A Re-do Repair of Tetralogy of Fallot With an Anomalous Coronary Artery Using a Valved Conduit for the Right Ventricular Outflow Tract Reconstruction.

Several techniques can be used for the repair of right ventricular outflow tract (RVOT) stenosis in patients with an anomalous coronary artery. Here, we report a case of conduit obstruction after re-operation following double-tract reconstruction and release of stenosis by main pulmonary artery transection and conduit replacement. The patient is a female child diagnosed with tetralogy of Fallot with an anomalous coronary artery (right coronary across right ventricle outflow) who underwent correction with a double-tract RVOT repair at the age of 10 months (weight: 8 kg). At the age of eight years (weight: 24 kg), a conduit re-implantation procedure was required because of an increase in body weight. Designing smooth conduits proved challenging due to the anomalous coronary artery and a short main pulmonary arterial trunk. RVOT stenosis was documented early postoperatively, and further intervention was deemed necessary. At 13 years of age (weight: 45 kg), the patient underwent implantation of an adult-size valved conduit. Transection of the main pulmonary artery and extensive mobilization of the bilateral pulmonary arteries were effective in creating a relative laminar blood flow. Postoperative evaluations confirmed that the conduit was well-shaped and had satisfactory valve functionality. This case highlights the potential difficulties involved with replacing an additional conduit after double-tract cardiac repair procedures, particularly due to anatomical constraints posed by a coronary artery and a short pulmonary arterial trunk. Main pulmonary artery transection and comprehensive mobilization of branch pulmonary arteries can be a solution to conduit design difficulties in RVOT reconstruction after double-tract cardiac repair procedures.

Evaluation of flow dynamics in distal stent graft-induced new entry using 4D flow MRI.

Distal stent graft-induced new entry may occur after stent grafting for aortic dissection. Four-dimensional magnetic resonance imaging is useful for predicting outcomes, showing accelerated flow and increased wall shear stress, indicating further false lumen expansion.

Melanocortin-4 receptor in macrophages attenuated angiotensin II-induced abdominal aortic aneurysm in mice.

Obesity is recognized as an independent risk factor for abdominal aortic aneurysm (AAA). While mutations in the melanocortin-4 receptor (MC4R) gene is the most common cause of obesity caused by mutations in a single gene, the link between MC4R function and vascular disease has still remained unclear. Here, by using melanocortin-4 receptor (MC4R) deficient mice, we confirmed MC4R deficiency promotes AAA and atherosclerosis. We demonstrated the contribution of two novel factors towards vascular vulnerability in this model: leptin signaling in vascular smooth muscle cells (VSMCs) and loss of MC4R signaling in macrophages. Leptin was shown to promote vascular vulnerability via PI3K-dependent upregulation of Spp1 expression in VSMC. Additionally, Ang II-induced AAA incidence was significantly reduced when MC4R gene expression was myeloid cell-specifically rescued in MC4R deficient (MC4RTB/TB) mice. Ex vivo analysis showed a suppression in NF-κB activity in bone marrow-derived macrophages from LysM(+);MC4RTB/TB mice compared to LysM(-);MC4RTB/TB mice, which exaggerates with endogenous MC4R ligand treatment; α-MSH. These results suggest that MC4R signaling in macrophages attenuates AAA by inhibiting NF-κB activity and subsequent vascular inflammation.

[Two-debranching Thoracic Endovascular Aortic Repair for Thoracic Aortic Aneurysm Complicated by Idiopathic Thrombocytopenic Purpura;Report of a Case].

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease in which the number of platelets decreases due to auto-antibodies against platelets. We report that thoracic endovascular aortic repair (TEVAR) was successfully performed for a thoracic aortic aneurysm complicated by ITP. The patient was a man of 77 years of age. He had a history of splenectomy due to ITP. He was admitted to our hospital with an aneurysm of the aortic arch that enlarged to a maximum minor axis of 63 mm. An operation was planned. Because of ITP, it was judged that replacement of the aortic arch using a cardio-pulmonary pump would be associated with a high risk of bleeding. Thus, 2-debranching TEVAR was selected and performed with no hemorrhagic complications. He was discharged from the hospital on the 12th day after surgery. We believe that 2-debranching TEVAR is effective for reducing perioperative bleeding in patients with ITP.

Pitfalls of Retrograde Cardioplegia Cannulation: Left Atrium Dissection.

Left atrial dissection is a rare complication of cardiac surgery. We present a case of left atrial dissection that occurred during a partial arch repair for Stanford acute type A aortic dissection. Because no entry was found in the left atrium by transesophageal echocardiography, and there were no issues weaning from cardiopulmonary bypass, we decided that no surgical intervention was necessary. Transthoracic echocardiography and computed tomography showed the remaining dissection on postoperative day 7; however, on postoperative day 14, it had completely disappeared.

Correction to: Outcomes of plasma exchange for severe dilated cardiomyopathy in children.

In original publication of the article, some of the co-author's names were not included. The correct author group is published in this article.

Outcomes of plasma exchange for severe dilated cardiomyopathy in children.

Antimyocardial autoantibodies are a cause of dilated cardiomyopathy (DCM). Immunoabsorption therapy for eliminating autoantibodies can improve cardiac function in adult DCM. The purpose of this study was to investigate the indication and efficacy of plasma exchange in children with DCM and their outcomes. We performed a single-center, retrospective study in children with DCM who had received plasma exchange (PE). Six patients in various degrees of heart failure (three patients in acute exacerbation phase, one patient in early phase, and two patients in chronic phase) received PE. The effects of first PE were that the left ventricular ejection fraction (LVEF) and New York Heart Association (NYHA) functional class were transiently increased in five of six patients (83 %) and in four of five patients (80 %), respectively. The median duration of improved cardiac function after first PE was 8 months. PE was performed a total of two times in two patients and three times in one patient. The effect of repeated PE was attenuated when compared with first PE. Improved LVEF and NYHA functional class were observed in two of four courses (50 %) and in one of four courses (25 %), respectively. The median duration of improved cardiac function was 1 month. PE can transiently improve cardiac function and clinical symptoms of DCM in children. PE may be an additional therapeutic option in children with refractory DCM. However, PE should only be considered as a bridge to ventricular assist device implantation or heart transplantation.

Benefits of Balloon-Dilatable Bilateral Pulmonary Artery Banding in Patients With Hypoplastic Left Heart Syndrome and Other Complex Cardiac Anomalies.

OBJECTIVES: The purpose of this study was to evaluate the potential of balloon-dilatable bilateral pulmonary artery banding (b-PAB) and its impact on the configuration of the pulmonary artery (PA). BACKGROUND: We have previously used balloon-dilatable b-PAB as first-stage palliation for patients with hypoplastic left heart syndrome (HLHS) and other complex cardiac anomalies. METHODS: Two pliable tapes were placed around each branch of the PA and tightened with 7-0 polypropylene sutures in a manner that allowed for the subsequent adjustment of PA diameters. We retrospectively examined the adjustability of PA diameters by balloon dilation and the need for surgical PA angioplasty at later stages. RESULTS: From January 2010 to October 2013, we performed b-PAB in 8 patients, including 3 borderline cases between biventricular repair (BVR) and univentricular repair (UVR). The b-PAB procedures were performed at a median age of 6.5 days (range, 2-10 days). Balloon dilations were performed in 10 lesions in 4 patients. All of the procedures were performed safely. Two patients reached definite BVR. The remaining 6 patients underwent open palliative procedures with univentricular physiologies that resulted in 2 deaths unrelated to the initial b-PAB. In all but 1 of the patients, the PA configuration was properly maintained and did not require surgical pulmonary angioplasty. CONCLUSIONS: Balloon-dilatable b-PAB can be performed safely and prevents PA distortion at later stages. This technique should be considered for patients with complex cardiac anomalies if uncertainty exists regarding the optimal surgical strategy (BVR or UVR) in early infancy.

[Right branch pulmonary artery stenosis with supravalvar aortic stenosis as a complication of Lecompte maneuver for tetralogy of Fallot associated with absent pulmonary valve].

The patient was diagnosed with tetralogy of Fallot associated with absent pulmonary valve syndrome and a low birth weight of 1,912 g. He suffered from respiratory distress on day 14 and received non-invasive positive pressure ventilation. At 5 months of age and 4.1 kg, he underwent intracardiac repair including right ventricular outflow repair with a monocusp patch, patch closure of the ventricular septum defect and right pulmonary transposition to the anterior of the ascending aorta following the Lecompte maneuver for airway decompression. He was subsequently discharged to home and exhibited an uneventful clinical course with non-invasive positive pressure ventilation for 5 months postoperatively. However, right pulmonary artery and supra-aortic stenosis was noted 2 years after the operation. Computed tomography (CT) and angiography showed ascending aorta strangulation by the right pulmonary artery with right ventricular outflow regurgitation. Right pulmonary artery reconstruction using polytetrafluoroethylene graft interposition and repeat right ventricular outflow repair with bicuspid hand-sewn valves was therefore performed;the postoperative course was uneventful. Pre- and post-operative management using non-invasive positive pressure ventilation and airway decompression with pulmonary artery translocation is a useful strategy in patients exhibiting symptomatic tetralogy of Fallot associated with absent pulmonary valve syndrome in the neonatal period.

Hybrid procedures for an infant with hypoplastic left heart syndrome with intact atrial septum.

A boy, prenatally diagnosed as hypoplastic left heart syndrome (HLHS) with intact atrial septum (IAS) was successfully treated by hybrid procedures. He underwent emergent catheter atrial septostomy and stent insertion in the atrial septum on Day 1 and then underwent bilateral pulmonary artery banding, ductal stent insertion, modified Norwood operation, bidirectional Glenn's operation and finally Fontan type operation at 2 years of age. Considering the presence of decompression pathway from the left atrium in HLHS with IAS, we should organize a treatment team for collaborative work and plan an appropriate treatment strategy before delivery. Although his clinical course has been uneventful until now, closer medical observation is warranted because he may have coexisting pulmonary disease.

Splenic artery aneurysm of the hepatosplenomesenteric trunk.

We herein report the case of a splenic artery aneurysm with a hepatosplenomesenteric trunk that presented in a pregnant woman. Catheter embolization was not performed due to the wide neck of the aneurysm and its close location to the trunk indicates a high risk of mesenteric trunk thrombosis. We instead performed surgical resection of the aneurysm after successful delivery of the infant by Caesarian section. The splenic artery was reconstructed by side-to-end anastomosis with the common hepatic artery.

Successful staged management of simultaneous abdominal aortic aneurysm and renal tumor: the novel minimally invasive treatment with endovascular aneurysm repair and retroperitoneal laparoscopic radical nephrectomy in an elderly and high-risk case.

The association between abdominal aortic aneurysm (AAA) and renal tumor is becoming more frequent, and the increasing incidence of this synchronous diseases raised questions about the procedures of treatment. In most of the previous cases, open nephrectomy and AAA surgery have been reported, however in high risk and elderly patients, the procedure is life threatening. We present a successful staged treatment of AAA and renal tumor using novel minimally invasive treatment with endovascular aneurysm repair and retroperitoneal laparoscopic radical nephrectomy in an elderly and high-risk case.

Long-term survival of Uhl's anomaly with total cavopulmonary conversion.

We report a long-term survivor with Uhl's anomaly who underwent one and a half ventricle repair combined with a partial right ventriculectomy in infancy, followed by successful total cavopulmonary conversion with right ventricular exclusion 5 years later. The combination of total cavopulmonary connection and right ventricular exclusion could be the optimal surgical option for a critically ill infant with Uhl's anomaly.