Echocardiographic follow-up of treated patients with carcinoid syndrome.

Carcinoid heart disease is a rare disease, and its prevalence is uncertain. The aim of the present prospective study was to assess the prevalence of carcinoid heart disease using annual echocardiographic follow-up. We studied 80 consecutive patients presenting with histologically proven digestive endocrine tumor and carcinoid syndrome. All patients underwent annual conventional transthoracic echocardiographic studies and measurement of biologic carcinoid markers. Right- and left-sided carcinoid heart disease and the score of the severity of carcinoid heart disease were systematically assessed. At baseline, the prevalence of right- and left-sided carcinoid heart disease was 33% and 8%, respectively. At the end of follow-up, the corresponding prevalence was 53% and 21%. The correlations were strong between urinary 5-hydroxyindoleacetic acid (5-HIAA) and the right-sided carcinoid heart disease score (r = 0.75, p <0.0001), between urinary 5-HIAA and the left-sided carcinoid heart disease score (r = 0.83, p = 0.001), and between urinary 5-HIAA and the overall carcinoid heart disease score (r = 0.84, p <0.0001). All patients with > or =3 years of carcinoid syndrome and increased level of urinary 5-HIAA presented with echocardiographic evidence of valve disease on routine monitoring. In conclusion, the prevalence of carcinoid heart disease remained high and increased during follow-up. Carcinoid heart disease progresses over time, highlighting the need for echocardiographic follow-up once carcinoid syndrome has been diagnosed.

Cardiovascular magnetic resonance and PET-CT of left atrial paraganglioma.

Cardiac paragangliomas are among the rarest primary cardiac tumors. We present a case of left atrial paraganglioma in a patient who presented with symptoms and signs of catecholamine excess in which cardiovascular magnetic resonance in multiple orientations and PET-CT played an important role in the diagnosis and tissue characterization.

Nephrotic-range proteinuria associated with right atrial myxoma.

A case of right atrial myxoma presenting with right heart failure and proteinuria is described. Proteinuria was variable and this corresponded with the degree of systemic venous congestion. On one occasion the proteinuria was within the nephrotic range. There was no evidence of intrinsic renal pathology. The right heart failure and proteinuria resolved after tumour removal, suggesting that the etiology of urinary protein loss was a reversible increase in glomerular permeability.

Cardiac pheochromocytomas.

Cardiac pheochromocytomas are rare. Thirty cases have been reported in the literature. We report the cases of 2 more patients in whom the diagnosis was established using coronary angiography and who underwent surgical resection using cardiopulmonary bypass. We also review the literature on the subject.