Early severe coronary heart disease and ischemic heart failure in homozygous familial hypercholesterolemia: A case report.

RATIONALE: Familial hypercholesterolemia (FH) is a common inherited cause of coronary heart disease (CHD) and premature death in an early age. Nevertheless, an ischemic heart failure (IHF) associated with FH seems to be rare, and an early diagnosis and therapy could influence the prognosis. PATIENT CONCERNS: In this 13-year-old girl, multiple xanthomas began to develop from the first day of birth. Until June, 2017, she was admitted to our center due to edema, oliguria, and dyspnea during exertion, which was attributed to a recent respiratory infection. DIAGNOSIS: Homozygous FH (HoFH), CHD, and IHF. INTERVENTIONS: The patient has been treated with statin, ezetimibe, aspirin, and traditional heart failure (HF) medications. In addition, the beta-blocker was simultaneously administered. OUTCOMES: Genotypes of this proband indicated homozygous mutations of low-density lipoprotein receptor (LDLR) and some co-segregated mutations, such as von Willebrand factor (VWF) and fibroblast growth factor receptors. At 6-month follow-up, we found a decreased level of plasma lipid profile, in addition to a significant improvement in 6-minute walk distance and functional class. Echocardiography indicated nonsignificant improvements in the structure and function of the heart. LESSONS: This case report indicates that HoFH can lead to dramatically progressive endothelial damages and ventricular remodeling, severe atherosclerosis, even IHF. Genetic outcomes indicate IHF with HoFH could possibly result from LDLR mutations and some co-segregated mutations influencing endothelial function and cardiovascular remodeling. In a short-term follow-up, a combination of statins, ezetimibe, aspirin, and traditional HF agents is safe and effective for IHF with HoFH, and there is a need for further identification of drugs to ameliorate endothelial function and cardiovascular remodeling which may play an important role in long-term treatment.

Agenesia de óstio de coronária direita: relato de caso / Right coronary ostium agenesis: case report

Coronária única é uma rara anomalia que é frequente associada a outras alterações congênitas, tais como transposição de grandes vasos, fístula coronária e válvula aórtica bicúspide. Esta doença apresenta uma prevalência reportada de 0,024% a 0,066% dos pacientes submetidos a angiografia coronariana, sendo a maioria dos casos achados incidentais, entretanto pode levar a várias apresentações clínicas, como angina, infarto, síncope e morte súbita. No presente caso se trata de uma paciente de 53 anos com sintomas de angina há um ano onde a angiotomografia de coronárias revelou artéria circunflexa ampla que se estende pelo sulco atrioventricular direito, subgrupo L-I de Lipton, e drenagem do seio venoso coronariano para o átrio esquerdo. Este subgrupo é usualmente considerado benigno, contudo há relatos de pacientes que tinham a coronária direita originando do segmento distal da circunflexa que necessitaram de cirurgia de revascularização miocárdica. O reconhecimento desta rara anomalia é importante para evitar repetidas cateterizações coronarianas, para o correto diagnóstico e direcionamento do tratamento adequado. Mais relatos de caso são necessários para o entendimento das implicações clínicas desta anomalia. (AU)

Fibrilación auricular en paciente de 22 meses durante palatoplastia / Atrial fibrillation in a 22-month-old patient during cleft palate surgery

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Escalas de cálculo del riesgo cardiovascular para pacientes con diabetes. ¿Qué son y de qué nos sirven? / Cardiovascular risk scores in patients with diabetes. What are they and what are they worth?

La enfermedad cardiovascular (ECV) representa la primera causa de mortalidad en las personas con diabetes mellitus, entre las cuales, el riesgo de mortalidad cardiovascular es 2-4 veces mayor que el de la población general. Las guías de práctica clínica recomiendan calcular el riesgo de ECV en la diabetes; sin embargo, se han desarrollado pocos modelos para estimar este riesgo en las personas con diabetes. Los primeros modelos de predicción de ECV en la diabetes mellitus tipo 2, que incluyeron junto a las variables de riesgo clásicas la HbA1c y los años de evolución, no son contemporáneos y no funcionan en poblaciones diferentes a las que participaron en su desarrollo. La creación de modelos de riesgo propios actuales y validados en diferentes poblaciones permitiría realizar intervenciones preventivas más agresivas y tempranas, y centradas en el paciente, con la finalidad de frenar la epidemia de ECV que padecen las personas con diabetes

Cardiovascular disease (CVD) remains the first cause of death in patients with diabetes mellitus. Cardiovascular mortality is between 2 and 4 times as high as the risk of matched controls in the general population. Although practice guidelines recommend calculating CVD risk in diabetes, few models for estimating cardiovascular risk have been developed specifically for diabetic patients. The first ones, taking into account HbA1c and diabetes duration plus classical risk factors, is not contemporary and perform sub-optimally in different populations with diabetes. Constructing updated population-derived and externally validated cardiovascular risk models will yield more aggressive patient-centered preventive interventions to curb the ongoing epidemic of CVD in patients with diabetes

Infarto agudo de miocardio en un lactante secundario a una anomalía coronaria congénita / No disponible

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Vascular endothelial growth factor-a in lactobacillus casei cell wall extract-induced coronary arteritis of a murine model.

BACKGROUND: Vascular endothelial growth factor (VEGF) is associated with Kawasaki disease (KD), the most commonly acquired heart disease in developed countries. This study investigated the involvement of VEGF-A expression and its related signaling pathway in Lactobacillus casei cell wall extract (LCWE)-induced murine coronary artery lesions (CALs), and analyzed this in regard to the inhibition of CALs by spleen tyrosine kinase (Syk). METHODS AND RESULTS: Wild-type BALB/C mice were intraperitoneally injected with LCWE (1mg/ml) to induce CALs. The aortic roots, ventricular myocardium, peripheral blood leukocytes (PBLs), spleen, liver, kidneys, and lungs were analyzed for VEGF-A expression. Phosphate buffered saline (PBS)-, lipopolysaccharide (LPS)-, and zymosan-treated mice served as controls, and an oral Syk inhibitor served as an arteritis-ameliorated reagent. In aortic roots and PBLs, LCWE induced an early upregulation and a late downregulation of VEGF-A expression. No differential VEGF-A expression was observed in the other organs. Most importantly, Syk inhibition significantly attenuated the LCWE-induced expression of VEGF-A, dimethylarginine dimethylaminohydrolase (DDAH)-1, and endothelial nitric oxide synthase in aortic roots. However, LCWE-induced aortic DDAH-2 expression remained higher, despite Syk inhibition. CONCLUSIONS: Local VEGF-A and its signaling pathway are associated with the development of LCWE-induced CALs. Therefore, the clinical correlation between VEGF and human KD and the role of the VEGF-A regulation and signaling pathway in murine CALs warrant further investigation.

Management of an unusual left coronary artery to right ventricular fistula in a neonate.

Congenital coronary arterial fistula is a rare cause of congestive heart failure in neonates. We describe a neonate who developed early symptoms because of an unusually large left coronary artery to right ventricular fistula. Computed tomography and coronary angiography delineated anatomy and facilitated successful surgical closure.

Giant congenital fistula of the circumflex coronary artery with drainage into right atrium.

A dilated and tortuous fistula from circumflex coronary artery draining into the right atrium was diagnosed by transthoracic echocardiography and selective angiography in a 64-year old female. Transcatheter closure was excluded by the cardiologist because of the presence of a large huge at the drainage site so the patient was scheduled for surgical ligation. Intraoperative and postoperative controls were performed by echocardiography. The Authors discuss on differential diagnosis, indications and types of treatment in all cases presenting coronary artery fistula.

Atresia congênita do tronco da coronária esquerda em adulto jovem: rasa e potencialmente fatal anomalia coronária / Congenital atresia of the left main coronary artery in a young adult: a rare and potentially fatal coronary anomaly

Relato do caso de paciente do sexo masculino, 35 anos de idade, com atresia congênita do tronco da artéria coronária esquerda, afecção extremamente rara e com poucos casos descritos na literatura. Nessa afecção, o óstio da coronária esquerda está ausente, o tronco da coronária esquerda termina em fundo cego e o fluxo sanguíneo se dá da coronária direita para a esquerda por pequenas artérias colaterais e retrogradamente. O diagnóstico diferencial envolve outras malformações congênitas (artéria coronária direita única e origem anômala da artéria coronária esquerda do tronco da artéria pulmonar) e doença aterosclerótica adquirida do tronco da coronária esquerda.

We report the case of a 35-year-old male patient with congenital atresia of the left main coronary artery, a raredisease with few cases reported in the literature. In this anomaly, the left coronary ostium is absent, the proximalleft main trunk ends blindly and blood flow is retrograde from the right to the left coronary artery by small collateralarteries. Differential diagnosis includes other congenital malformations (single right coronary artery and anomalousorigin of the left coronary artery from the pulmonary artery) and acquired atherosclerotic disease of the left main coronary artery.

Cierre percutáneo del ductus con diferentes dispositivos en adultos / Percutaneous closure of patent ductus arteriosus in adults using different devices

El cierre quirúrgico del conducto arterioso en adultos implica riesgos debido a sus cambios anatómicos e histológicos. Desde octubre de 1992 hasta agosto de 2008, fueron referidos a nuestro servicio 23 pacientes con conducto arterioso persistente aislado; edad, 16-75 años (mediana, 25,5); peso, 52-80 kg (mediana, 57); diámetro pulmonar, 1,8-5,8 mm (media, 3,5); presión media en la arteria pulmonar, 9-72 mmHg (media, 15). Las tasas de oclusión fueron del 85,7% con los dispositivos Rashkind-PDA y del 100% con Amplatzer Duct-Occluder y Nit-Occlud coil. Tiempo medio de internación y seguimiento: 24 h y 2 años respectivamente. La única complicación inmediata fue un hematoma inguinal; no hubo complicaciones alejadas. En adultos, el cierre del conducto arterioso con diferentes dispositivos, especialmente los de última generación, resultó seguro y eficaz, independientemente de su morfología y estado histológico (AU)

Surgical closure of patent ductus arteriosus in adults involves a number of risks because there are associated anatomic and histologic alterations. Between October 1992 and August 2008, 23 patients were referred to our department with isolated patent ductus arteriosus. Their age ranged from 16-75 years (median 25.5 years) and their weight from 52-80 kg (median 57 kg). The pulmonary diameter ranged from 1.8-5.8 mm (mean 3.5 mm), and pulmonary artery pressure, from 9-72 mmHg (mean 15 mmHg). The rate of ductal occlusion achieved with the Rashkind patent ductus arteriosus occluder was 85.7%, and it was 100% with the Amplatzer duct occluder and the Nit-Occlud coil. The average hospitalization time and follow-up duration were 24 hours and 2 years, respectively. The only immediate complication was an inguinal hematoma, and there were no late complications. In adults, closure of patent ductus arteriosus using a number of different devices, especially the latest generation devices, was safe and effective, regardless of morphologic and histologic characteristics (AU)

Ectopia cordis / No disponible

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Neonatal closure of a large coronary-cameral fistula in a case of single coronary artery: the utility of intraoperative angiography.

Isolated coronary artery fistula in the newborn is a very rare congenital anomaly. When symptomatic, treatment options include surgical management with ligation and/or patch closure or transcatheter coil embolization. We describe the case of a newborn with symptomatic left coronary-right ventricular fistula, which was managed operatively with off-pump ligation and on-table angiogram for confirmation of fistula obliteration and normal distribution of flow in the coronary artery. We point out the advantages of on-table angiography to evaluate and potentially treat residual structural pathology after cardiac surgery.

Evaluation of a coronary-cameral fistula: benefits of coronary dual-source MDCT angiography in children.

We describe the use of a dual-source 64-channel multidetector array CT (MDCT) scanner in the evaluation of a coronary-cameral fistula in a 10-month-old boy. Echocardiography, cardiac catheterization, and MRI are modalities often used to evaluate congenital and other pediatric heart diseases. Radiation dose concerns and the absence of established protocols are factors that have potentially limited the use of CT for work-up in these young children. We present a case in which recent advances in technology allowed effective low-dose diagnostic quality coronary MDCT angiography in an infant.

[The saccular coronary aneurysm associated with coronary artery to pulmonary artery fistulae].

Coronary artery fistulae are relatively rare congenital anomalies. Those associated with saccular coronary artery aneurysms are even rarer. Including the current case, only 65 such cases have been reported in Japan. A 62-year-old female was admitted to our hospital for evaluation of abnormal shadow on the chest X-ray. The enhanced chest computed tomography (CT) scan demonstrated a giant saccular coronary aneurysm on the left side of the pulmonary artery. Multi-detector row CT (MDCT) scan demonstrated the coronary artery aneurysm was connected to the left anterior descending artery. Coronary angiography revealed 2 aneurysms with bilateral coronary artery to pulmonary artery fistulae. The patient underwent aneurysmectomy and ligation of fistulae under cardiopulomonary bypass. The postoperative course was uneventful and postoperative coronary angiography revealed complete resection of the aneurysms and only slight blood flow through the fistulae. She was discharged on the 10th postoperative day.

Origen anómalo de la arteria coronaria izquierda originándose de la arteria pulmonar. Reporte de caso y revisión de literatura / Anomalous origin of the left coronary artery originating itself of the pulmonary artery. Report of case and revision of literature

Se presenta el caso de un lactante de 2 meses de edad que consultó por tos y dificultad respiratoria; al ser admitido en el servicio de urgencias, el abordaje inicial fue de una infección respiratoria baja (bronquitis) pero la evolución tórpida y la presencia de cardiomegalia hizo sospechar una cardiopatía, diagnosticándose origen anómalo de la arteria coronaria izquierda que nace de la arteria pulmonar, la cual fue tratada quirúrgicamente con resultado favorable.