[Repeated Pericardial Effusion Leading to the Diagnosis of Synovial Sarcoma:Report of a Case].

The patient is a 76-year-old man. His chief complaint of chest pain led to a diagnosis of pericardial effusion of unknown cause, and pericardial drainage was performed. On the 30th day, chest pain appeared again. Echocardiography revealed a pericardial fluid reaccumulation and a substantial mass in the pericardial space. Surgical drainage was performed to find the cause. A hematoma/mass was present on the epicardium. The pericardial sac was filled with hematoma. The hematoma was removed, but part of the mass infiltrated close to the anterior descending branch of the left coronary artery, and removal of that part was abandoned. The intrapericardial hematoma and epicardium were submitted to pathology leading to the diagnosis of synovial sarcoma. The patient was discharged home 14 days after surgery.

Surgical Outcomes in Children With Partial and Transitional Atrioventricular Septal Defect.

BACKGROUND: This study aimed to evaluate the long-term outcomes of partial and transitional atrioventricular septal defect repair, focusing on left atrioventricular valve reoperation. METHODS: We conducted a retrospective review of 104 children who underwent partial or transitional atrioventricular septal defect repair between August 1983 and January 2021. The major outcomes included patient survival and left atrioventricular valve reoperation. RESULTS: Median age at repair was 4.0 (0.1-17.0) years, with 17 patients being infants (age ≤ 1 year; 16.3%). All but eight patients (92.3%) underwent left atrioventricular valve cleft closure. After initial repair, there were 18 cases of moderate-to-severe left atrioventricular valve regurgitation (17.3%). Three in-hospital deaths (2.9%) and four late deaths (3.8%) occurred. At follow-up (median 14.3 years), actuarial survival was 95.1% and 93.0% at 1 and 20 years, respectively, and 16 patients (15.4%) had undergone a total of 19 left atrioventricular valve reoperations. Initial repair performed during infancy was associated with significantly higher mortality than a repair performed after infancy (35.3% vs 1.5%, P < .01, hazard ratio = 26.4). On multivariable analysis, repair during infancy was associated with mortality (P < .01, hazard ratio = 27.4, 95% confidence interval = 2.7-283). Partial or no cleft closure of left atrioventricular valve (P = .03, hazard ratio = 4.7, 95% confidence interval = 1.2-18.8) and moderate-to-severe left atrioventricular valve regurgitation after repair (P < .01, hazard ratio = 9.9, 95% confidence interval = 3.0-32.2) were associated with left atrioventricular valve reoperation. CONCLUSIONS: Partial and transitional atrioventricular septal defect repair outcomes were generally satisfactory. However, repair during infancy had worse survival outcomes, and moderate-to-severe left atrioventricular valve regurgitation after a repair was associated with future left atrioventricular valve reoperation.

Surgical outcomes of reoperation after Fontan completion.

OBJECTIVES: Patients who have achieved Fontan circulation may require reoperation. We reviewed the outcomes of reoperation after Fontan completion and assessed the risk factors for poor outcomes. METHODS: This was a retrospective study of 106 patients undergoing open-heart reoperations after Fontan completion in 2003 at a single institution. RESULTS: The mean age at reoperation was 24.6 ± 8.3 years. A history of Fontan failure or end-organ dysfunction was noted in 30 patients. The reoperations included 73 total cavopulmonary connection conversions, 29 atrioventricular or semilunar valve operations (17 with total cavopulmonary connection conversions) and 4 other operations. Eight early deaths occurred. During a median follow-up of 5.5 (0.01-16.2) years, there were 3 late deaths and 9 second cardiac operations. The 10-year survival rate after reoperation was 89.8%, and the 5-year second cardiac operation-free survival was 84.3%. The 10-year survival rates were significantly lower in patients who underwent surgery before 2011 (75.8% vs 100%), had a history of Fontan failure or end-organ dysfunction (71.7% vs 97.3%), had preoperative central venous pressure >15 mmHg (64.9% vs 96.5%) and were operated on with deep hypothermic circulatory arrest (DHCA) (60.0% vs 91.3%). A history of Fontan failure or end-organ dysfunction, preoperative central venous pressure >15 mmHg and requirement of DHCA were identified as risk factors for mortality. CONCLUSIONS: Reoperation after Fontan completion resulted in excellent mid-term outcomes. A history of failed Fontan circulation and the requirement of DHCA negatively affected survival outcomes.

Outcome of Pulmonary Autograft After the Ross Procedure.

BACKGROUND: The purpose of this study was to assess autograft function after the Ross procedure and to review surgical outcomes associated with autograft reoperations. METHODS: This is a retrospective study of patients undergoing the Ross procedure since 1993. Autograft function and autograft reoperation were studied. Autograft failure was defined as more than moderate autograft regurgitation or autograft dilatation to more than 50 mm diameter or z-score of more than +4 in children. One hospital death was excluded from analysis as were patients with unknown late autograft status. RESULTS: Among 75 patients analyzed, preoperative diagnosis before the Ross procedure included aortic regurgitation in 26, aortic stenosis in 19, combined lesions in 28, and 2 mechanical valve malfunctions. Median age at the Ross procedure was 12.1 (0.4-43.6) years with 44 children less than 15 years old. Six patients had greater than mild autograft regurgitation at post-Ross hospital discharge. During median follow-up of 14.9 years, there were 23 autograft failures. Eighteen autograft reoperations were performed on 17 patients (13 children), including 12 aortic valve replacements, 5 aortic root replacements (including 1 valve-sparing root replacement), and 1 Konno procedure. Freedom from autograft failure and autograft reoperation at 20 years after the Ross procedure was 52.0% and 66.3%, respectively. Multivariate analysis identified greater than mild autograft regurgitation at hospital discharge from Ross procedure as a risk factor for autograft failure (P < .01). All patients who underwent autograft reoperation survived and had good health status at a median of 6.9 years after the reoperation. CONCLUSIONS: The Ross procedure is effective in delaying prosthetic aortic valve replacement, although the time-related risk of autograft failure is a real consideration.

Right Ventricle to Pulmonary Artery Conduit With Tricuspid Expanded Polytetrafluoroethylene Valves.

BACKGROUND: Excellent outcomes of right ventricle to pulmonary artery conduits with expanded polytetrafluoroethylene (ePTFE) valves have been reported. The purpose of this study was to evaluate the outcomes of the different material conduits with tricuspid ePTFE valves. METHODS: Forty-one consecutive patients who received right ventricle to pulmonary artery conduit with tricuspid ePTFE valves for biventricular repair between April 2004 and December 2016 were studied. The conduits made of autologous pericardial roll or xenograft roll were used in 22 patients (group P) and the conduits made of ePTFE tube were used in 19 patients (group G). The conduit reoperation and the conduit dysfunction were analyzed. RESULTS: During the median follow-up of 5.8 years, no death related to the conduit was observed. There were four reoperations (three in group P and one in group G). Freedom from conduit reoperation at 5 years was 100% in both groups (P = .30). Freedom from more than moderate conduit stenosis at 5 years after operation was not significantly different between groups (46.9% in group P vs 76.3% in group G, P = .23) even though the group G conduits were significantly smaller and freedom from more than moderate conduit regurgitation at 5 years was significantly better in group G (63.3% in group P vs 94.1% in group G, P = .04). CONCLUSIONS: The conduit with ePTFE valves in the ePTFE tubes had better conduit function compared with the conduit with autologous pericardial or xenograft roll, especially in terms of conduit regurgitation.

Mid- to long-term surgical outcomes of partial anomalous pulmonary venous connection.

OBJECTIVE: To investigate mid- to long-term morbidity and mortality after the repair of partial anomalous pulmonary venous connections (PAPVCs). METHODS: We retrospectively analyzed the cases of 29 consecutive patients who underwent surgery in 1991-2010 at Tokyo Women's Medical University Hospital. Median follow-up period: 9.9 years. Median age at operation: 7.4 years. Median body weight: 22.4 kg. Right PAPVC (n = 23), left-PAPVC (n = 5), and mixed PAPVC (n = 1) were identified. RESULTS: In the 14 patients with a right upper pulmonary vein (PV) connected to the superior vena cava (SVC) and/or cavo-atrial junction, the following were performed: single-patch diversion (n = 6), double-patch diversion (n = 4), Warden procedure (n = 1), and atrial septation with the atrial wall (n = 3). In the nine patients with a right anomalous PV connected to the right atrium (RA) and/or coronary sinus, intra-atrial rerouting was performed (n = 7) and translocation of the atrial septum (n = 2). Intra-atrial rerouting of the left upper and lower PVs to the coronary sinus (n = 1) and direct anastomosis of the left upper PV to the left atrial appendage (n = 4) were performed. Double-patch diversion and direct anastomosis of the left upper PV to the left atrial appendage was performed in a patient with mixed PAPVC. There were no post-operative deaths or reoperations, although transient sinus node dysfunction occurred (n = 2 with atrial septation and intra-atrial rerouting), while mild stenosis of the SVC occurred (n = 1 with double-patch diversion). CONCLUSIONS: The various methods which we used for PAPVC repair all appear to have reasonable outcomes, although rhythm disturbance and stenotic complications were confirmed in a few patients.

Late Neo-Aortic Valve Regurgitation Long After Arterial Switch Operation.

BACKGROUND: The purpose of this study is to assess risk factors of neo-aortic regurgitation (AR) after an arterial switch operation and outcomes of neo-aortic valve surgery. METHODS: This is a retrospective study of 469 hospital survivors after arterial switch operation from 1982 to 2016. Preoperative diagnoses included 285 transpositions of the great arteries with intact septum, 140 transpositions with ventricular septal defect, and 44 double-outlet right ventricles. More than mild preoperative pulmonary regurgitation was found in 15 patients. Median age at the operation was 1.0 months (range, 0.1-81.5). Concomitant relief of left ventricular outflow tract obstruction was performed in 20 patients. Seventy-seven patients had more than mild neo-AR at hospital discharge. RESULTS: More than moderate neo-AR was found in 41 patients (8.6%) at a median follow-up of 19.0 years (range, 0.1-35.2). Multivariate analysis identified more than mild preoperative pulmonary regurgitation, concomitant relief of left ventricular outflow tract obstruction, and more than mild neo-AR at hospital discharge as risk factors for late neo-AR. Seventeen patients (3.6%) underwent neo-aortic valve surgeries, including 11 aortic valve replacements, 3 aortic valve plasties, 2 Konno procedures, and 1 aortic root replacement, with median age at the neo-aortic valve surgery of 16.5 years (range, 6.2-27.3). There were 2 late deaths and 5 reoperations to the neo-aortic valve in 4 patients. CONCLUSIONS: Concomitant relief of left ventricular outflow tract obstruction and preoperative more than mild pulmonary regurgitation increase the risk of neo-AR after the arterial switch operation. Reoperation for neo-AR can be done safely.

Repair of Atrioventricular Septal Defect and Transposition in Left Isomerism.

We report the successful primary anatomic correction of an atrioventricular septal defect with transposition of the great arteries and pulmonary stenosis in a 4-year-old girl with left isomerism by common atrioventricular valve division, Mustard-type atrial septation, and a Rastelli operation with ventricular septal defect enlargement. To the best of our knowledge, this is the first report of total correction for this combination of cardiac anomalies.