RESUMO
OBJECTIVES: Myocardial extracellular volume (ECV) on computed tomography (CT), an alternative to cardiac magnetic resonance (CMR), has significant practical clinical advantages. However, the consistency between ECVs quantified via CT and CMR in cardiac amyloidosis (CA) has not been investigated sufficiently. Therefore, the current study investigated the application of CT-ECV in CA with CMR-ECV as the reference standard. METHODS: We retrospectively evaluated 31 patients with CA who underwent cardiac CT and CMR. Pearson correlation analysis was performed to investigate correlations between CT-ECV and CMR-ECV at each segment. Further, correlations between ECV and clinical parameters were assessed. RESULTS: There were no significant differences in the mean global ECVs between CT scan and CMR (51.3% ± 10.2% vs 50.0% ± 10.5%). CT-ECV was correlated with CMR-ECV at the septal (r = 0.88), lateral (r = 0.80), inferior (r = 0.79), anterior (r = 0.77) segments, and global (r = 0.87). In both CT and CMR, the ECV had a weak to strong correlation with high-sensitivity cardiac troponin T level, a moderate correlation with global longitudinal strain, and an inverse correlation with left ventricular ejection fraction. Further, the septal ECV and global ECV had a slightly higher correlation with the clinical parameters. CONCLUSIONS: Cardiac CT can quantify myocardial ECV and yield results comparable to CMR in patients with CA. Moreover, a significant correlation between CT-ECV and clinical parameters was observed. Thus, CT-ECV can be an imaging biomarker and alternative to CMR-ECV. CLINICAL RELEVANCE STATEMENT: Cardiac CT can quantify myocardial ECV and yield results comparable to CMR in patients with CA, and CT-ECV can be used clinically as an imaging biomarker and alternative to CMR-ECV. KEY POINTS: ⢠A significant correlation was found between CT myocardial extracellular volume and cardiac MR myocardial extracellular volume in patients with cardiac amyloidosis. ⢠In CT and cardiac MR, the myocardial extracellular volume correlated well with high-sensitivity cardiac troponin T level, global longitudinal strain, and left ventricular ejection fraction. ⢠CT myocardial extracellular volume can be an imaging biomarker and alternative to cardiac MR myocardial extracellular volume.
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Amiloidose , Troponina T , Humanos , Volume Sistólico , Estudos Retrospectivos , Imagem Cinética por Ressonância Magnética/métodos , Função Ventricular Esquerda , Miocárdio/patologia , Imageamento por Ressonância Magnética , Amiloidose/diagnóstico por imagem , Biomarcadores , Valor Preditivo dos TestesRESUMO
AIMS: Wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) is often accompanied by atrial fibrillation (AF), atrial flutter (AFL), and atrial tachycardia (AT), which are difficult to control because beta-blockers and antiarrhythmic drugs can worsen heart failure (HF). This study aimed to investigate the outcomes of catheter ablation (CA) for AF/AFL/AT in patients with ATTRwt-CM and propose a treatment strategy for CA. METHODS AND RESULTS: A cohort study was conducted on 233 patients diagnosed with ATTRwt-CM, including 54 who underwent CA for AF/AFL/AT. The background of each arrhythmia and the details of the CA and its outcomes were investigated. The recurrence-free rate of AF/AFL/AT overall in ATTRwt-CM patients with multiple CA was 70.1% at 1-year, 57.6% at 2-year, and 44.0% at 5-year follow-up, but CA significantly reduced all-cause mortality [hazard ratio (HR): 0.342, 95% confidence interval (CI): 0.133-0.876, P = 0.025], cardiovascular mortality (HR: 0.378, 95% CI: 0.146-0.981, P = 0.045), and HF hospitalization (HR: 0.488, 95% CI: 0.269-0.889, P = 0.019) compared with those without CA. There was no recurrence of the cavotricuspid isthmus (CTI)-dependent AFL, non-CTI-dependent simple AFL terminated by one linear ablation, and focal AT originating from the atrioventricular (AV) annulus or crista terminalis eventually. Twelve of 13 patients with paroxysmal AF and 27 of 29 patients with persistent AF did not have recurrence as AF. However, all three patients with non-CTI-dependent complex AFL not terminated by a single linear ablation and 10 of 13 cases with focal AT or multiple focal ATs originating beyond the AV annulus or crista terminalis recurred even after multiple CA. CONCLUSION: The outcomes of CA for ATTRwt-CM were acceptable, except for multiple focal AT and complex AFL. Catheter ablation may be aggressively considered as a treatment strategy with the expectation of improving mortality and hospitalization for HF.
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Neuropatias Amiloides Familiares , Fibrilação Atrial , Flutter Atrial , Cardiomiopatias , Ablação por Cateter , Humanos , Ablação por Cateter/efeitos adversos , Masculino , Flutter Atrial/cirurgia , Flutter Atrial/etiologia , Feminino , Fibrilação Atrial/cirurgia , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/fisiopatologia , Idoso , Neuropatias Amiloides Familiares/cirurgia , Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/mortalidade , Cardiomiopatias/mortalidade , Cardiomiopatias/terapia , Resultado do Tratamento , Pessoa de Meia-Idade , Recidiva , Taquicardia Supraventricular/cirurgia , Taquicardia Supraventricular/etiologia , Taquicardia Supraventricular/fisiopatologia , Taquicardia Supraventricular/diagnóstico , Estudos Retrospectivos , Pré-Albumina/genética , Pré-Albumina/metabolismoRESUMO
AIMS: This study aimed to identify factors for attention leading to future pacing device implantation (PDI) and reveal the necessity of prophylactic PDI or implantable cardioverter-defibrillator (ICD) implantation in transthyretin amyloid cardiomyopathy (ATTR-CM) patients. METHODS AND RESULTS: This retrospective single-center observational study included consecutive 114 wild-type ATTR-CM (ATTRwt-CM) and 50 hereditary ATTR-CM (ATTRv-CM) patients, neither implanted with a pacing device nor fulfilling indications for PDI at diagnosis. As a study outcome, patient backgrounds were compared with and without future PDI, and the incidence of PDI in each conduction disturbance was examined. Furthermore, appropriate ICD therapies were investigated in all 19 patients with ICD implantation. PR-interval ≥220 msec, interventricular septum (IVS) thickness ≥16.9â mm, and bifascicular block were significantly associated with future PDI in ATTRwt-CM patients, and brain natriuretic peptide ≥35.7â pg/mL, IVS thickness ≥11.3â mm, and bifascicular block in ATTRv-CM patients. The incidence of subsequent PDI in patients with bifascicular block at diagnosis was significantly higher than that of normal atrioventricular (AV) conduction in both ATTRwt-CM [hazard ratio (HR): 13.70, P = 0.019] and ATTRv-CM (HR: 12.94, P = 0.002), whereas that of patients with first-degree AV block was neither (ATTRwt-CM: HR: 2.14, P = 0.511, ATTRv-CM: HR: 1.57, P = 0.701). Regarding ICD, only 2 of 16 ATTRwt-CM and 1 of 3 ATTRv-CM patients received appropriate anti-tachycardia pacing or shock therapy, under the number of intervals to detect for ventricular tachycardia of 16-32. CONCLUSIONS: According to our retrospective single-center observational study, prophylactic PDI did not require first-degree AV block in both ATTRwt-CM and ATTRv-CM patients, and prophylactic ICD implantation was also controversial in both ATTR-CM. Larger prospective, multi-center studies are necessary to confirm these results.
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Bloqueio Atrioventricular , Cardiomiopatias , Desfibriladores Implantáveis , Humanos , Pré-Albumina/genética , Estudos Retrospectivos , Estudos Prospectivos , Doença do Sistema de Condução Cardíaco , Bloqueio de Ramo , Ecocardiografia , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/terapiaRESUMO
BACKGROUND: The accurate sensitivity of amyloid deposition in extracardiac tissue (subcutaneous tissue and gastrointestinal tract) has not been evaluated in transthyretin amyloidosis cardiomyopathy (ATTR-CM) patients. This study aimed to evaluate the sensitivity of amyloid deposition in obtained endomyocardial and extracardiac biopsies.MethodsâandâResults: This study retrospectively evaluated 175 consecutive ATTR-CM patients (wild-type [ATTRwt]: 134, hereditary [ATTRv]: 41) who had positive findings on 99 mTc-labeled pyrophosphate (99 mTc-PYP) scintigraphy and underwent tissue biopsy of at least one organ (subcutaneous tissue, gastrointestinal tract, and endomyocardium). Amyloid deposition was observed in the subcutaneous tissue of 57/150 patients (38%), gastrointestinal tract of 80/131 patients (61%), and endomyocardium of 108/109 patients (99%). Compared to patients with ATTRv, ATTRwt had significantly lower sensitivity in subcutaneous tissue (73% vs. 25%, P<0.01) and tended to be lower in the gastrointestinal tract (74% vs. 57%, P=0.08) biopsies. Among 124 patients who underwent both subcutaneous tissue and gastrointestinal tract biopsies, amyloid was detected in at least 1 specimen in 91 (73%) patients. The sensitivity of the combination of extracardiac biopsies was 66% and 94% in ATTRwt-CM and ATTRv-CM, respectively. Multivariate analysis reveals that ATTRv was the only significant predictor of amyloid deposition in the subcutaneous tissue. CONCLUSIONS: Subcutaneous tissue and gastrointestinal tract biopsy sensitivity are inadequate, especially in patients with ATTRwt; however, the combination of these extracardiac biopsies contributes to increased sensitivity in patients with positive 99 mTc-PYP scintigraphy findings.
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Neuropatias Amiloides Familiares , Cardiomiopatias , Neuropatias Amiloides Familiares/diagnóstico por imagem , Biópsia , Cardiomiopatias/diagnóstico por imagem , Difosfatos , Humanos , Cintilografia , Estudos Retrospectivos , Pirofosfato de Tecnécio Tc 99mRESUMO
BACKGROUND AND AIMS: Although antithrombotic treatments are established for coronary artery disease (CAD), they increase the bleeding risk, especially in malnourished patients. The total thrombus-formation analysis system (T-TAS) is useful for the assessment of thrombogenicity in CAD patients. Here, we examined the relationships among malnutrition, thrombogenicity and 1-year bleeding events in patients undergoing percutaneous coronary intervention (PCI). METHODS AND RESULTS: This was a retrospective analysis of 300 consecutive CAD patients undergoing PCI. Blood samples obtained on the day of PCI were used in the T-TAS to compute the thrombus formation area under the curve. We assigned patients to two groups based on the geriatric nutritional risk index (GNRI): 102 patients to the lower GNRI group (≤98), 198 patients to the higher GNRI group (98<). The primary endpoint was the incidence of 1-year bleeding events defined by Bleeding Academic Research Consortium criteria types 2, 3, or 5. The T-TAS levels were lower in the lower GNRI group than in the higher GNRI group. Kaplan-Meier analysis showed worse 1-year bleeding event-free survival in the lower GNRI group compared with the higher GNRI group. The combined model of the GNRI and the Academic Research Consortium for High Bleeding Risk (ARC-HBR) had good calibration and discrimination for bleeding risk prediction. In addition, having a lower GNRI and ARC-HBR positivity was associated with 1-year bleeding events. CONCLUSION: A lower GNRI could reflect low thrombogenicity evaluated by the T-TAS and determine bleeding risk in combination with ARC-HBR positivity.
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Doença da Artéria Coronariana , Desnutrição , Intervenção Coronária Percutânea , Trombose , Idoso , Doença da Artéria Coronariana/diagnóstico , Doença da Artéria Coronariana/terapia , Hemorragia/induzido quimicamente , Humanos , Desnutrição/diagnóstico , Intervenção Coronária Percutânea/efeitos adversos , Inibidores da Agregação Plaquetária/efeitos adversos , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Trombose/diagnóstico , Trombose/epidemiologia , Trombose/etiologia , Resultado do TratamentoRESUMO
An 83-year-old man suddenly lost consciousness, and his blood pressure dropped. Results of blood analysis were within normal range. A chest radiograph showed enlargement of the left mediastinum. Computed tomographic scan of the chest showed a large mass surrounding the left common carotid artery in the left upper mediastinum. Histology revealed a large B-cell lymphoma. A Holter ECG showed transient sinus bradycardia and atrioventricular block. The power spectrum revealed increase in power of high frequency, suggesting that general vagal activity might be related to bradycardia. Evaluation of autonomic system from high frequency and low frequency components may be useful in examining the mechanisms of sudden bradycardia and/or hypotension of unknown origin.
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Linfoma , Neoplasias do Mediastino , Idoso de 80 Anos ou mais , Sistema Nervoso Autônomo , Bradicardia/diagnóstico , Bradicardia/etiologia , Humanos , Masculino , Neoplasias do Mediastino/complicações , Neoplasias do Mediastino/diagnóstico , Síncope/etiologia , Nervo VagoRESUMO
BACKGROUND: We investigated the clinical significance of the derivative of reactive oxygen metabolites (DROM), a new marker of reactive oxygen species (ROS), in patients with heart failure (HF) with reduced left ventricular ejection fraction (LVEF) (HFrEF). METHODS AND RESULTS: Serum DROM concentrations were measured in 201 consecutive patients with HFrEF (EF < 50%) in stable condition. DROM values were significantly higher in patients with HFrEF than in risk-matched patients without HF (P < 0.01). They also correlated significantly with high-sensitivity C-reactive protein and B-type natriuretic peptide. Kaplan-Meier analysis demonstrated significantly higher probabilities of HF-related events in the high-DROM group than in the low-DROM group (log-rank test, P < 0.01). Multivariable Cox hazard analysis revealed that DROM were independent and significant predictors of cardiovascular events. In a subgroup analysis, DROM levels were also measured at the aortic root and coronary sinus in 49 patients. The transcardiac gradient of DROM values was significantly higher in patients with HFrEF than in patients without HF (Pâ¯=â¯0.04), indicating an association between DROM production in the coronary circulation and HFrEF development. Changes in DROM following optimal therapy were significantly associated with LVEF improvement (râ¯=â¯0.34, Pâ¯=â¯0.04). CONCLUSIONS: The higher levels of DROM and their association with cardiovascular events suggest the clinical benefit of DROM measurements in the risk stratification of patients with HFrEF.
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Insuficiência Cardíaca , Humanos , Peptídeo Natriurético Encefálico , Estresse Oxidativo , Prognóstico , Volume Sistólico , Função Ventricular EsquerdaRESUMO
Cardiac amyloidosis (CA) has long been recognized as a rare disease. However, recent advances in cardiac imaging have led to increased identification of hidden CA in patients diagnosed with heart failure. This shift suggests that the actual incidence of CA is underestimated. The prognosis of CA is generally poor, especially in patients with advanced heart failure. However, recent developments in therapeutic interventions have improved the survival of patients with CA. An early diagnosis and interventions involving effective therapies are essential contributors to improved prognoses. Recent noninvasive diagnostic imaging modalities such as echocardiography, cardiac MRI, and nuclear imaging have facilitated the precise and early diagnosis of CA and enabled the initiation of appropriate management. The authors present an updated review of the clinical features of CA, including a discussion of current trends in noninvasive diagnostic imaging. ©RSNA, 2020.
Assuntos
Amiloidose/diagnóstico por imagem , Diagnóstico por Imagem/tendências , Cardiopatias/diagnóstico por imagem , Diagnóstico Precoce , Humanos , PrognósticoRESUMO
BACKGROUND: We previously reported that a high score (2 or 3 points) according to the Kumamoto criteria, a combination of high-sensitivity cardiac troponin T (hs-cTnT) ≥0.308 ng/mL, the length of QRS ≥ 120 ms in electrocardiogram, and left ventricular (LV) posterior wall thickness ≥ 13.6 mm, increases the pretest probability of 99m Tc-labeled pyrophosphate (99m Tc-PYP) scintigraphy in patients with suspected transthyretin amyloid cardiomyopathy (ATTR-CM). However, some patients with a low score (0 or 1 point) show positive findings on 99m Tc-PYP scintigraphy. Therefore, we evaluated the usefulness of additional examinations, including echocardiographic assessment of myocardial strain, to raise the pretest probability of 99m Tc-PYP scintigraphy for these patients. METHODS AND RESULTS: We examined 109 consecutive patients aged ≥70 years with low scores according to the Kumamoto criteria who underwent 99m Tc-PYP scintigraphy. Nineteen patients (17%) had positive 99m Tc-PYP scintigraphy findings. The relative apical longitudinal strain (LS) index (apical LS/ basal LS + mid LS) (RapLSI) was significantly higher in patients with positive than negative 99m Tc-PYP scintigraphy findings (1.04 ± 0.37 vs 0.70 ± 0.28, P < .01). Multivariable logistic regression analysis revealed that a high RapLSI (≥1.04) was significantly associated with 99m Tc-PYP positivity (odds ratio, 14.14; 95% confidence interval, 3.36-59.47; P < .01). The sensitivity, specificity, and accuracy of the diagnostic model using the RapLSI for identification of 99m Tc-PYP positivity were 53%, 94%, and 87%, respectively. CONCLUSIONS: A high RapLSI can raise the pretest probability of 99m Tc-PYP scintigraphy in patients with a low score according to the Kumamoto criteria. The RapLSI can assist clinicians in determining strategies for these patients.
Assuntos
Cardiomiopatias , Pré-Albumina , Cardiomiopatias/diagnóstico por imagem , Difosfatos , Humanos , Cintilografia , Compostos RadiofarmacêuticosRESUMO
BACKGROUND: After previous earthquakes, a high prevalence of deep vein thrombosis (DVT) has been reported. We examined DVT prevalence and risk factors in evacuees of the Kumamoto earthquakes by performing mobile DVT screening at various evacuation centers around the epicenter. MethodsâandâResults: For 1 month after the Kumamoto earthquake on 14 April 2016, mobile DVT screening using portable ultrasonography (US) was performed at 80 evacuation centers. Questionnaires, physical examination, and US of the lower limb were carried out, and simple D-dimer measurements were undertaken for DVT-positive examinees. The total number of examinees was 1,673, of whom 178 (10.6%) had DVT. The prevalence of DVT seemed to be gradually decreasing in the screening period, but age, use of sleep medication, prevalence of hypertension, dyslipidemia, leg edema, and lower leg varix were significantly higher in the DVT positive group than in the negative group. On multivariable logistic regression analysis, high age (≥70 years old), use of sleep medication, lower leg edema, and lower leg varix were significant predictors of DVT. In examinees with these 4 predictors, the DVT positive rate was 71.4%. CONCLUSIONS: In the first month after the Kumamoto earthquakes, DVT prevalence and severity, evaluated on D-dimer level, decreased with the passage of time. Mobile DVT screening indicated significant factors stratifying DVT risk in the evacuees.
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Terremotos , Trombose Venosa/etiologia , Adulto , Fatores Etários , Idoso , Edema , Feminino , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Humanos , Japão , Extremidade Inferior/patologia , Masculino , Pessoa de Meia-Idade , Prevalência , Fatores de Risco , Ultrassonografia , Varizes , Trombose Venosa/diagnóstico , Trombose Venosa/epidemiologiaRESUMO
A 16-year-old healthy boy visited our department because of a heart murmur. A 12-lead electrocardiogram showed left QRS axis deviation and repolarization abnormalities. Transthoracic echocardiography and a computed tomographic scan revealed a hypertrophied papillary muscle and a discrete ridge arising from the septal wall, causing mid-ventricular obstruction. Doppler echocardiography revealed that the pressure gradient at the obstruction was mild. The patient will be followed up annually, without medication or physical restriction.
Assuntos
Ecocardiografia , Eletrocardiografia , Músculos Papilares/anormalidades , Tomografia Computadorizada por Raios X , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Adolescente , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/fisiopatologia , Diagnóstico Diferencial , Sopros Cardíacos/etiologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Humanos , Masculino , Músculos Papilares/diagnóstico por imagem , Obstrução do Fluxo Ventricular Externo/complicações , Obstrução do Fluxo Ventricular Externo/patologiaRESUMO
We detected symptomatic atrial fibrillation in a 64-year-old man who had undergone mitral valvuloplasty. While performing transesophageal echocardiography (TEE) in the left lateral decubitus position, we detected an isoechoic mass lesion at the bottom of the left atrial appendage (LAA). After changing the patient's position from left to right, the mass lesion dropped down from the bottom of the LAA, spread out into the left atrium, and appeared as a spontaneous echocardiographic contrast with mobility. We therefore diagnosed the mass lesion as not a thrombus but sludge. Changing the patient's position during TEE is useful for distinguishing sludge from thrombi.
Assuntos
Apêndice Atrial/diagnóstico por imagem , Apêndice Atrial/fisiopatologia , Fibrilação Atrial/fisiopatologia , Ecocardiografia Transesofagiana/métodos , Posicionamento do Paciente/métodos , Trombose , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade , PosturaRESUMO
Wild-type transthyretin amyloidosis (ATTRwt) is often overlooked in elderly patients with left ventricular hypertrophy (LVH). Impaired atrial function, in addition to ventricular diastolic dysfunction, is one of the hallmarks of cardiac amyloidosis. Here, we assessed the hypothesis that atrial function evaluated by A-velocity in pulse Doppler echocardiography is useful to differentiate ATTRwt in elderly patients with LVH. We analyzed 133 consecutive patients who underwent tissue biopsy to rule out infiltrative cardiomyopathy in our institute. We excluded patients younger than 50 years, without LVH (LV thickness was less than 12 mm), with other types of cardiac amyloidosis and patients with chronic atrial fibrillation, and analyzed remaining 51 patients (ATTRwt: 16, non-ATTRwt: 35). ATTRwt patients were significantly older and had advanced heart failure compared with non-ATTRwt group. In echocardiography, E/A, E/e', and relative wall thickness was significantly higher in ATTRwt group than non-ATTRwt group. A-velocity was significantly decreased in ATTRWT group compared with non-ATTRwt group (40.8 ± 20.8 vs. 78.7 ± 28.2 cm/s, p = 0.0001). Multivariate logistic analysis using eight forced inclusion models identified trans-mitral Doppler A-wave velocity was more significant factor of cardiac amyloidosis in ATTRwt. In receiver operating characteristic (ROC) analysis, the area under the curve (AUC) for A-wave velocity in discrimination between ATTRwt and non-ATTRwt were 0.86 (CI 0.76-0.96, p < 0.001). The cut-off value was 62.5 cm/s, and it yielded the best combination of sensitivity (69.7%) and specificity (87.5%) for prediction of amyloidosis. We concluded that reduced A-velocity predicts the presence of ATTRwt in elderly patients with LVH in sinus rhythm.
Assuntos
Neuropatias Amiloides Familiares/complicações , Insuficiência Cardíaca/fisiopatologia , Ventrículos do Coração/fisiopatologia , Hipertrofia Ventricular Esquerda/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Neuropatias Amiloides Familiares/diagnóstico por imagem , Neuropatias Amiloides Familiares/patologia , Ecocardiografia Doppler , Feminino , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/etiologia , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/etiologia , Japão/epidemiologia , Estimativa de Kaplan-Meier , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Análise de Onda de Pulso , Curva ROC , Estudos RetrospectivosRESUMO
The 79th Annual Scientific Meeting of the Japanese Circulation Society was held in Osaka on April 24-26, 2015. The main theme was "Late-breaking Cardiovascular Medicine from Japan". Recently, optimal medical treatment has been guided by evidence-based medicine. We aim to emphasize the research findings and advances in cardiology from Japan, in the hope that Japan will become one of the leaders in the field worldwide. Unlike previous meetings, this annual scientific meeting was held in late April. Approximately 18,000 people, including medical doctors, healthcare professionals, and management staff, attended. The meeting was successfully completed, and included discussions on state-of-the art medicine.
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Cardiologia , Sociedades Médicas , Congressos como Assunto , Humanos , JapãoAssuntos
Insuficiência Cardíaca , Hipertensão Pulmonar , Pressão Arterial , Humanos , Volume SistólicoAssuntos
Cardiopatias Congênitas/fisiopatologia , Ventrículos do Coração/anormalidades , Hemodinâmica , Função Ventricular Direita , Adulto , Cateterismo Cardíaco , Ecocardiografia , Técnicas Eletrofisiológicas Cardíacas , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Imageamento por Ressonância MagnéticaRESUMO
An 81-year-old woman presented to our hospital with dyspnea. She had been treated with trastuzumab for nine years. Chest radiography revealed pleural effusion. Transthoracic echocardiography revealed dyskinesis of the interventricular septum (IVS). Cardiac magnetic resonance imaging revealed increased native T1 values at the interventricular septum and apex, indicating myocardial edema or fibrosis in these areas. A transthoracic echocardiogram after half a year revealed an increase in LVEF from 25% to 48%. Serial transthoracic echocardiography and cardiac magnetic resonance imaging were useful for evaluating the cardiac structure and function in the present case of delayed trastuzumab-induced myocardial injury.
RESUMO
Pregnancy is a major life event for most women that causes extensive physiological changes. Hence, it is associated with additional risks in women with congenital heart disease. No reports of pregnancy or a baby born to a woman with isolated right ventricular hypoplasia without an atrial septal defect have been published. In this case, the patient's right ventricle was very small with no contractility. The right atrium was highly enlarged, and its contractility resulted in pulmonary circulation without pulmonary hypertension. The size increased post-delivery than that before pregnancy. Fortunately, a healthy infant was born without any right heart failure symptoms.
RESUMO
RATIONALE AND OBJECTIVES: This study aimed to assess the utility of cardiac magnetic resonance imaging (MRI) T1 and T2 mapping as quantitative imaging biomarkers in transthyretin amyloid cardiomyopathy (ATTR-CM). MATERIALS AND METHODS: This study retrospectively evaluated 74 patients with confirmed wild-type ATTR-CM who underwent cardiac MRI, 99mTc-labeled pyrophosphate (99mTc-PYP) scintigraphy, and echocardiography. We assessed the quantitative disease parameters, for example, left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS) by echocardiography, native T1, extracellular volume fraction (ECV), and native T2 value by cardiac MRI, heart-to-contralateral ratio (H/CL) by 99mTc-PYP, and high-sensitive cardiac troponin T. Myocardial native T2 of ≥50 ms was defined as myocardial edema. Correlations between the disease's quantitative parameters were evaluated, and the ECV was compared to other parameters in ATTR-CM with/without myocardial edema. RESULTS: ECV in all patients with ATTR-CM revealed a strong correlation with native T1 (r = 0.62), a moderate correlation with hs-TnT (r = 0.59), LVEF (r = -0.48), GLS (r = 0.58), and H/CL (r = 0.48). Correlations between ECV and other quantitative parameters decreased in ATTR-CM with myocardial edema except for H/CL. Meanwhile, the correlations increased in ATTR-CM without myocardial edema. CONCLUSION: The presence of myocardial edema affected the interpretation of ECV assessment, although ECV can be a comprehensive imaging biomarker for ATTR-CM. ECV showed a significant correlation with various quantitative disease parameters and can be a reliable disease monitoring marker in patients with ATTR-CM when myocardial edema was excluded.
Assuntos
Amiloidose , Cardiomiopatias , Humanos , Pré-Albumina , Cardiomiopatias/diagnóstico por imagem , Pirofosfato de Tecnécio Tc 99m , Estudos Retrospectivos , Volume Sistólico , Função Ventricular Esquerda , Amiloidose/diagnóstico por imagem , Imageamento por Ressonância Magnética , Edema , BiomarcadoresRESUMO
Atrial amyloidosis is primarily caused by atrial natriuretic peptide (ANP) amyloid deposition. The main precursor protein causing cardiac amyloidosis is transthyretin (TTR), also known as TTR amyloid cardiomyopathy (ATTR-CM). A 73-year-old man, who presented with external dyspnea, was diagnosed with decompensated heart failure due to atrial fibrillation and severe mitral regurgitation. Left ventricular hypertrophy and elevated levels of high-sensitivity cardiac troponin T indicated cardiac amyloidosis. 99mtechnetium pyrophosphate scintigraphy findings and cardiac magnetic resonance imaging in the absence of monoclonal proteins were consistent with those of ATTR-CM. The patient underwent mitral valve repair, a maze procedure, and left atrial appendage (LAA) excision. While the histological analysis of the sampled left ventricular tissue led to diagnosis of ATTR-CM, the histological analysis revealed the coexistence of ANP and TTR amyloid deposition in the resected LAA. We report a case of ATTR-CM in which TTR and ANP amyloid deposition coexisted in the surgically resected LAA, indicating that both TTR and ANP amyloid correlate with atrial amyloidosis development in ATTR-CM. Learning objectives: Atrial natriuretic peptide (ANP) and transthyretin (TTR) amyloids can coexist in the same atrium. Not only TTR amyloids but also ANP amyloids can be correlated with the development of atrial amyloidosis in TTR amyloid cardiomyopathy with subsequent increased risk of atrial fibrillation.