Surgical management of complete atrioventricular septal defect with abnormal right ventricular outflow tract

1997 ◽  
Vol 6 (1) ◽  
pp. 30
Author(s):  
Tim Cartmill ◽  
Hitoshi Yaku ◽  
Graham Nunn ◽  
Richard Chard ◽  
Richard Hawker ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Surgical Management ◽  
Septal Defect ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Atrioventricular Septal Defect ◽  
Outflow Tract ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Atrioventricular

Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot revisited: making a case for primary repair in infancy

1998 ◽  
Vol 8 (4) ◽  
pp. 455-461 ◽  
Author(s):  
Doff B. McElhinney ◽  
V. Mohan Reddy ◽  
Norman H. Silverman ◽  
Michael M. Brook ◽  
Frank L. Hanley
Keyword(s):  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Primary Repair ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Atrioventricular Septal Defect ◽  
Outflow Tract ◽  
Complete Repair ◽  
The Right

AbstractAtrioventricular septal defect with common valvar orifice and tetralogy of Fallot is a rare combination of congenital cardiac anomalies. Approaches to this lesion have tended to emphasize either staged repair or complete repair beyond infancy. Between July 1992 and August 1997, nine patients underwent repair of complete atrioventricular septal defect with tetralogy of Fallot. One patient, aged 9.6 years at the time of repair, had previously undergone construction of a modified Blalock-Taussig shunt. Primary complete repair was performed in the other 8 patients at ages ranging from 2.5 to 16 months (median 4.6 months), and all but one were infants. All patients had a Rastelli type C defect, a single ventricular septal defect with inlet and outlet components, and malalignment of the muscular outlet septum with subpul-monary stenosis. A single patch technique, with closure of the zone of apposition (‘cleft’) in the left atrioventricular valve, was used in all eight patients undergoing primary repair, while a double patch was employed in the previously palliated older patient. In all cases of repair using a single patch, the anterosu-perior bridging leaflet was divided obliquely to the right, following the malaligned outlet septum, in order to avoid subaortic obstruction. Repair of the right ventricular outflow tract included infundibular myectomy in eight, pulmonary valvotomy in four, infundibular or transannular patching in three and one, respectively, and reconstruction with a valved allograft conduit in two patients. There was no early mortality or significant morbidity. At a median follow-up of 45 months, there had been one death related to non-cardiac causes and no reinterventions. Left atrioventricular valvar regurgitation was moderate or mild in two patients, and right atrioventricular valvar regurgitation was mild in one patient. No patient had more than mild pulmonary regurgitation or a gradient across the right ventricular outflow tract in excess of 18 mm Hg. Our results demonstrate that primary repair of atrioventricular septal defect with tetralogy of Fallot can be performed with excellent early and mid-term results in young infants. Although it has been suggested that a technique utilizing oblique division of the anterosuperior bridging leaflet may lead to high rates of atrioventricular valvar regurgitation, medium-term atrioventricular valvar function in the present cohort of patients has been excellent.

scholarly journals Unruptured Sinus of Valsalva Aneurysm with Right Ventricular Outflow Tract Obstruction and Supracristal Ventricular Septal Defect: A Rare Case

2015 ◽  
Vol 42 (5) ◽  
pp. 462-464
Author(s):  
Ganiga Srinivasaiah Sridhar ◽  
Muhammad Athar Sadiq ◽  
Wan Azman Wan Ahmad ◽  
Chitra Supuramaniam ◽  
Timothy Watson ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricular ◽  
Septal Defect ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Sinus Of Valsalva ◽  
Exertional Dyspnea ◽  
Sinus Of Valsalva Aneurysm ◽  
Ventricular Outflow Tract Obstruction

Unruptured right sinus of Valsalva aneurysm that causes severe obstruction of the right ventricular outflow tract is extremely rare. We describe the case of a 47-year-old woman who presented with exertional dyspnea. Upon investigation, we discovered an unruptured right sinus of Valsalva aneurysm with associated right ventricular outflow tract obstruction and a supracristal ventricular septal defect. To our knowledge, only 2 such cases have previously been reported in the medical literature. Although treatment of unruptured sinus of Valsalva aneurysm remains debatable, surgery should be considered for extremely large aneurysms or for progressive enlargement of the aneurysm on serial evaluation. Surgery was undertaken in our patient because there was clear evidence of right ventricular outflow tract obstruction, right-sided heart dilation, and associated exertional dyspnea.

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

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