Does Ascending Aorta Size Affect Norwood Outcomes in Hypoplastic Left Heart With Aortic Atresia?

Carvajal, Horacio G.; Canter, Matthew W.; Abarbanell, Aaron M.; Eghtesady, Pirooz

doi:10.1016/j.athoracsur.2020.02.037

Cited by 10 publications

(6 citation statements)

References 27 publications

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“…1–3 We report a case of HLHS with anomalous origin of right pulmonary artery from ascending aorta, a rare association not reported in literature. In neonates with HLHS, Carjaval and colleagues 4 observed that the mean ascending aortic diameter was 2.5 ± 0.9 mm, with only 8.56% of subjects having a relatively large aorta, defined as ascending aortic diameter ≥4 mm. The strikingly fair-sized ascending aorta served as the only arterial supply to the right lung in the absence of major aortopulmonary collaterals, via an anomalous right pulmonary artery with normal peripheral arborization, which provides a rationale for the unusual occurrence of a relatively large ascending aorta and aortic arch in this subset of HLHS.…”

Section: Discussionmentioning

confidence: 99%

Hypoplastic left heart syndrome with anomalous origin of right pulmonary artery

Mishra

Champaneri

Rana

et al. 2020

Asian Cardiovasc Thorac Ann

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Classic hypoplastic left heart syndrome is a rare but fatal congenital heart disease associated with variable underdevelopment of the left side of the heart. Complex forms of hypoplastic left heart syndrome have been reported to coexist with anomalous pulmonary venous drainage, transposition of the great arteries, or pulmonary valve dysplasia. We report a case of hypoplastic left heart syndrome with anomalous origin of the right pulmonary artery from the ascending aorta a rare association not reported in the literature. Preoperative comprehensive echocardiography is essential for diagnosis and accurate recognition of such rare anatomic variations.

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Section: Discussionmentioning

confidence: 99%

Hypoplastic left heart syndrome with anomalous origin of right pulmonary artery

Mishra

Champaneri

Rana

et al. 2020

Asian Cardiovasc Thorac Ann

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“…36 However, ascending aortic size does not appear to impact long-term outcomes after the Stage I palliation, with the exception of the increased rate of recoarctation. 36…”

Section: Small Ascending Aortamentioning

confidence: 95%

“…Recent data have confirmed that aortic diameters less than 1.5 mm have a higher rate of early adverse events and mortality. 36 However, ascending aortic size does not appear to impact long-term outcomes after the Stage I palliation, with the exception of the increased rate of recoarctation. 36 Alternative surgical methods for exceptionally small ascending aortas are increasingly described.…”

Section: Small Ascending Aortamentioning

confidence: 95%

“…36 However, ascending aortic size does not appear to impact long-term outcomes after the Stage I palliation, with the exception of the increased rate of recoarctation. 36 Alternative surgical methods for exceptionally small ascending aortas are increasingly described. One such technique involves anastomosing the pulmonary artery to the transverse aortic arch and placing an internal pulmonary artery band, leaving the atretic aorta in continuity with the arch.…”

Section: Small Ascending Aortamentioning

confidence: 95%

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High-Risk Anatomic Subsets in Hypoplastic Left Heart Syndrome

Geoffrion

Fuller

2022

World J Pediatr Congenit Heart Surg

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Despite overall improvements in outcomes for patients with hypoplastic left heart syndrome, there remain anatomic features that can place these patients at higher risk throughout their treatment course. These include severe preoperative obstruction to pulmonary venous return, restrictive atrial septum, coronary fistulae, severe tricuspid regurgitation, smaller ascending aorta diameter (especially if <2 mm), and poor ventricular function. The risk of traditional staged palliation has led to the development of alternative strategies for such patients. To further improve the outcomes, we must continue to diligently examine and study anatomic details in HLHS patients.

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“…For risk-adjusted modeling, we examined previously studied factors for Norwood mortality including prematurity, low birthweight, Hispanic ethnicity, female sex, older age at the time of operation, genetically related syndromes, HLHS diagnosis, significant neonatal AVV regurgitation, highly restrictive interatrial communication, reduced single ventricular function, small ascending aorta diameter, surgeon performing the operation, and type of Norwood. [8][9][10][11][12][13][14][15][16][17] Genetic syndromes included DiGeorge's, Down's, Turner's, Partial Trisomy 7, Distal 10q Deletion, and 18q Deletion. We defined premature birth as birth before 37 gestational weeks and low birthweight as <2,500 grams.…”

Section: Preoperative Risk Factorsmentioning

confidence: 99%

Outcomes Following Norwood Procedures: Analysis of a “Small Volume” Program

D’Angelo

Narasimhulu

Pourmoghadam

et al. 2022

World J Pediatr Congenit Heart Surg

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Background Institutional survival following Norwood procedures is traditionally correlated with a center's surgical volume. Multiple single and multi-institutional studies conducted at large-volume centers have recently demonstrated improved survival following Norwood procedures. We report both short- and long-term outcomes at a single, small-volume institution and comment on factors potentially influencing outcomes at this institution. Methods All patients undergoing Norwood procedures from January 1, 2005, to January 1, 2020, at our institution were included in this study. Kaplan-Meier survival and Cox regression risk factor analyses were performed in addition to first interstage risk factor scoring to compare observed versus expected survival. Results The cohort included 113 patients. Kaplan-Meier freedom from death or transplant was 88%, 80%, and 76% at 1, 5, and 10 years, respectively. Freedom from death following hospital discharge after Norwood procedures was 94%, 87%, and 83% at 1, 5, and 10 years, respectively. The presence of genetic syndromes was a significant risk factor for mortality. First interstage observed-to-expected mortality following discharge was 0.57 ( P = .04). Postoperative length of stay was comparable to that reported for the period 2015 to 2018 in the Society of Thoracic Surgeons Database. Conclusions Survival outcomes at this single, small-volume institution were similar to those reported by large-volume centers and multi-institutional collaborative studies. These results may be related to structural and functional features that have been demonstrated to influence outcomes in other studies. These factors are achievable by small-volume programs with sufficient resource allocation.

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Does Ascending Aorta Size Affect Norwood Outcomes in Hypoplastic Left Heart With Aortic Atresia?

Cited by 10 publications

References 27 publications

Hypoplastic left heart syndrome with anomalous origin of right pulmonary artery

Hypoplastic left heart syndrome with anomalous origin of right pulmonary artery

High-Risk Anatomic Subsets in Hypoplastic Left Heart Syndrome

Outcomes Following Norwood Procedures: Analysis of a “Small Volume” Program

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