[Fetal diagnosis and prognosis of Ebstein's anomaly]. / Diagnóstico y pronóstico fetal de la anomalía de Ebstein.

BACKGROUND: Currently fetal echocardiography may confirm the diagnosis in utero of Ebstein anomaly, as well as determine the perinatal outcome with high certainty. OBJECTIVE: To review 16 cases diagnosed with Ebstein anomaly, by fetal echocardiography, analyzing prognostic echocardiographic parameters set by Pavlova and colleagues. MATERIAL AND METHOD: A descriptive, observational, retrospective study was done in patients with fetal diagnosis of Ebstein anomaly, during January 2001 to December 2011. Echocardiographic parameters are analyzed and its correlation to perinatal evolution of 16 cases of Ebstein anomaly. RESULTS: Maternal age was of 27.94 + 5.7 years, gestational age was 31.3 +/- 3.6 weeks. In utero mortality represented 37.5% (n = 6), and neonatal mortality 50% (n = 8), two patients (12.5%) survived. Cardiothoracic index was of 0.61 +/- 0.074, ratio foramen ovale-atrial septal was of 0.6 +/- 0.015, obstruction of the outflow tract of the right ventricle was seen in 14 (87.5%) patients; 81% of the deceased had a degree of displacement valve > 2.5; ratio right ventricle-left ventricle 2.24 +/- 0.37. The umbilical vein was throbbing in 64% of the deceased, the tricuspid insufficiency was severe in 15 cases (94%, 21.62 +/- 2.82 mmHg), hydrops affected 18.7% of patients. CONCLUSIONS: The following factors are of bad prognosis in fetal stage of Ebstein anomaly: cardiothoracic index > 0.55, relative foramen ovale-atrial septal <0.3, the obstruction to the outflow tract, a degree of valve displacement > 2.5, absence of reverse flow in the duct arteriosus, ratio right ventricle-left ventricle > 2. The Ebstein anomaly diagnosed in utero has a perinatal mortality of 87.5%.

[Clinical guide practice. Patent ductus arteriosus]. / Guía de práctica clínica. Persistencia del conducto arterioso.

Patent ductus arteriosus (PDA) is the most common congenital heart disease in Mexico. The clinical manifestations of the PCA are from asymptomatic patients to the presence of heart failure. Its management should be individualized based on clinical, hemodynamic data and presence of pulmonary hypertension. Our objective was to provide current medical recommendations based on the best, available scientific evidence for the diagnosis, study and therapeutic decisions of the PCA. Established a standardized sequence to search for Practice Guidelines, based on the clinical questions about PCA diagnosis and treatment. Most of the recommendations were taken from selected guidelines and supplemented with the remaining material. The information is expressed in levels of evidence (E) and grade of recommendation (R) according to the characteristics of the study design and type of publications. Currently produces large amounts of medical information in a relatively short period of time which is necessary to have evidence-based CPG to facilitate and standardize the diagnostic decision-treatment to provide better care for children and adults with PCA.

[Right ventricular thrombous and pulmonary artery aneurysms in Behçet's disease. Report of one case]. / Trombo en ventriculo derecho y aneurismas de arterias pulmonares en enfermedad de Behçet. Reporte de un caso.

UNLABELLED: Behçet's disease uncommon in the pediatric population. Intracardiac thrombus and bilateral pulmonary artery aneurysms are uncommon manifestations. We are reporting one case. A 14 years old patient was admitted, with high fever, dyspnea and hemoptysis since 4 months ago. Two years ago, fever, oral ulcers, aphthae (gingival, palate, tonsils), nose ulceration in and arthralgias-arthritis were noted. Chest radiograph showed round mass in the right lower lung field. The chest tomographic computed scan and pulmonary centellography were done to investigate malformations arterial. An Intracardiac thrombus of 27 x 12 mm was identified in the right ventricle by transthoracic echocardiography. Catheterization and pulmonary angiography showed an aneurism located in the right and left lobares arteries. Medical management with immunosuppressive and anticoagulation therapy resulted in complete remission of the clinical manifestations. Due to heamodynamic compromise surgical removal of the intracardiac thrombus was done. IN SUMMARY: The Behçet's disease is rare disease in children. Intracardiac thrombus and bilateral pulmonary artery aneurysms are rare complications. Medical treatment (immunosupressive and anticoagulation) is the first line therapy with resolution of the mucous, skin, cardiac and pulmonary manifestations.

Trombo en ventriculo derecho y aneurismas de arterias pulmonares en enfermedad de Behçet: reporte de un caso / Right ventricular thrombous and pulmonary artery aneurysms in Behçet's disease: report of one case

Behçet's disease uncommon in the pediatric population. Intracardiac thrombus and bilateral pulmonary artery aneurysms are uncommon manifestations. We are reporting one case. A 14 years old patient was admitted, with high fever, dyspnea and hemoptysis since 4 months ago. Two years ago, fever, oral ulcers, aphthae (gingival, palate, tonsils), nose ulceration in and arthralgias-arthritis were noted. Chest radiograph showed round mass in the right lower lung field. The chest tomographic computed scan and pulmonary centellography were done to investigate malformations arterial. An Intracardiac thrombus of 27 x 12 mm was identified in the right ventricle by transthoracic echocardiography. Catheterization and pulmonary angiography showed an aneurism located in the right and left lobares arteries. Medical management with immunosuppressive and anticoagulation therapy resulted in complete remission of the clinical manifestations. Due to heamodynamic compromise surgical removal of the intracardiac thrombus was done. IN SUMMARY: The Behçet's disease is rare disease in children. Intracardiac thrombus and bilateral pulmonary artery aneurysms are rare complications. Medical treatment (immunosupressive and anticoagulation) is the first line therapy with resolution of the mucous, skin, cardiac and pulmonary manifestations.