We describe a case of a rare congenital heart disorder,scimitar syndrome,diagnosed in an adult woman presenting with dyspnea on exertion,chest pain and recurrent episodes of pulmonary infections.The hallmark of the sy...We describe a case of a rare congenital heart disorder,scimitar syndrome,diagnosed in an adult woman presenting with dyspnea on exertion,chest pain and recurrent episodes of pulmonary infections.The hallmark of the syndrome is the presence of an enlarged anomalous pulmonary vein draining into the inferior vena cava.Speckle tracking echocardiography,including the often-forgotten atrial strain evaluation,is a sensitive parameter that should be routinely used for a better clinical and prognostic evaluation of patients with congenital heart disease(CHD).展开更多
Scimitar Syndrome is a congenital condition resulting in anomalous pulmonary venous drainage from the lung into the inferior vena cava. It is often associated with cardiac and pulmonary abnormalities, both which are d...Scimitar Syndrome is a congenital condition resulting in anomalous pulmonary venous drainage from the lung into the inferior vena cava. It is often associated with cardiac and pulmonary abnormalities, both which are determinants in the severity and timing of presentation. True incidence of this condition is unknown and likely to be significantly underestimated as many patients remain asymptomatic through adult life. The increase in availability and use of medical imaging suggests that more asymptomatic and subacute cases will be detected. The management in those with the delayed presentation of this condition is debated, however the mainstay of therapy remains medical.展开更多
Background & Aims: Fermentation site and increasing time of symptomatic gas would be different between irritable bowel syndrome (IBS) and small intestine bacterial overgrowth (SIBO). The aim of this study was to d...Background & Aims: Fermentation site and increasing time of symptomatic gas would be different between irritable bowel syndrome (IBS) and small intestine bacterial overgrowth (SIBO). The aim of this study was to determine the time for increase in abdominal gas following ingestion of lactulose and the possibility of differential diagnosis of SIBO and IBS. Methods: A prospective study was conducted on a series of IBS patients (n = 14) who were referred to the Oroku-Hospital (Okinawa, Japan) from June 6, 2014 to December 30, 2014. Imaging was first performed in early morning after fasting. After ingestion of lactulose, 1 - 4 plain abdominal radiographs were taken for investigation of increased gas during the indicated timeframe. Regions of interest of the gas areas were highlighted on the images obtained. Gas images were divided into three areas, the stomach, small intestine, and large intestine, and each total area was calculated. Results: At a dose of 10 g lactulose and an observation time of two hours, patients displayed no symptoms, and the gas volume was only slightly increased. However, when the dose of lactulose was increased (13 g/50 kg), and the observation time for the lactulose challenge was extended to 240 - 300 minutes, the results clearly demonstrated an increase in the gas produced in IBS patients. Conclusions: An increased dose of lactulose coupled with an extended observation time for the lactulose challenge clearly demonstrated an increase in the gas produced in IBS patients. Alterations in diet rather than antibiotics might reduce IBS symptoms.展开更多
Importance:Scimitar syndrome(SS)is a rare type of congenital heart disease characterized by total or partial anomalous venous drainage of the right lung to the inferior vena cava.However,the surgical repair techniques...Importance:Scimitar syndrome(SS)is a rare type of congenital heart disease characterized by total or partial anomalous venous drainage of the right lung to the inferior vena cava.However,the surgical repair techniques for SS vary according to patients'anatomical and pathological features.Objective:This study was performed to analyze the mid-term results of a less invasive surgical correction technique for SS in children.Methods:Eleven patients with SS who underwent surgical repair from January 2012 to March 2020 were retrospectively analyzed.The anomalous scimitar vein(SV)was directly reimplanted to the left atrium,and the concomitant atrial septal defect was simultaneously repaired with cardiopulmonary bypass.Results:Three male and eight female patients were included in the study.Their mean age was 3.1±1.3 years,and their mean body weight was 12.8±3.0 kg.Most patients had symptoms,such as upper respiratory tract infection,dyspnea,and recurrent pneumonia,and two patients had pulmonary hypertension.None of the 11 patients who underwent direct SV reimplantation by right thoracotomy developed bleeding,arrhythmia,heart failure,or perioperative death,and no patients required reoperation during a mean follow-up period of 36.6±15.2 months.Postoperative echocardiography revealed no restenosis or obstruction of the anastomosis in any patients.Interpretation:Surgical repair for SS by right thoracotomy and direct anastomosis of the SV to the posterior wall of the left atrium is safe and effective,with good long-term patency of the reimplanted SV and a low mortality rate.展开更多
文摘We describe a case of a rare congenital heart disorder,scimitar syndrome,diagnosed in an adult woman presenting with dyspnea on exertion,chest pain and recurrent episodes of pulmonary infections.The hallmark of the syndrome is the presence of an enlarged anomalous pulmonary vein draining into the inferior vena cava.Speckle tracking echocardiography,including the often-forgotten atrial strain evaluation,is a sensitive parameter that should be routinely used for a better clinical and prognostic evaluation of patients with congenital heart disease(CHD).
文摘Scimitar Syndrome is a congenital condition resulting in anomalous pulmonary venous drainage from the lung into the inferior vena cava. It is often associated with cardiac and pulmonary abnormalities, both which are determinants in the severity and timing of presentation. True incidence of this condition is unknown and likely to be significantly underestimated as many patients remain asymptomatic through adult life. The increase in availability and use of medical imaging suggests that more asymptomatic and subacute cases will be detected. The management in those with the delayed presentation of this condition is debated, however the mainstay of therapy remains medical.
文摘Background & Aims: Fermentation site and increasing time of symptomatic gas would be different between irritable bowel syndrome (IBS) and small intestine bacterial overgrowth (SIBO). The aim of this study was to determine the time for increase in abdominal gas following ingestion of lactulose and the possibility of differential diagnosis of SIBO and IBS. Methods: A prospective study was conducted on a series of IBS patients (n = 14) who were referred to the Oroku-Hospital (Okinawa, Japan) from June 6, 2014 to December 30, 2014. Imaging was first performed in early morning after fasting. After ingestion of lactulose, 1 - 4 plain abdominal radiographs were taken for investigation of increased gas during the indicated timeframe. Regions of interest of the gas areas were highlighted on the images obtained. Gas images were divided into three areas, the stomach, small intestine, and large intestine, and each total area was calculated. Results: At a dose of 10 g lactulose and an observation time of two hours, patients displayed no symptoms, and the gas volume was only slightly increased. However, when the dose of lactulose was increased (13 g/50 kg), and the observation time for the lactulose challenge was extended to 240 - 300 minutes, the results clearly demonstrated an increase in the gas produced in IBS patients. Conclusions: An increased dose of lactulose coupled with an extended observation time for the lactulose challenge clearly demonstrated an increase in the gas produced in IBS patients. Alterations in diet rather than antibiotics might reduce IBS symptoms.
基金The research is supported by Pediatric Special Key Project from Beijing Hospitals Authority Pediatric Collaborative Development Center,Grant No.XTYB201819。
文摘Importance:Scimitar syndrome(SS)is a rare type of congenital heart disease characterized by total or partial anomalous venous drainage of the right lung to the inferior vena cava.However,the surgical repair techniques for SS vary according to patients'anatomical and pathological features.Objective:This study was performed to analyze the mid-term results of a less invasive surgical correction technique for SS in children.Methods:Eleven patients with SS who underwent surgical repair from January 2012 to March 2020 were retrospectively analyzed.The anomalous scimitar vein(SV)was directly reimplanted to the left atrium,and the concomitant atrial septal defect was simultaneously repaired with cardiopulmonary bypass.Results:Three male and eight female patients were included in the study.Their mean age was 3.1±1.3 years,and their mean body weight was 12.8±3.0 kg.Most patients had symptoms,such as upper respiratory tract infection,dyspnea,and recurrent pneumonia,and two patients had pulmonary hypertension.None of the 11 patients who underwent direct SV reimplantation by right thoracotomy developed bleeding,arrhythmia,heart failure,or perioperative death,and no patients required reoperation during a mean follow-up period of 36.6±15.2 months.Postoperative echocardiography revealed no restenosis or obstruction of the anastomosis in any patients.Interpretation:Surgical repair for SS by right thoracotomy and direct anastomosis of the SV to the posterior wall of the left atrium is safe and effective,with good long-term patency of the reimplanted SV and a low mortality rate.
