Double outlet right ventricle with anterior and left-sided aorta and subpulmonary ventricular septal defect

Double outlet right ventricle (DORV) is a heterogeneous group of abnormal ventriculoarterial connections where, by definition, both great arteries (pulmonary artery and aorta) arise primarily from the morphologically right ventricle. This condition affects 1-1.5% of the patients with congenital heart diseases, with a frequency of 1 in each 10,000 live births. We report the case of an 18-day-old infant with DORV and extremely rare anatomical features, such as anterior and left-sided aorta and subpulmonary ventricular septal defect (VSD). In addition to the anatomic features, the role of the echocardiogram for guiding the diagnosis and the surgical therapy of this congenital heart disease are discussed.

Absence of the aortic valve associated with hypoplastic left-sided heart syndrome

In this report we describe the twelveth case in the literature of absence of the aortic valve cusps, associated with hypoplastic left-sided heart syndrome in a neonate. Clinical and hemodynamic conditions in our patient resemble the classical features of this syndrome except for a greater development of the ascending aorta and the left ventricular cavity, due to aortic insufficiency. A patch was unsuccessfully inserted at the aortic annulus to exclude the left ventricle from the circulation. In addition the Norwood operation was performed.

The Brugada syndrome. Outcome of one case

The Brugada syndrome is a rare condition, and due to its mutating manner of presentation it may be difficult to diagnose. We report one case and discuss the diagnostic aspects and the clinical outcome of one patient with characteristic findings of this syndrome. These findings are especially defined by J-ST elevation in the right leads of serial electrocardiographic records, wide oscillations of J points and ST segments during 24-hour Holter monitoring, and nocturnal sudden death. We stress the importance of the Holter monitor findings for diagnostic complementation. Through this method it is possible to establish a correlation between vigil activities and sleep and the variability of the degree of impairment in ventricular repolarization.

Pregnancy after cardiac transplantation. Report of one case and review

A 14-year-old female patient became pregnant 6 years after heart transplantation. The pregnancy evolved uneventfully, and the newborn infant was healthy. Five months after delivery, the mother was in good condition with preserved ventricular function, and the baby had normal neuro-psychomotor development. Even though the case reported here was a success, pregnancy following cardiac transplantation is considered a high-risk condition and remains contraindicated.

One-year follow-up after primary coronary intervention for acute myocardial infarction in diabetic patients: a substudy of the STENT PAMI trial

OBJECTIVE - This analysis was undertaken to determine the composite incidence of cumulative adverse events (death, reinfarction, disabling stroke, and target vessel revascularization) at the end of the first year after acute myocardial infarction, in diabetic patients who underwent coronary stenting or primary coronary balloon angioplasty. METHODS - From the STENT PAMI trial, we analyzed the 6-month angiographic and 1-year clinical outcomes of 135 diabetic (112, noninsulin dependent) patients who underwent the randomization process of the trial and compared them with 758 nondiabetic patients. RESULTS - Coronary stenting did not significantly reduce the primary composite clinical end point when compared with PTCA (20 vs. 30%, p=0.2). A significant benefit from stenting was observed in patients with noninsulin dependent diabetes, with a trend toward a lesser need for new revascularization procedures (10 vs. 21%, p<.001), with a significant reduction in the primary composite clinical end point at 1 year (12 vs. 28%, p=. 04). At 6 months, the restenosis rate were significantly reduced only in nondiabetic patients (18 vs. 33%, p<. 001). Diabetic patients had the same restenosis rate (38%) either with stenting or balloon PTCA. CONCLUSIONS - Coronary Stenting in diabetics noninsulin dependent offered a significant reduction in the composite incidence of major clinical adverse events compared with balloon PTCA.

Block of the Mitral-pulmonary Isthmus During Ablation of a Single Left-sided Accessory Pathway Causing Different Patterns of Retrograde Atrial Activation

The case of a 16-year-old patient with atrioventricular tachycardia caused by a single left anterolateral accessory pathway is reported. When the patient underwent radiofrequency ablation, a lesion on the mitral annulus lateral wall produced changes in the retrograde atrial activation pattern determined by that pathway; changes ranged from a delay in depolarization of the annulus posterior portions to full left atrium counterclockwise activation. Such phenomena were probably caused by a block in the isthmus between the annulus and the lower left pulmonary vein ostium. This case illustrates the importance of the mitral-pulmonary isthmus in the process of left atrium activation, an alert to changes induced by its unintentional block during accessory pathway ablation.

Using one-year claim development to chose a large claim reserving technique

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