[Early Structural Valve Deterioration of Trifecta Aortic Bioprosthesis:Report of a Case].

Trifecta, an externally mounted bovine pericardial bioprosthetic aortic valve, provides excellent hemodynamic performance;however, early structural deterioration of this valve has been reported. A 60-year-old man with progressive dyspnea was admitted to the emergency unit of our institution. Seven years prior, he underwent aortic valve replacement with 23-mm Trifecta valve. Severe aortic valve regurgitation and stenosis due to structural valve deterioration was diagnosed and redo aortic valve replacement using an Inspiris valve was performed. Intraoperative findings revealed a large laceration in the left coronary cusp adjacent to the non coronary-left coronary commissure and leaflet calcification. Further, circumferential fibrous pannus ingrowth at the inflow portion was also noted. To avoid anticoagulation therapy and repeat surgery, mitral valve plasty and left atrial appendage clipping were performed simultaneously. Postoperative course was uneventful, and he was transferred to a rehabilitation facility on 36th postoperative day.

Giant coronary artery aneurysm associated with Kawasaki disease showing progressive dilation over 30 years.

A 33-year-old pregnant woman with a history of a giant coronary artery aneurysm (CAA) of the right coronary artery owing to Kawasaki disease (KD) was referred to our hospital for the management of pregnancy and delivery. The CAA was detected when she was 10 months old on the 24th day from the onset of KD and showed transient regression followed by progressive dilation and reached a size of 25 mm when she was 30 years old. The baby was delivered at 38 weeks of gestational age. Resection of the CAA and coronary artery bypass grafting were performed 5 months after the delivery. Pathological results suggest that progressive dilation of the CAA was owing to a reduction in elastic recoiling force caused by partial destruction of the internal elastic lamina and degenerated tunica media against an increase in blood pressure that accompanied the growth of the patient. The pathophysiology of CAAs with atypical clinical course may be different from that of typical CAAs owing to KD. .

Pseudoaneurysm of the sinus of Valsalva caused by infective endocarditis in a 7-year-old child with congenital heart disease.

We report a case of a pseudoaneurysm in the sinus of Valsalva, secondary to infective endocarditis in a child with trisomy 21. The patient had a history of subaortic stenosis, bicuspid aortic valve, and ventricular septal defect. Patch closure of the ostium of the pseudoaneurysm and aortic valve replacement was performed. The patient was discharged without severe complications.

Survival Outcomes of Two-Stage Intracardiac Repair in Large Ventricular Septal Defect and Trisomy 18.

BACKGROUND: Surgical management has not been encouraged in patients with trisomy 18 (T18) and congenital heart diseases due to poor survival. This study aimed to investigate (1) the appropriateness of palliative surgeries followed by intracardiac repair (ICR) (i.e., two-stage ICR) for patients with a large ventricular septal defect (VSD) and T18, and (2) its impact on their long-term outcomes. METHODS: Medical charts of patients with VSD and T18 who underwent two-stage ICR at the Japanese Red Cross Medical Center between January 2005 and December 2019 were retrospectively reviewed. Demographic data, timing, and types of palliative surgeries, information related to ICR, peri- and postoperative clinical information, postoperative survival, and cause of death were collected. The long-term prognosis of patients treated with two-stage ICR was compared with that of patients treated with primary ICR and palliative surgery without ICR. RESULTS: Overall, 18 (2 male, 16 female) patients underwent two-stage ICR. Pulmonary artery banding was the initial palliative surgery in all patients after a median duration of 19.5 (range 6-194) days of life. The median age and the mean body weight at the time of ICR were 18.2 (7.6-50.7) months and 6.0 ± 1.0 kg, respectively. The mean pulmonary artery pressure and pulmonary vascular resistance index before ICR were 19.1 ± 7.3 mmHg and 3.4 ± 2.0 U m2, respectively. Overall, 17/18 (94%) patients were discharged after ICR. Fourteen (78%) patients were alive during data collection. None of the patients died of cardiac insufficiency, and the median duration of survival was 46.3 (14.3-186.4) months since birth. Most patients required cardiac medications rather than pulmonary vasodilators at the last follow-up. During the study period, three patients underwent primary ICR, and 46 underwent palliative surgery without ICR. Of those who underwent primary ICR, two died in the hospital on the first and 48th day following ICR, and the third died 179 days after the ICR. The Log-rank test revealed a significantly longer survival for the patients treated with two-stage ICR compared with those treated with palliative surgery without ICR (P = 0.003). CONCLUSION: Two-stage ICR improves the long-term survival of patients with VSDs and T18. This safe surgical strategy can also prevent pulmonary hypertension in such patients.

Treatment of tumor thrombus in the superior mesenteric vein due to advanced colon cancer with complete surgical resection and chemotherapy: a case report.

BACKGROUND: Tumor thrombus in the superior mesenteric vein secondary to colon cancer is rare. We report a case of tumor thrombus in the superior mesenteric vein and liver metastasis due to advanced colon cancer that was treated with chemotherapy and complete surgical resection. CASE PRESENTATION: A 72-year-old man after transverse colectomy with lymph node dissection for advanced colon cancer was diagnosed with tumor thrombus in the superior mesenteric vein and liver metastasis. He underwent adjuvant chemotherapy and had complete surgical tumor resection involving tumor thrombectomy and hepatectomy. There has been no recurrence at 36 months after surgery. CONCLUSION: Herein, we report a rare case of tumor thrombus in the superior mesenteric vein related to advanced colon cancer. The combination of chemotherapy and complete surgical tumor resection may provide long-term survival.

[Hybrid Two-stage Repair of Syphilitic Aortic Aneurysm, Total Arch Replacement and Thoracic Endovascular Aortic Repair;Report of a Case].

Syphilitic aortic aneurysm is seldom seen in the antibiotic era. Statistically the number of patients is increasing today and 10% of them seem to develop syphilitic aortitis. A 59-year-old male visited the emergency room due to chest discomfort and general fatigue. Treponema pallidum latex agglutination (TPLA) and rapid plasma reagin (RPR) were both strongly positive on blood tests. White blood cell counts and C-reactive protein elevation were also found. He couldn't figure out how or when he was suffering from syphilis. He needed to undergo a hybrid 2-stage surgery urgently, Total arch replacement and thoracic endovascular aortic repair (TEVAR), because his thoracic aortic aneurysm was growing more rapidly. No complication has occurred during or after surgery. Computed tomography after surgery showed successful exclusion of the thoracic aneurysm. It is important not to forget that syphilis is one of the causes of aortic aneurysm.

[Bentall Procedure for Annuloaortic Ectasia in a Patient with Systemic Lupus Erythematosus;Report of a Case].

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease, rarely complicated with cardiac valvular abnormality or aortic aneurysm. An asymptomatic 40-year-old woman with a 15-year history of SLE developed annuloaortic ectasia and aortic valve regurgitation. She had long been maintained on steroid therapy for the treatment of SLE. Her serum anti-phospholipid antibodies were positive. An enhanced computed tomography revealed a dilated aortic root and ascending aorta (57 mm in diameter). Cardiac ultrasonography demonstrated severe aortic regurgitation. We performed the Bentall procedure using a composite graft with the Carrel patch technique. Histopathological examination of the aortic wall showed cystic medial necrosis without any evidence of vascu-litis. Careful long-term follow-up is mandatory for assessing the risk of later anastomotic dehiscence and pseudoaneurysm formation.

Axillofemoral Bypass Markedly Improved Acute Decompensated Heart Failure and Kidney Injury in a Patient with Severely Calcified Stenosis of Thoracoabdominal Aorta (Atypical Aortic Coarctation).

Atypical aortic coarctation (AAC) has been reported to occur anywhere along the aorta, except for the ascending aorta. The associated symptoms include hypotension in the lower half of the body, secondary hypertension in the upper half of the body, and heart failure. Here we present an 80-year-old Asian woman complaining of progressive exertional dyspnea. She was diagnosed with acute decompensated heart failure and kidney injury due to severely calcified stenosis of the thoracoabdominal aorta, the so called AAC. She received hemodiafiltration, and pulmonary congestion improved in part. Generally, surgical treatments are quite invasive in elderly patients. Endovascular stent graft placement is less invasive, however, fracture and rupture should be considered at severely calcified lesions like this case. Therefore, we selected extra-anatomical axillofemoral bypass. Her recovery after the surgery was remarkable. In a few days, she became free from hemodiafiltration, intravenous diuretics, and oxygen administration. We thought the contributive factors are the increase in kidney blood flow and the correction of afterload mismatch. The decrease in pulse pressure may reflect the reduction in systemic arterial compliance by axillofemoral bypass. The operative mortality of axillofemoral bypass was reported to be acceptable, although the patency of the axillofemoral bypass graft was not high enough. In conclusion, axillofemoral bypass is effective and feasible for elderly patients with acute decompensated heart failure and kidney injury due to AAC.

Left mammary artery bypass grafting rescued a patient with hypoplastic left heart syndrome with ascending aorta obstruction after norwood stage I procedure.

A three-month-old boy with hypoplastic left heart syndrome (mitral atresia, aortic atresia) and moderate tricuspid regurgitation developed ascending aorta obstruction two months after a Norwood stage I procedure. An emergent left mammary artery bypass grafting to the ascending aorta and extracorporeal membrane oxygenator support resulted in successful salvage. The patient subsequently underwent a bidirectional Glenn procedure and tricuspid valve repair at the age of five months. Follow-up at an outpatient clinic reveals no electrocardiographic evidence of ischemia, and echocardiography shows recovery of ventricular function.

Homograft use in patient with tetralogy of Fallot and absent pulmonary valve.

A neonate with tetralogy of Fallot and absent pulmonary valve presented with respiratory distress due to airway obstruction after birth. He was placed on mechanical respiration. At 28-days old, he underwent pulmonary artery reduction and pulmonary homograft insertion. Ventricular septal defect patch closure was performed under cardiopulmonary bypass. His postoperative course was complicated by prolonged mechanical ventilatory support because of tracheomalacia. After 1 year of follow-up, he was in good condition without pulmonary regurgitation.

Neonatal repair of right interrupted aortic arch with cerebro-myocardial perfusion technique.

Right interrupted aortic arch and descending aorta is exceedingly rare and most likely cause respiratory presentation, since patent ductus arteriosus (PDA) courses over the right mainstem bronchus. We report a case of successful neonatal biventricular repair of a right interrupted aortic arch (type B), with an aberrant right subclavian artery ventricular septal defect (VSD) in a 2.7 kg term neonate with DiGeorge syndrome. Patient presented in severe respiratory distress and acidosis at one day old. Two-dimensional (2D) echocardiography revealed aortic arch interruption beyond the common carotid arteries with large perimembranous outlet VSD. Aortic annulus diameter was 4.8 mm and there was no left ventricle (LV) outflow tract obstruction. Three-dimensional (3D) CT-scan confirmed these findings and identified a right-sided ductal arch that continued over the right mainstem bronchus into a right-sided descending aorta and aberrant right subclavian artery. Brachiocephalic perfusion and ductal perfusion was employed for cooling during cardiopulmonary bypass. Under deep hypothermia (27 °C rectal temperature), selective cerebro-myocardial perfusion was used for successful aortic arch repair without sacrificing the aberrant right subclavian artery. A direct tension-free anastomosis was attained. Her postoperative course was uneventful and her respiratory symptoms disappeared postoperatively. Early surgical correction is mandatory for these patients with unique anatomy and presentation.

Radical surgery for a ventricular septal defect associated with trisomy 18.

PURPOSE: Congestive heart failure is one of the major causes of early death of patients with trisomy 18. Ventricular septal defect (VSD) is the most common heart defect in patients with trisomy 18, and closure of the VSD may elongate the lifespan of the patient. Morphological characteristics of these patients, such as thoracic deformity, prominent right ventricular hypertrophy, and dysplastic tricuspid valve may complicate closure of the VSD. We report our initial experience of VSD closure in patients with trisomy 18 and estimate the feasibility of the surgical procedure. METHODS: Between June 2005 and September 2007, five female patients with trisomy 18 and VSD underwent radical operations. Four of them had undergone previous palliative surgery entailing pulmonary artery banding. RESULTS: All patients survived surgery. The average cardiac arrest time during surgery was 74 +/- 22 min. No early death occurred within 30 days of surgery. One patient with hydrocephalus treated by a previous ventriculoperitoneal shunt died in hospital owing to postoperative intracranial hypertension. Four of five patients were discharged from the hospital. Of the four discharged patients, a girl who underwent primary radical operation died of pneumonia. Another patient died of sudden cardiopulmonary arrest. The average survival of the patients in this study was 815 +/- 389 days at data acquisition, with two patients still alive. CONCLUSION: Closure of VSDs in patients with trisomy 18 was found feasible and was associated with extended survival.

A case of early phase dialysis associated effusive constrictive pericarditis with distinct surgical findings.

A 55-year-old male patient with a 10 year history of hemodialysis was admitted for examination of pericardial effusion. Specific echocardiography, MRI, and cardiac catheterization findings strongly suggested a diagnosis of effusive constrictive pericarditis. Pericardiectomy showed the following distinct findings. Two layers of fibrous tissue below the thickened pericardium emerged. The cardiac constriction had not been relieved until the inner layer was partially resected. We conclude that patients in dialysis associated constrictive pericarditis should undergo pericardiectomy before the adhesion between fibrous tissues becomes hard.

Cardiac surgery in patients with trisomy 18.

Cardiac surgery is infrequently but increasingly being used to repair congenital heart defects associated with trisomy 18. The clinical details of trisomy 18 patients undergoing cardiac surgery have rarely been reported. Seventeen patients with trisomy 18 and serious cardiac symptoms underwent cardiac surgery in our institution. Age at surgery ranged from 7 to 258 days (median, 66 days). One patient had an atrioventricular septal defect and coarctation of the aorta. The remaining patients had ventricular septal defects, including four patients with coarctation of the aorta. Fourteen patients had associated patent ductus arteriosus. Fourteen patients underwent palliative surgery without cardiopulmonary bypass, and four of these underwent a second-stage intracardiac repair. The other three patients underwent primary intracardiac repair. Postoperatively, 14 patients (82%) were discharged home with improved symptoms. Survival from birth ranged from 12 to 1384 days (median, 324 days). Eight patients survived longer than 1 year. Median postoperative survival was 179 days. Postoperative survival was significantly better after palliative surgery (0 to 1239 days; median, 257 days) than after primary intracardiac repair (1 to 179 days; median, 48 days). Only one patient died of heart failure, suggesting that cardiac surgery was effective in preventing heart failure-related death.

Right ventricular plication in a neonate with an unguarded tricuspid valvar orifice.

Unguarded tricuspid orifice is a congenital cardiac malformation characterized by absence of the tricuspid valvar leaflets in the setting of a normal atrioventricular junction and obligatory severe tricuspid regurgitation. A neonate with such a lesion presented with severe cardiac failure. The right atrium and ventricle were grossly dilated, and there were small muscular ventricular septal defects. We plicated the right-sided structures to create space for adequate expansion of the lungs and left ventricular filling, proceeding to staged conversion to the Fontan circulation. Our experience shows that neonates with cardiac failure due to dilated right-sided structures can be stabilized by surgical plication.

Intensive cardiac management in patients with trisomy 13 or trisomy 18.

Intensive cardiac management such as pharmacological intervention for ductal patency (indomethacin and/or mefenamic acid for closure and prostaglandin E1 for maintenance) and palliative or corrective surgery is a standard treatment for congenital heart defects. However, whether it would be a treatment option for children with trisomy 13 or trisomy 18 syndrome is controversial because the efficacy on survival in patients with these trisomies has not been evaluated. We retrospectively reviewed 31 consecutive neonates with trisomy 13 or trisomy 18 admitted to our neonatal ward within 6 hr of birth between 2000 and 2005. The institutional management policies differed during three distinct periods. In the first period, both pharmacological ductal intervention and cardiac surgery were withheld. In the second, pharmacological ductal intervention was offered as an option, but cardiac surgery was withheld. Both strategies were available during the third period. The median survival times of 13, 9, and 9 neonates from the first, second, and third periods were 7, 24, and 243 days, respectively. Univariate and multivariate analyses confirmed that the patients in the third period survived significantly longer than the others. Intensive cardiac management consisting of pharmacological intervention for ductal patency and cardiac surgery was demonstrated to improve survival in patients with trisomy 13 or trisomy 18 in this series. Therefore, we suggest that this approach is a treatment option for cardiac lesions associated with these trisomies. These data are helpful for clinicians and families to consider in the optimal treatment of patients with these trisomies.

Light-guided surgery to repair coronary sinus orifice atresia with left superior vena cava.

An 8-month-old male with coronary sinus orifice atresia, left superior vena cava, and single ventricle underwent light-guided coronary sinus unroofing concomitant with bidirectional cavopulmonary anastomosis to circumvent coronary sinus hypertension. During surgery, a 2.25 Fr angioscopic catheter was inserted into the coronary sinus via the left superior vena cava. The coronary sinus, lit by the illumination obtained from the catheter, was readily located from the left atrial interior, and unroofed. Light-guided coronary sinus unroofing is an easy, safe, and quick technique for the creation of unobstructed coronary sinus drainage in patients with coronary sinus orifice atresia and left superior vena cava.

Bronchial migration of a systemic-pulmonary shunt conduit.

Using an expanded polytetrafluoroethylene conduit, a subclavian artery-to-pulmonary artery shunt was created in an infant with tetralogy of Fallot. The postoperative course was complicated by sepsis, shunt occlusion, and pneumonia. Four years later, an obstructive mass was found in the right main bronchus on fluoroscopy and was retrieved on rigid endoscopy, which turned out to be the migrated conduit. This case implies that a vascular conduit anastomosed to a systemic artery can migrate into the airway without bleeding, pseudoaneurysm formation, or host artery occlusion.