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Biomedical subjects

A Nigri

Publications and source records attributed to A Nigri.

At least 19 recordsLinked to original sources

Treatment of homozygous and double heterozygous familial hypercholesterolemic children with LDL-apheresis.

Within the framework of a seven-year clinical experience on treatment of severe hyperlipoproteinemia with/without associated coronary heart disease, with therapeutic plasmapheresis (APO B-100-containing lipoprotein-apheresis), we focused the present report on two young patients aged 7 and 11 years, respectively. The older patient is a boy treated since 1990 by plasma-exchange, cascade filtration-low density lipoprotein apheresis (LDL-apheresis), and dextrane sulphate-LDL apheresis. Over the treatment period the patient was submitted to three consecutive coronary angiographies. The second is a girl first submitted to a coronary angiography and then treated with dextrane sulphate-LDL apheresis. Up to now, a total of one-hundred therapeutic plasmaphereses have been performed. The interval of treatment was of fifteen days, and a volume of 2-3000 ml of plasma was processed at each session. The systems used were the following: DIDECO Vivacell BT 798-A, DIDECO Vivacell BT 798-A + BT 803, DIDECO BT 985 (Dideco, Mirandola, Italy), KANEKA MA-01 (Kanegafuchi, Osaka, Japan). Mean (SD) plasma apo B-100-containing major lipoprotein-LDL, Lp(a)-levels during treatment, are reported below: [table: see text] The treatment was very well tolerated. Rare, moderate hypotensive events occurred. Nevertheless, all procedures were regularly completed. A mild hypochromic anemia, regressed using drug treatment, was observed in the boy. Along with the improvement of plasma atherogenic profile, a regression of skin xanthomas and unchanged favourable coronary angiograms, were obtained in the above mentioned patient.

Apolipoproteins A

Histomorphometric features predict 1-year outcome of patients with idiopathic dilated cardiomyopathy considered to be at low priority for cardiac transplantation.

Cardiac transplantation for patients with idiopathic dilated cardiomyopathy (IDC) and poor left ventricular function usually is postponed until symptoms have become intolerable. However, the short-term prognosis of this subset of patients has been defined poorly. Accordingly, the 1-year outcome was investigated in 30 patients with IDC with an ejection fraction < or = 25% who showed a stabilized clinical condition at assessment for transplantation and were therefore considered at low priority for surgery. During follow-up, 10 patients (group A) showed a poor outcome: 2 died suddenly, and 8 had hemodynamic failure (4 of whom underwent transplantation and 4 of whom died from heart failure while on the waiting list). The remaining 20 patients (group B) had a benign outcome. At assessment for cardiac transplantation, clinical and electrocardiographic features, left ventricular dimension, and ejection fraction were similar between the two groups. However, group A patients had higher left ventricular end-diastolic pressure (p < 0.03) and lower cardiac index (p < 0.02) and stroke volume index (p < 0.03) with respect to group B patients. In addition, the former had a lower myofibril volume fraction (p < 0.001) and a higher nuclear area (p < 0.001) compared with the latter. Multivariate analysis selected myofibril volume fraction (p < 0.001) and nuclear area (p < 0.005) as the only independent predictors of a poor 1-year outcome. The combination of myofibril volume fraction < or = 89% and nuclear area > 50 microns 2 was found in all group A patients (sensitivity 100%) but in only 2 group B patients (specificity 90%). It is concluded that in patients with IDC considered at low priority for cardiac transplantation: (1) the 1-year freedom from a cardiac event is lower than that currently expected with surgery; (2) histomorphometric features, that is, the concurrency of low myofibril volume fraction and increased nuclear area, predict short-term outcome; and (3) endomyocardial biopsy at assessment for cardiac transplantation might improve the rationalization of the timing of the procedure.

Adult

Angina and normal epicardial coronary arteries: radionuclide features and pathophysiological implications at long-term follow-up.

BACKGROUND: The aim of this study was to identify specific characteristics associated with modifications of symptoms and to evaluate the presence of a pathophysiological link between radionuclide abnormalities and delayed run-off of coronary contrast dye in patients with angina and normal coronary arteries. METHODS: We followed up 53 patients (21 men and 32 women, mean age: 52 +/- 10 years) with angina and angiographically normal epicardial coronary arteries, 21 of whom (40%) displayed a pattern of slow contrast dye run-off from coronary vessels, on visual assessment using a semiquantitative empirical score. Exercise tests showed ECG abnormalities in 29 patients (55%). RESULTS: All patients were alive 92 +/- 44 months after catheterization (140 +/- 79 months after beginning of symptoms). However, 30 patients (57%), who reported worsening or no change of symptoms, presented with a non-significant higher prevalence of conduction abnormalities at rest ECG (27 versus 17%), pathological exercise tests (57 versus 52%), and delayed run-off (47 versus 30%). Regional left ventricular function and perfusion were then simultaneously assessed at rest and peak exercise with 99mTc-sestamibi. Exercise-induced radionuclide abnormalities were detected in 27 patients (51%), who also presented with a non-significant higher prevalence of pathological exercise tests (63 versus 43%) and no improvement of symptoms (63 versus 46%). However, exercise-induced functional and perfusional abnormalities were simultaneously present in 29 out of 42 (69%) coronary territories supplied by vessels with delayed run-off, versus 21 out of 117 (17%) normal territories (P = 0.00032). CONCLUSIONS: Despite a good prognosis, some patients with angina and normal coronary arteries presented no improvement of symptoms at follow-up, and had functional and perfusional abnormalities in coronary territories supplied by vessels showing delayed contrast dye run-off.

Angina Pectoris

Clinical significance of small left-to-right shunts after percutaneous mitral valvuloplasty.

Left-to-right shunt after percutaneous mitral valvuloplasty was evaluated by contrast echocardiography in 29 patients at 24 hours and at 1, 3, 6, and 9 months after the procedure. The patients were divided into two groups: in group A (13 patients) the double-balloon technique was used; in group B (16 patients) the Inoue single-balloon technique was used. The two groups were comparable in terms of age, gender, and mitral valve area before and after percutaneous mitral valvuloplasty. A left-to-right shunt was detected in all patients 24 hours after the procedure. At 1 month follow-up the shunt was present in 12 patients of group A (92%) and in 13 of group B (81%) with a statistically significant difference (p < 0.001). At 3 months the values were 7 (54%) in group A and 6 (37.5%) in group B (p < 0.05); at 6 months the values were 3 (23%) in group A and 3 (19%) in group B (NS). At 9 months a left-to-right shunt was no longer detectable in any of the patients in either group. The disappearance of the shunt could be related to a healing process of the atrial septal injury that occurs within a few months after percutaneous mitral valvuloplasty. This process seems to be more rapid in group B patients, probably because of the smaller lesion that is produced in the atrial septum by the passage of the Inoue balloon.

Adult

Resting and ambulatory ECG predictors of mode of death in dilated cardiomyopathy.

With the purpose of verifying whether the electrocardiogram (ECG) pattern alone can predict the mode of death in dilated cardiomyopathy, data from 12-lead ECGs and 48-hour arrhythmia monitoring were evaluated in 67 patients with dilated cardiomyopathy. During a mean follow-up period of 3 +/- 2 years, death from congestive heart failure occurred in 18 patients (27%), whereas 10 (15%) died suddenly (NS). Multivariate analysis showed that left bundle branch block (p < 0.001) and left atrial enlargement (p < 0.001) were independently related to death from congestive heart failure. Ventricular arrhythmias of Lown grade 4A or 4B (p < 0.001) and repolarization time, as assessed by QTc-QRS interval (p < 0.05), were independent predictors of sudden death. It is concluded that ECG features alone may be helpful for risk factor characterization of dilated cardiomyopathy patients, provided that multiple ECG criteria are utilized at time of diagnosis.

Adolescent

Transcatheter ablation of the accessory pathway in 2 patients with life-threatening tachyarrhythmias.

Successful transcatheter ablation of an accessory pathway in 2 patients with life-threatening tachyarrhythmias is reported. In both cases, electrophysiological evaluation documented the posterior septal location of the anomalous atrioventricular connection. Radiofrequency current energy was delivered just below the coronary sinus orifice, and resulted in disappearance of preexcitation. During the follow-up, both patients remained free from tachyarrhythmias on no medication, and serial electrocardiographic recordings confirmed the persistent absence of preexcitation. It is concluded that ablation of accessory pathways can be accomplished easily and without risk by means of the radiofrequency catheter technique. This therapeutic modality requires a short hospitalization and can be recommended as the first option in patients with the Wolff-Parkinson-White syndrome.

Adult

Electrocardiographic correlates with left ventricular morphology in idiopathic dilated cardiomyopathy.

The purpose of the present study was to verify whether the electrocardiographic pattern of patients with idiopathic dilated cardiomyopathy (IDC) might be useful in predicting measurements of left ventricular (LV) morphology. A total of 12 electrocardiographic criteria for LV enlargement were evaluated in 67 patients with IDC, aged 14 to 68 years (mean 48), and were correlated to LV wall thickness, volume and mass, as assessed at angiography (all patients) and echocardiography (50 patients). Linear regression analysis showed weak correlations between multiple electrocardiographic criteria and LV wall thickness, volume and mass. Multiple logistic regression analysis showed that total 12-lead QRS amplitude, voltage criteria of Sokolow and Lyon, overshoot and U-wave inversion were the variables significantly related to LV wall thickness, as assessed by angiography (r = 0.55, p less than 0.005) and echocardiography (r = 0.43, p less than 0.025). The sum of T/R-wave ratios, the RV6/RV5 ratio and the Romhilt-Estes score were predictors of LV end-diastolic volume, as determined by angiography (r = 0.83, p less than 0.001) and echocardiography (r = 0.77, p less than 0.005). Total 12-lead QRS amplitude and the sum of T/R-wave ratios were the only independent predictors of LV mass, either angiographically (r = 0.81, p less than 0.001) or echocardiographically measured (r = 0.71, p less than 0.025). It is concluded that a single electrocardiographic criterion for prediction of LV morphology in patients with IDC is barely effective. Multiple electrocardiographic criteria should be utilized to better predict LV mass and distinguish reliably between LV wall thickening and dilatation.

Adolescent

[Relations between ACE inhibition with captopril and atrial natriuretic factor during an acute hemodynamic study].

Aim of this study was to evaluate if captopril treatment may directly alter the trial natriuretic factor (ANF) concentration. Six patients (2 male and 4 female) aged 53 +/- 11 years, with mitral stenosis, and atrial fibrillation, underwent cardiac catheterization in our Institution. The following parameters were evaluated: heart rate, right atrial and pulmonary capillary wedge pressure, aortic and pulmonary pressure, cardiac index, pulmonary and systemic resistances and ANF concentration in coronary sinus, pulmonary, artery, aorta, peripheral vein. All these parameters were measured before and 30 and 60 min after captopril administration (50 mg orally). No hemodynamic changes occurred after captopril administration. No changes in ANF concentration occurred in comparison with baseline levels, after 30 and 60 min in coronary sinus (199.8 +/- 151.5 vs 181.9 +/- 102.5 fmol/ml; 178.4 +/- 95.2 vs 181.9 +/- 102.5 fmol/ml), in pulmonary artery (58.3 +/- 36.6 vs 51.4 +/- 48.8 fmol/ml; 35.5 +/- 16.9 vs 51.4 +/- 48.8 fmol/ml), in aorta (29.7 +/- 22.7 vs 37.5 +/- 26.3 fmol/ml; 25.2 +/- 9.8 vs 37.5 +/- 26.3 fmol/ml); and in peripheral vein (14.6 +/- 7.9 vs 17.3 +/- 9.7 fmol/ml; 16.2 +/- 12.2 vs 17.3 +/- 9.7 fmol/ml). In conclusion our data show that, providing no hemodynamic changes occur, captopril administration does not alter ANF concentration.

Adult

[Primary pulmonary hypertension: a retrospective study of 13 patients].

Twenty-four patients (9M and 15F, mean age 28 years) with primary pulmonary hypertension underwent cardiac catheterization in our institution from 1955 to 1989. The prevalence of the disease in our population was lower (0.2%) than that reported by other Authors (1%). Thirteen of these patients (4 M and 9 F, mean age 32 years) evaluated between January 1979 and December 1989, were followed. Five were alive after 52 +/- 30 months (Group A) while 8 died after 11 +/- 9 months (Group B). In Group B mean pulmonary pressure was significantly higher than in Group A (66.7 +/- 17.2 vs 41.2 +/- 19.0 mmHg, p less than 0.05, respectively). Cardiac index and systolic volume index were lower in Group B than in Group A (2.07 +/- 0.85 vs 3.72 +/- 1.32 l/min/m2, p less than 0.01 and 24.43 +/- 10.25 vs 41.08 +/- 16.97 ml/m2, p less than 0.05, respectively). Pulmonary resistance index and systemic resistance index were higher in Group B than in Group A (3039 +/- 1519 vs 1181 +/- 1236 dyne x s x cm-5/m2, p less than 0.01; 4277 +/- 1794 vs 2309 +/- 1238 dyne x s x cm-5/m2, p less than 0.01). One patient underwent repeated cardiac catheterization after 2 years. This patient showed a deterioration of the hemodynamic parameters, consistent with the worsening of the clinical conditions. In conclusion, in our population of patients with primary pulmonary hypertension, an increase in pulmonary artery pressure and pulmonary resistances, as well as a decrease in cardiac index, are associated with a reduced life expectancy. On the other hand, right atrial pressure does not affect mortality.

Adolescent

[Winging effect in interatrial defect after transseptal mitral valvuloplasty: an anatomical study].

In the balloon catheters the redundancy of the deflated balloon produces 2 or 3 sort of thin wings. The presence of wings can reduce the catheter "pushability" or, in certain conditions, can determine tissue lesion (winging effect). Simulating a transeptal valvuloplasty of the mitral valve by technique of the 2 balloon in human fresh heart, we studied the winging effect over the interatrial septum in 12 hearts; in the first 6 the atrial septum was dilated with a 6 mm balloon (Group A), in the second 6 the atrial septum was dilated with a 10 mm balloon (Group B). The procedure was completed introducing consecutively 2 bigger balloons (15 + 20 mm). We suppose that the winging effect of the 2 bigger balloons (15 + 20 mm) could counter balance the theoretical advantage of a small balloon (6 mm). The atrial septal defect (ASD) after septal dilatation was 5.0 +/- 0.59 mm x 1.56 +/- 0.25 mm (long axis x short axis) in Group A and 6.53 +/- 0.35 x 2.16 +/- 0.39 mm in Group B (p less than 0.01). The final ASD (after introducing the 2 bigger balloons) was 7.04 +/- 1.06 x 2.36 +/- 0.57 mm in Group A and 7.03 +/- 0.18 x 2.16 +/- 0.32 mm in Group B (NS). Our data show that the winging effect can determine biological negative effects.

Catheterization

Hemodynamic and clinical effects of captopril in patients with severe congestive heart failure.

In a group of 10 patients (9 male, 1 female, aged 16 to 56, mean 43 years) with severe congestive heart failure (CHF), due to ischemic heart disease (8 cases) or dilatative cardiomyopathy (2 cases), the acute hemodynamic response to captopril (CPT) (90 min after 50 mg po) was evaluated. CPT was then given starting with 15 mg q 8 hr, gradually increased within 2 or 3 days to 50 or 75 mg q 8 hr. A hemodynamic re-evaluation was performed after 20 days, and, in 5 patients, after 1 year of CPT treatment. The clinical follow-up done every 3 months in the outpatient clinic. CPT acutely produced a decrease in mean pulmonary wedge pressure: 23.8 +/- 8.9 to 18.6 +/- 8.6 mmHg, p less than 0.01, and an increase in cardiac index: 2.43 +/- 0.73 to 2.91 +/- 0.85 l/min/m2, p less than 0.01; systemic and pulmonary resistances decreased significantly, with no significant changes in heart rate and in mean brachial artery pressure. This favourable hemodynamic response persisted after 20 days of CPT treatment. In the group of 5 patients, who underwent a third hemodynamic evaluation, no statistically significant differences were demonstrated in respect to the previous control values. All patients on chronic CPT treatment experienced a gradual clinical improvement, reaching a steady state after 2 to 3 weeks; the surviving patients remained stable in the improved functional class for at least 6 months. The mortality rate was 30% in the first year, increasing to 50% in the second and to 60% in the third year of treatment. After a mean follow-up 35.7 months (range 33 to 39) the 4 surviving patients remained in the same functional class as after the first year for therapy. In our CHF patients CPT improved the quality of life, with the best clinical and hemodynamic results the first few months of treatment; life expectancy probably was not affected.

Adolescent

[Idiopathic dilated cardiomyopathy: clinical and prognostic significance of the morpho-functional involvement of the right heart].

Aim of this study was to evaluate whether different severity of clinical and functional impairment of right heart in patients with idiopathic dilated cardiomyopathy (IDC) might condition distinct clinical features and prognosis. From 104 consecutive patients with hemodynamically assessed diagnosis of IDC studied between 1977 and 1987 in our Institute, 39 patients (28 males and 11 females, mean age 41 +/- 14 years) were selected on the basis of ejection fraction ranging from 35 to 50%, left ventricular end-diastolic pressure ranging from 13 to 20 mmHg and left ventricular end diastolic volume less than or equal to 150 ml/m2. A significant involvement of right heart (diagnosed according to a mean right atrial pressure greater than or equal to 9 mmHg, a right ventricular end-diastolic pressure greater than or equal to 9 mmHg and a right ventricular end-diastolic diameter greater than or equal to 30 mm) was assessed in 16 patients (41%), 11 males and 5 females, aged 40 +/- 15 years (Group A). On the contrary the remaining 23 patients (59%), 17 males and 6 females, aged 42 +/- 12 years, had a normal right heart (Group B). At entry into the study, clinical features appeared similar in the 2 groups of patients whereas patients of Group A had significantly higher incidence of atrial fibrillation (25% vs 4%, p less than 0.01) and complex ventricular arrhythmia (greater than or equal to 4 Lown class) (25% vs 4%, p less than 0.01) compared with patients of Group B. With respect to conduction defects no difference was found between Group A and Group B.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult

[Predictive value of myocellular hypertrophy in idiopathic dilated cardiomyopathy].

The role of endomyocardial biopsy (EMB) in assessing of idiopathic dilated cardiomyopathy (IDCM) is a well-recognised one. On the contrary, the value of correlating histological features, such as myocellular hypertrophy, with functional evaluation and outcome is still controversial. It was the purpose of the present study to appraise the correlation with hemodynamic data and the predictive role of histological features in 32 consecutive patients affected by IDCM who underwent left ventricular EMB between January 1984 and December 1986. Light microscopy findings were graded by means of a semiquantitative score system. In comparison with the 19 patients with mild myocellular hypertrophy, the 13 patients with marked hypertrophy showed significantly lower right ventricular end-diastolic pressure (10.4 +/- 5.8 vs 6.6 +/- 3.6 mmHg, p less than 0.05) and left ventricular end-diastolic pressure (26.9 +/- 9.0 vs 16.5 +/- 8.8 mmHg, p less than 0.01). On the contrary, different degrees of interstitial fibrosis, as well as of the other morphologic findings, could not identify patients with distinct hemodynamic patterns. However, there was a direct correlation between the amount of myocellular hypertrophy and interstitial fibrosis (p less than 0.01). During a mean follow-up period of 32 +/- 11 months (range: 12-48 months), 6 patients died and 3 further patients underwent heart transplantation. Univariate analysis of histological features (log-rank test) showed a mild degree of hypertrophy alone to be significantly related to a poor outcome (p = 0.02).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult