Valvular Heart Disease

In heart valve defects (cardiac vitias), a distinction is made between heart valve stenoses (valvular stenoses), heart valve weaknesses (valvular insufficiencies), and combined heart valve defects. There are congenital (congenital) and acquired heart defects or valvular defects.

Congenital heart defects or valvular heart defects (HKF)

Congenital heart defects are divided into:

Major congenital heart valve (HKF) defects:

  • Aortic isthmic stenosis (ISTA; synonym: coarctation of the aorta: coarctatio aortae); incidence: 6%; ICD-10-GM Q25.1: coarctation of the aorta) – narrowing of the isthmus aortae (“aortic stenosis“).
  • Atrial septal defect (ASD; atrial septal defect); incidence: 7-10%; ICD-10-GM Q25.1: Coarctation of the aorta) – gap in the septum between the two chambers of the heart.
  • Atrioventricular septal defect (AVSD; combination of a ventricular septal defect (hole in the septum of the ventricles) and an atrial septal defect ((hole in the septum of the atria)) (incidence: 40 per 100. 000 newborns; ICD-10-GM Q21.2: defect of the atrial and ventricular septum); frequent occurrence in children with a chromosomal anomaly in the form of trisomy 2 1 (Down syndrome).
  • Fallot’s tetralogy (also Fallot’s tetralogy) (10-15%; ICD-10-GM Q21. 3: Fallot’s tetralogy) – congenital heart malformation consisting of four components (hence tetralogy): A pulmonary stenosis (narrowing in the outflow tract from the right ventricle to the pulmonary artery), a ventricular septal defect (defect in the ventricular septum), an aorta riding over the ventricular septum, and subsequent right ventricular hypertrophy (pathological enlargement (hypertrophy) of the muscle tissue in the right ventricle of the heart).
  • Persistent ductus arteriosus botalli (synonyms: Ductus arteriosus persistens, persistent ductus arteriosus, PDA; ICD-10-GM Q25.0: patent ductus arteriosus) – this is present when the ductus arteriosus (ductus botalli), which provides a vascular connection between the aorta (the aorta) and the truncus pulmonalis (pulmonary artery) in the fetal (prenatal) circulation, has not closed three months after birth.
  • Pulmonary atresia (ICD-10-GM Q25.5: Atresia of the pulmonary artery) – failure to establish the pulmonary artery.
  • Pulmonary stenosis (frequency: 6%; ICD-10-GM Q24.3: Infundibular pulmonary stenosis) – narrowing of the truncus pulmonalis
  • Transposition of the great arteries (synonym: dextro-transposition (d-transposition) of the great arteries; d-TGA) (frequency: 6%; ICD-10-GM Q20.3: Discordant ventriculoarterial junction) – congenital malformation in which the aorta is connected to the right ventricle of the heart and the pulmonary artery is connected to the left ventricle of the heart
  • Tricuspid atresia (ICD-10-GM Q22.4: Congenital tricuspid valve stenosis) – absence of the tricuspid valve.
  • Ventricular septal defect (VSD; ventricular septal defect; defect of the ventricular septum) (incidence: 15-30%; most common congenital heart defect; ICD-10-GM Q21.0: Ventricular septal defect); four different VSD types are distinguished:
    • Perimembranous VSD: incidence approximately 80%.
    • Muscular VSD: surrounded by muscle tissue all around; when multiple defects are present, it is referred to as “Swiss-cheese” type.
    • Subarterial VSD (synonyms: subpulmonary defect, conus defect, “outlet” VSD): incomplete closure of the conus septum from
    • Inlet VSD (synonym: “AV canal” type): located immediately behind the tricuspid valve (heart valve between the right atrium and the right ventricle); clustered occurrence in trisomy 21.

Acquired heart defects or heart valve defects (HKF)

Acquired cardiac defects are divided into:

  • Valvular stenosis (“valvular stenosis”) with pressure insufficiencies.
  • Valve insufficiencies (“valve leakiness”) with volume loading.
  • Combined heart vitium, ie common occurrence of both forms.

Major acquired valvular heart disease (HKF):

  • Mitral valve stenosis (mitral stenosis; ICD-10-GM I05.0: mitral valve stenosis; ICD-10-GM I34.2: nonrheumatic mitral valve stenosis) (mitral valve stenosis) – most common acquired HKF; affects women more often than men; aortic stenosis is also present in approximately 40% of cases
  • Mitral valve regurgitation (mitral regurgitation, MI; ICD-10-GM I05.8: Other mitral valve diseases; ICD-10-GM I34.0: Mitral valve regurgitation) (leakiness of the mitral valve) – second most common valve disease in adults
  • Mitral valve prolapse (ICD-10-GM I34.1: Mitral valve prolapse) (protrusion of the mitral valve) – occurs in approximately 5% of the adult population; men are more commonly affected than women
  • Aortic valve stenosis (aortic stenosis; ICD-10-GM I35.0: aortic valve stenosis; ICD-10-GM I06.0: rheumatic aortic valve stenosis) (aortic valve stenosis).
  • Aortic valve insufficiency (aortic insufficiency; ICD-10-GM I35.1: aortic valve insufficiency; ICD-10-GM I06.1: rheumatic aortic valve insufficiency) (leakiness of the aortic valve)

The prevalence (disease incidence) for congenital heart defects is 0.8% of all newborns. Depending on the type of heart defect, the prevalence varies from 0.1-0.4% (cor triatriatum) and ventricular septal defect (15-30%). The most common valvular defect is mitral valve prolapse (up to 6%), followed by aortic valve stenosis (5%). Course and prognosis: Usually, valvular defects have a progressive course. If the valvular defect causes symptoms, the prognosis is rather poor. A damaged heart valve can become inflamed, which has an unfavorable effect on the course. In some cases, cardiac arrhythmias develop due to the valvular defect. Due to medical progress, approximately 90% of all people with a congenital heart defect reach adulthood in countries with appropriate intervention options.