Descripción anatómica, clínica y sobrevida de los pacientes con atresia pulmonar que han sido llevados a angioplastia con stent en el ductus arterioso persistente en el periodo 2009-2014 / Anatomical and clinical description and survival of patients with pulmonary atresia subjected to angioplasty with ductal stent in the period 2009-2014

Resumen Objetivo: describir las características anatómicas, la evolución clínica y la sobrevida de los pacientes con atresia pulmonar que han sido llevados a angioplastia con stent en ductus arterioso persistente y determinar si existen diferencias significativas en la saturación al inicio y final del procedimiento y a las 6, 12 y 24 horas posprocedimiento. Materiales y métodos: estudio analítico, descriptivo tipo cohorte. La población de estudio se tomó de las bases de datos del servicio de Cardiología pediátrica de una institución especializada en la ciudad de Bogotá, Colombia. Se incluyeron pacientes con diagnóstico de atresia pulmonar sometidos a angioplastia, en tanto que se excluyeron aquellos con cirugía cardiovascular previa. El análisis estadístico se realizó en STATA® 12,0, mediante estadística descriptiva, curvas de Kaplan-Meier y ANOVA de medidas repetidas. Resultados: se identificaron 237 pacientes con atresia pulmonar. Se seleccionaron 30 de los cuales se excluyeron 5. En total se construyó una cohorte de 25 pacientes que fueron llevados a angioplastia, 52% con atresia pulmonar con comunicación interventricular. Edad promedio 12,2 días, peso 2.698 g, diámetro del DAP 3,8 mm (DE = 1,1), índice de McGoon media 1,35. El implante del stent fue exitoso en todos los pacientes; sin embargo 2 fallecieron en la sala de procedimientos y uno durante las 6 horas siguientes. Las complicaciones más frecuentes fueron paro cardiorrespiratorio (n = 2), trombosis del stent (n = 2) y bacteriemia (n = 2). La mortalidad a 30 días fue del 28% (n = 7); durante el tiempo total fue del 36% (n = 9). Por medio del ANOVA no se encontraron diferencias significativas en los promedios de saturación en los cinco tiempos de interés. Conclusiones: la atresia pulmonar es una patología compleja que requiere intervención invasiva para el mantenimiento de la capacidad circulatoria de los pacientes. Este estudio muestra que a pesar de una intervención efectiva, la mortalidad a los 30 días es elevada. No se encontraron diferencias en los tiempos de saturación, lo que sugiere que mantiene la saturación de los pacientes de una forma no distinta que con la prostaglandina E (PGE1) pero garantiza la permeabilidad del ductus.

Abstract Objective: the aim of this study is to describe the anatomical characteristics, the clinical progression, and survival of patients with pulmonary atresia subjected to angioplasty with a stent in patent ductus arteriosus (PDA), and to determine if there are significant difference in the saturation at the beginning and the end of the procedure, and at 6, 12, and 24 hours after the procedure. Materials and methods: An analytical and descriptive cohort study was conducted on a population taken from the database of a Paediatric Cardiology Department of a specialised institution in the city of Bogota, Colombia. Patients with pulmonary atresia subjected to angioplasty were included, and those with previous cardiovascular were excluded. The statistical analysis was performed using STATA® 12.0, by means of descriptive statistics, Kaplan-Meier curves and repeated measurements ANOVA. Results: A total of 237 patients with pulmonary atresia were identified. Of the 30 that were selected, 5 were excluded, leaving a total of 25 patients that were subjected to angioplasty. Interventricular communication was observed in 52%. The mean age was 12.2 days, with a mean weight of 2.698 g, a mean PDA diameter of 3.8 mm (SD = 1.1), and a mean McGoon index of 1.35. Although the stent implant was successful in all patients, two died in the procedures room, and another one during the following 6 hours. The most common complications were, cardiorespiratory arrest in 2 patients, thrombosis of the stent in 2, and bacteraemia in two patients. The mortality at 30 days was 28% (7), and 36% (9) during the total time. Using ANOVA, no significant differences were found in the saturation means at the five times of interest. Conclusions: Pulmonary atresia is a complicated condition that requires invasive intervention in order to maintain the circulatory capacity of the patients. This study shows that, despite an effective intervention, the 30-day mortality is elevated. No differences were, found in the saturation times, which suggest that saturation is maintained in the patients in a way not unlike that with prostaglandin E (PGE1), but ensures the patency of the ductus.

Cardiac Screening in the First Trimester Using Three-dimensional Ultrasound with Spatiotemporal Image Correlation / 中山大学学报(医学科学版)

[Objective] To evaluate the value of cardiac screening in the first trimester by using three-dimensional ultrasound with spatiotemporal image correlation (STIC) and to analyze the value of different views for cardiac assessment.[Methods] This study retrospectively reviewed the eighty-seven fetuses with congenital heart diseases which were diagnosed in our institution between January 4th 2013 and June 30th 2016.We compared the detection rate of prenatal ultrasound screening with STIC in the first trimester and the results of postnatal echocardiography or autopsy.In addition,the detection rates of the different planes,including four-chamber view (4CV),outlet of the left ventricle (OLV),outlet of the right ventricle (ORV) and three vessels-trachea view (3VT) were analyzed.[Results] Severe cardiac anomalies were identified in 62 fetuses (71.3％) and non-severe cardiac anomalies were identified in 25 fetuses (28.7％).Seventy-eight cases (89.7％,78/87) with congenital heart diseases could be detected by using three-dimensional ultrasound with STIC.The detection ratesfor 4CV,4CV+OLV,4CV+ORV,4CV+3VT,4CV+OLV+ORV and 4CV+OLV+ORV+3VT were 48.3％,64.4％,66.7％,79.3％,69.0％ and 89.7％ respectively.[Conclusions] Three-dimensional ultrasound with STIC could detect congenital heart disease in the first trinester.The four-chamber view plus outlet of the left ventricle,outlet of the right ventricle and three vessels-trachea view could increase the detection rates of congenital heart diseases.

One-stage surgical repairs for interrupted aortic arch associated with cardiac defects in infants / 中华胸心血管外科杂志

Objective To sum up the clinical information and outcomes of one-stage surgical repairs for interrupted aortic arch (IAA) associated with cardiac defects through median stemotomy in infant.Methods From August 2005 to January 2012,23 IAA patients,aged 18 days to 3 years [mean age (8.61 ± 11.81) months],body weight 3.3-13.0 kg [mean (6.61 ± 3.26) kg] were reviewed underwent one-stage repair.There were 12 male and 11 female.The anatomic subtype ineluded type A 20 (87.0％),type B 3 (13.0％),and no type C in the records.All cases included ventricular septal defect and patent ductus artefious,some also with artirical-pulmonary window,aterical septal defect,bicuspid or unicuspid aortic valve,and subvalvular aortic stenosis.Diagnosis was determined in of the patients and suspected in by echocardiography.Also,64 layers helico-CT was employed to make a definite diagnosis for 18 patients and cardiac catheterization was used for 4 patients.All patients with cardiac anomalies underwent one-stage repair through median sternotomy.The aortic continuity was reestablished by anastomosis between the descending aortic segment and aortic arch.Results In all 23 cases,21 were successful.There was 2 (8.69％) postoperative death:one was due to surgical hemorrhage and severe low cardiac output during perioperative stage and the other was 2-month old due to crisis of pulmonary hypertension.CPB time was ranged from 53-215 min [(129.76 ± 38.98) min],and aortic crossclamp time was 34-125 min[(74.47 ± 24.30) min].The length of stay in ICU postoperatively was 96h averagely.The postoperative complications included severe low cardiac output syndrome in 3 patients,hypoxemia in 13,pneumonia in 7,and supraventricular tachycardia in 12.21 patients were followed up from 2 months to 6 years and were in good condition without recoactation.Conclusion The outcomes of early and medium term for one-stage repair of IAA and associated cardiac anomalies through median stemotomy is excellent.Technique of extended anastomosis between the descending aortic segment and aortic arch may reduce the incidence of recoarctation.It is simplified the procedure and improved life quality of patients.

Significance of screening chromosome 22q11.2 deletion syndrome in the congenital cardiovascular abnormalities / 国际儿科学杂志

Chromosome 22q11.2 deletion syndrome,also called DiGeorge syndrome or Velo-CardiacFacial syndrome,has all expansive phenotype involving essentially every organ and system,such as cardiovascular abnormalities,abnormal face,immunodeficiency,even psychiatric illnesses,and etc.Fluorescence in situ hybridization analysis test for the microdeletion from chromosome 22 at the q11.2 band is the comqrmed diagnostic method So far,it has not been known thoroughly in China and there has not been a normative screening system yet.Close relations between the microdeletion and congenital cardiovascular abnormalities especially conotruncal cardiac defects and arcus aortae abnormalities have been shown in reported cases.This review will describe the 22q11 DS and how to screen it in the congenital cardiovascular abnormalities so that it Can be diagnosed early and managed properly.which will benefit the patients and their later generations.