The outcome of biventricular (BV) repair for right-dominant unbalanced atrioventricular canal has remained poor, because it is difficult to predict left ventricular (LV) adequacy before surgery. Our aim was to determine whether preoperative echocardiographic parameters, specifically analysis of color inflow into the LV, would predict survival after BV repair in patients with right-dominant unbalanced atrioventricular canal. Subjects with right-dominant unbalanced atrioventricular canal diagnosed from 1994 to 2007 were included. The echocardiographic parameters were analyzed blinded to the palliation strategy and survival. The LV inflow index (LVII) was calculated as the secondary color inflow diameter indexed to the left atrioventricular valve (AVV) annulus diameter. Univariate analysis, survival analysis, and multivariate modeling with stepwise logistic regression were performed. Of the 45 subjects, 23 (51%) underwent single ventricle (SV) palliation and 22 (49%) underwent BV repair. Of the 23 who underwent SV palliation, 15 (65%) survived compared to 18 (82%) of 22 who underwent BV repair (p = 0.34). In the BV group, a greater LVII predicted survival (R2 = 0.46, p = 0.03). No subjects with a LVII <0.5 survived BV repair. Mortality in the BV group was associated with younger age at initial surgery (p <0.01) and abnormal left AVV morphology (p = 0.02). Of the BV subjects with a patent ductus arteriosus at the initial operation (n = 11), the nonsurvivors were more likely to have retrograde flow in the transverse arch (p <0.01). In the BV group, reoperation within 30 days of the initial repair was strongly associated with mortality (p <0.01). In conclusion, in cases of mild or moderate LV hypoplasia, a greater LVII predicted survival after BV repair in patients with right-dominant unbalanced atrioventricular canal. We propose incorporation of the LVII into the echocardiographic assessment of these patients.
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http://dx.doi.org/10.1016/j.amjcard.2010.08.052 | DOI Listing |
Ann Thorac Surg Short Rep
June 2024
Department of Cardiothoracic Surgery and Perfusion Services, The Heart Center, Nationwide Children's Hospital, Columbus, Ohio.
Background: Right ventricular (RV) failure after heart transplantation (HT) is common in those with pretransplantation elevated pulmonary vascular resistance (PVR). Mechanical circulatory support has been used as a bridge to recovery, with mixed outcomes. We describe a patient with failed single-ventricle palliation in whom severe RV failure developed after HT.
View Article and Find Full Text PDFInterdiscip Cardiovasc Thorac Surg
March 2024
Department of Cardiology, Children's Hospital Boston, Boston, MA, USA.
Objectives: Parachute left atrioventricular valve (LAVV) complicates atrioventricular septal defect (AVSD) repair. We evaluate outcomes of AVSD patients with parachute LAVV and identify risk factors for adverse outcomes.
Methods: We evaluated all patients undergoing repair of AVSD with parachute LAVV from 2012 to 2021.
Interdiscip Cardiovasc Thorac Surg
July 2023
Department of Cardiovascular Surgery, Kyushu University Hospital, Fukuoka, Japan.
We report the case of a 19-month-old girl with a right-dominant unbalanced atrioventricular septal defect and severe right-sided atrioventricular valve regurgitation who underwent biventricular repair using basal chordae resection, artificial chordae reconstruction and a left-sided atrioventricular valvuloplasty. At 14-month postoperative follow-up, the patient had minimal heart failure, gained weight and adapted to biventricular circulation.
View Article and Find Full Text PDFMultimed Man Cardiothorac Surg
October 2022
The patient is a 5-year-old girl who underwent a previous Kawashima procedure with a left-sided bidirectional cavopulmonary anastomosis as a stage I palliation for her functional single ventricle. Her cardiac defect consisted of an unbalanced, right-dominant complete atrioventricular septal defect and a double outlet right ventricle. She also had heterotaxy syndrome with left isomerism, polysplenia, and an interrupted inferior vena cava with azygous continuation to a right-sided superior vena cava.
View Article and Find Full Text PDFWorld J Pediatr Congenit Heart Surg
November 2021
Pediatric Cardiology, Cardiac Sciences, 37532Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates.
Surgical management of left ventricle outflow tract obstruction in infants with right-dominant unbalanced transitional atrioventricular septal defect poses difficulties. A two-month-old infant with transitional atrioventricular septal defect and complex left ventricle outflow tract obstruction presented in cardiogenic shock. The patient underwent successful biventricular repair.
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