RESUMO
Hemitruncus arteriosus refers to an uncommon congenital cardiovascular abnormality. It usually presents in infancy and leads to the development of pulmonary hypertension, heart failure, and in severe cases maybe death. Herein, we reported hemitruncus arteriosus in a 10-day-old neonate with respiratory distress, patent ductus arteriosus, and thrombocytopenia.
Assuntos
Permeabilidade do Canal Arterial/complicações , Trombocitopenia/complicações , Persistência do Tronco Arterial/complicações , Progressão da Doença , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/fisiopatologia , Permeabilidade do Canal Arterial/terapia , Evolução Fatal , Humanos , Recém-Nascido , Masculino , Síndrome do Desconforto Respiratório do Recém-Nascido/etiologia , Trombocitopenia/diagnóstico , Trombocitopenia/terapia , Persistência do Tronco Arterial/diagnóstico por imagem , Persistência do Tronco Arterial/fisiopatologia , Persistência do Tronco Arterial/terapiaRESUMO
Intrapericardial teratomas are rare primary cardiac tumors. These tumors, albeit benign in essence, can be fatal if they exert pressure on the cardiovascular and/or respiratory system. We describe a 34-day-old infant, who needed emergent surgery due to cardiovascular structure compromise. Proper anesthetic and surgical techniques conferred an uneventful postoperative course. Histologic examination confirmed the tumor as an intrapericardial teratoma. At 8 months' postoperative follow-up, the child had a good developmental status and a normal echocardiogram.
RESUMO
It is shown that phosphodiesterase type 5 (PDE5) inhibitors such as sildenafil can modulate pulmonary arterial hypertension (PAH) via increasing the level of guanosine-3,5-cyclic monophosphate (cGMP) and decreases pulmonary artery pressure (PAP). In this study we determined the effectiveness of sildenafil and compared its efficacy with inhaled nasal oxygen (O2) during cardiac catheterization in patients with congenital heart diseases (CHD) and PAH, as a test of feasibility for surgical repair of the patients. We studied 15 patients, 9 male and 6 female, with a mean age of 8.3 years. Hemodynamic measurements were made at baseline, after O2 administration for 20 min (5 L/min by mask), and then 45 min after administration of a single dose of sildenafil (0.5 mg/kg orally or via nasogastric tube). Mean PAP at baseline was 72.2 +/- 12.54 mm Hg and was reduced by sildenafil to 52.5 +/- 9.6 and by O2 to 61.3 +/- 10.39. Both sildenafil and O2 decreased PAP effectively (p = 0.08 and p = 0.04, respectively). Pulmonary vascular resistance (PVR) was calculated for 12 patients, with a baseline level of 9.08 +/- 1.09 mm Hg . L(-1) . min, which was significantly decreased by O2, to 3.74 +/- 0.43, and by sildenafil, to 5.93 +/- 0.75 (p = 0.005 and p = 0.05, respectively). Sildenafil, as a single oral dose, can effectively reduce PAP and PVR. This novel PDE5 inhibitor can be used for assessment of feasibility of operation for patients with CHD and PAH when inhaled NO is not available.