Fatal overwhelming postsplenectomy infection due to Streptococcus pneumoniae serotype 10A with atypical polysaccharide capsule in a patient with chromosome 22q11.2 deletion syndrome: A case report.

We report the first case of a teenage patient with chromosome 22q11.2 deletion syndrome who died of overwhelming postsplenectomy infection (OPSI) by Streptococcus pneumoniae despite appropriate prevention by pneumococcal vaccine. He had congenital heart disease and underwent several surgeries. Immunodeficiency had not been noticed clinically. Two years prior to death, splenectomy was performed for a drug-resistant idiopathic thrombocytopenic purpura and he was immunized with 23-valent pneumococcal polysaccharide vaccine (PPV23) 4 months after splenectomy. He died suddenly after a mild flu-like symptom. Autopsy was performed and OPSI was diagnosed. Blood culture was positive for S. pneumoniae. This isolated S. pneumoniae strain was serotypically un-typable by polyvalent serum agglutination test. On the contrary, multilocus sequence typing followed by DNA sequencing indicated the molecular serotype as 10A. Additional testing using monovalent and factor-specific sera confirmed the strain as serotype 10A. Ultrastructural observation of this S. pneumoniae strain showed that the polysaccharide capsule was thin and sparse. We speculate that the abnormal morphology of the capsule may have accounted for the polyvalent serum agglutination failure and may possibly be associated with severity of OPSI observed in this case. Chromosome 22q11.2 deletion syndrome is associated with certain immunodeficiency, especially susceptible to S. pneumoniae infections; however, fatal OPSI has not been reported. In addition to vaccination, prophylactic antibiotics may be necessary for these patients who are at risk of immunodeficiency.

Hepatic vein redirection to improve pulmonary arteriovenous malformations after Fontan completion in patients with absence of inferior vena cava and hemiazygos continuation.

Pulmonary arteriovenous malformation is an important complication after Fontan completion in patients with univentricular circulation. Lack of hepatic venous flow in a pulmonary artery has been identified as a cause of pulmonary arteriovenous malformation. We report our experience with a case of redirection of the hepatic vein to the hemiazygos vein using a conduit via left thoracotomy and median sternotomy for the correction of unequal distribution of hepatic venous flow in the pulmonary arteries. The pulmonary arteriovenous malformation improved, leading to increased arterial saturation levels.

Post-Extubation Inhaled Nitric Oxide Therapy via High-Flow Nasal Cannula After Fontan Procedure.

In 2014, our hospital introduced inhaled nitric oxide (iNO) therapy combined with high-flow nasal cannula (HFNC) oxygen therapy after extubation following the Fontan procedure in patients with unstable hemodynamics. We report the benefits of HFNC-iNO therapy in these patients. This was a single-center, retrospective review of 38 patients who underwent the Fontan procedure between January 2010 and June 2016, and required iNO therapy before extubation. The patients were divided into two groups: patients in Epoch 1 (n = 24) were treated between January 2010 and December 2013, receiving only iNO therapy; patients in Epoch 2 (n = 14) were treated between January 2014 and June 2016, receiving iNO therapy and additional HFNC-iNO therapy after extubation. There were no significant differences between Epoch 1 and 2 regarding preoperative cardiac function, age at surgery, body weight, initial diagnosis (hypoplastic left heart syndrome, 4 vs. 2; total anomalous pulmonary venous return, 5 vs. 4; heterotaxy, 7 vs. 8), intraoperative fluid balance, or central venous pressure upon admission to the intensive care unit. Epoch 2 had a significantly shorter duration of postoperative intubation [7.2 (3.7-49) vs. 3.5 (3.0-4.6) hours, p = 0.033], pleural drainage [23 (13-34) vs. 9.5 (8.3-18) days, p = 0.007], and postoperative hospitalization [36 (29-49) vs. 27 (22-36) days, p = 0.017]. Two patients in Epoch 1 (8.3%), but none in Epoch 2, required re-intubation. Our results suggest that HFNC-iNO therapy reduces the duration of postoperative intubation, pleural drainage, and hospitalization.

Fontan operation with bilateral conduits for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia after reconstruction of a non-confluent pulmonary artery.

We describe a case involving a successful Fontan procedure for the treatment of pulmonary arteriovenous fistulas in a patient with polysplenia. The patient was diagnosed with bilateral superior vena cavae, interrupted inferior vena cava with azygos connection, wide separate hepatic veins, and central pulmonary artery stenosis following repair of a non-confluent pulmonary artery. Bilateral extracardiac conduits were used to connect each hepatic vein to the pulmonary artery, opposite each superior vena cava. Clinical improvement in the pulmonary arteriovenous fistulas was observed within 4 months after surgery.

Prognosis in tetralogy of Fallot with absent pulmonary valve.

BACKGROUND: Tetralogy of Fallot with absent pulmonary valve (TF/APV) is a rare and severe congenital heart disease with high mortality. The aim of this study was to assess whether TF/APV prognosis is related to fetal and postnatal clinical course and pulmonary artery (PA) configuration. METHODS: The fetal and postnatal echocardiograms and clinical outcomes of 13 patients with TF/APV (diagnosed antenatally in 9 patients and postnatally in 4) were reviewed, and divided into two groups: group A (n = 6), alive; and group D (n = 7), dead. RESULTS: Fetal period: group A, polyhydramnios n = 0, hydrops fetalis (HF) n = 0, patent ductus arteriosus (PDA) n = 2; group D, polyhydramnios n = 3, HF n = 2, PDA n = 0. Postnatal period: group A, five patients underwent intracardiac repair, including one requiring artificial ventilation (AV). A further AV patient required three operations before extubation. Postoperative courses were all good. Group D, excluding the two intrauterine fetal deaths, four patients required AV (three of whom died neonatally or in early childhood) and one underwent intracardiac repair. PA configuration: all group A patients had bulbous expansion of left and right PA (clover type). Three patients in group D had bulbous expansion of main PA (balloon type). CONCLUSIONS: PDA was a factor associated with good prognosis. Hydramnion and HF were factors associated with poor prognosis. Given that there was a higher rate of postnatal AV and poorer prognosis in balloon type than in clover type PA (P < 0.05), PA configuration is also considered an important factor to predict postnatal outcome in TF/APV.

Pulmonary artery rupture after bilateral pulmonary artery banding in a neonate with Loeys-Dietz syndrome and an interrupted aortic arch complex: report of a case.

Loeys-Dietz syndrome (LDS) is a recognized connective tissue disorder characterized by progressive aortic aneurysm and dissection. Patients are at high risk of aortic dissection or rupture at an early age, but to our knowledge, surgery on the great arteries has never been attempted in the neonatal period. We report a case of LDS with dilated pulmonary arteries and an interrupted aortic arch complex in a neonate. We performed bilateral pulmonary artery banding, but 12 days after the procedure, the infant died of rupture of the distal portion of the banding sites following massive dilatation.

Contractility-afterload mismatch in patients with protein-losing enteropathy after the Fontan operation.

This study aimed to clarify the relationship between onset of protein-losing enteropathy (PLE) and Fontan circulation, with special reference to the development of contractility-afterload mismatch. The PLE group comprised 9 patients who experienced PLE after undergoing the Fontan operation, and the control group consisted of 32 patients had did not experienced PLE more than 10 years after the Fontan operation. The study compared the pre- and postoperative values of arterial elastance (Ea), end-systolic elastance (Ees), and contractility-afterload mismatch (Ea/Ees). Furthermore, the variations in the values were examined during the preoperative, postoperative, and midterm postoperative periods in seven PLE patients who underwent cardiac catheterization at the onset of PLE and during the pre- and postintervention periods in three PLE patients who underwent surgical intervention to improve the Fontan circulation after the onset of PLE. Comparison of the values obtained before and after Fontan operations showed that the Ea values increased significantly in the PLE group. However, the pre- and postoperative Ees values did not differ in the two groups. During the postoperative period, Ea/Ees increased significantly, and the Ea and Ea/Ees values increased continuously until the onset of PLE in the PLE group. In the patients who underwent surgical intervention to improve the Fontan circulation after the onset of PLE, the Ea/Ees decreased significantly, and the serum albumin levels improved after the intervention. Contractility-afterload mismatch, mainly caused by the increase in the afterload of the systemic ventricle, may have an important role in the development of PLE after the Fontan operation.

Mortality analysis of arterial switch operation for transposition of the great arteries with and without ventricular septal defect.

OBJECTIVES: We aimed to evaluate the 90-day mortality and effect of rescue or urgent coronary revascularization in children undergoing arterial switch operation for transposition of the great arteries with and without ventricular septal defect. METHODS: The 90-day mortality, risk factors for mortality and outcome of rescue or urgent coronary revascularization were analysed using 8 years of data from the Japan Cardiovascular Surgical Database. We only included patients with full data for all analyses. RESULTS: A total of 1084 patients (median weight: 3.0 kg; interquartile range: 2.8-3.3) underwent arterial switch operation at a median age of 10 days (interquartile range: 7-14). The 90-day mortality (5.2%, n = 56) was ∼1.6-fold higher than 30-day mortality (3.2%, n = 35). The cause was cardiac origin in 84% of non-survivors. Fifty-nine of the 1034 patients (5.7%) required extracorporeal membrane oxygenation (ECMO), with successful weaning in 44% (n = 26). Univariable or multivariable analyses revealed the following risk factors for mortality: body weight at operation <2.5 kg, aortic cross-clamp time, cardiac events and ECMO (P < 0.005-0.001). Ventricular septal defect was not a risk factor.Thirteen patients (1.2%) had either rescue or urgent coronary revascularization with salvage rates of 25% (2/8) and 100% (5/5), respectively. Only 5 ECMO patients (8%) underwent coronary revascularization with 1 survivor. CONCLUSIONS: The 90-day mortality represented perioperative outcomes better than 30-day mortality. Patients on ECMO, which extended the survival time, had 56% 90-day mortality. Coronary revascularization showed a salvaging effect, although the case number was small.

Smart Systems for Material and Process Designing in Direct Nanoimprint Lithography Using Hybrid Deep Learning.

A hybrid smart process and material design system for nanoimprinting is proposed, which is combined with a learning system based on experimental and numerical simulation results. Instead of carrying out extensive learning experiments for various conditions, the simulation learning results are partially complimented when the results can theoretically be predicted by numerical simulation. In other words, the data that are lacking in experimental learning are complimented by simulation-based learning results. Therefore, the prediction of nanoimprint results without experimental learning could be realized under various conditions, even for unknown materials. In this study, material and process designs are demonstrated for a low-temperature nanoimprint process using glycerol-containing polyvinyl alcohol. The experimental results under limited conditions were learned to investigate the optimum glycerol concentrations and process temperatures. Simulation-based learning was used to predict the dependence on press pressure and shape parameters. The prediction results for unknown glycerol concentrations agreed well with the follow-up experiments.

[Optimal timing of pediatric surgery to prevent deleterious effects on associated cardiac defects].

Although cardiac defects are thought to have deleterious effects on the outcome of general pediatric surgery due to low cardiac output syndrome and hypoxemia, both pediatric surgery and cardiac surgery can be performed at the optimal timing with good results. However, some conditions requiring pediatric surgery may have deleterious effects on the outcome of cardiac surgery. Airway obstructive diseases sometimes require concomitant repair of the associated cardiac defects. In particular, tracheal stenosis may be repaired in cooperation with not only general pediatric and cardiac surgeons but also with pediatric thoracic surgeons who work in other healthcare institutions. Low birth-weight infants with symptomatic patent ductal artery are at risk of poor outcome. For patients with right isomerism, midgut malrotation or sliding hernia should be diagnosed early and repaired at the optimal timing to prevent urgent surgical intervention. Pediatric surgery should be performed at the optimal timing to prevent any deleterious effects on cardiac defects. Moreover, respiratory infection and neurologic disease should be treated to reduce late deaths.

Active mitral valve endocarditis with widespread interventricular septal abscesses in a paediatric patient.

We present the case of a previously healthy 2-year-old boy with extensive infective mitral valve endocarditis, with 2 huge, mobile vegetations attached to the anterior leaflet of the mitral valve and to the left ventricular outflow tract, and interventricular septal abscesses extending into the left ventricular outflow tract without any septal defects. He underwent mitral valve repair and simultaneous drainage of the interventricular septal abscesses excluding the inlet portion to avoid postoperative complications.

Pulmonary venous obstruction after extracardiac total cavopulmonary connection in right atrial isomerism.

BACKGROUND: Patients with functional single ventricle and right atrial isomerism (RAI) often have multiform cardiac pulmonary venous (PV) connection, which could be a risk factor for pulmonary venous obstruction (PVO) after extracardiac total cavopulmonary connection (EC-TCPC) owing to compression of the conduit. OBJECTIVE: To investigate the anatomical risk factors for PVO after EC-TCPC in RAI. METHODS: Twenty-nine patients with RAI without extracardiac total anomalous pulmonary venous connection were enrolled. No patients had PVO before EC-TCPC. A total of 14 and 15 patients had PV orifices ipsilateral and contralateral to the extracardiac conduit, respectively. The former 14 patients were assigned to two groups based on development of PVO after EC-TCPC (groups O and N). The pre- and post-operative cardiac morphologies and their relationship with the conduit were compared. RESULTS: After the EC-TCPC, the pressure gradients between the atrium and the PV were 5.0 ± 2.5 and 0.44 ± 0.2 mmHg in groups O and N, respectively (p < 0.01); however, the pressure gradients in the left and right PVs were not significantly different, suggesting stenosis of the common PV orifice. The ratio of the horizontal distance from the vertebrae to the PV orifice and to the lateral edge of the atrium was significantly higher (0.38 ± 0.2 vs. 0.17 ± 0.1; p = 0.04) and the orifice was smaller (8.9 ± 2.0 vs. 15 ± 4.7 mm; p < 0.01) in group O than in group N. CONCLUSION: In cases with ipsilateral locations of the conduit and PV orifice, small size and more lateral location of the PV orifice may be preoperative risk factors for development of PVO.

Bilateral Pulmonary Artery Banding in Ebstein's Anomaly With Circular Shunting.

We present a case of critical Ebstein's anomaly with circular shunting, diagnosed in utero. The fetal cardiothoracic area ratio was elevated; tricuspid regurgitation and pulmonary regurgitation worsened with fetal hydrops. At 35 weeks 6 days of gestation, elective caesarean section delivery was performed. Planned bilateral pulmonary artery banding and pulmonary trunk ligation were performed as a palliative operation 4 hours after birth, with the infant in a stable condition. At age 5 days, we performed the Starnes operation. The postoperative course was uneventful and a bidirectional Glenn operation was performed at age 4 months.

Left ventricular function after a Fontan operation in patients with pulmonary atresia with an intact ventricular septum.

OBJECTIVES: In patients with pulmonary atresia with an intact ventricular septum (PA/IVS), there are no reports about the impact of sinusoidal communication (SC) on left ventricular (LV) performance after a Fontan operation; therefore, the purpose of this study was to fill this gap in the literature. METHODS: We performed a single-centre, retrospective study of 46 patients with PA/IVS. Nineteen patients who underwent the Fontan procedure were enrolled and divided into 2 groups: those with SC (SC group) and those without SC (N group). The 2 groups were compared in terms of postoperative cardiac function. RESULTS: Thirteen patients were in the SC group (4 patients with right ventricle-dependent coronary circulation) and 6, in the N group. Although 2 of the patients with right ventricular (RV)-dependent coronary circulation showed focal asynergy of the LV wall, others showed no findings of myocardial infarction. The patients' preoperative age and age during the observation period after the Fontan operation did not differ. There was no difference in LV ejection fraction, cardiac index and RV and pulmonary artery pressure before and after the Fontan operation. Preoperative RV volume in the N group was greater than that in the SC group. Brain natriuretic peptide levels were higher in the SC group after the Fontan operation. On ventricular efficacy analysis, contractility was lower (1.8 ± 0.32 vs 2.5 ± 0.40 mmHg/ml/m2, P = 0.001) and ventricular efficacy was worse (1.0 ± 0.15 vs 0.86 ± 0.11, P = 0.046) in the SC group during the postoperative period. CONCLUSIONS: Compared to patients with PA/IVS without SC, patients with PA/IVS with SC had a low cardiac contractility and decreased cardiac output efficiency after the Fontan procedure.

[Long term outcome of arterial switch surgery for transposition of the great arteries: evaluation of the reconstruction of the pulmonary artery].

We assessed the effect of reconstructing the pulmonary artery during arterial switch surgery for transposition of the great arteries on late pulmonary stenosis. Sixty-five patients who underwent Lecompte procedure between September 1991 and December 2006 were divided, by the procedure used chronologically to reconstruct the pulmonary artery, into group XP (single pantaloon patch with equine pericardium, n = 11), group P (direct reconstruction, n = 47), and group AP (single pantaloon patch with fresh autopericardium, n = 7). Outcome and pulmonary stenosis on the most recent ultrasound cardiography (UCG) were compared in the 3 groups. The median follow-up was 13, 7.5, and 1.3 years, respectively. Both early and late mortalities were 1.5% (1/65). Although percutaneous trans-pulmonary angioplasty was necessary in 1, 13, and 3 patients, there was 1, 1, and 0 reoperation for pulmonary stenosis in the 3 groups, respectively. Pulmonary stenosis (pulmonary arterial maximum flow velocity > 3 m/sec on UCG) was present in 4 (40%). 14 (30%). and 3 patients (43%). Although there was no significant difference among the 3 procedures in preventing pulmonary stenosis 10 years after arterial switch surgery, direct reconstruction of the pulmonary artery may show a superior outcome, in particular, over 10 years after arterial switch surgery.

Evaluation of Factors Associated With Achievement of Biventricular Repair After Bilateral Pulmonary Artery Banding in Patients With Interrupted Aortic Arch.

BACKGROUND: At our institution, we perform bilateral pulmonary artery banding (BPAB) as the first-stage palliation for interrupted aortic arch (IAA) with low birth weight or severe subaortic stenosis (SAS). The present study aimed to identify factors that may influence the decision regarding the type of second-stage operation, that is, univentricular palliation or biventricular repair, in these patients. METHODS: Cardiac catheterization and angiographic data of nine patients with IAA who underwent initial BPAB and subsequent univentricular or biventricular repair were retrospectively analyzed. RESULTS: Between 2004 and 2014, of nine patients with IAA who underwent initial BPAB, biventricular repair was subsequently performed in six patients (group B) and univentricular repair in three patients (group U). All patients survived. There was no significant intergroup difference in IAA classification, location of ventricular septal defect, presence of 22q11.2 deletion, presence of aberrant right subclavian artery, band diameter, or post-BPAB pulmonary artery pressure and index. Timing of BPAB and the body weight at the time of BPAB, however, differed significantly between the groups ( P = .02). Catheter data before BPAB were not significantly different between the groups, with the exception of the degree of subaortic stenosis (or hypoplasia of the left ventricular outflow tract) expressed as percentage of the normal end-systolic aortic valve annular diameter for patient body surface area. This metric (%SAS before BPAB) was significantly higher in group B (60%-68%) than in group U (47%-60%; P = .04). Among patients for whom baseline %SAS was < 60%, the %SAS did not increase after BPAB. CONCLUSION: The most important factor that allowed biventricular repair was not the pulmonary artery pressure or diameter but the degree of SAS. Patients who initially had more severe SAS ultimately underwent univentricular repair due to lack of substantial improvement in dimensions of the left ventricular outflow tract after BPAB.

Supra-Annular Mitral Valve Replacement in an Infant With Infective Endocarditis.

We report the case of a female neonate born at 40 weeks' gestation with no known risk factors. On postnatal day 27, she was transferred to the pediatric intensive care unit and intubated for cardiorespiratory failure; she was diagnosed with group B streptococcus infective endocarditis. Mitral valve vegetations did not improve with antibiotic therapy. Mitral valve surgery was performed on postnatal day 36. A mechanical mitral valve with short-segment polytetrafluoroethylene skirt was inserted into the supra-annular position; gentian violet was applied to the sewing ring and skirt. She was discharged from the hospital after 6 weeks with good cardiac function and normal sinus rhythm.

Clinical standard for valve area after common atrioventricular valve plasty for a single ventricle.

OBJECTIVES: To determine a clinical standard for post-repair common atrioventricular valve orifice area based on mid- to long-term valve function in patients with a functional single ventricle. METHODS: The medical records of 19 single-ventricle patients who underwent common atrioventricular valve plasty from July 1988 to January 2013 were retrospectively reviewed. Bivalvation valvuloplasty was performed in 7 patients with relatively hypoplastic leaflets. The relationship between the orifice area of the repaired common atrioventricular valve measured intraoperatively and valve function and ventricular volume in the early postoperative period (median, 9.5 months) and at mid- to long-term follow-up (median, 4 years) were analysed. RESULTS: Post-repair valve area was significantly positively correlated with valve regurgitation severity in the early postoperative period (P = 0.001, r = 0.69) and at mid- to long-term follow-up (P = 0.02, r = 0.57). Patients who did not undergo bivalvation had favourable valve function at mid- to long-term follow-up and in the early postoperative period when the post-repair valve area was 96-136% of the normal mitral valve area. Bivalvation patients had significantly more valve regurgitation in the early postoperative period than patients without bivalvation, despite equivalent repaired valve area (P = 0.02). CONCLUSIONS: The post-repair orifice area of the common atrioventricular valve is significantly related to postoperative valve function. The clinical standard of post-repair valve orifice area might be 96-136% of the normal mitral valve area in patients undergoing repair without bivalvation. Patients undergoing bivalvation require greater reduction to obtain favourable mid- to long-term valve function.

Temporary decompression of the right ventricle to assess the right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.

We repaired a case of pulmonary atresia with intact ventricular septum in which the blood supply to the left anterior descending coronary artery depended on the right ventricle. At the time of a bidirectional Glenn operation, total cardiopulmonary bypass with venous drainage from the right atrium was performed in order to evaluate the safety of right ventricular decompression required for a planned Fontan operation. We confirmed the dependence of the coronary perfusion on the right ventricle by demonstrating transient depression of the ST segment in the epicardial electrocardiogram during temporary decompression of the right ventricle. To prevent ischemic myocardial damage, we then performed an extracardiac Fontan operation with a temporary venous shunt and without cardiopulmonary bypass.

Site-specific nanopatterning of functional metallic and molecular arbitrary features in nanofluidic channels.

We established a versatile method for site-specific nanopatterning of functional metallic and molecular arbitrary features in glass nanofluidic channels, with well-controlled feature sizes ranging from tens to hundreds of nanometers and precisely controlled placements in the range of several tens of nanometers. With the method, we achieved the fabrication of quasi-0D, quasi-1D, 2D, and 3D gold nanopatterns in nanofluidic channels, as well as a high-density fluorescent molecular nanoarray in arrayed femtoliter nanofluidic channels. The method opens the way for precise functionalization of nanofluidic channels, which has been greatly challenging in the field of nanofluidics.