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Biomedical subjects

A Carpentier

Publications and source records attributed to A Carpentier.

At least 109 records · Page 6Linked to original sources
PubMed1998

Mechanical modifications to human pericardium after a brief immersion in 0.625% glutaraldehyde.

BACKGROUND AND AIMS OF THE STUDY: The use of human pericardium pretreated for 10 min with 0.625% glutaraldehyde (GLUT) in valvular repair or intracardiac reconstruction has produced good results. However, to date, no investigations have been made to determine the mechanical changes that occur in the tissue following such pretreatment. METHODS: Human pericardial samples were harvested from 25 patients and immersed in GLUT for increasing times (5, 10, 30, 60 min and 6 months). Either untreated human pericardium or bovine pericardium treated for six months with GLUT served as controls. Tensile tests were performed with a uniaxial load machine and a pulsative bench. Fatigue testing was for 14 days; each sample was tested at 1,200 cycles/min at a controlled pressure of 90-120 mmHg. RESULTS: Untreated tissue thickness was 0.44+/-0.16 mm, but after six months GLUT treatment it was 0.53+/-0.15 mm (p<0.001). There was a 13.7% shrinkage of tissue after six months immersion. Strain was significantly greater in treated tissue than in untreated tissue, while stiffness decreased with the duration of GLUT immersion. Young's modulus was significantly lower after six months GLUT treatment (0.26+/-0.06 MPa) compared with untreated, and 5-, 10- and 30-min GLUT treatment (0.32+/-0.15, 0.35+/-0.09, 0.32+/-0.09 and 0.36+/-0.10 MPa (p<0.05)), respectively. Creep was greater after six months GLUT treatment (0.5+/-0.03%) than in untreated and 10-, 30- and 60-min treatments (0.3+/-0.50, 0.27+/-0.01, 0.27+/-0.02, 0.3+/-0.01% (p<0.05)), respectively. Ultimate tensile stress (UTS) was greater in 10-min treated pericardium than in untreated tissue: 38.46+/-11.75 versus 22.17+/-8.30 MPa (p<0.05) respectively. Strain at rupture was greater in the 6-month group (30.62+/-2.54%) than for untreated and 10-, 30- and 60-min GLUT immersion 16.3+/-0.73, 21.85+/-0.75, 20.12+/-1.04 and 18.87+/-0.86% (p<0.05), respectively. Fatigue testing showed an increased length after five and 10 min, and six months, with a lengthening of 14.66, 12.53, 7.66%, respectively compared with 3.5% for untreated tissue (p<0.05). There were three failures in the untreated group (n = 5), none in the 5- and 10-min groups, and one in the 6-month group (p<0.05). CONCLUSION: Brief immersion of human pericardial tissue in 0.625% glutaraldehyde reduces the tissue's stiffness and improves its durability for use in cardiac surgery.

Biomechanical Phenomena↗
PubMed1998

The activity of c-myb antisense oligonucleotide to prevent intimal hyperplasia is nonspecific.

BACKGROUND: We sought to determine the efficacy and specificity of a new c-myb antisense by inhibiting neointimal hyperplasia in a rat abdominal aorta injury model. Using c-myb antisense oligonucleotides, inhibition of vascular smooth muscle cell proliferation has been reported. METHODS: Sixty-six male Wistar rats had a de-endothelialization of the abdominal aorta. Following a double blind randomization protocol, F127 pluronic gel containing one of the five oligonucleotides or plain gel was applied around the aorta: 1) 18-mer c-myb antisense (AS18) with four contiguous guanosines (G-quartet); 2) 15-mer c-myb antisense (AS15) without G-quartet; 3) 1-bp mismatch AS15 without G-quartet (MM1); 4) an oligonucleotide with G-quartet (4G), whereas the other bases were chosen at random; 5) 1-bp mismatch 4G without G-quartet (MM2). After 21 days all rats were sacrificed and aortas harvested for histomorphometric evaluation. Four rats were given fluorescent-labeled oligonucleotides to study in vivo localization after local advential delivery. RESULTS: Morphometric analysis showed significant suppression of neointimal hyperplasia in AS18 and 4G and MM2 groups compared with GEL, AS15 and MM1 groups (p<0.05). The oligonucleotide-labeled aortas showed penetration of the oligonucleotides into the media which increased with time. CONCLUSIONS: Our findings pointed to the potential non specificity of the c-myb antisense oligonucleotide in vivo. Such results will minimize the importance of antisense strategy as a potential therapeutic for preventing neointimal hyperplasia. The two oligonucleotides with a G-quartet inhibited neointimal hyperplasia in our model. Exploring a non-antisense mechanism, G-quartet oligonucleotides as potential drugs to reduce neointimal hyperplasia is attractive.

Animals↗
PubMed1998

Preoperative localization of parathyroid lesions in hyperparathyroidism: relationship between technetium-99m-MIBI uptake and oxyphil cell content.

UNLABELLED: The aim of this study was to assess the relationship between parathyroid oxyphil cell content and early or late phases of uptake of 99mTc-MIBI, a radioisotope preferentially retained in mitochondria-rich cells. METHODS: This study is a retrospective, single-blind analysis of all double-phase 99mTc-MIBI parathyroid scintigraphy studies performed before surgery in our institution between 1990 and 1995. A total of 18 parathyroid lesions in 14 patients were reviewed. This sample included 11 cases of primary hyperparathyroidism (8 adenomas, 1 adenocarcinoma and 2 hyperplasias) and 3 cases of tertiary hyperparathyroidism secondary to chronic renal failure. RESULTS: Uptake of 99mTc-MIBI in the early phase of scintigraphy was associated with larger parathyroid lesions (1.61 +/- 1.61 ml versus 0.33 +/- 0.27 ml; p < 0.02) and higher serum calcium levels (3.00 +/- 0.41 mM versus 2.67 +/- 0.14 mM; p < 0.02). More importantly, we found that a parathyroid oxyphil cell content greater than 25% was more often associated with a positive uptake of 99mTc-MIBI in the late phase of the test (positive late uptake in 78% of lesions with a high oxyphil cell content versus 33% in lesions with an oxyphil cell content between 1% and 25% and 0% in lesions with no oxyphil cells; p < 0.04). CONCLUSION: These findings suggest that the late retention of 99mTc-MIBI in double-phase scintigraphy is related to parathyroid oxyphil cell content.

Adenoma↗
PubMed1998

[Cavo-pulmonary anastomosis associated with intracardiac repair of Ebstein anomaly. Value in high-risk patients].

The surgical prognosis of Ebstein's anomaly depends on the quality of tricuspid valve repair and right ventricular function. In patients with right ventricular failure, a decrease in afterload was attained by a cavo-bipulmonary anastomosis associated with the intraventricular repair. Fifty-nine out of 111 patients operated for Ebstein's anomaly were considered to be at high risk and were selected for this study. The inclusion criteria were one or more of the following factors: massive tricuspid regurgitation, extensive atrialisation of the right ventricle, poor right ventricular function, chronic atrial fibrillation. This population was divided into two groups with the same preoperative features: Group I (45 operated patients: tricuspid valvuloplasty with longitudinal plicature of the right ventricule: Group II (14 operated patients): same intracardiac repair as Group I and associated cavo-bipulmonary anastomosis. The operative mortality was 24% (11/45) in Group I and 7% (1/14) in Group II (p < 0.05). The 5 year actuarial was 68.6% in Group I and 61.8% in Group II (NS). The reoperation rate was 11% (5/45) in Group I and 0% in Group II. In Group II, the persistence of significant tricuspid regurgitation was better tolerated and the frequence of reoperation was decreased with respect to Group I. The authors conclude that high risk patients with Ebstein's anomaly have a lower operative mortality and improved functional tolerance when there is persistent tricuspid regurgitation after cavo-bipulmonary anastomosis.

Adolescent↗
PubMed1998

Aortic valve surgery in osteogenesis imperfecta: report of two cases and review of the literature.

Aortic insufficiency is a well known but uncommon valvular dysfunction in patients with osteogenesis imperfecta. In such cases, aortic valve surgery has rarely been performed, and carries a high risk of perioperative complications. We report two patients with osteogenesis imperfecta, who underwent elective successful aortic valve replacement. The surgical problems encountered in this connective tissue disorder are also reviewed.

Adolescent↗
PubMed1998

[Coronary reoperation: indications, methods and immediate postoperative prognosis. Retrospective study of 184 reoperations].

The authors performed 184 coronary reoperations (172 redux, 12 tridux) between January 1986 and december 1995 in 177 patients (165 men, 12 women) with an average age at surgery of 62.9 +/- 7.6 years. The average interval between the surgical procedures was 9.5 +/- 4.5 years. The symptoms were recurrent angina, stable in 44%, unstable in 51%, and cardiac failure, 7%. Graft dysfunction was the cause in 94.5% of cases with progression of atheroma of the native coronary vessels in only 5.6% of cases. At reoperation 389 bypass procedures were performed (venous 39.5%, arterial 60.5%) (2.1 +/- 0.6 per patient) with 10 associated procedures (3 mitral valvuloplasties, 2 left ventricular aneurysmectomy, 3 aortic valve replacements, 1 replacement of the ascending aorta, 1 carotid endarteriectomy). The operative mortality was 10.9%. The causes of the 20 deaths were myocardial infarction (7), left ventricular failure (8), arrhythmias (2), mediastinitis (1) and multi-organ failure (2). The risk factors for death were: the date of surgery (19% before 1991 and 8% after: p = 0.03), age (18% after 60 years, 2% before: p = 0.015), the interval between the surgical procedures (33% after 15 years, p = 0.02), anterograde cardioplegic injection alone (15% versus 4.5% when mixed antero and retrograde perfusion was used: p = 0.02). The morbidity was 28% (52/184 patients); 132 patients (72%) had uncomplicated postoperative courses. The incidence of repeat coronary artery surgery is in constant progression. Improved medico-surgical management should continue to reduce the mortality which is still high.

Aged↗
PubMed1997

Distinct sensitivity of neuroblastoma cells for retinoid receptor agonists: evidence for functional receptor heterodimers.

Retinoic acid (RA) plays a major role in embryogenesis of the nervous system and has been reported to induce differentiation in neuroblastoma cell lines. To identify RA signaling pathways involved in such differentiation processes, two RA-sensitive neuroblastoma cell lines (LA-N-5 and SH-SY5Y) were extensively studied. Northern blot experiments determined that of the three RAR mRNAs, only RARalpha was significantly expressed, with respectively weak or undetectable levels of RARgamma and RARbeta. RXRs (alpha and beta) receptors were weakly expressed. Western blotting analysis confirmed the constitutive expression of RARalpha and absence of RARbeta and weak levels of RXRalpha. Treatment with all-trans-RA up-regulated RARalpha and induced a drastic increase of RARbeta (both at the RNA and protein level). To further characterize the function of RARalpha, RARbeta and RXRalpha in NB cells, nuclear extracts from LA-N-5 cells were analysed by EMSA studies. Three specific retarded complexes were observed which were significantly decreased or shifted in the presence of monoclonal antibodies to RARalpha, RARbeta and RXRalpha. RA treatment dramatically induced a DR5-binding RXRalpha-RARbeta heterodimer. Treatment with combinations of RARalpha or RARbeta agonists with a RXRalpha agonist or with a RARalpha agonist alone, induced neurite-outgrowth supporting the probability that both RXRalpha-RARalpha or RXRalpha-RARbeta heterodimers are involved in RA-mediated differentiation of NB cells. The availability of novel synthetic RA-specific receptor ligands should provide the possibility of tissue specific therapeutic regimes.

Cell Differentiation↗
PubMed1997

Video-assisted aortofemoral bypass: results in seven cases.

Between September 1995 and February 1996 we attempted to perform video-assisted aortofemoral bypass in nine patients. All patients were male with a mean age of 58.7 years, mean weight of 64.7 kg, and mean height of 1.69 m. In two patients it was necessary to switch to open laparotomy due to inadequate aortic exposure in one and extensive aortic calcification in the other. Aortobifemoral bypass was performed by the transperitoneal approach in three patients and unilateral aortofemoral bypass by the retroperitoneal approach in four patients. Exposure was more difficult by the transperitoneal approach. Postoperative graft patency was excellent in all patients. Video-assisted surgery did not shorten the delay to resumption of intestinal transit but it did reduce the duration of hospitalization and need for postoperative analgesia. Our experience suggests that video-assisted aortofemoral bypass without laparotomy can be performed and that it allows more rapid patient recovery.

Adult↗
PubMed1997

Inhibition of intimal hyperplasia by an antisense oligonucleotide of farnesyl transferase delivered endoluminally during iliac angioplasty in a rabbit model.

The major complication of vascular recanalization is intimal hyperplasia which is due mainly to proliferation and migration of smooth muscle cells (SMC). Activation of SMC results from stimulation of protooncogens (c-myb, c-myc, c-fos) by growth factors induced by activated ras-proteins. Ras-proteins become activated after receiving a farnesyl group in a reaction catalyzed by famesyl transferase. The purpose of this study was to test the effectiveness in preventing intimal hyperplasia of an antisense oligonucleotide of the alpha subunit of farnesyl-transferase delivered endoluminally during angioplasty of the common iliac artery in rabbit model. Twenty-one male New Zealand rabbits with a mean weight of 3.3 kg fed a high cholesterol diet underwent bilateral angioplasty of the common iliac artery using hydrogel-coated balloon catheters. On the right side three types of treatment were randomly performed by adding one of the following three oligonucleotides to the hydrogel precoating:antisense oligonucleotide of farnesyl transferase (n = 7), mismatch oligonucleotide (n = 7), and scramble oligonucleotide (n = 7). On the left side hydrogel was used with saline so that each animal served as its own control. Animals were killed 6 weeks after angioplasty and arteries were studied. The thickness and mean surface of the neointima (MTI and MSI) and the ratio (R) of the neointima to neointima + media were calculated. In the scramble and mismatch groups there was no difference between the treated and control arteries with regard to MTI, MSI, or R. In the antisense group mean all three values were significantly lower on the treated side than the control side (EMI: p < 0.02, SMI: p < 0.02, and R: p < 0.01). Treated arteries in the antisense group presented significantly lower EMI (p < 0.02), SMI (p < 0.02), and R (p < 0.01) than treated arteries in the other groups whereas the thickness and mean surface of the media were comparable. Endoluminal administration of an antisense oligonucleotide against the alpha subunit of farneysyl transferase inhibited intimal hyperplasia in our model.

Alkyl and Aryl Transferases↗
PubMed1997

Osteopontin is associated with bioprosthetic heart valve calcification in humans.

Calcification of non-osseous tissues such as heart valves or vessels is a major concern in clinical practice. The exact mechanism is still unknown. Numerous studies have shown that mineral deposits of crystalline hydroxyapatite within these tissues were associated with increased non-collagenous protein content. More recently osteopontin was found to be associated with calcification in living tissues such as vessels and native human aortic valves. The aim of this study was to determine whether or not non-collagenous proteins can also be found in non-living tissues such as glutaraldehyde-pretreated porcine valves after implantation in humans. Thirty-eight glutaraldehyde pretreated porcine bioprostheses were studied: 16 not implanted and 22 after 11 years of implantation in the aortic and mitral valve position in humans. In areas of calcification vizualized by Von Kossa staining and microradiography, immunostaining using polyclonal antibodies against calcium-binding proteins showed osteopontin positive staining and no staining for osteocalcin, bone sialoprotein or osteonectin. In uncalcified areas and in non-implanted values, staining for osteopontin or other calcium-binding proteins was negative. Western blot analysis of macroscopically calcified and uncalcified areas showed that several proteins were adsorbed in implanted values and confirmed the presence of osteopontin in the calcified areas, while no immunolabelling was found in non-calcified areas, in uncalcified valves and in non-implanted valves. Thus the presence of osteopontin in the calcified areas of bioprosthetic heart valves implanted in human indicates that this protein is associated with bioprosthetic valvular calcification. Since these values are made of non-living connective tissue, and no cell immunostained for osteopontin was found around the calcified area, this suggests that a non-cellular mediated mechanism involving protein adsorption may play a role in bioprosthetic valvular calcification.

Bioprosthesis↗
PubMed1997

Dynamic cardiomyoplasty: clinical follow-up at 12 years.

OBJECTIVE: The purpose of this study is to evaluate the long-term outcome of dynamic cardiomyoplasty. This surgical technique was conceived to assist the failing heart. The many proposed mechanisms of action of cardiomyoplasty are: (1) systolic assist; (2) limitation of ventricular dilation; (3) reduction of ventricular wall stress (sparing effect); (4) ventricular remodeling with an active girdling effect; (5) angiogenesis; and (6) a neurohumoral effect. METHODS: We investigated 95 patients in our hospital undergoing this procedure due to severe chronic heart failure, refractory to optimal medical treatment. Patients had a mean age of 51 +/- 12 years. The etiology of heart failure was ischemic 55%, idiopathic 34%, ventricular tumor 6%, and other 5%. The mean follow-up was 44 months. RESULTS: The mean New York Heart Association (NYHA) functional class improved postoperatively from 3.2 to 1.8. Average radioisotopic left ventricular (LV) ejection fraction increased from 17 +/- 5 to 27 +/- 4% (P < 0.05). Stroke volume index increased from 32 +/- 7 to 43 +/- 8 ml/beat per m2 (P < 0.05). The heart size remained stable over the long term. Following cardiomyoplasty, the number of hospitalizations due to congestive heart failure was reduced to 0.4 hospitalizations/patient per year (preoperative: 2.5, P < 0.05). Computed tomography scans showed at long term a preserved latissimus dorsi muscle structure in 84% of patients. Survival probability at 7 years is 54%. Six patients underwent heart transplant after cardiomyoplasty (mean delay: 25 months), due to the natural evolution of their underlying heart disease. There were no specific technical difficulties. CONCLUSIONS: Clinically, this procedure reverses heart failure, improves functional class and ameliorates quality of life. The latissimus dorsi muscle histological structure is maintained at long-term, when postoperative electrostimulation is performed, avoiding excessive stimulation. Cardiomyoplasty may delay or prevent the progression of heart failure and the indication of cardiac transplantation.

Actuarial Analysis↗
PubMed1997

Early extubation after mitral valve surgery: a target-controlled infusion of propofol and low-dose sufentanil.

OBJECTIVE: In the current study, the use of a target-controlled infusion of low-dose propofol was combined with a continuous infusion of sufentanil for patients undergoing mitral valve surgery. The purpose of the study was to evaluate the hemodynamic stability, the time to awakening and spontaneous ventilation, and the feasibility in an early extubation setting of a total intravenous anesthetic technique. DESIGN: Prospective study. SETTING: University hospital. PARTICIPANTS: Fifteen patients scheduled for elective mitral valve surgery. INTERVENTIONS: Induction of anesthesia consisted of sufentanil (1 microgram/kg), propofol (1 microgram/mL) target plasma concentration achieved over 3 minutes, and atracurium (0.5 mg/kg). The propofol target-controlled infusion was maintained at 1 microgram/mL throughout surgery and stopped at skin closure. A continuous infusion of sufentanil at 1.8 micrograms/kg/hr was started after induction and reduced to 0.9 microgram/kg/hr at the start of cardiopulmonary bypass and stopped at the end of bypass. Atracurium was infused at a rate of 0.5 mg/kg/hr up to sternal closure. No inhalation agents were used. MEASUREMENTS AND MAIN RESULTS: Hemodynamic data were within normal limits. Six patients (40%) responded to verbal commands within 15 minutes postoperatively, 10 (67%) within the first hour, and all patients recovered within 2 hours. Four patients (27%) resumed spontaneous ventilation within the first 15 postoperative minutes. The time to successful spontaneous ventilation was 169 +/- 42 minutes. Spontaneous ventilation was associated with a 21% increase in cardiac index. Total sufentanil dose was 328 +/- 28 micrograms (4.6 +/- 0.2 microgram/kg), whereas total propofol dose was 862 +/- 44 mg (13.1 +/- 1.2 mg/kg). No patient required reintubation. CONCLUSION: The simplicity of the method with only one change in infusion rate is a major advantage. The technique permits predictable recovery and return to spontaneous ventilation in all patients. Its use in patients entering early extubation protocols is appealing for its reproducibility, simplicity, and safety.

Adult↗
PubMed1997

Normal human immunoglobulins for intravenous use (IVIg) delay hyperacute xenograft rejection through F(ab')2-mediated anti-complement activity.

Xenotransplantation between discordant species leads to a hyperacute rejection mediated by natural antibodies, both of the IgG and IgM isotypes, activation of complement and endothelial cell activation. The combination of these mechanisms leads to a transplant survival of minutes to a few hours. Polyclonal human immunoglobulins for intravenous use (IVIg) from normal donors have proved effective in a number of antibody-mediated disorders, as well as in inflammatory disorders. We demonstrate that administration of IVIg in a guinea pig to rat model of cardiac xenografting can effectively delay hyperacute rejection. This effect is mediated by the F(ab')2 fragments of IVIg, and is correlated to an anti-complementary activity.

Acute Disease↗
PubMed1997

Experimental bilateral lobar lung transplantation and its application in humans.

BACKGROUND: The critical lack of donor organs from people of small size or children has created great difficulties in transplantation for recipients who are of smaller size. Surgical techniques of organ reduction and partial transplantation may to some extent solve the problem of disparity in organ size, be it liver or lung, and lessen the problem of scarcity of paediatric organs. METHODS: In a series of experiments on dogs the surgical technique of pulmonary partition of a large organ from a grown dog followed by transplantation of lobes, either unilaterally or bilaterally, into a young dog was studied. Two series of experiments were performed in two groups of animals; in group 1 transplantation of a single right lobe (n = 6) or single left lobe (n = 6) from a split adult lung was carried out and in group 2 (n = 10) animals received bilateral lobar transplants from a single split adult lung. The animals were sacrificed at fixed intervals (days 8-120 in group 1, days 7-10 in group 2) and the results of the surgical technique were assessed. RESULTS: Healing of lobar bronchial anastomoses was found to be excellent with no histological evidence of dehiscence or ulceration. There was one bronchial anastomotic stenosis and one arterial thrombosis. Morphological and functional adaptation of the lobes in the thorax was found to be excellent in both groups of animals. The technique has been applied in a clinical setting and the first patient with bilateral lobar lung transplantation followed for 30 months is reported. CONCLUSION: Lung partition and subsequent lobar transplantation, either unilaterally or bilaterally, is associated with satisfactory early results in an animal experimental model. Initial clinical experience in one patient has been successful.

Adult↗
PubMed1997

Tris-hydroxymethyl aminomethane and sodium bicarbonate to buffer metabolic acidosis in an isolated heart model.

Metabolic acidosis induces a decrease in the developed force of cardiac muscle by affecting every step of the excitation--contraction coupling pathway. Due to transient worsening in intracellular acidosis, the value of administering sodium bicarbonate therapeutically during acute acidosis has been questioned. An alternative therapeutic drug, Tris-hydroxymethyl aminomethane (THAM) has the advantage of diffusing into the intracellular space. This study was designed to evaluate the effects of metabolic acidosis on myocardial performance and to determine the effects of alkalinization with sodium bicarbonate, THAM, and their combination. Using a blood-perfused isolated heart preparation, left ventricular contractility and relaxation were measured at normal pH and during metabolic acidosis (pH = 7.0). Acidosis dramatically impaired myocardial contractility and relaxation. After buffering with sodium bicarbonate, although plasma bicarbonate concentration was normalized, pH remained below normal owing to an increased PaCO2. Contractility and relation were initially worsened, then slightly improved to return to control values. THAM uncompletely buffered acidosis but significantly improved contractility and relaxation. The combination of THAM with sodium bicarbonate perfectly buffered acidosis without modifying PaCO2 and significantly improved contractility. The combination of THAM with sodium bicarbonate is based on the ability of THAM to capture the CO2 produced by the sodium bicarbonate buffer. This combination achieves a perfect correction of metabolic acidosis and improves myocardial performance.

Acidosis↗
PubMed1997

Study of muscular and ventricular function in dynamic cardiomyoplasty: a ten-year follow-up.

BACKGROUND: The basic physiologic principle underlying cardiomyoplasty is long-term electrostimulation of a latissimus dorsi muscle (LDM) wrapped around the heart to obtain a phasic activity that could be integrated with ventricular kinetics. The aim of cardiomyoplasty is to prolong survival and to improve the quality of life of patients with severe chronic and irreversible myocardial failure by improving systolic contraction and correcting diastolic dysfunction. METHODS: To evaluate the long-term outcome of cardiomyoplasty, we investigated 82 patients electively undergoing this procedure in-our hospital. All patients had severe chronic heart failure that did not respond to optimal medical treatment. Patients had a mean age of 50 +/- 12 years (84% males). The cause of heart failure was ischemic (55%), idiopathic cardiomyopathy (34%), ventricular tumor (6%), and other (5%). The mean follow-up was 4.3 years. RESULTS: The mean New York Heart Association functional class improved after operation from 3.2 to 1.8. Average radioisotopic left ventricular ejection fraction increased from 17% +/- 6% to 28% +/- 3% (p < 0.05). Stroke volume index increased from 35 +/- 9 to 46 +/- 8 ml/beat/m2 (p < 0.05). The heart size remained stable at long term (evaluated by echo and computed tomography scanning). After cardiomyoplasty the number of successive hospitalizations resulting from congestive heart failure was reduced to 0.4 hospitalizations/patient/year (before operation 2.5, p < 0.05). Computed tomography scans showed at long-term a preserved LDM structure in 82% of patients who underwent operation. Survival probability at 7 years was 54% for the totality of patients, and 66% for patients who underwent operation in New York Heart Association functional class 3. Five patients underwent heart transplantation after cardiomyoplasty (mean delay 29 months), principally as a result of the natural evolution of their underlying heart disease, without major technical difficulties. CONCLUSIONS: Our 10-year clinical experience demonstrates that cardiomyoplasty increases ejection fraction, improves functional class, and ameliorates quality of life. Ventricular volumes and diameters remain stable long term. LDM structure is maintained long term if electrostimulation is performed avoiding excessive myostimulation. Patient selection is the most important determinant for early and late outcome. Late death in patients undergoing cardiomyoplasty is principally due to sudden death. Our future aim is to incorporate a cardioverter-defibrillator in the cardiomyostimulator, thus improving long-term results. Cardiomyoplasty may delay or prevent end-stage heart failure and the need for heart transplantation.

Adolescent↗
PubMed1997

[2 familial cases of metachromatic leukodystrophy of late onset].

We report here a familial observation of metachromatic leukodystrophy (MLD) in 2 sisters. The very beginning, with only psychiatric manifestations at adolescence, could be precisely established. The evolution towards a dementia, and the evidence of a pyramidal syndrome oriented later towards a clearly organic disease. A very wide bilateral and symmetrical demyelination was shown by Magnetic Resonance imaging. The deficiency in arylsulfatase A activity oriented towards MLD which was confirmed by metachromatic deposits in the nerve biopsy. Molecular biology evidenced in the two, compound heterozygoty with both the classical mutation of the infantile form with loss of a splicing site at the level of intron 2, and the ileu > Ser 179 mutation frequent in adult forms.

Aged↗