Long-term outcomes of hypoplastic left heart syndrome with analysis of the Norwood procedure in infants following bilateral pulmonary artery banding.

Objective: To investigate the early and long-term outcomes of the deferred Norwood procedure by bilateral pulmonary artery banding (BPAB) versus the neonatal Norwood procedure. Methods: This retrospective study examined 46 patients with hypoplastic left heart syndrome and its variants undergoing the Norwood procedure for single ventricle physiology between 2004 and 2022 at 3 institutions. The patients were divided into 2 groups: neonatal Norwood procedure (group N; n = 23) and staged Norwood procedure in infants following BPAB (group I; n = 23). Preoperative risk factors, surgical results, survival rates, Fontan candidacy, and long-term complications were compared. Results: Early survival rates after the Norwood procedure were 91.3% (21 of 23) in both groups. Late survival rates after the Norwood procedure were similar at the 10-year follow-up (group N, 76.3%; group I, 68.7%; P = .63). Fontan completion rates also were comparable in the 2 groups (group N, 77.8%; group I, 85.7%; P = .67). Group N showed a higher median pulmonary artery (PA) index before bidirectional cavopulmonary connection (group N, 177 [interquartile range (IQR), 147-243] mm2/m2; group I, 152 [IQR, 146-163] mm2/m2; P = .03); this trend continued until 5 years after Fontan completion (P = .01). Group N also had a lower rate of freedom from protein-losing enteropathy (PLE) at 9.0 years after the Fontan operation (90.0% vs 52.5% for group I; P = .04), although the incidences of other Fontan-associated events were not significantly different. Conclusions: Fontan candidacy and survival rates were similar regardless of the timing of the Norwood procedure. Early performance of the Norwood procedure may lead to lower rates of late Fontan-associated events, such as PLE.

Rapidly growing thrombus from a ductus arteriosus aneurysm in a neonate.

Ductus arteriosus aneurysm (DAA) is a rare cardiovascular anomaly, and thrombosis of DAA is even less common. The management of asymptomatic DAA with a thrombus is controversial. We here report a neonate with a thrombus from a DAA that grew rapidly into the pulmonary artery. The thrombus was detected incidentally in the main pulmonary artery by routine screening echocardiography. There was no clinical evidence of its presence until a few days after birth. The thrombus grew rapidly, despite administration of heparin. Six days after birth, the patient became cyanotic and had developed right ventricular pressure overload as a result of obstruction of the left pulmonary artery. The thrombus was immediately removed and the DAA resected. The patient was discharged home without any complications. Complications related to thrombus of a DAA can be critical and therefore require careful monitoring. Learning objective: A thrombus extending from a ductus arteriosus aneurysm into the pulmonary artery can have serious consequences; thus, careful monitoring is required. Any signs of such complications should prompt immediate consideration of removal of the aneurysm and thrombus.

Gluteal Blood Flow Monitoring in Endovascular Aneurysm Repair With Internal Iliac Artery Embolization.

BACKGROUND: When an internal iliac artery (IIA) has to be embolized during endovascular aneurysm repair (EVAR), buttock claudication sometimes poses problems. However, there is no established method to evaluate intraoperative blood flow to the gluteal muscles.Methods and Results:Gluteal regional oxygen saturation (rSO2) was monitored using near-infrared spectroscopy (NIRS) during surgery, and changes in rSO2were compared with treatment results. Twenty-seven patients who underwent EVAR and IIA embolization at our institution between April 2019 and May 2020 were included in this study. The association between intraoperative changes in rSO2and postoperative incidence of buttock claudication was analyzed. Furthermore, the presence or absence of communication between the superior and inferior gluteal arteries and the intraoperative changes in rSO2were compared to ascertain whether rSO2reflects blood flow change. Postoperative buttock claudication occurred in 4 of 19 patients (21%) with unilateral occlusion of IIA and in 4 of 8 patients (50%) with bilateral occlusion of IIAs. rSO2was found to decrease significantly further in patients with buttock claudication than in patients without buttock claudication (-15±12% vs. -4±16%, P<0.05). In addition, rSO2was predominantly lower in patients without the communication between the superior and inferior gluteal arteries than in those with the communication. CONCLUSIONS: Gluteal rSO2is useful as an indicator of intraoperative gluteal blood flow.

Novel Urinary Biomarkers for Acute Kidney Injury and Prediction of Clinical Outcomes After Pediatric Cardiac Surgery.

Acute kidney injury (AKI) is a serious complication of pediatric cardiac surgery, with high morbidity and mortality. We aimed to evaluate the perioperative risk factors for AKI, and the validity of novel diagnostic urinary biomarkers after pediatric cardiac surgery. We analyzed 103 consecutive pediatric patients (≤ 18 years old), who underwent cardiac surgery. AKI was defined by ≥ 50% increase in serum creatinine levels from baseline. Urinary liver-type fatty acid binding protein (L-FABP) and neutrophil gelatinase-associated lipocalin (NGAL) were measured postoperatively at the intensive care unit (ICU) admission, subsequently at 4, 12, and 24 h. Areas under the receiver-operating characteristic curves (AUC) were calculated at each assessment time. AKI had developed in 47 patients (45.6%) by the second postoperative day. Univentricular status, aortic cross-clamping time, and intraoperative fluid balance were independently associated with AKI (p = 0.02, 0.01 and 0.01, respectively). Urinary L-FABP and NGAL were significantly higher in the AKI group at each point (p < 0.05). The predictive abilities of both biomarkers (AUC = 0.78-0.90) at ICU admission and 4 h after were especially high. The patients with L-FABP greater than the cutoff value at ICU admission and 4 h after ICU admission had significantly longer intubation and hospitalization periods (p < 0.05). Those with elevated NGAL levels at admission, and 4 h and 24 h after ICU admission, had significantly longer intubation, ICU stay, and hospitalization (p < 0.05). L-FABP and NGAL can be useful biomarkers for detecting early AKI after pediatric cardiac surgery and predicting adverse clinical outcomes.

Extensibility of autologous pericardium roll conduit in non-confluent pulmonary artery: a case report.

BACKGROUND: There is great discussion about non-confluent pulmonary artery (PA) reconstruction, and several materials have been used. Autologous pericardium is considered feasible for infectious resistance, autoimmune response, extensibility, and growth potential. CASE PRESENTATION: The patient was born at 39 weeks (body mass = 2550 g). He was diagnosed with tetralogy of Fallot, pulmonary atresia, non-confluent PA, and bilateral patent ductus arteriosus. Right and left Blalock-Taussig shunts with patent ductus arteriosus ligations were placed on day 27 and 3 months, respectively. At 19 months (8.8 kg), definitive repair was performed with tricuspid valved conduit concurrent with PA reconstruction using an autologous pericardium roll conduit. The autologous pericardium was treated with glutaraldehyde (autologous pericardium fixed with 0.4% glutaraldehyde for 7 min and rolled as conduit - 12 mm in diameter and 30 mm in length). Following an incision on the visceral side of the PAs before the 1st branch, the autologous pericardial roll conduit was anastomosed. Follow-up angiographies on postoperative months 9 and 57 demonstrated that the PA, including the autologous pericardium roll conduit, had spontaneously enlarged. CONCLUSION: Particularly for non-confluent PA, the patients require increased pulmonary beds at an early age because of hypoplastic PA. While size mismatch between the graft and native PA develops as the child grows, size-adjustable extensibility of the PA graft should be noted.

Neo-aortic insufficiency late after staged reconstruction for hypoplastic left heart syndrome: impact of differences in initial palliative procedures.

The neo-aortic insufficiency in patients with hypoplastic left heart syndrome is an important sequela. We assessed the risks of the neo-aortic valve deterioration by the difference of initial palliations: Group I underwent primary Norwood (Nw) with systemic-to-pulmonary artery shunt (SPS), Group II underwent bilateral pulmonary artery banding (bPAB) and subsequent Nw with SPS (bPAB-Nw/SPS), Group III underwent bPAB and subsequent Nw with bidirectional Glenn (BDG) procedure (bPAB-Nw/BDG). The neo-aortic valve z score changes over time did not reach statistical significance in all groups (p = 0.43 for Group I, 0.20 for Group II, and 0.30 for Group III). The degree of neo-aortic valve insufficiency did not change significantly over time during this period except for Group III (p = 0.34 for Group I, 0.20 for Group II, and 0.02 for Group III). On the other hand, dimensions of the neo-aortic annulus and degrees of neo-aortic insufficiency did not differ significantly among the 3 groups at any pre-determined time. The presence or absence of incision into the sino-tubular junction at Nw did not affect the late neo-aortic valve z score or insufficiency. These data indicate that the difference of initial palliative procedures does not affect late neo-aortic valve insufficiency in Nw survivors. Because valve failure may develop in longer follow-up, further observation should be conducted.

Quick atrial access by subxiphoid approach in extracorporeal cardiopulmonary resuscitation after bidirectional Glenn procedure.

Bidirectional Glenn procedure outcomes are very good; therefore, extracorporeal membrane oxygenation use as extracorporeal cardiopulmonary resuscitation is uncommon. We describe a 13-month-old female who required extracorporeal cardiopulmonary resuscitation for ventricular tachycardia provoked by transient myocarditis 6 months post-bidirectional Glenn procedure. After extracorporeal membrane oxygenation induction with cannulation on the cervical vessels, small skin incision was created on the subxiphoid area without sternotomy and the atrium was cannulated. With adequate venous drainage and ventricular unloading, ventricular tachycardia eventually converted to sinus rhythm. The patient withdrew from extracorporeal membrane oxygenation and was discharged successfully. Our results suggest that for successful post-bidirectional Glenn extracorporeal cardiopulmonary resuscitation, quick atrial access and ventricular unloading are essential.

Establishment of a Healthcare System for Patients With Adult Congenital Heart Disease in Collaboration With Children's Hospital　- The Nagano Model.

BACKGROUND: Despite the best efforts of pediatricians, healthcare for adult patients with congenital heart disease (ACHD) has proven challenging because of the increased numbers. This study presents the process of establishing an ACHD care system as a collaborative effort between Shinshu University Hospital and Nagano Children's Hospital. Methods and Results: Establishing an outpatient clinic for transition, a cooperation agreement for in-patient care between the 2 hospitals, and quality management of diagnostic imaging and educational meetings for adult cardiologists were the 3 major challenges. Of the 99 patients who visited the transition clinic in the children's hospital between May 2014 and December 2016, 3 returned to the pediatrician's clinic. Between June 2013 and December 2017, 273 patients visited the ACHD center in Shinshu University Hospital. Until December 2017, mortality and fatal arrhythmia were noted in 3 and 2 cases, respectively. Catheter ablation for arrhythmia was performed in 12 cases, and 4 cases of pregnancy with moderate/severe ACHD or estimated as high risk were managed with healthy livebirths. Surgical interventions for moderate/severe ACHD were performed in collaboration with the children's hospital or Sakakibara Heart Institute. CONCLUSIONS: Patients were successfully transferred to adult cardiology departments. Surgical and nonsurgical interventions for ACHD were provided. Collaboration between adult and pediatric cardiologists assists in the establishment of healthcare systems for ACHD.

Rapid growth of pulmonary artery after intrapulmonary artery septation.

Intrapulmonary artery septation is employed in patients with unbalanced pulmonary artery growth. A baby girl received a systemic pulmonary shunt in the neonatal period and bilateral Glenn shunts in early infancy. Once the Glenn shunts were established, the right pulmonary artery became hypoplastic. We performed intrapulmonary artery septation involving the Glenn shunts to the left pulmonary artery and the systemic pulmonary shunt to the right pulmonary artery. As early as 3 months after intrapulmonary artery septation, right pulmonary artery growth was observed. Eventually, Fontan circulation was achieved with fenestration.

Pulmonary vein obstruction after primary sutureless pericardial repair of a total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection is a rare congenital heart disease. The development of pulmonary venous obstruction is one of the major risk factors for poor outcomes after surgical repair. Sutureless pericardial repair for a total anomalous pulmonary venous connection was introduced to decrease the risk of pulmonary venous obstruction after surgical repair, and favourable outcomes have been reported. Herein, we report the case of an infant with a total anomalous pulmonary venous connection who developed pulmonary venous obstruction after primary sutureless pericardial repair.

Valve-sparing reimplantation for neoaortic root dilatation and regurgitation with an unbalanced cusp after the arterial switch operation.

A 13-year-old male presented with neoaortic root dilatation and severe aortic valve regurgitation 13 years following an arterial switch operation. The valve cusps were unbalanced due to an enlarged non-coronary cusp. A valve-sparing reimplantation with a cusp plication was performed which resulted in a competent valve with trivial regurgitation. Thus, even in an unbalanced cusp, valve-sparing reimplantation can be used for neoaortic root dilatation and valve regurgitation after an arterial switch operation.

Refractory ventricular fibrillations after surgical repair of atrial septal defects in a patient with CACNA1C gene mutation - case report.

BACKGROUND: Congenital long QT syndrome (LQTS) can cause ventricular arrhythmic events with syncope and sudden death resulting from malignant torsades de pointes (TdP) followed by ventricular fibrillations (VFs). However, the syndrome is often overlooked prior to the development of arrhythmic events in patients with congenital heart diseases demonstrating right bundle branch block on electrocardiogram (ECG). We present a case of an adult patient with congenital heart disease who developed VFs postoperatively, potentially due to his mutation in a LQTS related gene, which was not identified on preoperative assessment due to incomplete evaluation of his family history. CASE PRESENTATION: A 64-year-old man was diagnosed as having multiple atrial septal defects. He presented with no symptoms of heart failure. His preoperative ECG showed complete right bundle branch block (CRBBB) with a corrected QT interval time of 478 ms. He underwent open-heart surgery to close the defects through median sternotomy access. Three hours after the operation, he developed multiple events of TdP and VFs in the intensive care unit. Cardiopulmonary resuscitation and multiple cardioversions were attempted for his repetitive TdP and VFs. He eventually reverted to sinus rhythm, and intravenous beta-blocker was administered to maintain the sinus rhythm. After this event, his family history was reviewed, and it was confirmed that his daughter and grandson had a medical history of arrhythmia. A genetic test confirmed that he had a missense mutation in CACNA1C, p.K1580 T, which is the cause for type 8. CONCLUSIONS: This case highlights the importance of paying attention to other ECG findings in patients with CRBBB, which can mask prolonged QT intervals.

Aortic Coarctation 28 Days after an Arterial Switch Operation in a Neonate.

Aortic coarctation rarely occurs after an arterial switch operation for D-transposition of the great arteries with intact ventricular septum. We report the case of a neonate patient in whom aortic coarctation developed 28 days after an uncomplicated arterial switch operation. Preoperatively, the aorta was noted to have an irregular shape, but there was no pressure gradient across the lesion. The patient underwent successful reoperation to correct the coarctation. We hope that our report raises awareness of a rare early complication after arterial switch operation with intact ventricular septum, and the need to carefully monitor the aortic isthmus in patients who have aortic irregularities, even in the absence of a pressure gradient.

Aortopulmonary collateral arteries: a rare complication after arterial switch operation for transposition of the great arteries.

Collateral vascular arteries from the descending aorta to the pulmonary arteries are uncommon after arterial switch operation. Here, we report the case of a baby girl treated with coil embolization for abnormal blood flow from the descending aorta to the pulmonary arteries after arterial switch operation. A baby girl weighing 1324 g was delivered at 32 weeks 4 days of gestation, and she had D-transposition of the great arteries and a ventricular septal defect. She underwent nitrogen inhalation to reduce pulmonary blood flow before arterial switch operation. After the operation, she presented with left heart failure due to the presence of abnormal blood flow from the descending aorta to the pulmonary arteries, and she was successfully treated with coil embolization. After the treatment, her condition improved dramatically, and she was discharged without any complications.

The analysis of ascending aortic dilatation in patients with a bicuspid aortic valve using the ratio of the diameters of the ascending and descending aorta.

BACKGROUND: A bicuspid aortic valve (BAV) is associated with premature valve dysfunction and abnormalities of the ascending aorta. The aim of our study was to assess the degree of ascending aortic dilatation by measuring the ratio of the dimension of the AAo to that of the descending aorta (DAo) using preoperative computerized tomography (CT). METHODS: A review of our institutional clinical database identified 76 patients undergoing aortic valve replacement (AVR) and 73 control patients undergoing off-pump coronary artery bypass (OPCAB group) between September 2009 and April 2012. RESULTS: There were 17 patients diagnosed with BAV (BAV group), and the remaining 59 patients had a tricuspid aortic valve (TAV group). The ratios of the dimensions of the AAo to that of the DAo (AAo/DAo) for each group were: BAV, 1.58 ± 0.25; TAV, 1.32 ± 0.11; and OPCAB, 1.29 ± 0.12. Interestingly, the AAo/DAo of the BAV group was significantly larger than that of the other groups. CONCLUSIONS: Although progressive AAo dilatation for BAV is well documented, the diameter of the AAo is currently the only estimate of aortic dilatation. In this study, we report that the ratio of the AAo and DAo diameters in patients with BAV can be a new index for assessing the dilatation of the AAo and differentiating the patients with BAV from those with TAV.

Pathophysiology of lung injury induced by common bile duct ligation in mice.

BACKGROUND: Liver dysfunction and cirrhosis affect vasculature in several organ systems and cause impairment of organ functions, thereby increasing morbidity and mortality. Establishment of a mouse model of hepatopulmonary syndrome (HPS) would provide greater insights into the genetic basis of the disease. Our objectives were to establish a mouse model of lung injury after common bile duct ligation (CBDL) and to investigate pulmonary pathogenesis for application in future therapeutic approaches. METHODS: Eight-week-old Balb/c mice were subjected to CBDL. Immunohistochemical analyses and real-time quantitative reverse transcriptional polymerase chain reaction were performed on pulmonary tissues. The presence of HPS markers was detected by western blot and microarray analyses. RESULTS: We observed extensive proliferation of CD31-positive pulmonary vascular endothelial cells at 2 weeks after CBDL and identified 10 upregulated and 9 down-regulated proteins that were associated with angiogenesis. TNF-α and MMP-9 were highly expressed at 3 weeks after CBDL and were less expressed in the lungs of the control group. CONCLUSIONS: We constructed a mouse lung injury model by using CBDL. Contrary to our expectation, lung pathology in our mouse model exhibited differences from that of rat models, and the mechanisms responsible for these differences are unknown. This phenomenon may be explained by contrasting processes related to TNF induction of angiogenic signaling pathways in the inflammatory phase. Thus, we suggest that our mouse model can be applied to pulmonary pathological analyses in the inflammatory phase, i.e., to systemic inflammatory response syndrome, acute lung injury, and multiple organ dysfunction syndrome.

Redo cardiac surgery for active prosthetic valve endocarditis associated with hereditary hemorrhagic telangiectasia: report of a case.

Hereditary hemorrhagic telangiectasia (HHT) is caused by an autosomal dominant gene and characterized by multiple arteriovenous malformations in several organs, leading to bleeding or shunting. These patients often suffer severe infections and heart failure, which should be managed in the perioperative period, when open heart surgery is indicated. We report a case of successful aortic root replacement for active prosthetic valve endocarditis and ventricular septal perforation in a patient with HHT, who had severe heart failure.