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

AbstractObjective: 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. (J Thorac Cardiovasc Surg 2000;120:778-82

Outcomes of inferior sinus venosus defect repair

ObjectiveInferior sinus venosus defect is an unusual form of interatrial communication with few published data on surgical outcomes. We sought to compare outcomes of surgical repair of inferior sinus venosus defect with those of large secundum atrial septal defects.MethodsPatients undergoing surgical closure of an isolated interatrial defect were reviewed, and those with inferior sinus venosus defect were identified on the basis of predetermined anatomic criteria. For each case, 2 controls with secundum atrial septal defect, matched for age and year of surgery, were selected. Technical outcome scores and other perioperative outcomes were compared.ResultsCompared with the secundum atrial septal defect group (n = 90), the inferior sinus venosus defect group (n = 45) had worse technical outcome scores (P = .02), a higher rate of reintervention (9% vs 1%, P = .04), longer median total cardiopulmonary bypass (48 vs 39 minutes, P < .001) and crossclamp (29 vs 20 minutes, P < .001) times, and were more likely to stay more than 1 day in the intensive care unit (20% vs 8%, P = .04) and more than 3 days in the hospital (29% vs 13%, P = .03). Only 16 (36%) of the patients with inferior sinus venosus defect had a correct diagnosis preoperatively. Patients with an incorrect diagnosis had worse technical outcome scores than the secundum atrial septal defect group (P = .003), whereas those with a correct diagnosis had scores similar to those of the secundum atrial septal defect group (P = .55).ConclusionsCompared with patients with secundum atrial septal defect, patients with inferior sinus venosus defect have more residual defects and longer durations of cardiopulmonary bypass and hospitalization. Rates of misdiagnosis of inferior sinus venosus defect are high and associated with worse technical outcome scores. Accurate preoperative diagnosis of this lesion may lead to improved outcomes

Perventricular device closure of muscular ventricular septal defects on the beating heart: technique and results

AbstractObjectiveBoth surgical management and percutaneous device closure of muscular ventricular septal defects have drawbacks and limitations. This report describes our initial experience with intraoperative device closure of muscular ventricular septal defects without cardiopulmonary bypass in 6 consecutive patients.MethodsA median sternotomy or a subxiphoid minimally invasive incision was performed. Under continuous transesophageal echocardiographic guidance, the right ventricle free wall was punctured, and a wire was introduced across the largest defect. The Amplatzer (AGA Medical Corporation, Golden Valley, Minn) muscular ventricular septal defect occluding device (a self-expandable double-disk device) was used. An introducer sheath was fed over the wire, with the sheath tip positioned in the left ventricle cavity. The device was then advanced inside the sheath and deployed by retracting the sheath. Associated cardiac lesions, if any, can then be repaired during cardiopulmonary bypass. A similar technique can also be applied for periatrial closure of complex atrial septal defects.ResultsThe initial 6 patients are presented. Cardiopulmonary bypass was not needed in any patient for placement of the device and needed in 4 patients for repair of concomitant malformations only (double-outlet right ventricle, aortic arch hypoplasia, pulmonary artery band removal). No complications from using this technique occurred. Discharge echocardiograms showed no significant shunting across the ventricular septum.ConclusionsPerventricular closure of multiple muscular ventricular septal defects is safe and effective. We believe that this could become the treatment of choice for any infant with muscular ventricular septal defects or any child with muscular ventricular septal defect and associated cardiac defects

Early repair of hemitruncus: Excellent early and late outcomes

ObjectiveAnomalous origin of 1 of the branch pulmonary arteries from the aorta with 2 normal semilunar valves (hemitruncus) is a rare entity. There have been several small case series reported. We report here our single-institution surgical experience with hemitruncus from 1982 to 2006.MethodsA retrospective case review of all cases of conotruncal anomalies at Children’s Hospital Boston revealed 16 patients with hemitruncus. Ten patients had surgery in the neonatal period (<30 days), 4 at 1 to 6 months, 1 at 8 months, and 1 at 2 years. Diagnosis of hemitruncus was the indication for operation in all but 1. Fourteen of the 16 had anomalous right pulmonary artery from aorta, and 2 had left pulmonary artery from aorta. Common associated anomalies included patent foramen ovale in 14, patent ductus arteriosus in 11, and ventricular septal defect in 4. All patients had elevated right ventricular pressures with systemic pressures in 5 and suprasystemic pressures in 9.ResultsThere was 1 operative death in this series in an infant who died from sepsis following ligation of a tracheoesophageal fistula. One patient required reoperation for supravalvar aortic stenosis and right pulmonary artery stenosis 1 year postoperatively. Three patients required 4 catheter-based interventions postoperatively. At 20 years, survival by Kaplan–Meier was 93%; freedom from reoperation, 93%; and freedom from catheter reintervention, 79%.ConclusionsEarly repair of hemitruncus results in excellent hemodynamic and anatomic results. Survival is excellent, with a low incidence of reoperation or reintervention

Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart

ObjectiveBorderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenoses, left ventricular hypoplasia) and endocardial fibroelastosis. The multilevel obstruction and impaired left ventricular systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of left ventricular rehabilitation—endocardial fibroelastosis resection with mitral or aortic valvuloplasty—on left ventricular function and clinical outcomes.MethodsAll patients with borderline left heart structures and endocardial fibroelastosis who underwent a primary left ventricular rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from preoperative and postoperative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after left ventricular rehabilitation. Preoperative and postoperative values were compared by paired t test.ResultsBetween 1999 and 2008, 9 patients with endocardial fibroelastosis and borderline left heart disease underwent left ventricular rehabilitation at a median age of 5.6 months (range, 1–38 months). There was no operative mortality, and at a median follow-up of 25 months (6 months to 10 years) there was 1 death from noncardiac causes and 2 patients required reoperations. Significant increases in ejection fraction and left ventricular end-diastolic volume were observed, whereas left atrial pressure and right ventricular/left ventricular pressure ratios decreased postoperatively.ConclusionIn patients with borderline left hearts, primary left ventricular rehabilitation with endocardial fibroelastosis resection and mitral and aortic valvuloplasty results in improved left ventricular systolic and diastolic performance and decreased right ventricular pressures. This approach may provide an alternative to single-ventricle management in this difficult patient group

Surgery for bilateral outflow tract obstruction in elastin arteriopathy

AbstractObjective: 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. (J Thorac Cardiovasc Surg 2000;120:755-63

A Novel Restorative Pulmonary Valve Conduit : early Outcomes of Two Clinical Trials

Objectives: We report the first use of a biorestorative valved conduit (Xeltis pulmonary valve-XPV) in children. Based on early follow-up data the valve design was modified; we report on the comparative performance of the two designs at 12 months post-implantation. Methods: Twelve children (six male) median age 5 (2 to 12) years and weight 17 (10 to 43) kg, had implantation of the first XPV valve design (XPV-1, group 1; 16 mm (n = 5), and 18mm (n = 7). All had had previous surgery. Based on XPV performance at 12 months, the leaflet design was modified and an additional six children (five male) with complex malformations, median age 5 (3 to 9) years, and weight 21 (14 to 29) kg underwent implantation of the new XPV (XPV-2, group 2; 18 mm in all). For both subgroups, the 12 month clinical and echocardiographic outcomes were compared. Results: All patients in both groups have completed 12 months of follow-up. All are in NYHA functional class I. Seventeen of the 18 conduits have shown no evidence of progressive stenosis, dilation or aneurysm formation. Residual gradients of >40 mm Hg were observed in three patients in group 1 due to kinking of the conduit (n = 1), and peripheral stenosis of the branch pulmonary arteries (n = 2). In group 2, one patient developed rapidly progressive stenosis of the proximal conduit anastomosis, requiring conduit replacement. Five patients in group 1 developed severe pulmonary valve regurgitation (PI) due to prolapse of valve leaflet. In contrast, only one patient in group 2 developed more than mild PI at 12 months, which was not related to leaflet prolapse. Conclusions: The XPV, a biorestorative valved conduit, demonstrated promising early clinical outcomes in humans with 17 of 18 patients being free of reintervention at 1 year. Early onset PI seen in the XPV-1 version seems to have been corrected in the XPV-2, which has led to the approval of an FDA clinical trial

Survival and Clinical Course at Fontan After Stage One Palliation With Either a Modified Blalock-Taussig Shunt or a Right Ventricle to Pulmonary Artery Conduit

ObjectivesWe sought to determine whether the type of shunt used at stage one palliation (S1P) affected the survival and the perioperative course through Fontan completion.BackgroundAlthough improved surgical and interstage survival have been demonstrated with the use of the right ventricle to pulmonary artery (RV-PA) conduit compared with a modified Blalock-Taussig shunt (BTS) at S1P, it is unknown whether this effect will be observed in long-term follow-up.MethodsAll patients who underwent a S1P during 2002 and 2003 (n = 80) at our institution were included for analysis. Patients were followed until death or June 1, 2007. Perioperative variables at Fontan completion were recorded.ResultsFor the entire cohort, cumulative survival for those who underwent a RV-PA conduit (n = 34) was 79.4% at 3 years compared with 65.8% in the modified BTS group (n = 46) (log-rank = 0.31). At Fontan (n = 44), when compared with those who had received a modified BTS, those who had a RV-PA conduit placed at S1P had no difference in the median duration of ventilation (21 h [range 10 to 96 h] vs. 26.5 h [range 7 to 204 h], p = 0.09) or hospital stay (9 days [range 5 to 29 days] vs. 10 days [range 6 to 48 days], p = 0.89), although length of stay in the intensive care unit was shorter (2 days [range 0 to 6 days] vs. 4 days [range 1 to 25 days], p = 0.01). Sixty-seven percent of the RV-PA conduit group had at least one PA intervention 3 years after S1P compared with 42.8% in the modified BTS group (log-rank = 0.11).ConclusionsNonstatistically significant trends toward improved cumulative survival and increased PA interventions were demonstrated in patients who had a RV-PA conduit placed at S1P. Longitudinal follow-up of larger groups of randomized patients is required to determine the influence of the RV-PA conduit on long-term outcomes