Presentación clínica y características ecocardiográficas en pacientes con miocardio no compacto / Clinical Presentation and Echocardiographic Characteristics of Patients with Left Ventricular Noncompaction

El miocardio no compacto es una miocardiopatía genética primaria ocasionada por la detención de la embriogénesis normal del endocardio y el miocardio. Esta anomalía se asocia frecuentemente con arritmias, insuficiencia cardíaca y eventos embólicos. El presente estudio se llevó a cabo con el objetivo de describir la modalidad de presentación clínica y las características electrocardiográficas y ecocardiográficas en portadores de esta miocardiopatía. Se analizaron 22 pacientes con diagnóstico de miocardio no compacto detectados en el laboratorio de ecocardiografía entre julio de 2004 y abril de 2009. Toda la población en estudio fue analizada mediante electrocardiograma y eco-Doppler cardíaco transtorácico y en 12 casos se registró Holter de 24 horas. Se determinaron el peso, la altura y el índice de masa corporal, que se compararon contra 66 pacientes distribuidos en tres grupos de 22 pacientes cada uno: 1) grupo control de personas sin cardiopatías, 2) portadores de miocardiopatía hipertrófica y 3) pacientes con miocardiopatía dilatada idiopática. En la población estudiada con miocardio no compacto predominó el sexo femenino y se observó menor peso e índice de masa corporal en relación con los grupos comparados y el síntoma preponderante fue la disnea. El electrocardiograma mostró ritmo sinusal con trastornos de conducción y arritmia ventricular repetitiva. El eco transtorácico mostró diferentes grados de disfunción ventricular izquierda sistólica y diastólica con áreas de miocardio no compacto predominantes en los territorios medial y apical de los segmentos inferior, posterior y lateral, en algunos casos complicados con trombos intraventriculares y ocasionalmente asociados con fístulas coronarias.(AU)

Left ventricular noncompaction is a primary genetic cardiom-yopathy caused by arrest of normal embryogenesis of the endocardium and myocardium. This anomaly is frequently associated with arrhythmias, heart failure and thromboem-bolic events. The goal of the present study was to describe the clinical presentation and the electrocardiographic and echocardiographic characteristics of patients with this cardiomyopathy. Twenty-two patients with left ventricular noncompaction detected by echocardiography between July 2004 and April 2009 were analyzed. Electrocar di ogram and transthoracic Doppler echocardiography was performed to all the patients, and 12 patients underwent 24-hour Holter monitoring. Weight, height and body mass index were calculated and compared with 66 patients distributed in three groups of 22 patients each: 1) control group without heart disease, 2) hypertrophic cardiomyopathy; and 3) idiopathic dilated cardiomyopathy. In the population with left ventricular noncompaction, fe-male gender prevailed, lower weight and body mass index was observed and dyspnea was the most common symptom. The electrocardiogram showed sinus rhythm, conduction distur-bances and repetitive ventricular arrhythmia. Transthoracic echocardiography showed different degrees of systolic and diastolic left ventricular dysfunction with areas of noncom-paction in the mid and apical inferior, posterior and lateral segments; intraventricular thrombi were occasionally seen and sometimes associated with coronary artery fistulas.(AU)

Presentación clínica y características ecocardiográficas en pacientes con miocardio no compacto / Clinical Presentation and Echocardiographic Characteristics of Patients with Left Ventricular Noncompaction

El miocardio no compacto es una miocardiopatía genética primaria ocasionada por la detención de la embriogénesis normal del endocardio y el miocardio. Esta anomalía se asocia frecuentemente con arritmias, insuficiencia cardíaca y eventos embólicos. El presente estudio se llevó a cabo con el objetivo de describir la modalidad de presentación clínica y las características electrocardiográficas y ecocardiográficas en portadores de esta miocardiopatía. Se analizaron 22 pacientes con diagnóstico de miocardio no compacto detectados en el laboratorio de ecocardiografía entre julio de 2004 y abril de 2009. Toda la población en estudio fue analizada mediante electrocardiograma y eco-Doppler cardíaco transtorácico y en 12 casos se registró Holter de 24 horas. Se determinaron el peso, la altura y el índice de masa corporal, que se compararon contra 66 pacientes distribuidos en tres grupos de 22 pacientes cada uno: 1) grupo control de personas sin cardiopatías, 2) portadores de miocardiopatía hipertrófica y 3) pacientes con miocardiopatía dilatada idiopática. En la población estudiada con miocardio no compacto predominó el sexo femenino y se observó menor peso e índice de masa corporal en relación con los grupos comparados y el síntoma preponderante fue la disnea. El electrocardiograma mostró ritmo sinusal con trastornos de conducción y arritmia ventricular repetitiva. El eco transtorácico mostró diferentes grados de disfunción ventricular izquierda sistólica y diastólica con áreas de miocardio no compacto predominantes en los territorios medial y apical de los segmentos inferior, posterior y lateral, en algunos casos complicados con trombos intraventriculares y ocasionalmente asociados con fístulas coronarias.

Left ventricular noncompaction is a primary genetic cardiom-yopathy caused by arrest of normal embryogenesis of the endocardium and myocardium. This anomaly is frequently associated with arrhythmias, heart failure and thromboem-bolic events. The goal of the present study was to describe the clinical presentation and the electrocardiographic and echocardiographic characteristics of patients with this cardiomyopathy. Twenty-two patients with left ventricular noncompaction detected by echocardiography between July 2004 and April 2009 were analyzed. Electrocar di ogram and transthoracic Doppler echocardiography was performed to all the patients, and 12 patients underwent 24-hour Holter monitoring. Weight, height and body mass index were calculated and compared with 66 patients distributed in three groups of 22 patients each: 1) control group without heart disease, 2) hypertrophic cardiomyopathy; and 3) idiopathic dilated cardiomyopathy. In the population with left ventricular noncompaction, fe-male gender prevailed, lower weight and body mass index was observed and dyspnea was the most common symptom. The electrocardiogram showed sinus rhythm, conduction distur-bances and repetitive ventricular arrhythmia. Transthoracic echocardiography showed different degrees of systolic and diastolic left ventricular dysfunction with areas of noncom-paction in the mid and apical inferior, posterior and lateral segments; intraventricular thrombi were occasionally seen and sometimes associated with coronary artery fistulas.

Impaired myocardial function in hypertrophic cardiomyopathy.

BACKGROUND: Tissue Doppler imaging (TDI) parameters of peak myocardial velocities (S', E', and A') has been employed to assess the regional left ventricular myocardial function. The global function index (GFI) derived from TDI has been recently employed to distinguish the different etiologies of left ventricular hypertrophy. OBJECTIVE: To analyze whether the GFI or individual TDI parameters of peak myocardial velocities (S', E', and A') allows detecting different degrees of regional myocardial dysfunction in the most frequent forms of hypertrophic cardiomyopathy (HCM). METHODS: GFI = (E/E')/S' (where E is the peak transmitral flow velocity, E' is the early diastolic myocardial velocity, and S' is the peak systolic myocardial velocity) and TDI peak myocardial velocities was measured in the septal and lateral mitral annulus in 101 patients with HCM (mean age 47.5 +/- 14 years, 58 women) and in age-matched group of 30 healthy controls (mean age 46 +/- 6 years, 16 women). RESULTS: Forty-five patients had nonobstructive asymmetric septal HCM, 20 patients had a subaortic gradient >or= 30 mm Hg, 21 p. had apical HCM, and 15 p. had other forms of HCM (midventricular, symmetric, and biventricular). All patients with HCM exhibited a decrease in early diastolic (E') and systolic (S') myocardial velocities, both in the lateral and septal-mitral annulus border, but more pronounced in septal-mitral annulus. Septal GFI was higher in HCM patients than in healthy subjects (1.8 (1.1-2.5) and (0.57 (0.31-0.92), respectively, P < 0.001), but no differences were seen when different forms of HCM were compared. CONCLUSIONS: In a selected population of patients with HCM and a preserved left ventricular(LV) systolic function, GFI and individual TDI parameters of peak velocity (S', E', and A') and E/E' ratio were similar in different forms of HCM, indicating that in all patients with HCM there is regional systolic and diastolic myocardial dysfunction, regardless of the location of hypertrophy.

Right and left atrial appendage function in patients with mitral stenosis and sinus rhythm.

Tissue Doppler imaging (TDI) is generally used for the assessment of ventricular function, and to a lesser extent, to evaluate the left atrial appendage (LAA). In the present study, we used TDI to analyze the contractile function of the right atrial appendage (RAA). The aim of this study was a comprehensive evaluation of RAA and LAA contractile function in patients with mitral stenosis and sinus rhythm. A total of 69 patients were assessed: group 1 (23 patients with severe MS, 38 +/- 11 years, 20 women), group 2 (23 patients with mild MS, 39 +/- 12 years, 19 women) and group 3 (23 healthy subjects, 42 +/- 14 years, 16 women). Multiplane transesophageal echo was performed in all patients. The RAA was visualized at 130 degrees and the LAA at 0 degrees . TDI sample volume was placed in the tip of both atrial appendages, with an ultrasound beam angle < 10 degrees . Flow velocities and myocardial velocities were measured. The presence of thrombus and/or spontaneous echo contrast (SEC) was assessed. TDI showed in normal subjects, myocardial contraction velocities in RAA similar to that of the LAA (21.8 +/- 4.2 vs. 20.1 +/- 4 cm/s, respectively, P = NS). In patients with MS, myocardial velocities in both atrial appendages were significantly lower than in normal subjects (RAA: 17.4 +/- 5.1 vs. 21.8 +/- 4.2 cm/s, respectively, P < 0.01, LAA: 9 +/- 5.1 vs. 20.1 +/- 4 cm/s, respectively, P < 0.001). Linear regression analysis showed a correlation between the impairment of systolic function of both appendages, pulmonary arterial pressure, valve area and transmitral gradient. Of the 46 patients with MS, patients with intense SEC had lower flow and myocardial velocities in the LAA than patients without SEC (16 +/- 5 vs. 50 +/- 3 cm/s, 6 +/- 2 vs. 10.6 +/- 5.6 cm/s, respectively, P < 0.001 and P < 0.001). In healthy subjects, myocardial contraction velocities in both appendages were similar. Patients with MS and sinus rhythm had contractile dysfunction of both appendages, shown by the decrease in myocardial velocities and related to the increase in atrial afterload. Both appendages exhibited a relation between contractile dysfunction and the presence of SEC, but dysfunction was less marked in the RAA, which might explain the lower prevalence of thrombi in the RAA.

Myocardial perfusion SPECT in the diagnosis of apical hypertrophic cardiomyopathy.

BACKGROUND: Apical hypertrophy cardiomyopathy (ACM) is a rare condition characterized by asymmetric myocardial hypertrophy of the apex of the left ventricle. When two-dimensional echocardiography is limited by a poor acoustic window, patients are often referred for MRI. Our hypothesis is that a cheaper and more widely available diagnostic modality like myocardial perfusion single photon emission computed tomography (SPECT) may be helpful in the diagnosis of ACM. OBJECTIVE: The purpose of this prospective study was to define the characteristics of rest and stress SPECT studies in patients with known ACM, and whether SPECT may be helpful in the diagnosis of ACM. METHODS: Adult patients with ACM were enrolled in the study. Diagnosis was made with 2-D echo. A rest and exercise or dipyridamole stress SPECT study was performed in all patients with Tc-99m sestamibi. RESULTS: We enrolled 20 patients (mean age 60 +/- 16 years), 9 were female, with ACM. SPECT at rest revealed in 15 patients (75%) an increased apical tracer uptake, a spade-like deformity of the left ventricular chamber, and the "Solar Polar" map pattern consistent with ACM. The sensitivity, specificity, positive predictive value, and negative predictive value of SPECT for detecting ACM were 75%, 100%, 100%, and 80%, respectively. CONCLUSION: Three-fourths of adult patients with ACM showed, on myocardial perfusion SPECT, characteristic findings which were not seen in age-matched control subjects, such as a significant increased apical tracer uptake, a spade-like deformity of the left ventricle, and the "Solar Polar" map. Nuclear physicians should be aware of these SPECT findings because many ACM patients may first end up in the nuclear labs due to their markedly abnormal ECG for exclusion of obstructive coronary artery disease.

Left and right atrial function after percutaneous mitral valvuloplasty in mitral stenosis and sinus rhythm.

BACKGROUND AND AIM OF THE STUDY: Percutaneous mitral valvuloplasty (PMV) is an effective method for treating patients with severe mitral stenosis. The study aim was to compare left and right atrial appendage functions before and after PMV. METHODS: Twenty-five patients (23 women, two men; mean age 38 +/- 9 years; range: 21-57 years) with severe mitral stenosis and sinus rhythm who underwent PMV were included in the study. Transesophageal echocardiography (TEE) was performed before and at six months after PMV, to evaluate the intensity of spontaneous echo contrast (SEC), left atrial appendage (LAA) dimensions and function, and right atrial appendage (RAA) function. RESULTS: After PMV, the SEC (at TEE) was decreased in all patients, while increases were observed in the contraction and relaxation velocity flows of the LAA (pre-PMV 18 +/- 5 cm/s; post-PMV 24 +/- 9 cm/s; p < 0.001 and pre-PMV 25.5 +/- 10.2 cm/s; post-PMV 32.9 +/- 12.6 cm/s; p < 0.006, respectively). The tissue myocardial velocities were also increased in the LAA (pre-PMV 6.92 +/- 3.77 cm/s; post-PMV 11.16 +/- 6.61 cm/s; p < 0.002) and RAA (pre-PMV 16.2 +/- 3.7 cm/s; post-PMV 19.1 +/- 4.1 cm/s; p < 0.001). CONCLUSION: In patients with mitral stenosis and sinus rhythm, improvements were noted in the left atrial, LAA and RAA systolic functions after PMV. This suggests that the relief of mitral stenosis may not only confer hemodynamic benefits to improve symptoms but also have a favorable influence on future thromboembolic complications. Thus, an early intervention might benefit patients with sinus rhythm by preventing the development of atrial fibrillation and systemic and pulmonary embolism.

Absence of myocardial dysfunction during cold pressor stress echocardiography in patients with endothelial dysfunction.

BACKGROUND: Several methods are available for the assessment of coronary endothelial function, but there are no reports to date regarding the usefulness of cold pressor stress echocardiography (CPSE). OBJECTIVE: To assess regional systolic and diastolic left ventricular function using CPSE in patients with endothelial dysfunction. METHODS: We studied 24 patients, of whom 10 were men, aged 27 to 68 years, who had coronary risk factors and a normal exercise MP-SPECT test. They were compared with 10 normal subjects (6 men), aged 21 to 44 years. All patients underwent a CPSE. RESULTS: The cold pressor-MP-SPECT revealed myocardial ischemia in 10 patients (Group I) and was normal in 14 patients (Group II). All normal subjects (Group III) had normal cold pressor-MP-SPECT. The cold pressor test caused a significant increase in systolic BP in the three groups (baseline 117 +/- 17 mmHg vs. postcold test 137 +/- 16 mmHg, P < 0.05), without changes in heart rate, PR interval, or the corrected QT interval. During the CPSE, no patient developed WMA in 2D echo or changes in regional systolic or diastolic LV function in the pulsed Doppler tissue imaging. CONCLUSIONS: In patients with endothelial dysfunction and no known coronary artery disease, the ischemic response to the cold pressor-MP-SPECT is not accompanied by WMA or changes in regional systolic or diastolic LV function during CPSE. Such negative findings indicate that the amount of ischemia that occurs secondarily to endothelial dysfunction does not involve sufficient myocardial mass to cause contractile dysfunction.

Valve replacement with bileaflet HP-Biplus mechanical prostheses versus standard prostheses: long-term results.

BACKGROUND AND AIM OF THE STUDY: At present, no reports are available regarding the hemodynamic assessment and survival of patients undergoing valve replacement with HP-Biplus prostheses. Hence, the present study was designed to acquire this information. The aim was to assess: (i) any potential hemodynamic differences between the HP-Biplus prosthesis and the 'standard' bileaflet mechanical valves (SJM, ATS, Sorin Bicarbon and CarboMedics); and (ii) the incidence of postoperative complications and long-term mortality. METHODS: The data from 242 patients (139 males, 103 females; mean age 58.4 +/- 14.0 years) who survived mitral or aortic valve surgery with mechanical bileaflet standard prostheses (SJM, ATS, Sorin Bicarbon or CarboMedics), between January 1985 and December 2005, were analyzed retrospectively. Evaluations were conducted consecutively with Doppler echocardiography, and compared with 35 patients (20 males, 15 females; mean age 52.2 +/- 12.8 years) who received an HP-Biplus prosthesis and underwent surgery between January 2000 and December 2005. RESULTS: At seven years after surgery, actuarial survival was 40% for the HP-Biplus prosthesis and 84% for the 'standard' prostheses (p < 0.0001). The HP-Biplus prostheses had a higher rate of reoperations for aortic valves (15.2% versus 1.7%; p < 0.003, OR 10.2), a higher rate of prosthesis dysfunction (62.9% versus 7.8%; p < 0.00001, OR 25), and a higher rate of total events (72% versus 21.8%; p < 0.0001, OR 11). CONCLUSION: The present study was the first to compare long-term results of the HP-Biplus prostheses with those obtained with 'standard' mechanical prostheses. Valve replacement with standard prostheses was shown to carry low morbidity and mortality (21.8%), whereas the HP-Biplus prosthesis showed high morbidity and mortality (92%). The data acquired may be very important when selecting prostheses with better hemodynamic characteristics, and show that the HP-Biplus prosthesis, in the authors' opinion, is not suitable for clinical use.

Early detection of left ventricular diastolic dysfunction in Chagas' disease.

BACKGROUND: Chagas' disease may cause left ventricular diastolic dysfunction and its early detection in asymptomatic patients would allow to stratify the risk and to optimize medical treatment. The aim of this study is to investigate if transmitral Doppler flow can detect early abnormalities of the diastolic left ventricular function in patients during the indeterminate phase of Chagas' disease, in which the electrocardiogram (ECG), chest x-ray and 2-D echocardiogram (2D-echo) are normal. METHODS: a group of 54 patients with Chagas' disease was studied and compared to a control group of 27 subjects of similar age. All were assessed with an ECG, chest X-ray, 2-D echo, and transmitral Doppler flow. RESULTS: both groups had similar values in the 2D-echo. In patients with Chagas' disease, the transmitral Doppler showed a higher peak A velocity (control group: 0.44 m/sec, Chagas group: 0.55 m/sec, p = 0.001), a lower E/A ratio (control group: 1.45, Chagas group: 1.22, p < 0.05), and a lengthening of the deceleration time of early diastolic filling (control: 138.7 +/- 26.8 msec, Chagas group: 167.9 +/- 34.6 msec, p = 001), thus revealing an early disorder of the diastolic left ventricular function in patients with Chagas' disease. CONCLUSION: in patients with Chagas' disease who are in the indeterminate phase, transmitral Doppler flow allowed to identify early abnormalities of the left ventricular diastolic function, which provide useful clinical information for prognostic stratification and treatment.

Usefulness of myocardial contrast echocardiography early after acute myocardial infarction.

OBJECTIVES: (1) Evaluate wall motion and perfusion abnormalities after reperfusion therapy of the culprit lesion, (2) delineate the ability of myocardial contrast echocardiography (MCE) to evaluate the microvasculature after reperfusion, in order to distinguish between stunning and necrosis in the risk area. METHODS: We analyzed 446 segments from 28 patients, 10 normal controls (160 segments), and 18 with a first AMI (286 segments). MCE was obtained with Optison and a two-dimensional echocardiography was performed at 3 months post acute myocardial infarction (AMI). RESULTS: In the group with AMI, we analyzed 286 segments, of which 107 had wall motion abnormalities (WMA) related to the culprit artery. Two subgroups were identified: Group I with WMA and normal perfusion (50 segments, 47%) and Group II with WMA and perfusion defects (57 segments, 53%). According to the 2D echocardiogram at 3 months, they were further subdivided into: Group IA: with wall motion improvement (stunning): 18 segments, 36%, Group IB: without wall motion improvement: 32 segments, 64%, Group IIA: with wall motion improvement: 12 segments, 21%, Group IIB: without wall motion improvement (necrosis): 45 segments, 79%. CONCLUSIONS: (1) The presence of myocardial perfusion in segments with WMA immediately after AMI reperfusion therapy predicts viability in most patients. Conversely, the lack of perfusion is not an absolute indicator of the presence of necrosis. (2) Perfusion defects allow to detect patients with thrombolysis in myocardial infarction (TIMI) 3 flow and "no-reflow" phenomenon who will not show improved wall motion in the 2D echocardiogram. However, some patients with initial no-reflow could have microvascular stunning and their regional contractile function will normalize after a recovery period.

Cinefluoroscopic assessment of mechanical disc prostheses: its value as a complementary method to echocardiography.

BACKGROUND AND AIM OF THE STUDY: Many types of mechanical prostheses are used for heart valve replacement, but it is difficult to distinguish between them using transthoracic echocardiography. Hence, cinefluoroscopy complements the echocardiographic evaluation of cardiac prostheses. The aims of the present study were to: (i) describe the contribution of cinefluoroscopy in identifying different prostheses; (ii) compare gradients obtained by Doppler echocardiography with the opening angle of the discs assessed by cinefluoroscopy; and (iii) assess the ability of cinefluoroscopy to distinguish normal from dysfunctional prostheses. METHODS: A total of 229 mechanical disc prostheses was prospectively evaluated with cinefluoroscopy. Eight prosthetic valves (six aortic, two mitral) were excluded due to the coexistence of severe left ventricular dysfunction. Thus, the final analysis comprised 221 prosthetic valves (146 aortic, 75 mitral). RESULTS: Based on the characteristics of the ring and the discs, cinefluoroscopy identified 87 single-leaflet and 134 bileaflet prostheses. Disc motion allowed distinction to be made between normal and dysfunctional prostheses (opening angle: 74 +/- 13 degrees versus 49 +/- 18 degrees). Fluoroscopy could not define disc profile or the ring in 6% of aortic valves and in 26% of mitral prostheses. The technique could be used to identify the TriTechnologies and HP-Biplus valves, but could not provide data on prosthetic function due to radiolucency of the discs. Among the 146 aortic prostheses, Doppler echocardiography helped to identify 109 normal valves and 37 dysfunctional valves. Among 75 mitral prostheses, 54 normal and 21 dysfunctional prosthetic valves were identified. When both methods were correlated, the sensitivity, specificity and positive and negative predictive values of fluoroscopy to distinguish normal from malfunctioning prostheses were 83%, 80%, 89%, and 71%, respectively. CONCLUSION: Each prosthesis type has radioscopic characteristics that allow its identification. Fluoroscopy permitted rapid and easy evaluation of mechanical prosthetic valve function, and in most cases allowed a distinction to be made between normal and dysfunctional prostheses. The presence of high gradients by Doppler echocardiography, with normal opening angles by fluoroscopy, and without pannus on transesophageal echocardiography, is indicative of patient-prosthesis mismatch. Fluoroscopy was superior to echocardiography in identifying disc motion, whilst Doppler study allowed the measurement of gradients and areas, and semiquantification of regurgitation. Thus, cinefluooscopy rapidly provides valuable information which is complementary to that obtained by echocardiography.

Evaluación ecocardiográfica del aneurisma del septum interauricular / Echocardiographic evaluation of atrial septal aneurysm

Se evaluó la presencia de cortocircuitos interauriculares y la asociación de eventos isquémicos cerebrovasculares en 20 pacientes portadores de aneurisma del septum interauricular (ASIA), realizándose ecocardiograma y Doppler color transtorácico (ETT) y transesofágico (ETE), con contraste en condiciones basales y durante la maniobra de Valsalva. Tuvo cortocircuito interauricular el 70 por ciento (14/20). De éstos, el 71 por ciento fue identificado por ETT con contraste y Valsalva y el 50 por ciento por ETE color, ETT y ETE con contraste en condiciones basales. El 15 por ciento de los pacientes con ASIA mostró accidente cerebrovascular isquémico, transitorio o constituído, de origen indeterminado (AU)

Evaluación ecocardiográfica del aneurisma del septum interauricular / Echocardiographic evaluation of atrial septal aneurysm

Se evaluó la presencia de cortocircuitos interauriculares y la asociación de eventos isquémicos cerebrovasculares en 20 pacientes portadores de aneurisma del septum interauricular (ASIA), realizándose ecocardiograma y Doppler color transtorácico (ETT) y transesofágico (ETE), con contraste en condiciones basales y durante la maniobra de Valsalva. Tuvo cortocircuito interauricular el 70 por ciento (14/20). De éstos, el 71 por ciento fue identificado por ETT con contraste y Valsalva y el 50 por ciento por ETE color, ETT y ETE con contraste en condiciones basales. El 15 por ciento de los pacientes con ASIA mostró accidente cerebrovascular isquémico, transitorio o constituído, de origen indeterminado