Background: Open heart surgery with cardiopulmonary bypass (CPB) causes haemostatic abnormalities which result in postoperative excessive bleeding. Objectives: To investigate haemostatic disorders before - after CPB and postoperative bleeding. Subjects and methods: Sixty congenital cardiac patients with and without cyanosis were recruited consecutively. Hematology and coagulation tests were done 1 day before operation, 15 minutes after protamine administration, 2 and 6 hours after the operation in the intensive care unit (lCU). Mediastinal chest tube drainage (MCTO) was measured for the first 6h in the ICU. Results: Significant differences between 2 groups could be found for red blood cells, hemoglobin, hematocrit, fibrinogen, D-dimer, fibrinogen degradation products (FOP), von Kaulla and platelet aggregation to epinephrine before operation (p < 0.05). There were not significant differences in platelets, PT, APTT, platelet aggregation to adenosine diphosphate (AOP) in the acyanotic and cyanotic patients (p > 0.05). Most hematology parameters were decreased significantly and hemostasis measurements were prolonged after operation in both groups (p < 0.05). There was also the significant difference in MCTO between 2 groups (p < 0.05). Conclusion: Coagulation, hemostasis and fibrinolysis disorders; thrombocytopenia and acquired transient platelet dysfunction may be responsible for bleeding complications after CBP \r\n', u'\r\n', u'
Heart Defects ; Congenital/ pathology ; epidemiology ; Hemostatic Disorders/ pathology ; diagnosis

Pulmonary arterial hypertension is a significant complication of congenital heart disease, which carries a recognized risk of morbidity and mortality. There have been remarkable advances in the field of pulmonary arterial hypertension over the past several decades. At the third world symposium on pulmonary arterial hypertension held in Venice, Italy, 2003, congenital cardiac shunts were classified in the same group as idiopathic pulmonary hypertension. This article discusses the recent advances in understanding the pathology, pathobiology, diagnosis, and treatment of pulmonary arterial hypertension associated pediatric congenital heart disease.
Diagnosis ; Heart Defects, Congenital ; Heart Diseases* ; Heart* ; Hypertension* ; Hypertension, Pulmonary ; Italy ; Mortality ; Pathology ; Pediatrics

Pulmonary arterial hypertension is a significant complication of congenital heart disease, which carries a recognized risk of morbidity and mortality. There have been remarkable advances in the field of pulmonary arterial hypertension over the past several decades. At the third world symposium on pulmonary arterial hypertension held in Venice, Italy, 2003, congenital cardiac shunts were classified in the same group as idiopathic pulmonary hypertension. This article discusses the recent advances in understanding the pathology, pathobiology, diagnosis, and treatment of pulmonary arterial hypertension associated pediatric congenital heart disease.
Diagnosis ; Heart Defects, Congenital ; Heart Diseases* ; Heart* ; Hypertension* ; Hypertension, Pulmonary ; Italy ; Mortality ; Pathology ; Pediatrics

MRI is useful for demonstrating the anatomy of various congenital cardiac lesions and plays an important role in the diagnosis of congenital cardiac lesions. Its large field of view and unlimited imaging planes enable the depiction of complex lesions, the complicated three-dimensional relations of the cardiac chambers and anomalies of the central pulmonary arteries, the systemic and pulmonary veins, and aorta. We describe the normal MR anatomy and MR imaging findings of a variety of congenital pathologies of the heart, in order to provide a better understanding and facilitate the interpretation of the MR features of various congenital heart diseases.
Aorta ; Diagnosis ; Heart ; Heart Defects, Congenital* ; Heart Diseases ; Magnetic Resonance Imaging* ; Pathology ; Pulmonary Artery ; Pulmonary Veins

Congenital left ventricular diverticulum is a very rare cardiac abnormality and it is not completely understood about its etiology, clinical manifestation, diagnosis, treatment and prognosis. This article presents a case of large congenital diverticulum of the left ventricle. The clinical manifestation included paroxysmal supraventricular tachycardia. The diagnosis was made by chest fluoroscopy observation and confirmed by 64-slice CT-angiography. The arrhythmia alleviated instead of antiarrhythmic drugs but by radiofrequency catheter ablation. Due to the rapid growth of the diverticulum, the patient underwent surgical resection finally. Owing to the fatal risks, clinicians should improve the understanding of this disease by deeply studying more cases, in order to standardize the treatment.
Adult ; Diverticulum ; diagnosis ; Female ; Fluoroscopy ; methods ; Heart Defects, Congenital ; diagnosis ; Heart Ventricles ; pathology ; Humans ; Young Adult

No abstract available.
Echocardiography ; Heart Defects, Congenital/*diagnosis/pathology ; Heart Ventricles ; Humans ; Magnetic Resonance Imaging ; Tomography, X-Ray Computed

No abstract available.
Echocardiography ; Heart Defects, Congenital/*diagnosis/pathology ; Heart Ventricles ; Humans ; Magnetic Resonance Imaging ; Tomography, X-Ray Computed

OBJECTIVETo improve the diagnostic accuracy of fetal pulmonary venous abnormalities through the analysis of the fetal pulmonary vein anatomy.

METHODS234 cases of congenital cardiac abnormalities were detected by echocardiography during pregnancy in An Zhen Hospital, Capital Medical University from May 2010 to August 2015. Autopsy was then performed. The type of fetal pulmonary venous malformation, cardiac abnormalities, systemic venous malformations, and other internal organs deformities were documented.

RESULTSThere were ninteen cases of pulmonary venous malformations among the 234 cases of fetal congenital heart disease. These included two cases of congenital pulmonary venous hypoplasia (CPVH) or atresia, four cases of partial anomalous pulmonary venous drainage (PAPVD), seven cases of total anomalous pulmonary venous drainage (TAPVD), five cases of atresia of common pulmonary vein (CPV), one case of congenital pulmonary venous hypoplasia with total anomalous pulmonary venous drainage. There were eleven cases with single ventricle, eight cases with right aortic arch, seven cases with single atrium and six cases with pulmonary valve stenosis. Eleven cases had pulmonary hypoplasia and nine cases had abnormal spleen.

CONCLUSIONSThere are many variations in pulmonary venous abnormalities associated with severe and complex cardiac abnormalities and internal organs malformation. Care should be exercised during autopsy examination to look for all branches of the pulmonary vein.

Autopsy ; Female ; Fetal Diseases ; Heart Defects, Congenital ; diagnosis ; Humans ; Pregnancy ; Pulmonary Veins ; abnormalities ; Spleen ; pathology

BACKGROUNDFetal congenital heart anomalies are the most common congenital anomalies in live births. Fetal echocardiography (FECG) is the only prenatal diagnostic approach used to detect fetal congenital heart disease (CHD). FECG is not widely used, and the antenatal diagnosis rate of CHD varies considerably. Thus, mastering the anatomical characteristics of different kinds of CHD is critical for ultrasound physicians to improve FECG technology. The aim of this study is to investigate the applications of a fetal CHD anatomic database in FECG teaching and training program.

METHODSWe evaluated 60 transverse section databases including 27 types of fetal CHD built in the Prenatal Diagnosis Center in Peking University People's Hospital. Each original database contained 400-700 cross-sectional digital images with a resolution of 3744 pixels × 5616 pixels. We imported the database into Amira 5.3.1 (Australia Visage Imaging Company, Australia) three-dimensional (3D) software. The database functions use a series of 3D software visual operations. The features of the fetal CHD anatomical database were analyzed to determine its applications in FECG continuing education and training.

RESULTSThe database was rebuilt using the 3D software. The original and rebuilt databases can be displayed dynamically, continuously, and synchronically and can be rotated at arbitrary angles. The sections from the dynamic displays and rotating angles are consistent with the sections in FECG. The database successfully reproduced the anatomic structures and spatial relationship features of different fetal CHDs. We established a fetal CHD anatomy training database and a standardized training database for FECG. Ultrasound physicians and students can learn the anatomical features of fetal CHD and FECG through either centralized training or distance education.

CONCLUSIONSThe database of fetal CHD successfully reproduced the anatomic structures and spatial relationship of different kinds of fetal CHD. This database can be widely used in anatomy and FECG teaching and training.

Cross-Sectional Studies ; Databases, Factual ; Female ; Fetus ; Heart Defects, Congenital ; diagnostic imaging ; pathology ; Humans ; Male ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography, Prenatal

BACKGROUNDThe incidence of congenital heart disease has been studied in developed countries for many years, but rarely in the mainland of China. Fetal echocardiographic screening for congenital heart disease was first performed in Beijing in the early 2000s, but the impact was not clear. The current study was undertaken to determine the incidence of congenital heart disease in Beijing, China and to estimate the impact of fetal echocardiography on the incidence of liveborn congenital heart disease.

METHODSThe study involved all infants with congenital heart disease among the 84 062 total births in Beijing during the period of January 1 and December 31, 2007. An echocardiographic examination was performed on every baby suspected to have congenital heart disease, prenatally or/and postnatally.

RESULTSA total of 686 infants were shown to have congenital heart disease among 84 062 total births. The overall incidence was 8.2/1000 total births. Mothers of 128 of 151 babies diagnosed prenatally were chosen to terminate the pregnancy. Two of the 151 infants died in utero. A specific lesion was identified for each infant and the frequencies of lesions were determined for each class of infants (total births, stillbirths and live births). The incidence of congenital heart disease in stillbirths and live births was 168.8/1000 and 6.7/1000, respectively. The difference between the incidence of total birth and the incidence of live birth was statistically significant (P < 0.001).

CONCLUSIONSThe incidence of liveborn congenital heart disease in Beijing is within the range reported in developed countries. Fetal echocardiography reduce significantly the incidence of liveborn congenital heart disease.

China ; epidemiology ; Echocardiography ; Female ; Fetal Heart ; diagnostic imaging ; Heart Defects, Congenital ; diagnostic imaging ; epidemiology ; pathology ; Humans ; Incidence ; Infant, Newborn ; Pregnancy ; Prenatal Diagnosis ; methods ; Stillbirth ; epidemiology