An infant with a vertical vein aneurysm in a supracardiac totally anomalous pulmonary venous connection.

Massive vertical vein aneurysm in a supracardiac total anomalous pulmonary venous connection is rare. Herein, vertical vein aneurysm with total anomalous pulmonary venous connection and additional pathological findings are reported in a young child.

Parathyroid carcinoma with pancreatitis causing hypercalcaemic emergency treated with extracorporeal membrane oxygenation-assisted parathyroid resection.

Summary: Emergencies due to malignancies usually have a severe clinical course and require urgent treatment. These scenarios are dubbed 'oncologic emergencies'. Parathyroid tumours often cause hypercalcaemia but not oncologic emergencies. We present a case of parathyroid carcinoma with severe hypercalcaemia and pancreatitis, resolved by surgical resection of the tumour assisted by extracorporeal membrane oxygenation (ECMO). A 66-year-old woman presented to our hospital because of haematuria. Laboratory findings were as follows: white blood cell count: 30 000, C-reactive protein: 17.7, calcium: 21.9, creatine kinase: 316, creatine kinase-myoglobin binding: 20, troponin I: 1415.8, amylase: 1046, lipase: 499, blood urea nitrogen: 57, and creatinine: 2.42. ECG was unremarkable. CT revealed a 4-cm low-density irregular tumour in the left lobe of the thyroid gland and severe pancreatitis. We diagnosed hypercalcaemia and pancreatitis due to parathyroid carcinoma. Volume expansion with isotonic saline was started immediately. Calcitonin, followed by denosumab, calcimimetic agents, and continuous hemodiafiltration were administered. The patient's general condition worsened due to uncontrolled hypercalcaemia. Urgent tumour resection was planned, assisted with ECMO for cardiopulmonary support and surgical field venous pressure reduction. Tumour histology was suggestive of parathyroid carcinoma. Hypercalcaemia and the patient's general condition improved gradually postoperatively. Hypercalcaemia is one of the oncologic emergency symptoms, commonly occurring because of lytic bone metastasis. However, reports about parathyroid carcinoma-causing life-threatening hypercalcaemia and pancreatitis are scarce; the fatality of this condition is estimated to be 30-70%. We report a case of survival of hypercalcaemia of malignancy. Learning points: Parathyroid carcinoma is relatively rare and sometimes causes emergent conditions such as hypercalcaemia and severe pancreatitis. General therapy for hypercalcaemia including aggressive saline dehydration, administration of furosemide, calcitonin, zoledronic acid, and evocalcet, and dialysis is sometimes ineffective for parathyroid carcinoma. Therefore, careful planning of therapy in case of exacerbation is important. During an emergency, rapid surgical treatment despite high calcium level is the best potential therapeutic strategy.

Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection.

OBJECTIVES: A few studies have described the outcomes of systemic-to-pulmonary artery shunt (SPS) for functional single ventricular heart with extracardiac total anomalous pulmonary venous connection (TAPVC). This study aimed to examine the outcomes of SPS with extracardiac TAPVC and identify the predictors of mortality before bidirectional Glenn operation (BDG). METHODS: Medical records of 41 children with single ventricular heart and extracardiac TAPVC who underwent SPS between 1998 and 2019 were reviewed retrospectively. The median age and body weight at SPS were 36 days and 3.4 kg, respectively. Surgical outcomes and predictors of mortality were investigated. RESULTS: Four operative deaths (10%) and 10 late deaths (27%) occurred before BDG. Of all the children, 19 underwent BDG at a median of 10 months since SPS and eight are waiting for BDG. In the multivariate analysis, preoperative pulmonary venous obstruction (p = 0.01) at initial surgery was most predictive of death before BDG. Patients who underwent simultaneous SPS and TAPVC repair were younger, had high preoperative rate of pulmonary venous obstruction, and more deaths before BDG. CONCLUSIONS: Survival outcomes of SPS for SVH with extracardiac TAPVC were improved as a whole due to the increase in knowledge and technique of management SPS. However, the patients who have preoperative pulmonary venous obstruction (PVO) and need SPS and TAPVC repair concomitantly in the early postnatal period have poor outcomes and still challenging. In such a case, staged TAPVC repair and SPS may be beneficial.

Outcomes of mitral valve replacement with bileaflet mechanical prosthetic valve in children.

OBJECTIVES: We examined the outcomes following mitral valve replacement with bileaflet mechanical prosthetic valve in children and identified the predictors for mortality and reoperation. METHODS: Medical records from 49 children who underwent mitral valve replacement between 1982 and 2015 were reviewed retrospectively. Median age and body weight at initial mitral valve replacement were 2.4 years and 9.7 kg, respectively. The median follow-up was 13 years. Surgical results and predictors for mortality and reoperation were investigated. RESULTS: There was no operative mortality; eight late deaths occurred. The actuarial survival rates were 89.5%, 84.2%, and 80.7% at 5, 10, and 15 years, respectively, after initial mitral valve replacement. The actuarial freedom rates from related complications were 89.5%, 78.3%, and 70.7% at 5, 10, and 15 years, respectively. Nineteen patients required 1st re-mitral valve replacement at a median of 5.9 years; six of these 19 required 2nd re-mitral valve replacement at a median of 8.9 years after 1st re-MVR. The actuarial freedom rates from re-mitral valve replacement were 86.0%, 56.8%, and 44.2% at 5, 10, and 15 years, respectively. No predictor for death was determined; however, the predictor for re-mitral valve replacement was initial valve diameter less than 19 mm. CONCLUSIONS: Survival outcomes among children after mitral valve replacement with bileaflet mechanical prosthetic valve in biventricular heart were satisfactory. However, complications, including re-mitral valve replacement, were frequent and the predictor was of a small prosthesis size.

Successful coronary transfer for transposition of the great arteries with bilateral intramural coronary arteries from a single aortic sinus.

We experienced an arterial switch operation in a patient with d-transposition of the great arteries associated with bilateral intramural coronary arteries from a single coronary ostium. In performing the coronary transfer, intramural course of bilateral coronary arteries was unroofed and coronary ostium was separated into two coronary buttons. The two coronary buttons were translocated to the neo-aorta using modified trap-door technique. The angiogram performed 6 years after the operation revealed well-developed coronary arteries without stenosis, and the patient is doing well 8 years after the operation.

Intermittent distal perfusion shortens hypothermic circulatory arrest time in aortic arch replacement surgery.

The procedure and efficacy of the intermittent distal perfusion during hypothermic circulatory arrest in total arch replacement was described. During hypothermic circulatory arrest, elephant trunk was fixed inside the descending aorta. Then, the AP Grid Catheter was inserted through the elephant trunk, and blood perfusion at a flow rate of 500 ml/min for 5 min was installed. After the perfusion, distal anastomosis was completed. Clinical results of 23 patients (Group I) with this technique were compared with these of 21 patients without the procedure (Group II). Continuous hypothermic circulatory arrest time was significantly shorter (32.7 vs. 72.7 min; p < 0.05) and postoperative serum creatinine level was significantly lower (1.29 vs. 1.68; p < 0.05) in Group I than Group II. The incidence of abdominal complication was also fewer in Group I. Intermittent distal perfusion shortens hypothermic circulatory arrest time and is protective for the lower body including kidneys.

Pericardial ectopic thymoma presenting with cardiac tamponade: report of a case.

Ectopic thymoma arising from organs other than the thymus, such as the neck, trachea, thyroid, lung and pericardium, is rare. To date, there have been only seven other cases of pericardial thymoma reported in the English literature. We herein report a case of pericardial ectopic thymoma that presented with cardiac tamponade. A 72-year-old Japanese male noticed body weight gain and leg edema. Chest computed tomography (CT) revealed pericardial effusion and an irregularly shaped mass in the pericardial space compressing the right atrium. He was considered to have cardiac tamponade due to a paracardiac tumor that developed following acute cardiac failure. The intraoperative frozen diagnosis was thymoma. Pericardectomy of the thickened pericardium, tumorectomy and thymectomy via a median sternotomy were performed. The final pathological diagnosis was pericardial ectopic thymoma associated with constrictive pericarditis. The differential diagnosis and complete resection of mediastinal tumors such as this rare case of thymoma are important to obtain a better prognosis, as patients with such tumors often present in a state of shock.