent of children were in New York Heart Association class I/II at latest follow-up. Conclusions: LAVVR results in significant clinical improvement and lasting recovery in ventricular chamber function and size. Valve repair offers survival advantage and should be aggressively attempted; however, it is only achievable in 50% of cases. Valve replacement is necessary in cases associated with complex LAVV morphology or following repair failure. At intermediate follow-up, patients continue to be at risk of major valve-related morbidity, requirement for re-intervention, and cardiac death. doi: 10.1111/j.1540-8191.2008.00784.x (J Card Surg ****;**:**-**)

Objective: There is lack of information regarding the long-term behavior of aortic valve reconstruction with pericardium (AoR). A 16-year follow-up is reported here. Methods: Between 1988 and 1995, 92 consecutive patients had AoR with bovine (Group I, nZ27) or glutaraldehydetreated autologous pericardium (Group II, nZ65). The mean age was 30 years (range 12–68). There were 65% males, 92% in sinus rhythm, 84% had rheumatic etiology and 36% had ‘other valve’ surgery. Mitral valve replacement with a mechanical prosthesis is a contraindication to the operation. Results: Hospital mortality was 2%. The reconstructed aortic valve performed well with excellent hemodynamics. The mean followup interval was 10.5G4 years, range 9–16 years (longer for group I,12 versus 10 years) with 4% late deaths and seven patients lost to follow-up. Survival rate was 85G4%. There were no episodes of thromboembolism. Freedom from reoperation for the whole group was 68G5% at 10 years and 47G6% at 16 years. For group I, it was 68G9% at 10 years and 48G10% at 16 years, while for group II it was 72G6 and 45G8% at 10 and 15 years, respectively. Excluding endocarditis (one in group I and seven in group II) and ‘other’ reasons for reoperation (two in group I and three in group II), the freedom from structural valve degeneration (SVD) at 10 and 16 years was 78G1 and 55G10% for group I. For group II, it was 80G5% at 10 years and 58G9% at 15 years. The mean interval at which the valve degenerated was 8.8 yearsG3.6 and did not differ between the two groups. Conclusions: AoR is feasible with good hemodynamics, low mortality and thromboembolic rate. Its behavior at 10 years is comparable to that of stentless aortic valve bioprosthesis. It can be performed with either xenopericardium or glutaraldehyde-treated autologous pericardium, but the latter has the advantage of being inexpensive and readily available. Q 2005 Elsevier B.V. All rights reserved.

Objective We aimed to identify characteristics differentiating children undergoing aortic valve replacement by using mechanical prostheses versus the Ross procedure and to compare survival and the need for aortic valve reoperation after each procedure. Methods From 1983 to 2004, 346 children underwent aortic valve replacement (215 underwent the Ross procedure and 131 underwent placement of a mechanical prosthesis). Factors associated with procedure choice were used to construct a propensity score for use as a covariate in regression models to adjust for potential confounding by indication. Results Patients undergoing the Ross procedure were younger, more likely to have a congenital cause, and less likely to have a rheumatic or connective tissue cause. They had a lower frequency of regurgitation, required more annular enlargement, and had less concomitant cardiac surgery. Competing-risk analysis showed that 16 years after aortic valve replacement, 20% of patients had died without subsequent aortic valve replacement, 25% underwent second aortic valve replacement, and 55% remained alive without further replacement. After propensity adjustment, factors associated with early-phase death included mechanical valves and a nonrheumatic cause. Mechanical valves were also associated with constant-phase mortality. Repeated aortic valve replacement was associated with the Ross procedure and a rheumatic cause. Both factors were also associated with all-cause cardiac reoperation. In children receiving mechanical prostheses, younger age and smaller valve size were significant risk factors for death. Freedom from homograft replacement after the Ross procedure was 82% at 16 years of follow-up. Conclusion Results from this study showed good outcomes and an acceptable complication rate with both valve choices. Given the significantly increased risk of early and late death in younger children receiving smaller mechanical valves, the Ross procedure confers survival advantage in this age group at the expense of increased reoperation risk, especially in patients with a rheumatic cause.

ObjectiveWe examined outcomes after mitral valve replacement in children younger than 8 years.MethodsMedical records of patients who underwent mitral valve replacement from 1990 to 2006 were reviewed. Competing-risks methodology determined time-related prevalence and associated factors for death, repeated valve replacement, and survival without reoperation.ResultsIn total, 79 patients, median age 24 months (40 days–8 years) underwent 91 mitral valve replacements (10 had repeated procedures). Underlying pathology was congenital heart disease in 95% of cases. Forty-six patients (58%) had undergone previous operations. Operative mortality was 18%, 30% for those 2-years old and younger and 6% for those older than 2 years. Competing-risks analysis showed that 10 years after initial mitral valve replacement, 40% of patients had died without repeated replacement, 20% had undergone a second replacement, and 40% remained alive without further replacement. Factors associated with death included higher prosthesis size/patient weight ratio (P < .0001) and longer crossclamp time (P < .0001). Second replacement 6 ± 4 years after initial replacement was necessary for 10 survivors. At second replacement, larger prostheses were implanted (mean 24 mm vs 19 mm initially). Repeated MVR was associated with younger age at surgery (p = .006). Permanent pacemaker implantation was eventually needed by 11% of hospital survivors.ConclusionsMortality and repeated valve replacement are common after mitral valve replacement in children younger than 8 years, especially younger patients with significantly oversized valves. At valve reoperation, larger prostheses could be implanted, suggesting continued annular growth.