Total anomalous pulmonary venous connection (TAPVC) is rarely associated with remarkably small left heart structures. In these types of cases, the hemodynamics resembles that of hypoplastic left heart syndrome, and the treatment strategy is controversial. We present the case of a 1-day-old girl with infracardiac TAPVC, small left heart structures (hypoplastic left heart complex), bilateral superior vena cava, and aberrant origin of the right subclavian artery. We performed a semi-emergent first-stage open palliation for repair of TAPVC, because of pulmonary venous obstruction. We concomitantly performed atrial septal defect (ASD) enlargement and bilateral pulmonary artery banding (BPAB). The postoperative course was uneventful and the left heart structures did not grow, so we performed the Norwood procedure and placed a right ventricle-pulmonary artery shunt with a 5.0 mm artificial graft. Subsequently, the left heart structures were not suitable for biventricular repair, so we chose univentricular repair. The patient underwent a bilateral bidirectional Glenn operation and Fontan completion at 6 and 23 months of age, respectively. TAPVC repair, BPAB, and ASD enlargement are reasonable surgical options for a patient with borderline small left heart structures and TAPVC, as they enable us to wait for growth in the left heart structures and to determine whether univentricular or biventricular repair is suitable.

Background : Connective tissue disease (CTD) is an idiopathic autoimmune disorder which causes systemic chronic inflammation. Inflammation causes various cardiovascular diseases. Systemic steroid use, which is usually the sole treatment for CTD, also causes arteriosclerosis. Although cardiovascular surgery is often necessary in patients with CTD, preexisting multiple organ dysfunction related to CTD, in addition to systemic administration of steroids or other immunosuppressants, is thought to increase the risk of surgery. However, little is known about how the disease process of CTD influences early and late cardiovascular surgery outcomes. Methods : To better understand these issues, we reviewed 31 patients with CTD (study group) and compared their outcomes to those of other patients (control group) who underwent cardiovascular surgery at our institution between April 2008 and November 2013. Results : There were 26 women and 5 men, and the average age was 64.4±16.7 years. CTD types included rheumatoid arthritis in 7 patients, systemic lupus erhythematosus in 6, aortitis syndrome in 6, polymyalgia rheumatica in 3, scleroderma in 3, polymyositis in 3, and others. The procedures included 10 valve cases, 10 coronary artery bypass grafting (CABG) or CABG-valve combination cases, and 11 isolated or complicated thoracic aortic surgery cases. Prior to undergoing these procedures, 24 patients (77.4%) were treated with steroids and/or immunosuppressant, and 6 patients had been diagnosed with interstitial pneumonia in the study group. Moreover, the rate of peripheral artery disease and carotid artery stenosis in the study group was significantly higher than that in the control group. There were no perioperative deaths in the study group. There were no significant differences in terms of major complications such as ischemic events, infection, acute kidney injury, lung injury, and others between the groups. We conducted a follow-up survey for the study group with an average period of 27.8±16.0 months. During the follow-up period, there were 4 late deaths. In addition, 8 patients required readmission, 6 for cardiovascular events and 2 for poor wound healing. All the survivors in the study group showed improved cardiac function and were in the NYHA functional class I and II. Conclusion : Cardiovascular surgery for patients with CTD can provide acceptable early and mid-term results.

Introduction : Prevention of embolic stroke is the key issue to perform aortic arch replacement in patients with a shaggy aorta. The aim of this study is to report the utility of the isolation technique for total arch replacement in patients with a shaggy aorta. Methods : Clinical results of seven patients (71.7 years old, all men) with a shaggy aorta who underwent total arch replacement between January 2017 and November 2018 were retrospectively reviewed. The operative indications were a distal arch or proximal descending aortic aneurysm in 6 patients and a thrombus inside brachiocephalic artery in one. A cerebral perfusion was established by inserting a cannula directly into all supra-aortic branches before starting systemic perfusion. Result : Utilizing the isolation technique with clamping of all branches in 4 patients and the functional isolation technique with clamping of two branches in 3, total arch replacement was performed in all patients (operation time : 513 min, selective cerebral perfusion time : 162 min). No operative death was observed and no newly developed stroke was encountered. Conclusion : The isolation technique is a useful method to prevent stroke during total arch replacement in patients with a shaggy aorta.

Among the less reported complications after thoracic endovascular aortic repair (TEVAR) is type II endoleak (T2EL). The intercostal and bronchial artery are known as feeder vessels to T2EL after TEVAR. We experienced two cases of successful treatment of percutaneous transarterial feeder vessels embolization via right costocervical trunk approach for patients with persistent T2EL and sac enlargement of an arch aneurysm after TEVAR. The costocervical trunk route is possible for key vessels to construct a collateral pathway to feeder vessels of the endoleak nidus of T2EL after TEVAR procedures for aortic arch aneurysm. A preembolizational Catheter-Directed CT angiogram (CTA) can be helpful to prevent harmful complications (e.g., spinal cord infarction).

Knitted Polyester prosthetic grafts can cause long-term dilatations and formation of anastomotic or non-anastomotic aneurysms, and rupture in result. We experienced a case of anastomotic pseudoaneurysm and recurrent non-anastomotic dilatation of the ascending aorta-abdominal aorta bypass by Cooley Double Velour Knitted Dacron (CDVKD) graft for a patient with atypical coarctation of the aorta (Takayasu Aortitis, type III), which case needed treatment two times over 30 years after the initial operation. The first additional treatment was Thoracic Endovascular Aortic Repair (TEVAR) for non-anastomotic aneurysm was done as 1st operation. Thirty-two years after the initial operation, the second treatment was a hybrid operation consisting of 4 procedures: bilateral axillo-external iliac bypass, taking down of the CDVKD graft at the proximal anastomotic site, endovascular repair (EVAR) with modified Double D Technique, and coil packing at the distal anastomotic site of the CDVKD graft. The patient was discharged at 37-POD. No complication and no endoleak has occurred in the 2.5 years since the operation.

We present a 62-year-old woman who was diagnosed with primary pulmonary arterial sarcoma with pulmonary hypertension. CT showed a large defect inside both main pulmonary arteries in accordance with the accumulation of FDG-PET. To relieve the symptom and to prevent sudden death, removal of a massive pulmonary tumor and postoperative chemotherapy were planned. Utilizing the intermittent systemic circulatory arrest under deep hypothermia (18°C), the pulmonary artery trunk and both main pulmonary arteries were opened. The tumor stacking inside the pulmonary artery was removed and its origin at the commissure of the pulmonary artery valve was resected. The defect was repaired with a pulmonary valve replacement. Histopathological examination revealed high grade sarcoma. Her postoperative course was uneventful ; however, she died of cerebral hemorrhage during chemotherapy six months after surgery.