Predictive value of bronchoscopy after infant cardiac surgery: a prospective study.

BACKGROUND AND AIMS: Airway evaluation following infant cardiac surgery often reveals evidence of tracheobronchial narrowing. We studied the association between airway narrowing and extubation failure (EF) in this population. METHODS: Prospective cohort study of infants (age ≤6 months) from March-September 2009. Flexible bronchoscopy (FB) evaluations were obtained using a standardised protocol after operative intervention. The primary endpoint was the development of extubation failure (EF; defined as the need for invasive mechanical ventilation ≤48 h after primary extubation) and several secondary endpoints. RESULTS: Fifty-three patients were evaluated at a median age of 81 [interquartile range (IQR) 13-164] days and weight of 4.2 (IQR 3.2-6.0) kg; 13 (25 %) of the patients had single ventricle palliations and two subsequently underwent heart transplantation. Significant airway narrowing was noted in 15 of 30 [50 %, 95 % confidence interval (CI) 31-69 %] patients who underwent FB; ten of the 53 patients (19 %, 95 %CI 10-32 %) subsequently developed EF. Narrowed airway calibre on bronchoscopy had a sensitivity and specificity of 50 % (95 %CI 28-71 %) and 50 % (95 %CI 28-71 %), respectively, for EF. The single greatest predictor of EF by univariate analysis was the need for preoperative ventilation [odds ratio (OR) 6.5, 95 %CI 1.3-33.2, p = 0.03]. Patients with EF had a greater likelihood of intensive care readmission (OR 4.8, 95 %CI 1.1-21, p < 0.04) during the same hospital admission. CONCLUSIONS: Airway narrowing on FB is noted frequently after infant cardiac surgery. Overall assessment and presence of narrowing on bronchoscopy had poor sensitivity and specificity for EF in our cohort. Expert assessment of tracheobronchial narrowing on FB has poor to moderate inter-rater reliability.

ABO-incompatible lung transplantation in an infant.

Waitlist mortality continues to be a limiting factor for all solid-organ transplant programs. Strategies that could improve this situation should be considered. We report the first ABO-incompatible lung transplantation in an infant. The recipient infant was ABO blood group A1 and the donor group B. The recipient was diagnosed with surfactant protein B deficiency, which is a fatal condition and lung transplantation is the only definitive therapy. At 32 days of age, a bilateral lung transplantation from a donation after cardiac death (DCD) donor was performed. Intraoperative plasma exchange was the only preparatory procedure performed. No further interventions were required as the recipient isohemagglutinins were negative before transplant and have remained negative to date. At 6 months posttransplant, the recipient is at home, thriving, with normal development. This outcome suggests that ABO-incompatible lung transplantation is feasible in infants, providing another option to offer life-saving lung transplantation in this age range.

Outcomes of prenatally diagnosed tetralogy of Fallot: Implications for valve-sparing repair versus transannular patch.

OBJECTIVES: To assess outcomes of prenatally diagnosed tetralogy of Fallot and determine factors associated with the choice to undergo a valvesparing repair versus transannular patch, and the use of prostaglandins at birth. METHODS: All cases at The Hospital for Sick Children (Toronto, Ontario) with a fetal diagnosis of tetralogy of Fallot from 1998 to 2006, were reviewed for demographic and fetal echocardiographic data to determine factors associated with the valve-sparing repair and need for perinatal support. RESULTS: Sixty-four fetuses met inclusion criteria (median gestational age 22 weeks) with 47 live births. Twenty-six underwent valve-sparing repair (median age 5.7 months) and 14 underwent transannular patch repair (median age 4.5 months). There were seven deaths before surgery and one post-transannular patch repair. One patient required a transannular patch repair after the initial valve-sparing repair. Twelve of 29 (41%) patients received prostaglandins at birth. Type of surgical repair, use of prostaglandins and postnatal death were among the outcomes investigated. The mean pulmonary valve (PV) z-score was -3.0+/-2.0 and the mean PV/aortic valve (AoV) ratio was 0.65+/-0.10. Lower PV z-score (P=0.04), smaller PV/AoV ratio (P=0.04) and the presence of nonantegrade arterial duct flow (P=0.02) were associated with prostaglandin use. A higher PV/AoV ratio was associated with valvesparing repair (P=0.04). Fetal z-scores of the PV, AoV and right pulmonary artery at 29 to 32 weeks gestational age correlated with respective postnatal z-scores (P=0.01). CONCLUSION: Fetal echocardiographic variables were associated with the use of prostaglandins and valve-sparing repair in fetuses with tetralogy of Fallot, and at 29 weeks, correlated with postnatal valve diameters.

Left ventricular morphology influences mortality after the Norwood operation.

BACKGROUND: Within the spectrum of congenital heart disease referred to as hypoplastic left heart syndrome (HLHS), there is variation in the morphology and function of the left ventricle which could influence outcomes after stage I Norwood palliation. OBJECTIVE: To determine if left ventricular (LV) morphology is associated with outcome after stage I Norwood palliation for HLHS. METHODS: Echocardiograms were reviewed from 108 patients who had undergone Norwood palliation at our institution over the past 11 years. Total cardiac diameter, thickness of the interventricular septum (IVS), LV area and LV myocardial area were calculated. Competing risk analysis was performed for survival to a stage II operation and to determine potential predictors. RESULTS: From the Norwood operation up to stage II operation, mortality was predicted by IVS thickness, while the absence of right ventricular (RV) dysfunction was predictive of survival to stage II operation. For the complete pathway, from Norwood to the Fontan operation, mortality was predicted by IVS, a lower RV fractional area change and the presence of significant tricuspid regurgitation. Cardiac transplantation during this period was predicted by a lower RV fractional area change (p = 0.02) and a larger LV area in diastole. CONCLUSIONS: These results indicate that LV hypertrophy and decreased RV function adversely effect survival after the Norwood operation. They suggest that LV morphology, especially septal hypertrophy, can influence outcomes in HLHS and should be considered when evaluating treatment options.

Stenting of the right ventricular outflow tract in the symptomatic infant with tetralogy of Fallot.

BACKGROUND: Repair of neonatal tetralogy of Fallot (TOF) has low mortality. Debate continues regarding the initial management of cyanotic or duct-dependent infants with TOF and adverse risk factors. While repair can and has been performed in these patients, it is associated with increased morbidity. OBJECTIVE: We review the effectiveness of right ventricular outflow tract (RVOT) stenting in the symptomatic young infant with TOF. METHODS: Clinical, echocardiographic, angiographic and haemodynamic data were reviewed for nine patients who underwent 11 RVOT stenting procedures from October 1994 to August 2007. RESULTS: The pulmonary valve was deemed unsalvageable in all patients (median valve diameter 3.7 mm (range 2.7-4.2), Z-score -6.7 (range -9.7 to -5.4). RVOT stenting improved arterial oxygen saturation from a median of 73% (60-85%) to 94% (90-98%) (p = 0.008). Median Z-score for the left pulmonary artery increased from -4.9 (-7.8 to -2.4) before stent implantation to -1.5 (-4.2 to -0.2) (p = 0.02) before surgical repair. Median Z-score for the right pulmonary artery increased from -3.7 (-6.8 to -1.9) to -0.8 (-2.5 to 0.1) (p = 0.008). Median Nakata index increased from 56 mm(2)/m(2) (21-77) to 150 mm(2)/m(2) (123-231) (p = 0.008). There were no procedural complications. Six patients have undergone successful repair. There were no deaths. CONCLUSIONS: In the symptomatic young infant with TOF, stenting of the RVOT provides a safe and effective management strategy, improving arterial oxygen saturation and encouraging pulmonary artery growth.

Postoperative cardiorespiratory instability following ligation of the preterm ductus arteriosus is related to early need for intervention.

OBJECTIVE: To investigate the effect of postnatal age at patent ductus arteriosus (PDA) ligation on postoperative need for cardiotropic support. STUDY DESIGN: A significant proportion of premature infants with a hemodynamically significant ductus arteriosus (HSDA) require surgical intervention. The relationship of postnatal maturation to postoperative cardiorespiratory stability is poorly understood. All preterm neonates who underwent PDA ligation between October 2002 and September 2004 were identified and divided according to postnatal age at ductal ligation, into early (4 weeks, n=36) groups. Neonatal birth demographics and morbidities, characteristics of the HSDA and reason for ligation were reviewed. Indices of cardiorespiratory stability (for example heart rate, mean arterial pressure) were recorded before and 1, 4, 8, 12 and 24 h following ligation. Details of perioperative cardiovascular interventions (for example crystalloid boluses, cardiotropic agents) were recorded. RESULT: There were no differences in baseline neonatal demographics between the groups although a greater number of babies requiring early ligation had contraindication to indomethacin as the main reason for ligation. Early PDA ligation was associated with increased postoperative cardiotrope requirement (27.6 vs 5.6%, P=0.02) and decreased systolic blood pressure 8 h after intervention (P<0.05). Although increased oxygenation index was observed in both groups during the first 12 h it was only sustained to 24 h in the early group (P<0.05). Age at ligation remained a significant risk factor for postoperative cardiotrope requirement on multiple logistic regression analysis. CONCLUSION: Surgical ligation of the ductus arteriosus in preterm infants less than 28 days old is associated with oxygenation difficulties, decreased systolic arterial pressure and an increased need for cardiotropic support in the initial 24 postoperative hours. These data suggest the need to prospectively characterize perioperative cardiovascular physiology and identify predictive factors of clinical deterioration.

Anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course: clinical profile and approach to management in the pediatric population.

An anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course (interarterial coronary artery [IACA]) is a rare congenital anomaly associated with sudden cardiac death. The cardiac and surgical databases at a single pediatric institution were reviewed for a description of the clinical profiles and associated risk factors of this coronary anomaly. From 1994 to 2006, IACA was diagnosed for 31 patients with a median age of 6.2 years (range, birth to 16 years). The symptoms for 6 (19%) of the 10 patients (32%) presenting with symptoms were deemed to be cardiac in origin. The symptoms for the remaining 21 patients (68%) were incidental findings. Of the 31 patients, 29 (94%) had normal resting electrocardiograms (ECG). A total of 17 patients underwent dobutamine stress echo. None had wall motion abnormalities, but two had ECG changes indicating ischemia, and 4 had abnormal coronary flow, as detected by Doppler echocardiography. Seven patients, with either acute symptoms or testing suggestive of ischemia, underwent surgery. All seven had an interarterial left main coronary artery. There was one cardiac-related death. No sudden death was found in either the surgery or nonsurgery group during the mean follow-up period of 23 and 58 months, respectively. Whether surgical intervention modifies the natural history of the anomaly or not remains to be determined.

Remote ischaemic preconditioning protects against cardiopulmonary bypass-induced tissue injury: a preclinical study.

OBJECTIVES: To test the hypothesis that remote ischaemic preconditioning (rIPC) reduces injury after cardiopulmonary bypass (CPB). DESIGN: Randomised study with an experimental model of CPB (3 h CPB with 2 h of cardioplegic arrest). Twelve 15 kg pigs were randomly assigned to control or rIPC before CPB and followed up for 6 h. INTERVENTION: rIPC was induced by four 5 min cycles of lower limb ischaemia before CPB. MAIN OUTCOME MEASURES: Troponin I, glial protein S-100B, lactate concentrations, load-independent indices (conductance catheter) of systolic and diastolic function, and pulmonary resistance and compliance were measured before and for 6 h after CPB. RESULTS: Troponin I increased after CPB in both groups but during reperfusion the rIPC group had lower concentrations than controls (mean area under the curve -57.3 (SEM 7.3) v 89.0 (11.6) ng.h/ml, p = 0.02). Lactate increased after CPB in both groups but during reperfusion the control group had significantly more prolonged hyperlactataemia (p = 0.04). S-100B did not differ between groups. Indices of ventricular function did not differ. There was a tendency to improved lung compliance (p = 0.07), and pulmonary resistance changed less in the rIPC than in the control group during reperfusion (p = 0.02). Subsequently, peak inspiratory pressure was lower (p = 0.001). CONCLUSION: rIPC significantly attenuated clinically relevant markers of myocardial and pulmonary injury after CPB. Transient limb ischaemia as an rIPC stimulus has potentially important clinical applications.

Assessment of the relationship between cerebral and splanchnic oxygen saturations measured by near-infrared spectroscopy and direct measurements of systemic haemodynamic variables and oxygen transport after the Norwood procedure.

OBJECTIVES: To evaluate the clinical utility of near-infrared spectroscopic (NIRS) monitoring of cerebral (ScO2) and splanchnic (SsO2) oxygen saturations for estimation of systemic oxygen transport after the Norwood procedure. METHODS: ScO2 and SsO2 were measured with NIRS cerebral and thoracolumbar probes (in humans). Respiratory mass spectrometry was used to measure systemic oxygen consumption (O2). Arterial (SaO2), superior vena caval (SvO2) and pulmonary venous oxygen saturations were measured at 2 to 4 h intervals to derive pulmonary (Qp) and systemic blood flow (Qs), systemic oxygen delivery (DO2) and oxygen extraction ratio (ERO2). Mixed linear regression was used to test correlations. A study of 7 pigs after cardiopulmonary bypass (study 1) was followed by a study of 11 children after the Norwood procedure (study 2). RESULTS: Study 1. ScO2 moderately correlated with SvO2, mean arterial pressure, Qs, DO2 and ERO2 (slope 0.30, 0.64. 2.30, 0.017 and -32.5, p < 0.0001) but not with SaO2, arterial oxygen pressure (PaO2), haemoglobin and O2. Study 2. ScO2 correlated well with SvO2, SaO2, PaO2 and mean arterial pressure (slope 0.43, 0.61, 0.99 and 0.52, p < 0.0001) but not with haemoglobin (slope 0.24, p > 0.05). ScO2 correlated weakly with O2 (slope -0.07, p = 0.05) and moderately with Qs, DO2 and ERO2 (slope 3.2, 0.03, -33.2, p < 0.0001). SsO2 showed similar but weaker correlations. CONCLUSIONS: ScO2 and SsO2 may reflect the influence of haemodynamic variables and oxygen transport after the Norwood procedure. However, the interpretation of NIRS data, in terms of both absolute values and trends, is difficult to rely on clinically.

Non-invasive assessment of ventricular force-frequency relations in the univentricular circulation by tissue Doppler echocardiography: a novel method of assessing myocardial performance in congenital heart disease.

OBJECTIVE: To describe the first clinical application of a novel tissue Doppler derived index of contractility, isovolumic acceleration (IVA), in the assessment of the ventricular myocardial force-frequency relation (FFR) in the univentricular heart (UVH). DESIGN: Prospective study. SETTING: Tertiary referral centre. INTERVENTIONS: Non-invasive assessment of the myocardial FFR by tissue Doppler echocardiography during atrial pacing. RESULTS: IVA was used to measure the FFR of the systemic ventricle in patients with structurally normal hearts and in patients with UVHs. Basal IVA of the normal hearts (mean (SD) 1.9 (0.3) m/s2) was significantly greater than that of UVHs in patients with a dominant right ventricle (RV) (1.0 (0.3) m/s2) or left ventricle (LV) (0.8 (0.7) m/s2; p < 0.05 for both). Neither the absolute nor percentage change from basal to peak values of IVA with pacing differed between the three groups. Peak force developed by the normal LV was significantly greater than that of the UVH, dominant LV group but not different from that of the UVH, dominant RV group. CONCLUSION: Contractility at basal heart rate is depressed in patients with UVH compared with the normal LV. Analysis of ventricular FFRs exposes further differences in myocardial contractility. There is no evidence that contractile function of the dominant RV is inferior to that of the dominant LV over a physiological range of heart rates.

Extracardiac conduit versus lateral tunnel cavopulmonary connections at a single institution: impact on outcomes.

OBJECTIVE: To compare outcomes of extracardiac conduit and lateral tunnel Fontan connections in a single institution over a concurrent time period. METHODS: Between January 1994 and September 1998, 60 extracardiac conduit and 47 lateral tunnel total cavopulmonary connections were performed. Age, sex, and weight did not differ between the 2 groups. Compared with the lateral tunnel group (LT group), patients undergoing the extracardiac conduit procedure (EC group) had a trend to a higher incidence of morphologically right ventricle (EC group 48% vs LT group 32%; P <.09), a higher incidence of isomerism/heterotaxy syndrome (EC 22% vs LT 0%; P <.001), worse atrioventricular valve regurgitation (EC 11% moderate-plus vs LT 0%; P <.06), and lower McGoon indices (EC 1.8 +/- 0.5 vs LT 2.1 +/- 0.5; P <.03). Preoperative arrhythmias, transpulmonary gradients, room air oxygen saturations, ejection fractions, ventricular end-diastolic pressure, and pulmonary artery distortion did not differ between groups. Cardiopulmonary bypass times and fenestration usage were similar in both groups. RESULTS: Overall operative mortality was 5.6% and did not differ between groups. The LT group had a significantly higher incidence of postoperative sinoatrial node dysfunction (45% vs EC group 15%; P <.007), supraventricular tachycardia (33% vs EC group 8%; P <.0009), and need for temporary postoperative pacing (32% vs 12%; P <.01). Median duration of intensive care unit stay (EC 2 days, range 1-10 days, vs LT 2.8 days, range 1-103 days; P <.07) and ventilatory support (EC 1 day, range 0.25-10 days, vs LT 1 day, range 0.25-99 days; P <.03) were all longer in the LT group. Median chest tube drainage (EC 8 days, LT 9 days) was similar in both groups. Follow-up averaged 2.5 +/- 1.4 years in the EC group and 2.8 +/- 1.9 years in the LT group. There were 2 late deaths. Overall survival is 94% at 1 month, 92% at 1 year, and 92% at 5 years. Late ejection fraction or atrioventricular valve function did not differ between groups. Intermediate follow-up Holter analysis showed a higher incidence of atrial arrhythmias in the LT group (23% vs 7%; P <.02). Multivariable analysis showed that (1) prolonged cardiopulmonary bypass time was the only independent predictor for perioperative mortality, prolonged ventilation and intensive care unit length of stay, and increased time to final removal of chest tube drains and (2) lateral tunnel Fontan connection is an independent predictor of early postoperative and intermediate atrial arrhythmias. CONCLUSIONS: Although patients in the EC group were at higher preoperative risk, their outcomes were comparable with those of the LT group. Use of the extracardiac conduit technique for the modified Fontan operation reduces the risk of early and midterm atrial arrhythmia.

Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes.

BACKGROUND: The historic outcome of the Fontan procedure in children with single ventricle and heterotaxy syndrome has been poor, and in the current era it has been incompletely described. METHODS: From January 1993 through April 2000, 30 patients (age range, 5.3 +/- 3.6 years) had total cavopulmonary connection for single ventricle and isomerism and heterotaxy syndrome. Right atrial isomerism and heterotaxy (n = 19) or left atrial isomerism and heterotaxy (n = 11) were associated with a morphologic right ventricle (n = 26), a common atrioventricular valve (n = 25), pulmonary atresia (n = 9) or stenosis (n = 17), anomalous pulmonary venous drainage (partial, n = 7; total, n = 11; obstructed, n = 4), and interrupted inferior vena cava (n = 13). A previous systemic to pulmonary artery shunt had been performed in 21 patients, and 3 patients had previous pulmonary artery banding. The Fontan procedure was staged in all but 3 patients at a median age of 12 months (range, 3 to 114 months). Before the Fontan procedure, atrial arrhythmia was present in 7 patients (23%). RESULTS: An extracardiac conduit was constructed in 22 patients (median diameter, 20 mm; range, 16 to 27 mm), and a lateral tunnel was constructed in 4 patients. Associated procedures included pulmonary artery augmentation (n = 17), repair of anomalous pulmonary venous drainage (n = 7), and repair of a regurgitant atrioventricular valve (n = 2). The mean cardiopulmonary bypass time was 134 +/- 57 minutes. Cardioplegic cardiac arrest was used in 12 patients for a mean duration of 62 +/- 28 minutes. There were four hospital deaths (13%), three that occurred in children having concomitant repair of anomalous pulmonary venous drainage (previously undetected in one). Thirteen children (41%) developed early postoperative atrial arrhythmias, 11 of the 13 children required temporary pacing. There was one late death. Follow-up (3.4 +/- 2.7 years) was available on 24 of 25 survivors. All but 2 patients were in sinus rhythm. CONCLUSIONS: Early and midterm outcomes after the Fontan procedure in this patient group improved and may be further ameliorated by detecting and repairing associated obstruction to pulmonary venous flow and performing atrioventricular valvuloplasty before the Fontan procedure.

Evolving strategies and improving outcomes of the modified norwood procedure: a 10-year single-institution experience.

BACKGROUND: This study reviews our 10-year experience with the modified Norwood procedure to determine its early and midterm outcomes. The focus is on the impact of evolving management strategies and accumulated institutional experience. METHODS: A modified Norwood operation was performed in 171 infants over a 10-year period. Sixty-eight percent of the infants were male, the median age at operation was 6 days (range 1 to 175 days), and the median weight was 3.3 kg (range 1.7 to 4.8 kg). The 10-year period was divided into three eras: era I; 1990 through 1993; era II; 1994 through 1997; and era III; 1998 into 2000. Outcomes and risk factors for mortality were sought. RESULTS: Hypoplastic left heart syndrome or a variant was the primary diagnosis in 118 infants (69%). The overall 5-year survival rate was 43%. Multivariate analysis revealed that only need of preoperative ventilatory support, earlier date of operation, and lower weight at operation were significant independent predictors of increased time-related mortality. Morphologic features such as a diagnosis other than hypoplastic left heart syndrome, ascending aortic size, and noncardiac anomalies were not significantly associated with an increased risk of death. The hospital survival rate for stage-one palliation in era III was 82%, significantly better than that in the preceding eras (p < 0.001). Attrition between stages one and two accounted for a 15% mortality rate among hospital survivors. CONCLUSIONS: With increasing experience and improvements in perioperative care and surgical technique, good outcomes can be expected for the first-stage modified Norwood procedure. Greater monitoring of patients in the interstage period may reduce interval mortality and improve overall survival.

Outcomes of intraoperative device closure of muscular ventricular septal defects.

BACKGROUND: The surgical management of muscular ventricular septal defects (mVSD) in the small infant is a challenge particularly when multiple and associated with complex cardiac lesions. Devices for percutaneous implantation have the advantage of ease of placement and for the double umbrella designs a wide area of coverage. We reviewed our experience and clinical outcomes of intraoperative mVSD device closure for such defects in small infants. METHODS: Since October 1989, intraoperative VSD device closure was a component of the surgical strategy in 14 consecutive patient implants (median age, 5.5 months; range, 3 to 11 kg), whose defects were thought difficult to approach using conventional techniques. Nine patients had associated complex cardiac lesions, 10 multiple mVSDs, and 4 patients had a previous pulmonary artery banding. RESULTS: There were 2 early deaths, 1 in a severely ill child who preoperatively had pulmonary hypertension and left ventricular failure and another in a patient with a hypoplastic left heart. Mean pulmonary to systemic flow ratio before device insertion was 3.5:1. Complete closure was achieved in 5 patients and clinically insignificant residual shunts persisted in 7. In 2 infants with significant residual lesions concomitant pulmonary artery banding was required. Postoperative mean pulmonary to systemic flow ratio was 1.7:1. In follow-up of the 12 surviving infants (mean, 41 months), 8 had complete closure and 3 persistent residual shunts. One patient with no residual shunting required heart transplantation for progressive ventricular failure 9 years after operation. All devices were well positioned on postoperative echocardiograms. There was 1 late death due to aspiration in a patient with a tiny residual shunt. CONCLUSIONS: Infants requiring operative intervention with mVSDs are difficult to manage and have an increased mortality and morbidity. Intraoperative VSD device placement for closure of mVSDs is feasible, can avoid ventriculotomy, division of intracardiac muscle bands, and is ideally suited for the neonate or infant.

Total cavopulmonary connections in children with a previous Norwood procedure.

BACKGROUND: Outcomes of the Fontan operation in children initially palliated with the modified Norwood procedure are incompletely defined. METHODS: From August 1993 to January 2000, 45 patients (mean age 2.6 +/- 1.1 years, weight 12.7 +/- 2.8 kg) who were palliated with staged Norwood procedures (hypoplastic left heart syndrome, n = 32; nonhypoplastic left heart syndrome, n = 13) underwent a modified Fontan operation. Preoperative features included moderate/severe atrioventricular valve regurgitation (n = 5, 11%), reduced ventricular function on echocardiography in 11 patients, McGoon index 1.56 +/- 0.38, and pulmonary artery distortion in 18 patients (40%). RESULTS: A lateral tunnel (n = 16) or an extracardiac conduit (n = 29) connection with fenestration in 38 patients (84%) was used. Concomitant procedures included pulmonary artery reconstruction (n = 24, 53%), atrioventricular valve repair (n = 4, 9%) or replacement (n = 1). Before Fontan, 12 patients (27%) had an intervention to address neoaortic obstruction, and 7 patients required balloon dilation/stenting of the left (n = 5) or right pulmonary artery (n = 5). Intraoperatively, left (n = 5) or right pulmonary artery (n = 1) stenting was performed in 5 patients (11%). On follow-up, 8 patients required additional interventional procedures to address left pulmonary artery narrowing (n = 5), or venous (n = 5) or arteriopulmonary collaterals (n = 1). Perioperative mortality was 4.4% (n = 2). There were 2 late deaths at a mean follow-up of 39 +/- 20 months. CONCLUSIONS: In relatively high-risk patients, midterm results of the Fontan operation for children initially palliated with the Norwood procedure were good. Combined interventional-surgical treatment algorithms can lead to improved outcomes.

Pediatric heart transplantation: improving results in high-risk patients.

OBJECTIVES: Our institutional experience with 73 pediatric patients undergoing cardiac transplantation between January 1, 1990, and December 31, 1999, was reviewed to determine the impact of unconventional donor and recipient management protocols implemented to extend the availability of this therapy. METHODS AND RESULTS: The introduction of donor blood cardioplegic solution with added insulin was associated with a significant improvement in patient and graft survival (hazard ratio [Cox] = 0.25, P =.08), despite significantly longer ischemic times with this protocol compared with the use of crystalloid-based donor procurement techniques (P <.01). Eleven patients underwent intentional transplantation of ABO-incompatible donor hearts with the aid of a protocol of plasma exchange on bypass. In this subgroup, there were 2 early deaths caused by nonspecific graft failure (n = 1) and respiratory complications with mild vascular rejection (n = 1), and there was 1 late death caused by lymphoma. ABO-incompatible transplantation was not a risk factor for death by multivariate analysis. The postoperative course in these patients suggests minimal reactivity directed against incompatible grafts on the basis of low anti-donor blood group antibody production, in association with a favorable rejection profile. Ten of 13 patients requiring preoperative support with an extracorporeal membrane oxygenator survived transplantation; there were 3 additional late deaths in this subgroup (hazard ratio = 2.88, P =.05). CONCLUSIONS: The results with pediatric cardiac transplantation continue to improve as a result of changes in both surgical and medical protocols permitting successful treatment of patients conventionally considered at high risk or unsuitable for transplantation.

Independent factors associated with longevity of prosthetic pulmonary valves and valved conduits.

OBJECTIVE: To evaluate the age dependence of variables predictive of pulmonary valve prosthesis replacement, we conducted the following analysis. METHODS: Retrospective analysis of 945 operations in 726 patients undergoing placement of pulmonary valve prostheses was performed. Age was identified as a strong independent predictor of valve failure. The database was stratified into age-based subsets and predictors of valve replacement were identified within each subset. RESULTS: For the entire cohort, freedom from valve replacement at 5 years was 81%. Younger age was strongly associated with decreased time to valve replacement by multivariable analysis (hazard ratio: 0.71/log-year, P <.001). Other independent factors included diagnosis, type of prosthesis, and time-dependent requirement for pulmonary valve stent placement. Important predictors of valve failure varied among age groups and are as follows: for Age Less Than 3 Months: valve type; for Age 3 Months To Less Than 2 Years: smaller normalized valve prosthesis size; for Age 2 Years To Less Than 13 Years: sex, smaller normalized valve prosthesis size, placement of endovascular stents, and valve type; for Age 13 Years To 65 Years: smaller normalized valve prosthesis size, placement of endovascular stents, and increased number of previous valve placements. CONCLUSION: Age is a dominant risk factor predictive of pulmonary valve prosthesis failure. A significant interaction exists between age and the effects of diagnosis, valve type, and size on prosthetic pulmonary valve longevity.

Aortic valve repair for adult congenital heart disease: A 22-year experience.

BACKGROUND: Aortic valve-preserving procedures have resulted in excellent outcomes in selected patients, particularly those with normal aortic valve leaflets and dilated aortic roots. However, several congenital heart lesions are associated with abnormal aortic valve leaflets. The long-term results of aortic valve repair for these lesions are not well defined. METHODS AND RESULTS: We reviewed the clinical records of 54 adult (age >18 years) patients who underwent repair of congenital abnormalities of the aortic valve between 1976 and September 1999. Follow-up data were available on 52 (96%) patients (mean 50+/-67 months, range 1 to 266). Patients underwent repair at a mean age of 34+/-14 years with associated diagnoses of subaortic stenosis (n=10), ventricular septal defect with prolapsing aortic valve (n=17), bicuspid aortic valve (n=23), sinus of Valsalva aneurysm (n=10), and bacterial endocarditis (n=2). There was 1 operative death (1.9%) and 3 late deaths. Survival at 5 and 10 years was 98+/-2% and 74+/-12%, respectively. Freedom from reoperation was 74+/-9% and 51+/-15% at 5 and 10 years, respectively. The presence of a ventricular septal defect predicted failure of valve repair (59% versus 22%, P:=0.01). A bicuspid aortic valve, subaortic stenosis, or the requirement for mitral valve surgery did not affect outcomes. CONCLUSIONS: Aortic valve repair in adult patients with congenital heart disease can be performed with minimal morbidity and mortality rates. The medium-term results of repair are acceptable, regardless of valvular or associated pathology. However, only 31 patients (57%) demonstrated long-term competence of the aortic valve, suggesting that most adult patients with congenital aortic valve disease will eventually require aortic valve replacement.

What is the optimal age for repair of tetralogy of Fallot?

BACKGROUND: Controversy regarding the timing for the repair of tetralogy of Fallot centers around initial palliation versus primary repair for the symptomatic neonate/young infant and the optimal age for repair of the asymptomatic child. We changed our approach from one of initial palliation in the infant to one of primary repair around the age of 6 months, or earlier if clinically indicated. We examined the effects of this change in protocol and age on outcomes. METHODS AND RESULTS: The records of 227 consecutive children who had repair of isolated tetralogy of Fallot from January 1993 to June 1998 were reviewed. The median age of repair by year fell from 17 to 8 months (P:<0.01). The presence of a palliative shunt at the time of repair decreased from 38% to 0% (P:<0.01). Mortality (6 deaths, 2. 6%) improved with time (P:=0.02), with no mortality since the change in protocol (late 1995/early 1996). Multivariate analysis for physiological outcomes of time to lactate clearance, ventilation hours, and length of stay, but not death, demonstrated that an age <3 months was independently associated with prolongation of times (P:<0.03). Each of the deaths occurred with primary repair at an age >12 months. The best survival and physiological outcomes were achieved with primary repair in children aged 3 to 11 months. CONCLUSIONS: On the basis of mortality and physiological outcomes, the optimal age for elective repair of tetralogy of Fallot is 3 to 11 months of age.

Are bilateral superior vena cavae a risk factor for single ventricle palliation?

BACKGROUND: Performing superior vena cava-to-pulmonary artery anastomosis, in the presence of bilateral superior vena cavae, can be technically challenging. Our clinical observation has been that bilateral superior vena cavae are a risk factor for poor outcome in children needing single ventricle palliation. METHODS: Detailed operative, angiographic, and follow-up data were analyzed in 39 children undergoing bilateral cavopulmonary anastomosis (b-CPA). Overall outcome was compared to 274 children having a unilateral cavopulmonary anastomoses (u-CPA). RESULTS: Nine patients (23%) with bilateral superior vena cavae were found to have thrombus in the cavopulmonary circulation after the b-CPA. Postoperative mean arterial oxygen saturation was significantly lower in those who had thrombus [69%+/-10% versus 82%+/-7%, (p < 0.01)]. Thrombus formation was associated with mortality. The indexed superior vena cavae size was not a risk factor for thrombosis. In follow-up studies the connecting pulmonary artery segment between the two cavopulmonary anastomosis was smaller than the pulmonary arteries adjacent to the hilum. Survivors of a b-CPA were less frequently converted to a Fontan circulation at 5 years of follow up (Kaplan-Meier 5-year estimates, 39% for b-CPA versus 74% for u-CPA [p = 0.02]). CONCLUSIONS: Bilateral superior vena cava-to-pulmonary artery anastomosis is associated with an increased risk of thrombus formation and unfavorable growth in the central pulmonary arteries. Modifications of surgical technique may alter flow patterns, thereby optimizing growth and diminishing the risk of thrombus formation. Anticoagulation therapy may be an important adjunct in children undergoing a b-CPA.