Comprehensive outcomes after primary arterial switch operation.

OBJECTIVE: To report the long-term outcomes of the arterial switch operation for transposition of the great arteries. METHODS: The data of 35 patients who underwent an arterial switch operation between April 2006 and May 2021 were retrospectively examined; five had double-outlet right ventricles, three had side-by-side transposition, and 27 had d-transposition. The punch-out technique was the basic method for coronary transplantation; however, if coronary artery kinking after additional peeling was a concern, the trap-door technique was used. In aortic reconstruction, the caliber difference between the neoascending aorta and the native pulmonary artery was fixed using the pulmonary artery flap. In pulmonary reconstruction, the pulmonary artery was bilaterally exposed over the second branch. RESULTS: The median age at operation and follow-up duration were 10.0 (8.0-12.5) days and 72.9 (40.7-139.5) months, respectively. Thirty-four patients survived, and one was lost to follow-up ([coronary transplantation]: right coronary artery, punch-out = 31, trap-door = 2; left coronary artery, punch-out = 28, trap-door = 7; [aortic regurgitation]: no-slight = 27, mild = 6, moderate = 1; [pulmonary regurgitation]: no-slight = 22, mild = 11, moderate = 1). Slight coronary bending occurred in two patients; ischemia was not observed. Catheter stent placement was performed in one of three patients with stenosis at the pulmonary artery bifurcation. Catheter balloon dilation was performed in one patient with supravalvular pulmonary stenosis. There were no cases of coronary, aortic, or pulmonary artery reoperations. The 15-year cumulative reintervention avoidance rate was 91.0%. CONCLUSIONS: There were no deaths or reoperations during the long-term follow-up period, and arterial switch operation outcomes at our hospital were satisfactory.

Coil Embolization and Left Ventricular Assist Device After Arterial Switch Operation.

Aortopulmonary collateral arteries are frequently found in patients with transposition of the great arteries after catheterization. Although most of them are asymptomatic, it may cause heart failure or pulmonary hemorrhage after an arterial switch operation. Here we report a case of symptomatic aortopulmonary collateral arteries after arterial switch, which necessitated support with a centrifugal pump and left ventricular assistive device because of severe mitral regurgitation and left atrial hypertension and emergent coil embolization. Hemodynamics dramatically improved after embolization. The postoperative period was uneventful, and the infant was released from the hospital on the 14th day.

Primary pulmonary artery reconstruction for functional single ventricle with absent central pulmonary artery and bilateral patent ductus arteriosus.

OBJECTIVE: This study aimed to evaluate and discuss the outcomes of creating a single systemic-pulmonary shunt and reconstruction of the pulmonary artery continuity in patients with a single functional ventricle, absent central pulmonary artery, and bilateral patent ductus arteriosus. METHODS: Six infants diagnosed with a functional single ventricle, absent central pulmonary artery, and bilateral patent ductus arteriosus were treated by creating a single systemic-pulmonary shunt and reconstructing the pulmonary artery continuity (primary operation) between January 2010 and September 2020. Pulmonary artery continuity was ensured using the remnant pulmonary artery and an autologous pericardial patch in five patients and a rolled autologous pericardium in one patient. RESULTS: All patients eventually underwent total cavopulmonary connection. Two patients underwent intrapulmonary artery septation before Glenn or total cavopulmonary connection procedure. The median follow-up period was 9.02 years (interquartile range, 3.90-9.53). No late deaths were observed. CONCLUSIONS: Our strategy of establishing a single systemic-pulmonary shunt with reconstruction of the pulmonary artery continuity was useful for treating patients with a functional single ventricle with absent central pulmonary artery and bilateral patent ductus arteriosus. This procedure helped accomplish pulmonary artery growth and ensured an appropriate volume load after total cavopulmonary connection.

Fatal septic embolism due to Staphylococcus lugdunensis-induced bacteremia.

Staphylococcus lugdunensis infective endocarditis (IE) is very rare in children. A female neonote presented with fever on the 29th postoperative day after undergoing a modified Norwood procedure (right ventricular-pulmonary artery [RV-PA] conduit). Blood cultures were positive for S. lugdunensis. Echocardiography did not demonstrate vegetation. Therefore, we made a diagnosis of catheter-related bacteremia instead of IE. Cultures were negative 3 days after treatment initiation with intravenous vancomycin. One month after discharge, she developed acute hypoxia and could not be revived because of obstruction of RV-PA conduit with large vegetation. Even in S. lugdunensis bacteremia without IE, surgical treatment should be considered early.

Impact of intrapulmonary-artery septation to pulmonary vein obstruction for two-lung Fontan.

OBJECTIVES: Patients with unbalanced pulmonary artery (PA) growth and decreased unilateral pulmonary circulation are considered unsuitable candidates for the Fontan procedure. Following our previous study on the utility of intrapulmonary-artery septation for patients with PA hypoplasia, we investigated its use in patients with pulmonary venous obstruction (PVO). METHODS: We recruited 42 patients who underwent intrapulmonary-artery septation for unilateral PA hypoplasia and/or PVO between 1998 and 2018 and classified them into no PVO or PVO group. We analysed overall survival, success of the Fontan procedure and data from catheterization and echocardiography. In PVO, we evaluated the functional lung area before the Fontan procedure and the relevance of this parameter to operative outcomes. RESULTS: The PVO and no-PVO group included 24 and 18 patients, respectively. One patient in the no-PVO and 6 patients in the PVO group died during follow-up; this difference was statistically significant (log-rank P = 0.040). In the no-PVO group, 15 (83%) patients achieved two-lung Fontan circulation. In the PVO group, 12 (50%) patients achieved two-lung Fontan circulation. Multivariate analysis revealed that functional lung area and shunt size [significantly larger in patients with functional lung area ≥50% in affected lung (P = 0.040)] were significant factors for successful two-lung Fontan procedure (P = 0.030). CONCLUSIONS: Intrapulmonary-artery septation may contribute to increase functional lung area after PVO release to establish two-lung Fontan circulation in patients with PVO who have unbalanced PA growth and/or decreased unilateral pulmonary circulation.

Complete repair with unifocalization of the only unilateral lung.

A boy with 22q11.2 deletion was diagnosed with pulmonary atresia with ventricular septal defect (PAVSD) and major aortopulmonary collateral arteries (MAPCAs). At 8 months, unifocalization of left MAPCAs and BT shunt was performed at another hospital. However, they occluded directly after surgery. An angiography revealed 2 MAPCAs supplying all segments of the right lung. Qp:Qs was 2.24. At 1 year 6 months, we performed VSD closure with unifocalization of the only right lung vascularity, using intraoperative PA flow study. We report a successful case of complete repair with unilateral lung in PAVSD and MAPCAs.

Midterm results and risk factors of functional single ventricles with extracardiac total anomalous pulmonary venous connection.

OBJECTIVES: To evaluate the clinical outcomes of surgical repair of an extracardiac total anomalous pulmonary venous connection (TAPVC) in a functional single-ventricle (f-SV) strategy. METHODS: This was a retrospective analysis of 48 consecutive cases of extracardiac TAPVC repair, from 1998 to 2015. Demographic and clinical variables were as follows: median age, 24 (range 0-744) days; median weight, 3.1 (range 2.0-9.6) kg; type of TAPVC-supracardiac, 21 patients, infracardiac, 8, and mixed, 19; right atrial isomerism, 45 patients; pulmonary atresia, 24 patients; and obstructed TAPVC, 30 patients. Concomitant procedures included systemic-to-pulmonary shunting in 15 patients, pulmonary artery banding in 12, ventricle-to-pulmonary artery shunting in 2, a Norwood procedure in one, a bidirectional Glenn procedure in 16, and a Fontan procedure in 1. RESULTS: The 1- and 5-year cumulative survival rates were 66.0% and 58.0%, respectively. Of the 28 survivors, 22 (78.6%) underwent Fontan completion and 4 (14.3%) a bidirectional Glenn procedure, and 1 (3.6%) was awaiting a bidirectional Glenn procedure. Recurrent pulmonary venous stenosis (PVS) was observed in 17 patients, with a 1- and 5-year rates of freedom from recurrent PVS 59.8% and 53.5%, respectively. Of the 20 post-operative deaths, only 4 were PVS related. Only pre-operative pulmonary atresia was identified as an independent risk factor of mortality after TAPVC repair. CONCLUSIONS: The midterm surgical outcomes of f-SV with extracardiac TAPVC were acceptable. Moreover, among survivors, Fontan completion can be sufficiently expected. Further improvement, with the development of a comprehensive treatment strategy, is required for this patient group.

Pediatric Case of Staphylococcus lugdunensis-Induced Infective Endocarditis at Bovine Jugular Vein.

A 2-year-old boy underwent repeat right ventricular outflow tract reconstruction (re-RVOTR) with a bovine jugular vein (BJV) graft. He presented with high fever on postoperative day 6. Blood and drainage effusion cultures were all positive for Staphylococcus lugdunensis and vancomycin was prescribed. Echocardiography showed vegetation at the BJV. Re-re-RVOTR was performed 51 days after re-RVOTR. In the operation, vegetation was adhered to the right-sided leaflet and three leaflets were degenerated. After complete BJV graft resection, an expanded polytetrafluoroethylene conduit with trileaflets was implanted. There was no sign of recurrent infection 8 months after the surgery.

Novel Left Atrioventricular Valvuloplasty for Atrioventricular Septal Defect.

An infant boy with 21 trisomy was diagnosed with complete atrioventricular septal defect-Rastelli type A with parachute left atrioventricular valve and absent left mural leaflet. Biventricular repair was difficult in infancy because the left ventricular volume and left atrioventricular valve annulus were too small. After 2 pulmonary artery bandings, the left atrioventricular valve annulus increased to 85% of normal mitral valve, and the left ventricular end-diastolic volume increased to 98% of normal. We report a successful instance of complete atrioventricular septal defect that achieved biventricular repair via novel left atrioventricular valvuloplasty for parachute left atrioventricular valve and absent left mural leaflet.

Long-term outcome of patients with right atrial isomerism after common atrioventricular valve plasty.

OBJECTIVES: To review long-term outcomes of patients with right atrial (RA) isomerism who underwent common atrioventricular valve (CAVV) plasty. METHODS: We retrospectively analysed 59 patients with RA isomerism operated on between January 2004 and April 2016. We divided patients into those with CAVV plasty (CAVV plasty (+), 29 patients) and without CAVV plasty (CAVV plasty (-), 30), and we compared the outcome between the groups. We further divided patients into those with CAVV plasty before bidirectional cavopulmonary shunt (BCPS) operation (group before BCPS, 13 patients) or CAVV plasty with or after BCPS (group with or after BCPS, 16), and we compared the outcome between these groups. We reviewed the outcomes of 7 neonatal patients who underwent CAVV plasty. RESULTS: Kaplan-Meier estimated survival rates at 10 years were 70 ± 10% and 69 ± 9% in the CAVV plasty (+) and CAVV plasty (-) groups, respectively ( P = 0.45). Kaplan-Meier estimated survival rates at 10 years were 47 ± 17% and 85 ± 10% in the group before BCPS and group with or after BCPS, respectively ( P = 0.01). Among 7 neonates in the group before BCPS, 4 are alive; Kaplan-Meier estimated survival rates at 1 year and 5 years were 60 ± 20% and 30 ± 24%, respectively. CONCLUSIONS: Patients who underwent CAVV plasty with or after BCPS had good outcomes; the outcome of patients with CAVV plasty was the same as that of those without CAVV plasty. Treatment for patients who require CAVV plasty before BCPS, especially neonates, is challenging.

Modification of hemi-Fontan operation for patients with functional single ventricle and anomalous pulmonary venous connection to the superior vena cava: mid-term results.

OBJECTIVES: Fontan candidates with mixed totally anomalous pulmonary venous connection often have postoperative pulmonary venous obstruction after cavopulmonary anastomosis. Because some pulmonary venous obstructions have no intimal hypertrophy at reoperation, we considered such pulmonary venous obstructions to be caused by 3D deformities arising from dissection or mobilization of the vessels, and hypothesized that keeping the pulmonary venous branches in a natural position could avoid such obstruction. Here, we evaluated a modified hemi-Fontan strategy consisting of minimal dissection with no division of vessels and patch separation between systemic and pulmonary venous flow. METHODS: We retrospectively reviewed clinical records of infants with a functional single ventricle and supracardiac anomalous pulmonary venous connection who had undergone this procedure between 2002 and 2012. RESULTS: Nine infants underwent this procedure (median age, 5.6 months; range 3.2-30), all with right atrial isomerism and several pulmonary venous branches directly and separately connecting to the superior vena cava. In 5 patients, all pulmonary veins drained into the superior vena cava; in 1, the right pulmonary veins drained into the superior vena cava and in 3, a pulmonary venous branch drained into the superior vena cava. The median follow-up was 6.9 years (0.8-13 years). Three patients underwent reoperation for postoperative pulmonary venous obstruction caused by intimal hypertrophy; however, we confirmed no pulmonary venous obstruction caused by 3D deformities on the pulmonary venous branches connecting separately to the superior vena cava. Although 2 patients were effectively relieved from pulmonary venous obstruction, 1 died due to recurrent pulmonary venous obstruction. There was no late death and no sinus-node dysfunction. Eight patients underwent successful Fontan operation and catheterization. The median interval from the Fontan operation to the latest catheterization was 3.7 years (0.9-3.7 years). The median arterial oxygen saturation was 94% (91-97%) and the central venous pressure was 12 mmHg (8-14 mmHg); no deficiency of pulmonary arteries and veins was noted. CONCLUSIONS: For patients with functional single ventricle and anomalous pulmonary venous connections to the superior vena cava, our novel strategy of second-stage palliation could avoid postoperative pulmonary venous obstruction caused by 3D deformities, but may not eliminate pulmonary venous obstruction caused by intimal hypertrophy.

Efficacy of the 'intrapulmonary-artery septation' surgical approach for Fontan candidates with unilateral pulmonary arterial hypoplasia†.

OBJECTIVES: The bilaterally unbalanced development of pulmonary arteries (PAs), as a result of unilateral pulmonary arterial hypoplasia (PAhypo) makes patients either ineligible for the Fontan operation or candidates for the one-lung Fontan operation. In the present study, we examined the efficacy of intrapulmonary-artery septation (IPAS), a technique we reported in 2007 in which a septation is constructed within the central PA, in patients with unilateral PAhypo. METHODS: Sixteen patients with unilateral PAhypo and an affected PA index of ≤60 mm(2)/m(2), including non-confluent PA (NCPA), underwent IPAS between January 2000 and March 2012; patients with pulmonary venous obstruction were excluded from this study. We compared the affected PA index values before and after IPAS and after the Fontan operation as well as the bilateral pulmonary blood flow ratio using pulmonary scintigraphy. The post-Fontan operation values of central venous pressure (CVP), pulmonary vascular resistance (PVR), ventricular end-diastolic pressure (VEDP), cardiac index (CI) and arterial oxygen saturation (SaO2) at the most recent cardiac catheterization, as well as the pre-IPAS and post-Fontan New York Heart Association (NYHA) classification levels were examined. RESULTS: One patient died after IPAS (survival rate, 93.8%). Thirteen (86.7%) of the surviving 15 patients underwent the Fontan operation. The mean PA indexes were 25.5 ± 18.9 mm(2)/m(2) before IPAS and 67.9 ± 34.2 mm(2)/m(2) after IPAS (P = 0.003); the mean PA index was 71.1 ± 50.0 mm(2)/m(2) after the Fontan operation. Restenosis did not occur after the Fontan operation, and the PA diameters were maintained. The mean affected/unaffected lung blood flow ratio was 0.89 ± 0.67. The most recent cardiac catheterization after the Fontan operation indicated the following values: CVP, 11.7 ± 1.8 mmHg; PVR, 1.3 ± 0.4 U m(2); EDP, 5.7 ± 2.0 mmHg; CI, 3.1 ± 0.5 l/min/m(2) and SaO2, 94.9 ± 2.0%. The mean pre-IPAS and post-Fontan NYHA levels were 2.3 ± 0.6 and 1.2 ± 0.4, respectively (P = 0.0002). CONCLUSIONS: With IPAS, the affected PA diameters increased significantly, and were maintained after the Fontan operation, and continuity of the native PAs was achieved. IPAS is very effective for patients suffering from otherwise intractable diseases.

Primary central pulmonary artery plasty for single ventricle with ductal-associated pulmonary artery coarctation.

BACKGROUND: Infants with a single ventricle who require a modified Blalock-Taussig shunt can have stenosis at the ductal insertion site on the pulmonary artery (PA). We hypothesized that primary PA plasty in the first palliation would limit PA stenosis and unbalanced pulmonary perfusion, and thereby facilitate safe accomplishment of the next operation. METHODS: From 1998 to 2012, functional single-ventricle patients with pulmonary atresia were managed as follows: initial operative strategy based on midline approach, using cardiopulmonary bypass, and central PA plasty (resection of all ductal tissue). All patients underwent catheter study and lung perfusion test before bidirectional cavopulmonary shunt (BCPS). RESULTS: Forty consecutive patients (heterotaxy, n = 27; others, n = 13) were enrolled in this study. Seven patients (17.5%) had repair of obstructed total anomalous pulmonary venous drainage, and 4 (10%) had repair of common atrioventricular valve at the first palliative operation. Fifteen patients (37.5%) underwent first palliation in the neonatal period. The mean PA index (mm(2)/m(2)) before BCPS was 230.7 ± 101.7. No pulmonary stenosis was detected before BCPS. Pulmonary artery diameter ratio (nonshunt side/shunt side) was 0.93 ± 0.25; pulmonary lung perfusion ratio (nonshunt side/shunt side) was 0.9 ± 0.2. A multivariate analysis identified persistent total anomalous pulmonary venous drainage (extracardiac type; p = 0.002) as a factor associated with unbalanced branch pulmonary arteries at BCPS. Thirty-five patients underwent BCPS, and 31 underwent Fontan procedures. There were 4 early deaths and 4 late deaths. CONCLUSIONS: The strategies we have employed facilitate safe accomplishment of the next-stage operation in diverse anatomic groups, with avoidance of unbalanced pulmonary vascular bed.

Permanent epicardial pacing lead implantation for scheduled multistage operations in Fontan candidate.

The use of an epicardial pacing lead may be essential in pediatric patients with rhythm disturbances. In patients with single-ventricle physiology who need scheduled multistage operations, the lead could be an obstacle because of the induction of tight adhesions and the need for dissection in the next operation. We propose a novel reproducible technique for the placement of an epicardial pacing lead followed by easy dissection of the entire length of the lead in the next operation in neonates or young infants who need multistage operations.