A 23-year-old non-dysmorphic female was admitted for congestive heart failure. She was diagnosed with mitral stenosis, congenital hypopituitarism confirmed by genetic tests (defect of gene encoding growth hormone secretion [GH1]; defect of gene encoding pituitary cell differentiation [PROP1]) and coeliac disease. Echocardiography and cardiac computed tomography (CT) showed a congenital mitral arcade causing severe stenosis [1–5]. Mitral arcade has some anatomical features in common with the parachute valve, such as deformed and restricted leaflet mobility and underdeveloped commissures. On the other hand, the arcade has two thick papillary muscles and elongated chordae tendinae, whereas the parachute valve presents one papillary muscle and short and thick chordae tendinae. The valve was replaced with a St Jude 27 prosthesis, because leaflets were unsuitable for successful repair. A mechanical valve was implanted as pregnancies are very unlikely due to congenital hypopituitarism. At one year of follow-up, the patient is in New York Heart Association (NYHA) functional class I.
Mitral Arcade Causing Severe Stenosis in an Adult Patient
D'Errico Ramirez A.;Milano A. D.
2022-01-01
Abstract
A 23-year-old non-dysmorphic female was admitted for congestive heart failure. She was diagnosed with mitral stenosis, congenital hypopituitarism confirmed by genetic tests (defect of gene encoding growth hormone secretion [GH1]; defect of gene encoding pituitary cell differentiation [PROP1]) and coeliac disease. Echocardiography and cardiac computed tomography (CT) showed a congenital mitral arcade causing severe stenosis [1–5]. Mitral arcade has some anatomical features in common with the parachute valve, such as deformed and restricted leaflet mobility and underdeveloped commissures. On the other hand, the arcade has two thick papillary muscles and elongated chordae tendinae, whereas the parachute valve presents one papillary muscle and short and thick chordae tendinae. The valve was replaced with a St Jude 27 prosthesis, because leaflets were unsuitable for successful repair. A mechanical valve was implanted as pregnancies are very unlikely due to congenital hypopituitarism. At one year of follow-up, the patient is in New York Heart Association (NYHA) functional class I.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
