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Heart, Lung and Circulation

Septum Primum Malposition: An Uncommon Cause of Right Ventricular Volume Overload

  • Andrei George Iosifescu
    Correspondence
    Corresponding author at: Emergency Institute for Cardiovascular Diseases “Prof. C.C. Iliescu”, Sos.Fundeni 258, Bucharest 022322, Romania.
    Affiliations
    Carol Davila University of Medicine and Pharmacy, Bucharest, Romania

    Department of Cardiac Surgery, Emergency Institute for Cardiovascular Diseases “Prof.C.C. Iliescu”, Bucharest, Romania
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  • Roxana Enache
    Affiliations
    Carol Davila University of Medicine and Pharmacy, Bucharest, Romania

    Department of Cardiology, Emergency Institute for Cardiovascular Diseases “Prof.C.C. Iliescu”, Bucharest, Romania
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Published:September 10, 2022DOI:https://doi.org/10.1016/j.hlc.2022.08.008
      A 24-year-old woman was hospitalised for a systolic murmur with exertional fatigue. Transthoracic echocardiography found an enlarged right heart, mild pulmonary hypertension (39 mmHg systolic pressure), and an interatrial septum exhibiting a leftward deviation of its dorsocranial insertion (Figure 1A; Supplementary Video 1). On Doppler examination, partial anomalous pulmonary venous drainage (PAPVD) to the right atrium (RA) was suspected (Figure 1B; Supplementary Video 2). Transoesophageal echocardiography demonstrated an interatrial septum aneurysm and a patent foramen ovale with a right-to-left shunt (Figure 1C; Supplementary Video 3). No right pulmonary veins (PVs) were draining to the left atrium; some unusual RA Doppler flows (Figure 1D; Supplementary Video 4) increased suspicion of a PAPVD to the RA. Computed tomography angiography discovered all right PVs to be draining anomalously to the RA (Figure 2A, Supplementary Video 5). The patient had situs solitus and exhibited no sign of heterotaxy. Surgical repair was undertaken. All four right PVs ended on the posterior RA wall (Figure 2B). The interatrial septum consisting almost exclusively of the septum primum (SP) was found in a sagittal position (Figure 2C); after SP resection and a baffle technique repair, the fresh pericardium neoseptum adopted a normal frontal plane position (Figure 2D). The outcome of the case was uneventful.
      Figure thumbnail gr1
      Figure 1Echocardiographic findings.
      (A) TTE (transthoracic echocardiography): malposition of the interatrial septum (arrow). (B) TTE: drainage of right pulmonary veins to the right atrium (asterisks). (C) TOE (transoesophageal echocardiography): interatrial septum aneurysm and patent foramen ovale (arrow) with a small right-to-left shunt. (D) TOE Doppler exam: right atrial flow suggestive of partial anomalous pulmonary venous drainage (arrow).
      Figure thumbnail gr2
      Figure 2Anomalous RA (right atrial) drainage of all right PV (pulmonary veins).
      (A) Three-dimensional computed tomography angiography reconstruction: four PVs draining into the RA (asterisks). Intraoperative RA views: (B) four anomalous PVs ending in the RA; (C) sagittal position of septum primum due to its leftward malposition; (D) neoseptum placed in a frontal plane after baffle technique repair.
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