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.

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.

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.

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.

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.

Interventions associated with minimal fontan mortality.

BACKGROUND: The operative mortality rate for the first 400 Fontan procedures at this institution was 15% but declined to 4% for the next 100 procedures. METHODS: The cases of 100 consecutive patients receiving the Fontan procedure and associated with this change in mortality rate were reviewed to determine associations. RESULTS: The mortality rate in the first and second 50 patients was 16% and 0%, respectively. There were no differences in age, number of risk factors, diagnosis, or operating surgeon between the two groups. Patients in the lower-mortality era were significantly more likely to have had a cavopulmonary anastomosis before a Fontan procedure (90% versus 70%) and to have an extracardiac Fontan procedure (38% versus 8%), shorter cross-clamp (45+/-24 minutes versus 58+/-22 minutes) and cardiopulmonary bypass times (121+/-42 minutes versus 141+/-45 minutes), magnesium-rich cardioplegia (100% versus 39%), hemoconcentration after bypass (67% versus 4%), and institution of pharmacologic support in the operating room. CONCLUSIONS: Patient characteristics and risk factors were similar in the two groups. However, several interventions that were increasingly utilized in the lower-mortality era, including the extracardiac Fontan procedure and modified ultrafiltration after bypass, are associated with lower mortality. Each one had the potential to improve postoperative myocardial function.

A novel repair for patients with atrioventricular septal defect requiring reoperation for left atrioventricular valve regurgitation.

OBJECTIVE: Left atrioventricular valve regurgitation (LAVVR) is the most frequent indication for reoperation following atrioventricular septal defect (AVSD) repair. We estimate from our experience that within 10 years of initial repair, 14% of patients undergoing repair of atrioventricular septal defect (AVSD) require reoperation for this complication. We have developed a novel leaflet augmentation technique for LAVVR which may avoid failure of conventional repair and/or the need for valve replacement. METHOD: The novel technique consists of insertion of a glutaraldehyde-treated autologous pericardial patch to augment the bridging leaflets of the atrioventricular valve. We describe the outcome of eight patients in whom this technique was used and compared them to 68 other patients with AVSD undergoing reoperation for LAVVR by either conventional repair (n=54) or valve replacement (n=14). RESULTS: There were no early deaths or major complications following patch repair. The mean follow-up is 2.3 years (range 1-8.5 years) during which there were no late deaths. Two patients underwent reintervention at 3.5 and 5 years after patch repair for LAVVR and were successfully rerepaired. Mild residual LAVVR was seen at last echocardiography in six patients and mild to moderate in two. These results compare favorably with the 68 patients who underwent conventional surgery. The 3-year freedom from reoperation was 86% for both repair groups. Dysplastic valve tissue appears to be a major risk factor for failure of conventional repair or for valve replacement. Failure of conventional valve repair led to valve replacement in six of seven patients. CONCLUSIONS: For patients with late LAVVR after AVSD repair, pericardial leaflet augmentation is durable and may avoid failure of conventional repair or valve replacement in patients with dysplastic valves.

Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.

BACKGROUND: Congenital absence of the pericardium (CAP) is a rare clinical entity. METHODS: We identified from the two hospital databases all patients with isolated CAP, reviewed their data, and invited them for prospective clinical evaluation with electrocardiography, chest x-ray findings (CXR), echocardiography, and magnetic resonance imaging (MRI). RESULTS: Ten patients (3 males, 7 females) presented at a median age of 21 years (range, 2-53 years) with paroxysmal stabbing chest pain, largely nonexertional (9), and heart murmur with an abnormal CXR (1). Three patients had partial and 7 had complete CAP (all 7 had marked lateral displacement of the cardiac apex). CXR combined with MRI were key to establishing the diagnosis; a "tongue" of lung tissue interposing between the main pulmonary artery and aorta was the most consistent diagnostic feature. Four patients underwent pericardioplasty, 3 for debilitating symptoms and 1 for left atrial appendage herniation, followed by improvement or resolution of symptoms. At a mean of 10.5 years from presentation all patients were alive. No complications were seen in the nonsurgical group. CONCLUSIONS: Isolated CAP has a common presentation pattern with periodic stabbing chest pain mimicking coronary artery disease. CXR and MRI are required for definitive diagnosis. Symptomatic patients with the complete form may benefit from pericardioplasty.

Does the degree of cyanosis affect myocardial adenosine triphosphate levels and function in children undergoing surgical procedures for congenital heart disease?

OBJECTIVE: The outcome of children with cyanosis after cardiac surgical procedures is inferior to that of children who are acyanotic. Animal studies indicated detrimental effects of chronic hypoxia on myocardial metabolism and function. We studied whether the presence or the degree of cyanosis adversely affected myocardial adenosine triphosphate, ventricular function, and clinical outcome in children. METHODS: Forty-eight children who underwent repair of tetralogy of Fallot were divided according to their preoperative saturation: group I, 90% to 100% (n = 14 patients); group II, 80% to 89% (n = 16 patients); and group III, 65% to 79% (n = 18 patients). Adenosine triphosphate was measured from right ventricular biopsy specimens taken before ischemia, at 15 minutes of ischemia, at end-ischemia, and at 15 minutes of reperfusion. Ejection fraction was measured by echocardiography. RESULTS: Even before surgical ischemia, compared with groups I and II, group III had lower preoperative ejection fraction (59% +/- 2.9% vs 67% +/- 1.7% and 68% +/- 1.0%; P <.01) and lower preischemic adenosine triphosphate levels (15.1 +/- 2.1 vs 19.1 +/- 1.9 and 21.4 +/- 1.5 micromol/g dry weight; P <.01). After 15 minutes of ischemia, group III had lower adenosine triphosphate levels (11.2 +/- 1.8 vs 14.77 +/- 2.3 and 17. 6 +/- 3.1 micromol/g dry weight; P <.01). With reperfusion, both cyanotic groups lost further adenosine triphosphate compared with partial recovery in the acyanotic group (-22% +/- 3.8%, -20% +/- 3. 1% vs +18% +/- 1.8%; P <.01). Children in group III had a more complicated postoperative course as evidenced by longer ventilatory support (85 +/- 25 hours vs 31 +/- 15 and 40 +/- 21 hours; P =.07), inotropic support (86 +/- 23 hours vs 38 +/- 12 and 36 +/- 4 hours; P <.01), and intensive care unit stay (160 +/- 35 hours vs 60 +/- 10 and 82 +/- 18 hours; P =.02). CONCLUSIONS: The degree of cyanosis adversely affects myocardial adenosine triphosphate, function, and clinical outcome of children who undergo cardiac operation. Children with cyanosis should be identified as a higher risk group that could be targeted for supportive interventions.

Precoronary stenosis after stage I palliation for hypoplastic left heart syndrome.

We report a patient with stenosis of the native ascending aorta after palliation of hypoplastic left heart syndrome and aortic atresia. We describe the approach to diagnosis, temporary support with extracorporeal membrane oxygenation, and successful surgical reintervention. Stenosis of the native ascending aorta is an important, potentially reversible cause of acute, early postoperative ventricular dysfunction.

Equivalent survival following cavopulmonary shunt: with or without the Fontan procedure.

OBJECTIVES: In 1992, an analysis of our experience with the cavopulmonary shunt (CPS) demonstrated equivalent long-term survival, with or without subsequent conversion to a Fontan circulation. Before 1992 (era 1) intervention was deferred until mandated by clinical deterioration. Since 1992 (era 2), timing of both CPS and Fontan was compressed in an effort to improve survival. Survival following CPS is analyzed to ascertain whether Fontan confers any survival advantage over no further definitive intervention. METHODS: From 1962 to 1997 inclusive, 490 patients underwent CPS, excluding those who had a CPS concomitant with a Fontan. In 55 patients the CPS was performed at or after a biventricular repair (BVR), or after a Fontan, and these patients are excluded. The 435 patients remaining followed a surgical protocol which included a subsequent BVR (n = 28), or a subsequent Fontan operation (n = 220), or no further definitive surgery (CPS only, n = 187). Between eras the mean age at surgery decreased for all procedures. RESULTS: Long-term survival 20 years after a CPS in 435 patients is 56 +/- 5%. Survival at 20 years among the 220 patients who were subsequently converted to a Fontan circulation is 65 +/- 8% compared to 50 +/- 11% for the 187 patients who did not have a Fontan. However, most of their survival difference is because all early deaths after a CPS occurred in the non-Fontan group. Multivariable analysis demonstrated that proceeding to a Fontan did have a small survival advantage which was not evident by univariate analysis. Independent risk factors for death, at any time, are a common atrioventricular valve, or pulmonary artery banding. The era had no effect on survival. CONCLUSIONS: The single ventricle circulation appears to have a limited durability of, an average, 30-40 years. There is a slight survival advantage in converting patients after a CPS to a Fontan circulation. A marked reduction in age at CPS and at Fontan has, as yet, not improved survival.