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.

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.

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)

Unruptured right sinus of Valsalva aneurysm in a Maltese dog: a case report

A rare case of an unruptured sinus of Valsalva aneurysm (SVA) in a 2-month-old male Maltese terrier weighing 1.0 kg with a heart murmur is presented. A right SVA and a ventricular septal defect (VSD) were diagnosed by echocardiography and cardiac catheterization. The dog died due to a worsening of his condition. The necropsy revealed the sinus of Valsalva to have a diameter of 7 mm and a VSD hole was on the opposite surface. This report is the first to describe an unruptured SVA in the right coronary cusp of a small dog.

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.

Open Surgical Repair Using the Femoral Vein for a Mycotic Superior Mesenteric Artery Aneurysm

Superior mesenteric artery (SMA) aneurysms are rare and often fatal. A 72-year-old man had previously been admitted to the emergency room with epigastric pain and heart murmur. The echocardiographic diagnosis was vegetation on the aortic and mitral valves, with moderate regurgitation from both valves due to infective endocarditis. No aneurysm was detected on abdominal computed tomography, and emergency double-valve replacement was performed. On postoperative day 25, the patient experienced abrupt abdominal pain, and computed tomography revealed a mycotic SMA aneurysm. Open surgical repair of the SMA aneurysm was performed using the femoral vein, and the patient's postoperative course was uneventful.

Aneurisma del seno de Valsalva roto con fístula a ventrículo derecho. Presentación de un caso clínico y revisión de la literatura / Aneurisma do seio de Valsalva roto com fístula para ventrículo direito. Apresentação de um relato de caso e revisão da literatura / Valsalva sinus aneurysm ruptured with right ventricular fistula. Presentation of a clinical case and review of the literature

La presencia de un aneurisma de seno de Valsalva es bastante rara. Esta condición anatómica puede terminar en la ruptura y fistulización a diferentes estructuras cardíacas, con una posible evolución clínica catastrófica. El diagnóstico de esta enfermedad puede resultar laborioso; sin embargo, un dato clínico importante es la presencia de un soplo continuo, que aunque no es específico de esta patología, orienta en el abordaje y detección de posibles causas. La conjunción de la clínica, los hallazgos ecocardiográficos y hemodinámicos, mediante angiocardiografía, resulta primordial en la adecuada caracterización de esta anomalía. De acuerdo a su historia natural, todos los pacientes deben ser tratados quirúrgicamente.

The presence of a Valsalva sinus aneurysm is quite rare. This anatomical condition can end in the rupture and fistulization to different cardiac structures, with a possible catastrophic clinical evolution. The diagnosis of this disease can be laborious; however, an important clinical finding is the presence of a continuous murmur, which although not specific to this pathology, guides in the approach and detection of possible causes. The conjunction of clinic, echocardiographic and hemodynamic findings, through angiocardiography, is primordial in the adequate characterization of this anomaly. According to their natural history, all patients should be treated surgically.

A presença de um aneurisma do seio de Valsalva é muito rara. Esta condição anatômica pode resultar em ruptura e fistulización de diferentes estruturas cardíacas, com uma possível evolução clínica catastrófica. O diagnóstico desta doença pode ser trabalhoso; no entanto, um importante achado clínico é a presença de um sopro contínuo, que embora não seja específico para esta doença, orientada na abordagem e detecção de possíveis causas. A combinação de achados clínicos, ecocardiográficos e hemodinâmicos por angiografia é fundamental na caracterização adequada desta anomalia. De acordo com a sua história natural, todos os pacientes devem ser tratados cirurgicamente.

Ecocardiograma: da Solicitação do Exame pelo Pediatra à Realização pelo Cardiologista Pediátrico / Echocardiography: The Examination Request by the Pediatriciant Achieving the Pediatric Cardiologist

Fundamento: O ecocardiograma é uma ferramenta diagnóstica utilizada para avaliar anomalias cardíacas e esclarecer dúvidas quanto ao caráter benigno de certos achados do exame clínico cardiovascular. Objetivos: Identificar os principais motivos da solicitação do ecocardiograma pelo pediatra e avaliar o grau de ansiedade dos familiares gerado por essa conduta. Métodos: Foram incluídos pacientes com idade inferior a 18 anos, sem diagnóstico prévio de cardiopatia e encaminhados para o primeiro exame de ecocardiograma. Os familiares responsáveis pelos pacientes responderam a um questionário para avaliar o grau de ansiedade deles desde o pedido até a realização do ecocardiograma, com pontuação entre 0 e 19. Os dados foram analisados através de porcentagens, desvio padrão e teste T de Student. Resultados: Estudados 30 pacientes com idade média de 4,45 anos. O motivo de encaminhamento mais prevalente foi sopro cardíaco (23 casos), dos quais 70% não tiveram a hipótese de cardiopatia congênita confirmada após o ecocardiograma. A pontuação média no questionário de ansiedade foi 11 ± 6, sendo relacionado significativamente à presença de cardiopatia, com média de 13,0 ± 5,3 vs. 9,3 ± 5,2 no grupo sem a hipótese da mesma (p = 0,007). Conclusão: O achado de sopro foi o principal motivo para solicitação de ecocardiograma pelo pediatra. A confirmação de cardiopatia foi maior nos menores de 1 ano e com achado de sopro. Apesar do grau de ansiedade ter sido maior no grupo dos pacientes que tiveram o diagnóstico de cardiopatia congênita, este não foi desprezível nos familiares no grupo dos pacientes sem cardiopatia

Background: Echocardiography is a diagnostic tool used to evaluate cardiac anomalies and clarify doubts about the benign nature of certain findings of cardiovascular clinical examination. Objectives: To identify the main reasons for the request of echocardiography by pediatrician and evaluate the degree of anxiety of the family generated by this conduct. Methods: We included patients younger than 18 years without previous diagnosis of heart disease and referred for the first echocardiogram. Family members responsible for the patients answered a questionnaire to assess the degree of their anxiety from order to the performance of the echocardiogram, with scores between 0 and 19. Data were analyzed using percentages, standard deviation and Student's t test. Results: Studied 30 patients with a mean age of 4.45 years. The most prevalent reason for referral was heart murmur (23 cases), of which 70% patients had no congenital heart disease hypothesis confirmed, after echocardiography. The average score on the anxiety questionnaire was 11 ± 6 significantly associated with the presence of heart disease, with a mean of 13.0 ± 5.3 vs. 9.3 ± 5.2 in the group without the possibility of it (p = 0.007). Conclusion: The murmurs findings was the main reason for echocardiography request by the pediatrician. Confirmation of heart disease was higher in children under 1 year and with murmurs findings. Despite the anxiety level was higher in the group of patients who were diagnosed with congenital heart disease, this was not negligible in the family in the group of patients without heart disease

Screening and follow-up for congenital heart disease in children aged 0-3 years in rural areas of Chongqing, China / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To examine the incidence of congenital heart disease (CHD) in children aged 0-3 years in the rural areas of Chongqing, and to determine the suitable "screening-diagnosis-follow-up" system and screening indicators for CHD in these areas.</p><p><b>METHODS</b>Children aged 0-3 years from rural areas of the Fuling Disctrict of Chongqing were selected by cluster sampling. Using the "screening-diagnosis-evaluation system" employed at the levels of village/town, district/county, and province/city, the children were screened for seven indicators, i.e., family history of CHD, dyspnea, cyanosis, unique facial features, other congenital malformations, heart murmurs, and blood oxygen saturation (SpO<95%). Children who were positive for one or more indicators accepted echocardiography (ECG) for the diagnosis of CHD. CHD patients were evaluated for disease progression, given guided treatments, and followed-up by pediatric cardiologists.</p><p><b>RESULTS</b>Screening was performed for 10 005 out of the 10 281 children enrolled in the study (97.32% response rate). Among the 175 children who were positive for the indicators, 166 underwent ECG and 60 (0.6‰) were diagnosed with CHD, including 46 cases of simple CHD (76.65%), 11 cases of combined CHD (18.33%), and 3 cases of complex CHD (5.00%). Of the 7 screening indicators, heart murmur had the largest area under the ROC curve for the diagnosis of CHD. In addition, a combination of screening indicators (heart murmur, unique facial features, and other congenital malformations) was most effective for screening out CHD. The CHD patients were given surgical or intervention treatments, and followed up for 6 to 18 months. Ten patients improved without treatment, 13 patients received interventional or surgical treatment, 1 patient died of non-cardiac reasons. The remaining 36 patients were subjected to further follow-up.</p><p><b>CONCLUSIONS</b>Heart murmur alone and in combination with unique facial features and other congenital malformations are valuable tools for CHD screening in children aged 0-3 years. The "village/town-district/county-province/city" screening-diagnosis-evaluation systems are useful for the early detection, diagnosis, and treatment of CHD in infants and young children from the rural areas of Chongqing.</p>

Right Coronary Artery to Left Ventricular Fistula with Giant Right Coronary Artery Aneurysm / 대한내과학회지

A 35-year-old male with exertional dyspnea was transferred to our hospital for evaluation of a diastolic murmur and cardiomegaly discovered by chest X-ray. Two-dimensional and Doppler echocardiographic studies revealed an aneurysmal dilation of the right coronary artery (RCA) and diastolic inflow from the RCA to the left ventricle (LV) through a myocardial defect 11 mm in diameter in the basal inferior LV wall. Coronary computed tomography angiography (CCTA) confirmed the presence of a giant RCA to LV fistula. Due to the risks for developing overt left heart failure and spontaneous rupture of the giant RCA, the RCA was obliterated surgically. Coronary-cameral fistulas are a rare congenital coronary artery anomaly. A fistula between the coronary artery and LV, especially when combined with coronary artery aneurysm, is a rare occurrence in the literature. Here, we report a case of a giant RCA to LV fistula detected by echocardiography and CCTA.

Classic Peripheral Signs of Subacute Bacterial Endocarditis

A 50-year-old female patient with visual disturbances was referred for further evaluation of a heart murmur. Fundoscopy revealed a Roth spot in both eyes. A physical examination showed peripheral signs of infective endocarditis, including Osler nodes, Janeway lesions, and splinter hemorrhages. Our preoperative diagnosis was subacute bacterial endocarditis with severe aortic regurgitation. The patient underwent aortic valve replacement and was treated with intravenous antibiotics for 6 weeks postoperatively. The patient made a remarkable recovery and was discharged without complications. We report this case of subacute endocarditis with all 4 classic peripheral signs in a patient who presented with visual disturbance.

Busca ativa por cardiopatias congênitas é factível? Experiência em oito cidades Brasileiras / Active search for congenital heart disease is possible? Experience from eight brazilian cities

Fundamentos: As cardiopatias congênitas são causa de alta morbimortalidade na faixa etária pediátrica. Em regiões em desenvolvimento, a falta de diagnóstico precoce pode acarretar a piora do quadro; uma busca ativa por cardiopatias congênitas pode colaborar na resolução dessa situação.Objetivo: Descrever os principais achados de uma busca ativa por cardiopatias na infância, realizada em oito cidades brasileiras do estado da Paraíba. Métodos: Estudo realizado em oito cidades brasileiras do estado da Paraíba. A busca ativa compreendeu duas fases. Na primeira, os centros de saúde locais realizaram a triagem de crianças com sintomas ou história clínica de doenças cardiovasculares. Na segunda fase, realizada uma consulta clínica com posterior realização de ecocardiograma para identificação de cardiopatias. As frequências obtidas foram analisadas. Variáveis categóricas foram comparadas utilizando-se o teste do qui-quadrado. Resultados: Atendidas 440 crianças. Destas, 192 (43,63%) apresentaram alterações ao ecocardiograma. As presenças de sopro e da síndrome de Down mostraram correlação significativa (p<0,05) com a cardiopatia congênita. A maioria dos casos era de cardiopatia congênita por shunt (64,1%), seguida pelos defeitos valvares (12,5%) e pelas cardiopatias obstrutivas acianogênicas (8,3%). Conclusão: A busca ativa realizada foi capaz de identificar um número expressivo de cardiopatias congênitas na infância, não reconhecidas previamente, e inserir esses pacientes nos serviços de saúde para o tratamento adequado, garantindo-lhes melhor qualidade de vida.

Background: Congenital heart defects are the cause of high morbidity and mortality in pediatric patients. In developing regions, the lack of early diagnosis may lead to worsening of the condition; an active search for congenital heart diseases can assist in resolving this situation. Objective: To describe the key findings of an active search for heart diseases in childhood, held in eight Brazilian cities in the state of Paraíba. Methods: A study conducted in eight Brazilian cities in the state of Paraíba. The active search included two phases. In the first, local health centers screened children with symptoms or a history of cardiovascular diseases. In the second phase, a clinical consultation with echocardiogram to identify diseases was conducted. The frequencies were then analyzed. Categorical variables were compared using the chi-square test. Results: 440 children were assisted. Of these, 192 (43.63%) had abnormalities on echocardiogram. The presence of heart sounds and Down’s syndrome showed a significant correlation (p <0.05) with the congenital heart disease. Most cases were congenital heart disease by shunt (64.1%), followed by valve defects (12.5%) and acyanogenic obstructive heart diseases (8.3%). Conclusion: The active search conducted was able to identify a significant number of congenital heart diseases in childhood not previously recognized and inserting these patients in health services for appropriate treatment, thus ensuring them a better quality of life.

Hypertrophic cardiomyopathy secondary to severe right and left ventricular outflow tract obstruction in a Maltese dog / 대한수의학회지

An 8-year-old spayed female Maltese (2.5 kg of body weight) presented with the primary complaint of loud heart murmur and exercise intolerance. Diagnostic imaging revealed severe pulmonic stenosis (peak velocity 5.2 m/s) with right ventricular hypertrophy. The dog revisited after 2 years, at which time, diagnostic imaging revealed severe biventricular hypertrophy, dynamic left ventricular outflow tract obstruction, left atrial dilation and pulmonary hypertension with worsened pre-existing pulmonic stenosis. Postmortem investigation revealed hypertrophic cardiomyopathy and regional myocardial infarction. The case was diagnosed as hypertrophic cardiomyopathy secondary to severe right and left ventricular outflow tract obstruction.

Características clínicas de los pacientes con soplos atendidos en una consulta de cardiopediatría / Clinical characteristics of patients with murmurs cared in a pediatric cardiology consultation

Fundamento: La auscultación de un soplo cardíaco durante el examen físico de un niño es frecuente, por lo que constituye el principal motivo de interconsulta en cardiología pediátrica. Objetivo: Identificar las características clínicas de los pacientes con soplos atendidos en una consulta de cardiopediatría. Metodología: Se estudiaron 118 niños durante el periodo de septiembre 2011 a diciembre 2012. Las variables utilizadas fueron edad, sexo, antecedentes patológicos familiares, síntomas asociados, localización del soplo, intensidad, complementarios (telecardiograma, electrocardiograma y ecocardiograma doppler). Resultados: Predominó el grupo de cero días a cinco años, y el sexo masculino. El dolor precordial, las palpitaciones y la disnea constituyeron los síntomas más frecuentes asociados al soplo. En 94 niños el soplo se ubicó en la región mesocárdica y en 106 fueron grado II. Conclusiones: La auscultación de un soplo cardíaco durante el examen físico de un niño es un hecho frecuente, por lo que constituye motivo de interconsulta periódicamente en la cardiología pediátrica.

Background: auscultation of a heart murmur during a physical examination of a child is common, which is the main interclinical reason in pediatric cardiology. Objective: To identify clinical characteristics of patients with murmurs cared in a pediatric cardiology consultation. Methodology: 118 children were studied from September 2011 to December 2012. The variables used were age, gender, family medical history, associated symptoms, location of the murmur, intensity, complementary exams (telecardiogram, electrocardiogram and echocardiogram doppler). Results: The group of 0 days to five years and male sex predominated. Chest pain, palpitations and dyspnea were the most common symptoms associated with the murmur. In 94 children the murmur was located in the mesocardiac region and 106 were grade II. Conclusions: Auscultation of a heart murmur during a physical examination of a child is frequent, that is why it is a periodically interclinical reason in pediatric cardiology consultation.

Total Anomalous Pulmonary Venous Return in Siblings

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR have been reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery was performed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.