RESUMO
El objetivo de esta revisión es analizar y actualizar los aspectos más relevantes de la insuficiencia cardiaca en niños, contrastando con lo que ocurre en la insuficiencia cardiaca en adultos; esto con el propósito de resaltar los elementos comunes entre los dos grupos y, principalmente, destacar las diferencias, por ejemplo, el hecho de que la presencia de la insuficiencia cardiaca en niños no necesariamente implica el evento final de una enfermedad y que en la mayoría de estos casos el pronóstico es mejor. También se revisan en esta primera parte los aspectos históricos, las definiciones y los conceptos, así como la epidemiología, la fisiopatología y el diagnóstico.
The aim of this paper is to review and update, the most relevant aspects of heart failure in children, comparing them with heart failure in adults, in order to establish the common elements between both groups and also to emphasize the differences between both groups. For instance, heart failure in children not necessarily implied poor prognosis and in general, the prognosis in children is better. The history, concepts and definitions, epidemiology, physiopathology and clinical picture are discussed in this first part.
RESUMO
Two pairs of twins with specific concordance in congenital heart lesions are presented. We reviewed all the monozygotic twins with specific concordance in congenital heart lesions in the last 18 years. Two pairs were found. First, a pair of 13 years-old monozygotic female twins; a severe infundibular and pulmonary valve stenosis was discovered in both girls. In the second pair of twins (8 years-old boys), Tetralogy of Fallot was diagnosed by echocardiogram, cardiac catheterization and angicardiography. In fluorescence hybridization test in situ all patients were negative for microdeletion in chromosome 22q11. Anatomic differences between twins are not enough explained on genetic bases; it's necessary to consider the role of other factors, probably acquired during the first stages of embryonic development. These are the two first pairs of twins with specific concordance in congenital cardiac lesions reported in Mexico.
Assuntos
Doenças em Gêmeos/genética , Estenose da Valva Pulmonar/genética , Tetralogia de Fallot/genética , Adolescente , Criança , Feminino , Humanos , Masculino , Gêmeos MonozigóticosRESUMO
The experience with echocardiographic diagnosis of five cases of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) is reviewed. MATERIAL AND METHODS: all cases with a diagnosis of ALCAPA during a 10 year period were included. RESULTS: two age groups were clearly identified: infants and older patients. In the former, the echocardiographic findings included a dilated left ventricle with low ejection fraction (19% and 26%), mitral regurgitation, dilated right coronary artery and difficulties in identifying the origin of the left coronary artery. An 11-year-old asymptomatic boy, a 17-year-old young woman with dyspnea on effort and a 55-year-old woman with angina formed the older group. In these three cases, an abnormal upward flow was detected within the ventricular septum related to the collaterals and the inverse flow of the anterior descending artery. A reverse flow within the origin of the left coronary artery, probably related to an origin in the pulmonary artery, was observed. In all five cases the diagnosis was corroborated using selective right coronary artery angiography. CONCLUSIONS: In the infants, the dilated left ventricle with impaired systolic function, mitral regurgitation and dilated right coronary artery make it necessary to discard the ALCAPA diagnosis. In the older group, even in asymptomatic patients, an abnormal ascending flow within the ventricular septum, combined with a dilated coronary artery, was the most reliable echocardiographic evidence for a diagnosis of ALCAPA.
Assuntos
Adolescente , Criança , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Anormalidades Múltiplas , Anomalias dos Vasos Coronários , Artéria Pulmonar/anormalidades , Artéria PulmonarRESUMO
UNLABELLED: Congenital coronary artery fistulas are uncommon anomalies, however themselves may resemble the whole spectrum of cardiac manifestations. Clinical presentations vary considerably from adults to children. MATERIAL AND METHODS: patients with coronary artery fistula diagnosed by selective coronariography in the period from 2000 to 2007 were included. RESULTS: 7 cases were found, 4 children, mean age 6.5 years, and 3 adults, mean age 25.6 years. The fistulae originate from the left coronary artery in 3 children, 2 connect to the pulmonary artery an one to the right atrium, in one child the fistula originated from the right coronary artery and terminated at the right ventricle. In the 3 adult patients, the fistula originated from the right ventricle, two connected to the right ventricle and one to the coronary sinus. Two children were operated on successfully and are asymptomatic 1 and 4 years later. In an adult patient with surgical ligation was performed, 4 weeks later developed an uncomplicated myocardial infarction. We reviewed the literature available and were compared with our cases. CONCLUSIONS: The clinical manifestations and hemodynamic features differ in childhood and adulthood. Coronary angiography is the primary diagnostic tool. Surgical treatment has low mortality and morbidity.
Assuntos
Anomalias dos Vasos Coronários/diagnóstico por imagem , Fístula Vascular/congênito , Fístula Vascular/diagnóstico por imagem , Adulto , Criança , Pré-Escolar , Anomalias dos Vasos Coronários/cirurgia , Feminino , Humanos , Masculino , Radiografia , Fístula Vascular/cirurgia , Adulto JovemRESUMO
Congenital coronary artery fistulas are uncommon anomalies, however themselves may resemble the whole spectrum of cardiac manifestations. Clinical presentations vary considerably from adults to children. MATERIAL AND METHODS: patients with coronary artery fistula diagnosed by selective coronariography in the period from 2000 to 2007 were included. RESULTS: 7 cases were found, 4 children, mean age 6.5 years, and 3 adults, mean age 25.6 years. The fistulae originate from the left coronary artery in 3 children, 2 connect to the pulmonary artery an one to the right atrium, in one child the fistula originated from the right coronary artery and terminated at the right ventricle. In the 3 adult patients, the fistula originated from the right ventricle, two connected to the right ventricle and one to the coronary sinus. Two children were operated on successfully and are asymptomatic 1 and 4 years later. In an adult patient with surgical ligation was performed, 4 weeks later developed an uncomplicated myocardial infarction. We reviewed the literature available and were compared with our cases. CONCLUSIONS: The clinical manifestations and hemodynamic features differ in childhood and adulthood. Coronary angiography is the primary diagnostic tool. Surgical treatment has low mortality and morbidity.
Assuntos
Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Adulto Jovem , Anomalias dos Vasos Coronários , Fístula Vascular/congênito , Fístula Vascular , Anomalias dos Vasos Coronários , Fístula VascularRESUMO
UNLABELLED: The experience with echocardiographic diagnosis of five cases of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) is reviewed. MATERIAL AND METHODS: all cases with a diagnosis of ALCAPA during a 10 year period were included. RESULTS: two age groups were clearly identified: infants and older patients. In the former, the echocardiographic findings included a dilated left ventricle with low ejection fraction (19% and 26%), mitral regurgitation, dilated right coronary artery and difficulties in identifying the origin of the left coronary artery. An 11-year-old asymptomatic boy, a 17-year-old young woman with dyspnea on effort and a 55-year-old woman with angina formed the older group. In these three cases, an abnormal upward flow was detected within the ventricular septum related to the collaterals and the inverse flow of the anterior descending artery. A reverse flow within the origin of the left coronary artery, probably related to an origin in the pulmonary artery, was observed. In all five cases the diagnosis was corroborated using selective right coronary artery angiography. CONCLUSIONS: In the infants, the dilated left ventricle with impaired systolic function, mitral regurgitation and dilated right coronary artery make it necessary to discard the ALCAPA diagnosis. In the older group, even in asymptomatic patients, an abnormal ascending flow within the ventricular septum, combined with a dilated coronary artery, was the most reliable echocardiographic evidence for a diagnosis of ALCAPA.
Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Anomalias dos Vasos Coronários/diagnóstico por imagem , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Adolescente , Criança , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , UltrassonografiaRESUMO
In patients with Bidirectional Glenn who have undergone surgery, the superior caval venous flow provides the only pulmonary blood supply. This is the effective pulmonary flow and at the same time its volume is not enough to overflow the single ventricle. The unsaturated, inferior vena cava flow is not oxygenated, since it goes across the interatrial septal communication and gets mixed in the left ventricle with the pulmonary venous blood. In this work, a bidirectional Glenn case is analyzed. The hemodynamic data before and after the operation are shown. It was evident from this case that the use of the Fick method to measure pulmonary flow in patients with bidirectional Glenn operation is not appropriate. Alternative methods, such as Doppler echocardiography and Magnetic Resonance Imaging, are recommended. A literature review on this subject was carefully done.
Assuntos
Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/cirurgia , Fluxo Sanguíneo Regional , Atresia Tricúspide/cirurgia , Veia Cava Superior/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , HumanosRESUMO
Clinical term of association refers to a not randomized congenital malformations which are present in one single subject. The term MURCS is an acronym for (MU) Mullerian, (R) Renal, (C) Cervicothoracic, (S) Somite abnormalities. We communicate a case of a phenotipically normal 16 years old female patient with primary amenorrhea due to müllerian malformations and cervicothoracic dysplasia integrating the MURCS association diagnosis.