RESUMEN
Pre-capillary pulmonary hypertension was the presenting sign of a CREST syndrome in a 65 year old woman. The diagnosis of this form of scleroderma is based on the association of a number of features (calcinosis, Raynaud's phenomenon, oesophageal dyskinesia, sclerodactylia and telangectasia). Scleroderma is the systemic disease which is usually complicated by pre-capillary pulmonary hypertension. This complication is observed in about 13% of CREST syndromes, but very rarely as severe pre-capillary pulmonary hypertension. The diagnosis of pre-capillary pulmonary hypertension carries a poor prognosis with a 2 year survival rate of about 40%. Treatment is usually with calcium inhibitors but with no effect on prognosis. The use of prostacycline and its analogue, iloprost, is an interesting therapeutic strategy, currently under evaluation. Cardiopulmonary transplantation is the only treatment of very severe forms, despite the progressive character of the condition. All cases of pre-capillary pulmonary hypertension require complete aetiological investigation to exclude a systemic disease, especially a scleroderma and, above all, a CREST syndrome.
Asunto(s)
Síndrome CREST/complicaciones , Hipertensión Pulmonar/complicaciones , Anciano , Electrocardiografía , Femenino , HumanosRESUMEN
A 32 year-old-man admitted to hospital on the third day of myocardial infarction, three months after aortic valve replacement with a mechanical Medtronic-Hall prosthesis. The embolic character of the disease was evident on coronary angiography which showed a large thrombus in the left main coronary artery. The thrombosis of the valve prosthesis was not detected by non-invasive investigations but at aortography, confirmed at surgery. Thrombolysis with rt-PA did not dissolve the clot and reoperation was necessary (valve replacement with aorto-coronary bypass grafting).
Asunto(s)
Trombosis Coronaria/etiología , Embolia/etiología , Prótesis Valvulares Cardíacas/efectos adversos , Adulto , Válvula Aórtica , Insuficiencia de la Válvula Aórtica/cirugía , Angiografía Coronaria , Puente de Arteria Coronaria , Trombosis Coronaria/diagnóstico por imagen , Trombosis Coronaria/terapia , Embolia/diagnóstico por imagen , Embolia/terapia , Estudios de Seguimiento , Prótesis Valvulares Cardíacas/métodos , Humanos , Masculino , Activadores Plasminogénicos/uso terapéutico , Falla de Prótesis , Reoperación , Activador de Tejido Plasminógeno/uso terapéuticoRESUMEN
The authors report two new cases of congenital quadricuspid aortic valve complicated by pure, severe, chronic, aortic regurgitation. This abnormality was diagnosed fortuitously at transoesophageal echocardiography in one case and at aortography in the other, and confirmed at surgery. This congenital abnormality was associated with superior displacement of the left coronary ostium.