Prediction of future insulin-deficiency in glutamic acid decarboxylase autoantibody enzyme-linked immunosorbent assay-positive patients with slowly-progressive type 1 diabetes.

AIMS/INTRODUCTION: This study aimed to identify risk factors that contribute to the progression of slowly-progressive type 1 diabetes by evaluating the positive predictive value (PPV) of factors associated with the progression to an insulin-dependent state. MATERIALS AND METHODS: We selected 60 slowly-progressive type 1 diabetes patients who tested positive for glutamic acid decarboxylase autoantibodies (GADA) at diagnosis from the Japanese Type 1 Diabetes Database Study. GADA levels in these patients were concurrently measured using both radioimmunoassay (RIA) and enzyme-linked immunosorbent assay (ELISA) techniques. RESULTS: Compared with the non-progressor group (fasting C-peptide [F-CPR] levels maintained ≥0.6 ng/mL), the progressor group showed a younger age at diagnosis, lower body mass index (BMI), lower F-CPR levels and a higher prevalence of insulinoma-associated antigen-2 autoantibodies (IA-2A). The PPV of RIA-GADA increased from 56.3 to 70.0% in the high titer group (≥10 U/mL), and further increased to 76.9, 84.2, 81.0 and 75.0% when combined with specific thresholds for age at diagnosis <47 years, BMI <22.6 kg/m2 , F-CPR <1.41 ng/mL and IA-2A positivity, respectively. In contrast, the PPV of ELISA-GADA (71.8%) remained the same at 73.1% in the high titer group (≥180 U/mL), but increased to 81.8, 82.4 and 79.0% when evaluated in conjunction with age at diagnosis, BMI and F-CPR level, respectively. CONCLUSIONS: Our findings show that, unlike RIA-GADA, ELISA-GADA shows no association between GADA titers and the risk of progression to an insulin-dependent state. The PPV improves when age at diagnosis, BMI and F-CPR levels are considered in combination.

A case of type 2 diabetes mellitus with weight gain and worsening of glycemic management after tezepelumab administration for severe bronchial asthma.

Some cases of bronchial asthma are refractory to conventional therapies. As the pathogenesis of bronchial asthma has been clarified, new treatments, such as bronchial thermoplasty and biological drugs, have been developed. Tezepelumab, an anti-thymic stromal lymphopoietin antibody, has been reported to inhibit the exacerbation of severe asthma; however, its adverse effects on glucose metabolism have not yet been reported. We encountered a case of weight gain and worsening glycemic management in a patient with type 2 diabetes and refractory bronchial asthma after the initiation of tezepelumab treatment. It has been reported that the overexpression of thymic stromal lymphopoietin in mice resulted in an enhanced release of free fatty acids from adipose tissues and the liver; thus, the administration of anti-thymic stromal lymphopoietin antibodies in the present case might have caused obesity, fatty liver and lower glucose tolerance.

Impact of coronavirus disease 2019 in seasonal variation of hemoglobin A1c in adults with type 1 diabetes and the effect of the mode of treatment: a single-center retrospective study for 2019 and 2021 and analysis by the mode of treatment.

The study was aimed to investigate the seasonal variation of hemoglobin A1c (HbA1c) in adults with type 1 diabetes (T1D) and the impact of coronavirus disease 2019 (COVID-19) by comparing 2019 and 2021 data and differences in treatment modes. This was a single-center retrospective observational study including 52 adult patients with T1D who regularly visited hospital in 2019 and 2021. Twenty-five patients used multiple daily injections (MDI)/self-measurement of blood glucose (SMBG), 16 used MDI/intermittently scanned continuous glucose monitoring (isCGM), 9 used sensor-augmented pump (SAP), and 2 used continuous subcutaneous insulin infusion (CSII)/isCGM. The mean HbA1c level was calculated for each month. The correlation between monthly means of temperature and HbA1c was investigated. Similar analyses were performed for the MDI/SMBG, MDI/isCGM, and SAP + CSII/isCGM groups. HbA1c levels in 2019 decreased in summer and increased in winter and showed a significant negative correlation with temperature (r = -0.652, p = 0.022). However, HbA1c in 2021 showed no seasonal variation and no correlation with temperature (r = -0.134, p = 0.678) and tended to decline after the three emergency declarations. HbA1c in the MDI/SMBG group showed the same trend as the whole group in 2019 and 2021. However, the effect of seasonal variation in HbA1c was lower in the MDI/isCGM group and the lowest in the SAP + CSII/isCGM group in 2019. The impact of emergency declaration on HbA1c level was small for the MDI/isCGM group and smaller for the SAP + CSII/isCGM group in 2021. The COVID-19 pandemic has affected the seasonal variation of HbA1c levels in T1D; the variation differed according to the treatment mode.

Graft failure after allogeneic islet transplantation in a patient with type 1 diabetes and a high anti-glutamic acid decarboxylase antibody titer.

Pancreatic islet transplantation is a ß-cell replacement therapy for people with insulin-deficient diabetes who have difficulty in glycemic control and suffer from frequent severe hypoglycemia. However, the number of islet transplantations carried out is still limited in Asia. We report a case of allogeneic islet transplantation in a 45-year-old Japanese man with type 1 diabetes. Although the islet transplantation was successfully carried out, graft loss was observed on the 18th day. Immunosuppressants were used in accordance with the protocol, and donor-specific anti-human leukocyte antigen antibodies were not detected. Autoimmunity relapse was also not observed. However, the patient had a high titer of anti-glutamic acid decarboxylase antibody from before the islet transplantation, and autoimmunity might thus have affected the ß-cells in the transplanted islet. The evidence is still scarce to reach conclusions, and further data accumulation is required to enable proper patient selection before islet transplantation.

New-onset type 1 diabetes and Graves' disease after antiretroviral therapy in a patient with human immunodeficiency virus infection.

Patients with human immunodeficiency virus (HIV) infection receiving antiretroviral therapy can develop autoimmune diseases, referred to as immune-inflammatory reconstitution syndrome. Nevertheless, only a few reports on the onset of type 1 diabetes as immune-inflammatory reconstitution syndrome are available. A 40-year-old Japanese man with HIV infection was initiated with antiretroviral therapy at the age of 29 years. He developed Graves' disease at 35 years and diabetes, with a hemoglobin A1c of 6.5%, and maintained insulin secretion at 38 years. His antiglutamic acid decarboxylase antibody level was >2,000 U/mL, and he was diagnosed with slowly progressive type 1 diabetes. At the age of 40 years, he was admitted to our hospital with diabetic ketosis. We retrospectively assayed his stored plasma samples for thyroid-stimulating hormone receptor antibody and antiglutamic acid decarboxylase antibody, which showed positive conversion after initiating antiretroviral therapy, suggesting that Graves' disease and type 1 diabetes developed as a probable result of immune-inflammatory reconstitution syndrome.

Clinical significance of tumor markers in patients with type 2 diabetes: a retrospective observational study.

Aim: To cross-sectionally and longitudinally investigate the association between tumor markers (Cancer embryonic antigen (CEA) Carbohydrate antigen 19-9 (CA19-9)) and malignancies in type 2 diabetes patients without evidence of malignancy. Materials and Methods: The study included 707 patients admitted for the treatment of diabetes from 1 August 2010 to 1 September 2018. Serum CEA and CA19-9 levels were measured for screening of malignancies at admission. Abdominal ultrasonography, computed tomography, and endoscopy were performed for close examination. The percentage of patients diagnosed with malignancy was calculated, and among those without malignancy, the incidence of malignancies was examined after discharge. Results: A total of 26 patients (3.7%) were newly diagnosed with malignancy during hospitalization. The optimal cut-off value of CEA and CA19-9 by receiver operating characteristic analysis was 5.0 ng/mL and 75 U/mL, and their positive predictive values (PPV) were 8.7% and 22.5%, respectively. The addition of CA19-9 to age, smoking status, body mass index, and glycated hemoglobin significantly improved classification performance for malignancy using net reclassification improvement (0.682, 95% CI 0.256-1.107) and integrated discrimination improvement (0.150, 95% CI 0.007-0.294). Among 681 patients without malignancies during hospitalization, 30 patients (4.4%) developed malignancies during an average follow-up of 3.9 years. CA19-9 (hazard ratio: 1.005, 95% CI: 1.003-1.008) was associated with the development of malignancies. Conclusions: PPV of serum CEA and CA19-9 for detecting malignancy was high in type 2 diabetes patients with poor glycemic control. Measuring CA19-9 was found to be valuable to cross-sectionally and longitudinally detect malignancies. Supplementary Information: The online version contains supplementary material available at 10.1007/s13340-022-00594-x.

The patient-centered diabetes management during the COVID-19 pandemic.

Since December 2019, in the fight against the coronavirus disease 2019 (COVID-19) pandemic, we observed that glycemic control in people with diabetes is easily affected by lifestyle changes. To maintain a good health condition, a patient-centered approach with mental support and close monitoring is required. For these, telemedicine and online continuous glucose monitoring (CGM), are effective systems. Therefore, based on our experience during the two-year period, we reviewed the literature for appropriate actions required for the management of diabetes to prevent COVID-19 infection and avoid unfavorable outcomes in COVID-19 cases. Once infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), there is a high risk of a poor prognosis in patients with diabetes. Glucocorticoid therapy in severe COVID-19 cases leads to further hyperglycemia. Since good glycemic control has been shown to improve outcomes, strict glycemic control using CGM is recommended. Using CGM data, insulin can be adequately titrated without causing hypoglycemia, and remote data monitoring can reduce the risk of infection for health care professionals, by reducing the frequency of patient contact. Among patients with COVID-19, some are found to have newly-diagnosed diabetes at admission. Those newly diagnosed patients present with a higher risk of poor prognosis compared to those with pre-existing diabetes. Therefore, glycemic status should be evaluated in all patients with COVID-19 admitted to hospitals.

Efficacy of non-obstructive aortic angioscopy for detecting a thoracic aortic graft rupture: a case report.

BACKGROUND: Non-anastomotic thoracic aortic graft rupture is extremely rare and difficult to diagnose. Non-obstructive general angioscopy can help monitor the aortic intima and detect the locations of abnormal findings, while aortic angioscopy can detect vulnerable plaques in the aorta, which are difficult to visualize using conventional diagnostic methods. Herein, we report the case of a patient with non-anastomotic thoracic aortic graft rupture diagnosed using non-obstructive aortic angioscopy. CASE PRESENTATION: An 85-year-old man who had undergone total arch replacement 5 years prior complained of chest pain. Emergent contrast-enhanced computed tomography (CT) revealed an intra-mediastinal hematoma around the vascular graft of the ascending aorta and angiography revealed pooling of contrast medium on the dorsal side of the vascular graft. We suspected extravasation of the thoracic vascular graft. Aortic angioscopic examination revealed a red vascular graft defect that matched extravasation at the contralateral level of the prosthetic left common carotid artery branch. Subsequently, non-anastomotic thoracic aortic graft rupture was diagnosed. The patient underwent a two-debranching thoracic endovascular aortic repair (Zone 0) with a right subclavian artery-left common carotid artery-left subclavian artery bypass. Postoperative angiography revealed disappearance of the extravasation from the graft rupture site, patent grafted vessels with flow, and no endoleak. Follow-up CT at 6 months postoperatively showed no extravasation. CONCLUSIONS: To our knowledge, this is the first report of non-anastomotic thoracic aortic graft rupture detected using non-obstructive aortic angioscopy. Aortic angioscopy can help establish a definitive diagnosis in patients with aortic graft rupture.

Living and working environments are important determinants of glycemic control in patients with diabetes during the COVID-19 pandemic: A retrospective observational study.

AIM: To investigate (1) the association of lifestyle changes and living and working conditions with glycemic control and (2) whether treatment was intensified appropriately in patients with diabetes under the first COVID-19 state of emergency in Japan. MATERIALS AND METHODS: A total of 321 participants were included. Participants completed a questionnaire regarding lifestyle changes, including diet, physical activity, and living and working conditions during the COVID-19 pandemic. The change in hemoglobin A1c (HbA1c) levels was estimated before (June 1, 2019 to August 31, 2019) and during (June 1, 2020 to August 31, 2020) the pandemic. Factors associated with changes in HbA1c levels were examined by multiple linear regression analysis. The proportion of patients who received treatment intensification for diabetes was compared between before and during the pandemic. RESULTS: There was no significant change in HbA1c levels before the pandemic and during the pandemic (7.13 ± 0.98% vs 7.18 ± 1.01%, P = 0.186). Teleworking (estimate 0.206, P = 0.004) and living with a dog (estimate -0.149, P = 0.038) were significantly associated with changes in HbA1c levels after adjusting for covariates. There was no significant difference in the proportion of patients who received treatment intensification for diabetes during the pandemic and before the pandemic in either the elderly or non-elderly patients. CONCLUSIONS: Overall glycemic control did not worsen during the pandemic. Nonetheless, environmental factors, including telework, were found to influence glycemic control in patients with diabetes. Further studies are needed to clarify whether the COVID-19 pandemic could affect treatment intensification for diabetes.

Impact of newly diagnosed diabetes on coronavirus disease 2019 severity and hyperglycemia.

AIMS/INTRODUCTION: Diabetes is associated with poor clinical outcomes of coronavirus disease 2019 (COVID-19). However, the impact of newly diagnosed diabetes on prognosis has not been clarified. The objective of this study was to show the features and outcome of COVID-19 patients with newly diagnosed diabetes in Japan. MATERIALS AND METHODS: We retrospectively analyzed 62 patients with diabetes hospitalized for COVID-19 between 1 April and 18 August 2021 at the National Center for Global Health and Medicine in Tokyo, Japan. We evaluated the worst severity of COVID-19 and plasma blood glucose levels in patients with newly diagnosed diabetes or pre-existing diabetes. RESULTS: This study included 62 confirmed COVID-19 patients with diabetes, including 19 (30.6%) patients with newly diagnosed diabetes and 43 (69.4%) patients with pre-existing diabetes. Patients with newly diagnosed diabetes significantly progressed to a critical condition more frequently during hospitalization than patients with pre-existing diabetes (52.6% vs 20.9%, P = 0.018). In addition, patients with newly diagnosed diabetes had significantly higher average plasma blood glucose levels for the first 3 days after admission than those with pre-existing diabetes. CONCLUSIONS: Our study suggests that the proportion of COVID-19 patients who are newly diagnosed with diabetes is high, and they have an increased risk of developing severe disease than those with pre-existing diabetes. It might be advisable that at the point of COVID-19 diagnosis, blood glucose and glycated hemoglobin levels be assessed in all patients.

Glycemic control using intermittently scanned continuous glucose monitoring in patients with diabetes requiring methylprednisolone therapy for severe COVID-19.

In patients with severe coronavirus disease 2019 (COVID-19) with diabetes, glycemic control is essential for a better outcome, however, we face difficulty controlling hyperglycemia induced by high-dose glucocorticoids. We report five cases of severe COVID-19 patients with diabetes, whose glycemic control was managed using an intermittently scanned continuous glucose monitoring (isCGM) system during methylprednisolone therapy. Patients using isCGM showed significantly lower average blood glucose levels and significantly higher total daily insulin dose during the methylprednisolone therapy, compared to patients under regular blood glucose monitoring. The use of isCGM enables remote glucose monitoring, and this can reduce the risks of healthcare workers who have frequent contact with the patients. Thus, we suggest that using isCGM should be considered in hospitalized patients with diabetes under the COVID-19 pandemic to achieve better glycemic control and to minimize the possible risks of healthcare workers.

The work style and living condition survey of diabetologists and the expectations for the Japan Diabetes Society: results of questionnaires about the current state and the future prospect of their carrier in 2017.

The Japan Diabetes Society's Committee to Promote Female Diabetologists conducted a questionnaire survey from May to June 2017 to investigate the work style and living situation of diabetologists. The survey targeted 5298 Board Certified Diabetologists (diabetologists), with answers obtained from 1566 diabetologists (male, n = 1003: females, n = 563). Ninety-four percent of the males and 72% of the females worked full time. Twenty-one percent of the male subjects and 7% of the female subjects were heads of clinical departments, and 23% of the male subjects and 13% of the female subjects were diabetes training instructors, showing that there were fewer women than men in both roles. Regarding the allocation of time per day, men spent 10.7 h working, while women spent 8.5 h working. Both men and women slept 6.3 h. Men spent 1.0 h on housework, while women spent 3.3 h on housework. Men spent 0.7 h on childcare and nursing care, while women, spent 2.8 h. Among diabetologists in the childrearing generation, men spent 1.4 h providing childcare and nursing care, while women spent 4.9 h, showing that women spent significantly more time on these tasks than men. To encourage female diabetologists to work more actively, to reduce overworking on the part of male diabetologists, and to enhance the careers of both men and women as diabetologists, we conclude it necessary to improve the workplace environment and for the Japan Diabetes Society to offer support.

Tissue-Specific Metabolic Regulation of FOXO-Binding Protein: FOXO Does Not Act Alone.

The transcription factor forkhead box (FOXO) controls important biological responses, including proliferation, apoptosis, differentiation, metabolism, and oxidative stress resistance. The transcriptional activity of FOXO is tightly regulated in a variety of cellular processes. FOXO can convert the external stimuli of insulin, growth factors, nutrients, cytokines, and oxidative stress into cell-specific biological responses by regulating the transcriptional activity of target genes. However, how a single transcription factor regulates a large set of target genes in various tissues in response to a variety of external stimuli remains to be clarified. Evidence indicates that FOXO-binding proteins synergistically function to achieve tightly controlled processes. Here, we review the elaborate mechanism of FOXO-binding proteins, focusing on adipogenesis, glucose homeostasis, and other metabolic regulations in order to deepen our understanding and to identify a novel therapeutic target for the prevention and treatment of metabolic disorders.

FCoR-Foxo1 Axis Regulates α-Cell Mass through Repression of Arx Expression.

Pancreatic endocrine cell development into differentiated α- and ß-cells is highly regulated and involves multiple transcription factors. However, the mechanisms behind the determination of α- and ß-cell masses remains unclear. We previously identified Foxo1 CoRepressor (FCoR), which inhibits Foxo1 by acetylation. Here we demonstrate that Fcor-knockout mice (FcorKO) exhibit significantly increased α-cell mass, expression of the master α-cell regulatory transcription factor Aristaless-related homeobox (Arx), which can be normalized by ß-cell-specific FCoR overexpression (FcorKO-ßFcor), and exhibit ß-to-α-cell conversion. Compared with FcorKO, ß-cell-specific Foxo1 knockout in the FcorKO (DKO) led to decreased Arx expression and α-cell mass. Foxo1 binding to Arx promoter led to DNA methyltransferase 3a (Dnmt3a) dissociation, Arx promoter hypomethylation, and increased Arx expression. In contrast, FCoR suppressed Arx through Foxo1 inhibition and Dnmt3a recruitment to Arx promoter and increased Arx promoter methylation. Our findings suggest that the FCoR-Foxo1 axis regulates pancreatic α-cell mass by suppressing Arx expression.

Transarterial Embolization With Complementary Surgical Ligation of Gastroduodenal Artery for Ruptured Pancreaticoduodenal Artery Aneurysm.

The association between pancreaticoduodenal artery aneurysm (PDAA) and local hemodynamic changes in pancreaticoduodenal arcades is well established. However, there are few case reports of PDAA associated with acute aortic dissection. In this article, we outline and discuss the case of a 61-year-old man diagnosed with a type A acute aortic dissection who underwent emergency surgery and developed sudden-onset severe abdominal pain and shock 10 days later. Contrast-enhanced computed tomography showed a ruptured PDAA with feeding vessels from the gastroduodenal and superior mesenteric arteries, with evidence that the celiac artery was diverged from a false lumen. Transarterial embolization via the superior mesenteric artery alone was not expected to achieve hemostasis, so we performed a hybrid procedure involving transarterial embolization cannulated from superior mesenteric artery with complementary surgical ligation of the gastroduodenal artery. The postoperative course was uneventful, and follow-up contrast-enhanced computed tomography showed no persistence of the aneurysm 8 days after the second operation. This case proposed that visceral arterial malperfusion due to acute aortic dissection can cause PDAA in the early postoperative period. Although previous reports suggest that endovascular treatment is preferable, it may not always be feasible. Since ruptured PDAAs are often not detected during surgery, surgical treatment can be overly invasive. Whereas, transarterial embolization with complementary clamping or ligation of the gastroduodenal artery for ruptured PDAA is less invasive and can control hemorrhage, especially when cannulation to the celiac artery is impossible. Notably, the technique did not cause organ ischemia, presumably because the small collateral vessels of the pancreaticoduodenal arcades permitted sufficient blood flow. If endovascular treatment is unable to achieve rapid hemostasis, this technique may be a useful option for ruptured PDAA.

[Successful Use of Percutaneous Left Ventricular Assist Device( Impella) and Veno Artery Extracorporeal Membrane Oxygenation( VA-ECMO) for Ischemic Cardiogenic Shock;Report of a Case].

A 73-year-old male was referred to our hospital for acute congestive heart failure. His cardiac and respiratory conditions were worsening with cardiogenic shock requiring intubation. Coronary angiography revealed severe triple vessel disease, and echocardiography showed severe left ventricular dysfunction. Therefore, he underwent veno artery extracorporeal membrane oxygenation (VA-ECMO) followed by percutaneous left ventricular assist device (Impella). His cardiac condition improved and VA-ECMO and Impella were removed on the 2nd day and the 4th day after surgery, respectively. He underwent off-pump coronary artery bypass grafting (OPCAB) without any complication on the 36th day. Postoperative course was uneventful and he was discharged on postoperative day 30. Concomitant use of Impella and VA-ECMO (Ecpella) remarkably improved ischemic cardiogenic shock by unloading the left ventricle and increasing the cardiac output.

Predictors of patent false lumen of the aortic arch after hemiarch replacement.

BACKGROUND: Hemiarch replacement for acute type A aortic dissection is less invasive than total arch replacement but involves increased risk of late aortic arch dilation because of patent false lumen of the aortic arch. If we can predict this risk, it may be a valuable prognostic indicator for selecting surgical procedures for acute type A aortic dissection. METHODS: We reviewed our surgical experience to predict patent false lumen. From January 2009 to November 2014, we performed 108 hemiarch replacement procedures for acute type A aortic dissection that had patent false lumen of the ascending aortic arch. We identified 56 patients who had preoperative and postoperative contrast-enhanced computed tomography. Patients' preoperative characteristics, preoperative and postoperative contrast-enhanced computed tomography findings, intraoperative findings and postoperative course were investigated. RESULTS: Of the 56 patients, 32 (57.1 %) were men and their mean age at surgery was 63.7 ± 11.8 years. Overall in-hospital mortality rate was 7.1 % (4 patients). According to postoperative imaging findings, 56 patients were classified into two groups: group A (39 patients), with patent false lumen, and group B (17 patients), with thrombosed false lumen. Logistic regression analysis revealed that brachiocephalic artery dissection and no tear resection contributed to postoperative patent false lumen of the aortic arch more strongly than did other factors. CONCLUSIONS: Brachiocephalic artery dissection and no tear resection are potential predictors of patent false lumen of the aortic arch after hemiarch replacement.

Emergency Pulmonary Embolectomy Using Minimally Invasive Cardiac Surgery.

A 78-year-old man who had undergone operation for acute type A aortic dissection presented with dyspnea and shock. Chest computed tomography revealed pulmonary embolism. Minimally invasive cardiac surgery was performed through a right fourth intercostal skin incision using cardiopulmonary bypass through the right femoral artery and vein. The right pulmonary artery below the superior vena cava was incised vertically, and the thrombus was extracted directly by balloon catheter. The patient was weaned off cardiopulmonary bypass uneventfully. The postoperative course was also uneventful. In redo cardiac surgery, pulmonary embolectomy through minimally invasive right thoracotomy can be easily performed, with quick recovery.

Oblique venotomy with parallelogram anastomosis in sequential bypass grafting.

The proximal vein graft is incised obliquely and anastomosed to the coronary artery to ensure that the proximal rim of the vein incision is adjusted to the lateral side of the coronary artery incision adjacent to the proximal rim. So the vein graft can be placed perpendicular to the coronary artery without kinking, and the anastomosis orifice can be made wider than those made with diamond anastomosis. Oblique venotomy with parallelogram anastomosis provides sufficient anastomotic flow, allows long incisions, and prevents kinking.

Mitral and tricuspid valve surgery for Coffin-Lowry syndrome.

Coffin-Lowry syndrome is a rare X-linked disorder characterized by craniofacial and skeletal abnormalities, mental retardation, short stature, and hypotonia. An 18-year-old man with morphologic features characteristic of Coffin-Lowry syndrome was referred to our institution for valve disease surgery for worsening cardiac failure. Echocardiography showed severe mitral valve regurgitation associated with tricuspid valve regurgitation. Mitral valve implantation with a biological valve and tricuspid annular plication with a ring was performed. The ascending aorta was hypoplastic. Both the mitral papillary muscle originating near the mitral annulus and the chordae were shortened. The patient's postoperative course was uneventful and his cardiac failure improved.