scholarly journals Ventricular Septal Defect in Children with Down Syndrome - Two Case Reports

1970 ◽  
Vol 3 (1) ◽  
pp. 107-109
Author(s):  
MK Hassan ◽  
KA Hasan ◽  
SA Quader ◽  
R Sarker ◽  
F Ahmed ◽  
...  

The association of Down syndrome (DS) with congenital cardiovascular malformation is well established. Complete atrioventricular septal defects have been associated most commonly with DS. There are also reports of VSD, ASD, TOF and PDA with DS. We here reported two patients of Down syndrome with ventricular septal defect (VSD), underwent repair of VSD, diagnosis was suggested by echocardiography and confirmed by surgery and chromosomal study. Both the patient discharged from hospital with good result. Survival and quality of life have been improving in patient with Down syndrome after repairing VSD. Key words: Down syndrome; Ventricular septal defect. DOI: 10.3329/cardio.v3i1.6438Cardiovasc. j. 2010; 3(1): 107-109

2015 ◽  
Vol 42 (2) ◽  
pp. 166-168 ◽  
Author(s):  
Firat H. Altin ◽  
Okan Yildiz ◽  
Mehmet Karacalilar ◽  
Oyku Tosun ◽  
Ozgen Ilgaz Kocyigit ◽  
...  

Atrioventricular septal defects constitute 4% of all congenital cardiac malformations. Patients with complete atrioventricular septal defect rarely survive for decades without surgical treatment. Pulmonary stenosis can provide a delicate balance between the pulmonary and systemic circulations and thereby increase longevity. We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. We discuss her successful surgical treatment in terms of the available medical literature.


1989 ◽  
Vol 11 (4) ◽  
pp. 99-100
Author(s):  
Edward B. Clark

Editor's note: In response to a reader's question about cardiac evaluation of patients with Down syndrome, we asked Dr Clark for a review and recommendation for workup by the general pediatrician. RJH Infants with Down syndrome are at 50% risk for congenital cardiovascular malformations1 compared with a 0.3% (3 of 1000) risk for infants with normal chromosomes.2 These data confirm the original estimates of Rowe and Uchida3; other estimates have ranged from 20% to 70%. The types of heart defects found in persons with Down syndrome are severe: complete atrioventricular canal, ventricular septal defect, partial atrioventricular canal, atrial septal defect, tetralogy of Fallot, and patent ductus arteriosus.4


2019 ◽  
Vol 8 (2) ◽  
pp. 444
Author(s):  
Bun Yurizali ◽  
AM Hanif

Atrioventricular septal defect (AVSD) adalah kelainan berupa defek pada septum atrioventrikular (AV) di atas atau bawah katup AV, disertai kelainan katup AV; terjadi akibat pertumbuhan yang abnormal dari endokardial cushion pada masa janin. AVSD mewakili 4% sampai 5% bawaan cacat jantung. Dilaporkan seorang pasien laki–laki 20 tahun dengan keluhan sesak nafas saat beraktivitas dan berkurang dengan istirahat, disertai bibir dan kuku jari yang membiru, sakit kepala hilang timbul, muka kemerahan, demam, lemah letih lesu dan dada rasa berdebar-debar. Adanya sianosis, kulit kemerahan, Konjungtiva hiperemis, peningkatan JVP, hepatojugular refluks, bentuk dada abnormal. Bunyi jantung reguler, terdengar bising sistolik di RIC VI, blowing, grade 4/6, punctum maximum di apeks, penjalaran ke Axilla. Bising sistolik di RIC V linea strenalis dektra, grade 4/6, blowing punctum maksimun di RIC V linea sternalis dektra. Pada pemeriksaan laboratorium didapatkan polisitemia. Pada rontgent dada terdapat kardiomegali, dari ekokardiografi adanya CAVSD, hipertensi pulmonal Moderate-severe, Left arch, fungsi RV yang menurun. Pada pasien dilakukan prosedur flebotomi untuk mengurangi kepekatan darah. Pengobatan hipertensi pulmonal pada pasien ini adalah dengan pemberian diuretik dan dorner.


2019 ◽  
Vol 8 (2) ◽  
pp. 444
Author(s):  
Bun Yurizali ◽  
AM Hanif

Atrioventricular septal defect (AVSD) adalah kelainan berupa defek pada septum atrioventrikular (AV) di atas atau bawah katup AV, disertai kelainan katup AV; terjadi akibat pertumbuhan yang abnormal dari endokardial cushion pada masa janin. AVSD mewakili 4% sampai 5% bawaan cacat jantung. Dilaporkan seorang pasien laki–laki 20 tahun dengan keluhan sesak nafas saat beraktivitas dan berkurang dengan istirahat, disertai bibir dan kuku jari yang membiru, sakit kepala hilang timbul, muka kemerahan, demam, lemah letih lesu dan dada rasa berdebar-debar. Adanya sianosis, kulit kemerahan, Konjungtiva hiperemis, peningkatan JVP, hepatojugular refluks, bentuk dada abnormal. Bunyi jantung reguler, terdengar bising sistolik di RIC VI, blowing, grade 4/6, punctum maximum di apeks, penjalaran ke Axilla. Bising sistolik di RIC V linea strenalis dektra, grade 4/6, blowing punctum maksimun di RIC V linea sternalis dektra. Pada pemeriksaan laboratorium didapatkan polisitemia. Pada rontgent dada terdapat kardiomegali, dari ekokardiografi adanya CAVSD, hipertensi pulmonal Moderate-severe, Left arch, fungsi RV yang menurun. Pada pasien dilakukan prosedur flebotomi untuk mengurangi kepekatan darah. Pengobatan hipertensi pulmonal pada pasien ini adalah dengan pemberian diuretik dan dorner.


2020 ◽  
pp. 1-6
Author(s):  
Hirohito Doi ◽  
Jun Muneuchi ◽  
Mamie Watanabe ◽  
Yuichiro Sugitani ◽  
Ryohei Matsuoka ◽  
...  

Abstract Objective: Infants with complete atrioventricular septal defect occasionally accompany pulmonary hypertension; however, the pulmonary circulation can be altered by pulmonary vascular conditions as well as the left heart lesions. This study aimed to explore whether the left heart lesions were related to the pulmonary circulation among them. Methods: We performed echocardiography and cardiac catheterisation in 42 infants with complete atrioventricular septal defect and studied relationships between the pulmonary haemodynamic parameters and the left heart morphology. Results: Age and weight at preoperative evaluation were 65 days (47-114) (the median following interquartile range) and 5.5 kg (4.0-7.1), respectively. There were 27 individuals with Down syndrome. Gestational age was 38 weeks (37-39). Catheterisation showed mean pulmonary arterial pressure: 36 (29-46) mmHg, the ratio of pulmonary to systemic blood flow: 3.45 (2.79-4.98), pulmonary vascular resistance: 2.20 Wood units·m2 (1.53-3.65), and pulmonary arterial compliance: 2.78 (1.86-4.10) ml/Hg/m2. Echocardiography showed the Rastelli classification type A in 28 and type C in 14, moderate or severe left atrioventricular valve regurgitation in 19 patients (45%), atrioventricular valve index of 0.67 (0.56-0.79), left ventricular end-diastolic volume z score of 4.46 (1.96-7.78), and aortic valve diameter z score of −0.70 (−1.91 to 0.20). Multivariable regression analysis revealed that preoperative pulmonary vascular resistance was significantly correlated to gestational age (p = 0.002), and that preoperative pulmonary arterial compliance was significantly correlated to gestational age (p = 0.009) and Down syndrome (p = 0.036). Conclusions: The pulmonary circulation does not depend upon the presence of left heart lesions but gestational age and Down syndrome in infants with complete atrioventricular septal defect.


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