Multimodality imaging for pre-clinical assessment of Fabry's cardiomyopathy.

Anderson Fabry's disease (AFD) is a rare but underdiagnosed intracellular lipid disorder which can cause left ventricular hypertrophy (LVH). Pre-clinical diagnosis of Fabry's disease is important as it permits early stratification for enzyme replacement therapy, improving the patient's long-term prognosis, avoiding progression to irreversible fibrosis, and preventing cardiovascular complications. Combinations of imaging modalities that integrate the strengths of each modality and at the same time eliminate weaknesses of an individual modality can offer improved diagnostics, therapeutic monitoring, and pre-clinical assessment of Fabry's disease.

Electrical plasticity and cardioprotection in myocardial ischemia--role of selective sodium channel blockers.

The concept of electrical protection of the ischemic myocardium is in constant evolution and has recently been supported by experimental and clinical studies. Historically, antiplatelet agents, angiotensin-converting enzyme inhibitors, β-blockers, and statins have been all proposed as drugs conferring anti-ischemic cardioprotection. This was supported by the evidence consistently indicating that all these drugs were capable of reducing mortality and the risk of repeat myocardial infarction.

Segmental diastolic compression of circumflex coronary artery secondary to pericardial constriction: an uncommon cause of angina pectoris.

Diastolic segmental compression of a native coronary artery is an uncommon cause of chest pain. Here we describe a 24-year-old woman with constrictive pericarditis who had retrosternal chest pain, progressive dyspnea, tachycardia, and bilateral leg edema. She was diagnosed with compression of the first and second obtuse marginal branches of left circumflex coronary artery secondary to constrictive pericarditis.

Acute spinal cord ischemia during aortography treated with intravenous thrombolytic therapy.

Acute anterior spinal cord ischemia is a rare but disastrous complication of endovascular aortic procedures. Although intravenous thrombolysis with recombinant tissue plasminogen activator is an effective treatment for acute brain ischemia, its use for the treatment of spinal cord ischemia has not previously been reported. We report the case of a patient who developed anterior spinal cord ischemia during diagnostic aortography He was treated with intravenous recombinant tissue plasminogen activator within 3 hours after the onset of symptoms.

Aneurysm sac re-expansion after thrombolysis.

Purpose: To present a case of delayed abdominal aortic aneurysm (AAA) re-expansion after thrombolysis for endograft limb occlusion.

Case Report: A 68-year-old man underwent AAA exclusion with an AneuRx stent-graft in 1999. Five years later, he developed right limb thrombosis of the endograft.

Carotid stenting.

From my point of view, carotid artery stenting, in 2005, is clearly here to stay. "Houston, the Eagle has landed." The rest is up to you and me as we encourage our surgical colleagues and our various medical Societies to embrace this safe, effective, and durable technology and make it available to the patients who will really benefit from it.

Left ventricular unloading with an assist device results in receptor relocalization as well as increased beta-adrenergic receptor numbers: are these changes indications for outcome?

Background: The use of left ventricular (LV) assist devices (LVADs) can improve performance and recovery of failing human hearts.

Aim: Following our alpha-adrenergic receptor work, we hypothesized that mechanical unloading in patients with low output syndrome and LV failure would yield similar results with beta-adrenergic receptors ((beta)AR), that being increased numbers and intra-myocytic relocalization.

Methods: (beta)AR density and localization were investigated by fluorescence deconvolution microscopy and compared at LVAD insertion and removal in 13 heart failure patients, the patients therefore acting as their own control.

West Nile virus infection after cardiac transplantation.

West Nile virus is a mosquito-borne RNA Flavivirus infection transmitted to humans and other vertebrates, mainly by the Culex species of mosquito. Since the mid-1990s, the frequency and apparent clinical severity of West Nile virus outbreaks have increased. We report the case of a patient who developed West Nile virus encephalitis shortly after undergoing cardiac transplantation.

Acquired commissural fusion of aortic valves in patients with left ventricular assist devices.

Background: Left ventricular assist devices (LVADs) currently are used as bridges for patients who deteriorate clinically while awaiting cardiac transplantation. At the time of transplantation or subsequent death, we can assess the effect of long-term device implantation on cardiac structural changes. We have noted, in our years of experience, that aortic valve commissures may fuse in patients supported by LVADs.

Intravenous milrinone in treatment of advanced congestive heart failure.

Phosphodiesterase inhibitors such as milrinone can relieve symptoms and improve hemodynamics in patients with advanced congestive heart failure. We retrospectively evaluated the hemodynamic and clinical outcomes of long-term combination therapy with intravenous milrinone and oral beta-blockers in 65 patients with severe congestive heart failure (New York Heart Association class IV function and ejection fraction <25%) refractory to oral medical therapy. Fifty-one patients successfully began beta-blocker therapy while on intravenous milrinone.

Implantation of the Jarvik 2000 left ventricular assist device without the use of cardiopulmonary bypass.

In this report, we describe successful implantation of a Jarvik 2000 left ventricular assist device (Jarvik Heart, Inc, New York, NY) without the use of cardiopulmonary bypass in a patient who was a member of the Jehovah's Witness faith. To accomplish this, we had to change our implantation technique. The modified technique, which minimizes the risk of bleeding and end-organ dysfunction, can also be used to decrease cardiopulmonary bypass time.

Inappropriate shock delivery and biventricular pacing cardiac defibrillators.

In the United States, physicians adapt currently available defibrillators to accommodate leads for biventricular pacing in those congestive heart failure patients who might benefit from cardiac resynchronization and who are additionally at risk for sudden cardiac death. The adaptation of the lead system of available defibrillators to also allow them to function as biventricular pacemakers presents occasions in which inappropriate shocks are delivered due to double counting of the right and left ventricular depolarizations by the implantable cardiac defibrillator. We reviewed a series of inappropriate shock deliveries that occurred after the implantation of biventricular pacing cardiac defibrillators at our institution; all of these shocks were related to ventricular double counting.

Coronary artery bypass surgery versus coronary stenting: risk-adjusted survival rates in 5,619 patients.

We used the Texas Heart Institute Cardiovascular Research Database to retrospectively identify patients who had undergone their 1st revascularization procedure with coronary artery bypass surgery (CABG; n=2,826) or coronary stenting (n=2,793) between January 1995 and December 1999. Patients were classified into 8 anatomic groups according to the number of diseased vessels and presence or absence of proximal left anterior descending coronary artery disease. Mortality rates were adjusted with proportional hazards methods to correct for baseline differences in severity of disease and comorbidity.

Pseudo-false-positive exercise treadmill testing caused by systolic anterior motion of the anterior mitral valve leaflet.

Subendocardial ischemia as indicated by electrocardiography during exercise, in association with severe systolic anterior motion of the anterior mitral valve leaflet without left ventricular hypertrophy, has not been well described. We report the case of a 42-year-old man who presented with symptoms of exertional angina and 2-mm ST depression on treadmill electrocardiography but had a normal perfusion scan and coronary angiogram. Initially the negative angiographic results caused us to regard the treadmill results as false-positive.

Unstable angina and non-ST-segment elevation myocardial infarction: perspectives on combination therapy.

We reviewed recent clinical data on the management of unstable angina and non-ST segment elevation myocardial infarction. We concentrated on the use of new therapies, particularly in combination with both older agents and other new methods, in order to present health care providers with an overview of available treatment options. The clinical trials reviewed herein provide strong evidence and proof of principle that combination therapies targeting 1) platelet function (aspirin, thienopyridines, and GP IIb/IIIa antagonists), 2) the coagulation cascade (unfractionated heparin and low-molecular-weight heparin), and 3) the physical characteristics of the active lesion (percutaneous intervention) reduce the risk of death or ischemic complications after thrombotic progression of coronary atherosclerosis.

Gender and cardiac arrhythmias.

The incidence of certain clinical arrhythmias varies between and women. Clinical and experimental observations suggest the existence of true differences in electrophysiologic properties between the sexes. We review these differences, possible mechanisms, clinical implications, and therapeutic considerations in the treatment of various arrhythmias in women.

Cutaneous T-cell lymphoma in a cardiac transplant recipient.

Mycosis fungoides, an uncommon form of cutaneous T-cell lymphoma, arises in the skin and frequently progresses to generalized lymphadenopathy Although the cause of cutaneous T-cell lymphoma is unknown, chronic immunosuppression may play a role. A few cases have been reported in renal transplant recipients; however, ours appears to be the 1st report of cutaneous T-cell lymphoma in a cardiac transplant recipient. In our patient, cutaneous manifestations of the disease were noted less than 1 year after transplantation.

Carotid stenting for post-endarterectomy restenosis and radiation-induced occlusive disease.

Surgical treatment of carotid restenosis and radiation-induced occlusive disease is challenging because of the high morbidity and mortality associated with this procedure. Carotid stenting has been proposed as an alternative approach. We report a series of 8 patients who were treated via the percutaneous approach for either carotid restenosis (n = 4) or radiation-induced occlusive disease (n = 4).

Closure of large percutaneous access sites using the Prostar XL Percutaneous Vascular Surgery device.

Purpose: To report early experience using a vascular closure device following endovascular aortic aneurysmal repair in which large-bore sheaths are used.

Technique: A 10F Prostar XL Percutaneous Vascular Surgery device is used to deploy sutures around sheath entry sites up to 16F. At the completion of the procedure, the sutures are tied with a sliding knot to ensure adequate hemostasis.

Echocardiographic diagnosis of left ventricular outflow tract obstruction caused by an acquired subaortic membrane after mitral valve replacement.

Although an acquired subaortic membrane has been reported as a cause of left ventricular outflow tract (LVOT) obstruction in various clinical settings, it previously has not been reported after mitral valve surgery. We describe 3 cases of acquired LVOT obstruction that resulted from development of a subaortic membrane after mitral valve replacement. This report emphasizes the role of an acquired subaortic membrane in LVOT obstruction after mitral valve replacement, the use of echocardiography in diagnosing this condition, and the importance of early intervention.