Bailout procedure in obstructed supracardiac total anomalous pulmonary vein drainage / Procedimiento de rescate en drenaje venoso pulmonar anómalo total supracardíaco obstructivo

Abstract Total anomalous pulmonary venous drainage is a rare and diverse anomaly, accounting for 1% to 3% of patients with congenital heart disease. Newborns with diagnosis of an obstructed total anomalous pulmonary venous dainage are extremely ill soon after birth and often present with severe cyanosis, pulmonary hypertension and low cardiac output requiring urgent surgical intervention. Transcatheter palliative stenting of the obstructive vertical vein can be an acceptable alternative as a bailout intervention before complete surgical correction is undertaken. This report of two cases highlights the feasibility, safety and effectiveness of the inter ventional palliative procedure and confirms that this technique can be an acceptable and attractive bridge in the algorithm of medical decisions during the evaluation of these critical patients.

Resumen El drenaje venoso pulmonar anómalo total es una enfermedad poco frecuente y de presentación diversa y se observa en el 1% a 3% de las cardiopatías congénitas. Si se asocia a obstrucción, se convierte en una afección grave en el recién nacido, mostrando cianosis intensa, hipertensión arterial pulmonar y bajo gasto cardíaco con indicación de intervención quirúrgica de urgencia. El implante de stent por cateterismo de forma paliativa para aliviar la obstrucción puede ser una alternativa aceptable de tratamiento como intervención de rescate antes de la corrección quirúrgica definitiva. Presentamos dos casos de intervención percutánea paliativa mostrando que esta técnica puede ser eficaz como puente al tratamiento quirúrgico definitivo para ser incorporado en la toma de decisiones de estos pacientes críticos.

Mal-positioning of dialysis catheter in anomalous left superior pulmonary vein in a patient with acute type a dissection, a case report

Abstract The partial anomalous pulmonary vein drainage is a rare congenital defect. The pulmonary vein drains in to a systemic vein instead of draining in to the left atrium. In this rare birth defect, the right sided pulmonary vein involvement is more prevalent than the left sided pulmonary veins. We present a case where the anomalous left superior pulmonary vein was diagnosed when a renal dialysis catheter (size = 12F x 16cm) was mal-positioned in to the Anomalous left superior pulmonary vein, demonstrating confusing blood results. We describe how a systematic multidisciplinary approach and use of advanced imaging techniques can recognise and deal with this rare clinical dilemma.

Conexión venosa pulmonar anómala: reporte de un caso / Anomalous pulmonary venous connection: a case report

RESUMEN: Las conexiones anómalas de venas pulmonares fueron descritas por primera vez en 1739. Las anomalías de drenaje de las venas pulmonares se deben a una alteración temprana (día 27-30 del desarrollo) momento en el cual los plexos venosos pulmonares están en conexión con las venas de los sistemas cardinal, umbilical y vitelino, estas conexiones involucionan y el drenaje venoso deriva hacia el atrio izquierdo a través de la vena pulmonar común. En este trabajo presentamos una conexión venosa pulmonar anómala parcial, su conocimiento es relevante pues se asocia en un 80 % a defectos cardiacos, principalmente comunicaciones interatriales. Disección de rutina de un cadáver adulto, de sexo masculino, a nivel del pedículo pulmonar izquierdo. Se describe una vena originada en el lobo pulmonar superior izquierdo que abandona el hilio pulmonar por delante del bronquio, realizando un trayecto ascendente de 10 cm de longitud desde el hilio para terminar desembocando en la vena braquiocefálica izquierda. La vena anómala drena el territorio del lobo superior del pulmón izquierdo. Por su disposición y relaciones, esta vena de trayecto anómalo tiene su origen en la persistencia de las conexiones del sistema venoso pulmonar, en especial las que se establecen con el sistema cardinal anterior izquierdo. Este hallazgo destaca la riqueza y relevancia clínica de la disección en la formación de profesionales de la salud.

SUMMARY: The anomalous connections of the pulmonary veins were first described in 1739. The drainage anomalies of the pulmonary veins are due to an early alteration (day 27-30 of development), during this period the pulmonary venous plexuses are in connection with the cardinal, umbilical and vitelline vein systems. These venous connections return and drain into the left atrium through the common pulmonary vein. In this work we present a partial anomalous pulmonary venous connection. Knowledge of this connection is relevant because it is associated to 80 % of cardiac defects, mainly interatrial communications. During a routine dissection of an adult male cadaver, a vein originating in the left upper lobe was found. The vein originates at the pulmonary hilum in front of the bronchus, with an ascending path of a 10 cm length from the hilum, concluding its trajectory in the left brachiocephalic vein. The anomalous vein drains the left upper lobe of the lung. This anomalous vein originates within the connections of the pulmonary venous system, particularly those found in the left anterior cardinal vein system. This finding highlights the importance and clinical relevance of dissection in the training of health professionals.

Surgical treatment strategy and outcome of functional univentricular heart with total anomalous pulmonary vein drainage / 中国胸心血管外科临床杂志

@#Objective     To explore the surgical management method and outcome of functional univentricular heart with total anomalous pulmonary vein drainage (UVH-TAPVD). Methods     We reviewed the surgical procedures and results for 44 UVH-TAPVD patients in our hospital between the year 2010 and 2016. There were 34 males and 10 females. The age of the patients was 312 (77-4 220) d when they accepted the first surgical treatment. Results     There were 8 deaths in stage Ⅰ palliation, 1 death in stage Ⅱ palliation and 5 deaths during the follow-up. The overall survival rate was 68.2% (30/44). Glenn operation was undertaken in 34 patients with 5 deaths. Fontan operation was undertaken in 9 patients with 2 deaths. Conclusion     Surgical strategies for UVH-TAPVD should be planned according to different anatomical and pathophysiological conditions in different patients. Staged palliations can reduce mortality and morbidity. But pulmonary venous obstruction and heterotaxy syndrome are still risk factors for these patients.