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.

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)

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.

Left ventricular diverticulum, a rare echocardiographic finding: two adult patients and review of the literature.

Congenital diverticulum of the left ventricle is a very rare cardiac abnormality characterized by a local embryological development failure of the ventricular muscle. It may present as an isolated disorder or may be associated with other cardiac abnormalities. Clinically, it has been reported that it can lead to chest pain, arrhythmias, cardiac rupture, and sudden death, although frequently the course is asymptomatic. This article presents two illustrative cases of left ventricular diverticulum. The diagnosis was made by two-dimensional echocardiography and was confirmed by cardiac catheterization. There were no other thoracoabdominal or cardiac abnormalities. The patient was asymptomatic and surgical resection was not indicated. Given the few cases reported in the medical literature, the information available is scarce; hence, physicians should be trained to make the appropriate diagnosis.

Intermittent acute aortic regurgitation of a mechanical bileaflet aortic valve prosthesis: diagnosis and clinical implications.

Intermittent aortic regurgitation (AR) is an unusual complication after a mechanical prosthetic replacement. We describe a rare case of intermittent dysfunction of a bileaflet mechanical aortic prosthetic valve in a 41-year-old man with a 21 mm Tri-technologies prosthetic valve implanted 4 years before. Transthoracic echocardiography (TTE) before discharge was normal and prosthesis-patient mismatch was ruled out. He was admitted to our hospital because of mild dyspnoea at effort. TTE revealed acute and severe intermittent AR. The patient underwent surgery, during which abnormal proliferation of subvalvular pannus overgrowth on the inflow aspect of the prosthesis was found impeding the normal closure of one of the discs of the prosthesis. The pannus formation was resected, the Tri-technologies prosthetic valve was prophylactic explanted and a 23 mm St Jude Medical bileaflet mechanical prosthesis valve was implanted. We describe the role of TTE and the limitation of the cinefluoroscopy in the diagnosis of Tri-technologies prosthetic dysfunction.

[Usefulness of pulsed Doppler tissue imaging in the early detection of diastolic myocardial abnormalities in first-degree relatives of patients with familial hypertrophic cardiomyopathy]. / Utilidad del Doppler pulsado tisular en la detección precoz de anormalidades diastólicas en familiares de primer grado de pacientes con miocardiopatía hipertrófica familiar.

INTRODUCTION AND OBJECTIVES: Doppler tissue imaging (DTI) enables regional myocardial function to be assessed irrespective of preload. Our aim was to determine the usefulness of DTI in healthy relatives of patients with familial hypertrophic cardiomyopathy. PATIENTS AND METHOD: We studied 47 first-degree relatives of patients with familial hypertrophic cardiomyopathy (group GI) and 47 normal subjects (group GII). Echocardiographic studies, including DTI, were performed in both groups. DTI was used to measure myocardial velocities during systole (S'), early diastole (E') and atrial contraction (A') at the mitral annulus, septum, and tricuspid annulus. Isovolumic relaxation and contraction times were corrected for heart rate. With DTI assessment of regional diastolic myocardial function and use of the E'/A' ratio, normal (i.e., E'/A'>1) and abnormal (i.e., E'/A'<1) diastolic function could be distinguished. RESULTS: There were no abnormalities in regional diastolic function in group GII (right E'/A' 1.78 (0.58), septum E'/A' 2.03 (0.53), and left E'/A' 2.55 (0.80). However, in group GI, two subgroup could be distinguished using E'/A' ratio values: group GIa comprised 37 first-degree relatives with normal diastolic function (right E'/A' 1.8 (0.44), septum E'/A' 2.07 (0.41), and left E/A 2.35 (0.6) who did not differ significantly from control subjects, and group GIb comprised 10 first-degree relatives with abnormal right diastolic function (right E'/A' 0.70 (0.28), P<.001) but with less significant differences at the septum (1.57 (0.49), P<.01) and mitral annulus (1.85 (0.53), P<.01). CONCLUSIONS: In 10 of 47 (21.3%) first-degree relatives of patients with familial hypertrophic cardiomyopathy, DTI enabled the detection of ventricular diastolic abnormalities, which were more evident in the right ventricle. They could be a preclinical manifestation of disease.

Utilidad del Doppler pulsado tisular en la detección precoz de anormalidades diastólicas en familiares de primer grado de pacientes con miocardiopatía hipertrófica familiar / Usefulness of pulsed doppler tissue imaging in the early detection of diastolic myocardial abnormalities in first-degree relatives of patients with familial hypertrophic cardiomyopathy

Introducción y objetivos. El Doppler pulsado tisular (DPT) permite analizar la función miocárdica regional independientemente de la precarga. Se realizó DPT a los familiares de primer grado de pacientes con miocardiopatía hipertrófica familiar (MHF) para detectar anormalidades precoces. Pacientes y método. Se estudió a 47 familiares de primer grado de pacientes con MHF (grupo GI) y se los comparó con un grupo control de 47 sujetos normales (grupo GII). Se realizó ecocardiografía 2D y Doppler pulsado de los flujos mitral y tricuspídeo. Mediante DPT en la pared lateral del ventrículo izquierdo, el ventrículo derecho y el septo interventricular se midieron las velocidades pico S', E' y A' y los períodos de relajación y contracción isovolumétricos. Se consideró función diastólica regional normal a una relación E'/A' > 1 y disfunción diastólica a una relación E'A'< 1. Resultados. No hubo alteraciones de la relajación ventricular en el GII (E'A' derecho, 1,78 ± 0,58; septo, 2,03 ± 0,53; izquierdo, 2,55 ± 0,80), mientras que en el GI la relación E'/A' permitió diferenciar 2 subgrupos: GIa de 37 familiares de primer grado que presentaron patrón de relajación normal (E'/A' derecho, 1,8 ± 0,44; septo, 2,07 ± 0,41; izquierdo, 2,35 ± 0,6, sin diferencias significativas con el grupo control) y GIb de 10 familiares de primer grado con relajación prolongada en el ventrículo derecho (E'/A', 0,70 ± 0,28; p < 0,001), con menor tendencia significativa en el septo (1,57 ± 0,49; p < 0,01) y el lateral izquierdo (1,85 ± 0,53; p < 0,01). Conclusiones. En familiares de primer grado de pacientes con MHF, el DPT permitió diferenciar anormalidades de la relajación ventricular en 10 de los 47 casos (21,3%), más manifiestas en el ventrículo derecho, que podrían ser un marcador preclínico de la enfermedad

Introduction and objectives. Doppler tissue imaging (DTI) enables regional myocardial function to be assessed irrespective of preload. Our aim was to determine the usefulness of DTI in healthy relatives of patients with familial hypertrophic cardiomyopathy. Patients and method. We studied 47 first-degree relatives of patients with familial hypertrophic cardiomyopathy (group GI) and 47 normal subjects (group GII). Echocardiographic studies, including DTI, were performed in both groups. DTI was used to measure myocardial velocities during systole (S'), early diastole (E') and atrial contraction (A') at the mitral annulus, septum, and tricuspid annulus. Isovolumic relaxation and contraction times were corrected for heart rate. With DTI assessment of regional diastolic myocardial function and use of the E'/A' ratio, normal (i.e., E'/A'>1) and abnormal (i.e., E'/A'<1) diastolic function could be distinguished. Results. There were no abnormalities in regional diastolic function in group GII (right E'/A' 1.78 (0.58), septum E'/A' 2.03 (0.53), and left E'/A' 2.55 (0.80). However, in group GI, two subgroup could be distinguished using E'/A' ratio values: group GIa comprised 37 first-degree relatives with normal diastolic function (right E'/A' 1.8 (0.44), septum E'/A' 2.07 (0.41), and left E/A 2.35 (0.6) who did not differ significantly from control subjects, and group GIb comprised 10 first-degree relatives with abnormal right diastolic function (right E'/A' 0.70 (0.28), P<.001) but with less significant differences at the septum (1.57 (0.49), P<.01) and mitral annulus (1.85 (0.53), P<.01). Conclusions. In 10 of 47 (21.3%) first-degree relatives of patients with familial hypertrophic cardiomyopathy, DTI enabled the detection of ventricular diastolic abnormalities, which were more evident in the right ventricle. They could be a preclinical manifestation of disease

Left cavities spontaneous contrast opacification.

We report a transesophageal echocardiography study of a patient in a critical care unit under mechanical respiratory assistance in whom left cavities spontaneous contrast opacification was seen, suggesting systemic gas embolism as the probable mechanism.