Diagnosis and therapeutic evaluation of a pediatric case of cardiomyopathy using phosphorus-31 nuclear magnetic resonance spectroscopy

Glenn J.R. Whitman, Britton Chance, Hans Bode, John Maris, John Haselgrove, Richard Kelley, Bernard J. Clark, Alden H. Harken

Research output: Contribution to journalArticle

Abstract

An 8 month old girl presented with undiagnosed non-anatomic congenital cardiomyopathy with massive car-diomegaly on chest X-ray film. Her older sibling had died suddenly at 6 months of age from what appeared to be a similar abnormality. Utilizing phosphorus-31 nuclear magnetic resonance (P-31 NMR) surface coil spectroscopy, a metabolic disorder was demonstrated in both her myocardium and skeletal muscle, revealing a phos-phocreatine (PCr) to inorganic phosphate (Pi) ratio of half of that for a normal control infant. Manipulation of serum substrate availability indicated that medium chain triglycerides alone did not improve myocardial metabolism, but that intravenous glucose or oral carbohydrate loading raised the myocardial PCr/Pi ratio from 1.0 ± 0.05 to 1.8 ± 0.1 (p < 0.01) without significantly affecting the PCr/Pi value of her resting skeletal muscle. This study demonstrates the feasibility of using P-31 nuclear magnetic resonance to evaluate the biochemistry of the human myocardium in vivo and to diagnose a metabolic abnormality. Phosphorus-31 nuclear magnetic resonance can thus be used to optimize therapy for human disease.

Original languageEnglish (US)
Pages (from-to)745-749
Number of pages5
JournalJournal of the American College of Cardiology
Volume5
Issue number3
DOIs
StatePublished - Jan 1 1985

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ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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