RESUMO
Meandering right pulmonary vein (MRPV) is an exceedingly rare congenital pulmonary vascular variant with less than 20 reported cases. MPVR is a tortuous aberrant venous drainage of the right pulmonary upper lobe anastomosing with the right lower pulmonary vein instead of directly joining the left atrium. The variant is benign with an orthotopic venous drainage without any kind of shunting. The differential diagnosis mostly comprises the scimitar syndrome but also primary varix, arteriovenous malformations, and pulmonary sequestration. We report a case of MRPV fortuitously diagnosed in a 70-year-old patient. Multi-detector computed tomographic angiography (CTA) with MPR, MIP, and 3D reconstructions allowed the diagnosis obviating the need for invasive procedures.
Assuntos
Angiografia por Tomografia Computadorizada , Tomografia Computadorizada Multidetectores , Veias Pulmonares/anormalidades , Idoso , Meios de Contraste , Humanos , Imageamento Tridimensional , Achados IncidentaisRESUMO
Scimitar or pulmonary venolobar syndrome, a rare pulmonary anomaly, consists basically of anomalous pulmonary venous drainage of the right lung to the inferior caval vein, anomalous systemic arterial supply to the right lower lobe from the descending aorta, hypoplasia of the right lung, and dextroposed heart. We present a rare case with constellation of all these findings of scimitar syndrome, but with the aberrant pulmonary vein draining into the left atrium.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Átrios do Coração/diagnóstico por imagem , Veias Pulmonares/anormalidades , Síndrome de Cimitarra/diagnóstico , Malformações Vasculares/diagnóstico , Adolescente , Diagnóstico Diferencial , Humanos , Masculino , Veias Pulmonares/diagnóstico por imagem , Radiografia Torácica , Tomografia Computadorizada por Raios X , Malformações Vasculares/cirurgiaRESUMO
Meandering right pulmonary vein is a rare vascular anomaly that requires accurate diagnosis to avoid unnecessary procedures and unintended vascular injury during operation. We describe an unusual meandering right upper lobe pulmonary vein draining into the left atrium via the right middle lobe pulmonary vein.
Assuntos
Veias Pulmonares/anormalidades , Adolescente , Feminino , Átrios do Coração/anatomia & histologia , Átrios do Coração/diagnóstico por imagem , Humanos , Veias Pulmonares/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Congenital anomalies of the pulmonary veins are rare. Meandering right pulmonary vein, considered a part of the Scimitar syndrome spectrum, is often an incidental finding during chest imaging. We present the case of a 4-year-old girl diagnosed with meandering pulmonary vein, who developed pulmonary hypertensive disease with an aggressive course, in spite of absence of hypoxia or elevated pulmonary wedge pressure.
Assuntos
Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Angiografia , Pré-Escolar , Feminino , Humanos , Achados Incidentais , Radiografia , Síndrome de Cimitarra , Tomografia Computadorizada por Raios XRESUMO
Background: Total anomalous pulmonary venous connection (TAPVC), with an intrapulmonary segment (IPV), a meandering abnormally located confluence and obligatory drainage of one lung into the other before entering the systemic circulation, is a rare anomaly and we term it as the meandering intrapulmonary TAPVC (MITAPVC). Material and methods: We report five patients with an unusual variation of the TAPVC channel. A review of literature was done to identify this association of TAPVC with an intrapulmonary vein and absence of a confluence in its usual location. Results: In our study, 4 patients with neo-confluence creation had excellent outcome while one with partial correction required catheter-based intervention, but succumbed to persistent pulmonary hypertension refractory to therapy. A literature search showed 25 additional such patients. Two groups were noted, one with isolated lesions (N = 16) and the other with heterotaxy or complex intracardiac lesions (N = 14). Of the 20 surgical interventions, only 12 survived, most of them in the isolated group (N = 10). Mortality was due to incomplete surgery (4/4), inappropriate surgery (3/3), and complete and appropriate surgery (1/11) respectively. Conclusion: The MITAPVC is often associated with heterotaxy and complex lesions. However, the isolated version is being increasingly recognised. Non-recognition or inappropriate surgical correction of MITAPVC is associated with fatal outcomes. Evaluation by a computerised tomography (CT) scan, meticulous dissection and demonstration of the entire channel, creation of a neo-confluence and appropriate palliation for the heterotaxy is the key to ensure good outcome. This is not a new entity, but deserves a separate subclassification under TAPVC. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-021-01290-2.
RESUMO
Meandering pulmonary vein (MPV) is a rare entity that can be associated with an additional cardiac and pulmonary venous variations, including left-to-right shunts. Clinicians should consider further workup with dedicated cardiac imaging to evaluate for associated cardiovascular abnormalities after an abnormal pulmonary vein draining is initially identified on routine computed tomography or echocardiogram. Pulmonary venous variations in MPV represent a spectrum of disorders, and no consistent nomenclature has been established.
RESUMO
Anomalies of the pulmonary venous circulation are extremely uncommon. We report a case of an anomalous unilateral single pulmonary vein also referred to as a meandering pulmonary vein. A single large tortuous pulmonary vein was seen on the right side draining into the left atrium with associated ipsilateral absence of the middle lobe bronchus and interlobar fissures. This is considered a variant of the classical scimitar syndrome. The venous anatomy was depicted with considerable accuracy using multidetector computed tomography (MDCT) non-invasively.
Assuntos
Veias Pulmonares , Malformações Vasculares , Brônquios , Átrios do Coração/anormalidades , Humanos , Pulmão , Tomografia Computadorizada Multidetectores , Veias Pulmonares/anormalidades , Síndrome de Cimitarra/complicaçõesRESUMO
A 56-year-old Chinese man presented with giddiness and vertigo. Subsequent chest radiography showed the classic scimitar sign of an abnormal pulmonary venous return. Further evaluation with non-contrast computed tomography substantiated the finding of a partial anomalous venous drainage pattern and identified an associated rare lung anomaly, horseshoe lung. The imaging findings of scimitar syndrome and its association with horseshoe lung are reviewed.