Heart disease and pregnancy: State of the art. / Cardiopatia e gravidez ­ o estado da arte.

The association between heart disease and pregnancy is increasingly prevalent. Although most women with heart disease tolerate the physiological changes of pregnancy, there are heart conditions that manifest for the first time during pregnancy and others that totally contraindicate a pregnancy. It is therefore important to establish multidisciplinary teams dedicated to the management of women with heart disease who intend to become, or who already are, pregnant. The aim of this article is to systematically review current knowledge on the approach to women with high-risk cardiovascular disease during pregnancy.

Hypercalcemic crisis and primary hyperparathyroidism: Cause of an unusual electrical storm. / Crise hipercalcémica e hiperparatiroidismo primário: causa de tempestade arrítmica invulgar.

Hypercalcemia is a known cause of heart rhythm disorders, however its association with ventricular arrhythmias is rare. The authors present a case of a fifty-three years old male patient with a ischemic and ethanolic dilated cardiomyopathy, and severely reduced ejection fraction, carrier of cardiac resynchronization therapy (CRT) with cardioverter defibrillator (ICD), admitted in the emergency department with an electrical storm, with multiple appropriated ICD shocks, refractory to antiarrhythmic therapy. In the etiological investigation was documented severe hypercalcemia secondary to primary hyperparathyroidism undiagnosed until then. Only after the serum calcium level reduction ventricular tachycardia was stopped.

[Novel mutation in long QT syndrome in a patient with prior diagnosis of epilepsy]. / Nova mutação na síndroma de QT Longo em doente com diagnóstico prévio de epilepsia.

Congenital long QT syndrome (LQTS) can present as syncope or seizures, secondary to polymorphic ventricular tachycardia, mimicking a primary seizure disorder. In patients treated with an implantable cardioverter-defibrillator (ICD), the recurrence of arrhythmias with subsequent frequent therapeutic shocks may cause adverse reactions, which can be psychogenic. We report the case of a 22-year-old woman with syncope and seizures who was diagnosed in childhood as epileptic and in whom LQTS was diagnosed only in adulthood. Beta-blocker therapy failed and an ICD was implanted. However, as arrhythmias persisted, left cardiac sympathetic denervation was performed. After surgery, three-month follow-up showed a significant reduction in arrhythmias. The genetic study identified a heterozygous mutation, c.1817 C>T p.S606F, on the KCNH2 gene that has not previously been reported in the literature. We also report the rare occurrence of an electrical storm in the course of H1N1 infection. This case illustrates the difficulties in the diagnosis and treatment of LQTS. The possibility of a common genetic basis for arrhythmic diseases and epilepsy is discussed.

Electrical storm induced by H1N1 A influenza infection.

Pandemic H1N1 influenza A virus is spreading worldwide. We report a case of electrical storm during H1N1 influenza A infection in a patient with congenital long-QT syndrome (LQTS) that was previously stable on beta-blocker therapy. Possible causes for the association of A flu and LQTS recurrent ventricular arrhythmias are discussed.