scholarly journals Anesthetic Management of a Patient With Emanuel Syndrome

2016 ◽  
Vol 63 (4) ◽  
pp. 201-203
Author(s):  
Masanori Tsukamoto ◽  
Takashi Hitosugi ◽  
Kanako Esaki ◽  
Takeshi Yokoyama
Keyword(s):  
Congenital Heart ◽  
Septal Defect ◽  
Anesthetic Management ◽  
Genetic Material ◽  
Supernumerary Chromosome ◽  
Pulmonary Artery Stenosis ◽  
Chromosome 11 ◽  
Close Attention ◽  
Seizure Disorders ◽  
Emanuel Syndrome

Emanuel syndrome is associated with supernumerary chromosome, which consists of the extra genetic material from chromosome 11 and 22. The frequency of this syndrome has been reported as 1 in 110,000. It is a rare anomaly associated with multiple systemic malformations such as micrognathia and congenital heart disease. In addition, patients with Emanuel syndrome may have seizure disorders. We experienced anesthetic management of a patient with Emanuel syndrome who underwent palatoplasty. This patient had received tracheotomy due to micrognathia. In addition, he had atrial septal defect, mild pulmonary artery stenosis, and cleft palate. Palatoplasty was performed without any complication during anesthesia. Close attention was directed to cardiac function, seizure, and airway management.

scholarly journals On non-healing of the interventricular septum of the heart (Roger's disease)

1935 ◽  
Vol 31 (1) ◽  
pp. 27-35
Author(s):  
F. F. Piaid
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Septal Defect ◽  
Interventricular Septum ◽  
Artery Stenosis ◽  
Pulmonary Artery Stenosis

Simple uncomplicated forms of congenital heart disease in adults - pulmonary artery stenosis, non-healing of the Botall's duct - although they are a rarity, still studied more or less enough, and their lifelong recognition is not particularly difficult. Unfortunately, this cannot be said of another heart anomaly, a ventricular septal defect.

scholarly journals Rare combination of aortopulmonary septal defect with other heart defects

2001 ◽  
Vol 82 (6) ◽  
pp. 457-458
Author(s):  
V. A. Lukanikhin ◽  
G. I. Kharitonov ◽  
N. G. Shigabutdinova ◽  
B. A. Ostroumov
Keyword(s):  
Pulmonary Artery ◽  
Congenital Heart ◽  
Septal Defect ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Coarctation Of The Aorta ◽  
Ascending Aorta ◽  
Pulmonary Artery Stenosis ◽  
Patent Ductus

Defects between the ascending aorta and the pulmonary artery are among the rare congenital heart defects (CHD), occurring at a rate of 0.27%. In 10-15% of cases this CHD is combined with patent ductus arteriosus (PDP), coarctation of the aorta. Single cases of its combination with pulmonary artery stenosis have been described in the literature. We present a case of successful correction of aortopulmonary septal defect (ASD) combined with PDP and stenosis of the bicuspid pulmonary valve.

Prenatal differential ultrasound diagnosis of congenital heart diseases with interventricular septal defect and dextroposed aorta

2017 ◽  
Author(s):  
M.V. Medvedev, M.V. Kubrina
Keyword(s):  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Prenatal Ultrasound ◽  
Ultrasound Diagnosis ◽  
Congenital Heart Diseases

Main prenatal ultrasound differential patterns of congenital heart diseases including interventricular septal defect and dextroposed aorta are presented.

Early Extubation in a Pediatric Cardiac Surgery Program Located at High Altitude

2021 ◽  
Vol 12 (4) ◽  
pp. 473-479
Author(s):  
Orlando José Tamariz-Cruz ◽  
Luis Antonio García-Benítez ◽  
Hector Díliz-Nava ◽  
Felipa Acosta-Garduño ◽  
Marcela Barrera-Fuentes ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Operating Room ◽  
Sea Level ◽  
Congenital Heart ◽  
Heart Surgery ◽  
Anesthetic Management ◽  
Fontan Procedure ◽  
Congenital Heart Surgery ◽  
Serum Lactate ◽  
Early Extubation

Background: Early extubation is performed either in the operating room or in the cardiovascular intensive care unit during the first 24 postoperative hours; however, altitude might possibly affect the process. The aim of this study is the evaluation of early extubation feasibility of patients undergoing congenital heart surgery in a center located at 2,691 m (8,828 ft.) above sea level. Material and Methods: Patients undergoing congenital heart surgery, from August 2012 through December 2018, were considered for early extubation. The following variables were recorded: weight, serum lactate, presence or not of Down syndrome, optimal oxygenation and acid–base status according to individual physiological condition (biventricular or univentricular), age, bypass time, and ventricular function. Standardized anesthetic management with dexmedetomidine–fentanyl–rocuronium and sevoflurane was used. If extubation in the operating room was considered, 0.08 mL/kg of 0.5% ropivacaine was injected into the parasternal intercostal spaces bilaterally before closing the sternum. Results: Four hundred seventy-eight patients were operated and 81% were early extubated. Mean pre- and postoperative SaO2 was 92% and 98%; postoperative SaO2 for Glenn and Fontan procedures patients was 82% and 91%, respectively. Seventy-three percent of patients who underwent Glenn procedure, 89% of those who underwent Fontan procedure (all nonfenestrated), and 85% with Down syndrome were extubated in the operating room. Reintubation rate in early extubated patients was 3.6%. Conclusion: Early extubation is feasible, with low reintubation rates, at 2,691 m (8,828 ft.) above sea level, even in patients with single ventricle physiology.

scholarly journals Identification and characterization of a novel ELN mutation in congenital heart disease with pulmonary artery stenosis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Cuilan Hou ◽  
Junmin Zheng ◽  
Wei liu ◽  
Lijian Xie ◽  
Xiaomin Sun ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Molecular Mechanism ◽  
Protein Level ◽  
Congenital Heart ◽  
Artery Stenosis ◽  
Pulmonary Artery Stenosis ◽  
Functional Component ◽  
Functional Changes

AbstractCongenital heart defects, one of the most common birth defects, affect approximately 1% of live birth globally and remain the leading cause of infant mortality in developed countries. Utilizing the pathogenicity score and inheritance mode from whole exome sequencing results, a heterozygous mutation (NM_001278939.1: c.1939G>T, p.Gly647Ter) in elastin (ELN) was identified among 6,440 variants in a female proband born with an atrial septal defect accompanied by pulmonary artery stenosis. Results of RT-PCR showed that the mutation (NM_001278939.1: c.1939G>T, p.Gly647Ter) did not affect the expression levels of ELN mRNA but increased protein level. The content of ELN truncate (functional component) was significantly lower in both the intracellular and extracellular compartments after mutation. These results indicate that the ELN mutation (NM_001278939.1: c.1939G>T, p.Gly647Ter) affected the protein truncate, which may be a functional component of ELN and play crucial roles for this pedigree. Here we report of an ELN heterozygous variant associated with congenital heart disease accompanied with pulmonary artery stenosis, which is less common. Based on our results, we speculate that this may be the main molecular mechanism underlying the mutation-led functional changes, and propose that the decrease of ELN protein level may cause this pedigree vascular abnormality, especially pulmonary artery stenosis, and reinforce the view that ELN insufficiency is the primary cause of these vascular lesions. This may be the main molecular mechanism underlying the mutation-led functional changes. Thus, systematic analysis not only enables us to better understand the etiology of this disease but also contributes to clinical and prenatal diagnosis.

Atrial septal defect occlusion by a catheter during reperfusion of homograft in a case of living donor liver transplantation

2021 ◽  
pp. 1-2
Author(s):  
Shiro Baba ◽  
Takuya Hirata ◽  
Hideaki Okajima
Keyword(s):  
Liver Transplantation ◽  
Atrial Septal Defect ◽  
Living Donor ◽  
Living Donor Liver Transplantation ◽  
Septal Defect ◽  
Liver Graft ◽  
Pulmonary Artery Stenosis ◽  
Donor Liver ◽  
Easy Method ◽  
Donor Liver Transplantation

Abstract Liver transplantation for patients with atrial septal defect and pulmonary artery stenosis, causing high right atrium pressure, raises concerns about embolism in systemic vessels during reperfusion of the donor liver graft. Temporal atrial septal defect occlusion by a catheter is a simple and easy method of preventing the complication.

Pediatric Ventricular Assist Device Implantation: An Anesthesia Perspective

2021 ◽  
pp. 108925322199854
Author(s):  
Dash F. T. Newington ◽  
Fabrizio De Rita ◽  
Alan McCheyne ◽  
Claire Louise Barker
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Extracorporeal Membrane Oxygenation ◽  
Congenital Heart ◽  
Anesthetic Management ◽  
Inotropic Support ◽  
Ventricular Assist Devices ◽  
Ventricular Assist ◽  
Membrane Oxygenation ◽  
Delayed Sternal Closure

Background Ventricular assist devices (VADs) are increasingly being implanted in children, yet there is little literature to guide anesthetic management for these procedures. Aims To describe the pediatric population presenting for VAD implantation and the anesthetic management these patients receive. To compare (a) children under and over 12 months of age and (b) children with and without congenital heart disease. Methods Retrospective review of patients aged 0 to 17 years who underwent VAD implantation at a single center between 2014 and 2019. Results Seventy-seven VADs were implanted in 68 patients (46 left VADs, 24 biventricular VADs, 6 right VADs, and 1 univentricular VAD). One procedure was abandoned. Preoperatively, 20 (26%) patients were supported with extracorporeal membrane oxygenation and 57 (73%) patients were ventilated. Intraoperative donor blood products were required in 74 (95%) cases. Postimplantation inotropic support was required in 66 (85%) cases overall and 46 (100%) patients receiving a left VAD. Infants under 12 months were more likely to require preoperative extracorporeal membrane oxygenation (42% vs 19%), have femoral venous access (54% vs 28%), receive an intraoperative vasoconstrictor (42% vs 24%), and have delayed sternal closure (63 vs 22%). Mortality was higher in patients under 12 months (25% vs 19%) and in patients with congenital heart disease (25% vs 20%). Conclusions Children undergoing VAD implantation require high levels of preoperative organ support, high-dose intraoperative inotropic support, and high-volume blood transfusion. Children under 12 months and those with congenital heart disease are particularly challenging for anesthesiologists and have worse overall outcomes.

scholarly journals Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Heart Defects ◽  
Descriptive Analysis ◽  
Atrioventricular Septal Defect

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

scholarly journals Prognostic Value of CTA-Derived Left Ventricular Mass in Neonates with Congenital Heart Disease

Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1215
Author(s):  
Stephan Ellmann ◽  
Julie-Marie Nickel ◽  
Rafael Heiss ◽  
Nouhayla El Amrani ◽  
Wolfgang Wüst ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricular Mass ◽  
Congenital Heart ◽  
Left Ventricular ◽  
Surgical Approaches ◽  
Pulmonary Artery Stenosis ◽  
Operating Characteristics ◽  
Ventricular Mass ◽  
Therapeutic Decisions

For therapeutic decisions regarding uni- or biventricular surgical repair in congenital heart disease (CHD), left ventricular mass (LVM) is an important factor. The aim of this retrospective study was to determine the LVM of infants with CHD in thoracic computed tomography angiographies (CTAs) and to evaluate its usefulness as a prognostic parameter, with special attention paid to hypoplastic left heart (HLH) patients. Manual segmentation of the left ventricular endo- and epicardial volumes was performed in CTAs of 132 infants. LVMs were determined from these volumes and normalized to body surface area. LVMs of patients with different types of CHD were compared to each other using analyses of variances (ANOVA). An LVM cutoff for discrimination between uni- and biventricular repair was determined using receiver operating characteristics. Survival rates were calculated using Kaplan–Meier statistics. Patients with a clinical diagnosis of an HLH had significantly lower mean LVM (21.88 g/m2) compared to patients without applicable disease (50.22 g/m2; p < 0.0001) and compared to other CHDs, including persistent truncus arteriosus, left ventricular outflow tract obstruction, transposition of the great arteries, pulmonary artery stenosis or atresia, and double-outlet right ventricle (all, p < 0.05). The LVM cutoff for uni- vs. biventricular surgery was 33.9 g/m2 (sensitivity: 82.3%; specificity: 73.7%; PPV: 94.9%). In a subanalysis of HLH patients, a sensitivity of 50.0%, specificity of 100%, PPV of 100%, and NPV of 83.3% was determined. Patient survival was not significantly different between the surgical approaches or between patients with LVM above or below the cutoff. LVM can be measured in chest CTA of newborns with CHD and can be used as a prognostic factor.

ABNORMAL CAPILLARY MORPHOLOGY IN CONGENITAL HEART DISEASE

PEDIATRICS ◽  
1966 ◽  
Vol 37 (2) ◽  
pp. 316-322
Author(s):  
Stella B. Kontras ◽  
JoAnn G. Bodenbender
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Chronic Hypoxia ◽  
Normal Children ◽  
Vascular Abnormalities ◽  
Definitive Surgery ◽  
Vascular Compartment

Capillary morphology has been studied in 111 normal children. In 98% of these cases, straight hairpin forms make up over 80% of the capillaries examined. Thirty children with congenital heart disease showed 80% abnormal capillary patterns consisting of dilated, tortuous, and branched loops. These were most marked in cyanotic tetralogy of Fallot and ventricular septal defect with pulmonary hypertension. These cases also were associated with microscopic hemorrhages, increased capillary fragility and actual postoperative hemorrhagic complications. Definitive surgery in two cases, though improving the patient, did not result in changes in the abnormal capillary patterns. It is suggested that the role of the capillary structure in rheology of blood in the microvasculature has largely been ignored and that abnormalities in this vascular compartment may affect perfusion of tissues. The association of abnormalities in coagulation studies, hemorrhage, and thrombosis with congenital heart disease may in part be related to the morphologic vascular abnormalities. The abnormal capillary findings may be due to maturational arrest or dysmaturity of this portion of the cardiovascular system or may be the result of chronic hypoxia.

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