Late gadolinium enhancement distribution pattern of left ventricular wall in patients with dilated cardiomyopathy: a preliminary study / 中华心血管病杂志

Objective: To investigate the distribution pattern of late gadolinium enhancement (LGE) in left ventricular free wall of patients with dilated cardiomyopathy (DCM). Methods: A total of 130 consecutive DCM patients who were hospitalized in our hospital, underwent both CMR and CTA examinations and met the inclusion and exclusion criteria including negative results of coronary angiography or coronary CTA, were retrospective included in this study. The LGE pattern, extent and distribution in left ventricular free wall were analyzed. Results: Left ventricular free wall LGE was detected in 56 out of 130 DCM patients. LGE was observed in both septal and free wall in 53 out of 56 patients with LGE (94.6%). Prevalence of NYHA classification Ⅲ/Ⅳ, intraventricular block, paroxysmal ventricular tachycardia, and secondary mitral insufficiency was significantly higher, while left ventricular ejection fraction was significantly lower, left ventricular end-diastolic/systolic volume, left ventricular end-diastolic/systolic volume index and left ventricular end-diastolic diameters values were larger in patients with LGE than without LGE (all P<0.05). In terms LGE pattern among these 56 patients, percent of involved myocardial segments in patients with subepicardial LGE (n=19) was significantly higher than patients with intermural LGE (n=30), patients with transmural LGE (n=21), and patients with subendocardial LGE (n=9)(60.8%(127/209) vs. 32.4%(107/330), 32.5%(75/231), 26.3%(26/99), respectively, all P < 0.01). Transmural LGE was most likely to involve the left ventricular inferior lateral basal (18/21) and mid (13/21) segment, followed by anterior lateral basal (15/21) and mid (11/21) segments and inferior mid segment (9/21). Subepicardial LGE was more likely to occur in the inferior lateral basal (13/19) and mid (16/19) segment, anterior lateral basal (13/19) and mid (15/19) segment, anterior lateral basal (13/19) and mid (15/19) segment, lateral apical (13/19), anterior and inferior mid segment (12/19 and 10/19), and apical segment (15/19 and 10/19). Intermural LGE mostly involved the anterior and inferior basal (19/30, 16/30) and mid (18/30 and 14/30) segment. There were 33 cases of single LGE pattern and 23 cases of multiple LGE pattern. Percent of involved myocardial segments was significantly higher in multiple LGE group than single LGE group (60.9% (154/253) vs. 49.9%(181/363), P = 0.007). Of 130 patients, 23 received heart transplantation, of which 6 patients had septal LGE alone and 17 patients had septal and free wall LGE. The rate of heart transplantation in the latter group was higher (32.1% (17/53)vs. 13.6%(6/44), P=0.034). Conclusions: There are several LGE distribution patterns in left ventricular wall among DCM patients.

Miocardiopatía dilatada secundaria a hipotiroidismo primario. Reporte de un caso / Dilated cardiomyopathy secondary to primary hypothyroidism. A case report

Resumen El vínculo entre el hipotiroidismo y varias enfermedades cardiovasculares va más allá de la asociación con los factores de riesgo vascular clásicos. Sin embargo, la miocardiopatía dilatada es una forma rara de presentación del hipotiroidismo. Se reporta el caso de una paciente femenina de 52 años que acude a urgencias por presentar tos húmeda, edema en miembros inferiores y disnea de esfuerzo, así como otros síntomas y signos típicos de hipotiroidismo. La radiografía de tórax revela cardiomegalia y el ecocardiograma informa ventrículo izquierdo dilatado, fracción de eyección disminuida y derrame pericárdico. La TSH estaba aumentada y T4 libre disminuida. Luego de comenzar tratamiento hormonal con levotiroxina se alcanzó la resolución de los síntomas y de las alteraciones radiológicas y ecocardiográficas. El presente caso resalta la importancia de realizar un interrogatorio exhaustivo y examen físico completo que aporten las pistas diagnósticas para sospechar el hipotiroidismo en pacientes con miocardiopatía dilatada.

ABSTRACT The link between hypothyroidism and several cardiovascular diseases goes beyond the association with classical vascular risk factors. However, dilated cardiomyopathy is a rare form of presentation of hypothyroidism. We report the case of a 52-year-old female patient who comes to the emergency room for having wet cough, lower limb edema and dyspnea on exertion, as well as other typical symptoms and signs of hypothyroidism. Chest x-ray reveals cardiomegaly and the echocardiogram reports dilated left ventricle, decreased ejection fraction and pericardial effusion. TSH was increased and free T4 was decreased. After starting hormonal treatment with levothyroxine, resolution of symptoms and radiological and echocardiographic alterations was achieved. The present case highlights the importance of a thorough interrogatory and complete physical examination that provide the diagnostic clues to suspect hypothyroidism in patients with dilated cardiomyopathy.

Speckle Tracking and Transthyretin Amyloid Cardiomyopathy / Rastreamento de Pontilhados e Cardiomiopatia Amiloidótica por Mutação da Transtirretina

Abstract Background: Amyloidosis is a disease caused by deposits of insoluble fibrils in extracellular spaces. The most common type of familial amyloidosis is mediated by mutation of transthyretin, especially Val30Met. Symptoms and ejection fraction decrease may occur in cardiac amyloidosis only in case of poor prognosis. Myocardial strain detected by two-dimensional speckle tracking echocardiography can indicate changes in myocardial function at early stages of the disease. Objective: To determine the accuracy of left ventricular longitudinal strain by two-dimensional speckle tracking echocardiography in patients with familial amyloidosis caused by Val30Met transthyretin mutation. Methods: Eighteen consecutive patients, carriers of transthyretin mutation, were evaluated by two-dimensional speckle tracking echocardiography, by which myocardial strain curves were obtained, following the American Society of Echocardiography recommendations. Results: Patients were divided into three groups: 1- Val30Met with cardiac amyloidosis; 2-Val30Met with extracardiac amyloidosis; 3 - Val30Met without evidence of disease. As the three groups were compared by the Mann-Whitney test, we found a statistically significant difference between groups 1 and 2 in the mean longitudinal tension (p=0.01), mean basal longitudinal strain (p=0.014); in mean longitudinal tension and mean longitudinal strain between groups 1 and 3 (p=0.005); and in the ratio of longitudinal strain of apical septum segment to longitudinal strain of basal septum (p=0.041) between groups 2 and 3. Conclusion: Left ventricular longitudinal strain detected by two-dimensional speckle tracking echocardiography is able to diagnose left ventricular dysfunction in early stages of familial amyloidosis caused by transthyretin Val30Met mutation.

Resumo Fundamento: A amiloidose é uma doença de depósito de fibrilas insolúveis nos espaços intercelulares. A forma mais comum de amiloidose familiar é mediada por mutação da transtirretina, sendo a Val30Met a mutação mais frequente. A amiloidose cardíaca só causa sintomas e queda da fração de ejeção em fases tardias quando o prognóstico é pobre. A deformação miocárdica obtida com speckle tracking bidimensional pode detectar alterações da função miocárdica em estágios precoces da doença. Objetivos: Determinar a acurácia da deformação longitudinal do ventrículo esquerdo obtida com speckle tracking bidimensional em um grupo de pacientes com amiloidose familial por mutação da transtirretina Val30Met. Métodos: Foram examinados 18 pacientes consecutivos com a mutação da transtirretina com speckle tracking bidimensional obtendo curvas de deformação miocárdica segundo normas da American Society of Echocardiography. Resultados: Os pacientes foram divididos em três grupos: 1- Val30Met com amiloidose cardíaca; 2- Val30Met com amiloidose extra-cardíaca; 3- Val30Met sem doença aparente. Ao compararmos os três grupos com o teste de Mann-Whitney encontramos diferença estatística significativa entre os grupos 1 e 2 na tensão longitudinal média (p=0,01), deformação longitudinal basal média (p=0,014); entre os grupos 1 e 3 na tensão longitudinal média (p=0,005), deformação longitudinal média (p=0,002); entre os grupos 2 e 3 na relação de deformação longitudinal do septo apical/deformação longitudinal do septo basal (p=0,041). Conclusão: A deformação longitudinal do ventrículo esquerdo obtida com speckle tracking bidimensional é capaz de diagnosticar disfunção do ventrículo esquerdo em fases precoces da amiloidose familial por mutação Val30Met da transtirretina.

Miocardiopatía dilatada reversible asociada a hipotiroidismo severo / Reversible dilated cardiomyopathy in hypothyroidism, case report

Dilated cardiomyopathy is usually not reversible. In a few cases diverse etiologies can be treated and cardiomyopathy may disappear or improve significantly. The case of a young man with hypothyroidism and dilated cardiomyopathy is presented. After thyroid hormone replacement, a severe left ventricular dysfunction became reverted.

Evolución clínica y ecográfica posterior a terapia de resincronización cardíaca / Clinical and echocardiographi follow up after cardiac resynchronization

Antecedentes: La terapia de resincronización cardíaca (TRC) está adquiriendo importancia como tratamiento complementario en insuficiencia cardíaca avanzada. En Chile, el seguimiento clínico y ecográfico de este grupo de pacientes no ha sido suficientemente investigado y se desconoce la realidad nacional. Objetivos: Describir los cambios clínicos y eco-gráficos posterior a TRC en los pacientes del servicio de cardiología del Hospital Carlos Van Buren de Valparaíso, sometidos al procedimiento entre los años 2013 y 2015. Métodos: Se realizó un análisis descriptivo de 10 pacientes con diagnóstico de miocardiopatía dilatada sometidos a TRC, recolectándose variables clínicas y ecográficas previo y al sexto mes post-procedimiento. Se utilizó frecuencias y medianas para describir los resultados. Se definió como respondedores aquellos pacientes que mejoraron su capacidad funcional luego de la intervención. Resultados: Del total de pacientes sometidos a TRC, hubo un 30% de no respondedores, en los cuales no se encontraron predictores de fracaso. En los respondedores se observó mejoría de la capacidad funcional y al menos una de las siguientes: aumento de la fracción de eyección, disminución del diámetro telediastólico del ventrículo izquierdo y reducción del grado de regurgitación mitral. No se observó reducción en el número de hospitalizaciones al año. Conclusiones: Se demuestra la utilidad de la TRC en el tratamiento de la insuficiencia cardíaca avanzada en un grupo seleccionado de pacientes. Se necesitan estudios locales con mayor número de paciente para representar nuestra realidad nacional.

Background: Cardiac resynchronization therapy (CRT) is gaining relevance as an important therapy in patients with advanced heart failure. In Chile, the clinical and echocardiographic follow-up of these patients has not been sufficiently documented. Aim: To describe the clinical and echocardiographic changes after CRT in patients treated at the cardiology department of the Carlos Van Buren Hospital in Valparaiso, undergoing the procedure between 2013 and 2015. Methods: A descriptive analysis of 10 patients with a diagnosis of dilated cardiomyopathy submitted to CRT was performed. Clinical and echocardiographic data was collected before and sixth months after the procedure. Frequency and median were used to describe the results. Responders were defined as patients who improved their functional capacity after the intervention. Results: Of all patients undergoing CRT, there were 3 (30%) non-responder patients. In responders improvement in functional capacity and increased ejection fraction or decreased left ventricular diastolic diameter or reduction of mitral regurgitation was observed. No reduction was observed in the number of hospitalizations annually. Conclusion: The results support the usefulness of CRT in treating advanced heart failure in a selected group of patients. Further studies are needed to better define our national reality and predictors of a favorable response after the procedure.

Three-Dimensional Endo-Cardiovascular Volume-Rendered Cine Computed Tomography of Isolated Left Ventricular Apical Hypoplasia: A Case Report and Literature Review

We report multidetector computed tomography (MDCT) and cardiac magnetic resonance (CMR) findings of a 34-year-old female with isolated left ventricular apical hypoplasia. The MDCT and CMR scans displayed a spherical left ventricle (LV) with extensive fatty infiltration within the myocardium at the apex, interventricular septum and inferior wall, anteroapical origin of the papillary muscle, right ventricle wrapping around the deficient LV apex, and impaired systolic function. MDCT visualized morphologic and also functional findings of this unique cardiomyopathy.

Sequential functional analysis of left ventricle from 2D-echocardiography images.

The sequential changes in shape of left ventricle (LV), which are the result of cellular interactions and their levels of organizational complexity, in its long axis view during one cardiac cycle are obtained. The changes are presented in terms of shape descriptors by processing of images obtained from a normal subject and two patients with dilated left ventricular cardio-myopathy. These images are processed, frame by frame, by a semi-automatic algorithm developed by MATLAB. This is consisting of gray scale conversion, the LV contour extraction by application of median and SRAD filters, and morphological operations. By filling the identified region with pixels and number of pixels along its contour the area and perimeter are calculated, respectively. From these the changes in LV volume and shape index are calculated. Based on these the stroke volume (SV) and ejection fraction (EF) are calculated. The changes in LV area, perimeter, volume and shape index in cardiac patients are less than that of normal subject. The calculated SV and EF of normal subject are within the range as obtained by various imaging procedures.

Ressonância magnética cardíaca na cardiomiopatia dilatada: atualidades / Cardiac magnetic resonance in dilated cardiomyopathy: an update

JUSTIFICATIVA E OBJETIVOS: A cardiomiopatia dilatada(CMD) é a mais comum das cardiomiopatias, compreendendo mais de 90% de todos os casos. O seu diagnóstico é feito, sobretudo,com a utilização da ecocardiografia, a qual apresenta algumas limitações quanto à identificação da etiologia. A ressonância magnética cardíaca (RMC) tem se destacado como exame coadjuvante, permitindo melhor caracterização morfofuncional e tecidual do músculo cardíaco. O objetivo deste estudo foi descrevero papel da RMC no diagnóstico e avaliação das diversas causas de CMD.CONTEÚDO: A RMC apresentou bons resultados na aplicaçã oclínica em diversas causas de CMD, não possuindo capacidade de diferenciar todos os seus tipos, porém, contribuindo para a presunção diagnóstica.CONCLUSÃO: Com o avanço tecnológico da RMC, múltiplas indicações clínicas têm surgido em algumas ocasiões, com um aspecto complementar a determinados exames, em outras, como solução de dilemas diagnósticos. Tudo isso com acurácia superior aos demais métodos de imagem, poucas contraindicações e mínimos riscos de efeitos adversos.(AU)

BACKGROUND AND OBJECTIVES: Dilated cardiomyopathy (DCM) is the most common cause of cardiomyopathies,including more than 90% of all cases. Its diagnostic is done using, especially, echocardiography; which presents some limitations regarding the etiology's identification. Cardiac magnetic resonance (CMR) has been very useful as a more precise study, allowing better characterization of myocardium tissue and morphology. The objective of this article was to describe the role of the CMR in the diagnosis and evaluation of the diverse causes of DCM.CONTENTS : The CMR presented good results in the clinical applicationin diverse causes of DCM, not having capacity to differentiate all their types; however, better contributing to the diagnostic and patients follow up.CONCLUSION: With the technological development of CMR, several clinical indications have been created, in a few occasions with a complementing aspect to certain exams, in others with solutions of diagnostic dilemmas. All with higher accuracy in comparison to others image modalities, few contraindications and minimum adverse risks effects.(AU)

Naxos disease.

Naxos disease (OMIM 601214) characterized by wooly hair, palmoplantar keratoderma and dilated cardiomyopathy in two Indian children of the same family is described.

value of myocardial perfusion imaging in differentiating between idiopathic dilated cardiomyopathy from the ischemic form

Differentiating between ischemic cardiomyopathy [ICM] and idiopathic dilated cardiomyopathy [IDCM] is important as coronary revascularization can improve prognosis in the ischemic subgroup. Due to inherent problems of coronary angiography in patients with depressed ejection fraction [EF] introducing a noninvasive tool to diagnose those who will benefit from angiography seems to be rewarding. We examined usefulness of myocardial perfusion scan in this group of patients. Study was performed on 64 patients [62 male and 2 female] aged 57.1 +/- 6.7y [mean +/- SD] all with dilation of the left ventricular [LV] cavity and ejection fraction less than 40% by echocardiography. Myocardial perfusion scan was performed in stress and rest phases. All the patients had coronary angiography which was used as the gold standard test. On each set of images, heart was arbitrary divided into 17 segments and perfusion abnormality in each segment was scored by a 5 grade scoring system [0-4]. Summed Stress Score was used as the scan criteria to differentiate dilated ischemic from idiopathic cardiomyopathy. Scores more than 17 were considered ischemic, and less than that, idiopathic. Results were compared with angiography. From total 40 cases of ischemic cardiomyopathy [proved by angiography] 39 were correctly diagnosed by scan and only one case was miscategorized as IDCM. All 24 cases of IDCM were correctly diagnosed by scintigraphy. Sensitivity, specificity, positive predictive value, and negative predictive value of myocardial perfusion imaging for discrimination between ischemic and idiopathic dilated cardiomyopathy were 97.5%, 100%, 100%, and 96% respectively. Considering excellent accuracy of myocardial perfusion scan with scoring system in discrimination of ischemic dilated cardiomyopathy from idiopathic dilated cardiomyopathy, this noninvasive test could be considered the main diagnostic test

Frequency and echocardiographic study of dilated cardiomyopathy in children presenting with cardiac failure

To evaluate the role of echocardiography in diagnosis of dilated cardiomyopathy as a cause of cardiac failure in children. Design: This was descriptive study. Children presenting with cardiac failure from indoor patients were selected and echocardiography along with chest X- ray, ECG, cardiac enzymes and ASO titre was performed in all patients. Setting: Hospitalized patients included in the study were selected from the Department of Child Health, Khyber Teaching Hospital. Fifty hospitalized patients with congestive heart failure were selected consecutively from hospitalized patients. Main Outcome: Role of echocardiography in the diagnosis of dilated cardiomyopathy in children presenting with cardiac failure. Out of fifty patients admitted with cardiac failure 27 [54%] cases were found to be dilated cardiomyopathy while congenital heart disease, myocarditis and rheumatic heart disease were found in 12 [24%], 8 [16%] and 3 [6%] cases respectively. Dilated cardiomyopathy is an important cause of cardiac failure in children and echocardiography is an important tool to diagnose and differentiate dilated cardiomyopathy from other causes of cardiac failure