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Biomedical subjects

G Jondeau

Publications and source records attributed to G Jondeau.

At least 37 records · Page 2Linked to original sources
PubMed2000

[Dystrophic aortic insufficiency].

Dystrophic aortic regurgitation is the result of 2 diseases: (1) aortic regurgitation, consequence of (2) aortic dilatation due to decreased aortic wall resistance. Marfan syndrome, which is a genetic disease, should be looked for systematically, with the help of an ophthalmologist and a rheumatologist. Aortic dilation is responsible for the increased mortality because of aortic dissection. Diagnosis is often made when the aorta is dilated wheras the aortic regurgitation is minimal or moderate; when the patient is asymptomatic. This has 2 consequences: siblings of Marfan patient should be examined by echocardiography; surgical replacement of the ascending aorta is often performed because of the aortic dilation, not because of the aortic regurgitation.

Aortic Valve Insufficiency↗
PubMed2000

Near-maximal fractional oxygen extraction by active skeletal muscle in patients with chronic heart failure.

Systemic oxygen uptake and deep femoral vein oxygen content were determined at peak exercise in 53 patients with chronic heart failure with impaired systolic function (mean left ventricular ejection fraction 0.18; n = 41) or preserved systolic function (mean left ventricular ejection fraction 0.70; n = 12) and in 6 age-matched sedentary normal subjects. At peak exercise, deep femoral vein oxygen content in heart failure patients with impaired systolic function and preserved systolic function were similar, both significantly lower than that of normal subjects (2.5 +/- 0.1, 2.9 +/- 0.2, and 5.0 +/- 0.1 ml/100 ml, respectively; P < 0.05). Deep femoral venous oxygen content was lower in patients with the greater impairment of aerobic capacity, regardless of the underlying systolic function (r = 0.72, P < 0.01). Fractional oxygen extraction in the skeletal muscle at peak exercise is enhanced in patients with chronic heart failure when compared with normal subjects, in proportion to the degree of aerobic impairment.

Aged↗
PubMed2000

[Aortic dissection at 6 months gestation in a women with Marfan's syndrome. Simultaneous Bentall intervention and cesarean section].

A 31 year old woman with Marfan's syndrome had a dilatation of the aortic root (55-60 mm at the beginning of pregnancy). Pregnancy was continued with beta-blocker therapy and with regular echocardiographic follow-up. The aortic dilatation increased (62-65 mm) at the last control and, at the 34th week of pregnancy, the patient suffered a dissection of the ascending aorta. A caesarean section was performed with a Bentall procedure during the same operative session. The mother and baby girl are well two years later. The problems of pregnancy in patients with Marfan's syndrome are discussed.

Adrenergic beta-Antagonists↗
PubMed2000

Marfan syndrome and fibrillin disorders.

Marfan syndrome is the second most common inherited connective tissue disorder after osteogenesis imperfecta. Musculoskeletal abnormalities are at the forefront of the clinical picture and count among the major diagnostic criteria for Marfan syndrome, together with cardiovascular and ocular system involvement. Early diagnosis is of the utmost importance since preventive measures significantly increase life expectancy and prevent the occurrence of impairments and disabilities. Marfan syndrome is due to mutations within the fibrillin-1 gene, which is the main protein of the microfibril network. Microfibrils play a crucial role in the trophicity and function of elastic tissue. Multidisciplinary management of the patients and their families is vital.

Extracellular Matrix Proteins↗
PubMed1999

Central pulse pressure is a major determinant of ascending aorta dilation in Marfan syndrome.

BACKGROUND: In patients with Marfan syndrome (MFS), brachial pulse pressure (PP) has been recognized as a risk factor for aortic dilatation, leading to aortic dissection, the main cause of premature death. However, the relationships between aortic PP, aortic stiffness, and aortic root dilation have not been investigated. Our main objective was to determine whether central PP, which takes into account wave reflections and aortic stiffness, is a better determinant of ascending aorta diameter than brachial PP in MFS patients. METHODS AND RESULTS: Twenty patients with confirmed MFS and 20 age- and sex-matched control subjects were included in this cross-sectional, noninvasive study. Elastic properties of the abdominal aorta and common carotid, common femoral, and radial arteries were calculated from the pulsatile changes in arterial diameter and pressure. The ascending aorta diameter, measured with conventional echocardiography, was 37% larger in MFS than in control subjects (P<0.001). Arterial distensibility was 38% lower in MFS than in control subjects at the site of the abdominal aorta (P<0.01) but not at other sites (common carotid, common femoral, and radial arteries). Independently of age and body surface area, ascending aorta diameter was positively correlated with carotid PP in MFS (P<0. 01) and negatively in control subjects (P<0.01) but was not correlated with brachial PP and mean blood pressure. CONCLUSIONS: In patients with MFS, local PP, estimated from carotid PP, was a major determinant of ascending aorta diameter, whereas brachial PP was not. Increased arterial stiffness was confined to the aorta.

Adult↗
PubMed1999

Addition of angiotensin II receptor blockade to maximal angiotensin-converting enzyme inhibition improves exercise capacity in patients with severe congestive heart failure.

BACKGROUND: Incomplete suppression of the renin-angiotensin system during long-term ACE inhibition may contribute to symptomatic deterioration in patients with severe congestive heart failure (CHF). Combined angiotensin II type I (AT1) receptor blockade and ACE inhibition more completely suppresses the activated renin-angiotensin system than either intervention alone in sodium-depleted normal individuals. Whether AT1 receptor blockade with losartan improves exercise capacity in patients with severe CHF already treated with ACE inhibitors is unknown. METHODS AND RESULTS: Thirty-three patients with severe CHF despite treatment with maximally recommended or tolerated doses of ACE inhibitors were randomized 1:1 to receive 50 mg/d losartan or placebo for 6 months in addition to standard therapy in a multicenter, double-blind trial. Peak aerobic capacity (V(O2)) during symptom-limited treadmill exercise and NYHA functional class were determined at baseline and after 3 and 6 months of double-blind therapy. Peak V(O2) at baseline and after 3 and 6 months were 13.5+/-0.6, 15.1+/-1.0, and 15.7+/-1.1 mL. kg-1. min-1, respectively, in patients receiving losartan and 14.1+/-0.6, 14.3+/-0.9, and 13.6+/-1.1 mL. kg-1. min-1, respectively, in patients receiving placebo (P<0.02 for treatment group-by-time interaction). Functional class improved by at least one NYHA class in 9 of 16 patients receiving losartan and 1 of 17 patients receiving placebo. CONCLUSIONS: Losartan enhances peak exercise capacity and alleviates symptoms in patients with CHF who are severely symptomatic despite treatment with maximally recommended or tolerated doses of ACE inhibitors.

Adult↗
PubMed1999

Bone mineral density in sixty adult patients with Marfan syndrome.

Sixty adult patients (40 women, 20 men) with Marfan syndrome (MFS) according to the Berlin criteria had a full clinical examination and bone mineral density (BMD) measurement by dual-energy X-ray absorptiometry of the hip and nondominant forearm. BMD was expressed as a Z-score and compared with the reference population of the Hologic database. In MFS men, BMD (g/cm(2)) was compared with the BMD of 45 normal tall Caucasian adults. Osteocalcin was measured by radioimmunoassay. In patients with MFS, BMD was compared between patients with and without previous fractures and according to the phenotypic severity of MFS. The mean age of the patients was 32.9 +/- 9.3 years (women 32.5 +/- 9.7, men 33.4 +/- 8.6), mean height was 180.3 +/- 10.3 cm (women 176.3 +/- 9.2, men 188.1 +/- 7.5) and mean body mass index 20.9 +/- 3.6 kg/m(2) (women 20.8 +/- 3.4, men 20.95 +/- 3.97). Hyperlaxity score (Beighton criteria) was 6.9 +/- 1. 1. Six patients (10%) had a previous fracture. Thirty per cent of patients had had at least one previous operation for scoliosis, aortic dilatation or eye problems. BMD values in the 60 patients were as follows: Z-score of the hip, -1.26 +/- 0.93, p<10(-9) (neck, -0.93 +/- 1.09, p<10(-9); trochanter, -1.31 +/- 0.85, p<10(-9); intertrochanter, -1.39 +/- 0.99, p<10(-9); Ward's triangle, -0.93 +/- 1.88, p<10(-9)); Z-score of the radius: -1.6 +/- 1.06, p<10(-9) (1/3 proximal, -1.29 +/- 1.03; mid-radius, -1.94 +/- 1.04; ultradistal, -0.68 +/- 1.1, p<10(-9)). The decrease in BMD was similar in men and women at both the hip and the radius. BMD in MFS patients was significantly decreased at cortical compared with trabecular sites (radius 1/3 proximal vs ultradistal, p<0.0001; total femur vs Ward's triangle, p<0.0005). No difference in BMD was found between MFS patients with or without previous fractures and those with severe or less severe phenotypic expression of MFS. An influence of height and weight in MFS on BMD is suspected. Osteocalcin was not increased in our group of MFS patients. Thus both men and women with MFS have a significant deficit of BMD at the hip and radius. The decrease in BMD is present equally in both sexes and is more pronounced at predominantly cortical sites. In our group of patients we found no increase in fractures and no relation between decreased BMD and phenotypic expression of the syndrome.

Absorptiometry, Photon↗
PubMed1999

[Evaluation of a specific French scale of activity in chronic heart failure. A national multicenter study. Group for Cardiac Insufficiency and Cardiomyopathy of the French Society of Cardiology].

Many systems have been proposed to evaluate the functional incapacity caused by chronic cardiac failure. The classification of the New York Heart Association (NYHA) is the best known. It is subjective, poorly reproducible and has a poor predictive value on effort. The authors propose a Specific French Scale of Activity with the object of a more accurate functional evaluation of cardiac failure, easier to use by the doctor and more specific to French patients and their life styles. A French multicentre study was set up in hospital departments by the French Society of Cardiology working group on Cardiomyopathy and Cardiac Failure to assess this new classification with respect to the NYHA classification and peak VO2 (Weber's classification). Eight centres participated in the study. A total of 124 patients with chronic cardiac failure and a mean age of 61 years (102 men) were included. Cardiac failure was due to ischaemic heart disease in 72 cases, hypertension in 10 cases, dilated cardiomyopathy in 40 cases and aortic regurgitation in 2 cases. Eighty-two patients underwent a double evaluation using the French Scale: 40 patients by 2 physicians and 42 patients by a physician and a nurse. Good reproducibility was found between the assessment by the 2 physicians in 35 cases (87%) and between the physician and nurse in 30 cases (71%). When compared with peak VO2, the classification was concordant in 47% of cases using the NYHA and in 61% of cases using the French Scale, with variation of one class in 40% of cases with the NYHA and 35% of cases with the French Scale. These results show good reproducibility and correspondence of classification with the exercise test which was better using the French Scale than the NYHA classification.

Aged↗
PubMed1998

[Value of multidisciplinary consultation in diagnosis and survival of Marfan syndrome].

OBJECTIVES: To facilitate diagnosis, define a strategy for prevention and treatment and obtain further insight concerning Marfan's disease, we conducted a series of multidisciplinary consultations in French clinics. PATIENTS AND METHODS: Five specialists (a genetics specialist, a pediatrician or a rheumatologist, an ophthalmologist, and a psychologist) saw all the patients successively in the same clinic. A synthesis of the clinical files was prepared for analysis. Since the first consultation on 1 January 1995 and the study end on 30 June 1997, 494 patients (67% adults) participated in primary consultations, 143 were seen at follow-up consultations. RESULTS: The diagnosis of Marfan's disease was affirmed in 41% of the patients, disaffirmed in 48% and uncertain in 11%. Among the Marfan's patients, 75% were seen again within the framework of a multidisciplinary follow-up consultations. Annual work-up included cardiology (echocardiography with measurement of the proximal aorta diameter in search for indication for beta-blocker therapy or preventive valve replacement), ophthalmology (lens, retina) and rheumatology examinations (skeletal involvement). CONCLUSION: Early diagnosis and rigorous follow-up can help prevent ocular and cardiac complications in Marfan's disease. The multidisciplinary approach provides more precise data for diagnosis and possible phenotype-genotype correlations.

Adult↗
PubMed1998

[The value of transesophageal echocardiography for the diagnosis of pulmonary embolism with acute pulmonary heart disease].

Transoesophageal echocardiography is a method of visualising intracardiac thrombi and could therefore be useful for the diagnosis of pulmonary embolism, but its diagnostic value is unknown. The authors carried out a prospective study with this diagnostic tool in massive pulmonary embolism. The study protocol was to perform transthoracic echocardiography in patients with suspected acute pulmonary embolism and then to perform transoesophageal echocardiography when there were signs of acute cor pulmonale. The results of both echocardiographic investigations were compared with two reference radiological techniques: the spiral CT scan and/or pulmonary angiography. Fifty-six patients underwent transthoracic echocardiography. In the 34 patients with transthoracic echocardiographic signs of acute cor pulmonale, the positive predictive value of the investigation for pulmonary embolism was 91% and the negative predictive value was 54%. Twenty of these 34 patients underwent transoesophageal echocardiography. The sensitivity and specificity for the diagnosis of proximal embolism were 85% and 86% respectively. The limitations of the method were poor visualisation of the left pulmonary artery in which only one thrombus was detected, compared with 6 by spiral CT scan, and the absence of visualisation of lobar arteries. Consequently, the real sensitivity of transoesophageal echocardiography for visualisation of all thrombi in the pulmonary arteries in acute cor pulmonale was only 55%. In acute cor pulmonale, the diagnostic value of transoesophageal echocardiography is poor because the sensitivity for visualisation of intra-pulmonary arterial thrombi is low compared with other radiological techniques. However, in patients with proximal emboli in the right or main pulmonary artery, the diagnosis may be established in a few minutes without the need of other more invasive techniques. Nevertheless, normal transoesophageal echocardiography does not rule out the presence of proximal in the left pulmonary artery or distal emboli in the lobar arteries.

Acute Disease↗
PubMed1998

[The echocardiographic examination in adult cardiomyopathy].

Echocardiography allows distinction between the diastolic dysfunction of hypertrophic or restrictive cardiomyopathies and the systolic dysfunction of dilated cardiomyopathy. The diagnosis and prognosis may be deduced from echocardiographic parameters. In hypertrophic cardiomyopathy systolic function is normal and there is asymmetric left ventricular hypertrophy (> 13 mm) associated with a reduced diastolic dimension and atrial dilatation resulting from diastolic dysfunction. The prognosis could be related to the severity of left ventricular hypertrophy; right ventricular hypertrophy is uncommon and its severity seems to be related to that of left ventricular hypertrophy. Restrictive cardiomyopathies are less common and amyloidosis is the commonest cause. Symmetric hypertrophy with reduced diastolic dimensions is observed; right ventricular involvement occurs in about 30% of cases. The prognosis seems to be related to the degree of parietal infiltration and, at advanced stages, systolic function is abnormal (fractional shortening < 20% with a left ventricular diastolic dimension > or = 55 mm) and rapidly fatal. Dilated cardiomyopathy is diagnosed when wall thickness is normal but left ventricular diastolic dimensions > 27 mm/m2 and ejection fraction < 45%. Right and left ventricular dimensions of the same size, left ventricular diastolic dimensions > 70 mm and left ventricular ejection fractions < 20% are poor prognostic indicators.

Adult↗
PubMed1997

Relation of functional improvement in congestive heart failure after quinapril therapy to peripheral limitation.

Angiotensin-converting enzyme inhibitors have been shown to increase maximal muscle blood flow in parallel to peak VO2 in patients with congestive heart failure (CHF). Whether this increase shifts factors limiting peak aerobic capacity from periphery (skeletal muscle or vessels) to central factors (cardiac or respiratory) is unknown. Comparison of peak oxygen consumption (VO2) obtained during leg cycling (VO2 leg) with peak VO2 obtained during combined leg cycling and arm cranking (VO2 arm + leg) allows determination of the relative role of central or peripheral factors. We compared VO2 leg with VO2 arm + leg before and after 3 months of therapy with quinapril 40 mg in 16 patients with CHF (age 53 +/- 13 years) due to left ventricular systolic dysfunction (ejection fraction 0.25 +/- 0.07). Before quinapril, VO2 arm + leg was significantly higher than VO2 leg (19.0 +/- 3.3 vs 16.9 +/- 3.8 ml/kg/min, p < 0.001), whereas after therapy these 2 values were similar (20.3 +/- 4.3 vs 21.0 +/- 4.3 ml/kg/min; p = NS), indicating that patients were no longer limited by peripheral factors. Besides, VO2 leg increase after therapy was higher in patients in whom difference between VO2 arm + leg and VO2 leg was the greatest (i.e., in patients who were initially more limited by peripheral factors). Simultaneously, calf peak reactive hyperemia and circumference significantly increased, indicating an improvement in vascular dilating capacity and an increase in skeletal muscle mass. No significant modification occurred in the forearm. Thus, patients who improved the most after 3 months of quinapril therapy were those who were initially limited by peripheral factors. The restricting role of these factors was reduced after quinapril therapy.

Angiotensin-Converting Enzyme Inhibitors↗
PubMed1997

Familial ligand-defective apolipoprotein B-100: simultaneous detection of the ARG3500-->GLN and ARG3531-->CYS mutations in a French population.

Familial ligand-defective apolipoprotein B-100 (FDB) is an autosomal dominant disorder leading to plasma LDL cholesterol elevation and coronary artery disease (CAD). Two specific mutations in the APOB gene--R3500Q and R3531C--induce FDB. We report an original method to detect both mutations simultaneously, based upon PCR-mediated, site-directed mutagenesis and double restriction of a unique PCR product. With this method we have investigated the prevalence of these mutations in 1,040 French patients. The R3500Q mutation was found in five probands. Genotypes were determined for 10 APOB polymorphic markers and were consistent with the common European ancestral haplotype previously reported. The only exception was one FDB proband who did not harbor the 48 repeat allele of the 3'HVR. Additionally, the first two R3531C mutations were identified in French probands. Genotypes were consistent with a previously reported haplotype, suggesting that this is another mutation of European ancestry.

Adult↗
PubMed1997

Angiotensin II-receptor blockade further reduces afterload safely in patients maximally treated with angiotensin-converting enzyme inhibitors for heart failure.

Combined therapy with an angiotensin-II type I receptor (AT1) antagonist and an angiotensin-converting enzyme (ACE) inhibitor results in more complete suppression of the renin-angiotensin system. Accordingly, the blood-pressure response and safety of combining AT1-receptor blockade with losartan for ACE inhibition were evaluated in patients with congestive heart failure who were already treated with maximally recommended or tolerated doses of an ACE inhibitor. Forty-three patients with symptomatic congestive heart failure were evaluated biweekly for 1 month before addition of losartan and weekly during administration of losartan at a daily dose of 25 mg for the first week and 50 mg for the second week. Systolic blood pressure, which remained unchanged before addition of losartan, decreased from 122 +/- 18 mm Hg to 112 +/- 17 and 107 +/- 17 mm Hg (p < 0.001) after 1 week of 25 mg and 1 week of 50 mg losartan, respectively. Diastolic blood pressure also significantly decreased. The decreases in blood pressure were well tolerated by all patients, even by those in whom symptomatic hypotension developed during uptitration of ACE inhibition. Serum potassium and sodium and parameters of renal function remained unchanged. Combining AT1-receptor blockade with losartan to maximally recommended or tolerated ACE inhibition appears safe and leads to further vasodilatation in symptomatic patients with congestive heart failure.

Aged↗
PubMed1997

Left main coronary artery compression during primary pulmonary hypertension.

Primary pulmonary hypertension (PPH) is often associated with angina-like chest pain, the mechanism of which is controversial. A 37-year-old woman with severe PPH and angina had transient ischemic ECG changes and reversible anterior perfusion defect on 201thallium scintigraphy. Coronary angiography revealed severe stenosis of the left main coronary artery (LMCA) and otherwise normal vessels. After heart-lung transplantation, examination of the explanted heart showed normal coronary arteries. Compression of the LMCA by the dilated pulmonary artery trunk was responsible for myocardial ischemia. This mechanism should be considered in patients with PPH and angina and might contribute to the high sudden death rate.

Adult↗
PubMed1997

[Marfan's syndrome. Cardiovascular manifestations and therapeutic indications].

Marfan's syndrome is a disease of collagen microfibrils, the essential composition of which, fibrillin, is defective. The coding gene of this large protein has been localised on chromosome 15 and partially cloned. Its mutation is transmitted in the autosomal dominant mode. It is common, affects many organs, especially those rich in collagen. They include the skeleton, the eye and cardiovascular system. The prognosis of these patients depends on the degree of cardiovascular involvement; the aorta is the principal site of the lesions, particularly the aortic root which tends to develop aneurysmal dilatation, then dissection, the main cause of premature death of these patients. Mitral valve prolapse giving rise to mitral regurgitation may also be observed. Much progress has been made is recent years in the management of Marfan's syndrome, especially in ultrasound follow-up of the aortic root and the definition of risk factors for dissection. In parallel, advances have been made in the treatment, both medical with the use of betablockers to try to slow progression to aneurysm, and surgical, in replacement of the aorta and aortic valve in time and under optimal conditions. Life expectancy is thereby significantly increased. Similar progress has been made to allow these patients to have a pregnancy, classically thought to carry a high risk of maternal death.

Adrenergic beta-Antagonists↗