A functional understanding of moderate to complex congenital heart disease and the impact of pregnancy. Part II: Tetralogy of Fallot, Eisenmenger’s syndrome and the Fontan operation

Background: Varying single center data exist regarding the posttransplant outcomes of patients with single ventricle circulation, particularly following the Fontan operation. We sought to better elucidate these results in patients with congenital heart disease (CHD) through combining two national databases. Methods: The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS), an administrative database with 71% of UNOS patients matched. Patients undergoing transplantation at a PHIS hospital from 2006 to 2017 were categorized as single ventricle or biventricular strategy based on their diagnoses and procedures in 90% of patients. When known, single ventricle patients were further analyzed by their palliative stage post-Glenn or post-Fontan (known in 31%). Results: A total of 1,517 CHD transplantations were identified, 67% with single ventricle strategy (1,016). Single ventricle, biventricular, and indeterminate patients had similar survival (log-rank P > .1). Risk factors for mortality in patients with CHD were extracorporeal membrane oxygenation (ECMO) support at transplant (hazard: 2.27), ABO blood type incompatibility (hazard: 1.61), African American recipient (hazard 1.42), and liver dysfunction (hazard 1.29). A total of 130 confirmed Fontan and 185 confirmed bidirectional Glenn patients underwent transplantation, each with survival equivalent to biventricular patients (log-rank P > .500). For Fontan patients, renal dysfunction (hazard: 5.40) and transplant <1 year after Fontan (hazard 2.82) were found to be associated with mortality. Conclusions: Single ventricle patients, as a group, experience similar outcomes as biventricular patients with CHD undergoing transplantation, and this extends to Fontan patients. Risk factors for mortality correlate with end-organ dysfunction as well as race and ABO blood type incompatibility in the CHD population.

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Modified Fontan operation for complex congenital heart disease.

Japanese Journal of Cardiovascular Surgery ◽

10.4326/jjcvs.15.325 ◽

1986 ◽

Vol 15 (4) ◽

pp. 325-326

Author(s):

T. Abe

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Fontan Operation ◽

Complex Congenital Heart Disease ◽

Modified Fontan Operation

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Moderate to complex congenital heart disease

10.1093/med/9780198713333.003.0040 ◽

2016 ◽

Author(s):

Daryl P. Dob ◽

Elspeth E. Pickering ◽

Michael A. Gatzoulis

Keyword(s):

Quality Of Life ◽

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Early Death ◽

Western World ◽

Complex Congenital Heart Disease ◽

Congenital Aortic Stenosis ◽

Eisenmenger’S Syndrome

Children born with congenital heart disease no longer face the prospect of early death and a poor quality of life. In fact, most neonates with moderate to complex congenital heart disease have a survival rate to adulthood of over 80%. The ratio of adults to children with congenital heart disease is increasing, due to better surgical repairs, and longer survival with a better quality of life. In the Western world, there are more adults than children alive with congenital heart disease. This remarkable medical effort has allowed young women with congenital heart disease to mature to an age where they wish to have babies of their own. Early generations of women, palliated with Mustard or Senning repairs, have shown it is possible to face the cardiovascular challenges of pregnancy and survive. As the number of women with congenital heart disease is predicted to grow by 25% in the next decade and more women with congenital heart disease become pregnant; a better understanding of moderate to complex heart disease, different surgical repair procedures, and residual anomalies is paramount. This chapter examines the management of parturients with transposition complexes (both classical and congenitally corrected), tetralogy of Fallot, the Fontan circulation, Eisenmenger’s syndrome, and congenital aortic stenosis, taking into consideration the effect of pregnancy, labour, delivery, and anaesthesia on each circulation.

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Heart Transplantation in Congenital Heart Disease: In Whom to Consider and When?

Journal of Transplantation ◽

10.1155/2013/376027 ◽

2013 ◽

Vol 2013 ◽

pp. 1-12 ◽

Cited By ~ 8

Author(s):

Christine H. Attenhofer Jost ◽

Dörthe Schmidt ◽

Michael Huebler ◽

Christian Balmer ◽

Georg Noll ◽

...

Keyword(s):

Heart Failure ◽

Congenital Heart Disease ◽

Heart Disease ◽

Heart Transplantation ◽

Lung Transplantation ◽

Congenital Heart ◽

Complex Congenital Heart Disease ◽

Eisenmenger’S Syndrome ◽

Number Of Patients ◽

Increased Risk

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger’s syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future.

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Abstract 18813: Outcomes of Patients with Congenital Heart Disease following Heart Transplantation: the Impact of Disease Type, Prior Thoracic Surgeries and End-Organ Dysfunction

Circulation ◽

10.1161/circ.130.suppl_2.18813 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Matthew J Lewis ◽

Jonathan Ginns ◽

P. C Schulze ◽

Matt Lippel ◽

Paul Chai ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Heart Transplantation ◽

Organ Dysfunction ◽

Congenital Heart ◽

Fontan Operation ◽

Pulmonary Atresia ◽

Patient Specific ◽

Increased Risk ◽

The Impact

Introduction: Adults with congenital heart disease (ACHD) are at increased risk for early adverse outcomes following heart transplantation (Htx). Despite the need for improved risk stratification, small cohorts have constrained identification of patient-specific factors associated with poor prognosis. We hypothesized that type of CHD, number of sternotomies and prior end-organ dysfunction would be associated with an increased risk for mortality post-HTx. Methods: We performed a retrospective, observational cohort study of all patients with ACHD who underwent HTx at our institution from 1/1997 to 1/2014. The primary endpoint was death. Exposures of interest included type of CHD, number of sternotomies and hepatic dysfunction secondary to passive congestion, measured as high Models for End-Stage Liver Disease Excluding INR (MELD-XI) score. Results: 48 ACHD patients were followed (mean age at HTx: 36±1.7 years). Diagnoses included: Tetralogy of Fallot (TOF)/pulmonary atresia/double outlet right ventricle in 15 (31%), D-transposition of the great arteries in 10 (21 %), tricuspid atresia/double inlet left ventricle in 9 (19%), VSD/ASD in 4 (8%), heterotaxy in 3 (6%), congenitally corrected transposition of the great arteries in 2 (4%), and 5 (10%) with other diagnoses. Pre-transplant cardiac surgeries included: Fontan operation in 12 (25%), Mustard/Senning repair in 9 (19%), TOF repair in 5 (11%), Rastelli repair in 5 (11%), VSD/ASD closures in 3 (6%), other another congenital heart surgery in 8 (17%) and no prior cardiac surgery in 5 (11%). Over a median follow-up time of 3.9 years, 14 patients died (29%) and 10 (71%) deaths occurred within 30 days of HTx. Survival at 1 and 5 years was 77%. Deaths within 30 days of surgery were secondary to hemorrhage in 4 (40%), graft failure in 3 (30%) and multi-system organ failure in 3 (30%). By multivariate analysis, ≥3 sternotomies (HR 8.5, p=0.02), MELD-XI score >18 (HR 6.2, p=0.009) and heterotaxy (HR 9.8, p=0.01), were significant predictors of mortality. Conclusion: In our cohort of patients with CHD undergoing Htx, ≥3 sternotomies, MELD-XI score >18 and heterotaxy syndrome were significantly associated with death. These findings may be important in patient selection and timing of Htx in this population.

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