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Uncommon cause of late onset anasarca after cardiac surgery
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  1. Chintan Bhatt1,
  2. S Radhakrishnan2,
  3. Anupama Nair2
  1. 1Fortis Escorts Heart Institute, New Delhi, India
  2. 2Department of Pediatric Cardiology, Fortis Escorts Heart Institute, New Delhi, India
  1. Correspondence to Dr Chintan Bhatt, Fortis Escorts Heart Institute, Okhla Road, New Delhi 110025, India; chintan18bhatt{at}yahoo.co.in

https://doi.org/10.1136/heartasia-2015-010697

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    A 10-year-old girl presented with a history of having loose stools and generalised swelling for a 1-month period. She had complex congenital cyanotic heart disease—double outlet right ventricle with anatomy unsuitable for two-ventricle repair. She had undergone Fontan surgery 3 years previously. Fontan completion was staged after pulmonary artery (PA) banding and bidirectional Glenn surgery. Her laboratory parameters showed hypoproteinemia with hypocalcaemia (serum protein 3.4 gm/dL, serum albumin 1.3 gm/dL, serum calcium 5.6 mg/dL). The patient's clinical condition was highly suggestive of a particular syndrome and she was started on treatment for that condition. Her echocardiogram showed significant haemodynamic findings (figure 1). She was referred for cardiac catheterisation and a percutaneous intervention was performed (figure 2A, B). Following the intervention, she improved dramatically with subsidence of symptoms and signs within 3 days.

    Figure 1
    Figure 1

    Color Doppler echocardiography image with anterior tilt.

    Figure 2
    Figure 2

    (A, B) Fluoroscopy images with cranial 30° view.

    Question

    What is the significant unexpected haemodynamic finding identified in this patient's echocardiographic image (figure 1)?

    1. Ventricular septal defect (VSD) shunt

    2. Atrial septal defect (ASD) shunt

    3. Antegrade flow in the right ventricular outflow tract (RVOT)

    4. Left ventricular outflow tract obstruction (LVOTO)

    Answer

    This girl is suffering from protein losing enteropathy (PLE) due to a failing Fontan circuit.

    This four chamber echocardiographic view with anterior tilt shows antegrade flow through the PA which had previously been banded. Such flow is detrimental in Fontan physiology and can have serious haemodynamic consequences.1 ,2 Antegrade flow may increase PA pressures and lead to increased resistance in the Fontan circulation, which in turn may increase resistance in the mesenteric circulation. This may contribute to the development of PLE. On cardiac catheterisation the patient had a high mean PA pressure (17 mm Hg). Therefore, option 3 is the correct answer.

    The patient underwent device (Amplatzer duct occluder) closure of the RVOT through a Glenn shunt (figure 2A, B). Understanding the pathophysiology may be useful in the management of PLE in Fontan physiology (figure 3).3

    Figure 3
    Figure 3

    Algorithm protocol for management of patients with protein losing enteropathy. GI, gastrointestinal.

    A large VSD is part of the primary condition, and the ASD, if flowing right to left, may simply reflect the elevated pressure in the system (so options 1 and 2 are not correct). There is no left ventricular outflow obstruction (so option 4 is not correct).

    References

    1. ↵
      1. Rychik J,
      2. Goldberg D,
      3. Rand E, et al
      . End-organ consequences of the Fontan operation: liver fibrosis, protein-losing enteropathy and plastic bronchitis. Cardiol Young 2013;23:831–40. doi:10.1017/S1047951113001650
      OpenUrlCrossRefPubMed
    2. ↵
      1. Tanamati C,
      2. Guimarães VA,
      3. Penha JG, et al
      . Fontan postoperative complication: antegrade pulmonary flow. Rev Bras Cir Cardiovasc 2011;26:137–9. doi:10.1590/S0102-76382011000100025
      OpenUrlPubMed
    3. ↵
      1. Rychik J
      . Protein-losing enteropathy after Fontan operation. Congenit Heart Dis 2007;2:288–300. doi:10.1111/j.1747-0803.2007.00116.x
      OpenUrlCrossRefPubMed
    View Abstract

    Footnotes

    • Contributors CB prepared the manuscript, AN helped in reviewing the manuscript, and SR re-reviewed the manuscript and helped in preparing the final draft.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; internally peer reviewed.