Exercise Capacity and Reoperation Late After Transatrial Fallot Repair.

BACKGROUND: The exercise capacity long after repair of tetralogy of Fallot, when performed exclusively with a transatrial repair, is unclear. It is also unknown whether echocardiography and cardiopulmonary exercise testing can predict the risk of reoperation in this patient group. METHOD: We retrospectively reviewed the clinical records of 59 patients who underwent cardiopulmonary exercise testing after transatrial Fallot repair at a single centre. Patients underwent cardiopulmonary exercise testing at a mean age of 16.6±4.4 years, and at 15.3±4.1 years after Fallot repair. RESULTS: At testing, the volume of oxygen consumption at maximal exercise (VO2 max) was 71%±13% and the oxygen pulse was 80%±17% of predicted values. Seventeen (17) patients (29%) had a VO2 max superior to 80% of the predicted value. Thirty-two (32) patients (56%) had severe pulmonary regurgitation, three (5%) had moderate pulmonary regurgitation, and 12 (21%) had mild pulmonary regurgitation. After a mean of 7.8±3.9 years following cardiopulmonary exercise testing (23±5.3 years after the repair), 21 (40%) patients underwent reoperation. Right ventricular dilation and systolic function on echocardiography were both significantly associated with subsequent reoperation rates. Patients who had severe right ventricular dilation were eight times more likely to undergo subsequent reoperation (hazard ratio 8.67; 1.82-41.3; p=0.007). No cardiopulmonary exercise testing variable independently predicted reoperation. CONCLUSIONS: The exercise capacity at adolescence following transatrial repair of tetralogy of Fallot is maintained at around 70% of predicted values. Only the patients with normal right ventricular size and normal right ventricular function seemed to be protected from reoperation over the subsequent decade. We found no exercise variables which predicted reoperation.

Long-term outcomes of tetralogy of Fallot repair: A 30-year experience with 960 patients.

OBJECTIVE: This study evaluates the long-term results of tetralogy of Fallot repair and assesses the risk factors for adverse outcomes. METHODS: This retrospective study included 960 patients who underwent transatrial transpulmonary tetralogy of Fallot repair between 1990 and 2020. RESULTS: A transannular patch was placed in 722 patients, and pulmonary valve preservation was achieved in 233 patients. The median age at tetralogy of Fallot repair was 9.4 (interquartile range, 6.2-14.2) months. The median follow-up duration was 10.6 (interquartile range, 5.4-16.3) years. There were 8 early deaths (0.8%) and 20 late deaths (2.1%). Genetic syndrome and pulmonary valve annulus Z score less than -3 were risk factors for mortality. The survival was 97.7% (95% confidence interval, 96.4-98.5) and 94.5% (95% confidence interval, 90.9-96.7) at 10 and 30 years, respectively. Freedom from any reoperation was 86.4% (95% confidence interval, 83.6-88.7) and 65.4% (95% confidence interval, 59.8-70.4) at 10 and 20 years, respectively. Postoperative right ventricular outflow tract peak gradient of 25 mm Hg or greater correlated with reoperation. Propensity score-matched analysis demonstrated that freedom from pulmonary valve replacement at 15 years was higher in the pulmonary valve preservation group compared with the transannular patch group (98.2% vs 78.4%, P = .004). Freedom from reoperation for right ventricular outflow tract obstruction at 15 years was lower in the pulmonary valve preservation group compared with the transannular patch group (P = .006). CONCLUSIONS: The long-term outcomes of tetralogy of Fallot repair are excellent. A postoperative right ventricular outflow tract peak gradient less than 25 mm Hg appears to be optimal to prevent reoperation. If the pulmonary valve size is suitable, pulmonary valve preservation reduces the risk of pulmonary valve replacement, yet increases the reoperation rate for right ventricular outflow tract obstruction.

Long-term Outcomes of Transatrial-Transpulmonary Repair of Tetralogy of Fallot With Anomalous Coronary Arteries.

Repair of tetralogy of Fallot (TOF) can be complicated by the presence of an anomalous coronary artery (ACA) crossing the right ventricular outflow tract (RVOT). This study sought to evaluate the late outcomes of a policy of transatrial-transpulmonary repair for this condition. The transatrial-transpulmonary approach was used in 864 consecutive TOF repairs between 1993 and 2018 at a single institution, of which 55 (6%) patients had an ACA. Nineteen (35%,19/55) patients underwent prior palliation. Late survival and freedom from reoperations were compared with the general cohort of 809 patients who underwent complete repair during the same period. Early mortality was 2% (1/55). Median follow-up was 15.6 years. Late mortality was 6% (3/54). Absence of a preoperative diagnosis of ACA was not a risk factor for worse outcomes in terms of late re-interventions, acute coronary syndrome, residual RVOT gradient, and late mortality. Survival was 91% (95% confidence interval [CI]: 77-96%) at 20 years and was comparable to the general TOF cohort (95%, 95% CI: 90-98%, P = 0.12). Actuarial freedom from any re-intervention was 46% (95% CI: 27-62%) at 20 years, which was also comparable to the general cohort (31%, 95% CI: 20-42%, P = 0.19). The presence of an ACA does not appear to affect late survival or re-intervention rates in patients undergoing transatrial-transpulmonary repair of TOF.

Long-term Outcomes of Staged Complete Repair of Pulmonary Atresia With Ventricular Septal Defect.

BACKGROUND: The optimal strategy for pulmonary atresia with ventricular septal defect (PA-VSD) without major aortopulmonary collateral arteries is yet to be defined. It remains unclear how primary complete repair compares with staged repair. METHODS: This study reviewed the records of 107 patients with PA-VSD who were managed with systemic-to-pulmonary shunts from February 1989 to November 2019. Tables presenting summary statistics of patient and surgical data and Kaplan-Meier curves showing repair, mortality, and reoperation were created. Logistic regression analysis was performed to identify risk factors for repair, morbidity, and mortality. RESULTS: A total of 91 (85%) patients achieved complete repair at a median age of 1 year (interquartile range [IQR], 8-20 months). Survival was as follows: 97 (90%) patients at 6 months, 91 (85%) at 20 months, and 87 (81%) at 10 years. A shunt diameter of 3.5 mm or less (P = .006) and shunt after 2009 (P < .001) were associated with earlier progression to complete repair. A shunt diameter of 3.5 mm or less was an independent risk factor for interstage morbidity (hazard ratio, 4.5; IQR, 1.1-18.5; P = .039), but it was not associated with complete repair (P = .75) or mortality (P = .62). The median follow-up period was 10.5 years (IQR, 3.6-18.8 years) from birth. CONCLUSIONS: The complete repair and long-term survival rates of staged PA-VSD repair are high. The use of smaller shunts has resulted in earlier complete repair and is associated with increased risk of morbidity but not mortality.

Long-term outcomes of staged repair of tetralogy of Fallot.

BACKGROUND: The optimal management strategy for symptomatic young infants with tetralogy of Fallot (TOF) is yet to be determined. We aimed to evaluate the long-term outcomes of a staged approach with initial shunt palliation followed by complete repair. METHODS: Between January 1993 and July 2021, 160 children with TOF underwent a systemic-to-pulmonary shunt at our institution, including 65 neonates (41%). The mean duration of follow-up was 12.3 ± 8.1 years. RESULTS: Hospital mortality was 3% (4 of 160), all occurring in patients with a shunt size-to-weight ratio ≥1.2 mm/kg. Composite morbidity-defined as cardiac arrest, postoperative mechanical circulatory support, or unplanned reoperation-occurred in 21% (33 of 160). On multivariable analysis, a shunt size-to-weight ratio ≥1.2 mm/kg and prematurity were independent predictors of composite morbidity. Interstage mortality was 3% (4 of 156). A limited transannular patch was used in 75% (113 of 150) of TOF repairs. Actuarial survival at 20 years after shunt was 90% (95% confidence interval [CI], 79%-95%). Actuarial freedom from reinterventions at 20 years after TOF repair was 40% (95% CI, 28%-52%). Neonates had comparable composite morbidity, mortality, and late risk of reinterventions to older children. CONCLUSIONS: Staged repair of TOF in symptomatic young infants results in low mortality but high rates of reinterventions at long-term follow-up. A shunt size-to-weight ratio ≥1.2 mm/kg is a significant risk factor for mortality and morbidity prior to complete repair. Neonates undergoing shunt insertion have comparable outcomes to older children.

Right-Sided Infective Endocarditis: The Importance of Vegetation Size.

BACKGROUND: Right-sided infective endocarditis (IE) carries favourable prognosis compared to left-sided IE. However, the prognostic significance of vegetation size in right-sided IE is less well defined. This study reports the clinical, microbiological, and echocardiographic findings associated with right-sided IE and examines the predictors of adverse outcomes. METHODS: Consecutive adults admitted with isolated right-sided IE at an Australian tertiary referral centre between June 1999 and May 2017 were retrospectively reviewed. Patients were stratified according to intravenous drug user (IVDU) status. Culprit organisms, sepsis severity, treatment regimens, inpatient complications, and vegetation size were recorded. Hospital survivors were followed mean 6.9±4.8 years for late mortality and IE recurrence. RESULTS: Of 318 consecutive cases of IE, 60 (19%) were isolated right-sided IE and included in this study. Forty-three (43) (72%) patients were current IVDUs, who were younger and more likely to have hepatitis. The majority (90%) of patients were medically managed with multi-agent antimicrobial regimens (median three agents) for a total duration of median 91 days. In-hospital mortality was 3% (2/60). Septic emboli were found in 82% (49/60) of patients, were significantly more common among IVDUs but were not related to vegetation size. Survival after hospital discharge was 100% at 1 year, 96% at 3 years, and 89% at 5 years. Vegetation size >2 cm, chronic kidney disease, and Pitt bacteraemia score were independent predictors of all-cause late mortality. Freedom from IE recurrence was 93% at 1 year, 87% at 3 years, and 84% at 5 years. Vegetation >2.5 cm, prisoner status, and multivalvular IE involvement conferred higher risks of recurrence. CONCLUSIONS: Patients with right-sided IE and small vegetations do well with medical management and this should continue to be the preferred strategy. However, those with large vegetations have poorer late outcomes and may require more aggressive treatment and closer follow-up.

Single-ventricle palliation in children with atrioventricular septal defect and transposition of the great arteries: 45 years of experience.

BACKGROUND: The association of atrioventricular septal defect and transposition of the great arteries is very rare. As a rule, these patients have unbalanced ventricles. However, there have been no studies describing the results of single-ventricle palliation in these children. METHODS: All children who underwent surgery with a diagnosis of atrioventricular septal defect and transposition of the great arteries were included in the study. Data were obtained from medical records. RESULTS: A total of 38 patients with atrioventricular septal defect and transposition of the great arteries underwent single-ventricle palliation at the study institution between 1971 and 2016. The mean follow-up was 12.4 years (median: 14.6 years, range 2-43.3 years). Most children had unbalanced atrioventricular septal defect (94.7%, 36/38). Survival was 67.6% (95% confidence interval [CI]: 50.0-80.2%) at 10 years and 57.8% (95% CI: 38.0-73.4%) at 20 years. By 10 years, 58.6% (95% CI: 40.8-72.7%) had progressed to Fontan completion, while 32.5% (95% CI: 18.2-47.6%) had died. In patients achieving Fontan completion, 20-year event-free survival was 73.3% (95% CI: 34.8-91.3%), while 5.0% (95% CI: 0.4-20.5%) had undergone cardiac transplantation and 21.7% (95% CI: 3.2-50.8%) had undergone takedown of the Fontan circulation. Freedom from atrioventricular valve surgery was 57.0% (95% CI: 37.2-72.7%) at 10 and 20 years. CONCLUSIONS: The association of atrioventricular septal defect and transposition of the great arteries is very rare, and most of these children have unbalanced ventricles. Single-ventricle palliation results in 25-year overall survival of 50%. However, in patients, who had Fontan completion, survival was 75% at 25 years after Fontan operation.

Long-term outcomes of mitral valve endocarditis: improved survival through collaborative management.

BACKGROUND: Infective endocarditis (IE) of the mitral valve is an illness associated with significant morbidity and mortality. We describe the long-term outcomes of mitral valve endocarditis at a single centre. METHODS: All patients who presented with IE to the study institution between 2000 and 2015 were included. Data were obtained by retrospective review of the medical records. RESULTS: There were 163 patients who presented with mitral valve IE. Mean age was 58 ± 16.8 years. A history of intravenous drug use was present in 18% (30/163) of patients. The most common infective agents were Staphylococcus aureus in 42% (69/163) (7% (5/69) were methicillin resistant), Streptococcus viridans species in 15% (25/163) and Enterococcus faecalis in 10% (17/163). Surgery was performed in 29% (47/163) of patients. Hospital mortality was 23% (38/163). Survival was 71% (95% confidence interval (CI) 63.1-77.6%) at 1 year, 56% (95% CI 46.0-64.9%) at 5 years and 44% (95% CI 36.4-59.7%) at 10 years follow-up. There was no survival difference between medical and surgical management (P = 0.55). On multivariate Cox regression analysis, need for renal replacement therapy (P = 0.003) and increasing age (P = 0.014) were found to be risk factors while infectious diseases consult during index admission (P = 0.007) was found to be protective. CONCLUSION: Mitral valve endocarditis is associated with survival of <50% at 10 years follow-up. Surgical and medical management were associated with similar outcomes. Increasing age and need for renal replacement therapy were associated with mortality, and infectious diseases consultation was associated with improved survival.

Does transatrial-transpulmonary approach improve outcomes compared with transventricular approach in non-neonatal patients undergoing tetralogy of Fallot repair?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether the transatrial-transpulmonary approach to tetralogy of Fallot repair in non-neonatal patients provides superior outcomes compared with the transventricular approach. Altogether, 175 papers were found using the reported search, of which 11 represented the best evidence to answer the clinical question. Two randomized controlled trials (RCTs) and 3 observational studies showed that the transatrial approach resulted in better preservation of right ventricular (RV) function, whereas 4 observational studies showed no significant difference. Three observational studies showed better attenuation of RV dilatation, whereas 3 showed no difference. One RCT and 2 observational studies showed lower incidence of postoperative ventricular arrhythmias, while 1 RCT and 4 observational studies showed no difference. Two observational studies demonstrated greater freedom from reoperation, 1 RCT and 2 observational studies showed no difference, while 1 retrospective study observed a higher incidence of residual RV outflow tract obstruction and lower freedom from reoperation in infants. Two observational studies reported lower risk of requiring pulmonary valve replacement, whereas 2 reported no difference. Three observational studies reported superior exercise capacity, while 1 reported no difference. No difference in long-term survival was demonstrated. The results presented suggest that transatrial repair of tetralogy of Fallot confers superior or equivalent outcomes in terms of preservation of RV function and volume, ventricular arrhythmias, need for pulmonary valve replacement, and exercise capacity compared with transventricular repair. However, the incidence of residual RV outflow tract obstruction may be higher in infants undergoing transatrial repair.

Quality of life in adult survivors after paediatric heart transplantation in Australia.

BACKGROUND: Paediatric heart transplantation in Australia is centralised at The Royal Children's Hospital, Melbourne. Survival to adulthood is improving but the ongoing need for complex medical therapy, surveillance, and potential for late complications continues to impact on quality of life. Quality of life in adults who underwent heart transplantation in childhood in Australia has not been assessed. METHODS: Cross-sectional quality of life data were collected from paediatric heart transplant survivors >18 years of age using Rand 36-Item Health Survey. Self-reported raw scores were transformed to a 0-100 scale with higher scores indicating better quality of life. Mean scores were compared to National Health Survey Short Form-36 Population Norms data using the independent sample t-test. RESULTS: A total of 64 patients (64/151) who underwent transplantation at The Royal Children's Hospital between 1988 and 2016 survived to adulthood. In total 51 patients (51/64, 80%) were alive at the time of the study and 27 (53%) responded with a mean age of 25 ± 6 years, being a median of 11 years (interquartile range 7-19) post-transplantation. Most self-reported quality of life subscale scores were not significantly different from the Australian normative population data. However, self-reported 'General Health' was significantly worse than normative data (p = 0.02). Overall, 93% (25/27) reported their general health as being the same or better compared to 1-year ago. CONCLUSION: Adult survivors after paediatric heart transplantation in Australia report good quality of life in multiple domains and demonstrate independence in activities of daily living and employment. However, lifelong medical treatment may affect perceptions of general health.

Partition of Common Atrioventricular Valve in a Patient With Dextrocardia and Univentricular Circulation.

Long-term outcomes in children with atrioventricular septal defect (AVSD) and univentricular palliation are of concern, with <60% survival at 25 years.1 Common atrioventricular valves (AVV) often become insufficient in patients with univentricular physiology, leading to heart failure.1,2 We have recently observed that outcomes of children with AVSD who reach Fontan circulation are not as bad as previously thought, provided that the AVV remains competent.1 Common AVV surgery is associated with substantial mortality and reoperation rates.3 Although successful AVV repair is associated with better survival and freedom from reoperation, good quality repair is difficult to achieve in univentricular circulation,3 especially in patients with dextrocardia.4 Herein, we report a patient with unbalanced AVSD and dextrocardia who underwent AVV repair using the "polytetrafluoroethylene (Gore-Tex, W.L. Gore & Associates, Flagstaff, AZ) bridge" technique5 with excellent early outcome.

Cost-effectiveness of the National Pediatric Heart Transplantation Program in Australia.

OBJECTIVES: Cost data for pediatric heart transplantation are scarce. We examined hospital cost of the national pediatric heart transplantation program in Australia and assessed factors associated with increased costs. METHODS: The hospital cost of all children who underwent heart transplantation at a national referral center between January 2003 and June 2015 and were followed more than 1 year was retrospectively analyzed. Lifetime follow-up costs were adjusted for quality of life and projected to life expectancy. All costs were reported in 2016 US dollars. RESULTS: Of 70 children who underwent heart transplantation in the study period, 61 were followed more than 1 year after transplantation (mean, 4.3 ± 2.5 years). Mean cost of primary heart transplantation was $278,480 (95% confidence interval, 219,282-337,679) and did not change over time. Pretransplant mechanical circulatory support was required in 36% (22/61) of children. On multivariable analysis, greater admission costs were associated with ventricular assist device and pretransplant length of stay. Mean annual follow-up cost after discharge was $55,823 (95% confidence interval, 47,631-64,015) in the first year and $12,119 (95% confidence interval, 8578-15,661) thereafter. Increased first-year follow-up costs were associated with endomyocardial biopsies and length of readmissions. Cost per quality-adjusted life-year gained varied from $29,161 to $44,481 on sensitivity analysis. Freedom from treated rejections was 65.5% at 1 year, 63.2% at 3 years, and 59.5% at 5 years. Endomyocardial biopsies contributed to 52% of first-year follow-up costs. CONCLUSIONS: Primary pediatric heart transplantation in Australia is cost-effective for long-term survivors, even for those supported by ventricular assist device. Surveillance endomyocardial biopsy was a major contributor to post-transplantation costs. Selective targeting of surveillance biopsies may be cost-saving.

Long-Term Outcome After Pulmonary Artery Banding in Children With Atrioventricular Septal Defects.

BACKGROUND: Patients with atrioventricular septal defect (AVSD) may require pulmonary artery banding (PAB), either as a part of a staged univentricular palliation or to allow delayed biventricular repair in patients presenting with early heart failure. The long-term outcomes of PAB in children with AVSD have not been previously reported. METHODS: All children with AVSD who underwent PAB at a single institution were included in the study. Data were obtained from medical records and correspondence with general practitioners and cardiologists. RESULTS: A total of 68 patients with complete AVSD underwent PAB, of whom 58.8% of patients (40 of 68) had balanced AVSD (bAVSD) and underwent PAB with intent to subsequently perform biventricular repair. The remaining 41.2% of patients (28 of 68) had unbalanced AVSD (uAVSD) and underwent PAB as part of staged univentricular repair. PAB was not associated with a short-term increase in atrioventricular valve (AVV) regurgitation (p = 0.24). In patients with bAVSD, 83.8% (95% confidence interval [CI]: 67.4% to 92.4%) achieved biventricular repair. Survival was 73.4% (95% CI: 54.3% to 85.5%) and freedom from left AVV operation was 60.0% (95% CI: 36.1% to 77.4%) at 20 years of follow-up. In patients with uAVSD, 61.9% (95% CI: 40.5% to 77.5%) had achieved Fontan completion at 10 years of follow-up. Survival was 60.9% (95% CI: 36.2% to 78.5%) and freedom from AVV operation was 78.6% (95% CI: 55.5% to 90.6%) at 20 years. CONCLUSIONS: PAB can be used in patients with AVSD without compromising AVV function. Most patients with bAVSD progress to biventricular repair, albeit with a high rate of AVV reoperation. Patients with uAVSD who undergo PAB have similar outcomes to the overall uAVSD population.

Propensity score matched analysis of partial atrioventricular septal defect repair in infancy.

OBJECTIVE: Partial atrioventricular septal defect (pAVSD) is usually repaired between 2 and 4 years of age with excellent results. Repair during infancy has been associated with poorer outcomes. However, most infants in reported series had heart failure or significant left atrioventricular valve (LAVV) regurgitation. The impact of surgery during infancy on outcomes remains unclear. METHODS: All children at three institutions who underwent repair of pAVSD from 1975 to 2015 were included. Infants (aged <1 year) were compared with older children in a propensity score matched analysis. Variables used to generate propensity scores were: failure to thrive, congestive heart failure, preoperative LAVV regurgitation, associated congenital heart disease, sex and the presence of trisomy 21. RESULTS: pAVSD repair was performed on 430 children, 17.4% (75/430) were infants. Infants (mean age 0.5±0.3 years) had higher rates of LAVV regurgitation, heart failure and additional cardiac malformations than older children (mean age 4.7±3.5 years). At 30 years, survival for infants was 82.1% (95% CI 70.1% to 89.6%) compared with 95.7% (95% CI 91.3% to 97.9%) in older children (P<0.001).Propensity score matching yielded 52 well-matched pairs. Survival at 30 years was 87.9% (95% CI 75.0% to 94.4%) for infants compared with 98.1% (95% CI 87.1% to 99.7%) for older children (P=0.04). There was no significant difference in freedom from reoperation between the groups. CONCLUSIONS: Despite matching for risk factors, survival after repair of pAVSD during infancy is lower than that when repair is performed in older children, with no difference in reoperation rates. This suggests that elective repair of pAVSD should be deferred until after infancy.

Successful atrioventricular valve repair improves long-term outcomes in children with unbalanced atrioventricular septal defect.

BACKGROUND: Atrioventricular valve regurgitation is a significant cause of morbidity and mortality in patients with unbalanced atrioventricular septal defect. However, knowledge of the outcomes of atrioventricular valve repair in children with unbalanced atrioventricular septal defect and univentricular physiology is limited. METHODS: We conducted a retrospective review of all patients with unbalanced atrioventricular septal defect treated with single-ventricle palliation who underwent atrioventricular valve surgery at The Royal Children's Hospital. RESULTS: Between 1976 and 2016, 139 children with unbalanced atrioventricular septal defect underwent single-ventricle palliation, of whom 31.7% (44/139) required atrioventricular valve surgery. Repair of the atrioventricular valve was attempted in 97.7% (43/44) of patients, of whom 4.7% (2/43) were converted to replacement during the initial operation. Replacement of the atrioventricular valve without attempted repair was performed in 2.3% (1/44) of patients. Early mortality was 18.2% (8/44). Freedom from death or transplantation at 10 years was 66.0% (95% confidence interval, 49.1-78.5) and at 20 years was 53.3% (95% confidence interval, 32.1-70.6). In multivariable analysis, significant predischarge atrioventricular valve regurgitation (hazard ratio, 6.4; P = .002), age less than 1 year (hazard ratio, 8.3; P = .01), and repair before stage II palliation (hazard ratio, 3.4; P = .04) were associated with death. Freedom from reoperation at 10 years was 61.9% (95% confidence interval, 41.9-76.8) and at 20 years was 56.3% (95% confidence interval, 35.3-72.8). Moderate or greater atrioventricular valve regurgitation at discharge was associated with an increased risk of reoperation (hazard ratio, 1.8; P = .03). Of transplant-free survivors, atrioventricular valve regurgitation was less than moderate in 60.0% (15/25) at the most recent follow-up. CONCLUSIONS: Atrioventricular valve surgery in patients with unbalanced atrioventricular septal defect is associated with substantial mortality and a high rate of reoperation. Successful atrioventricular valve repair is associated with better survival and freedom from reoperation.

Ventricular assist devices for the failing univentricular circulation.

INTRODUCTION: Improved survival following single ventricle palliation has led to a large population of patients with a univentricular circulation, many of whom develop heart failure. Increasing experience with ventricular assist devices (VAD) in children has paved the way for VAD support in those with failing univentricular circulation. Areas covered: The use of VADs to support the failing univentricular circulation is a relatively new concept. Most studies have focused on supporting patients with the failing systemic ventricle. There are limited reports of VAD support of the pulmonary circulation in patients with Fontan failure despite preserved ventricular function. None of the current VADs have been designed to support the pulmonary circulation. Novel low-pressure, high-flow pumps, specifically designed to support the pulmonary circulation, are under development. Expert commentary: The failing univentricular circulation is one of the great challenges in the field of congenital heart disease. While current VADs are designed to support the systemic circulation, many patients require support of the pulmonary circulation. A fully implantable VAD for support of the pulmonary circulation as destination therapy would be beneficial for patients with preserved systolic function, but must have low energy requirements, negligible risk of stroke and low risk of device thrombosis and failure.