Answer for BIR CoW 03 July 2022
RIGHT PULMONARY ARTERY TO LEFT ATRIAL FISTULA
Findings
Right ventricle and right atrium appears dilated. Ostium secundum type of atrial septal defect is noted Large focal outpouching noted arising from the right inferior pulmonary artery Inferomedial portion of the dilatation appears to communicate with left atrium. IMPRESSION: Dilated Right ventricle and right atrium with Ostium secundum type of atrial septal defect. Right pulmonary artery to left atrium fistula with right lower lobe pulmonary veins draining into it No evidence of myocardial / Right ventricular outflow tract scar.
Discussion
Abnormal communication (fistula) between right pulmonary artery & left atrium (RPA-LA) occurs rarely, and is distinct from pulmonary AVM. Mostly congenital in origin, it can also be post-traumatic. Due to its rarity and diagnostic complexity, o. nly about a hundred cases exist in the literature, and its true prevalence is not documented Classification is into four types, based on pulmonary veins configuration. Surgical correction is curative in most cases and early intervention is advised. Sysubtle crescentic right paracardiac opacity is present in all cases CT / catheter mptoms are centred around cyanosis and exertional dyspnoea. Direct RPA-LA fistula produces persistent central cyanosis, whereas aneurysmal fistula produces intermittent cyanosis. Chest X-ray demonstrates oligaemic lung fields with left-sided cardiomegaly in extreme cases. A very angiography clearly demonstrates the fistulous communication, which most often arises from the posterior wall of the descending branch of RPA, and inserts into the LA. Both right & left pulmonary veins are of normal configuration in type-I fistula. PDA is identified in 70% of cases of neonatal presentation. Accurate anatomical identification facilitates choice of proper incision approach and surgical technique. Cardiopulmonary bypass may be required if dissection & ligation of the aneurysm is technically difficult
REFERENCES:
1] Chowdhury UK, Kothari SS, Airan B, et al (2005) Right pulmonary artery to left atrium communication. Ann Thorac Surg 80:365–370. [2] Chowdhury UK, Airan B, Kothari SS, et al (2005) Right pulmonary artery-to-left atrium communication: report of 5 cases. Ann Thorac Surg 80:336–338. [3] Orlick AE, Hultgren HN, Stoner JD, et al (1979) Traumatic pulmonary artery—left atrial fistula: An unusual case of cyanosis in an adult. American Heart Journal 98:366–370. [4] Krishnamoorthy K (2001) Pulmonary artery to left atrial fistula. European Journal of Cardio-Thoracic Surgery 20:1052–1053. [5] Zhu J, Xi E-P, Yan M, Zhu S-B (2015) Right Pulmonary Artery to Left Atrial Fistula Confirmed by 320-slice Computerized Tomography. Chin Med J 128:2549–2550 [6] Biocina B, Sutlić Z, Husedinović I, et al (1993) Direct communication of the pulmonary artery with the left atrium. Lijec Vjesn 115:160–162. [7] Krause DW, Kuehn HJ, Sellers RD, Wilson WJ (1974) Roentgen sign associated with an aberrant vessel connecting right main pulmonary artery to left atrium. Radiology 111:177–178 [8] Jimenez M, Fournier A, Choussat A (1989) Pulmonary artery to the left atrium fistula as an unusual cause of cyanosis in the newborn. Pediatr Cardiol 10:216–220. [9] Krishnakumar N, Krishna MR, Tharakan JA (2007) Right pulmonary artery-to-left atrial fistula: a differential diagnosis of cyanosis without structural heart disease. J Invasive Cardiol 19:401–402. [10] Mongé MC, Russell HM, Popescu AR, Robinson JD (2014) Right pulmonary artery to left atrial fistula in a neonate: case report and review of the literature. World J Pediatr Congenit Heart Surg 5:306–310. [11] Abe T, Kuribayashi R, Sato M, Nieda S (1972) Direct communication of the right pulmonary artery with the left atrium. A case report and review of the literature. J Thorac Cardiovasc Surg 64:38–44.
Findings
Right ventricle and right atrium appears dilated. Ostium secundum type of atrial septal defect is noted Large focal outpouching noted arising from the right inferior pulmonary artery Inferomedial portion of the dilatation appears to communicate with left atrium. IMPRESSION: Dilated Right ventricle and right atrium with Ostium secundum type of atrial septal defect. Right pulmonary artery to left atrium fistula with right lower lobe pulmonary veins draining into it No evidence of myocardial / Right ventricular outflow tract scar.
Discussion
Abnormal communication (fistula) between right pulmonary artery & left atrium (RPA-LA) occurs rarely, and is distinct from pulmonary AVM. Mostly congenital in origin, it can also be post-traumatic. Due to its rarity and diagnostic complexity, o. nly about a hundred cases exist in the literature, and its true prevalence is not documented Classification is into four types, based on pulmonary veins configuration. Surgical correction is curative in most cases and early intervention is advised. Sysubtle crescentic right paracardiac opacity is present in all cases CT / catheter mptoms are centred around cyanosis and exertional dyspnoea. Direct RPA-LA fistula produces persistent central cyanosis, whereas aneurysmal fistula produces intermittent cyanosis. Chest X-ray demonstrates oligaemic lung fields with left-sided cardiomegaly in extreme cases. A very angiography clearly demonstrates the fistulous communication, which most often arises from the posterior wall of the descending branch of RPA, and inserts into the LA. Both right & left pulmonary veins are of normal configuration in type-I fistula. PDA is identified in 70% of cases of neonatal presentation. Accurate anatomical identification facilitates choice of proper incision approach and surgical technique. Cardiopulmonary bypass may be required if dissection & ligation of the aneurysm is technically difficult
REFERENCES:
1] Chowdhury UK, Kothari SS, Airan B, et al (2005) Right pulmonary artery to left atrium communication. Ann Thorac Surg 80:365–370. [2] Chowdhury UK, Airan B, Kothari SS, et al (2005) Right pulmonary artery-to-left atrium communication: report of 5 cases. Ann Thorac Surg 80:336–338. [3] Orlick AE, Hultgren HN, Stoner JD, et al (1979) Traumatic pulmonary artery—left atrial fistula: An unusual case of cyanosis in an adult. American Heart Journal 98:366–370. [4] Krishnamoorthy K (2001) Pulmonary artery to left atrial fistula. European Journal of Cardio-Thoracic Surgery 20:1052–1053. [5] Zhu J, Xi E-P, Yan M, Zhu S-B (2015) Right Pulmonary Artery to Left Atrial Fistula Confirmed by 320-slice Computerized Tomography. Chin Med J 128:2549–2550 [6] Biocina B, Sutlić Z, Husedinović I, et al (1993) Direct communication of the pulmonary artery with the left atrium. Lijec Vjesn 115:160–162. [7] Krause DW, Kuehn HJ, Sellers RD, Wilson WJ (1974) Roentgen sign associated with an aberrant vessel connecting right main pulmonary artery to left atrium. Radiology 111:177–178 [8] Jimenez M, Fournier A, Choussat A (1989) Pulmonary artery to the left atrium fistula as an unusual cause of cyanosis in the newborn. Pediatr Cardiol 10:216–220. [9] Krishnakumar N, Krishna MR, Tharakan JA (2007) Right pulmonary artery-to-left atrial fistula: a differential diagnosis of cyanosis without structural heart disease. J Invasive Cardiol 19:401–402. [10] Mongé MC, Russell HM, Popescu AR, Robinson JD (2014) Right pulmonary artery to left atrial fistula in a neonate: case report and review of the literature. World J Pediatr Congenit Heart Surg 5:306–310. [11] Abe T, Kuribayashi R, Sato M, Nieda S (1972) Direct communication of the right pulmonary artery with the left atrium. A case report and review of the literature. J Thorac Cardiovasc Surg 64:38–44.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!