Adult ALCAPA: from histological picture to clinical features.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly that results in high mortality if left untreated. Our aim was to extend our knowledge of the histological, angiographic, and clinical characteristics of ALCAPA in order to deepen our understanding of this rare entity. CASE PRESENTATION: We were involved in the assessment, treatment, and pathological evaluation of two adult ALCAPA patients who were rescued from ventricular fibrillation and then surgically treated to establish a dual coronary artery system. Histological studies indicated various chronic ischemic changes in the myocardium, patchy fibrosis, and severely thickened arteriolar walls in both ventricles. The first patient is alive and well 11.5 years after surgical correction without any implantable cardioverter defibrillator (ICD) activations. The second patient required re-do surgery 9 months after the initial operation but subsequently died. Histologically, chronic ischemic alteration of the myocardium and thickened arteriolar walls persisted even after surgical correction, and coronary angiography (CAG) showed an extremely slow flow phenomenon even after surgical correction in both patients. The average postoperative opacification rate in the first case was 7.36 + 1.12 (n = 2) in the RCA, 3.81 + 0.51 (n = 3) in the left anterior descending (LAD) artery, and 4.08 + 0.27 (n = 4) in the left circumflex (LCx) artery. The slow flow phenomenon may represent persistent high arteriolar resistance in both ventricles. CONCLUSIONS: Seldom reported or new findings in adult ALCAPA were identified in two cases. More frequent diagnosis of adult ALCAPA can be expected because of the widespread availability of resuscitation and more advanced diagnostic modalities. Accumulation of pathological and clinical findings and confirmation of the long-term follow-up results after treatment may contribute to expanding our knowledge of this rare entity and establishing optimal treatment.

Patency of Saphenous Vein Grafts Using the PAS-Port System During Coronary Artery Bypass Surgery.

BACKGROUND: Several proximal anastomosis devices have been developed to shorten the time required for a proximal anastomosis and to avoid aortic cross-/side-clamping during coronary artery bypass grafting. This study retrospectively examined the patency of saphenous vein grafts (SVGs) using the PAS-Port System (Cardia Inc, Redwood City, CA). METHODS: From 2004 to 2014, 451 patients underwent coronary artery bypass graft operations requiring at least 1 proximal anastomosis using a PAS-Port device. A total of 802 PAS-Port devices were used, and 95.0% (762 of 802) were implanted successfully. Among the successfully implanted anastomoses, 76.8% (585 of 762) were evaluated using coronary angiography or multidimensional computed tomography, or both. The evaluations were performed between postoperative days 4 and 3,182 (mean, 319 ± 624 days). The early (1 to 365 days) and the midterm to long-term (more than 366 days) occlusion rates were examined. A complete postoperative clinical course was recorded for 70.7% of the patients. RESULTS: Overall, 93.8% (549 of 585) of the device-dependent SVGs were patent. The patency rates of device-dependent SVGs that were 1, 2, 3, 4, 5, 6, 7, and 8 years old were 90.1% ± 1.8%, 87.1% ± 2.3%, 86.1% ± 2.5%, 82.9% ± 3.3%, 80.6% ± 3.9%, 77.2% ± 5.0%, 77.2% ± 5.0%, and 70.2% ± 8.1%, respectively. The longest follow-up period was 3,182 days (8.7 years). The occlusion rate for device-dependent SVGs tended to decrease as the number of patients accumulated. CONCLUSIONS: The PAS-Port system provided acceptable SVG patency and clinical outcome for the early and midterm to long-term. There may be a learning curve for the use of PAS-Port device that affects the device-dependent SVG patency.

Two adult cases of Bland-White-Garland syndrome with lethal arrhythmia due to coronary steal phenomenon during physical or mental stress.

We experienced two adult cases of anomalous origin of the left coronary artery from the pulmonary artery, so-called Bland-White-Garland (BWG) syndrome, that presented with ventricular tachycardia (VT) and ventricular fibrillation during exertion in daily life. They presented to our hospital with syncope due to VT, and recovered following application of an automated external defibrillator with cardiopulmonary resuscitation. We diagnosed BWG syndrome by multi-detector computed tomography angiography and coronary angiography. We analyzed the mechanisms of lethal arrhythmias in relation to myocardial ischemia on exertion. Coronary flow modification and implantable cardioverter defibrillator implantation were performed in order to prevent future lethal arrhythmia due to myocardial ischemia. It is important to be aware of congenital heart disease in ordinary cases. .

Xenopericardial roll graft replacement for infectious pseudoaneurysms and graft infections of the aorta.

BACKGROUND: Which graft material is the optimal graft material for the treatment of infected aortic aneurysms and aortic graft infections is still a matter of controversy. Orthotopic aortic reconstruction with intraoperatively prepared xenopericardial roll grafts without omentopexy was performed as the "initial" operation to treat aortic infection or as a "rescue" operation to treat graft infection. Mid-term outcomes were evaluated. METHODS: Between 2009 and 2013, orthotopic xenopericardial roll graft replacement was performed to treat eight patients (male/female: 6/2; mean age: 69.5 [55-80] yr). Graft material: equine/bovine pericardium: 2/6; type of operation: initial 4/rescue 4; omentopexy 0. Additional operation: esophagectomy 2. Mean follow-up period: 2.6 ± 1.6 (1.1-5.1) years. RESULTS: Replacement: ascending 3, arch 1 (reconstruction of neck vessels with small xenopericardial roll grafts), descending 3, and thoracoabdominal 1. Pathogens: MRSA 2, MSSA 1, Candida 1, E. coli 1, oral bacillus 1, and culture negative 2. Postoperative local recurrence of infection: 0. Graft-related complications: stenosis 0, calcification 0, non-infectious pseudoaneurysm of anastomosis 2 (surgical repair: 1/TEVAR 1). In-hospital mortality: 2 (MOF: initial 1/rescue 1); Survival rate exclusive of in-hospital deaths (~3 y): 100 %, but one patient died of lung cancer (3.6 yr). CONCLUSIONS: Because xenopericardial roll grafts are not composed of synthetic material, the replacement procedure is simpler and less invasive than the standard procedure. Based on the favorable results obtained, this procedure may have the possibility to serve as an option for the treatment of aortic infections and aortic graft infections not only as a "rescue" treatment but as an "initial" treatment as well.

Clinical efficacy of intermittent pressure augmented-retrograde cerebral perfusion.

OBJECTIVE: During aortic surgery under hypothermic circulatory arrest, retrograde cerebral perfusion (RCP) is commonly used as a cerebroprotective method to extend the duration of circulatory arrest safely. Kitahori and colleagues described a novel protocol of RCP using intermittent pressure augmented (IPA)-RCP in 2005. The aim of the present study was to determine the clinical effectiveness of this novel protocol. METHODS: A total of 20 consecutive patients undergoing total replacement of the aortic arch were assigned to a conventional RCP (n = 10) or an IPA-RCP group (n = 10). Cerebral perfusion was provided at a continuous venous pressure of 25 mm Hg in the conventional RCP, and venous pressure was intermittently provided at 20 mm Hg for 120 seconds and at 45 mm Hg for 30 seconds in the IPA-RCP group. The clinical outcomes were compared between the 2 groups. Regional cerebral oxygen saturation (rSO(2)) was measured using near infrared spectroscopy every 10 minutes from the beginning of RCP initiation. To represent the brain oxygen consumption, the decline ratio of rSO(2) was calculated. RESULTS: There was no surgical mortality or major neurologic complications in either group. The interval from the end of surgery to full wakefulness was significantly shorter in the IPA-RCP group (85 ± 64 minutes) than in the conventional RCP group (310 ± 282 minutes; P < .05). Although the initial rSO(2) value did not show significant difference in both groups, the rSO(2) with IPA-RCP was greater than that with conventional RCP from 10 to 70 minutes (P < .05). The decline ratio of rSO(2) was lower in the IPA-RCP group than in the RCP perfusion group at all points (P < .05). CONCLUSIONS: IPA-RCP might provide more homogenous cerebral perfusion and a more effective oxygen supply to the brain with better clinical results than conventional RCP.

Equine pericardial roll graft replacement of infected pseudoaneurysm of the ascending aorta.

The standard procedure for treating infected aortic aneurysms is to resect the infected aorta, debridement of the surrounding tissue, in situ graft replacement, and omentopexy. However, the question of which graft material is optimal is still a matter of controversy. We recently treated a patient with an infected ascending aortic aneurysm. Because of previous abdominal surgery, the omentum was unavailable. The ascending aorta was replaced in situ with equine pericardial roll grafts. The patient is alive and well 29 months after the operation.

Equine pericardial roll graft replacement of infected pseudoaneurysm of the aortic arch.

Resection of the infected aorta, debridement of the surrounding tissue, in situ graft replacement, and omentopexy is the standard procedure for treating infected aortic aneurysms, but the question of which graft material is optimal is still a matter of controversy. We recently treated a patient with an infected thoracic aortic aneurysm. The aneurysm was located in the proximal aortic arch. Because the patients had previously undergone abdominal surgery, the aortic arch were replaced in situ with a branched equine pericardial roll grafts. The patient is alive and well 23 months after the operation.

Three-dimensional replica of a life-sized model of aortic arch aneurysm for preoperative assessments.

PURPOSE: In the treatment of extended distal aortic arch aneurysms with open stent grafting, detailed preoperative minute evaluation is essential to determine the optimal operative strategy and reduce the incidence of complications. We describe a three-dimensional replica of a life-sized aortic arch aneurysm model for aiding preoperative assessments. DESCRIPTION: Life-sized replicas of an aortic arch aneurysm were made for 4 patients who underwent total aortic arch replacement with open stent-grafting. These replicas were used to determine the curve of the CLATE, a newly developed open stent delivery system (Senko Medical Instrument Mfg, Co, Ltd, Tokyo, Japan), and the depth of the open stent grafting. The replica and the CLATE system were also used to obtain informed consent from the patients. EVALUATION: Surgeons could simulate the operative procedure precisely. Insertion of the graft was smooth in all patients. Use of the replica made it easier for the patients to understand the form and site of the aneurysm, as well as the operative procedure. CONCLUSIONS: The combination of the three-dimensional replica and the CLATE system may be useful for both surgeons and patients in the treatment of extended distal aortic arch aneurysms.

How to do it: proximal elephant trunk insertion technique for preventing stroke during replacement of the descending aorta through a left thoracotomy.

To reduce the risk of stroke during open surgery for the treatment of descending thoracic or thoracoabdominal aortic diseases, we attempted to insert a proximal elephant trunk to stabilize the atherosclerosis at the site of the proximal anastomosis. Although the patients had dense atherosclerotic lesions, they recovered well without neurologic complications. This technique is simple and may be effective for preventing stroke when replacing the descending thoracic or thoracoabdominal aorta through a left thoracotomy.

Ticlopidine-induced hepatotoxicity in a GSH-depleted rat model.

We investigated hepatotoxicity induced by ticlopidine (TIC) in glutathione (GSH)-depleted rats by pre-treatment of a well-known GSH synthesis inhibitor, L-buthionine-S,R-sulfoxinine (BSO). Although sole administration of either TIC or BSO showed no signs of hepatotoxicity, combined administration of TIC with BSO induced hepatotoxicity, which was characterized by centrilobular necrosis of the hepatocytes and an elevation of plasma alanine aminotransferase activity. Administration of radio-labeled TIC in combination with BSO resulted in significantly higher covalent binding to rat liver proteins than that observed after sole dosing of radio-labeled TIC. Pre-treatment of 1-aminobenzotriazole, a non-specific inhibitor of P450s, completely suppressed both hepatotoxicity and the increased hepatic covalent binding caused by TIC co-treatment with BSO. The results obtained in this animal model suggest that GSH depletion and covalent binding may be involved in hepatotoxicity induced by TIC. These observations may help to understand the risk factors and the mechanism of hepatotoxicity of TIC in humans.

Excision of right coronary artery aneurysm.

A coronary artery aneurysm (CAA), the etiology and treatment of which are still not fully understood, is uncommonly encountered. A giant CAA is extremely rare and only a limited number of cases have been reported. We report the case of a 37-year-old female with a giant right coronary artery aneurysm that was successfully treated by surgical excision.

Total aortic arch replacement under intermittent pressure-augmented retrograde cerebral perfusion.

Kitahori, Kawata, Takamoto et al. described the effectiveness of a novel protocol for retrograde cerebral perfusion that included intermittent pressure augmentation for brain protection in a canine model. Based on their report, we applied this novel technique clinically. Although the duration of circulatory arrest with retrograde cerebral perfusion was long, the patient recovered consciousness soon after the operation and had no neurological deficit. Near-infrared oximetry showed recovery of intracranial blood oxygen saturation every time the pressure was augmented.

Cardiac swinging calcified amorphous tumors in end-stage renal failure patients.

We recently encountered 2 patients with mobile cardiac calcified amorphous tumors who were successfully treated by surgery. Both patients had mitral annular calcification and were on hemodialysis. These tumors showed swinging motion on echocardiography and they grew rapidly. Intraoperatively, the tumors were found to be fragile and they easily detached from their origin. The histologic findings were thrombus with angiogenesis, fibrin, and calcium deposition. This rapid-growing mobile tumor in end-stage renal failure patients is speculated to increase the risk of embolic events and should be included as a special entity of cardiac amorphous tumors.

Clinical application of intermittent pressure-augmented retrograde cerebral perfusion.

Brain protection is important during aortic arch surgery, especially in patients with cerebral ischemia. We clinically applied the effectiveness of a novel protocol of retrograde cerebral perfusion with intermittent pressure augmentation for brain protection in a canine model, as described in a previous report. Although, in our patient the brachiocephalic artery and left subclavian artery were occluded as a result of aortitis, there was a history of right cerebral infarction, recovery of consciousness, and no neurologic sequelae. Near-infrared oximetry showed recovery of intracranial blood oxygen saturation every time the pressure was augmented.

[Acute aortic dissection with left main coronary malperfusion treated with precedent stenting followed by a definitive surgery].

Coronary malperfusion due to acute aortic dissection (AAD) is a relatively rare, but fatal condition. We experienced a case of acute myocardial infarction (AMI) with cardiogenic shock due to compression of the left main trunk (LMT) by a false lumen of an AAD. Percutaneous coronary intervention (PCI) to the LMT was firstly performed, followed by a definitive surgery under a stable hemodynamic condition. Stanford type A AAD is an indication for emergency operation and stenting to the LMT is controversial. However, it might be an essential strategy to perform precedent PCI to the LMT which quickly relieves global ischemia, and achieves hemodynamic stability for the following definitive surgery.

Metabolism of ticlopidine in rats: identification of the main biliary metabolite as a glutathione conjugate of ticlopidine S-oxide.

We have identified several novel metabolites of ticlopidine, a well known antiplatelet agent and have revealed its metabolic route in rats. The main biliary metabolite of ticlopidine was characterized as a glutathione (GSH) conjugate of ticlopidine S-oxide, in which conjugation had occurred at carbon 7a in the thienopyridine moiety. Quantitative analysis revealed that 29% of the dose was subjected to the formation of reactive intermediates followed by conjugation with GSH after oral administration of ticlopidine (22 mg/kg) to rats. In vitro incubation of ticlopidine with rat liver 9000 g supernatant fraction (S9) fractions led to the formation of multiple metabolites, including 2-oxo-ticlopidine, the precursor for the pharmacologically active ticlopidine metabolite, [1-(2-chlorobenzyl)-4-mercaptopiperidin-(3Z)-ylidene] acetic acid. A novel thiophene ring-opened metabolite with a thioketone group and a carboxylic acid moiety has also been detected after incubation of 2-oxo-ticlopidine with rat liver microsomes or upon incubation of ticlopidine with rat liver S9 fractions.

Metabolism-dependent hepatotoxicity of amodiaquine in glutathione-depleted mice.

We investigated the hepatotoxicity induced by AQ using a glutathione (GSH)-depleted mice model. Although sole administration of either AQ or L-buthionine-S,R-sulfoxinine (BSO), a well-known GSH synthesis inhibitor, produced no significant hepatotoxicity, combined administration of AQ with BSO induced hepatotoxicity characterized by centrilobular necrosis of the hepatocytes and an elevation of plasma alanine aminotransferase activity. Pretreatment of aminobenzotriazole, a nonspecific inhibitor for P450s, completely suppressed the above hepatotoxicity caused by AQ co-treatment with BSO. Administration of radiolabeled AQ in combination with BSO exhibited significantly higher covalent binding to mice liver proteins than that observed after sole dosing of radiolabeled AQ. The results obtained in this GSH-depleted animal model suggest that the reactive metabolite of AQ formed by hepatic P450 binds to liver proteins, and then finally leads to hepatotoxicity. These observations may help to understand the risk factors and the mechanism for idiosyncratic hepatotoxicity of AQ in humans.

Epicardial electrical isolation of the right atrial appendage on the beating heart with an infrared coagulator.

To determine the clinical efficacy of application of infrared energy to the beating heart as a means of creating electrical blocks, an original infrared coagulator (KIRC-119; Phomec Inc, Tokyo, Japan) was applied to the atrium of a patient with atrial fibrillation. A postoperative electrophysiologic study was performed to confirm its efficacy. The coagulator was applied epicardially to the beating heart concomitant with an on-pump beating heart coronary artery bypass graft procedure. Sinus rhythm was restored during the operation, and the electrophysiologic study revealed that a bidirectional block had been created on the right atrial appendage. The infrared coagulator may facilitate performance of the epicardial Maze procedure on the beating heart.