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Outcomes of Berlin Heart EXCOR ® pediatric ventricular assist device support in patients with restrictive and hypertrophic cardiomyopathy
This study reviews the North American experience with Berlin Heart EXCOR® ventricular assist device implants in children with such physiology. The Berlin Heart clinical database was reviewed. Patients with primary diastolic dysfunction are included in this study. Twenty pediatric patients with restrictive cardiomyopathy (n = 13), hypertrophic cardiomyopathy (n = 3), or congenital heart disease with restrictive physiology (n = 4) who were supported with EXCOR® were identified. Of these, nine (45%) were successfully bridged to transplant, one (5%) weaned from support, and 10 (50%) died after support was withdrawn. Of...
Source: Pediatric Transplantation - September 1, 2017 Category: Transplant Surgery Authors: Jennifer A. Su, Jondavid Menteer Tags: ORIGINAL ARTICLE Source Type: research

Demands and challenges for patients with sickle ‐cell disease requiring hematopoietic stem cell transplantation in Saudi Arabia
In this study, we estimated the number of Saudi patients with SCD who are candidates for HSCT. We used the presence of overt stroke, recurrent ACS, and frequent severe pain crisis as indications for HSCT. We calculated the frequencies of these complications among a Saudi SCD cohort of 376 patients with SCD, 250 from SW and 126 from Eastern (E) provinces. We found that 59 (23.6%) of SW patients were transplant candidates compared to 22 (17.4%) from E province. It is estimated that about 61 000 patients with SCD live in Saudi Arabia. Thus, the projected number of Saudi patients with SCD who are candidates for HSCT is 10 53...
Source: Pediatric Transplantation - July 30, 2016 Category: Transplant Surgery Authors: Abdulrahman Alsultan, Wasil Jastaniah, Sameera Al Afghani, Muneer H. Al Bagshi, Zaki Nasserullah, Ahmed M. Al ‐Suliman, Mohammed K. Alabdulaali Tags: Original Article Source Type: research

Challenges managing end ‐stage renal disease and kidney transplantation in a child with MTFMT mutation and moyamoya disease
We describe a four‐yr‐old female with features of moyamoya disease referred to our center for kidney transplant evaluation with ESRD secondary to presumed renal dysplasia along with concern for cerebral vascular anomalies. With her constellation of organ involvement, a genetic workup revealed a homozygous, frameshift mutation in the mitochondrial methionyl‐tRNA formyltransferase gene. Given her vascular anomalies and evidence of prior infarcts seen on cerebral imaging, it was felt that her risk of future stroke events was high and that hypotension or intravascular volume depletion would further exacerbate this risk. ...
Source: Pediatric Transplantation - July 7, 2016 Category: Transplant Surgery Authors: Aris Oates, Jessica Brennan, Anne Slavotinek, Adnan Alsadah, Daniel Chow, Marsha M. Lee Tags: Case Report Source Type: research

Challenges managing end‐stage renal disease and kidney transplantation in a child with MTFMT mutation and moyamoya disease
We describe a four‐yr‐old female with features of moyamoya disease referred to our center for kidney transplant evaluation with ESRD secondary to presumed renal dysplasia along with concern for cerebral vascular anomalies. With her constellation of organ involvement, a genetic workup revealed a homozygous, frameshift mutation in the mitochondrial methionyl‐tRNA formyltransferase gene. Given her vascular anomalies and evidence of prior infarcts seen on cerebral imaging, it was felt that her risk of future stroke events was high and that hypotension or intravascular volume depletion would further exacerbate this risk. ...
Source: Pediatric Transplantation - July 7, 2016 Category: Transplant Surgery Authors: Aris Oates, Jessica Brennan, Anne Slavotinek, Adnan Alsadah, Daniel Chow, Marsha M. Lee Tags: Case Report Source Type: research

Difficult decision: What should we do when a VAD‐supported child experiences a severe stroke?
Source: Pediatric Transplantation - December 30, 2014 Category: Transplant Surgery Authors: Stefanie Benoit, Armand H. Matheny Antommaria, Norbert Weidner, Angela Lorts Tags: Commentary Source Type: research

The use of a Berlin Heart EXCOR LVAD in a child receiving chemotherapy for Castleman's disease
We present the unique case of a pediatric patient who received chemotherapy for a diagnosis of CD, while mechanically supported with a Berlin EXCOR LVAD secondary to restrictive cardiomyopathy. A four‐yr‐old previously healthy male with restrictive cardiomyopathy required MCS after cardiac arrest but was diagnosed with multicentric CD, a non‐malignant lymphoproliferative disorder fueled by excessive IL‐6 production. Treatment with IL‐6 blockade (tocilizumab) every two wk and methylprednisolone had no effect on his lymph nodes or cardiac function while on temporary RotaFlow. A Berlin LVAD was placed for treatment...
Source: Pediatric Transplantation - December 1, 2014 Category: Transplant Surgery Authors: Tamara O. Thomas, Shanmuganathan Chandrakasan, Maureen O'Brien, John L. Jefferies, Thomas D. Ryan, Ivan Wilmot, Michael L. Baker, Peace C. Madueme, David Morales, Angela Lorts Tags: Case Report Source Type: research

Mechanical embolectomy for ischemic stroke in a pediatric ventricular assist device patient
We describe a nine‐yr‐old patient on BiVAD support who experienced threatened AIS on two separate occasions. He was treated successfully via mechanical embolectomy on both occasions and survived to transplantation with minimal neurologic deficits.
Source: Pediatric Transplantation - March 20, 2014 Category: Transplant Surgery Authors: Eileen Rhee, Robert Hurst, Bryan Pukenas, Rebecca Ichord, Anne Marie Cahill, Joseph Rossano, Stephanie Fuller, Kimberly Lin Tags: Case Report Source Type: research