Wild-Type Transthyretin Amyloidosis in Female Patients　- Consideration of Sex Differences.

Background: With recent advances in non-invasive diagnostic tools, some studies indicate that wild-type transthyretin amyloidosis (ATTRwt) may be more common in females than previously reported. However, the clinical characteristics of female ATTRwt patients have not been determined. Methods and Results: Of the 78 consecutive patients with ATTRwt in our cohort, 14 (17.9 %) were female. Compared with male patients, female ATTRwt patients had smaller left ventricular (LV) wall thicknesses (ventricular septum thickness 12.9 vs. 14.2 mm [P=0.081]; posterior wall thickness 12.7 vs. 13.6 mm [P=0.035]) and a higher LV ejection fraction (EF; mean [±SD] 58.4±8.9% vs. 48.9±11.8%; P=0.006). However, the severity of heart failure (HF), as assessed by HF stage, New York Heart Association functional class and B-type natriuretic peptide concentrations, did not differ between female and male patients. Moreover, LV mass index and relative wall thickness were increased and the stroke volume index was reduced in both female and male patients. In organ biopsies, female patients had a higher sensitivity to transthyretin deposition from abdominal fat than male patients (positive abdominal fat biopsy 80.0 % vs. 26.5%; P=0.016). Conclusions: This study suggests that a relatively large proportion of elderly females have ATTRwt. Female ATTRwt patients had HF symptoms even at the stage of mild LV hypertrophy and preserved EF. Abdominal fat biopsy may be useful to diagnose ATTRwt, especially in female patients with HF.

Validation of the Kumamoto criteria for prediction of 99m technetium pyrophosphate scintigraphy positivity as a strategy for diagnosis of transthyretin cardiac amyloidosis: A retrospective cohort study in Kochi.

BACKGROUND: Early diagnosis of transthyretin cardiac amyloidosis (ATTR-CA) is important. The aim of this study was to validate the 'Kumamoto criteria' for prediction of technetium-99m pyrophosphate (99mTc-PYP) scintigraphy positivity. METHODS: One hundred fifty patients (median age: 79.4 years, 117 males) with the possibility of ATTR-CA who underwent 99mTc-PYP scintigraphy were assessed. We divided the patients into 4 groups (groups with score of 0-3) according to the Kumamoto criteria by total points for the following 3 factors: high-sensitivity cardiac troponin T (hs-cTnT) ≥0.0308 ng/ml, left ventricle posterior wall thickness ≥13.6 mm, and wide QRS (QRS ≥ 120 ms). RESULTS: Seventy patients (46.7%) were positive for 99mTc-PYP scintigraphy. 99mTc-PYP positivity rates in the groups with score of 0, 1, 2, and 3 were 4%, 39%, 69%, and 89%, respectively. Compared with the original Kumamoto cohort, our patients in the score 1 group showed a relatively high rate of 99mTc-PYP positivity because hs-cTnT as one of the positive factors had high ability to discriminate the disease. The sensitivity and negative predictive value of hs-cTnT ≥0.0308 ng/ml for 99mTc-PYP positivity were 97.1% and 93.9%. CONCLUSIONS: In the Kochi validation cohort, the Kumamoto criteria were useful for predicting 99mTc-PYP positivity. However, patients in the score 1 group should be assessed cautiously for the possibility of ATTR-CA if the hs-cTnT value is high.

Preoperative Clinical Features and High Pulmonary Wedge Pressure with a Discordant Pattern as Prognostic Factor in Hemodialysis Patients with Severe Aortic Valve Stenosis.

Hemodialysis (HD) is one of the important risks for the development of cardiovascular disease, including aortic valve stenosis (AS). Although aortic valve replacement (AVR) is a beneficial treatment for AS, HD patients are known to show a high rate of mortality after AVR than non-HD patients.We retrospectively studied 109 patients who underwent AVR for severe AS, 18 of which were HD patients. Survival rate after AVR, preoperative clinical data, and surgical procedure were investigated.In preoperative clinical features, left ventricular end-diastolic diameter was larger, intraventricular septum thickness (IVST) was thicker, left ventricular mass index (LVMI) was higher, left ventricular ejection fraction was lower, E/e' was higher, and pulmonary arterial wedge pressure (PAWP) was higher in the HD group than in the non-HD group. During a follow-up period of 3.2 ± 2.3 years after AVR, patients receiving HD had a worse prognosis than those without HD treatment: the 3-year survival rate after surgery in the HD group was 36.2% and that in the non-HD group was 84.9%. With regard to prognostic factors in the whole cohort, significant differences were found in IVST, LVMI, E/e', PAWP, and HD. In patients receiving HD, abnormally high PAWP for their right atrial pressure (RAP) was observed, suggesting that PAWP and RAP were discordant, and univariate analysis revealed that high PAWP was the only predictor of mortality in HD patients after surgery.Preoperative PAWP with a discordant pattern in HD patients might be an important prognostic predictor after AVR.

Prediction of Medium-Term Mortality in Japanese Patients With Wild-Type Transthyretin Amyloidosis.

Background: The prognostic factors in Japanese patients with wild-type transthyretin amyloidosis (ATTRwt) have not been elucidated. Methods and Results: In this study we retrospectively analyzed the clinical characteristics and outcomes of 47 patients with ATTRwt (mean (±SD) age at diagnosis 80.3±4.6 years; 41 males). Fifteen patients died within 2 years of their diagnosis. Receiver operating characteristic and Kaplan-Meier analyses revealed that the best predictors of 2-year mortality were low serum albumin (≤3.75 g/dL), elevated high-sensitivity cardiac troponin T (hs-cTnT; >0.086 ng/mL), and reduced left ventricular ejection fraction (LVEF; <50%). According to the total number of these 3 risk factors, patients were stratified into 4 subgroups: low risk (no risk factors; n=15), intermediate-low risk (1 risk factor; n=15), intermediate-high risk (2 risk factors; n=7), and high risk (3 risk factors; n=10). The estimated 2-year survival rate of patients classified as low risk, intermediate-low risk, intermediate-high risk, and high risk was 93%, 80%, 83%, and 11%, respectively (P<0.001). Conclusions: Low serum albumin, elevated hs-cTnT, and reduced LVEF are associated with a worse prognosis in Japanese patients with ATTRwt. The combination of these factors may be useful for predicting medium-term mortality in patients with ATTRwt.

Relationship between the fall in blood pressure in the standing position and diaphragmatic muscle thickness: proof of concept study.

BACKGROUND: The diaphragm is an important muscle of respiration, and regulates the intrathoracic pressure. Blood pressure is regulated by the baroreceptor reflex system, and is also affected by intrathoracic pressure. We examined the relationship between the diaphragmatic muscle thickness and the degree of drop in blood pressure in the standing position. METHODS: We prospectively studied 15 healthy subjects. The diaphragmatic muscle thickness was measured using a B-mode ultrasonic imaging device. The blood pressure before and after standing was measured by a head-up tilt test. RESULTS: The diastolic blood pressure difference during expiration and inspiration showed a significant correlation with the diaphragmatic muscle thickness (r = 0.578, P = 0.024 and r = 0.518, P = 0.048, respectively). CONCLUSION: The diaphragmatic muscle thickness was related to the fall in diastolic blood pressure in the standing position. This indicates that adequate diaphragmatic muscle thickness helps to maintain intrathoracic pressure and prevents excessive drop in blood pressure in the standing position.

Complex venous malformation in an elderly female: A case report.

A 76-year-old female taking oral medications for chronic atrial fibrillation and hypertension was admitted to our hospital for examination of bilateral leg edema. Transthoracic echocardiography showed an enlarged coronary sinus of about 40 mm. Contrast-enhanced computed tomography revealed absence of the left innominate vein, a persistent left superior vena cava, and absence of the hepatic segment of the inferior vena cava. The interrupted inferior vena cava connected with the hemiazygos vein. The left superior vena cava also continued to the hemiazygos vein with reflux to the enlarged coronary sinus. A diagnosis of heterotaxy syndrome associated with polysplenia was made. No other congenital cardiac abnormalities were found. The patient was also diagnosed as having right cardiac failure due to atrioventricular valve regurgitation associated with chronic atrial fibrillation. The bilateral leg edema improved rapidly with oral medications. Although complex venous malformations such as those described above are rarely observed in the elderly, absence of the hepatic segment of the inferior vena cava, as in this case, is a particularly pathognomonic malformation in left isomerism in heterotaxy syndrome.

Impact of infant health problems on postnatal depression: pilot study to evaluate a health visiting system.

This pilot study aimed to elucidate whether or not mothers who receive neonatal home visits tend to suffer from depressive disorders during the first postpartum year and to examine what kinds of factors were related to the postnatal depression observed in these mothers. The subjects consisted of 70 Japanese mothers who received neonatal home visits and completed the 1-year study. At their first visit, the health visitors asked about their personal backgrounds and early postpartum mood, and depression was assessed by a Japanese translation of the Edinburgh Postnatal Depression Scale (EPDS). At 12 months postpartum, a diagnostic interview using the Structured Clinical Interview for DSM-IV (SCID) was performed to confirm the maternal diagnosis by a psychiatrist. An assessment of infant development using the Denver Developmental Screening Test (DDST) was administered to each infant by a psychologist. Nineteen mothers (27%) were categorized as having had a new onset of depression (12 major depressive disorders and seven depressive disorders not otherwise specified). In comparison to the non-depressed mothers, infant-related health problems that required either outpatient treatment or hospitalization were significantly related to postnatal depression. Pediatric diseases in infants did not always precede their mother's depression. The extra burden of caring for a sick infant tends to increase the risk of developing postpartum depression. As a result, neonatal home visits by health visitors may therefore be a potentially crucial opportunity to provide emotional support, particularly for mothers with the extra burden of having to care for sick infants.

Accumulation and degradation of thiamin-binding protein and level of thiamin in wheat seeds during seed maturation and germination.

Changes in the levels of thiamin-binding globulin and thiamin in wheat seeds during maturation and germination were studied. The thiamin-binding activity of the seed proteins increased with seed development after flowering. The thiamin content of the seeds also increased with development. Thiamin-binding activity decreased during seed germination. On the other hand, immunological analysis using an antibody directed against the thiamin-binding protein isolated from wheat seeds showed that the thiamin-binding globulin accumulated in the aleurone layer of the seeds during maturation, and then the protein was degraded and disappeared during seed germination. These results suggested that the thiamin-binding globulin of wheat seeds was synthesized and accumulated in the aleurone layer of the seeds with seed development, similar to the thiamin-binding albumin in sesame seeds, and that thiamin bound to the thiamin-binding globulin in the dormant wheat seeds for germ growth during germination.