Atrial septal defect with intermittent hypoxia due to venous return through a persistent left superior vena cava return draining into a contiguous coronary sinus. Case report.

A case is reported of a 38-month-old female patient with an ostium secundum-type atrial septal defect, 25 mm in diameter, with intermittent hypoxemia and hypoplasia of the right ventricular cavity and tricuspid valve. The right-to-left shunt through the atrial septal defect, responsible for these alterations, is explained by its proximity to the coronary sinus, and exacerbated by a persistent left superior vena cava. The cyanosis disappeared and normal hemodynamic status was achieved after closure of the atrial septal defect.

Atrial septal defect with intermittent hypoxia due to venous return through a persistent left superior vena cava return draining into a contiguous coronary sinus. Case report.

A case is reported of a 38-month-old female patient with an ostium secundum-type atrial septal defect, 25 mm in diameter, with intermittent hypoxemia and hypoplasia of the right ventricular cavity and tricuspid valve The right-to-left shunt through the atrial septal defect, responsible for these alterations, is explained by its proximity to the coronary sinus, and exacerbated by a persistent left superior vena cava. The cyanosis disappeared and normal hemodynamic status was achieved after closure of the atrial septal defect.

[Mild pulmonary valve stenosis: the possible spontaneous cure in the natural history of the defect]. / Estenose pulmonar valvar discreta: a possível cura espontânea na evolução natural do defeito.

OBJECTIVE: To determine the long-term natural history of patients with mild pulmonary valve stenosis. METHODS: Throughout a 24-year evolutive period (1980 to 2004), 83 patients with pulmonary valve stenosis were evaluated, with 29 of them presenting a mild form of the defect. Of these 29, 13 patients had a long-term follow up. The mean age at the first and last follow-up visits was 34 months (1 month to 15 years) and 10.5 years (3 to 24 years), respectively. In addition to evolutive elements, those regarding clinical, electrocardiographic and echocardiographic aspects were also assessed. RESULTS: All patients remained asymptomatic throughout the study. Regarding the pulmonary valve pressure gradient, 5 (38.4%) presented a decrease, 3 (23%) remained unchanged, 4 (30.7%) presented elimination and 1 (7.6%) presented accentuation to a moderate degree of involvement. The mean initial and final gradient was 24.2 mmHg (15-30) and 13.6 mmHg (0-54), respectively. The initial gradients of the 4 patients who achieved spontaneous cure were 30, 19, 30 and 20 mmHg. The systolic murmur and right ventricular overload had a direct correlation to the gradient pressure degree, subdivided at each 10 mmHg. CONCLUSION: The spontaneous cure of the mild pulmonary valve stenosis is possible to achieve, similarly to other acyanogenic anomalies.

Ascending aortic aneurysm after pediatric heart transplantation: case report of an unusual complication.

A 28-month-old boy, weighing 11 kg, with severe dilated cardiomyopathy, was transplanted on December 1995. Hypertension and supraventricular tachycardia were detected in the immediate post-operative period, with favorable outcome. After 5 months of clinically asymptomatic follow-up, a dilation in the ascending aorta was observed on routine echocardiogram. Nuclear magnetic resonance imaging (NMRI) confirmed an ascending aortic aneurysm, with a diameter of 38 mm. An operation was performed, a bovine pericardium patch was sutured with reconstruction of the aortic wall, excluding the aneurysm. Good recovery was obtained and the child was discharged on Day 7 postoperatively. A post-operative echocardiogram showed absence of the aortic aneurysm and good surgical results. Another NMRI was done 5 months later, showing an intact ascending aorta. After 64 months, the patients clinical condition was confirmed as normal by echocardiogram. Surgical treatment was successful and the positive results have been maintained.

[Prevailing right ventricular myocardiopathy for previous myocarditis or arrhythmogenic dysplasia?]. / Miocardiopatia preponderante de ventrículo direito por miocardite prévia ou por displasia arritmogênica?

A clinical case of a 10-year-old male patient is reported. His dilated and prevailing right ventricular myocardiopathy shows diagnostic difficulties between previous myocarditis etiology and arrhythmogenic dysplasia. As the elements are not pathognomonic of one or other cause, the increase of cardiac enzymes in subacute stage maybe tends to the supposition of previous myocarditis. Hence, the questioning that many cases labeled as arrhythmogenic dysplasia can truly correspond to the possibility of evolutional myocarditis. The controversial clinic management is disputable.