ABSTRACT
Organ transplantation from ABO blood group-incompatible (ABOi) donors requires accurate detection, effective removal and subsequent surveillance of antidonor antibodies. Because ABH antigen subtypes are expressed differently in various cells and organs, measurement of antibodies specific for the antigen subtypes in the graft is essential. Erythrocyte agglutination, the century-old assay used clinically, does not discriminate subtype-specific ABO antibodies and provides limited information on antibody isotypes. We designed and created an ABO-glycan microarray and demonstrated the precise assessment of both the presence and, importantly, the absence of donor-specific antibodies in an international study of pediatric heart transplant patients. Specific IgM, IgG, and IgA isotype antibodies to nonself ABH subtypes were detected in control participants and recipients of ABO-compatible transplants. Conversely, in children who received ABOi transplants, antibodies specific for A subtype II and/or B subtype II antigens-the only ABH antigen subtypes expressed in heart tissue-were absent, demonstrating the fine specificity of B cell tolerance to donor/graft blood group antigens. In contrast to the hemagglutination assay, the ABO-glycan microarray allows detailed characterization of donor-specific antibodies necessary for effective transplant management, representing a major step forward in precise ABO antibody detection.
Subject(s)
ABO Blood-Group System/immunology , Blood Group Incompatibility/immunology , Heart Transplantation , Immune Tolerance/immunology , Isoantibodies/immunology , Polysaccharides/immunology , B-Lymphocytes/immunology , Case-Control Studies , Child , Child, Preschool , Female , Follow-Up Studies , Graft Survival/immunology , Humans , Infant , Infant, Newborn , Male , Microarray Analysis , PrognosisABSTRACT
The purpose of this study was to expand the American Academy of Pediatrics' (AAP) car safety seat testing recommendation to include high-risk infants following cardiac surgery. Car safety seat testing (< or =4 days prior to discharge) was retrospectively reviewed for 66 postoperative infants. Car safety seat testing was performed according to AAP guidelines. Failure of the test was defined as the occurrence of apnea, bradycardia, or oxygen desaturation. Average birth weight was 3.1 +/- 0.5 kg. Two patients were born <37 weeks of gestation. Surgical procedures included modified Blalock-Taussig shunt (15), arterial switch operation (12), Norwood-Sano modification (11), coarctation repair (8), repair of tetralogy of Fallot (6), repair of truncus arteriosus (4), repair of total anomalous pulmonary venous return (3), pacemaker (2), repair of interrupted aortic arch and ventricular septal defect (VSD) (1), repair of coarctation/VSD (1), orthotopic heart transplant (1), repair of VSD (1), and patent ductus arteriosus ligation (1). Average age at discharge was 28 +/- 21 days. Four patients (6%) failed car safety seat testing secondary to a decrease in oxygen saturation. One of four passed on retesting after parental education; three of 4 (75%) were discharged home in a supine car safety seat. There was no relationship between the type of surgery and car safety seat test failure. It may be beneficial to extend the AAP recommendations for car safety seat testing to include this high-risk patient population.
Subject(s)
Apnea/prevention & control , Bradycardia/prevention & control , Cardiac Surgical Procedures , Equipment Safety/standards , Heart Defects, Congenital/surgery , Infant Equipment/standards , Patient Discharge , Apnea/etiology , Bradycardia/etiology , Gestational Age , Guidelines as Topic , Humans , Hypoxia/blood , Hypoxia/etiology , Hypoxia/prevention & control , Infant , Infant Equipment/adverse effects , Infant, Newborn , Postoperative Period , Retrospective Studies , Risk Assessment/methods , Supine Position/physiologyABSTRACT
In adults, pulmonary value replacement (PVR) shows improvement in right ventricular (RV) volume and function and reduces QRS duration. In addition, RV volume correlates with QRS duration and QRS change. This has not been shown in pediatric patients. The purpose of this study was to evaluate serial magnetic resonance imaging (MRI) and electrocardiogram measurements before and after early PVR in a pediatric population with repaired Tetralogy of Fallot and whether QRS duration and QRS change correlated with RV volume. A retrospective review of MRIs and electrocardiograms was conducted on 10 patients. Median age at repair was 2.1 +/- 0.7 years, and median age at PVR was 11.5 +/- 2.0 years. There were significant decreases in RV end diastolic volume (EDV)/body surface area (BSA) (p < 0.0004), end systolic volume (ESV)/BSA (p = 0.02), RVEDV/left ventricular (LV) EDV (p < 0.001), RV ejection fraction (p < 0.04), RV stroke volume (SV)/BSA (p < 0.0002), and (RVSV - LVSV)/BSA (p = 0.0007). No significant change in QRS duration occurred (p = 0.08). QRS duration (pre-r = 0.44, p = 0.20; post-r = 0.34, p = 0.33) and QRS change (r = -0.08, p = 0.83) did not correlate with RVEDV. We propose early consideration of PVR in pediatric patients. PVR improves RV volumes and function and may provide beneficial electromechanical effects by slowing the progression of QRS duration.
Subject(s)
Electrocardiography , Heart Conduction System/physiopathology , Heart Valve Prosthesis Implantation , Pulmonary Valve/surgery , Tetralogy of Fallot/surgery , Ventricular Function, Right , Child , Child, Preschool , Hemodynamics , Humans , Infant , Magnetic Resonance Imaging , Postoperative Period , Retrospective Studies , Tetralogy of Fallot/physiopathologySubject(s)
Apnea/etiology , Atrial Appendage/abnormalities , Heart Defects, Congenital/diagnosis , Hernia/congenital , Image Processing, Computer-Assisted , Imaging, Three-Dimensional , Magnetic Resonance Imaging , Pericardium/abnormalities , Syncope/etiology , Atrial Appendage/pathology , Child , Diagnosis, Differential , Heart Defects, Congenital/surgery , Hernia/diagnosis , Herniorrhaphy , Humans , MaleSubject(s)
Heart Atria/abnormalities , Heart Defects, Congenital/surgery , Heart Septal Defects, Atrial/surgery , Vena Cava, Inferior/abnormalities , Bronchopulmonary Dysplasia/diagnosis , Cardiac Catheterization , Child , Comorbidity , Cyanosis/etiology , Echocardiography , Follow-Up Studies , Heart Defects, Congenital/diagnosis , Heart Septal Defects, Atrial/diagnosis , Humans , Infant, Newborn , Male , PhlebographyABSTRACT
We report the use of nesiritide in two infants following cardiac surgery. Both infants had increased intracardiac filling pressures postoperatively, despite traditional afterload reduction and diuretics. Both infants demonstrated clinical improvement with nesiritide therapy. There is currently limited data available describing the use of nesiritide in infants.
Subject(s)
Cardiac Surgical Procedures , Natriuretic Agents/therapeutic use , Natriuretic Peptide, Brain/therapeutic use , Aortic Coarctation/surgery , Aortic Valve Stenosis/congenital , Aortic Valve Stenosis/surgery , Blood Pressure/drug effects , Cardiac Surgical Procedures/adverse effects , Heart Septal Defects, Ventricular/surgery , Humans , Infant , Mitral Valve Insufficiency/congenital , Mitral Valve Insufficiency/surgery , Stroke Volume/drug effects , Ventricular Pressure/drug effectsABSTRACT
BACKGROUND: Significant tricuspid valve regurgitation (TR) occurs with other congenital heart defects, typically after repair of right-sided obstructive lesions. Since 1991, we applied the De Vega tricuspid annuloplasty technique for TR in children. METHODS: Forty-one children, aged 5 months to 22.7 years (mean, 9.9 years) underwent 42 De Vega tricuspid annuloplasties for moderate or severe TR during correction of other heart defects. One child had a De Vega during primary ventricular septal defect repair. The remaining patients had prior repair of tetralogy of Fallot or pulmonary atresia, or both (19 patients), double-outlet right ventricle (6 patients), pulmonary stenosis (4 patients), pulmonary atresia and intact ventricular septum (3 patients), complete atrioventricular septal defect (3 patients), and other diagnoses (6 patients). At the time of the De Vega, 37 patients (88%) had pulmonary valve replacement or right ventricular to pulmonary artery conduit replacement. Other procedures included aortic or mitral repair or replacement (6 patients), atrial septal defect and ventricular septal defect closure (5 patients), pulmonary arterioplasty (6 patients), and tracheoplasty (1 patient). RESULTS: There were no deaths at follow-up of 3.4 +/- 2.1 years; 1 child required cardiac transplantation 17 months postoperatively. Early postrepair echocardiography quantified TR as absent or mild (34 patients; 81%), mild-to-moderate (4 patients), moderate (3 patients), and severe (1 patient). The most recent echocardiogram showed moderate TR in 11 patients and severe TR in 2 patients (both with recurrent right ventricular hypertension). One child required tricuspid valve replacement 3 years later and 1 child had redo De Vega at the time of conduit re-replacement. No other child has symptomatic TR, significant tricuspid stenosis, or De Vega-related pacemaker implantation. CONCLUSIONS: The De Vega tricuspid annuloplasty safely provides excellent relief of TR, usually in children undergoing pulmonary valve replacement or conduit replacement. Although echocardiographic TR tends to increase with time (especially with right ventricular hypertension), it rarely requires reintervention or causes symptoms.
Subject(s)
Heart Defects, Congenital/surgery , Tricuspid Valve Insufficiency/surgery , Tricuspid Valve/surgery , Adolescent , Adult , Child , Child, Preschool , Female , Follow-Up Studies , Heart Defects, Congenital/physiopathology , Heart Valve Prosthesis Implantation , Hemodynamics/physiology , Humans , Infant , Male , Postoperative Complications/physiopathology , Reoperation , Suture Techniques , Tricuspid Valve/physiopathology , Tricuspid Valve Insufficiency/physiopathologyABSTRACT
BACKGROUND: Historically, warfarin has been discontinued or rapidly reversed with fresh frozen plasma in patients awaiting heart transplantation because of concerns regarding excessive bleeding. Because preoperative warfarin may have effects on bleeding after cardiac operations, we reviewed our experience to determine the risks in patients undergoing heart transplantation while maintained on warfarin. METHODS: The records of consecutive adult patients undergoing heart transplantation from January 1996 to December 1998 were reviewed. Preoperative and 24-hour postoperative data were obtained, including patient demographics; hematologic laboratory values; medication use; repeat or primary sternotomy data; allogeneic blood product administration; and chest tube drainage. Multivariate linear and logistic regression analyses were performed using these variables to determine risk factors for bleeding after heart transplantation. RESULTS: Ninety adult patients, mean age 50 years, underwent orthotopic heart transplantation during the 36-month period. No relationships existed between preoperative international normalized ratio (INR, mean = 1.83 +/- 0.1, p = 0.84) or postoperative INR (mean = 2.2 +/- 0.9, p = 0.63) and chest tube drainage (mean = 721 +/- 63 mL). Relationships were observed between total blood product administration and preoperative INR (partial r = 0.30, p = 0.01) and postoperative INR (partial r = -0.37, p = 0.002); however, preoperative INR did not correlate (p = 0.29) when perioperative use of fresh frozen plasma was factored as a covariate. Inverse relationships were evident between postoperative INR and total blood product exposures, as well as transfusions of platelets (partial r = -0.26, p = 0.03), fresh frozen plasma (partial r = -0.28, p = 0.02), and red cells (partial r = -0.25, p = 0.04). CONCLUSIONS: Although we noted no correlations between INR and chest tube output, inverse relationships were observed with transfusion requirements in the first 24 hours after transplantation. Preoperative warfarin may be safely continued in patients awaiting heart transplantation.
Subject(s)
Heart Transplantation , Postoperative Hemorrhage/chemically induced , Warfarin/adverse effects , Adult , Aged , Aged, 80 and over , Aspirin/adverse effects , Aspirin/therapeutic use , Blood Transfusion , Chest Tubes , Humans , International Normalized Ratio , Logistic Models , Middle Aged , Multivariate Analysis , Platelet Count , Postoperative Care , Preoperative Care , ROC Curve , Risk Factors , Warfarin/administration & dosageABSTRACT
OBJECTIVE: To evaluate the outcome of aortic root augmentation by the Konno-aortoventriculoplasty technique as part of reoperative aortic valve replacement. METHODS: Since 1983, 15 patients, 12 males and three females, had repeat aortic valve replacement (AVR) with concomitant Konno aortoventriculoplasty. Age ranged from 1.2 to 18 years (mean 12.5 years). The underlying anatomic diagnoses were valve and subvalvar aortic stenosis in 11, truncal valve insufficiency in one, endocarditis in one, Shone's complex in one and severe aortic insufficiency associated with a ventricular septal defect in one patient. All patients had had previous AVR. The causes for reoperation were prosthetic valve stenosis due to growth in ten and paravalvular leak in one, homograft failure in two, xenograft failure in one, and left ventricular outflow tract obstruction (LVOTO) after mitral valve replacement in one patient. The mean size of explanted prostheses was 19.2 mm (13-23 mm) while the mean size of the implanted prostheses was 24.3 mm (19-27 mm) (P<0.01). Previous aortic root enlargement had been performed in 11 patients in conjunction with AVR. The Manougian technique was used previously in two, Konno aortoventriculoplasty in eight, and both techniques in one patient. The newly implanted aortic valves were a homograft in one patient and mechanical prostheses in 14 patients. RESULTS: There was one operative death (1 of 15 or 6.6%) in a 17.5 year old patient with previous AVR and posterior root enlargement, due to low cardiac output state. Follow-up ranged from 6 months to 17 years (mean 7.2 years). The only late death occurred in an 11.6-year-old patient due to prosthetic valve endocarditis. Two patients had complete heart block and had permanent pacemaker insertion (2 of 15 or 13.3%). One patient had pulmonary valve replacement because of combined stenosis and insufficiency 5 years after operation. All 13-surviving patients are asymptomatic at latest follow up. CONCLUSION: Konno aortoventriculoplasty with repeat AVR may be safely performed. Excellent results may be achieved despite previous aortic root enlargement. It is a good surgical option for complex LVOTO and may even reduce reoperation in children by allowing placement of a larger prosthesis.
Subject(s)
Aortic Valve/surgery , Ventricular Outflow Obstruction/surgery , Adolescent , Child , Child, Preschool , Female , Follow-Up Studies , Heart Valve Prosthesis , Humans , Infant , Male , Reoperation , Transplantation, Homologous , Treatment Outcome , Ventricular Outflow Obstruction/etiologyABSTRACT
BACKGROUND: Management of hypoplastic aortic arch associated with coarctation in infancy can be challenging. Reverse subclavian flap aortoplasty plus coarctation resection offers simplicity without needing foreign material or cardiopulmonary bypass. METHODS: Since 1988, 46 of 162 infants less than 3 months undergoing coarctation repair had hypoplastic arch enlargement with reverse subclavian flap aortoplasty. Median age was 11 days; mean weight was 3.2 kg. Thirty-seven patients (80%) had associated cardiac defects including single or multiple ventricular septal defects (14 infants), transposition of the great arteries (7), aortic or mitral stenosis (5), and complete atrioventricular septal defect (5 infants). Twenty-eight patients had pulmonary artery banding; 2 had an arterial switch operation through a separate median sternotomy. RESULTS: There were two hospital deaths: one 4 months postoperatively in a patient requiring a Norwood procedure the next day for underestimated left ventricular hypoplasia; the other of sepsis more than 1 month postoperatively. On follow-up from 1 to 129 months (mean, 38 months), there were five recurrent obstructions: three at the coarctation site treated with balloon dilatation and two at the arch site. Twenty-six children had their heart defects corrected with 29 subsequent operations including an arterial switch operation for transposition of the great arteries/ ventricular septal defect (3 infants), relief of aortic or mitral stenosis +/- ventricular septal defect closure (5), multiple ventricular septal defect closure (3), a bidirectional Glenn (2), complete atrioventricular septal defect (2), and anomalous left coronary with ventricular septal defect repair (1 infant). Four children await debanding and ventricular septal defect closure or Glenn anastomosis. There have been two late deaths (overall survival, 91%). CONCLUSIONS: Reverse subclavian flap aortoplasty is excellent for relief of arch hypoplasia and coarctation in infants with low recurrence rates and acceptable operative and intermediate survival.
Subject(s)
Aorta, Thoracic/abnormalities , Aortic Coarctation/surgery , Subclavian Artery/transplantation , Surgical Flaps , Aorta, Thoracic/diagnostic imaging , Aorta, Thoracic/surgery , Aortic Coarctation/diagnostic imaging , Aortic Coarctation/mortality , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Male , Postoperative Complications/diagnostic imaging , Radiography , Survival Rate , Suture TechniquesABSTRACT
BACKGROUND: Management of newborns with interrupted aortic arch (IAA) remains challenging. Associated severe left ventricular outflow tract obstruction (LVOTO) have often led to increased mortality with neonatal biventricular repair. We review our experience with an alternative approach for this complex surgical problem. METHODS: From May 1991 to June 1999, 28 neonates were treated for IAA. Thirteen of 28 neonates (46%) had type B IAA, ventricular septal defect (VSD) and severe LVOTO (Z value -2 to -7; mean -5 +/- 1.7). Mean age was 8 days (3 to 23 days old) with average weight of 3.3 kg (2.4 to 4.2 kg). Eight of 13 (62%) had anomalous right subclavian artery. Ten of 13 (77%) had thymic aplasia and chromosome 22 region qll deletion. All 13 patients were treated initially with a modified Norwood procedure. RESULTS: There were no perioperative deaths. Complications included 2 patients with recurrent arch stenosis treated with balloon dilatation. Two patients had systemic arterial shunt revision. Follow-up ranged from 2 to 99 months old (mean 39 months). There were 2 late deaths unrelated to any operation. Nine of 12 patients had a second stage palliation consisting of a bidirectional Glenn shunt. Six patients went on to have biventricular repairs (3 Ross-Konno, 2 Rastelli, 1 VSD closure with LVOT resection). One patient had a modified Fontan operation and 5 patients are awaiting potential biventricular repair. CONCLUSIONS: Children with IAA and severe LVOTO may be managed by initial Norwood palliation with an excellent outcome likely. This initial "univentricular" approach has enabled eventual successful biventricular repair despite severe LVOTO.
Subject(s)
Aortic Coarctation/surgery , Heart Ventricles/surgery , Postoperative Complications/surgery , Ventricular Outflow Obstruction/surgery , Aortic Coarctation/mortality , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Male , Postoperative Complications/mortality , Reoperation , Survival Rate , Ventricular Outflow Obstruction/mortalityABSTRACT
BACKGROUND: The total cavopulmonary connection (TCPC) design continues to be refined on the basis of flow analysis at the connection site. These refinements are of importance for myocardial energy conservation in the univentricular supported circulation. In vivo magnetic resonance phase contrast imaging provides semiquantitative flow visualization information. The purpose of this study was to understand the in vivo TCPC flow characteristics obtained by magnetic resonance phase contrast imaging and compare the results with our previous in vitro TCPC flow experiments in an effort to further refine TCPC surgical design. METHODS: Twelve patients with TCPC underwent sedated three-dimensional, multislice magnetic resonance phase contrast imaging. Seven patients had intraatrial lateral tunnel TCPC and 5 had extracardiac TCPC. RESULTS: In all patients in both groups a disordered flow pattern was observed in the inferior caval portion of the TCPC. Flow at the TCPC site appeared to be determined by connection geometry, being streamlined at the superior vena cava-pulmonary junction when the superior vena cava was offset and flared toward the left pulmonary artery. Without caval offset, intense swirling and dominance of superior vena caval flow was observed. In TCPC with bilateral superior vena cavae, the flow patterns observed included secondary vortices, a central stagnation point, and influx of the superior vena cava flow into the inferior caval conduit. A comparative analysis of in vivo flow and our previous in vitro flow data from glass model prototypes of TCPC demonstrated significant similarities in flow disturbances. Three-dimensional magnetic resonance phase contrast imaging in multiple coronal planes enabled a comprehensive semiquantitative flow analysis. The data are presented in traditional instantaneous images and in animated format for interactive display of the flow dynamics. CONCLUSIONS: Flow in the inferior caval portion of the TCPC is disordered, and the TCPC geometry determines flow characteristics.
Subject(s)
Heart Bypass, Right , Heart Defects, Congenital/pathology , Heart Defects, Congenital/surgery , Magnetic Resonance Imaging , Adolescent , Blood Flow Velocity , Child , Child, Preschool , Heart Defects, Congenital/physiopathology , Humans , Magnetic Resonance Imaging/methodsABSTRACT
Symptomatic vascular rings are uncommon in adults. We report a case of dysphagia in a 48-year-old woman from a right-sided aortic arch with aberrant left subclavian artery and left-sided ligamentum arteriosum. Through a left posterolateral thoracotomy, the ligamentum was divided, relieving the patient's dysphagia. Twenty-three cases of adult-onset dysphagia lusoria attributable to this anatomy have been reported. We recommend a left thoracotomy with division of the ligamentum as treatment.
Subject(s)
Deglutition Disorders/etiology , Esophageal Stenosis/etiology , Subclavian Artery/abnormalities , Aortography , Deglutition Disorders/diagnostic imaging , Deglutition Disorders/surgery , Diagnosis, Differential , Esophageal Stenosis/diagnostic imaging , Esophageal Stenosis/surgery , Female , Humans , Ligaments/abnormalities , Ligaments/diagnostic imaging , Ligaments/surgery , Middle Aged , Subclavian Artery/diagnostic imaging , Subclavian Artery/surgeryABSTRACT
BACKGROUND: Historically, panel reactive antibody (PRA) analysis to detect HLA antibodies has been performed using cell-based complement-dependent cytotoxicity (CDC) techniques. Recently, a flow cytometric procedure (FlowPRA) was introduced as an alternative approach to detect HLA antibodies. The flow methodology, using a solid phase matrix to which soluble HLA class I or class II antigens are attached is significantly more sensitive than CDC assays. However, the clinical relevance of antibodies detected exclusively by FlowPRAhas not been established. In this study of cardiac allograft recipients, FlowPRA was performed on pretransplant sera with no detectable PRA activity as assessed by CDC assays. FlowPRA antibody activity was then correlated with clinical outcome. METHODS: PRA analysis by anti-human globulin enhanced (AHG) CDC and FlowPRA was performed on sera corresponding to final cross-match specimens from 219 cardiac allograft recipients. In addition, sera collected 3-6 months posttransplant from 91 patients were evaluated. The presence or absence of antibodies was correlated with episodes of rejection and patient survival. A rejection episode was considered to have occurred based on treatment with antirejection medication and/or histology. RESULTS: By CDC, 12 patients (5.5%) had pretransplant PRA >10%. In contrast, 72 patients (32.9%) had pretransplant anti-HLA antibodies detectable by FlowPRA (34 patients with only class I antibodies; 7 patients with only class II antibodies; 31 patients with both class I and class II antibodies). A highly significant association (P<0.001) was observed between pretransplant HLA antibodies detected by FlowPRA and episodes of rejection that occurred during the first posttransplant year. Fifteen patients died within the first year posttransplant. Of nine retrospective flow cytometric cross-matches that were performed, two were in recipients who had no pretransplant antibodies detectable by FlowPRA. Both of these cross-matches were negative. In contrast, five of seven cross-matches were positive among recipients who had FlowPRA detectable pretransplant antibodies. Posttransplant serum specimens from 91 patients were also assessed for antibodies by FlowPRA. Among this group, 58 patients had FlowPRA antibodies and there was a trend (although not statistically significant) for a biopsy documented episode of rejection to have occurred among patients with these antibodies. CONCLUSIONS: Collectively, our data suggest that pre- and posttransplant HLA antibodies detectable by FlowPRA and not AHG-CDC identify cardiac allograft recipients at risk for rejection. Furthermore, a positive donor reactive flow cytometric cross-match is significantly associated with graft loss. Thus, we believe that detection and identification of HLA-specific antibodies can be used to stratify patients into high and low risk categories. An important observation of this study is that in the majority of donor:recipient pairs, pretransplant HLA antibodies were not directed against donor antigens. We speculate that these non-donor-directed antibodies are surrogate markers that correspond to previous T cell activation. Thus, the rejection episodes that occur in these patients are in response to donor-derived MHC peptides that share cryptic determinants with the HLA antigens that initially sensitized the patient.
Subject(s)
HLA Antigens/immunology , Heart Transplantation/immunology , Antibodies/analysis , Antibody Specificity , Cytotoxicity, Immunologic , Flow Cytometry , Graft Rejection/diagnosis , Graft Rejection/immunology , Graft Rejection/therapy , Humans , Sensitivity and Specificity , Transplantation, HomologousABSTRACT
OBJECTIVE: Recurrent aortic narrowing after repair of aortic coarctation or interrupted aortic arch, as well as diffuse, long-segment aortic hypoplasia, can be difficult to manage. Extra-anatomic ascending aorta-descending aorta bypass grafting through a sternotomy is an alternative approach for this problem. METHODS: Since 1985, 19 patients aged 2 months to 18 years (mean 10.7 years) underwent extra-anatomic bypass with 10- to 30-mm Dacron grafts. The initial diagnosis was coarctation with hypoplastic arch in 15, interrupted aortic arch in 3, and diffuse long-segment aortic hypoplasia in 1. Seventeen of the children had a total of 22 previous operations: transthoracic interposition or bypass graft (n = 7), end-to-end anastomosis (n = 7), subclavian arterioplasty (n = 6), and synthetic patch (n = 2). The mean time from initial repair was 8.0 years (range 0.6-18 years). Three children had previous sternotomies. Cardiopulmonary bypass was avoided in all but 6 patients (5 with simultaneous intracardiac repairs). RESULTS: No hospital or late deaths occurred. On follow-up from 4 months to 14.7 years (mean 7.9 years), no reoperations for recurrent stenosis were performed. Two patients have arm-to-leg pressure gradients: 20 mm Hg at rest in 1 patient and a 60-mm Hg systolic exercise gradient with no resting gradient in the other. One patient required exclusion of an aortic aneurysm at the old coarctation repair site 13 years after extra-anatomic bypass. Three children had subsequent successful cardiac operations. CONCLUSIONS: Extra-anatomic bypass is an effective and relatively easy approach for selected cases of complex or reoperative aortic arch obstruction. It should be considered as an alternative operative technique for complex aortic arch reconstruction.
Subject(s)
Aortic Valve Stenosis/surgery , Blood Vessel Prosthesis Implantation/methods , Adolescent , Anastomosis, Surgical , Aortic Valve Stenosis/etiology , Child , Child, Preschool , Female , Humans , Infant , Male , Polyethylene Terephthalates , Sternum/surgery , Treatment OutcomeABSTRACT
Four infants aged 20-115 days (mean, 57.8 days) who had undergone stage I Norwood procedure for hypoplastic left heart syndrome came to early cardiac catheterization (6-112; mean, 47.3 days) following surgery because of significant arterial desaturation (pulse oximetry indicating oxygen saturations consistently in the 40%-70% range). Cardiac catheterization demonstrated a significant systolic pressure gradient between the ascending aorta and innominate artery (30-65; mean, 51 mm Hg) as the likely cause of diminished pulmonary blood flow in these patients. Routine angiography by itself was not conclusive in identifying a discrete area of obstruction, but selective angiography coupled with a knowledge of the obstruction did reveal the stenosis. All patients were successfully treated with balloon dilatation of the stenotic area, with the pressure gradient being reduced to 7-25 (mean, 17 mm Hg) immediately following dilatation. On follow-up catheterization in three patients, the systolic gradients were 3, 6, and 9 mm Hg. Arterial oxygen saturations rose from 63.5% predilatation to 77.3% immediately postdilatation and 81% on follow-up evaluation. In conclusion, innominate artery stenosis is an important cause of diminished blood flow through a modified right Blalock-Taussig shunt. Routine angiography will often miss the diagnosis. Pressure gradients and selective angiograms are necessary in order to make the diagnosis, although careful noninvasive assessment should also be diagnostic of this problem. Catheter dilatation is therapeutic in this situation and can be performed early after surgery in the absence of a fresh suture line.
Subject(s)
Angioplasty, Balloon , Arterial Occlusive Diseases/therapy , Brachiocephalic Trunk , Hypoplastic Left Heart Syndrome/surgery , Postoperative Complications/therapy , Angiography , Arterial Occlusive Diseases/diagnostic imaging , Brachiocephalic Trunk/diagnostic imaging , Brachiocephalic Trunk/injuries , Female , Humans , Hypoplastic Left Heart Syndrome/diagnostic imaging , Infant , Infant, Newborn , Lung/blood supply , Male , Oxygen/blood , Postoperative Complications/diagnostic imagingABSTRACT
BACKGROUND: Children with chronic cyanotic heart disease often develop systemic-to-pulmonary collateral arteries that can be deleterious at the time of a Fontan procedure due to excessive pulmonary blood flow. We therefore occlude all significant collaterals during cardiac catheterization. METHODS: From June 1993 to May 1998, 93 children aged 1.5 to 15.8 years (median 2.5 years) underwent a fenestrated lateral tunnel Fontan procedure. Eighty-nine (96%) had a previous bidirectional Glenn anastomosis, including 31 (33%) with a Norwood procedure. RESULTS: Preoperatively, 33 children (35%) required occlusion of 1 to 11 (mean 3.6) collateral vessels. Two of the three perioperative deaths (operative survival 97%) were due to excessive pulmonary blood flow from unrecognized collaterals in one and uncontrollable collaterals in the other. Postoperatively, 19 children (20%) required coil occlusion of 1 to 21 (mean 5.6) collaterals for elevated pulmonary artery pressures, heart failure, or prolonged chest tube drainage. Duration of inotropic support, postoperative ventilation, intensive care unit stay, and postoperative hospitalization were all significantly longer in the patients who had postoperative occlusion of collaterals. On follow-up of 2 to 67 months (mean 35 months), there have been four late deaths (two infections, two heart failures); 6 patients underwent successful cardiac transplantation for refractory heart failure. All 8 patients with ventricular failure required occlusion of significant collaterals postoperatively. CONCLUSIONS: Hemodynamically significant collaterals are not uncommon in Fontan candidates, and aggressive control can result in good operative and medium-term survival. After the Fontan, significant collaterals may be a marker for eventual cardiac failure because 8 of 18 patients requiring postoperative coils went on to transplantation or died of heart failure.
Subject(s)
Collateral Circulation , Embolization, Therapeutic , Fontan Procedure , Heart Defects, Congenital/surgery , Pulmonary Circulation , Adolescent , Cardiac Catheterization , Cardiotonic Agents/therapeutic use , Child , Child, Preschool , Fontan Procedure/mortality , Heart Defects, Congenital/physiopathology , Humans , Infant , Length of Stay , Postoperative Care , Preoperative Care , Respiration, Artificial , Survival RateABSTRACT
BACKGROUND: Cardiac transplantation is an accepted treatment for children with end-stage heart failure or complex or inoperable congenital defects. METHODS: Since 1988, 95 transplants have been performed in 89 children aged 4 days to 18 years (median 6.9 years, 42 patients 0-5 years). Fifty-eight (61%) had congenital or acquired heart disease, 31 (33%) had idiopathic cardiomyopathy, and 6 (6%) were retransplants. Fifty-seven of the patients had prior cardiac surgery with a range of one to eight procedures (mean 3.4 procedures/patient). At the time of transplantation, 53 (56%) were United Network for Organ Sharing (UNOS) status I, including 23 children on mechanical ventilation and 4 with mechanical circulatory support. RESULTS: Thirty-day survival in this group was 96%. Posttransplant results showed a median time of ventilation of 1 day (mean 3.0+/-5.7 days), median duration of inotropic support of 2 days (mean 2.7+/-2.3 days), median intensive care unit (ICU) stay of 4 days (mean 6.9+/-9.6 days), and median hospitalization of 9 days (mean 14.3+/-13.9 days). Follow-up from 1 month to 10.3 years (mean 3.1 years) has demonstrated a 1-year actuarial survival of 79% and a 5-year actuarial survival of 69%. Rejection, both acute and chronic, accounted for the vast majority of deaths. CONCLUSIONS: Pediatric heart transplantation can be accomplished with excellent early survival despite multiple prior cardiac operations and relative severity of illness. Parameters such as postoperative ventilation, inotropic support, ICU stay, and hospitalization can be kept at reasonable levels with acceptable long-term results, although rejection remains a serious problem.
Subject(s)
Heart Defects, Congenital/surgery , Heart Failure/surgery , Heart Transplantation , Postoperative Complications/mortality , Actuarial Analysis , Adolescent , Child , Child, Preschool , Female , Follow-Up Studies , Graft Rejection/mortality , Heart Defects, Congenital/mortality , Heart Failure/mortality , Humans , Infant , Infant, Newborn , Male , Reoperation , Survival RateABSTRACT
Continued miniaturization of permanent pacing systems has promoted use of this technology in younger and smaller pediatric patients. Intermedics ThinLine 438-10 active fixation pacing leads (4.5 Fr lead body) were implanted in 26 patients (17 males/9 females; 9.9 +/- 6.9 years). Twenty of 26 patients received dual chamber systems, 6 of 26 patients single lead systems. Each patient has been followed 3 months. Pacemaker analysis at implant and 6 months later evaluated pulse width thresholds at 2.5 V (atrial 0.07 +/- 0.02 vs 0.13 +/- 0.02 ms [P = 0.01]; ventricular 0.08 +/- 0.04 ms vs 0.20 +/- 0.04 ms [P = 0.01]); sensing thresholds (atrial 4.1 +/- 0.41 mV vs 4.0 +/- 4.2 mV [P = NS]; ventricular 9.7 +/- 0.72 vs 9.3 +/- 0.94 mV [P = NS]); and impedance (atrial 345 +/- 12 vs 370 +/- 120 O [P = 0.04]; ventricular 412 +/- 17 vs 458 +/- 190 O [P < 0.01]). One volt lead failed with exit block at approximately 6 weeks. The youngest (9 months to 5 years) and smallest (6.5-18.0 kg) ten patients have each shown by venography to have at least mild venous stenosis at the lead(s) insertion site; five patients demonstrated collateral formation around asymptomatic obstruction, with no thrombus formation. The Intermedics 438-10 ThinLine pacing lead has demonstrated good and stable early postimplant electrical parameters. Angiographic evaluation in our smaller patients has shown evidence for asymptomatic venous obstruction.