576 Zdrav Vestn | november – december 2019 | Letnik 88 Srce in ožilje 1 International center for cardiovascular diseases, MC Medicor, Izola, Slovenia 2 Division of internal medicine, Izola General hospital, Izola, Slovenia 3 Department of Intensive Internal Medicine, Division of Internal Medicine, University Medical Centre Ljubljana, Ljubljana, Slovenia Korespondenca/ Correspondence: Maja Rojko, e: rojko. maja@gmail.com Ključne besede: okvara mepreddvornega pretina; odprto ovalno okno; perkutano zapiranje Key words: atrial septal defect; patent foramen ovale; percutaneous closure Prispelo: 17. 9. 2018 Sprejeto: 15. 3. 2019 @publisher.id: 2872 @primary-language: sl, en @discipline-en: Microbiology and immunology, Stomatology, Neurobiology, Oncology, Human reproduction, Cardiovascular system, Metabolic and hormonal disorders, Public health (occupational medicine), Psychiatry @discipline-sl: Mikrobiologija in imunologija, Stomatologija, nevrobiologija, onkologija, r eprodukcija človeka, Srce in ožilje, Metabolne in hormonske motnje, javno zdravstvo (varstvo pri delu), Psihiatrija @article-type-en: Editorial, Original scientific article, Review article, Short scientific article, Professional article @article-type-sl: Uvodnik, izvirni znanstveni članek, Pregledni znanstveni članek, Klinični primer, Strokovni članek @running-header: Percutaneous closure of interatrial defects @reference-sl: Zdrav Vestn | november – december 2019 | Letnik 88 @reference-en: Zdrav Vestn | November – December 2019 | Volume 88 Percutaneous closure of patent foramen ovale and atrial septal defect: A case report Perkutano zapiranje odprtega ovalnega okna in okvare medpreddvornega pretina: Prikaz primera Maja Rojko, 1 Nataša Černič Šuligoj, 1,2 Metka Zorc, 1 Saibal Kar, 1 Marko Noč 1,3 Abstract A 67-year old man with a haemodynamically significant type secundum atrial septal defect (ASD), large patent foramen ovale (PFO) and a significant septal aneurism presented with shortness of breath and limited exercise tolerance. There was no evidence of additional structural abnorma- lities or significant coronary artery disease. Simultaneous percutaneous closure of both defects was planned. Since the wire could only be passed through PFO while the second wire could not be passed through the ASD, only PFO was closed with a 35 mm Amplatz PFO occluder. After 3 months, which served for tissue ingrowth of Amplatz PFO occluder and aneurism stabilization, ASD located in the posterior-inferior part of the fossa ovalis documented by three-dimensional transesophageal echocardiography (3D-TEE) was easily crossed and successfully closed with a 12 mm Amplatz ASD occluder. Stable position without unwanted interference between the de - vices was obtained. There was no residual shunting on colour Doppler and no bubble shunting during Valsalva maneuver. Within 6 months after the procedure, symptoms significantly impro- ved and the right heart chambers decreased. 3D-TEE revealed both devices in good position with only trivial shunting through the PFO occluder documented by colour Doppler. Izvleček 67-letnega moškega s pomembno hemodinamsko okvaro medpreddvornega pretina (ASD) tipa secundum v kombinaciji z velikim odprtim ovalnim oknom (PFO) in anevrizmo medpreddvor- nega pretina smo obravnavali zaradi težkega dihanja med naporom in omejene telesne zmo- gljivosti. Dodatnih strukturnih nepravilnosti ali pomembne koronarne bolezni nismo potrdili. Načrtovali smo sočasno perkutano zapiranje obeh okvar. Z žico smo prečkali PFO, z drugo žico pa preko ASD nismo uspeli priti. Zato smo zaprli PFO s 35-milimetrskim zapiralom Amplatz PFO. Po 3 mesecih, ki so služili za vraščanje zapirala in stabiliziranje anevrizme pretina, smo s tridi- menzionalnim transezofagealnim ultrazvokom (3D-TEE) jasno umestili ASD, ki je bil v postero- -inferiornem delu t. i. fosse ovalis. Uspešno smo ga prečkali in zaprli z 12-milimetrskim zapiralom Amplatz ASD. Obe zapirali sta bili v stabilni legi, preostalega pretoka pri barvni Dopplerjevi ultra- zvočni preiskavi ali prehoda mehurčkov med Valsalvo pa ni bilo. V 6 mesecih so se simptomi po- membno izboljšali, desne srčne votline pa zmanjšale. 3D-TEE je pokazal dobro lego obeh zapiral in minimalen preostali pretok skozi PFO zapiralo. Citirajte kot/Cite as: r ojko M, Černič Šuligoj n, Zorc M, Kar S, noč M. Percutaneous closure of patent foramen ovale and atrial septal defect: A case report. Zdrav Vestn. 2019;88(11–12):576–81. DOI: 10.6016/ZdravVestn.2872 Percutaneous closure of interatrial defects 577 KliniČni PriMer 1 Introduction Atrial septal defect (ASD) accounts for up to 30 % of all congenital heart abnormalities. There are four types in‑ cluding type primum, secundum, sinus venosus superior/inferior and sinus co‑ ronarius (1). Most of the patients with ASD are asymptomatic until their for‑ ties and early fifties (2) when shortness of breath and limited exercise tolerance gradually develops due to increasing left to right shunting (3). Only ASD type se‑ cundum is amenable for percutaneous closure which is the preferred treatment in case of appropriate anatomic chara‑ cteristics. Patent foramen ovale (PFO), on the other hand, is present in about 30 % of population and may be the culprit for paradoxical embolization resulting in ischemic stroke, myocardial infarcti‑ on or peripheral ischemic event (4). Percutaneous closure may reduce the in‑ cidence of repeat paradoxical emboliza‑ tion and represents an effective and safe alternative to lifelong antiaggregation or/and anticoagulation therapy in these predominantly younger patients (5‑7). Large ASD or PFO is frequently asso‑ ciated with interatrial septal aneurism (ISA) which may make percutaneous closure more challenging and increases the risk of device embolization. We he‑ rein report a patient in whom large PFO and ASD accompanied with significant ISA were successfully closed using two Amplatz occluders implanted in sequen‑ tial procedures. 2 Case report A 67‑year ‑old man with arterial hypertension and paroxysmal atrial fi‑ brillation presented with shortness of breath and limited exercise tolerance. Several years earlier, a 7 mm ASD esti‑ mated to be hemodynamically insigni‑ ficant, was documented. No additional cardiac abnormality including PFO or ISA has been documented at that time. A 12‑lead electrocardiogram recorded at our institution revealed sinus rhythm with borderline first‑degree AV block and signs of left ventricular hypertrophy without evidence of right bundle branch block (Figure 1). Transthoracic echocar ‑ diography (TTE) showed moderately en‑ larged right ventricle (end‑diastolic area 29 cm 2 ) and right atrium (area 36 cm 2 ). Left ventricle showed concentric hyper‑ trophy, normal ejection fraction and transmitral Doppler flow pattern sugge‑ stive of mild diastolic dysfunction. Also left atrium was moderately dilated (area Figure 2: Two-dimensional transesophageal echocardiographic view (90 degrees) on interatrial septum showing significant left to right color Doppler flow through atrial septal defect (ASD) and very long patent foramen ovale (PFO) with some in-tunnel color Doppler flow. LA = left atrium; RA = right atrium; Ao = aorta. Figure 1: 12-lead electrocardiogram recorded on admission. 578 Zdrav Vestn | november – december 2019 | Letnik 88 Srce in ožilje of 36 cm 2 ). Transesophageal echocardio‑ graphy (TEE) revealed PFO with a very long tunnel which widely opened during Valsalva maneuver and was associated with immediate massive bubble appea‑ rance in left atrium. Additionally, large ISA with oval ASD (12 × 7 mm) with si‑ gnificant left to right shunting (Qp/Qs 2.6:1.0) was demonstrated (Figure 2 and Figure 3 ). Cardiac MRI did not show additional abnormalities. Coronary angiography revealed mild nonobstructive disease. Right atrial pressure was 8/7 mm Hg (mean 5 mm), pulmonary artery pressure 38/9 mm Hg (mean 22 mm) and left atrial pressure measured by passage of the catheter through the PFO was 9/7 mm Hg (mean 6 mm Hg). Calculated Qp/Qs based on oximetry performed on room air was 1.9:1.0. Simultaneous percutaneous closure of both defects was planned. We routi‑ nely use fluoroscopy and TEE guidance under conscious sedation without en‑ dotracheal intubation (9‑11). A J‑tipped wire was easily passed through the PFO. The second wire, nor J‑tipped not hydrophilic, could not have been pas‑ sed through the ASD even when PFO was completely occluded with the sizing Figure 3: Two-dimensional transesophageal echocardiographic view (90 degrees) on interatrial septum during Valsalva maneuver showing atrial septal defect (ASD), very large patent foramen ovale (PFO) and significant aneurism of interatrial septum with large amount of abundant tissue. LA = left atrium; RA = right atrium; Ao = aorta. Figure 4: Three dimensional transesophageal echocardiographic view from left atrium showing the left disk of Amplatz PFO occluder and atrial septal defect (ASD) at the infero- posterior part of fossa ovalis. Ao = aorta; MV = mitral valve. Figure 5: Fluoroscopic view (LAO 30/2 degrees) showing Amplatz PFO occluder and wire with contrast-filled sizing balloon through the atrial septal defect (ASD). Percutaneous closure of interatrial defects 579 KliniČni PriMer balloon. At that point, the decision was made to close PFO using 35 mm Amplatz PFO occluder and perform ASD closure in a staged procedure after ingrowth of the device and ISA stabilization. After 3 months, the patient was brought aga‑ in to the catheterization laboratory. Three‑ dimensional (3‑D) TEE revealed a 10 mm ASD located at infero‑poste‑ rior border of fossa ovalis just below the previously implanted Amplatz PFO occluder which was in a good position (Figure 4 ). A standard J‑tipped wire was this time easily passed through the ASD and following balloon sizing (Figure 5 ), a 12 mm Amplatz ASD occluder was su‑ ccessfully deployed. Complete closure without unwanted interference between the both devices assessed by fluoroscopy (Figure 6 a) and 3‑D TEE (Figure 6 b) was documented. There was no residual color Doppler flow through any device or interatrial septum. The patient was discharged next morning without any complications. At 6 months follow up, he reported si‑ gnificantly decreased shortness of breath and improved exercise tolerance. He ro‑ ughly estimated improvement of symp‑ toms for about 70 % from the baseline and reported only two brief episodes of, most likely, paroxysmal atrial fibrillation. TTE showed that right ventricular area significantly decreased from 29 cm 2 to 15 cm 2 and right atrium area from 36 cm 2 to 28 cm 2 . 3‑D TEE showed both closure devices in adequate position with persisting small overlap (Figure 7 A) and trivial color flow through the Amplatz PFO occluder (Figure 7 B). 3 Discussion We described a patient with a com‑ plex pathology of aneurismatic interatri‑ al septum with large PFO and ASD whi‑ ch were successfully closed in a staged procedure using two Amplatz occluders. This case nicely illustrates the important role of TEE in such complex procedures and in particular the added value of 3‑D TEE which served to accurately locate ASD and its relation to PFO occluder which stabilized ISA. Such clear imaging was essential for easy wire crossing of ASD during the staged procedure. With good cooperation between interventio‑ nal cardiologist and echocardiographist, even such complex procedure becomes more predictable, safer and requires less fluoroscopy. Instead of TEE, intracardiac echocardiography (ICE) using percuta‑ neous probe introduced via femoral vein could also have been used. We have pre‑ Figure 6: Fluoroscopic (A) and three-dimensional transesophageal echocardiographic side view (B) showing both implanted Amplatz occluders in appropriate position. LA = left atrium; RA = right atrium. Figure 7: Three-dimensional transesophageal echocardiographic view from left atrium showing both implanted Amplatz occluders (A) and two dimensional echocardiographic view (90 degrees) of interatrial septum showing trivial color flow through the Amplatz PFO occluder (B) 6 months after the procedure. 580 Zdrav Vestn | november – december 2019 | Letnik 88 Srce in ožilje viously described successful closure of ASD in several patients (10). This tech ‑ nology is undoubtedly more comforta‑ ble for a patient but it does not provide a 3‑D image and is unfortunately associa‑ ted with significant additional cost. We have developed considera‑ ble experience in PFO (9,11) and ASD closure (10) which has been as we write, successfully performed in 213 patients using different closure devices (Table 1). In PFO patients, who had concomi‑ tant ISA in 33 %, a 6‑ month closure rate assessed by TEE was in excess of 90 % without device embolization/thrombo‑ sis/pericardial effusion (9). In ASD pati‑ ents, successful closure was achieved in all patients of whom 3 (13 %) had mul‑ tiple defects requiring two Amplatz occluders. Only 3 of 140 PFO patients (2.1 %) presented with combined PFO/ASD defects. In one patient, both defect were successfully closed using only one, but larger device resulting in complete closure also at 6 month TEE. In a second patient, FlatStent for PFO and Amplatz for ASD were used. Despite complete initial closure, follow up TEE after 6 months demonstrated residual shunting through the PFO tunnel for which antiaggregation therapy without additional closure attempt has been ad‑ vised. Also herein reported patient with only trivial shunt at 6 months will be further followed to document eventual increase in shunting. These observations indicate importance of systematic TEE follow up to document possible remo‑ deling of the tunnel and septum after closure device(s) over time with reoc‑ currence of shunts. We recently demon‑ strated such dynamic changes after in‑ ‑tunnel PFO closure using FlatStent (11). In conclusion, our patient illustrates the capability of current percutaneous closure procedures to achieve optimal defect closure even in complex anato‑ mies thereby avoiding open heart surge‑ ry. It also emphasizes the need for close intraprocedural cooperation between interventional cardiologist and echocar‑ diographist who now, with 3‑D TEE, has a very valuable tool to guide percutaneo‑ us structural interventions. The patient gives his consent to the publication. Table 1: Percutaneous structural interventions at MC Medicor between October 2006 and end of December 2017. Number of patients PFO closure 181 ASD closure-single defect 26 ASD closure-multiple defects 3 PFO + ASD closure 3 VSD closure 1 Left atrial appendage closure 29 TAVI 11 Percutaneous paravalvular leak closure 1 Alcohol septal ablation for HOCM 8 All percutaneous structural interventions 262 Legend: PFO = patent foramen ovale; ASD = atrial septal defect type secundum; VSD = ventricular septal defect; TAVI = transcatheter aortic valve implantation; HOCM = hypertrophic obstructive cardiomyopathy Percutaneous closure of interatrial defects 581 KliniČni PriMer References 1. Dolenc j , Koželj M, Prokšelj K. najpogostejše zmote pri obravnavi odraslih bolnikov s prirojenimi srčnimi napakami. Zdrav Vestn. 2012;81:312–9. 2. Berger F, Ewert P . Atrial septal defect: waiting for symptoms remains an unsolved medical anachronism. Eur Heart j . 2011 Mar;32(5):531–4. https://doi.org/10.1093/eurheartj/ehq377 PMiD:20971748 3. Humenberger M, r osenhek r, Gabriel H, rader F, Heger M, Klaar U, et al. Benefit of atrial septal defect closure in adults: impact of age. eur Heart j . 2011 Mar;32(5):553–60. https://doi.org/10.1093/eurheartj/ ehq352 PMiD:20943671 4. Dolenc j , cerar A, c vijić M, Prokšelj K, Mirta K. l ong-term follow up after transcatheter closure of atrial septal defect and patent foramen ovale in adults. Zdrav Vestn. 2013;82:809–17. 5. carroll jD, Saver jl, Thaler De, Smalling r W, Berry S, MacDonald l A, et al.; reSPec T investigators. closure of patent foramen ovale versus medical therapy after cryptogenic stroke. n engl j Med. 2013 Mar;368(12):1092– 100. https://doi.org/10.1056/nejMoa1301440 PMiD:23514286 6. Meier B, Kalesan B, Mattle HP, Khattab AA, Hildick-Smith D, Dudek D, et al.; Pc Trial investigators. Percuta- neous closure of patent foramen ovale in cryptogenic embolism. n engl j Med. 2013 Mar;368(12):1083–91. https://doi.org/10.1056/nejMoa1211716 PMiD:23514285 7. Furlan Aj , r eisman M, Massaro j , Mauri l, Adams H, Albers GW, et al.; cloSUre i investigators. closure or medical therapy for cryptogenic stroke with patent foramen ovale. n engl j Med. 2012 Mar;366(11):991–9. https://doi.org/10.1056/nejMoa1009639 PMiD:22417252 8. Webb G, Gatzoulis MA. Atrial septal defects in the adult: recent progress and overview. circulation. 2006 oct;114(15):1645–53. https://doi.org/10.1161/ circ Ul ATionAHA.105.592055 PMiD:17030704 9. Černič Šuligoj n, Zorc M, Grad A, Kar S, noč M. Perkutano zapiranje ovalnega okna-izkušnje z novejšimi tipi zapiral. Slov Kardiol. 2011;8:13–7. 10. Černič Šuligoj n, Zorc M, Kar S, noč M. Perkutano zapiranje defekta preddvornega pretina tipa sekundum pri odraslih bolnikih- naše izkušnje. Slov Kardiol. 2012;9:4–9. 11. noc M, cernic Suligoj n, Zorc M, Kar S. in-tunnel closure of patent foramen ovale with FlatStent. Kardiol Pol. 2015 Mar 3. https://doi.org/10.5603/KP.a2015.0026.