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D Le Guludec

Publications and source records attributed to D Le Guludec.

At least 19 recordsLinked to original sources
PubMed2006

The regulatory background of nuclear cardiology in Europe: a survey by the European Council of Nuclear Cardiology.

PURPOSE: Nuclear cardiology is a well-validated, non-invasive imaging modality that is highly cost-effective as a diagnostic and prognostic tool in the evaluation of patients with known or suspected coronary artery disease. However, the number of procedures in Europe is very far from that which would be expected on the basis of epidemiological data, particularly when comparison is made with the USA. As a preliminary step for future action aimed at improving and increasing nuclear cardiology practice in Europe, the European Council of Nuclear Cardiology performed a survey to identify the regulatory issues and the training components pertaining to the practice of nuclear cardiology. METHODS: a questionnaire was sent to 31 national nuclear medicine societies and to 40 national cardiology societies. The main areas covered by the survey were: (1) the license requirements, (2) the theoretical and practical aspects of training and (3) supervision of the stress test during a nuclear cardiology study. RESULTS: The results show that, in a setting of wide heterogeneity of national regulations, education and professional practice, nuclear medicine is a restricted and closely regulated specialty. This situation guarantees the quality and safe use of radionuclides; at the same time, however, it limits integration of nuclear medicine into the clinical arena. CONCLUSION: Cardiologists should become more involved in nuclear cardiology, to further stimulate the use of this powerful diagnostic and prognostic imaging modality.

Cardiology↗
PubMed2003

Comparative impact of standard approach, FDG PET and FDG dual-head coincidence gamma camera imaging in preoperative staging of patients with non-small-cell lung cancer.

We prospectively compared the impact of the standard approach, of fluorodeoxyglucose positron emission tomography (FDG PET) and of FDG dual-head coincidence gamma camera imaging (DHC) in preoperative staging of patients with non-small-cell lung cancer (NSCLC). In addition to traditional staging, 42 patients were studied with a PET system and a DHC system. The number of lesions detected on DHC and on PET were compared independently of the proof of a tumoural invasion. Then, for the sub-group of lesions with the proof of a tumoural invasion, the sensitivity of the different imaging modalities was compared. Finally, stagings were compared with final staging established by histopathological findings (n=28), additional imaging modalities (n=4), clinical and traditional imaging follow-up over at least 4 months. DHC detected 105 of the 145 lesions considered as pathological on PET (73%, P=0.01), with a concurrence of 89% (NS) in lesions larger than 1.5 cm, and only 17% (P=0.03) in those smaller or equal to 1 cm. Traditional staging detected 87 of the 114 verified tumoural lesions (76%), PET 110/114 (96%, P=0.01 vs traditional staging), DHC 88/114 (77%, NS vs traditional staging, P=0.01 vs PET). PET correctly predicted the N stage in 39/42 (93%) patients, DHC in 38/42 (90%), and computed tomography in 32/42 (76%). PET correctly predicted the M stage in 42/42 (100%) patients, DHC in 41/42 (98%), and traditional staging in 38/42 (90%). Identical NM staging was obtained with DHC and PET in 38/42 (90%) patients. Compared to traditional NM staging, PET correctly up-staged 9/42 (21%) patients and down-staged 3/42 (7%), with one additional false N up-staging. DHC correctly up-staged 7/42 (17%) patients and down-staged 3/42 (7%), with one additional false N down-staging. PET correctly reclassified 4/42 (9.5%) patients from resectable to unresectable and incorrectly reclassified one. DHC correctly reclassified 3/42 (7%) patients without false therapeutic reclassification. Although DHC detected fewer lesions than PET, DHC is a possible alternative to PET since the impact on staging was high as compared with traditional staging and was very similar to that of PET.

Adult↗
PubMed2003

[Is coincidence detection emission tomography (CDET) as reliable as positron emission tomography (PET) in head and neck cancer?].

OBJECTIVE: Positron emission tomography (PET) is becoming more and more useful in head and neck tumour detection, guiding diagnostic and therapeutic decision. However, techniques, similar to PET, but using modified conventional tomography equipment, are used. The most commonly used is the dual-head positron emission tomography using coincidence detection (CDET). This study was aimed at searching if CDET could be as reliable as PET in some of its indications. PATIENTS AND METHODS: Between 1997 and 2001, 19 patients, with head and neck cancer, had a CDET, for 3 indications. We studied retrospectively sensitivity (Se) and specificity (Sp) rates for these 3 indications. RESULTS: 1 degree) Detection of unknown primary head and neck cancers with lymph node manifestation: Se = 100%, Sp = 66,7%. 2 degrees) Detection of body metastasis: Se = 50%, Sp = 33%. 3 degrees) Detection of local recurrent cancer: Se = 100%, Sp = 25%. CONCLUSION: CDET seems to be useful in finding unknown primary cancers with lymph node manifestation but is not reliable to look for metastasis and local recurrences. It cannot be used in all indications for PET. If no other tool is available, this investigation can be used, keeping in mind its limitations.

Diagnosis, Differential↗
PubMed2001

Myocardial muscarinic receptor upregulation and normal response to isoproterenol in denervated hearts by familial amyloid polyneuropathy.

BACKGROUND: Patients with familial amyloid polyneuropathy, a rare hereditary form of amyloidosis, have progressive autonomic neuropathy. The disease usually does not induce heart failure but is associated with sudden death, conduction disturbances, and an increased risk of complications during anesthesia. Although cardiac sympathetic denervation has been clearly demonstrated, the postsynaptic status of the cardiac autonomic nervous system remains unelucidated. METHODS AND RESULTS: Twenty-one patients were studied (age, 39+/-11 years; normal coronary arteries; left ventricular ejection fraction 68+/-9%). To evaluate the density and affinity constants of myocardial muscarinic receptors, PET with (11)C-MQNB (methylquinuclidinyl benzilate), a specific hydrophilic antagonist, was used. Cardiac beta-receptor functional efficiency was studied by the heart rate (HR) response to intravenous infusion of isoproterenol (5 minutes after 2 mg of atropine, 5, 10, and 15 ng/kg per minute during 5 minutes per step). The mean muscarinic receptor density was higher in patients than in control subjects (B'(max), 35.5+/-8.9 versus 26.1+/-6.7 pmol/mL, P=0.003), without change in receptor affinity. The increase in HR after injection of atropine as well as of MQNB was lower in patients compared with control subjects despite a similar basal HR (DeltaHR after atropine, 11+/-21% versus 62+/-17%; P<0.001), consistent with parasympathetic denervation. Incremental infusion of isoproterenol induced a similar increase in HR in patients and control subjects. CONCLUSIONS: Cardiac autonomic denervation in familial amyloid polyneuropathy results in an upregulation of myocardial muscarinic receptors but without change in cardiac beta-receptor responsiveness to catecholamines.

3-Iodobenzylguanidine↗
PubMed2001

Prognostic value of radionuclide angiography in patients with right ventricular arrhythmias.

BACKGROUND: The prognosis of patients with right ventricular (RV) arrhythmias remains uncertain. This study prospectively evaluated the prognostic value of RV and left ventricular (LV) involvement assessed by radionuclide angiography (RNA) as predictors for sudden death. METHODS AND RESULTS: Patients (n=188) with severe arrhythmias originating from the RV were followed up for a mean of 45+/-34 months. Data on clinical presentation, resting and stress ECG, signal-averaged ECG, 24-hour Holter monitoring, and programmed stimulation were collected along with RNA. Patients were classified as group I (n=82) with normal RNA or group II (n=106) with an abnormal RV suggestive of arrhythmogenic RV cardiomyopathy, classified as diffuse or localized disease, with or without associated LV abnormalities. During follow-up, 14 patients died suddenly, all in group II. None of the clinical and electrical data were predictive of death. An abnormal RNA study was a highly predictive factor for death (P<0.005), as well as the presence of LV abnormalities (P<0.01). CONCLUSIONS: The present study confirms that arrhythmogenic RV cardiomyopathy is a severe disease with a high risk for cardiac death. Evidence of RV abnormalities in patients presenting with RV arrhythmias is highly predictive for sudden death, as is its association with LV involvement.

Adolescent↗
PubMed2001

Myocarditis in patients with clinical presentation of myocardial infarction and normal coronary angiograms.

OBJECTIVES: The aim of this study was to assess the diagnosis of myocarditis in patients presenting with acute myocardial infarction (MI) and normal coronary angiograms. BACKGROUND: Most often in these patients, the etiologic diagnosis remains unclear once they are found to have normal coronary arteries. The diagnosis of myocarditis mimicking MI is clinically relevant, because numerous arguments suggest a relation between myocarditis and dilated cardiomyopathy. Myocardial indium-111 (111In)-antimyosin antibody (AMA)/rest thallium-201 (201Tl) imaging allows noninvasive detection of myocarditis. METHODS: Forty-five patients admitted to three intensive care units for suspicion of acute MI, with normal coronary angiograms, were investigated. Indium-111-AMA planar images and then a dual-isotope rest AMA/201Tl tomographic study were performed. Six-month echocardiographic follow-up was obtained in 80% of the patients with initial left ventricular (LV) wall motion abnormalities. RESULTS: In eight patients, AMA and 201Tl scintigraphy were negative. In two patients, a matched 201Tl defect and focal AMA uptake suggested acute MI (due to prolonged vasospasm or spontaneously reperfused coronary occlusion). In 17 patients, diffuse AMA uptake over the whole LV suggested diffuse myocarditis. In 18 patients, focal AMA uptake with a normal 201Tl scan suggested diffuse but heterogeneous, or focal myocarditis. Complete functional recovery was observed in 81% of the patients with a pattern of myocarditis. CONCLUSIONS: Among 45 patients presenting with acute MI and normal coronary angiograms, 38% had diffuse myocarditis and 40% had a scintigraphic pattern of heterogeneous or focal myocarditis. Short-term follow-up showed complete LV functional recovery in 81% of these patients.

Adult↗
PubMed2001

Prognostic value of 201Tl myocardial scintigraphy after coronary artery bypass grafting.

BACKGROUND: 201Tl myocardial scintigraphy (201Tl SPECT) is of strong prognostic value in various populations with suspected or known coronary artery disease. However, its value in patients with coronary artery bypass grafting (CABG) is not fully assessed. METHODS: We examined 115 consecutive patients to determine the relation between clinical data/stress 201Tl SPECT performed 5+/-3 years after CABG, and subsequent cardiac events. RESULTS: Thirteen patients (11%) had stress-induced angina, 22 (19%) had electrical positivity, and 97 (84%) had abnormal scintigraphy, including 62 (54%) with reversible defects. During follow-up (35+/-22 months), there were nine cardiac deaths, seven myocardial infarctions, and 20 revascularization procedures. Multivariate Cox analysis identified the delay between CABG and scintigraphy (P<0.01, relative risk (RR) = 1.01), the extent of stress 201Tl defects (P = 0.04, RR = 1.18), and increased stress 201Tl lung uptake (P = 0.03, RR = 3.56) as significant predictors of cardiac deaths/infarctions. Delay between CABG and scintigraphy (P < 0.001, RR = 1.01), the extent of stress 201Tl defects (P = 0.03, RR = 1.15), and that of reversible defects (P = 0.05, RR = 1.13) were the only significant predictors of total events. CONCLUSIONS: Besides the delay between CABG and scintigraphy, the scintigraphic parameters were the only significant and additive predictors of cardiac events in 115 patients with CABG.

Adult↗
PubMed2001

Somatostatin receptor scintigraphy and gallium scintigraphy in patients with sarcoidosis.

UNLABELLED: Somatostatin receptor scintigraphy (SRS) has been shown to reveal sarcoidosis sites. The aim of this study was to prospectively compare SRS and gallium scintigraphy in the evaluation of pulmonary and extrapulmonary involvement in patients with proven sarcoidosis. METHODS: Eighteen patients with biopsy-proven sarcoidosis were included. Nine were or recently had been receiving steroid therapy at the time of the examination. Planar gallium scintigraphy (head, chest, abdomen, and pelvis) and thoracic SPECT were performed at 48-72 h after injection of a mean dose of 138 +/- 21 MBq 67Ga. Planar SRS and thoracic SPECT were performed at 4 and 24 h after injection of a mean dose of 148 +/- 17 MBq 111n-pentetreotide. RESULTS: Gallium scintigraphy found abnormalities in 16 of 18 patients (89%) and detected 64 of 99 clinically involved sites (65%). SRS found abnormalities in 18 of 18 patients and detected 82 of 99 clinically involved sites (83%). Of the 9 treated patients, gallium scintigraphy found abnormalities in 7 (78%), detecting 23 of 39 clinically involved sites (59%), whereas SRS found abnormalities in 9, detecting 32 of 39 clinically involved sites (82%). CONCLUSION: This study suggests that, compared with gallium scintigraphy, SRS appears to be accurate and contributes to a better evaluation of organ involvement in sarcoidosis patients, especially those treated with corticosteroids.

Adrenal Cortex Hormones↗
PubMed2001

[Evaluation of the ejection fraction tomoscintigraphy synchronised with an electrocardiogram].

Electrocardiographic gated myocardial scintigraphy (gated SPECT) allows simultaneous evaluation of left ventricular perfusion and contractility. The aim of this study was to assess the impact of the type of tracer and temporal sampling on the measurement of the ejection fraction by gated SPECT. Eighty-six consecutive patients (59 men, 27 women, average age 62 +/- 11 years) with a history of myocardial infarction (anterior N = 50--inferior N = 36) were studied. All had measurement of the isotopic ejection fraction by equilibrium angioscintigraphy and by gated SPECT sampled at 8 (N = 45, 52%) or 16 images/cycle RR (N = 41, 48%). After filtered retroprojection, the ejection fraction was calculated by a validated method (QGS). A better correlation between gated SPECT and equilibrium angioscintigraphy was observed at 16 images/cycle (r = 0.90) compared with 8 (r = 0.80). The Bland-Altman curves showed improved accuracy of the measurement of the ejection fraction by gated SPECT with 16 compared with 8 images/cycles sampling (mean differences of 0.37 +/- 6.06% and 4.3 +/- 7.71%, respectively). Multivariable analysis showed that temporary sampling was the only parameter to significantly affect the difference between the two methods. There was no difference between the use of Thallium and that of 99mTc-sestamibi. Gated SPECT, therefore, provides an accurate measurement of the ejection fraction for routine clinical usage, providing the acquisition is made at 16 images/cycle.

Electrocardiography↗
PubMed2001

Electrocardiographically gated blood-pool SPECT and left ventricular function: comparative value of 3 methods for ejection fraction and volume estimation.

UNLABELLED: The current major limitation to development of electrocardiographically (ECG) gated blood-pool SPECT (GBPS) for measurement of the left ventricular (LV) ejection fraction (LVEF) and volumes is the lack of availability of clinically validated automatic processing software. Recently, 2 processing software methods for quantification of the LV function have been described. Their LVEFs have been validated separately, but no validation of the LV volume measurement has been reported. METHODS: We compared 3 processing methods for evaluation of the LVEF (n = 29) and volumes (n = 58) in 29 patients: automatic geometric method (GBPS(G)), semiautomatic activity method (GBPS(M)), and 35% maximal activity manual method (GBPS(35%)). The LVEF provided by the ECG gated equilibrium planar left anterior oblique view (planar(LAO)) and the LV volumes provided by LV digital angiography (Rx) were used as gold standards. RESULTS: Whereas the GBPS(G) and GBPS(M) methods present similar low percentage variabilities, the GBPS(35%) method provided the lowest percentage variabilities for the LVEF and volume measurements (P < 0.04 and P < 0.02, respectively). The LVEF and volume provided by the 3 methods were highly correlated with the gold standard methods (r > 0.98 and r > 0.83, respectively). The LVEFs provided by the GBPS(35%) and GBPS(M) methods are similar and higher than those of the GBPS(G) method and planar(LAO) method, respectively (P < 0.0001). For the LVEF, there is no correlation between the average and paired absolute difference for the 3 GBPS methods against the planar(LAO) method, and the limits of agreement are relatively large. LV volumes are lower when calculated with the GBPS(M), GBPS(G), and Rx methods (P < 0.0001). However, the GBPS(35%) and Rx methods provide LV volumes that are similar. There is no linear correlation between the average and the paired absolute difference of volumes calculated with the GBPS(G) and GBPS(35%) methods against Rx LV volumes. However, a moderate linear correlation was found with the GBPS(M) method (r = 0.6; P = 0.0001). The 95% limits of agreement between the Rx LV volumes and the 3 GBPS methods are relatively large. CONCLUSION: GBPS is a simple, highly reproducible, and accurate technique for the LVEF and volume measurement. The reported findings should be considered when comparing results of different methods (GBPS vs. planar(LAO) LVEF; GBPS vs. Rx volume) and results of different GBPS processing methods.

Electrocardiography↗
PubMed2001

Clinical impact of combination of scatter, attenuation correction, and depth-dependent resolution recovery for (201)Tl studies.

UNLABELLED: A lack of specificity for myocardial perfusion imaging has been widely reported, mostly related to false-positive defects on the inferior wall. The application of depth-dependent resolution recovery (RR), attenuation correction (AC) using external source devices, and scatter correction has been proposed to resolve this pitfall. METHODS: We studied the clinical benefit of depth-dependent RR, nonuniform AC using a scanning line source, and scatter correction (photon energy recovery [PER]) compared with filtered backprojection alone. Eighty-two patients were included: 40 healthy volunteers with a low likelihood of coronary artery disease (control group) and 42 patients with proven right or circumflex coronary artery disease but without involvement of the left anterior descending artery. Among these 82 patients, the images of 33 were also processed with PER. RESULTS: RR did not alter the performance of filtered backprojection alone. AC + RR greatly improved specificity and the rate of normal (201)Tl SPECT findings in the control population (from 56% to 95% and from 53% to 100%, respectively) but significantly decreased sensitivity (from 92% to 54%). AC + RR generated a false anteroapical defect in 21% of patients and reverse redistribution of the apex in 23%. AC + RR significantly decreased the extent of the stress defect (from 4.09 to 3.21 segments, P < 0.003) and increased the perfusion score of the stress defect (from 0.78 +/- 0.72 to 1.47 +/- 1.11, P < 0.00061). Moreover, AC + RR generated overcorrection on the inferior wall, leading to false estimation of viability for 11 of 15 patients with an old inferior myocardial scar without evidence of residual viability. PER decreased overcorrection on the inferior wall, but without improving sensitivity. PER did not significantly reduce the number of anteroapical false-positives or the number of apical reverse distribution cases. CONCLUSION: AC + RR improved the specificity and normalcy rate of (201)Tl SPECT myocardial perfusion imaging but generated overcorrection on the inferior wall, leading to low sensitivity and to false evaluation of myocardial viability in 73% of the patients with inferior infarction. AC + RR also generated anteroapical artifacts. The addition of scatter correction did not significantly reduce these drawbacks.

Coronary Circulation↗
PubMed2000

Relationship between resting 201Tl reverse redistribution, microvascular perfusion, and functional recovery in acute myocardial infarction.

UNLABELLED: 201TI reverse redistribution is a common finding early after reperfusion therapy for myocardial infarction. Its mechanism and clinical implications remain unclear. The aim of this study was to clarify the relationships between reverse redistribution, microvascular perfusion, and myocardial viability. METHODS: Resting, 10-min-postinjection, and redistribution 201TI data obtained for 33 patients 8 and 42 d after the onset of acute myocardial infarction were compared with echocardiographic wall motion measured acutely and on day 42. Microvascular perfusion was assessed by myocardial contrast echocardiography performed 10 min after restoration of complete patency of the infarct artery. RESULTS: Marked significant reverse redistribution was found on day 8 (absolute change, 7.5%+/-7.9% of the 10-min-postinjection defect size; P<5x0.000001) and significantly decreased on day 42 (2.7%+/-6.8%; P = 0.004 between days 8 and 42). The 10-min-postinjection defect size best predicted the final infarct size on day 42 and was closely related to microvascular perfusion. Patients with adequate reperfusion had a smaller postinjection defect on day 8 (21.1%+/-14.6%) and a larger reverse redistribution (10.2%+/-6.1%) than did patients with no reflow (35.3%+/-13% and 3.2%+/-9.2%, respectively; P<0.04 for both). CONCLUSION: Reverse redistribution was marked early after myocardial infarction in patients with complete patency of the infarct artery and decreased in subsequent weeks. Reverse redistribution was associated with restoration of adequate microvascular reperfusion and with myocardial salvage and viability. The early postinjection scans on day 8 were the relevant images for assessing myocardial salvage and predicting wall motion recovery.

Coronary Circulation↗
PubMed2000

Diagnosis of extensive coronary artery disease: intrinisic value of increased lung 201 T1 uptake with exercise SPECT.

UNLABELLED: Exercise lung 201 TI uptake calculated with planar imaging has an important diagnostic and prognostic value in patients with coronary artery disease (CAD). However, its value with SPECT imaging raises methodological concerns and is controversial. We studied its value for the discrimination between extensive (E) and limited (L) angiographic CAD with exercise SPECT. METHODS: Four methods of lung-to-heart ratio quantification were calculated in patients with a low likelihood (< 5%) of CAD (n = 62). Their dependent variables were defined, and corresponding correction equations were derived. Receiver operating characteristic (ROC) analysis was performed in a pilot group (L-CAD, n = 49; E-CAD, n = 126) to define the optimal method of calculation of the lung-to-heart ratio. Its best threshold providing the best sensitivity for a specificity of 90% was defined. After correction for dependent variables, the 4 methods were also compared by ROC analysis and the optimal corrected method was compared with the optimal uncorrected method using ROC analysis and the best threshold. The consistency of these results in the validation group (L-CAD, n = 41; E-CAD, n = 122) and of the results of visual analysis of lung 201TI uptake were then verified. RESULTS: On ROC analysis in the pilot group, the optimal method of calculation of the lung-to-heart ratio was the mean activity in a region of interest drawn at the base of the lungs to the mean activity over the heart (Lb/H). For the best threshold, Lb/H presented a sensitivity of 34%. Corrected Lb/H still remained the best method of calculation on ROC analysis compared with the other corrected methods. On ROC analysis, there was no difference between corrected and uncorrected Lb/H. For the best threshold, corrected Lb/H presented a similar sensitivity of 37% compared with uncorrected Lb/H. When applied to the validation group (L-CAD, n = 41; E-CAD, n = 122), the best-defined threshold in the pilot group for corrected Lb/H presented a diagnostic value similar to that in the pilot group (sensitivity, 41%; specificity, 90%), but uncorrected Lb/H presented a higher sensitivity (47%; P < 0.04) and a slightly lower specificity (80%). Results of lung 201TI uptake visual analysis were inconsistent between pilot and validation groups (42% versus 58% sensitivity, P = 0.012; 86% versus 66% specificity, P = 0.023). CONCLUSIONS: For evaluation of E-CAD versus L-CAD, quantification of the exercise lung-to-heart 201TI uptake ratio with SPECT is feasible, reproducible, more discriminate than simple visual analysis, and best calculated as Lb/H. It presents an intrinsic diagnostic value even after correction for other clinically valuable dependent variables.

Case-Control Studies↗
PubMed2000

111In-pentetreotide scintigraphy in patients with Langerhans' cell histiocytosis.

UNLABELLED: Langerhans' cell histiocytosis is a granulomatous disease that may involve multiple organs and the prognosis of which is highly variable. Because the prognosis depends particularly on the number of tissues involved, the accurate identification of the organs involved by granulomatous lesions is of critical importance. We hypothesized that 111In-pentetreotide scintigraphy would be useful for evaluation of patients with Langerhans' cells histiocytosis. METHODS: Thirteen patients (38.3+/-10.4 y) with Langerhans' cell histiocytosis (8 patients with unifocal lung disease, 5 with multifocal disease) received intravenous 111In-pentetreotide (111-222 MBq), and planar images were obtained at 24 h after injection. Pulmonary uptake was quantified using a lung-to-background ratio (L/B) and compared with a population of 10 normal scintigrams. For the other sites, uptake of radioactivity in disease-related areas was visually assessed. RESULTS: Ten of 12 patients with lung involvement had increased lung uptake (UB, 2.23+/-0.49 versus 1.34+/-0.07; P < 0.001). In the patients with multifocal disease, increased 111In-pentetreotide uptake was found in disease-related areas such as the salivary glands, the skin, the soft tissues, and the bones. However, somatostatin receptor imaging was insensitive for detecting central nervous system and liver involvement and most skin lesions. CONCLUSION: 111In-pentetreotide imaging may be useful in Langerhans' cell histiocytosis. Further study will indicate whether 111In-pentetreotide is a relevant tracer in the management of histiocytosis.

Adult↗
PubMed1999

Cardiac sympathetic denervation in familial amyloid polyneuropathy assessed by iodine-123 metaiodobenzylguanidine scintigraphy and heart rate variability.

Familial amyloid polyneuropathy (FAP) is a rare and severe hereditary form of amyloidosis, due to nervous deposits of a genetic variant transthyretin produced by the liver and characterized by both sensorimotor and autonomic neuropathy. Left ventricular systolic dysfunction is rare, but conduction disturbances and sudden deaths can occur. The neurological status of the heart has not been elucidated, and an alteration of the sympathetic nerves may be involved. We studied 17 patients (42+/-12 years) before liver transplantation by iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy, heart rate variability analysis, coronary angiography, radionuclide ventriculography, rest thallium single-photon emission tomography (SPET) and echocardiography. Coronary arteries, left ventricular systolic function and rest thallium SPET were normal in all patients. Only mild evidence of amyloid infiltration was found at echocardiographic examination. Cardiac MIBG uptake was dramatically decreased in patients compared with age-matched control subjects (heart-to-mediastinum activity ratio at 4 h: 1.36+/-0.26 versus 1.98+/-0.35, P<0.001), while there was no difference in MIBG washout rate. Heart rate variability analysis showed a considerable scatter of values, with high values in four patients despite cardiac sympathetic denervation as assessed by MIBG imaging. The clinical severity of the polyneuropathy correlated with MIBG uptake at 4 h but not with the heart rate variability indices. Cardiac MIBG uptake and the heart rate variability indices did not differ according to the presence or absence of conduction disturbances. Patients with FAP have sympathetic cardiac denervation as assessed by MIBG imaging despite a preserved left ventricular systolic function and cardiac perfusion, without correlation with conduction disturbances. Results of the heart rate variability analysis were more variable and this technique does not seem to be the best way to evaluate the extent of cardiac sympathetic denervation in FAP patients.

3-Iodobenzylguanidine↗