The patient was a 57-year-old woman. Even though she had received a diagnosis of tetralogy of Fallot as an student of elementary school, she did not consent to undertake surgical repair. Consequently, she had been observed until age 56, although she suffered from atrial fibrillation during this period. She decided to undergo surgical correction as she suffered from severe heart failure. The surgical repair included Maze procedure, a patch repair of ventricular septal defect, a repair of the right ventricular outflow tract and a pulmonary valve replacement. The right ventricular pressure reduced to around two-fifths of high left ventricular pressure postoperatively, and she was discharged without any complication.

There are few reports of cone reconstruction for neonatal Epstein's anomaly. We report 14-day-old girl with symptomatic Ebstein's anomaly with prenatal diagnosis prenatally and who was born by caesarean section. Continuous infusion of prostaglandin was started immediately and she was transferred to our institution. The cardiothoracic ratio (CTR) was 74% on chest roentgenograph. The echocardiograph showed severe tricuspid valve regurgitation (TR) and functional pulmonary atresia. Despite aggressive medical therapy, congestive heart failure had advanced and early surgical intervention was required. The anterior leaflet was severely plastered to free the wall, like the Carpentier type C. After cone reconstruction, the intraoperative echocardiography showed mild to moderate TR, antegrade flow to the pulmonary arteries and good function of both ventricles. The patient was extubated on the 6th postoperative day and stayed in the intensive care unit for another 2 days. The CTR was 58%, and the echocardiography showed a well-functioning RV with mild to moderate TR and a right to left transatrial shunt flow with an arterial saturation level of over 90%. Cone reconstruction might even be effective in such border line cases of biventricular repair with dysplastic anterior leaflet of tricuspid valve.

A 27-year-old man who presented with worsening dyspnea was transferred to our hospital due to congestive heart failure with multiple organ dysfunction. Echocardiogram showed severe left ventricular systolic dysfunction and a huge thrombus in the left ventricle. An urgent operation was performed to remove the thrombus simultaneously with the placement of bilateral extracorporeal ventricular assist devices. After the operation, despite a rapid improvement in the liver function, renal dysfunction persisted and he remained anuric for nearly a month. We continued maximal circulatory support with biventricular assist device to optimize his end-organ function. His renal function gradually improved, allowing him to be registered as a heart transplant candidate on the 140th postoperative day. On the 146th postoperative day, the patient underwent successful removal of the right ventricular assist device, and the left extracorporeal device was replaced by an implantable device (HeartMate II). He was discharged 78 days after the implantation. We present here a case where adequate support with biventricular assist device enabled a successful bridge to transplantation even in a patient with end-stage heart failure having end-organ dysfunction.

A 61-year-old woman presented with loss of consciousness. Echocardiography revealed a hypoechoic, round mass of 12×13 mm with a smooth border in the left ventricular outflow tract, leading to a diagnosis of loss of consciousness caused by left ventricular outflow tract obstruction, and surgical excision of the mass was performed. The mass, with a smooth, elastic soft surface and filled with yellow, creamy contents, was observed within a range from the subannular region of the left coronary cusp to the anterior mitral leaflet. Pathological examination showed central degeneration and liquefaction, as well as cystic, coarse-granular calcium deposition surrounded by inflammatory cell infiltration and fibroblast proliferation. No tumor cells were seen. Abscess was excluded by preoperative clinical presentations, hematologic data, and culture testing, and thus the mass was considered as caseous calcification of the mitral annulus. Here, we report a case of caseous calcification of the mitral annulus, a rare nonneoplastic lesion thought to be a variant of mitral annular calcification, with literature review.

Central diabetes insipidus (CDI) is a disease that caused by insufficient or no anti-diuretic hormone (ADH) secretion from the posterior pituitary, which results in an increase in urine volume. CDI is controlled with ADH supplementation thereby reducing urine output and correcting electrolyte imbalance. However, reports on perioperative management for CDI patient are scarce, especially for patients who underwent cardiac surgery. We herein report our experience of the management of a CDI patient who underwent surgery for valvular heart disease.

The case is a 72-year-old woman who developed secondary CDI after pituitary tumor removal. She had been controlled with orally administered desmopressin acetate hydrochloride. She underwent aortic valve replacement and mitral valve repair for severe aortic, and moderate mitral regurgitation. Immediately after surgery, we started vasopressin div, which yielded good urine volume control. However, once we started to switch vasopressin to oral desmopressin administration, the control became worse. We thus made a sliding scale for subcutaneous injection of vasopressin every 8 h according to the amount of urine output, which resulted in good control. Overlapping administration of vasopressin and oral desmopressin between postoperative day 12 and 17 resulted in successful transition. The patient was discharged with oral desmopressin administration. Management with sliding scale for vasopressin subcutaneous injection after surgery was useful in controlling a CDI patient who underwent major cardiac surgery.

The patient was 62-year-old woman was brought to the emergency room with chest pain and dyspnea. Computed tomography revealed a hematoma around the ascending aorta, a notch in the aortic wall, pericardial effusion and a hematoma around the pulmonary artery. We diagnosed early thrombotic type of acute aortic dissection. An ascending aorta replacement was performed via median sternotomy under hypothermic circulatory arrest. Upon operation, there was a 1.0 cm intimal tear just above the left main trunk and there was no specific evidence of aortic dissection. So we diagnosed spontaneous aortic rapture. Her postoperative course was uneventful and she was discharged 18 days after surgery.

We report a case of reoperation for proximal pseudoaneurysmal formation of the ascending aorta and distal enlargement of the dissecting arch and descending aorta after ascending aorta replacement for acute type A aortic dissection. The patient was a 47-year-old man who had undergone ascending aorta replacement and aortic valve replacement for acute type A aortic dissection three months previously. Pseudoaneurysm of the ascending aorta and enlargement of the dissecting arch and descending aorta were revealed by computed tomography. Therefore, we performed extensive replacement of the aortic root, arch and descending aorta. Median re-sternotomy with left anterolateral thoracotomy the (“Door open method”) was applied as the surgical approach. After reconstructing the aortic root using the modified Bentall procedure, we replaced the arch and descending aorta using antegrade continuous coronary perfusion with systemic blood through the composite graft of the aortic root under non-cardioplegic arrest. Despite the long duration of extracorporeal circulation, the duration of cardioplegic arrest was relatively short, and the postoperative cardiac function was not deteriorated at all. The patient is currently doing well with no problems at 1.5 years after the surgery. The Door open method was a useful approach providing good operative exposure in this case requiring extensive replacement of the thoracic aorta. Antegrade continuous blood coronary perfusion was useful for performing the arch and descending aortic replacement under non-cardioplegic arrest, and it was a reliable strategy for ensuring myocardial protection and avoiding prolonged duration of cardiac ischemia.

Endovascular treatment for chronic aortic dissection in patients with Marfan syndrome is still controversial. A 60-year-old man developed an extended chronic type B dissection involving the aortic arch and thoraco-abdominal aorta with a large entry at the distal aortic arch and patent false lumen. He had undergone David procedure for type A aortic dissection at age 42, and aortic valve replacement for recurrent aortic valve insufficiency at 58, which was complicated with mediastinitis. He also suffered drug-induced interstitial pneumonitis. Considering his complicated surgical history and impaired pulmonary function, conventional graft replacement of thoraco-abdominal aorta was thought to be quite a high risk. Thus, we chose debranch TEVAR with a staged approach. First, debranching and Zone 0 TEVAR with the chimney technique were performed. Then, 4 months later, abdominal debranching and TEVAR was performed. The patient tolerated both procedures well and was discharged home. Two years after last procedure, he is in good condition and computed tomography shows that complete entry closure and false lumen had thrombosed. This strategy may be worthy to be considered even for a patient with Marfan syndrome, in case the patient's condition is unsuitable for conventional surgery.

We encountered a case of ruptured sinus of Valsalva aneurysm caused by an unusual event. A 38-year-old-man was referred to our hospital owing to cough and orthopnea after being struck in the chest while playing baseball. Echocardiography showed a ruptured right sinus of Valsalva aneurysm and ventricular septal defect (VSD). Intraoperative findings revealed rupture of the sinus of Valsalva aneurysm into the right ventricle, with a typical “windsock” appearance. The fistula and VSD were closed with Dacron patches at both the right coronary cusp and right ventricular outflow tract. The patient was discharged on the 11th postoperative day without any complications.

We report a case of type A acute aortic dissection in a patient with situs inversus totalis. A 51-year-old man was hospitalized with sudden-onset back pain. Contrast-enhanced computed tomography revealed Stanford type A acute aortic dissection and situs inversus totalis. Total arch replacement using selective cerebral perfusion and mild hypothermic circulatory arrest was successfully performed. He was discharged home 23 days after the operation.

Aortopulmonary fistula with an arch aortic aneurysm is a rare disease that is difficult to diagnose and often presents with sudden, life-threatening heart failure. Here we report a case of aortopulmonary fistula for which we performed a thoracic endovascular aortic repair (TEVAR) with favorable results. A 79-year-old man presented with slurring of speech and body malaise at a neighborhood clinic. A distal arch aortic aneurysm was detected on chest computed tomography (CT) scans, and the patient was referred to our hospital for further management. We identified a saccular aneurysm and the dilated pulmonary artery, with maximum vessel diameters of 80 and 38 mm, respectively, on preoperative chest CT scans. He was diagnosed with an impending aortic rupture and a TEVAR was performed after preparing for a cervical ramification bypass. Intraoperatively, the aortopulmonary fistula had invaded the pulmonary artery, and the shunt created by the invasion was responsible for the sudden exacerbation of heart failure symptoms in the patient. The diameter of the saccular lump did not increase in the postoperative CT and follow-up visits were scheduled for subsequent monitoring. In the absence of significant complications and with improvement of heart failure symptoms, the patient was discharged from our hospital on the 37th postoperative day. He was later transferred to a neighborhood clinic for rehabilitation and subsequently discharged for further recuperation at home in the fifth postoperative month.

Endovascular treatment is a first-line treatment for renal arteriovenous malformations (AVMs). Endovascular treatment might be effective in patients with aneurysmal-type renal AVMs, which involve one feeding artery and one drainage vein, because control of the feeding artery, rather than the aneurysm itself, could have a therapeutic effect. Herein, we describe two cases of patients with renal AVM with multiple renal artery aneurysms, who were treated by controlling the arterial inflow alone. In Case 1, the patient was a 76-year-old woman with renal AVM discovered during examination for another medical condition. A computed tomography scan revealed four renal aneurysms (φ38/44/24/35 mm) ranging from an intimal defect in the right renal artery to the drainage vein running into the inferior vena cava (IVC). Although we had planned to use a covered stent in the right renal artery to cover the intimal defect without embolization of the aneurysms, a minor artery proximal to the aneurysm was found near the orifice of the right renal artery. Therefore, we used a covered stent in the right renal artery after embolization of the most proximal aneurysm was performed. In Case 2, a 78-year-old man was referred to our facility because a renal AVM was found during examination for lower back pain. The distal posterior branch of the right renal artery attached to the multiple aneurysms and directly drained into the IVC, which was diagnosed as an aneurysmal-type renal AVM. Because there were no arteries arising from the aneurysms in the right renal artery, which fed the renal parenchyma, embolization of only the inflow artery was performed. For both patients, renal blood flow was maintained without any decrease of the renal function. In these patients, although renin-angiotensin system activity was within the normal range, and blood pressure became better controlled postoperatively. In addition, there was significant improvement in the brain natriuretic peptide (BNP) levels postoperatively. Thus, we believe that unstable hypertension and/or high-output heart failure as well as the aneurysmal size should be assessed in the management of renal AVMs.