Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV

2018 ◽  
Vol 11 (4) ◽  
pp. NP182-NP185
Author(s):  
Raghav Murthy ◽  
Nicole L. Herrick ◽  
Howaida El-Said ◽  
Paul Grossfeld ◽  
John Moore ◽  
...  
Keyword(s):  
Arterial Switch Operation ◽  
Ductus Arteriosus ◽  
Rare Condition ◽  
Ehlers Danlos Syndrome ◽  
Arterial Switch ◽  
Type Iv ◽  
Vascular Type ◽  
Switch Operation ◽  
Long Term Follow Up ◽  
Danlos Syndrome

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.

Early and Late Outcomes of Cardiovascular Surgery in Patients With Ehlers-Danlos Syndrome

2021 ◽  
Vol 12 (6) ◽  
pp. 773-777
Author(s):  
Mohamed F. Elsisy ◽  
Alberto Pochettino ◽  
Joseph A. Dearani ◽  
Thomas C. Bower ◽  
Robert D. McBane ◽  
...  
Keyword(s):  
Surgical Outcomes ◽  
Time Frame ◽  
Ehlers Danlos Syndrome ◽  
Vital Status ◽  
Type Iv ◽  
Vascular Type ◽  
Baseline Characteristics ◽  
Danlos Syndrome ◽  
Adult Cardiac Surgery

Background Cardiovascular surgical outcomes reports are few for vascular type IV of Ehlers- Danlos Syndrome (vEDS) compared to non-vascular types I-III (nEDS). Methods To define cardiovascular surgical outcomes among adult patients (≥18 years) with EDS types, a review of our institution's in-house STS Adult Cardiac Surgery Database-compliant software and electronic medical records from Mayo Clinic (1993–2019) was performed. Outcomes were compared for vEDS patients and nEDS patients. Demographics, baseline characteristics, operative, in-hospital complications and follow-up vital status were analyzed. Results Over the study time frame, 48 EDS patients underwent surgery (mean age 52.6 ± 14.6 years; 48% females). Of these, 17 patients had vEDS and 31 patients had nEDS. Six patients (12.5%) underwent prior sternotomy. Urgent or emergent surgery was performed in 10 patients (20.8%). Aortic (vEDS 76.5% vs. nEDS 16.1%) and mitral procedures (vEDS 11.8% vs. nEDS 48.4%) were the two most common cardiovascular surgeries performed (p < .01 and p = .007, respectively). Cardiopulmonary bypass time (CPB) (165 ± 18 vs. 90 ± 13 min; p = .015) and aortic cross clamp times (140 ± 14 vs. 62 ± 10 min; p < .001) were longer for vEDS patients. There was 1 (2.1%) early and 7 (14.6%) late deaths; 6 among vEDS and 2 among nEDS patients. Survival at 5 (80% vs. 93%), 10 (45% vs. 84%) and 15 years (45% vs. 84%) was lower in patients with vEDS (p = .015 for each comparison). Conclusion Cardiovascular surgeries are significantly more complex with longer bypass and cross clamp times for type IV vEDS compared to nEDS patients. Reduced overall survival underscores the complexity and fragility of vEDS patients.

Clinical and Genetic Features of Ehlers–Danlos Syndrome Type IV, the Vascular Type

2000 ◽  
Vol 342 (10) ◽  
pp. 673-680 ◽  
Author(s):  
Melanie Pepin ◽  
Ulrike Schwarze ◽  
Andrea Superti-Furga ◽  
Peter H. Byers
Keyword(s):  
Syndrome Type ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Vascular Type ◽  
Genetic Features ◽  
Danlos Syndrome

Acute brain ischemia as a complication of the Ehlers–Danlos syndrome, the case series

Vascular ◽  
2013 ◽  
Vol 22 (5) ◽  
pp. 341-345 ◽  
Author(s):  
Michal Pajak ◽  
Marcin A Majos ◽  
Wojciech Szubert ◽  
Ludomir Stefanczyk ◽  
Agata Majos
Keyword(s):  
Brain Ischemia ◽  
Case Series ◽  
Vascular Complications ◽  
Limb Ischemia ◽  
Radiology Department ◽  
Artery Dissection ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Vascular Type ◽  
Danlos Syndrome

Vascular type of Ehlers–Danlos syndrome involves many severe complications leading not only to organ-specific symptoms but often ends in a sudden death. The aim of this paper was to present a diagnostic possibilities and its efficiency rate in patients with vascular complications of Ehlers–Danlos syndrome who suffered from artery dissection resulting in acute brain or limb ischemia. We analysed three patients with diagnosed Ehlers–Danlos syndrome who were referred to radiology department for diagnostic imaging of affected vascular beds, each experienced brain ischemia. The paper also aims at offering some general recommendations for patients suffering from possible complications of type IV Ehlers–Danlos syndrome basing on our own experience and available literature data.

scholarly journals Manajemen Perioperatif Operasi Arterial Switch pada Transposition of The Great Arteries with Intact Ventricular Septum

2020 ◽  
Vol 12 (2) ◽  
pp. 11-22
Author(s):  
Dian Kesumarini ◽  
Herdono Poernomo
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Myocardial Protection ◽  
Septal Defect ◽  
Arterial Switch Operation ◽  
Ductus Arteriosus ◽  
Intact Ventricular Septum ◽  
Arterial Switch ◽  
Switch Operation ◽  
Patent Ductus

Latar belakang: Penyakit jantung bawaan (PJB) berkontribusi terhadap hampir sepertiga dari kelainan kongenital secara keseluruhan. Transposition of the great arteries (d-TGA) adalah satu kelainan jantung bawaan (PJB) yang kompleks. Tindakan arterial switch operation (ASO) menjadi pilihan koreksi pada kasus TGA. Tindakan ini mempunyai risiko morbiditas dan mortalitas yang cukup tinggi.Kasus: Bayi berusia 42 hari dengan berat badan 3100 gram dirujuk ke Rumah Sakit Jantung dan Pembuluh Darah (RSJPD) Harapan Kita karena kelainan jantung. Pasien dilakukan diagnosik ekokardiografi dan didapatkan TGA dengan septum ventrikular yang intak (TGA-IVS), atrium septal defect (ASD) sekundum L-R shunt, dan patent ductus arteriosus (PDA). Prosedur pembedahan meliputi ASO menggunakan manuver Le Compte, pemotongan PDA, ASD ditutup sebagian dan disisakan 3mm. Durasi cardiopulmonary bypass (CPB) 136 menit dengan cross clamp 85 menit, diberikan tranfusi PRC, FFP, dan TC, lalu dipindahkan ke intensive care unit (ICU) dengan support adrenalin 0.05 mcg/kg/menit dan milrinone 0.375 mcg/kg/menit. Ekstubasi dilakukan 72 jam pascaoperasi.Pembahasan: Operasi arterial switch merupakan tindakan berisiko tinggi, dengan angka kematian dan morbiditas yang tinggi. Konsiderasi perianestesia pada pasien TGA ini di antaranya tatalaksana preanestesi, manajemen selama operasi, topangan hemodinamik, aritmia yang diakibatkan masalah pembuluh darah koroner, dan penilaian ekokardiografi epikardial pascaoperasi. Manajemen pascaoperasi penting untuk mengantisipasi efek dari CPB yang berpengaruh pada miokardium, sindroma curah jantung rendah, risiko infeksi, dan komplikasi lain yang sering terjadi pada infant setelah pembedahan ini.Kesimpulan: Manajemen preoperatif dengan mengenali faktor risiko, tatalaksana anestesia intraoperatif, myocardial protection, serta perawatan komprehensif pascaoperasi di ICU sangat menentukan outcomepasien yang menjalani prosedur ini. 

scholarly journals Ehlers-Danlos Syndrome Type IV, Vascular Type, Which Demonstrated a Novel Point Mutation in the COL3A1 Gene

2010 ◽  
Vol 49 (16) ◽  
pp. 1797-1800 ◽  
Author(s):  
Rinako Sadakata ◽  
Atsushi Hatamochi ◽  
Keiji Kodama ◽  
Akiko Kaga ◽  
Takefumi Yamaguchi ◽  
...  
Keyword(s):  
Point Mutation ◽  
Syndrome Type ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Vascular Type ◽  
Col3a1 Gene ◽  
Danlos Syndrome

Clinical and Genetic Features of Ehlers-Danlos Syndrome Type IV, the Vascular Type

2000 ◽  
Vol 55 (8) ◽  
pp. 469-471 ◽  
Author(s):  
Melanie Pepin ◽  
Ulrike Schwarze ◽  
Andrea Superti-Furga ◽  
Peter H. Byers
Keyword(s):  
Syndrome Type ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Vascular Type ◽  
Genetic Features ◽  
Danlos Syndrome

scholarly journals Impact of pulmonary artery reduction during arterial switch operation: 14 years follow-up

2020 ◽  
Vol 30 (6) ◽  
pp. 917-924
Author(s):  
Fernanda Lübe Antunes Pereira ◽  
Cristiane Nunes Martins ◽  
Roberto Max Lopes ◽  
Matheus Ferber Drummond ◽  
Fernando Antonio Fantini ◽  
...  
Keyword(s):  
Arterial Switch Operation ◽  
Z Score ◽  
Valve Regurgitation ◽  
Arterial Switch ◽  
Switch Operation ◽  
Long Term Follow Up ◽  
Z Scores ◽  
The Mean

Abstract OBJECTIVES Pulmonary artery/aorta (PA/Ao) size discrepancy plays an important role in the development of neoaortic root growth and valve regurgitation. Since 2004, we started using PA reduction to manage severe great vessels root mismatch at the time of arterial switch operation. The purpose of this study is to evaluate the impact of this technique in the mid- and long-term follow-up. METHODS Patients considered to have severe PA/Ao mismatch (&gt;2:1 ratio) underwent resection of a 3- to 4-mm flap of the posterior PA wall. Patients submitted to this technique were followed up with clinical and image examinations. Echocardiographic findings were reviewed, and Z-scores were recorded to evaluate the incidence and progression of neoaortic root dilatation and valve regurgitation. RESULTS The median (Q1–Q3) follow-up time was 8 years (3–11). Before arterial switch operation, the median (Q1–Q3) Z-score of the PA annulus was 2.90 (2.75–3.75). At the latest follow-up, the median Z-score of the neoaortic annulus was 1.34 (0.95–1.66). The mean difference between the Z-scores of PA annulus and neoaortic annulus was 1.56 (P &lt; 0.0001). The mean value of the sinus of Valsalva was +0.29 ± 1, that of sinotubular junction was +0.71 ± 0.6 and that of ascending Ao was +1.09 ± 0.7. There was no severe dilatation of the neoaortic annulus, neoaortic root or ascending Ao during follow-up. Neoaortic valve regurgitation was none or mild in 93% of patients. CONCLUSIONS PA reduction proved to be a feasible and low-risk procedure to approach PA/Ao mismatch in arterial switch operation. Mid- and long-term follow-up showed a tendency towards stabilization of the neoaortic root dilatation and satisfactory valve performance after the procedure. Further investigation is required with a larger population and longer-term follow-up.

A Critical Analysis of the Perioperative Management of Patients with Ehlers Danlos Type IV (Vascular) Syndrome

2017 ◽  
Vol 27 (9) ◽  
pp. 196-199
Author(s):  
J Martin
Keyword(s):  
Perioperative Management ◽  
Emergency Situation ◽  
Joint Hypermobility ◽  
Connective Tissue Disorders ◽  
Care Needs ◽  
Ehlers Danlos Syndrome ◽  
Type Iv ◽  
Vascular Type ◽  
Theatre Staff ◽  
Danlos Syndrome

This paper discusses the perioperative care needs of patients with Ehlers Danlos type IV (vascular) syndrome. Ehlers Danlos syndrome (EDS) is a heritable group of connective tissue disorders characterised by varying degrees of tissue, blood vessel and internal organ fragility as well as skin and joint hypermobility (De Paepe & Malfait 2012). In 1997 EDS was revised and classified into six subtypes: classical (Types I and II), hypermobility, vascular (Type IV), kyphoscoliosis and arthrochalasia type. Each classification has been based on the following aspects: diagnostic uniformity, natural history, management, genetics, and the identification of potential areas for research (Beighton et al 1998). Vascular type EDS has serious implications for any form of surgical procedure and it is therefore imperative that theatre staff are fully conversant with the needs of this patient group. Overall awareness needs to be increased, particularly as most patients will most frequently be seen in an emergency situation, as elective procedures are avoided as far as possible.

Sign in / Sign up

Export Citation Format

Share Document