Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy

Larsen, Ranae L.; Eguchi, Jim; Mulla, Neda F.; Johnston, Joyce K.; Fitts, J.; Kuhn, Marie; Razzouk, Anees J.; Chinnock, Richard; Bailey, Leonard L.

doi:10.1016/s0002-9149(02)02325-1

Cited by 41 publications

(18 citation statements)

References 13 publications

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“…The success of heart transplantation as a therapy for hypoplastic left heart syndrome led pediatric transplantation centers to apply heart transplantation as primary therapy in other conditions for which there was a poor natural history with standard surgical therapy. These conditions have included pulmonary atresia with intact septum and right ventricledependent coronary circulation 54,55 and complex heterotaxy syndromes 56,57 in which a functional single ventricle can be associated with anomalous pulmonary venous return and severe atrioventricular or semilunar valve disease.…”

Section: Congenital Heart Diseasementioning

confidence: 99%

Indications for Heart Transplantation in Pediatric Heart Disease

Canter¹,

Shaddy²,

Bernstein³

et al. 2007

Circulation

261

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Background— Since the initial utilization of heart transplantation as therapy for end-stage pediatric heart disease, improvements have occurred in outcomes with heart transplantation and surgical therapies for congenital heart disease along with the application of medical therapies to pediatric heart failure that have improved outcomes in adults. These events justify a reevaluation of the indications for heart transplantation in congenital heart disease and other causes of pediatric heart failure. Methods and Results— A working group was commissioned to review accumulated experience with pediatric heart transplantation and its use in patients with unrepaired and/or previously repaired or palliated congenital heart disease (children and adults), in patients with pediatric cardiomyopathies, and in pediatric patients with prior heart transplantation. Evidence-based guidelines for the indications for heart transplantation or retransplantation for these conditions were developed. Conclusions— This evaluation has led to the development and refinement of indications for heart transplantation for patients with congenital heart disease and pediatric cardiomyopathies in addition to indications for pediatric heart retransplantation.

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Section: Congenital Heart Diseasementioning

confidence: 99%

Indications for Heart Transplantation in Pediatric Heart Disease

Canter¹,

Shaddy²,

Bernstein³

et al. 2007

Circulation

261

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“…Conversely heart transplantation is a valid alternative in children with visceral heterotaxy. Larsen and associates 21 recently reported a 68% 5-year and 50% 10-year survival after OHT in 29 patients with visceral heterotaxy who were at high risk for standard palliation because of AV valve insufficiency, ventricular dysfunction or aortic atresia. Lamour et al 22 reported successful OHT in 3 cases with failed Fontan physiology.…”

Section: Discussionmentioning

confidence: 99%

Orthotopic Heart Transplantation for Congenital Heart Disease: An Alternative for High-Risk Fontan Candidates?

et al. 2003

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Objective-Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for congenital heart disease. Methods-Between 1988 and 2002, 43 patients (mean age 9.1Ϯ7.2 years) underwent 44 OHT for complex TGA (6), DORV (4), single ventricle (21), and other end-stage structural heart disease (11). Two discernible ventricular chambers were present in 18 pts (41.8%). Previous reconstructive or palliative procedures had been previously accomplished in 35 pts (83.3%), including atrial switch (5), systemic-to-pulmonary shunts (10), cavopulmonary anastomosis (9), Fontan completion (6), and others (5). Results-30-day survival for the 2-ventricle subgroup was 94.4Ϯ5.4% compared with 67.2Ϯ9.5% for the single ventricle subgroup (Pϭ0.04) (overall 78.6%Ϯ3.3%). OHT following single ventricle staging to bi-directional cavopulmonary anastomosis exhibited 100% early survival, as opposed to 62.5Ϯ17.1% for OHT after systemic-to-pulmonary shunts, and 33.3Ϯ19.2% for OHT following failing Fontan (Pϭ0.010). HLHS diagnosis (0.0085) and failing Fontan (Pϭ0.003) were identified as independent predictors of early mortality by regression logistic modeling, while Fontan stage represented the only predictor of overall mortality by Cox proportional hazard. Overall 10-year survival was 54.3Ϯ11%. Conclusions-OHT for structural congenital heart disease with single ventricle physiology entails substantial early mortality and bi-directional cavopulmonary anastomosis enables the best transition to heart transplant. OHT should be considered in the decision making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.

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“…Patients with heterotaxy have poor long-term outcomes post-transplant and OHT should be considered judiciously in this group (46). Other recognized but unquantified risk factors are shown in Box 1.…”

Section: Mortality In Sv Heart Transplantationmentioning

confidence: 99%

Transplantation in the single ventricle population

Kenny¹,

DeRita²,

Nassar³

et al. 2018

Ann. Cardiothorac. Surg.

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The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution's practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair.Progress in surgical palliative strategies has impacted upon the present composition of the population, with growing numbers of hypoplastic left heart syndrome patients and those with late physiology failure following Fontan completion. Multiple prior surgeries, immunological sensitivity and multi-organ involvement impart high peri-operative risk but can be mitigated in part by careful pre-operative planning by a dedicated multidisciplinary team addressing issues such as planning of concurrent reconstructive surgery, minimizing the post-operative effect of collaterals, timely harvesting, oversizing of donor organs to minimize graft failure, and strategies to address anticipated post-operative elevation in pulmonary vascular resistance. Determining optimal timing for transplant in these patients remains unclear, but understanding the risk of alternative surgical options can help guide decision making with regards to listing.

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Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy

Cited by 41 publications

References 13 publications

Indications for Heart Transplantation in Pediatric Heart Disease

Indications for Heart Transplantation in Pediatric Heart Disease

Orthotopic Heart Transplantation for Congenital Heart Disease: An Alternative for High-Risk Fontan Candidates?

Transplantation in the single ventricle population

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