Reversible acute global left ventricular dysfunction in a patient with autosomal recessive dystrophic epidermolysis bullosa.
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Biomedical subjects
A Sgreccia
Publications and source records attributed to A Sgreccia.
Congestive heart failure in hypertensive patients with normal systolic function and dynamic left ventricular outflow obstruction.
BACKGROUND: Hypertensive patients with left ventricular hypertrophy and normal systolic function can develop congestive heart failure refractory to conventional drug therapy with digoxin, diuretic, and vasodilators. METHODS: We studied 8 patients with a history of systemic hypertension (6 females and 2 males, mean age 69+/-6 years), affected by New York Heart Association (NYHA) class IV congestive heart failure notwithstanding conventional drug therapy with digoxin, diuretic, and vasodilators. After clinical history and physical examination, blood chemistry including cardiac enzymes, arterial blood gases, chest roentgenogram, standard 12-lead ECG, and complete echocardiographic study were performed in all patients. RESULTS: In all cases, a left ventricle with increased wall thickness, normal cavity size, and normal or supernormal systolic function was shown. All patients had left ventricular systolic dynamic obstruction, with peak gradient between 36 and 130 mmHg (mean 83+/-31). After having stopped treatment with nitrates, digoxin, and diuretics, drug therapy with calcium channel antagonists or beta-blockers was started, and rapid clinical improvement with disappearance of left ventricular outflow obstruction was observed. CONCLUSIONS: Sometimes, a distinction between several forms of heart failure is clinically impossible. However, when conventional therapy is not effective in patients with longstanding history of systemic hypertension and ECG signs of left ventricular hypertrophy, diastolic heart failure and/or dynamic left ventricular obstruction should be suspected. Thus, an early echocardiographic study should be performed.
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Systemic sclerosis (scleroderma). A case of recovery of cardiomyopathy after vitamin E treatment.
A 60-year-old woman with systemic sclerosis, systemic hypertension, and chronic renal failure, presented with clinical manifestations of heart failure. An echocardiogram showed a mildly dilated left ventricle and global hypokinesis. A six-month treatment including reduced sodium intake, furosemide, and nifedipine did not change the clinical and instrumental findings. Casually, vitamin E (600 mg daily) was added. After 6 months, clinical manifestations of heart failure were disappeared and the echocardiogram showed a normally-sized left ventricle with normal wall motion.
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Left ventricular diastolic function in systemic sclerosis.
OBJECTIVE: To assess left ventricular diastolic function in patients with systemic sclerosis (SSc) and to verify if a "primary" diastolic dysfunction might exist. METHODS: In total 124 patients and 41 healthy subjects underwent complete echocardiographic examination. The following pulsed wave Doppler variables were evaluated: peak velocity during early filling (E), peak velocity during late atrial filling (A), E/A ratio, and early filling deceleration time. RESULTS: Seventy-seven patients (62.1%) had conditions potentially affecting left ventricular diastolic function (Group A) and 47 patients (37.9%) formed a homogeneous group without cardiac involvement or other causes of abnormal diastolic function (i.e., systemic and/or pulmonary hypertension, ventricular hypertrophy, pericardial disease, systolic dysfunction, valvular heart disease, coronary artery disease) (Group B). The entire SSc population and Group A showed significant differences in the Doppler variables of diastolic function compared to the control group. No significant differences were found between Group B and controls. CONCLUSION: In patients with SSc, left ventricular diastolic dysfunction was found only in patients with conditions potentially affecting left ventricular diastolic function. In patients without conditions potentially affecting left ventricular diastolic function no differences were seen in comparison with controls. SSc does not seem to cause "primary" diastolic abnormalities.
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Cardiac involvement in patients with Wegener's granulomatosis.
Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis, which could potentially affect any organ system. However, there have only been a few reports on cardiac involvement. We described the echocardiographic findings in nine patients affected by WG. A complete M-mode, two-dimensional, Doppler and color-Doppler transthoracic echocardiogram was performed in nine patients (seven females and two males) affected by WG. In each patient, cardiac abnormality, for example, valvular damage, left ventricular global systolic dysfunction, or pericardial effusion, was detected. In particular, heart valve disease was found in eight patients, and in three cases, aortic valve insufficiency, which was severe enough to require surgical valve replacement, was observed. Cardiac involvement in patients with WG is common. In particular, there is a high frequency of aortic valve abnormalities. Thus, an echocardiographic study should be routinely performed.
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Pulmonary hypertension is associated with impaired exercise performance in patients with systemic sclerosis.
OBJECTIVE: The aim of this study was to evaluate the exercise tolerance by expired gas analysis during stress test in patients with Systemic Sclerosis (SSc). METHODS: Eighteen women (mean age 48.56+/-12.48 years) affected by SSc were studied. A complete echocardiographic examination including pulmonary artery systolic pressure estimation, pulmonary function tests, diffusion lung capacity for carbon monoxide (DLCO), and exercise test were performed. During exercise, breath-by-breath expired gas analysis was performed. RESULTS: Seven patients (39%) had baseline pulmonary systolic hypertension (group A) and 11 patients (61%) did not (group B). Six patients had reduced DLCO values. Both maximal oxygen consumption (VO2max) and anaerobic threshold (VO2AT) values were markedly decreased compared to the predicted values. Seven of 18 patients were unable to complete a maximal exercise (5 of whom affected by pulmonary systolic hypertension). Group A patients showed reduced VO2max, VO2AT, and O2 pulse compared with patients with group B patients (p=0.004, 0.017, and 0.013, respectively); VO2max, VO2AT and O2 pulse were significantly correlated to baseline pulmonary artery systolic pressure. CONCLUSIONS: An exercise intolerance in patients affected by SSc is present. Impairment of exercise performance is associated with pulmonary hypertension.
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Multiple cerebral infarcts from dislodgement of mobile apical left ventricular thrombus: therapeutic implications.
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QT interval and QT dispersion in systemic sclerosis (scleroderma).
OBJECTIVES: To measure QT interval and QT dispersion, and to evaluate possible relationships between these measurements, heart rate variability parameters, and early myocardial involvement in patients with systemic sclerosis (SSc). DESIGN: Prospective study. SETTING: Tertiary care centre, University 'La Sapienza', Rome, Italy. SUBJECTS: Thirty-eight patients with SSc (35 females and three males, mean age 47 +/- 11 years), 19 patients with the diffuse form of disease and 19 with the limited form, and 17 healthy controls (11 females and six males, mean age 43 +/- 10 years) were studied. INTERVENTIONS: Both patients and control subjects underwent resting 12-lead electrocardiogram and 24-hour Holter monitoring. Moreover, resting myocardial scintigraphy with 99 m Tc-sestamibi was performed in all SSc patients. MAIN OUTCOME MEASURES: Bazett's formula was used to obtain rate corrected value of QT interval (QTc). QT and QTc dispersion were defined as the difference between maximum and minimum QT or QTc interval, respectively. Twenty-four-hour heart rate variability was analysed both in the frequency and in the time domain. RESULTS: Twenty-three SSc patients (60.5%) had myocardial resting perfusion defects (group A) and 15 (39.5%) did not (group B). Maximum QTc interval, QT and QTc dispersion were significantly increased in SSc patients compared to the control subjects. No significant differences between group A and group B were observed for all QT measurements. Furthermore, maximum QTc interval, QT dispersion and QTc dispersion were significantly increased in group A patients compared to the control group. Total power, low-frequency, and high-frequency values were significantly lower in all SSc patients, whether in group A or group B, than in control subjects. On the other hand, low-frequency/high-frequency ratio was similar in all groups. Heart rate variability in time domain analysis showed no statistically significant differences between groups. CONCLUSIONS: Patients with SSc have increased QTc interval, QT dispersion, and QTc dispersion. The role of autonomic nervous system and myocardial involvement on ventricular repolarization in patients with SSc needs further investigation.
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Relationship between cutaneous and pulmonary involvement in systemic sclerosis.
OBJECTIVE: Pulmonary disease may shorten survival in patients affected by systemic sclerosis (SSc). However, pulmonary involvement may commonly be silent, whereas skin fibrosis is usually the clinical feature drawing most attention. We investigated the relationship between cutaneous and pulmonary involvement during SSc. METHODS: We studied 52 patients (mean age 50.2 +/- 13.7 years) affected by SSc (mean duration of disease 13.8 +/- 9.5 years). Twenty-eight had the diffuse form of the disease (dSSc) and 24 the limited form (lSSc). All patients underwent pulmonary function studies, high resolution computed tomography (HRCT) of the lungs, and complete echocardiographic examination. Pulmonary artery systolic pressure was measured by Doppler echocardiography. Pulmonary interstitial fibrosis and skin fibrosis were evaluated using a point system. RESULTS: Mean percentages of predicted values of forced vital capacity and total lung capacity were significantly reduced in patients with dSSc compared to lSSc (80.0 +/- 18.9 vs 98.4 +/- 16.8%, p < 0.001; and 81.3 +/- 13.9 vs 92.1 +/- 14.2%, p < 0.01, respectively). The overall HRCT score was 6.1 +/- 4.9, with no significant differences between disease subgroups. However, a HRCT score of 10 or more was present in 10 patients with dSSc vs 2 patients with lSSc (p = 0.02). Pulmonary hypertension was present in 27 patients, 15 with lSSC and 12 with dSSc (p = NS). No significant correlation was observed between skin score and lung volumes, carbon monoxide diffusing capacity, HRCT score, or pulmonary artery systolic pressure for all patients and subgroups. CONCLUSION: Extent and severity of cutaneous and pulmonary involvement in SSc are not directly correlated. Nevertheless, different patterns of pulmonary involvement between SSc subgroups were observed. Restrictive lung disease was more frequent in patients with dSSc, while a trend to higher prevalence of pulmonary hypertension was observed in patients with lSSc.
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Noninvasive assessment of myocardial involvement in patients with systemic sclerosis: role of signal averaged electrocardiography.
OBJECTIVE: To assess the role of late ventricular potentials (LVP) in detecting early myocardial involvement in patients with systemic sclerosis (SSc). METHODS: Seventy-seven patients with SSc (68 women, 9 men, mean age 50 +/- 13 yrs) and 33 control subjects (18 women, 15 men, mean age 46 +/- 10 yrs) underwent resting electrocardiogram (ECG), 24 h Holter monitoring, complete echocardiographic and Doppler echocardiographic examination, and signal averaged ECG at high pass setting of 40 Hz, with the low pass fixed at 250 Hz. Patients with SSc underwent resting myocardial scintigraphy and radionuclide angioventriculography. RESULTS: The prevalence of LVP at 40 Hz was 20.5%. Compared to control subjects, patients with SSc showed higher prevalence of septal infarction pattern (p = 0.05), complex ventricular arrhythmias (p = 0.03), pulmonary arterial hypertension (p < 0.001), and LVP (p = 0.02). Forty-four patients with SSc (57.1%) had resting perfusion defects by myocardial scintigraphy. Fourteen of 15 patients with LVP showed perfusion defects compared to 29 of 58 without LVP (p = 0.002). Linear regression analysis showed that myocardial perfusion defect score was significantly correlated to either the filtered QRS duration, or the duration of low amplitude signals < 40 microV of the terminal QRS, or the root mean square voltage of the last 40 ms of the QRS complex. After a mean followup period of 20 months, 8 patients died. In 2 patients who died suddenly, LVP were present. CONCLUSION: Signal averaged ECG is a sensitive and inexpensive technique in the clinical assessment and followup of patients with SSc.
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Relationships between electrocardiographic and echocardiographic findings in systemic sclerosis (scleroderma).
We assessed the prevalence of electrocardiographic abnormalities in patients with systemic sclerosis and evaluated their functional significance through a comparison with echocardiographic findings. Seventy-two patients with systemic sclerosis and 64 controls underwent resting electrocardiogram (ECG) and M-mode, two-dimensional, Doppler and color Doppler echocardiography. Electrocardiographic abnormalities were observed in 48.7% of patients. Conduction disturbances (27.7%) infarction pattern (13.8%), non-specific ST-T wave changes (13.8%) and right ventricular hypertrophy (11.1%) were the most frequent abnormalities. QTc interval was significantly longer in patients with systemic sclerosis than in controls. Significant differences between patients and controls were found in the prevalence of long QTc interval (p = 0.0016) infarction pattern (p = 0.0016), right ventricular hypertrophy (p = 0.007) and non-specific ST-T wave abnormalities (p = 0.0016). All patients with infarction pattern and 90% of patients with prolonged QTc interval had some echocardiographic abnormalities. Electrocardiographic signs of right ventricular hypertrophy were 16% sensitive and 93% specific for pulmonary hypertension; the sensitivity and specificity of the combination of right ventricular hypertrophy, right atrial enlargement and right bundle branch block were 35% and 90%, respectively. Standard ECG is useful to assess cardiac involvement in patients with systemic sclerosis. If infarction pattern, right ventricular hypertrophy or long QTc interval are present, a cardiac involvement is very likely.
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Twenty-four hour heart period variability in systemic sclerosis.
OBJECTIVE: In patients with systemic sclerosis (SSc), an autonomic dysfunction has been implicated in the pathogenesis of Raynaud's phenomenon and gastrointestinal symptoms. To evaluate cardiovascular autonomic function we analyzed the heart rate variability (HRV) on 24 h electrocardiogram recordings. METHODS: In 50 patients diagnosed with SSc and 24 control subjects HRV was analyzed for both frequency and time domain. RESULTS: Frequency domain analysis showed reduction of low frequency and high frequency values in the patients with SSc compared to controls (5.52 +/- 0.74 vs 6.04 +/- 0.78 In(ms2), p = 0.007; and 4.17 +/- 0.97 vs 4.74 +/- 0.97 In(ms2), p = 0.021, respectively). Analysis in time domain showed no significant differences in any variable between groups. There were no significant differences between the diffuse and the limited form of SSc. Number of ventricular ectopic beats, prevalence of frequent ventricular ectopy, and supraventricular tachycardia episodes were increased in the patients with SSc compared to controls. CONCLUSION: Spectral analysis of heart period variability suggests cardiovascular autonomic dysfunction in patients with SSc. Better understanding of this complication could help delineate new strategies for the treatment of SSc.
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Atrial septal aneurysm and cardiac arrhythmias.
Atrial septal aneurysm has been associated with thromboembolic events, interatrial shunting, mitral valve prolapse, and systolic click. An association between atrial septal aneurysm and cardiac arrhythmias has been also described. Twenty patients with atrial septal aneurysm and 19 control subjects performed 24-h Holter monitoring. Frequent (> 10/h) atrial premature beats were observed in seven patients vs. none of the controls (P = 0.008). The mean number of episodes of supraventricular tachycardia and the prevalence of ventricular tachycardia were also higher in the atrial septal aneurysm group (P = 0.044 and P = 0.046, respectively). Left atrial enlargement, mitral valve prolapse and left ventricular hypertrophy were more frequent than in the normal subjects. In conclusion, atrial and ventricular 'complex' arrhythmias occurred more frequently in patients with atrial septal aneurysm than in normal subjects. Further studies in patients with atrial septal aneurysm without other associated echocardiographic abnormalities need to be done to ascertain a potential arrhythmogenicity of this condition.
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Pulmonary arterial hypertension responsive to immunosuppressive therapy in systemic lupus erythematosus.
Two female patients with recent diagnosis of systemic lupus erythematosus (SLE) are reported. Pulmonary arterial hypertension was diagnosed by Doppler echocardiography. Immunosuppressive therapy was started at the time of diagnosis of SLE. After 2 months of therapy, Doppler echocardiography was repeated and the estimated pulmonary artery systolic pressure was substantially decreased from 78 to 42 mmHg and from 67 to 42 mmHg, respectively, along with significant improvement of the clinical conditions.
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Cardiac involvement in Behçet's disease.
To assess the prevalence and the extent of cardiac involvement in patients with Behçet's disease and to investigate the possible causes that may predispose to this involvement, 30 patients affected by Behçet's disease and 30 normal control subjects were submitted to M-mode, two-dimensional, and Doppler echocardiographic evaluation. Moreover, antinuclear and anticardiolipin autoantibodies were determined in the sera of both patients and control subjects. Finally, HLA-B51 positivity was assessed in the patients and in a historical control group. Mitral valve prolapse was observed in 50% and proximal aorta dilatation in 30% of the patients. There was a significant difference in the rate of these abnormalities in comparison with the control group. Left ventricular function parameters were similar between the two groups. The positivity rate of antinuclear and anticardiolipin autoantibodies was very low (7%), without differences between the groups. HLA-B51 was detected in 82.7% of the patients versus 21.7% in the control group (p < 0.00001). In conclusion, this study demonstrates a high rate of cardiac abnormalities in patients with Behçet's disease.
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Primary aspergillosis of the larynx in a patient with Felty's syndrome.
Herein we report the first case of primary aspergillosis of the larynx in a patient with Felty's syndrome. A 53-year-old man, a florist by profession, with a 12-year history of rheumatoid arthritis and on treatment with steroids, was admitted because of hoarseness, and intermittent fever of 2 weeks' duration. On admission, physical examination and laboratory data showed, among other findings, splenomegalia and neutropenia. At bone marrow examination, normal cellularity with mild dyserythropoiesis was observed. A fiberoptic laryngoscopy showed white plaques on both the true vocal cords. Both culture and microscopic examination of these lesions provided the diagnosis of invasive process by Aspergillus flavus. A computed tomography of the middle ears, paranasal sinuses, and chest was normal. Thus, primary aspergillosis of the larynx and Felty's syndrome was diagnosed, and the patient was successfully treated with granulocyte colony-stimulating factor and systemic antifungal agents. Felty's syndrome, corticosteroid use, and occupational risk probably rendered our patient susceptible to Aspergillus infection.
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