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1.
J Chem Phys ; 139(14): 144303, 2013 Oct 14.
Artigo em Inglês | MEDLINE | ID: mdl-24116615

RESUMO

We report new experimental data for the Rb2 a(3)Σu(+) and 2(3)Π0g states obtained using the Perturbation Facilitated Infrared-Infrared Double Resonance (PFIIDR) technique. The results include ro-vibrational term values of the 2(3)Π0g state and resolved fluorescence spectra of the 2(3)Π0g→a(3)Σu(+) transitions for a wide range of rotational and vibrational quantum numbers. An analysis of these data confirms the initial assignment of the transitions to the a(3)Σu(+) state reported in our earlier work [B. Beser, V. B. Sovkov, J. Bai, E. H. Ahmed, C. C. Tsai, F. Xie, L. Li, V. S. Ivanov, and A. M. Lyyra, J. Chem. Phys. 131, 094505 (2009)]. The potential energy functions of the Rb2 a(3)Σu(+) and 2(3)Π0g states are derived from a simultaneous fit of the available experimental data. The improved potential function of the Rb2 a(3)Σu(+) state spans both the attractive and repulsive regions starting with internuclear distance R ∼ 4.5 Å.

2.
J Chem Phys ; 135(2): 024303, 2011 Jul 14.
Artigo em Inglês | MEDLINE | ID: mdl-21766938

RESUMO

Sets of experimental data on the Cs(2) a(3)Σ(u)(+) and 1(g) (3(3)Π(1g)) states, including the bound-bound and bound-free fluorescence spectra, are analyzed simultaneously to produce the potential energy curves of both states in the form of the Morse long range multiparameter function. The attractive branch of the a(3)Σ(u)(+) state potential is improved relative to the one reported in our earlier work [F. Xie, V. B. Sovkov, A. M. Lyyra, D. Li, S. Ingram, J. Bai, V. S. Ivanov, S. Magnier, and L. Li, J. Chem. Phys. 130, 051102 (2009)], in which the data on this state alone were analyzed. Besides, the new potential of this state also includes the repulsive branch in the range spanned by the bound-free fluorescence spectra. We have not found experimental evidence of the double minimum character of the 3(3)Π(1g) state potential, predicted by ab initio calculations, at least up to v = 8. This fact testifies that the upper state observed is better described by the Hund coupling case (c), in which the case (a) electronic basis states are intermixed by the strong spin-orbit interaction.

3.
J Chem Phys ; 130(24): 244307, 2009 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-19566153

RESUMO

A theoretical investigation of the electronic structure of the K(2) molecule, including spin-orbit effects, has been performed. Potential energies have been calculated over a large range of R up to 75a(0) for the 88 Omega(g,u)(+/-) states dissociating adiabatically into the limits up to K(4p (2)P(3/2))+K(4p (2)P(3/2)). Equilibrium distances, transition energies, harmonic frequencies, as well as depths for wells and heights for barriers are reported for all of the bound Omega(g,u)(+/-) states. Present ab initio calculations are shown to be able to reproduce quite accurately the small structures (wells and barrier) displayed at very long-range (R>50a(0)) by the (2,3)1(u) and (2)0(g)(-) purely long-range states. As the present data could help experimentalists, we make available extensive tables of energy values versus internuclear distances in our database at the web address http://www-lasim.univ-lyon1.fr/spip.php?rubrique99.

4.
J Chem Phys ; 130(5): 051102, 2009 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-19206950

RESUMO

We have observed the vibrational levels v(") = 0-40 of the Cs(2) a (3)Sigma(u)(+) state by perturbation facilitated infrared-infrared double resonance excitation and spectrally resolved fluorescence measurements, and derived a multiparameter Morse long range potential and molecular constants based on these data.

5.
J Chem Phys ; 129(17): 174301, 2008 Nov 07.
Artigo em Inglês | MEDLINE | ID: mdl-19045341

RESUMO

We report a comparison between experimental and theoretical electronic transition dipole moment values for the (7)Li(2) A (1)Sigma(u) (+)-X (1)Sigma(g) (+) system. The experimental results are based on measuring the absolute magnitude of the transition dipole matrix elements from Autler-Townes splitting of rovibrational transitions for different R-centroid values. The ab initio theoretical calculations of the transition dipole moment for the (7)Li(2) A (1)Sigma(u) (+)-X (1)Sigma(g) (+) system were performed using two different quantum-mechanical models: an all-electron valence bond self-consistent-field method and a pseudopotential molecular orbital method. As expected for the smallest molecule with core electrons, the agreement between experiment and theory is very good.

6.
Neuromuscul Disord ; 3(1): 31-42, 1993 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-8392409

RESUMO

Two brothers, aged 27 and 20 months, born from consanguineous healthy parents, presented with cardiomyopathy, lactic acidosis and carnitine abnormalities in serum and muscle, without clinical evidence of muscle involvement. The histochemical reaction for cytochrome c oxidase (COX) activity was negative in all muscle fibres, although the holoenzyme and subunits were present at a normal level, as shown by immunocytochemistry. The COX activity was, respectively, 5 and 25% of control values, in muscle biopsies. Partial deficiency of complex IV was confirmed in fresh isolated muscle mitochondria from patient 2 and was associated with a defect of complex I. Patient 1 died at age 3 yr 6 months. Partial improvement of cardiomyopathy in patient 2 was obtained under carnitine therapy, but seizures occurred and CT scan and magnetic resonance imaging (MRI) revealed thalamic hypodensity. Thus, the disorder appears to be progressive despite the clinical stabilization of the cardiomyopathy. This further demonstrates the complexity and clinical heterogeneity of combined respiratory chain complex deficiencies.


Assuntos
Cardiomiopatias/genética , Deficiência de Citocromo-c Oxidase , Mitocôndrias Cardíacas/enzimologia , Mitocôndrias Musculares/metabolismo , Músculos/patologia , NAD(P)H Desidrogenase (Quinona)/deficiência , Biópsia , Cardiomiopatias/enzimologia , Cardiomiopatias/patologia , Carnitina/metabolismo , Consanguinidade , Complexo IV da Cadeia de Transporte de Elétrons/metabolismo , Feminino , Humanos , Lactente , Masculino , Mitocôndrias Musculares/enzimologia , Mitocôndrias Musculares/ultraestrutura , Músculos/ultraestrutura , NAD(P)H Desidrogenase (Quinona)/metabolismo , Consumo de Oxigênio , Valores de Referência , Succinato Desidrogenase/análise
7.
Ann Thorac Surg ; 61(4): 1251-3, 1996 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-8607698

RESUMO

Diffuse supravalvular aortic stenosis can be treated by a variety of surgical approaches. In this case of severe diffuse supravalvular aortic stenosis in a child, we used the combination of an apicoaortic conduit followed 6 years later by aortic valve replacement, replacement of the ascending aorta and aortic arch, and an ascending to thoracic descending aorta bypass graft.


Assuntos
Síndrome de Williams/cirurgia , Adolescente , Aorta Torácica/cirurgia , Valva Aórtica , Prótese Vascular , Próteses Valvulares Cardíacas , Humanos , Masculino , Polietilenotereftalatos , Reoperação/métodos , Síndrome de Williams/diagnóstico
8.
Eur J Pediatr Surg ; 10(2): 142-4, 2000 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-10877087

RESUMO

A case of thoracic extra-adrenal pheochromocytoma was observed in a 13-year-old girl. The initial clinical symptoms consisted of her being unwell after effort over a period of 3 years. The diagnosis was suggested after a recorded episode of paroxysmal ventricular tachycardia with concomitant hypertension, which reproduced the characteristic symptoms. Biochemical analysis confirmed hypersecretion of catecholamines and morphological examinations showed the presence of an isolated left median thoracic tumor. One year after surgical excision, the patient remained completely asymptomatic, in spite of resuming intense sporting activity. The diagnosis of ectopic pheochromocytoma should be considered as a possible etiology of cardiac rhythm disturbances or hypertension in children. Thorough biochemical and radiological examination including MIBG radioisotope scanning should be carried out systematically before surgical excision; pheochromocytomas in children are frequently extra-adrenal (30%), multifocal (50%) or associated with multiple endocrine neoplasms (NEM).


Assuntos
Neoplasias do Mediastino/diagnóstico , Feocromocitoma/diagnóstico , Taquicardia Ventricular/etiologia , Adolescente , Feminino , Humanos , Neoplasias do Mediastino/complicações , Feocromocitoma/complicações
9.
Arch Mal Coeur Vaiss ; 74(5): 609-14, 1981 May.
Artigo em Francês | MEDLINE | ID: mdl-6794483

RESUMO

Seven out of 348 cases of Fallot's tetralogy (2 %) referred over an eight year period were complicated by aortic incompetence (AI). The incidence of AI was much lower than in VSD (7.6 %), probably because of the prevailing haemodynamics: in high VSD, the left-to-right shunt may damage the aortic cusps whilst in Fallot's tetralogy the output of both ventricles is ejected directly into the aorta with no alteration of aortic valve function. In this series, infective endocarditis was the predominant cause of AI (3 cases); surgery had to be undertaken in the infectious phase in 2 cases. AI was coincidental in two other cases (rheumatic valvular disease and calcified bicuspid valve). In the remaining two cases, the role of increased aortic flow in late distension of the aortic valve ring is discussed. AI should be corrected in the same operative time as the Fallot's tetralogy. Late onset AI occurring after repair of Fallot's tetralogy should also be corrected; in this situation, it is often difficult to diagnose the origin of the diastolic murmur. Without surgery the prognosis is poor as the diastolic regurgitation occurs in a ventricle whose function is often already compromised.


Assuntos
Insuficiência da Valva Aórtica/etiologia , Tetralogia de Fallot/complicações , Adolescente , Adulto , Insuficiência da Valva Aórtica/diagnóstico , Insuficiência da Valva Aórtica/fisiopatologia , Insuficiência da Valva Aórtica/cirurgia , Endocardite Bacteriana/complicações , Feminino , Comunicação Interventricular/complicações , Comunicação Interventricular/fisiopatologia , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Febre Reumática/complicações
10.
Arch Mal Coeur Vaiss ; 72(1): 98-106, 1979 Jan.
Artigo em Francês | MEDLINE | ID: mdl-107888

RESUMO

The choice of method of contraception in cardiac patients is often peremptory, as combined oestrogen-progesterone preparations and intra uterine devices are often contraindicated. A pure progesterone mini-pill, lynoestrenol (500 microgram) has been used in our department for several years, and would appear to be a possible solution. Its daily, uninterrupted administration for 6 to 30 months in 40 cardiac patients, many considered to be high risk cases (28 cases), has confirmed its contraceptive action: totally effective, excellent reversibility and satisfactory acceptability despite definite menstrual changes. Above all, it was almost totally innocuous, an essential factor in cardiac patients. No haemodynamic, hypersensitive or thromboembolic side effects were observed in any pateint. No changes were observed in glucose or lipid metabolism, hepatic function or blood coagulation after 3 to 6 months, in 6 to 14 patients at high risk. Platelet aggregation, factors II, V, VII and X, fibrinogen and anti thrombin II were normal. Although this is a small series of patients, the use of microdosage lynoestrenol seems an acceptable method of oral contraception for cardiac patients, providing they collaborate and are closely followed up from the cardiac point of view.


Assuntos
Cardiopatias , Hemodinâmica/efeitos dos fármacos , Linestrenol , Adolescente , Adulto , Avaliação de Medicamentos , Feminino , Seguimentos , Cardiopatias/fisiopatologia , Humanos , Linestrenol/administração & dosagem , Linestrenol/efeitos adversos
11.
Arch Mal Coeur Vaiss ; 86(5): 639-41, 1993 May.
Artigo em Francês | MEDLINE | ID: mdl-8257277

RESUMO

Cardiorespiratory adaptation of congenital atrioventricular block is not well known. The authors present the case of a 20 year old patient with excellent muscular force and maximal aerobic capacity despite severe chronotropic insufficiency on exercise, the heart rate not exceeding 80/mn. This condition may be explained by increased peripheral oxygen uptake and an increased oxygen transport due to high hemoglobin level.


Assuntos
Adaptação Fisiológica , Bloqueio Cardíaco/congênito , Adulto , Aptidão , Flutter Atrial/fisiopatologia , Gasometria , Teste de Esforço , Bloqueio Cardíaco/fisiopatologia , Humanos , Masculino , Prognóstico , Esportes
12.
Arch Mal Coeur Vaiss ; 69(7): 711-9, 1976 Jul.
Artigo em Francês | MEDLINE | ID: mdl-821442

RESUMO

A haemodynamic and 2-plane cine-angiographic study of 23 patients with obstructive cardiomyopathy has shown that the mitral valve has a limited opening capacity, an unusually anterior position in early systole and is displaced anteriorly during systole. Mitral incompetence is present in more than half of the cases, but usually remains as the only lesion. When it is severe, it is because there are additional lesion: perforation, rupture of the cordae. The behaviour of the mitral valve was fairly constant in all 15 cases of hypertrophic, hyperkinetic, cardiomyopathy, and mitral incompetence was common, and usually found in isolation. The mechanism is discussed, and the physiopathological and therapeutic implications of these facts are reviewed.


Assuntos
Cardiomiopatia Hipertrófica/complicações , Insuficiência da Valva Mitral/etiologia , Adolescente , Adulto , Idoso , Cardiomiopatia Hipertrófica/fisiopatologia , Criança , Cineangiografia , Feminino , Próteses Valvulares Cardíacas , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Insuficiência da Valva Mitral/fisiopatologia , Insuficiência da Valva Mitral/cirurgia
13.
Arch Mal Coeur Vaiss ; 77(13): 1547-50, 1984 Dec.
Artigo em Francês | MEDLINE | ID: mdl-6440504

RESUMO

Idiopathic right atrial dilatation is a rare and probably congenital malformation. Two new cases are reported, in an 18 months old infant and a six week old baby, both presenting with supraventricular tachyarrhythmias. Clinical examination was normal. Chest X-ray showed bulging of the right heart border and electrocardiography revealed partial right bundle branch block. The diagnosis was made by echocardiography which showed aneurysmal dilatation of the right atrium. There were no signs of valvular heart disease or of an intracardiac shunt; the septal tricuspid leaflet was in its normal position. M-mode recordings showed no signs of Ebstein's anomaly. The diagnosis was confirmed by cardiac catheterisation in the first case; this investigation seemed to be pointless in the second case. Both children experienced many episodes of supraventricular tachyarrhythmias. The first developed tricuspid regurgitation but the second remains asymptomatic.


Assuntos
Átrios do Coração/anormalidades , Taquicardia/etiologia , Ecocardiografia , Feminino , Hemodinâmica , Humanos , Lactente , Masculino , Taquicardia/fisiopatologia , Valva Tricúspide/anatomia & histologia , Valva Tricúspide/fisiologia
14.
Arch Mal Coeur Vaiss ; 78(4): 657-60, 1985 Apr.
Artigo em Francês | MEDLINE | ID: mdl-3159371

RESUMO

Idiopathic left ventricular aneurysms are rare in childhood as are the congenital epigastric muscular diverticula which form part of a complex congenital malformation. The aneurysms are usually symptomatic and present either with cardiac failure or another complication. The authors report a very rare case in which the aneurysm effectively realised a double chamber left ventricle. The aneurysm was larger than the true ventricle, muscular, lined by normal endocardium and covered by coronary vessels. Surgical ablation was indicated for cardiac failure. Two similar cases have been previously reported.


Assuntos
Cardiomegalia/etiologia , Aneurisma Cardíaco/complicações , Aneurisma Cardíaco/diagnóstico , Humanos , Lactente , Masculino
15.
Arch Mal Coeur Vaiss ; 71(12): 1347-55, 1978 Dec.
Artigo em Francês | MEDLINE | ID: mdl-85437

RESUMO

The charges of left ventricular function provoked by coupled electrical stimulation were studied in 17 patients with severe chronic aortic incompetence and 9 controls. These results were compared with those obtained after isolated extrasystole in 24 cases of aortic incompetence and 10 controls, and during paired electrical stimulation in 12 cases of aortic incompetence. Left ventricular function increased uniformly after post-extrasystolic potentiation whatever the mode of stimulation. The preload did not change significantly, the increased ventricular function being mainly due to increased contractility. Coupled electrical stimulation mobilised the greatest contractility reserve (ejection fraction : +32 % in controls and +37 % in aortic incompetence and averaged circumferentiel fibre shortening +55 % in controls and +47 % in aortic incompetence). The contractility reserve is inversely proportional to the initial ejection fraction except when its value is less than 35 %. The myocardial response then becomes variable so that left ventricles with probable irreversible hypocontractility may be distinguished from those whose ventricular funciton would improve after surgical correction.


Assuntos
Insuficiência da Valva Aórtica/fisiopatologia , Adulto , Complexos Cardíacos Prematuros/fisiopatologia , Diástole , Estimulação Elétrica , Ventrículos do Coração/fisiopatologia , Humanos , Pessoa de Meia-Idade , Contração Miocárdica
16.
Arch Mal Coeur Vaiss ; 71(3): 348-56, 1978 Mar.
Artigo em Francês | MEDLINE | ID: mdl-416808

RESUMO

Disordered left ventricular function and changes in the sub-valvular apparatus appear to be associated in patients with isolated mitral stenosis (MS). Left ventricular cine-angiography has demonstrated two groups of patients, according to the presence (group II) or absence (group I) of changes in the sub-valvular apparatus. There was no significant difference between the two groups in terms of age, heart rate, left ventricular end diastolic pressure, surface area of the mitral valve, or ventricular ejection time. However, group II patients had a lowered ejection fraction (EF), systolic ejection volume (SEB), speed of shortening of circular fibres (VCF), and systolic work index (SWI) compared with group I cases (respectively 0.49 +/- 0.06 and 0.58 +/- 0.04 for the EF, 36 +/- 7 ml.m-2 and 41 +/- 7 ml.m-2 for the SEV, 0.85 +/- 0.12 circ.s-1 and 1.09 +/- 0.16 circ.s-1 for the VCF, 45 +/- 11 gm.m-2 and 57 +/- 11 gm.m-2 for the SWI. Nevertheless, it appears that changes in the sub-valvular apparatus indicate rather than cause dysfunction of the left ventricle, which occurs in a certain number of patients who have a normal sub-valvular apparatus.


Assuntos
Ventrículos do Coração/fisiopatologia , Estenose da Valva Mitral/fisiopatologia , Contração Miocárdica , Adulto , Angiocardiografia , Volume Cardíaco , Cineangiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estenose da Valva Mitral/diagnóstico por imagem
17.
Arch Mal Coeur Vaiss ; 71(12): 1375-82, 1978 Dec.
Artigo em Francês | MEDLINE | ID: mdl-106791

RESUMO

132 patients with pure mono-valvular cardiopathies (mitral incompetence, aortic stenosis and aortic incompetence) were classified into two groups according to the values of the systolic work index/myocardial mass ratio (SWI/MLV). Normal values of the ejection function (EF) and mean velocity of circumferential fibre shortening (VCF) for each cardiopathy were so obtained. Only patients with aortic stenosis of group I (SWI/MLV greater than or equal to 0.75 gm . g-1) had normal EF. All the other patients had EF and VCF values below normal although this did not always imply impaired myocardial function. Therefore the myocardial mass should also be considered in the evaluation of myocardial function and it would seem desirable to take this parameter into account in the management of these patients.


Assuntos
Insuficiência da Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/fisiopatologia , Insuficiência da Valva Mitral/fisiopatologia , Adulto , Ventrículos do Coração/fisiopatologia , Humanos , Cinética , Pessoa de Meia-Idade , Contração Miocárdica , Sístole
18.
Arch Mal Coeur Vaiss ; 80(10): 1505-11, 1987 Sep.
Artigo em Francês | MEDLINE | ID: mdl-3125810

RESUMO

The records of 43 patients older than 18 years presenting with tetralogy of Fallot were retrospectively examined to determine the semiological characteristics of the disease in adults. One or more palliative operations had previously been performed in 27 patients. Full correction was carried out in 38 patients with results described below. Tetralogy of Fallot has the following characteristics in adults as compared with children: clinically, heart failure, attacks of angina, haemoptysis and sequelae of previous complications are more frequent; at electrocardiography, right atrial hypertrophy, right bundle disorders of conduction and ventricular extrasystoles are also more frequent; radiology shows that cardiomegaly is no longer exceptional; haemodynamic studies demonstrate an increase in right atrial and right ventricular end-diastolic pressures. These characteristics indicate a deterioration of haemodynamic adjustment to the disease with age. The operative morbidity mostly consisted of haemorrhages (55% of the patients), more frequent in subjects with permeable anastomoses (p less than 0.01), and heart failure (50% of the patients) the frequency of which increased with the subject's age, the duration of the operation and the use of an infundibulo-pulmonary prosthesis (p less than 0.05). The operative mortality (18%) depended on the extent of the pulmonary stenosis and on the presence of a previous anastomosis (p = 0.04). An analysis of the causes of death reported in the literature showed that in adults the presence of an anastomosis constitutes a separate risk factor in complete repair. The excellent long-term results of corrective surgery concerning cardiac function and survival suggest that except for those rare cases where the operative risk is very high all adults with tetralogy of Fallot should undergo complete repair.


Assuntos
Tetralogia de Fallot/cirurgia , Adulto , Envelhecimento , Feminino , Seguimentos , Hemorragia/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/mortalidade , Gravidez , Estenose da Valva Pulmonar/cirurgia , Reoperação , Estudos Retrospectivos , Tetralogia de Fallot/fisiopatologia
19.
Arch Mal Coeur Vaiss ; 88(5): 705-10, 1995 May.
Artigo em Francês | MEDLINE | ID: mdl-7646281

RESUMO

For over 20 years, different methods of interventional catheterisation have partially replaced surgical closure of patent ductus arteriosus (PDA). The authors report the results of a new operative technique, video-thoracoscopy, derived from endoscopic surgery. Under general anaesthesia and after tracheal intubation, two trocarts of 5 mm diameter are introduced into the thorax for the passage of the instruments required for dissection and closure of the PDA. Two hooks are also introduced to retract the lung and dissect the ductal region. Two 9 mm titanium clips are positioned under videoscopic control. Forty-five children underwent this procedure between February 1992 and July 1994. The average age at the time of operation was 13.8 months (range: 3 to 32 months) with an average weight of 14.5 kg (range: 2 to 48 kg) including 10 (22%) with a body weight of less than 6 kg. The surgical indications were haemodynamic in 27% of cases (large shunts with pulmonary hypertension) and prophylactic against endocarditis in 73% of cases. There were no operative fatalities. The immediate complications included: chylothorax (1 case) and left recurrent nerve paralysis (2 cases). A residual shunt was observed in 3 of the 45 cases (6%). In one of these cases, a supplementary clip was effective in suppressing the residual shunt. In the other 2 cases, the residual shunt was respected after a second failure of clipping the duct in one of the cases. The final closure rate was 95.6%. Closure of PDA by video-thoracoscopy is a rapid and safe technique.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Permeabilidade do Canal Arterial/cirurgia , Toracoscopia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Métodos , Complicações Pós-Operatórias , Grampeadores Cirúrgicos
20.
Arch Mal Coeur Vaiss ; 88(5): 737-42, 1995 May.
Artigo em Francês | MEDLINE | ID: mdl-7646286

RESUMO

The authors report 5 cases of the long QT syndrome. The patients had malaises and syncopes triggered by exercise or emotional stress, occurring before 10 years of age. The diagnosis was delayed by an average of 38 months (range: 2 to 72 months) from the first symptom and systematic familial study which was positive in 4 of the 5 cases. The corrected QT intervals varied from 0.46 to 0.56 second. All patients were prescribed the betablocker nadolol, resulting in total regression of symptoms with no deaths during an average follow-up period of 40 months (range: 26 to 64 months). The long QT syndrome is a rare hereditary disorder comprising several entities. Recent genetic advances have improved our understanding of the condition. All have an idiopathic prolongation of the QT interval (> 0.44 second) in common with a predisposition to syncope due to torsades de pointes which may cause sudden death. The spontaneous arrhythmic mortality, which was early and high, has been remarkably decreased by betablocker therapy, from 73% to 6% at 10 years. The authors find nadolol to be extremely reliable and well tolerated, and recommend it as the drug of choice in this indication.


Assuntos
Antagonistas Adrenérgicos beta/uso terapêutico , Síndrome do QT Longo/congênito , Nadolol/uso terapêutico , Criança , Pré-Escolar , Relação Dose-Resposta a Droga , Eletrocardiografia , Teste de Esforço , Feminino , Humanos , Síndrome do QT Longo/tratamento farmacológico , Síndrome do QT Longo/fisiopatologia , Masculino , Fatores de Risco
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