Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children

Iannucci, Glen; Adisa, Olufolake; Oster, Matthew E.; McConnell, Michael V.; Mahle, William T.

doi:10.14503/thij-15-5610

Cited by 2 publications

(2 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In terms of cardiac transplantation in patients with sickle cell, there are only 2 adults and 1 child described who underwent cardiac transplantation with SCT, 1 of whom had a coexisting rare red cell membrane disorder . There are also case reports describing patients with SCD undergoing cardiac transplant . An adult patient with a baseline HbS of 6% (due to transfusion) received a heart transplant with an exchange transfusion, a 10‐year‐old patient with a heart transplant 2 years earlier being treated for humoral rejection, 5 children with congenital heart disease who underwent heart transplantation with SCD, and a patient who underwent a retransplant and who died 4 years later after having a postoperative cerebrovascular accident.…”

Section: Discussionmentioning

confidence: 99%

“…Despite these challenges, there are several reports of successful outcomes for patients with SCD undergoing cardiac surgery, both with and without red cell exchange, including our center's experience of pulmonary endarterectomy with deep hypothermic circulatory arrest . In terms of cardiac transplantation, there are reports of only 2 adults and 1 child who underwent cardiac transplantation with SCT, and 1 adult and 6 children who underwent cardiac transplantation with SCD . Many heart transplant units that serve Afro‐Caribbean/African American populations do not specifically test for SCT during heart transplant assessment.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Catastrophic sickling crisis in patient undergoing cardiac transplantation with sickle cell trait

Ali

Besser

Goddard

et al. 2019

American Journal of Transplantation

View full text Add to dashboard Cite

There is debate in the literature regarding management of patients with sickle cell trait (SCT) undergoing cardiac surgery, since it is recognized that cardiopulmonary bypass presents many precipitating risk factors for a sickling crisis. Despite this, many report successful outcomes without any modification to perioperative management. A 49‐year‐old woman with SCT (HbS 38%) with postpartum cardiomyopathy underwent cardiac transplantation. The patient was cooled to 34.0°C and retrograde cold blood cardioplegia was infused continuously. The cold ischemic time was 219 minutes and warm ischemic time 46 minutes. After weaning from bypass, she developed global cardiac dysfunction requiring veno‐arterial extracorporeal membrane oxygenation. The circuit suddenly stopped, requiring emergency reinstitution of bypass; the circuit had clotted. Transesophageal‐echocardiogram revealed thrombus within the left atrium and ventricle. There was no recovery of cardiac function and the patient developed multiorgan failure. At postmortem there was extensive myocardial infarction with evidence of widespread catastrophic intravascular red‐cell sickling. This case highlights the danger of complacency in patients with SCT, offering a learning opportunity for the cardiothoracic community to highlight the most serious complication that can occur in this group of patients. We have learned that SCT and cardiac surgery is not a benign combination.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%