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

• Published in: Journal of cardiac surgery Vol. 36; no. 8; pp. 2850 - 2856
• Main Authors: Papakonstantinou, Nikolaos A., Kanakis, Meletios A., Bobos, Dimitrios, Giannopoulos, Nicholas M.
• Format: Journal Article
• Language: English
• Published: United States 01.08.2021
• Subjects: acquired heart disease; congenital heart disease; discrete subaortic stenosis; double‐chambered right ventricle; left ventricular outflow tract obstruction; right ventricular outflow tract obstruction; congenital heart disease; double-chambered right ventricle; right ventricular outflow tract obstruction; discrete subaortic stenosis; acquired heart disease; left ventricular outflow tract obstruction
• ISSN: 0886-0440; 1540-8191
• DOI: 10.1111/jocs.15588

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.