Right pulmonary artery to left atrial fistula: a description of two cases, emphasising a diagnostic approach

Abstract

Right pulmonary artery to left atrial fistula is a rare pathology characterised by a right to left shunt. Another important aspect of this pathology is the difficulty encountered in making a diagnosis, which is why the diagnosis is frequently delayed into adulthood. A description of two cases is used to emphasise the importance of the different modes of echocardiography as a diagnostic tool in diagnosis, as well as the two different clinical forms that it adopts: a group of patients suffering cardiac failure and cyanosis without apparent cause generally in neonates and a second group of mostly older patients with dyspnoea and cyanosis without apparent cause. Symptoms thus differ depending on the time of presentation and are related to the size of the fistula.

Transthoracic Echocardiography for Diagnosis of Right Pulmonary Artery to Left Atrial Fistula

“…Selective RPA angiography remains the most definitive investigation for confirmation and accurate imaging of this anomaly, but is invasive . We found that TTE was an effective, noninvasive, inexpensive, and reproducible diagnostic method . Of the 3 cases, 2 were diagnosed by TTE, and the other was misdiagnosed as a pulmonary arteriovenous fistula by contrast echocardiography.…”

“…9,18 We found that TTE was an effective, noninvasive, inexpensive, and reproducible diagnostic method. 13 Of the 3 cases, 2 were diagnosed by TTE, and the other was misdiagnosed as a pulmonary arteriovenous fistula by contrast echocardiography.…”

“…Since the first description of RPA‐LAF by Friedlich and associates in 1950, less than 100 cases have been reported, and almost all of these have been presented as case reports or as reports of computed tomography or angiography findings . There is still a lack of systematic description of the echocardiographic characteristics of this condition.…”

Renal thromboembolism from a large pulmonary artery to a pulmonary vein fistula in an asymptomatic adolescent

“…2 Interestingly, when discussing direct fistulous connections with the left atrium, there is a strong male predominance estimated at 3:1. 3 The presentation can vary depending on the size of the shunt and can include exertional dyspnoea, cyanosis, cerebrovascular accidents, and systemic embolisation. 2,3 Studies including children and adults report an incidence of neurological events related to pulmonary arteriovenous fistula as 37% for transient ischaemic attacks and 18% for stroke.…”

“…3 The presentation can vary depending on the size of the shunt and can include exertional dyspnoea, cyanosis, cerebrovascular accidents, and systemic embolisation. 2,3 Studies including children and adults report an incidence of neurological events related to pulmonary arteriovenous fistula as 37% for transient ischaemic attacks and 18% for stroke. 4 Systemic, non-cerebral embolisms account for 5-10% of all paradoxical events; however, recent studies demonstrate that this is a rare occurrence, even in patients with hypercoagulopathy.…”