Cardiac amyloidosis responding to bortezomib: case report and review of literature.

We report a case of a 53-year old patient with symptoms of congestive heart failure in whom a restrictive cardiomyopathy and a kappa-chain monoclonal gammopahty were diagnosed. Treatment with eight cycles of Bortezomib, a proteasome inhibitor, resulted in a significant regression of myocardial amyloid deposition and a notable clinical and hemodynamic improvement. Over the last few years, the management of cardiac amyloidosis has taken advantage of many of the advances of the chemotherapeutic regimens, as well as the wider availability of stem cell transplantation. The management of cardiac amyloidosis is also expected to evolve and improve with the better understanding of the specific mechanisms of amyloidogenesis and myocardial deposition. This will probably make certain molecules targeting specific sites in this process, as potentially effective and minimally toxic compared therapy with the currently used ones. In this article, we describe one of the first reported cases of cardiac amyloidosis, successfully treated with Bortezomib. We describe and discuss the mechanisms of action of Bortezomib and provide a detailed review of cardiac amyloidosis, from pathophysiology to diagnosis and treatment.

ST segment elevation in lead aVR: what to expect from this orphan?

Standard 12-lead electrocardiography is used to diagnose acute myocardial infarctions in patient presenting with ST elevation. The specificity of ST segment elevation for the corresponding area is more than 90 percent. It has been suggested that ST-segment elevation in lead aVR may indicate left main disease. We will present a patient who had an ST segment elevation in this lead. We will review the current data about this finding, as well as the significance of ST segment elevation in other leads.

Atrial flutter and myotonic dystrophy in a male adolescent treated with radiofrequency catheter ablation.

A variety of cardiomyopathies are due to familial disease. Most are primarily associated with cardiac involvement and can lead to hypertrophic, dilated, or restrictive cardiomyopathy. Myotonic dystrophy (MD) is a multisystem disease with autosomal dominant inheritance and variable penetrance. Cardiac diseases are important causes of morbidity and mortality in MD patients. Patients with primary MD should be carefully investigated with an electrocardiogram, stress test, and an echocardiogram to identify preclinical cardiac involvement and to prevent life-threatening complications. Any new onset of atrial flutter or atrial fibrillation in a young patient without any underlying cardiac abnormality should be investigated for underlying myopathy. The authors report on a male adolescent with MD who presented with atrial flutter. The patient had been diagnosed with MD at birth. He had an impaired ejection fraction of 38% to 45%. The patient described sharp chest pain in the retrosternal area, with no radiation, that was induced by exercise.

Initial description of radiofrequency catheter ablation as treatment for atrial flutter in Marfan's syndrome: a case report and literature review.

Marfan's syndrome is a common connective tissue disease with different musculoskeletal, ophthalmic and cardiac manifestations. Marfan's patients carry increased risk for cardiac arrhythmias. Only three cases of atrial flutter in Marfan's patients are described in the literature. We report a fourth case of a young Marfan's patient who presents with typical atrial flutter after motor vehicle accident. After electrical cardioversion, sinus rhythm was restored but he had recurrent atrial flutter on follow up. The patient then underwent electrophysiological study and successful radiofrequency catheter ablation of the flutter circuit. Since discharge, the patient has had no documented arrhythmias on follow up.

Methadone and torsade de pointes: case report and review of the literature.

Methadone, a synthetic opioid, has been used for more than 35 years to treat heroin addiction. It is also an effective therapy for chronic pain. An increase in the mean dose used nationally to treat these patients has been reported. There is an ongoing debate over whether it is necessary to screen patients with an ECG before and during therapy with methadone. We present a patient who developed QT prolongation and sustained ventricular arrhythmias after her methadone dose was increased. Previous literature, as well as the mechanism and risk factors, will be reviewed in this article. Recommendations will be given as to when an ECG is preferably to be performed.

Tricuspid valve malfunction and ventricular pacemaker lead: case report and review of the literature.

Pacemaker implantation can be associated with several complications, including myocardial perforation with or without pericardial effusion, venous thrombosis, vegetations of the tricuspid valve (TV) or pacing lead, and tricuspid regurgitation (TR). The TR is thought to be derived from deformity or perforation of the TV by the pacing lead or secondary to atrioventricular discordance with asynchronous ventricular pacing. Severe TR can be deleterious to the patient because it raises the central venous pressure by increasing the right sided preload. Chronically, the increase in right sided blood volume can result in an increase in the right atrial pressure leading to a decrease in venous return and low cardiac output. Severe TR from leaflet adhesion to the pacemaker lead has not been reported before. With the aging of the population and the expanding use of pacemakers and implantable cardioverter defibrillators (ICD) in clinical practice, this complication may be seen more frequently. We present a patient diagnosed with severe TR, years after his pacemaker implantation. His TR was thought to be caused by adhesion of the tricuspid valve to his pacemaker lead.

Idiopathic polymorphic ventricular tachycardia with normal QT interval in a structurally normal heart.

Polymorphic ventricular tachycardia (PVT) is a life-threatening arrhythmia that is typically related to long QT syndrome, organic heart disease, electrolyte abnormalities, cardiotoxic drugs, or adrenergic stimulation. A review of the literature reveals that PVT with normal QT interval and without underlying cause is quite rare. We report a case of idiopathic spontaneous PVT with structurally normal heart and without electrolyte abnormalities, drug reactions, or evidence of catecholamine induced arrhythmia. We also review the literature on the electrocardiographic characteristics and management of idiopathic PVT.

Alveolar hemorrhage: an underdiagnosed complication of treatment with glycoprotein IIb/IIIa inhibitors.

OBJECTIVE: Alveolar hemorrhage (AH) is a rare complication of treatment with GP IIb/IIIa inhibitors. Hemoptysis, a constant sign, lacks in specificity, and may occur in confounding syndromes such as pulmonary edema, pulmonary infarction, and pneumonia. Nonspecific symptoms and signs often delay the diagnosis, thereby allowing serious or even fatal disease progression. Here, we performed a large-scale retrospective analysis to define the incidence and risk factors of AH in the setting of GP IIb/IIIa inhibitors therapy. BACKGROUND: Randomized controlled trials demonstrate that treatment with glycoprotein IIb/IIIa (GP IIb/IIIa) inhibitors may improve the outcome of acute myocardial infarction (AMI) and angioplastic procedures. However, this treatment may rarely lead to severe hemorrhagic complications, in particular AH. Unfortunately, the incidence and risk factors of AH remain poorly defined. METHODS: We reviewed for the period extending from August 1998 to January 2005 consecutive histories of AMI patients receiving coronary arteriography and treatment with either eptifibatide or abciximab. Concomitantly admitted AMI patients not treated with GP IIb/IIIa inhibitors were reviewed and served as a control group. The diagnosis of AH required the demonstration of typical symptoms and signs including dyspnea, hemoptysis, arterial hypoxemia, pulmonary radiographic changes, and, when available, bronchoscopic signs for AH. Potential covariates including pulmonary disease, pulmonary hypertension, smoking, and use of other anticoagulant or antiplatelet agents were evaluated. RESULTS: Six of 1,810 patients (0.33%) receiving eptifibatide and five of 3,648 patients (0.14%) receiving abciximab exhibited typical symptoms and signs of AH. Contrarily, only one of 4,136 patients (0.025%) receiving no GP IIb/IIIa inhibitors presented with similar symptoms and signs. There was no fatal outcome, though two patients required blood transfusions. Statistically significant differences were found between control patients and patients receiving eptifibatide alone (P = 0.004). There was also a significant difference between untreated patients and those receiving eptifibatide and abciximab (P = 0.017). No differences were found between eptifibatide and abciximab-treated patients (P = 0.19) or between abciximab and untreated control patients (P = 0.105). CONCLUSIONS: AH is a rare complication of treatment with GP IIb/IIIa inhibitors. Its incidence ranged from 0.14% in patients treated with abciximab to 0.33% in those receiving eptifibatide. Compared to a control group, patients treated with GP IIb/IIIa inhibitors had a statistically increased risk for AH.

Tako-tsubo syndrome as a consequence of transient ischemic attack.

An 82-year-old woman presented to the emergency department with chest pain after sustaining a transient ischemic attack 1 week prior to presentation. Electrocardiography revealed ST-segment elevation in leads I, II, aVF, and V3 through V6. Coronary angiography demonstrated nearly normal coronaries but left ventriculography showed apical akinesis and basal hyperkinesis. One month later her follow-up echocardiography showed no wall motion abnormalities. Several reports of tako-tsubo syndrome or transient left ventricular apical ballooning have been described, especially in Japan. We present a case with the typical features of the syndrome after a cerebrovascular accident.

Papillary fibroelastoma involving the left ventricular wall.

A 71-year-old white woman presented to her primary care physician for a routine visit and was found to have a new, previously undocumented cardiac murmur. A subsequent transthoracic echocardiogram revealed a 1 cm mobile mass arising from the lateral free wall of the left ventricle. Transesophageal echocardiography later confirmed these findings. The patient underwent a left ventriculotomy and excision of a.7 cm friable mass, which was later identified as a papillary fibroelastoma (PFE) by routine histopathologic studies. We present this unique case ith a review of the literature.