RESUMO
Obstructive cardiomyopathy has been reported in cases of male and female Turner phenotype with normal chromosomes. We report here a case of a male dwarf with bilateral undescended testes and biventricular obstructive cardiomyopathy. Clinical features included choreoathetoid movements, chorioretinitis, bilateral nystagmus, and unusual red color of the hair, associated with some features of Turner phenotype. Endocrine studies were normal except for a lack of thyrotrophic stimulating hormone (TSH) stimulation. Propranolol removed the gradient during cardiac catheterization and relieved the exertional chest pain clinically.
Assuntos
Cardiomiopatias/complicações , Criptorquidismo/complicações , Nanismo/complicações , Adulto , Nanismo/sangue , Humanos , Masculino , Tireotropina/sangue , Hormônio Liberador de Tireotropina , Síndrome de Turner/complicaçõesRESUMO
An 18-year old white youth presented with severe right heart failure and was found to have an obliterated and funnel-shaped right ventricle, massive tricuspid regurgitation, and mitral regurgitation. The haemodynamic findings were those of cardiomyopathy with obliteration on the right side. He underwent surgery consisting of decortication and peeling off of a thickened right ventricular endocardium, tricuspid valve replacement, and mitral valve repair. After surgery there was pronounced haemodynamic as well as clinical improvement. The pathological picture was that of constrictive endocarditis. We recommend this method of treatment for patients with obliterate cardiomyopathy on either side of the heart.
