Home About us Contact
|
Home About us Contact | ||
|
|
||
Complex Congenital Heart Disease (complex + congenital_heart_disease)
Selected AbstractsCraniorachischisis and Heterotaxia with Heart Disease in Twins: Link or Change Nature?CONGENITAL HEART DISEASE, Issue 5 2010Sebastiano Bianca MD ABSTRACT Craniorachischisis is a rare neural tube defect in which both acrania and a complete schisis of the vertebral column are present. Heterotaxy results from failure to establish normal left,right asymmetry during embryonic development and is characterized by a variable group of congenital anomalies that include complex cardiac malformations and situs inversus or situs ambiguous. We report a diamniotic twin pregnancy with two malformed fetuses affected one by craniorachischisis and the other by heterotaxya with paired right-sided viscera, asplenia, and complex congenital heart disease. The occurrence of severe congenital anomalies in both members of the twin pair implies a strong influence of genetic factors. At present, the genetic basis determining the different phenotypes observed in our twins is unknown. Our case with the simultaneous presence of both midline and laterality defects in twins supports the hypothesis that the midline plays a critical role in establishing left,right asymmetry in the body and that a mutation in a gene responsible for both heterotaxy and midline defects may be strongly supposed. [source] Open-chest Epicardial Approach to Transcatheter Pulmonary Artery Stenting Following Heart Transplantation in an InfantCONGENITAL HEART DISEASE, Issue 1 2007Todd M. Gudausky MD ABSTRACT We describe an open-chest epicardial approach to transcatheter pulmonary artery stenting in a critically ill infant following heart transplantation. Technical considerations, indications, and feasibility are discussed. This case provides another example of the value of a "hybrid" approach (combining surgery and interventional cardiology) to complex congenital heart disease. [source] Use of rituximab to treat refractory Diamond-Blackfan anemiaEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 5 2005Akira Morimoto Abstract:, We report here the first case with Diamond-Blackfan anemia (DBA) who responded to rituximab. The patient is an 8-yr-old Japanese girl with refractory DBA accompanied by complex congenital heart disease. She received two doses of rituximab, 375 mg/m2/wk. She became transfusion independent 6 months after the treatment without any serious side effect. However, after 8 months of transfusion-free period, her condition returned to the pretreatment level with recovery of peripheral B cells. Rituximab may be a successful therapy for refractory DBA where B cells play a crucial role in the pathogenesis of the severe anemia. [source] Dental caries experience in children with congenital heart disease: a case-control studyINTERNATIONAL JOURNAL OF PAEDIATRIC DENTISTRY, Issue 2 2004C. Stecksén-Blicks Summary. Objectives., To compare the dental health of a group of children with complex congenital heart disease with that of age and gender matched healthy controls. Design., Case-control study. Setting., Faculty of Medicine and Odontology/Pediatric cardiology and Pedodontics, Umeĺ University, Sweden. Sample and Methods., All the cases and their controls lived in the county of Västerbotten in northern Sweden. Each group comprised 41 children with a mean age of 6·5 years. Data were collected from medical and dental records while all bitewing radiographs were read separately by one of the authors. Results., Children with congenital heart disease had significantly more caries in their primary teeth than the control group. The mean dmfs-value was 5·2 ± 7·0 in the cardiac group compared to 2·2 ± 3·5 in the control group (P < 0·05). Twenty-six of the children had all four 6-year-molars, and their mean DMFS-values were 0·9 ± 1·9 in the cardiac group compared to 0·3 ± 0·6 in the control group (P > 0·05). The children with congenital heart disease had received more caries prevention based on the use of fluorides than the control group. There was a significant correlation between the number of fluoride varnish treatments and the dmfs value of the child (r = 0·411, P < 0·01). Fifty-two per cent of the children in the cardiac group had been prescribed fluoride tablets on one or more occasions compared to 17% in the control group (P < 0·01). Number of months on digoxin medication and the dmfs-value had a significant correlation (r = 0·368, P < 0·05). Ten of the children had been on digoxin medication between 6 and 87 months; this subgroup had a mean dmfs-value of 10·1 ± 8·5. Conclusion., Swedish children with complex congenital heart disease have poorer dental health than healthy age and gender matched controls in spite of intensive preventive efforts. In many cases, intervention had been given when caries were present. A closer cooperation between paediatric cardiology and paediatric dentistry is needed. [source] Cardiac arrest at induction of anesthesia in a child with undiagnosed right-ventricular dependent coronary circulation: a case reportPEDIATRIC ANESTHESIA, Issue 11 2006TODD A. BROWN MD Summary Pediatric perioperative cardiac arrest occurs in 1.4 per 10 000 anesthetics, with an overall mortality rate of 26%. The etiology of the arrest is identifiable in the majority of these patients. We report the case of a child with a complex congenital heart defect, who sustained a cardiac arrest at induction of anesthesia, secondary to right-ventricular dependent coronary circulation. We discuss the incidence, risks, anesthetic management and outcomes of pediatric cardiac arrest in the perioperative period especially in patients with complex congenital heart disease. [source] Management of a cadaveric orthotopic liver transplantation in a pediatric patient with complex congenital heart diseasePEDIATRIC ANESTHESIA, Issue 6 2006DENNIS E. FEIERMAN MD PhD Summary Pediatric orthotopic liver transplantations (OLT) are commonly performed nowadays. Two primary reasons for OLT in children are complications from either extrahepatic biliary atresia (EHBA) or inborn errors of metabolism. However, congenital liver disease may be associated with significant other congenital abnormalities. We present a case of a successful OLT in a pediatric patient with a history of EHBA, situs inversus, and complex congenital heart disease. The cardiac anomalies include dextrocardia, absence of the atrial septum (single atrium), single atrioventricular valve (a-v canal), and an incomplete ventricular septum. Prior surgery include a Kasai procedure for EHBA, banding of the proximal main pulmonary artery, and Broviac catheter placement. We present the anesthesia concerns and management for this complicated case. [source] Spinal anaesthesia in a child with hypoplastic left heart syndromePEDIATRIC ANESTHESIA, Issue 3 2003S. Sacrista MD Summary Hypoplastic left heart syndrome (HLHS), a complex congenital heart disease, is the most common lethal cardiac defect in neonates. Its treatment includes cardiac transplantation and/or surgical palliation. Associated extracardiac congenital abnormalities are exceptional. We report the case of a neonate with HLHS and anorectal atresia who required urgent surgical management to relieve intestinal obstruction. The surgery was successfully performed under spinal anaesthesia. [source] Horseshoe lung and facio-auriculo-vertebral sequence: A previously unreported associationPEDIATRIC PULMONOLOGY, Issue 6 2006Lisa D'Alessandro MD Abstract We describe a case of horseshoe lung in an infant with facio-auriculo-vertebral (FAV) sequence that included mild hemifacial microsomia, ear anomalies, a missing left rib, left hemivertebrae (T2,T4), and complex congenital heart disease. Of the approximately 40 cases of horseshoe lung described since 1962, most are reported in association with scimitar syndrome, and only four reported cases were associated with left lung hypoplasia. None of these cases included malformations consistent with a diagnosis of FAV sequence. Pediatr Pulmonol. © 2006 Wiley-Liss, Inc. [source] Domino Heart Transplantation Involving InfantsAMERICAN JOURNAL OF TRANSPLANTATION, Issue 11 2007T. L. Astor Domino heart transplantation has been well described in adults, but has not previously been reported in infant patients. We report the successful transplantation of a ,domino' heart from a 3-month-old infant with primary pulmonary hypertension undergoing heart,lung transplantation, into a 3-month-old infant with complex congenital heart disease. Both infants have survived past 1 year post-transplant, and neither infant has experienced any clinically significant allograft-related complications. Echocardiography and cardiac catheterization of the domino heart have consistently demonstrated stable hypertrophy of the right ventricle (RV) and interventricular septum, but good right and left ventricular function. Domino heart transplant surgery may be an effective way to provide ,pre-conditioned' donor hearts to infants urgently in need of heart transplantation. [source] Perioperative Monitoring of Thromboelastograph on Hemostasis and Therapy for Cyanotic Infants Undergoing Complex Cardiac SurgeryARTIFICIAL ORGANS, Issue 11 2009Yongli Cui Abstract This study investigated features and treatments of perioperative coagulopathies in cyanotic infants with complex congenital heart disease (CCHD). Thirty-six infants with cyanotic CCHD were involved and divided into two groups: In group H (n = 20), hematocrit (HCT) > 54%, and in group L (n = 16), HCT < 54%. Blood was sampled at anesthesia induction (T1), rewarming to 36°C (T2), after heparin neutralization (T3), and 4 h after operation (T4). The hemostatic changes were evaluated by thromboelastograph (TEG). After surgery, group H was treated with fibrinogen-combined platelets (PLT), while group L was treated with PLT only. We observed the effect at T4. At T1, the hemostatic function in group H, deteriorating with the increase of HCT (P < 0.01), was obviously lower than that in group L (P < 0.01), but the PLT function was still complete. In group H, the hemostatic function at T2 decreased with a significant drop of PLT function (P < 0.01) and had little change of functional fibrinogen (Ffg) (P > 0.05). At T3, compared with T2, there were improvements in hemostatic function and Ffg (P < 0.01, respectively) without increase of PLT (P > 0.05) in group H. After therapy, PLT function in both groups restored to T1 level (P > 0.05); Ffg at T4 was significantly better than at T1 (P < 0.01) in group H, but Ffg at T4 with still normal function was lower than at T1 in group L (P < 0.01). Whole hemostatic function at T4 was back to normal and had no differences between two groups. So, we proposed that fibrinogen and PLT transfusion in combination should be better for infants with high HCT CCHD, but PLT alone might be enough for low HCT ones. [source] New transcatheter techniques for creation or enlargement of atrial septal defects in infants with complex congenital heart diseaseCATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS, Issue 5 2007Carlos A.C. Pedra MD Abstract Objectives: To describe a series of 8 consecutive infants (5 with transposition of the great arteries [TGA] and 3 with hypoplastic left heart syndrome [HLHS]) who underwent nonconventional septostomy techniques. Background: For some complex congenital heart defects, an unrestrictive atrial septal defect (ASD) is essential to achieve an adequate cardiac output and/or systemic saturation. In some scenarios, the use of conventional septostomy techniques may be technically difficult, hazardous, and/or ineffective. Methods: Use of transhepatic approach, cutting balloons, and radiofrequency perforation with stenting of the atrial septum. Results: The size of the ASD and the oxygen saturation increased in all patients with no major complications. In those with TGA, the ASDs were considered to be of good size at the arterial switch operation. Two of the 3 patients with hybrid palliation for HLHS have developed some degree of obstruction within the interatrial stent over 2,3 months. At surgery, the stents were found to be secured within the septum with one showing significant fibrous ingrowth after uneventful removal. The other had some nonobstructive ingrowth. Conclusions: Creation or enlargement of ASDs in infants using new nonconventional transcatheter techniques is feasible, safe, and effective, at least in the short-to-mid-term follow-up. Infants with TGA seem to benefit the most because the procedure results in satisfactory clinical stability for subsequent early surgical intervention. In infants with HLHS palliated by a hybrid approach, stent implantation to the atrial septum seems to buy enough time to bring them to the phase II safely despite progressive in-stent obstruction. © 2007 Wiley-Liss, Inc. [source] Cardiac magnetic resonance imaging in valvular heart diseaseCLINICAL PHYSIOLOGY AND FUNCTIONAL IMAGING, Issue 4 2009Juha W. Koskenvuo Summary Cardiac magnetic resonance imaging (CMR) has rapidly gained acceptance as an accurate, reproducible and non-invasive imaging method for assessment of a wide range of cardiovascular diseases. However, CMR has not been used widely for diagnostic purposes in valvular heart disease (VHD). Unlike echocardiography it has no body habitus-related limitations and can thus be used to complement echocardiography. It is an especially good alternative for clinical follow-up in patients with VHD, as it allows accurate measurement of valvular dysfunction and related ventricular burden. Additionally, CMR is an ideal method for evaluating complex congenital heart disease and determining the significance of its components. It can also be used to study the physiological course of valvular dysfunction and response to therapeutic interventions. In this review, we present a basic introduction to CMR methodology, including its advantages and potential problems, and the physiology and quantification in VHD. We also discuss clinical applications of CMR in VHD. Furthermore, we describe how a CMR study statement should be structured in order to increase clinical use of this valuable methodology in cardiology. [source] | ||||||||||