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Fifty-seven-year-old man with progressive dyspnoea
  1. Nikoloz Koshkelashvili,
  2. Priya Kohli,
  3. Jason Linefsky
  1. Division of Cardiology, Emory University School of Medicine, Atlanta, Georgia, USA
  1. Correspondence to Dr Nikoloz Koshkelashvili, Division of Cardiology, Emory University School of Medicine, Atlanta, GA 30322, USA; nkoshke{at}emory.edu

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Clinical introduction

A 57-year-old man from the USA with a history of atrial fibrillation and hypertension was evaluated for progressive dyspnoea and decreased energy. The patient denied a history of congestive heart failure, systemic symptoms or myocardial infarction. He was found to have a 3/6 holosystolic murmur radiating to the axilla. Transthoracic echocardiography (TTE) reported a reduced ejection fraction of 40%, mitral valve regurgitation and absence of interatrial shunts. The remaining cardiac valves were without abnormality. A nuclear stress test reported 18% fixed inferolateral defect. Subsequent coronary angiography was negative for obstructive coronary artery disease. To better evaluate the mitral valve apparatus, transoesophageal echocardiography (TEE) was performed.

Question

Which of the following best explains the TEE findings (figure 1) of the mitral valve?

  1. Carcinoid heart disease.

  2. Drug-induced valvular heart disease.

  3. Infective endocarditis.

  4. Ischaemic mitral regurgitation (MR).

  5. Rheumatic mitral valve disease.

Figure 1

(A) Transoesophageal echocardiography (TEE) mid-oesophageal view at 94° during diastole. (B) TEE mid-oesophageal view at 94° during systole. (C) TEE mid-oesophageal view at 0° with colour Doppler. (D) TEE 3D view of the mitral valve from the left atrium.

Answer: B

The mitral valve demonstrates leaflet thickening, thickening and shortening of chordae tendineae and restricted posterior leaflet mobility that led to severe regurgitation from incomplete coaptation (figure 1A–C). Morphological changes are similar in patients with rheumatic mitral valve and carcinoid heart diseases, but the patient did not have any commissural fusion (figure 1D) or mitral stenosis (online supplementary file), making rheumatic mitral valve disease less likely. The patient’s tricuspid and pulmonic valves were normal, and there was no interatrial shunt to explain mitral valve involvement from carcinoid. Morphological changes of the leaflet and subvalvular apparatus, along with absence of significant coronary artery disease, make ischaemic or secondary MR unlikely diagnosis.1 Findings of infective endocarditis, including valvular vegetation or leaflet perforation, are not seen here. There were no systemic findings presented to suggest active infection.

Supplemental material

The patient when questioned reported prior use of fenfluramine and phentermine for weight loss, confirming drug-induced valvular heart disease (DIVHD). DIVHD is a diagnosis of exclusion with a positive history of valvulopathic drug use. Pathologies such as rheumatic heart disease, carcinoid, Libman-Sacks endocarditis and congenital abnormalities should be ruled out during the evaluation.2–4 DIVHD is associated with the use of ergot alkaloids and appetite suppressants.4 Serotonin 5HT2B receptors are expressed on valves and medications acting as agonist on these receptors induce the pathological changes.5 The patient was evaluated for mitral valve replacement for a primary MR but due to the absence of social support he was deemed to be a poor surgical candidate and is managed by medical therapy.

References

Footnotes

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors

  • Competing interests None declared.

  • Patient consent Not required.

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