Two Cases of Aortic Root Replacement After Fontan Completion.

Aortic root dilatation is a well-known complication in patients with congenital aortic valve malformation, tetralogy of Fallot, or a double outlet right ventricle. We report two rare patients who underwent composite graft replacement of the aortic root with a mechanical valve, the so-called Bentall-type operation, after Fontan completion. The pathological examination on the resected aortic wall revealed mucoid degeneration in tunica media and elastic fiber fragmentation. Our report emphasizes the need for close observation of these patients over a long-term period.

[Aortitis Syndrome Requiring Redo Bentall Procedure with Coronary Artery Bypass Grafting due to Graft Detachment;Report of a Case].

A 53-year-old woman was referred to us for reoperation of aortic root. Twenty-three years ago, she was diagnosed with aortitis and suffered from annuloaortic ectasia, aortic regurgitation, severe stenosis of left carotid and subclavian arteries and occulusion of the main trunk of left coronary artery. The left coronary system was perfused by collateral arteries from right coronary artery. She had undergone original Bentall procedure( Cooley graft 26 mm+SJM valve 25 mm) with coronary artery bypass grafting (CABG)[saphenous vein graft (SVG)-left anterior descending artery (LAD)]. When she was 52 years old, she suffered from angina on efforts. Computed tomography and catheter angiocardiogram revealed pseudoaneurysm formation due to detachment of right coronary artery button and occulusion of SVG. We underwent redo aortic root replacement (Hemashield graft 26 mm+On-X valve 23 mm) with redo CABG (SVG-LAD). Eight mm graft was interposed between composite valve graft and right coronary button. Postoperative course was uneventful. She was discharged from the hospital on the 17th day, and is now doing well without any symptoms.

Root aneurysm following aortic valve replacement 20 years after an arterial switch operation.

Late development of annuloaortic ectasia (AAE) and progression of aortic regurgitation (AR) are widely recognized outcomes following an arterial switch operation (ASO). We treated a 29-year-old male with AAE and rapid aortic root expansion, who underwent ASO as a neonate and aortic valve replacement (AVR) as an adult. He was diagnosed as having dextro-(D-loop) transposition of the great arteries after birth and underwent ASO at the age of 13 months. At the age of 19 years, AVR was performed for progressive AR. AAE developed after AVR. In patients who have undergone neonatal ASO, AAE may occur following AVR decades later.

[Migration of a Temporary Epicardial Pacing Lead into the Vascular Graft].

A 48-year-old man was refered to our hospital for the treatment of bicusp aortic valve and severe aortic regurgitation. He underwent Bentall procedure (SJM 25mm+HemashieldØ 28 mm composite graft). Temporary epicardial pacing lead( TPL) was placed on the right ventricle at the opetation, and was cut on his skin surface prior to discharge because of difficulty in traction removal. After 15 years from operation, follow up computered tomography and echocardiography showed migration of 2 retaining TPLs extending from vascular graft of ascending aorta to the right innominate artery. We performed Redo-median sternotomy and removed the TPLs. This is the 1st reported case of a migrated temporary pacing lead into the vascular graft of ascending aorta under noninfectious conditions.

Open-heart surgery using a centrifugal pump: a case of hereditary spherocytosis.

BACKGROUND: Hereditary spherocytosis is a genetic, frequently familial hemolytic blood disease characterized by varying degrees of hemolytic anemia, splenomegaly, and jaundice. There are few reports on adult open-heart surgery for patients with hereditary spherocytosis. CASE PRESENTATION: We report a rare case of an adult open-heart surgery associated with hereditary spherocytosis. A 63-year-old man was admitted for congestive heart failure due to bicuspid aortic valve, aortic valve regurgitation, and sinus of subaortic aneurysm. The family history, the microscopic findings of the blood smear, and the characteristic osmotic fragility confirmed the diagnosis of hereditary spherocytosis. Furthermore, splenectomy had not been undertaken preoperatively. The patient underwent a successful operation by means of a centrifugal pump. Haptoglobin was used during the cardiopulmonary bypass, and a biological valve was selected to prevent hemolysis. No significant hemolysis occurred intraoperatively or postoperatively. CONCLUSION: There are no previous reports of patients with hereditary spherocytosis, and bicuspid aortic valve. We have successfully performed an adult open-heart surgery using a centrifugal pump in an adult patient suffering from hereditary spherocytosis and bicuspid aortic valve.

Bileaflet mechanical valve replacement: an assessment of outcomes with 30 years of follow-up.

OBJECTIVES: Heart valve replacement with a bileaflet mechanical valve is a well-established procedure. However, the long-term results of valve replacement using the bileaflet mechanical valve remain unclear, especially for follow-up periods over 30 years. Additionally, it is important to identify predictors of long-term mortality and valve-related events. METHODS: We performed a retrospective cohort analysis of 2727 patients (mean ± standard deviation age, 52.8 ± 1.6 years) who underwent valve replacement with a St. Jude Medical valve at our institute from 1978 to 2012. Data were collected using a questionnaire and chart review or physician contact. The cohort included 950 aortic valve replacements (AVRs), 1255 mitral valve replacements (MVRs) and 522 double valve replacements (DVRs). Follow-up was 91% complete, and the analysis included a total of 39 187 patient-years. RESULTS: Operative mortality rates were 2.3% for AVR, 2.2% for MVR and 3.6% for DVR. The 30-year survival rate (actuarial method) was 38.0% (AVR, 44.5%; MVR, 34.9%; and DVR, 37.5%). The 30-year rates of freedom from valve-related mortality, thromboembolic events and bleeding events were 86.3% (AVR, 88.6%; MVR, 85.4%; and DVR, 84.3%), 83.5% (AVR, 89.8%; MVR, 80.0%; and DVR, 81.4%) and 91.5% (AVR, 94.4%; MVR, 90.1%; and DVR, 90.2%), respectively. The incidence rates of valve-related morbidity, thromboembolic events and bleeding events were significantly higher among patients with MVR and DVR than among those with AVR. Significant risk factors for late death and other late events included male sex, age >65 years and atrial fibrillation. CONCLUSIONS: Low late mortality and a low incidence of valve-related events can be achieved for at least 30 years using mechanical bileaflet valve replacement. Persistent atrial fibrillation is a significant risk factor for morbidity and mortality.

The impact of preoperative identification of the Adamkiewicz artery on descending and thoracoabdominal aortic repair.

OBJECTIVE: To investigate the impact of preoperative identification of the Adamkiewicz artery (AKA) on prevention of spinal cord injury (SCI) through the multicenter Japanese Study of Spinal Cord Protection in Descending and Thoracoabdominal Aortic Repair (JASPAR) registry. METHODS: Between January 2000 and October 2011, 2435 descending/thoracoabdominal aortic repairs were performed, including 1998 elective repairs and 437 urgent repairs, in 14 major centers in Japan. The mean patient age was 67 ± 13 years, and 74.2% were males. There were 1471 open repairs (ORs), including 748 descending and 137 thoracoabdominal extent [Ex] I, 136 Ex II, 194 Ex III, 115 Ex IV, and 138 Ex V, and 964 endovascular repairs (EVRs). Of the 2435 patients, 1252 (51%) underwent preoperative magnetic resonance or computed tomography angiography to identify the AKA. RESULTS: The AKA was identified in 1096 of the 1252 patients who underwent preoperative imaging (87.6%). Hospital mortality was 9.2% (n = 136) in those who underwent OR and 6.4% (n = 62) in those who underwent EVR. The incidence of SCI was 7.3% in the OR group (descending, 4.2%; Ex I, 9.4%; Ex II, 14.0%; Ex III, 14.4%; Ex IV, 4.2 %; Ex V, 7.2%) and 2.9% in the EVR group. The risk factors for SCI in ORs were advanced age, extended repair, emergency, and occluded bilateral hypogastric arteries. In ORs of the aortic segment involving the AKA, having no AKA reconstruction was a significant risk factor for SCI (odds ratio, 2.79, 95% confidence interval, 1.14-6.79; P = .024). CONCLUSIONS: In descending/thoracoabdominal aortic repairs, preoperative AKA identification with its adequate reconstruction or preservation, especially, in ORs of aortic pathologies involving the AKA, would be a useful adjunct for more secure spinal cord protection.

Surgery for aortic regurgitation and aortic root dilatation in Takayasu arteritis.

BACKGROUND: Special consideration may be required for surgical treatment in Takayasu arteritis because inflammation may cause serious complications such as valve detachment or dilatation of the residual aorta. We evaluated our surgical outcome of treatment for aortic regurgitation and aortic root dilatation in Takayasu arteritis. METHODS: Between December 1983 and January 2013, 22 cardiac operations were performed in 20 patients with aortic regurgitation due to Takayasu arteritis. Aortic valve replacement was carried out in 6 patients, and aortic root replacement in 16. Of these 16 patients, composite graft replacement was undertaken in 6, composite graft replacement plus coronary artery bypass grafting in 2, composite graft replacement plus total or partial arch replacement in 7, and valve-sparing aortic root replacement with hemiarch replacement in one. RESULTS: The operative mortality (within 30 days) was 4.5% and the 5-year survival rate was 90.9%. Early surgical reintervention was not required in any patient. Neither valve detachment nor composite graft detachment was noted. Two patients required redo aortic root replacement due to pseudoaneurysm formation and severe aortic regurgitation during follow-up. Late dilatation of the residual thoracoabdominal or abdominal aorta was observed in 2 patients, and both were treated surgically. CONCLUSIONS: The early surgical outcome was acceptable but surgical reintervention was required because of late dilatation of the residual aorta or recurrent aortic regurgitation due to annular dilatation, and longstanding careful follow-up will be needed.

Surgical strategy for Kommerell's diverticulum: total arch replacement.

OBJECTIVE: Kommerell's diverticulum is a rare congenital aortic arch anomaly. Various surgical techniques have been reported; however, the surgical strategy is still controversial. In our institute, total arch replacement (TAR) and anatomic reconstruction of the subclavian artery (SCA) has been selected for the treatment of Kommerell's diverticulum to release the vascular ring completely and prevent postoperative complications, including dissection, rupture, hand ischemia, and subclavian steal syndrome. METHODS: From 2000 to 2012, 4 patients (aged 38-72 years) underwent TAR and anatomic reconstruction of the SCA for Kommerell's diverticulum. All patients had a right aortic arch with an aberrant left SCA. The indications for surgery were dysphagia and dilatation of Kommerell's diverticulum. TAR, using hypothermic cardiopulmonary bypass and circulatory arrest, was performed through a median sternotomy and right anterolateral thoracotomy. After resection of Kommerell's diverticulum, the SCA was reconstructed with a graft. RESULTS: No hospital deaths or major complications occurred, but a 72-year-old patient required prolonged hospitalization for respiratory failure. All patients were discharged from the hospital and were free of symptoms, rupture, dissection, hand ischemia, and subclavian steal syndrome at 5 months to 11 years postoperatively. CONCLUSIONS: TAR is a reasonable surgical technique for Kommerell's diverticulum, because it enables the vascular ring to be completely released, preventing recurrence, rupture, and dissection. Anatomic reconstruction of the SCA was effective to prevent hand ischemia and subclavian steal syndrome.

Transatrial repair of submitral left ventricular pseudoaneurysm.

In a 57-year-old man with Wolff-Parkinson-White syndrome, a left ventricular pseudoaneurysm developed, induced by the pop phenomenon, after radiofrequency catheter ablation of the posterolateral accessory pathway. The pseudoaneurysm was positioned at the atrioventricular groove with moderate pericardial adhesions, and the rupture site was located just below the P3 annulus of the posterior mitral leaflet. With this anatomy and in the presence of adhesions, we repaired the rupture site from within the heart through an incision made in the posterior mitral leaflet. This transmitral approach might be a reasonable surgical option for treating this type of submitral left ventricular pseudoaneurysm.

Rupture of thoracoabdominal aortic aneurysm associated with Behcet's disease.

Surgical treatment of arterial Behcet's disease (BD) has a higher incidence of graft-related complications such as anastomotic pseudoaneurysm or graft occlusion. A 64-year-old man presented with a rupture of the thoracoabdominal aortic aneurysms associated with BD. Evaluation shows a large hematoma in the retroperitoneum and multiple aneurysms of the thoracoabdominal aorta. Physical examination and past history fulfills the diagnostic criteria of BD. The abdominal aorta was replaced with an allograft and the major branches were reconstructed with its branches. The postoperative course was uneventful. A 10-month follow-up computed tomographic scan did not show any graft-related complications. This case suggests the usefulness of an allograft for arterial involvement of BD.

Delayed enhancement on computed tomography in abdominal aortic aneurysm wall.

The purpose of this study was to evaluate delayed enhancement (DE) of the aortic wall of atherosclerotic aneurysms using computed tomography and to evaluate the relationships between DE and wall thickness of abdominal aortic aneurysm (AAA), diameter of AAA, serum levels of C-reactive protein (CRP) which indicate inflammation status, and pathological findings. Computed tomographic images of atherosclerotic AAA in 110 patients were studied between July 2001 and March 2003. Computed tomography (CT) scanning included unenhanced, enhanced early, and enhanced delayed phases. Pathological findings were obtained from 19 of the 110 patients. We determined DE of the AAA wall and assessed the association between DE and AAA wall thickness, AAA diameter, serum levels of CRP, and pathological findings. Delayed enhancement on CT was demonstrated in 66 of 110 patients with atherosclerotic AAA (60.0%). Patients with DE demonstrated significantly larger AAA diameter (4.8 +/- 0.9 versus 3.9 +/- 0.6 cm, P < 0.0001) and significantly higher levels of CRP (5.0 +/- 6.0 versus 2.3 +/- 2.9 mg/l, P = 0.033) than those patients without DE. Patients with DE also had significantly thicker and more severe atheroma and a tendency toward more prominent inflammation and vascularity in pathologic findings. There was no significant difference in wall thickness between AAA with and without DE (1.44 +/- 0.7 versus 1.24 +/- 0.22 mm, P = 0.352). Delayed enhancement on CT demonstrated in over half of atherosclerotic AAA may be associated with chronic inflammation by atherosclerosis.

Long-term results of modified Bentall procedure using flanged composite aortic prosthesis and separately interposed coronary graft technique.

We have selected the flanged composite aortic prosthesis and separately interposed coronary graft technique for the aortic root replacement over seven years. We sought to evaluate the long-term results of aortic root replacement with this technique. Between April 1996 and September 2003, 71 patients (mean age 46.1+/-12.9 years, 67.6% males) underwent aortic root replacement with this technique. Sixty-two patients had annuloaortic ectasia, and seven patients acute type A aortic dissection. Marfan syndrome was recognized in 35 patients. Two separate 8-10 mm knitted Dacron grafts were interposed between a valved composite graft and both coronary ostia to avoid kinking of coronary arteries. The early mortality rate was 4.2%. The actuarial survival rate was 93.9+/-3.0% at 5 years. The freedom from operation related complications was 86.7+/-4.1% at 5 years. No patients had anticoagulant-related hemorrhage, valve thrombosis, reoperation, graft thrombosis, or coronary pseudoaneurysm. The separately interposed coronary graft and the flanged composite graft technique is predictable and safe. Coronary pseudoaneurysm and graft thrombosis have been eliminated.

Characteristics of hypertrophic obstructive cardiomyopathy refractory to medical treatment and selection of surgical methods.

BACKGROUND AND AIM OF THE STUDY: Treatment for hypertrophic obstructive cardiomyopathy (HOCM) has been reported; however, there has been no report on the characteristics of medication-responsive and -refractory hypertrophic obstructive cardiomyopathy (HOCM). Using the classification of systolic anterior movement (SAM) which has been previously reported, we tried to identify the characteristics and use them to treat HOCM appropriately. METHODS: The clinical, echocardiographic, catheterization, and surgical data of 29 hospitalized patients with HOCM during 1980 to 1999 were analyzed retrospectively. We classified SAM in all patients by echocardiography. Nineteen patients improved with medical treatment (medical group), and 10 patients underwent surgical treatment because of ineffectiveness of medication (surgical group). We studied the relation between types of SAM and medical/surgical groups, and examined the relation between types of SAM and the surgical methods. RESULTS: Type I SAM was significantly more frequent in the medical group, while type II SAM was more frequent in the surgical group (p = 0.047). Patients in the surgical group underwent mitral valve replacement (MVR), myectomy, or a combination of MVR and myectomy. Left ventricular outflow gradient (LVOG) of over 100 mmHg was recognized in almost all patients with type II SAM. CONCLUSIONS: It was suggested that patients with medication-responsive HOCM tended to have type I SAM and those with refractory HOCM tended to have type II SAM. We consider that in type I SAM, if the position of the papillary muscles changed with medication or myectomy, shift of the chordae and type I SAM were reduced or disappeared. However, in type II SAM, even if the position of the papillary muscles changed, SAM did not disappear because lifting of the mitral leaflets remained. It is therefore suggested that patients with type II SAM should undergo at least MVR.

Pravastatin, a 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitor, reduces delayed neuronal death following transient forebrain ischemia in the adult rat hippocampus.

Recent evidence indicates that statins have beneficial effects on the brain in the ischemic condition. However, there is a lack of studies related to the effect of statins on delayed neuronal death. We investigated the effect of prophylactic therapy with pravastatin on delayed neuronal death in the rat hippocampus. The rats were given a daily dose of 20 mg/kg of pravastatin orally for 14 days. Transient forebrain ischemia was induced by the four-vessel occlusion method. Three days after ischemia, surviving neurons of the hippocampal CA1 subfield were counted. Our results demonstrated that prophylactic statin treatment significantly reduced delayed neuronal death after transient forebrain ischemia. Our findings suggest that prophylactic statin treatment may be useful in preventing functional neurological disorders after transient cerebral ischemic insult.

Matrix metalloproteinase 2 is suppressed by trapidil, a CD40-CD40 ligand pathway inhibitor, in human abdominal aortic aneurysm wall.

BACKGROUND: The activation of inflammatory cells and the production of matrix metalloproteinases (MMPs) are important in the pathogenesis of abdominal aortic aneurysm (AAA). Previous studies have demonstrated that the antiplatelet agent trapidil has multiple actions, including suppression of MMP expression through the inhibition of the CD40-CD40 ligand (CD40-CD40L) pathway in cultured cells. A recent clinical study suggested that trapidil might have functions beyond its antiplatelet action. Methods and results In the present study, we performed immunohistochemical analysis and semiquantitative reverse transcription-polymerase chain reaction to evaluate the effect of trapidil on the production of MMPs in cultured aortic tissues from patients with infrarenal AAA (n = 9) and control patients with aortoiliac occlusive disease (n = 7). The tissue concentrations of both MMP-2 and MMP-9 were significantly higher in AAA walls than in control aortic walls. Both trapidil and an anti-CD154 (CD40L) antibody significantly suppressed the protein production and mRNA expression of MMP-2 but did not inhibit those of MMP-9 in organ cultures of AAA wall specimens. MMP-9 was produced by macrophages and a lot of neutrophils in AAA tissues, whereas MMP-2 was derived from macrophages. CD40 was expressed on macrophages but not on neutrophils, and this expression could explain the differential effect of trapidil on the production of MMP-2 and MMP-9. CONCLUSIONS: Trapidil, a CD40-CD40L pathway inhibitor, suppressed mRNA expression and protein production of MMP-2 in AAA tissues, suggesting a potential therapeutic approach for the prevention or treatment of AAA.