Idiopathic, nonobstructive cardiomyopathy in children (original) (raw)
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A case of non-compaction cardiomyopathy
Scripta Scientifica Medica, 2013
A female patient was admitted to the hospital for the first time on the occasion of sudden breath shortness and palpitations. Blood pressure was 220/120 mm Hg and antihypertensive medications were administered. The patient had no history of arterial hypertension. However, she reported a similar episode a few weeks ago. Family history showed a sister with arettrtial hypertension. Physical examination at admission revealed a sinus rhythm, heart rate of 110/ min, T3 gallop, crepitation in the lower lung fields, and blood pressure of 145/100 mm Hg. CT of the thorax demonstrated pulmonary congestion with dilated pulmonary arteries: pulmonary trunk-26 mm; right main branch of pulmonary artery-16,5 mm and the left main branch of pulmonary artery-20 mm. There were no signs of pulmonary thromboembolism (Fig. 1). Electrocardiogram (ECG) displayed a sinus rhythm, intraventricular conduction disturbancies, QRS-109 msec, and non-specific ST-T changes (Fig. 2). The following imaging methods were used for diagnostic purposes: Echocardiography Echocardiography demonstrated trabecularization of the segments of the left ventricle with a
American Journal of Case Reports, 2013
Challenging differential diagnosis Background: Non-compaction cardiomyopathy (NCM) is a rare congenital cardiomyopathy characterized by increased trabeculation in one or more segments of the ventricle. The left ventricle is most commonly affected. However, biventricular involvement or right ventricle predominance has also been described. Clinical features of NCM are non-specific and can range from being asymptomatic to symptoms of congestive heart failure, arrhythmia, and systemic thromboembolism. Case Report: 22-year-old Hispanic male presented with two month history of chest discomfort. Laboratory workup revealed an elevated brain-natriuretic-peptide of 1768 pg/ml. ECG and chest x-ray was nonspecific. Transthoracic echocardiogram revealed prominent trabeculae and spongiform appearance of the left ventricle (LV) with an ejection-fraction of 15-20%; 5 of 9 segments of the LV were trabeculated with deep intertrabecular recesses also involving the right ventricle (RV) with demonstrated blood flow in these recesses on color-doppler. The biventricular spongiform appearance was morphologically suggestive for NCM with involvement of the RV. Confirmatory cardiac MRI was performed, demonstrating excessive trabeculation of the left-ventricular apex and mid-ventricular segments. Hypertrabecularion was exhibited at the apical and lateral wall of the RV. Cardiac catheterization showed an intact cardiac vessel system. The patient was discharged on heart failure treatment and was placed on the heart transplantation list. Conclusions: NCM is a unique disorder resulting in serious and severe complications. The majority of the reported cases describe the involvement of the left ventricle. However, the right ventricle should be taken into careful consideration. The early diagnosis may help to increase the event-free survival.
Unusual cause of left ventricular dysfunction in a child
Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology, 2018
Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery is a rare congenital heart disease and a cause of myocardial ischemia during childhood. Most undiagnosed cases die in the first year of life as an extensive collateral network is essential for survival. The diagnosis requires a high index of clinical suspicion. The authors present the case of an 8-year-old black asymptomatic child referred from Cape Verde Island in order to clarify left ventricular dilatation and dysfunction with systo-diastolic turbulent flows observed at the interventricular septum. At the age of 3 months, she was diagnosed with heart failure, in the context of showing dilated cardiomyopathy. She was managed and clinically improved with anticongestive therapy, which she was still taking at the time of admission to our Center. The echocardiogram findings suggested Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery and the diagnosis was confirmed by computerized angiotomograph...
Cardiomyopathy complicated by left ventricular aneurysms in children
Heart, 1976
Ventricular aneurysms in children are unusual. Three patients with cardiomyopathy associated with angiographically proved left ventricular aneurysms in this age group are reported. Two of them weregirls. The ages were 20 months, 7 years, and 14 years. Heart failure was present in all patients. There was radiological evidence of cardiomegaly in all three, and the electrocardiogram showed signs of necrosis in two of them. Selective left ventricular angiography disclosed generalized hypokinesis in all patients. One child had an aneurysm of the diaphragmatic wall. In another the aneurysm was localized in the muscular ventricular septum, causing severe subpulmonary stenosis by encroaching in the right ventricular outflow tract during systole. The third patient had an aneurysm of the left ventricular free wall partly encircling the left ventricle. The coronary arteries appeared normal in all cases. The clinical features of the underlying disease were not altered by the presence of the aneurysm except in the patient with the septal aneurysm and subpulmonary stenosis. In this patient the aneurysm was successfully resected. The commonest cause of left ventricular aneurysm aneurysms occurring almost exclusively in Africans in adults is ischaemic heart disease (Cheng, 1971). (Pocock et al., 1965) have also been described in Unusual causes for this lesion include syphilis this age group. (Braunstein, Bass, and Thomas, 1940), tuberculous It is the purpose of this communication to granuloma (Beheyt and VandenPutte, 1958), present three children with cardiomyopathies and mycotic embolism (Layman and January, 1967), angiographically proven left ventricular aneurysms. Chagas's disease (Moia, Rosenbaum, and Hojmen, 1955), fungal endocarditis (Mershon, Samuelson, Case reports and Layman, 1968), nonspecific myocarditis
ARRHYTHMOGENIC CARDIOMYOPATHY OF THE RIGHT VENTRICLE: CLINICAL AND PHYSIOPATHOLOGICAL ASPECTS (Atena Editora), 2024
A cardiomiopatia arritmogênica do ventrículo direito (CAVD) é uma entidade clínica hereditária progressiva, caracterizada fisiologicamente por mutações que codificam o músculo cardíaco, e afeta predominantemente o ventrículo direito. O objetivo do estudo realizado é avaliar e consolidar as principais evidências disponíveis sobre cardiomiopatia arritmogênica do ventrículo direito e seus principais aspectos clínicos e fisiopatológicos, utilizando estudos e artigos publicados nos últimos 5 anos. Revisão integrativa, de nível exploratório e qualitativa de pesquisa, por meio de buscas nas bases de dados científicas PubMed, BVS e SciELO. Os critérios de inclusão estabelecidos foram: artigos originais disponíveis em formato eletrônico, na íntegra, redigidos em português, inglês ou espanhol, com delimitação de tempo de 2019 a 2024. A investigação foi realizada em março de 2024. Com a pesquisa realizada, foram encontrados 360 artigos, destes 210 foram selecionados para leitura e, conforme o objetivo do presente trabalho, 10 foram incluídos na pesquisa. Em síntese, apesar de ser rara a CAVD relaciona-se com alterações potencialmente fatais, tais como arritmias malignas que cursam com morte súbita, o que torna evidente a importância de estudos conservadores e aprofundados para definir bem a incidência na população geral que ainda é mal conhecida e seu mecanismo etiopatogênico, como doença genética ou adquirida, ou sequela de miocardite.