Cardiac amyloidosis: A case report and review of literature

BACKGROUND Cardiac amyloidosis,a disease caused by the precipitation of amyloid proteins in the myocardial extracellular matrix has been historically difficult to diagnose due to lack of specific clinical manifestations and necessity of biopsy to demonstrate amyloid deposition. However,advances in cardiovascular imaging techniques have facilitated earlier recognition of this disease. In addition,while once thought of as incurable,treatment strategies are emerging for cardiac amyloidosis,making early diagnosis essential.CASE SUMMARY We outline the case of a 73 years old African American female who was admitted with sudden onset shortness of breath and found to be in cardiogenic shock.Cardiac amyloidosis was suspected due to discordance between electrocardiogram and echocardiogram findings and this was subsequently confirmed with the aid of scintigraphy and an endomyocardial biopsy.CONCLUSION Our objective is to highlight the diagnostic evaluation and clinical implications of cardiac amyloidosis.

Left ventricular pseudoaneurysm: A case report and review of the literature

Left ventricular(LV) pseudoaneurysm is a rare complication that is reported in less than 0.1% of all patients with myocardial infarction. It is the result of cardiac rupture contained by the pericardium and is characterized by the absence of myocardial tissue in its wall unlike true aneurysm which involves full thickness of the cardiac wall. The clinical presentation of these patients is nonspecific, making the diagnosis challenging. Transthoracic echocardiogram and cardiac magnetic resonance imaging are the noninvasive modalities whereas coronary arteriography and left ventriculography are invasive modalities used for the diagnosis. As this condition is lethal, prompt diagnosis and timely management is vital.

Elusive left ventricular thrombus:Diagnostic role of cardiac magnetic resonance imaging-A case report and review of the literature

Identification of left ventricular mural thrombus(LVT) may be challenging depending on the imaging modality used. We present a case of LVT which was incidentally identified on cine cardiac magnetic resonance imaging(CMR). A sixty-four years old female presented with worsening dyspnea on exertion with troponin elevation. Transthoracic echocardiography(TTE) revealed a dilated left ventricle(LV) and ejection fraction(EF 30%) with thinning and akinesis of inferior/inferolateral wall was noted with basal and mid inferior wall aneurysm, and thrombus was not identified. CMR done to ascertain viability of myocardium revealed a mural thrombus within basal inferior aneurysm. This was not visualized on transthoracic echocardiography with and without use of contrast. She underwent coronary artery bypass grafting, bioprosthetic mitral valve replacement, resection and plication of posterior left ventricular aneurysm with removal of mural thrombus, and was started on anticoagulation with warfarin postoperatively for the apical thrombi. Cardiac magnetic resonance is a well suited imaging modality in detecting LVT due to its high resolution images and is more reproducible than TTE. In our patient, conventional TTE despite administration of echo-contrast agents failed to diagnose the presence of LVT in the basal inferior aneurysm as well as the apical thrombi. Delayedenhancement CMR provides the greatest sensitivity for detection of left ventricular thrombus, superiorto standard transthoracic and contrast-enhanced transthoracic echocardiography.

Coumadin ridge: An incidental finding of a left atrial pseudotumor on transthoracic echocardiography

Coumadin ridge is a normal anatomic variant that is occasionally found in the left atrium. It can present as a linear or nodular mass which can undulate with cardiac motion and if particularly prominent, can easily be mistaken for a tumor or thrombus. Careful evaluation and consideration of the common variants discussed in this review can help limit misdiagnosis, as well as unnecessary workup and treatment. We present a case of coumadin ridge that was found on a patient using two-dimensional transthoracic echocardiography.

Side matters: An intriguing case of persistent left superior vena-cava

Persistent left superior vena cava, usually an incidental finding, is the most common thoracic vein anatomical variation draining into the coronary sinus. Central venous catheter procedures may be complicated secondary to the presence of a persistent left superior vena cava, leading to life-threatening complications such as arrhythmias, cardiogenic shock, and cardiac arrest. We present a case of persistent superior vena cava diagnosed on transthoracic echocardiogram(TTE) in a patient with congestive heart failure. A dilated coronary sinus was identified on TTE, followed by injection of agitated saline into the left antecubital vein resulting in filling of the coronary sinus prior to the right atrium-an indication of persistent left superior vena-cava. This also was confirmed on cardiac computed tomography. Such a diagnosis is critical in patients who may undergo central venous catheter procedures such as our patient's potential requirement for an implantable cardiovertor defibrillator due to severe global left ventricular systolic dysfunction. The presence of a persistent left superior vena cava should always be suspected when the guidewire takes a left-sided downward course towards the rightatrium at the level of the coronary sinus. Therefore, special attention should be paid to the imaging work-up prior to central venous catheter procedures.

Transthoracic echo: A sensitive tool for detecting cardiac extension of renal cell carcinoma?

Renal cell carcinoma is a common urological malignancy with the unique ability to invade the inferior vena cava(IVC) and to extend into the right atrium of the heart. Of those with Renal cell carcinoma only 4%-25% are found to have IVC invasion and of those only 2%-10% extend into the right atrium. If treated surgically, extension of tumor thrombus is not a determinant of survival; therefore it is imperative to determine the presence and extent of tumor thrombus in order to determine surgical approach and tumor resection. To date this has been primarily accomplished by magnetic resonance imaging and computed tomography. We present a case of 61 years old African American woman in which transthoracic echocardiography provided a more accurate determination/characterization of the presence and degree of tumor thrombus and extension.

Atrium of stone:A case of confined left atrial calcification without hemodynamic compromise

Dystrophic cardiac calcification is often associated with conditions causing systemic inflammation and when present,is usually extensive,often encompassing multiple cardiac chambers and valves.We present an unusual case of dystrophic left atrial calcification in the setting of end stage renal disease on hemodialysis diagnosed by echocardiography and computed tomography.Significant calcium deposition is confined within the walls of the left atrium with no involvement of the mitral valve,and no hemodynamic effects.

Noncoronary sinus of Valsalva rupture into the right atrium with a coexisting perimembranous ventricular septal defect

Ruptured sinus of Valsalva is very uncommon,and is < 1% of all congenital defects.The incidence ranges from 0.1%-3.5%.There is a male to female predominance of 4:1,with the highest incidence in the Asian population.Higher incidence is also seen in patients with Marfan's syndrome and Ehlers Danlos syndrome.There is a higher association of ruptured sinus of Valsalva with ventricular septal defect(VSD),aortic stenosis,and bicuspid valve defect.While most patients with VSD often have rupture of their right coronary sinus of Valsalva into the right ventricle due to poor structural integrity,we present a rare case of a patient with VSD who had rupture of his noncoronary sinus of Valsalva into the right atrium.

Saphenous graft on transesophageal echocardiogram masquerading as an abnormal vascular communication into the right atrium

An unknown aberrant f low in the right atrium observed on doppler with transesophageal echocardiogram(TEE) in a patient with prior coronary bypass. TEE revealed normal size left ventricle with severely dilated left atrium. There was moderate aortic regurgitation and moderate aortic stenosis noted. Patient was incidentally found to have an abnormal vascular communication noted to the right atrium. To further evaluate this f inding, the patient underwent cardiac magnetic resonance angiography which revealed that the tubular structure noted on TEE was actually a graft that was abutting onto the coronary sinus, and the f low anomaly was really the graft coming up and running adjacent to the coronary sinus.

Incidental findings of pericardial calcification

Calcification of the pericardium is a relatively rare finding and often has an uncertain etiology. Incidental findings of pericardial calcification may increase due to widespread application of cardiac computed tomography for the assessment of coronary atherosclerosis in the appropriate clinical setting using coronary artery calcium scoring and/or coronary angiography. Pericardial calcification alone is asymptomatic and is neither necessary nor sufficient for the diagnosis of pericardial constriction. Its presence may suggest of diffused pericardial scarring and consequently, its pathological involvement with pericardial constriction. Calcification of the pericardium must be evaluated with full clinical knowledge to facilitate an accurate diagnosis and an appropriate therapy when required. Our objective is to present a case of asymptomatic pericardial calcification and to discuss the importance of its clinical implications.

Low-Gradient,Low Ejection Fraction Severe Aortic Stenosis:Still a Management Conundrum

The management of patients with severe symptomatic aortic stenosis and a high transvalvular gradient is straightforward.It requires aortic valve replacement.Management of aortic stenosis patients with low flow,low ejection fraction and low gradient is fraught.Such patients rarely fulfi ll all the tenets of“severity”and also have severe left ventricular dysfunction.Both circumstances make the outcome of aortic valve replacement uncertain.Thus the management of this group of patients requires integration of all diagnostic modalities available.The physical examination,degree of valve calcification,the appearance of the valve during sonographic examination and the presence of inotropic reserve all contribute importantly in judging stenosis severity and the likelihood of successful valve replacement.

Physiology of in-situ arterial revascularization in coronary artery bypass grafting: Preoperative,intraoperative and postoperative factors and influences

Surgical revascularization with coronary artery bypass grafting(CABG) has become established as the most effective interventional therapy for patients with moderately severe and severe stable ischemic heart disease(SIHD). This recommendation is based on traditional 5-year outcomes of mortality and avoidance of myocardial infarction leading to reintervention and/or cardiac death. However, these results are confounded in that they challenge the traditional CABG surgical tenets of completeness of anatomic revascularization, the impact of arterial revascularization on late survival, and the lesser impact of secondary prevention following CABG on late outcomes. Moreover, the emergence of physiologic-based revascularization with percutaneous cardiovascular intervention as an alternative strategy for revascularization in SIHD raises the question of whether there are similar physiologic effects in CABG. Finally, the ongoing ISCHEMIA trial is specifically addressing the importance of the physiology of moderate or severe ischemia in optimizing therapeutic interventions in SIHD. So it is time to address the role that physiology plays in surgical revascularization. The long-standing anatomic framework for surgical revascularization is no longer sufficient to explain the mechanisms for short-term and long-term outcomes in CABG. Novel intraoperative imaging technologies have generated important new data on the physiologic blood flow and myocardial perfusion responses to revascularization on an individual graft and global basis. Long-standing assumptions about technical issues such as competitive flow are brought into question by real-time visualization of the physiology of revascularization. Our underestimation of the impact of Guideline Directed Medical Therapy, or Optimal Medical Therapy, on the physiology of preoperative SIHD, and the full impact of secondary prevention on post-intervention SIHD, must be better understood. In this review, these issues are addressed through the perspective of multi-arterial revascularization in CABG, which is emerging(after 30 years) as the "standard of care" for CABG. In fact, it is the physiology of these arterial grafts that is the mechanism for their impact on long-term outcomes in CABG. Moreover, a better understanding of all of these preoperative, intraoperative and postoperative components of the physiology of revascularization that will generate the next, more granular body of knowledge about CABG, and enable surgeons to design and execute a better surgical revascularization procedure for patients in the future.

Biventricular pulsus alternans: An echocardiographic finding in patient with pulmonary embolism

Pulsus alternans is characterized by regular rhythm with beat-to-beat alternation of systolic pressures. Left ventricular alternans is usually found in severe left ventricular dysfunction due to cardiomyopathy, coronary artery disease, systemic hypertension, and aortic stenosis. Right ventricular alternans is usually associated with left ventricular alternans, right ventricular dysfunction, pulmonary embolism, and pulmonary hypertension. Biventricular alternans is rare and associated with severe left ventricular dysfunction and left anterior descending coronary artery disease. The exact mechanism of pulsus alternans has not been clearly delineated, and it has been remained a subject of investigation and conjecture since the nineteenth century. Two fundamental mechanisms have been proposed to explain ventricular alteration. The first, based on the Frank-Starling mechanism, proposes beat-to-beat alteration in end-diastolic volume accounted for the alternating contractile force. The second proposed mechanism which explains the physiology of pulsus alternans involves the abnormal calcium handling by cardiac myocytes. To the best of our knowledge, biventricular alternans in pulmonaryembolism has not been previously reported in the medical literature. We present and discuss the mechanisms of pulsus alternans and its clinical implications.

Case of early right ventricular pacing lead perforation and review of the literature

We report a case of a 77-year-old patient with complete atrioventricular block. She underwent permanent pacemaker implantation complicated by right ventricular lead perforation. This was suspected on transthoracic echocardiogram and confirmed by chest computed tomography. The lead was repositioned in the cardiac electrophysiology lab followed by an uneventful course thereafter.

循证医学分析:全科医学 研究诚信与"没有存在感"的法律

循证医学可以说是现代最重要的创新之一,但出版偏倚和研究透明度不足是影响循证实践基础的严重问题。令人担心的是,这些重要问题在很长一段时间内基本未解决或解决不力。目前监管工作已被证明不足以消除这些问题。幸运的是,在过去的5年里,我们看到了一些有望解决这些问题的进展,将来可以把这些问题作为"污点故事"来回顾。不过时间才是仲裁者,以检验这项补救措施的最终效果。本文按照时间顺序对这些问题进行分析,记录了为解决这些问题所经历的失败、可行和正在进行的措施,以期真正解决这些问题。