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Heart transplantation for restrictive cardiomyopathy: development of cardiac amyloidosis in preexisting monoclonal gammopathy.

A 54-year-old woman died 6 months after heart transplantation for treatment of restrictive cardiomyopathy. A monoclonal gammopathy without other signs of malignant disease was present preoperatively, and up to 6 weeks before transplantation no evidence of amyloidosis was established in the rectal, bone marrow, and cardiac specimens. At autopsy there was amyloidosis type AL in the kidneys, bone marrow, liver, spleen, recipient atrium, and donor heart. Retrospectively, the explanted heart also revealed amyloidosis. We conclude that in patients undergoing heart transplantation for treatment of restrictive cardiomyopathy with a preexisting monoclonal gammopathy, a thorough evaluation, including multiorgan biopsy for amyloidosis with electron microscopic workup, should be performed.

Amyloidosis↗

Postnatal development of cardiac and respiratory control following prenatal drug exposure.

Of primary importance in the consideration of animal models of prenatal drug exposure is the recognition of interdependence among physiologic systems, the pervading influence of sleep and waking states, and the potential interaction between drug-induced structural changes and physiologic function. It is difficult for a drug effect on the cardiovascular system not to modify respiratory pattern; any change in blood pressure, systemic perfusion, or heart rate is immediately reflected in breathing; respiratory pattern, in turn, through negative thoracic pressure and other sensory activity, can alter cardiac patterning. The respiratory system itself is one component of a somatic motor system, and neural motor control structures undergo substantial modification by drug action. Enhancing or diminishing any aspect of interaction is the influence of sleep states, which nonlinearly can alter relationships between physiologic systems, occasionally dissociating systems, as in the case of temperature effects on breathing during REM sleep. Finally, drug-induced structural changes that alter morphology of the upper airway can result in obstructive respiratory events, leading to pronounced cardiovascular sequelae.

Child, Preschool↗