Paeds · cardiology
Atrial septal defect and partial anomalous pulmonary venous return
Also known as atrial septal defect · ASD · secundum ASD · partial anomalous pulmonary venous return · PAPVR · partial anomalous pulmonary venous connection · PAPVC · scimitar syndrome
A fellowship approach to atrial septal defect and partial anomalous pulmonary venous return: the auscultatory signature of a wide fixed split second heart sound, the left-to-right shunt that silently loads the right ventricle, the echo that confirms and quantifies, and the device-versus-surgery closure decision that must be made before pulmonary vascular disease becomes irreversible.
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Target exams
Red flags
- A wide, fixed, split second heart sound is the auscultatory signature of a significant ASD — never dismiss it as an innocent murmur
- Do not close an ASD if the pulmonary vascular resistance is high and the shunt has reversed — closing an Eisenmenger defect kills
- A sinus venosus ASD is almost always accompanied by partial anomalous pulmonary venous return, so recognise it on echo and image with CT or MRI before surgery
- An infant presentation of scimitar syndrome carries severe pulmonary hypertension and a high mortality — refer urgently
- Primum ASD and atrioventricular septal defect sit on a spectrum — always look for and repair an atrioventricular valve cleft at the time of surgical closure
- Aspirin or anticoagulation is given for three to six months after device closure because endothelialisation takes time and thrombus can form on the device
Life stages
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Board mappings
- Cardiology - recognise and investigate common congenital heart lesions
- General and Community Paediatrics
- Growth and Development
- Cardiology - recognise, investigate and manage atrial septal defect and anomalous pulmonary venous return
- Congenital heart disease - interpret echocardiography and plan closure
- Renewed curriculum for first-year trainees from 2027 - Learning goal 6: Clinical management - essential general paediatrics
- Cardiology 10-12
- Neonatal and congenital cardiac 10-12
- Clinical Applications
- Long Cases
- Short Cases
- 8. Cardiology - congenital heart disease including septal defects
- 1. Neonates and infants with cardiology: ASD and anomalous venous return
- 4. Professional skills and knowledge: Patient management
- Foundation of Practice (FOP)
- Theory and Science (TAS)
- Applied Knowledge in Practice (AKP)
- Clinical
- History
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- General Pediatrics Content Outline - D15 Cardiology (5%)
- General Pediatrics Content Outline - D01 Newborn (3%)
- General Pediatrics Content Outline - Universal Task 4: Management and Treatment
- Patient Care 4: Clinical Reasoning
- Patient Care 5: Patient Management
- Medical Knowledge 1: Congenital anomalies and genetics
- Systems-Based Practice 1: Patient Safety
- Medical Expert
- Pediatrics: Foundations EPA #8 - Communicating assessment findings and management plans to patients and/or families
- Pediatrics: Cardiology applied domain
A four-year-old is referred for an asymptomatic heart murmur heard at a preschool check; or a twelve-year-old presents with exertional dyspnoea and two episodes of pneumonia in a year; or a young woman in her twenties is investigated for new atrial fibrillation and is found to have a large secundum defect. The fellowship task in each is the same: confirm with echocardiography, quantify the shunt, exclude pulmonary vascular disease, and arrange closure before the right ventricle declares itself. [5] [1]
Remember the pathway as four moves. Recognise the wide, fixed, split second heart sound and the soft pulmonary murmur. Confirm with echocardiography, measuring defect size, rims, right ventricular size, and the shunt ratio. Close with a device for secundum defects with adequate rims, or surgery for primum, sinus venosus, coronary sinus, or partial anomalous venous return. Protect the patient with lifelong follow-up because arrhythmia and pulmonary hypertension surveillance never ends, and never close a defect that has progressed to Eisenmenger. [1] [6]
Overview & Definition
An atrial septal defect is a hole in the atrial septum that allows shunting between the atria, and partial anomalous pulmonary venous return is a drainage error in which one or more pulmonary veins empty into the systemic venous circulation instead of the left atrium. The two conditions are paired here because they produce the same haemodynamic problem — a left-to-right shunt that volume-loads the right heart — and because they so often coexist, particularly with sinus venosus defects. [5] [4]
The fellowship framing turns on a single haemodynamic truth. Atrial septal defects are pressure-low, volume-high shunts, unlike the pressure-driven shunts of a ventricular septal defect. The right atrium is thin and compliant, so it accepts extra volume without a big pressure rise, the right ventricle dilates, and the pulmonary circulation is flooded with blood rather than pressure. This is why the disease is silent for so long, and why the damage accumulates insidiously. [5] [2]
The clinical importance of the condition comes from what happens if it is left untreated. A significant defect causes right ventricular dilation, exercise intolerance, recurrent respiratory infections, atrial arrhythmia, paradoxical embolism, and ultimately pulmonary vascular disease with Eisenmenger physiology. Closure of a significant defect in childhood or early adulthood prevents all of these, and the right ventricle usually remodels back to normal. The task is to find the defect, measure it, and close it in time. [1] [5]
References10ShowHide
- [1]Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 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. J Am Coll Cardiol, 2019.PMID 30121239
- [2]Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J, 2021.PMID 32860028
- [3]Feltes TF, Bacha E, Beekman RH 3rd, Cheatham JP, Feinstein JA, Gomes AS, Hijazi ZM, Ing FF, de Moor M, Morrow WR, Mullins CE, Taubert KA, Zahn EM. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation, 2011.PMID 21536996
- [4]Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules JW, Graham TP Jr, Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GD. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol, 2008.PMID 19038677
- [5]Geva T, Martins JD, Wald RM. Atrial septal defects. Lancet, 2014.PMID 24725467
- [6]Alkashkari W, Albugami S, Hijazi ZM Current practice in atrial septal defect occlusion in children and adults. Expert Rev Cardiovasc Ther, 2020.PMID 32441165
- [7]Aristizabal AM, Guzmán-Serrano CA, Mondol-Villamil NV, et al. Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary care center. Int J Cardiovasc Imaging, 2024.PMID 38634941
- [8]Sehgal A, Loughran-Fowlds A Scimitar syndrome. Indian J Pediatr, 2005.PMID 15812123
- [9]C S SL, Sharma K, Manaswini M, et al. Holt-Oram Syndrome With Atrial Septal Defect. Cureus, 2024.PMID 39156428
- [10]Egidy Assenza G, Krieger EV, Baumgartner H, et al. AHA/ACC vs ESC Guidelines for Management of Adults With Congenital Heart Disease: JACC Guideline Comparison. J Am Coll Cardiol, 2021.PMID 34736567