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ACI (Acta Cardiologia Indonesiana) (Vol.4 No.2): 117-121

Atrial Arrythmia in Patient: A Case Report and Review of Literature

Indah Paranita*, Lucia Kris Dinarti, Bambang Irawan

Department of and Vascular Medicine, Faculty of Medicine, Public Health and Nursing Universitas Gadjah Mada, Yogyakarta, Indonesia

*Corresponding author : Indah Paranita, MD, - email: [email protected] Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing Universitas Gadjah Mada, Yogyakarta, Indonesia Jalan Farmako no1 Sekip Utara, Yogyakarta 55281

Manuscript submitted: April 8, 2018; Revised and accepted: August 18, 2018

ABSTRACT (AF) and atrial flutter are the most common cardiac arrhythmias associated with atrial septal defects (ASD) in adult patients. The incidence could be as high as 52% in patients ages 60 years or more.Patient with congenital disease who developed atrial arrhythmias had a >50% increased risk. Nevertheless, studies regarding the pathophysiological mechanism underlying the high incidence of atrial fibrillation in adult patients with ASD remain relatively few. We reported a female 46 years referred to Sardjito hospital with chest discomfort and palpitation. ECG showed atrial flutter, 90 beat per minute, incomplete RBBB, RAD and RVH. Transthoracal shown ASD left to right shunt with diameter 1.2 -1.8 cm, LA, RA and RV dilatation, with normal systolic function. From right heart catetherization, the result is ASD High Flow Low Resistance, with (mPAP 44 mmHg).The consequences of left to right shunt across an ASD is RV and pulmonary overcirculation. Atrial arrhytmia are a common result of long standing right side heart volume and overload. The idea of combining ASD closure and arrhythmia intervention is another approach to consider.

Keywords : atrial fibrillation ; atrial septal defect; atrial arrytmia.

INTISARI Aritmia atrial yang paling sering terjadi pada pasien defek septum (DSA) adalah atrial fibrilasi dan atrial flutter. Insidensinya akan meningkat lebih dari 52% pada pasien dengan usia lebih dari 60 tahun keatas.11 Pasien dengan penyakit jantung kongenital yang mengalami aritmia atrial akan memiliki resiko kejadian stroke >50%. Namun, penelitian mengenai mekanisme patofisiologi yang mendasari tingginya kejadian atrial fibrilasi pada pasien DSA dewasa dengan ASD masih sangat sedikit. Kami laporkan seorang wanita 46 tahun yang dirujuk ke RS Sardjito dengan keluhan dada tidak nyaman dan berdebar. EKG menunjukkan gambaran atrial flutter, 90 kali per menit, RBBB inkomplit, RAD (right axis deviation) and RVH (right ventrikel hypertrophy). Ekokardiografimenunjukkan hasil ASD left to right shunt dengan diameter 1.2 -1.8 cm, dilatasi LA, RA and RV dengan fungsi sistolik normal. Hasil penyadapan jantung kanan menunjukkan ASD High Flow Low Resistance, dengan hipertensi pulmonal (mPAP 44 mmHg). Efek darialiran left to right shunt yang melewati DSA adalah overload volume di ventrikel kanan dan over sirkulasi pulmonal. Aritmia atrial adalah hasil yang didapatkan dari proses overload volume dan tekanan yang lama dari jantung kanan.Ide untuk mengkombinasikan penutupan DSA dengan intervensi aritmia adalah suatu pendekatan yang harus dipertimbangkan.

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Paranita et al.,2018 ACI (Acta Cardiologia Indonesiana) (Vol.4 No.2): 117-121

INTRODUCTION CASE PRESENTATION Atrial fibrillation (AF) and atrial A female 46 years old referred to flutter are the most common cardiac Sardjito hospital with chest discomfort and arrhythmias associated with atrial septal palpitation. Since 2 years before admission defects (ASD) in adult patients. The the patient often complained chest incidence could be as high as 52% in discomfort and palpitation. Then, the patient patients ages 60 years or more. Patient check to the primary health care, but the with congenital heart disease who complaints didn`t reduce. After 1 years developed atrial arrhythmias had a >50% there was no improvement, the patient was increased stroke risk. Atrial and ventricular referred to cardiologist in Kebumen district geometrical remodelling secondary to the hospital. Patient is performed physical intracardiac shunt promotes evolution of the examination, (ECG), electrical substrate, predisposing the chest x-ray and echocardiography. From all patient to atrial fibrillation and other of the examination, the cardiologist arrhythmias. Nevertheless, studies concludes that there is atrial septal defect regarding the pathophysiological and patient is referred to Cardiology mechanism underlying the high incidence of Department of Dr. Sardjito General Hospital atrial fibrillation in adult patients with ASD for further investigation. remain relatively few. Closure of an ASD reveales reduces the immediate and long-term holosystolic murmur 3/6 in lower sternal prevalence of atrial arrhythmias, but the border and wide fixed sound. An evidence suggests that patients remain at ECG revealed atrial flutter, incomplete right an increased long-term risk in comparison bundle branch block (RBBB), right axis with the normal population. deviation (RAD) and right ventricle hypertrophy (RVH) (figure 1).

Figure 1. An ECG showed atrial flutter, 90 x/minutes, incomplete RBBB, RAD, and RVH

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Paranita et al.,2018 ACI (Acta Cardiologia Indonesiana) (Vol.4 No.2): 117-121

Figure 2. Chest X-ray patient with atrial septal defect

Chest X-ray shows cardiomegaly, 15% of patients in older surveys.5 The link RVH, dilatation of left atrium and left between AF and ASD is fairly complex and ventricle, with prominent pulmonal artery. It entails modifications in electrophysiologic, indicates the present of pulmonary contractile and structural properties, at the hypertension. Figure 2 is chest X-ray from cellular and tissue level, of both atria, the patient. mainly due to chronic atrial stretch and Laboratory examination is within dilation.1 This association has important normal limit. Transthoracal clinical implications for the antithrombotic echocardiography (TTE) shows an ASD management of patients with previous with left to right shunt, ASD diameter is 1.2 stroke or transient ischaemic attack (TIA) -1.8 cm, left atrial, right atrial and right and an atrial septal defect. 5 ventricle dilatation with normal systolic Atrial flutter and fibrillation are function, left ventricle ejection fraction relatively common in ASD and their (LVEF) is 58 %. Intraventricular septal incidence increases steeply with age. The paradoks ( LV- D shaped) is observed, proneness to these arrhythmias is sistolic function of RV is decreased (TAPSE demonstrated by their common occurrence 11 mm), moderate tricuspid regurgitation, in patients undergoing surgical repair of the moderate pulmonnary hypertension, mild defect, in which postoperative, reversible mitral regurgitation, and mild pulmonary atrial arrhythmias occur in the majority of regurgitation. The result from right heart patients older than 35 years of age.6 The catetherization (RHC) is ASD with high flow prevalence atrial arrhythmias in ASD is < low resistance and pulmonary hypertension 2% in ages around 20 years old, 10-15% in (mPAP 44 mmHg). patients ages 40 years old and 20-40% in Patient is diagnosed as ASD patients ages 60 years old (measured pre- secundum with left to right shunt, high flow closure).4 low resistance, moderate pulmonary The estimated incidence of atrial hypertension NYHA functional class II and arrhythmia is approximately 10 % under the atrial flutter. The patient is treated with oral age of 40 years in unoperated adults, rising sildenafil 20 mg t.i.d, digoxin 0.125 mg q.i.d to at least 20% with increased age, and warfarin 2 mg q.i.d. pulmonary arterial pressure and systemic hypertension.4 To reduce the morbidity associated with atrial flutter and fibrillation, DISCUSSION the timely closure of atrial septal defects is Atrial septal defect (ASD) warranted.9 Additional studies have associated with atrial fibrillation (AF) in 10 – suggested that the incidence is increased in

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Paranita et al.,2018 ACI (Acta Cardiologia Indonesiana) (Vol.4 No.2): 117-121

male patients, those with chronic 0,5% at ages 50-59% years to almost 9% at obstructive pulmonary disease, reduced ages 80-89% years. Oliver et al. (2002) ejection fraction and hypertension.4 shows a significant increase in the The consequences of left to right prevalence of AF with each increased shunt across an ASD are RV volume decade of age in ASD. It shows the rate of overload and pulmonary overcirculation. 10 AF is <2% in patient <30 years, 15% in Atrial arrhytmia are a common result of long patients in their 50 years, and >60% in standing right side heart volume and patients in their 70 years.13 pressure overload. Changes in atrial Adult patients with an ASD and refractoriness, ionic currents and atrial atrial tachyarrhythmias get benefit from conduction properties (electrical defect closure. Closure of the defect may remodeling), along with tissue remodeling lead to regression of atrial flutter, whereas due to atrial fibrosis (structural remodeling) in older patients (>25 years) with AF, generate a favorable substrate for the restoration of normal hemodynamics initiation and domestication of AF.12 Chronic alone may not be sufficient.2 Device RA stretch because of ASD causes closure of ASD leads to improvement of electrical remodeling with modest increases both RV and LV function as well as in RA effective refractory period (ERP), reduction in LA volume. These conduction delay at the crista terminalis, hemodynamic improvements provide and sinus node dysfunction. Conduction insights into the symptomatic benefits delay at the crista terminalis persists gained in closure of ASD using the beyond ASD closure and may contribute to transcatheter approach. Whether a the long-term atrial arrhythmia substrate in reduction in LA volume predicts a reduction this condition. 12 in probability of later arrhythmias in a given On the other hand, as a result of patient remains to be determined.15 chronic dilatation of the RV, the LV in ASD The management of patients with patients is subject to paradoxical septal congenital heart disease and AF is similar motion, diminished impaired diastolic filling, to the management of AF encountered in diminished , and failed cardiac other forms of heart disease. Acute output recruitment in management involves anticoagulation and exercise.Nevertheless, the structural rate control as needed, followed by remodeling in the left atrium under chronic consideration of cardioversion to restore right atrial and right ventricular dilatation in sinus rhythm.8 Nonpharmacological ASD patients remains largely unknown.11 therapies for rhythm management include An ASD is associated with chronic LA catheter or surgical ablation.7 Successful stretch, which results in remodeling control of AF has been reported after characterized by LA enlargement, loss of combined RA and LA Maze procedures, myocardium, and electrical scar that results which may be considered in patients in widespread conduction abnormalities but requiring to correct with no change or an increase in ERP. hemodynamic issues. There are also very These abnormalities were associated with a limited report of radiofrequency catether greater propensity for sustained AF.14 ablation prior to device closure of ASD at a The prevalence of AF is almost separate procedur. In Santangeli study16 similar in surgical and non surgical patient. state that radiofrequency catheter ablation It relates to advanced age, left atrial of AF is feasible, safe, and effective in enlargement and grade of severity of mitral patients with ASD closure devices. and tricuspid regurgitation. Gender, Transseptal access can be obtained in anatomic type, defect size, Qp:Qs, portions of the native septum in the majority pulmonary artery pressure, right ventricular of cases. Direct transseptal puncture of the dimension, left ventricular systolic function device is feasible and safe but requires or previous surgical repair are not longer time for each transseptal access. significantly related to late AF. Age >25 years at the time of surgical closure of ASD CONCLUSION was the only predictor for AF in surgically treated patients independent of age at the An ASD are frequently associated time of study.13 Data from the Framingham with atrial arrhythmias and their incidence study indicate that its prevalence double increase steeply with age. Atrial Arrythmias with each advancing decade of age, from in ASD patients is mainly the byproduct of

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RA and LA dilatation. Changes in atrial Cardiovasc electrophysiol, 23:102- refractoriness, ionic currents and atrial 104. conduction properties (electrical 8. Darby A.E., DiMarco J.P. 2012. remodeling), along with tissue remodeling Management of atrial fibrillation in due to atrial fibrosis (structural remodeling) patients with structural heart disease. generate a favorable substrate for the Circulation, 125:945-957. initiation and domestication of atrial 9. Gatzoulis M.A., Freeman M.A., Siu tachyarrhythmias. For the patient was S.M., Webb G.D., Harris L. 1999. recommended surgical closure to improve Atrial arrhytmia after surgical closure exercise capacity, to prevents right to left of atrial septal defect in adults. N shunt, and to eliminates . Engl J Med, 340:839-846. 10. Gelernter-Yaniv L., Lorber A. 2007. The familial form of atrial septal REFERENCES defect. Acta Paediatr, 96: 726–730. 1. Blake G.E., Lakkireddy D. 2008. 11. Lin J.L. 2009. Atrial fibrillation in atrial Atrial septal defect and atrial septal defect : A problem of right fibrillation: the known and unknown. J atrium or left atrium?, Heart Rhytm, Atr Fibrillation, 1:45. 6:1007-1008. 2. Berger F., Vogel M., Kramer A. 12. Morton J.B., Sanders P., Vohra J.K., (1999). Incidence of atrial flutter/ Sparks P.B., Morgan J.G., Spence fibrillation in adults with atrial septal S.J., et al. 2003. Effect of chronic defect before and after surgery. Ann right atrial stretch on atrial electrical Thorac Surg, 68:75–78. remodeling in patients with an atrial 3. Bernstein D. 2007. ‘Congenital . Circulation, 107:1775- disease’. In: Kliegman RM, Behrman 1782. RE, Jenson HB, Stanton BF, Nelson 13. Oliver J.M., Gallego P., Gonzalez A., textbook of pediatrics, Edisi ke-18, Benito F., Mesa J.M., Sobrino J.A., Saunders Elsevier, Philadelphia,pp 2002, Predisposing condition for 1878-81. atrial fibrillation in atrial septal defect 4. Chubb H., Whitaker J., William S.E., with and without operative closure. Head C.E., Chung N.A.Y., Wright Am J Cardiol, 89:39-43. M.J., et al. 2014. Patophysiology and 14. Roberts-Thomson K.C., John B., management of arrhytmias Worthley S.G., Brooks A.G., Stiles associated with atrial septal defect M.K., Lau D.H., et al. 2009. Left atrial and patent . Arrhythm remodelling in patient with atrial Electrophysiol Rev, 3:168-172. septal defect. Heart Rhytm, 6:1000- 5. Camm A.J., Kirchhof P., Lip G.Y.H., 1006. Schotten U., Savelieva I., Ernst S., et 15. Salehian O., Horlick E., al. 2010, Guidelines for the Schwerzmann M., Haberer K., management of atrial fibrillation: the McLaughlin P., Siu S.C., et al. 2005. Task Force for the Management of Improvements in cardiac form and Atrial Fibrillation of the European function after transcatheter closure of Society of Cardiology (ESC). Eur secundum atrial septal defects. J Am Heart J, 31:2369-2429. Coll Cardiol, 45:499-504. 6. Craig R.J., Selzer A. 1968. Natural 16. Santangeli P., Di Biase L., Burkhardt history and prognosis of atrial septal J.D., Horton R., Sanchez J., Bailey defect. Circulation, 37:805-815. S., et al. 2011. Transeptal acces and 7. Crandall M.A., Daoud E.G., Daniels atrial fibrillation ablation guided by C.J., Kalbflleisch S.J. 2012. intracardiac echocardiography in Percutaneous radiofrequency patient with atrial septal closure catheter ablation for atrial fibrillation devices. Heart Rhythm, 8:1669-1675. prior to atrial septal defect closure. J

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