Carney's syndrome: complex myxomas. Report of four cases and review of the literature.

Cardiac myxomas are rare tumors. They usually appear as a sporadic isolated condition in the left atrium of middle-aged women with no other coincidental pathology. Carney and others have described in young people a special complex group of cardiac myxomas associated to a distinctive complex pathology, giving identity to the "Syndrome Myxoma" or "Carney's Syndrome". Four additional cases of this syndrome, treated from 1977 to 1999 at the Hospital Clínico de la Universidad de Chile are presented here with a comprehensive review of the literature, accumulating 100 cases. The main features of our cases include the presence of malignant non cardiac tumors, a familial trend, follow-up of 23 years and an iterative recurrence in the elder case. To date all patients are tumor free. Reviewing the literature, patients with Carney's Syndrome were younger, with a mean age of 26 years and female predominance (62%). Cardiac myxomas affected the four chambers of the heart: 64% the left atrium; 44% the right atrium; 14% the left ventricle and 12% the right ventricle. They were multiple tumors in 41% and involved more than one chamber in 31%, being synchronous or metachronous. There was a marked familial trend (52%), a high incidence of recurrence (20%), with more than one occurring in half the cases. Extra-cardiac involvement consisted of: 68% pigmented skin lesions, 40% cutaneous myxomas, 37% adrenal cortical disease, 27% myxoid mammary fibroadenoma and 34% male patients with testes tumors. A low percentage had pituitary adenoma, melanotic schwannomas and thyroid disease. The diagnosis is made when two or more of these criteria are present. In agreement with these findings the four chambers of the heart should be examined at surgery for atypical myxoma locations, right atriotomy and combined superior-transseptal approach improve exposure of the cavities, careful screening of the first degree family members should be conducted, and closed short and long term follow up controls are important. Complex myxoma appears as a multi-systemic disorder, occasionally having an ominous prognosis and malignant potentiality, and is still undergoing investigation for better understanding and identification.

Is the addition of ECG gating to technetium-99m sestamibi SPET of value in the assessment of myocardial viability? An evaluation based on two-dimensional echocardiography following revascularization.

The main goal of this study was to evaluate whether the addition of ECG gating to technetium-99m sestamibi single-photon emission tomographic (SPET) perfusion imaging assists the prediction of recovery of regional wall motion abnormalities after revascularization. Thirty-six patients with coronary artery disease were included in the study. All had wall motion abnormalities, and 31 (86%) had a clinical history of myocardial infarction. Coronary artery bypass surgery was performed in 18 patients and angioplasty in the remainder. All underwent ECG-gated and non-gated SPET at rest and after intravenous dipyridamole. Two-dimensional echocardiography was performed at a mean of 27 days before revascularization and at a mean of 69 days following revascularization to assess segmental wall motion changes. Perfusion prior to revascularization was analysed qualitatively and quantitatively on gated and non-gated SPET, and the results compared with those of echocardiography. Bullseye parameters were obtained from a normal database, generated from data in 40 normal volunteers, using dipyridamole ECG-gated and non-gated sestamibi SPET. There was good concordance between gated and non-gated qualitative analysis (79% with kappa=0.65) for normal, viable or necrotic segments. Gated SPET predicted functional recovery in 27 of 35 (77%) segments showing echocardiographic improvement while non-gated SPET did so in 30 of 39 (77%) such segments. Gated SPET predicted no functional recovery in 20 of 45 (44%) segments that did not show improved wall motion after revascularization, while with non-gated SPET the figure was 18 of 51 (35%). The positive predictive values of gated and non-gated SPET with regard to the recovery of wall motion following revascularization were 52% and 48%, while the negative predictive values were 71% and 67%, respectively. 99mTc-sestamibi had a low predictive value for recovery of function if visual assessment was used in the analysis of SPET data. Quantitative bullseye sestamibi parameters (defect extension and severity, reversibility and percentage change in extension), from gated or non-gated studies, appear best to distinguish which segments will display improved motility on the echocardiogram after revascularization. The addition of ECG gating does not significantly increase the predictive value of SPET imaging with regard to recovery of function.

[Evaluation of coronary revascularization: comparison between perfusion with dipyridamole Tc99m sestaMIBI and amrinone-echocardiography as predictors of viability]. / Evaluación de revascularización coronaria: comparación entre perfusión con Tc99m sestaMIBI-dipiridamol gatillado y ecografia-amrinona como predictores de viabilidad.

We studied left ventricular perfusion and motility in 37 patients with coronary artery disease and disturbances of parietal motility, aged 57 +/- 9 years old, before and after revascularization. Perfusion was assessed with dipyridamole-tc99m MIBI SPECT and motility was assessed with two dimensional echocardiography with amrinone. Myocardial segments were defined as normal, viable or necrotic in both studies. Eighteen subjects were subjected to angioplasty and 19 to coronary bypass surgery. Submitted SPECT and echocardiography were repeated 64 +/- 15 and 69 +/- 23 days after revascularization respectively. The concordance between SPECT and echocardiographic pre-revascularization diagnosis was 60.1% Eighty one percent of segments considered viable with SPECT and 71% thus considered with echocardiography improved after revascularization. Likewise, 50% of segments considered necrotic with SPECT and 68% of segment thus considered with echocardiography did not improve. It is concluded that although there is a good concordance between both methods, they have limitations on the study of myocardial viability and should be considered as second choice.

[Tomographic myocardial perfusion (SPECT) using 99mTc sestamibi-dipyridamole and 2-d-echocardiography with amrinone pre and post myocardial revascularization in 2 cases]. / Perfusión miocárdica tomografica (SPECT) con sestamibi Tc99m-dipiridamol y ecocardiografia-2D bajo acción de amrinona pre y post revascularización coronaria en dos casos.

We report two male patients aged 50 and 55 years old with coronary artery disease who were treated with surgical revascularization and angioplasty respectively. To assess viable and ischemic myocardial territories, they were studied before and after revascularization with radionuclide perfusion SPECT with Tc99m-Sestamibi under Dipyridamole and with 2-D echocardiography at baseline and after the use of amrinone. Improvement in relative perfusion on SPECT and wall motion on 2-D echocardiography indicated tissue recovery.

[Infectious endocarditis: clinical and echocardiographic initial follow-up]. / Endocarditis infecciosa: seguimiento inicial clínico-ecocardiográfico.

We analyzed the clinical and echocardiographic features of 24 patients with infective endocarditis followed for a mean of 20.2 month (range 3-84) after discharge. Mean age was 38 years, male to female ratio was 2:1 and 87% of cases had a subacute clinical course; 17% of patients had late prosthetic endocarditis. Positive blood cultures were obtained in only 50% of patients. Vegetations were detected by echocardiography in 88%, 66% of them located at the aortic valve. Heart failure (62%) was the main complication, leading to valve replacement in 4 patients. Four patients died during follow up, 3 males due to heart failure and a female from systemic emboli. Twenty patients survive at the end of follow up (84%), 50% of them in FC I or II, 40% in FC III or IV (2 lost to follow up). Eleven patients had a late echocardiogram at a mean of 12 months after discharge: 6 of them showed persistence of vegetations (55%).