ABSTRACT
Brugada syndrome is a rare arrhythmogenic syndrome associated mainly with pathogenic variants in the SCN5A gene. Right ventricle outflow tract fibrosis has been reported in some cases of patients diagnosed with Brugada syndrome. Pulmonary atresia with an intact ventricular septum is characterized by the lack of a functional pulmonary valve, due to the underdevelopment of the right ventricle outflow tract. We report, for the first time, a 4-year-old boy with pulmonary atresia with an intact ventricular septum who harbored a pathogenic de novo variant in SCN5A, and the ajmaline test unmasked a type-1 Brugada pattern. We suggest that deleterious variants in the SCN5A gene could be implicated in pulmonary atresia with an intact ventricular septum embryogenesis, leading to overlapping phenotypes.
Subject(s)
Brugada Syndrome , NAV1.5 Voltage-Gated Sodium Channel , Pulmonary Atresia , Humans , Pulmonary Atresia/genetics , Pulmonary Atresia/pathology , Male , Brugada Syndrome/genetics , Brugada Syndrome/pathology , Child, Preschool , NAV1.5 Voltage-Gated Sodium Channel/genetics , Heart Defects, Congenital/genetics , Heart Defects, Congenital/pathology , Ventricular Septum/pathologyABSTRACT
Marfan´s syndrome is a multisystemic, autosomal dominant congenital abnormality of variable penetrance that affects the integrity of connective tissue. In the cardiovascular system, the dysfunction of the physiology of the aortic root and the myocardial fibrosis originates non-ischemic cardiomyopathy independent of valve lesions. Few data have been reported on the prevalence of arrhythmias and its impact on heart function. We present a 21-year-old man with Marfan's syndrome and heart failure with frequent supraventricular arrhythmias and aortic root dilation. After ablation in the posteroseptal area of the mitral ring and Tirone David Surgery, there was clinical improvement, the left ventricular ejection fraction increased dramatically from 33% to 46%, the left ventricular end-diastolic volume decreased from 90 ml/m2 to 77 ml/m2 and the NT-proBNP decrease from 1100 pg/mL at 180 pg/mL.
El síndrome de Marfan es una anomalía congénita multisistémica, autosómica dominante y de penetrancia variable que afecta a la integridad del tejido conectivo. En el sistema cardiovascular, también se ha descrito la disfunción de la fisiología de la raíz aórtica y la fibrosis miocárdica que origina una miocardiopatía no isquémica independiente de las lesiones valvulares. Se han comunicado pocos datos sobre la prevalencia de arritmias y su repercusión en la función cardiaca. Presentamos el caso de un varón de 21 años con síndrome de Marfan e insuficiencia cardiaca con frecuentes arritmias supraventriculares y dilatación de la raíz aórtica. Después de la ablación en la zona posteroseptal del anillo mitral y la cirugía de Tirone David, hubo mejoría clínica, la fracción de eyección ventricular izquierda aumentó espectacularmente del 33 al 46%, el volumen telediastólico ventricular izquierdo disminuyó de 90 a 77 mL/m* y el NT-proBNP disminuyó de 1100 a 180 pg/mL.
ABSTRACT
Durante los últimos años, ha cambiado el perfil de los pacientes que pueden someterse a procedimientos de cirugía ambulatoria, en centros quirúrgicos ambulatorios independientes. Es muy importante considerar la disponibilidad, competencias, flujos de atención del personal asistencial, asimismo el estudio integral del paciente, la capacidad resolutiva de dichos centros y las evaluaciones con los indicadores de calidad de atención, para obtener así resultados óptimos. El presente caso fue de un paciente de sexo masculino, de 34 años de edad quien presentó por primera vez fibrilación auricular rápida y sintomática, documentada en el postoperatorio inmediato. Se describe la atención, la estrategia diagnóstica y el tratamiento, ya que requirió cardioversión eléctrica.
In recent years, the profile of patients who can undergo ambulatory surgery procedures in independent ambulatory surgical centers has changed. It is very important to consider the availability, skills and experience of the care staff, as well as the integral study of the patient, the resolution capacity of these centers and evaluations with quality care indicators to obtain optimal results. The present case was of a 34-year-old male, first presentation of rapid and symptomatic atrial fibrillation documented in the immediate postoperative period. Patient care, diagnostic strategy and treatment were described, since the patient required electrical cardioversion.
ABSTRACT
Congenital long QT syndrome (LQTS) represents a group of heart diseases of genetic origin characterized by prolongation of the QT interval and an abnormal T wave on the electrocardiogram (ECG). They can have a dominant or recessive expression, the latter associated with sensorineural deafness. In both cases, its clinical presentation is associated with recurrent syncope and sudden death as a consequence of ventricular tachycardia, specifically Torsades de Pointes. Currently they are classified according to the specific genetic defect, being able to compromise around 16 genes and almost 2000 mutations. It should be suspected in individuals with related symptoms, electrocardiographic findings, and family history. Management is based on the reduction or elimination of symptoms, and concomitantly the prevention of sudden death (SD), in those children with congenital deafness, the management requires the application of the otolaryngologist specialist's own measures. The cardiovascular management implies the modification of lifestyles, mainly the prohibition of competitive sports, including swimming, avoiding exposure to loud sounds or triggers. The medications used include beta-blockers, and more rarely flecainide, ranozaline, and verapamil; invasive management consists of the implantation of a cardioverter defibrillator or even left sympathetic denervation, each with its own risks and benefits. In any of the cases, we must avoid the circumstances that increase the QT interval, as well as carry out the appropriate analysis of the benefits and risks of each possible invasive measure.
ABSTRACT
Objective: This study aims to describe the clinical, electrophysiological and therapeutic characteristics of pediatric patients with supraventricular tachycardia undergoing radiofrequency ablation. Materials and methods: Observational, descriptive, retrospective study of children admitted for supraventricular tachycardia treatment at the Instituto Nacional de Salud del Niño during 2018 to 2021. Results: Data from 62 procedures were collected, corresponding to 59 patients (mean age: 9.91 years, 61% male), 23% of procedures were performed in patients under 15kg, and two were in patients under 5kg. 63% of patients had a structurally normal heart, while 37% had some type of congenital heart disease, the most common being Ebstein's anomaly. The most widely used group of drugs were beta-blockers, mainly propranolol. Among the arrhythmias treated, 21.7% had preexcitation Syndrome, 33.3% had at least one hidden accessory pathway, 5% corresponded to permanent reciprocating junctional tachycardia, 5% intranodal tachycardia, 11.7% atrial tachycardia, 10 % atrial flutter, 5% had other types of tachycardia and in 5 cases no tachycardia was induced. Of the total procedures, 76% corresponded to electrophysiological study and ablation, four patients recurred. Conclusions: The most common supraventricular tachycardia substrate in children was the presence of some accessory pathway. Radiofrequency catheter ablation could be applied in children with a high success rate and a low complication rate.
ABSTRACT
BACKGROUND: Micra transcatheter pacemaker system (TPS) usually achieves low implant pacing threshold (IPT). However, IPT may increase in some patients during follow-up. AIM: To apply implant parameters in predicting long-term occurrence of very high pacing threshold (VHPT) in patients with Micra-TPS. METHODS: A cohort of 110 consecutive patients implanted with a Micra-TPS from 2014 to 2018 was evaluated at discharge and at 1, 12, 24, 36, and 48 months follow-up. VHPT was defined as greater than 2 V/0.24 ms. VHPT predictors were identified. RESULTS: Micra-TPS was implanted successfully in 108 patients (98.2%). During a mean follow-up of 24 ± 16 months, 18 patients (16.7%) died of causes nonpacemaker-related, and 4 (3.8%) developed VHPT. Patients with VHPT had higher IPT and lower implant impedance than patients with non-VHPT: 1 ± 0.31 vs 0.55 ± 0.29 V/0.24 ms (P = .003) and 580 ± 59 vs 837 ± 232 Ω (P = .03), respectively. IPT and impedance had excellent discriminative power to predict VHPT (area under the curve: 0.85 ± 0.07 and 0.91 ± 0.05, respectively). Negative predictive value (NPV) of IPT ≤ 0.5 V/0.24 ms was 100%; positive predictive value (PPV) was 8% throughout follow-up. Implant impedance ≤ 600 Ω had NPV of 99% throughout follow-up, whereas PPV varied: 16%, 21%, 16%, and 28% at 1, 12, 24, and 36 months, respectively. Sequential combination of IPT greater than 0.5 V/0.24 ms and impedance ≤ 600 Ω improved PPV to 25%, 35%, 27%, and 44%, respectively, whereas NPV remained 99% throughout follow-up. CONCLUSION: Despite favorable long-term electrical performance of Micra-TPS, a small percent of patients developed VHPT during follow-up. A sequential combination of IPT and impedance could allow the implanter to identify patients who will develop VHPT during long-term follow-up.
Subject(s)
Arrhythmias, Cardiac/therapy , Cardiac Pacing, Artificial , Pacemaker, Artificial , Adult , Aged , Aged, 80 and over , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/mortality , Arrhythmias, Cardiac/physiopathology , Cardiac Pacing, Artificial/adverse effects , Cardiac Pacing, Artificial/mortality , Electric Impedance , Electric Power Supplies , Equipment Design , Female , Humans , Male , Middle Aged , Time Factors , Treatment Outcome , Young AdultABSTRACT
BACKGROUND: The Micra transcatheter pacing system (Micra TPS) is often implanted in patients with atrial fibrillation and thus with increased thromboembolic risk. It is unknown whether the use of anticoagulants, associated with the use of a large venous introducer, implies an increased risk of bleeding in this group of patients. OBJECTIVE: The purpose of this study was to assess the incidence of bleeding and thromboembolic complications after Micra TPS implantation with and without therapeutic anticoagulation. METHODS: This single-center observational study included 107 consecutive patients receiving the Micra TPS from 2014 to 2018. At procedure completion, a figure-of-eight suture was placed at the femoral puncture site after sheath withdrawal and was maintained for 24 hours. In patients receiving enoxaparin or new oral anticoagulants, treatment was discontinued 12 or 24 hours before the procedure, respectively, and was reinitiated 4-6 hours postprocedure. In those receiving vitamin K antagonists (VKAs), dosing was not discontinued and the procedure was performed if the international normalized ratio was less than 3. RESULTS: Sixty-four patients (60%) did not receive anticoagulants. Of the 43 (40%) who did, 29 (67%) received VKAs, 8 (19%) received new oral anticoagulants, and 6 (14%) received enoxaparin. Two patients presented hemorrhagic or thromboembolic complications during short-term follow-up: 1 woman receiving VKAs presented hemorrhagic pericardial effusion without tamponade and 1 woman not receiving anticoagulants presented thrombosis of the ipsilateral saphenous vein. CONCLUSION: Bleeding and thromboembolic complications after receiving Micra TPSs are infrequent. The use of anticoagulant therapy, regardless of the type, does not increase the complications associated with the procedure.
Subject(s)
Anticoagulants , Atrial Fibrillation , Catheterization, Peripheral , Hemorrhage , Thromboembolism , Anticoagulants/administration & dosage , Anticoagulants/adverse effects , Anticoagulants/classification , Atrial Fibrillation/blood , Atrial Fibrillation/therapy , Blood Coagulation/drug effects , Catheterization, Peripheral/adverse effects , Catheterization, Peripheral/instrumentation , Catheterization, Peripheral/methods , Female , Hemorrhage/chemically induced , Hemorrhage/diagnosis , Hemorrhage/epidemiology , Humans , Male , Medication Therapy Management , Middle Aged , Outcome and Process Assessment, Health Care , Pacemaker, Artificial , Risk Adjustment/methods , Thromboembolism/epidemiology , Thromboembolism/etiology , Thromboembolism/prevention & controlABSTRACT
AIMS: Riata® implantable cardioverter-defibrillator (ICD) leads from St. Jude Medical are prone to malfunction. This study aimed to describe the rate of this lead's malfunction in a very long-term follow-up. METHODS: This single-centre observational study included 50 patients who received a Riata 7Fr dual-coil lead between 2003 and 2008. Follow-up was conducted both in person and remotely, and analysed at 8-month intervals. We evaluated the rates of cable externalization (CE), electrical failure (EF), and the interaction of these two complications. Structural lead failure was defined as radiographic CE. Oversensing of non-cardiac signal or sudden changes in impedance, sensing, or pacing thresholds constituted EF. RESULTS: During a mean follow-up of 10.2⯱â¯2.9 years, 16 patients (32%) died. We observed lead malfunction in 13 patients (26%): three (23%) due to CE, six (46%) to EF and four (31%) to both complications. Of the malfunctioning leads, 77% failed after seven years of follow-up. The incidence rate (IR) of overall malfunction per 100 patients per year was 0.9 during the first seven years post-implantation, increased to 7.0 after the 7th year and more than doubled (to 16.7) after 10 years. Beyond seven years post-implantation, IR per 100 patient-years increased in both EF and CE (from 0.6 to 5.6 vs. 0.3 to 4.2, respectively). Presence of CE was associated with a 4-fold increase in the proportion of EF. CONCLUSION: The incidence of Riata ICD lead malfunction, both for EF and CE, increased dramatically after seven years and then more than doubled after 10 years post-implantation.
ABSTRACT
El síndrome de Tako-Tsubo es caracterizado por una disfunción transitoria del ventrículo izquierdo (VI), con cambios electrocardiográficos y elevación de enzimas cardiacas que simulan un infarto agudo de miocardio (IAM) en ausencia de enfermedad coronaria obstructiva (ECO). Los reportes de síndrome de Tako-Tsubo luego de una cirugía cardiaca son excepcionales. Se describe el caso clínico de una mujer de 58 años que tras cirugía de reemplazo valvular mitral electiva desarrolla disfunción ventricular severa y shock cardiogénico, su evolución fue favorable con recuperación de la contractilidad hasta la normalidad. El síndrome de Tako-Tsubo debe ser considerado como un diagnostico diferencial en pacientes que presentan shock cardiogénico después de una cirugía cardiaca.
