Elevation of IGF-2 receptor and the possible underlying implications in end-stage heart failure patients before and after heart transplantation

Introduction and Background: Arrhythmogenic ventricular cardiomyopathy (AVC) is a hereditable disorder characterized by fibro-fatty infiltration of the right ventricular myocardial wall. The purpose of this study was to describe the clinicopathologic phenotype of AVC in pediatric end-stage heart failure patients who underwent heart transplantation (HTx). Hypothesis: We hypothesized that AVC is misclassified in the young population who require heart transplant. Methods: We investigated 371 consecutive cases of primary pediatric (≤21 years) heart transplantation performed at Texas Children’s hospital between 1989 and 2018. Heart re-transplants were excluded from the study. Explanted hearts and tissue blocks were examined by a cardiac pathologist after HTx. Histological Diagnosis of AVC was based on the presence of major and minor diagnostic criteria according to the 2010 Revised Task Force Criteria. Results: Over half of the patients who underwent HTx had cardiomyopathy (212/371=57%) as the underlying primary diagnosis. After comprehensive histological evaluation of explanted hearts, 8 cases (8/212=3.8%) were diagnosed with AVC. Predominantly right ventricular disease was seen in 2/8 (25%), and biventricular involvement in 6/8 (75%) patients on pathological examination. Six out of 8 patients (6/8=75%) were misclassified, 4 as dilated cardiomyopathy, 1 as viral myocarditis, and 1 as restrictive cardiomyopathy. The median age at heart transplant (AVC cohort) was 11 years. Four of 8 (50%) were males. Six of 8 (75%) had a significant ventricular arrhythmia burden manifesting as non-sustained ventricular tachycardia requiring antiarrhythmic therapy. Genetic testing was undertaken in 3 of 8 (37.5%); all three were found to have pathogenic mutations in the PKP2 gene. Conclusion: Arrhythmogenic ventricular cardiomyopathy is often misclassified in the young population who require heart transplant. Pediatric dilated and restrictive cardiomyopathy phenotypes with end-stage heart failure waitlisted for a heart transplant (HT) who have a significant ventricular arrhythmia burden should be investigated for AVC.

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The Use of Intranasal Fentanyl for the Palliation of Incident Dyspnea in Advanced Congestive Heart Failure: A Pilot Study

Journal of Palliative Care ◽

10.1177/0825859718777343 ◽

2018 ◽

Vol 34 (2) ◽

pp. 96-102 ◽

Cited By ~ 4

Author(s):

Jana Pilkey ◽

Allison Pedersen ◽

James W. Tam ◽

Amrit Malik ◽

Jonathan Wong

Keyword(s):

Heart Failure ◽

Pilot Study ◽

Vital Signs ◽

Shortness Of Breath ◽

Heart Failure Patients ◽

Before And After ◽

Similar Distance ◽

End Stage Heart Failure ◽

End Stage ◽

Minute Walk

Background: Dyspnea is distressing in palliative patients with end-stage heart failure and many are hospitalized to optimize this symptom. We hoped to conduct a pilot study to determine whether the administration of intranasal fentanyl would decrease activity-induced dyspnea in this patient population. Methods: Patients performed two 6-minute walk tests with and without the administration of 50 μg of intranasal fentanyl. Vital signs were recorded before and after each walk, as were participant reported dyspnea and adverse events scores. Results: Twenty-four patients were screened, 13 were deemed eligible, and 6 completed the study. Dyspnea scores changed from a mean of 6.00 immediately after the walk without fentanyl to a mean of 3.83 after the walk with fentanyl ( P = .048). Mean respiratory rate decreased from 21.0 to 18.7 ( P = .034) breaths per minute and was considered a favorable outcome by the participants. Distance walked did not significantly increase with the fentanyl pretreatment (136.0-144.2 m; P = .283), although the participants reported feeling better while walking a similar distance. Conclusions: In this pilot study, the preadministration of intranasal fentanyl prior to activity in palliative, end-stage hospitalized heart failure patients, safely reduced tachypnea, and the feeling of shortness of breath. This approach may help palliate advanced heart failure patients by alleviating symptoms brought on by exertional activities.

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