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.
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.
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.
A 22 year old African immigrant female student who is otherwise well and fit was incidentally found to have an elevated blood pressure during a physical examination for evaluation of headache. She denied frequent use of over the counter medication, intravenous drug or recreation drug use. There is no family history of HTN, diabetes, coronary artery disease, sudden cardiac death, polycystic kidney disease or other polyglandular autoimmune endocrine disease. On initial examination blood pressure was 190/90 mm Hg in both upper extremities. On fundoscopic examination there was no papilledema; the rest of the examination was otherwise unremarkable. ECG and C-x-ray was essentially unremarkable. A cardiology consultation was placed; on reassessment, there was ejection click over aortic area, a continuous harsh murmur over interscapular space and radio-femoral delay of the pulse. Doppler echocardiography showed a bicuspid aortic valve with mild aortic regurgitation. Head CT was negative for aneurysm or bleeding. Careful review of the frontal chest radiographs revealed rib notching along the inferior aspect of the third to the eighth ribs. Diagnosis of aortic coarctation was suspected and a spiral CT of the thoracic aorta with three-dimensional reconstruction confirmed postductal coarctation. A cardiothoracic surgery consultation was obtained and the patient underwent successful repair. Coarctation of the aorta is an acyanotic congenital heart disease. Most patients are identified accidentally during routine medical examination. Coarctation accounts for approximately 5% of all congenital heart diseases. Coarctation is a discrete infolding of the posteriolateral wall of the aorta, which results in an obstructing membrane at the level of the ductus arteriosus. There are several classifications of the coarctation; it is most commonly classified into adult (postductal) type and infantile (preductal) type. However, many patients with preductal lesions do not present until adulthood. The age of the patient at presentation is dependent on the severity rather than the site of obstruction. Usually those with severe obstruction or associated cardiac anomalies present early, as a result of cardiac failure or occasionally cerebrovascular accident, aortic dissection, or endocarditis. 25-50% of patients will also have a bicuspid aortic valve. Patent ductus arteriosus, ventricular septal defects and Turner syndrome are other common associations with aortic coarctation. This case emphasizes the importance of a careful history and physical examination, which remain the cornerstones of diagnosis of coarctation.
carditis. On September 14, 2004, the patient underwent repeat cardiac surgery. The intraoperative findings included 1) a potential space between the sewing ring and the annulus; 2) the suspected prosthetic mitral valve mass was identified as a previously preserved primary chord to the anterior leaflet, which had a small portion of papillary muscle attached to it. This was prolapsing into the left ventricular outflow tract through the aortic valve. The surgeon performed a primary repair of the paravalvular leak and excision of the residual anterior leaflet chordae with attached papillary muscle. The papillary muscle presumably ruptured and resulted in its prolapse into the left ventricular outflow tract through the aortic valve. Pathology demonstrated infarcted papillary muscle tissue attached to chordae consistent with the contention of a ruptured papillary muscle.
A 42-year-old man presented to his primary care provider office with symptoms of upper respiratory tract infection and episodic dull chest pain for 3 days. Chest pain was located over anterior precordium and retrosternum, increased in intensity with inspiration and body movement. On examination his vitals were stable except for pulse of 115 beats per minute. Physical examination was otherwise unremarkable. Electrocardiogram performed in the office revealed diffuse ST-segment elevation of 1-2 mm and diffuse PR-segment depression. Portable chest x-ray was unremarkable without mediastinal widening. He was diagnosed with acute pericarditis. However due to suspicion of acute coronary syndrome he was referred to emergency room for possible admission to coronary care unit for observation. In the emergency room on re-examination he is alert but uncomfortable. Blood pressure was 140/82 mm Hg in all extremities; pulse was 103 beats per minute and regular. His chest is clear and careful cardiac examination revealed a 2/6 early diastolic murmur in aortic region. First set of cardiac enzymes was negative. Repeat ECG was similar to previous one. Incidentally ordered CT scan of chest was reported as Stanford Type-A thoracic dissection. He was immediately started on IV labetolol drip and admitted to intensive care unit. Emergent cardiothoracic surgery consultation was placed. Patient successfully underwent aortic repair without complication. Thoracic aortic dissection is a life-threatening condition associated with high rates of morbidity and mortality. It is the most common acute aortic condition requiring emergent surgical therapy. The incidence of aortic dissection has been estimated at from 5 to 30 per 1 million people per year. Dissection is a dynamic process that can occur anywhere within the aorta and is characterized by separation of the layers within the aortic wall.Thoracic aortic dissection has long been known to mimic multitude of clinical conditions. It is usually associated with severe chest pain and or back pain and is the most frequent fatal condition in the spectrum of chest pain syndromes. We present a young patient with cystic medial necrosis who presented with atypical clinical symptoms and ascending thoracic aortic dissection was diagnosed by CT imaging. Patients with cystic medial necrosis and aortic dissection may not present with a classic acute chest pain syndrome. Due to variable clinical presentation, dissection of thoracic aorta can be a diagnostic challenge for physicians. Therefore a high clinical index of suspicion is necessary.
carditis. On September 14, 2004, the patient underwent repeat cardiac surgery. The intraoperative findings included 1) a potential space between the sewing ring and the annulus; 2) the suspected prosthetic mitral valve mass was identified as a previously preserved primary chord to the anterior leaflet, which had a small portion of papillary muscle attached to it. This was prolapsing into the left ventricular outflow tract through the aortic valve. The surgeon performed a primary repair of the paravalvular leak and excision of the residual anterior leaflet chordae with attached papillary muscle. The papillary muscle presumably ruptured and resulted in its prolapse into the left ventricular outflow tract through the aortic valve. Pathology demonstrated infarcted papillary muscle tissue attached to chordae consistent with the contention of a ruptured papillary muscle.
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