Histopathology of resected tissue from repair of anomalous aortic origin of a coronary artery: Potential mechanism of coronary artery compression.

Objective: This study aimed to describe the histomorphologic characteristics of resected (unroofed) common wall tissue from repair of anomalous aortic origin of a coronary artery and to determine whether the histologic features correlate with clinical and imaging findings. Methods: The histology of resected tissue was analyzed and reviewed for the presence of fibrointimal hyperplasia, smooth muscle disarray, mucoid extracellular matrix accumulation, mural fibrosis, and elastic fiber disorganization and fragmentation using hematoxylin and eosin and special stains. Clinical, computed tomography imaging, and surgical data were correlated with the histopathologic findings. Results: Twenty specimens from 20 patients (age range, 7-18 years; 14 males) were analyzed. Anomalous aortic origin of a coronary artery involved the right coronary in 16 (80%), and a slit-like ostium was noted in 18 (90%). By computed tomography imaging, the median proximal coronary artery eccentricity index was 0.4 (range, 0.20-0.90). The median length of intramural course was 8.2 mm (range, 2.6-15.2 mm). The anomalous vessel was determined to be interarterial in 14 patients (93%, 15 had evaluable images). The median distance from a commissure was 2.5 mm above the sinotubular junction (STJ) (range: 2 mm below the STJ-14 mm above the STJ). Prominent histopathologic findings included elastic fiber alterations, mural fibrosis, and smooth muscle disarray. The shared wall of the aorta and intramural coronary artery is more similar to the aorta histologically. Mural fibrosis and elastic fiber abnormalities tended to be more severe in patients >10 years of age at the time of surgery, but this did not reach statistical significance. The extent of vascular changes did not appear to have a clear relationship with the imaging features. Conclusions: The findings confirm the aortic wall-like quality of the intramural segment of the coronary artery and the presence of pathologic alterations in the wall microstructure.

Predictors of anesthesia ready time: Analysis and benchmark data.

Objective: Patients undergoing congenital cardiac surgery require induction of anesthesia. Our objective was to identify the median anesthesia ready time and the predictors of this time. Methods: By using the Society of Thoracic Surgeons Congenital Heart Surgery Database, we identified patients who underwent cardiopulmonary bypass procedures from 2017 to 2021. Univariate and multivariable regression modeling to predict the anesthesia ready time was performed using mixed-effects linear regression. Results: After exclusion of outliers, 44,418 cases were analyzed. The median anesthesia ready time was 51 minutes (interquartile range, 38-66). On multivariable analysis, independent predictors of a longer anesthesia ready time included decreasing weight (0.3 min/10 kg, 95% CI, 0.1-0.6; P = .011), prematurity (1.5 minutes, 95% CI, 0.8-2.2; P < .001), and presence of chromosomal abnormality (3.4 minutes, 95% CI, 1.5-5.2; P < .001). An increase in the duration in anesthesia ready time was seen with increasing Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery mortality category with an additional 7.8 minutes (95% CI, 5.2-10.4; P < .001) for a Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery 5 procedure compared with Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery 1. Emergency versus elective case designation was associated with an anesthesia ready time reduction of 3.6 minutes (95% CI, 1.1-6.1; P = .005), and an afternoon case start was associated with an anesthesia ready time reduction of 4.2 minutes (95% CI, 2.8-5.6; P < .001). The presence of an anesthesia trainee increased the anesthesia ready time by 3.8 minutes (95% CI, 2.6-5.0; P < .001). The presence of an airway in situ decreased the anesthesia ready time by 3.6 minutes (95% CI, 1.6-5.5; P < .001), whereas an in situ arterial line decreased the anesthesia ready time by 7.4 minutes (95% CI, 4.6-10.2; P < .001). Placement of a central venous line increased the anesthesia ready time by 8.5 minutes (95% CI, 5.9-11.1; P < .001). Conclusions: The median anesthesia ready time was 51 minutes. For patients with characteristics associated with prolonged anesthesia ready time, consideration should be given to allocation of additional anesthesia staffing to improve efficiency.

The Yasui operation: A single institutional experience over 30 years.

Objective: The Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation. Methods: We retrospectively reviewed all patients undergoing the Yasui operation between 1989 and 2021. Results are reported as median with interquartile range (IQR). Results: Twenty-five patients underwent a Yasui operation (19 primary), at 11 days (IQR, 7-218 days) of life and weight of 3 kg (IQR, 2.8-4.1 days). Fundamental diagnosis was ventricular septal defect/interrupted aortic arch in 11 patients and ventricular septal defect/aortic atresia in 14. Follow-up was 96% (24 out of 25) at 5 years (IQR, 1.4-14.7) with 92% survival. Freedom from LVOTO reoperation was 91% at late follow-up with 2 patients requiring baffle revision at 6 and 9 years. Latest echocardiogram showed 100% of patients had normal biventricular function and 87% (20 out of 23) less than mild LVOTO at 5 years (IQR, 2.3-14.9). Diagnosis, aortic valve morphology, and material used were not predictors of LVOTO. Freedom from right ventricle-to-pulmonary artery conduit reoperation was 48% at a median of 5 years (IQR, 1.4-14.7). Conduit type was not a predictor of reintervention. Conclusions: The Yasui operation can be performed with low morbidity and mortality in patients with 2 acceptable-size ventricles and aortic atresia or interrupted aortic arch with severe LVOTO. Despite some burden of reoperation, midterm reoperation for LVOTO is not common and ventricular function is preserved.

Computed tomography angiography (CTA) of anomalous aortic origin of a coronary artery (AAOCA): Which measurements are accurate and reliable?

BACKGROUND: The variation and accuracy of computed tomography angiography (CTA) features of anomalous aortic origin of a coronary artery (AAOCA) have not been completely characterized. We evaluated anomalous right (AAORCA) and left (AAOLCA) coronary arteries by CTA, with an emphasis on reproducibility, comparison with surgical measurements, and effect of nitroglycerin. METHODS: CTAs were interpreted for location of coronary origin & exit from the aorta, course, and dominance; minor & major diameters of the proximal & distal coronaries; shape of orifice; and intramural length. Relationships between vessel measurements, body surface area (BSA), and nitroglycerin use were evaluated. Comparisons between CTA and surgical measurements included intramural length, surgical probe size pre-intervention vs. CTA proximal AAOCA diameter, and surgical probe size post-intervention vs. CTA distal AAOCA diameter. RESULTS: Of 104 patients [81 (78%) AAORCA], all but 1 were intramural. Compared to AAOLCA, AAORCA patients were more likely to have a high origin (91% vs. 31%, p â€‹< â€‹0.01), and slit-like orifice (58% vs. 26%, p â€‹< â€‹0.01). When CTAs with nitroglycerin were compared to those without, no difference in proximal AAOCA dimensions was identified; however distal AAOCA and normal-origin coronary dimensions were larger in scans with nitroglycerin. Aside from slit-like orifice, reliability of coronary CTA measurements, including proximal AAOCA minor diameter and intramural length, was moderate to good between readers. In a subgroup of 54 patients, proximal AAOCA minor diameter and intramural length had good agreement and correlation with surgery. CONCLUSION: Proximal AAOCA dimensions and intramural length are reproducible variables. The lack of difference in proximal AAOCA dimensions with nitroglycerin may reflect abnormal vessel mechanics. Multicenter studies are an important next step in understanding the generalizability of our findings.

Patch augmentation of small ascending aorta during stage I procedure reduces the risk of morbidity and mortality.

OBJECTIVES: Hypoplastic left heart syndrome (HLHS) with aortic atresia (AA) patients are prone to coronary insufficiency due to a small ascending aorta. Prophylactic patch augmentation of the small ascending aorta during the stage I procedure (S1P) may reduce the risk of coronary insufficiency as marked by ventricular dysfunction, need for extracorporeal membrane oxygenator (ECMO) support or mortality. METHODS: Retrospective analysis of patients with HLHS with AA who underwent an S1P was completed. Baseline ascending aorta size, right ventricular (RV) function and outcome variables of transplant-free survival, ECMO support after the stage 1 operation and RV function at the time of the bidirectional Glenn and latest follow-up were collected. RESULTS: Between January 2010 and April 2020, 11 patients underwent prophylactic ascending aorta augmentation at the time of the S1P as a planned portion of the procedure. A total of 125 patients underwent S1P during this period as a comparison. Overall survival was 100% for the augmented group and 74% for the control group (P = 0.66). A composite end point of transplant-free survival, no post-S1P ECMO and less than moderate RV dysfunction was created. At the time of BDG, this composite end point was 100% for the augmented group and 61.8% for the control group (P = 0.008) and at most recent follow-up was 100% for the augmented group and 59.3% for control (P = 0.007). Eight patients required a rescue procedure for the clinical evidence of coronary insufficiency following S1P that included ascending aorta patch augmentation or stent placement. When comparing these rescue versus prophylactic ascending aortic augmentations, there were also differences in the composite outcome 100% for augmented and 60% for rescue (P = 0.009) and at the time of most recent follow-up 100% for augmented and 50% for rescue (P = 0.029). CONCLUSIONS: Prophylactic patch augmentation of the ascending aorta in HLHS patients with AA may reduce the risk of mortality, ECMO and reduced RV function. Patients not initially undergoing augmentation but then requiring a rescue procedure have particularly poor outcomes. Patch augmentation for smaller ascending aortic diameters should be considered and further clinical experience may help delineate aorta diameter threshold for augmentation.

Hybrid Left Heart Bypass Circuit for Repair of the Descending Aorta in an 8-kg Williams Syndrome Patient.

A 1-year old male patient with Williams syndrome and multiple prior interventions presented for surgical repair of his descending aorta (DA) through a left thoracotomy. Concerns for significant bleeding and spinal cord protection led the care team to consider a left heart bypass (LHB) circuit with options for pump sucker use, heat exchange capacity, and the possibility of converting to traditional cardiopulmonary bypass (CPB). A traditional CPB circuit with a roller-head arterial pump was assembled with a bypass line around the cardiotomy venous reservoir (CVR). Excluding the CVR with this line allowed for a closed LHB circuit. A second pump head was integrated to both recirculate the CVR volume and to serve as a means for controlled volume administration to the closed LHB circuit. Pump sucker return directed to the CVR could easily be transfused back to the patient. The patient was placed on the hybrid LHB circuit and cooled to 32°C. DA clamps were placed. Upper body dynamic blood pressure was managed for a target mean of 50 mmHg, the left atrial pressure (LAP) was maintained in the 5-7 mmHg range, and the nonpulsatile lower body blood pressure was targeted at 40-50 mmHg. Cerebral near-infrared spectroscopy (NIRS) helped guide volume and pressure management. The surgeons placed two long-segment patches on the DA, moving clamps as needed. The patient was rewarmed and separated from the hybrid LHB circuit after 82 minutes. Closed circuit LHB can be provided with a roller-head hybrid circuit incorporating an oxygenator for gas exchange, central cooling and warming, and arterial line filtration along with a CVR for pump sucker use and controlled transfusion to the patient.

The Use of Regional Catheters in Children Undergoing Repair of Aortic Coarctation.

OBJECTIVE: The objective was to assess the effectiveness and safety of peripheral regional anesthesia in congenital cardiac surgical patients undergoing thoracotomy for aortic coarctation. DESIGN: A retrospective chart review of pediatric patients (<18 years) who underwent surgical repair of congenital heart diseases via thoracotomy between September 2013 and July 2018 was done. Among patients who underwent coarctation repair, a propensity score was used to match patients who received a regional catheter (C) versus traditional medical treatment only (M). SETTING: A single center children's hospital. PARTICIPANTS: The median age was 172 days (IQR 64-1315) in group C and 176 days (IQR 71-1146) in group M (SMD = 0.07). The median weight was 6.8 kg (IQR 4.8-13.6) in group C and 7.7 kg (4.6-17.4) in group M (SMD = 0.003). MEASUREMENTS AND MAIN RESULT: Outcomes assessed were postoperative hospital length of stay, median pain scores in the first 24 and 48 hours, and total morphine equivalent use in the first 24 and 48 hours. Complications related to the catheters were reviewed. The median oral morphine equivalent dose administered in the first 24 hours was lower in group C than group M (0.8 mg/kg, IQR 0.5-1.1 vs. 1.4 mg/kg, IQR 0.9-1.7, p = 0.019). There were no major complications related to the catheters, including hematoma. CONCLUSIONS: Peripheral regional catheters may be used to reduce opioid requirements in patients after CoA repair. Due to the low risk of these catheters, they should be considered as part of a pain management strategy for pediatric patients undergoing thoracotomy and should be incorporated into strategies to improve outcomes.

Relationship of Preoperative Thyroid Dysfunction to Clinical Outcomes in Pediatric Cardiac Surgery.

CONTEXT: Thyroid function may be assessed in children before cardiac surgery because of concerns that hypothyroidism or thyrotoxicosis might adversely affect cardiac function perioperatively. However, the relationship between preoperative thyroid dysfunction and surgical outcomes is unknown. OBJECTIVE: Determine the relationship between preoperative thyroid dysfunction and outcomes of pediatric cardiac surgery. METHODS: Retrospective cohort study (January 2005 to July 2019). SETTING: Academic pediatric hospital. PATIENTS: All patients <19 years old who underwent cardiac surgery with cardiopulmonary bypass and had thyrotropin (TSH) measured within 14 days preoperatively. Exclusion criteria included neonates (≤30 days), preoperative extracorporeal life support, salvage operations, or transplantation procedures. MAIN OUTCOME MEASURES: Subjects were stratified by preoperative TSH concentration (mIU/L): low (<0.5), normal (0.5-5), mildly high (5.01-10), or moderately high (>10). Outcomes were compared among subjects with normal TSH (control) and each group with abnormal TSH concentrations. The primary outcome was 30-day mortality. Secondary outcomes included time to extubation, intensive care unit and hospital length of stay, and operative complications. RESULTS: Among 592 patients analyzed, preoperative TSH was low in 15 (2.5%), normal in 347 (58.6%), mildly high in 177 (29.9%), and moderately high in 53 (9.0%). Free thyroxine was measured in 77.4% of patients and was low in 0 to 4.4% of subjects, with no differences among TSH groups. Thirty-day mortality was similar among TSH groups. There were no differences in any secondary outcome between patients with abnormal TSH and patients with normal TSH. CONCLUSION: Preoperative mild to moderate subclinical hypothyroidism was not associated with adverse postoperative outcomes in children undergoing cardiopulmonary bypass procedures.

Mechanical Properties of Autologous Pericardium Change With Fixation Time: Implications for Valve Reconstruction.

Autologous pericardium, fixed in glutaraldehyde, is a common patch material used in heart valve reconstruction. Competence of a reconstructed valve depends on patch dimensions and also on how much the patch deforms when the closed valve is pressurized. In this study, we used biaxial testing to evaluate the deformability of fixed autologous pericardium, and several commercial alternatives, under typical cardiac loads. We found that deformability of fixed autologous pericardium varies predictably with fixation time. This information can be used by surgeons in designing patches for valve repair.

Complications of Transthoracic Intracardiac and Central Venous Lines in Neonates Undergoing Cardiac Surgery.

Neonates undergoing congenital heart surgery require central venous access for diagnostic information and medication administration. There are multiple options for central access including peripherally inserted central catheters, umbilical, central venous, and transthoracic intracardiac lines. We retrospectively identified all patients younger than 30 days who underwent cardiac surgery in a 1-year period. Data were collected on demographic and medical characteristics, adverse events, pre-emptive transfusion of blood products prior to line removal, and transfusion of blood products following removal of transthoracic intracardiac lines and central venous lines. In our cohort of 124 neonates, 176 transthoracic intracardiac lines were placed in 113 patients. Eighty-two patients had pre-existing central venous access including 35 umbilical venous lines, 21 PICC lines, and 18 CVLs. Ninety-eight patients received a CVL in the operating room by anesthesia. Five patients were transfused to correct laboratory derangement prior to transthoracic intracardiac line removal. Transfusion of packed red blood cells (pRBC) occurred after transthoracic intracardiac line removal in 25 patients. Thrombus formation was present in one patient with a transthoracic intracardiac line and three patients with CVLs. One patient underwent surgical intervention for repositioning of a transthoracic intracardiac line. There were no cases of cardiac arrest, extracorporeal life support, or deaths attributable to lines. In this cohort, transthoracic intracardiac lines were generally safe. There were very few complications of thrombus, infection, or requirement for surgical intervention; however, transfusion of pRBCs following transthoracic intracardiac line removal occurred in 20% of patients.

Impact of a Composite Valved RV-PA Graft After Stage 1 Palliation.

BACKGROUND: The use of a valved right ventricular to pulmonary artery shunt (RVPAS) has been reported by some to improve pulmonary artery growth after stage 1 palliation (S1P). METHODS: We retrospectively reviewed all patients undergoing an S1P with an RVPAS between January 2013 and May 2017, stratified by RVPAS type: a ring-reinforced polytetrafluoroethylene (PTFE) graft or a composite graft that included a distal valved femoral or saphenous vein homograft. We examined the association of RVPAS type on postoperative hemodynamics, time to reintervention, pulmonary artery growth, and survival. RESULTS: Among 94 infants, 56 (60%) underwent PTFE-only shunt, 24 (25%) underwent femoral vein homograft, and 14 (15%) underwent saphenous vein homograft, and no relevant risk factor differences were found between the groups. Arterial saturation was 2.3% higher (p = 0.014) and serum lactic acid was 1.24 mg/dL lower (p = 0.03) in the femoral vein homograft group than in the PTFE-only group, although venous saturation was similar. By 60 days, 50% of patients with saphenous vein homograft had a reintervention compared with 5% with PTFE graft (p < 0.0001) and 12% with femoral vein homograft (p = 0.2 versus PTFE). At the time of stage 2 palliation, no differences were found in pulmonary artery size or growth over time by either echocardiogram or angiography or in the density of aortopulmonary collaterals or degree of tricuspid regurgitation. The 12-month survival was similar between the groups. CONCLUSIONS: The use of an interposition femoral vein homograft into the RVPAS may enhance perioperative stability, but it does not substantially improve interstage growth of the pulmonary arteries. Use of saphenous vein homograft is associated with earlier time to reintervention after S1P.

Twenty Years of Anesthetic and Perioperative Management of Patients With Tetralogy of Fallot With Absent Pulmonary Valve.

OBJECTIVE: Review the authors' institutional experience of the induction and perioperative airway management of children with tetralogy of Fallot with an absent pulmonary valve. DESIGN: Retrospective chart review. SETTING: Large academic children's hospital. PARTICIPANTS: Patients with the diagnosis of tetralogy of Fallot with absent pulmonary valve undergoing primary cardiac repair over a 20-year period. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Forty-four patients were identified with tetralogy of Fallot with an absent pulmonary valve from January 1995 through August 2014. Forty-two patients (95%) required surgery in their first year of life. Sixteen patients (36%) required mechanical ventilation preoperatively, including 11 neonates. Of the 28 patients not intubated preoperatively, only 1 (3.8%) exhibited minor airway obstruction following induction that was managed uneventfully. All intubations in the operating room were performed in the supine position. Five patients who were mechanically ventilated preoperatively in a lateral or prone position were supinated in the operating room without significant cardiopulmonary compromise. There were no patients who suffered cardiopulmonary arrest or required mechanical circulatory support. The median time to extubation was 2 days (range 1-13 days) in those patients who were not mechanically ventilated prior to their surgery. Ultimately, 5 patients required tracheostomy and 1 patient underwent lobectomy. Although, there was no mortality at 30 days, 4 children died within 1 year of their surgery. All the children who died had a genetic syndrome and required mechanical ventilation preoperatively. CONCLUSIONS: While there are theoretical concerns specific to the induction and airway management of tetralogy of Fallot with an absent pulmonary valve, there were no episodes of cardiorespiratory arrest or extracorporeal membrane oxygenation in the authors' series. Neonatal age at the time of surgery, preoperative need for mechanical ventilation, and concomitant genetic syndromes are risk factors for respiratory morbidity. Mortality in this study was low compared to historic reports, likely reflecting improvement in surgical technique and intensive care management.

Clinical Utility of Preimplantation Homograft Cultures in Patients Undergoing Congenital Cardiac Surgery.

Institutional practice at our hospital (Boston Children's Hospital) is to culture homografts before implantation during congenital cardiac surgery. Over a 4-year period, 5% (73 of 1376) of these cultures were positive, but the results had minimal clinical impact. Our experience demonstrates that there is limited utility in preimplantation cultures of cardiac homografts.

Phosphodiesterase Inhibitor-Based Vasodilation Improves Oxygen Delivery and Clinical Outcomes Following Stage 1 Palliation.

BACKGROUND: Systemic vasodilation using α-receptor blockade has been shown to decrease the incidence of postoperative cardiac arrest following stage 1 palliation (S1P), primarily when utilizing the modified Blalock-Taussig shunt. We studied the effects of a protocol in which milrinone was primarily used to lower systemic vascular resistance (SVR) following S1P using the right ventricular to pulmonary artery shunt, measuring its effects on oxygen delivery (DO2) profiles and clinical outcomes. We also correlated Fick-based assessments of DO2 with commonly used surrogate measures. METHODS AND RESULTS: Neonates undergoing S1P were treated according to best clinical judgment prior to (n=32) and following (n=24) implementation of a protocol that guided operative, anesthetic, and postoperative management, particularly as it related to SVR. A majority of the subjects (n=51) received a modified right ventricular to pulmonary artery shunt. In a subset of these patients (n=21), oxygen consumption (VO2) was measured and used to calculate SVR, DO2, and oxygen debt. Neonates treated with the protocol had significantly lower SVR (P=0.02), serum lactate (P<0.001), and Sa-vO2 difference (P<0.001) and a lower incidence of CPR requiring extracorporeal membrane oxygenation (E-CPR, P=0.02) within the first 72 postoperative hours. DO2 was closely associated with SVR (r2=0.78) but correlated poorly with arterial (SaO2) and venous (SvO2) oxyhemoglobin concentrations, the Sa-vO2 difference, and blood pressure. CONCLUSIONS: A vasodilator protocol utilizing milrinone following S1P effectively decreased SVR, improved serum lactate, and decreased postoperative cardiac arrest. DO2 correlated more closely with SVR than with Sa-vO2 difference, highlighting the importance of measuring VO2 in this population. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT02184169.

Direct heart shunt placement for CSF diversion: technical note.

The authors report a complex case of an 18-year-old male with a history of hydrocephalus secondary to intraventricular hemorrhage of prematurity, with more than 30 previous shunt revisions, who presented to the authors' institution with shunt malfunction. After exhausting his peritoneal cavity and pleural space as possible distal sites of shunt placement, he underwent a direct heart shunt placement when it was discovered he had thrombosis of his subclavian vein precluding a standard wire-guided atrial cannulation. His course was complicated by postoperative distal catheter migration and repeat surgery for reimplantation of the shunt directly into the atrium. At the 16-month follow-up visit, the patient showed no symptoms of shunt malfunction or pericardial effusion. Imaging studies demonstrated a functioning shunt system. This is the second reported successful ventricle to direct heart shunt placement in an adult. The authors report on the technical aspects of the case and review the relevant literature.

Outcomes of surgery for young children with multivessel pulmonary vein stenosis.

OBJECTIVE: We pursued a multimodality approach to the treatment of patients with pulmonary vein stenosis, incorporating sutureless surgical repair, catheter interventions, and adjunctive chemotherapy. We report our outcomes after surgery. METHODS: Between January 2007 and August 2013, 49 patients with multivessel pulmonary vein stenosis underwent operations at our institution. We retrospectively reviewed data from a pulmonary vein stenosis registry and the medical records. RESULTS: At the time of the index operation, the median patient age was 6 months (range, 32 days-48 months) and weight was 4.9 kg (range, 2.1-13.4 kg). Fourteen patients (28%) died during the follow-up period (median follow-up was 0.5 years [range, 0.04-4.9 years]). There were 2 deaths (4%) within 30 days. Age at repair <6 months, weight at repair <3 kg, and a preoperative right ventricular systolic pressure < ¾ systemic were found to be associated with mortality. One patient required repeat operation for recurrent stenosis. Thirty-nine patients (80%) underwent postoperative catheterizations. The median number of catheterizations per patient was 2 (range, 0-14). Twenty-nine patients (59%) underwent catheterizations with pulmonary vein intervention. The median number of catheterizations with intervention per patient was 1 (range, 0-14). There were no identifiable associations with need for or number of catheterizations with intervention. Ten patients were listed for lung transplantation: 4 patients were de-listed, 3 patients died waiting, and 3 patients underwent transplant. CONCLUSIONS: Using a multimodality approach, we observed acceptable early survival after operation in patients with pulmonary vein stenosis, despite the need for catheter reinterventions. Lung transplantation remains a viable option.

Valve reconstruction for congenital mitral valve disease.

The surgical treatment of mitral valve disease in children is a challenging problem. Mitral stenosis and regurgitation may occur in isolation or together. Mitral valve repair is almost always preferable to replacement. Mitral valve replacement is not an ideal alternative to repair due to limitations of size, growth, structural valve degeneration, anticoagulation and poor survival. Surgical repair of congenital mitral stenosis must address the multiple levels of obstruction, including resection of the supramitral ring, thinning of leaflets and mobilization of the subvalvular apparatus. Sometimes leaflet augmentation is required. Repair of mitral regurgitation in children may involve simple cleft closures, edge-to-edge repairs, triangular resections and annuloplasties. Techniques used in adults, such as annuloplasty bands or artificial chords, may not be appropriate for children. Overall, an imperfect mitral valve repair may be more acceptable than the negative consequences of a replacement in a child.

Transposition of the great arteries and sinus venosus defect with partially anomalous pulmonary venous return: physiological and anatomic considerations.

Transposition of the great arteries is a common congenital heart defect causing cyanosis in neonates, occurring in 0.2 per 1000 live births. It has been reported to occur with other associated congenital heart lesions. However, its association with a superior sinus venosus defect and partially anomalous pulmonary venous return has not been reported. We present a neonate with transposition of the great arteries, superior sinus venosus defect with partially anomalous pulmonary venous return that underwent successful complete neonatal repair, and discuss important physiological and anatomic considerations.