Noonan syndrome: An anesthesiologist′s perspective
Noonan syndrome (NS) is one of the most common non chromosomal syndrome presenting to the cardiac anesthesiologist for the management of various cardiac lesions, predominantly pulmonary stenosis (PS) (80%) and hypertrophic obstructive cardiomyopathy (HOCM) (30%). The presence o...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2011-01-01
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| Series: | Annals of Cardiac Anaesthesia |
| Subjects: | |
| Online Access: | http://www.annals.in/article.asp?issn=0971-9784;year=2011;volume=14;issue=3;spage=214;epage=217;aulast=Aggarwal |
| _version_ | 1818266958842298368 |
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| author | Aggarwal Vikram Malik Vishwas Malhotra Kapoor Poonam Kiran Usha |
| author_facet | Aggarwal Vikram Malik Vishwas Malhotra Kapoor Poonam Kiran Usha |
| author_sort | Aggarwal Vikram |
| collection | DOAJ |
| description | Noonan syndrome (NS) is one of the most common non chromosomal syndrome presenting to the cardiac anesthesiologist for the management of various cardiac lesions, predominantly pulmonary stenosis (PS) (80%) and hypertrophic obstructive cardiomyopathy (HOCM) (30%). The presence of HOCM in NS makes these children susceptible to acute congestive heart failure due to hemodynamic fluctuations, thus necessitating optimization of drug and fluid therapy, careful conduct of anesthesia and providing adequate analgesia in the perioperative period. We describe a case of four year old boy with NS who presented to us for the management of PS and HOCM. In our case, transesophageal echocardiography (TEE) played a major role in confirmation of the preoperative findings, detection of any new anomalies missed during the preoperative evaluation, intraoperative monitoring and assessment of the adequacy of repair in the immediate postoperative period. TEE provided invaluable help in taking critical surgical decisions, resulting in a favorable outcome. |
| first_indexed | 2024-12-12T20:14:58Z |
| format | Article |
| id | doaj.art-391c813223b44d74b0ee56ae5f13d8f2 |
| institution | Directory Open Access Journal |
| issn | 0971-9784 0974-5181 |
| language | English |
| last_indexed | 2024-12-12T20:14:58Z |
| publishDate | 2011-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Annals of Cardiac Anaesthesia |
| spelling | doaj.art-391c813223b44d74b0ee56ae5f13d8f22022-12-22T00:13:25ZengWolters Kluwer Medknow PublicationsAnnals of Cardiac Anaesthesia0971-97840974-51812011-01-01143214217Noonan syndrome: An anesthesiologist′s perspectiveAggarwal VikramMalik VishwasMalhotra Kapoor PoonamKiran UshaNoonan syndrome (NS) is one of the most common non chromosomal syndrome presenting to the cardiac anesthesiologist for the management of various cardiac lesions, predominantly pulmonary stenosis (PS) (80%) and hypertrophic obstructive cardiomyopathy (HOCM) (30%). The presence of HOCM in NS makes these children susceptible to acute congestive heart failure due to hemodynamic fluctuations, thus necessitating optimization of drug and fluid therapy, careful conduct of anesthesia and providing adequate analgesia in the perioperative period. We describe a case of four year old boy with NS who presented to us for the management of PS and HOCM. In our case, transesophageal echocardiography (TEE) played a major role in confirmation of the preoperative findings, detection of any new anomalies missed during the preoperative evaluation, intraoperative monitoring and assessment of the adequacy of repair in the immediate postoperative period. TEE provided invaluable help in taking critical surgical decisions, resulting in a favorable outcome.http://www.annals.in/article.asp?issn=0971-9784;year=2011;volume=14;issue=3;spage=214;epage=217;aulast=AggarwalHypertrophic obstructive cardiomyopathyNoonan syndromepulmonary stenosistransesophageal echocardiography |
| spellingShingle | Aggarwal Vikram Malik Vishwas Malhotra Kapoor Poonam Kiran Usha Noonan syndrome: An anesthesiologist′s perspective Annals of Cardiac Anaesthesia Hypertrophic obstructive cardiomyopathy Noonan syndrome pulmonary stenosis transesophageal echocardiography |
| title | Noonan syndrome: An anesthesiologist′s perspective |
| title_full | Noonan syndrome: An anesthesiologist′s perspective |
| title_fullStr | Noonan syndrome: An anesthesiologist′s perspective |
| title_full_unstemmed | Noonan syndrome: An anesthesiologist′s perspective |
| title_short | Noonan syndrome: An anesthesiologist′s perspective |
| title_sort | noonan syndrome an anesthesiologist x2032 s perspective |
| topic | Hypertrophic obstructive cardiomyopathy Noonan syndrome pulmonary stenosis transesophageal echocardiography |
| url | http://www.annals.in/article.asp?issn=0971-9784;year=2011;volume=14;issue=3;spage=214;epage=217;aulast=Aggarwal |
| work_keys_str_mv | AT aggarwalvikram noonansyndromeananesthesiologistx2032sperspective AT malikvishwas noonansyndromeananesthesiologistx2032sperspective AT malhotrakapoorpoonam noonansyndromeananesthesiologistx2032sperspective AT kiranusha noonansyndromeananesthesiologistx2032sperspective |