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

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.

Spontaneous closure of an isolated congenital perimembranous ventricular septal defect in two dogs

“…In addition to these mechanisms, in the case of perimembranous VSDs, when the defect is in close proximity to the tricuspid and aortic valves, reduplication of the tricuspid valve tissue and prolapse of an aortic valve leaflet have been described as the cause of spontaneous VSD closure [ 12 ]. The way in which turbulent blood flow results in a jet lesion and consequently a proliferation of endocardial fibrous tissue is similar to the mechanism described in the pathogenesis of double-chambered right ventricle and discrete subaortic stenosis [ 16 ]. The cause of closure of the VSDs in the present report cannot be known as both dogs remained alive and post-mortem examination has yet to be performed.…”

Genomic and Proteomic Analysis of Patients with Reoccurring Discrete Subaortic Stenosis

“…Tryptic peptides were separated in a reverse-phase C18 column in a pipet tip. Peptides were eluted and separated into fifteen fractions using a stepwise gradient of increasing acetonitrile (2,4,6,8,10,12,14,16,18,20,22,24, 26, 28, 30% Acetonitrile) at pH 10 then combined to five fractions (2+12+12, 4+14+24, 6+16+26, 8+18+28, 10+20+30) and vacuum dried. The dried peptide samples were resuspended with 5% methanol, 0.1% formic acid in water and analyzed on Orbitrap Fusion mass spectrometers (Thermo Fisher Scientific) coupled with an Easy-nLC 1000 nanoflow LC system (Thermo Fisher Scientific) using an in-housed trap column packed with 1.9 μm Reprosil-Pur Basic C18 beads (2 cm × 100 μm) and a 5 cm × 150 μm capillary separation column packed with 1.9 μm Reprosil-Pur Basic C18 beads with 75-min discontinuous gradient of 4-26% acetonitrile, 0.1% formic acid at a flow rate of 800 nl/min.…”

“…Discrete sub-aortic stenosis (DSS) is a congenital heart condition in which a fibrous membrane forms in the left ventricular outflow track (LVOT) of patients 1, 2 . This condition accounts for 6% of all congenital heart disorders and 8-30% of all cases of LVOT obstruction in the pediatric population 3, 4 .…”

Progressive Right Ventricular Obstruction Caused by a Double-Chambered Right Ventricle Resulting in Shunt-Reversal via a Concomitant Congenital Ventricular Septal Defect and Subsequent Erythrocytosis in a Dog

“…Double-chambered right ventricle (DCRV) is characterized by a discrete muscular band and/or fibrous tissue within the right ventricular chamber, leading to abnormal septation and an obstruction to blood flow [ 4 , 5 , 6 ]. Although DCRV is often listed among congenital heart diseases, it is more of a development anomaly with a congenital substrate, similar to subaortic stenosis [ 4 , 5 , 6 ]. Though VSD is among the five most common congenital heart diseases both in dogs and humans, DCRV is rare in both species [ 5 , 6 , 7 , 8 , 9 ].…”

“…Interestingly, the simultaneous presence of a DCRV and a VSD in the same heart is relatively common, where the high-velocity jet resulting from the left-to-right shunting VSD-flow is thought to lead to the tissue proliferation in the right ventricle, contributing to the development of a DCRV (also known as Gasul phenomenon) and a progressive right ventricular outflow tract obstruction [ 7 , 8 , 9 ]. Depending on the severity of the obstruction and the anatomical localization of the VSD, the obstruction caused by DCRV can increase the right ventricular pressure to such a degree that a supra-systemic systolic pressure develops, and flow reversal via the VSD takes place, resulting in an intracardiac right-to-left shunting [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. A right-to-left shunting VSD can cause hypoxemia and on long-term also erythrocytosis because of increased renal erythropoietin production [ 13 ].…”