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CLINICAL STUDIES

Echocardiographic and biochemical evaluation of the development and progression of carcinoid heart disease

William D. Denney, MD^*, W. Evans Kemp, Jr., MD^*, Lowell B. Anthony, MD, John A. Oates, MD and Benjamin F. Byrd, III, MD, FACC^*

^* Division of Cardiology, Vanderbilt University, Nashville, Tennessee, USA
Department of Internal Medicine, Vanderbilt University, Nashville, Tennessee, USA
Division of Medical Oncology, Tulane University, New Orleans, Louisiana, USA

Manuscript received July 21, 1997; revised manuscript received May 18, 1998, accepted June 2, 1998.

Address for correspondence: Dr. W. Evans Kemp, Jr., 315 MRB II, 2220 Pierce Ave., Vanderbilt University Medical Center, Nashville, Tennessee 37232
evans.kemp{at}mcmail.vanderbilt.edu

Objectives. To study the applicability of a newly developed echocardiographic scoring system in the assessment of carcinoid valvular heart disease.

Background. We investigated prospectively the development, progression and regression of carcinoid valvular heart disease in patients with carcinoid syndrome by serial echocardiography, correlating these features with urinary 5-HIAA levels and clinical data collected during therapy with somatostatin analog.

Methods. Twenty-three patients with carcinoid syndrome underwent serial echocardiographic examinations. An echocardiographic carcinoid valvular heart disease (CVHD) % score was determined from points assigned for tricuspid and pulmonary valve structure and function.

Results. Fifteen patients had no CVHD at study entry (group 1), while 8 patients had findings of CVHD (group 2). Five patients in group 1 developed new CVHD (1B), while one demonstrated progression of CVHD (2B). The remaining patients did not develop (1A) or had no progression of CVHD (2B). Despite major declines in 5-HIAA levels during therapy in most patients, CVHD did not regress. There were significantly lower levels of median baseline 5-HIAA (98.8 vs. 256 mg/24 h), posttreatment 5-HIAA (50.3 vs. 324 mg/24 h) and posttreatment 5-HIAA time integral (37.3 vs. 192 g/24 h* days) in group A vs. B (p < 0.05). However, only posttreatment 5-HIAA levels independently predicted the development or progression of CVHD by multiple step-wise regression analysis (p < 0.005), with a threshold observed in the 100 mg/24 h range.

Conclusions. We designed a new echocardiographic scoring system to evaluate CVHD. Correlating echocardiographic scores with biochemical and clinical markers showed that only posttreatment 5-HIAA levels independently predicted the development or progression of CVHD. This study strengthens the association between serotonin secretion and CVHD, as well as introducing a new technique for serial follow-up of these patients.

Abbreviations and Acronyms   5-HIAA = 5-hydroxyindoleacetic acid   CVHD = carcinoid valvular heart disease   CT = computed tomography   2D = two-dimensional   TR = tricuspid regurgitation   PI = pulmonic valve insufficiency   PS = pulmonic valve stenosis   RA = right atrium

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