1/11. Alkaptonuric aortic stenosis: a case report.alkaptonuria is a rare disease of phenylalanine, aromatic amino acids, and tyrosine metabolism. Because of a genetic deficiency of the enzyme homogentisic acid oxidase, an accumulation of homogentisic acid causes ochronotic pigment deposition. The most common clinical manifestations are arthropathy, urinary calculi and discoloration, cutaneous and cartilaginous pigmentation, and cardiac valvular disease. Arthropathy and aortic stenosis are the most debilitating manifestations of the disease. A case of alkaptonuric aortic stenosis is described. A 75-year-old woman with a history of alkaptonuria presented in the emergency department with complaints of progressive dyspnea. Upon examination, the patient was hypertensive, tachypneic, and tachycardic with premature ventricular contractions. She had pitting edema of the lower extremities and complaints of generalized weakness. Chest x-rays revealed congestive heart failure and pulmonary edema. diuretics were administered, and a continuous nitroglycerin infusion was initiated in the emergency department. The patient was admitted for further evaluation. The patient's respiratory status continued to decline. She was intubated endotracheally 1 day after admission. Subsequent cardiac evaluation revealed an ejection fraction of 35%, severe aortic stenosis, mild coronary artery disease, ischemic cardiomyopathy, and anteroapical akinesis. A dobutamine infusion was instituted for persistent hypotension, and renal dose dopamine was initiated for oliguric renal failure. The patient underwent an emergency operation for an aortic valve replacement with a Dacron patch 10 days after admission. cardiopulmonary bypass and mild hypothermia were used during the procedure. The patient's hemodynamic status remained tenuous throughout the procedure. Although the first attempt to wean off cardiopulmonary bypass failed, the second attempt was successful with the aid of an intra-aortic balloon pump, inotropic support, and atrioventricular pacing. These measures were maintained during transport to the surgical intensive care unit. In the intensive care unit, the patient did not have an audible blood pressure or a palpable pulse without the support of the intra-aortic balloon pump and atrioventricular pacing. Coarse atrial fibrillation was the underlying electrocardiogram rhythm in the absence of atrioventricular pacing. sodium bicarbonate was given without improvement. After discussion with the family, all life support measures were discontinued. The patient died 10 minutes after her arrival in the intensive care unit. alkaptonuria's pathogenesis is manifested as both local and systemic in nature. collagen vascular diseases share a similar pattern of multisystem involvement. Despite the negative outcome for the patient described, valuable insight can be obtained by studying this case and noting the anesthetic considerations specific to collagen vascular diseases in general.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/11. azoospermia due to testicular amyloidosis in a patient with familial mediterranean fever.We describe a patient suffering from familial mediterranean fever (FMF) who presented to our clinic with secondary infertility of 2 years due to amyloid A amyloidosis. His spermiogram disclosed azoospermia. A testicular biopsy revealed hyalinized tubules devoid of full spermatogenesis and containing abundant amyloid, confirmed by congo red stain. We suggest that testicular amyloidosis be taken into consideration when dealing with azoospermic FMF patients. In view of the progressive nature of amyloid accumulation in the testis we propose to follow routinely the spermiogram of FMF patients with renal amyloidosis. Furthermore, consideration of sperm cryopreservation is suggested in these cases. In FMF patients with azoospermia consideration of testicular biopsy is recommended as early as possible in order to increase the chance of sperm retrieval.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/11. Combined paravertebral lumbar plexus and parasacral sciatic nerve block for reduction of hip fracture in a patient with severe aortic stenosis.PURPOSE: To report the use of a combined paravertebral lumbar plexus and parasacral sciatic nerve block for reduction of hip fracture in an elderly patient with severe aortic stenosis. Clinical features: In an 87-yr-old lady with severe aortic stenosis and fracture of the right trochanter due to a fall, a combined right-sided paravertebral lumbar plexus and parasacral sciatic nerve block was used successfully for operative reduction of the fracture. A moderate amount of phenylephrine was required to maintain adequate systemic blood pressure despite the largely unilateral nature of the blocks. CONCLUSION: Combined paravertebral lumbar plexus and parasacral sciatic nerve block can be a viable alternative to general anesthesia and epidural or spinal block for hip surgery in patients with severe aortic stenosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/11. A cluster of cases of aspergillus endocarditis after cardiac surgery.aspergillus endocarditis is an ominous condition whose prevalence is increasing in the hospital population. Despite the life-threatening nature of the disease, detection of the source, establishment of the diagnosis, and treatment remain highly challenging. A cluster of three cases of aspergillus endocarditis recently encountered at the Montreal Heart Institute are presented.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/11. Continuous wave Doppler signal: a mystery.Doppler echocardiography is an established noninvasive cardiovascular investigational tool. However, the physical nature of the ultrasound beam may lead to unexpected findings in routine echocardiographic examination, which may lead to misdiagnosis and subsequent wrong clinical decisions.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/11. Iatrogenic left main coronary artery stenosis following PTCA or valve replacement.We report three patients who developed iatrogenic severe left main coronary artery stenosis. In two, it was secondary to coronary cannulation during aortic valve replacement and in one it followed distention of the artery during balloon dilatation of a proximal lesion in the left anterior descending artery. In all three, the stenosis was clinically manifest a few months after the intervention. All were successfully treated by aortosaphenous coronary bypass. A common mechanism for the three cases may be mechanical distention of the left main coronary artery resulting in intimal damage with secondary fibrosis and stenosis. The percutaneous transluminal coronary angioplasty-related stenosis is, to our knowledge, the first reported case of this nature, and represents a previously unrecognized complication of this procedure.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/11. Severe left ventricular dysfunction in critical aortic stenosis--reversal following aortic valve replacement.A case of aortic valve stenosis is reported with progressive left ventricular dysfunction demonstrated by radionuclide angiography. A gradient of 120 mmHg was present across the aortic valve. At the time of surgery, the patient was in severe cardiac failure with a left ventricular ejection fraction of 22%. Two months postoperatively, the patient was asymptomatic, and repeat radionuclide angiography demonstrated an ejection fraction of 93%. The reversible nature of this patient's ventricular dysfunction suggests that the cardiac failure was related to 'afterload mismatch' caused by the stenotic valve, rather than due to depressed contractility. The encouraging result in this patient reinforces the view that surgical intervention is warranted in some patients with critical aortic stenosis and extreme impairment of left ventricular function, and that the close relation between ejection fraction and operative survival may not hold true in aortic stenosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/11. Calcific stenosis of a glutaraldehyde-treated porcine bioprosthesis in the aortic position.The porcine xenograft bioprosthesis is used widely at the present time, although its long-term durability is still being evaluated. Calcification resulting in stenosis of the glutaraldehyde-treated bioprosthesis has been reported infrequently. In the patient described herein, severe calcific stenosis of a porcine xenograft in the aortic position occurred 45 months after implantation. This report emphasizes the life-threatening and rapidly progressive nature of this complication in the long-term survivor after valve replacement.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/11. The williams syndrome: evidence for possible autosomal dominant inheritance.We recently evaluated a mother and son with the williams syndrome. documentation of the clinical phenotype in two generations of this family suggests that some cases of the williams syndrome are autosomal dominantly inherited. Recognition of the heritable nature of the williams syndrome should prompt careful clinical evaluation of other at-risk relatives in order to provide accurate recurrence risk counseling.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/11. Fixed subaortic stenosis associated with hypertrophic cardiomyopathy: report of a rare familial occurrence.INTRODUCTION: Fixed subaortic stenosis is considered to be an acquired condition. It is often associated with congenital heart disease, creating a turbulence in the left ventricle outflow tract. Familial forms of fixed subaortic stenosis are very unusual. We report a remarkable familial cluster in which fixed subaortic stenosis is associated with hypertrophic cardiomyopathy. methods: Fourteen relatives of a patient affected with hypertrophic cardiomyopathy and fixed subaortic stenosis underwent cardiological examination, electrocardiogram and echo-doppler study. RESULTS: Two of the proband's sisters showed an association between asymmetrical hypertrophic cardiomyopathy and fixed subaortic stenosis. The brother presented a subaortic ridge and concentric left ventricular hypertrophy. The other members of the family (another brother and the third-generation relatives) were unaffected. CONCLUSIONS: While the association between fixed subaortic stenosis and hypertrophic cardiomyopathy has commonly been reported, there is little in the literature to suggest the family-related nature of this association. The familial occurrence of this association reveals genetic transmission, with a recessive autosomal pattern of inheritance. This finding goes against the usual autosomal dominant pattern of inheritance in hypertrophic cardiomyopathy. Familial studies of FSS are needed in order to gain a better understanding of the genetic background of these patients.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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