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Cardiac valvular defect, developmental(CVDP1)

MedGen UID:
1823949
Concept ID:
C5774175
Disease or Syndrome
Synonyms: CARDIAC VALVULAR DYSPLASIA 1; CVDP1
 
Gene (location): PLD1 (3q26.31)
 
Monarch Initiative: MONDO:0008913
OMIM®: 212093

Definition

Cardiac valvular dysplasia-1 (CVDP1) is characterized by congenital malformations of the pulmonic, tricuspid, and mitral valves. Structural cardiac defects, including atrial and ventricular septal defects, single left ventricle, and hypoplastic right ventricle have also been observed in affected individuals (Ta-Shma et al., 2017). Genetic Heterogeneity of Cardiac Valvular Dysplasia CVDP2 (620067) is caused by mutation in the ADAMTS19 gene (607513) on chromosome 5q23. [from OMIM]

Clinical features

From HPO
Hydronephrosis
MedGen UID:
42531
Concept ID:
C0020295
Disease or Syndrome
Severe distention of the kidney with dilation of the renal pelvis and calices.
Urethral diverticulum
MedGen UID:
57776
Concept ID:
C0152443
Disease or Syndrome
The presence of a diverticulum (sac or pouch) in the wall of the urethra.
Hydroureter
MedGen UID:
101073
Concept ID:
C0521620
Anatomical Abnormality
The distention of the ureter with urine.
Patent foramen ovale
MedGen UID:
8891
Concept ID:
C0016522
Congenital Abnormality
Failure of the foramen ovale to seal postnatally, leaving a potential conduit between the left and right cardiac atria.
Atrial septal defect
MedGen UID:
6753
Concept ID:
C0018817
Congenital Abnormality
Atrial septal defect (ASD) is a congenital abnormality of the interatrial septum that enables blood flow between the left and right atria via the interatrial septum.
Ventricular septal defect
MedGen UID:
42366
Concept ID:
C0018818
Congenital Abnormality
A hole between the two bottom chambers (ventricles) of the heart. The defect is centered around the most superior aspect of the ventricular septum.
Mitral regurgitation
MedGen UID:
7670
Concept ID:
C0026266
Disease or Syndrome
An abnormality of the mitral valve characterized by insufficiency or incompetence of the mitral valve resulting in retrograde leaking of blood through the mitral valve upon ventricular contraction.
Mitral valve prolapse
MedGen UID:
7671
Concept ID:
C0026267
Disease or Syndrome
One or both of the leaflets (cusps) of the mitral valve bulges back into the left atrium upon contraction of the left ventricle.
Mitral stenosis
MedGen UID:
44466
Concept ID:
C0026269
Disease or Syndrome
An abnormal narrowing of the orifice of the mitral valve.
Tricuspid regurgitation
MedGen UID:
11911
Concept ID:
C0040961
Disease or Syndrome
Failure of the tricuspid valve to close sufficiently upon contraction of the right ventricle, causing blood to regurgitate (flow backward) into the right atrium.
Tricuspid valve prolapse
MedGen UID:
11912
Concept ID:
C0040962
Disease or Syndrome
One or more of the leaflets (cusps) of the tricuspid valve bulges back into the right atrium upon contraction of the right ventricle.
Tricuspid stenosis
MedGen UID:
21678
Concept ID:
C0040963
Disease or Syndrome
A narrowing of the orifice of the tricuspid valve of the heart.
Left atrial enlargement
MedGen UID:
536845
Concept ID:
C0238705
Finding
Increase in size of the left atrium.
Tricuspid atresia
MedGen UID:
67034
Concept ID:
C0243002
Congenital Abnormality
Failure to develop of the tricuspid valve and thus lack of the normal connection between the right atrium and the right ventricle.
Pulmonary artery atresia
MedGen UID:
82723
Concept ID:
C0265908
Congenital Abnormality
A congenital anomaly with a narrowing or complete absence of the opening between the right ventricle and the pulmonary artery.
Double inlet left ventricle
MedGen UID:
488862
Concept ID:
C0344622
Congenital Abnormality
The condition in which both atria are joined to the left ventricle each by its own atrioventricular valve. Usually there is a hypoplastic right ventricle, which may be on the opposite side of the heart as usual.
Muscular ventricular septal defect
MedGen UID:
473253
Concept ID:
C0685707
Congenital Abnormality
The trabecular septum is the largest part of the interventricular septum. It extends from the membranous septum to the apex and superiorly to the infundibular septum. A defect in the trabecular septum is called muscular VSD if the defect is completely rimmed by muscle.
Valvular pulmonary stenosis
MedGen UID:
348595
Concept ID:
C1860336
Congenital Abnormality
A cardiovascular malformation associated with narrowing of the outflow tract of the right ventricle immediately at the level of the pulmonary valve.
Pulmonic stenosis
MedGen UID:
408291
Concept ID:
C1956257
Disease or Syndrome
A narrowing of the right ventricular outflow tract that can occur at the pulmonary valve (valvular stenosis), below the pulmonary valve (infundibular stenosis), or above the pulmonary valve (supravalvar stenosis).
Arteria lusoria
MedGen UID:
1372352
Concept ID:
C2004632
Congenital Abnormality
Usually, three large arteries arise from the arch of the aorta
Subpulmonary stenosis
MedGen UID:
757434
Concept ID:
C3165028
Disease or Syndrome
A cardiovascular malformation associated with narrowing of the outflow tract of the right ventricle immediately below the pulmonary valve.
Left aortic arch with cervical origin of the right subclavian artery
MedGen UID:
868870
Concept ID:
C4023281
Anatomical Abnormality
Hypoplasia of right ventricle
MedGen UID:
903846
Concept ID:
C4082954
Anatomical Abnormality
Underdevelopment or reduced size of the heart right ventricle, often due to a reduced number of cells.
Inguinal hernia
MedGen UID:
6817
Concept ID:
C0019294
Finding
Protrusion of the contents of the abdominal cavity through the inguinal canal.
Edema
MedGen UID:
4451
Concept ID:
C0013604
Pathologic Function
An abnormal accumulation of fluid beneath the skin, or in one or more cavities of the body.
Cyanosis
MedGen UID:
1189
Concept ID:
C0010520
Sign or Symptom
Bluish discoloration of the skin and mucosa due to poor circulation or inadequate oxygenation of arterial or capillary blood.
Hydrops fetalis
MedGen UID:
6947
Concept ID:
C0020305
Disease or Syndrome
The abnormal accumulation of fluid in two or more fetal compartments, including ascites, pleural effusion, pericardial effusion, and skin edema.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Yi JS, Perla S, Bennett AM
Cardiovasc Drugs Ther 2023 Dec;37(6):1193-1204. Epub 2022 Feb 14 doi: 10.1007/s10557-022-07324-0. PMID: 35156148
Hammerness PG, Cassidy AR, Potts H, Richardson A, Fogler J, Daly KP, Augustyn M
J Dev Behav Pediatr 2021 May 1;42(4):340-342. doi: 10.1097/DBP.0000000000000961. PMID: 33859122
Ohlow MA
Cardiology 2006;106(2):63-72. Epub 2006 Apr 12 doi: 10.1159/000092634. PMID: 16612072

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