Single dose myocardial protection technique utilizing del Nido cardioplegia solution during congenital heart surgery procedures.

BACKGROUND: The single dose cardioplegia technique for myocardial protection during congenital heart surgery is a viable alternative to multidose protocols. METHODS: Thirty-four pediatric patients with aortic cross clamp times greater than 90 minutes were grouped by modified adult (MA) multidose solution or del Nido (dN) single dose solution. Also, data from eight patients where the cross clamp times were greater than two hours on one dose of dN solution were included. RESULTS: In the 90-minute plus arm of the study, there were no significant differences between the groups when comparing the risk adjustment for congenital heart surgery (RACHS) (p=0.6), cardiopulmonary bypass times (CPB) (p=0.5), aortic cross camp times (p=0.5), weights (p=0.7) and number of intraoperative exogenous blood units (p=0.5). There were significant differences between the groups (p<0.05) in the number of cardioplegia doses and with perioperative glucose levels. In the greater than two hours group, the incidence of complete heart block (CHB) was 0.125% and there were no deaths or mechanical circulatory support (MCS) devices used. CONCLUSION: del Nido cardioplegia solution is a reasonable tool for myocardial protection during congenital heart surgery that significantly decreased the number of cardioplegic interventions and perioperative glucose values in our study groups.

The role of surgical therapy for pulmonary vein atresia in childhood.

UNLABELLED: The outcome for children with pulmonary vein atresia has been historically poor. This report describes clinical outcomes after surgical treatment of primary and secondary pulmonary vein atresia. All patients undergoing surgery for pulmonary vein atresia at Children's Hospital Boston from 1 January 2005 to 1 January 2009 were identified. The data available for analysis included demographic characteristics, findings based on cardiac catheterization and other imaging, clinical information, operative surgical intervention, and pulmonary vein histology. The study-specific information included the calculated number of atretic pulmonary veins as well as surgical interventions performed during the study period. The study end points included successful relief of pulmonary vein atresia, recurrence of pulmonary atresia or stenosis, overall pulmonary vein disease progression, and patient survival. A total of 16 patients with one or more pulmonary vein atresias were identified in the cardiac surgery registry database. Of these 16 patients, 5 had primary pulmonary atresia, and 11 had secondary atresia after repair of anomalous pulmonary venous drainage. Surgical recanalization of atretic pulmonary veins was achieved for more than 80% of the patients. At the most recent follow-up assessment, 7 (44%) of the 16 patients had recurrence of atresia. Five of the patients had development of new atresia in other previously healthy pulmonary veins. The median follow-up period was 4 months for primary and 17 months for secondary pulmonary vein atresia. The three main predictors of pulmonary vein disease progression after surgery were the presence of underlying lung disease (P = 0.036), prematurity (P = 0.035), and a history of recurrent pulmonary vein atresia. The overall mortality rate for the patients with primary or secondary pulmonary vein atresia was 56% at the most recent follow-up assessment (100% for the patients with primary vein atresia and 36% for the patients with secondary vein atresia). CONCLUSIONS: Surgical recanalization of pulmonary vein atresia is possible, but prognosis remains poor. Recurrence of atresia can take place as early as a few months in the presence of underlying lung disease, prematurity, or history of recurrent pulmonary vein atresia. Further research is needed to identify mechanisms for attaining and sustaining pulmonary vein patency after surgery.

Biventricular repair in patients with heterotaxy syndrome.

OBJECTIVE: Complex intracardiac and extracardiac anatomy is often confronted during biventricular repair in patients with heterotaxy syndrome. We examined factors affecting surgical outcomes in these patients. METHODS: Between January 1990 and July 2007, 371 patients received a diagnosis of heterotaxy syndrome; 91 (91/371, 24.5%) underwent biventricular repair. Left atrial isomerism was present in 73% (66/91) and right atrial isomerism in 10% (9/91), with indeterminate atrial anatomy in 17% (16/91). Median age at biventricular repair was 6.8 months (5 days to 22.3 years). Systemic venous anomalies were present in 75 patients, pulmonary venous anomalies in 26, and endocardial cushion defects in 36. Transposition complexes were present in 15 patients with atrioventricular discordance in 10; 8 underwent double switch, 2 received a physiologic repair, 2 underwent arterial switch, and 3 underwent the Rastelli operation. Other conotruncal anomalies included double-outlet right ventricle in 10 patients, tetralogy of Fallot in 3, and hemitruncus in 2. Separation of systemic from pulmonary venous return included intra-atrial baffling in 48 patients and extracardiac grafting in 2. Combined lesions were common, occurring in 99% (90/91). Statistical analysis with Kaplan-Meier and Cox proportional hazards models were performed. RESULTS: Average follow-up was 44.9 +/- 57.5 months (3 days to 189.3 months). Kaplan-Meier estimated survival was 93.4% at 10 years; unbalanced complete atrioventricular canal was the only risk factor for mortality (P = .006). Subsequent procedures were common with a 10-year freedom from reoperation or reintervention of 38% +/- 7.5%. Arrhythmias occurred in 36 (39.6%) patients; bradyarrhythmia in 27 (29.7%) and tachyarrhythmia in 15 (16.5%). Freedom from any arrhythmia was 53.9% +/- 6.7% at 10 years. CONCLUSIONS: Excellent survival for patients with heterotaxy undergoing biventricular repair can be expected, even for multiple, complex lesions. Reintervention is common, and arrhythmia is a long-term concern. This experience shows that patients with heterotaxy syndrome and complex cardiac anatomy can be considered for biventricular repair. Patients with unbalanced complete atrioventricular canal are a high-risk group for which selection criteria are particularly important.

A review of ductal stenting in hypoplastic left heart syndrome: bridge to transplantation and hybrid stage I palliation.

There is increasing interest in applying ductal stenting technology to high-risk patients with hypoplastic left heart syndrome (HLHS). In this review, we present the complete history and a comprehensive up-to-date analysis of all available data on the use of ductal stenting as part of various hybrid strategies for the combined medical and surgical management of HLHS.

Anomalous left coronary artery from aorta with Prinzmetal's angina: a postoperative dilemma successfully managed.

Anomalous aortic origin of one of the coronary arteries is uncommon. There have been few reports of the left coronary artery arising from the noncoronary sinus. The occurrence of Prinzmetal's angina in association with anomalous aortic origin is extremely rare. We report a case of such a combination and discuss the diagnostic options and therapeutic decision making.

Hybrid pediatric cardiac surgery.

Minimally invasive strategies can be expanded by combining standard surgical and interventional techniques. We performed a longitudinal prospective study of all pediatric patients who have undergone hybrid cardiac surgery at the University of Chicago Children's Hospital. Hybrid cardiac surgery was defined as combined catheter-based and surgical interventions in either one setting or in a planned sequential fashion within 24 hours. Between June 2000 and June 2003, 25 patients were treated with hybrid approaches. Seventeen patients with muscular ventricular septal defects (mVSDs) (mean age, 4 months; range, 2 weeks-4 years) underwent either sequential Amplatzer device closure in the catheterization laboratory followed by surgical completion (group 1A, n = 9) or one-stage intraoperative off-pump device closure (group IB, n = 8) with subsequent repair of any concomitant heart lesions. Eight patients with branch pulmonary artery (PA) stenoses (group 2) underwent intraoperative PA stenting or stent balloon dilatation along with concomitant surgical procedures. All patients survived hospitalization. Complications from the hybrid approach were mostly confined to groups 1A and 2. At a mean follow-up of 18 months, 2 group 1A patients died suddenly several months after discharge. All other patients are doing well. Hybrid pediatric cardiac surgery performed in tandem by surgeons and cardiologists is safe and effective in reducing or eliminating cardiopulmonary bypass. Patients with mVSDs who are small, have poor vascular access, or have concomitant cardiac lesions are currently treated in one setting with the perventricular approach.

Multicenter experience with perventricular device closure of muscular ventricular septal defects.

Hybrid procedures are becoming increasingly important, especially in the management of congenital heart lesions for which there are no ideal surgical or interventional options. This report describes a multicenter experience with perventricular muscular venticular septal defect (VSD) device closure. Three groups of patients (n = 12) were identified: infants with isolated muscular VSDs (n = 2), neonates with aortic coarctation and muscular VSDs (n = 3) or patients with muscular VSDs and other complex cardiac lesions (n = 2), and patients with muscular VSDs and pulmonary artery bands (n = 5). Via a sternotomy or a subxyphoid approach, the right ventricle (RV) free wall was punctured under transesophageal echocardiography guidance. A guidewire was introduced across the largest defect. A short delivery sheath was positioned in the left ventricle cavity. An Amplatzer muscular VSD occluding device was deployed across the VSD. Cardiopulmonary bypass was needed only for repair of concomitant lesions, such as double-outlet right ventricle, aortic coarctation, or pulmonary artery band removal. No complications were encountered using this technique. Discharge echocardiograms showed either mild or no significant shunting across the ventricular septum. At a median follow-up of 12 months, all patients were asymptomatic and 2 patients had mild residual ventricular level shunts. Perventricular closure of muscular VSDs is safe and effective for a variety of patients with muscular VSDs.

Optimal surgical approach for repair of aortopulmonary window.

BACKGROUND: This is a review of the experience over 26 year in a single institution with surgical repair of aortopulmonary window. METHODS: Between July 1973 and March 1999, 38 patients underwent surgery for aortopulmonary window at a median age of 5 weeks, and with a median weight of 3.9 kg. Median follow-up was 6.6 years, with a range from 0.8 to 26 years. Additional defects were present in 25 (65%) patients, including interruption of the aortic arch in 7, tetralogy of Fallot in 7, ventricular septal defect in 5, functionally univentricular anatomy in 3, aortic coarctation in 2, and anomalous origin of a coronary artery in 1. We approached via an aortotomy in 45%, an incision through the defect in 31%, and using a pulmonary arteriotomy in 24% of patients. Closure was achieved using a single patch in 30 patients (79%). RESULTS: There were 3 (7.9%) in-hospital deaths. Actuarial patient survival was 88% at 10 years. Three patients required reinterventions for stenoses of the great arteries. Freedom from any reintervention was 70% at 10 years. By multivariate analysis, the approach through a pulmonary arteriotomy was shown to result in a higher need for reintervention (p = 0.01). CONCLUSIONS: Repair of aortopulmonary window can be done with excellent results. A pulmonary arteriotomy should be avoided.

Long-term results after early primary repair of tetralogy of Fallot.

OBJECTIVE: Early primary repair of tetralogy of Fallot has been routinely performed at Children's Hospital, Boston, since 1972. We evaluated the long-term outcome of this treatment strategy including the influence of a transannular patch. METHODS: Fifty-seven patients less than 24 months of age (median 8 months) underwent primary repair of tetralogy of Fallot between January 1972 and December 1977. Thirty-one patients had a transannular patch. Survival and freedom from reintervention were determined by the Kaplan-Meier method with 95% confidence intervals. RESULTS: There were 8 early deaths, and 1 patient died 24 years after initial repair. Recent follow-up was obtained for 45 of the 49 long-term survivors (92%). Median follow-up was 23.5 years. Ten patients underwent reintervention, 8 of whom underwent relief of right ventricular outflow tract obstruction. Right ventricular outflow tract obstruction occurred in 6 patients without a transannular patch and 2 with a transannular patch (33% vs 6%, P =.04). One pulmonary valve replacement was performed at another institution 20 years after the repair. Forty-one long-term survivors were in New York Heart Association class I and 4 were in class II. Actuarial survival was 86% at 20 years (95% confidence intervals = 80%-92%). Freedom from reintervention was 93% at 5 years (95% confidence intervals = 87%-99%) and 79% at 20 years (95% confidence intervals = 70%-86%). No significant differences were found between patients with and without a transannular patch (survival, P =.34; freedom from reintervention, P =.09, log-rank tests). CONCLUSIONS: Long-term survival is excellent and the freedom from reintervention is satisfactory after early primary repair of tetralogy of Fallot in the 1970s. Use of a transannular patch does not reduce late survival and is associated with a lower incidence of right ventricular outflow tract obstruction.

Valve-sparing operation for balloon-induced aortic regurgitation in congenital aortic stenosis.

OBJECTIVE: Aortic regurgitation after balloon dilation of congenital aortic stenosis may be treated with valve repair as an alternative to replacement. METHODS: Charts and echocardiograms of all patients undergoing aortic valve operations after balloon dilation of congenital aortic stenosis at our institution between January 1988 and December 1999 were reviewed. RESULTS: Twenty-one patients underwent valvuloplasty for predominant aortic regurgitation 9 months to 15 years (mean, 6.1 years) after balloon dilation. The mean +/- SD age at the time of the operation was 11 +/- 7 years. Aortic regurgitation was caused by a combination of commissural avulsion (10), cusp dehiscence with retraction (9), cusp tear (5), central incompetence (2), perforated cusp (1), or cusp adhesion to the aortic wall (1). Repair techniques included commissural reconstruction with a pericardial patch (8), pericardial patch cusp augmentation (6), primary suture repair (6), raphae release and debridement (4), commissurotomy (4), commissural resuspension with sutures (3), and cusp release (1). There were no deaths. At a mean follow-up of 30.1 months (range, 9 months-8 years), all patients were asymptomatic, and the grade of aortic regurgitation had been significantly reduced (P <.001). Left ventricular end-diastolic dimension z scores and proximal regurgitant jet/aortic anulus diameter ratios were significantly reduced (P <.001) and remained so over time. Freedom from reoperation for late failure was 100%, and overall freedom from reintervention was 80% at 3 years. CONCLUSION: Aortic valve repair for balloon-induced aortic regurgitation is reproducible and durable at medium-term follow-up.

Surgery for coarctation of the aorta in infants weighing less than 2 kg.

BACKGROUND: Low- and very low-birth weight infants are now candidates for reparative cardiac surgery. Outcomes after coarctation repair have not been characterized in this patient population. METHODS: We performed a retrospective review of 18 consecutive neonates less than 2 kg who underwent repair of aortic coarctation between August 1990 and December 1999. RESULTS: Median weight was 1,330 g, and median gestational age was 31 weeks. A ventricular septal defect was present in 5 patients, and Shone's complex in 4. Sixteen patients had resection and end-to-end anastomosis, and 2 had resection and subclavian flap. Median clamp time was 15.5 minutes. One patient died during hospitalization. Two patients died late postoperatively (5-year estimated survival 80%). Mean follow-up was 28.5 months. Eight patients (44%) had a residual or recurrent coarctation, 5 underwent balloon dilation, and 3 underwent reoperation. Freedom from reintervention for recoarctation was 60% at 5 years. Shone's complex or a hypoplastic arch was an independent risk factor for decreased survival (p < 0.001). Very low birth weight was a multivariate predictor for increased risk of recoarctation (p = 0.01). CONCLUSIONS: Coarctation repair in less than 2-kg premature non-Shone's infants can be performed with a low mortality. The rate of recoarctation is higher in the very low-birth weight infants, but can be managed with low risk.

Minimal sternotomy approach for congenital heart operations.

BACKGROUND: In recent years, minimal access cardiac operations have increased in application in both the adult and pediatric population. As our experience has grown with these approaches to atrial septal defect closure, we have expanded the same approach to the repair of more complex congenital heart disease. METHODS: At the Children's Hospital in Boston, from August 1996 to November 1999, a minimal sternotomy approach was used to surgically correct 104 children with congenital heart defects other than atrial septal defect. The approach, in most patients, consisted of a skin incision based over the xiphisternum, 3.5 to 5 cm in length, with division of the xiphoid only and elevation of the sternum by fixed retractor. All patients underwent cannulation for cardiopulmonary bypass through the great vessels in the chest using this same incision. The lesions corrected included ventricular septal defect in 41 patients, tetralogy of Fallot in 27, common atrioventricular canal in 15, mitral valve operation in 3.5, and other defects in 18 patients. There were 53 male and 51 female patients. Mean age at operation was 1.4 years (range, 2 weeks to 11 years). RESULTS: There were no deaths. The mean cardiopulmonary bypass time was 71 minutes (standard deviation, 19 minutes), mean cross-clamp times 40.8 minutes (standard deviation, 13 minutes), and length of stay 4.5 days (standard deviation, 1.9 days). Complications included transient atrioventricular block in 2 patients, pleural effusion requiring drainage in 4, and pericardial effusion in 3 patients. When compared to similar lesions repaired using a full sternotomy approach there was no difference in operating times and length of stay tended to be shorter in the minimal sternotomy group. CONCLUSIONS: A minimal sternotomy approach can be used to repair congenital cardiac lesions other than atrial septal defects. It gives good exposure, particularly for transatrial repairs, does not prolong ischemic times, and may lead to shorter hospital stay.

Comprehensive management of branch pulmonary artery stenosis.

Branch pulmonary artery stenosis may occur as an isolated finding or in conjunction with other cardiac malformations; it may be congenital or acquired postoperatively. Transcatheter management with balloon pulmonary angioplasty (BPA) and/or endovascular stenting is generally considered the procedure of choice for most patients. With the introduction of high-pressure balloons, the results of BPA have improved significantly (75%). Stent implantation has increased the success rate to over 90%. However, BPA continues to be the initial procedure of choice for distal stenoses, or those associated with branching points, and in small children or infants. Stent implantation is preferred over BPA for central or proximal stenoses, and those due to kinking or tenting, external compression, intimal flaps, failed balloon dilation, and early postoperative procedures. Certain lesions, such as supravalvar pulmonary stenosis, or stenosis at the branch pulmonary arteries bifurcation, are better dealt with at surgery. High-risk situations for the development of postoperative pulmonary artery (PA) stenosis exist in small infants with hypoplastic branch PAs after a conotruncal procedure, after duct ligation, after shunt insertion, after PA band placement, or after pulmonary arteriotomies or anastomoses of any kind. A combined collaborative transcatheter and surgical approach is essential for many patients with PA stenosis, particularly in complex forms of tetralogy of Fallot.

Functional living trileaflet heart valves grown in vitro.

BACKGROUND: Previous tissue engineering approaches to create heart valves have been limited by the structural immaturity and mechanical properties of the valve constructs. This study used an in vitro pulse duplicator system to provide a biomimetic environment during tissue formation to yield more mature implantable heart valves derived from autologous tissue. METHODS AND RESULTS: Trileaflet heart valves were fabricated from novel bioabsorbable polymers and sequentially seeded with autologous ovine myofibroblasts and endothelial cells. The constructs were grown for 14 days in a pulse duplicator in vitro system under gradually increasing flow and pressure conditions. By use of cardiopulmonary bypass, the native pulmonary leaflets were resected, and the valve constructs were implanted into 6 lambs (weight 19+/-2.8 kg). All animals had uneventful postoperative courses, and the valves were explanted at 1 day and at 4, 6, 8, 16, and 20 weeks. Echocardiography demonstrated mobile functioning leaflets without stenosis, thrombus, or aneurysm up to 20 weeks. Histology (16 and 20 weeks) showed uniform layered cuspal tissue with endothelium. Environmental scanning electron microscopy revealed a confluent smooth valvular surface. Mechanical properties were comparable to those of native tissue at 20 weeks. Complete degradation of the polymers occurred by 8 weeks. Extracellular matrix content (collagen, glycosaminoglycans, and elastin) and DNA content increased to levels of native tissue and higher at 20 weeks. CONCLUSIONS: This study demonstrates in vitro generation of implantable complete living heart valves based on a biomimetic flow culture system. These autologous tissue-engineered valves functioned up to 5 months and resembled normal heart valves in microstructure, mechanical properties, and extracellular matrix formation.

Surgery for bilateral outflow tract obstruction in elastin arteriopathy.

OBJECTIVE: A number of patients with Williams syndrome or other forms of elastin arteriopathy have stenoses of pulmonary arteries in addition to supravalvular aortic stenosis. We sought to investigate the effect of the degree of pulmonary arterial stenosis on the prognosis after an operation for supravalvular aortic stenosis to help define the optimal treatment strategy for patients with severe forms of elastin arteriopathy. METHODS: Between 1960 and 1999, 33 patients underwent operations for supravalvular aortic stenosis while having significant stenoses of the pulmonary arteries. We retrospectively reviewed patient charts, obtained current follow-up information, and determined risk factors for survival and reoperation. RESULTS: Fifteen patients with moderate right-sided obstructions (confirmed by pulmonary artery Z-scores and right ventricular/descending aortic pressure ratio) underwent operations for supravalvular aortic stenosis only. Eighteen patients had more severe right-sided obstructions and underwent surgical relief of pulmonary arterial stenoses or right ventricular outflow tract obstruction in addition to operations for supravalvular aortic stenosis. Eight patients had undergone preoperative balloon dilations of stenotic pulmonary arteries. There were 6 early deaths and 1 late death in our series. Survival at 10 and 20 years was 76% (70% confidence interval, 68%-84%) and freedom from reintervention was 59% (70% confidence interval, 46%-71%) at 10 years and 49% (70% confidence interval, 35%-62%) at 20 years. Multivariate analysis revealed that patients with a right ventricular/descending aortic pressure ratio of 1.0 or more were at higher risk for reintervention but not for death. CONCLUSIONS: Surgical treatment of pulmonary artery obstructions in elastin arteriopathy is palliative but, in conjunction with balloon dilation of peripheral pulmonary arteries, offers good long-term survival to patients with the severest form of elastin arteriopathy.

Evaluation of hemostatic and coagulation factor abnormalities in patients undergoing the Fontan operation.

OBJECTIVE: Low-velocity and nonlaminar flow patterns in the Fontan circulation, as well as abnormal liver function in some patients, may partly account for the coagulation abnormalities seen. We examined (1) coagulation factor abnormalities before and after the Fontan procedure and (2) regional coagulation factor abnormalities in the Fontan circulation. METHODS: Levels of factors V, VII, VIII, X, antithrombin III, prothrombin fragment F1+2, protein C, and protein S were measured in 2 groups of patients: In 14 patients undergoing the Fontan procedure, blood was analyzed before the operation and 5 days after the operation (group 1). The median age in this group was 3.2 years. In 10 patients who had undergone the Fontan procedure, cardiac catheterization was performed and samples were taken from the femoral vein, inferior vena cava, right atrium, and pulmonary artery (group 2). The median age in this group was 6.2 years and the median follow-up from the Fontan procedure was 4.1 years. RESULTS: In group 1 a significant increase was noted postoperatively in the concentration of factor VIII (P<.001), factor X (P<.001), and prothrombin fraction F1+2 (P <.001). A significant decrease in the levels of antithrombin III (P <.001), protein C (P<.004), and protein S (P<.02) was also found. The increase in factors VIII and X persisted at 4 years' follow-up in group 2 patients. In group 2, no significant regional differences were observed between the coagulation factors measured at different sites. CONCLUSIONS: There is an increased tendency toward coagulation after the Fontan procedure. A prothrombotic state is supported by thrombin generation associated with reduced antithrombin III concentration. This increase in coagulation may contribute to the early and late risks of thromboembolism observed after the Fontan procedure. We did not find any regional differences in coagulation abnormalities in patients late after the Fontan procedure. Therefore, the mechanisms and causes of the coagulation abnormalities remain unclear.

Minimal access approach for the repair of atrial septal defect: the initial 135 patients.

BACKGROUND: From May 1996 to August 1998 a minimal access approach was used for 135 of 200 consecutive surgical atrial septal defects closures in children through young adults ranging in age from 6 months to 25 years (median 5 years). METHODS: A 3.5- to 5-cm midline incision was centered over the xiphoid with division of the xiphoid alone (transxiphoid) or of the lower sternum (ministernotomy); both groups underwent bicaval venous cannulation through the incision. Cardioplegia and aortic cross-clamping were administered through the incision. Cephalad retraction of the sternum with a fixed-arm retractor aided exposure. RESULTS: There have been no early or late deaths and no bleeding or wound complications. No procedure required conversion to a full sternotomy, and no cannulation attempt was abandoned for an alternate site. Cross-clamp and cardiopulmonary bypass times were equivalent to those in the full sternotomy group. The mean length of hospital stay in the ministernotomy group was 2.7 days. CONCLUSIONS: The closure of atrial septal defects can be performed through a transxiphoid or ministernotomy approach, conferring a satisfactory cosmetic result without compromising the safety or accuracy of the repair.

Surgical treatment of primary pulmonary sarcomas.

OBJECTIVE: We sought to identify the long-term prognosis after surgical treatment for primary pulmonary sarcoma. METHODS: Twenty-three patients were retrospectively identified as having been treated surgically for primary pulmonary sarcoma between 1981 and 1996. The records of all patients were reviewed, and the histopathology reexamined by a pathologist. RESULTS: Fifteen patients were male and eight female; their ages ranged from 20 to 78 (mean 51) years. Tumors measured between 0.9 and 12.0 (mean 5.2) cm across the greatest diameter. The histologic diagnoses were malignant fibrous histiocytoma (8, three grade 1 or 2, two grade 3), synovial sarcoma (4), malignant schwannoma (3), leiomyosarcoma (3), and one case each of angiosarcoma, intimal sarcoma, epitheloid hemangioendothelioma, fibrosarcoma and primitive neuroectodermal tumor. Three patients were found to be unresectable. All three underwent radiation and chemotherapy. Lobectomies or bilobectomies were performed in 13 patients including two sleeve resections, one carinal resection, and one chest wall resection. Four patients underwent radical pneumonectomies. Three patients with invasion of the pulmonary artery, pulmonary veins or atrial wall underwent extended resections with the use of cardiopulmonary bypass. In two, a homograft was used to reconstruct the right ventricular outflow tract. Of the resected patients, six had a positive resection margin, and four had at least one positive lymph node in the specimen. Three patients underwent repeat pulmonary resections for recurrences. Eleven patients received postoperative chemotherapy and eight had radiation therapy. Follow-up was available on 22 patients, and ranged from 2 to 183 (mean 48) months; 14 patients are disease free, six died of disease, one died of surgical complications (operative mortality 5%), and two are alive with disease. Actuarial 3- and 5-year survival of the resected patients was 69%. Size and grade were not found to be correlated with significantly increased survival, but completeness of resection was (P<0.05). CONCLUSIONS: Resection of primary pulmonary sarcomas can produce an acceptable survival rate if the resection is complete. Cardiopulmonary bypass can be a useful adjunct when tumors involve a resectable area of the heart or great vessels.