scholarly journals A Single Misstep in Cardiac Development Explains the Co-Occurrence of Tetralogy of Fallot and Complete Atrioventricular Septal Defect in Down Syndrome

2014 ◽  
Vol 165 (1) ◽  
pp. 194-196 ◽  
Author(s):  
Hoang H. Nguyen ◽  
Patrick Y. Jay
Keyword(s):  
Down Syndrome ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Cardiac Development ◽  
Atrioventricular Septal Defect ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Atrioventricular

Characteristics of the pulmonary circulation in infants with complete atrioventricular septal defect

2020 ◽  
pp. 1-6
Author(s):  
Hirohito Doi ◽  
Jun Muneuchi ◽  
Mamie Watanabe ◽  
Yuichiro Sugitani ◽  
Ryohei Matsuoka ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Gestational Age ◽  
Pulmonary Circulation ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Left Heart ◽  
Complete Atrioventricular Septal Defect ◽  
Pulmonary Arterial ◽  
Heart Lesions ◽  
Complete Atrioventricular

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.

Surgical management of complete atrioventricular septal defect with tetralogy of Fallot: results from a national audit database

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
D Dorobantu ◽  
R Tulloh ◽  
S Stoica
Keyword(s):  
Tetralogy Of Fallot ◽  
Surgical Management ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
National Audit ◽  
Treatment Pathways ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Repair ◽  
Complete Atrioventricular

Abstract Background Complete atrioventricular septal defect (CAVSD) with tetralogy of Fallot (CAVSD-ToF) is an uncommon association, and the management is more difficult than for each lesion in isolation. Depending on the anatomy, clinical status and ventricular morphology, definitive repair is offered either as staged or single operation with few undergoing single or one and a half ventricle procedures. Currently available data are limited to single centre studies. Purpose We aim to use data from a national audit dataset to describe outcomes in the surgical management of CAVSD-ToF with data from the national audit. Methods All children with CAVSD and tetralogy of Fallot (including double outlet right ventricle and pulmonary atresia variants) undergoing the first cardiac procedure between 2000–2013 were included and all subsequent procedures extracted. Three treatment pathways were defined: definitive primary procedure (“primary complete repair”), staged repair or “unbalanced ventricles repair” (Glenn shunt or modified Fontan). Continuous data is expressed as median (range). Survival data obtained using the Kaplan Meier method and subgroup comparisons done using the log-rank test. Results A total of 125 patients were included, 50.4% male, first procedure at 150 (1–2770) days. Down syndrome was present in 79.4% (63/79 of those with reported non-cardiac comorbidities), while 8% (10/125) had heterotaxy syndrome. “Primary complete repair” was performed in 40% (n=50), at 319 (33–2770) days. Staged repair was undertaken in 28% (n=35), first palliation at 80 (1–733) days. An “unbalanced ventricles” approach was used in 16.8% (n=21), first procedure at 135 (1–1742) days). The other 15.2% (n=19), first procedure at 53 (range 1–436) days, had at least one palliation procedure, but no identifiable committed pathway. Survival at 5 years from the first procedure in “primary complete repair” compared to the remainder of the group is shown in the Figure. No significant differences in survival were found. Specifically in the “unbalanced ventricle repair” group, just 1 patient died during follow-up. A total of 85 patients (68%) achieved either primary or staged “complete repair”. Early mortality (30 day) after the “complete repair” was 6.6% (similar for both approaches, p=0.9). Survival at 5 years after “complete repair” was 82.7% (similar for both approaches, p=0.8). Freedom from any cardiac re-intervention at 5 years was 69.5% (77.6% after primary, 58.1% after staged repair, p=0.03). Conclusion Early-life mortality in this heterogeneous group of CAVSD-ToF remains high, even in patients receiving definitive “complete repair”. We found no survival differences in the medium term between primary repair and a palliation approach, but more cardiac re-interventions were undertaken after staged complete repair. Surprisingly good results were seen in the unbalanced ventricles group, but longer follow up is needed to examine their attrition and longer term outcome. Survival at 5 years by treatment option Funding Acknowledgement Type of funding source: None

Complete atrioventricular septal defect, Down syndrome, and surgical outcome: risk factors

2003 ◽  
Vol 75 (2) ◽  
pp. 412-421 ◽  
Author(s):  
Amira A Al-Hay ◽  
Stephanie J MacNeill ◽  
Magdi Yacoub ◽  
Darryl F Shore ◽  
Elliot A Shinebourne
Keyword(s):  
Risk Factors ◽  
Down Syndrome ◽  
Surgical Outcome ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Atrioventricular

Repair of Complete Atrioventricular Septal Defect with Tetralogy of Fallot:

2004 ◽  
Vol 19 (2) ◽  
pp. 175-183 ◽  
Author(s):  
Edvin Prifti ◽  
Massimo Bonacchi ◽  
Massimo Bernabei ◽  
Marzia Leacche ◽  
Fabio Bartolozzi ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Atrioventricular
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