atrial septal defect (ASD)
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Etiology
Epidemiology
- other than bicuspid aortic valve, most frequent form of congenital heart disease in adults
Pathology
- left to right shunt lesions may produce elevated pulmonary pressure & pulmonary vascular disease (infrequent)[3]
- right heart failure may occur by 3rd-4th decade of life
- shunting occurs during diastole
- initially left to right
- right atrial enlargement occurs
- increased pulmonary blood flow may lead to pulmonary hypertension
- pulmonary hypertension may cause decreased right ventricular compliance & shunt reversal
- shunt reversal (right to left) or Eisenmenger's syndrome results in cyanosis
Genetics
- defects in NKX2-5 associated with atrial septal defect with atrioventricular conduction defects
Clinical manifestations
- dyspnea, fatigue
- fixed splitting of S2
- nearly pathognomonic for atrial septal defect
- increased P2 suggests pulmonary hypertension
- jugular venous distension
- early-mid systolic murmur at right upper sternal border
- large left to right shunt may cause tricuspid diastolic flow rumble
- murmur often found in pregnancy from increased intravascular volume[3]
- prominent right parasternal impulse
- right ventricular heave[3]
- may be felt at left sternal border
- atrial arrhythmia may be presenting symptom[3] (although generally absent)
- clubbing of fingers & toes[3]
- *distinguish from Eisenmenger's syndrome - clubbing of toes
Diagnostic procedures
- electrocardiogram
- may show arrhythmia
- ostium secundum ASD:
- right axis deviation
- incomplete right bundle branch block[3]
- ostium primum ASD:
- sinus venosus ASD: abnormal P axis
- ostium secundum ASD:
- may show arrhythmia
- transthoracic echocardiogram, US of atrial septum
- right ventricular enlargement
- increased pulmonary artery flow
- cardiac catheterization
- pulmonary hypertension[3]
- identification & closure of defect
Radiology
- chest X-ray
- right heart may be enlarged
- prominent pulmonary artery
- increased pulmonary vascularity
Complications
- right ventricular enlargement, cor pulmonale
- mitral regurgitation, tricuspid insufficiency
- pulmonary hypertension (rare)
- atrial fibrillation
- thromboembolism, stroke
- residual shunt post surgical repair (rare)[3]
Management
- surgical closure[3][4]
- indications
- symptomatic patients, dyspnea, right heart failure
- patients with pulmonary/systemic blood flow of > 1.5[3]
- atrial arrhythmias
- contraindications: shunt reversal (right to left)
- closure contraindicated if pulmonary vascular resistance is very high & shunt reversal is present[3]
- reported to increase survival in patients > 40 years of age
- percutaneous device closure for ostium secundum ASD
- open surgical closure for ostium primum ASD & mitral valve defects[3]
- asymptomatic patients without right heart enlargement with small atrial septal defects (pulmonary/systemic blood flow of < 1.5) can be followed clinically & with echocardiography[3]
- aspirin + clopidogrel may reduce number of migraine headaches relative to aspirin alone after surgical closure[5]
- indications
- pharmacologic therapy
- treatment of hypertension
- treatment of coronary artery disease
- prognosis of patients with significant right to left shunts is poor (even with surgery)
More general terms
More specific terms
- atrial septal defect type 2 (ASD2)
- atrial septal defect type 3 (ASD3)
- atrial septal defect type 4 (ASD3)
- atrial septal defect type 5 (ASD5)
- atrial septal defect type 6 (ASD6)
- persistent ostium primum (partial atrioventricular canal)
- persistent ostium secundum
- persistent sinus venosus
Additional terms
- Eisenmenger's syndrome
- ostium primum (interatrial foramen primum)
- ostium secundum (interatrial foramen secundum)
References
- ↑ DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 868
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 45-46
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
- ↑ 4.0 4.1 Humenberger M, Rosenhek R, Gabriel H et al Benefit of atrial septal defect closure in adults: impact of age. Eur Heart J. 2011 Mar;32(5):553-60 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20943671
- ↑ 5.0 5.1 Rodes-Cabau J et al Effect of Clopidogrel and Aspirin vs Aspirin Alone on Migraine Headaches After Transcatheter Atrial Septal Defect Closure. The CANOA Randomized Clinical Trial. JAMA. Published online November 09, 2015 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/26551304 <Internet> http://jama.jamanetwork.com/article.aspx?articleid=2469193
- ↑ Brida M, Chessa M, Celermajer D et al Atrial septal defect in adulthood: a new paradigm for congenital heart disease. Eur Heart J. 2022 Jul 21;43(28):2660-2671. PMID: https://www.ncbi.nlm.nih.gov/pubmed/34535989 Review.