Partial anomalous pulmonary venous connection or scimitar. In the latter case the hemodynamic condition is totally different from the former because the systemic circulation is maintained. Part of this mixture passes through the atrial septum atrial septal defect into the left atrium. Cardiovascular mr angiography in partial anomalous. An associated high sinus veno sus atria1 septal defect is shown by dashed lines. Cardiac type of partial anomalous pulmonary venous. Partial anomalous pulmonary venous connection associated with lutembachers syndrome. Partial anomalous pulmonary venous connection is a congenital abnormality in which one or more of the pulmonary veins connect to a systemic vein or directly to the right atrium potentially producing a left to right shunt. Partial anomalous pulmonary venous connection in a 72yearold woman. Partial anomalous pulmonary venous connection papvc is one of the rare congenital cardiac diseases with a prevalence of 0.
Blood from one or more pulmonary veins returns abnormally to the right atrium, either directly or indirectly through a variety of systemic venous pathways that connect with the anomalous pulmonary vein. Leftupper lobe partial anomalous pulmonary venous return. This relatively uncommon congenital anomaly is found in 0. If the anomaly compromises 50% or more of the pulmonary venous flow, it may become clinically significant. Partial anomalous pulmonary venous return papvr, or partial anomalous pulmonary venous connection, is a rare congenital cardiovascular anomaly where some of the pulmonary veins drain into the systemic circulation, commonly at the level of the inferior vena cava, instead of the left atrium. Sinus node dysfunction after partial anomalous pulmonary. The most common forms encountered in adults are papvc of the left upper lobe pulmonary. We studied the prevalence of pah both in patients with isolated papvc or associated with other. Partial anomalous pulmonary venous connection papvc causes a lefttoright shunt from the anomalous pulmonary vein pv to a systemic vein. Partial anomalous pulmonary venous connection papvc was first described by winslow in 1739. Partial anomalous pulmonary venous connection papvc is a congenital abnormality characterized by the failure of fusion of embryologic pulmonary venous system with the left atrium.
Cardiovascular mr angiography can measure the associated lefttoright shunting and can depict the anomalous vasculature threedimensionally. Partial anomalous pulmonary venous connection request pdf. Fulltext pdf partial anomalous pulmonary venous return in a lung transplant. Partial anomalous pulmonary venous connection in a dog. Partial anomalous pulmonary venous connection has also recently been reported for the first time in a cat and cta confirmed the diagnosis. Partial anomalous pulmonary venous return, sometimes called partial anomalous pulmonary venous connection, is a heart defect present at birth congenital in which some of the pulmonary veins carrying blood from the lungs to the heart flow into other blood vessels or into the hearts upper right chamber right atrium, instead of correctly entering the hearts upper left chamber left atrium. Partial anomalous pulmonary venous drainage papvd describes the connection of at least one pulmonary vein, but not all, to the systemic venous system or. Abstract the surgical management of 15 patients with partial anomalous pulmonary venous connection papvc. Partial anomalous pulmonary venous return overview.
The left pulmonary veins as well as the right upper lobe pulmonary vein connected and drained normally. Partial cardiopulmonary bypass is instituted with venous return from the in. Total anomalous pulmonary venous connection tapvc frequency clinical signs etiology diagnosis treatment references abstract pulmonary venous return anomaly consists of partial papvc and total tapvc anomalous pulmonary venous connections. Isolated partial anomalous pulmonary venous connection papvc has been implicated as a cause of pulmonary arterial hypertension pah, however this condition is often overlooked in the diagnostic work up of patients with ph. Although the atriotomy is limited in this technique, a sufficiently large systemic venous chamber overlapping to the outside of the superior vena cava can be created.
Tapvc is found in about 2% of autopsied cases with congenital heart disease and has a male predominance. The goal of this publication is to present the case of diagnosis. Partial anomalous pulmonary venous connection papvc is a rare congenital anomaly. Isolated partial anomalous pulmonary venous connection ncbi. Request pdf partial anomalous pulmonary venous connection anomalous pulmonary venous connection is the 15th most common congenital heart defect 1. Partial anomalous pulmonary venous connection detected. Leftsided partial anomalous pulmonary venous connection papvc is a congenital defect where pulmonary veins from the left lung drain into the right atrium. Tapvc is almost always associated with some type of atrial septal defect asd for life to be sustained beyond the newborn period. Abstract objectives to systematically describe the imaging features and clinical correlates of a partial anomalous pulmonary venous connection diagnosed on computed tomography ct in adults.
Sinus node dysfunction after partial anomalous pulmonary venous connection repair carlo pace napoleone, md,a elisabetta mariucci, md,b emanuela angeli, md,a guido oppido, md,a and gaetano d. In partial anomalous pulmonary venous connection some but not all pulmonary veins of the left or right lung recircle oxygenated blood towards the right of the heart. Partial anomalous pulmonary venous connection to the. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava herbert e. Partial anomalous pulmonary venous connection papvc. The pulmonary veins connect normally and directly to the left atrium papvc is caused by.
Partial anomalous pulmonary venous connection in a 72yearold. On the contrary, partial anomalous venous connection of right pulmonary veins to the left vertical vein is described in just few reports 1, 2. A anomalous pulmonary venous connection to the superior oena cava svc well above the ravoatrial junction. In total anomalous pulmonary venous return, sometimes called total anomalous pulmonary venous connection, the blood vessels that lead from the lungs pulmonary veins attach to the heart in incorrect places, instead of attaching correctly to the upper left heart chamber left atrium. Partial anomalous pulmonary venous return radiology. Clinical implications of partial anomalous pulmonary. Partial anomalous pulmonary venous connection in a 72year. Generally, it is seen on the right side and is associated with an atrial septal defect. Both of these dogs had an anomalous vessel that was visualized on echocardiography. Right upper lobe partial anomalous pulmonary venous connection. The abnormal venous connection may affect only some of the pulmonary veins partial anomalous venous return or all of them total anomalous venous return.
We present an alternative surgical technique for the repair of a partial anomalous pulmonary venous connection to the higher segment of the superior vena cava. As the name suggests, in papvc, the blood flow from a few of the pulmonary veins return to the right atrium instead of the left atrium. If left untreated, papvc may result in severe right ventricular failure and pulmonary vascular disease. A technique for repair of partial anomalous pulmonary vein. Blood from one or more pulmonary veins returns abnormally to the right atrium, either directly or indirectly through a variety of systemic venous pathways that connect. Partial anomalous pulmonary venous return partial anomalous pulmonary venous return papvr, also known as partial anomalous pulmonary venous connection papvc, is a rare congenital cardi. This chapter includes definition, types, morphology, clinical manifestations, diagnosis, natural course, treatment, and fetal diagnosis of partial anomalous pulmonary venous connection and scimitar syndrome. Many cases do not cause symptoms and are diagnosed incidentally. Partial anomalous pulmonary venous return papvr is a lefttoright shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. Scimitar syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. Repair of partial anomalous pulmonary venous connection to. In most cases the anomalous venous connection involves the right pulmonary veins 1, 3 that drain into the superior vena cava svc or the right atrium. Partial anomalous pulmonary venous connection is usually associated with a normal or near normal life span. Anomalous pulmonary venous drainage journal of congenital.
Partial anomalous pulmonary venous connection papvc was. A right axillary minithoracotomy is done through the fourth intercostal space. Partial anomalous pulmonary venous connection papvc is a rare congenital anomaly characterized by the failure of fusion of embryologic pulmonary venous system with the left atrium, which manifested as 1 or more, but not all, of the pulmonary veins draining into the. Embryological approach for understanding pulmonary venous developmental anomalies. They described a technique that consisted of dividing the svc right above the anomalous pulmonary vein, oversewing the lower svc end, connecting the svc upper end to the right atrial appendage, and. Rengarajan rajagopal, niraj pandey, mumun sinha, sanjiv sharma. The prevalence of partial anomalous pulmonary venous connections is 0. Cardiovascular mr angiography can measure the associated lefttoright shunting and can depict. From there it goes into the left ventricle, then into the aorta and out to the body. We describe the surgical technique of reimplantation of the right superior pulmonary vein into the left atrium in 2 patients with partial anomalous pulmonary venous connection to the superior vena cava without atrial septal defect. Methods twentynine adults with a partial anomalous pulmonary venous connection on ct were retrospectively identified.
Isolated partial anomalous pulmonary venous connection papvc of the entire left lung is a rare congenital anomaly with incidental diagnosis and vague symptoms, if any, until late adulthood. An alternative method for repair of partial anomalous. Partial anomalous pulmonary venous connection differential. Cardiac type of partial anomalous pulmonary venous connection. Partial anomalous pulmonary venous return papvr, also known as partial anomalous pulmonary venous connection papvc, is a rare congenital cardiovascular condition in which some of the pulmonary veins, but not all, drain into the systemic circulation rather than in the left atrium. In the right atrium, oxygenrich red blood from the pulmonary veins mixes with lowoxygen bluish blood from the body. Partial anomalous pulmonary venous connection with pulmonary. We report a case of a 45yearold male with papvr to superior vena cava which was incidentally discovered during a.
There were 19 women and 10 men, with a mean age of 53 range. Surgical correction of this previously unreported defect was accomplished by creating an atrial septal defect and constructing a baffle to direct the blood flow from the azygous vein to the. Repair of partial anomalous pulmonary venous connection. The repair of a partial anomalous pulmonary venous connection papvc to the high superior vena cava svc was simplified by the surgical procedure reported by warden et al1 in 1984. Multislice computed tomography is useful for evaluating. Partial anomalous pulmonary venous connection papvc, also known as partial anomalous pulmonary venous return, encompasses a spectrum of congenital cardiovascular anomalies. Partial anomalous pulmonary venous connection in adults. Volumerendered images, derived from multidetectorrow computed tomography mdct, can facilitate assessment of the morphology of partial anomalous pulmonary venous connection and are thus useful in preoperative planning to prevent surgical morbidity and assist postoperative evaluations. Partial anomalous pulmonary venous connection to the superior. Repair of isolated partial anomalous pulmonary venous. Partial anomalous pulmonary venous connection papvc is an anomaly in which some pulmonary veins, but not all, are connected to the right atrium ra, its venous tributaries including the coronary sinus. These are partial and total forms of pulmonary venous drainage into the systemic venous system. The clinical and roentgenographic features suggested the correct diagnosis.
Surgical repair of partial anomalous pulmonary venous. Isolated partial anomalous pulmonary venous connection papvc has been implicated as a cause of pulmonary arterial hypertension pah. Most commonly one or more pulmonary veins pvs from the right lung connect to the svc or right atrium ra, or both. Images in cardiovascular mr angiography in partial. Patients with papvc usually have a clinical course that consists in. Partial anomalous pulmonary venous connection papvc is a rare congenital cardiac defect. Case report right upper lobe partial anomalous pulmonary. Pdf partial anomalous pulmonary venous connections papvcs are a heterogeneous group of congenital heart lesions in which at least one. Isolated partial anomalous pulmonary venous connection of.
The papvc may occur as an isolated anomaly or may be combined with atrial septal defect asd. Partial anomalous pulmonary venous connection papvc is a condition in which some, but not all, pulmonary veins connect to the right atrium or its tributaries, rather than to the left atrium. Partial anomalous pulmonary venous connection with. Partial anomalous pulmonary venous connection associated. Partial anomalous pulmonary venous connection papvc encompasses a group of cardiovascular anomalies that are caused by the abnormal return of one or. Partial anomalous pulmonary venous connection papvc is found in around 0. Total anomalous pulmonary venous return overview mayo. Leftsided partial anomalous pulmonary venous connections. Case report right upper lobe partial anomalous pulmonary venous connection christostourmousoglou, 1 christinakalogeropoulou, 2 efstratioskoletsis, 1 nikolaoscharoulis, 1 christosprokakis, 1 panagiotisalexopoulos, 1 emmanoilmargaritis, 1 anddimitriosdougenis 1 cardiothoracic department, university hospital of patra, rio, patra, greece.