Association of right ventricle diastolic diameter with pulmonary function, exercise tolerance and exacerbation period in patients with chronic obstructive pulmonary disease: A prospective study.

BACKGROUND: The echocardiogram is a useful method for monitoring the cardiovascular complications of chronic obstructive pulmonary disease (COPD), but it is not clear if morphological and functional cardiac variables are able to identify patients with unfavorable COPD progression. OBJECTIVE: The aim of this study was to investigate morphological and functional echocardiographic variables associated with exacerbation of the disease, pulmonary function parameters and functional capacity evaluation in COPD patients. METHOD: A longitudinal observational study including 91 patients diagnosed with COPD, who were stratified in two groups according to their basal right ventricle diastolic diameter (RVDD) measuring greater and less than 35 mm. They underwent clinical evaluation, echocardiography, spirometry and 6-minute walking-test (6MWT). RESULTS: The study found that patients with RVDD greater than 35 mm showed worse functional capacity in the 6MWT (p = 0.05) and more exacerbation of the disease during the one-year follow-up (p = 0.05). There were correlations of greater RVDD vs forced vital capacity (R = -0.27; p = 0.02) and distance in the 6MWT (R = 0.55; p = 0.03) in patients with exacerbation. In the univariate regression analysis, the RVDD was responsible for 55% of the variation in distance in the 6MWT (-0.75 m) in COPD patients. CONCLUSION: The RV dilation is associated with unfavorable prognostic markers in COPD, such as the worsening of the functional capacity and more frequent exacerbations. The echocardiogram may be a useful tool to identify patients who need more aggressive strategies to control the evolution of the disease with potential impact in pulmonary rehabilitation.

Relationship Between Postnatal Pulmonary Arterial Pressure and Altered Diastolic Function in Neonates with Down Syndrome.

OBJECTIVE: To assess the influence of diastolic dysfunction on the evolution of pulmonary hypertension in neonates with Down Syndrome over the early newborn period. STUDY DESIGN: This was a prospective observational cohort study. Echocardiography was performed three times over the first week of life in both Down syndrome and control cohorts. Measurements of pulmonary arterial pressure in addition to left ventricular (LV) and right ventricular systolic and diastolic function were collected. RESULTS: Seventy babies with Down syndrome and 60 control infants were enrolled. Forty-eight of the infants with Down syndrome (69%) were born with congenital heart disease (CHD). Echocardiography surrogates of pulmonary hypertension and myocardial function remained significantly impaired in the Down syndrome group in comparison with control infants (all P < .01). In the Down syndrome group, LV early diastolic strain rate was independently associated with measures of pulmonary hypertension while controlling for gestational age, cesarean delivery, and the presence of CHD (P < .01). CONCLUSIONS: Intrinsic LV diastolic impairment is directly associated with higher indices of pulmonary hypertension in infants with Down syndrome and may be a contributing factor to its evolution.

Association between diastolic stress test and H2FPEF score. / Asociación entre prueba de estrés diastólico y puntuación H2FPEF

OBJECTIVE: Heart failure with preserved ejection fraction is a highly prevalent disease; some advances for improving the diagnosis are the development of the H2FPEF score and the diastolic stress test for the evaluation of diastolic function. The objective is to describe the clinical and echocardiographic characteristics of patients referred for stress tests, and the association between the H2FPEF score and the results of the diastolic test. METHODS: This is an analytical, observational, retrospective study. An exercise stress test was performed. The Chi-square test was used to establish an association between H2FpEF score and diastolic stress test results. Patients over the age of 18, in sinus rhythm, with the left ventricular ejection fraction > 54%, with no more than mild diastolic dysfunction on the baseline echocardiogram were included in the study. RESULTS: A total of 99 patients met the eligibility criteria. About 49.5% were women, median age was 62.2 years. The H2FPEF score was low in 27.2%, intermediate 71.7%, and 1% in the high range. There was a high prevalence of hypertension 58.6%, diabetes 12.1%, and coronary disease 20.2%. The stress test was positive for diastolic dysfunction in 36.4% of the patients. A statistically significant association was found between the H2FPEF score and the diastolic stress test (p = 0.02). CONCLUSIONS: Although clinical scores such as H2FPEF help identify patients, a high percentage of patients are classified in the intermediate range. The diastolic stress test can help to make the diagnosis of diastolic function in this group of patients.

OBJETIVO: La insuficiencia cardíaca con fracción de eyección preservada (FCFEp) es una enfermedad de alta prevalencia, sin embargo, aún falta una estrategia de referencia para establecer el diagnóstico de FCFEp. Algunos de los avances más importantes para mejorar el diagnóstico de FCFEp son la puntuación H2FPEF y la prueba de esfuerzo para la evaluación de la función diastólica durante el ejercicio. El objetivo es describir las características clínicas y ecocardiográficas de los pacientes remitidos para pruebas de esfuerzo en un centro de referencia colombiano, y la asociación entre el puntaje H2FPEF con los resultados de la prueba de estrés diastólica. MÉTODOS: Se trata de un estudio analítico, observacional y retrospectivo. Se realizó una ecocardiografía de esfuerzo solicitada por disnea o fatiga. La prueba Chi cuadrado se utilizó para establecer una asociación entre la puntuación H2FpEF y los resultados de la prueba diastólica. Se incluyeron pacientes mayores de 18 años, en ritmo sinusal, con fracción de eyección del ventrículo izquierdo igual o superior al 55%, con una disfunción diastólica no mayor de leve en el ecocardiograma basal. RESULTADOS: Un total de 99 pacientes cumplieron los criterios de inclusión. El 49,5% de la población eran mujeres, la edad media fue de 62.2 años. La puntuación H2FPEF fue baja en 27.2%, intermedia 71,7% y 1% en el rango alto. Hubo una alta prevalencia de hipertensión 58.6%, diabetes 12.1% y enfermedad coronaria 20.2%. La prueba de esfuerzo fue positiva para disfunción diastólica en el 36,4% de los pacientes. Se encontró una asociación estadísticamente significativa entre la puntuación H2FPEF y la prueba de esfuerzo diastólico (p = 0.02). CONCLUSIONES: Aunque puntuaciones clínicas como H2FPEF ayudan a identificar a los pacientes, un alto porcentaje de pacientes se clasifican en el rango intermedio. La prueba de esfuerzo diastólico puede ayudar a realizar el diagnóstico de función diastólica en este grupo de pacientes.

Alternative treatment for patent ductus arteriosus: a therapeutic challenge / Tratamento alternativo do canal arterial pervio: um desafio terapêutico

Abstract The ductus arteriosus is a fetal structure that spontaneously closes in 90% of newborns. Patency 3 months after birth is considered a congenital heart disease that, if untreated, can progress to serious cardiovascular complications. This report aims to review an alternative treatment with an aortic endoprosthesis in a 49-year-old man who presented with dyspnea on moderate exertion associated with a heart murmur. He was diagnosed with persistent ductus arteriosus (PDA) with cardiac complications. Clinical management was unsuccessful and surgical treatment was indicated. Endovascular treatment with a thoracic endoprosthesis was indicated and performed successfully. Percutaneous closure is the preferred method in adult patients. Endovascular intervention using an endoprosthesis is a safe and effective option, in addition to being applicable regardless of the anatomy of the PDA. This case demonstrates the natural history of the pathology and presents a safe and effective alternative for its management.

Resumo O canal arterial é uma estrutura fetal que se fecha espontaneamente em 90% dos recém-nascidos. A permeabilidade por mais de 3 meses é considerada uma cardiopatia congênita que, se não tratada, pode evoluir para complicações cardíacas graves. Este trabalho relata um tratamento alternativo com endoprótese aórtica. Trata-se de um homem, 49 anos, que apresentou dispneia aos moderados esforços, associada com um sopro cardíaco. Foi diagnosticado com canal arterial persistente (PCA) já com complicações cardíacas. Tentou-se manejo clínico sem sucesso, sendo indicado tratamento cirúrgico. Optou-se pelo tratamento endovascular com uma endoprótese torácica, o qual foi realizado com sucesso. O fechamento percutâneo é o método preferido em pacientes adultos. A intervenção endovascular com o uso da endoprótese é uma possibilidade segura e eficaz, além de ser aplicável independentemente da anatomia do PCA. Este caso, além de demonstrar a história natural desta patologia, apresenta uma alternativa segura e eficaz empregada no seu manejo.

Sobrevida e características ecocardiográficas de gatos com e sem cardiomiopatias / Survival and echocardiographic characteristics of cats with and without cardiomyopathies

Cardiopatias felinas apresentam importante relevância na rotina veterinária, todavia os seus aspectos epidemiológicos em gatos não são conhecidos regionalmente. O objetivo do estudo foi descrever a frequência das cardiopatias na região Norte e Vale do Itajaí no estado de Santa Catarina e determinar a sobrevida de pacientes cardiopatas e não cardiopatas. O estudo contou com a análise retrospectiva de 74 laudos ecocardiográficos e prontuários médicos de gatos oriundos de estabelecimentos veterinários da região, de janeiro de 2017 a dezembro de 2019. Tutores ou veterinários responsáveis foram contatados para averiguar a sobrevida dos animais. Os animais do estudo em sua maioria eram machos (n=40/74) e sem raça definida (n=47/74). Cardiomiopatia foi o diagnóstico mais comum (n=21/74), com destaque para o fenótipo hipertrófica (n=13/21). As cardiomiopatias foram diagnosticadas mais comumente em gatos acima de oito anos de idade. Os principais sinais clínicos nos gatos sintomáticos (n=41/74) foram sopro (n=15/41) e dispneia (n=6/41). Os principais achados ecocardiográficos foram hipertrofia concêntrica da parede livre do ventrículo esquerdo (n=18/41) e dilatação do átrio esquerdo (n=12/41). A mediana de sobrevida dos 74 gatos foi de 303±209.8 dias, estando altamente relacionado com a classe do estadiamento clínico (P=0,006). Gatos com fenótipo dilatada tiveram menor média de sobrevida (180.5 dias). As doenças concomitantes mais observadas foram doença renal crônica (n=7/15), hipertensão (n=5/15) e/ou hipertireoidismo (n=3/15). Gatos com cardiomiopatias, sintomáticos e com estágios mais avançados de remodelamento cardíaco, demostraram viver menos se comparados com aqueles em estágio inicial da cardiopatia. Bem como pacientes com doenças de base associada apresentaram menor expectativa de vida.

Feline cardiopathies are relevant on veterinary practice although lack of regional epidemiogical description. The purpose of this study is to determine cardiopathy prevalence and survival of cardiac and non-cardiac patients on Santa Catarina's northern and Itajai valley regions. The retrospective study included 74 echocardiographic exams and medical records from January 2017 to December 2019. Veterinarians and owners were contacted to check survival on cardiac and non-cardiac patients. The animals were most male (n=40/74) and mongrel (n=47/74). Cardiomyopathies were the most common diagnosis (n=21/74), specially the hypertrophic phenotype (n=13/21). The cardiomyopathy diagnosis was evidenced in cats above eight years old. The most usual clinical findings on symptomatic patients (n=41/74) were cardiac murmur (n=15/41) and dyspnea (n=6/41). Left ventricular free wall concentric hypertrophy (n=18/41) and left atrium dilation (n=12/41) were the main echocardiographic findings. Median survival from 74 cats was 303±209,8 days and related to clinical staging (p=0,006). Cats with dilated cardiomyopathy phenotype presented lower mean survival (180,5 days). Concomitant diseases included chronic renal disease (n=7/15), systemic arterial hypertension (n=5/15) and/or hyperthyroidism (n=3/15). In conclusion, cats with cardiomyopathies, symptomatic and with more advanced stages of cardiac remodeling, have shown to live less than those in early stage of heart disease. As well as patients with associated concomitant diseases, they had a lower life expectancy.

Bridging the Cardiac Needs of a Large, Underserved Immigrant and Resettled Refugee Population.

OBJECTIVE: To describe a monthly outreach pediatric cardiology clinic established to better understand the cardiac needs of immigrant/resettled refugee children. STUDY DESIGN: Data obtained between 2014 and 2017 from a monthly pediatric cardiology clinic at a Federally Qualified Health Center were analyzed using descriptive statistics. RESULTS: A total of 366 patients (222 male, 61%) were evaluated. Indications for referral included murmur (242, 66%), nonexertional symptoms (31, 9%), exertional symptoms (16, 4%), history of cardiac surgery/transcatheter interventions (15, 4%), previous diagnosis of heart conditions without intervention (13, 4%), arrhythmia/bradycardia (13, 4%), and others (36, 10%). Echocardiograms were performed on 136 patients (67 were abnormal, 49%). The most common final diagnoses include innocent murmur in 201 (55%), simple congenital heart disease in 61 (16%), complex congenital heart disease in 3 (1%), and acquired heart disease in 3 (1%). A total of 15 patients (4%) were ultimately determined to require surgical or cardiac catherization as an intervention. Patients have been followed for a median of 0.7 years (range 0-3.3 years). CONCLUSIONS: Rates of abnormal echocardiograms suggest a greater likelihood of congenital or acquired heart disease at time of initial consultation compared with nonimmigrant/refugee populations. The most common indication for referral to the outreach pediatric cardiology clinic was a murmur. Collaborative efforts between physicians and support services are essential in assisting this vulnerable population access pediatric subspecialty care.

Del soplo inocente a la hipertensión arterial pulmonar / From an innocent heart murmur to pulmonary arterial hypertension

Resumen Introducción: La detección de cardiopatías congénitas en la etapa neonatal a partir de un soplo cardiaco o cianosis no es efectiva. Las cardiopatías congénitas críticas, como el tronco arterioso común (TAC), causan la mayoría de las muertes neonatales por malformaciones congénitas. El tamizaje por oximetría de pulso en los recién nacidos detecta hasta el 70% de estas cardiopatías. El TAC presenta una alta mortalidad en el primer año de vida. Caso clínico: Se presenta el caso de un paciente de sexo femenino de 4 años de edad con soplo cardiaco, palpitaciones, disnea y cianosis perioral, con diagnóstico al nacimiento de soplo inocente. Se detectó TAC mediante una ecocardiografía. Las resistencias vasculares pulmonares fueron evaluadas por medio de cateterismo cardiaco derecho, con hallazgo de hipertensión arterial pulmonar y vasorreactividad pulmonar. Se realizó corrección quirúrgica. A la fecha, la hipertensión arterial pulmonar continúa presente, por lo que se implementó Bosentan® (Actelion, USA) como tratamiento a largo plazo. Conclusiones: En recién nacidos, el tamizaje por oximetría de pulso después de las 24 horas de vida es un método efectivo para el diagnóstico oportuno de cardiopatías congénitas críticas antes de los signos de colapso cardiovascular. Por ello, resulta una herramienta diagnóstica fundamental para reducir la morbimortalidad. Aunque la corrección quirúrgica de cardiopatías congénitas con hipertensión arterial pulmonar es factible en algunos pacientes, su manejo subsecuente es complejo e impacta de manera adversa en la calidad de vida.

Abstract Background: The detection of congenital heart disease in newborns, based on a heart murmur or cyanosis is not effective. Critical congenital heart diseases, such as truncus arteriosus (TA), cause most of neonatal deaths due to congenital malformations. The screening for pulse oximetry in newborns detects up to 70% of these heart diseases. TA presents high mortality in the first year of life. Case report: A 4-year-old female patient with a heart murmur, palpitations, dyspnea, and perioral cyanosis was diagnosed with an innocent heart murmur at birth. TA was detected by echocardiography. Pulmonary vascular resistances were evaluated through right cardiac catheterization, and pulmonary arterial hypertension and pulmonary vasoreactivity were diagnosed as well. Surgical correction was performed. Currently, pulmonary arterial hypertension persists, for which Bosentan® (Actelion, USA) has been implemented as a long-term treatment. Conclusions: In newborns, the pulse oximetry screening after 24 hours of life is an effective method for suitable diagnosis of critical congenital heart disease before the signs of cardiovascular collapse. Therefore, it has become an essential diagnostic tool to reduce morbidity and mortality. Although the surgical correction of congenital heart disease with pulmonary arterial hypertension is feasible in some patients, its subsequent management is complex and has an adverse impact on the quality of life.

From an innocent heart murmur to pulmonary arterial hypertension.

Background: The detection of congenital heart disease in newborns, based on a heart murmur or cyanosis is not effective. Critical congenital heart diseases, such as truncus arteriosus (TA), cause most of neonatal deaths due to congenital malformations. The screening for pulse oximetry in newborns detects up to 70% of these heart diseases. TA presents high mortality in the first year of life. Case report: A 4-year-old female patient with a heart murmur, palpitations, dyspnea, and perioral cyanosis was diagnosed with an innocent heart murmur at birth. TA was detected by echocardiography. Pulmonary vascular resistances were evaluated through right cardiac catheterization, and pulmonary arterial hypertension and pulmonary vasoreactivity were diagnosed as well. Surgical correction was performed. Currently, pulmonary arterial hypertension persists, for which Bosentan® (Actelion, USA) has been implemented as a long-term treatment. Conclusions: In newborns, the pulse oximetry screening after 24 hours of life is an effective method for suitable diagnosis of critical congenital heart disease before the signs of cardiovascular collapse. Therefore, it has become an essential diagnostic tool to reduce morbidity and mortality. Although the surgical correction of congenital heart disease with pulmonary arterial hypertension is feasible in some patients, its subsequent management is complex and has an adverse impact on the quality of life.

Introducción: La detección de cardiopatías congénitas en la etapa neonatal a partir de un soplo cardiaco o cianosis no es efectiva. Las cardiopatías congénitas críticas, como el tronco arterioso común (TAC), causan la mayoría de las muertes neonatales por malformaciones congénitas. El tamizaje por oximetría de pulso en los recién nacidos detecta hasta el 70% de estas cardiopatías. El TAC presenta una alta mortalidad en el primer año de vida. Caso clínico: Se presenta el caso de un paciente de sexo femenino de 4 años de edad con soplo cardiaco, palpitaciones, disnea y cianosis perioral, con diagnóstico al nacimiento de soplo inocente. Se detectó TAC mediante una ecocardiografía. Las resistencias vasculares pulmonares fueron evaluadas por medio de cateterismo cardiaco derecho, con hallazgo de hipertensión arterial pulmonar y vasorreactividad pulmonar. Se realizó corrección quirúrgica. A la fecha, la hipertensión arterial pulmonar continúa presente, por lo que se implementó Bosentan® (Actelion, USA) como tratamiento a largo plazo. Conclusiones: En recién nacidos, el tamizaje por oximetría de pulso después de las 24 horas de vida es un método efectivo para el diagnóstico oportuno de cardiopatías congénitas críticas antes de los signos de colapso cardiovascular. Por ello, resulta una herramienta diagnóstica fundamental para reducir la morbimortalidad. Aunque la corrección quirúrgica de cardiopatías congénitas con hipertensión arterial pulmonar es factible en algunos pacientes, su manejo subsecuente es complejo e impacta de manera adversa en la calidad de vida.

Consultorio de orientaciòn de cardilogía: epidemiología y perfil de la población / Cardiology guidance clinic: Epidemiology and profile of the population

Introducción: Las cardiopatías congénitas (CC) son las anomalías congénitas más frecuentes. Representan el 0,8-1,2% de todos los defectos del nacimiento y tienen una prevalencia de alrededor de 5,8 por cada 1000 personas. El Servicio de Cardiología del Hospital Garrahan es un centro de referencia nacional y de países limítrofes donde se realizan 18000 consultas anuales. Los pacientes que concurren por primera vez se atienden en el consultorio de orientación. Objetivo: Describir la epidemiologia y perfil de los pacientes que asisten diariamente al consultorio de orientación de cardiología infantil en un hospital pediátrico de tercer nivel de Buenos Aires. Métodos: Entre septiembre de 2017 y febrero de 2018 se recolectaron los datos de 1000 pacientes atendidos en forma consecutiva en el consultorio de orientación de cardiología. A la totalidad de los pacientes se les realizó anamnesis, examen físico cardiovascular, electrocardiograma, y en los casos en los que se consideró necesario, saturometría, radiografía de tórax y/o ecocardiograma. Las variables a considerar fueron edad, procedencia, presencia o ausencia de cardiopatías congénitas o adquiridas, soplo, cianosis, insuficiencia cardíaca, estado nutricional, síndromes genéticos asociados, métodos diagnósticos e indicaciones terapéuticas implementadas. Se subdividió la población en cinco grupos: Grupo A (pacientes con cardiopatía congénita), Grupo B (cardiopatías operadas), Grupo C (miocardiopatías), Grupo D (arritmias), Grupo E (corazón sano). Resultados: La edad mediana fue 4.86 años (0.03 a 18.9 años). El 64% de los pacientes procedían de la provincia de Buenos Aires. Los motivos de consulta fueron: interconsultas internas 29.5%, derivación por cardiopatía 27.2%, soplo 17.6%, síncope 7%, segunda opinión 5.1%, arritmias 4.8%, precordialgia 3.1%, palpitaciones 2.6%, episodio paroxístico 1.4%, cardiomegalia 0.7%, disnea 0.5%, mal progreso de peso 0.3%. El 10.6% tenían un síndrome genético. Grupo A: 252 pacientes con una edad mediana de 1.9 años. Las cardiopatías acianóticas con hiperflujo pulmonar fueron las más frecuentes (66.66%, 168/252). Grupo B: 51 pacientes, 23.52%(12/51) fueron Fallot reparados en otra institución. Grupo C: 22 pacientes, siendo la miocardiopatía hipertrófica la más frecuente. Grupo D: 47 pacientes, la preexcitación ventricular fue el hallazgo más frecuente (34,04%, 16/47). Grupo E: 628 pacientes, 45.70% (287/628) derivados por pediatras del área ambulatoria, principalmente para valoración de pacientes con enfermedades sistémicas o síndromes genéticos. Conclusión: Los motivos de derivación al consultorio de orientación de cardiología fueron muy diversos. La mayoría de los pacientes provenían de provincia de Buenos Aires. Solamente el 37.2% presentó algún problema cardiológico de base. El 91% de los pacientes que consultaron por soplo, no tuvieron cardiopatía. El grupo correspondiente a los pacientes con cardiopatías no operadas (grupo A) fue el de menor edad (mediana de 1.9 años) y las cardiopatías simples no cianóticas con hiperflujo pulmonar representaron el 66.66% de las cardiopatías. La implementación del ecocardiograma portátil en el consultorio de orientación permitió confirmar el diagnóstico y definir la conducta terapéutica en el 29.4% de los pacientes durante la primer consulta (AU)

Introduction: Congenital heart defects (CHD) are the most common congenital abnormalities. They account for 0.8-1.2% of all birth defects and have a prevalence of around 5.8 per 1000 people. The Department of Cardiology of Garrahan Hospital is a national and bordering-country reference center, receiving 18000 consultations annually. Patients seen for the first time are assessed at the cardiology guidance clinic. Objective: To describe the epidemiology and profile of patients who seen daily at the child cardiology guidance clinic of a third-level pediatric hospital in Buenos Aires. Methods: Between September 2017 and February 2018, data from 1000 patients consecutively seen at the cardiology guidance clinic were collected. All patients underwent anamnesis, cardiovascular physical examination, electrocardiogram and, if considered necessary, pulse oximetry, chest x-ray, and/or echocardiogram. The variables considered were age, place of origin, presence or absence of congenital or acquired heart disease, murmur, cyanosis, heart failure, nutritional status, associated genetic syndromes, diagnostic methods, and treatment. The population was divided into five groups: Group A (patients with congenital heart defects), Group B (operated cardiopathies), Group C (myocardiopathies), Group D (arrhythmias), Group E (healthy heart). Results: Median age was 4.86 years (0.03 to 18.9 years). Overall, 64% of patients came from the province of Buenos Aires. The reasons for consultation were: internal consultations 29.5%, cardiac shunt 27.2%, murmur 17.6%, syncope 7%, second opinion 5.1%, arrhythmias 4.8%, precordialgia 3.1%, palpitations 2.6%, paroxysmal episode 1.4%, cardiomegaly 0.7%, dyspnea 0.5%, 0.3% poor weight gain. A genetic syndrome was identified in 10.6%. Group A: 252 patients with a median age of 1.9 years. Acyanotic congenital heart defect with pulmonary hyperflow was the most common (66.66%, 168/252). Group B: 51 patients, 23.52% (12/51) had tetralogy of Fallot repaired at another institution. Group C: 22 patients, in whom hypertrophic cardiomyopathy was the most common. Group D: 47 patients, in whom ventricular preexcitation was the most common finding (34.04%, 16/47). Group E: 628 patients, 45.70% (287/628) referred by pediatricians from the outpatient clinics, mainly for the assessment of systemic diseases or genetic syndromes. Conclusion: The reasons for referral to the cardiology guidance clinic were varied. Most of the patients came from the province of Buenos Aires. Only 37.2% had an underlying heart disease. Of the patients who consulted because of a murmur, 91% did not suffer from heart disease. The group of patients with congenital heart disease who had not undergone surgery (group A) was the youngest (median 1.9 years) and simple non-cyanotic heart disease with pulmonary hyperflow accounted for 66.66% of heart diseases. The implementation of the portable echocardiography in the guidance clinic confirmed the diagnosis and defined the management in 29.4% of patients during the first consultation (AU)

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.

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

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.

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.

Divertículo congênito de ventrículo direito associado à Tetralogia de Fallot / Congenital right ventricular diverticulum associated with Tetralogy of Fallot

APRESENTAÇÃO DO CASO: Paciente de 45 dias de vida admitido com diagnóstico de Tetralogia de Fallot (T4F). Mãe referia sopro cardíaco auscultado desde o 1° dia de vida e, atualmente, cansaço, cianose às mamadas e perda de fôlego após choro. Ao exame físico, apresentava cianose leve, dispneia e hepatomegalia, além de sopro sistólico 1+/4+ na BEE alta, com segunda bulha única e normofonética. O ecocardiograma transtorácico evidenciou, além dos achados próprios da T4F, uma câmara acessória conectada ao ventrículo direito (VD) através de ampla comunicação, mais anterior, muscularizada e com boa função contrátil, podendo corresponder a divertículo. Para complementação diagnóstica foi realizada ressonância magnética de coração, que corroborou com diagnóstico do ecocardiograma. Caso discutido com equipe, sendo optado por correção cirúrgica da Tetralogia de Fallot, sem abordagem do divertículo ventricular. DISCUSSÃO: O divertículo ventricular congênito é uma patologia rara, com incidência de cerca de 0.013%. É mais comumente encontrado no ventrículo esquerdo e associado a mal-formações extracardíacas da linha média. Quando presente no VD, é ainda mais raro, podendo estar associado a mal-formações congênitas intracardíacas cianogênicas, como a T4F. Sua origem ainda é desconhecida, porém acredita-se que tenha relação com os altos níveis pressóricos no VD causado por obstruções de sua via de saída ou com o aumento de fluxo ventricular ocasionado pela comunicação interventricular. Possui miocárdio em sua parede, contraindo sincronicamente com o ventrículo, sendo denominado também como câmara acessória. Seu diagnóstico pode ser realizado pelo ecocardiograma, podendo ser complementado com outros métodos de imagem. Não há abordagem cirúrgica definida, podendo ou não ser ressecado, dependendo da sua localização, conexões anatômicas e sua contribuição em relação a função contrátil do miocárdio ventricular remanescente. Apesar de raras, possíveis complicações incluem formação de trombo, insuficiência cardíaca e ruptura ventricular. CONSIDERAÇÕES FINAIS: O divertículo do VD é uma patologia extremamente rara, porém de fácil suspeita diagnóstica pelo ecocardiograma. O seu tratamento deve ser considerado caso a caso, dependendo principalmente da importância do divertículo na função ventricular. No caso descrito, o diagnóstico foi primeiramente realizado pelo ecocardiograma e, devido boa função contrátil diverticular, optado por apenas correção da cardiopatia de base. (AU)