Congenital, acquired, or both? The only two congenitally based, acquired heart diseases

J Card Surg. 2021 Aug;36(8):2850-2856. doi: 10.1111/jocs.15588. Epub 2021 Apr 28.

Abstract

Discrete subaortic stenosis (DSS) is a type of left ventricular outflow tract obstruction whereas double-chambered right ventricle is a form of right ventricular outflow tract obstruction. Both of these cardiac malformations share lots of similar characteristics which classify them as acquired developmental heart diseases despite their congenital anatomical substrate. Both of them are frequently associated to ventricular septal defects. The initial stimulus in their pathogenetic process is anatomical abnormalities or variations. Subsequently, a hemodynamic process is triggered finally leading to an abnormal subaortic fibroproliferative process with regard to DSS or to hypertrophy of ectopic muscles as far as double-chambered right ventricle is concerned. In many cases, these pathologies are developed secondarily to surgical management of other congenital or acquired heart defects. Moreover, high recurrence rates after initial successful surgical therapy, particularly regarding DSS, have been described. Finally, an interesting coexistence of DSS and double-chambered aortic ventricle has also been reported in some cases.

Keywords: acquired heart disease; congenital heart disease; discrete subaortic stenosis; double-chambered right ventricle; left ventricular outflow tract obstruction; right ventricular outflow tract obstruction.

Publication types

  • Review

MeSH terms

  • Aorta
  • Discrete Subaortic Stenosis*
  • Heart Defects, Congenital* / surgery
  • Heart Septal Defects, Ventricular*
  • Heart Ventricles / diagnostic imaging
  • Humans
  • Ventricular Outflow Obstruction* / etiology
  • Ventricular Outflow Obstruction* / surgery