As originally published in 1988: Results of total correction of tetralogy of Fallot performed in adults. Updated in 1996.
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Biomedical subjects
Publications and source records attributed to P Presbitero.
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We describe a case of a verrucous non-bacterial endocarditis on the native mitral valve in a patient with polymyositis. This case, to our knowledge, represents the first report in the literature we could get. The case reached our attention after an episode of acute limb ischemia which lead to an echocardiographic examination that showed vegetations on the valve. No cardiac signs or symptoms were evident. We discuss the possible relation between the two disorders. The possibility of autoimmune diseases, other than lupus or lupus-related disorders, to produce this kind of lesions should be confirmed by a systematic echocardiographic study of these patients even if they have no evidence of cardiac involvement.
Pregnancy and delivery in women with congenital heart disease remain inadvisable for patients at high risk due to the following congenital diseases: (1.) Severe left ventricular outflow tract obstruction (increase of pressure gradient); (2.) Eisenmenger syndrome (increase of right to left shunt with worsening of cyanosis and fetal growth retardation); (3.) Marfan syndrome with enlarged aortic root (risk of aortic dissection). In women suffering from cyanotic congenital heart disease the main risk is fetal complications. These are correlated to oxygen saturation and to the type of maternal disease, particularly if a palliative shunt operation has never been performed. Women with left to right shunt, right outflow tract obstruction or previous correction of the disease can go through pregnancy with low risk of mortality. The complications (mainly heart failure and arrhythmias) can be well managed with medical treatment, and the fetal outcome is similar to that in the general population.
OBJECTIVE: To assess the clinical course of adult patients with corrected transposition of the great arteries without associated anomalies. DESIGN: All patients with corrected transposition of the great arteries without associated anomalies were reviewed with complete clinical and echocardiographic assessment. The complications were evaluated in each decade. SETTING: Tertiary centre with a specific unit dealing with "grown-up" adolescent and adult congenital heart disease, designated as a quaternary centre and a general hospital with a referral centre for "grown-up" congenital heart disease. PATIENTS: 18 patients (nine male and nine female) aged 16-61 years followed for 1-30 years (mean 10 years). RESULTS: There were no deaths. Six patients had a worsening ability index during follow up. Complications were: (a) complete heart block in seven, three of whom required pacemaker insertion; (b) significant left atrioventricular valve regurgitation in 50%, appearing only in the third decade (12%), with increasing frequency thereafter. Infective endocarditis was responsible for increasing left atrioventricular valve regurgitation in only one patient; (c) supraventricular arrhythmia appeared in the fifth decade, and occurred in all patients over the age of 60 years. One patient aged 61 had recurrent sustained ventricular tachycardia; and (d) congestive heart failure developed only after 50 years in 66%. One patient had severe left atrioventricular valve regurgitation; the function of the systemic ventricle was only moderately reduced in the other three. Three of the nine women had seven uneventful pregnancies. CONCLUSIONS: Patients with corrected transposition of the great arteries without associated defects may remain undiagnosed until adult life. Symptoms occur rarely before the fourth and fifth decades, when rhythm disturbance, left atrioventricular valve regurgitation, and moderately impaired systemic ventricular function cause congestive cardiac failure. The role of pacemaker insertion or surgery for left atrioventricular valve regurgitation needs further assessment.
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In a series of 416 women with congenital heart disease seen in the Royal Brompton National Heart and Lung Hospital, London, and the Hospital Giovanni Bosco, Torino, Italy, there were 822 pregnancies. The outcomes of 96 pregnancies in 44 patients with cyanotic congenital heart disease were studied. Patients with the Eisenmenger reaction were excluded. Patients were divided arbitrarily into groups according to the type of maternal congenital cardiac anomaly, and factors influencing maternal and fetal outcome were evaluated. The incidence of maternal cardiovascular complications was high (32%), with one death from endocarditis 2 months after delivery. Forty-one (43%) of 96 pregnancies resulted in a live birth; 15 (37%) were premature. Mean weight of full-term infants was 2575 g. Univariate analysis suggested that maternal disease, Ability Index, hemoglobin, and arterial oxygen saturation before the pregnancy were factors that discriminated between successful and unsuccessful fetal outcome, with hemoglobin and arterial oxygen saturation being the most important predictors. Women with cyanotic congenital heart disease can go through pregnancy with a low risk to themselves, with frequent treatable complications, but there is a high incidence of miscarriage, premature births, and low birth weights. An incidence of congenital heart disease in the fetus of 4.9% (2 of 41 live births) is higher than that found in the normal population.
Aim of this study was to assess the long-term results of PTCA in patients with a recent myocardial infarction. The study population consisted of 244 consecutive patients who underwent a PTCA < or = 30 days after myocardial infarction either because of angina (116 patients), or because of provoked ischemia (62 patients), or in the absence of both (66 patients). "Simple" balloon angioplasty, without adjunctive devices, was performed. Most of the patients were seen at 1 year intervals at the outpatient clinic; otherwise information was gathered either from the family physician or by telephone. The following events were noted during a follow-up of 6-102 months (median 39 months): death, myocardial infarction, need for re-PTCA or surgical revascularization, recurrence of angina. Survival and event-free curves were calculated according to the method of Kaplan and Meier; statistical comparisons between the curves were obtained by the log-rank test. Periprocedural deaths, infarctions and surgical revascularizations are included in the actuarial curves. Differences were examined between patients with Q and non-Q wave infarctions, single vessel versus multivessel disease, left ventricular ejection fraction > or 50% versus < 50%. A clinical success was achieved in 85% of the procedures. Sixteen patients were lost at follow-up. Overall, within the first 30 days and during follow-up, 12 (4.9%) patients died, 19 (7.7%) suffered from myocardial infarction, 34 (13.9%) required re-PTCA or surgical revascularization, and 63 (25.8%) experienced angina again.(ABSTRACT TRUNCATED AT 250 WORDS)
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OBJECTIVE: To define the clinical and angiographic features and the therapeutic problems in patients with coronary artery disease after therapeutic irradiation of the chest. DESIGN: An observational retrospective study. SETTING: The cardiac catheterisation laboratory, university medical school. PATIENTS: 15 subjects (8 men and 7 women, aged 25-56 years, mean 44) examined in the cardiac catheterisation laboratory, who had significant coronary artery disease years after having radiation treatment to the chest and anterior mediastinum. In the early stages of the study angiography was performed because of typical symptoms of ischaemic heart disease. Later on it was performed because of a high index of suspicion in people with signs of extensive radiation heart damage. MAIN OUTCOME MEASURES: Clinical and electrocardiographic evidence of ischaemic heart disease; echocardiographic signs of pericardial, myocardial or valvar involvement; angiographic evidence of coronary arterial stenosis, with special attention to the ostia; haemodynamic and angiographic signs of pericardial, myocardial, and valvar disease. Survival and symptomatic and functional status were ascertained after medical or surgical treatment. RESULTS: The patients were relatively young and had no risk factors. Seven patients had no signs or symptoms of ischaemic heart disease. Ten patients had ostial stenosis, which was associated with extensive involvement of other cardiac structures in nine of them. Seven required surgical treatment for coronary artery disease. Two died, one at surgery and the other one six months later. Five patients had complications associated with irradiation. CONCLUSIONS: Coronary arterial disease can be reasonably ascribed to the effects of chest irradiation when the patients are young and free from risk factors, especially if the obstructions are ostial and there is important damage to other cardiac structures. In patients with damage to other cardiac structures angina and infarction are often absent and coronary angiography seems to be mandatory. Patients often require surgical treatment and postoperative complications are common.
PURPOSE OF THE STUDY: to assess the diagnostic value of the commonly adopted haemodynamic criteria for the diagnosis of obstructive and non-obstructive hypertrophic cardiomyopathy. PATIENTS: 16 cases of hypertrophic cardiomyopathy, 7 cases of the non-obstructive form, 16 fixed subaortic stenosis, 8 valvular aortic stenosis, and 7 cases of hypertensive heart disease. METHODS: left and right heart catheterisation, with particular attention to the pressure gradient and to the aortic pulse pressure by means of simultaneous pressure recording from the left ventricle and aorta under basal conditions, after a ventricular premature beat, during the Valsalva manoeuvre, during amyl nitrite inhalation, and isoproterenol administration. RESULTS AND CONCLUSION: the Valsalva manoeuvre increases significantly (p < 0.001) the pressure gradient, and the aortic pulse pressure in the post-extrasystolic beat decreases significantly (p < 0.001) in patients with hypertrophic obstructive cardiomyopathy. This response is significantly different from that of the other 3 disease groups. If the non-obstructive form is defined by strict criteria (i.e. no gradient above 30 mmHg even after provocative manoeuvres), then it cannot be separated from other forms of secondary left ventricular hypertrophy.
INTRODUCTION: Haemostatic and fibrinolytic parameters were evaluated in patients with chronic pulmonary hypertension following pulmonary embolism (CPE). The diagnosis of CPE was based on lobar or segmental defects on perfusion lung scans and by pulmonary angiograms which showed complete or partial obstruction of main or segmentary lobar arteries. METHODS: Antithrombin III (AT III), protein C, protein S, and lupus anticoagulant (LA) were assayed in 8 patients with CPE; in 6 out of 8 patients plasma fibrinolytic activity was assessed both under basal conditions and after venous stasis. The control group consisted of 4 normal subjects. Protein C and protein S antigens were assayed by an electrophoretic method. Protein C and protein S biological activities were assayed by a manual clotting system. AT III was assayed by chromogenic method. Fibrinolytic total activity was studied on fibrin plates, tPA and PAI-1 activities by chromogenic method; tPA and PAI-1 antigens by ELISA technique. RESULTS: One patient out of 8 showed a protein C deficiency and 3 patients out of 8 were positive for a LA. All patients had a statistically significant reduction of plasma fibrinolytic activity (p < 0.001) and of tPA activity (p < 0.0005) after venous stasis as compared to the control group. CONCLUSIONS: Our data show that significant haemostatic abnormalities may underlie this disease. In particular, a) an impairment of fibrinolytic plasma activity and low levels of plasminogen activator may be found, and b) the undiagnosed presence of a LA may be the cause of these thrombotic events. The meaning of these results needs further assessment.
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A dipyridamole-echocardiographic test (DET) was carried out to find out how safe and useful it was in predicting clinical outcome and in identifying patients at risk. The test was performed in 107 asymptomatic patients early (5 to 8 days) after a first acute uncomplicated myocardial infarction managed with thrombolytic therapy. All patients were followed up for a mean of 15 months and 94 underwent coronary angiography. The test was considered positive if transient asynergy of contraction was newly detected either in the infarct and adjacent areas or in the remote zones; two subsets were studied, according to the dose of dipyridamole (0.56 or 0.84 mg.kg-1) needed to induce ischaemia. The test was accomplished satisfactorily in 96% of patients. Intra-inter-observer agreements were 88% and 91% respectively. The test also proved safe at the high infusion dose. During the follow-up period, two patients died, one experienced re-infarction and 12 (12%) developed recurrence of angina. DET was abnormal in 32 patients (adjacent and remote asynergy in 28 and four patients respectively): 18 had a critical and two a non-critical stenosis in the infarct-related vessel, and nine had an occluded artery with collateral distal flow. Multivessel disease was present in 11 patients considered positive, four in the remote and seven in the adjacent zones. However, 20 patients with negative DET results had multivessel disease. Of the positive DET patients, seven had angina. There were eight total events in the 71 negative DET patients, five of whom had multivessel disease. Abnormality was more pronounced in positive DET patients, but did not influence the outcome.(ABSTRACT TRUNCATED AT 250 WORDS)
108 women with congenital heart disease in child-bearing age (16-38 years, mean age 28) were followed up for a period of ten years. Possible complications of contraceptive methods, and incidence of full-term pregnancies, spontaneous abortions and cardiovascular complications during pregnancy and delivery, were considered. Half of these patients had a cyanotic congenital heart disease, 23 had left-to-right shunt mainly at atrial level, 18 had right and 14 left ventricular outflow tract obstruction. 60 women had previous surgical procedures for their congenital heart disease. 83% of the patients were in NYHA functional class I-II. No complications were found during oral contraceptive regimen during intrauterine device insertion with antibiotic prophylaxis, except for the development of pulmonary hypertension in one patient with an atrial septal defect. 146 pregnancies occurred, but only 89 were full-term. There was a high number of interrupted pregnancies, rarely for medical reasons. The incidence of spontaneous abortion was similar to that of normal population (18% versus 10-15%). Maternal cardiovascular complications were found in the 22% of the full-term pregnancies, but were well controlled by medical treatment. In conclusion, good family planning and pregnancies are possible in most young females with congenital heart disease. In the absence of cyanosis and pulmonary hypertension, oral contraception does not carry any particular risk. With appropriate medical care intrauterine devices may be an alternative in high risk patients. In the presence of a good cardiac function, a normal full-term pregnancy with an healty baby is the rule. A cesarean section is seldom needed.
BACKGROUND: Cardiac surgery has become a possible cause of ascending aortic dissection, particularly in patients who have undergone aortic valve replacement. METHODS: From January 1979 to December 1989, 134 patients with angiographic evidence of aortic dissection, were studied in the Haemodinamic Laboratory of the University of Turin. Twelve of these patients (7 men and 5 women), mean age 51.6 years, had been previously operated for aortic valve replacement. We evaluated incidence, clinical and anatomic features of ascending aortic dissection in this group. RESULTS: A dilated ascending aorta with a mean diameter of 4.92 cm. was noted in all these patients. The interval between valve replacement and diagnosis of aortic dissection was 57 months (range 1 day to 9 years). Reoperation was performed in 11 patients. Four intraoperative deaths occurred and 2 other patients died in the early post-operative period. Survival rate at 12 months was 50%. Our incidence of ascending aortic dissection after aortic valve replacement was 0.66% (10 patients out of 1499 operated for aortic valve replacement) and it is in agreement with reports from other Authors in literature. CONCLUSIONS: Replacement of the ascending aorta must be considered if an important dilatation of aortic root is found at time of aortic valve surgery. New efforts must be undertaken to identify patients at high risk for this complication because an elevated mortality rate is observed in reoperation for aortic dissection.
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