scholarly journals Concurrent End-Stage Cardiomyopathy and Aortic Disease in Patients with Marfan Syndrome: A Case Report and Literature Review

2021 ◽  
Author(s):  
Timothy Smith ◽  
Jose Sleiman ◽  
Nikita Zadneulitca ◽  
Cedric Sheffield ◽  
Viviana Navas ◽  
...  
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Literature Review ◽  
Marfan Syndrome ◽  
Aortic Disease ◽  
Connective Tissue Disorder ◽  
Orthotopic Heart Transplantation ◽  
Bentall Procedure ◽  
Life Saving ◽  
End Stage

Abstract Background: Marfan syndrome (MFS) is a connective tissue disorder that can lead to aortic disease, arrhythmias and heart failure. Many centers are reluctant to offer orthotopic heart transplantation (OHT) for patients with MFS with concurrent aortic disease due to complexity of the surgery and perceived inferior results when compared to patients without MFS. Methods: We present a case of a patient with MFS with previous Bentall procedure who underwent successful OHT, accompanied by a literature review on OHT performed for patients with MFS. Results and Conclusions: Patients with MFS who underwent OHT had no difference in mortality compared to patients without MFS. Even though OHT is technically more challenging when combined with concurrent intervention for aortic disease, it should be considered as a life-saving operation for patients with MFS.

Two-Stage Total Cardioaortic Replacement for End-Stage Heart and Aortic Disease in Marfan Syndrome: Case Report and Review of the Literature

2009 ◽  
Vol 28 (9) ◽  
pp. 958-963 ◽  
Author(s):  
Keshava Rajagopal ◽  
Joseph G. Rogers ◽  
Andrew J. Lodge ◽  
Jeffrey G. Gaca ◽  
Richard L. McCann ◽  
...  
Keyword(s):  
Case Report ◽  
Marfan Syndrome ◽  
Aortic Disease ◽  
Review Of The Literature ◽  
Two Stage ◽  
End Stage

Donors after circulatory death heart trial

2021 ◽  
Vol 17 (1) ◽  
pp. 11-17
Author(s):  
Yasuhiro Shudo ◽  
Rhodalene Benjamin-Addy ◽  
Tiffany K Koyano ◽  
William Hiesinger ◽  
John W MacArthur ◽  
...  
Keyword(s):  
Heart Failure ◽  
Gold Standard ◽  
Orthotopic Heart Transplantation ◽  
Donation After Circulatory Death ◽  
Clinical Trial Registration ◽  
Life Saving ◽  
End Stage Heart Failure ◽  
Standard Life ◽  
End Stage ◽  
Circulatory Death

Orthotopic heart transplantation is the gold standard treatment for end-stage heart failure. However, the persistent shortage of available donor organs has resulted in an ever-increasing waitlist and longer waiting periods for transplantation. On the contrary, increasing the number of heart transplants by preserving extended criteria donors and donation after circulatory death hearts with the Organ Care System™ (OCS) Heart System has the potential to provide the gold standard, life-saving treatment to patients with end-stage heart failure. The objective of the Donation After Circulatory Death Heart Trial is to evaluate the effectiveness of the OCS Heart System to preserve and assess hearts donated after circulatory death for transplantation to increase the pool of donor hearts available for transplantation, which can potentially provide patients with end-stage heart failure with the life-saving treatment. Clinical Trial Registration: NCT03831048 ( ClinicalTrials.gov )

Laparoscopic Bypass in Morbidly Obese Patient with End-Stage Heart Failure: Case Report

2021 ◽  
Author(s):  
Christian Lamar Scheibe ◽  
Luis Eduardo Veras Pinto ◽  
José Aparecido Valadão ◽  
Caio Márcio Barros de Oliveira ◽  
Ed Carlos Rey Moura ◽  
...  
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Obese Patient ◽  
Morbidly Obese ◽  
Morbidly Obese Patient ◽  
End Stage Heart Failure ◽  
End Stage ◽  
Heart Failure Case

scholarly journals A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

scholarly journals Marfan Syndrome with Atypical Presentation: A Case Report

2014 ◽  
Vol 4 (2) ◽  
pp. 111-114
Author(s):  
Sharmin Mahbuba ◽  
Fauzia Mohsin ◽  
Rubaiya Islam ◽  
Tahmina Begum
Keyword(s):  
Case Report ◽  
Marfan Syndrome ◽  
Autosomal Dominant ◽  
Connective Tissue Disorder ◽  
Atypical Presentation ◽  
Dominant Trait ◽  
Autosomal Dominant Trait ◽  
Excessive Sweating ◽  
Molecular Cytogenetic ◽  
Molecular Cytogenetic Techniques

Marfan syndrome is an inherited connective tissue disorder that is transmitted as an autosomal dominant trait. These cases can be diagnosed by molecular cytogenetic techniques. A modified Ghent criteria using systemic scoring system can also identify these cases in absence of molecular cytogenetic techniques.We report a case of a 6 year 5 month old boy who presented with the complaints of excessive sweating sinceinfancy and protrusion of both eye balls which was non progressive since early childhood. On examination, some skeletal features of Marfan syndrome was found and echocardiogram showed huge dilatation of root of aorta which helped in diagnosis by scoring system.Birdem Med J 2014; 4(2): 111-114

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