1/48. 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 = pressure (Clic here for more details about this article) |
2/48. Acute cardiovascular changes of partial left ventriculectomy without mitral valve repair.We assessed the acute cardiovascular changes of partial left ventriculectomy (PLV) in a patient with idiopathic dilated cardiomyopathy (IDCM) without mitral regurgitation. Acutely, PLV reduced left ventricular (LV) end-diastolic dimension and volume while increasing LV ejection fraction and cardiac output due to increased HR and SV. Substantial increases in LV filling pressure, possibly due to high LV end-systolic and diastolic elastances, were of concern clinically and the mechanism(s) of change remain unclear. However, one year follow-up showed remarkable improvements in NYHA and VO2 max while maintaining reduced LV volume, increased LVEF, and trivial MR.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
3/48. hepatopulmonary syndrome associated with cardiorespiratory disease.BACKGROUND/AIMS: hepatopulmonary syndrome is defined as a clinical triad including chronic liver disease, abnormal pulmonary gas exchange resulting ultimately in profound arterial hypoxaemia, and evidence of intrapulmonary vascular dilatations. We report five patients with liver cirrhosis diagnosed with hepatopulmonary syndrome who had associated chronic obstructive or restrictive respiratory diseases. methods: Clinical, radiographic and constrast-enhanced echocardiographic findings, and systemic and pulmonary haemodynamic and gas exchange, including ventilation-perfusion distributions, measurements were assessed in all five patients. RESULTS: echocardiography was consistent with the presence of intrapulmonary vasodilation without intracardiac abnormalities, and high resolution computed tomographic scan features were compatible with clinical (3 cases) or histopathological diagnoses (2 cases) of associated respiratory disorders. The most common prominent functional findings were moderate to severe arterial hypoxaemia, caused by moderately to severely increased intrapulmonary shunting and/or mild to moderate low ventilation-perfusion areas, and hypocarbia along with an increased cardiac output and a low pulmonary artery pressure and vascular resistance. CONCLUSIONS: These functional characteristics, classically reported in the setting of clinically stable, uncomplicated hepatopulmonary syndrome, conform to a distinctively unique, chronic gas exchange pattern. Equally important, these pulmonary haemodynamic-gas exchange hallmarks are not influenced by the co-existence of chronic cardiorespiratory disease states. These data may have clinical relevance for elective indication of hepatic transplantation in patients with life-threatening hepatopulmonary syndrome.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
4/48. Acute dilated cardiomyopathy and central nervous system toxicity following propranolol intoxication.OBJECTIVE: We report a case of a 16-year-old boy who developed central nervous system (CNS) depression and acute dilated cardiomyopathy following ingestion of 3200 mg of propranolol in a suicide attempt. Early echocardiographic findings were the only sign of cardiac toxicity. DESIGN: A case report. SETTING: Pediatric intensive care unit of a teaching hospital. RESULTS: This child developed significant acute dilated cardiomyopathy and severe CNS depression 2 hours after ingesting 3200 mg of propranolol. The child was treated with gastric lavage, activated charcoal, and mechanical ventilation. Following the echocardiographic findings, treatment with isoprenaline hydrochloride and glucagon were given intravenously. Echocardiographic examination 12 hours following treatment showed normal left ventricular size and function. No change in pulse rate or blood pressure was reported on admission and during his hospitalization. DISCUSSION: In the early stages of propranolol and other lipophilic beta-blocker intoxication, severe CNS depression can develop in the absence of clinical signs of cardiac toxicity. Early echocardiographic evaluation is important and may prevent delay in diagnosis and treatment of cardiac toxicity.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
5/48. Long term treatment of refractory congestive heart failure by continuous positive airway pressure.A 26-year-old woman developed congestive heart failure (CHF) secondary to idiopathic dilated cardiomyopathy. Despite aggressive pharmacological therapy, her disease progressed over the next year, causing massive edema and dyspnea at rest. Although a sleep study showed no clinically significant sleep apnea, she was treated with nocturnal continuous positive airway pressure (CPAP). Following application of CPAP, a remarkable improvement in her condition was observed, with resolution of her edema and alleviation of dyspnea. Left ventricular ejection fraction increased from 29% to 43% and left ventricular dimensions decreased. Previous studies have demonstrated that nocturnal CPAP exerts a number of favourable effects on cardiovascular function in patients with CHF who suffer from a coexisting sleep apnea disorder. This report illustrates that CPAP can also have beneficial long term effects on the failing heart even in the absence of clinically significant sleep apnea.- - - - - - - - - - ranking = 1.25keywords = pressure (Clic here for more details about this article) |
6/48. 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 = 0.25keywords = pressure (Clic here for more details about this article) |
7/48. Dilated phase of hypertrophic cardiomyopathy with mid-ventricular obstruction after 20-year follow-up.This paper reports a case of dilated phase in hypertrophic cardiomyopathy with mid-ventricular obstruction. Following the first cardiac catheterization and endomyocardial biopsy, the patient was diagnosed as having hypertrophic cardiomyopathy (HCM) with mid-ventricular obstruction. He had been first diagnosed at the age of 38 years and was subsequently followed for 20 years. Echocardiogram revealed gradually progressive dilatation of the left ventricle, associated with disappearance of the mid-ventricular obstruction. The second cardiac catheterization and endomyocardial biopsy performed at the age of 58 disclosed that the patient was in the dilated phase of HCM with a dip-and-plateau pattern diastolic pressure trace.- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
8/48. Aortic valve replacement combined with endoventricular circulatory patch plasty (Dor operation) in a patient with aortic valve stenosis and severe ischemic cardiomyopathy.A 58-year-old woman with ischemic cardiomyopathy and aortic valve stenosis, underwent aortic valve replacement and simultaneous endoventricular circulatory patch plasty (Dor operation). She underwent coronary artery bypass grafting for severe triple vessel disease 10 years ago. Recently she started to show severe congestive heart failure. aortic valve stenosis with pressure gradient of 85-mmHg was also found. Coronary bypasses were all patent, but the left ventricle (LV) was severely dilated (LVDd/Ds=71/61 mm) and the ischemic cardiomyopathy was considered as the cause. She successfully underwent aortic valve replacement and endoventricular circulatory patch plasty. The initial postoperative course was complicated with intractable ventricular arrhythmia, but subsequent course was smooth and the patient was discharged with improved symptoms (NYHA Class II). Postoperative catheterization showed decreased left ventricular volume and improved contractility. This case implies the role of LV remodeling procedure in the ischemic cardiomyopathy combined with aortic valve lesion- - - - - - - - - - ranking = 0.25keywords = pressure (Clic here for more details about this article) |
9/48. Combined spinal-epidural anesthesia for cesarean section in a patient with peripartum dilated cardiomyopathy.PURPOSE: To report a case of peripartum dilated cardiomyopathy associated with morbid obesity and possible difficult airway presenting for elective cesarean section, which was successfully managed with combined spinal-epidural anesthesia. CLINICAL FEATURES: A morbidly obese parturient with a potentially difficult airway, suffering from idiopathic peripartum cardiomyopathy (ejection fraction 20%), was scheduled for an elective cesarean section. A combined spinal epidural anesthesia was performed and 6 mg of bupivacaine were injected into the subarachnoid space. This was supplemented after 60 min with 25 mg of bupivacaine injected epidurally. The patient's hemodynamic status was monitored with direct intra-arterial blood pressure and central venous pressure measurements. The patient's perioperative course was uneventful. CONCLUSION: In patients suffering from peripartum cardiomyopathy, undergoing cesarean section, combined spinal-epidural anesthesia may be an acceptable anesthetic alternative.- - - - - - - - - - ranking = 0.5keywords = pressure (Clic here for more details about this article) |
10/48. Long-term treatment of ischemic dilated cardiomyopathy with continuous positive airway pressure.An 81-year-old man with ischemic dilated cardiomyopathy complained of frequently awakening from sleep due to choking; subsequent polysomnography revealed cheyne-stokes respiration (CSR) with sleep apnea. With continuous positive airway pressure (CPAP) through a nasal mask, both the CSR and symptoms disappeared. After 6-12 months, chest X-ray and echocardiographic findings continued to improve without any change in pharmacological treatment. For three years, CPAP had been effective to eliminate CSR during sleep. Long-term CPAP treatment, which is rarely applied for congestive heart failure in japan, is useful in alleviating the adverse effects of CSR and, thereby, maintaining a good quality of life in these patients.- - - - - - - - - - ranking = 1.25keywords = pressure (Clic here for more details about this article) |
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