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Pheochromocytoma multisystem crisis with transient stress cardiomyopathy due to ruptured pheochromocytoma
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  1. Kenji Sakamoto,
  2. Suano Kojima,
  3. Seiji Hokimoto,
  4. Hisao Ogawa
  1. Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan
  1. Correspondence to Dr Kenji Sakamoto, sakakenn{at}kumamoto-u.ac.jp

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Description

A 64-year-old woman was transported to emergency room, with intense breast pain with impending shock. She had been diagnosed with mammary carcinoma, and pheochromocytoma was discovered during preoperative examinations. Reduced ejection fraction (EF; 30%) with apical ballooning indicated acute coronary syndrome or Takotsubo cardiomyopathy,1 we inserted an intra-aortic balloon pump (IABP) after confirming intact coronary arteries via sequential coronary angiograms.

Seven hours after use of the IABP started, the sudden intense breast pain recurred, with mental confusion. Blood pressure (BP) increased to 190/110 mm Hg with 160 bpm tachycardia, so deep sedation and tracheal intubation were started as we suspected a pheochromocytoma multisystem crisis (PMC).2 Subsequently, BP decreased to 50 mm Hg and EF was 8% (video 1), causing us to introduce percutaneous cardiopulmonary support. Careful administration of prazosin was initiated, titrated to a dose of 5 mg/day. Sufficient improvement of cardiac function on day 6 (video 2) allowed withdrawal of the mechanical devices. Complicated liver and kidney failure as well as disseminated intravascular coagulation improved with multidisciplinary treatment under continuous deep sedation to avoid recurrence. Laparoscopic adrenalectomy on day 16 and sequential breast cancer resection were completed without awakening the patient. Macroscopic bleeding in the adrenal gland was found by the surgeon.

Histopathologically, adrenomedullary cell-like cells with abundant granular cytoplasm proliferation with nest formation were observed among severe hemorrhagic necrosis. Intense immunoreactivity for CgA diagnosed pheochromocytoma (figure 1).

Figure 1

Macroscopic and microscopic tumour findings.

Video 1

Ejection fraction was reduced to 8% on cardiac echo several hours after intra-aortic balloon pump insertion. This finding made us decide to introduce additional support with percutaneous cardiopulmonary support.

Video 2

Improved cardiac function on day 6.

Although the precise trigger was unknown, ruptured pheochromocytoma caused PMC with stepwise cardiogenic shock due to severe transient cardiomyopathy.

Learning points

  • Ruptured pheochromocytoma can cause pheochromocytoma multisystem crisis.

  • Continuous sedation and aggressive prazosin administration allowed adrenalectomy without recurrent crisis.

  • Immediate intervention is required after diagnosis of pheochromocytoma.

Acknowledgments

The authors thank Floyd Hodgins for his review and editing advice. They also thank Koji Enomoto for data collection.

References

View Abstract

Footnotes

  • Competing interests None declared.

  • Patient consent Obtained.

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