scholarly journals Clinical presentation and surgical results in a patient who has multiple congenital heart defects

1997 ◽  
Vol 18 (7) ◽  
pp. 1191-1193
Author(s):  
R. ZEYBEK ◽  
E. AKSOY
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Clinical Presentation ◽  
Heart Defects ◽  
Surgical Results

scholarly journals Williams-Beuren Syndrome:A Rare Case from Western India

2016 ◽  
Vol 1 (1) ◽  
pp. 1
Author(s):  
Pankaj K. Gadhia ◽  
Salil N. Vaniawala
Keyword(s):  
Williams Syndrome ◽  
Congenital Heart Defects ◽  
Rare Case ◽  
Congenital Heart ◽  
Clinical Presentation ◽  
Heart Defects ◽  
Genetic Disorder ◽  
Western India ◽  
Renal Anomalies ◽  
Live Births

Williams-Beuren Syndrome (WBS) also known as Williams Syndrome (WS) is a rare multisystem genetic disorder having incidence of 1 in 20,000 to 50,000 live births. WS caused by deletion of 26 - 28 contiguous genes including elastin (ELN) on chromosome 7q11.23. It is characterized by congenital heart defects, skeletal and renal anomalies. We report herein two rare cases of WS (One male and one female) from Western India varying clinical presentation. The confirmation was carried out by cytogenetic analysis and FISH test.

Clinical Profile and Etiological Agents Involved in Pediatric Infective Endocarditis

2021 ◽  
Vol 15 (10) ◽  
pp. 2787-1089
Author(s):  
Muhammad Imran ◽  
Muhammad Naveed ◽  
Fazal ur Rehman ◽  
Khurram Shahnawaz ◽  
Farhan Zahoor ◽  
...  
Keyword(s):  
Infective Endocarditis ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Clinical Presentation ◽  
Heart Defects ◽  
Underlying Disease ◽  
Etiological Agent ◽  
Coagulase Negative Staphylococcus ◽  
Shortness Of Breath ◽  
Etiological Agents

Objective: To find out clinical profile and etiological agents involved in Pediatric infective endocarditis. Study Design: A descriptive observational study. Place and Duration of the Study: The Department of Pediatrics, Sughra Shafi Medical Complex, Sahara Medical College, Narowal, Pakistan from April 2017 to March 2021. Material and Methods: A total of 195 children of both genders aged below 14 years and having endocarditis were enrolled. Endocarditis was labeled as per modified Duke’s criteria. Data was entered and analyzed using SPSS version 26.0. Quantitative data like age was represented as mead and standard deviation. Qualitative data like gender, area of residence, clinical presentation, types of isolated microorganisms and underlying disease were shown as frequencies and percentages. Results: Out of a total of 195 children, 124 (63.6%) were male. Overall, mean age was noted to be 5.48+2.1 years. Fever was the commonest type of clinical presentation noted among 148 (75.9%) children followed by shortness of breath 101 (51.8%) and chest pain/discomfort 48 (24.6%). Congenital heart defects were the commonest form of underlying disease observed in 95 (48.7%) children. Blood culture findings revealed 112 (57.4%) samples to produce no growth while coagulase negative staphylococcus was the commonest etiological agent noted in 37 (19.0%) children. Conclusion: Majority of the children with infective endocarditis were male. Most common types of clinical presentation were fever and shortness of breath. Congenital heart defects formed major chunk of underlying disease. Coagulase negative staphylococcus was found to be the most common etiological agent. Keywords: Infective endocarditis, fever, shortness of breath, congenital heart defects.

Minimally invasive partial inferior sternotomy for congenital heart defects in children

2006 ◽  
Vol 54 (S 1) ◽  
Author(s):  
R Seipelt ◽  
T Tirilomis ◽  
T Paul ◽  
H Dörge ◽  
F Schoendube ◽  
...  
Keyword(s):  
Minimally Invasive ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects

Surgical management of congenital heart defects in adolescent and adult patients, between years 2001–2008

2009 ◽  
Vol 150 (37) ◽  
pp. 1739-1743 ◽  
Author(s):  
István Hartyánszky ◽  
Andrea Székely ◽  
László Király ◽  
Zsolt Prodán ◽  
Sándor Mihályi ◽  
...  
Keyword(s):  
Surgical Management ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Adult Patients

A felnőttkorban operált veleszületett szívhibák között vezetnek az I. rekonstrukciós beavatkozások: a) frissen felismert betegségek, b) megelőzően inoperábilisnak ítélt kórképek, c) pulmonalis hypertonia, jobbkamra-elégtelenség miatt „elkésett” műtétek. Növekszik a II. REDO műtétek száma: a) residuumok korrigálása, b) kinőtt, diszfunkciós homograftok cseréje, c) műtéti/intervenciós korrigálás utáni recoarctatio (aneurysma, dissectio) sebészete, d) aorta valvulotomia/valvuloplastica, illetve társvitiumok (TGA) korrigálásának következményeként Ross-műtét, műbillentyű-beültetés . Betegek, eredmények: A 2001–2008 között végzett 4496 műtét közül 166 volt fiatal-felnőtt korú (16–52, átlagéletkor: 28 év) (Ia: 77, Ib: 15, Ic: 4, IIa: 11, IIb: 22, IIc: 9, IId: 28). Műtéti mortalitás nem volt, 1 beteg pulmonalis hypertoniás krízisben, 1 jobbkamra-elégtelenség miatti malignus ritmuszavarban, 2 többszerv-elégtelenségben halt meg. Konklúzió: A rizikófaktorokat a pulmonalis hypertonia és a jobbkamra-elégtelenség jelenti. A bonyolult sebészi megoldások a „congenitalis szívsebész” számára nem jelentenek problémát, de koraibb diagnózisok, terápiában az extracorporalis membránoxigenátor használata az eredményeket javíthatja.

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