Treatment with intravenous and oral cardiotonics and afterload reduction resulted in markedly improved cardiac function and good recovery in a moribund neonate with spongy cardiomyopathy.
Case Report (n=1)
Medical management with cardiotonics and afterload reduction can successfully improve cardiac function in neonates presenting with cardiogenic shock due to isolated ventricular noncompaction.
Isolated noncompaction of ventricular myocardium is a rare cardiomyopathy, presumed to originate from a developmental abnormality in the evolution of the heart, and resulting in sponge-like myocardium. Isolated ventricular noncompaction can present with a variety of symptoms, but usually includes heart failure. The diagnosis is often made by echocardiography, which reveals a very distinct image of the myocardium, with many deep, confluent recesses and dense trabeculations. We encountered such findings in a moribund neonate presenting with cardiogenic shock with extremely low shortening fractions. After treatment with intravenous and oral cardiotonics, coupled with afterload reduction, we were able to optimize the balance between ventricular filling and myocardial contractility, resulting in markedly improved cardiac function as judged clinically, and as measured by echocardiography. As far as we know, this is one of the youngest patients yet reported to have a good recovery.
Halbertsma et al. (Sun,) conducted a case report in Isolated noncompaction of ventricular myocardium (spongy cardiomyopathy) (n=1). Intravenous and oral cardiotonics with afterload reduction was evaluated on Cardiac function recovery. Treatment with intravenous and oral cardiotonics and afterload reduction resulted in markedly improved cardiac function and good recovery in a moribund neonate with spongy cardiomyopathy.
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