Typical Features of Carcinoid Heart Disease by 3D Transthoracic Echocardiography

by Alex Felix, MD1*; Denisa Muraru, MD, PhD2
1National Institute of Cardiology and Ecocardiografia Americas Medical City/Samaritano – Brazil
2Department of cardiac, thoracic and vascular sciences, University of Padua, School of Medicine, Padua, Italy

Clinical Case

  • 57-year-old man with type 2 diabetes mellitus
  • History of surgical removal of small bowel tumor (20 years before current presentation)
  • Diagnosed with recurrence of tumor and hepatic metastases
  • Progressive shortness of breath and edema of lower limbs (NYHA II with diuretics)

Two-dimensional PLAX view, showing a dilated right ventricle (RV) with paradoxycal septal motion

Two-dimensional 4-chamber view showing the characteristic aspect of carcinoid tricuspid valve (TV) disease: hyperecongenic, thickened and immobile leaflets, fixed in semi-open position

Two-dimensional color Doppler showing the severe, “free-flowing” tricuspid regurgitation with some antegrade flow acceleration due to mild degree of valve stenosis

Two-dimensional PSAX view focused on pulmonary valve (PV) showing the thickened and immobile leaflets, fixed in open position

Two-dimensional color Doppler confirming the typical steno-insufficiency of PV in carcinoid heart disease

A 3D full-volume dataset of the RV was acquired from the apical approach, in order to obtain more detailed information on valve morphology and also to quantitatate the RV volumes and function. In this multi-slice display of RV 3D dataset, there is a marked RV dilation with thickening of trabeculae and of TV subvalvular apparatus, as well as a severe thickening of TV leaflets which appear immobile, semi-open throughout the cardiac cycle.

RVEDV= 287 ml
RVESV=180 ml
EF=37%

Transthoracic 3D rendering of the TV and PV from the apical approach, showing the thickened and immobile TV leaflets en face, which enables to better appreciate the residual orifice of both valves. In the same view, the mitral valve (MV) appears unaffected.

On still 3D images of TV in the frame of maximal opening, one can obtain an estimate of TV area by planimetry (in this case TV area = 4.2 cm2).

Transthoracic 3D rendering of the TV from the parasternal approach (ventricular view), showing the thickened and stiff TV leaflets with “board-like” motion, and the thickening and retraction of subvalvular apparatus.

Transthoracic 3D rendering of the TV from the parasternal approach (atrial view), showing the thickened and immobile TV leaflets, and the TV open throughout the cardiac cycle; mitral valve (MV) and aortic valve (AV) are unaffected.

Transthoracic 3D rendering of the TV from the parasternal approach (longitudinal view), showing the thickening, shortening and fusion of subvalvular apparatus. In the right atrium, the orifices of inferior vena cava and coronary sinus are seen.

Important, a full-volume 3D dataset of optimal quality can serve for multiple purposes:

  • qualitative assessment (visual estimation of global RV size, shape, morphology and function in multi-slice display);
  • quantitative assessment (RV volumes, RV ejection fraction, TV area etc);
  • dynamic anatomy and function of cardiac valves seen en face
  • rapid appreciation of morphology of entire TV complex (including subvalvular apparatus)

Key Messages

2D echocardiography:

  • Revealed the characteristic aspect of carcinoid valve disease affecting both tricuspid and pulmonary valves
  • Was enough to establish the diagnosis of carcinoid heart disease

3D echocardiography:

  • Provided unique anatomic insights for a better understanding of carcinoid disease features
  • Provided realiable quantitative information on RV volumes and ejection fraction
  • Enabled the direct measurement of residual TV area, making possible to evaluate the presence and severity of TV stenosis despite the associated severe regurgitation (when Doppler gradients are not useful).

References

  • Muraru D, Badano LP, Sarais C et al. Evaluation of tricuspid valve morphology and function by transthoracic three-dimensional echocardiography. Curr Cardiol Rep 2011;13(3):242-249
  • Bhattacharyya S, Toumanakis C, Burke M et al. Features of carcinoid heart disease identified by 2- and 3-dimensional echocardiography and cardiac MRI. Circ Cardiovasc Imaging 2010;3(1):103-11

DrAlexFelix

Dr Alex Felix

Dr Alex Felix is a cardiologist from Rio de Janeiro (Brasil) with special interest in right heart pathologies. In 2013, he has attended the 3D Echo Intensive Course at Padua University, and ever since he continued his training in 3D transthoracic and transoesophageal echocardiography in his institution.