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1.
World J Pediatr Congenit Heart Surg ; 15(3): 270-276, 2024 05.
Article in English | MEDLINE | ID: mdl-38404011

ABSTRACT

Background: We sought to evaluate the influence of the rudimentary ventricle on long-term outcomes after the Fontan operation, focusing on exercise capacity and cardiac performance. Methods: Between 1995 and 2021, 290 patients underwent a total cavopulmonary connection. "Two-ventricle" Fontan circulation was defined as a rudimentary ventricle >30% of the dominant ventricle or >50% of its predicted normal value. This cohort was compared with patients with single-ventricle Fontan circulation. The primary endpoint was Fontan failure, and the secondary endpoints were VO2 and cardiac catheterization data at ten years postoperatively. Results: The median follow-up after the Fontan operation was 7.9 years (interquartile range: 1.9-13.9). No significant difference was found in Fontan failure-free survival between the "two-ventricle" Fontan circulation group and the single-ventricle Fontan circulation group (83/91, 93% vs 156/199, 78%), respectively at 20 years; P = .11). No significant difference was found in VO2 or cardiac performance except cardiac index, with 2.9 (2.3-4.8) versus 2.5 (2.3-4.3) L/min/m2 (P = .047). Fifty-one patients in the "two-ventricle" Fontan circulation group were followed up for over ten years after the Fontan operation. In the subgroup analysis of this cohort, the rudimentary ventricular end-diastolic volume/dominant ventricular end-diastolic volume ratio showed a significant positive correlation with dominant ventricular end-diastolic pressure (r = 0.58 [95% CI 0.35-0.74], P = .002) and a significant negative correlation with VO2 (r = -0.61 [95% CI -0.80 to -0.28], P = .001). Conclusions: The rudimentary ventricle was not significantly associated with any clinical disadvantages regarding Fontan failure. However, a large rudimentary ventricle was significantly associated with higher end-diastolic pressure and lower exercise capacity.


Subject(s)
Fontan Procedure , Heart Defects, Congenital , Heart Ventricles , Humans , Female , Male , Heart Defects, Congenital/surgery , Heart Ventricles/abnormalities , Heart Ventricles/surgery , Heart Ventricles/physiopathology , Child , Retrospective Studies , Child, Preschool , Follow-Up Studies , Treatment Outcome , Exercise Tolerance/physiology , Adolescent , Cardiac Catheterization
2.
Pediatr Cardiol ; 44(4): 933-939, 2023 Apr.
Article in English | MEDLINE | ID: mdl-36538051

ABSTRACT

This study aimed to evaluate the outcome of coarctation of the aorta (CoA) repair with a special interest in bovine arch anatomy. Fifty-six patients who underwent CoA repair between 2010 and 2021 were included in this retrospective study. Of these, 11 patients had bovine arch anatomy. Surgical outcomes were reviewed. Computed tomography was used to analyze aortic arch geometry for all cases preoperatively. The gap between anastomotic sites was calculated at the linear region of the lesser curvature of the aortic arch between the distal ascending aorta and the proximal descending aorta. CoA repair was performed at a median age of 39 days (median body weight 3.3 kg). Thirty-two patients underwent extended direct anastomosis, 22 patients underwent direct anastomosis, and two patients underwent lesser curvature patch augmentation. The median follow-up period was 47 months. There were no early deaths. In patients who underwent direct and extended direct anastomosis, nine recoarctation and one left pulmonary venous obstruction events occurred. Moreover, freedom from these adverse events was 81% in normal arch and 50% in bovine arch patients at 10 years (P = 0.04). Two patients with a bovine arch anatomy who underwent lesser curvature patch augmentation had good outcomes. The distal arch was narrower and longer, and the gap between anastomotic sites was longer in patients with a bovine arch anatomy than with a normal arch (P < 0.01). In CoA with a bovine arch anatomy, the gap between anastomotic sites was long. This adversely influenced the outcomes of the CoA repairs.


Subject(s)
Aortic Coarctation , Humans , Infant , Aortic Coarctation/diagnostic imaging , Aortic Coarctation/surgery , Retrospective Studies , Aorta/surgery , Aorta, Thoracic/diagnostic imaging , Aorta, Thoracic/surgery , Anastomosis, Surgical , Treatment Outcome , Follow-Up Studies
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