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Described herein is a 67-year-old woman who underwent replacement of both tricuspid and pulmonic valves because of severe isolated right-sided systolic heart failure. The cause of the heart failure preoperatively was believed to be the result of left breast radiation a year earlier. At operation, however, the pulmonic valve was excised and a biopsy of the stiff-walled right atrium was performed, and histologic examination of each was classic of carcinoid heart disease. She never awoke postoperatively. Postoperatively, computed tomography disclosed numerous masses in the liver. Retrospectively, clues to the presence of carcinoid heart disease include thickening of both the tricuspid and pulmonic valve leaflets by echocardiogram, a pressure gradient, albeit small, across the pulmonic valve, the plastering of the septal tricuspid-valve leaflet to the ventricular septum, the total absence of left-sided heart disease, and the presence of extremely low 12-lead QRS electrocardiographic voltage., (Copyright © 2014 Elsevier Inc. All rights reserved.)

A 53-year-old woman is described who underwent mitral and aortic valve replacement and tricuspid valve annuloplasty for pure regurgitation at all 3 valve sites for unrecognized carcinoid heart disease without the carcinoid syndrome 22 days before death. Metastatic carcinoid was not recognized until necropsy, which disclosed a probable ovarian primary but with large hepatic metastases and left-sided cardiac involvement either greater than or equal to the right-sided involvement. Pulmonary hypertension, very unusual in carcinoid heart disease, persisted postoperatively and probably played a role in the patient's early death. Hepatic metastasis with ovarian primary is most unusual in this circumstance., (Copyright © 2011 Elsevier Inc. All rights reserved.)

The aim of this study was to assess (1) the incidence of patent foramen ovale (PFO) in carcinoid syndrome (CS) and (2) the feasibility of percutaneous closure procedure in selected patients with CS. One hundred eight patients were prospectively studied: 54 with CS and an age- and gender-matched control group. All patients underwent conventional and contrast echocardiography. Patients with clinical signs of dyspnea (New York Heart Association class > or =III), cyanosis, carcinoid heart disease (CHD), and severe PFO were referred for the percutaneous closure of PFO. The prevalence of PFO was 41% in patients with CS and 22% in the control group (p = 0.03) and was significantly higher in patients with CHD (59%, p = 0.009). Four patients (14% of those with CHD) were referred for the percutaneous closure of PFO, and 3 patients ultimately underwent PFO closure (using Amplatzer septal occluders). At 6-month follow-up, New York Heart Association class was improved in all patients, as well as arterial blood gas results (p = 0.04) and 6-minute walking distance (p = 0.03), but all patients presented residual right-to-left shunts. In conclusion, this prospective study demonstrates that in patients with CHD, the prevalence of PFO is high and that percutaneous closure of PFO is feasible, with a reduction in symptoms but with residual shunting.

Because the venous drainage of the ovary bypasses the portal circulation, carcinoid heart disease in patients with primary ovarian carcinoid tumors may develop in the absence of liver metastasis. We describe 4 patients who presented with symptomatic carcinoid heart disease in association with primary ovarian carcinoid tumor.

Mitral valve replacement is considered when there is severe mitral stenosis, severe mitral insufficiency or a combination of the two. Ordinarily, surgical replacement is considered only for patients who are in functional classes III or IV and do not respond to medical management. Patients with symptomatic mitral stenosis should be treated with mitral commissurotomy whenever possible. Patients selected for commissurotomy should have a pliable valve, no other major valve dysfunction, sinus rhythm, no systemic embolism and good left ventricular function. Early operation is not ordinarily required. Mitral insufficiency may require mitral valve replacement in six rather common settings: rheumatic disease, rupture of mitral chordae tendineae, postinfarction rupture of a papillary muscle, intractable infective endocarditis, floppy mitral valve and malfunction of a prosthetic valve. Rupture of mitral chordae tendineae can usually be recognized from the history, physical examination, echocardiogram and angiocardiogram. Severe left ventricular papillary muscle dysfunction is usually due to cardiac infarction, and occurs within the first 9 days of infarction. When only a papillary muscle tip is ruptured the patient may survive long enough for a mitral valve replacement. In infective endocarditis, operation is more often needed because of congestive heart failure than because of refractory infection. Evidence of mitral stenosis or insufficiency in a patient with a previously implanted prosthetic valve usually indicates an urgent need for study and early operation. Uncommon causes of mitral incompetence that may require valve replacement are endocardial fibroelastosis, Marfan's syndrome, calcified mitral anulus, osteogenesis imperfecta, methysergide-induced heart disease and carcinoid heart disease.

Previously reported cardiac manifestations of carcinoid disease include right-sided valvular dysfunction and vasomotor disturbances. This report describes a 62-year-old white man who had electrocardiographically documented widespread elevation of the S-T segment, arrhythmias, and cardiac arrest after exercise. Noninvasive and laboratory studies confirmed the diagnosis of malignant carcinoid disease with tricuspid valve involvement. Coronary angiography revealed mild atherosclerosis. Thus, coronary artery spasm is a previously unrecognized manifestation of carcinoid heart disease and introduces what may be an important relationship of endogenous, excessive vasoactive amines to human coronary vasospasm.

Plasma atrial natriuretic peptide (ANP) concentration was determined and cardiac ultrasound studies were performed in 50 patients with malignant mid-gut carcinoid tumors. The extent of carcinoid-related heart disease varied among the patients. The patients with the most severe right-sided heart disease, who often had signs of right ventricular failure, had significantly (p less than 0.001) higher plasma ANP concentrations than either patients with less or no abnormal ultrasound findings or age- and sex-matched healthy control subjects. ANP levels were serially determined for 0.5 to 4 years (median 2.1) in 12 patients. The levels increased above the reference range in patients with clinical findings of right ventricular failure. In patients without cardiac decompensation the levels remained within the reference range. In 3 patients who had successful tricuspid and pulmonary valve replacements, signs and symptoms of right ventricular failure disappeared and plasma ANP levels declined and normalized. Five patients with progressive right ventricular failure and increasing plasma ANP levels during follow-up eventually died from cardiac decompensation. This study demonstrates the predictive value of serial determinations of plasma ANP in carcinoid heart disease. Such measurements can be an additional guide in the clinical management of these patients.

A woman with tricuspid regurgitation due to carcinoid heart disease underwent successful tricuspid valvular replacement. This was performed as soon as the patient became symptomatic, enabling her to return to a normal life untroubled by slowly growing liver metastases. Few early valvular replacements have been undertaken in this disease. This case report demonstrates its usefulness.

To correlate clinical and laboratory variables in carcinoid heart disease, clinical data, echocardiograms, 24-hour urinary 5-hydroxyindoleacetic acid levels and liver function tests were evaluated in 30 patients with the carcinoid syndrome. The dominant cardiac lesion of carcinoid heart disease by echocardiography and Doppler was severe tricuspid regurgitation with right ventricular volume overload. A characteristic finding was thickened, retracted tricuspid valve leaflets that were fixed in a partially open position. Carcinoid heart disease was progressive and often fatal. The 17 patients with echocardiographic evidence of carcinoid heart disease had higher peak levels of urinary 5-hydroxyindoleacetic acid (331 +/- 231 vs 58 +/- 78 mg, p less than 0.001) and more severe hepatic dysfunction than the 13 patients without carcinoid heart disease. Although duration of symptoms of the carcinoid syndrome before echocardiography was similar for patients with and without carcinoid heart disease (5.4 +/- 6.4 vs 6.2 +/- 5.9 years, respectively, p greater than 0.1), survival after echocardiography was shorter for those with carcinoid heart disease (1.9 +/- 1.4 vs 3.8 +/- 2.9 years, p = 0.05). The findings support the concept that long-term exposure of the endocardium to serotonin in the right side of the heart leads to the development of heart lesions; in addition, progressive hepatic dysfunction may allow more serotonin to bypass liver enzymes and reach the right side of the heart.