1/13. Solitary renal myofibromatosis: an unusual cause of infantile hypertension.INTRODUCTION: Renovascular disease accounts for the vast majority of cases of infantile hypertension with complications resulting from umbilical arterial catheterization predominating in the neonatal period and fibrodysplastic lesions of the renal artery predominating outside the neonatal period. We report a previously undescribed cause of renovascular hypertension: solitary renal myofibromatosis. CASE REPORT: A 9-month-old male infant was transported to the intensive care unit at Children's Hospital in Denver, colorado, for evaluation and treatment of a dilated cardiomyopathy and severe systemic hypertension. The child was full-term with no perinatal problems. Specifically, the child never required umbilical arterial catheterization. He was well until 6 months of age when his parents noted poor weight gain. At 9 months of age, he was evaluated at the referral hospital for failure to thrive. On examination he was noted to have a blood pressure of 170/110 mm Hg, but no other abnormalities. A chest radiograph showed cardiomegaly. Laboratory studies demonstrated normal electrolytes, blood urea nitrogen, and creatinine. However, urinalysis demonstrated 4 protein without red blood cells. An echocardiogram showed severe left ventricular dilatation with an ejection fraction of 16%. On admission the child was noted to be cachectic. His vital signs, including blood pressure, were normal for age. The physical examination was unremarkable. serum electrolytes, blood urea nitrogen, and creatinine were normal. Echocardiographic studies suggested a dilated hypertrophic cardiomyopathy. He was started on digoxin and captopril. Subsequently, he demonstrated episodic hypertension ranging from 170/90 to 220/130 mm Hg. A repeat echocardiogram 24 hours after admission demonstrated a purely hypertrophic cardiomyopathy. verapamil and nifedipine were added to the treatment regimen in an effort to better control the blood pressure without success. urine and blood for catecholamines and plasma renin activity, respectively, were sent and treatment with phentolamine instituted because of a possible pheochromocytoma. A spiral abdominal computerized tomographic scan revealed a markedly abnormal right kidney with linear streaky areas of calcification around the hilum and also an area of nonenhancement in the posterior upper pole. The adrenals and the left kidney were normal. Doppler ultrasound revealed a decrease in right renal arterial flow. The urinary catecholamines were normal and surgery was scheduled after the blood pressure was brought under control by medical treatment. At surgery, tumorous tissue and thrombosis of the renal artery were found in the right upper pole. A right nephrectomy was performed. Pathologic examination of the kidney showed the presence of a diffuse spindle cell proliferation in the interstitium of the kidney. The angiogenic/angiocentric character of the proliferation was demonstrated in several large renal vessels. The lumen of most vessels was narrowed and some vessels were totally occluded with recanalization and dystrophic calcifications observed. Immunostaining of the tumor demonstrated strong desmin and vimentin positivity and minimal actin positivity in the spindle cells. Mitotic activity was not noted in the spindle cell process. These pathologic changes were consistent with a diagnosis of infantile myofibromatosis (IM). The child's preoperative plasma renin activity was 50 712 ng/dL/h (reference range, 235-3700 ng/dL/h). DISCUSSION: The causes of systemic hypertension in infancy are many although renal causes are by far the most common. Renal arterial stenosis or thrombosis accounts for 10% to 24% of cases of infantile hypertension. renal artery thrombosis is usually a consequence of umbilical arterial catheterization, which can also lead to embolization of the renal artery. renal artery stenosis may result from fibrodysplastic lesions (74%), abdominal aortitis (9%), a complication of renal transplantation (5%), and ren- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
2/13. The spectrum of dilated cardiomyopathy. The Johns Hopkins experience with 1,278 patients.This report describes the evaluation of 1,278 patients referred to The Johns Hopkins Hospital with dilated cardiomyopathy. After a careful history and physical examination, selected laboratory tests, and endomyocardial biopsy, a specific diagnosis was made in 49% of cases. In 16% of cases the biopsy demonstrated a specific histologic diagnosis. myocarditis and coronary artery disease were the most frequent specific diagnoses; 51% of patients were classified as idiopathic. Thus a rigorous and systematic search can demonstrate an underlying cause for approximately one-half of patients with unexplained cardiomyopathy. Endomyocardial biopsy plays a crucial role in this evaluation. Six cases are presented which demonstrate the utility of endomyocardial biopsy in specific clinical situations. In addition to its routine use in monitoring rejection in heart transplant recipients, endomyocardial biopsy is indicated in the evaluation of possible infiltrative cardiomyopathy, in differentiating restrictive cardiomyopathy from constrictive pericarditis, and in diagnosing and monitoring doxorubicin cardiotoxicity. The importance of diagnosing myocarditis remains controversial, and disagreement persists about the utility of immunosuppressive therapy in these patients. A combination of clinical and histologic features can divide patients with myocarditis into 4 subgroups--acute, fulminant, chronic active, and chronic persistent. This classification provides prognostic information and may identify those patients who may respond to immunosuppression, as well as those likely to have adverse outcomes from such treatment. The continued development of novel molecular techniques may allow endomyocardial biopsy to provide greater prognostic and therapeutic information in the future.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
3/13. myocarditis and cardiomyopathy associated with clozapine.BACKGROUND: clozapine is effective for resistant schizophrenia. After two sudden deaths in physically well young men soon after starting clozapine, we investigated the cardiovascular complications for this drug. methods: From January, 1993, to March, 1999, 8000 patients started clozapine treatment in australia, and were registered with a mandatory monitoring service. We identified cases of myocarditis and cardiomyopathy from voluntary reports to the Australian Adverse Drug Reaction Committee and sought details of the relevant diagnostic studies, necropsies that had been done in suspicious cases, or both. FINDINGS: 23 cases (20 men, three women, mean age 36 years [SD 9]) were identified: 15 of myocarditis and eight of cardiomyopathy associated with clozapine treatment. Six patients died. All cases of myocarditis (five deaths) occurred within 3 weeks of starting clozapine. Cardiomyopathy (one death) was diagnosed up to 36 months after clozapine was started. Necropsy results showed mainly eosinophilic infiltrates with myocytolysis, consistent with an acute drug reaction. INTERPRETATION: clozapine therapy may be associated with potentially fatal myocarditis and cardiomyopathy in physically healthy young adults with schizophrenia.- - - - - - - - - - ranking = 0.39275631734321keywords = physical (Clic here for more details about this article) |
4/13. First clinical experience with the DeBakey VAD continuous-axial-flow pump for bridge to transplantation.BACKGROUND: A shortage of donor organs and increased numbers of deaths of patients on the waiting list for cardiac transplantation make mechanical circulatory support for a bridge to transplantation a standard clinical procedure. Continuous-flow rotary blood pumps offer exciting new perspectives. methods AND RESULTS: Two male patients (ages 44 and 65 years) suffering from end-stage left heart failure were implanted with a DeBakey VAD axial-flow pump for use as a bridge to transplant. In the initial postoperative period, the mean pump flow was 3.9 /-0.5 L/min, which equals a mean cardiac index (CI) of 2.3 /-0.2 L. min(-1). m(-2). In both patients, the early postoperative phase was characterized by a completely nonpulsatile flow profile. However, with the recovery of heart function 8 to 12 days after implantation, increasing pulse pressures became evident, and net flow rose to 4.5 /-0.6 L/min, causing an increase of mean CI up to 2.7 /-0.2 L. min(-1). m(-2). patients were mobilized and put through regular physical training. hemolysis stayed in the physiological range and increased only slightly from 2. 1 /-0.8 mg/dL before surgery to 3.3 /-1.8 mg/dL 6 weeks after implantation. CONCLUSIONS:The first clinical implants of the DeBakey VAD axial-flow pump have demonstrated the device to be a promising measure of bridge-to-transplant mechanical support.- - - - - - - - - - ranking = 0.1963781586716keywords = physical (Clic here for more details about this article) |
5/13. Importance of physical rehabilitation before and after cardiac transplantation in a patient with myotonic dystrophy: a case report.patients with muscular dystrophy and concomitant cardiomyopathy are only reluctantly accepted for heart transplantation because of the perioperative risk secondary to respiratory muscle weakness. We describe a man with Steinert's disease (myotonic dystrophy) who received a cardiac allograft because of end-stage dilated cardiomyopathy. This case shows the importance of uninterrupted physiotherapeutic training and assistance to minimize respiratory infections and ventilatory insufficiency in patients with muscle diseases under high-dose immunosuppression. To our knowledge, this is the first heart transplantation reported in a patient with Steinert's disease who has clinically overt muscular impairment.- - - - - - - - - - ranking = 0.78551263468642keywords = physical (Clic here for more details about this article) |
6/13. Peripartum cardiomyopathy and biventricular thrombi.Peripartum cardiomyopathy is a rare cardiac disorder characterized by the development of heart failure in the last month of pregnancy or up to 5 months postpartum in women without other determinable causes of cardiac failure. Intracardiac thrombi have been found at autopsy in some patients with this condition and have been demonstrated in the left or right ventricles on 2-dimensional echocardiography. A 23-year-old woman presented with peripartum cardiomyopathy and biventricular thrombi on echocardiography. The thrombi were spherical, pedunculate, shaggy and irregular in configuration, and freely mobile, suggesting that they were fresh. She was treated with conventional heart failure therapy and anticoagulants. Four days later, the apical thrombi within both ventricles had disappeared and there was no evidence of embolism on physical examination. The hypercoagulable state of the peripartum period and the severe biventricular dysfunction most likely led to the formation of biventricular thrombi.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
7/13. A case of reversible dilated cardiomyopathy after alpha-interferon therapy in a patient with renal cell carcinoma.A 47-year-old man with renal cell carcinoma underwent nephrectomy, and postoperative chemotherapy was performed with recombinant alpha-interferon. Five years later, he experienced dyspnea during physical exertion. An echocardiogram revealed dilatation and systolic dysfunction of the left ventricle, and thallium-201 myocardial scintigraphy showed diffuse heterogeneous perfusion. We diagnosed congestive heart failure because of cardiomyopathy induced by alpha-interferon therapy. Withdrawal of interferon therapy and the combination of an angiotensin-converting enzyme inhibitor, diuretics, and digitalis improved left ventricular systolic function. Furthermore, myocardial scintigraphy using [123I] beta-methyl-p-iodophenylpentadecanoic acid (123I-BMIPP) or [123 I]metaiodobenzylguanidine (123I-MIBG) revealed normal perfusion after the improvement of congestive heart failure. This is a rare case of interferon-induced cardiomyopathy that resulted in normal myocardial images in 123I-BMIPP and 123I-MIBG scintigrams after withdrawal of interferon therapy.- - - - - - - - - - ranking = 0.1963781586716keywords = physical (Clic here for more details about this article) |
8/13. Suppression of electrical storm by biventricular pacing in a patient with idiopathic dilated cardiomyopathy and ventricular tachycardia.This study presents a patient with idiopathic dilated cardiomyopathy who had suffered from multiple ICD shocks. amiodarone and a beta-blocker failed to suppress ventricular tachycardia. His ECG showed a very wide QRS complex with an intraventricular conduction delay, so biventricular (BV) pacing was attempted. The BV pacing successfully prevented the multiple ICD shocks accompanied with an improvement in left ventricular systolic function and physical activity.- - - - - - - - - - ranking = 0.1963781586716keywords = physical (Clic here for more details about this article) |
9/13. Reversible cardiomyopathy caused by an uncommon form of congenital adrenal hyperplasia.A child with 11, beta-hydroxylase deficiency, an unusual variant of congenital adrenal hyperplasia, presented with dilated cardiomyopathy. Congestive heart failure and dilatation of ventricles resolved with hydrocortisone replacement therapy. This case highlights the importance of complete history and physical examination of children with dilated cardiomyopathy.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
10/13. Dilated cardiomyopathy in a 7-year-old girl.Pediatric patients with dilated cardiomyopathy can initially be present for medical attention with non-specific and misleading signs and symptoms. We present the case of a 7-year-old girl with vague complaints of fever, vomiting, and abdominal pain and cardiac murmur on physical exam who progressed to congestive heart failure before her dilated cardiomyopathy was diagnosed. Clinicians should maintain a high index of suspicion for dilated cardiomyopathy in any patient with cardiac murmur and systematic symptoms.- - - - - - - - - - ranking = 0.1963781586716keywords = physical (Clic here for more details about this article) |
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