Left Ventricular Involvement Research Articles

Technetium-99m Sestamibi/Tetrofosmin Myocardial Perfusion Scanning in Cardiac and Noncardiac Sarcoidosis

Left ventricular (LV) and right ventricular (RV) involvement in sarcoidosis must be firmly confirmed to determine patients’ prognosis. We examined whether myocardial perfusion images using technetium-99m single photon emission computed tomography (SPECT) have a diagnostic benefit in the evaluation of biventricular involvement. Sixteen patients with sarcoidosis, aged 21–78 (54 ± 12) years old, 5 males and 11 females, complicated with cardiac disease (cardiac sarcoidosis, n = 6) including tachyarrhythmias of ventricular origin (n = 5), atrioventricular block (n = 4), and congestive heart failure (NYHA ≧ II, n = 1), were enrolled in this study. Myocardial SPECT using technetium-99m sestamibi or tetrofosmin was performed and semiquantitatively scored for comparison with 25 control subjects. Perfusion abnormalities were more frequently recognized in sarcoidosis (LV 5/16, 31% and RV 14/16, 88% vs. LV 0/25, 0% and RV 8/25, 32% in controls). LV involvement had a close correlation with atrioventricular block and with congestive heart failure, and multiple sites of RV involvement correlated with ventricular tachyarrhythmia of RV origin. Total number of defect segments were highest in cardiac sarcoidosis (18/30, 60% vs. 19/60, 32% in noncardiac sarcoidosis, and 11/150, 7% in controls, p = 0.0001), and semiquantitatively evaluated total LV and RV scores (ranging from 0 to 18) were higher than those of controls (15.1 ± 1.8 vs. 11.4 ± 3.0 in noncardiac sarcoidosis, and 9.0 ± 5.0 in cardiac sarcoidosis) and exhibited a significant positive linear correlation with the RV ejection fraction (y = 19.8 + 1.83x, r = 0.786, p = 0.001). Biventricular SPECT using technetium-99m is clinically useful for the noninvasive evaluation of both ventricular involvements in sarcoidosis.

Arrhythmogenic Right Ventricular Cardiomyopathy as a Cause of Sudden Unexplained Death

Objective:Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive cardiac muscle disease characterized as a progressive fibrofatty replacement of the right ventricle, severe ventricular arrhythmia, and sudden death. However, there is has been no report reports of ARVC as a cause of sudden death in Korea. Methods and Results:Postmortem studies were done to on 115 cases of sudden unexplained death at the department Department of legal Legal medicineMedicine, school School of medicineMedicine, Kyungpook national National university University in year 1999. We identified 7 cases (6%) of typical ARVC with no other identifiable cause of sudden death. The subjects included 5 males and 2 females, ranging in age from 19 to 41 years (mean 29.7 years). All were found dead at in bed (5 cases) or in the workshop (2 cases). Five cases were fibrofatty types and two cases were fatty types. Right ventricular aneurysm, inflammatory infiltrates and left ventricular involvement were found in 4, 2 and 1 cases, repectivelyrespectively. Two cases had a family history of sudden death before age 40. No one was None were suspected of having cardiovascular disease or ARVC before death. Conclusion:These findings indicate that ARVC in the Taegu-Kyungpook area may be more frequent than previously thought. ARVC may be a major cause of sudden unexplained death. (Korean Circulation J 2001;31(3):335-340)

Risk stratification of sudden cardiac death and malignant ventricular arrhythmias in right ventricular dysplasia-cardiomyopathy

Arrhythmogenic right ventricular dysplasia-cardiomyopathy is in most cases a benign cause of ventricular arrhythmias in young patients. The major reason of mortality is sudden arrhythmic death with an annual rate of 2–3% as the first manifestation of the disease in most cases. Little is known about risk factors of sudden arrhythmic death so far. The purpose of the retrospective study was to classify risk factors from invasive and non-invasive examinations. Methods: In a cohort of 121 consecutive patients sampled from 1986 to 1998 the value of right ventricular dilatation, left ventricular involvement analysed by angiocardiography or echocardiography and standard ECG parameters such as precordial T wave inversions, right precordial ST elevation, precordial QRS dispersion, left precordial JT interval prolongation and complete right bundle branch block were determined. The whole cohort of patients were divided into two groups with high arrhythmic risk (aborted or non-aborted sudden death, recurrent ventricular tachycardia despite medical treatment, recurrent syncopes) and low risk (frequent ventricular premature beats, non sustained ventricular tachycardia, uneventful course under medical therapy). Results: From angiocardiography or echocardiography in a quantitative approach right ventricular dilatation ( p<0.0001) and additional left ventricular abnormalities ( p<0.0001) could be identified as major risk factors. From an ECG point of view increased precordial QRS dispersion ≥50 ms ( p<0.01) with complete right bundle branch block and right ventricular dilatation in most cases and precordial T wave inversions beyond V3 ( p<0.0001) and the phenomenon of left precordial JT interval prolongation (JT dispersion ≥30 ms) in cases of additional left ventricular abnormalities represented non-invasive predictors of recurrent arrhythmic events. Right precordial ST segment elevation could be excluded as risk factor of sudden arrhythmic death. Conclusions: Right ventricular dilatation with ECG depolarisation abnormalities and additional left ventricular involvement with striking ECG repolarisation abnormalities could be identified as strong risk factors of recurrent arrhythmic events in ARVD with unfavorable prognosis.

Arrhythmogenic right ventricular dysplasia masquerading as dilated cardiomyopathy

This is a case report of 2 patients with severe left ventricular dysfunction and ventricular arrhythmias. At the time of cardiac transplantation they were found to have right ventricular dysplasia with left ventricular involvement.

Atrial natriuretic peptide is not degraded by the lungs in humans.

In an attempt to identify and quantify the sites of atrial natriuretic peptide (ANP) degradation, particularly the lungs, a new tracer method to study ANP metabolism in vivo in humans was developed and applied to patients with left ventricular dysfunction. Thirteen male, normotensive, cardiac patients with different degrees of left ventricular myocardial involvement were enrolled in the study. The study protocol required constant infusion (3 patients) or bolus injection (10 patients) of 125I-labeled ANP just upstream of the right atrium and blood sampling from different sites (pulmonary artery, aorta, inferior vena cava, and femoral vein) during the hemodynamic study. Data analysis was based on a kinetic model consisting of three blocks in series (right heart, lungs and left heart, and periphery) supplied by the same plasma flow (plasma cardiac output). Plasma levels of native ANP were measured with a sensitive and specific immunoradiometric assay method. ANP values measured in the aorta (163.9 +/- 144.8 pg/mL, n = 80) were superimposable on those measured in the pulmonary artery (161.8 +/- 136.5 pg/mL, n = 80). Negligible extraction of 125I-labeled ANP was found in the lungs and left heart block (on average 0.08 +/- 3.92%), whereas the peripheral block extraction (46.2 +/- 7.8%) accounted for almost total hormone removal from the blood (whole body extraction was 46.4 +/- 6.6%). ANP metabolic clearance rate (3.11 +/- 1.48, range 1.4-6.8 L/min) declined with the progression of left ventricular dysfunction (plasma cardiac output 3.46 +/- 1.08, range 1.2-5.7 L/min), and a close correlation between metabolic clearance rate and cardiac output was evident. Our data suggest that lungs do not extract, or extract only very small amounts, of labeled ANP administered iv to patients with different degrees of left ventricular myocardial involvement, and whole body extraction of labeled ANP remains relatively stable with the progression of disease, and the large reductions in clearance values observed in our patients can be ascribed mainly to the reductions in cardiac output.

Cardiac Rhabdomyomas and Obstructive Left Heart Disease: Histologically but Not Functionally Benign

Background. Cardiac rhabdomyoma is the most common primary heart tumor in infants. Spontaneous regression of such tumors is common, particularly with smaller lesions, followed by resolution of symptoms. Based on our data on spontaneous involution, our institutional philosophy has been one of expectant management in the absence of life-threatening symptoms. However, surgical intervention sometimes is required for the extirpation of a rhabdomyoma from the left ventricular outflow tract. Methods. A retrospective review was conducted of 30 children in whom a rhabdomyoma was diagnosed over a 27-year period. Results. Twenty-three percent (7/30) of the children required surgical extirpation of the tumor from within their left ventricular outflow tract, although a total of 94% had left ventricular involvement. There were no deaths. To date, no child has required reexcision of tumor. Conclusions. The natural history of rhabdomyoma is one of spontaneous regression (the 23 children who did not undergo surgical intervention are alive and continue to be followed up medically). We recommend surgical excision to alleviate acute outflow tract obstruction with reliance on the tumor’s natural history of regression to achieve long-term freedom from reoperation. Although operation has been recognized as lifesaving, we were somewhat surprised to find that greater than 20% of our pediatric population required operative intervention.

Comparison of Clinical and Morphologic Cardiac Findings in Patients Having Cardiac Transplantation for Ischemic Cardiomyopathy, Idiopathic Dilated Cardiomyopathy, and Dilated Hypertrophic Cardiomyopathy

This article compares intergroup and intragroup clinical and morphologic findings in patients with ischemic cardiomyopathy (IC), idiopathic dilated cardiomyopathy (IDC), and dilated hypertrophic cardiomyopathy (HC) undergoing cardiac transplantation (CT). Few previous publications have described findings in native hearts explanted at the time of CT. The explanted heart in 92 patients having CT was examined in uniform manner with particular attention to the sizes of the ventricular cavities and the presence of and extent of ventricular scarring. Of the 92 hearts examined, 47 had IC, 35 had IDC, and 10 had dilated HC. Although considerable degrees of intragroup variation occurred, the mean degree of left ventricular dilatation was similar among the patients with IC, IDC, and dilated HC. All patients with IC had left ventricular free wall scarring more extensive than that involving the ventricular septum, but the intragroup variation in the amounts of scarring was considerable. Nine of the 10 patients with dilated HC also had ventricular wall scarring, but it was more extensive in the ventricular septum than in the left ventricular free wall and involvement of the right ventricular wall also was present. Eight (23%) of the 35 IDC patients also had grossly visible ventricular scars but they were small and only 1 of the 8 had coronary narrowing and that was not in the distribution of the scarring. Narrowing of 1 or more epicardial coronary arteries >75% in cross-sectional area by plaque was present in all 47 IC patients, in 8 of the 35 IDC patients (7 had no ventricular scars), and in none of the 10 dilated HC patients. Coronary angiography was the major clinical tool allowing separation of the IC, IDC, and HC patients. Coronary angiography did not detect narrowing in any of the 8 patients with IDC who were found to have coronary narrowing on anatomic study. Thus, among patients with IC, IDC, and dilated HC having CT, distinctive anatomic features allow separation of patients with IC, IDC, and dilated HC, but within each group considerable variation in left ventricular cavity size and extent of ventricular scarring occurs.

Clinical characteristics of patients with arrhythmogenic right vontricular cardiomyopathy and left ventricular involvement

Clinical characteristics of patients with arrhythmogenic right vontricular cardiomyopathy and left ventricular involvement

Spectrum of Clinicopathologic Manifestations of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Multicenter Study

Objectives. The aim of the present investigation was to redefine the clinicopathologic profile of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC), with special reference to disease progression and left ventricular (LV) involvement.Background. Long-term follow-up data from clinical studies indicate that ARVC is a progressive heart muscle disease that with time may lead to more diffuse right ventricular (RV) involvement and LV abnormalities and culminate in heart failure.Methods. Forty-two patients (27 male, 15 female; 9 to 65 years old, mean [±SD] age 29.6 ± 18) from six collaborative medical centers, with a pathologic diagnosis of ARVC at autopsy or heart transplantation, and with the whole heart available, were studied according to a specific clinicomorphologic protocol.Results. Thirty-four patients died suddenly (16 during effort); 4 underwent heart transplantation; 2 died as a result of advanced heart failure; and 2 died of other causes. Sudden death was the first sign of disease in 12 patients; the other 30 had palpitations, with syncope in 11, heart failure in 8 and stroke in 3. Twenty-seven patients experienced ventricular arrhythmias (ventricular tachycardia in 17), and 5 received a pacemaker. Ten patients had isolated RV involvement (group A); the remaining 32 (76%) also had fibrofatty LV involvement that was observed histologically only in 15 (group B) and histologically and macroscopically in 17 (group C). Patients in group C were significantly older than those in groups A and B (39 ± 15 years vs. 20 ± 8.8 and 25 ± 9.7 years, respectively), had significantly longer clinical follow-up (9.3 ± 7.3 years vs. 1.2 ± 2.1 and 3.4 ± 2.2 years, respectively) and developed heart failure significantly more often (47% vs. 0 and 0, respectively). Patients in groups B and C had warning symptoms (80% and 87%, respectively, vs. 30%) and clinical ventricular arrhythmias (73% and 82%, respectively, vs. 20%) significantly more often than patients in group A. Hearts from patients in group C weighed significantly more than those from patients in groups A and B (500 ± 150 g vs. 328 ± 40 and 380 ± 95 g, respectively), whereas hearts from both group B and C patients had severe RV thinning (87% and 71%, respectively, vs. 20%) and inflammatory infiltrates (73% and 88%, respectively, vs. 30%) significantly more often than those from group A patients.Conclusions. LV involvement was found in 76% of hearts with ARVC, was age dependent and was associated with clinical arrhythmic events, more severe cardiomegaly, inflammatory infiltrates and heart failure. ARVC can no longer be regarded as an isolated disease of the right ventricle.

Prognostic Significance of Precordial ST-Segment Changes in Acute Inferior Wall Myocardial Infarction

To examine the clinical significance of precordial ST-segment changes in patients with acute Q-wave inferior wall myocardial infarction. Prospective evaluation (clinical follow-up) of Q-wave inferior wall myocardial infarction over a 6-year period in patients who fulfilled the inclusion criteria. Coronary care unit at a university hospital. Two hundred consecutive patients with acute Q-wave inferior wall myocardial infarction admitted to the coronary care unit within 24 h from the onset of chest pain. Precordial ST-segment depression resolved <24 h (transient) after admission in 84 patients, lasted > or = 24 h (persistent) in 48 patients, and was absent in 68 patients, while ST-segment elevation in V4R was detected in 60 patients. Seventy-one patients had major in-hospital complications and 18 patients died in the hospital. When nine variables were used in the multivariate analysis, right ventricular dilatation and persistent precordial ST-segment depression were the important factors related to major in-hospital complications, whereas age, alveolar arterial oxygen difference, and persistent precordial ST-segment depression were important for in-hospital deaths. Left ventricular posterior wall involvement, diagnosed by persistent precordial ST-depression, was an independent and stronger predictor of major in-hospital complications and deaths than right ventricular involvement in patients with acute Q-wave inferior wall myocardial infarction.

Age related dilatation of the right ventricle in arrhythmogenic right ventricular dysplasia-cardiomyopathy

‘Progression’ in typical arrhythmogenic right ventricular dysplasia-cardiomyopathy could be mainly demonstrated in case reports and generally implies decreasing left ventricular function; systematic follow-up data are only available on clinical features such as recurrent arrhythmias, late potentials or left ventricular ejection fraction. Due to methodological problems, continuous data on structural and functional right ventricular performance are not available. In order to get some more information about basic mechanisms of possible progression in arrhythmogenic right ventricular dysplasia-cardiomyopathy, the age-dependancy of right ventricular enddiastolic volume, right ventricular ejection fraction, number of bulges, left ventricular involvement and the form of tachycardias was analysed at a single examination after having made the diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy. In the whole cohort of patients ( n = 66), age-dependancy of right ventricular volume could be demonstrated using linear regression analysis, chi-square-test and paired t-test ( P < 0.01); global right ventricular ejection fraction and the number of right ventricular bulges did not differ significantly in younger and older patients. The same was true for mild left ventricular impairment and sustained or non-sustained ventricular tachycardias, respectively. In a follow-up, two older patients developped dramatic left ventricular dilatation and reduction of left ventricular function requiring heart transplantation. A third older patient died from isolated right heart failure. Increasing age-related right ventricular dilatation is possibly a marker of disease progression as a basis for right heart failure. Dramatic left heart involvement seems to be an event with abrupt onset in most cases due to acute or chronic myocarditis.

Usefulness of electron-beam computed tomography in arrhythmogenic right ventricular dysplasia. Relationship to electrophysiological abnormalities and left ventricular involvement.

Electron-beam computed tomography (CT) may be useful for detecting myocardial fat infiltration and diagnosing arrhythmogenic right ventricular dysplasia (ARVD). There are several characteristic electron-beam CT findings of ARVD. However, the incidence, their relation to electrophysiological abnormalities, and the usefulness of electron-beam CT for evaluating left ventricular involvement are unknown. This study aimed to clarify these issues. Electron-beam CT was performed in 14 patients with ARVD (ARVD group), 16 age- and sex-matched patients with right ventricular enlargement and/or dysfunction without ARVD (RV enlargement group), and 13 control subjects (control group). The incidences of abnormal electron-beam CT findings in the three groups were examined. Furthermore, we examined the endocardial fat-infiltrated areas detected by electron-beam CT (CT-A) and electrophysiologically abnormal areas detected in the mapping electrophysiology study (EPS-A) and compared the relationship between them in the ARVD group. (1) The frequencies of abundant epicardial adipose tissue, low-attenuation trabeculations, scalloping of the right ventricular free wall, and intramyocardial fat deposits were 86%, 71%, 79%, and 50%, respectively, in the ARVD group, whereas these findings were not observed in the RV enlargement and control groups. (2) Three ARVD patients (21%) had adipose tissue involvement of the left ventricle. (3) The relationship between CT-A and EPS-A was as follows: CT-A > EPS-A, 71%; CT-A = EPS-A, 14%; and EPS-A only, 14%. Characteristic electron-beam CT findings are frequently observed only in patients with ARVD. Electron-beam CT is useful for evaluating for left ventricular involvement and can estimate EPS-A.

Endomyocardial fibrosis: electron-beam CT features.

To present morphologic and functional electron-beam computed tomographic (CT) features of endomyocardial fibrosis. Twelve patients with histologic evidence of endomyocardial fibrosis and/or definitive echocardiographic and angiocardiographic findings were examined with electron-beam CT. Endomyocardial fibrosis was depicted as linear calcifications and/or a thin tissue band of low attenuation within the endomyocardium. In right ventricular involvement, CT depicted obliteration of the apex associated with extension along the free wall and/or papillary muscles of the tricuspid valve. In left ventricular involvement, a large amount of endocardial tissue led to severe obliteration within the inflow tract and/or of the apex. At ventricular volume analysis, restrictive patterns of the disease were determined. Electron-beam CT allows direct visualization of endomyocardial fibrosis and the resulting volumetric changes and enables distinction of the disease from constrictive pericarditis.

Age related dilatation of the right ventricle in arrhythmogenic right ventricular dysplasia-cardiomyopathy

<h2>Abstract</h2> ‘Progression' in typical arrhythmogenic right ventricular dysplasia-cardiomyopathy could be mainly demonstrated in case reports and generally implies decreasing left ventricular function; systematic follow-up data are only available on clinical features such as recurrent arrhythmias, late potentials or left ventricular ejection fraction. Due to methodological problems, continuous data on structural and functional right ventricular performance are not available. In order to get some more information about basic mechanisms of possible progression in arrhythmogenic right ventricular dysplasia-cardiomyopathy, the age-dependancy of right ventricular enddiastolic volume, right ventricular ejection fraction, number of bulges, left ventricular involvement and the form of tachycardias was analysed at a single examination after having made the diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy. In the whole cohort of patients (<i>n</i> = 66), age-dependancy of right ventricular volume could be demonstrated using linear regression analysis, chi-square-test and paired <i>t</i>-test (<i>P</i> < 0.01); global right ventricular ejection fraction and the number of right ventricular bulges did not differ significantly in younger and older patients. The same was true for mild left ventricular impairment and sustained or non-sustained ventricular tachycardias, respectively. In a follow-up, two older patients developped dramatic left ventricular dilatation and reduction of left ventricular function requiring heart transplantation. A third older patient died from isolated right heart failure. Increasing age-related right ventricular dilatation is possibly a marker of disease progression as a basis for right heart failure. Dramatic left heart involvement seems to be an event with abrupt onset in most cases due to acute or chronic myocarditis.

Value of the Echocardiographic Findings of Arrhythmogenic Right Ventricular Dysplasia with Left Ventricular Involvement in a Child

Value of the Echocardiographic Findings of Arrhythmogenic Right Ventricular Dysplasia with Left Ventricular Involvement in a Child

Right ventricular dysplasia as a generalized cardiomyopathy? findings on magnetic resonance imaging.

The aim of our study was to define cardiac morphological and functional abnormalities of right ventricular dysplasia by magnetic resonance imaging. Twenty-two healthy volunteers (age, 37.7 +/- 14.2 years) free of cardiac or respiratory diseases (group I) and 12 patients (age, 41.9 +/- 15.8 years) with clinical, electrophysiological and cineangiographic diagnosis of right ventricular dysplasia (group II) underwent magnetic resonance imaging at 0.2 Tesla. End-diastolic diameter, trabecular disarray and segmental wall motion abnormalities were evaluated for the right ventricle as were adipose replacement and fractional shortening for both ventricles. The right ventricular end-diastolic diameter was significantly enlarged in group II (P = 0.0023). Right ventricular trabecular disarray was mild in two group I subjects, and moderate in seven and massive in five group II patients. Right ventricular systolic bulges were found in seven group II patients, aneurysms in five. Excellent agreement was found between magnetic resonance imaging and cineangiography for bulges, aneurysms and tricuspid regurgitation (P < 0.0001). On spin-echo images, signal hyperintensities, due to adipose replacement, were found in 44 cardiac regions in group II: right ventricular outflow tract (12), sub-tricuspid posterobasal region (8), right ventricular apex (9), right ventricular anterior wall (6), interventricular septum (4), left ventricular lateral wall (4), left ventricular apex (1). Significant signal-to-noise ratio differences were found between group II abnormal areas and group I myocardial tissue for the right (P < 0.0001) and left ventricles (P = 0.0006). Fractional shortening in the right and left ventricles were significantly reduced in group II (P = 0.0002 and P = 0.00016, respectively). Magnetic resonance imaging can be considered a very useful diagnostic tool for the detection of features typical of right ventricular dysplesia, such as adipose replacement, trabecular disarray, bulges and aneurysms and provides useful information about cardiac function and regional wall motion. It indicates that left ventricular involvement occurs in a significant fraction of patients, and suggests that right ventricular dysplasia may be a generalized cardiomyopathy.

Right Ventricular Dysplasia: The Mayo Clinic Experience

To determine the initial clinical manifestations and echocardiographic features of right ventricular dysplasia as encountered in a major cardiovascular referral center in the United States. We conducted a retrospective study of cases of right ventricular dysplasia diagnosed at the Mayo Clinic between January 1978 and January 1993. In an institutional data-base search, we identified 20 patients with right ventricular dysplasia. Echocardiographic, electrophysiologic, Holter monitoring, cardiac catheterization, and endomyocardial biopsy results were analyzed. The mean duration of follow-up was 7 years. In the 12 female and 8 male patients (mean age, 30 years; range, 3 to 60), the initial manifestations of right ventricular dysplasia included ventricular arrhythmia (45%), congestive heart failure (25%), heart murmur (10%), asymptomatic (10%), complete heart block (5%), and sudden death (5%). First-order relatives were affected in 30% of the patients. Ventricular tachycardia with morphologic features of left bundle branch block was inducible in seven of nine patients. On Holter monitoring, all but 2 of 15 patients studied had frequent ventricular ectopic activity (Lown grade 2 or more). Characteristic fatty infiltration of the myocardium was present in 7 of 13 right ventricular biopsy specimens. Inordinate right ventricular enlargement was present in 60% of the patients at first echocardiographic assessment and in two other patients on follow-up assessment. Variable left ventricular involvement was noted in 50% of the cases. During the follow-up period, four patients died: two died suddenly, one died of congestive heart failure, and one died of respiratory failure after a coronary artery bypass operation. Of the 16 living patients, 8 are doing well, 3 have an implanted cardiac defibrillator, 3 are receiving antiarrhythmic agents, and 2 have undergone cardiac transplantation because of progressive biventricular failure. Patients with right ventricular dysplasia have varied initial manifestations and a high frequency of serious cardiovascular symptoms and complications.

Long-term evolution of right ventricular dysplasia-cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart-muscle disease primarily affecting the right ventricle (RV) and potentially causing sudden death in young people. The natural history of the disease is firstly characterized by a concealed form progressing over a biventricular involvement. Three different cases coming from the same family are presented together with a review of the literature.Multi-parameter analysis including imaging and electrocardiographic analysis is presented since the first medical referral with follow-up ranging from 11 to 38 years. Case 1 presented a typical RV involvement in agreement with the ECG pattern. Case 2 presented a prevalent left ventricular involvement leading from the beginning to a pattern of dilated cardiomyopathy in agreement with his ECG evolution over the years. On the other side, Case 3 came to observation with a typical RV involvement (similar to Case 1) but with ECG evolution of typical left ventricle involvement (similar to Case 2). The genetic analysis showed a mutation in desmoglein-2 (DSG2) gene: p. Arg49His. Comparison between size and localization of ventricular dyskinesia at cardiovascular imaging and the surface 12 lead electrocardiography are proposed.ARVC may lead to an extreme phenotypic variability in clinical manifestations even within patients coming from the same family in which ARVC is caused by the same genetic mutation. ECG progression over time reflects disease evolution and in particular cases may anticipate wall motion abnormalities by years.

913-113 Detection of Left Ventricular Involvement with 1-123 MIBG SPECT in Patients with Arrhythmogenic Right Ventricular Dysplasia: Comparison with Electron-beam CT, MRI and Left Ventriculography

Histological, functional abnormalities in the left ventricular (LV) myocardium have been observed in some patients (pts) with arrhythmogenic right ventricular dysplasia (ARVD). It is recently reported that 1 – 123 MIBG (MIBG) SPECT allowed to detect early stage in ARVD. But comparing MIBG SPECT with other noninvasive examination has not been studied. We performed MIBG (111 MBq) SPECT, electron-beam CT (EBCT), MRI and left ventriculography (LVG) in 16 ARVD pts to evaluate the best method forthe early diagnosis of LV involvement. ARVD was diagnosed on the basis of RV wall motion abnormality, left bandle branch block type ventricular tachycardia, and myocardial fatty infiltration and/or fibrosis in RV endomyocardial biopsy. We divided the LV myocardium into 5 segments (apical, anterior, septal, posterior and lateral), and we assessed the findings in each segment. Abnormalities of regional tracer uptake in MIBG delayed (4 hr) SPECT images were evaluated by using the extent bull's-eye map which exhibits reduced %uptake under mean-2SD of 10 normal controls. Abnormal segments in EBCT, MRI and regional wall motion abnormalities (rWM) were highly matched with MIBG defects (93% in EBCT, 100% in MRI and 83% in decreased rWM). Abnormal segments in MIBG, EBCT and MRI were poorly matched with decreased rWM (45% in MIBG, 67% in EBCT and MRI). Fifteen segments which showed MIBG defects revealed no abnormalities in EBCT, MRI and rWM. Massive fatty infiltration detected by EBGT and MRI highly correlated with MIBG defects and decreased rWM. But the sympathetic abnormalities as shown by MIBG defects appeared to precede massive fatty infiltration and rWM abnormalities. a MIBG defects EBCT MRI decreased rWM Pt(n) 14 8 7 8 Abnormal segments 44 15 * 12 * 24 * Matched with MIBG defects - 14(93%) 12(100%) 20 (83%) Matched with decr. rWM 20 (45%) 10(67%) 8(67%) - * p &lt; 0.05 vS. MIBG defects Regional reduction of MIBG myocardial uptake indicating abnormal sympathetic innervation is a very sensitive marker of LV involvement, comparing EBCT, MRI and regional wall motion abnormalities, in ARVD.

Right ventricular dysplasia: Right and left ventricular involvement morphometrically evaluated

Right ventricular dysplasia (RVD) is a cardiac anomaly characterized by replacement of variable amounts of right ventricular myocardium by adipose tissue. This condition is believed to be a selective disorder involving extensively the right ventricle, but there are occasional reports of concomitant “minor” abnormalities of the left ventricle. The object of this report concerns a patient who died after heart transplantation because of an unsuspected RVD of the donor heart. We present a morphometric study of the heart in order to evaluate the distribution of the fat on both ventricles and to understand the structural basis of the heart failure. The results show that a large portion of the right ventricle is replaced by fat with a quite homogeneous distribution; the left ventricle is also largely replaced by adipose tissue that is primarily localized at the apex and decreases from the apex to the basis. The remodeling of the heart is attributable to a conspicuous increase in volume of the right ventricle associated with a normal number of myocytes that are longer than normal. For these reasons, according to Starling's law, the heart develops congestive failure.