A case of torsades de pointes due to takotsubo cardiomyopathy after common atrial flutter ablation.

Takotsubo cardiomyopathy (TCM) is a transient acute cardiac disorder often associated with QT prolongation, but this rarely leads to torsades de pointes (TdP). Additionally, it is a rare complication of catheter ablation. Here we report a case of TCM that developed after catheter ablation for common atrial flutter, which led to TdP. The patient was an 85-year-old male who had persistent supraventricular tachycardia, which was considered atrial flutter. The patient was hospitalized for congestive heart failure. Although the response to diuretic administration was unfavorable, heart failure improved with the combined use of rate control by landiolol. Catheter ablation was performed because of the possibility of tachycardia-induced cardiomyopathy. Tachycardia disappeared following ablation to the cavotricuspid isthmus, but the patient complained of severe pain during the ablation. Approximately 2 h after the treatment, the patient's heart failure re-exacerbated. The next day, electrocardiogram confirmed a marked QT prolongation, and TdP occurred. Although the phenomenon we experienced is rarely reported, it should be considered a complication following catheter ablation. Adequate analgesia, care for anxiety about treatment, and evaluation of cardiac condition after treatment are considered important. .

Coronary intervention for severe stenosis in the ostial right coronary artery with cardiac angiosarcoma: a case report.

BACKGROUND: Although rare, angiosarcoma is the most common type of cardiac primary malignancy. This disease can cause life-threatening complications and the prognosis remains poor. There is no standard approach to care, and clinical judgement is exercised on a case-by-case basis. Tumour progression causes serious complications, such as heart failure and vascular disruption. CASE SUMMARY: A 64-year-old Japanese woman presenting with a right atrial tumour was referred to our department. Tumour biopsy revealed that the patient suffered from angiosarcoma. We performed a lumpectomy to excise the tumour, but due to tissue adhesions in and around the right atrium, the malignancy could not be completely removed. After 3 years of chemotherapy, the patient was admitted to our hospital with increased chest pain. Emergency coronary angiogram revealed severe stenosis of the ostial right coronary artery. Intravascular ultrasound (IVUS) and computed tomography suggested coronary compression due to cardiac angiosarcoma. In this study, we report a unique case of advanced cardiac angiosarcoma, presenting as unstable angina, which was successfully treated with percutaneous coronary intervention using stent implantation. DISCUSSION: Due to the rarity of cardiac primary angiosarcoma, many symptoms are misdiagnosed until mechanical complications arise, such as coronary compression. The clinical course and various imaging modalities are useful for differentiating angiosarcomas from coronary stenosis.

Impact of Distal Protection with Filter-Type Device on Long-term Outcome after Percutaneous Coronary Intervention for Acute Myocardial Infarction: Clinical Results with Filtrap®.

AIM: Although distal embolization during percutaneous coronary intervention (PCI) for acute myocardial infarction (AMI) deteriorates cardiac function, whether distal protection (DP) can improve prognosis is still controversial. We investigated whether a filter-type DP device, Filtrap®, could improve long-term outcomes after PCI for AMI. METHOD: We studied 164 patients (130 men, mean age: 65.7 years) who underwent PCI. Patients were divided into two groups based on the use of Filtrap®. The occurrence of congestive heart failure (CHF) and major adverse cardiac events (MACE) defined as cardiac death, recurrent AMI, and target vessel revascularization were compared. RESULT: Between DP (n=53, 41 men, mean age: 65.5 years) and non-DP (n=111, 89 men, mean age: 65.8 years) groups, although there was significantly greater plaque area in the DP group than in the non-DP group, there were no significant differences in coronary reperfusion flow after PCI. Interestingly, patients with CHF in the non-DP group exhibited a higher CK level than those in the DP group. During a 2-year follow-up period, cumulative CHF was significantly lower in the DP group than in the non-DP group (log-rank p=0.018), and there was no significant difference in the MACE rate (log-rank p=0.238). The use of DP device could not predict MACE, but could predict CHF by multivariate analysis (odds ratio=0.099, 95% CI: 0.02-0.42, p=0.005). CONCLUSION: These results demonstrate that favorable clinical outcomes could be achieved by the filter-type DP device in AMI, particularly in patients with CHF.

Impact of thin-cap fibroatheroma on predicting deteriorated coronary flow during interventional procedures in acute as well as stable coronary syndromes: insights from optical coherence tomography analysis.

The occurrence of deteriorated coronary flow associated with distal embolization during percutaneous coronary intervention results in impaired myocardial perfusion and worsens the clinical prognosis. This study aimed to examine the impact of optical coherence tomography (OCT)-determined coronary plaque morphology on the prediction of deteriorated coronary flow after stent implantation in acute as well as stable coronary syndromes (ACS and SAP, respectively). We studied 126 patients who underwent OCT during stenting for ACS (n = 44) and SAP (n = 82) with a de novo lesion. Angiographic deteriorated coronary flow was defined as the deterioration of TIMI flow grade after mechanical dilatation in the absence of a mechanical obstruction on angiograms. Patients could be divided into the deteriorated flow group (n = 21) and the reflow group (n = 105). Under these conditions, the presence of thin-cap fibroatheroma (TCFA) was more frequently observed in the deteriorated flow group than in the reflow group in both ACS and SAP. A multivariable logistic regression model revealed that TCFA was an independent predictor of deteriorated coronary flow (hazard ratio: 12.32; 95 % confidence interval: 3.02-50.31; p = 0.0005). These results demonstrate that TCFA detected by OCT could be a strong predictor of the occurrence of deteriorated coronary flow during stent implantation in ACS as well as SAP.

Comparison of sirolimus-eluting, paclitaxel-eluting, and bare-metal stents in a patient with angina pectoris: histopathological autopsy findings of the third month.

A 67-year-old man with a more than 15-year-old history of hypertension, dyslipidemia, and glucose intolerance presented at our hospital with exertional angina. Coronary angiography showed considerable stenosis of 3 vessels. A diffuse calcified lesion in the left anterior descending coronary artery was pre-treated using rotational atherectomy followed by sirolimus-eluting stent (SES) implantation. A lesion in the proximal right coronary artery was treated by bare-metal stent (BMS) implantation, and the tandem lesion in the left circumflex artery was treated using paclitaxel-eluting stent (PES) implantation. All the procedures were performed within 1 month of the initial presentation and yielded good angiographic results. 3 months after the final stenting, the patient was re-admitted because of congestive heart failure (CHF). While recovering from CHF, he suddenly developed cardiopulmonary arrest and died during hospitalization. Autopsy examination of the coronary arteries showed that both drug-eluting stents (DESs: SES and PES) and the BMS had characteristic histopathological features. Inflammatory responses in the neointima were greater in both the DESs than in the BMS. SES and PES showed different inflammatory infiltration pattern or fibrin deposition status; these histopathological differences observed in the DES environments have implication to cause adverse clinical events such as late stent thrombosis or late catch-up phenomena.

Intravascular ultrasound appearance of scattered necrotic core as an index for deterioration of coronary flow during intervention in acute coronary syndrome.

In acute coronary syndrome (ACS) patients with deterioration of coronary flow during percutaneous coronary intervention (PCI), a scattered necrotic core pattern (SNC) is observed by intravascular ultrasound virtual histology (VH-IVUS). The purpose of this study was to evaluate the impact of SNC on deterioration of coronary flow during PCI in ACS. A total of 38 ACS patients were imaged using VH-IVUS before PCI. In addition to conventional definitions of thin-cap fibroatheroma by VH-IVUS (ID-TCFA), the SNC was defined as necrotic core foci with a maximum diameter of <14 pixels on a 400 × 400 VH-IVUS image in the presence of >50% plaque burden except in the ID-TCFA frame. Patients were divided into deterioration of coronary flow group (n = 15) and normal-reflow group (n = 23). The incidence of residual thrombus and plaque rupture, the external elastic membrane, plaque and fibrous volumes, the incidence of ID-TCFA and the average number of SNC per frame was significantly greater in deterioration of coronary flow group than in normal-reflow group (all parameters P < 0.05). Multivariate analysis revealed that the average number of SNC per frame was independently associated with deterioration of coronary flow in ACS patients (odds ratio 1.18, P < 0.05). In conclusion, an increased number of SNC is associated with deterioration of coronary flow during PCI in ACS patients.

Spontaneous healing of posttraumatic focal coronary aneurysm: a case report.

We report on the spontaneous healing of a posttraumatic focal coronary aneurysm in a previously healthy 61-year-old man after his involvement in a motor vehicle accident, resulting in blunt chest trauma that injured the anterior wall of his left ventricle. Left-sided cardiac catheterization and selective coronary angiography 1 month after the accident showed an aneurysm in the proximal part of the left anterior descending artery, and normal coronary arteries otherwise. Intravascular ultrasound revealed that the lesion was a pseudoaneurysm protruding toward the myocardium. Surgical removal of the aneurysm was not considered, and the patient was discharged after 2 months of uneventful hospitalization. Follow-up coronary angiography and intravascular ultrasound at 3 months and 1 year after the accident showed a total regression of the aneurysm. The patient has remained asymptomatic, with no residual ischemia 3 years after the accident. This case indicates that careful conservative treatment is a therapeutic option for posttraumatic coronary pseudoaneurysms.

A KCR1 variant implicated in susceptibility to the long QT syndrome.

The acquired long QT syndrome (aLQTS) is frequently associated with extrinsic and intrinsic risk factors including therapeutic agents that inadvertently inhibit the KCNH2 K(+) channel that underlies the repolarizing I(Kr) current in the heart. Previous reports demonstrated that K(+) channel regulator 1 (KCR1) diminishes KCNH2 drug sensitivity and may protect susceptible patients from developing aLQTS. Here, we describe a novel variant of KCR1 (E33D) isolated from a patient with ventricular fibrillation and significant QT prolongation. We recorded the KCNH2 current (I(KCNH2)) from CHO-K1 cells transfected with KCNH2 plus wild type (WT) or mutant KCR1 cDNA, using whole cell patch-clamp techniques and assessed the development of I(KCNH2) inhibition in response to well-characterized KCNH2 inhibitors. Unlike KCR1 WT, the E33D variant did not protect KCNH2 from the effects of class I antiarrhythmic drugs such as quinidine or class III antiarrhythmic drugs including dofetilide and sotalol. The remaining current of the KCNH2 WT+KCR1 E33D channel after 100 pulses in the presence of each drug was similar to that of KCNH2 alone. Simulated conditions of hypokalemia (1mM [K(+)](o)) produced no significant difference in the fraction of the current that was protected from dofetilide inhibition with KCR1 WT or E33D. The previously described α-glucosyltransferase activity of KCR1 was found to be compromised in KCR1 E33D in a yeast expression system. Our findings suggest that KCR1 genetic variations that diminish the ability of KCR1 to protect KCNH2 from inhibition by commonly used therapeutic agents constitute a risk factor for the aLQTS.

Transition of the ST segment from a J wave to a coved-type elevation before ventricular fibrillation induced by coronary vasospasm in the precordial leads.

We describe the case of a 63-year-old man whose electrocardiogram showed transition of the ST segment from a J wave to a coved-type elevation in precordial leads before ventricular fibrillation induced by right coronary artery vasospasm. Simultaneously, the ST segment in inferior leads was gradually depressed with a J wave. Considering the sudden death of his son, induced ventricular fibrillation by programmed electrical stimulation, and modulations of the ST segment in the precordial and inferior leads by pilsicainide, some abnormalities in repolarization associated with Brugada syndrome or early repolarization syndrome might have caused these atypical ST-segment manifestations.

Complete atrioventricular block associated with takotsubo cardiomyopathy.

An 82-year-old woman was admitted to the hospital due to repeated episodes of syncope with incontinence. Electrocardiography showed torsades de pointes, complete atrioventricular (AV) block, T-wave inversions and a prolonged QTc interval. Urgent coronary angiography showed no significant coronary stenosis and left ventriculography demonstrated typical abnormal wall motion of takotusbo cardiomyopathy. Electrophysiology study suggested that the damaged structure might be the bundle of His. After temporary transvenous pacing and administration of intravenous lidocaine, no recurrence of torsade de pointes was found. Symptoms of worsening heart failure were not found. Although abnormal left ventricular wall motion improved, a complete AV block remained and the patient needed pacemaker implantation on Day 18 after admission. This case demonstrated that complete AV block associated with takotsubo cardiomyopathy may persist after improvement of left ventricular wall motion, and implantation of a pacemaker may be needed.

Combination therapy with oral sildenafil and beraprost for pulmonary arterial hypertension associated with CREST syndrome.

Pulmonary arterial hypertension (PAH) is commonly associated with CREST (Calcinosis, Raynaud phenomenon, Esophageal motility disorders, Sclerodactyly, and Telangiectasia) syndrome. Sildenafil, an oral phosphodiesterase type-5 inhibitor, may offer benefits in the pharmacological management of PAH. However, little is known about the long-term hemodynamic effects of sildenafil, and the potential role of sildenafil in long-term combination with beraprost, an oral prostacyclin analogue, remains unclear. We therefore examined the hemodynamic effect of oral sildenafil alone and when coadministered with beraprost in a patient with PAH associated with CREST syndrome. Traces of the acute hemodynamic effects of beraprost (20 microg) disappeared after 2 hours. In contrast, the acute hemodynamic effects of sildenafil (50 mg) produced a greater reduction in PAP (31%) and PVR (40%), and these effects also disappeared after 5 hours. After 1 month of combination therapy of sildenafil (25 mg) twice daily and beraprost (20 microg) 3 times daily, the fall in pulmonary artery pressure and pulmonary vascular resistance was sustained (31% in both). Furthermore, the patient had significantly improved her 3-minute walk test and NYHA function class without significant adverse effects at the reported doses. The findings indicate that oral sildenafil is a potent pulmonary vasodilator that appears to act synergistically with oral beraprost to cause sustained pulmonary vasodilatation in a patient with PAH associated with CREST syndrome.

Gene mutations in adult Japanese patients with dilated cardiomyopathy.

BACKGROUND: Some patients with dilated cardiomyopathy (DCM) have mutations of the genes that encode sarcomeric or cytoskeletal proteins of cardiomyocytes, but the prevalence of these mutations in Japan remains unclear. METHODS AND RESULTS: A group of 99 unrelated adult patients with DCM (familial n=27, sporadic n=72) were screened for the following genes: cardiac beta-myosin heavy chain, cardiac myosin-binding protein C (MYBPC3), regulatory and essential myosin light chains, alpha cardiac actin, alpha tropomyosin, cardiac troponin T, cardiac troponin I, cardiac troponin C, dystrophin, and lamin A/C. A mutation (R820Q) in MYBPC3 was found in an aged patient. In addition, dystrophin mutations were identified in 3 male patients (2 with exon 45-48 deletion and 1 with exon 48-52 deletion). The prevalence of dystrophin mutations in male patients with DCM was 4.4% (3 of 68). No mutations involving amino acid changes were identified in the other genes. CONCLUSIONS: Although cases of adult patients with DCM caused by mutations of the genes encoding sarcomeric or cytoskeletal proteins of cardiomyocytes are infrequent in Japan, it may be advisable to screen older DCM patients for MYBPC3 mutations, and male patients with familial DCM for dystrophin mutations.

A novel mutation Lys273Glu in the cardiac troponin T gene shows high degree of penetrance and transition from hypertrophic to dilated cardiomyopathy.

Familial hypertrophic cardiomyopathy (HC) can be caused by mutations in 9 different genes encoding sarcomere proteins expressed in cardiac muscle. To date, only 13 different mutations in the cardiac troponin T (cTnT) gene have been reported to cause HC. Clinical characteristics and prognosis associated with mutations of this gene have not been well characterized owing to the small size and composition of affected families. The aim of this study was to determine the characteristic phenotype of patients with HC caused by a novel cTnT gene mutation, Lys273Glu. Two hundred Japanese probands with HC were screened for mutations in the cTnT gene. The Lys273Glu missense mutation was present in 9 persons from 2 unrelated pedigrees. They exhibited different cardiac morphologies: 1 had a dilated cardiomyopathy-like feature, 7 had left ventricular hypertrophy with normal left ventricular systolic function, and the 6 of them had asymmetric septal hypertrophy. A 1-year-old boy was not evaluated with echocardiography. The mean maximum wall thickness was 18.0 +/- 5.5 mm (range 8 to 24). There were 7 histories of sudden death in 1 of the 2 families. The Lys273Glu substitution in the cTnT gene shows a high degree of penetrance (100% in persons aged >20 years), a high incidence of sudden death, and a partial transition from hypertrophic to dilated cardiomyopathy. Because the location of a mutation appears to influence the development of a phenotype, we suggest that the precise definition of the disease-causing mutation can provide important prognostic information about affected members.