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Heart involvement is a frequent cause of morbidity and mortality in autoimmune diseases. All cardiac structures can be involved: pericardium, endocardium, myocardium, coronary circle, and conduction system. In the last decade many patients affected by autoimmune diseases have been treated with hematopoietic stem cell transplantation; the vast majority of these transplants have been autologous, and most have been within the context of phase I and II clinical trials; now, phase III trials are ongoing. Patients affected by autoimmune disease often have cardiac involvement which potentially puts them at higher risk from acute cardiotoxicity due to alkylating agents such as cyclophosphamide. The authors propose an algorithm for cardiac assessment before stem cell transplantation in order to identify those patients at highest risk, prior to administering any drug, to avoid further worsening of heart involvement and possible organ failure.A baseline assessment includes physical examinations, ECG to highlight arrhythmias and conduction abnormalities, chest X-ray to evaluate the presence of pericardial effusion and cardiothoracic ratio.A second-step evaluation includes echocardiography (which assesses the following parameters: left ventricular ejection fraction, diastolic function, tricuspid gradient, pulmonary acceleration time, right ventricular diameter and pericardial effusion, wall motion), Holter ECG that may highlight the presence of arrhythmias and biohumoral parameters such as brain natriuretic peptide and troponin I. If these parameters show abnormalities, a further step is required before transplantation. Cardiac catheterization allows to identify ischemic coronary diseases and pulmonary artery hypertension. Intensive monitoring with life card assessment before inclusion might establish ischemic coronary diseases or complex arrhythmias requiring pacing. Magnetic resonance imaging and single-photon emission computed tomography with dipyridamole are useful tools to evaluate the coronary flow. Treatment of ischemic coronary disease (assessment for revascularization), cardiac failure, pulmonary artery hypertension and arrhythmias constitutes the final step. The aim is to optimize cardiac status in order to allow intense immunosuppressive treatments.
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PMID:Hematopoietic stem cell transplantation in autoimmune diseases: algorithm for cardiovascular assessment. 1732 34

Cardiovascular dysfunction in patients with hemolytic-uremic syndrome (HUS) may be related to secondary issues such as volume overload, hypertension or electrolyte disturbances including hyperkalemia. Additionally, primary myocardial involvement has been increasingly recognized as a potential comorbid feature of HUS. We report a 9-month-old child with HUS who developed clinical signs of poor myocardial function with depressed myocardial function noted by echocardiography. Supportive care including mechanical ventilation and inotropic agents were necessary for approximately 10 days. Follow-up echocardiography revealed return of normal ventricular function. Previous reports of primary cardiac involvement with HUS have included thrombotic microangiopathy of the coronary vasculature resulting in myocardial ischemia, myocardial infarction or depressed myocardial function, myocarditis, congestive heart failure with dilated cardiomyopathy and pericardial effusion with tamponade. Given the potential for morbidity and mortality during the preoperative period in patients with HUS, anesthesiologists involved in the care of such patients should be aware of the potential for myocardial involvement in this disease process. Preoperatively, the routine evaluation of myocardial function may be indicated.
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PMID:Hemolytic-uremic syndrome and myocardial dysfunction in a 9-month-old boy. 1749 23

Heart failure in sub-Saharan Africans is mainly due to non-ischaemic causes, such as hypertension, rheumatic heart disease, cardiomyopathy and pericarditis. The two endemic diseases that are major contributors to the clinical syndrome of heart failure in Africa are cardiomyopathy and pericarditis. The major forms of endemic cardiomyopathy are idiopathic dilated cardiomyopathy, peripartum cardiomyopathy and endomyocardial fibrosis. Endomyocardial fibrosis, which affects children, has the worst prognosis. Other cardiomyopathies have similar epidemiological characteristics to those of other populations in the world. HIV infection is associated with occurrence of HIV-associated cardiomyopathy in patients with advanced immunosuppression, and the rise in the incidence of tuberculous pericarditis. HIV-associated tuberculous pericarditis is characterised by larger pericardial effusion, a greater frequency of myopericarditis, and a higher mortality than in people without AIDS. Population-based studies on the epidemiology of heart failure, cardiomyopathy and pericarditis in Africans, and studies of new interventions to reduce mortality, particularly in endomyocardial fibrosis and tuberculous pericarditis, are needed.
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PMID:Contemporary trends in the epidemiology and management of cardiomyopathy and pericarditis in sub-Saharan Africa. 1789 Jun 92

Transthoracic echocardiography is a primary non-invasive modality for investigation of heart transplant recipients. It is a versatile tool which provides comprehensive information about cardiac structure and function. Echocardiographic examinations can be easily performed at the bedside and serially repeated without any patient's discomfort. This review highlights the usefulness of Doppler echocardiography in the assessment of left ventricular and right ventricular systolic and diastolic function, of left ventricular mass, valvular heart disease, pulmonary arterial hypertension and pericardial effusion in heart transplant recipients. The main experiences performed by either standard Doppler echocardiography and new high-tech ultrasound technologies are summarised, pointing out advantages and limitations of the described techniques in diagnosing acute allograft rejection and cardiac graft vasculopathy. Despite the sustained efforts of echocardiographic technique in predicting the biopsy state, endocardial myocardial biopsies are still regarded as the gold standard for detection of acute allograft rejection. Conversely, stress echocardiography is able to identify accurately cardiac graft vasculopathy and has a recognised prognostic in this clinical setting. A normal stress-echo justifies postponement of invasive studies. Another use of transthoracic echocardiography is the monitorisation and the visualisation of the catheter during the performance of endomyocardial biopsy. Bedside stress echocardiography is even useful to select appropriately heart donors with brain death. The ultrasound monitoring is simple and effective for monitoring a safe performance of biopsy procedures.
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PMID:Usefulness and limitations of transthoracic echocardiography in heart transplantation recipients. 1819 Jul 12

Hypertension is an uncommon but significant problem in high-risk neonates and infants, and the spectrum of potential causes is broad. Here, we describe an extremely premature infant (birth weight, 728 g; gestational age, 27 weeks) with multiple complications and hypertension. During admission, umbilical artery catheters were used for a period of time, and he suffered from respiratory distress syndrome, intraventricular hemorrhage, pulmonary hemorrhage, patent ductus arteriosus, pericardial effusion, heart failure, repeated sepsis, anemia, thrombocytopenia, chronic lung disease, and progressive liver damage. He was treated with multiple medications, including erythropoietin, indomethacin, epinephrine, dopamine, aminophylline, multiple antibiotics, amphotericin B, and total parenteral nutrition. Hypertension was first noted when he was 41 days old, with spontaneous remission. It then recurred, reaching higher than 100 mmHg when he was almost 4 months old. After stopping erythropoietin, hypertension subsided for a short period of time and went up again. Multiple factor-related hypertension in this premature infant was considered. Related literature on hypertension in premature infants is reviewed. In conclusion, multiple factors can influence blood pressure and may induce hypertension in high-risk premature infants. Thus, blood pressure should be closely monitored in high-risk premature infants. Judicious use of all medications and interventions are crucial to decrease the incidence of hypertension in high-risk premature infants.
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PMID:Conditions associated with hypertension in a high-risk premature infant. 1881 44

Given the increasing numbers of patients requiring long-term hemodialysis, there is an inevitably increasing population of patients with occluded central venous inflow (subclavian, innominate, and caval) despite access or access possibilities in the arm. In an effort to avoid sternotomy, we have attempted to treat these patients with a substernally tunneled subclavian to right atrial bypass. Patients treated in this fashion have an existing fistula with symptomatic venous hypertension or good fistula options but complete central vein obstruction, a patent subclavian/axillary vein to the costoclavicular junction, and no other options in the contralateral arm. Claviculectomy is performed and the subclavian vein isolated. Through a third intercostal space "minipericardiotomy," the right atrial appendage is exposed. A retrosternal tunnel is fashioned, and bypass is performed from the subclavian vein to atrial appendage. Eleven patients aged 20-70 (mean 46) years underwent surgery at our institution between February 2004 and March 2007. Three bypasses were performed with autogenous vein (two femoral and one saphenous), while eight were performed with polytetrafluoroethylene in an effort to preserve the superficial femoral vein for later leg bypass. There was one early mortality due to sepsis, and early morbidity was limited to one patient with a symptomatic pericardial effusion. Mean follow-up was 16 (range 3-43) months. Sixty-seven percent and 33% of arteriovenous fistulas remained functional at 6 and 10 months, respectively; and one patient's fistula remained functional at 21 months. Four patients (36%) developed central bypass stenosis or occlusion, one requiring a redo bypass and three angioplasty. Infection occurred in two patients (18%), with removal of autogenous vein graft in one. While a significant number of these bypasses fail, upper extremity access is maintained in a reasonable number of patients (67% at 6 months) who are not candidates for local repair or stenting and would thus have no other upper extremity access options. This technique offers an alternative to sternotomy and brachiocephalic vein reconstruction, although the superiority of one method over the other will require direct comparison.
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PMID:Subclavian vein to right atrial appendage bypass without sternotomy to maintain arteriovenous access in patients with complete central vein occlusion, a new approach. 1935 37

A 74-year-old woman was at the emergency department for acute chest pain, dyspnea and severe transient hypotension. History was arterial hypertension and external electrical cardioversion (EEC) for persistent atrial fibrillation (AF) 8 days before admission. At that time echocardiography was normal. The patient underwent coronary angiography with no evidence of significant coronary arteries disease. At echocardiography a large multi-loculated mass occupying most of the left atrial space and obstructing left ventricular inflow was evident. There was mild pericardial effusion. The patient was operated and a large thrombus totally encompassed in the left atrial wall was removed. Initial tearing into the pericardial space was revealed. Post-surgical follow-up was uneventful and at 3-6 months normalization of the atrial cavity with blending of atrial endocardium and epicardium was demonstrated. No apparent etiological factor was found. We have provided evidence of the possible rapid formation of a large intramural atrial hematoma. Spontaneous atrial wall dissection should be considered in the differential diagnosis of chest pain.
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PMID:A fast-growing obstructive left atrial intramural hematoma causing acute prolonged chest pain. 1944 3

Diagnosing acute Stanford type A aortic dissection with the uncommon involvement of the left main coronary artery(LMCA) remains challenging for the emergency physician because it can resemble acute myocardial infarction with cardiogenic shock. The following case report illustrate this infrequent but critical situation. A 52-year-old woman with a history of hypertension awakened with acute retrosternal chest pain accompanied by nausea and vomiting. She was referred to our hospital for primary coronary intervention because of acute myocardial infarction with cardiogenic shock. Coronary angiography indeed revealed LMCA occlusion. Subsequently successful percutaneous coronary intervention with stent implantation was performed, followed by immediate clinical improvement of the patient. Soon after admission at the coronary care unit, severe chest pain, hypotension, and electrocardiographic signs of diffuse myocardial ischemia relapsed. Control coronary angiography,however, showed no in-stent thrombosis. Review of clinical examination revealed an aortic regurgitation murmur. Because of this dynamic pattern of (1) signs of acute myocardial ischemia, (2) relapse of hemodynamic collapse, and (3) unaltered control coronary angiography together with the confirmed aortic regurgitation at transthoracic echocardiography, the patient was suspected of having aortic dissection. Transesophageal echocardiography revealed Stanford type A aortic dissection with severe eccentric aortic regurgitation and no pericardial effusion. Emergent valve-sparing aortic replacement was performed. The patient recovered completely. In this case, the lifesaving element was primary coronary intervention with stenting of the LMCA preventing extensive myocardial damage followed by a surgical correction of the aorta.
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PMID:Acute myocardial infarction with cardiogenic shock in a patient with acute aortic dissection. 1968 31

Cases of small pericardial effusion have been reported in association with dengue fever (DF), largely with dengue hemorrhagic fever during epidemic outbreaks. However, cardiac tamponade developed by a patient with DF has not yet been reported in the English literature. We report a case of cardiac tamponade in a patient with DF and lupus nephritis. We describe the characteristic features to differentiate pericardial effusion of lupus origin from that of viral etiology. A 59-year-old Hispanic woman presented to the emergency department with complaints of 5 to 6 days of fever, myalgia, headache, and retro-orbital pain. Her symptoms started 3 days after returning from the Dominican Republic, where a dengue outbreak was reported. Her past medical history was significant for hypertension and lupus nephritis diagnosed 3 months earlier. On day 2, patient developed a large pericardial effusion that progressed to tamponade over the next 2 days, requiring surgical drainage. Subsequently, the patient improved; however, serological analysis did not suggest any lupus flare-up. Pericardial fluid analysis showed hypocellularity without lupus erythematosus cell and biopsy revealed only reactive mesothelial cells suggestive of viral etiology. Dengue serology was reported as markedly elevated, supporting a diagnosis of classic DF (both immunoglobulin M [IgM] titer 2.93 and IgG titer 12.13 by enzyme-linked immunosorbent assay [ELISA]; reference range: <0.90 for both). Absence of rise in serum antinuclear antibody (ANA) titer correlated with lack of inflammatory changes on the pericardium favored viral etiology over lupus origin. This differentiation is pertinent from a management perspective.
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PMID:Cardiac tamponade in a patient with dengue fever and lupus nephritis: a case report. 2008 24

Patients with advanced idiopathic pulmonary artery hypertension have often a chronic pericardial effusion. It is the result of increased transudation and impaired re-absorption due to elevated venous pressure. These patients have pre-existent symptoms and signs of chronic right heart failure. High degree of suspicion is required to detect of development of an atypical form of tamponade with isolated compression of left heart chambers as shown in present case report. Transthoracic echocardiography provides a rapid access to the correct diagnosis, a prompt relief of symptoms following the ultrasound guided pericardiocentesis and important diagnostic tool for regular follow up of patients thereafter as shown in our case report.
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PMID:Echocardiography of isolated subacute left heart tamponade in a patient with cor pulmonale and circumferential pericardial effusion. 2063 52


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