The usefulness of staged extra-anatomic bypass for coarctation of the aorta with arch hypoplasia, from premature neonate to adult.

Various surgical techniques have been developed for coarctation of the aorta. However, coarctation repair in neonates with arch hypoplasia remains challenging. We herein report a case in which a premature neonate under 1500 g with coarctation of the aorta and arch hypoplasia underwent an extra-anatomical bypass at 18 days old. A second extra-anatomical bypass was performed at 3 years of age, and a third extra-anatomic bypass for recurrent coarctation was performed in adulthood. By increasing the size of the graft as the patient grows, extra-anatomic bypass can be a useful surgical option for premature neonates with coarctation and arch hypoplasia.

Pseudoaneurysm after aortopulmonary window repair and bilateral lung transplantation for eisenmenger syndrome: a case report.

BACKGROUND: Aortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by communication between the main pulmonary artery and ascending aorta. There are various surgical techniques, and the short- and long-term results are excellent if the surgical repair is performed early in life. To our knowledge, there have been no reports of pseudoaneurysm after APW repair. Herein, we present a case of a 30-year-old woman with an ascending aortic pseudoaneurysm found at the site of APW repair nine months after the APW repair and bilateral lung transplantation. CASE PRESENTATIONS: A 30-year-old woman presented with APW and Eisenmenger syndrome. The patient underwent APW repair and bilateral lung transplantation. We transected the communication between the aorta and pulmonary artery and closed the aortic side directly with strips of felts. Nine months after the surgery, the patient complained of chest pain. Cardiac computed tomography revealed an ascending aortic pseudoaneurysm at the anastomotic site. Emergent graft replacement of the ascending aorta was performed and the postoperative course was uneventful. CONCLUSIONS: We have presented a case of a pseudoaneurysm at the anastomotic site after APW repair and bilateral lung transplantation. The choice of surgical technique should be based on the patient's background requiring lung transplantation, and in these cases close postoperative follow-up is required.

Novel surgical technique for anomalous origin of a left coronary artery from the right sinus of Valsalva with a single coronary orifice.

Anomalous origin of a left coronary artery from the right sinus of Valsalva with a single coronary orifice is a rare congenital anomaly, which has been associated with myocardial ischemia and sudden death. Surgical repair is recommended upon its detection. A 14-year-old boy was diagnosed with anomalous origin of a left coronary artery from the right sinus of valsalva with a single coronary orifice after a syncope attack. The patient underwent relocation of the left coronary orifice. The postoperative course was uneventful, with no ventricular arrhythmia or syncope. The patient did not develop cardiac ischemia or infarction on exercise Tc-99 m myocardial scintigraphy 8 months after the procedure.

Cardiomyocyte deoxyribonucleic acid damage and cardiac recovery in paediatric dilated cardiomyopathy.

OBJECTIVES: The goal of this study was to identify the clinical significance of the deoxyribonucleic acid (DNA) damage response marker, phosphorylated H2A histone variant X, on the bridge to recovery in low-weight paediatric patients with dilated cardiomyopathy (DCM) after having a Berlin Heart EXCOR implanted. METHODS: Consecutive paediatric patients with DCM who had an EXCOR implanted for DCM at our hospital between 2013 and 2021 were reviewed. Patients were classified into 2 groups according to the degree of DNA damage in the left ventricular cardiomyocytes-the low DNA damage group and the high DNA damage group-using the median value as the threshold. We examined and compared the preoperative factors and histologic findings associated with cardiac functional recovery following the explant procedure in the 2 groups. RESULTS: Competing outcome analysis of 18 patients (median body weight, 6.1 kg) showed that the incidence of an EXCOR explant was 40% at 1 year after the implant procedure. Serial echocardiography revealed significant left ventricular functional recovery in the low DNA damage group 3 months after the implant. The univariable Cox proportional hazards model revealed that the percentage of phosphorylated H2A histone variant X-positive cardiomyocytes was the significant factor associated with cardiac recovery and the EXCOR explant (hazard ratio, 0.16; 95% confidence interval, 0.027-0.51; P = 0.0096). CONCLUSIONS: The degree of DNA damage response to the EXCOR implant may aid in predicting the bridge to recovery with EXCOR among low-weight paediatric patients with DCM.

Univentricular Conversion for Acquired Severe Right Ventricular Failure.

In a 14-year-old boy, prolonged right ventricular (RV) dysfunction due to postmyocarditis cardiomyopathy developed, whereas left ventricular function recovered with conservative treatment. On catheterization, the RV end-diastolic volume index was 184 mL/m2, and mean pulmonary artery pressure was 16 mm Hg. Despite one-and-a-half ventricle repair, RV dilation continued to worsen; therefore, 3 years after the operation, RV exclusion and extracardiac total cavopulmonary connection were performed. Postoperatively, left ventricular function was maintained, and no further RV dilation was observed. Univentricular conversion may be an effective procedure for the treatment of acquired severe isolated right-sided heart failure.

Fontan operation for tricuspid atresia with absent pulmonary valve: a case series.

Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative courses.

Thoracoscopic repair of diaphragmatic hernia following ventricular assist device implantation.

BACKGROUND: Diaphragmatic hernia is a rare complication of ventricular assist device (VAD), mainly developing after explantation of the device. We herein report a case of diaphragmatic hernia that developed following the implantation of VAD. CASE PRESENTATION: A 4-month-old girl with a diagnosis of dilated cardiomyopathy underwent VAD implantation for a bridge to heart transplantation. Three months later, intermittent vomiting developed, and left-sided diaphragmatic hernia was confirmed on plain X-ray and computed tomography. Without any findings of ischemia, we performed elective thoracoscopic repair of the diaphragmatic hernia. In the right decubitus position, thoracoscopy revealed the small intestine to be herniated into the left thorax. After reduction of the herniated intestine, the defect of the diaphragm (3 × 2 cm in size) was directly closed with interrupted non-absorbable sutures. Her postoperative course was uneventful. CONCLUSION: Thoracoscopic repair of diaphragmatic hernia associated with VAD implantation may be a safe approach.

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.

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.

Antibiotic Prophylaxis for Open Chest Management After Pediatric Cardiac Surgery.

OBJECTIVES: Although open chest management optimizes hemodynamics after cardiac surgery, it increases postoperative infections and leads to increased mortality. Despite the importance of antibiotic prophylaxis during open chest management, no specific recommendations exist. We aimed to compare the occurrence rates of bloodstream infection and surgical site infection between the different prophylactic antibiotic regimens for open chest management after pediatric cardiac surgery. DESIGN: Retrospective, single-center, observational study. SETTING: PICU at a tertiary children's hospital. PATIENTS: Consecutive patients younger than or equal to 18 years old with open chest management after cardiac surgery followed by delayed sternal closure, between January 2012 and June 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We compared the composite occurrence rate of postoperative bloodstream infection and surgical site infection within 30 days after cardiac surgery between three prophylactic antibiotic regimens: 1) cefazolin, 2) cefazolin + vancomycin, and 3) vancomycin + meropenem. In 63 pediatric cardiac surgeries with open chest management, 17 bloodstream infections, and 12 surgical site infections were identified postoperatively. The composite occurrence rates of bloodstream infection and surgical site infection were 10 of 15 (67%), 10 of 19 (53%), and nine of 29 (31%) in the cefazolin, cefazolin + vancomycin, and vancomycin + meropenem regimens, respectively (p = 0.07). After adjusting for age, open chest management duration, extracorporeal membrane oxygenation use, and nasal methicillin-resistant Staphylococcus aureus colonization in multivariable analysis, there was no significant difference between the cefazolin and the cefazolin + vancomycin regimens (p = 0.19), while the vancomycin + meropenem regimen had a lower occurrence rate of bloodstream infection and surgical site infection than the cefazolin regimen (odds ratio, 0.0885; 95% CI, 0.0176-0.446; p = 0.003). CONCLUSIONS: In this study, a lower occurrence rate of postoperative bloodstream infection and surgical site infection was observed among patients with broad-spectrum antibiotic regimen after pediatric cardiac surgery with open chest management. Further studies, ideally randomized controlled studies investigating the efficacy of broad-spectrum antibiotics and their complications, are warranted before routine implementation of broad-spectrum prophylactic antibiotic regimen.

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.

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.

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.

[Postoperative Atrial Fibrillation in Patients who Underwent Thoracic Aortic Surgery].

PURPOSE: Postoperative atrial fibrillation (POAF) is a very common and important complication occurring after open heart surgery. Risk factors and prevention measure including ß blocker use have been reported mainly in regard to patients who underwent coronary artery bypass grafting, while little is known about POAF following thoracic aortic surgery. In the present study, we examined risk factors related to POAF and effective prevention of POAF in patients who underwent thoracic aortic surgery. PATIENTS AND METHODS: We evaluated 95 consecutive patients who underwent thoracic aortic surgery since 2010. We analyzed the relationship between perioperative factors and occurrence of POAF in the study cohort, as well as in 62 patients who had perioperative intravenous ß blocker (landiolol) administration. RESULTS: Following surgery, 21 (22%) of the patients had new onset POAF. The occurrence of POAF was related to that of stroke. Univariate analysis showed that age was a risk factor for POAF. In patients with landiolol, risk factors for POAF were age, arch aneurysm, and timing of landiolol administration. Six patients developed POAF during landiolol administration. However, of 56 patients who had not have POAF during landiolol administration, 8 developed POAF after stopping landiolol. CONCLUSION: Our findings show that the risk factor of POAF after thoracic aortic surgery is age, while perioperative administration of landiolol may reduce POAF incidence.

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.