Completely detached mechanical aortic valve prosthesis stuck to the aortic arch in a patient with Behçet's disease.

BACKGROUND: Although detachment of the implanted valve prosthesis was a well-known complication in patients with Behçet's disease, complete detachment of an aortic bileaflet valve prosthesis has never been reported. CASE PRESENTATION: An 18-year-old boy with Behçet's disease (HLA-A26 positive) who had previously undergone aortic valve replacement with an 18-mm ATS-Advanced Performance (ATS-AP) valve (ATS Medical, Inc., Minneapolis, MN) at the age of 12 years, presented sudden-onset general fatigue and was emergently transferred to the regional hospital. Chest X-ray showed displacement of the implanted mechanical valve. An echocardiogram revealed mobile valve prosthesis and severe aortic regurgitation. Just before leaving for our hospital for surgical treatment, a completely detached valve prosthesis was floating in the ascending aorta. On arrival, the valve prosthesis was stuck to the transverse arch. Emergent removal of the previous mechanical valve from the aortic arch and redo aortic valve replacement with a 24-mm ATS-AP valve were performed under total circulatory arrest. Infectious endocarditis was denied by histopathological examination. The patient was back to the intensive care unit with extracorporeal membrane oxygenation support, which was successfully decannulated 5 days later. CONCLUSIONS: This was the first report of a patient with Behçet's disease who encountered a complete detachment of implanted aortic valve prosthesis. The patient could be rescued by emergent surgery.

Successful Percutaneous Thrombectomy of the Left Pulmonary Artery in a Neonate After a Patent Ductus Arteriosus Clipping.

We report a neonate with a successful percutaneous thrombectomy of a total thrombotic occlusion of the left pulmonary artery (LPA) after a surgical clipping for a patent ductus arteriosus (PDA). We suspected the compression of the LPA by the clipping and postoperative hemodynamic instability caused the LPA obstruction. After the surgical removal of the PDA clip and division of the PDA, we could safely retrieve the LPA thrombus with a non-hydrodynamic thrombectomy catheter for coronary arteries.

A rare case of pediatric primary cardiac tumor in a patient with Down syndrome.

Although hematological malignancies are a known complication of Down syndrome, few reports have described cases involving solid tumors. We describe the case of a 3-year-old Down syndrome girl with a primary solid cardiac tumor. Outpatient echocardiography after intracardiac repair of a ventricular septal defect at 6 months of age revealed a highly mobile pedunculated mass (8 × 9 mm) on the free wall of the right atrium. Due to potential incarceration of the mass in the tricuspid orifice, it was excised under extracorporeal circulation and cardiac arrest. Macroscopically, the tumor closely resembled a papillary fibroelastoma, although histopathological tests were inconclusive.

Hybrid Palliation for Interrupted Aortic Arch With Small Aortic Valve.

OBJECTIVES: Open heart surgery for interrupted aortic arch in the neonatal period is still a high-risk procedure related in part to patient factors such as low birth weight, other morphologic anomalies, and, especially, small aortic valve size. Recently, we performed hybrid palliation with bilateral pulmonary artery banding and ductal stenting as the first-stage palliation for such cases. In this study, the outcomes of this procedure were examined. METHODS: Six cases of interrupted aortic arch with a small aortic valve underwent the hybrid procedure in the neonatal period in our institute from 2010 to 2015 (mean age: 6.8 days, mean body weight: 3.2 kg, mean z score of the aortic valve annulus: -8.3). Their postoperative clinical courses and results of the second-stage surgery were evaluated. RESULTS: No mortality or severe morbidity was seen in association with initial hybrid palliation. Five of six patients were discharged from the hospital; the one exception had a significant urinary tract anomaly. None needed an additional catheter intervention or surgical procedure postoperatively. All surviving patients underwent second-stage surgery; three had biventricular repair by the conventional method or Damus-Kaye-Stansel anastomosis with the Rastelli procedure and the other three proceeded toward staged Fontan reconstruction. Growth of the aortic valve was seen in four patients, and increased indexed left ventricle volume was recognized in one after the palliation. CONCLUSION: Hybrid palliation could be useful not only to avoid high-risk neonatal surgery but also to allow for eventual selection of the second-stage surgery based on the observations of potential interval development of left ventricular structures.

De Novo Migraine With Aura After Surgical Repair of Aortic Coarctation.

Coarctation of the aorta (CoA) is reported to be associated with an increased risk for migraine. We describe here the case of a 13-year-old female patient who presented migraine with aura after surgical repair of CoA with a stent. As possible reasons for her condition, we postulate host responses to stent placement and/or disturbed cerebral autoregulation related to intracranial hypertension before the surgical repair and hypotension afterward, leading to hypoperfusion. This case demonstrates that de novo migraine with aura can occur after surgical repair of CoA and should be recognized as a potential complication.

Evaluation of 60 cases of systemic-pulmonary shunt with cardiopulmonary bypass.

OBJECTIVE: In recent years, the median sternotomy approach with cardiopulmonary bypass has been increasingly chosen when systemic-pulmonary shunt surgery is performed as initial palliation for congenital heart diseases with decreased pulmonary blood flow to secure a stable surgical field and to maintain a stable circulation and oxygen supply. Since 2007, this strategy has been applied in our institute. This time, we examine the advantage and disadvantage of this procedure by evaluating the intraoperative and postoperative courses. METHODS: The study investigated 60 cases that underwent systemic-pulmonary shunt surgery under cardiopulmonary bypass at our facility after August 2007. Original diagnosis, age and body weight at surgery, shunt procedure, concomitant procedure, and surgical times were evaluated. The postoperative course of each case and the results of subsequent surgeries were also examined. RESULTS: No death or severe complication occurred during surgery or in the perioperative period. The age at surgery was 4 days-12 years (median 5.1 months), and the body weight was 2.3-28.1 (median 4.7) kg. Surgical procedures were as follows: right modified Blalock-Taussig shunt (mBTS): 35, left mBTS: 11, and central shunt: 14. In addition, the following simultaneous surgeries were conducted: pulmonary artery plasty: 11, unifocalization: 5, main pulmonary artery ligation: 2, interatrial communication enlargement: 4, and total anomalous pulmonary venous drainage repair: 2. CONCLUSION: The outcomes at our facility have validated the safety of systemic-pulmonary shunt surgeries under cardiopulmonary bypass, and even the disadvantages of concern were believed to be within acceptable limits. Further innovation and examination are important in pursuit of even less invasive surgeries.

Giant epicardial cyst presenting as acute cardiac tamponade in a 2-year-old boy.

Cystic structures within the pericardial cavity are rare. They are divided into epicardial and pericardial variants. Pericardial and epicardial cysts rarely cause symptoms. This report describes a case of epicardial cyst with acute cardiac tamponade in a 2-year-old boy with no previous cardiac history who was transferred to our hospital because of hemodynamic instability. Emergency drainage of the pericardial effusion and complete excision of the cyst were performed through a median full sternotomy.

Influence of bidirectional cavopulmonary anastomosis and concomitant valve repair on atrioventricular valve annulus and function.

BACKGROUND: The relationship between atrioventricular valve regurgitation (AVVR) and valve annulus after bidirectional cavopulmonary anastomosis (BCPA) and adequate indications for valve repair are unclear. METHODS: We evaluated the size of the valve annulus and the grade of AVVR before and immediately after BCPA, and at the most recent follow-up before the Fontan operation in 37 patients with a functional single ventricle. RESULTS: Nine patients underwent concomitant valve surgery. The mean z value of the valve annulus was significantly lower postoperatively than preoperatively in the 28 patients who were not treated by valve surgery (0.45 vs 1.51, p=0.01). However, mean regurgitation scores did not significantly change after BCPA (1.60 vs 1.78, p=0.08). The most recent assessment showed that the mean z value increased compared with that immediately after BCPA (1.36 vs 0.45, p=0.005). This increase was significant in the patients with moderate regurgitation. The mean z value of the valve annulus of the patients treated by concomitant valvuloplasty was significantly lower postoperatively than preoperatively (-0.25 vs 3.9, p=0.0001) and remained low at the latest evaluation. Mean regurgitation scores also significantly decreased after BCPA (2.25 vs 3.37, p=0.007). CONCLUSIONS: Unloading the systemic ventricle by BCPA leads to a decrease in the relative size of the atrioventricular valve. However, this decrease does not improve the degree of AVVR in the absence of concomitant valve repair. Concomitant valve repair is justified in patients with moderate or worse AVVR and an abnormal valve structure.

A case of ventricular septal defect and mitral insufficiency after blunt trauma.

Few reports have described traumatic heart injury in children. We describe a case of acute mitral regurgitation associated with papillary muscle rupture, traumatic ventricular septal defect, and impending left ventricular free wall rupture due to blunt trauma in a 2-year-old girl. The papillary muscle was sutured to the left ventricular free wall. The septal defect and surrounding ruptured muscle were covered with a pericardial patch, and a Hemashield patch was used to close the ventriculotomy. A residual defect caused by dehiscence of the pericardial patch necessitated reoperation 10 months later. The patient is currently being observed on an outpatient basis.

Cardiopulmonary bypass through a left thoracotomy using venous drainage from the innominate vein in congenital heart surgery.

Cardiopulmonary bypass (CPB) through a left lateral thoracotomy is a useful approach for some congenital heart procedures, although vascular access for the arterial and venous cannulation can be challenging in the selective patients. Six patients underwent successful extracorporeal circulation through a left lateral thoracotomy using the innominate vein for venous drainage. No operative deaths or major complications occurred. Venous drainage solely from the innominate vein was adequate to establish partial bypass without the need for pericardiotomy. Total bypass was established with combined venous drainage from the innominate vein and the main pulmonary artery. Exposure of the systemic atrioventricular valve was excellent through a left thoracotomy. Venous drainage from the innominate vein without using atrial drainage can safely be used for extracorporeal circulation through a left lateral thoracotomy without compromising the procedure and it is a useful approach to congenital heart surgery in selected patients.

Unilateral pulmonary artery banding to promote contralateral pulmonary artery growth.

A full-term baby with double-outlet right ventricle and total anomalous pulmonary venous connection (TAPVC) complicated with left main pulmonary artery (PA) stenosis, presented with heart failure caused by increased pulmonary blood flow. Based on significant discrepancies in size and development between the left and right PAs, we performed right PA banding concomitant with TAPVC repair to promote left PA growth and restrict overall PA flow. PA-graphy performed 3 months after surgery showed marked increase in the left PA size with appropriately low pressure, which enabled us to successfully complete Glenn anastomosis. Under appropriate patient selection, unilateral PA banding for patients with unbalanced peripheral PA size could serve as an effective and less invasive strategy to simultaneously promote PA growth and control PA flow.

Coronary re-implantation after completion of neo-aortic reconstruction in arterial switch operation: accurate intraoperative assessment for the optimal re-implantation site.

The arterial switch operation (ASO) has evolved into the treatment of the choice for most forms of transposition of the great arteries (TGA). Despite advancement in the technical aspects of the procedure, certain anatomical variations of the coronary arteries are still considered as surgical risks. We have recently employed a novel technique for coronary artery reconstruction in ASO to achieve further improvement of coronary transfer in cases with complex coronary anatomy. The technical key of the procedure is that reconstruction of the coronary arteries is preceded by neo-aortic anastomosis. After neo-aortic reconstruction is accomplished, the neo-aorta is temporarily distended with removal of the cross-clamp. The distended neo-aorta informs us its postsurgical geometry, which facilitates accurate assessment for the optimal site of coronary button transfer. The technique was feasible in 13 of 15 children who were consecutively treated by our group between 2003 a nd 2008. All patients recovered uneventfully and no coronary perfusion issue has occurred during the follow-up period. However, the complex anatomy of the coronary arteries in two children was not amenable to this technique. One with double loops (1RL; 2Cx) accompanied by side-by-side relationship of the great arteries underwent the open trapdoor technique, while the other with intramural coronary artery underwent the Imai method, that is one of procedure in which the coronary arteries are left in situ. The coronary re-implantation after neo-aortic reconstruction is promising to minimize postsurgical coronary ischemia and suitable for most ASO cases. However, various modifications of coronary transfer are required in a few variations of the coronary anatomy and we have to pursue further technical refinement of coronary artery transfer in ASO.

Bridge to transplantation with a Toyobo-NCVC left ventricular assist device in a 3-year-old girl.

In Japan, no pulsatile ventricular assist devices are available specifically for use in children. Pumps designed for adults are thus often used in children. We report herein a case of end-stage heart failure in a 3-year old girl (height 100.4 cm; body weight 16.2 kg; body surface area 0.66 m2) who underwent implantation with an adult-sized Toyobo-NCVC left ventricular assist device (Toyobo-National Cardiovascular Center, Osaka, Japan) in our unit. We started with the driving mode to "full-fill, full-empty" mode. The problem was difficult-to-treat hypertension due to excessive stroke volume induced by the left ventricular assist device. Aggressive administration of antihypertensive therapy was needed. Successful heart transplantation was performed in Germany 5 months after beginning support with the Toyobo-NCVC left ventricular assist device.

Successful use of the Toyobo left ventricular assist device in a 16-kg girl awaiting cardiac transplantation.

In Japan, no mechanical circulatory support is available for children. We report a case of terminal stage cardiac failure in a 16-kg girl who was implanted with an adult-sized Toyobo-NCVC left ventricular assist device (Toyobo-National Cardiovascular Center, Osaka, Japan) in our unit. She successfully underwent heart transplantation in the United States 5 months later.

Importance of luxury flow for critically ill patients receiving a left ventricular assist system.

The presence of a significant organ dysfunction does not immediately exclude patients from consideration for treatment with a left ventricular assist system (LVAS). However, in treating morbid circulatory shock patients with multiple organ failure, it is important to know the preoperative and postoperative factor or factors related to the recovery of the damaged organ function. In this study, we retrospectively analyzed patients receiving a LVAS at our institution and tried to determine the important factors related to the survival of patients with multisystem failure. Twenty-seven patients who underwent LVAS placement at Saitama Medical School Hospital between 1993 and 2003 were included in this study. The preoperative risk factors analyzed were renal dysfunction, respiratory dysfunction, hepatic dysfunction, the existence of active infection, and the combination of all four factors. As a postoperative factor, the pump flow index (mean LVAS pump flow during the first 2 weeks after LVAS surgery divided by the body surface area) was analyzed. None of the analyzed preoperative factors could predict survival after LVAS surgery, but a pump flow index of less than 2.5 l/min/m2 had a significant relationship with death after LVAS surgery. Further analysis revealed that all the patients with a pump flow index of 3.0 l/min/m2 or more could overcome preoperative organ dysfunction. Congestive heart failure patients with multisystem failure need luxury pump flow for successful LVAS surgery; this factor could be especially important in device selection and postoperative management.

[Successful salvage of a patient with cardiac arrest caused by multiple pulmonary emboli and lung abscesses due to infective endocarditis of the tricuspid valve: a case report].

A 28-year-old female developed infective endocarditis in the tricuspid valve and multiple lung abscesses due to septic pulmonary emboli early after intensive therapy for ulcerative colitis. The pathogen was methicillin-resistant Staphylococcus aureus. Usual antibiotic agents and linezolid were administered. Three weeks later, she fell into cardiopulmonary arrest due to further pulmonary emboli and required mechanical circulatory assist. Fatal brain damage was suggested at first. Two days later, she fully regained consciousness and underwent tricuspid valve replacement using mechanical valve and extirpation of septic pulmonary embolus. Mechanical circulatory assist was discontinued on the next day. After strenuous administration of linezolid and other drugs for 9 weeks, she was discharged from hospital on foot. We believe that early surgical intervention should be considered in patients with infective endocarditis in the right heart and subsequent septic pulmonary emboli. Linezolid was very useful in this patient.

[Active infective endocarditis remaining latent for six weeks after discontinuation of antibiotic therapy: a case report].

A 75-year-old man was treated for 4 weeks with penicillin administration for infective endocarditis in the mitral valve caused by Enterococcus faecalis. The infection recurred, so he received penicillin administration for a further 6 weeks. He remained afebrile and all laboratory examinations were within normal limits for 6 weeks after the antibiotic treatment was discontinued, but the vegetation remained large and highly mobile. Since the onset, possible embolic episodes had occurred three times. He underwent mitral valve repair with annuloplasty. Although the infection appeared to have healed by antibiotic therapy, resected tissue was strongly positive for Enterococcus faecalis. This case suggests that surgery should be aggressively considered if the vegetation does not shrink markedly.

Lethal thrombus in the carotid artery during operation for acute aortic dissection with cerebral malperfusion.

A drowsy patient with acute type A aortic dissection and cerebral malperfusion required emergency operation. Because the right carotid artery was totally obstructed, cerebral perfusion was first restored by cannulating it and the left femoral artery before midline sternotomy. However, a long fresh thrombus was found flowing backward from the obstructed carotid artery. This thrombus was removed, and both arteries were connected through a Y-shaped extracorporeal circulation circuit to reperfuse the brain. During the subsequent aortic procedure, both arteries were used for arterial inflow. Such thrombi can cause grave postoperative neurologic dysfunction. Carotid artery cannulation is mandatory in such cases.