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The causes, clinical indications and diagnosis and differential diagnosis of cardiac disorders which may lead to cerebral symptoms are illustrated on the basis of a review of the present day level of scientific research. Principally involved are cerebral ischaemias arising from cerebral embolisms or from reduction of cardiac output in cardiovalvular and myocardial disorders. The incidence of all embolisms of cardiac origin makes up 10% of all ischaemic cerebral infarcts, with auricular fibrillation, irrespective of its origin, mitral stenosis, myocardial infarct, mitral insufficiency and combined mitral valve defects, and, in younger patients, mitral valve prolapse, being, in this order of frequency, of primary clinical significance. The other cardiovalvular and myocardial disorders have, in comparison, a relatively low incidence of cerebral embolisms. Haemodynamically induced cerebral ischaemias frequently occur in the form of complications following acute cardiac arrest, in myocarditis and in case of primary cardiomyopathies resulting from cardiac insufficiency or complicating bradyarrhythmia. They are clinically apparent in the form of syncope, and other impairments of consciousness of various levels of seriousness with and without indications of cerebral origin, extending up to coma. In view of the high incidence of 25% of acute cerebral ischaemias in cases of cardiac disease, not only neurological but also detailed cardiological investigation is vital in all cases for a correct diagnosis and for the selection of a suitable course of treatment. Cerebral complications in bradyarrhythmia and endocarditis are discussed in the context of a review of the relevant literature together with consideration of their epidemiology, aetiology, pathophysiology and clinical profile. Pathological sinus-bradycardia, bradyarrhythmia absoluta, sinu-atrial and atrio-ventricular blockages, carotid-sinus and sick-sinus node syndrome, paroxysmal atrial tachycardia, AV-node tachycardias, and auricular fibrillation and flutter, taken as a whole, lead to cerebral complications affected patients in 5 to 10% of afflictions of the central nervous system occur in 50% of patients suffering from complete AV blockage and, at a not precisely definable frequency, in patients suffering from other bradyarrhythmias. In addition to transitory, uncharacteristic symptoms such as dizziness, vertigo, impairment of vision and balance, presyncope, syncope and Adams-Stokes syndrome dominate the clinical profile. Endocarditis, with an incidence of 0.01 to 0.05% in the overall population, results in central nervous system complications in 12 to 25% of cases on average.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Heart diseases as a cause of cerebral symptoms and syndromes]. 222 59

Since the clinical, 2-dimensional and Doppler echocardiographic and pathologic findings in infantile Marfan syndrome have not been documented in detail, a study of 9 such infants was performed. The previously reported 64 cases were reviewed and the salient findings in 22 additional cases were discussed. The age at diagnosis in our 9 cases ranged from birth to 12 months (mean 2.7 months). Mitral valve prolapse was demonstrated in all, with mitral regurgitation in 8. Tricuspid valve prolapse was present in 8, with tricuspid regurgitation in 6. Marked aortic root dilatation was present in all, and was progressive. The aortic root assumed a "clover leaf" appearance in the parasternal short-axis view. Aortic regurgitation was documented initially in 1 patient, and developed during follow-up in 4 of 7 infants. Dilation of the pulmonary arterial root and pulmonary regurgitation were found in 3 of 7 infants. Severe heart failure associated with mitral or tricuspid regurgitation was present in 7 of the 9 patients. Four infants died during the first year of life. The salient pathologic features were myxomatous thickening and redundancy of the mitral and tricuspid leaflets, marked elongation of chordae tendineae and prominent dilatation of the aortic and pulmonary roots. Histologically, the collagen and elastic fibers were severely disrupted, disarrayed and fragmented with increased interstitial ground substance. These data document that infantile Marfan syndrome is characterized by clinical and morphologic features that are distinctly different from the classic syndrome seen in adolescents and adults. The aforementioned findings should facilitate early clinical and echocardiographic diagnosis of infantile Marfan syndrome.
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PMID:Two-dimensional and Doppler echocardiographic and pathologic characteristics of the infantile Marfan syndrome. 233 33

A case of WPW syndrome combined with mitral regurgitation caused by infective endocarditis underwent surgical division of accessory pathway and mitral valve replacement preserving posterior leaflet simultaneously. A 56-years old woman suffered atrial fibrillation with pseudo VT and cardiac failure caused by mitral regurgitation. Electro-physiological study (EPS) revealed accessory pathway in postero-lateral wall in left atrium and atrio-fascicular pathway like James bundle in AV node. ECHO cardiography showed mitral valve prolapse and severe regurgitation. Accessory pathway was divided surgically and deep freeze coagulation was followed. Perforation of anterior leaflet and chordal rupture of posterior leaflet caused by infective endocarditis were repaired by annuloplasty (Kay and McGoon method) at first, but regurgitation retained moderately. After re-clamping of aorta, mitral valve was replaced with prosthesis (SJM 29 mm) preserving posterior leaflet. Postoperative examination revealed division of accessory pathway and no regurgitation of mitral prosthesis.
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PMID:[Simultaneous operation of WPW syndrome combined with mitral regurgitation caused by infective endocarditis]. 234 36

Clinical pictures and prognosis in 24 patients (nine males, 15 females) with mitral valve prolapse (MVP) aged 60 years and older were examined at our institution. Valvular redundancy was noted in seven (29%) of the 24 patients. During a follow-up period of 38 +/- 18 months, four patients had mitral valve replacement and one died of congestive heart failure due to severe mitral regurgitation (MR). There was no episode of embolism or sudden death. Among 80 patients (47 males, 33 females) with MVP aged 60 years and older at St. George's Hospital in England, on the other hand, valvular redundancy was noted in 49 (61%) of 80 patients. During the same follow-up period of 39 +/- 28 months, 18 patients underwent surgery and seven patients died of heart failure (31%). There were 11 patients who had embolism (14%), but no sudden death. Although there was no significant difference in the incidence of severe MR in these two groups, valvular redundancy and embolism were less frequent in Japanese patients at our institution than the English patients in St. George's Hospital. These differences should be taken into consideration when one evaluates clinical profiles and prognosis of elderly MVP patients.
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PMID:[Prognosis of elderly patients with mitral valve prolapse: the difference between Japanese and English patients]. 239 94

Our study group included 12 patients (4 males, 8 females), mean age 60 yr, with symptomatic or threatening tachyarrhythmias (Lown classes IV A, B, V); 2 patients were suffering from mitral valve prolapse syndrome, 2 from ischemic heart disease; 4 from cardiac insufficiency caused by hypertensive or ischemic heart disease; 4 had no evident clinical signs of cardiopathy. Patients suffering from: cardiac insufficiency (F.C. III e IV NYHA); II and III degree BAV; atrial flutter and fibrillation; long QT syndrome; acute ischemic heart disease were excluded from the study. During short-term treatment, patients received placebo for four days and subsequently flecainide 200 mg daily for four days. During medium-term treatment patients received flecainide 200 mg daily (for six months). Several Holter/24-hour monitorings were performed for evaluation of therapy. No significant reduction in the number of ectopic ventricular beats (B.E.V.) was found with placebo whereas reductions of B.E.V. number (97% and 95%, respectively) were found during short and medium-term treatment with flecainide. Flecainide produced: changes in Lown class: from IV A, B and V to II and I; a marked reduction of subjective symptoms (dyspnea, giddiness syncope, precordial pain); ECG changes: increases in: PR: 5-25%; QRS: 11-12%; QT: 11-22%. Flecainide produced no pro-arrhythmic effects or changes in echocardiographic ventricular function index. Flecainide can be considered one of the most effective new antiarrhythmic drugs.
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PMID:[Short- and medium-term treatment of ventricular hyperkinetic arrhythmia with flecainide]. 252 12

Different anatomo-clinic aspects from three mitral valve prolapse cases are compared to those commonly presented in the literature and are also utilized as a basis for a new classification of this disease. The patients are more than 77 years old, what is in contrast with the current concept of MVP as a disease of young-middle aged women. The first case shows marked ostial dilation and many ruptured chordae: as a consequence, this patient showed severe cardiac dysfunction. The anterior, rather than the posterior leaflet, presented intense myxoid degeneration. In the second case, no ruptured chordae were detected and, consequently, the degree of heart failure was lesser than the first one, in spite of the same degree of ostial dilation. Both leaflets showed the same degree of myxoid degeneration. The third patient, who does not have heart failure, showed myxomatous degeneration of both cusps, but no ostial dilation or chordal rupture were present. These aspects reinforce the impression that isolated mixomatous degeneration of the cusps is not so deleterious when compared to those cases where the mitral valvar ring is dilated or its chordae are also involved by that degenerative process. Therefore ostial dimension (normal or enlarged) and the state of the chordae (with or without rupture) appears to be important points to be considered in MVP.
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PMID:[Mitral valve prolapse in the 8th and 9th decades of life. Anatomo-pathologic study of 3 cases]. 262 89

Myxoid heart disease (MHD), more commonly known as mitral valve prolapse, is a very common cardiac abnormality affecting 5-10% of the general population. This article reviews the history, symptoms, physical findings, pathology, associated conditions and complications of this entity. Special emphasis is given to sudden cardiac death, which occurs as a result of acute heart failure or due to a fatal arrhythmia. Theories regarding the origin of the arrhythmias are discussed. Once the origin is known, those at risk for this devastating complication can be identified and perhaps a preventative therapeutic regimen developed.
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PMID:Myxoid heart disease: a review with special emphasis on sudden cardiac death. 265 60

To evaluate the early and late results of mitral valve replacement and reconstruction for mitral insufficiency due to ruptured chordae tendineae respectively, 74 consecutive cases were analyzed. Fifty-five (74.3%) of the patients were men, and the mean age was 48 +/- 12 years old (range 16 to 76). The causes of the mitral disease were idiopathic in 50 (67.6%), rheumatic in 7 (9.4%) and infective endocarditis in 11 (14.9%) patients. In idiopathic 50 cases, 24 had mitral valve prolapse and 16 had both mitral valve prolapse and hypertension. Forty-one (55.4%) of the patients were in NYHA functional class III or IV preoperatively. Thirty (40.5%) cases underwent surgery within one year after their initial symptoms of heart failure onsets including six emergency operation cases due to uncontrollable acute lung edema. Chordae to anterior mitral leaflet were ruptured in 31 (a5, m16, p10)[41%] patients, to the posterior mitral leaflet in 45 (a4, m23, p18)[59%], and to both leaflets in one patient. Mitral valve replacement was performed in 68 patients (91.9%) and 6 patients (8.1%) underwent mitral valve repairs. Twenty cases underwent associated procedures that included tricuspid valve annuloplasty in 8, aortic valve replacement in 5 and myocardial revascularization in 4 cases. There were two operative deaths (2.4%); both occurred after replacement, left ventricular rupture in one and DIC in one. Mean follow-up period was 4.5 years (range 1 to 17) in 67 cases. There were four late deaths; all occurred after replacement. However five patients sustained mild mitral insufficiency after mitral valve repair including one that became worse of regurgitation three years after isolated Kay's annuloplasty, there were no cases that had needed reoperation and no late death after reconstruction. Left ventricular function and pulmonary arterial pressure were almost normalized in more than 90% cases postoperatively. Our data indicated that mitral valve reconstruction (McGoon's plus Kay's method as standardized maneuver) was the procedure of choice for selected patients with mitral insufficiency owing to ruptured chordae tendineae to the posterior mitral leaflet, including more limited patients with ruptured chordae to the anterior mitral leaflet.
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PMID:[Mitral insufficiency due to ruptured chordae tendineae--clinical features, early and late results of valve replacement and repair]. 273 33

Seventy-seven patients with drug-refractory sustained ventricular tachycardia (VT) (28 patients) or ventricular fibrillation (VF) (49 patients) underwent implantation of an automatic cardioverter defibrillator (AICD). The 67 men and 10 women, with a mean age of 60 +/- 12 years (range 18 to 79), had coronary artery disease (60 patients), idiopathic cardiomyopathy (eight patients), mitral valve prolapse (four patients), hypertensive heart disease (one patient), Ebstein's anomaly (one patient), long QT syndrome (one patient), and primary electrical disease (two patients). The mean left ventricular ejection fraction was 35 +/- 16% (range 10% to 75%). Sustained VT/VF was induced in 64 patients (83%) at baseline electrophysiologic testing. A mean of 4.1 +/- 1.3 antiarrhythmic drugs failed to control the arrhythmia. Associated surgery at AICD implantation included coronary artery bypass in 19 patients, coronary bypass with aneurysmectomy in six patients, and aneurysmectomy alone in one patient. Five patients had only prophylactic patches implanted during aneurysmectomy or coronary bypass and the AICD device was subsequently implanted under local anesthesia to prevent arrhythmia recurrence or to control persistently inducible VT. Operative mortality was 2.6% with two deaths from intractable VF. Fifty-two patients (69%) continued receiving antiarrhythmic drugs to suppress spontaneous VT. During a mean follow-up of 15 +/- 13 months (range 1 to 63), six patients died: two suddenly due to probable pulse generator failure (greater than 2 years old), one of acute myocardial infarction, two of heart failure, and one of respiratory failure.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical experience in seventy-seven patients with the automatic implantable cardioverter defibrillator. 277 68

Between December 1985 and June 1987, 38 consecutive patients with mitral regurgitation underwent mitral valve reconstruction (MVP) with Carpentier rings. There were 16 men and 22 women, ranging in age from 16 to 63 years (mean 36.4 +/- 14.4). The underlying causes were rheumatic heart disease (55%), degenerative valvular disease (42%), and congenital heart disease (3%). Thirty patients were categorized in the New York Heart Association's functional classification III or IV preoperatively. The concomitant procedures included aortic valve replacement (AVR) in 6 patients, tricuspid valve repair (TVP) in 9, and closure of atrial septal defect in one. Hospital death happened to one patient (3%). All but one patient were followed up at 31 months postoperatively (rate 98.6%). There was one late death due to myocardial failure not related to the valves. The actuarial survival rate at 31 months was 96.8%. The thromboembolic rate was 1.44% per patient-year. No reoperation or endocarditis was encountered. All 36 survivors were in functional classes I and II. Twenty-one patients underwent Doppler echocardiography 3 to 12 months after surgery and 17 (81%) showed no or mild mitral regurgitation and 4 (19%) had moderate regurgitation. We conclude that MVP with Carpentier rings is a satisfactory method with low mortality and complication rates in Chinese patients.
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PMID:Mitral valve reconstruction with Carpentier ring for mitral regurgitation: experience with Chinese patients. 279 33


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