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.

Incidental finding of pulmonary arterial sling during patent ductus arteriosus surgery in a patient with Mowat-Wilson syndrome.

Mowat-Wilson syndrome is a genetic condition due to a mutation in the ZEB2 gene; it affects many systems including the cardiovascular system. The pulmonary arterial sling originates from a failure of development of the proximal portion of the left sixth aortic arch, resulting in an anomalous left pulmonary artery origin from the posterior wall of the right pulmonary artery and the left pulmonary artery crossing to the left lung between the trachea and the oesophagus. We present a 4-month-old infant with Mowat-Wilson syndrome and left pulmonary arterial sling, and discuss the association of these two rare conditions. Pulmonary arterial sling is significantly more frequent in patients with Mowat-Wilson syndrome than in the general population.

Teeth infection may "shunt" through Fontan in high-altitude conditions.

The Fontan surgery involves the creation a conduit between the inferior vena cava and the right pulmonary artery. This conduit has a small fenestration that shunts the blood from right to left in case the pulmonary blood flow is limited; namely, if the pulmonary vascular resistance (PVR) is increased then the shunt is increased. Bacteria may bypass the pulmonary circulation and easily get access to the systemic circulation (bacteremia). We report the case of a patient that underwent Fontan surgery in 2010 and remained in a high-altitude city for 7 years, during this time he was asymptomatic until 2017 when he developed a brain abscess due to Streptococcus gordonii, a pathogen of dental plaque. Since high-altitude may raise PVR in response to reduction in the partial pressure of oxygen, we conclude that the long-term outcome of increased altitude on Fontan hemodynamics can lead to the shunt of teeth flora and consequently leading to severe infections.

Interrupción proximal de la arteria pulmonar derecha aislada e hipoplasia pulmonar ipsilateral: Presentación de caso / Pulmonary Artery Right Interruption Isolated With Ipsilateral Pulmonary Hypoplasia: Case Report

Las cardiopatías congénitas complejas se presentan con una gran variedad de malformaciones que pueden afectar el tronco de la arteria pulmonar y las arterias pulmonares. Es infrecuente encontrar de manera aislada la interrupción de una de las mismas. En este caso se explican las ayudas diagnósticas imaginológicas que llevaron al diagnóstico de interrupción proximal de la arteria pulmonar derecha sin asociación con otra malformación congénita, en una paciente a quien se le diagnosticó, inicialmente, síndrome de drenaje venoso anómalo.

The complex congenital heart disease may present with a variety of malformations in the main pulmonary artery. However, it is not common to find in isolation the absence of one of the pulmonary arteries. In this case report, we explain the diagnostic imaging aid that led to the diagnosis of proximal interruption of the right pulmonary artery without association to other congenital malformation in a patient in whom a syndrome of anomalous venous drainage was initially thought.