Pulmonary arterial hypertension after atrial septal defect closure: a case report

https://doi.org/10.19106/JMedSci005304202111

Lucia Kris Dinarti(1*), Dyah Wulan Anggrahini(2), Muhammad Reyhan Hadwiono(3), Abdul Majid Halim Wiradhika(4), Vera Dewanto(5), Anggoro Budi Hartopo(6)

(1) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(2) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(3) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(4) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(5) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(6) Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta
(*) Corresponding Author

Abstract


Most patients with congenital heart disease (CHD) who underwent successful shunts defect repair can be remained asymptomatic for years. It leads to a high number of losses to follow-up after patients discharge. After closure, pulmonary hypertension (PH) prevalence seems to be high and associated with increased morbidity and mortality. We reported a 55 y.o. female diagnosed with pulmonary arterial hypertension (PAH) and atrial fibrillation (AF) 31 years after atrial septal defect (ASD) closure by surgery, who never had routine follow-up evaluation because she remained asymptomatic for years. Physical examination revealed heart enlargement with irregular rhythm and pan systolic murmur in the fourth left sternal border. Electrocardiogram showed AF normal ventricular response, right axis deviation and suggestive for right ventricular hypertrophy. Laboratory testing found the NT pro-BNP level was 2,476 pg/mL. The chest X-ray showed enlargement of the heart and was representative of PH. From echocardiography study, transthoracic and transoesophageal echocardiography, we found no sign of residual shunt. There were right atrial and right ventricular dilatation, severe tricuspid regurgitation (TR) and a high probability of PH with TR velocity of 4.46 m/s. Right heart catheterization concluded mean pulmonary arterial pressure 46 mmHg, flow ratio 1.1, and pulmonary artery resistance index 15.5 Woods unit.m-2. We highlight this case because of the high incidence of PH long after defect closure. The high number of lost to follow-up patients can lead to morbidity and mortality.


Keywords


atrial septal defect; follow-up after closure; pulmonary hypertension

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References


  1. Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007; 115(8):1039-50. https://doi.org/10.1161/CIRCULATIONAHA.105.592386
  2. Dinarti LK, Hartopo AB, Kusuma AD, Satwiko MG, Hadwiono MR, Pradana AD, et al. The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry: a descriptive study from single-center hospital registry of adult congenital heart disease and pulmonary hypertension in Indonesia. BMC Cardiovasc Disord 2020; 20(1):163. https://doi.org/10.1186/s12872-020-01434-z
  3. Lammers AE, Bauer LJ, Diller GP, Helm PC, Khaliq HA, Bauer UMM, et al. Pulmonary hypertension after shunt closure in patients with simple congenital heart defects. Int J Cardiol 2020; 308:28-32. https://doi.org/10.1016/j.ijcard.2019.12.070
  4. D'Alto M, Romeo E, Argiento P, D’Andrea A, Vanderpool R, Correra A, et al. Accuracy and precision of echocardiography versus right heart catheterization for the assessment of pulmonary hypertension. Int J Cardiol 2013; 168(4):4058-62. https://doi.org/10.1016/j.ijcard.2013.07.005
  5. Galiè N, McLaughlin VV, Rubin LJ, Simonneau G. An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J 2019; 53(1):1802148. https://doi.org/10.1183/13993003.02148-2018
  6. Humbert M, Montani D, Evgenov OV, Simonneau G. Definition and classification of pulmonary hypertension. Handb Exp Pharmacol 2013; 218:3-29. https://doi.org/10.1007/978-3-642-38664-0_1
  7. Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019; 53(1):1801913. https://doi.org/10.1183/13993003.01913-2018
  8. Oster M, Bhatt AB, Zaragoza-Macias E, Dendukuri N, Marelli A. Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 2019; 139(14):e814-30. https://doi.org/10.1161/CIR.0000000000000605
  9. Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J 2021; 42(6):563-645. https://doi.org/10.1093/eurheartj/ehaa554
  10. Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37(1):67-119. https://doi.org/10.1093/eurheartj/ehv317
  11. Akseer S, Horlick E, Vishwanath V, Hobbes B, Huszti E, Mak S, et al. Prevalence and outcomes of pulmonary hypertension after percutaneous closure of atrial septal defect: a systematic review and meta-analysis. Eur Respir Rev 2020; 29(15):200099. https://doi.org/10.1183/16000617.0099-2020
  12. Zwijnenburg RD, Baggen VJM, Geenen LW, Voigt KR, Roos-Hesselink JW, van den Bosch AE. The prevalence of pulmonary arterial hypertension before and after atrial septal defect closure at adult age: A systematic review. Am Heart J 2018; 201:63-71. https://doi.org/10.1016/j.ahj.2018.03.020
  13. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Brobrg CS, Colman JM, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019; 73(12):1494-563. https://doi.org/10.1016/j.jacc.2018.08.1028
  14. van Riel AC, Blok IM, Zwinderman AH, Wajon EMCJ, Sadee ASJM, Bakker-de Boo M, et al. Lifetime Risk of Pulmonary Hypertension for All Patients After Shunt Closure. J Am Coll Cardiol 2015; 66(9):1084-86. https://doi.org/10.1016/j.jacc.2015.06.1318
  15. Lau EM, Manes A, Celermajer DS, Galiè N. Early detection of pulmonary vascular disease in pulmonary arterial hypertension: time to move forward. Eur Heart J 2011; 32(20):2489-98. https://doi.org/10.1093/eurheartj/ehr160



DOI: https://doi.org/10.19106/JMedSci005304202111

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Copyright (c) 2021 Lucia Kris Dinarti, Dyah Wulan Anggrahini, Muhammad Reyhan Hadwiono, Abdul Majid Halim Wiradhika, Vera Dewanto, Anggoro Budi Hartopo

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