Partial anomalous pulmonary venous connection associated with hemoptysis

Abstract

Rationale: Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly characterized by the failure of fusion of embryologic pulmonary venous system with left atrium. Patient Concerns: A 45-year-old male patient with PAPVC who was hospitalized because of mild hemoptysis. Images showed the anomalous vein originated from the left upper pulmonary vein and flowed into the left brachiocephalic vein. No other underlying causes for hemoptysis were detected. Diagnosis: After multi-disciplinary discussion, the patient was diagnosed as PAPVC of left upper pulmonary vein draining into the left brachiocephalic vein with intact atrial septum. Interventions: Although surgical correction of PAPVC was feasible, left upper lobectomy was performed as the definitive treatment for both hemoptysis and PAPVC. Outcomes: The patient had an uneventful postoperative hospital course and was followed up for nearly 2 years without recurrence of hemoptysis. Lessons: PAPVC is associated with atrial septal defect in 80% to 90% of cases while isolated PAPVC with intact atrial septum is an extremely rare entity. We present a rare isolated PAPVC patient with hemoptysis. To our best knowledge, PAPVC associated with hemoptysis has never been reported before.

Múltiples anomalías congénitas en el contexto de cardiopatía congénita con retorno venoso anómalo

“…La aurícula primitiva forma una vena pulmonar común que luego se une con el lecho vascular pulmonar formado por separado (7). El RVPA se caracteriza por la falla de fusión de una parte o todo del sistema venoso pulmonar con la aurícula izquierda (11); las venas anómalas del lado derecho pueden regresar a la aurícula derecha (como en el caso presentado); la vena cava superior, la vena cava inferior, la vena ácigos, la vena hepática o la vena porta y las venas anómalas del lado izquierdo podrían drenar en la vena innominada, el seno coronario y la vena hemiácigos (12), y los drenajes aberrantes son predominantemente derechos, alrededor del 80 % (7).…”

“…En nuestra paciente se observó el segundo tipo de RVPA, el retorno venoso pulmonar anómalo parcial (RVPAP), el cual representa por sí solo del 0,4 % al 0,7 % de las CC (11,16), con prevalencias en la vida adulta de entre el 0,1 % y el 0,2 % (12). La variante más común (el 60 %) está constituida por un drenaje venoso pulmonar total, en el que hay un tronco venoso único en el pulmón afectado, que se forma por la reunión de las venas pulmonares de origen intersegmentario y drena a alguno de los sitios antes mencionados.…”

Left partial anomalous pulmonary venous connection (PAPVC), a rare pulmonary venous variant

“…Patients often have no specific cardiac or respiratory symptoms therefore a chest x-ray test is the first step [2] but an association with hemoptysis is possible [6] . There is a frequent association with ASD and PAPVC in 80%-90% of cases [10] which further increases the volume of left to right shunt with an increase of the likelihood of pulmonary hypertension [5] .…”

Surgical repair of left-sided partial anomalous pulmonary venous connection associated with situs inversus totalis

“…Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly characterized by failure of normal connection of one or more of (but not all) the pulmonary veins to the left atrium. Alternatively, the anomalous pulmonary veins connect to the right atrium or one of its tributaries (5,6). The overall prevalence of PAPVC among general population has been reported to be between 0.4-0.7% (7).…”