Corrigendum: Use of the index of pulmonary vascular disease for predicting longterm outcome of pulmonary arterial hypertension associated with congenital heart disease.

[This corrects the article DOI: 10.3389/fcvm.2023.1212882.].

Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease.

Aims: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH. Methods: This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death. Results: The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD <2.0 (P = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45-13.73; P = .009). Conclusions: The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered.

[Arterial Switch Operation for the Criss-cross Heart Complicated by Muscular Ventricular Septal Defect:Report of a Case].

Criss-cross heart is an extreamly rare anomaly characterized by abnormal rotation of the heart on its long axis. Almost always there are associated cardiac anomalies such as pulmonary stenosis, ventricular septal defect (VSD) and ventriculoarterial connection discord, and most cases are candidates for Fontan procedure due to hypoplasia of right ventricle or straddling atrioventricular valve. We report a case of arterial switch operation for criss-cross heart with muscular ventricular septal defect. The patient was diagnosed with criss-cross heart, double outlet right ventricle, subpulmonary VSD, muscular VSD and patent ductus arteriosus (PDA). PDA ligation and pulmonary artery banding (PAB) was performed in the neonatal period, and an arterial switch operation (ASO) was planed at 6 months of age. Preoperative angiography showed nearly normal right ventricular volume and echocardiography showed normal subvalvular structures of atrioventricular valves. ASO, intraventricular rerouting and muscular VSD closure by sandwitch technique were successfully performed.

[Asymptomatic Left Atrial Appendage Aneurysm:Report of a Case].

We successfully performed resection of a large asymptomatic left atrial appendage aneurysm( LAAA) under non-cardiac arrest. Although the patient was asymptomatic, chest X-ray showed projection of the third left aortic arch, and computed tomography angiography showed a huge left atrial appendage aneurysm. Therefore, we decided to perform LAAA resection via a partial median sternotomy. Aneurysmectomy was performed by placing the patient on pump beating heart. The size of the resected aneurysm was approximately 80 mm in diameter. Because the base of the LAAA was constricted and the tissue was not fragile, resection was performed using an automatic excision anastomosis device( Echelon Flex). The patient's postoperative course was uneventful. Left atrial appendage aneurysm is believed to be congenital;however, it is often detected in adulthood. Long-term follow-up is necessary to ensure maintenance of optimal cardiac function.

[Double Switch Operation for Congenitally Corrected Transposition of the Great Arteries after the Loose Pulmonary Artery Banding].

Congenitally corrected transposition of the great arteries( ccTGA) is a rare complex cardiac anomaly, where the heart twist and the ventricles are reversed during fetal development. Through a double switch operation (DSO), the morphologic left ventricle (LV) can be corrected back to pumping blood for systemic circulation. For successful DSO, the morphologic LV may need training with a pulmonary artery banding (PAB) before surgery. There have been reports of early LV failure in patients who underwent PAB training and a DSO, due to rapid pressure loading on the myocardium. We reported a case of DSO in a 16 years old male after prolonged LV training with a loose PAB. He was scheduled for a DSO, due to severe tricuspid regurgitation( TR). At eight years old, he underwent a loose PAB training with left ventricular pressure to right ventricular pressure ratio (LVp/RVp) of 0.5. After eight years, the LVp/RVp ratio increased to 0.77;he successfully underwent a DSO with good LV function.

Neonatal intra-atrial baffle repair for isolated ventricular inversion with left isomerism: a case report.

BACKGROUND: Discordant atrioventricular connection with concordant ventriculoarterial connection, otherwise known as isolated ventricular inversion (IVI), is an extremely rare congenital cardiac malformation. Reports on the corrective surgery for this anomaly in neonates are few, and the procedure is difficult and complicated. Herein, we report our use of atrial septostomy as a palliative procedure followed by corrective surgery for the repair of neonatal IVI with situs ambiguous(inversus) morphology. CASE PRESENTATION: A 2-day-old girl weighing 3.5 kg was admitted to our hospital with a low oxygen saturation (SpO2) of 70% She was diagnosed with IVI [situs ambiguous(inversus), D-loop, and D-Spiral], atrial septal defect, patent ductus arteriosus (PDA), interrupted inferior vena cava with azygos continuation to the left superior vena cava (SVC), and polysplenia by transthoracic echocardiography and cardiac computed tomography. We planned to perform corrective surgery and decided to first increase interatrial mixing by performing surgical atrial septostomy and PDA ligation 7 days after birth. However, despite the surgical septostomy, pulmonary venous blood flowed toward the right ventricle via the tricuspid valve rather than toward the left-sided atrium and hypoxemia persisted. We decided to perform the intra-atrial switch procedure at the age of 17 days via a re-median sternotomy. The cardiopulmonary bypass (CPB) circuit was established with ascending aorta and venous drainage through the SVC and hepatic veins. Utilizing a left-sided atrium(l-A) approach, a bovine pericardial patch was used for the intra-atrial baffle, which was trimmed into a trouser-shaped patch. Continuous suture using the patch was lying from the front of the right-sided upper pulmonary vein and rerouted SVC, hepatic vein, and coronary sinus to the tricuspid valve. Overall, CPB weaning proceeded smoothly; however, direct current cardioversion was performed for junctional ectopic tachycardia. The postoperative course was uneventful. Her postoperative SpO2 improved (approximately 99-100%); overall, the patient showed clinical improvement. Discharge echocardiography showed normal biventricular function and an intact atrial baffle with no venoatrial or atrioventricular obstruction. CONCLUSION: We successfully performed an intra-atrial switch procedure for isolated ventricular inversion in a neonate. Long-term follow-up will be necessary to ensure the maintenance of optimal cardiac function.

[Revisiting Damus-Kaye-Stansel Procedure for Taussig-Bing Anomaly in Adults;Report of a Case].

We successfully performed the DKS+Rastelli procedure for Taussig-Bing anomaly (TBA) in an adult. A 22-year-old male was admitted to our hospital due to persistent lower leg edema and dyspnea. He had been diagnosed with TBA. On physical examination, his peripheral oxygen saturation (Spo2) was 73% in room air. Chest X-ray showed congestive heart failure. We decided to perform arterial switch operation (ASO) and VSD closure. Operation was performed via a re-median sternotomy. It seemed to be very difficult to dissect and mobilize great arteries and coronary arteries for ASO. Therefore, we decided to perform DKS, aortic valve closure, left ventricule to pulmonary artery rerouting, and right ventricular outflow reconstruction using expanded polytetrafluoroethylene (ePTFE) valved conduit. Postoperative course was uneventful, an oxygen saturation was improved. At 5 years after surgery, his oxygen saturation is about 97% in room air and he is now doing well without any symptoms. Long-term follow-up is necessary to ensure maintenance of optimal function.

[Emergency Patch Closure of Coronary Artery Fistula During the Neonatal Period;Report of a Case].

We describe surgical treatment of a coronary artery fistula (CAF) in a neonate. A 5-day-old male neonate was diagnosed with CAF at another hospital and was transferred to our hospital. Transthoracic echocardiography revealed a right coronary artery-to-right ventricle (RV) fistula. Emergency surgery was performed to treat progressive congestive heart failure due to a significant left-to-right shunt through the large CAF. Intraoperatively, we confirmed that the large CAF drained into the inlet of the RV through multiple openings. Intracardiac CAF closure was performed with a bovine pericardial patch. The neonate showed an uneventful postoperative recovery, and postoperative echocardiography revealed a trivial residual shunt with good left ventricular function without increased pulmonary artery pressure.