Advertisement
Mayo Clinic Proceedings Home

Patent Ductus Arteriosus Can Coexist With Pulmonary Sequestration in Adults, Conditioning Pulmonary Hypertension

  • Guillermo Cueto-Robledo
    Correspondence
    Correspondence: Address to Guillermo Cueto-Robledo, MD, Pulmonary Circulation Clinic, Hospital General de Mexico “Dr Eduardo Liceaga,” Mexico City, Mexico
    Affiliations
    Pulmonary Circulation Clinic, Hospital General de Mexico “Dr Eduardo Liceaga,” Mexico City, Mexico

    Cardiorespiratory Emergencies, Hospital General de Mexico “Dr Eduardo Liceaga,” Mexico City, Mexico

    Faculty of Medicine, National Autonomous University of Mexico, Mexico City, Mexico
    Search for articles by this author
  • Carlos Narvaez-Oriani
    Affiliations
    Department of Cardiology, ISSSTE Hospital General Tacuba, Mexico City, Mexico
    Search for articles by this author
  • Ernesto Roldan-Valadez
    Correspondence
    Ernesto Roldan-Valadez, MD, MSc, DSc, Directorate of Research, Hospital General de Mexico "Dr Eduardo Liceaga". Dr. Balmis 148, Colonia Doctores, Delegacion Cuauhtemoc, 06726 Mexico City, Mexico.
    Affiliations
    Directorate of Research, Hospital General de Mexico “Dr Eduardo Liceaga,” Mexico City, Mexico

    Department of Radiology, I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia
    Search for articles by this author
      A woman in her early 40s attended the hospital for dyspnea. Her chest radiograph revealed cardiomegaly, prominence of the cone of the pulmonary artery, and hyperflow (Figure A). The electrocardiogram revealed growth of the atrium and right ventricle. The chest computed tomography angiogram depicted right intralobar pulmonary sequestration (PS) with a patent ductus arteriosus (Figure B); the blood supply of the sequestrum originated from the abdominal aorta with venous drainage toward the pulmonary vein and the left atrium (Figure C and D). Right heart catheterization found severe precapillary pulmonary hypertension (PH) (Table).
      • Cueto-Robledo G.
      • Jurado-Hernandez M.Y.
      • Navarro-Vergara D.I.
      • et al.
      Cateterismo cardiaco derecho en hipertensión arterial pulmonar.
      The echocardiogram reported PH, a jet of tricuspid regurgitation (Supplemental Figure A, available online at http://www.mayoclinicproceedings.org), and the dilation of the heart’s right chambers (Supplemental Figure B).
      Figure thumbnail gr1
      FigureA, Posteroanterior chest radiograph shows a prominent pulmonary artery cone with an increased vascular pattern and an aortic atheroma. B, Sagittal plane, computed tomography pulmonary angiogram showing a patent calcified ductus arteriosus (white arrow). C and D, Three-dimensional reconstructions show the intralobar sequestration artery originating from the abdominal aorta (white arrows) and the patent ductus arteriosus (white asterisk).
      TableRHC Depicted Severe Precapillary Pulmonary Arterial Hypertension in the Upper Row; Normal RHC Numbers Are Shown in the Lower Row
      VariableHR (beats/min)RA (mm Hg)RV s/d (mm Hg)PAP s/d/m (mm Hg)CI (L/min per meter squared)PCWP (mm Hg)PVR (WU)Pvo2 (mm Hg)Svo2Pao2 (mm Hg)Sao2
      Patient10411123/8122/67/873.51114.83861%5180%
      Normal60/90625/425/2/142.4-44/12<335/4575%>65>90%
      CI, cardiac index; HR, heart rate; Pao2, partial pressure in oxygen; PAP, pulmonary arterial pressure; PCWP, pulmonary capillary wedge pressure; Pvo2, mixed venous oxygen tension; PVR, pulmonary vascular resistance; RA, right atrium; RHC, right heart catheterization; RV, right ventricle; s/d, systolic/diastolic; s/d/mean, systolic/diastolic/mean; Sao2, oxygen saturation level in arterial blood; Svo2, mixed venous oxygen saturation; WU, Wood units.
      Data extracted from Manual para la realización de cateterismo cardíaco derecho.
      • Cueto-Robledo G.
      • Jurado-Hernandez M.Y.
      • Navarro-Vergara D.I.
      • et al.
      Cateterismo cardiaco derecho en hipertensión arterial pulmonar.
      Pulmonary sequestration corresponds to 0.15% to 6.4% of all congenital pulmonary malformations, an abnormal mass of tissue that does not communicate with the bronchial tree or the pulmonary arteries.
      • Savic B.
      • Birtel F.J.
      • Tholen W.
      • Funke H.D.
      • Knoche R.
      Lung sequestration: report of seven cases and review of 540 published cases.
      Patent ductus arteriosus and PH in the intralobar PS have not been described.
      • Felker R.E.
      • Tonkin I.L.
      Imaging of pulmonary sequestration.
      This case illustrates the association of intralobar PS and the effect of PH due to congenital heart disease, which was not surgically treated because of severe pulmonary vascular disease; our patient was maintained on sildenafil and macitentan.

      Potential Competing Interests

      The authors report no competing interests.

      Supplemental Online Material

      References

        • Cueto-Robledo G.
        • Jurado-Hernandez M.Y.
        • Navarro-Vergara D.I.
        • et al.
        Cateterismo cardiaco derecho en hipertensión arterial pulmonar.
        in: Cueto-Robledo G. Navarro-Vergara D.I. Manual para la realización de cateterismo cardíaco derecho. 1st ed. Permanyer Mexico, 2020: 39-54
        • Savic B.
        • Birtel F.J.
        • Tholen W.
        • Funke H.D.
        • Knoche R.
        Lung sequestration: report of seven cases and review of 540 published cases.
        Thorax. 1979; 34: 96-101https://doi.org/10.1136/thx.34.1.96
        • Felker R.E.
        • Tonkin I.L.
        Imaging of pulmonary sequestration.
        AJR Am J Roentgenol. 1990; 154: 241-249