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Percutaneous Closure of Atrial Septal Defect EXPERT OPINION AND POSITION PAPER Percutaneous closure of atrial septal defect A consensus document of the joint group of experts from the Association of Cardiovascular Interventions and the Grown ‑Up Congenital Heart Disease Section of the Polish Cardiac Society Marek Grygier1, Robert Sabiniewicz2, Grzegorz Smolka3, Marcin Demkow4, Aleksander Araszkiewicz1, Danuta Sorysz5, Jacek Kusa6,7, Zenon Huczek8, Monika Komar9, Tadeusz Przewłocki10, Michał Hawranek11, Wojciech Wojakowski3, Jacek Białkowski12, Grażyna Brzezińska ‑Rajszys13, Stanisław Bartuś5 1 Chair and 1st Department of Cardiology, Poznań University of Medical Sciences, Poznań, Poland 2 Department of Pediatric Cardiology and Congenital Heart Disease, Medical University of Gdańsk, Gdańsk, Poland 3 Division of Cardiology and Structural Heart Diseases, Medical University of Silesia, Katowice, Poland 4 Department of Coronary and Structural Heart Diseases, National Institute of Cardiology in Warsaw, Warsaw, Poland 5 Department of Cardiology and Cardiovascular Interventions, Jagiellonian University Hospital in Kraków, Kraków, Poland 6 Pediatric Cardiology Department, Medical University of Silesia, Katowice, Poland 7 Pediatric Cardiology Department, Regional Specialist Hospital, Wrocław, Poland 8 1st Department of Cardiology, Medical University of Warsaw, Warsaw, Poland 9 Clinic for Heart and Vascular Diseases, Jagiellonian University Collegium Medicum, Institute of Cardiology, John Paul II Hospital in Kraków, Kraków, Poland 10 Jagiellonian University Collegium Medicum, Institute of Cardiology, John Paul II Hospital in Kraków, Kraków, Poland 11 3rd Department of Cardiology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland 12 Department of Congenital Heart Diseases and Pediatric Cardiology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland 13 Cardiology Department and Cardiovascular Interventional Laboratory, The Children’s Memorial Health Institute, Warsaw, Poland KEY WORDS ABSTRACT atrial septal defect, Atrial septal defect is the most common congenital heart lesion in adults. Although atrial septal defect closure congenital defects, is recommended in those with right heart enlargement or paradoxical embolism, data informing such indications consensus document, in adults are quite limited. This population has many unique characteristics and needs. In recent years, percutaneous closure significant progress has been made with regard to diagnostic modalities that facilitate the diagnostic workup of these patients. However, the decision ‑making process, especially in selected adult patients population (advanced age, various concomitant diseases, poor LV function [both systolic and diastolic], pulmonary hypertension, concomitant arrhythmias or multiple defects, or deficient rims) is still not easy. Available data are predominantly focused on imaging endpoints and short ‑term morbidity and mortality rather than long‑ ‑term. The evidence base for outcomes with or without defect closure comes from various studies with different observation periods. Moreover, the clinical experience in diagnosing and treating that subgroup of patients Correspondence to: is inhomogeneous between individual physicians (cardiologists, imaging specialists, operators) and between Prof. Marek Grygier, MD, PhD, small and large experience centers. In the view of the above, the joint group of experts from the Association Chair and 1st Department of Cardiology, Poznań University of of Cardiovascular Interventions and the Grown ‑Up Congenital Heart Disease Section of the Polish Cardiac Medical Sciences, ul. Długa 1/2, Society developed the following consensus opinion in order to standardize the principles of diagnosis, indications 61‑848 Poznań, Poland, for treatment, methods of performing procedures, and tenets of postoperative care in Poland. phone: +48 618549228, email: [email protected] Received: September 14, 2020. Background information on atrial septal communication. There are 5 types of anatom‑ Accepted: September 24, 2020. Published online: defect Atrial septal defect (ASD) is one of ic defects known: September 30, 2020. the most prevalent congenital heart defects • Secundum ASD (ASD II): 80% to even more Kardiol Pol. 2020; 78 (10): 1066‑1083 and may frequently remain undiagnosed un‑ than 90% of ASDs in the adult population; lo‑ doi:10.33963/KP.15629 1‑4 Copyright by the Polish Cardiac til adulthood. It is a consequence of various cated in the fossa ovalis region and its sur‑ Society, Warsaw 2020 embryological disorders that cause interatrial rounding tissue. 1066 KARDIOLOGIA POLSKA 2020; 78 (10) • Primum ASD: 10% to 15% of ASDs; also RV compliance (pulmonary arterial hyperten‑ known as partial atrioventricular septal de‑ sion [PAH], RV outflow tract obstruction), pul‑ fect or partial atrioventricular canal; locat‑ monary arteries stenosis or other RV diseases) ed near the crux of the heart, nearly always or tricuspid valve stenosis and / or regurgitation associated with a cleft in the anterior mitral may decrease left ‑to ‑right shunt or in some cas‑ valve leaflet. es cause shunt reversal which results in cyanosis. • Superior sinus venous defect: up to 5% of ASDs; located near the superior vena cava Prevalence and genetics Although ASDs are entry and often associated with an abnor‑ usually sporadic, some are inherited as autoso‑ mal connection of the right upper pulmonary mal dominant syndromes, and heart lesions vein to the superior vena cava or the right could be associated with other congenital de‑ atrium (RA). fects such as Holt–Oram syndrome in which • Inferior sinus venosus defect: less than 1% pre axial limb defects are caused by mutations of ASDs; located in the inferior portion of in the TBX5 gene on chromosome 12q24.1. Fam‑ the atrial septum, leading to an overriding ily forms of ASD with progressive atrioventric‑ of the inferior vena cava (IVC), often associ‑ ular blocks may suggest mutations or haploin‑ ated with anomalous connection of the right sufficiency of the Nkx2.5 gene on chromosome lower pulmonary vein to the IVC. 5, while familial ASD without conduction ab‑ • Unroofed coronary sinus defect or coronary normalities may be associated with GATA4 mu‑ sinus septal defect: less than 1% of ASDs; par‑ tations. Also, defects in genes responsible for tially or completely missing roof of coronary the formation of the atrial septum (eg, MYH6, sinus with lack of separation from the left TBX20) can lead to defects of the intra ‑atrial atrium (LA), often associated with a persis‑ septum. Exposure to teratogenes during preg‑ tent left superior vena cava that drains into nancy is another potential cause of ASD as ob‑ the coronary sinus. served in fetal alcohol syndrome.9‑12 Atrial septal defect may also occasionally be diagnosed together with other congenital car‑ Natural history, clinical presentation, diac abnormalities such as anomalous pulmo‑ and management of unoperated patients nary venous return, persistent left superior vena Patients with ASD usually remain asymptom‑ cava, pulmonary valve stenosis, or mitral valve atic until adulthood. Majority of them may de‑ prolapse. In some of these circumstances, such velop symptoms after the fourth or fifth decade as in patients with Ebstein anomaly and pul‑ of life, including: shortness of breath, poorer monary stenosis, the pathophysiology related exercise tolerance (dyspnea and fatigue), and to ASD is much more complex, and closure of supraventricular arrhythmias (atrial fibrilla‑ the defect could cause clinical deterioration.4‑6 tion, atrial flutter, atrial tachycardia, or sick si‑ Therefore, these recommendations address only nus syndrome). Sometimes paradoxical embo‑ isolated ASD and not ASD associated with com‑ li, heart failure or RV failure, and pulmonary plex cardiac defects. vascular disease can also be observed (5% to The shunt volume through ASD depends on 9% of patients). the defect size, right and left ventricular (LV) Studies describing unoperated patients with compliance, and left and right atrial pressures. large ASD (before 1980) revealed that nearly A simple ASD causes a left ‑to ‑right shunt (sig‑ 25% of these patients died before the age of 27 nificant when the defect size is relevant, usually years and 90% before the age of 60 years. Life ex‑ more than 10 mm) secondary to a higher compli‑ pectancy is still reduced if closure is performed ance of the right ventricle (RV) compared with after the age of 25 years, but survival is much the LV, and results in RV volume overload and better than previously assumed. Pulmonary ar‑ an increased pulmonary flow. This increase re‑ tery pressure (PAP) can be normal, but usually sults in late right heart failure with diminished increases with age. Severe increase of resistant functional capacity and fatigue. Atrial enlarge‑ PH is rare (less than 5%) and its development is ment, which develops with time, leads to fre‑ related to additional multifactorial factors sim‑ quent atrial arrhythmias (atrial fibrillation and ilar to idiopathic PH.13 After the fifth decade of atrial flutter) and higher risk of thromboembol‑ life, the severity of PH increases and tachyar‑ ic complications.6‑8 A clinically significant ASD rhythmias are more common. Symptoms vary may lead to late pulmonary hypertension (PH). widely with age at presentation and shunt vol‑ ASDs, like patent foramen ovale (PFO), may also ume, which means that symptoms alone can‑ permit paradoxical embolism resulting in stroke not guide therapy.1 or transient
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