Abstract
While unilateral pulmonary venous atresia (UPVA) most commonly presents as an extremely rare late embryological defect resulting in complete occlusion or absence of the PV pathway, it may also be an acquired pathology. We present a 3-year-old boy who presented with mild respiratory distress. Neonatal echocardiographic investigations revealed normal mediastinal anatomy and pulmonary vasculature with a bicuspid aortic valve. However, follow-up Doppler investigation revealed a pulmonary artery size difference with minimal forward flow and reverse flow during diastole. Absence of the left pulmonary veins and the presence of collaterals draining to the innominate vein confirmed the diagnosis of acquired UPVA. Our case represents the first case of acquired UPVA in conjunction with a normally functioning bicuspid aortic valve. The Doppler flow patterns discussed might be of significant interest to pediatricians, cardiologists and imaging specialists. These findings suggest that acquired UPVA should be considered in the differential diagnosis of such patients when radiographic and echocardiographic findings may rule out other more common diagnoses. While the management of such a condition remains unclear and conservative management was agreed upon for our patient, the vulnerability of such cases warrants timely diagnosis and routine monitoring.
Riassunto
Mentre l’atresia polmonare venosa unilaterale (UPVA) più comunemente si presenta come un rarissimo difetto di ritardo embriologico, con conseguente occlusione completa o assenza della PV, può essere anche una patologia acquisita. Presentiamo il caso di un bambino di 3 anni con distress respiratorio lieve. L’ecocardiografia neonatale ha rivelato normale anatomia del mediastino e della vascolarizzazione polmonare, con una valvola aortica bicuspide. Tuttavia, il follow-up Doppler ha rivelato una differenza di dimensioni dell'arteria polmonare con portata minima e flusso inverso durante la diastole. L’assenza delle vene polmonari di sinistra e la presenza di collaterali con drenaggio nella vena anonima hanno confermato la diagnosi di UPVA acquisita. Il nostro caso rappresenta il primo di UPVA acquisita in combinazione con il normale funzionamento di una valvola aortica bicuspide. I modelli di flusso Doppler discussi potrebbero essere di interesse significativo per pediatri, cardiologi e specialisti di imaging. Questi risultati suggeriscono che l’UPVA acquisita dovrebbe essere considerata nella diagnosi differenziale di tali pazienti quando i reperti radiografici ed ecocardiografici possano escludere altre diagnosi più comuni. Mentre la gestione di tale condizione non è chiara ed è stato concordato il trattamento conservativo per il nostro paziente, la vulnerabilità di tali casi richiede diagnosi tempestiva e monitoraggio di routine.
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Conflict of interest
Mohammed Firdouse, Arnav Agarwal, Lars Grosse-Wortmann, and Tapas Mondal declare that they have no conflict of interest.
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The authors confirm that all human and animal studies have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the Helsinki Declaration of 1975 and its late amendments. Additional informed consent was obtained from all patients for which identifying information is not included in this article.
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Firdouse, M., Agarwal, A., Grosse-Wortmann, L. et al. Acquired unilateral pulmonary vein atresia in a 3-year-old boy. J Ultrasound 18, 73–78 (2015). https://doi.org/10.1007/s40477-014-0135-0
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DOI: https://doi.org/10.1007/s40477-014-0135-0