Product information and disease description Place of product in therapy Approaches to treatment Current treatment options and standard of care

Mechanical circulatory support

11 evidence topics · 10 sources

GeneReviews: caution with durable support because of biventricular involvement

“Durable mechanical circulatory support should be used w/caution given biventricular involvement of disease.” (opens the source at this quote in a new tab)

AHA/ACC/HFSA heart failure guideline: anatomic criteria for durable support, in text that does not name Danon disease

“There are anatomic and physiologic criteria that make durable MCS inappropriate for some patients; it is most appropriate for those with HFrEF and a dilated ventricle.” (opens the source at this quote in a new tab)

AHA/ACC hypertrophic cardiomyopathy guideline: limited data on circulatory support, in text that does not name Danon disease

“Limited data are available on the role of temporary or biventricular mechanical circulatory support in patients with HCM.” (opens the source at this quote in a new tab)
“Data on the role of mechanical circulatory support in children with HCM are similarly limited.” (opens the source at this quote in a new tab)

Frequency of left ventricular assist device use in the International Danon Disease Registry

Group	Number with a left ventricular assist device	Percentage
Male participants (n = 52)	“2”	“3.8”
Female participants (n = 64)	“2”	“3.1”
All participants (n = 116)	“4”	“3.4”

Frequency of left ventricular assist device use in a Japanese nationwide survey

Group	Left ventricular assist device
Males	“1/17 (6%)”
Females	“4/22 (18%)”

Donor shortage and bridging in Japan

“However, due to the shortage of donor hearts in Japan, patients must wait three years until they can undergo heart transplantation.” (opens the source at this quote in a new tab)
“Therefore, an LVAD may help patients survive to transplantation.” (opens the source at this quote in a new tab)

First reported left ventricular assist device implantations: two cases

“This is the first report of Danon cardiomyopathy managed with a left ventricular assist device (LVAD).” (opens the source at this quote in a new tab)
“We present two cases of successful implantation of an LVAD for Danon cardiomyopathy.” (opens the source at this quote in a new tab)
“Patient 2 had dilatated cardiomyopathy with Danon cardiomyopathy and received transplantation 990 days after LVAD implantation without myopathy or intellectual disability.” (opens the source at this quote in a new tab)

Bridge to transplantation in an adult male: single case

“However, as most adult male patients with Danon disease are deemed ineligible for both LVAD and HTx, because of various severe comorbidities other than cardiac disease, reports concerning LVAD and/or HTx therapies for adult male patients with Danon disease are lacking.” (opens the source at this quote in a new tab)
“The clinical course after LVAD implantation was almost satisfactory except for a stroke on postoperative day 3, which was completely recanalized by emergent catheter angioplasty without any neurological deficits.” (opens the source at this quote in a new tab)

Biventricular support for 791 days in a 12-year-old girl: single case

“However, reports on VAD implantation for cardiomyopathy in Danon disease patients are limited.” (opens the source at this quote in a new tab)
“Owing to severe right ventricular failure, she was unable to be weaned off the cardiopulmonary bypass, and a right VAD implantation was undertaken using a Gyro pump (Kyosera, Kyoto, Japan).” (opens the source at this quote in a new tab)
“Despite several VAD-related complications, including aortic and pulmonary valve insufficiency and a driveline infection requiring hospitalization, the patient was able to attend junior high school during the waiting period for a heart transplantation.” (opens the source at this quote in a new tab)
“After a total waiting period of 882 days, including 791 days of biventricular Heartware VAD assistance, the patient underwent a heart transplantation.” (opens the source at this quote in a new tab)

Bridge to transplantation for 34 days in an adolescent: one of two brothers

“We report two cases of successful heart transplantation (HT) in adolescent brothers with DD, including one who was bridged to HT for 34 days with a HeartWare left ventricular assist device.” (opens the source at this quote in a new tab)
“These cases highlight that both HT and ventricular assist device support are feasible in patients with DD.” (opens the source at this quote in a new tab)

Death with a left ventricular assist device in the European registry: single participant

“Cerebrovascular event in context of ventricular assist device” (opens the source at this quote in a new tab)