Carcinoid and Echocardiography

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Clin Cardiol. 2006 Mar;29(3):134
Pathognomonic echocardiographic features of carcinoid syndrome.
de Diego C, Marcos Alberca P, Cabrera JA, Pindado J, Farre J, Pal RK.
Division of Cardiology, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California, USA.

Circulation. 2006 Feb 21;113(7):e160-1.
Images in cardiovascular medicine. Carcinoid heart disease.
Jaber WA, Klarich KW.
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, USA.

Circulation. 2005 Nov 22;112(21):3320-7. Epub 2005 Nov 14.
Prognosis of carcinoid heart disease: analysis of 200 cases over two decades.
Moller JE, Pellikka PA, Bernheim AM, Schaff HV, Rubin J, Connolly HM.
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA.

BACKGROUND: The long-term prognosis of patients who develop carcinoid heart disease and the effect of cardiac surgery on outcome are not well established. METHODS AND RESULTS: In this retrospective study, we identified 200 patients with carcinoid syndrome referred for echocardiography in whom the diagnosis of carcinoid heart disease was confirmed. Patients were divided into 3 groups of similar size according to the date from first diagnosis of carcinoid heart disease. Group A comprised patients diagnosed from 1981 through June 1989; group B, diagnosed July 1989 through May 1995; and group C, June 1995 through 2000. The end point was all-cause mortality. Median survival was significantly lower in group A (1.5 years, 95% CI 1.1 to 1.9 years) compared with groups B (3.2, 95% CI 1.3 to 5.1 years) and C (4.4, 95% CI 2.4 to 7.1 years; P=0.009). In a multivariate model adjusted for treatment and clinical characteristics, the risk of death in groups B (hazard ratio 0.67, 95% CI 0.46 to 0.99, P=0.04) and C (hazard ratio 0.61, 95% CI 0.39 to 0.92, P=0.006) was significantly reduced relative to group A. Cardiac surgery was performed in 87 patients. When cardiac surgery was included as a time-dependent covariate in a multivariate analysis, it was associated with a risk reduction of 0.48 (95% CI 0.31 to 0.73, P<0.001), whereas the time period of diagnosis was no longer significant. CONCLUSIONS: The prognosis of patients with recognized carcinoid heart disease has improved over the past 2 decades at our institution. This change in survival may be related to valve replacement surgery.

Am Heart Hosp J. 2005 Spring;3(2):132-5.
Carcinoid heart disease: echocardiographic recognition and differential diagnosis.
Pearlman AS.
Division of Cardiology, University of Washington School of Medicine, Seattle, WA 98195, USA.

N Engl J Med. 2003 Mar 13;348(11):1005-15.
Comment in:
N Engl J Med. 2003 Jun 5;348(23):2359-61; author reply 2359-61.
Factors associated with progression of carcinoid heart disease.
Moller JE, Connolly HM, Rubin J, Seward JB, Modesto K, Pellikka PA.
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minn 55905, USA.

BACKGROUND: By releasing vasoactive substances into the circulation, carcinoid tumors can cause right-sided valvular heart disease. Factors associated with the progression of carcinoid heart disease are poorly understood. We conducted a retrospective study to identify such factors. METHODS: Our sample included 71 patients with the carcinoid syndrome who underwent serial echocardiographic studies performed more than one year apart and 32 patients referred directly for surgical intervention after an initial echocardiographic evaluation. A score for carcinoid heart disease was determined on the basis of an assessment of valvular anatomy and function and the function of the right ventricle. An increase of more than 25 percent in the score between studies was considered suggestive of disease progression. Tumor progression was assessed on the basis of abdominal computed tomographic scans and changes in the level of urinary 5-hydroxyindoleacetic acid (5-HIAA), a metabolite of serotonin. RESULTS: Of the patients with serial echocardiographic studies, 25 (35 percent) had an increase of more than 25 percent in the cardiac score. As compared with patients whose score changed by 25 percent or less, these patients had higher urinary peak 5-HIAA levels (median, 265 mg per 24 hours [interquartile range, 209 to 593] vs. 189 mg per 24 hours [interquartile range, 75 to 286]; P=0.004) and were more likely to have biochemical progression (10 of 25 patients vs. 9 of 46, P=0.05) and to have received chemotherapy (13 of 25 vs. 10 of 46, P=0.009). Logistic-regression analysis showed that a higher peak urinary 5-HIAA level and previous chemotherapy were predictors of an increase in the cardiac score that exceeded 25 percent (odds ratio for each increase in 5-HIAA of 25 mg per 24 hours, 1.08 [95 percent confidence interval, 1.03 to 1.13]; P=0.009); odds ratio associated with chemotherapy, 3.65 [95 percent confidence interval, 1.74 to 7.48]; P=0.001). CONCLUSIONS: Serotonin is related to the progression of carcinoid heart disease, and the risk of progressive heart disease is higher in patients who receive chemotherapy than in those who do not. Copyright 2003 Massachusetts Medical Society

J Am Coll Cardiol. 2002 Oct 2;40(7):1328-32.
Metastatic carcinoid tumor to the heart: echocardiographic-pathologic study of 11 patients.
Pandya UH, Pellikka PA, Enriquez-Sarano M, Edwards WD, Schaff HV, Connolly HM.
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.

OBJECTIVE: We sought to investigate the clinical and echocardiographic (echo) characteristics of metastatic carcinoid tumor in the heart. BACKGROUND: Right-sided valvular dysfunction is the hallmark of carcinoid heart disease. Cardiac metastases are uncommon in carcinoid syndrome. Features of patients with metastatic carcinoid tumor involving the heart (MCH) have not been well described. METHODS: From 1985 through 1999, 11 patients (8 male, 3 female), mean age +/- standard deviation, 58 +/- 6 years, were seen who had pathologically confirmed MCH. All patients had echoes, which were reviewed retrospectively. RESULTS: All patients with MCH had carcinoid syndrome. The primary carcinoid tumor was in the small bowel in 83% of patients, and all patients had hepatic metastases. On pathologic review, the 11 patients had 15 MCH tumors. All metastases were intramyocardial. The MCH involved the right ventricle in 40%, left ventricle in 53%, and ventricular septum in 7%. The average size of macroscopic tumors was 1.8 +/- 1.2 cm. Nine MCH tumors were detected by echo in 6 of the 11 patients (55%). Mean echo-detected tumor size was 2.4 cm (range, 1.2 to 4). All tumors noted by echo were well circumscribed, non-infiltrating, and homogeneous. In the 5 other patients, review of autopsy records revealed 6 macroscopic tumors, mean size 0.35 cm (range, 0.2 to 0.4), none detected by echo even retrospectively. Carcinoid valve disease was present in 8 of the 11 MCH patients. The tricuspid valve was affected in all 8 patients (73%), pulmonary valve in 7 (64%), and left sided valves in 4 (36%) All patients with MCH identified by echo had cardiac surgery, 3 primarily for carcinoid valve disease and 2 for non-carcinoid cardiac disease; in 1 patient, MCH was the primary indication for cardiac surgery. CONCLUSIONS: MCH is uncommon but can be easily identified by echo if tumor size is >/=1.0 cm. In patients without valvular dysfunction, MCH may be the only manifestation of carcinoid heart disease. A search for MCH should be an integral part of the echo exam in patients with carcinoid syndrome.

Circulation. 2002 Sep 24;106(12 Suppl 1):I51-I56.
Carcinoid heart disease: impact of pulmonary valve replacement in right ventricular function and remodeling.
Connolly HM, Schaff HV, Mullany CJ, Abel MD, Pellikka PA.
Division of Cardiovascular Diseases, Department of Anesthesiology, and Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minn., USA.

BACKGROUND: Carcinoid heart disease characteristically affects tricuspid (TV) and pulmonary valves (PV), and TV replacement is helpful in selected patients. There is uncertainty, however, regarding optimal surgical management of PV regurgitation. METHODS AND RESULTS: We reviewed 22 patients having operation for carcinoid heart disease and compared those having TV and PV replacement (n=12), to those who underwent TV replacement and excision of the PV (n=10). Pre- and postoperative right ventricular (RV) size and dysfunction were assessed by consensus of 2 echocardiographers blinded to type of surgical treatment. RV dysfunction was graded as none (0), mild (1), moderate (2), or severe (3). RV size was graded as normal (0), or mild (1), moderate (2), or severe (3) enlargement. Preoperatively, RV size (2.2+/-0.8 [no PVR]versus 2.7+/-0.6 [with PVR], P=0.15), RV dysfunction (0.9+/-0.9 [no PVR]versus 1.4+/-0.7 [with PVR], P=0.14), and NYHA class were similar in the 2 groups. Postop RV size decreased inpatients with PVR, 2.7+/-0.6 to 1.7+/-1.0 (P=0.008), but did not change appreciably in those without PVR, 2.2+/-0.8 to 2.3+/-0.8 (P=0.67). There was no significant change in RV dysfunction after surgery, 1.4+/-0.7 to 1.8+/-0.9 with PVR (P=0.26) and 0.9+/-0.9 to 1.6+/-0.9 without PVR (P=0.07). CONCLUSIONS: PV replacement appears to have a beneficial effect on RV size in patients after surgery for carcinoid heart disease. This may have important implications for RV remodeling after PV replacement.

Neth J Med. 2002 May;60(4):181-5.
Echocardiographic diagnosis in carcinoid heart disease.
Wonnink-De JW, Knibbeler-Van RC, Van der Heul C, Pasteuning WH.
St. Elisabeth Hospital, Department of Cardiology, Tilburg, The Netherlands.

In this case report the typical echocardiographic features of carcinoid heart disease are presented. Newer treatments such as the use of a somatostatin analogue, interferon and hepatic de-arterialisation have improved the prognosis in patients with carcinoid syndrome. Nevertheless this syndrome portends a poor prognosis in patients with cardiac involvement. Cardiac lesions are mainly located in the right side of the heart. Regurgitation and stenosis of the tricuspid and pulmonary valve, leading to right heart failure, are the most common cardiac manifestations of the disease. Elevated levels of serotonin are probably responsible for the development of these cardiac lesions. Despite treatment resulting in significant reductions of urinary levels of 5-HIAA, regression of the cardiac manifestations in carcinoid syndrome has not been observed. Two-dimensional and Doppler echocardiography are the main tools to establish the diagnosis and severity of carcinoid heart disease. Cardiac surgery for carcinoid heart disease might improve symptoms and longevity, but the scarce data report on early mortality of over 35%.

Neth J Med. 2002 Mar;60(1):10-6.
Cardiovascular abnormalities in patients with a carcinoid syndrome.
Meijer WC, van Veldhuisen DJ, Kema IP, Van den Berg MP, Boomsma F, Willemse PH, De Vries EG.
University Hospital Groningen, Department of Medical Oncology, The Netherlands.

BACKGROUND: Heart failure is an important reason for morbidity and mortality in patients with carcinoid. Carcinoid heart disease is caused by increased levels of circulating serotonin. Because carcinoids also produce catecholamines, we evaluated cardiovascular manifestations of autonomic dysfunction in patients with a carcinoid syndrome. METHODS: Twenty patients with a midgut carcinoid, who had a carcinoid syndrome with a median duration of 72 months, and markedly elevated urinary 5-hydroxyindoleacetic acid (5-HIAA) excretion were studied. RESULTS: Ten patients had no symptoms of heart failure, i.e. New York Heart Association (NYHA) functional class I, 6 had class II, and 4 class III heart failure. Transthoracic echocardiography (TTE) showed right-sided valvular abnormalities in 13 of 19 evaluable patients (mild n=8, severe n=5). Fourteen of the 20 patients (70%) had an elevated concentration of plasma N-terminal atrial natriuretic peptide (N-ANP), which correlated with NYHA class, TTE abnormalities, and increased urinary metanephrine excretion. Heart rate variability (HRV) parameters, in particular those associated with increased sympathetic activity (low frequency power, p=0.002 versus healthy individuals), were impaired but were independent of NYHA class and TTE findings and correlated with urinary metanephrine excretion (r=-0.49, p<0.05). CONCLUSION: In these 20 carcinoid patients with substantial secretory activity of the tumour, overt cardiac morphological changes were present in a minority of patients. However, N-ANP values and HRV profile were markedly abnormal, and related to enhanced urinary excretion of catecholamine and metabolites, suggesting autonomic derangement. These abnormalities possibly herald the development of more severe cardiac dysfunction and may be indicative of the need for preventive drug treatment.

J Clin Anesth. 2002 Feb;14(1):57-63.
Carcinoid heart disease: a case report and literature review.
Botero M, Fuchs R, Paulus DA, Lind DS.
Department of Anesthesiology, University of Florida College of Medicine, and Veterans Affairs Medical Center, Gainesville, FL 32610-0254, USA.

We report a patient who presented for elective exploratory laparotomy, and resection of a pelvic mass, which was thought to be ovarian carcinoma. Intraoperative transesophageal echocardiography demonstrated right-sided valvular heart lesions, which suggested the diagnosis of carcinoid syndrome before a pathologic confirmation was obtained. This article discusses the classical presentation and anesthetic management of patients with carcinoid syndrome and emphasizes the importance of proper preoperative diagnosis and careful planning if the incidence and severity of the symptoms that this condition can provoke are to be reduced.

Br J Surg. 2001 Jun;88(6):865-72.
Prediction of prognosis by echocardiography in patients with midgut carcinoid syndrome.
Westberg G, Wangberg B, Ahlman H, Bergh CH, Beckman-Suurkula M, Caidahl K.
Department of Surgery, Sahlgrenska University Hospital, Goteborg, Sweden.

BACKGROUND: The association between malignant midgut carcinoid tumours and right-sided cardiac lesions is well known, but the pathogenetic link between tumour secretion and valvular disease is still obscure. The purpose of this investigation was to describe the morphological and functional changes of valvular heart disease in a large patient series and to correlate these findings with hormonal secretion and prognosis. METHODS: Of 64 consecutive patients with the midgut carcinoid syndrome followed between 1985 and 1998, valvular heart disease was evaluated in 52 patients by two-dimensional echocardiography, Doppler estimation of valvular regurgitation and flow profiles. A majority was also evaluated with exercise electrocardiography and spirometry. RESULTS: Structural and functional abnormalities of the tricuspid valve were found in 65 per cent of patients, while only 19 per cent had pulmonary valve regurgitation. Long-term survival was related to excessive urinary excretion of 5-hydroxyindole acetic acid of over 500 micromol in 24 h, but the main predictor of prognosis was the presence of severe structural and functional abnormalities of the tricuspid valve. Although advanced tricuspid abnormalities were prevalent in this series, only one patient died from right ventricular heart failure. CONCLUSION: Tricuspid valvular disease is a common manifestation of the midgut carcinoid syndrome and advanced changes are associated with poor long-term survival. Active surgical and medical therapy of the tumour disease reduced the hormonal secretion and, combined with cardiological surveillance, made right ventricular heart failure a rare cause of death in these patients.

Echocardiography. 2000 Aug;17(6 Pt 1):571-4.
Carcinoid heart disease in association with a primary ovarian carcinoid tumor: diagnostic role of echocardiography.
Franko DM, Berger M.
The Heart Institute, Beth Israel Medical Center, First Avenue at 16th Street, New York, New York 10003, USA.

We describe a patient with carcinoid heart disease secondary to a primary ovarian carcinoid tumor who initially presented with unexplained right-sided heart failure. The patient did not exhibit any of the typical clinical manifestations of the carcinoid syndrome. Echocardiography demonstrated typical features of carcinoid heart disease and played an important role in the detection and management of a condition that was previously unrecognized.

Arch Mal Coeur Vaiss. 2000 Mar;93(3):277-83.
Use of echocardiography in the diagnosis of carcinoid tumors.
Report of 4 cases
Avierinos JF, Habib G, Garcia M, Lefevre J, Medail C, Dufaut F, Philip E, Ambrosi P, Perrier H, Luccioni R. Service de cardiologie B, hopital La Timone, Marseille.

Carcinoid tumours are the most common neuro-endocrine tumours but cardiac involvement is rarely symptomatic although often observed at post-mortem and rarely revelatory of the disease. The authors report 4 cases in which echocardiographic detection of characteristic right ventricular involvement led to the confirmation of the diagnosis of carcinoid tumour leading to the secondary diagnosis of the primary carcinoid tumour. The clinical, physiopathological echocardiographic and therapeutic characteristics of this condition are discussed.

J Am Coll Cardiol. 1998 Oct;32(4):1017-22.
Echocardiographic and biochemical evaluation of the development and progression of carcinoid heart disease.
Denney WD, Kemp WE Jr, Anthony LB, Oates JA, Byrd BF 3rd.
Division of Cardiology, Vanderbilt University, Nashville, Tennessee, USA.

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 q 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.

Circulation. 1995 Aug 15;92(4):790-5.
Carcinoid heart disease. Correlation of high serotonin levels with valvular abnormalities detected by cardiac catheterization and echocardiography.
Robiolio PA, Rigolin VH, Wilson JS, Harrison JK, Sanders LL, Bashore TM, Feldman JM.
Division of Cardiology, Duke University Medical Center, Durham, NC 27710, USA.

BACKGROUND: Although serotonin has been postulated as an etiologic agent in the development of carcinoid heart disease, no direct evidence for different ambient serotonin levels in cardiac and noncardiac patients has been reported to date. METHODS AND RESULTS: The present study reviews our experience with 604 patients in the Duke Carcinoid Database. Nineteen patients with proven carcinoid heart disease (by cardiac catheterization and/or echocardiogram) were compared with the remaining 585 noncardiac patients in the database with regard to circulating serotonin and its principal metabolite, 5-hydroxyindole acetic acid (5-HIAA). No significant demographic differences existed between the cardiac and noncardiac groups; however, typical carcinoid syndrome symptoms (ie, flushing and diarrhea) were almost threefold more common in the cardiac group (P < .001). Compared with the noncardiac group, heart disease patients demonstrated strikingly higher (P < .0001) mean serum serotonin (9750 versus 4350 pmol/mL), plasma serotonin (1130 versus 426 pmol/mL), platelet serotonin (6240 versus 2700 pmol/mg protein), and urine 5-HIAA (219 versus 55.3 mg/24 h) levels. The spectrum of heart disease among the 19 patients showed a strong right-sided valvular predominance, with tricuspid regurgitation being the most common valvular dysfunction (92% by cardiac catheterization; 100% by echocardiogram). CONCLUSIONS: These data suggest that serotonin plays a major role in the pathogenesis of the cardiac plaque formation observed in carcinoid patients.

Am J Cardiol. 1995 Mar 1;75(7):485-8.
Predictors of outcome of tricuspid valve replacement in carcinoid heart disease.
Robiolio PA, Rigolin VH, Harrison JK, Lowe JE, Moore JO, Bashore TM, Feldman JM.
Division of Cardiology, Duke University Medical Center, Durham, North Carolina 27710.

The cardiac valvular surgical experience of patients in the Duke Carcinoid Database was reviewed to assess operative outcome. Of the 604 patients in the database, 19 patients with carcinoid heart disease were identified by cardiac catheterization or echocardiography, or both. Eight of these underwent tricuspid valve replacement surgery with bioprostheses (2 also had open pulmonic valvuloplasty). Compared with patients medically managed, surgically treated patients were similar with the exception that they had higher right atrial mean (17 +/- 6 vs 9 +/- 4 mm Hg, p = 0.03) and v-wave (27 +/- 6 vs 17 +/- 7 mm Hg, p = 0.04) pressures. Of the 8 surgical patients, 5 (63%) died within 30 days. Causes of death included tricuspid valve thrombosis, cerebral vascular accident, coagulopathy, renal failure, and intractable right heart failure. High comorbidity was present in all 8 patients. There was a weak trend (p = 0.17) toward lower Charlson comorbidity indexes in survivors (6.7 +/- 0.6) compared with nonsurvivors (7.6 +/- 0.9). Age was significantly lower (p = 0.036) in survivors (46 +/- 13 years) compared with nonsurvivors (69 +/- 4 years). Extended follow-up revealed 2 patients who survived beyond a decade. Review of 47 carcinoid valve replacement cases (Duke Carcinoid Database and 39 published cases) revealed a 30-day mortality of 56% for patients > 60 years of age, and 0% for those < or = 60 years of age (p < 0.0001). Although valve replacement surgery can afford prolonged palliation from carcinoid heart disease, it is associated with a significant mortality risk. Careful preoperative risk stratification by age and comorbidity may provide a means for optimal selection of surgical candidates.

J Heart Valve Dis. 1997 Nov;6(6):625-30.
Incidence and evolution of carcinoid syndrome in the heart.
Moyssakis IE, Rallidis LS, Guida GF, Nihoyannopoulos PI.
Department of Echocardiography, Hammersmith Hospital, London, UK.

BACKGROUND AND AIMS OF THE STUDY: Carcinoid syndrome originates from carcinoid tumors localized in the gastrointestinal system, pancreas, biliary vessels, bronchi, ovaries and testes. The condition is characterized by flushing, telangiectasias, diarrhea, bronchoconstriction and cardiac involvement, while cardiac lesions may occur in 50% of patients. This study aimed to investigate-non-invasively-the frequency, severity and evolution of carcinoid heart involvement. METHODS AND RESULTS: Among 87 patients with carcinoid syndrome undergoing routine echocardiography, 39 (45%) had cardiac involvement; 36 of these had thickened, shortened, immobile tricuspid leaflets. Tricuspid regurgitation was present in all 36, it was moderate or severe in 22. Pressure half-time rose to 117 +/- 35 ms, implying mild tricuspid stenosis. The pulmonary valve was also thickened, retracted and immobile with stenotic appearance in 19 patients; regurgitation was present in 16, and mild in 11. Nine patients had pericardial effusion. Of 39 patients with cardiac lesions, 18 had at least one follow up study within 16 +/- 7 months; in 15 cases the valve lesions deteriorated. Tricuspid regurgitation became more severe in six and pressure half-time rose from 107 +/- 46 to 132 +/- 50 ms. Pulmonary regurgitation deteriorated in two, while three patients had new pulmonary valve involvement. The peak gradient across the pulmonary valve increased from 18 +/- 7 to 25 +/- 9 mmHg. Twenty-two patients without heart involvement in the first examination had a follow up study (19 +/- 5 months) during which seven developed valvular lesions, with four moderate and three mild tricuspid regurgitation; mild stenosis was present in all cases. Two patients with mild tricuspid regurgitation had mild mixed pulmonary valve involvement, while of those with moderate tricuspid regurgitation, one also had moderate pulmonary stenosis with mild regurgitation. Five patients with both tricuspid and pulmonary valve diseases had interventional treatment: three underwent balloon valvuloplasty on both valves simultaneously due to stenotic lesions but later relapsed; the other two underwent double valve replacement with biological prostheses but in both cases the pulmonary valve developed severe destruction leading to one death. The other four patients survived for 36, 41, 25 and 16 months, respectively. CONCLUSIONS: Right valvular heart disease occurs frequently in patients with carcinoid syndrome. Evolution of lesions is rapid, leading to right heart failure, though death usually occurs from progressive systemic disease and rarely from pulmonary stenosis. Surgery is the most effective treatment and balloon valvulotomy is only palliative. However, therapy of the systemic condition is predominantly the treatment of choice.

Eur Heart J. 1993 Dec;14(12):1721-3.
Carcinoid heart disease: two cases of left heart involvement diagnosed by transthoracic and transoesophageal echocardiography.
Le Metayer P, Constans J, Bernard N, Roudaut R, Pellegrin JL, Lacoste D, Beylot J, Leng B, Conri C, Warin JF.
Service de Medecine Interne et Pathologie Cardio-vasculaire Hopital St-Andre, Bordeaux, France.

We report two observations of significant left heart involvement in patients with the carcinoid syndrome assessed by transthoracic and transoesophageal echocardiography. Echocardiographic lesions of this kind have only been reported twice. In the present cases, there was mitral involvement with mitral regurgitation in one case and a mitro-aortic involvement with mitral and aortic regurgitation in the other. The mechanism of left heart lesions is unclear since in both cases no right-to-left cardiac shunt was present, as attested by colour Doppler and saline contrast transoesophageal echocardiography. The location of the primary tumour was unknown in one case and ileal in the other; no pulmonary metastasis was detected. The use of transoesophageal echocardiography might make it possible to detect left-sided cardiac lesions more frequently since they were found in anatomical series, in 30% of patients with carcinoid syndrome.

Can J Anaesth. 1993 Jul;40(7):664-6.
Transoesophageal echocardiography and the intraoperative diagnosis of left atrial invasion by carcinoid tumour.
Neustein SM, Cohen E, Reich D, Kirschner P.
Department of Anesthesiology, Mount Sinai Medical Center, New York, NY 10029.

A 41-yr-old woman with pulmonary carcinoid tumour presented for thoracotomy and lung resection. However, intraoperative transoesophageal echocardiography (TEE) revealed that the tumour had invaded the left atrium, and the planned resection was aborted to allow resection under cardiopulmonary bypass at a later date. Although the incidence of cardiac involvement by lung cancer at the time of pulmonary resection is unknown, transoesophageal echocardiography can be useful in identifying the extension of hilar lung tumours. This should preferentially be done preoperatively, but can be done intraoperatively as described in this case report.

Circulation. 1993 Apr;87(4):1188-96.
Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients.
Pellikka PA, Tajik AJ, Khandheria BK, Seward JB, Callahan JA, Pitot HC, Kvols LK.
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minn. 55905.

BACKGROUND. The carcinoid syndrome is a rare cause of acquired valvular heart disease. Although the typical echocardiographic features of carcinoid heart disease are well recognized, this large series provides new information about unusual manifestations of the disease as well as the role of Doppler echocardiography. METHODS AND RESULTS. Between 1980 and 1989, 132 patients with carcinoid syndrome underwent echocardiographic study. The echocardiographic, Doppler, and clinical features of the 74 patients (56%) with echocardiographic evidence of carcinoid heart disease are described. Among these patients, 97% had shortened, thickened tricuspid leaflets. Tricuspid regurgitation was present in all 69 patients with carcinoid heart disease who underwent Doppler examination, and it was of moderate or severe degree in 62 patients (90%). Severe tricuspid regurgitation was characterized by a dagger-shaped Doppler spectral profile with an early peak pressure and rapid decline. The pressure half-time was prolonged (mean, 116 msec), which is consistent with associated tricuspid stenosis. The pulmonary valve appeared thickened, retracted, and immobile in 36 patients (49%) and was diminutive to the extent of not being visualized in an additional 29 patients (39%). Among the 47 patients who underwent Doppler evaluation of the pulmonary valve, regurgitation was present in 81%, and stenosis was present in 53%. Left-sided valvular involvement was present in five patients (7%), four of whom had patent foramen ovale or carcinoid tumor involving the lung. Previously undescribed myocardial metastases were present in three patients (4%) and were confirmed by biopsy in each case. Small pericardial effusions were present in 10 patients (14%). Patients with and without echocardiographic evidence of carcinoid heart disease did not differ with regard to sex, age, location of the primary tumor, duration of diagnosis, or duration of symptoms of carcinoid syndrome. However, the mean pretreatment level of urinary 5-hydroxyindoleacetic acid was higher in patients with carcinoid heart disease than in patients without carcinoid heart disease (270 versus 131 mg/24 hrs, p < 0.001). The symptom of dyspnea was more prevalent among patients with carcinoid heart disease than in patients without the disease (54% versus 27%, p = 0.003); as expected, heart murmurs were also noted more frequently in patients with disease (92% versus 43%, p < 0.0001). Treatment regimens and response to therapy were similar in the two groups. Survival of patients with echocardiographic evidence of carcinoid heart disease was reduced compared with those without cardiac involvement (p = 0.0003). ECG and chest roentgenographic findings in patients with carcinoid heart disease were nonspecific. CONCLUSIONS. The broad spectrum of carcinoid heart disease is detailed in this large series. This includes not only right-sided valvular lesions but also left-sided involvement, pericardial effusion, and myocardial metastases.

Neth J Med. 1992 Aug;41(1-2):17-21.
Additive value of transoesophageal echocardiography in the visualization of carcinoid heart disease.
van Veldhuisen DJ, Hamer JP, Andriessen MP, de Vries EG, Lie KI.
Department of Cardiology/Thorax Centre, University Hospital Groningen, Netherlands.

A 65-yr-old woman with atypical complaints and a tricuspid insufficiency murmur underwent transthoracic echocardiography, which showed right-sided abnormalities, but did not allow clear visualization of the valves. Subsequent transoesophageal imaging, however, raised the suspicion of carcinoid heart disease, because of the typical lesions. The diagnosis was thereafter confirmed by biochemical investigations, scintigraphy and abdominal ultrasound. This case suggests that transoesophageal echocardiography may have additive value in the detection of carcinoid heart disease.

J Am Soc Echocardiogr. 1992 Mar-Apr;5(2):147-52.
Measurement of left ventricular dp/dt by simultaneous Doppler echocardiography and cardiac catheterization.
Chung N, Nishimura RA, Holmes DR Jr, Tajik AJ.
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, MN 55905.

Left ventricular dp/dt is a useful isovolumic index for evaluating acute directional changes in myocardial contractility. To test the hypothesis that Doppler echocardiography can measure left ventricular dp/dt by using the mitral regurgitation velocity curve, 14 patients with at least a mild degree of mitral regurgitation (four with coronary artery disease, four with valvular heart disease, four with dilated cardiomyopathy, one with carcinoid, and one with mitral valve prosthesis) were studied by continuous-wave Doppler echocardiography. Simultaneously, left ventricular pressure was measured with a manometer-tipped catheter to generate actual dp/dt. Curves of left ventricular pressure and mitral regurgitant Doppler-derived velocities of three cardiac cycles were digitized at 1-msec intervals. The rate of Doppler-derived velocity increase was converted to a rate of pressure increase by using the modified Bernoulli equation. Mean dp/dt during various time intervals of the mitral regurgitation velocity envelope (1 to 2 m/sec, 2 to 3 m/sec, and 1 to 3 m/sec) corresponding to left ventricular-left atrial pressure differences of 12, 20, and 32 mm Hg, respectively, were calculated. Doppler-derived left ventricular dp/dt (y) correlated with catheter-derived left ventricular dp/dt (x) as follows: at the 1 to 2 m/sec interval, y (mm Hg/sec) = 0.84x + 137, r = 0.91, SEE = 90; at the 2 to 3 m/sec interval, y = 1.1x - 89, r = 0.96, SEE = 80; and at the 1 to 3 m/sec interval, y = 1.1x + 23, r = 0.98, SEE = 50.(ABSTRACT TRUNCATED AT 250 WORDS)

Rev Esp Cardiol. 1991 Apr;44(4):283-7.
Tricuspid and pulmonary percutaneous valvulopathy in the cardiopathy of the carcinoid syndrome. A case report.
Alcibar Villa J, Gochi Ibarra R, Onate Landa A, Inguanzo Balbin R, Alvarez Blanco A, Barreiro Garcia G.
Seccion de Hemodinamica, Hospital de Cruces, Baracaldo, Vizcaya.

We present a 36-year-old woman with a malignant carcinoid syndrome, liver metastases and right heart involvement with severe tricuspid and pulmonic valve stenosis. She was admitted with severe heart failure and poor general status. A combined tricuspid and pulmonic valvuloplasty was performed and she exhibited great clinical improvement that was maintained during the follow-up. We point out the importance of two-dimensional echocardiography in the diagnosis of this pathology and the role of Doppler measurements for the evaluation of the valvuloplasty's results.

Br Heart J. 1990 Sep;64(3):190-4.
Transoesophageal echocardiography improves the diagnostic value of cardiac ultrasound in patients with carcinoid heart disease.
Lundin L, Landelius J, Andren B, Oberg K.
Department of Cardiology, University Hospital, Uppsala, Sweden.

Transthoracic and transoesophageal cardiac echocardiography and Doppler investigations were performed in 31 consecutive patients with malignant midgut carcinoid tumours. The transoesophageal images allowed measurement of the thickness of the atrioventricular valve leaflets and the superficial wall layers on the cavity side of both atria. The mean thickness of the anterior tricuspid leaflet was significantly greater than that of the mitral valve--a difference not seen in a control group of age-matched patients without carcinoid tumours and with normal cardiac ultrasound findings. In addition, the edges of the tricuspid leaflets were thickened giving them a clubbed appearance. Tricuspid incompetence was detected transoesophageally in 71% of the patients with carcinoid compared with 57% by transthoracic investigation. The inner layer of the right atrial wall in the carcinoid patients was significantly thicker than that of the left atrium and that of both atria in the controls. Furthermore, patients with other signs of severe carcinoid heart disease had significantly thicker mean right atrial luminal wall layer than those with less or no signs of right heart disease. Transoesophageal cardiac ultrasound investigation improved the diagnostic accuracy and seemed to show the structural changes typical of carcinoid heart disease established by histopathological investigations.

Int J Cardiol. 1989 Oct;25(1):129-30.
Right heart failure as the sole presentation of carcinoid syndrome.
Lachter JH, Lavy A, Eidelman S.
Department of Internal Medicine, Rambam Medical Center, Haifa, Israel.

We report the case of a woman with symptomatic right heart failure. Despite lack of the characteristic features of the carcinoid syndrome, echocardiographic and histologic investigations confirmed the diagnosis of carcinoid disease with cardiac involvement.

Am J Cardiol. 1989 Feb 1;63(5):347-52.
Clinical and echocardiographic comparison of patients with the carcinoid syndrome with and without carcinoid heart disease.
Himelman RB, Schiller NB.
Division of Medicine, University of California, San Francisco 94143.

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.

Am J Cardiol. 1982 Oct;50(4):762-8.
Echocardiographic features of carcinoid heart disease.
Callahan JA, Wroblewski EM, Reeder GS, Edwards WD, Seward JB, Tajik AJ.

We reviewed the records of the Mayo Clinic patients with known carcinoid syndrome in whom echocardiographic studies had been done. Nineteen patients had M-mode and 2-dimensional echocardiographic examinations, and 1 patient had an M-mode examination only. Of the 20 patients, 8 had no evidence by echocardiogram of carcinoid heart disease; 2 had changes in the tricuspid valve echogram suggestive of early carcinoid heart disease, and the other 10 patients had the following distinctive echocardiographic findings: (1) the pattern of right ventricular volume overload (enlarged right ventricle with abnormal septal motion); (2) abnormal right-sided valves, including (a) a striking appearance of the tricuspid valve, the leaflets appearing thickened, retracted, and fixed in a semiopen position throughout the cardiac cycle, and (b) thickened, retracted pulmonic valve cusps, when visualized; and (3) the left-sided valves and chambers rarely involved. These echocardiographic features are distinctive of advanced carcinoid heart disease and correlate closely with pathologic findings.

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