bims-hylehe Biomed news on
Hypoplastic Left Heart Syndrome
Issue of 2018‒02‒25
eight papers selected by
Richard James
University of Pennsylvania


  1. Indian Heart J. 2018 Jan - Feb;70(1):doi: 10.1016/j.ihj.2017.03.00870(1):
    Abstract:  OBJECTIVE: To study the echocardiographic features of criss-cross heart (CCH), a congenital cardiac anomaly characterized by crossed ventricular inflow streams, in Indian patients. METHODS: In this retrospective observational study, all pediatric echocardiograms performed in a single tertiary care institution in South India over a three-year period were scrutinized for a diagnosis of CCH. Demographic, clinical and echocardiographic data were collected from patients' medical records and echocardiographic database. Crossed ventricular inflow streams was identified when there was inability to visualize both atrio-ventricular valves in a single imaging plane in cardiac four chamber view. RESULTS: CCH was diagnosed in five patients from 10,500 pediatric echocardiographic studies. The age at diagnosis ranged from one month to 8 years. Cyanosis was present in all but one of the five cases. Crossed ventricular inflow streams was present by definition in all cases, whereas superior-inferior ventricular relationship was present in only three cases. All cases were associated with ventricular septal defects. Atrio-ventricular discordance was seen in three cases and concordance in two. Ventriculo-arterial discordance was seen in three cases, concordance in one and double outlet right ventricle in one. Three cases had pulmonary stenosis and the other two had pulmonary arterial hypertension. Straddling of AV valve was observed in four cases and hypoplastic aortic arch in one case. CONCLUSION: CCH is an extremely rare congenital cardiac anomaly. Superior-inferior ventricular relationship often co-exists with CCH, but is not necessarily present in all cases. CCH requires early diagnosis because of its common association with diverse cardiac anomalies.
    Keywords:  Criss-cross heart; Echocardiography; Heart defects congenital
  2. Heart Vessels. 2018 Feb 17. doi: 10.1007/s00380-018-1137-1
    Abstract:  Although the number of pregnancies in women with cardiac disease is increasing worldwide, there are few data concerning their clinical characteristics and peripartum outcomes. Using the Diagnosis Procedure Combination database between 2008 and 2014 in Japan, we retrospectively identified pregnant women who underwent high-risk delivery due to obstetric or non-obstetric comorbidities. We classified eligible women into those with pre-existing cardiac disease (cardiac disease group) and those with non-cardiac comorbidities (non-cardiac disease group) and compared their characteristics and peripartum outcomes. Of 94,364 women undergoing high-risk delivery at 556 hospitals, 857 (0.91%) had pre-existing cardiac disease (302, congenital heart disease; 190, arrhythmia; 176, valvular heart disease; 120, ischemic heart disease; 65, cardiomyopathy; 4, pericardial disease). Women in the cardiac disease group were more likely to be treated at university hospitals (51.1 versus 28.6%; p < 0.001) and in intensive care units (33.5 versus 18.8%; p < 0.001) than those in the non-cardiac disease group. The proportion of cesarean deliveries was 69.4% (emergency, 28.4%; elective, 41.1%) in the cardiac disease group and 73.4% (emergency, 38.4%; elective, 35.0%) in the non-cardiac disease group. Epidural analgesia during vaginal delivery was used significantly more frequently in the cardiac disease than non-cardiac disease group (15.6 versus 2.3%; p < 0.001). Heart failure occurred more frequently in the cardiac disease than the non-cardiac disease group (10.2 versus 0.3%; p < 0.001). In cardiac subgroup comparisons, heart failure occurred more frequently in women with congenital heart disease (12.3%), valvular heart disease (12.5%), or cardiomyopathy (12.3%) than in women with arrhythmia (6.3%) or ischemic heart disease (5.8%). Multivariable logistic regression analysis showed a significant positive association between pre-existing cardiac disease and risk of heart failure (adjusted odds ratio, 24.7; 95% confidence interval, 17.6-34.6; p < 0.001). No woman in the cardiac disease group died, whereas 18 women (0.02%) in the non-cardiac disease group did (p = 1.000). These findings suggest that pregnant women with pre-existing cardiac disease are at a higher risk of heart failure during the peripartum period than those with non-cardiac comorbidities.
    Keywords:  Cardiac disease; Congenital heart disease; Delivery; Heart failure; Pregnancy
  3. Ann Thorac Surg. 2018 Mar;doi: 10.1016/j.athoracsur.2017.10.008105(3):
    Abstract:  A baby with pulmonary atresia with intact ventricular septum and hypoplastic right ventricle, with suspected right ventricle to coronary communications, was operated on for placing an aortopulmonary shunt. Postoperatively, the baby deteriorated with features of myocardial ischemia. Postmortem examination revealed anomalous origin of left coronary artery from pulmonary artery that caused significant coronary ischemia on ligation of the ductus arteriosus. Although coronary anomalies, including right ventricle dependent coronary circulation, has been well described, this is the first report of anomalous origin of coronary artery from pulmonary artery in a baby with pulmonary atresia and intact ventricular septum.
  4. J Clin Psychol Med Settings. 2018 Feb 17. doi: 10.1007/s10880-018-9554-y
    Abstract:  Living with congenital heart disease (CHD) presents survivors with numerous stressors, which may contribute to emotional problems. This study examined (a) whether coping with CHD-related stress predicts symptoms of depression and anxiety, and (b) whether associations between coping and emotional distress are moderated by involuntary stress reactivity. Adolescents and young adults diagnosed with CHD (Mage = 26.4) were recruited from pediatric and adult outpatient cardiology clinics. Participants (N = 168) completed online self-report measures. Hierarchical multiple regression analyses revealed that secondary control coping (e.g., cognitive restructuring, positive thinking) predicted lower depression and anxiety. Primary control coping (e.g., problem-solving) and stress reactivity (e.g., rumination, emotional numbing) interacted to predict depression and anxiety: the higher individuals were in involuntary stress reactivity, the stronger was the association between primary control coping and lower depression and anxiety. These results can inform clinical efforts to prevent or reduce emotional distress among CHD survivors.
    Keywords:  Anxiety; Congenital heart disease; Coping; Depression; Stress responses
  5. Indian Heart J. 2018 Jan - Feb;70(1):doi: 10.1016/j.ihj.2017.12.00570(1):
    Abstract:  This article reviews important features to improve the diagnosis of congenital heart disease (CHD) by applying ultrasound in prenatal cardiac screening. As low and high-risk pregnancies for CHD are subject to routine obstetric ultrasound, the diagnosis of structural heart defects represents a challenge that involves a team of specialists and subspecialists on fetal ultrasonography. In this review, the images highlight normal anatomy of the heart as well as pathologic cases consistent with cardiac malposition and isomerism, septal defects, pulmonary stenosis/atresia, aortic malformations, hypoplastic left ventricle, conotruncal anomalies, tricuspid dysplasia, and Ebstein's anomaly, and univentricular heart, among other congenital cardiovascular defects. Anatomical details of most CHD in fetuses were provided by two-dimensional (2D) ultrasound with higher quality imaging, enhancing diagnostic accuracy in a variety of CHD. Moreover, the accuracy of the cardiac defects in obstetrics ultrasound improves the outcome of most CHD, providing planned delivery, aided genetic counseling, and perinatal management.
    Keywords:  Congenital heart disease; Echocardiography; Prenatal diagnosis; Ultrasound imaging
  6. Indian Heart J. 2018 Jan - Feb;70(1):doi: 10.1016/j.ihj.2017.10.01770(1):
    Abstract:  OBJECTIVES: To study pregnancy outcomes in operated vs non-operated cases of congenital heart disease cases during pregnancy. MATERIALS AND METHODS: A total of 55 patients of congenital heart disease who delivered in the authors unit in last 10 years were taken in this retrospective study. These were divided into two groups Group 1:29 (52.7%) patient who had no cardiac surgery and Group 2: 26(47.2%) who had cardiac surgery to correct their cardiac defect before pregnancy. All patients were evaluated for cardiac complications and outcome during pregnancy. Obstetric complications, mode of delivery and fetal outcome was compared in the two groups using statistical analysis. RESULT: The commonest lesion was atrial septal defect (ASD) seen in 22(40%) patients followed by ventricular septal defect (VSD) in 16(29%) .Congenital valvular disease 8(14.5%) and patent ductus arteriosus in 4(7.2%) cases. The mean age was 25.9 ± 3.15 years in Group 1 and 26.3 ± 4.53 years in Group 2. The baseline characteristics were similar in the two groups. There was no difference in cardiac complications, NYHA deterioration and need of cardiac drugs in the two groups. Obstetric complications and mode of delivery were also similar in the two groups.Mean birth weight was 2516.65 ± 514.04 gm in Group 1 and 2683.00 ± 366.00 gm in Group 2 and was similar. APGAR < 8, stillbirth rate and other neonatal complications were also similar in two groups. CONCLUSION: The maternal and fetal outcome was excellent in patients with congenital heart disease and was similar in unoperated and operated cases.
    Keywords:  Cardiac surgery; Congenital heart disease; NYHA deterioration; Obstetric complications
  7. J Allergy Clin Immunol. 2018 Feb 14. doi: 10.1016/j.jaci.2018.01.037
    Keywords:  T cell; Thymus; autoimmunity; cancer; congenital heart defect; immunology; infection; thymectomy
  8. Bratisl Lek Listy. 2018 ;doi: 10.4149/BLL_2018_018119(2):
    Abstract:  Liver dysfunction in heart failure is common and usually clinically significant, especially in patients with advanced or severe acute heart failure. Lesions are caused by an impaired hepatic circulation due to congestion and hypoperfusion. Congestive lesions are more common and typically manifested by painful hepatomegaly and increased direct bilirubin and alkaline phosphatase. The inferior vena cava and hepatic veins are usually dilated. Congestive lesions are characterized by dilatation of the central vein with fibrotic changes in the surrounding areas on histological examination. Isolated ischaemic lesions are rare and occur due to severe and prolonged ineffective perfusion, often accompanied by hypoxemia. Ineffective perfusion is reflected by an increase in total bilirubin and significantly increased transaminase levels. The prognosis of ischaemic lesions without an adequate treatment of the cause of hypoperfusion is poor. Increased levels of bilirubin and liver function tests, as well as signs of impaired liver proteosynthetic function, are associated with a poor prognosis. Knowledge of the phenotypes of hepatic lesions in heart failure is important to select the appropriate treatment for an acute decompensation. Changes in biochemical markers, hepatic perfusion or stiffness of the liver can be used to evaluate the effectiveness of diuretic treatment and achieve euvolemic status in the patients with heart failure (Tab. 1, Fig. 3, Ref. 28).
    Keywords:  heart failure congestion.; liver dysfunction