scholarly journals Translational value of partial resection of the pulmonary artery wall for congenital heart disease-induced bronchial compression

2019 ◽  
Vol 47 (10) ◽  
pp. 4949-4957
Author(s):  
Xiangyang Wu ◽  
Jie Zhu ◽  
Hao Liu ◽  
Wensheng Chen ◽  
Yalin Wei ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Treatment Group ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Partial Resection ◽  
Control Group ◽  
Artery Wall ◽  
Severe Congenital Heart Disease ◽  
Bronchial Compression

Objective This study was performed to evaluate the clinical efficacy and safety of a novel surgical procedure in treating tracheal or bronchial compression related to severe congenital heart disease. Methods The clinical data of 28 patients with tracheal or bronchial compression related to severe congenital heart disease were retrospectively analyzed. In the control group, 12 patients underwent surgery for congenital cardiac malformations. In the treatment group, 16 patients underwent surgery for congenital cardiac malformations combined with partial resection of the pulmonary artery wall. The cardiothoracic ratio, pulmonary arterial pressure, left ventricular end-diastolic dimension, diameter of the pulmonary artery, and diameter of the trachea in the stenotic segment were quantitatively measured before and 9 days after the operation. Results The diameter of the pulmonary artery and diameter of the trachea in the stenotic segment were almost restored to the normal range in the treatment group. Patients in the treatment group recovered more rapidly and effectively than those in the control group. Conclusion Partial resection of the pulmonary artery wall is an efficacious and safe technique in the treatment of tracheal or bronchial compression related to severe congenital heart disease.

scholarly journals Effects of Remifentanil and Sufentanil Anesthesia on Cardiac Function and Serological Parameters in Congenital Heart Surgery

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Zhigang Qin ◽  
Younian Xu
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Treatment Group ◽  
Cardiac Function ◽  
Congenital Heart ◽  
Heart Surgery ◽  
Congenital Heart Surgery ◽  
Control Group ◽  
Muscle Rigidity ◽  
Significant Difference

In this study, we have investigated feasibility of remifentanil and sufentanil anesthesia in children with congenital heart disease surgery and its effects on cardiac function and serological parameters. For this purpose, a retrospective study was conducted on 120 children with congenital heart disease who underwent repair of ventricular septum or atrial septum in our hospital, specifically from January 2016 to January 2018, and 60 patients in each group were randomly divided into the control and treatment groups, respectively. The control group was anesthetized with sufentanil, and the treatment group was anesthetized with remifentanil. The heart function, serological indexes, and adverse reactions were observed and compared. We have observed that there was no significant difference in HR levels between these groups ( P > 0.05 ), but SDP and DBP values of the two groups were decreased after anesthetic induction ( P < 0.05 ). ACH, cortisol, and lactic acid in the treatment group were significantly lower than those in the control group, and the difference was statistically significant ( P < 0.05 ). The incidence of bradycardia, nausea and vomiting, hypotension, muscle rigidity, and respiratory depression in the treatment group was 16.67% lower than that in the control group ( P < 0.05 ). Remifentanil has less influence on hemodynamics and a better analgesic effect than fentanyl in inhibiting stress response in congenital heart surgery, which provides reference and basis for children congenital heart surgery.

scholarly journals Evaluation and Cardiac Rehabilitation on a patient with a congenital heart disease

2021 ◽  
Vol 20 (Supplement_1) ◽  
Author(s):  
A Felix
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Cardiac Rehabilitation ◽  
Congenital Heart ◽  
Ductus Arteriosus ◽  
Interindividual Variation ◽  
Initial Assessment ◽  
Control Group ◽  
Developmental Outcome

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Severe congenital heart disease (CHD) requiring surgical intervention occurs in 6 of 1000 live-born infants. Substantial improvements in surgical treatment and perioperative care have led to excellent long-term cardiac outcomes for most of these patients and, consequentially, to a significant increase in survival rates. Formal evaluations of preschool, school-aged children and adolescents born with complex CHD demonstrate a pattern of neurodevelopmental sequelae, which may appear alone, or in combination. Purpose F. is a 21 years-old patient with repaired complex transposition of the great arteries with ventricular septal defect, who have already undergone three surgeries: a) an initial palliative surgery (pulmonary artery banding and ductus arteriosus ligation) at the neonatal period; b) a corrective surgery (arterial switch and VSD closure) at the end of the first year of life and c) Pulmonary artery plasty for residual pulmonary stenosis at the age of 16 years-old. Now, he was admitted to our department for urgent wide-complex tachycardia treatment.  His initial assessment documented a subjectively impaired motor development, and subsequently, a formal assessment was performed. Methods The Bruininks-Oseretsky Test of Motor Proficiency, second edition (BOT-2) is an individually administrated test that resorts to a series of engaging, goal-directed activities to measure a wide array of motor skills in individuals ages 4 through 21. The BOT-2 uses a subtest and composite structure that highlights motor performance in the broad functional areas of stability, mobility, strength, coordination, and object manipulation. Results Patient"s performance in the four main motor skill areas will be report, namely: Fine Manual Control, Manual Coordination, Body Coordination, and Strength and Agility. Because Fabio completed all four parts of the BOT-2, a comprehensive score from all four areas, the Total Motor Composite, will also be reported. Fabio"s scores were compared to the ones of a control group of age and gender-matched individuals. This Patient started sessions of Cardiac Rehabilitation. The rehabilitation sessions are conducting for 1 hour three times a week for 1 year. This program still in progress, but we speculate that the will improve his motor abilities and developmental skills. Conclusion The risk of a poor developmental outcome varies according to the specific cardiac effect. In addition, there is significant interindividual variation in developmental outcome even among children with the same cardiac defect. Other factors may contribute to neurologic dysfunctions. These factors can be divided into three main categories and time-frames: prenatal, perioperative and post-discharge.

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.

scholarly journals Anomalous Origin of the Left Anterior Descending Coronary Artery in an Adult

2021 ◽  
Vol 10 (01) ◽  
pp. e9-e10
Author(s):  
Keisuke Shibagaki ◽  
Chikara Shiiku ◽  
Hiroyuki Kamiya ◽  
Yoichi Kikuchi
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Coronary Artery ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Left Coronary Artery ◽  
Internal Thoracic Artery ◽  
Artery Bypass ◽  
Anomalous Origin ◽  
Bypass Grafting

AbstractAn anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease. Among the variants, an anomalous origin of the left anterior descending coronary artery from the pulmonary artery (ALADPA) is extremely rare. Here, we report a case of ALADPA in an adult that was treated with coronary artery bypass grafting using the left internal thoracic artery.

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