Síndrome de QT largo congénito y bradicardia fetal: la importancia del diagnóstico / Congenital long QT syndrome and fetal bradycardia: the importance of the diagnosis

Antecedentes: El síndrome de QT largo es una canalopatía que afecta a la repolarización ventricular y aumenta el riesgo de sufrir arritmias ventriculares graves. Puede ser congénito o adquirido, y es una causa conocida de muerte súbita. Caso clínico: Gestante primigesta, de 28 años, sin antecedentes de interés. En ecografías prenatales se objetivó en el feto bradicardia sinusal mantenida desde la semana 28, sin repercusión hemodinámica, que persistió hasta la finalización de la gestación (semana 37+3). Al nacimiento se realizaron electrocardiogramas seriados que mostraron alteraciones en la repolarización con alargamiento del intervalo QT corregido. Se realizó estudio genético que confirmó síndrome de QT largo tipo 1 y se inició tratamiento oral con beta-bloqueantes, con buena respuesta. Conclusiones: El síndrome de QT largo suele diagnosticarse posnatalmente. Es importante conocer sus características clínicas prenatales para poder establecer un diagnóstico precoz y minimizar así el riesgo de muerte súbita de estos pacientes.

Background: Long QT syndrome is a channelopathy that affects ventricular repolarization and increases the risk of severe ventricular arrhythmias. It can be congenital or acquired, and is a known cause of sudden cardiac death. Case report: A 28-year-old primigravida with no significant medical history. Prenatal ultrasounds revealed sustained fetal sinus bradycardia from week 28, without hemodynamic repercussion, which persisted until the end of gestation (at 37+3 weeks). Serial electrocardiograms were performed after birth, showing repolarization abnormalities with prolonged corrected QT interval. A genetic study confirmed long QT syndrome type 1, and oral treatment with beta-blockers was initiated, showing a positive response. Conclusions: Long QT syndrome is often diagnosed postnatally. It is important to be aware of his prenatal clinical features in order to establish an early diagnosis and minimize the risk of sudden death in these patients.

Syncope due to non-sustained episodes of Torsade de Pointes associated to androgen-deprivation therapy use: a case presentation.

BACKGROUND: Abiraterone is a medication frequently used for metastatic castrate-resistant prostate cancer. We report a case of non-sustained episodes of TdP associated with severe hypokalemia due to androgen-deprivation therapy. Few case presentations describe this association; the novelty lies in the potentially lethal cardiovascular events among cancer patients receiving hormonal therapy. CASE PRESENTATION: A 70-year-old male presented with recurrent syncope without prodrome. ECG revealed frequent ventricular ectopy, non-sustained episodes of TdP, and severe hypomagnesemia and hypokalemia. During potassium and magnesium infusion for repletion, the patient underwent temporary transvenous atrial pacing. As part of the work-up, coronary angiography revealed a mild coronary artery disease, and transthoracic echocardiogram showed a moderately depressed ejection fraction. After electrolyte disturbances were corrected, the QT interval normalized, and transvenous pacing was no longer necessary. Abiraterone was discontinued during the admission, and the patient returned to baseline. CONCLUSIONS: Cancer treatment is complex and requires a multidisciplinary approach. We presented a case of non-sustained TdP associated with androgen-deprivation therapy in an elderly patient with mild coronary artery disease and moderately reduced ejection fraction. Close follow-up and increased awareness are required in patients with hormonal treatment, especially in the setting of other cardiovascular risk factors.

Recommendations for the measurement of the QT interval during the use of drugs for COVID-19 infection treatment. Updatable in accordance with the availability of new evidence.

COVID-19 infection has shown rapid growth worldwide, and different therapies have been proposed for treatment, in particular, the combination of immune response modulating drugs such as chloroquine and hydroxychloroquine (antimalarials) alone or in combination with azithromycin. Although the clinical evidence supporting their use is scarce, the off label use of these drugs has spread very quickly in face of the progression of the epidemic and the high mortality rate in susceptible populations. However, these medications can pathologically prolong the QT interval and lead to malignant ventricular arrhythmias such that organized guidance on QT evaluation and management strategies are important to reduce morbidity associated with the potential large-scale use.

Effects of metformin on QT and QTc interval dispersion of diabetic rats.

BACKGROUND: Several drugs can cause prolonged QT interval, as well as prolonged QT dispersion (QTd) in electrocardiographic (EKG) recordings. QTd may be a potentially sensitive marker of increased risk of cardiac arrhythmias and sudden cardiac death. Metformin is an effective antihyperglycemic agent used in the treatment of diabetes. However, studies have correlated dose-dependent effects of metformin on glycemia and cardiovascular risk markers. OBJECTIVE: To evaluate the dose-response effects of metformin on QT and QTd of diabetic rats. METHODS: Male Wistar rats were distributed in five groups: non-treated control (C), non-treated diabetics (D), diabetics treated with metmorfin at the doses of 3.5, 30 and 74 microg/kg/bw (DM 3.5, DM 30 and DM 74). Diabetes was induced by an alloxan injection (40 mg/kg, IV). EKG was recorded (days 1, 15 and 30) using four electrodes inserted into the subcutaneous layer of the paws. Both RR and QT intervals were measured, and then corrected QT and QT dispersion values were calculated. RESULTS: The DM 3.5 and DM 30 groups showed a significant reduction of glycemia (p< 0.05) when compared with the high dose (DM 74). Rats of the DM 74 group presented prolonged QTc, QTd and QTcd intervals, whereas rats of the DM 3.5 and DM 30 groups presented less prolonged intervals. CONCLUSION: Metformin at high doses provided greater dispersion of the QT interval probably because of the increased ventricular repolarization inhomogeneity, whereas at low doses decreased QT intervals were observed in diabetic rats.

Presentacion de un niño con síndrome de QT largo / Long-QT syndrome: a case report

El síndrome de QT largo es una enfermedad caracterizada por cambios a nivel electrocardiográficos en la repolarización,prolongación del intervalo QT( corregido para la frecuencia cardíaca)síncope y muerte súbita.En su forma congénita,múltiples mutaciones han sido descriptas en 5 diferentes genes,responsables delas alteraciones en la expresión de los canales iónicos de sodio y potasio.Debido a la creciente aparición de la forma adquirida,asociada a un importante número de fármacos utilizados en la práctica pediátrica habitual,este síndrome ha despertado recientemente considerable atención en las publicaciones internacionales.Tanto en la forma congénita como en la adquirida el síndrome se asocia a una arritmia ventricular rápida polimorfa,denominada torsión de punta(torsades des pointes) que en no pocos casos degenera en fibrilación ventricular.EL diagnósticos permite instituir un tratamiento adecuado y con el seguimiento,disminuir la frecuencia de muerte súbita cardíaca,de alta incidencia en esta población pediátrica particular