Background: The etiology of isolated right ventricular hypertrophy (RVH) is distinct from other forms of hypertrophic cardiomyopathy. RVH is typically seen in the setting of pulmonary valve stenosis or Tetralogy of Fallot. A rare cause of isolated RVH is premature closure of the patent ductus arteriosus (PDA) in utero that results in pulmonary hypertension. This can have a range of outcomes, from spontaneous resolution to fetal demise.
Methods: This case report describes a term infant who presented with respiratory distress and striking isolated RVH, pulmonary hypertension, and no PDA. She was treated conservatively with supplemental oxygen.
Results: The patient was gradually weaned off oxygen over the course of two weeks and follow-up echocardiography showed resolution of her RVH and pulmonary hypertension by 14 weeks of age.
Conclusions: The presentation and course of this patient with severe isolated RVH is consistent with spontaneous premature closure of the ductus arteriosus in utero.
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