[Echocardiographic study in heart transplantation].
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Echocardiography↗
Biomedical subjects
G Fasoli
Publications and source records attributed to G Fasoli.
The natural history of cardiac involvement in myotonic dystrophy: an eight-year follow-up in 17 patients.
We evaluated the progression of conduction system and myocardial disease in 17 asymptomatic myotonic dystrophy patients by clinical evaluation, electrocardiography, vector cardiography, and echocardiography. An exercise test was done in 10 patients. After 8 years, a follow-up study of 12 of the 17 original patients was done with a similar protocol. During this period, 2 patients died: one of sudden death while the other had acute left ventricular failure. In our first control study, we found EKG abnormalities in 15 of our patients, consisting mostly of conduction defects or pseudonecrotic patterns. In our second control, all patients had conduction system disease and, in addition, 3 of them had premature ventricular beats. One patient developed dilated cardiomyopathy. In 6 patients, structural involvement of the right ventricle was found. We conclude that even in asymptomatic myotonic dystrophy patients a conduction system deficit is present and progresses, and cardiac death may occur in about 12% of these patients.
Adult↗
Echocardiographic findings in left ventricular septal aneurysm.
The unusual echocardiographic findings are described in a case of congenital interventricular septal aneurysm. The aneurysm involved the middle part of the interventricular septum and was associated with a cleft of the mitral valve and an atrial septal defect but with no evidence of an atrioventricular septal defect. There was extensive fibroelastosis of the right ventricle. The patient presented with brief runs of ventricular tachycardia.
Adolescent↗
Juvenile sudden death and effort ventricular tachycardias in a family with right ventricular cardiomyopathy.
A family with occurrence of juvenile sudden death and effort polymorphous ventricular tachycardias is reported. Nineteen members aged 9 to 63 years were investigated. Four of them died suddenly in their youth. Postmortem investigation performed in 2 deceased subjects disclosed an apparently normal heart at macroscopy but fibro-fatty substitution of the right ventricular free wall was noted at histologic examination. The 14 living members underwent physical examination, resting electrocardiography, chest X-radiography, Holter monitoring, exercise stress testing, and M-mode and cross-sectional echocardiography. Four patients underwent hemodynamic and electrophysiologic studies. All 14 subjects had normal physical examination as well as normal electrocardiographic and cardiothoracic indices. Localized right ventricular structural and dynamic abnormalities were noted at cross-sectional echocardiographic and angiographic investigation of 9 of the patients. The right ventricular volumes in these subjects were normal or slightly increased. In 7 of them, polymorphous ventricular tachycardias were induced by exercise stress testing. The arrhythmias which were responsive to beta-blockade, do not seem to depend on reentry. Enhanced automaticity appeared to be the more likely mechanism of their production. These data demonstrate that right ventricular cardiomyopathy may occur in an occult form with life-threatening electrical instability.
Adolescent↗
Familial occurrence of right ventricular dysplasia: a study involving nine families.
Right ventricular pathologic involvement, with autopsy evidence of fibrous and fatty infiltration of the right ventricle, was investigated in members of families in which cases of juvenile sudden death had occurred. Seventy-two subjects from nine families were studied. Sixteen died at a young age and 56 are living. Postmortem investigation in 11 cases (mean age at death 24 years) revealed massive replacement of the right ventricular free wall by fat or fibrous tissue. In the 56 living patients clinical examination included an electrocardiogram (ECG) at rest, ambulatory ECG recording, posteroanterior and lateral chest roentgenograms, M-mode and two-dimensional echocardiograms and exercise stress tests. In 14 patients, hemodynamic, angiographic and electrophysiologic studies were also carried out; right ventricular endomyocardial biopsy was performed in four. Structural and dynamic right ventricular impairment was detected in 30 living patients (mean age 25 years), and concomitant mild left ventricular abnormalities were present in 4. In eight of the nine families studied at least two members were affected. Ventricular arrhythmias (Lown grade greater than or equal to 4a) were recorded in more than half of the cases. The data reveal that right ventricular dysplasia shows a familial clustering and causes electrical instability that may place affected subjects at risk of sudden death. The mean age of these subjects suggests that the disease is manifested at a young age with a polymorphic clinical and arrhythmic profile. Finally, because this disease is a primary disorder of the ventricular myocardium, it should be included among the cardiomyopathies.
Adolescent↗
[Quantitative echocardiographic study of the left ventricular function in acute aortic infective endocarditis].
Left ventricular (LV) function in 45 patients with native aortic valve infective endocarditis was studied in order to identify high surgical risk patients and the pattern of irreversible myocardial damage. LV function was studied by M-mode and 2D-echocardiography (LV volumes; ejection fraction, EF; peak systolic pressure to end-systolic volume ratio (PAP/ESV) as an index of myocardial contractility; LV mean systolic wall stress as an index of LV afterload and the radius to thickness ratio (R/Th). Thirteen patients underwent aortic valve replacement with an overall operative mortality of 15%. The cause of death was intractable heart failure. Different EF vs stress relationships could be described for different level of myocardial contractility: patients with intractable heart failure had a severely depressed myocardial contractility so that for a given level of LV stress, EF was significantly lower. High operative risk patients were identified by the PAP/ESV vs R/Th relation. All surgical deaths occurred in patients with a severely depressed myocardial contractility (PAP/ESV less than 2) and inadequate hypertrophy (R/Th greater than 4). Reversal of LV dysfunction in patients with moderately depressed myocardial contractility depended on the pattern of LV hypertrophy; a normal post-operative EF was achieved only in patients with adequate hypertrophy (R/Th less than 4).
Acute Disease↗
Observations on the capability of the electrocardiogram to detect left ventricular function in chronic severe aortic regurgitation.
Echocardiographic parameters of left ventricular (LV) function were compared with the electrocardiographic findings in 40 patients with chronic severe aortic regurgitation. Total 12-lead QRS amplitude was linearly correlated with LV mass and its 'relative reduction' (less than 250 mm) indicated LV hypertrophy. Patients with an electrocardiographic 'strain' pattern had a marked increase LV stress (573 +/- 68 dyn). The association of high total 12-lead QRS amplitude and 'strain' indicate a moderately depressed ejection fraction (EF = 43 +/- 3). Finally, 'strain' and a 'relative reduction' of total 12-lead QRS amplitude were present in patients with a severe depression of EF and contractility. The predictive value of these electrocardiographic indices was confirmed during a mean follow-up of 49 months. In conclusion, the electrocardiogram remains an excellent, easy tool for anatomical and functional investigation of patients with aortic regurgitation.
Adolescent↗
A polymorphic form of familial arrhythmogenic right ventricular dysplasia.
Thirty-two members of a family were studied. Three of them died in their youth and had evidence of arrhythmogenic right ventricular (RV) dysplasia. The other 29 members underwent clinical examination, electrocardiography, chest x-ray and M-mode and 2-dimensional echocardiography. Fourteen patients found to have structural abnormalities of the right ventricle underwent 24-hour ambulatory electrocardiographic recording and symptom-limited bicycle stress testing. Hemodynamic and angiographic studies were performed in 6 of these patients. In this family the arrhythmogenic RV dysplasia showed a wide variation of abnormalities, ranging from mild, local alterations to generalized involvement of the right ventricle. The patients were separated into 3 groups on the basis of both the clinical profile and noninvasive/invasive studies: 3 subjects who died suddenly; 3 subjects who had severe ventricular arrhythmias; and 8 subjects in whom RV impairment was not associated with any significant arrhythmias. There was no close relation between the severity of the RV abnormality and presence of ventricular arrhythmias. The variability of the RV abnormality and the high prevalence of this condition in this family is consistent with a genetic pattern of autosomal dominance with incomplete penetrance.
Adolescent↗
Effects of unloading and positive inotropic interventions on left ventricular function in asymptomatic patients with chronic severe aortic insufficiency.
The effect of an unloading (nifedipine, 20 mg sublingually) and of a combined unloading and positive inotropic intervention (nifedipine plus digoxin, 0.5 mg intravenously) on left ventricular performance was assessed in 48 patients with chronic severe aortic insufficiency. The left ventricular pump function-myocardial contractility relation (ejection fraction, EF vs. peak arterial pressure to end-systolic volume ratio, PAP/ESV), and the pump function-afterload relation (EF vs. mean systolic wall stress, MWS) were constructed by means of quantitative M-mode and two-dimensional echocardiography. In patients with normal control pump function (n = 14), nifedipine markedly decreased MWS, moving the patients to a new, more advantageous EF-MWS relation. In the 34 patients with abnormal pump function, the myocardial contractility level was the mean factor conditioning the response to pharmacological intervention. Patients with a value of PAP/ESV greater than 2.5 (n = 22) had normalization of EF after nifedipine and were upgraded to a more advantageous outlook for left ventricular mechanics EF-MWS and EF-PAP/ESV relations. Of the 12 patients without normalization of EF after nifedipine, only the 4 patients with PAP/ESV greater than 2 had normalization of pump function indices after combined administration of nifedipine and digoxin.
Adult↗
[Ebstein disease and pregnancy].
Three patients with Ebstein's disease became pregnant 11 times, 6 of which ended as spontaneous abortion, 2 newborn deaths and 3 successful term pregnancies. Ventricular overloading, arterial hypoxemia and arrhythmias are the major physiopathologic determinants. The ventricular overloading is the most important risk factor for the mother, so as the hypoxemia and the arrhythmias for the fetus. Pregnancy in these patients must be considered at high risk, and a close cardiologic assess with continuous hormonal controls of fetus maturity during the last trimester should be done. A team including obstetricians, cardiologists and anesthesiologists is suggested for the care of these patients in the view of a successful outcome.
Abortion, Incomplete↗
Progression of myocardial dysfunction in asymptomatic patients with severe aortic insufficiency.
By serial echocardiographic examinations, we investigated the parameters predictive of left ventricular (LV) deterioration in 38 asymptomatic patients with severe aortic insufficiency (AI). Based on their first echocardiograms, patients were divided into two groups: Group 1 (n = 30) had normal fractional shortening (FS); Group 2 (n = 8) had abnormal FS. Similar values of LV end-diastolic, end-systolic dimension (EDD, ESD), FS, radius/thickness ratio, and mean wall stress characterized stable and unstable patients of Group 1; unstable patients, who developed abnormal values of FS during the follow-up, showed significantly lower values of the contractility index (peak arterial pressure/end-systolic volume ratio, PAP/ESV). The unstable patients, moreover, were distributed near the onset of the downslope and moved along the descending limb of the curvilinear pump performance-myocardial contractility relation. No changes occurred in Group 2 patients during follow-up. Three unstable Group 1 patients and the 8 patients of Group 2 underwent aortic valve replacement (AVR): postoperative EDD was less than 65 mm and the changes from before to after AVR were greater than 11 mm in all cases. We conclude that the pump performance-myocardial contractility relation visualized the unstability of LV function in asymptomatic patients and that AVR can be delayed after the appearance of an abnormal FS for at least 36 months.
Age Factors↗
Variability of positive inotropic response to acute amrinone administration in chronic cardiac failure.
To determine the factors conditioning the variability of positive inotropic response after intravenous acute amrinone administration, 14 patients with chronic cardiac failure were studied by quantitative M-mode and cross-sectional echocardiography. Six patients had idiopathic dilated cardiomyopathy and 8 patients had severe chronic aortic insufficiency. Myocardial contractility (evaluated as peak arterial systolic pressure/end-systolic volume ratio: PAP/ESV) did not change in patients with idiopathic cardiomyopathy, a significant increase of myocardial contractility occurred in patients with aortic regurgitation only if the control value of PAP/ESV was greater than 1. Mean systolic wall stress decreased significantly in all patients, independent of aetiology of cardiac failure and was the factor determining the improvement of left ventricular performance (evaluated as fractional shortening) in patients without changes in myocardial contractility. Maximum improvement in left ventricular performance occurred 10 minutes after amrinone administration. It is concluded that the possibility of detecting the positive inotropic properties of amrinone in man depends on the aetiology of the cardiac failure and on the basal level of myocardial contractility.
Adult↗
[Heart transplant patients: outlines for the prevention, diagnosis and therapy of rejection, infections and other complications].
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Graft Rejection↗
[Hypertrophic cardiomyopathy and juvenile sudden death. A clinical case and re-evaluation of the problem].
A 29-year-old man with non-familiar hypertrophic cardiomyopathy died suddenly. The patient experienced recurrent episodes of palpitation both at rest and during exercise. M-mode and 2-D echocardiograms showed severe asymmetric septal hypertrophy (IVS/LVPW ratio 3:1) and systolic anterior motion of anterior leaflet of the mitral valve. The ECG demonstrated deep "Q" waves from the inferior leads and inverted "T" waves in V1 to V6, but failed to register arrhythmias. Holter monitoring had been planned in order to achieve information for pharmacologic therapy, but unfortunately the patient died during the stand-by. Postmortem examination showed severe asymmetric septal hypertrophy with fibrosis and wide-spread "myocardial disarray".
Adult↗
Uncommon pattern of tricuspid stenosis in Ebstein's anomaly.
The echocardiographic and cineangiographic features of an unusual type of tricuspid stenosis in Ebstein's anomaly are described. The displaced septal and mural leaflets of the tricuspid valve are inserted to an anomalous apical muscle shelf and cause a restrictive communication between the atrialized portion and the trabecular zone of the right ventricle. The antero-superior leaflet is not involved.
Adult↗
Effect of muzolimine in aortic insufficiency with different levels of left ventricular function.
The effect of muzolimine (initial dose 60 mg followed by 30 mg/day for 14 days) on 15 patients with aortic and mitral incompetence and chronic cardiac failure, functional class 3, was analyzed on the basis of repeated clinical observations, metabolic studies as well as mono and bidimensional echo changes 2, 24 hours and 15 days after the initial dose. An important variation was a reduction in body weight by 3-4% after the first day and by a further 1-2% after 2 weeks. No significant variations were observed regarding heart rate and blood pressure at rest. Plasma level of electrolytes as well as other important biochemical parameters in the serum (BUN, glucose and creatinine) were uninfluenced. A small increase was observed in both the plasma level of chloride and in the red cell count. Urine volume increased by two to threefold after the first day; this increase was still present after 2 weeks. Changes in the left ventricular end-diastolic and end-systolic volumes were not significant after the first day; however a slight but significant reduction was observed after 2 weeks and was in parallel with the improvement of the functional class of the patients. Data, in particular the possible mode of action of muzolimine after acute administration and after short-term therapy, are discussed.
Adult↗
Relation between myocardial contractility, hypertrophy and pump performance in patients with chronic aortic regurgitation: an echocardiographic study.
The aim of this study was to establish prognostic indices and to detect irreversible left ventricular dysfunction before aortic valve replacement in patients with chronic aortic regurgitation. Therefore, we determined the left ventricular pump/contractility relation (expressed as a ration between ejection fraction and peak systolic pressure/end-systolic volume), afterload mismatch (the relation between the ejection fraction and mean systolic wall stress) and the left ventricular end-diastolic radius/posterior wall thickness ratio in 52 patients with chronic aortic regurgitation by means of M-mode echocardiography. These indices were also calculated in 14 patients with idiopathic dilated cardiomyopathy and in 20 normal controls. The indices allowed separation of normals and patients with mild to moderate aortic insufficiency from patients with idiopathic dilated cardiomyopathy indicating that these indices could distinguish between patients with a reversible afterload mismatch and those with irreversible impaired muscle function. The 38 patients with severe aortic insufficiency showed a large overlap between normal and abnormal indices. Twelve of these patients had a pump/contractility index and a ratio between ejection fraction and wall stress similar to that found in patients with idiopathic dilated cardiomyopathy. All these patients, however, had a left ventricular end-diastolic radius/thickness ratio greater than 4 indicating "inadequate hypertrophy". Of these, 3 patients died perioperatively and 4 developed congestive heart failure within 12 months postoperatively. We conclude that an end-diastolic radius/thickness ratio greater than 4 is a sensitive but not a specific preoperative indicator of irreversible left ventricular damage in patients with chronic aortic regurgitation. This index is readily obtained preoperatively by M-mode echocardiography.
Adolescent↗
Dysplasia of the atrioventricular valves associated with conduction system anomalies.
Clinical, vectorcardiographic, and echocardiographic data from two siblings with atrial septal defects and dysplasia of the mitral and tricuspid valves are reported. Vectorcardiograms showed that both siblings had abnormal ventricular activation with initial electrical forces directed posteriorly. One sibling died after surgery, and necropsy showed incomplete differentiation of the leaflets and tensor apparatus producing anomalies resembling "mitral arcade." Serial histological examination of the conducting tissue showed that the atrioventricular node was located on the left side of the atrial septum, that the central fibrous body and the membranous septum were hypoplastic, and that an accessory nodoventricular pathway originating in the compact node joined the left side of the ventricular septum. This accessory pathway was probably the cause of the unusual ventricular activation. Dysplasia of the mitral and tricuspid valves together with hypoplasia of the central fibrous body and the presence of accessory pathways are probably part of a malformative complex caused by incomplete differentiation of both the cardiac atrioventricular valves and the junctional area.
Child↗