Two Ventricles Are Not Better Than One in the Fontan Circulation: Equivalent Late Outcomes

Marathe, Supreet P; Zannino, Diana; Shi, William Y.; Plessis, Karin du; Kehr, Jascha; Perumal, Gopinath; Sun, Jessica; Gentles, Thomas L.; Ayer, Julian; Sholler, Gary F.; Verrall, Charlotte E; Alphonso, Nelson; Andrews, David; d’Udekem, Yves; Winlaw, David S.

doi:10.1016/j.athoracsur.2018.08.024

Cited by 20 publications

(16 citation statements)

References 23 publications

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“…A recent study comparing patients with a 2-ventricle versus a 1-ventricle Fontan demonstrated no difference in survival between the 2 groups, calling into question the wisdom of resorting to a single ventricle strategy among patients with 2 working ventricles. 8 In the face of these sobering SVP results, institutions must consider whether a more optimal outcome can be achieved with SVP or a higher-risk biventricular repair.…”

Section: The Unavoidable Fontan Physiologymentioning

confidence: 99%

The Ventricular Switch: An Alternative Strategy Toward Biventricular Repair in Moderately Hypoplastic Left Ventricles or Complex Connections

Najm

2022

World J Pediatr Congenit Heart Surg

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Hypoplastic left heart complex (HLHC) constitutes a spectrum of left ventricular hypoplasia and valvar disease and can be associated with increased morbidity and mortality. Traditionally, management of these lesions involves single ventricle palliation (SVP). However, the SVP may be associated with substantial long-term consequences related to its physiology. Alternative management strategies have been employed to achieve biventricular circulation. We have proposed a novel technique, termed the “ventricular switch” procedure, in which the hypoplastic or unroutable left ventricle can be harnessed to function as the subpulmonic ventricle, and the right ventricle functions as the systemic ventricle. Herein, we describe our rationale for considering biventricular circulation and the ventricular switch procedure in HLHC and in other complex lesions.

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Section: The Unavoidable Fontan Physiologymentioning

confidence: 99%

The Ventricular Switch: An Alternative Strategy Toward Biventricular Repair in Moderately Hypoplastic Left Ventricles or Complex Connections

Najm

2022

World J Pediatr Congenit Heart Surg

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“…2,3 Further, a widely held belief that the outcomes among single ventricle (SV) patients with "2" ventricles (ie, those in whom septation was not done for other anatomic reasons), are superior to outcomes among SV repairs with only "one" ventricle, led to general acceptance of SV strategies in this population. However, the advent of advanced imaging and 3D modeling coupled with recent literature 4,5 challenging the idea that a 2-ventricle Fontan performs better than a 1-ventricle Fontan, encouraged our group to develop novel techniques to achieve a BiV circulation among patients with heterotaxy in whom septation to achieve a twoventricle or one-and-a-half ventricle repair is limited by inability to route the pulmonary venous return toward the left ventricle (LV). This report describes our initial experience in septation/BiV conversion among 5 consecutive patients, utilizing the LV as the subpulmonary ventricle, essentially recapitulating the physiology of congenitally corrected transposition of the great arteries (ccTGA).…”

Section: Perspective Statementmentioning

confidence: 99%

Biventricular Conversion in Unseptatable Hearts: “Ventricular Switch”

Najm

Karamlou

Ahmad

et al. 2021

Seminars in Thoracic and Cardiovascular Surgery

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Patients with complex systemic and pulmonary venous anatomy, common atrioventricular canal defects and conotruncal anomalies have traditionally been routed to univentricular palliation and labeled as "unseptatable." This report describes our initial experience in septation/biventricular conversion ("ventricular switch"), utilizing the left ventricle (LV) as the subpulmonary ventricle, essentially recapitulating the physiology of congenitally corrected transposition of the great arteries. Five consecutive patients with challenging anatomic configuration underwent septation. All patients were severely cyanotic and had important functional limitations. All patients required complex atrial septation. Ventricular septation was precluded by fixed pulmonary vascular resistance in 2 patients. Systemic venous return was diverted to the morphologic LV as part of physiological 2V (n = 4) or 1.5 V repair (n = 1). Median conversion age was 9 years (range 11 monthsÀ46 years). Four patients had 12 previous cardiac surgical procedures in preparation for univentricular repair elsewhere. Three dimensional-printed heart models evaluated feasibility of septation. All patients are alive at a median follow-up of 0.6 years (range 0.08À2.7 years). Median hospital stay was 13 (range 10À60) days. LV recruitment improved functional status and significantly increased systemic oxygen saturation in all patients (79 § 7% vs 95 § 5%, P = 0.003). We report a novel paradigm for successfully utilizing both ventricles with the morphologic LV as the subpulmonary ventricle, in a complex population thought to be unseptatable. This approach is versatile and can likely be extrapolated to other complex anatomic configurations. Although we utilized this strategy in patients of variable age, earlier ventricular switch may yield the best results.

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“…In general, the operation has demonstrated its success with a growing population of patients having this unique Fontan circuit physiology. However, with long-term follow up, the circulation of these patients have recently shown to have multisystem effects with both expected and unexpected sequelae (62)(63)(64).…”

Section: Fontan Palliation For Single Ventricle Patientsmentioning

confidence: 99%

Surgical outcomes in complex adult congenital heart disease: a brief review

2020

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Life expectancy of patients with congenital heart disease (CHD) continues to increase dramatically over the last few decades, primarily due to significant medical and surgical advancements in the field. Adult congenital heart disease (ACHD) patients are now living longer but continue to face morbidity and reduced long-term survival when compared to the general population. We briefly describe the growth of ACHD with a focus on surgical procedures and outcomes in the more complex congenital heart defects including Tetralogy of Fallot, Ebsteins Anomaly of the tricuspid valve, Coarctation of the Aorta, Transposition of the Great Arteries and single ventricle lesions. The advancing role of mechanical circulatory support and cardiac transplantation in ACHD patients is also highlighted. Cardiac surgery in these patients continues to improve with low surgical mortality rate and outstanding long-term outcomes and efficacy.

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Two Ventricles Are Not Better Than One in the Fontan Circulation: Equivalent Late Outcomes

Abstract: Dr d'Udekem discloses a financial relationship with MSD and Actelion.

Cited by 20 publications

References 23 publications

The Ventricular Switch: An Alternative Strategy Toward Biventricular Repair in Moderately Hypoplastic Left Ventricles or Complex Connections

The Ventricular Switch: An Alternative Strategy Toward Biventricular Repair in Moderately Hypoplastic Left Ventricles or Complex Connections

Biventricular Conversion in Unseptatable Hearts: “Ventricular Switch”

Surgical outcomes in complex adult congenital heart disease: a brief review

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