Background : Several studies have shown that Fontan circulation may lead to liver congestion and possible structural liver alteration. The aim of this study is to analyze the relationships between biochemical fibrosis markers and hemodynamic parameters in the long term after the Fontan operation.
Methods : The study enrolled 51 patients who underwent total cavopulmonary connection between March 1994 and July 2010. We analyzed the relationships between the 5 liver fibrosis markers (hyaluronic acid, retinol-binding protein, procollagen type III peptide, type IV collagen 7S, type IV collagen) and the 6 hemodynamic parameters (pulmonary artery pressure, pulmonary artery index, pulmonary vascular resistance, ejection fraction, atrioventricular valve regurgitation, cardiac index).
Results : Hyaluronic acid and type IV collagen 7S positively correlated with pulmonary artery pressure. Hyaluronic acid negatively correlated with ejection fraction, and type IV collagen 7S positively correlated with atrioventricular valve regurgitation in patients followed up for more than 8 years after Fontan completion. Pulmonary artery pressure was significantly higher in patients in whom type IV collagen 7S was elevated. Hyaluronic acid correlated with pulmonary vascular resistance (p=0.0035) and ejection fraction (p=0.014), as well as type IV collagen 7S with pulmonary artery pressure (p=0.0001) by multiple regression analysis.
Conclusion : Hyaluronic acid and type IV collagen 7S reflected the degree of hepatic congestion, and cardiac function, in the long term after the Fontan operation.

Persistent sciatic artery (PSA) is a very uncommon congenital malformation. Its incidence is estimated to be 0.01-0.06%. In this article, we have described the case of 59-year-old woman presenting with a left lower limb ischemia. She had a good pulse in her femoral artery. However, no pulsation of her left popliteal, pedal and posterior tibial artery was observed. The ankle brachial index (ABI) was 1.05 on the right side and 0.65 on the left side. Preoperative angiography and CT scan showed that persistent sciatic artery had been branched from left internal iliac artery and the distal side had completely occluded. This persistent sciatic artery had no aneurysmal changes. The left superficial femoral artery was hypoplastic. Blood supply to the left peroneal artery, anterior and posterior tibial arteries by collateral arteries was detected. The left leg ischemia was diagnosed due to chronic occlusion of the persistent sciatic artery. Then, the bypass grafting was performed from the left common femoral artery to the above-knee popliteal artery using an ipsilateral reversed great saphenous vein graft. Postoperatively, ABI improved to 0.89 on the left side.

A 58-year-old man who complained of dyspnea on effort was given a diagnosis of decompensated congestive heart failure. Echocardiography revealed severe aortic regurgitation and cardiomegaly. We decided to perform aortic valve replacement with a mechanical valve, however his past history made us suspicious of allergy to metal. From his previous patient records, we determined he was allergic to many metals : gold, iron, platinum, cobalt, chrome, bronze, and zinc. Newly performed skin patch tests showed positive reactions to aluminum, tin, palladium, indium, iridium and stainless steel. We selected a CarboMedics mechanical valve made of nickel-titanium alloy. Aortic valve replacement with a 27-mm CarboMedics mechanical valve was performed by median sternotomy. At sternum closure, we used polyester non-absorbable suture thread, instead of surgical steel wire, because it contains stainless steel. His postoperative progress was good and he was discharged on the 10th postoperative day. One year after surgery he is doing well without any allergic symptoms.

A 78-year-old woman had been undergoing medical treatment for hypertension since she delivered a son in her early twenties. Three months previously, she was admitted with heart failure. She had felt leg fatigue for a long time, and the pressure gradient between the upper and lower limbs was about 60 mmHg. On further examinations, she was found to have an atrial septal defect (ASD), tricuspid valve regurgitation, atrial fibrillation, and severe coarctation of the aorta (CoA) with well-developed collateral arteries. We performed ASD closure, tricuspid annuloplasty with a flexible ring, left atrial maze operation and extra-anatomic bypass from the ascending to the abdominal aorta through a median sternotomy and upper median laparotomy. She had no postoperative complications and the pressure gradient between the upper and lower limbs improved remarkably postoperatively. It is rare for a patient over 70 years old who for the first time was given a diagnosis of CoA and ASD with other heart disease and who underwent surgical correction. We think one stage surgery with extra-anatomic bypass from the ascending to the abdominal aorta is a safe and effective technique for patients suffering CoA with heart disease.

A 63-year-old man underwent Y-graft (16×8 mm Hemashield Gold^®) replacement for an abdominal aortic aneurysm in our department in December 2008. In December 2010, the patient was given a diagnosis of right hydronephrosis in another department, for which a right ureteral stent was placed. The condition was resolved and the stent removed in July 2011. Thereafter in October, the patient developed abdominal distension and macroscopic hematuria. Under a diagnosis of recurrent right hydronephrosis, another ureteral stent was placed, though macroscopic hematuria persisted. He was referred to our department, and was admitted for detailed examinations and treatment. Contrast-enhanced computed tomography showed no obvious ureteroarterial fistula or contrast media leakage in the ureter. Because of the history of long-term ureteral stenting and prolonged macroscopic hematuria, regardless of the absence of a hemorrhagic lesion in the urinary tract, an ureteroarterial fistula in the anastomotic site of the right prosthetic leg and right common iliac artery was strongly suspected. There were no findings indicating an infectious complication, thus endovascular treatment was performed for hemostasis from a ureteroarterial fistula. We performed endovascular treatment with coil embolization (TORNADO^® 7 mm×3, 5 mm×2) of the right internal iliac artery and a covered stent (fluency plus^® 10 mm×80 mm) placement from the right prosthetic leg to the right external iliac artery, after which the hematuria disappeared. Although long-term outcomes following endovascular treatment for ureteroarterial fistula have not been reported, such treatment is considered to be quick and effective, with a low level of invasiveness, thus it should be considered as an effective therapeutic option for such cases in addition to open surgery.

A 67-year-old man was admitted with right heart failure. He had severe peripheral edema of his lower limbs. The heart failure was treated by diuretics, but after 3 months, he was re-admitted with facial edema and pleural effusion. At this time, the pericardium thickened diffusely and rapidly, constricting the heart. Pericardiectomy was performed to alleviate symptoms of heart failure. The thickened pericardium firmly adhered to the epicardium, from which it was inseparable. A partial pericardiectomy was done. The diagnosis was not confirmed during operation, but primary malignant pericardial mesothelioma was diagnosed on immunohistological examination with carletinin. The patient died from massive pleural effusion and heart failure on the 22nd postoperative day. Primary malignant pericardial mesothelioma is an extremely rare pathology, which is difficult to diagnose and has a poor prognosis. However, this pathology is the disease which we should always mention as a cause of constrictive pericarditis.

A girl was given a diagnosis of Takayasu arteritis (TA) with thoracic aortic aneurysm (TAA) at 11 years old, and began receiving medical treatment using steroids. At age 19, her biological inflammation markers were within normal limit ; however, contrast-enhanced CT showed the enlargement of the TAA to 60 mm in diameter. The risks and benefits of traditional revascularization vs endovascular procedures were carefully considered. The presence of suitable anatomical conditions, including adequate 20-mm-long landing zones, led us to select an endovascular approach. We exposed the right common iliac artery via a retroperitoneal approach, then, original stent grafts, using Z stents covered with thin-wall polytetrafluoroethylene, were positioned in the descending aorta from the level of the left subclavian artery to the level of the celiac artery. Intraoperative arteriography showed a patent stent graft without endoleaks or enlargement of the TAA. The postoperative course was uneventful, and follow-up CT scan performed 4 years after the repair showed shrinkage of the aneurysm diameter, and no evidence of any endoleaks. We concluded that endovascular treatment for TA-related aneurysm can be useful and safe, but further follow-up is needed in this case.

We report a rare case of lipoma arising from the right ventricle. A 66-year-old woman was admitted to our hospital for exertional chest pain and fatigability. She was diagnosed of mild aortic stenosis and regurgitation (ASR), mild mitral regurgitation (MR), and asymptomatic cardiac tumor in the right ventricle about two years previously, for which she had been followed up at other local hospital. A recheck transthoracic echocardiography revealed moderate MR. No evidence of deterioration of ASR and cardiac lipoma were detected. The patient underwent mitral annuloplasty and replacement of aortic valve, plus resection of the right ventricle tumor through the tricuspid valve. Pathological examination of the resected tumor showed mature adipose tissue infiltrated into normal cardiac muscle without atypical cells, which suggested intramyocardial lipoma. Postoperative course was uneventful. Fourteen months after the operation, the patient remains asymptomatic and regular echocardiographic checkup demonstrates no tumor recurrence or residual MR.

Introduction : Although there are various diagnostic tools like computed tomography, magnetic resonance imaging, and positron emission tomography, it is sometimes difficult to precisely diagnose cardiac tumors. Early pathological diagnosis is crucial for possible chemotherapy and/or radiation therapy in cardiac malignant lymphoma. Objectives : To assess the diagnostic value of pericardial excisional biopsy and cytology of pericardial effusion for the pathological diagnosis of cardiac lymphoma. Methods : Five patients had a clinical diagnosis of cardiac tumor with no pathological diagnosis. The pericardial biopsy and pericardial effusion were obtained without sternotomy through the subxyphoid by a small incision under local anesthesia. Results : All procedures were completed without complications. In 3 cases, the cytology of pericardial effusion yielded a diagnosis of malignant lymphoma. One patient who had a negative cytology result in whom surgical resection was performed for definitive diagnosis and tumor volume reduction was found to have malignant lymphoma. The remaining patient underwent tumor biopsy via a cardiac catheter and benign lymphoma was diagnosed. There were no specific findings in the pericardial excisional biopsy in 3 cases. Conclusion : The analysis of cytology of pericardial effusion obtained through the subxyphoid may be useful for the diagnosis of cardiac malignant lymphoma. Pericardial excisional biopsy may not be necessary for the diagnosis of cardiac malignant lymphoma.

Extra-anatomical aortic bypass through median sternotomy for aortic recoarctation allows concomitant repair of associated cardiac defects while avoiding potential complications of anatomic repair, including hemorrhage, bleeding from adhesions, nerve damage and spinal cord ischemia. We describe here the case of a 13-year-old boy who presented with aortic regurgitation and aortic recoarctation after two previous anatomic repairs with a prosthetic graft through thoracotomies. Ascending-abdominal aortic bypass and aortic valve replacement (AVR) were performed concomitantly through a median sternotomy. Extra-anatomical aortic bypass through a median sternotomy is useful for aortic recoarctation. Careful observation is required for younger patients.