The Relationship Between Gastric Myoelectric Activity and SCN5A Mutation Suggesting Sodium Channelopathy in Patients With Brugada Syndrome and Functional Dyspepsia: A Pilot Study

BACKGROUND/AIMS: SCN5A encodes the cardiac-specific NaV1.5 sodium channel, and Brugada syndrome is a cardiac conduction disorder associated with sodium channel alpha-subunit (SCN5A) mutation. The SCN5A-encoded NaV1.5 channel is also found on gastrointestinal smooth muscle and interstitial cells of Cajal. We investigated the relationship between functional dyspepsia (FD) and SCN5A mutation to evaluate sodium channelopathy in FD. METHODS: Patients with Brugada syndrome or FD were examined using upper endoscopy, electrogastrography (EGG), FD symptom questionnaire based on Rome III criteria and genetic testing for SCN5A mutation. Symptom scores of FD and EGG findings were analyzed according to SCN5A mutation. RESULTS: A total of 17 patients (4 Brugada syndrome and 13 FD) participated in the study. An SCN5A mutation was noted in 75.0% of the patients with Brugada syndrome and in 1 (7.7%) of the patients with FD. Of 4 patients with SCN5A mutation, 2 (50%) had FD. Postprandial tachygastria and bradygastria were noted in 2 (50%) and 1 (25%) of the patients with SCN5A mutation, respectively. The EGG findings were not significantly different between positive and negative mutation in 17 patients. CONCLUSIONS: Although we did not find statistically significant results, we suggest that it is meaningful to attempt to identify differences in symptoms and gastric myoelectric activity according to the presence of an SCN5A mutation by EGG analysis. The relationship between FD and sodium channelopathy should be elucidated in the future by a large-scale study.

The Effects of Obstacles on the Dynamics of Ventricular Fibrillation

BACKGROUND AND OBJECTIVES: The effects of artificial obstacles on the dynamics of ventricular fibrillation have been extensively investigated with an electrical mapping system. This study was performed to assess the influence of transmural obstacles on the dynamics of wavefronts, and determine whether these can convert ventricular fibrillation to ventricular tachycardia by stabilizing the wavefronts in the fibrillating right ventricular tissues of pigs, using an optical mapping system. MATERIALS AND METHODS: The right ventricles of pigs (n=15) were excised and placed in a tissue perfusion system, with the epicardium facing up. Holes, with increasing sizes, from 2 to 8 mm in diameter, were created using a skin biopsy punch. Another 8 mm sized hole was then made adjacent to the first, and the changes in the wavefront dynamics and cycle length of the optical action potential waves investigated. RESULTS: In 14 of the 20 obstacles, in ten tissues, transient attachment of electrical activities along the rim of obstacles and transient rotation of the wavefronts were observed. During baseline ventricular fibrillation, the fibrillation cycle length was 118.5+/-24.7 msec, which was increased to 135.4+/-30.2 msec after creation of the first hole, and to 159.4+/-47.7 msec after the second (p=0.01). There was a positive correlation between the obstacle size and cycle length (r=0.43, p=0.007). In three tissues, conversion to ventricular tachycardia from ventricular fibrillation was observed after creation of the two holes. CONCLUSION: Obstacles of an appropriate size had anti-fibrillatory effects in tissues with ventricular fibrillation, which was partly explained by the temporary attachment of wavefronts to the obstacles.

A Case of Antiphospholipid Antibody Syndrome Accompanied by Valvular Heart Disease and Moya Moya Syndrome

A case of antiphospholipid antibody syndrome, accompanied by valvular heart disease and Moya moya syndrome, has never been reported. Here, we report on a case that had mitral regurgitation and Moya moya syndrome, associated with antiphospholipid antibody syndrome secondary to systemic lupus erythematosus. This patient underwent a mitral valve replacement for mitral valve regurgitation. The postoperative course was uneventful, and the pathological findings of the mitral valve showed a degenerative change, due to chronic inflammation, a proliferative fibrous change and calcification, but without thrombus formation. However, the patient returned to the hospital with a cerebral hemorrhage, which was caused by Moya moya syndrome. Surgical drainage was performed, and the patient was discharged without any complications. The patient is on anticoagulation and immunosuppression drugs, with no problems to date.

B-Type Natriuretic Peptide Predicts Clinical Presentations and Ventricular Overloading in Patients with Heart Failure

Brain natriuretic peptide (BNP), a neurohormone secreted from the ventricular myocardium in response to hemodynamic load/wall stress, in congestive heart failure (CHF). This study was performed to evaluate the correlation between BNP level and clinical presentations and hemodynamic parameters obtained by echo-Doppler (echo-Doppler) analysis, and its relation with disease severity and ventricular load/wall stress. CHF patients (n=246) were subgrouped by clinical presentations and echo-Doppler findings into 4 groups: diastolic HF only, chronic HF, acute HF, and chronic HF with acute exacerbation. A BNP level of 81.2 pg/ml showed a sensitivity/ specificity of 53.3%/98.4% for detecting CHF (AUC, 0.882; p< 0.0001), and was found to be closely related with the NYHA classification (p< 0.0001). Log BNP was related with LVEF (r2=0.3015, p< 0.0001) and the Meridional wall stress index (r2=0.4052, p< 0.0001). The difference between the BNP levels of the subgroups and BNP control was significant (p< 0.0001), exept between the HF group and the controls; control (n=114, 20.9 +/- 31.4pg/ml), only diastolic HF (n=84, 89.8 +/- 117.6pg/ml), chronic HF (n=60, 208.2 +/- 210.2pg/ml), acute HF (n=28, 477.9 +/- 498.4 pg/ml), chronic HF with acute exacerbation (n= 74, 754.1 +/- 419.2pg/ml). The BNP level was significantly higher in the only diastolic HF group than in the asymptomatic control group with diastolic dysfunction (89.8 +/- 12.8 vs. 22.8 +/- 5.1pg/ml, p< 0.0001). BNP may be a good indicator for the differential diagnosis of a broad spectrum of heart failures. And, elevated BNP might help to diagnose diastolic HF in patients with diastolic dysfunction.

Favorable Outcome of Endovascular Stent-Graft Implantation for Stanford Type B Aortic Dissection

BACKGROUND AND OBJECTIVES: To evaluate the feasibility and the short- and mid-term follow-up outcomes of endovascular stent-graft implantation in patients with Stanford type B aortic dissection. SUBJECTS AND METHODS: Twenty-eight patients with Stanford type B aortic dissection were evaluated. An aortogram was performed immediately after the procedure and a follow-up computed tomography (CT) scan was performed within one week, between 3 and 6 months, and annually thereafter. Clinical status was also evaluated at the same time. RESULTS: Endovascular stent-graft implantation at the target site was successful in 27 patients (96.4%). There were primary endoleaks in 6 patients and one case of procedure failure owing to migration of the stent-graft; and no procedure-related mortality. The number of patients with early complications requiring treatment was 2 (2/27, 4%). Fourteen patients experienced postimplantation syndrome (14/27, 52%). The average follow-up period was 22.1+/-17.5 months. Complete resolution or thrombosis of the false lumen was achieved in 14 patients and partial thrombosis was achieved in 10 patients. Operative treatments were required in three patients due to a progressing dissection or new dissection. There were no deaths and no instances of aneurysm or aortic rupture during the follow-up period. CONCLUSION: Endovascular stent-graft implantation for Stanford type B aortic dissection is a feasible, safe, and effective treatment modality. All patients who underwent surgery had a persisting leak. Therefore, regular evaluation of the aortic dissection and management of endoleaks were crucial for a favorable outcome in endovascular stent-graft implantation for a Stanford type B aortic dissection.