Combined Liver and Heart Transplantation for End-Stage Iron-Induced Organ Failure in an Adult with Homozygous Beta-Thalassemia

Olivieri, Nancy F.; Sher, Graham D.; Daly, Paul; Greig, Paul D.; McCusker, Patricia; Collins, Anne; Francombe, William H.; Templeton, Douglas M.; Butany, Jagdish

doi:10.1056/nejm199404213301605

Cited by 63 publications

(34 citation statements)

References 20 publications

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“…Although myocardial dysfunction in our patients was less severe than that reported in subjects undergoing CHLT for other diseases, [7][8][9][10][11][12][13][14][15] it would have implied an unacceptable risk for heart failure during or after LT, considering the probability of progression of cardiomyopathy after this operation in patients with such a non-Val30Met mutation as ATTR Glu89Gln. 3,4 Moreover, we previously had the experience of two patients with a Val30Met variant who died shortly after LT of cardiac failure after moderate perioperative hypotension despite apparently normal preoperative heart function.…”

Section: Discussionmentioning

confidence: 82%

Combined heart and liver transplantation for familial amyloidotic neuropathy: Considerations from the hepatic point of view

Grazi¹

2003

Liver Transplantation

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Few cases of combined heart and liver transplantation (CHLT) for familial amyloidotic polyneuropathy have been reported, and the technique for the operation is far from being consolidated. Three patients with amyloidogenic transthyretin (ATTR)-related (variant Glu89Gln to ATTR Glu89Gln) cardiomyopathy underwent CHLT at our institution. Patient 1 had no serious involvement of other organs, whereas patients 2 and 3 had evident peripheral neuropathy and gastrointestinal motility alterations. Patient 3 also had high-grade orthostatic hypotension. All three patients underwent cardiac and sequential hepatic transplantation using the piggyback technique with organs procured from the same donor. Venovenous bypass (VVB) was used only in patient 1, with an uncomplicated procedure. After CHLT, his cardiac performance remained normal, and no progression of amyloidosis was observed. Patient 2 had no intraoperative complications, but experienced postoperative bleeding, renal failure, sepsis, and heart failure and eventually died of multiorgan failure 2 months after transplantation. In patient 3, right hemicolectomy was required intraoperatively because of intestinal ischemia without significant hemodynamic perturbations, whereas extracardiac symptoms of amyloidosis gradually worsened postoperatively. Two patients (no. 1 and 3) currently are alive after 38 and 18 months, respectively. CHLT for ATTR Glu89Gln can be performed successfully, even in patients with advanced disease. However, the most compromised patients are more exposed to intraoperative risks, postoperative complications, and worsening of extracardiac and extrahepatic symptoms. The need for VVB remains to be evaluated. (Liver Transpl 2003;9:986-992.)

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Section: Discussionmentioning

confidence: 82%

Combined heart and liver transplantation for familial amyloidotic neuropathy: Considerations from the hepatic point of view

Grazi¹

2003

Liver Transplantation

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“…26 Patients with IOC and particularly those with hemoglobinopathies and other secondary causes of iron overload are not good candidates for heart transplantation, in general, because of the multiorgan injury caused by iron overload and the chronic blood-borne infections, including hepatitis B and C. 73 As a result, heart transplantation has only been performed in a limited number of selected patients. [73][74][75][76] According to a report in 2005, heart transplantation had been performed in 16 patients with IOC, including 11 with primary hemochromatosis, 4 with thalassemia major, and 1 with DiamondBlackfan anemia, with an overall 10-year survival rate of 41%. 75 A successful combined liver and heart transplantation has also been reported in a patient with thalassemia major.…”

Section: Kremastinos and Farmakismentioning

confidence: 99%

Iron Overload Cardiomyopathy in Clinical Practice

2011

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“…In homozygous betathalassemia, CLHT may be the only option for patients with end-stage, iron-induced, cardiac and liver disease, and it should be considered for any patient with iron loading and severe cardiac dysfunction associated with biopsy-proven cirrhosis [2].…”

Section: Discussionmentioning

confidence: 99%

Advantages of inferior vena caval flow preservation in combined transplantation of the liver and heart

Detry

Honoré

Meurisse

et al. 1997

Transplant International

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Advantages of inferior vena caval flow preservation in combined transplantation of the liver and heartAbstract Only a few cases of combined liver and heart transplantation have been reported in the literature, and no standard surgical procedure has yet been established. We report the successful transplantation of both liver and heart in a 28-year-old patient suffering from homozygous beta-thalassemia. We used Belghiti's technique of inferior vena caval flow preservation for liver transplantation, which avoids inferior vena cava occlusion by a side-to-side caval anastomosis. Applied to combined liver and heart transplantation, preservation of caval flow during liver transplantation

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Combined Liver and Heart Transplantation for End-Stage Iron-Induced Organ Failure in an Adult with Homozygous Beta-Thalassemia

Cited by 63 publications

References 20 publications

Combined heart and liver transplantation for familial amyloidotic neuropathy: Considerations from the hepatic point of view

Combined heart and liver transplantation for familial amyloidotic neuropathy: Considerations from the hepatic point of view

Iron Overload Cardiomyopathy in Clinical Practice

Advantages of inferior vena caval flow preservation in combined transplantation of the liver and heart

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