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).

Aortic Dissection and Supravalvular Aortic Stenosis With Williams Syndrome Complicated by Infection.

Williams syndrome (WS) is a contiguous gene syndrome involving the connective tissue, central nervous system, and cardiovascular disorders, such as supravalvular aortic stenosis (SVAS), pulmonary artery stenosis, and coronary artery anomaly, that carry a risk of sudden death. Although aortic dissection with SVAS in WS is extremely rare, it could be 1 of the triggers of sudden cardiac death. In this case, a patient with WS underwent ascending aorta replacement for aortic dissection that was potentially influenced by infective endocarditis and SVAS repaired by modified Brom aortoplasty.

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

Unlabelled: 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.

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 (rSO) was monitored using near-infrared spectroscopy (NIRS) during surgery, and changes in rSOwere compared with treatment results.

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.

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).

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Cardiac sarcoidosis diagnosed by histological assessment of a left ventricular apical core excised for insertion of a left ventricular assist device.

A 58-year-old male with no history of heart disease was admitted to hospital for congestive heart failure due to severe left ventricular dysfunction, and clinically diagnosed with dilated cardiomyopathy. He developed recurrent heart failure requiring several admissions to hospital and was finally referred to our institution with severe congestive heart failure. Despite medical treatment with inotropic agents, his symptoms gradually worsened.

Manouguian double-valve replacement in a 6-year-old girl.

A 6-year-old girl with a diagnosis of aortic regurgitation with stenosis and mitral regurgitation because of short chordae was referred to us for surgery. Echocardiography revealed that the aortic and the mitral annular diameters were 16 and 23 mm, respectively. The Ross procedure and mitral valvuloplasty were scheduled.

Surgical results for functional univentricular heart with total anomalous pulmonary venous connection over a 25-year experience.

Background: Surgical results for functional univentricular heart with total anomalous pulmonary venous connection (TAPVC) have been unsatisfactory to date.

Methods: During a 25-year period until December 2009, 207 TAPVC patients underwent surgical repair at our institute, including 56 with a univentricular heart. The 10-year survival rate was 51.