Neonatal Cardiomyopathy

  • Wallis G
  • Fricker F
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1. Neonatologists may be unfamiliar with the different causes of hypertrophic and dilated cardiomyopathies. 2. Neonatologists may be unfamiliar with the complex evaluation needed for patients who have hypertrophic or dilated cardiomyopathy. Abstract Cardiomyopathy is a disease that affects the myocardium and causes mechanical or electrical cardiac dysfunction. The clinical presentation of neonates who have cardio-myopathies is varied, as are the possible causes of the condition and the severity of disease presentation. In our experience, the best approach for evaluating a neonate who has a potential cardiomyopathy is to identify the possible type of cardiomyopathy and subsequently determine the potential cause(s). This review provides a summary of the clinical, laboratory, radiographic, and electrocardiac findings of an infant who has hypertrophic or dilated cardiomyopathy. This summary also provides a detailed overview of the diseases and syndromes associated with both types of cardiomyopathy. Objectives After completing this article, readers should be able to: 1. Describe the clinical manifestations of a neonate who has dilated or hypertrophic cardiomyopathy. 2. Provide an overview of the laboratory evaluation of a neonate who has dilated or hypertrophic cardiomyopathy. 3. Describe the underlying diseases associated with hypertrophic and dilated cardiomyopathies in a neonate. Case Presentation A full-term neonate was born to a 30-year-old primigravida woman with negative results on serologic tests. The mother had received prenatal care and had normal findings on fetal ultrasound evaluations. The male infant was born by vaginal delivery and was brought to the radiant warmer. He had decreased respiratory effort requiring bag-mask ventilation. He appeared mottled and hypotonic and had Apgar scores of 5, 5, and 8 at 1, 5, and 10 minutes, respectively. Because of continued poor respiratory effort, the neonatology team intubated the infant and brought him to the NICU for further evaluation and management. On arrival to the NICU, the infant had a right arm blood pressure of 70/40 mm Hg, which was similar to the blood pressures in his other extremities. He continued to appear mottled with weak distal pulses. He had a hyperactive precordium without mur-mur or gallop, and his breath sounds were clear. A chest radiograph showed significant cardiomegaly without lung disease or pleural effusions. As a result of these findings, the NICU team requested a cardiology consultation. Echo-cardiography revealed that the infant had cardiomyopathy with severe left ventricular dys-function and severe ventricular dilation.

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  • Gonzalo Wallis

  • Frederick Jay Fricker

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