Bilateral Cardiac Sympathetic Denervation for Treatment-Resistant Ventricular Arrhythmias in Heart Failure Patients with a Reduced Ejection Fraction.

The sympathetic nervous system plays an important role in life-threatening ventricular arrhythmias (VAs). Bilateral cardiac sympathetic denervation (BCSD) is performed for refractory VAs. We sought to assess our institutional experience with BCSD in managing treatment-resistant monomorphic ventricular tachycardia (MMVT) in heart failure patients with a reduced ejection fraction (HFrEF).Four patients with HFrEF (EF 30.0 ± 8.2%, New York Heart Association [NYHA] class IV 1) underwent BCSD for MMVT (VT storm 3, repetitive VT requiring implantable cardioverter defibrillator [ICD] therapy 1) refractory to antiarrhythmic drugs, catheter ablation and ICD therapy. BCSD was effective for suppressing VT in 3 patients for whom deep sedation was effective for suppressing VT. One patient remained alive after 14 months of follow-up without episodes of VT. One patient died of acute myocardial infarction before discharge and 1 patient died from unknown cause at 3 days post-discharge. In contrast, BCSD was completely ineffective for suppressing VT in a patient with NYHA class IV for whom deep sedation and stellate ganglion block were ineffective. This patient died on the 10th post-CSD day, despite left ventricular assist device implantation. In all cases, BCSD was successfully performed without procedure-related complications.Despite the limited number of cases, our results showed that BCSD in patients with HFrEF suppressed refractory MMVT in acute-phase except for a patient with NYHA class IV; however, the prognoses were not good. BCSD may be a treatment option at an earlier stage of NYHA and a bridge to orthotopic heart transplantation, even if BCSD is effective for suppressing VAs.

Multiple arrhythmic and cardiomyopathic phenotypes associated with an SCN5A A735E mutation.

BACKGROUND: SCN5A mutations are associated with multiple arrhythmic and cardiomyopathic phenotypes including Brugada syndrome (BrS), sinus node dysfunction (SND), atrioventricular block, supraventricular tachyarrhythmias (SVTs), long QT syndrome (LQTS), dilated cardiomyopathy and left ventricular noncompaction. Several single SCN5A mutations have been associated with overlap of some of these phenotypes, but never with overlap of all the phenotypes. OBJECTIVE: We encountered two pedigrees with multiple arrhythmic phenotypes with or without cardiomyopathic phenotypes, and sought to identify a responsible mutation and reveal its functional abnormalities. METHODS: Target panel sequencing of 72 genes, including inherited arrhythmia syndromes- and cardiomyopathies-related genes, was employed in two probands. Cascade screening was performed by Saner sequencing. Wild-type or identified mutant SCN5A were expressed in tsA201 cells, and whole-cell sodium currents (INa) were recorded using patch-clamp techniques. RESULTS: We identified an SCN5A A735E mutation in these probands, but did not identify any other mutations. All eight mutation carriers exhibited at least one of the arrhythmic phenotypes. Two patients exhibited multiple arrhythmic phenotypes: one (15-year-old girl) exhibited BrS, SND, and exercise and epinephrine-induced QT prolongation, the other (4-year-old boy) exhibited BrS, SND, and SVTs. Another one (30-year-old male) exhibited all arrhythmic and cardiomyopathic phenotypes, except for LQTS. One male suddenly died at age 22. Functional analysis revealed that the mutant did not produce functional INa. CONCLUSIONS: A non-functional SCN5A A735E mutation could be associated with multiple arrhythmic and cardiomyopathic phenotypes, although there remains a possibility that other unidentified factors may be involved in the phenotypic variability of the mutation carriers.

The effect of bilateral cardiac sympathetic denervation for refractory ventricular tachycardia in ischemic cardiomyopathy.

Recent studies have shown that cardiac sympathetic denervation (CSD) is effective in the treatment of refractory ventricular tachyarrhythmia in patients with structural heart disease. This case report aimed to highlight the effect of bilateral CSD in suppressing treatment-resistant ventricular tachycardia in patients with ischemic cardiomyopathy.

Deep Vein Thrombus Occurring Immediately After Blunt Abdominal Trauma.

CASE: A 13-year-old adolescent girl with blunt abdominal injury was transferred to our hospital. Enhanced computed tomography (CT) showed not only retroperitoneal hematoma around the inferior vena cava and left common iliac vein but also thrombus extending from the left common iliac vein to the femoral vein. OUTCOME: Enhanced CT performed on the second day revealed no increase in retroperitoneal hematoma and a new small thrombus in the popliteal vein. Anticoagulant therapy was therefore started with administration of unfractionated heparin. Administration of warfarin was started on the 12th day and heparin administration was stopped on the 14th day. The patient was discharged on the 19th day with continuation of warfarin administration. Enhanced CT performed 10 months after injury showed no thrombus, and the administration of warfarin was then stopped. CONCLUSIONS: She was successfully treated with the appropriate start time and control of anticoagulation therapy based on careful evaluation of her general condition.

Remarkable regression of massive deep vein thrombosis in response to intensive oral rivaroxaban treatment.

Deep vein thrombosis (DVT) is a common disease and is associated with pulmonary embolism (PE). Proximal iliofemoral DVT may lead to severe PE and chronic venous insufficiency. The standard therapy for DVT is anticoagulant therapy using heparin and a vitamin K antagonist, but a recent clinical study showed that rivaroxaban, an oral Xa inhibitor, was comparable to standard therapy and had less bleeding complications. Intensive high-dose anticoagulation is recommended during the initial 3 weeks of DVT treatment. The present report describes a case of a 77-year-old male showing a remarkable regression of DVT in response to rivaroxaban treatment within the initial 3 weeks of therapy and who did not experience any adverse events. His DVT was massive and was accompanied by proximal iliofemoral vein thrombus and iliac vein compression syndrome. Rivaroxaban, especially in intensive high-dose treatment, might be a safe and effective therapeutic choice for massive DVT.

The time-adjusted gradual replacement injection method enables better visualization of the right heart.

BACKGROUND: Despite the improvement of cardiac CT, right heart visualization remains challenging. OBJECTIVE: We herein describe a new method, called the time-adjusted gradual replacement injection protocol. The aim of this study was to compare this protocol with the split-bolus injection protocol. METHODS: Fifty-two patients who had undergone dual-source cardiac CT were retrospectively recruited. Twenty-six patients were injected by using the split-bolus injection protocol, and 26 patients were injected by using the time-adjusted gradual replacement injection protocol. For this method, we injected contrast medium for 10 seconds at a flow rate of 0.07 × body weight mL/s, then gradually replaced the contrast material with saline until 2 seconds before finishing the scans. The CT attenuation was measured in 4 chambers, the aorta, and the coronary arteries. The visualization of the anatomic structures and the occurrence and severity of streak artifacts were scored for the cardiac structures in the heart. For the analyses, either Welch t-test or Student t-test was performed. RESULTS: In the right heart, the CT values and visualization scores were significantly higher in the time-adjusted replacement injection group than in the split-bolus injection group, whereas the artifact scores were comparable between the 2 groups. The CT values, visualization scores, and artifact scores of the left heart were not significantly different between the 2 groups. CONCLUSIONS: In this study, the time-adjusted gradual replacement injection protocol provided excellent attenuation for visualization of the right heart. This method may help to accurately evaluate the right cardiac anatomy and thereby identify any potential diseases.

Novel KCNQ1 splicing mutation in patients with forme fruste LQT1 aggravated by hypokalemia.

BACKGROUND: Several KCNQ1 splicing mutations have been identified in patients with type-1 long QT syndrome (LQT1). It was suggested that the clinical severity may differ according to the aberrant splicing products. There may be precipitating factors that cause cardiac events in those with a mild clinical phenotype (forme fruste LQT1). METHODS AND RESULTS: We analyzed the KCNQ1, KCNH2, SCN5A, KCNE1, and KCNE2 genes in 31 consecutive LQTS patients. A novel KCNQ1 1251+1G>A (IVS9+1G>A) mutation was identified in three probands and their two relatives. The QT interval in all of the five individuals with mutation was not much prolonged in the absence of precipitating factors (mean QTc was 461±30ms.). Two of the five individuals with mutation were symptomatic. One patient (a 38-year-old female) had experienced recurrent episodes of syncope due to ventricular tachyarrhythmias (VTAs) accompanied by QT prolongation (QTc: 750ms) when the serum potassium concentration ([K(+)]) was 2.7mEq/L. After correction of [K(+)], the QTc interval was shortened to 515ms, and the occurrence of VTAs ceased. Another patient (a 22-year-old female) was resuscitated from cardio-pulmonary arrest due to VTAs. Just after resuscitation, the QTc interval was 629ms, and [K(+)] was 2.9mEq/L. After correction of [K(+)], the QTc interval was dramatically shortened to 440ms. In order to identify abnormal splicing products of the responsible mutation, we analyzed the reverse transcription-polymerase chain reaction products from peripheral bloods of the mutation carrier, and identified exon 9-skipping (Δ9) and cryptic sequential exons 8 and 9-skipping (Δ8-9) products, as well as a no exon-skipping product. CONCLUSIONS: We identified a novel KCNQ splicing mutation 1251+1G>A in forme fruste LQT1, which induces cryptic splicing. Two of the five individuals with mutation experienced VTAs in the setting of hypokalemia, emphasizing the need to increase awareness of the significance of hypokalemia in this subgroup of LQT1 patients.

Intravascular Ultrasound Analysis of Correlation between Plaque-Morphology and Risk Factors in Peripheral Arterial Disease.

OBJECTIVE: To analyze relationships between plaque-morphology classified by intravascular ultrasound (IVUS) and risk factors in patients with peripheral arterial disease (PAD). METHODS: We performed IVUS in 203 patients with PAD. Multiple regression and logistic analysis were used to assess relationships between plaque-morphology (degree of calcification, presence of a lipid core, intimal flap and thrombus) and risk factors including diabetes mellitus, hypertension, dyslipidemia, estimated glomerular filtration rate (eGFR), HbA1c and the homeostasis model assessment-insulin resistance ratio (HOMA-IR). RESULTS: IVUS data led to 22% of lesions being classified as soft, 18% as fibrous, 32% as calcified, and 28% as mixed. Calcification was present in the superficial and deep layers in 65% and 35% of cases, respectively, and a lipid core, intimal flap and thrombus were found in 31%, 5.4% and 3.0%, respectively. The calcified angle correlated with HbA1c and eGFR (p < 0.05). Associations were found between deep calcification and HOMA-IR (odds ratio: 4.4, p < 0.05) and a lipid core and hypercholesterolemia (odds ratio: 3.2, p < 0.05). The odds ratio for intimal flap was 15.6 times with hypercholesterolemia (p < 0.05) and 16.9 times with a high HOMA-IR (p < 0.01). CONCLUSION: Plaque calcification and morphology are associated with chronic kidney disease, insulin resistance and dyslipidemia in PAD patients.