Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso / Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

Comunicação interventricular pequena: conduta clínica expectante em longo prazo / Comunicación interventricular pequeña: conducta clínica expectante a largo plazo / Small ventricular septal defect: long-term expectant clinical management

FUNDAMENTO: A comunicação interventricular (CIV) pequena apresenta geralmente boa evolução clínica, mesmo em longo prazo. OBJETIVO: Verificar evolução clínica de pacientes com CIV pequena, a fim de determinar continuidade ou não da conduta expectante, em vista do baixo risco operatório, o que ocasiona maior liberalidade da indicação cirúrgica. MÉTODOS: No período de outubro de 1976 a dezembro de 2007, foram examinados 187 casos com CIV pequena (diâmetro menor que 3 mm pelo ecocardiograma) e seguidos 155 deles em longo prazo. Estudaram-se a época de exteriorização do sopro e aspectos evolutivos como fechamento espontâneo do defeito (grupo I) - 64 casos, continuidade do tamanho inicial (grupo II) - 74 casos e diminuição do mesmo (grupo III) -17 casos, além de intercorrências clínicas. RESULTADOS: A exteriorização do sopro ocorreu na maioria no primeiro mês de vida, correspondendo a 48 (75 por cento), 54 (72,9 por cento) e 12 (70,5 por cento) pacientes, nos três grupos respectivos, e acima do primeiro ano em 11 (5,8 por cento) pacientes. Fechamento espontâneo ocorreu no primeiro ano em 48 casos (75 por cento), média de 7,6 m e de 1 a 5,5 anos em 15 pacientes (23,4 por cento), em acompanhamento máximo de 18 anos. Verificou-se continuidade do defeito até 40 anos. A diminuição do defeito ocorreu em média de 15 meses, seguidos até 9 anos. Probabilidade de fechamento da CIV, pela curva actuarial, foi de 34,38 por cento em 1 ano e de 49,89 por cento em 5 anos. Não houve intercorrências clínicas. CONCLUSÃO: Evolução favorável da CIV pequena em longo prazo dispensa intervenção operatória, com preocupação da profilaxia antibiótica rigorosa.

BACKGROUND: The small ventricular septal defect (VSD) usually presents good clinical evolution, even at long-term follow-up. OBJECTIVE: To verify the clinical evolution of patients with small VSD in order to determine the continuation or not of the expectant conduct, considering the low operative risk, which results in a more liberal indication for surgery. METHODS: From October 1976 to December 2007, 187 cases of small VSD (diameter < 3 mm at the echocardiogram) were evaluated and 155 of them were assessed at long-term follow-up. Time of the clinical manifestation of the murmur and evolution aspects such as the spontaneous closure of the defect (group I) - 64 cases, persistence of the initial size (group II - 74 cases and decrease in the size of the defect (group III) - 17 cases, in addition to clinical complications, were studied. RESULTS: The clinical manifestation of the murmur occurred, in the majority of cases, during the first month of life, corresponding to 48 (75 percent), 54 (72.9 percent) and 12 (70.5 percent) patients, in the three groups, respectively and after the first year of life in 11 (5.8 percent) patients. Spontaneous closure occurred in the first year of life in 48 cases (75 percent), mean of 7.6 months and from 1 to 5.5 years in 15 patients (23.4 percent), with a maximum follow-up of 18 years. The persistence of the defect until 40 years of age was observed. The decrease in the size of the defect occurred on a mean of 15 months, followed for up to 9 years. The probability of VSD closure by the actuarial curve was 34.38 percent in 1 year and 49.89 percent in 5 years. There were no clinical complications. CONLCUSION: A favorable evolution of the small VSD at long-term follow-up does not require surgical intervention, with concerns regarding the strict antibiotic prophylaxis.

FUNDAMENTO: La comunicación interventricular (CIV) pequeña presenta generalmente buena evolución clínica, aun a largo plazo. OBJETIVO: Verificar la evolución clínica de pacientes con CIV pequeña, a fin de determinar continuidad o no de la conducta expectante, en vista del bajo riesgo operatorio, resultando una mayor liberalidad de la indicación quirúrgica. MÉTODOS: En el período de octubre de 1976 a diciembre de 2007, se examinaron 187 casos con CIV pequeña (diámetro menor que 3 mm por el ecocardiograma) y se siguieron a 155 de ellos a largo plazo. Se estudiaron la época de exteriorización del soplo y los aspectos evolutivos, como cierre espontáneo del defecto (grupo I): 64 casos; continuidad del tamaño inicial (grupo II): 74 casos; y disminución del mismo (grupo III):17 casos; además de intercurrencias clínicas. RESULTADOS: En la mayoría de los casos, la exteriorización del soplo tuvo lugar en el primer mes de vida, correspondiendo a 48 (75 por ciento), 54 (72,9 por ciento) y 12 (70,5 por ciento) pacientes, en los tres grupos respectivos, y después del primer año en 11 (5,8 por ciento) pacientes. Cierre espontáneo ocurrió en el primer año en 48 casos (75 por ciento), promedio de 7,6 m y de 1 a 5,5 años en 15 pacientes (23,4 por ciento), en seguimiento máximo de 18 años. Se verificó continuidad del defecto hasta 40 años. La disminución del defecto ocurrió en promedio de 15 meses, seguidos hasta 9 años. Probabilidad de cierre de la CIV, por la curva actuarial, fue de un 34,38 por ciento en 1 año y de un 49,89 por ciento en 5 años. No hubo intercurrencias clínicas. CONCLUSIÓN: Evolución favorable de la CIV pequeña a largo plazo dispensa intervención operatoria, con preocupación de la profilaxis antibiótica rigurosa.

Endocarditis verrucosa secundaria a saccharomyces cerevisiae: caso clínico / Saccharomyces cerevisiae endocarditis in a preterm infant: report of one case

We report a preterm infant with 30 weeks of gestation, that received broad spectrum antimicrobials during the first days of life. At nine days of life, the infant appeared with abdominal distension and hematochezia. A systolic murmur with changing auscultatory features also appeared. An echocardiography showed an atrial vegetation. A yeast, that was identified as the emergent pathogen Saccharomyces cerevisiae appeared in two blood cultures. Treatment with amphotericin B was started, the dose was adjusted calculating the minimal inhibitory concentration of amphotericin B, and measuring plasma levels of the antimicrobial. Therefore the minimal effective dose was prescribed, avoiding its deleterious effects. After 14 days of antifungal therapy, a new echocardiography showed a reduction in the size of the atrial vegetation. At 35 days, it disappeared and amphotericin B was discontinued. On the outpatient follow up, the infant has shown a normal growth and a normal cardiac auscultation

Tumores cardíacos y pericárdicos en el niño: revisión de 15 años / Cardiac and pericardial tumors in children: review of 15 years

Rvisión de 14 casos de tumores cardiacos y pericárdicos en niños diagnosticados desde 1985 hasta 1999, en el Servicio de Cardiovascular del Hospital Luis Calvo Mackenna. Objetivo: evaluar el tipo de tumor, forma de presentación y pronóstico. El diagnóstico es mediante la ecocardiografía. Los tumores fueron rabdomiomas en 71 por ciento, mixoma, lipoma, teratoma quístico y sarcoma de Ewing. Edad de presentación: período antenatal en 3 pacientes y hasta 15 años; 50 por ciento en menores de 3 meses. Los síntomas má frecuentes fueron soplos cardiacos y arritmias. La mayoría de los rabdomiomas eran múltiples, ubicados en las distintas cavidades cardiacas. Un tercio de ellos se asoció con esclerosis tuberosa. El pronóstico dependió del tipo de tumor y de su ubicación. Fallecieron dos pacientes, un niño con sarcoma de Ewing y otro con rabdomioma intramural con foco arritmogénico ventricular

Defeito do septo interventricular adquirido no curso de endocardite infecciosa / Acquired ventricular septal defect during infective endocarditis

We studied five patients with acquired ventricular septal defect during the course of an infective endocarditis. All patients were male and had a previous aortic valve disease associated with an aortic ring abscess. Clinical examination was useful for the diagnosis of 4 cases, emphasizing the following findings: systolic murmur and/or left paraesternal thrill and right-heart failure. Incidence of congestive heart failure was 60//, while 40//of all patients died during the in-hospital course. No specific etiologic agent was detected. Therefore, acquired ventricular septal defect should be suspected in patients with infective endocarditis of the aortic valve that evolve with a systolic murmur and/or paraesternal thrill and right-heart failure. Immediate surgical treatment is mandatory in these cases

Etiology of isolated mitral regurgitation: a clinico-echocardiographic study.

One hundred and ninety two consecutive patients with isolated mitral regurgitation (MR) with an audible murmur were studied for determination of etiology. There were 95 males & 97 females (mean age 24.5 +/- 10.8 years; range 13 to 58 years) with 102 patients in NYHA classes I and II, 81 in class III and 9 in NYHA class IV. The etiological grouping was: rheumatic 74 (38.5%), probable rheumatic 28 (15%), mitral valve prolapse 26 (13.5%), dilated cardiomyopathy 15 (8%), infective endocarditis 12 (6%), isochaemic heart disease 10 (5%), miscellaneous group (including rupture chordae tendinae, aortoarteritis etc) 9 (4.5%) and patients with indeterminate etiology 18 (9.5%). Etiology could be determined in 174 out of 192 cases. The clinical methods combined with echocardiography were helpful in 79 cases while echocardiography alone could diagnose etiology in 89 cases. Clinical features alone gave the diagnosis in 6 patients. The findings of gross morphology of the surgically removed mitral valves in 30 patients of this study were similar to their pre-operative etiologic diagnosis based on clinical and echocardiographic features. These findings may be of value in planning treatment and prophylactic strategies in cases of isolated MR.

Double orifice mitral valve: report of 4 cases.

Double Orifice Mitral Valve (DOMV) is a rare congenital abnormality. Reports of 4 patients with DOMV are presented. As associated anomalies, one had ostium primum atrial septal defect (ASD). Second one had Ebstein's anomaly. Third one had grade IV tricuspid regurgitation, thickened tricuspid valve and right atrial mass. Fourth one had sinus venosus ASD. Echocardiography is the gold standard investigation. Surgical treatment depends upon the mitral valve function and associated anomalies.

Electrocardiographic demostration of dual atrioventricular nodal pathways in children

Dual atrioventricular nodal, pathaways, as evaluated by electrocardiography, were found in two children without arrhythmias over a six year period. Although none have developed tachycardia, they have the substrate to develop atrioventricular reentrant tachycardia

Lobar agenesis of the left upper lung: a case report

Lung agensis is a rare developmental anomaly. It can range from total bronchial and parechymal agensis to mild pulmonary parenchymal hypoplasia of one or both lungs. A case of lobar agenesis of the left upper lung in a 15-year-old boy is presented. The patient had mild exertional dyspnea. Pulmonary angiography revealed the absence of the left upper pulmonary artery and vein. Bronchography showed no branching of bronchus to the left upper lobe. Intravenous pyelography revealed incomplete duplication of the right urinary tract.