Congenital diverticulum of the right ventricle associated with coarctation of aorta, atrial and ventricular septal defect and ductus.

Congenital right ventricular muscular diverticula are extremely rare and are usually associated with other congenital cardiac anomalies, (in half of the cases tetralogy of Fallot). They functionally behave like an accessory ventricular chamber which contracts synchronously with the normal ventricles. Less than 30 patients with a right ventricular diverticulum have been reported in literature. An apical right ventricular diverticulum occurs in patients with thoraco-abdominal midline defects or abnormalities of the cardiac position([1]). However, an antero-superior diverticulum is usually associated with other congenital cardiac defects, such as a ventricular septal defect, tetralogy of Fallot, double outlet right ventricle and pulmonary stenosis([2--9]). We report an 11-year-old boy with an antero-superior diverticulum of the right ventricle associated with a coarctation of aorta, ductus arteriosus, and atrial and ventricular septum defects. To the best of our knowledge, such an association has not been reported before.

[Role of adenosine triphosphate (ATP) in head-up tilt-induced syncope]. / Papel del adenosín trifosfato (ATP) en la inducción de síncope mediante test de basculación.

INTRODUCTION AND OBJECTIVES: Recent studies have demonstrated that adenosine is an endogenous modulator of the cardiac excitatory afferent nerves, and could provoke a vasovagal response during head-up tilt test. Isoproterenol has been the drug of choice to increase the sensitivity of this testing. The aim of the present study was to analyze the role of adenosine in head-up tilt-induced syncope in susceptible patients, and to compare the relative sensitivities of adenosine and isoproterenol. METHODS: Thirty patients with unexplained syncope (16 female and 14 male, mean age 37.1 +/- 18 years), no heart disease and negative baseline head-up tilt test were studied. After the baseline test, patients were randomized to receive adenosine triphosphate (bolus injections of 3, 6 and 9 mg/ 5 min) or isoproterenol (bolus injections of 2, 4 and 6 micrograms/5 min) and underwent a second tilt test. After 15 min at rest, patients received the alternative drug and a third test was performed. Eleven normal control subjects were tested with adenosine in the upright position to determine its effects. RESULTS: A vasovagal response was induced in 7 patients (23.3%) after ATP administration. Nine patients (30%) showed a positive response with isoproterenol. Only 2 patients (6.6%) showed a positive response with both drugs. Of the control subjects, one (9%) had a vasovagal response after ATP administration. CONCLUSIONS: We conclude that adenosine triphosphate seems to be a useful tool to provoke vasovagal reaction in susceptible patients during head-up tilt test.

[Long-term prognosis of patients with syncope of unknown origin in prolonged asystole induced by the head-up tilt test]. / Pronóstico a largo plazo de pacientes con síncope de origen desconocido y asistolia prolongada inducida mediante test de basculación.

INTRODUCTION AND OBJECTIVES: Prognosis and therapeutic assessment of patients with syncope and prolonged asystole during head-up tilt test remain unclear. The aim of the present study was to analyze the clinical evolution of patients with syncope of unknown origin, no heart disease and severe cardioinhibitory response induced by head-up tilt. METHODS: A prospective follow-up study was performed in 12 patients (6 male and 6 female, mean age 31 +/- 20 years) with recurrent syncope, no heart disease and affected by severe cardioinhibitory syncope induced by head-up tilt test. This was defined as syncope or near-syncope induced by baseline or isoproterenol tilt with asystole of > or = 3 seconds. All patients were re-tilted twice: with salt and fluid and with metoprolol (25 mg/b.i.d). According to the results of these tests, 5 patients were discharged with dietetic measures (salt & fluid) and 5 with metoprolol. In 2 patients who showed recurrent prolonged asystole a DDD pacemaker was implanted. RESULTS: After follow-up of 34 +/- 20 months all patients ae alive. The number of recurrences was small (2 syncopes and 2 near-syncopes). No relationship was observed between the number of syncopal recurrences and the applied treatment. CONCLUSIONS: We conclude that prolonged asystole induced by head-up tilt test does not confer an adverse prognosis in patients with syncope of unknown origin and no heart disease, thus, the clinical evolution of these patients is benign.