Management of the Failing Systemic Right Ventricle

doi:10.1053/j.semtcvs.2005.02.009

Seminars in Thoracic and Cardiovascular Surgery

Volume 17, Issue 2, Summer 2005, Pages 160-169

https://doi.org/10.1053/j.semtcvs.2005.02.009 Get rights and content

Conditions in which the right ventricle serves as the systemic pumping chamber are frequently complicated by the development of right ventricular failure and tricuspid valve regurgitation. The right ventricle is the systemic ventricle in conditions of ventriculoarterial discordance with atrioventricular concordance (transposition of the great arteries) or with atrioventricular discordance (congenitally corrected transposition of the great arteries). Concerns regarding actual or potential systemic right ventricular failure in these cases may lead to surgical evaluation and treatment designed to reestablish the left ventricle as the systemic pump. In cases where the left ventricle has prolonged exposure to low pressures in the pulmonary circulation, the left ventricle must be “retrained” to assume a systemic pressure load. Anatomic repair, with or without a preparatory period of left ventricular retraining, is a consideration for three clinically relevant scenarios: (1) patients with transposition of the great arteries after an atrial level switch (Senning or Mustard procedure), (2) patients with congenitally corrected transposition who are unoperated or who have undergone physiologic (“classic”) repair, and (3) unoperated patients with transposition who present after the neonatal period.

Section snippets

Anatomic and Physiologic Differences Between the Left and Right Ventricles

The left ventricle is anatomically suited to withstand a pressure load while the right ventricle is better suited to serve as a low-pressure, volume pump. Van Praagh has elegantly summarized anatomic differences between the left and right ventricles that provide a significant advantage for the left ventricle in the systemic circulation (Table 1).¹ The developmental origin of the left ventricle derives from the primitive ventricle, while the right ventricle is a more recent evolutionary

Right Ventricular Dysfunction in TGA After Atrial Inversion Procedures

Follow-up of patients with TGA after atrial inversion procedures in the 1980s suggested a reasonable expectation of survival with symptomatic heart failure occurring in perhaps 10% after 10 years.2, 3 However, longer periods of follow-up have demonstrated further evolution of heart failure in these patients. Piran found that 22% of patients developed symptomatic heart failure after a Mustard procedure during a mean follow-up of nearly 16 years.⁴ Further, most of these patients experienced a

Therapeutic Options for Systemic Right Ventricular/Tricuspid Valve Dysfunction

Our approach to patients with ventriculoarterial discordance and dysfunction of the systemic right ventricle or tricuspid valve in the setting of TGA after atrial inversion or ccTGA has been well described.32, 33, 34, 35, 36, 37, 38, 39, 40 Mild systemic right ventricular dysfunction with no more than mild to moderate tricuspid regurgitation is treated conservatively with afterload reduction and control of arrhythmias. Careful echocardiographic follow-up with exercise testing when appropriate

Results for Anatomic Conversion of TGA After Atrial Inversion

Our most recent results for staged anatomic reconstruction for this patient group were published in 2004.³² Over a 20-year period at two institutions (The Royal Children’s Hospital, Melbourne and The Cleveland Clinic), 39 patients who had previously undergone an atrial inversion procedure entered a surgical program of left ventricular reconditioning with a view to an arterial switch operation and atrial reseptation. The median age of the patients was 11 years (range 13 months to 24 years).

Summary and Conclusions

The poor long-term outlook for patients with ventriculoarterial discordance with or without atrioventricular discordance who possess a systemic right ventricle and tricuspid valve justifies attempts to perform anatomic correction. Patients with TGA after atrial inversion demonstrate higher mortality with left ventricular retraining and anatomic correction compared with ccTGA patients. This is at least partially due to progressively later age at presentation for patients with TGA who have

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Cited by (32)

Systemic Atrioventricular Valve Surgery in Patients With Congenitally Corrected Transposition of the Great Vessels
2023, Journal of the American College of Cardiology
Limited data exist regarding the long-term outcomes of systemic atrioventricular valve (SAVV) intervention (morphologic tricuspid valve) in congenitally corrected transposition (ccTGA).
The goal of this study was to evaluate the mid- and long-term outcomes of SAVV surgery in ccTGA.
We performed a retrospective review of 108 ccTGA patients undergoing SAVV surgery from 1979 to 2022. The primary outcome was a composite endpoint of mortality, cardiac transplantation, or ventricular assist device implantation. The secondary outcome was long-term systemic right ventricular ejection fraction (SVEF). Cox proportional hazard and linear regression models were used to analyze survival and late SVEF data.
The median age at surgery was 39.5 years (Q1-Q3: 28.8-51.0 years), and the median preoperative SVEF was 39% (Q1-Q3: 33.2%-45.0%). Intrinsic valve abnormality was the most common mechanism of SAVV regurgitation (76.9%). There was 1 early postoperative mortality (0.9%). Postoperative complete heart block occurred in 20 patients (18.5%). The actuarial 5-, 10-, and 20-year freedom from death or transplantation was 92.4%, 79.1%, and 62.9%. The 10- and 20-year freedom from valve reoperation was 100% and 93% for mechanical prosthesis compared with 56.6% and 15.7% for bioprosthesis (P < 0.0001). Predictors of postoperative mortality were age at operation (P = 0.01) and preoperative SVEF (P = 0.04). Preoperative SVEF (P < 0.001), complex ccTGA (P = 0.02), severe SAVV regurgitation (P = 0.04), and preoperative creatinine (P = 0.003) were predictors of late postoperative SVEF.
SAVV surgery remains a valuable option for the treatment of patients with ccTGA, with low early mortality and satisfactory long-term outcomes, particularly in those with SVEF ≥40%. Timely referral and accurate patient selection are the keys to better long-term outcomes.
Management Options for Congenitally Corrected Transposition: Which, When, and for Whom?
2022, Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual
Citation Excerpt :
Importantly, in this series, the median age at double switch was 7 years, highlighting the powerful impact of early PA banding. In patients diagnosed later with a failing mRV and TR, a PA band should be considered, placed as an ‘open-ended’ strategy.14,22,23,26–30 Depending on the patient's age and response, the band may allow for eventual anatomical repair.
Management strategies for congenitally corrected transposition of the great arteries (ccTGA) historically consisted of a physiologic repair, resulting in the morphologic right ventricle (mRV) supporting systemic circulation. This strategy persisted despite the development of heart failure by middle age because of the reasonable short-term outcomes, and the natural history of some patients with favorable anatomy (felt to demonstrate the mRV's ability to function in the long-term), and due to the less-than-optimal outcomes associated with anatomical repair. As outcomes with anatomical repair improved, and the long-term risk of systemic mRV dysfunction became apparent, more have begun to realize its advantages. In addition to the decision on whether or not to pursue anatomical repair, and the optimal timing, studies demonstrating the nuance to morphologic left ventricle retraining have demonstrated its feasibility. Further considerations in ccTGA have begun to be better understood, including: the management of a poorly functioning mRV, systemic tricuspid valve regurgitation, the utility of morphologic left ventricle outflow tract obstruction (native or surgically created) and pacing strategies. While some considerations are apparent: biventricular pacing is superior to univentricular, tricuspid regurgitation must be managed early with either progression towards anatomical repair (pulmonary artery banding if needed for retraining) or tricuspid replacement (not repair) based on the patient's age; others remain to be completely elucidated. Overall, the heterogeneity of ccTGA, as well as the unique presentation with each patient regarding ventricular and valvular function and center-to-center variability in management strategies has made the interpretation of published data difficult. That said, more recent long-term outcomes favor anatomical repair in most situations.
The Fate of Congenitally Corrected Transposition of the Great Arteries Unoperated Before Adulthood
2021, Annals of Thoracic Surgery
Citation Excerpt :
Graham and colleagues1 reported that SAVV regurgitation was associated with SV dysfunction, which was consistent with our study. For adult ccTGA patients with severe SAVV regurgitation, it was difficult to achieve anatomical repair.7-10 SAVV replacement or valvuloplasty may be considered the standard surgical option to reduce SAVV regurgitation and protect SV function.
The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear.
From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect, and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19.0 (IBM, Armonk, NY).
At the first visit, no patients suffered operation. The PS group had the least systemic atrioventricular valve regurgitation and the greatest systemic ventricular ejection fraction. All 49 patients underwent surgery. From the first visit to last follow-up, systemic ventricular ejection fraction of unoperated ccTGA decreased significantly. In the intact ventricular septum group, patients receiving systemic atrioventricular valve replacement/valvuloplasty had a significantly increased systemic ventricular ejection fraction and statistically more freedom from death and transplant than unoperated. In the ventricular septum defect group the late systemic ventricular ejection fraction of operated patients was not statistically different from their basic data at first visit. In the PS group patients receiving physiologic repair had significantly decreased systemic ventricular ejection fractions. Severe systemic atrioventricular valve regurgitation, physiologic repair, and systemic ventricular dysfunction (ejection fraction <40%) were risk factors for mortality, transplant, and congestive heart failure.
PS protects against systemic atrioventricular valve regurgitation and ventricular dysfunction. Systemic atrioventricular valve replacement/valvuloplasty improved systemic ventricular function for ccTGA with an intact ventricular septum. Physiologic repair was not ideal for ccTGA with PS. Severe systemic atrioventricular valve regurgitation and systemic ventricular dysfunction were associated with suboptimal outcomes.
Surgical Management of the Failing Systemic Ventricle
2018, Heart Failure in the Child and Young Adult: From Bench to Bedside
Heart failure (HF) is a complex physiologic phenomenon that, prior to the modern era, was poorly understood despite being widely recognized. The typical symptoms of dyspnea, cachexia, and edema were known in antiquity and were a portent of death. Today, it is recognized as a progressive clinical syndrome with a variety of etiologies and pathophysiologies and is becoming better understood at a molecular level. Compared to older patients, HF in children and young adults not only includes many of the same diagnoses but also the sequelae of complex congenital heart disease. Many of these are rare conditions and defy a simple classification system. All of congenital heart surgery may be considered as treatment for or prevention of HF. In this chapter, we will focus on emerging surgical strategies for the systemic right ventricle, the failing Fontan, and the borderline left ventricle.
Echocardiography-Derived Left Ventricular Outflow Tract Gradient and Left Ventricular Posterior Wall Thickening Are Associated with Outcomes for Anatomic Repair in Congenitally Corrected Transposition of the Great Arteries
2017, Journal of the American Society of Echocardiography
Congenitally corrected transposition of the great arteries is a rare form of congenital heart disease. Management is controversial; options include observation, physiologic repair, and anatomic repair. Assessment of morphologic left ventricle preparedness is key in timing anatomic repair. This study's purpose was to review the modalities used to assess the morphologic left ventricle preoperatively and to determine if any echocardiographic variables are associated with outcomes.
A retrospective review of patients with congenitally corrected transposition of the great arteries eligible for anatomic repair at Lucile Packard Children's Hospital from 2000 to 2016 was conducted. Inclusion criteria were (1) presurgical echocardiography, magnetic resonance imaging, and cardiac catheterization and (2) clinical follow-up information. Echocardiographic measurements included left ventricular (LV) single-plane Simpson's ejection fraction, LV eccentricity index, LV posterior wall thickening, pulmonary artery band (PAB)/LV outflow tract (LVOT) pressure gradient, and LV and right ventricular strain. Magnetic resonance imaging measurements included LV mass, ejection fraction, eccentricity index, and LV thickening. LV pressure, PAB/LVOT gradient, right ventricular pressure, pulmonary vascular resistance, and Qp/Qs constituted catheterization data. Outcomes included achieving anatomic repair within 1 year of assessment in patients with LVOT obstruction or within 1 year of pulmonary artery banding and freedom from death, transplantation, or heart failure at last follow-up.
Forty-one patients met the inclusion criteria. PAB/LVOT gradients of 85.2 ± 23.4 versus 64.0 ± 32.1 mm Hg (P = .0282) by echocardiography and 60.1 ± 19.4 versus 35.9 ± 18.9 mm Hg (P = .0030) by catheterization were associated with achieving anatomic repair and freedom from death, transplantation, and heart failure. Echocardiographic LV posterior wall thickening of 35.4 ± 19.8% versus 20.6 ± 15.0% (P = .0017) and MRI LV septal wall thickening of 37.1 ± 18.8% versus 19.3 ± 18.8% (P = .0306) were associated with achieving anatomic repair. Inter- and intraobserver variability for echocardiographic measurements was very good.
PAB/LVOT gradient and LV posterior wall thickening are highly reproducible echocardiographic measurements that reflect morphologic LV performance and can be used in assessing patients with congenitally corrected transposition of the great arteries undergoing anatomic repair.
Surgical Management of the Failing Systemic Ventricle
2017, Heart Failure in the Child and Young Adult: From Bench to Bedside
Heart failure (HF) is a complex physiologic phenomenon that, prior to the modern era, was poorly understood despite being widely recognized. The typical symptoms of dyspnea, cachexia, and edema were known in antiquity and were a portent of death. Today, it is recognized as a progressive clinical syndrome with a variety of etiologies and pathophysiologies and is becoming better understood at a molecular level. Compared to older patients, HF in children and young adults not only includes many of the same diagnoses but also the sequelae of complex congenital heart disease. Many of these are rare conditions and defy a simple classification system. All of congenital heart surgery may be considered as treatment for or prevention of HF. In this chapter, we will focus on emerging surgical strategies for the systemic right ventricle, the failing Fontan, and the borderline left ventricle.

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Management of the Failing Systemic Right Ventricle

Section snippets

Anatomic and Physiologic Differences Between the Left and Right Ventricles

Right Ventricular Dysfunction in TGA After Atrial Inversion Procedures

Therapeutic Options for Systemic Right Ventricular/Tricuspid Valve Dysfunction

Results for Anatomic Conversion of TGA After Atrial Inversion

Summary and Conclusions

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