[A case report of Williams and Beuren syndrome with associated aggravation of in situ distal pulmonary vascular obstruction due to thrombotic disease]. / Une observation de syndrome de Williams et Beuren avec aggravation de l'obstruction vasculaire pulmonaire distale par maladie thrombotique in situ associée.

We report a case of Williams and Beuren syndrome in which the obstruction of the pulmonary vascular bed, linked with peripheral stenoses of the pulmonary arteries, associated here with an interventricular communication, was aggravated due to the coexistence of in situ thrombotic disease, leading to a progressive hypoxaemia, requiring long-term anticoagulant treatment, and compromising the long-term prognosis. This case report puts into question the so-called favourable prognosis of pulmonary arterial branch stenoses in this malformation syndrome.

[Diagnosis of cardiac tumor by percutaneous thromboaspiration. A case report]. / Diagnostic d'une tumeur cardiaque par thromboaspiration percutanée. A propos d'un cas.

Percutaneous endovascular thromboaspiration is a valuable tool as illustrated by the case of a patient suffering from a large intracardiac tumour. Histological and immuno-histochemical analysis of the tumour fragments provided the diagnosis of a cardiac angiosarcoma. The patient was a 44 year old man admitted for a large sero-sanguinous pericardial effusion which recurred after drainage. The case was complicated by a haemorrhagic cerebrovascular accident unrelated to a secondary deposit. Initially suspected after transthoracic echocardiography, the diagnosis of a tumour invading the right atrium was confirmed by transoesophageal echocardiography and cardiac CT scan. Surgery was declined and as the diagnosis of lymphoma could not be excluded, the patient underwent biopsy by an original method of percutaneous thromboaspiration. This minimally invasive, low cost technique would appear to be a valuable alternative to other endovascular biopsy techniques (saber, biotome) and to surgical biopsy, and could be proposed as the technique of first intent in an a priori non-operable intra-cardiac tumour or when lymphoma is suspected.

[Reversible left intraventricular obstruction after treatment of pheochromocytoma and hyperthyroidism]. / Obstruction intraventriculaire gauche réversible après traitement d'un phéochromocytome et d'une hyperthyroïdie.

The authors report an original case of the association of three pathologies: pheochromocytoma, hyperthyroidism and cardiomyopathy with left ventricular outflow tract obstruction. This type of cardiac disease has occasionally been described in cases of pheochromocytoma and are usually induced by the endocrine disturbance because they regress with treatment of the pheochromocytoma. The associated hyperthyroidism observed in this case is very rare and may have increased the left ventricular pressure gradient. Medical treatment before surgery of the pheochromocytoma was unusual in that a triple therapy was used including betablockers, classically contra-indicated in pheochromocytoma alone. In this case, it provided excellent control of the blood pressure and decreased the left ventricular obstruction during the perioperative period.

[Effects of aging on left ventricle-arterial coupling in man]. / Effets du vieillissement sur le couplage "fonctionnel" ventricule gauche-système artériel chez l'homme.

Twenty-five normotensive men without any cardiac or arterial pathology, aged 22 to 68 years, 12 less than 45 year old, 13 over 45 years, underwent cardiac catheterisation and angiography. The following parameters were calculated: 1) a global index of arterial function (Ea) and its determining factors (Ea = LVESP/SV where LEVSP = left ventricular end systolic pressure and SV = left ventricular stroke volume); Ea = (HR x SVR) + Ea' where HR = heart rate, SVR = total systemic vascular resistance and Ea' = (LVESP - MAP/SV) (MAP = mean arterial pressure); 2) an index of global left ventricular pump function: ELV (ELV = LVESP/LVESV, where LVEDV = left ventricular end systolic volume; 3) an index of LV-arterial coupling: the Ea/ELV ratio. With aging, both Ea (by increase in SVR) and Ea' and ELV increased significantly. Ea/ELV (inverse of the ejection fraction-1) increased with age but ELV less than Ea. Ea/ELV was significantly higher in patients over 45 years of age but the correlation between ejection fraction and age was not statistically significant (p = 0.10). These results suggest that with aging, the improvement in LV pump function approximately corresponds to the degradation in arterial transport function: the left ventricular-arterial coupling as assessed by the Ea/ELV ratio (and therefore the ejection fraction) is maintained in the majority of cases.

[An unusual complication of percutaneous aortic valvuloplasty: rupture of an aberrant mitral chorda]. / Une complication exceptionnelle de la valvuloplastie aortique percutanée: la rupture d'un cordage mitral aberrant.

The case of a 77-year old woman who died of refractory pulmonary oedema 36 hours after percutaneous valvuloplasty for tight calcified aortic valve stenosis is reported. Post-mortem examination showed satisfactory opening of the aortic orifice but also rupture of an aberrant chorda which crossed the outflow tract below the aortic sigmoid valves, between the mitral valve and the interventricular septum. This case suggests that before all aortic valvuloplasties the absence of aberrant chorda or suspicious subaortic acceleration should be confirmed by Doppler echocardiography; moreover, during the procedure the balloon should not be inserted too deeply into the left ventricle.

[Evaluation of static elasticity and characteristic impedence of the aorta. Their relationships with age, aortic pressure and ventricular ejection resistance]. / Evaluation de l'élasticité statique et de l'impédance caractéristique de l'aorte. Ses relations avec l'âge, la pression aortique et la résistance à l'éjection ventriculaire

The static elasticity (Ep) (Peterson) and the characteristic impedance of the aorta (Zo) (Mac Donald) were evaluated by the simultaneous measurement of the pressure and diameter of the ascending aorta in 30 patients: 18 subjects were free from any aortic pathology (Group I), subjects had persistant arterial hypertension (Groupe II), and 3 subjects had aortic incompetence (Group III). In the group I patients, Ep increased significantly with age, and with the aortic pressure and diameter. In groupe II, Ep increased significantly only with aortic pressure. A narrow correlation was found between the characteristic impedance of the ascending aorta and the systolic ejection resistance, except in those patients with aortic incompetence, in whom it seems that Zo is a better measure of ejection resistance. In the group I patients, impedance increased significantly with age. No significant difference was found in the characteristic impedence between patients of groups I and II, the aortic diameter being greater in the latter group.

[Changes in the physical properties of the arterial system and left ventricular performance with age and in permanent arterial hypertension: their interrelation]. / Modifications des propriétés physiques du système artériel et de la performance ventriculaire gauche avec l'âge et au cours de l'hypertension artérielle permanente: leurs interrelations.

The purpose of this work was to study interactions between physical properties of the arterial system and left ventricular performance during aging in normal and in hypertensive patients. 50 patients were studied; 28 normal patients (age range 22 to 68 years) and 22 patients with essential hypertension (age range 23 to 63 years). In hypertensive patients, the end systolic pressure-volume ratio (ESP/ESV), modulus of chamber stiffness (kp), left ventricular wall thickness (h), mass (m), m/LVEDV ratio (LVEDV: left ventricular end diastolic volume), systemic arterial resistance (SAR), pulse wave velocity (C) and characteristic impedance of the ascending aorta (Zc) were increased compared to normal subjects of similar age. The ejection fraction (EF), the mean velocity of fiber shortening (VCF) and dp/dt max were unchanged. In normal patients: the ESP/ESV ratio, kp, h, m, m/LVEDV ratio, SAR, C and Zc increased with age; there were no age related changes in EF, VCF or dp/dt max. In both groups, there was a close relationship between the m/LVEDV ratio and Zc, the characteristic impedance of the ascending aorta. These results suggest that: 1. aging and arterial hypertension lead to similar changes in the physical properties of the arterial system and in left ventricular performance; 2. in both cases, the development of concentric cardiac hypertrophy is closely related to the physical properties of the arterial system.

[Clinical application of the alkalosis induction test for coronary artery spasm]. / Application clinique du test de provocation par l'alcalose du spasme artériel coronaire.

Alkalosis was used for stress testing for coronary artery spasm in 70 patients (average age: 56 years) with resting angina. A rapid intravenous infusion of an alkaline buffer (THAM) immediately followed by 5 minutes' maximal ventilation increased the arterial pH to 7.67 +/- 0.5. Anginal pain and ECG changes were observed in 24 Patients, with ST elevation in 10 cases and ST depression in 14 cases. The ischaemic changes occurred during hyperventilation in 16 cases and in the 3 minutes following the test in 8 cases. The heart rate increased from 66 +/- II to 71 +/- 14 bpm (p less than 0,01) but systolic blood pressure fell from 139 +/- 12 to 130 +/- 12 mm Hg during hyperventilation; there was no significant change in the rate-pressure product (1130 +/- 1750 to 8990 +/- 2690). In all cases, the angina and ischaemic changes regressed after intravenous Trinitrin. Coronary angiography was performed in 56 patients: in the 24 patients with positive responses (Group I) and in 30 of the 46 patients with negative responses (Group II). Significant coronary artery narrowing (greater than 70%) was observed in 21 patients of Group I: in the 3 patients without coronary lesions an intravenous injection of 0.4 mg methylergometrine provoked coronary spasm. In Group II, significant narrowing was demonstrated in 18 patients: in the 12 other patients, coronary spasm could not be induced by methylergometrine. Therefore, in the absence of organic coronary lesions, an excellent correlation has been shown between the alkalosis and methylergometrine tests. This stress test was repeated in 16 of the 24 patients in Group I one hour after administration of 20 mg of Nifedepine: the test was negative in all cases. We conclude that the alkalosis test could be useful in the coronary care unit as a stress test for coronary spasm to determine the antianginal treatment of choice and to evaluate its efficacity.

[Hemodynamic and coronary effects of intravenous verapamil in coronary insufficiency]. / Effets hémodynamiques et coronaires du vérapamil intraveineux dans l'insuffisance coronaire.

Verapamil inhibits calcium influx through the slow calcium canals. The coronary an haemodynamic effects of intravenous Verapamil were studied in 8 patients with chronic coronary insufficiency documented by coronary arteriography. The following measurements were made in spontaneous rhythm and during atrial pacing under basal conditions and 10 minutes after intravenous Verapamil (0.10 to 0.17 mg/kg) relayed with a continuous infusion of 5 x 10(-3) mg/Kg/mn: heart rate, cardiac output, left ventricularr pressure (Millar 5 F micromanometer), femoral artery pressure, coronary sinus flow by continuous thermodilution, oxygen and lactate concentrations in arterial and arterio-venous oxygen difference, and index of myocardial oxygen consumption and the coefficient of lactate extraction were then calculated. The coronary and haemodynamic effects of atrial pacing were similar before and after Verapamil at a given rate. Left ventricula end diastolic pressure decreased, cardiac output and total systemic resistance were unchanged, dP/dt max increased but to a lesser degree after Verapamil (P less than 0.05). Coronary arterio-venous oxygen difference decreased after Verapamil. The coronary and haemodynamic effects of Verapamil were similar in spontaneous rhythm and during atrial pacing. In spontaneous rhythm, the heart rate and left ventricular end diastolic pressure increased. In spontaneous and paced rhythm, femoral artery pressure, total systemic resistance and dP/dt max decreased. Cardiac output remained the same. Myocardial oxygen consumption decreased mainly because of a reduced coronary arterio-venous oxygen difference and because of unchanged coronary flow in spontaneous rhythm oxygen consumption seems to have a favourable effect on the myocardial energy equilibrium as shown by the increased coefficient of lactate extraction during atrial pacing after Verapamil. This study shows the negative inotropic and arterial vasodilator effects of Verapamil to be responsible for the reduced myocardial oxygen consumption. It also caused coronary artery vasodilation.

[A platypnea-orthodeoxia syndrome]. / Un syndrome platypnée-orthodéoxie.

We report the case of a 68 years old patient with platypnea orthodeoxia syndrome who has been clinically suspected on cutaneous saturation position's variation. It has been confirmed by transthoracic and transesophageal echocardiography (TEE). TEE showed the size of patent foramen ovale (PFO), visualised the right to left shunt. A right heart angiography confirmed echocardiographic data and allowed successful closure by a Cardioseal percutaneous transcatheter button device implantation. Symptom's disappearance and a TEE control after 2 months confirmed the success of the procedure.

[Rupture of a post-infarct ventricular aneurysm. Apropos of 3 cases cured surgically]. / Rupture d'anévrysme ventriculaire post-infarctus. A propos de trois cas guéris chirurgicalement.

The authors report 3 cases of post-infarction left ventricular aneurysm with localised rupture into the pericardium. The patients (3 men aged 54, 58 and 67 years old) had left ventricular aneurysms (2 anterior, I posterior) which ruptured early, between the second and tenth week. All presented with cardiogenic shock. The diagnosis was made by 2D echocardiography (I case) or by pericardial aspiration and angiography (2 cases). Good results were obtained by surgical evacuation of the hemopericardium and resection of the ventricular aneurysm with a follow-up of 3 to 12 months. The rarity of these cases is underlined: only three other similar cases appear to have been previously reported.

[Left ventricular hypertrophy in patients with hypertension. A comparative study between black Africans and white Europeans]. / L'hypertrophie ventriculaire gauche chez l'hypertendu. Une étude comparative entre des sujets Noirs africains et Blancs européens.

OBJECTIVE: The objective of this study was to compare clinical and ultrasonographic findings in 20 consecutive hypertensive Black Africans, with those of 20 hypertensive White Europeans matched for age in order to study modifications of left ventricular (VG) geometry. METHOD: 20 consecutive Black men (B), recently emigrated from Africa and applying for asylum, referred for HT, were assessed by measuring systolic blood pressure (SBP), diastolic blood pressure (DBP) and pulse pressure (PP), body mass index (BMI), echocardiography with measurement of the end-diastolic thickness of the septum, posterior wall (Epp), LV diameter (DTD), calculation of the LV mass index (LVMI) according to the Penn convention and the thickness/radius ratio (t/r). These subjects were matched for age with 20 consecutive European White men (W) sent to the echocardiography laboratory for assessment of HT, in whom the same parameters were measured. RESULTS: The mean age was 41.6 +/- 9.2 (B) vs 42.1 +/- 8.8 years (W) (NS). The BMI was 26.3 +/- 3.2 (B) vs 27.3 +/- 3.4 (W) (NS). SBP was 175 +/- 24 (B) vs 156 +/- 15 mmHg (W) (p < 0.01). DBP was 108 +/- 13 (B) vs 94 +/- 9 mmHg (W) (p < 0.01). PP was 67 +/- 20 (B) vs 63 +/- 10 mmHg (W) (NS). LVMI was 131 +/- 43 (B) vs 91 +/- 19 g/m2 (W) (p = 0.001). The t/r ratio was 0.48 +/- 0.08 (B) vs 0.38 +/- 0.07 (W) (p < 0.001). DTD was 47.1 +/- 3.9 (B) vs 48.6 +/- 4.4 mm (W) (NS). Epp was 11.2 +/- 1.9 (B) vs 9.1 +/- 1.4 mm (W) (p < 0.001). A poor correlation was observed between SBP and LVMI (r = 0.23, p = 0.17), and between SBP and t/r (r = 0.21, p = 0.21). CONCLUSION: Compared to a age-matched population of European hypertensive patients with similar morphology, our homogeneous group of recently emigrated patients was characterized by a greater severity of HT and the presence of concentric left ventricular hypertrophy. The marked difference in socio-economic status and access to care of these two populations must be stressed.

[Effects of sublingual nifedipine on the velocity of pulse wave evaluated by Doppler echocardiography of the thoracic aorta]. / Effets de la nifédipine sublinguale sur la vélocité de l'onde de pouls évaluée par échocardiographie Doppler de l'aorte thoracique.

Sublingual nifedipine has a rapid and powerful hypotensive effect. Its action on arterial distensibility can be assessed by measurement of pulse wave velocity (PWV) by Doppler echocardiography (DE). Ten patients were used to evaluate intra-observer reproducibility (Group 1). Fifteen hypertension patients (Group 2), not taking a calcium antagonist, 64 +/- 13 years-old, were studied by DE before and 15 minutes after 10 mg of SL nifedipine. The following were measured: systolic and diastolic blood pressures (SBP and DBP), heart rate (HR), echocardiographic shortening fraction (SF) and PWV, with sternal length (SL) taken as being identical to the length of the abdominal aorta between the isthmus and the coeliac region, and by measuring delta t: time interval for propagation of the pulsed Doppler velocimetric wave between the isthmic aorta and the coeliac aorta located by echocardiography: PWV = SL/delta t. Evidence was found in the nifedipine group (n = 15) of a fall in SBP (159.5 +/- 27.8 cf. 140.9 +/- 21 mmHg; p = 0.002); in DBP (88.6 +/- 13 cf. 79.6 +/- 8.3 mmHg; p = 0.005) and an increase in HR (72.3 +/- 10.7 cf. 76.8 +/- 12.9 bpm; p = 0.04). PWV decreased after nifedipine (10.5 +/- 3.9 cf. 7.15 +/- 12.9 bpm; p = 0.002). There was a linear correlation between the percentage reduction in PWV and left ventricular HR: y = 194x - 53; r = 0.56; p = 0.029.(ABSTRACT TRUNCATED AT 250 WORDS)

[Coronary insufficiency in hypercholesterolemic xanthomatosis. Angiographic aspects]. / L'insuffisance coronaire de la xanthomatose hypercholestérolémique. Aspects angiographiques.

Coronary arteriography for severe coronary artery disease was carried out in 21 patients with hypercholesterolaemic xanthomatosis (group I) and in 42 patients with serum lipids (group II). Both groups were matched with regard to age, sex and cardiovascular risk factors (hypertension, smoking habits, diabetes). The films were examined by an observer unaware of the patients' clinical features; the severity of the lesions observed was assessed by means of a coronary score system. Group I patients had significantly more severe coronary artery lesions and a significantly higher incidence (52% versus 7%; p less than 0.001) of left main coronary artery stenosis carrying a high risk of sudden death. The percentages of affected vessels amenable to surgery and abnormalities in left ventricular function were similar in both groups. It therefore seems reasonable to envisage early coronary angiography followed, if necessary, by aorto-coronary bypass in patients with hypercholesterolaemic xanthomatosis who experience anginal attacks, show ECG abnormalities on exercise and have persistent pain after myocardial infarction.

[Arterial hypertension in black Africans. Clinical and echographic study in 50 patients]. / Hypertension artérielle du noir Africain. Etude clinique et échographique chez 50 sujets.

OBJECTIVES: Clinical and electrocardiographic and echocardiographic data were collected in 50 black Africans with hypertension to study modifications in left ventricular geometry. METHODS: Thirty men and 20 women with hypertension (mean age 40.9 +/- 10.2 years) who had recently immigrated from Africa were included in the study. Parameters recorded were: systolic and diastolic pressures, body mass index (BMI = weight/height2); ECG; echocardiography (36 patients): end diastolic septal and posterior wall thickness, left ventricle diameter, left ventricle mass (LVM) and relative wall thickness. RESULTS: Systolic and diastolic pressures and BMI were 169 +/- 24 mmHg, 105 +/- 14 mmHg and 26.7 +/- 3.9 kg/m2). LVM was 129 +/- 41 g/m2 (Normal < 134 g/m2) in men and 113 +/- 35 g/m2 (Normal = 110 g/m2) in women. Concentric remodeling was found in 8 patients (LVM = normal, relative wall thickness > or = 0.45), concentric left ventricle hypertrophy in 10 (LVM > normal, relative wall thickness > or = 0.45), excentric left ventricular hypertrophy in 6 (LVM > normal, relative wall thickness < 0.45), asymmetric septal hypertrophy in 4 and normal echocardiogram in 8. There was a negative correlation between age and end diastolic left ventricle diameter (r = -0.34, p < 0.05) and age and end systolic ventricle diameter (r = -0.74, p < 0.05). There was a positive correlation between body surface and septal thickness (r = 0.34, p < 0.05), posterior wall thickness (r = 0.56, relative wall p < 0.001), and relative wall thickness (r = 0.45, p = 0.05). CONCLUSION: This population of young subjects with hypertension showed a high prevalence of modifications in left ventricular geometry with predominant concentric modeling with (8/36) and without (10/36) left ventricle hypertrophy.

[Functional coupling of the left ventriculo-arterial system and aging in man]. / Couplage "fonctionnel" ventricule gauche-système artériel et vieillissement chez l'homme.

In a haemodynamic and angiographic study at rest conducted in 25 men aged from 22 to 68 years who had no more than another risk factor for arterial disease and showed no evidence of cardiac or arterial disease several parameters were measured or calculated. Firstly, a global index of arterial system function (Ea) and its various factors: Ea = ESP/SV [ESP: left ventricular end-systolic pressure; SV: systolic volume]; Ea = (HR x TSR)+Ea' [HR: heart rate/min; TSR: total systemic resistance] where Ea' = (ESP - AoP/SV) [AoP: mean aortic pressure]. Secondly, the parameters concerning the left ventricle were the mass (m) and the m/EDV ratio [EDV: end-diastolic volume] and indices of the left ventricular systolic and diastolic functions, such as ELV = ESP/ESV [ESV: end-systolic volume], kp: the volumic distensibility module of the left ventricular chamber; relations EF - o ES [EF: ejection fraction; o ES: end-systolic constraint], and kp - m/EDV as indice of left ventricular muscle distensibility. In parallel with the subjects' age, Ea increased by joint augmentation of TSR and Ea' while m, m/EDV, ELV and kp also significantly increased. The inotropic quality of the left ventricular muscle and its intrinsic distensibility were found to be decreased in a few subjects aged over 45. Ea/ELV (reverse of ejection fraction -1) tended to increase (ELV relatively less than Ea), but this increase was not significant (P = 0.10). These results show that in the ageing man the improvement observed in the ejection fraction of the left ventricular pump corresponds roughly to the degradation of the arterial system transfer function, and the arterial system-left ventricle coupling, evaluated by the Ea/ELV ratio, is maintained (better in fact than in arterial hypertension and heart failure). This improvement is achieved by increases of m and, chiefly, m/EDV which compensate for both the increase of Ea and the relative decline of left ventricular muscle contractile quality. There is a disorder of the left ventricular pump diastolic function which is due to geometric changes in the chamber and to changes in the intrinsic distensibility of the left ventricular muscle.

[Ischemic cardiomyopathy: remodeling, hypertrophy, subendocardial risk. Can processes be controlled?]. / La myocardiopathie ischémique: remodelage, hypertrophie, sous-endocarde à risque. Des processus que l'on peut enrayer?

Ischaemic cardiomyopathy reflects the myocardial dysfunction caused by coronary disease. It results from the association of 1. segmental infarction(s) responsible for ventricular "remodelling", i.e. expansion of the necrotic area(s) and hypertrophy-dilatation of the rest of the ventricle, eventually concurring to heart failure; 2. areas which are viable but with a function that is reversibly compromised by severe acute or chronic ischaemia (myocardial sideration or hibernation) affecting mainly the subendocardium. The spontaneous course of cardiomyopathy towards the worst can be arrested by 1. revascularisation of the myocardium at risk by coronary reperfusion performed either as an emergency in case of infarct in the process of formation, or after detection of the viable myocardial areas by isotopic methods; 2. prevention or limitation of ventricular remodelling by coronary reperfusion and improvement of the ventricular load by administration of angiotensin-converting enzyme inhibitors and nitroglycerin. The Survival and Ventricular Enlargement study (SAVE) has been the first to demonstrate the relationship between limitation of ventricular remodelling and improvement of the secondary prognosis of infarction obtained by angiotensin-converting enzyme inhibitors.