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Cardiac CT in Mixed Type Atrial Septum Defects

Anita Sadeghpour, MD, FASE, FACC 1 ; and Fatemeh Moudi, MD 1, *
1 Rajaie Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran
*Corresponding author: Fatemeh Moudi, MD, Fellowship of Echocardiography, Echocardiography Department, Rajaie Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran. Tel: +98-9155121586, E-mail: drfatemehmoodi@yahoo.com.
Archives of Cardiovascular Imaging. 2014 February; 2(1): e18044 , DOI: 10.5812/acvi.18044
Article Type: Discussion; Received: Feb 1, 2014; Accepted: Feb 5, 2014; epub: Feb 17, 2014; ppub: Feb 2014

Keywords: Secundum Type Atrial Septal Defect (ASD); Sinus Venosus Type ASD; Partial Anomalous Pulmonary Venous Connection (PAPVC)

1. Introduction


A 38-year-old woman presented with dyspnea on exertion [New York Heart Association (NYHA) functional class II]. Physical examination showed no cyanosis or clubbing of the fingers or toes. Elevated jugular venous pressure, holo-systolic (II/VI) murmur at the left sternal border, and wide fixed S2 splitting were present. Transthoracic echocardiography revealed severe right ventricular enlargement with moderate to severely reduced systolic function, severe right atrial (RA) enlargement, and severe pulmonary artery hypertension (pulmonary artery pressure = 90 mm Hg). According to the computed tomography (CT) figures, presented below, what is your diagnosis?

2. Answer


A large secundum type atrial septal defect (ASD) associated with moderate size sinus venosus, superior vena cava (SVC) type ASD with partial anomalous pulmonary venous connection (PAPVC) of the right upper pulmonary vein (RUPV) to the RA.

3. Comment


The SVC type of sinus venosus defects is located immediately below the junction of the SVC and the RA and varies from small to non-restricted. The orifice of the SVC may override the defect, which is therefore bi-atrial (1).

The most common type of the ASD is ostium secundum in fossa ovalis location. Moreover, 10-15% of ostium secundum ASDs are associated with PAPVC, while 80-90% of the SVC type of sinus venosus defects are associated with the anomalous connection of the RUPV to the RA or the SVC (2); also interestingly in about 10% of patients with Sinus venosus atrial septal defect, an associated secundum type ASD is present (3).

Footnotes

Authors’ Contribution: The authors’ contribution in preparing of this interesting image is equal.
Financial Disclosure: There is no financial disclosure.

References


  • 1. Webb G, Gatzoulis MA. Atrial septal defects in the adult: recent progress and overview. Circulation. 2006;114(15):1645-53. [DOI] [PubMed]
  • 2. Oliver JM, Gallego P, Gonzalez A, Dominguez FJ, Aroca A, Mesa JM. Sinus venosus syndrome: atrial septal defect or anomalous venous connection? A multiplane transoesophageal approach. Heart. 2002;88(6):634-8. [PubMed]

Figure 1.

Figure 2.