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.

Intra-Extracardiac Total Cavopulmonary Connection for Patients With Anatomical Complexity.

BACKGROUND: Currently, the extracardiac conduit total cavopulmonary connection (eTCPC) is the most widely used for Fontan modification worldwide. Nevertheless, there have been some cases that are difficult for performing eTCPC because of their anatomical complexity, such as apicocaval juxtaposition. For such cases, in 2002, we introduced the intra-extracardiac TCPC (ieTCPC). METHODS: We reviewed our 20-year single-center experience with 316 TCPC patients to compare eTCPC (n = 277) and ieTCPC (n = 39) in terms of mortality and morbidity. ieTCPC was indicated for the cases in which there was concern that the TCPC conduit would be too curved for ordinary eTCPC. RESULTS: Early death occurred in 1 patient and late death occurred in 15 patients. The actuarial survival rate in the eTCPC and the ieTCPC groups at 10 years were 95.1% and 100.0%, respectively. There was no significant difference in actuarial survival between eTCPC and ieTCPC patients. In the multivariate analysis, preoperative superior vena cava pressure and preoperative oxygen saturation were found to be the independent predictor for postoperative mortality. There was also no significant difference in actuarial rate of freedom from late-occurring complications between eTCPC and ieTCPC groups. In the multivariate analysis, dominant right ventricle and preoperative SVC pressure were independent predictors for late-occurring complications. CONCLUSIONS: The clinical outcomes in patients who undergo eTCPC and ieTCPC appear to be excellent, with low mortality and morbidity rates in the midterm. ieTCPC may be a good option for TCPC cases with anatomical complexity such as apicocaval juxtaposition and separated hepatic vein drainage.

Interannular Bridge: A Novel Approach to Address Congenital Mitral Regurgitation.

A male infant with Kabuki syndrome had trivial congenital mitral regurgitation diagnosed at birth. At the age of 2 years and 9 months, the regurgitation worsened from mild to severe; thus, expedited surgical treatment was pursued. The primary operative finding was severe dysplastic two-leaflet disease. After completing chordal replacement as a conventional repair procedure, more-than-moderate central regurgitation caused by establishing a shallow coaptation between the anterior and posterior leaflets persisted. We report a successful case of mitral valve repair involving the novel option of interannular bridge for valvuloplasty to address congenital mitral regurgitation.

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.

Successful staged repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries in an extremely low birth weight infant.

An extremely low birth weight infant (810 g) was born with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries accompanied by pulmonary overcirculation, which eventually resulted in intestinal hypoperfusion and focal intestinal perforation from the very early stage of life. Based on the echographic findings, we performed banding operations twice to regulate the pulmonary blood flow on day 2 and day 9. At 6 months of age, a definitive repair simultaneous with unifocalization of major aortopulmonary collateral arteries was performed. At 1 year of age, the right ventricle/left ventricle pressure ratio was 0.44 after balloon angioplasty was performed for the right-sided pulmonary artery stenosis. The patient is in a stable condition and was followed-up for more than 2 years after definitive repair. This is the first known successful repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries in an extremely low birth weight infant.

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.

One-stage definitive repair of complete atrioventricular septal defect and pulmonary atresia with major aortopulmonary collateral arteries.

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is considered the most severe end of the tetralogy of Fallot spectrum, whereas complete atrioventricular septal defect associated with tetralogy of Fallot is a rare congenital defect. However, the combination of pulmonary atresia, major aortopulmonary collateral artery and complete atrioventricular septal defect is extremely rare, and surgical repair has not been reported. We describe a successful, 1-stage correction in a 7-month-old boy with this condition, who had an additional complication of a single aortic outlet from the right ventricle and significant atrioventricular valve regurgitation.

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.

A successful staged Fontan operation for a right atrial isomerism neonate having major aortopulmonary collateral arteries and extracardiac total anomalous pulmonary venous connection.

Right atrial isomerism and pulmonary atresia combined with major aortopulmonary collateral arteries are very rare. Surgical treatment becomes more challenging when an extracardiac total anomalous pulmonary venous connection (TAPVC) requiring surgical repair neonatally is also present. We describe a successful staged Fontan operation for a neonate with a single ventricle, right atrial isomerism, pulmonary atresia with major aortopulmonary collateral arteries, a small central pulmonary artery and a supracardiac TAPVC. Unifocalization of the pulmonary vasculature was achieved by ligating all dual-supply major aortopulmonary collateral arteries at their origins, concomitant with supracardiac TAPVC repair and modified Blalock-Taussig shunt placement at 15 days. The patient underwent patch augmentation of the small stenosed central pulmonary artery at 8 months and bilateral bidirectional Glenn shunt at 14 months. The Fontan operation was completed at 27 months using an intra-extracardiac conduit with fenestration. One year later, catheter examination revealed good Fontan circulation with acceptable central venous pressure, oxygen saturation and cardiac function. We also describe a new angiographic concept of central pulmonary arteriography to visualize more accurately the complex pulmonary circulation system with both major aortopulmonary collateral arteries and a central pulmonary artery.

Pulmonary root translocation for borderline complex coarctation of aorta and multiple ventricular septal defects.

We describe a case of complex left ventricular outflow tract obstruction, multiple muscular ventricular septal defects, aortic coarctation and a hypoplastic aortic arch, where staged biventricular repair was performed successfully using pulmonary root translocation.

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.

Extra-aortic commissuroplasty concomitant with fenestrated Fontan operation for neoaortic valve regurgitation after Norwood reconstruction.

We introduce a simple, less invasive surgical technique for treating neoaortic valve regurgitation (neoAR) after the Norwood procedure, with the aim of delaying reoperation for neoAR. A 31-month-old girl, with hypoplastic left heart syndrome, previously underwent 4 median sternotomies and was admitted to our hospital for a fenestrated Fontan operation. She presented with moderate neoAR, originating from a tricuspid neoaortic valve (neoAV), with the regurgitation oriented from the centre. Her neoAV annulus was dilated to twice its normal size. With the aim of delaying future neoAV intervention and minimizing the surgical invasiveness, we performed extra-aortic commissuroplasties on the 2 commissures that could be approached from the front during the Fontan operation, without inducing cardiac arrest. We used direct echocardiography and transoesophageal echocardiography to confirm the feasibility before applying this procedure. Her postoperative course was uneventful, and the postoperative echocardiography did not reveal any residual neoAR 5 months postoperatively. We believe that this technique is a useful surgical option for patients with moderate neoAR oriented from its centre and well-balanced tricuspid native pulmonary valves, and it might help to delay future neoAV interventions, with minimal surgical risk.

Modified Nikaidoh procedure with double-root translocation in a 1-year-old boy.

A 1-year-old boy weighing 10.4 kg underwent successful biventricular repair for transposition of the great arteries, a ventricular septal defect, and a left ventricular outflow tract (LVOT) obstruction with moderate pulmonary stenosis of the bicuspid pulmonary valve (z score of -4.4 for the pulmonary valve) by means of a modified Nikaidoh procedure with double root translocation by use of a valve-spared pulmonary root. The postoperative echocardiogram showed no LVOT obstruction, no aortic valve regurgitation, and mild pulmonary stenosis and pulmonary valve regurgitation. No reintervention has been required during the 6-year follow-up, with annular growth of the pulmonary valve.