Cardiovasc Interv and Ther DOI 10.1007/s12928-013-0240-6

CASE REPORT

Transcathether closure of a right pulmonary artery-to-left atrial fistula using Amplatzer muscular ventricular septal defect occluder Ibrahim Ece • Abdurrahman Uner Ferhat Cuce • Sevket Balli



Received: 30 October 2013 / Accepted: 21 December 2013 Ó Japanese Association of Cardiovascular Intervention and Therapeutics 2014

Abstract A right pulmonary artery-to-left atrial fistula is a very rare cyanotic congenital heart defect and is characterized by cyanosis and normal auscultation of the heart. Interventional closure of the fistula using occluder devices and coils has been rarely reported. We report the successful closure of a RPA-to-left atrial fistula using an Amplatzer muscular ventricular septal defect occluder in a child with cyanosis. The two-dimensional echocardiogram with bubble contrast study demonstrated the communication between right pulmonary artery and left atrium. Computerized tomography confirmed the diagnosis and delineated the anatomy. Keywords Cyanosis  Left atrium  Pulmonary artery fistula  Transcatheter closure

Introduction Right pulmonary artery (RPA)-to-left atrial fistula is a very rare form of cyanotic congenital heart disease (CHD) and was first reported by Friedlich et al. [1]. Such patients usually present with central cyanosis with clubbing of fingers, exertional dyspnea, or uncommonly with

I. Ece (&)  A. Uner Department of Pediatric Cardiology, Faculty of Medicine, Yuzuncu Yil University, Van, Turkey e-mail: [email protected] F. Cuce Department of Radyology, Van Millitary Hospital, Van, Turkey S. Balli Department of Pediatric Cardiology, Balıkesir Atatu¨rk Hospital, Balıkesir, Turkey

complications of embolism [2, 3]. To our knowledge, there are 61 such cases in the literature [4]. Surgical repair was the only treatment available till the introduction of interventional procedures [2, 5]. We report the successful closure of a RPA-to-left atrial fistula using an Amplatzer muscular ventricular septal defect (VSD) occluder (AGA Medical, Plymouth, MN, USA) in a child with cyanosis.

Case report An 8-year-old female child presented with cyanosis and effort limitation. Clinical examination was unremarkable except for significant central cyanosis and clubbing of fingers. Systemic arterial saturation was 79 % in room air. Electrocardiography showed a normal sinus rhythm. Echocardiography showed elongated left atrium (LA) with posterior expansion of this chamber in a modified 4-chamber view and a communication between RPA and LA in a high-parasternal short-axis view. The narrowest diameter was measured 5.4 mm. Agitated saline injection showed early appearance of contrast bubbles in the LA within two beats (Fig. 1a, c). There was no atrial septal defect (ASD) or patent foramen ovale (PFO). Computerized tomogram (CT) angiography confirmed a large aneurismal sac adjacent to the LA and the communication between RPA and LA. The narrowest diameter of fistula was measured 5.6 mm by CT (Fig. 2a, b). The child was planned for transcatheter closure of the RPA-to-left atrial fistula, under general anesthesia. Access from both the femoral veins and right femoral artery was obtained. Selective right pulmonary arteriogram performed in the 30° caudal projection demonstrated a fistula between the proximal RPA and a large aneurysmal sac of the LA. The narrowest portion of fistula was measured 5.6 mm

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I. Ece et al. Fig. 1 a Transthoracic echocardiogram showing fistulous (arrow) communication between right pulmonary artery (RPA) and left atrium (LA) in a highparasternal short-axis view; c bubble contrast echocardiogram demonstrating filling of LA from the fistula; b well-positioned Amplatzer muscular ventricular septal defect occluder within the fistulous tract; d control echocardiography showed no contrast bubbles on the left side of the heart appeared after agitated saline injection. RA right atrium

Fig. 2 Computerized tomogram angiography in axial plane (a) and three-dimensional volume-rendering computed tomography (b) showing a right pulmonary artery (RPA)-to-left atrial fistula (arrows) with a sac behind the left atrium. LPA left pulmonary artery

Fig. 3 a Selective right pulmonary artery (RPA) angiography in the 30° caudal projection shows a connection (arrows) between the RPA and the left atrium. b The control angiogram reveals no residual shunt

and no obstruction of the right pulmonary artery before detachment. c The control angiogram after detachment. Arrows indicate the position of the Amplatzer muscular ventricular septal defect occluder

(Fig. 3a). The fistula was crossed with the Judkins right 4 catheter and hydrophilic 0.035-in. guide wire and this was exchanged with 0.035-in. extra stiff guide wire. The fistula

was crossed from the right heart with a 6-F long sheath and a 6-mm Amplatzer VSD occluder was deployed at the narrowest site and before releasing the device, right

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Right Pulmonary Artery to Left Atrial Fistula

pulmonary arteriogram was repeated at a 30° caudal position with a second catheter (Fig. 3b). After the procedure, arterial oxygen saturation immediately increased to 96 % and control angiography demonstrated complete occlusion of the fistula (Fig. 3c). There were no procedure-related complications. The patient was discharged from the hospital on day 2 with aspirin (5 mg/kg). One month later, control echocardiography showed the well-seated device and no obstruction of the RPA and no contrast bubbles on the left side of the heart appeared after agitated saline injection (Fig. 1b, d). Patient’s clinical symptom was improved from New York Heart Association Functional Class II to I.

Discussion RPA-to-left atrial fistula is a rare CHD that is typically associated with a normal cardiovascular system examination except for central cyanosis. Often there is no murmur and diagnosis is delayed. Such patients usually present with cyanosis with clubbing, polycythemia, exertional dyspnea, or uncommonly with complications of cerebral or systemic embolism [2, 3]. Since the first description in 1950 [1], nearly 61 cases of RPA–left atrial fistula have been reported [4]. Surgical ligation of the fistula has been the preferred treatment of this condition until recently [2, 5]. There have been 7 reported cases of transcatheter closure of RPA-left atrial fistula. Transcatheter occlusion of RPAto-left atrial fistula with different devices has been employed with good results [4, 6–8]. de Souza e Silva et al. [9] categorized communication between the RPA and LA into three types based on the anatomy of the fistula and the pattern of the pulmonary venous drainage. Ohara et al. [10] added a fourth type. In type I, the RPA branches normally, pulmonary venous return is normal, and an additional fistulous channel connects the RPA to the LA. In type II, the right lower pulmonary vein is absent and the lower lobe RPA branch drains directly into the LA, forming an aneurysmal sac. In type III, all pulmonary veins drain into the abnormal channel that connects the RPA to the LA. In type IV, a very proximal pulmonary arteriovenous fistula joins the LA. According to this classification, our patient had type I anomaly. Device choice depends on the size of the vessel and the anatomy of the narrowing. The retrograde deployment from the LA side is a method by a duct occluder device in the presence of ASD or PFO; however, in the absence of an ASD or PFO, double-disc devices may be used such as VSD or ASD occluder device. We occluded the RPA-left atrial fistula using a 6-mm Amplatzer VSD occluder from the pulmonary arterial side. Amplatzer septal occluder may be deployed directly through the

pulmonary artery side, but the LA disc may assume an oblong shape, and there may be a concern that the globular configuration of the right atrial disc may predispose to a potential risk of pulmonary artery erosion. The LA disc may erode the aneurysmal wall. Duct occluder is not ideally suited for closing the fistula from the pulmonary arterial side, because the pulmonary artery pressure is likely to be higher than LA pressure. Vascular plugs may be used directly through the pulmonary artery side in the tunnel shape fistula. Amplatzer VSD occluder device has been reported to be effective and safe in RPA-left atrial fistula and it can be implanted from the pulmonary arterial side [8]. Before the operation, we evaluated the anatomy of the fistula well using echocardiogarphy and CT angiography. Multi-position pulmonary angiography was performed to learn more about lesion’s anatomy and embouchure of the pulmonary veins. We did not use balloon-sizing evaluation of the diameter of the fistula due to prolonged procedure time and increased complication risks. Also we did not use an oversized device to avoid the obstruction of pulmonary artery and veins and blood vessels injury.

Conclusion We occluded the RPA-to-left atrial fistula using an Amplatzer VSD occluder device from the pulmonary arterial site without trans-septal puncture and forming venovenous loop. According to our experience, the Amplatzer VSD occluder device appears to be effective tool for occlusion of RPA-to-left atrial fistula. Acknowledgments

None.

Conflict of interest

None.

References 1. Friedlich A, Bing RJ, Blount SG Jr. Physiological studies in congenital heart disease: IX. Circulatory dynamics in the anomalies of venous return to the heart including pulmonary arteriovenous fistula. Bull Johns Hopkins Hosp. 1950;86:20–57. 2. Zeebregts CJ, Nijveld A, Lam J, van Oort AM, Lacquet LK. Surgical treatment of a fistula between the right pulmonary artery and the left atrium: presentation of two cases and review of literature. Eur J Cardiothorac Surg. 1997;11:1056–61. 3. Gelfland MS, Stephen DS, Howell E, Alford RH, Kaiser AB. Brain abscess: association with pulmonary arteriovenous fistula and hereditary hemorrhagic telangiectasia: report of 3 cases. Am J Med. 1988;85:118–20. 4. Vadlamudi K, Verma S, Christopher J, Penumatsa RR, Koneti NR. Transcatheter closure of large right pulmonary artery-to-left atrial fistula. Ann of Pediatr Card. 2013;6:188–90. 5. Krishnamoorthy KM, Rao S. Pulmonary artery to left atrial fistula. Eur J Cardiothorac Surg. 2001;20:1052–3.

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I. Ece et al. 6. Slack MC, Jedeikin R, Jones JS. Transcatheter coil closure of a right pulmonary artery to left atrial fistula in an ill neonate. Catheter Cardiovasc Interv. 2000;50:330–3. 7. Ergul Y, Nisli K, Aydogan U. Transcatheter closure of a fistula between the right pulmonary artery and left atrium using the amplatzer septal occluder. Turk Kardiyol Dern Ars. 2011;39:231–4. 8. Baspinar O, Irdem A, Toprak Y. Transcatheter closure of a large left atrial aneurysm with a right pulmonary artery-to-left atrium

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connection using the Amplatzer ventricular septal occluder. Pediatr Cardiol. 2012;33:1443–5. 9. de Souza e Silva NA, Giuliani ER, Ritter DG, Davis GD, Pluth JR. Communication between right pulmonary artery and left atrium. Am J Cardiol. 1974;34:857–63. 10. Ohara H, Ito K, Kohguchi N, et al. Direct communication between the right pulmonary artery and the left atrium. A case report and review of the literature. J Thorac Cardiovasc Surg. 1979;77:742–7.

Transcathether closure of a right pulmonary artery-to-left atrial fistula using Amplatzer muscular ventricular septal defect occluder.

A right pulmonary artery-to-left atrial fistula is a very rare cyanotic congenital heart defect and is characterized by cyanosis and normal auscultati...
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