scholarly journals Management of a giant aortic root aneurysm in a young patient with Marfan syndrome: a case report

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Jiayu Shen ◽  
Changping Gan ◽  
R. D. T. Rajaguru ◽  
Dou Yuan ◽  
Zhenghua Xiao

Abstract Introduction Marfan syndrome (MFS) is a common heritable connective tissue disease involving multiple organs. Even though the clinical manifestations of MFS can be various, aortic root aneurysm is estimated as one of the most serious complications. We herein describe an individualized treatment decision-making process for a 23-year-old male with MFS, suffering from a giant but stable aortic root aneurysm which is extremely rare at his age. Case The patient, a 23-year-old male with a family history of MFS, presented to our cardiovascular department because of progressive exertional chest distress, fatigue and occasional precordial pain. Physical examinations revealed 190.5 cm of height, high myopia, and a diastolic murmur at the aortic valve area. Laboratory examinations for systemic vasculitis and infectious diseases were negative. Transthoracic echocardiography and enhanced thoracic computed tomography (CT) scan revealed the existence of a giant aortic root aneurysm (125.1 mm in short-axis), severe aortic valve regurgitation, cardiac dilatation (LV; 99 mm in diastolic diameter) and a poor ejection fraction (EF; 18%). Considering the risk of rupture or dissection of the dilated aortic root, we performed Bentall procedure based on the results of multidisciplinary team discussion and intraoperative exploration. Postoperative thoracic CT scan revealed a normal sized reconstructed aortic root, and the patient was discharged uneventfully 7 days later. Conclusion It is extremely rare to report such a giant aortic root aneurysm in a young patient. In the treatment decision-making process, the patient’s specific situation should be taken into consideration. A mechanical Bentall procedure seems to be an acceptable option for some selected cases.

2020 ◽  
Author(s):  
JIAYU SHEN ◽  
Changping Gan ◽  
R.D.T. Rajaguru ◽  
Dou Yuan ◽  
ZHENGHUA XIAO

Abstract IntroductionMarfan syndrome (MFS) is a common heritable connective tissue disease involving multiple organs. Even though the clinical manifestations of MFS can be various, aortic root aneurysm is estimated as one of the most serious complications. We herein describe an individualized treatment decision-making process for a 23-year-old male with MFS, suffering from a giant but stable aortic root aneurysm which is extremely rare at his age.CaseThe patient, a 23-year-old male with a family history of MFS, presented to our cardiovascular department because of progressive exertional chest distress, fatigue and occasional precordial pain. Physical examinations revealed 190.5cm of height, high myopia, and a diastolic murmur at the aortic valve area. Laboratory examinations for systemic vasculitis and infectious diseases were negative. Transthoracic echocardiography and enhanced thoracic computed tomography (CT) scan revealed the existence of a giant aortic root aneurysm (125.1 mm in short-axis), severe aortic valve regurgitation, cardiac dilatation (LV; 99 mm in diastolic diameter) and a poor ejection fraction (EF; 18%). Considering the risk of rupture or dissection of the dilated aortic root, we performed Bentall procedure based on the results of multidisciplinary team discussion and intraoperative exploration. Postoperative thoracic CT scan revealed a normal sized reconstructed aortic root, and the patient was discharged uneventfully 7 days later.ConclusionIt is extremely rare to report such a giant aortic root aneurysm in a young patient. In the treatment decision-making process, the patient’s specific situation should be taken into consideration. A mechanical Bentall procedure seems to be an acceptable option for some selected cases.


2020 ◽  
Author(s):  
JIAYU SHEN ◽  
Changping Gan ◽  
R.D.T. Rajaguru ◽  
Dou Yuan ◽  
ZHENGHUA XIAO

Abstract Introduction: Marfan syndrome (MFS) is a common heritable connective tissue disease involving multiple organs. Even though the clinical manifestations of MFS can be various, aortic root aneurysm is estimated as one of the most serious complications. We herein describe an individualized treatment decision-making process for a 23-year-old male with MFS, suffering from a giant but stable aortic root aneurysm which is extremely rare at his age. Case: The patient, a 23-year-old male with a family history of MFS, presented to our cardiovascular department because of progressive exertional chest distress, fatigue and occasional precordial pain. Physical examinations revealed six-foot-three inches of height, high myopia, and a diastolic murmur at the aortic valve area. Laboratory examinations for systemic vasculitis and infectious diseases were negative. The transthoracic echocardiography (TTE) and enhanced thoracic computed tomography (CT) scan revealed the existence of a giant aortic root aneurysm (125.1 mm in short-axis), severe aortic valve regurgitation, cardiac dilatation (LV; 99 mm in diastolic diameter) and a poor ejection fraction (EF; 18%). Considering the risk of rupture or dissection of the dilated aortic root, we successfully performed the Bentall procedure based on the intraoperative exploration results. Postoperative thoracic CT scan revealed a normal sized reconstructed aortic root, and the patient was discharged uneventfully 7 days later. Conclusion It is extremely rare to report such a giant aortic root aneurysm in a young patient. In the treatment decision-making process, the patient’s specific situation should be taken into consideration. The composite replacement of the aortic valve and ascending aorta should be performed if the patient is not suitable for valve-sparing operation.


2017 ◽  
Vol 63 (10) ◽  
pp. 640-645
Author(s):  
Jiří Ničovský ◽  
Jiří Ondrášek ◽  
Jan Černý ◽  
Daniela Žáková ◽  
Petr Němec

2020 ◽  
pp. 096973302094575
Author(s):  
Ni Gong ◽  
Qianqian Du ◽  
Hongyu Lou ◽  
Yiheng Zhang ◽  
Hengying Fang ◽  
...  

Background: Independent decision-making is one of the basic rights of patients. However, in clinical practice, most older cancer patients’ treatment decisions are made by family members. Objective: This study attempted to analyze the treatment decision-making process and formation mechanism for older cancer patients within the special cultural context of Chinese medical practice. Method: A qualitative study was conducted. With the sample saturation principle, data collected by in-depth interviews with 17 family members and 12 patients were subjected to thematic analysis. Ethical considerations: The study was approved by the ethics committees of Sun Yat-sen University. All participants provided verbal informed consent after being told their rights of confidentiality, anonymity, and voluntary participation. They had the right to refuse to answer questions and could withdraw at any time. Results: Three themes emerged: (1) complex process; (2) transformation of family decision-making power; and (3) individual compromise. Family members inevitably had different opinions during the long process of treatment decision-making for older cancer patients. The direction of this process could be regarded as an extension of the family power relationship. The patient usually compromised the decision to survive, which was made by family members. Conclusion: This study describes the treatment decision-making process of older cancer patients in the context of Chinese culture. The reasons underlying this process are related to the views on life and death and family values. An individual is a part of the family, which is often seen as the minimal interpersonal unit in Chinese society. It is significant that while emphasizing patient autonomy in the decision-making process, health professionals should also pay attention to the important roles of culture and family.


Psychotherapy ◽  
2016 ◽  
Vol 53 (3) ◽  
pp. 257-261 ◽  
Author(s):  
Jake Park ◽  
Jonathan Goode ◽  
Kelley A. Tompkins ◽  
Joshua K. Swift

2019 ◽  
Vol 13 (1) ◽  
pp. 61
Author(s):  
Sindi Paratika

Dupak primary health care is one of health care which have complete HIV and Acquired Immuno Deficiency Syndrom (AIDS) prevention program. The number of HIV-AIDS sufferers increasing every year, but the number of new HIV positive cases taking Anti Retroviral Virus(ARV) treatment at Dupak primary health care are low. The purpose of this research was analyzed the HIV treatment decision making process of patients at Dupak primary health care. The method in this research was descriptive quantitive research through questionnaires. Variables of this study are individual characteristics include age, level of education, knowledges, status of severity with decision making process of treatment. The results of this study indicate that most respondents (50%) have a high knowledge of HIV treatment. Decision-making process of HIV positive patient treatment have 2 stages that most respondents didn’t do. There are stage of the information search (75%) and stage of alternative evaluation (56.25%). The conclusion of this research are most respondents didn’t do information searching and alternative evaluation in decision making process at Dupak primary health care. According suggested from this study are to improve HIV related socialization programs through promotional activities and discussions related to HIV treatment services to improve knowledge and activeness of seeking information through print and electronic media.


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