Contemporary Outcomes of Combined Heart-Liver Transplant in Patients With Congenital Heart Disease

Bryant, Roosevelt; Rizwan, Raheel; Zafar, Farhan; Shah, Shimul A.; Chin, Clifford; Tweddell, James S.; Morales, David L.S.

doi:10.1097/tp.0000000000001978

Cited by 57 publications

(34 citation statements)

References 18 publications

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“…The latest International Society for Heart and Lung Transplantation (ISHLT) data show overall adult OHT 1-year survival of 85%-89%. 5,15,31,35,36 Risk factors for adverse outcome include protein losing enteropathy, acute heart failure, and kidney disease, all of which were relatively common among our cohort of patients. 31 Orthotopic heart transplantation alone in the adult Fontan patient is associated with a relatively high mortality of 20% in recent meta-analyses.…”

Section: Discussionmentioning

confidence: 99%

“…33,34 While some reports demonstrate transplant mortality to be equivalent in Fontan versus non-Fontan patients, this is less true in the older adult Fontan patient. 5,15,31,35,36 Risk factors for adverse outcome include protein losing enteropathy, acute heart failure, and kidney disease, all of which were relatively common among our cohort of patients. 5 Moreover, Fontan patients frequently have multiple prior sternotomies and complex anatomy that requires pulmonary artery and venous reconstruction.…”

Section: Discussionmentioning

confidence: 99%

“…Post-operatively six patients were extubated at a median of 3 days (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19), and the other three required tracheostomy at a median of 17 days (14)(15)(16)(17)(18)(19)(20)(21)(22)(23). Post-operatively six patients were extubated at a median of 3 days (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19), and the other three required tracheostomy at a median of 17 days (14)(15)…”

Section: Peri-operativementioning

confidence: 99%

“…(5-96) and 29 ) days, respectively. Post-operatively six patients were extubated at a median of 3 days (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19), and the other three required tracheostomy at a median of 17 days (14-23). Inotropic support with dopamine, phenylephrine, epinephrine, and/or norepinephrine was continued for a median of 10.5 days (5-59), with the two patients with the longest time of support needing octreotide, midodrine, and methylene blue to treat persistent vasoplegia.…”

Section: Peri-operativementioning

confidence: 99%

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Short‐term outcomes of en bloc combined heart and liver transplantation in the failing Fontan

Vaikunth

Concepción

Daugherty

et al. 2019

Clinical Transplantation

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Patients with failing Fontan physiology and liver cirrhosis are being considered for combined heart and liver transplantation. We performed a retrospective review of our experience with en bloc combined heart and liver transplantation in Fontan patients > 10 years old from 2006 to 18 per Institutional Review Board approval. Six females and 3 males (median age 20.7, range 14.2-41.3 years) underwent en bloc combined heart and liver transplantation. Indications for heart transplant included ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, and/or lymphatic abnormalities. Indication for liver transplant included portal hypertension and cirrhosis. Median Fontan/single ventricular end-diastolic pressure was 18/12 mm Hg, respectively. Median Model for End-Stage Liver Disease excluding International Normalized Ratio score was 10 (7-26), eight patients had a varices, ascites, splenomegaly, thrombocytopenia score of ≥ 2, and all patients had cirrhosis. Median cardiopulmonary bypass and donor ischemic times were 262 (178-307) and 287 (227-396) minutes, respectively. Median intensive care and hospital stay were 19 (5-96) and 29(13-197) days, respectively. Survival was 100%, and rejection was 0% at 30 days and 1 year post-transplant. En bloc combined heart and liver transplantation is an acceptable treatment in the failing Fontan patient with liver cirrhosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Peri-operativementioning

confidence: 99%

Section: Peri-operativementioning

confidence: 99%

See 2 more Smart Citations

Short‐term outcomes of en bloc combined heart and liver transplantation in the failing Fontan

Vaikunth

Concepción

Daugherty

et al. 2019

Clinical Transplantation

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show abstract

“… 7 The 10-year survival rate after combined transplants is even better for congenital heart diseases compared to single heart transplants (83% vs. 39%, p = 0.03). 80 The treatment decision for combined heart-liver transplantations must consider the model of liver damage, the level of liver fibrosis, the degree of liver impairment and the number of organ failures. In cases of pure congestive hepatopathy or associated liver diseases with a fibrosis stage between F0 and F2 and the absence of HCC, a heart transplantation alone should be proposed.…”

Section: Treatment Of Congestive Hepatopathymentioning

confidence: 99%

From congestive hepatopathy to hepatocellular carcinoma, how can we improve patient management?

et al. 2021

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Summary Heart failure and liver disease often coexist because of systemic disorders and diseases that affect both organs as well as complex cardio-hepatic interactions. Heart failure can cause acute or chronic liver injury due to ischaemia and passive venous congestion, respectively. Congestive hepatopathy is frequently observed in patients with congenital heart disease and after the Fontan procedure, but also in older patients with chronic heart failure. As congestive hepatopathy can evolve into cirrhosis and hepatocellular carcinoma, screening for liver injury should be performed in patients with chronic cardiac diseases and after Fontan surgery. Fibrosis starts in the centro-lobular zone and will extend progressively to the portal area. Chronic liver injury can be reversible if heart function improves. However, in the case of terminal heart failure, uncontrolled by medical resources or by assistive device support, the combination of heart and liver transplants must be discussed in patients with chronic advanced liver fibrosis. In this review of the literature, we will focus on congestive hepatopathy and its complications, such as liver fibrosis and hepatocellular carcinoma, with the aim of improving the management and surveillance of patients experiencing these complications.

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Simultaneous Heart–Liver Transplantation for Congenital Heart Disease in the United States: Rapidly Increasing With Acceptable Outcomes

et al. 2021

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Background and Aims There are more adults than children living with congenital heart disease (CHD) in the United States, with a growing proportion requiring heart–liver transplantation (HLT). Our aim was to ascertain the frequency, outcomes, and prognostic factors in this patient population. Approach and Results United Network for Organ Sharing data on adult patients who underwent heart transplantation (HT) from 2009 through March 2020 were analyzed. The primary study outcome was patient survival. Cox proportional‐hazards modeling assessed for mortality associations. There were 1,084 HT recipients: 817 (75.4%) CHD HTs only, 74 (6.8%) CHD HLTs, 179 (16.5%) non‐CHD HLTs, and 14 (1.3%) heart–liver–kidney transplants. The number of CHD HLTs increased from a prior rate of 4/year to 21/year in 2019. Among patients with CHD, the 5‐year survival rates were 74.1% and 73.6% in HTs only and HLTs, respectively (P = 0.865). There was a higher rate of allograft failure attributable to rejection in CHD HTs only compared with CHD HLTs (3.2% versus 0.4%; P = 0.014). Only 25 out of 115 HT‐performing hospitals undertook CHD HLTs. Higher‐volume centers (averaging one CHD HLT per year) had a 5‐year patient survival rate of 83.0% compared with 61.3% in lower‐volume centers (P = 0.079). Among HLT recipients, total bilirubin (hazard ratio [HR], 1.06; 95% confidence interval [CI], 1.01‐1.12) and diabetes (HR = 2.97, 95% CI = 1.21‐7.31) were independently associated with increased mortality risk, whereas CHD and age were not. Conclusions The rate of HLT for adult CHD in the United States is rising dramatically. The survival outcomes between CHD HT only and CHD HLT groups are comparable; however, the HLT group had lower rates of acute rejection. Among HLT recipients, diabetes and elevated bilirubin are associated with increased posttransplant mortality risk. An average of one CHD HLT per year could be considered a minimum quality metric at transplant centers.

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Contemporary Outcomes of Combined Heart-Liver Transplant in Patients With Congenital Heart Disease

Abstract: The posttransplant outcome of CHLT, with and without CHD, is comparable. However, there is a trend toward better survival for CHLT for CHD compared with isolated heart transplant for CHD.

Cited by 57 publications

References 18 publications

Short‐term outcomes of en bloc combined heart and liver transplantation in the failing Fontan

Short‐term outcomes of en bloc combined heart and liver transplantation in the failing Fontan

From congestive hepatopathy to hepatocellular carcinoma, how can we improve patient management?

Simultaneous Heart–Liver Transplantation for Congenital Heart Disease in the United States: Rapidly Increasing With Acceptable Outcomes

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