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.
The authors present the first report of an ABO‐incompatible lung transplantation in an infant.
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.
We review the effectiveness of right ventricular outflow tract (RVOT) stenting in the symptomatic young infant with TOF.
Clinical, echocardiographic, angiographic and haemodynamic data were reviewed for nine patients who underwent 11 RVOT stenting procedures from October 1994 to August 2007.
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.
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.
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.
Congenital absence of the pericardium (CAP) is a rare clinical entity.
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).
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.
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.
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.
To determine if left ventricular (LV) morphology is associated with outcome after stage I Norwood palliation for HLHS.
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.
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.
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.
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 (V̇o2). 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 V̇o2. 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 V̇o2 (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.
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. (J Thorac Cardiovasc Surg 2000;120:1022-31)
The purpose of this study is to determine the incidence, risk factors, and outcomes for chylothorax in children undergoing cardiothoracic surgery.
Hospital databases were used to identify chylothorax ...cases. Surgical databases were used to identify all patients undergoing cardiothoracic surgery. Medical records were reviewed, including daily records of drainage volumes and management.
From September 2000 to December 2002, there were 48 cases of chylothorax in 1,257 surgeries—an incidence of 3.8% (95% confidence interval: 2.8% to 4.8%). Overall mortality rate was similar, but cases had longer postoperative hospital stays (median, 22 versus 8 days; p < 0.001). Incidence of chylothorax was significantly higher with heart transplantation and Fontan procedures. Diagnosis was made at a median of 6 days after surgery. Duration of drainage was a median of 15 days, with 11 patients draining more than 30 days. Longer duration of drainage was associated with cavopulmonary anastomosis procedures and longer time to diagnosis of chylothorax. Nutritional management included low fat diet, enteral feeds enriched with medium-chain triglycerides, and parenteral nutrition. Five patients were treated with octreotide, 4 with thoracic duct ligation, and 1 with pleurodesis. Octreotide was associated with a variable effect on drainage. Thoracic duct ligation reduced, but did not stop drainage.
Chylothorax increases duration of hospitalization after cardiovascular surgery in children. Early diagnosis may reduce the duration of chylothorax. Nutritional strategies remain the cornerstone for management of postoperative chylothorax. The impact of octreotide and surgical intervention is limited when reserved for patients with severe or prolonged drainage.
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.
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.
PDF
