Aortic Stenosis in a Patient With Sjogren's Syndrome.

Sjogren's syndrome (SS) is an autoimmune disease characterized by dryness of the mouth and the eyes. Systemic involvement in SS is well known, however, obvious cardiac manifestations, particularly significant valve disorders, are extremely rare and only three cases of significant valve disease associated with SS that required surgical intervention have been previously described. We report a case of aortic stenosis (AS) associated with SS in an elderly patient. The diagnosis of primary SS had been made based on clinical features, positive ocular signs, and positive serologic findings. Echocardiography showed severe calcification, elevated mean pressure gradient (57 mmHg), and a small orifice area (0.45 cm(2)) of the aortic valve. At surgery, severe calcification of the aortic cusps and the annulus was the mechanism of AS, and the aortic valve was replaced with a bioprosthetic valve. Valve pathology showed nodular calcification and hyaline degeneration, but lymphocyte infiltration was not evident. The etiologic relation of SS to the valve lesions is not clear pathologically in this case, however, chronic inflammation related to immunologic reactions in SS could have some effect on exacerbation for degeneration of the valve tissue.

Structural valve deterioration in a starr-edwards mitral caged-disk valve prosthesis.

BACKGROUND: The durability of the Starr-Edwards (SE) mitral caged-disk valve, model 6520, is not clearly known, and structural valve deterioration in the SE disk valve is very rare. METHODS AND RESULTS: Replacement of the SE mitral disk valve was performed in 7 patients 23-40 years after implantation. Macroscopic examination of the removed disk valves showed no structural abnormalities in 3 patients, in whom the disk valves were removed at <26 years after implantation. Localized disk wear was found at the sites where the disk abutted the struts of the cage, in disk valves excised >36 years after implantation in 4 patients. Disk fracture, a longitudinal split in the disk along its circumference at the site of incorporation of the titanium ring, was detected in the valves removed 36 and 40 years after implantation, respectively, and many cracks were also observed on the outflow aspect of the disk removed 40 years after implantation. CONCLUSIONS: Disk fracture and localized disk wear were found in the SE mitral disk valves implanted >36 years previously. The present results suggest that SE mitral caged-disk valves implanted >20 years previously should be carefully followed up, and that those implanted >30 years previously should be electively replaced with modern prosthetic valves

A case report of atrial fibrillation in a patient with heparin resistance associated with an antithrombin III deficiency successfully treated by radiofrequency catheter ablation using a direct thrombin inhibitor.

BACKGROUND: Pulmonary vein antrum isolation has proven to be a useful strategy for radiofrequency catheter ablation (RFCA) of atrial fibrillation (AF) worldwide. Anticoagulation therapies are necessary to avoid thromboembolic events before, during, and after RFCA of AF. During the RFCA procedure for AF, it is recommended that the activated coagulation time be maintained between 300 s and 400 s using heparin as an anticoagulation therapy. CASE SUMMARY: An 81-year-old man with symptomatic and drug-refractory paroxysmal AF underwent RFCA. We found that he had a severe heparin resistance during the RFCA procedure, and heparin had little effect on him. Thus, a direct thrombin inhibitor, Argatroban Hydrate®, was used instead of heparin for anticoagulation therapy during the procedure. Finally, the AF was successfully treated by RFCA without any complications. With a post-procedural inspection, we found that he had a Type-1 antithrombin III (AT-III) deficiency. DISCUSSION: Atrial fibrillation is the most common clinical arrhythmia and is associated with significant clinical morbidity and increased mortality. An AT-III deficiency is a well-known autosomal dominant hereditary disease and congenital blood coagulation abnormality occurring in about 1:500-5000 live births that may sometimes cause thrombophilia. Thus, the physicians may occasionally come across patients with an AT-III deficiency in real-world clinical practice, even though they have no history of thrombophilia. Argatroban Hydrate® may be effective and feasible for anticoagulation therapy during the RFCA procedure of AF in patients with heparin resistance such as in this present case.

Radiofrequency catheter ablation of premature ventricular contractions from near the His-bundle.

OBJECTIVE: This study aimed to investigate the effects of radiofrequency catheter ablation (RFCA) and clinical and electrophysiological characteristics in symptomatic patients with premature ventricular contractions (PVCs) from near the His-bundle (His-PVCs). METHODS: The patient characteristics, prevalence of complications with any life style related disease (ALSRD) including hypertension, dyslipidemia, or diabetes mellitus, and/or cardiovascular disease (CVD) including coronary artery disease, cerebrovascular disease, renal dysfunction, or cardiomyopathy, clinical status, frequency of PVCs evaluated by 24hour Holter monitoring, echocardiography including the left ventricular diastolic dysfunction (LVDD) parameters, and electrophysiological findings were evaluated in 14 consecutive symptomatic patients with His-PVCs. RESULTS: The prevalence of males, being elderly and/or slightly obese, current and/or history of smoking, ALSRD or CVD related complications, and LVDD probably resulting from ALSRD and/or CVD complications were higher in patients with His-PVCs. RFCA of His-PVCs steadily decreased the PVC frequency and improved the systolic function, LV dilation, and clinical status, but not the LVDD. There was a significant relationship between the accordance rate of the QRS polarity between sinus rhythm and His-PVCs and the distance between the successful ablation site and His-bundle. CONCLUSION: The analysis of the QRS duration and accordance rate of the QRS polarity between sinus rhythm and His-PVCs before the RFCA may help to determine the distance between the origin of the PVCs and His-bundle. Further, the appropriate ablation catheter may be selected during the RFCA procedure. Finally, RFCA may be one of the most effective, feasible, and safest therapies for symptomatic patients with His-PVCs.

Paroxysmal Atrial Fibrillation in Patients Successfully Treated by Radiofrequency Catheter Ablation with Severely Compression, Lateral Displacement, and Clockwise Rotation of Their Hearts due to Severe Pectus Excavatum.

Two cases with severe pectus excavatum and symptomatic atrial fibrillation (AF) underwent radiofrequency catheter ablation (RFCA). Their chest X-ray and computed tomography (CT) findings revealed lateral displacement and clockwise rotation of their hearts, and severe right atrial and mild right ventricular compression against the sternum, but no left atrium compression against the spinal column. The procedure was therefore carefully performed under guidance with CT, intra-cardiac echography, atriography, and a three-dimensions mapping system. Finally, the AF was successfully treated by RFCA without any complications. These findings underscore the importance of understanding cases of abnormal anatomy and carefully designing a strategy before performing any procedure.

A case of paroxysmal atrial fibrillation in a patient successfully treated by radiofrequency catheter ablation with a severely right-sided dislocation of the heart after a total right lung excision.

A 72-year-old woman with symptomatic and drug-refractory paroxysmal atrial fibrillation (AF) underwent radiofrequency catheter ablation (RFCA). She had a history of a total right lung excision. Her chest X-ray and computed tomography (CT) revealed a severely sight-sided dislocation of the heart. Thus, the procedure was carefully performed under guidance of a CT, intracardiac echogram, atriography, and 3D mapping system. Finally, the AF was successfully treated by RFCA without any complications.

Inappropriate Sinus Tachycardia Diagnosed and Treated as Depression Successfully Treated by Radiofrequency Catheter Ablation.

We experienced a man in his 20s with inappropriate sinus tachycardia (IST) initially diagnosed and treated as depression who was steadily treated with radiofrequency catheter ablation (RFCA) using an EnSite™ system. The patient has remained well without any symptoms or medications, including antidepressants, for two years since the RFCA. To avoid missing IST and treating it as an emotional problem and/or mental illness such as depression, physicians - including cardiologists - should be aware of these conditions when examining patients with multiple and incapacitating complaints including palpitations and general fatigue and/or tachycardia, especially characterized by an elevated resting heart rate or a disproportionate increase in the heart rate with minimal exertion.

A case of premature ventricular complexes/ventricular tachycardia from the left ventricular outflow tract successfully ablated from the distal great cardiac vein.

We experienced a 41-year-old male with premature ventricular complexes/ventricular tachycardia from the left coronary cusp and distal great cardiac vein of the left ventricular outflow tract successfully treated by radiofrequency catheter ablation utilizing a 3D mapping system (EnSiteNavX/Velocity™ Cardiac Mapping System, St. Jude Medical, St. Paul, MN, USA) without any complications. .

Clinical benefits of deep sedation with a supraglottic airway while monitoring the bispectral index during catheter ablation of atrial fibrillation.

BACKGROUND: Pulmonary vein antrum isolation (PVAI) under sedation has proven to be a useful strategy for catheter ablation of atrial fibrillation (AF). METHODS: To evaluate the clinical benefits of respiratory management using supraglottic airways (SGAs) under deep sedation while monitoring the bispectral (BIS) index during the PVAI and the durations from admission to the catheterization room to starting the radiofrequency energy delivery (Time α), and from starting the radiofrequency energy delivery to completion of the PVAI (Time ß), X-ray time, frequency of dislocations of the three-dimensional maps (D3DM), procedure-related complications, and proportion of an AF-free rate 15 months after the PVAI (PAFFR) in patients who received deep sedation without SGAs (Group A: n=48) and those with SGAs (Group B: n=51) were evaluated. RESULTS: There were no significant differences in patient characteristics, Time α (77±3 versus 78±2 min; p=0.816), complications of cardiac tamponade (2% versus 2%; p=0.966), or PAFFR (81% versus 88%; p=0.313) between the two groups. However, the Time ß (84±4 versus 67±3; p=0.001), X-ray time (53±2 versus 34±2; p<0.001), and minor complications of nasal bleeding (25% versus 0%; p=0.001) were significantly shorter and lower in Group B than in Group A, in accordance with a reduction in the hypoxia (15% versus 0%; p=0.007) and D3DM (31% versus 8%; p=0.003). CONCLUSIONS: These results may demonstrate the clinical benefits of deep sedation with SGAs while monitoring the BIS index without any hypoxia during PVAI in patients with AF.

A case of fulminant myocarditis treated with very long-term support by cardiopulmonary support system.

Fulminant myocarditis (FM) sometimes causes severe left ventricular dysfunction and lethal arrhythmias leading to cardiogenic shock and critical conditions. Thus, mechanical circulation support with intra-aortic balloon pumping and/or a cardiopulmonary support system (CPS) is sometimes needed to save lives. The special recommended therapies for FM for that classified as class I (evidence level C) in the guidelines of the Japanese Circulation Society are intra-aortic balloon pumping, CPS, percutaneous cardiac pacing, and a left ventricular assist device (LVAD), and they are well established in evidence-based medicine. We experienced a case of FM that we were able to save by long-term stable CPS support. Because, unfortunately, the LVAD was not commercially available in Japan at that time, intensive treatments including CPS were continued in our hospital. Finally, a good course of the illness was achieved without any adverse complications. Thus, these intensive treatments in the present case may be one of the optional effective strategies for FM, especially in hospitals and/or countries where the LVAD is not (commercially) available, and when an LVAD may not be suitable because of complications associated with infectious disease. .

Silent myocardial ischemia in asymptomatic patients with type 2 diabetes mellitus without previous histories of cardiovascular disease.

BACKGROUND: The number of patients with type 2 diabetes mellitus (T2DM) continues to increase all over the world. Cardiovascular disease (CVD), especially coronary artery disease (CAD), is a major cause of the morbidity and mortality in patients with T2DM. The prognosis of patients with silent myocardial ischemia (SMI) is worse than that in those without. METHODS AND RESULTS: Thus, to assess how many patients with SMI existed among those patients, CVD screening tests were performed in 128 asymptomatic patients with T2DM without previous histories of CVD. SMI could be detected in 24 patients (19%) by exercise stress tests and/or the coronary fractional flow reserve. Their 12-lead electrocardiogram and cardiac ultrasonography were both normal. Compared to those without SMI, those with had a statistically significant longer history of T2DM (17±1 versus 11±1years, p=0.006), and the co-existence of a family history of CVD (42% versus 21%, p=0.037). Furthermore, these factors were demonstrated as independent risk factors of SMI by a multivariate analysis (Odds ratio 1.060 and 4.000, respectively), and in accordance with the disease duration of T2DM, the prevalence of patients with SMI has been increasing (p=0.019). CONCLUSIONS: Physicians should be aware of these conditions when examining patients with T2DM, especially with a family history of CVD and/or long disease duration (>11years) of T2DM, even though they have no symptoms, previous histories of CVDs, and/or abnormal findings on the 12-lead electrocardiogram and cardiac ultrasonography. This may be an effective, safe, and attractive diagnostic strategy for those asymptomatic patients with T2DM.

Non-occlusive condition with the Better-Header roller pump: impacts of flow dynamics and hemolysis.

PURPOSE: Impacts on hemolysis and backflow using a non-occlusive setting with the Better-Header (BH) roller pump were investigated. METHODS AND RESULTS: Pump flow of a non-occlusion setting was measured with a pump speed of 3 L/min and 5 L/min against various after-loads. With the non-occlusive setting (BH-NO350), backflow was less than 10% if the pump pressure head was <300 mmHg. When the outlet line is occluded, 80% of the set flow was shunted through the pressure relief valve and outlet pressure did not develop hazardous overpressure. During surgery with the BH-NO350, flow loss was <5% while the pump pressure was maintained at approximately 200 mmHg. An in vitro hemolysis test was conducted at 5 L/min against 350 mmHg, using the standard occlusion (BH-SO), the non-occlusion (BH-NO350), and the centrifugal pump (CP). The CP demonstrated less hemolysis than the other two groups; the BH-SO and the BH-NO350 had similar hemolytic characteristics. During cardiopulmonary bypass, no significant differences in hemolysis were seen among the BH-SO, the BH-NO350, and the CP. CONCLUSION: Possible flow loss of the non-occlusion setting with the BH should be almost negligible in most clinical situations. The BH-NO350 demonstrated hemolytic characteristics similar to those of the BH-SO, but not as good as those of the CP.

Anomalous origin of the right coronary artery: report of a case.

A 63-year-old man was admitted with a complaint of dyspnea. Echocardiography showed severe aortic regurgitation (AR), and moderate mitral regurgitation (MR). Coronary angiography revealed that the right coronary artery (RCA) arose from the ascending aorta with a high takeoff and a significant stenosis at the distal segment of the RCA. Scintigraphy with Thallium showed a transient perfusion defect on the inferior wall. The diagnosis of AR and MR associated with anomalous origin of the RCA and myocardial ischemia was made. After successful catheter intervention for stenosis of the RCA, an operation was performed on the aortic and mitral valve. At surgery, the orifice of the RCA was located above the commissure of the right and left coronary cusps and the shape was obliquely elliptical. The RCA originated at an acute angle from the ascending aorta, and its proximal segment was incorporated in the wall of the aorta. After aortic valve replacement and mitral valve repair, a neo-ostium without unroofing of the intramural segment of the RCA was created at the proximal RCA, and the intima of the RCA was fixed to the intima of the aorta. The patient recovered uneventfully and is doing well without findings of myocardial ischemia at present 40 months after operation.

Ebstein's anomaly in adult patients over 50 years of age.

Ebstein's anomaly (EA) is a rare congenital heart disease of the tricuspid valve, and less than 5% of patients with EA survive beyond the age of 50. We report two unoperated cases of EA in adult patients aged over 50 years. Two patients, a 70-year-old Japanese woman and a 59-year-old Chinese woman, were referred to us for tachyarrhythmias. Transthoracic echocardiography demonstrated apical displacement (>8 mm/m(2) body surface area) of the septal leaflet of the tricuspid valve from the atrioventricular ring with tricuspid regurgitation in both patients. The former suddenly expired 20 months later after suffering from repetitive supraventricular tachyarrhythmias and/or heart failure, and the latter is alive with minimal signs of heart failure 12 months after the diagnosis of EA. Although the natural history of EA is extremely variable, these two patients are exceptional in that they tolerated EA well for over 50 years without any surgical intervention.