These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Progression of left ventricular apical hypoplasia-like restrictive cardiomyopathy with severe pulmonary hypertension: Follow-up from fetal stage.
    Author: Sato R, Ishigaki M, Ito H, Mitsushita N, Sakamoto K, Iwamoto Y, Ishido H, Taketazu M, Senzaki H, Masutani S.
    Journal: J Cardiol Cases; 2021 Oct; 24(4):161-164. PubMed ID: 35069941.
    Abstract:
    Left ventricular (LV) apical hypoplasia is a rare restrictive cardiomyopathy subtype with an unclear pathophysiology. LV apical hypoplasia typically presents with elongated right ventricle (RV) wrapping around a truncated and spherical LV with a deficient apex (the "banana-shape" of the RV). Here we report a case of a young boy with apparent LV apical hypoplasia that developed after birth; no "banana-shaped" RV was observed during the fetal period. Moreover, suprasystemic pulmonary hypertension (PH) developed even after a mitral valve replacement was performed for progressive mitral stenosis and regurgitation at 14 months of age. He underwent surgery for the Potts shunt, a shunt between the pulmonary artery and aorta, at 13 years to secure systemic output. His PH ameliorated and his heart failure remained stable for 3 years after the operation. This case indicates that the "banana-shaped" RV seen in this condition is not always congenital but that it can form and develop after birth. Furthermore, this case supports the usefulness of the Potts shunt as a therapeutic option in patients with severe PH due to LV apical hypoplasia. <Learning objective: Left ventricular apical hypoplasia typically presents with elongated right ventricle wrapping around a truncated and spherical left ventricle with a deficient apex. However, this characteristic may not be always congenital and can also form and develop after birth. The Potts shunt, a shunt between the pulmonary artery and aorta, may be a therapeutic option in patients with severe pulmonary hypertension due to left ventricular apical hypoplasia.>.
    [Abstract] [Full Text] [Related] [New Search]