Objective: To investigate the representability and etiological diagnostic value of myocardium samples obtained from patients with hypertrophic cardiomyopathy (HCM) by transthoracic echocardiography-guided percutaneous intramyocardial septal biopsy (myocardial biopsy of Liwen procedure). Methods: This study was a retrospective case-series analysis. Patients with HCM, who underwent myocardial biopsy of Liwen procedure and radiofrequency ablation in Xijing Hospital, Air Force Military Medical University from July to December 2019, were included. Demographic data (age, sex), echocardiographic data and complications were collected through electronic medical record system. The histological and echocardiographic features, pathological characteristics of the biopsied myocardium of the patients were analyzed. Results: A total of 21 patients (aged (51.2±14.5) years and 13 males (61.9%)) were enrolled. The thickness of ventricular septum was (23.3±4.5)mm and the left ventricular outflow tract gradient was (78.8±42.6)mmHg (1 mmHg=0.133 kPa). Eight patients (38.1%) were complicated with hypertension, 1 patient (4.8%) had diabetes, and 2 patients (9.5%) had atrial fibrillation. Hematoxylin-eosin staining of myocardial samples of HCM patients before radiofrequency ablation evidenced myocytes hypertrophy, myocytes disarray, nuclear hyperchromatism, hypertrophy, atypia, coronary microvessel abnormalities, adipocyte infiltration, inflammatory cell infiltration, cytoplasmic vacuoles, lipofuscin deposition. Interstitial fibrosis and replacement fibrosis were detected in Masson stained biopsy samples. Hematoxylin-eosin staining of myocardial samples of HCM patients after radiofrequency ablation showed significantly reduced myocytes, cracked nuclear in myocytes, coagulative necrosis, border disappearance and nuclear fragmentation. Quantitative analysis of myocardial specimens of HCM patients before radiofrequency ablation showed that there were 9 cases (42.9%) with mild myocardial hypertrophy and 12 cases (57.1%) with severe myocardial hypertrophy. Mild, moderate and severe fibrosis were 5 (23.8%), 9 (42.9%) and 7 (33.3%), respectively. Six cases (28.6%) had myocytes disarray. There were 11 cases (52.4%) of coronary microvessel abnormalities, 4 cases (19.0%) of adipocyte infiltration, 2 cases (9.5%) of inflammatory cell infiltration,6 cases (28.5%) of cytoplasmic vacuole, 16 cases (76.2%) of lipofuscin deposition. The diameter of cardiac myocytes was (25.2±2.8)μm, and the percentage of collagen fiber area was 5.2%(3.0%, 14.6%). One patient had severe replacement fibrosis in the myocardium, with a fibrotic area of 67.0%. The rest of the patients had interstitial fibrosis. The myocardial specimens of 13 patients were examined by transmission electron microscopy. All showed increased myofibrils, and 9 cases had disorder of myofibrils. All patients had irregular shape of myocardial nucleus, partial depression, mild mitochondrial swelling, fracture and reduction of mitochondrial crest, and local aggregation of myofibrillary interfascicles. One patient had hypertrophy of cardiomyocytes, but the arrangement of muscle fibers was roughly normal. There were vacuoles in the cytoplasm, and Periodic acid-Schiff staining was positive. Transmission electron microscopy showed large range of glycogen deposition in the cytoplasm, with occasional double membrane surround, which was highly indicative of glycogen storage disease. No deposition of glycolipid substance in lysozyme was observed under transmission electron microscope in all myocardial specimens, which could basically eliminate Fabry disease. No apple green substance was found under polarized light after Congo red staining, which could basically exclude cardiac amyloidosis. Conclusion: Myocardium biopsied samples obtained by Liwen procedure of HCM patients are representative and helpful for the etiological diagnosis of HCM.
Biopsy/adverse effects* ; Cardiomegaly/pathology* ; Cardiomyopathy, Hypertrophic/diagnosis* ; Eosine Yellowish-(YS) ; Fibrosis ; Heart Defects, Congenital ; Hematoxylin ; Humans ; Lipofuscin ; Male ; Myocardium/pathology* ; Retrospective Studies

Atrial Fibrillation ; pathology ; Heart Defects, Congenital ; pathology ; Heart Septal Defects, Atrial ; pathology ; Humans ; Male ; Middle Aged ; Pulmonary Veins ; pathology ; Vena Cava, Superior ; pathology

BACKGROUND: Pulmonary artery hypertension associated with congenital heart disease (PAH-CHD) occurs predominantly among patients with uncorrected CHD. Treatment of severe pediatric PAH-CHD remains a major intractability. This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction. METHODS: The data for 59 children with severe PAH-CHD who underwent surgical correction, with or without postoperative medication, between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively. A regression analysis, receiver-operating characteristic (ROC) curves, and Kaplan-Meier curves were used for survival analysis. RESULTS: Fifty-nine children with severe PAH-CHD underwent heart catheterization and correction, with or without specific anti-PAH drugs postoperatively, were included in this study. The pulmonary pressure, heart function, and ending events were observed and median observation period was 49 ± 20 months. Twenty-eight patients (50%) received at least one additional anti-PAH drug after correction. The survival rate after 2 years was 91.5% (54/59); two patients were in a critical condition, and three were lost to follow-up. Twelve patients (29%) still received over one additional PAH-specific therapy at follow-up, whereas 42 (75%) had successfully stopped drug treatment. Two patients (3.5%) died and one underwent a second thoracotomy to remove the ventricular septal defect patch. Acute vasoreactivity test (AVT) criteria had limited efficacy in predicting pediatric PAH-CHD, whereas pulmonary vascular resistance (PVR) ≤ 6.65 Wood units (WU)/m or PVR/systemic vascular resistance (SVR) ≤ 0.39 during AVT indicated a good prognosis after surgical correction with an AUC of 98.3% (95% confidence interval [CI]: 96.0-100%), 98.4% (95% CI: 96.0-100%) sensitivity of 100%, 100% and specificity of 82.1%, 92.9%, respectively. CONCLUSIONS Although the criteria for positive AVT currently used are unsuitable for pediatric patients with PAH-CHD, PVR and PVR/SVR during AVT are excellent predictors of outcome in pediatric PAH-CHD. Surgery aided by anti-PAH drugs is an effective strategy and should be recommended for severe pediatric PAH-CHD with PVR ≤ 6.65 WU/m and PVR/SVR ≤ 0.39 after iloprost aerosol inhalation.
Adolescent ; Child ; Child, Preschool ; Echocardiography ; Female ; Heart Defects, Congenital ; pathology ; surgery ; Humans ; Hypertension, Pulmonary ; pathology ; surgery ; Kaplan-Meier Estimate ; Male ; Prognosis ; ROC Curve ; Retrospective Studies ; Risk Factors

Cerebral Hemorrhage ; pathology ; physiopathology ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; pathology ; physiopathology ; Hemodynamics ; physiology ; Humans ; Infant ; Male ; Risk Factors ; Stroke ; pathology ; physiopathology ; Vascular Malformations ; pathology ; physiopathology

To investigate the incidence of postoperative abnormal liver function test (aLFT) for the children with heart surgery, and to analyze the clinical characteristics and risk factors.
 Methods: A total of 143 children younger than 18 years old who underwent heart surgery in 2017 were enrolled in this study. The liver function were examined one day preoperation and consecutive 5 days after operation. The clinical data of perioperative period were recorded and the risk factors for aLFT were analyzed.
 Results: There were 43/143 (30%) cases had aLFT, including 5/143 (3.5%) acute liver injury (ALI). In the 6 liver function tests, total bilirubin, and glutamic-oxalacetic aminotransferase and glutamic-pyruvic aminotransferase increased to the peak at the first day and the second day after operation, respectively, and albumin declined to the lowest level at the fourth day after operation. aLFT happened most common at the first day after operation(22/43, 51.2%). The patients in the aLFT(+) group had smaller body size, more proportion of the risk adjustment for congenital heart sugery-1 (RACHS-1) score ≥ 3 and cyanosis, longer cardiopulmonary bypass (CPB) time, higher postoperative cardiac troponin (cTnI) value, higher inotropic score (IS), more transfusion, and longer mechanical ventilation time than those in the aLFT(-) group. The aLFT(+) group had longer ICU and hospital time, higher morbidity and mortality than those in the aLFT(-) group (P<0.05). Logistic regression showed that RACHS-1≥3, cyanosis, CPB time, cTnI, IS, transfusion, and mechanical ventilation time were the risk factors for aLFT. Multiple factor analysis showed the mechanical ventilation time was an independent risk factor for aLFT (P<0.05).
 Conclusion: aLFT is common in children after congenital heart operation, which could deteriorate to poor outcome. The mechanical ventilation time is an independent risk factor for aLFT.
Adolescent ; Cardiac Surgical Procedures ; Child ; Child, Preschool ; Heart Defects, Congenital ; surgery ; Humans ; Liver ; injuries ; pathology ; Logistic Models ; Postoperative Complications ; Respiration, Artificial ; Retrospective Studies ; Risk Factors ; Treatment Outcome

BACKGROUNDCongenital heart disease (CHD) is the most common congenital malformations with high mortality and morbidity. The prevalence of CHD reported previously ranged from 4 per 1000 live births to 50 per 1000 live births. In this cross-sectional study, we aimed to document the prevalence of CHD in Langfang district of Hebei Province, China by analyzing data collected by hospitals located in 11 the counties of the district, as supported by a public health campaign.

METHODSA total of 67,718 consecutive 3-month-old infants were included from July 19, 2012 to July 18, 2014. Structural abnormalities were diagnosed based on echocardiography findings, including two-dimensional and color Doppler echocardiography results.

RESULTSOf the 67,718 infants, 1554 were found to have cardiac structural abnormalities. The total prevalence of CHD was 22.9 per 1000 live births, a value significantly higher than the previously reported prevalence of 8 cases per 1000 live births. The top five most common cardiac abnormalities were as follows: atrial septal defect (ASD, 605 cases, 8.93‰); ventricular septal defect (550 cases, 8.12‰); patent ductus arteriosus (228 cases, 3.37‰); pulmonary stenosis (66 cases, 0.97‰); and tetralogy of Fallot (32 cases, 0.47‰). The CHD prevalence differed by gender in this study ( χ2 = 23.498,P < 0.001), and the majority of ASD cases were females. Regional differences in prevalence were also found ( χ2 = 24.602,P < 0.001); a higher prevalence was found in urban areas (32.2 cases per 1000 live births) than in rural areas (21.1 cases per 1000 live births). There was a significant difference in the prevalence of CHD in preterm versus full-term infants ( χ2 = 133.443,P < 0.001). Prevalence of CHD in infants of maternal aged 35 years or over was significantly higher ( χ2 = 86.917,P < 0.001).

CONCLUSIONSThe prevalence of CHD in Langfang district was within the range reported using echocardiography. Echocardiography can be used to early diagnose the CHD.

China ; Cross-Sectional Studies ; Ductus Arteriosus ; pathology ; Echocardiography ; Female ; Heart Defects, Congenital ; pathology ; Heart Septal Defects, Atrial ; pathology ; Humans ; Male ; Prevalence ; Pulmonary Valve Stenosis ; pathology ; Tetralogy of Fallot ; pathology

Tetralogy of Fallot (TOF) is one of the most common forms of cyanotic congenital heart disease usually managed by serial surgical repairs. The repaired prosthetic valve or conduit is susceptible to life-threatening infection. FDG-PET is an effective alternative to evaluate the source of infection when other examinations are inconclusive. We report an unusual case of an infected pulmonary artery conduit after TOF repair although the echocardiogram was negative for vegetation, which was later confirmed by surgery and pathology. The case highlights the role of FDG-PETas a problem-solving tool for potential endocarditis and cardiac device infection cases after complex cardiac surgery.
Endocarditis ; Heart Defects, Congenital ; Pathology ; Pulmonary Artery ; Tetralogy of Fallot ; Thoracic Surgery

No abstract available.
Abnormalities, Multiple/*genetics/pathology ; *Chromosomes, Human, Pair 14 ; Clubfoot/*genetics/pathology ; Female ; Gene Duplication ; Heart Defects, Congenital/*genetics/pathology ; Humans ; Infant, Newborn ; Karyotype ; Oligonucleotide Array Sequence Analysis

OBJECTIVETo discuss the experience of surgical treatment of total anomalous pulmonary venous connection (TAPVC) in infants.

METHODSThe clinic data of 84 cases with TAPVC under 6 months of age underwent surgical treatment at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center from January 2012 to October 2015 were analyzed retrospectively. There were 58 male and 26 female patients. The patients were aged 1 days to 6 months with a mean of (2.4±2.2) months at surgery, including 22 newborns. Body weight was 1.8 to 6.8 kg with a mean of (4.3±1.2) kg. There were 24 cases of intracardiac type, 46 cases of supracardiac type, 10 cases of infracardiac type and 4 cases of mixed type. There were 26 cases received emergent operation. There were 14 cases used Sutureless technique in operations and 46 cases used conventional methods in the no-intracardiac type cases, and 2 cases enlarged the anastomsis with autologous pericardium. According to the condition, corrective surgeries of other anomalies were performed in the meantime, including 3 Warden operations (right side), 3 bilateral bidirectional Gleen operation, 2 correction of unroofed coronary sinus syndrome, 1 coarctation of aorta correction with deep hypothermic circulation arrest, and 1 repair of ventricular septal defect.

RESULTSThe ratio of newborn was higher in Sutureless technique group than in conventional methods group (7/14 vs. 32.6%, χ(2)=4.927, P=0.043), and mean age was less ((1.8±0.4) months vs. (2.4±2.2) months, F=4.257, P=0.042), but there were no difference in body weight, cardiopulmonary bypass time and aorta clamped time between the two groups. Followed up for 1 to 46 months, 10 cases (11.9%) died overall and the mortality of intracardiac (3/10) and mixed (2/4) type were much higher than in intracardiac (4.2%) and supracardiac (13.0%) type. The mortality were no difference between newborn and infants, or whether emergent operation, or Sutureless technique and conventional methods. The maximal pulmonary venous flow velocity was abnormal speed-up >1.8 m/s at 1 week and 1 to 3 months post-operation mostly.

CONCLUSIONSThe mortality of TAPVC was differed by different types. Intrinsic pulmonary vein stenosis maybe the main cause of mortality. The high quality of anastomsis could reduce the operative mortality.

Aortic Coarctation ; Body Weight ; Cardiac Surgical Procedures ; methods ; Circulatory Arrest, Deep Hypothermia Induced ; Female ; Heart Defects, Congenital ; mortality ; surgery ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Infant, Newborn ; Male ; Postoperative Period ; Pulmonary Veins ; pathology ; surgery ; Retrospective Studies

Fontan surgery is a widely used palliative procedure that significantly improves the survival period of patients with complex congenital heart disease (CHD). However, it does not decrease postoperative complication rate. Previous studies suggested that elevated mean pulmonary artery pressure (mPAP) and vascular resistance lead to decreased exercise tolerance and myocardial dysfunction. Therapy with endothelial receptor antagonists (Bosentan) has been demonstrated to improve the patients' prognosis. A double-blind, randomized controlled trial was performed to explore the efficacy of Bosentan in treating patients who underwent the Fontan procedure. Eligible participants were randomly divided into Bosentan group and control group. Liver function was tested at a local hospital and the results were reported to the phone inspector every month. If the results suggested abnormal liver function, treatment would be adjusted or terminated. All the participants finished the follow-up study, with no patients lost to follow-up. Unblinding after 2-year follow-up, no mortality was observed in either group. However, secondary end-points were found to be significantly different in the comparable groups. The cardiac function and 6-min walking distance in the Bosentan group were significantly superior to those in the control group (P=0.018 and P=0.027). Bosentan could improve New York Heart Association (NYHA) functional status and improve the results of the 6-min walking test (6MWT) in Fontan patients post-surgery, and no other benefits were observed. Furthermore, a primary meta-analysis study systematically reviewed all the similar clinical trails worldwide and concluded an overall NYHA class improvement in Fontan patients who received Bosentan treatments.
Adolescent ; Child ; Double-Blind Method ; Female ; Follow-Up Studies ; Fontan Procedure ; Heart Defects, Congenital ; drug therapy ; pathology ; surgery ; Humans ; Liver ; drug effects ; pathology ; Male ; Palliative Care ; Prognosis ; Sulfonamides ; administration & dosage ; Treatment Outcome