The multisystem nature of isomerism: left isomerism complicated by Abernethy malformation and portopulmonary hypertension.
Cardiol Young
; 31(4): 532-540, 2021 Apr.
Article
en En
| MEDLINE
| ID: mdl-33731242
ABSTRACT
Isomerism, also referred to as "heterotaxy" is a complex set of anatomic and functional perturbations. One of the most obvious manifestations of isomerism is the disturbance of organ arrangement, such that the thoracic organs are no longer asymmetric on the left and right. We report the case of a 14-year-old female in whom exercise-induced dyspnea led to a late diagnosis of left isomerism complicated by Abernethy malformation and portopulmonary hypertension. A comprehensive evaluation revealed two anatomic left lungs and hyparterial bronchi, bilateral left atria, an interrupted inferior caval vein with azygos continuation, multiple spleens, sinus node dysfunction, hepatic hypertrophy with focal nodular hyperplasia, and absence of the portal vein. Pulmonary vasodilator therapy was initiated resulting in clinical improvement. This case exhibits unique features including a late diagnosis of isomerism with Abernethy malformation and portopulmonary hypertension. The patient's presentation, medical workup, and future treatment emphasise the importance of multidisciplinary care in children with complex multisystem disease. We review the multiple cardiac and extracardiac manifestations of isomerism.
Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Malformaciones Vasculares
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Síndrome de Heterotaxia
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Hipertensión
Tipo de estudio:
Diagnostic_studies
Límite:
Adolescent
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Child
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Female
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Humans
Idioma:
En
Revista:
Cardiol Young
Asunto de la revista:
ANGIOLOGIA
/
CARDIOLOGIA
/
PEDIATRIA
Año:
2021
Tipo del documento:
Article
País de afiliación:
Estados Unidos