Rescue percutaneous repair of ischemic acute severe mitral regurgitation.

Acute severe mitral regurgitation (MR) because of secondary left ventricular impaired regional contractility can present with severe acute heart failure, associated with a high risk for rapid decompensation, pulmonary edema and cardiogenic shock. Frequently, in these highly unstable patients, surgical risk can be prohibitive. Evidence for percutaneous repair of acute MR is scarce, but a few case series show that this approach could be safe and effective for bailing out hemodynamically unstable patients. We report a case of an 84-year-old man with acute ischemic severe MR post-acute myocardial infarction (MI), who remained hemodynamically unstable despite coronary revascularization, positive pressure non-invasive ventilation, vasodilator therapy and intra-aortic balloon pump (IABP) support. In heart team discussions, he was considered a high risk surgical candidate. We decided on rescue off-label percutaneous mitral valve repair with a MitraClip device (Abbott Vascular, Santa Clara, California), with good clinical result, allowing weaning from the supports and discharge seven days after the procedure. At one-year follow-up, the patient maintained a MV repair results and had a good functional status. In unstable patients with acute ischemic MR, percutaneous MV repair could be a rescue therapeutic option to consider, allowing hemodynamic compensation with potential persistent MR improvement up to one-year follow-up.

Impella support for cardiogenic shock and high-risk percutaneous coronary intervention: A single-center experience.

INTRODUCTION AND OBJECTIVES: The use of mechanical circulatory support is increasing in cases of cardiogenic shock (CS) and high-risk percutaneous coronary intervention (HR-PCI). The Impella® is a percutaneous ventricular assist device that unloads the left ventricle by ejecting blood to the ascending aorta. We report our center's experience with the use of the Impella® device in these two clinical settings. METHODS: We performed a single-center retrospective study including all consecutive patients implanted with the Impella® between 2007 and 2019 for CS treatment or prophylactic support of HR-PCI. Data on clinical and safety endpoints were collected and analyzed. RESULTS: Twenty-two patients were included: 12 were treated for CS and 10 underwent an HR-PCI procedure. In the CS-treated population, the main cause of CS was acute myocardial infarction (five patients); hemolysis was the most frequent device-related complication (63.7%). In-hospital, cumulative 30-day and one-year mortality were 58.3%, 66.6% and 83.3%, respectively. In the HR-PCI group, all patients had multivessel disease (mean baseline SYNTAX I score: 44.1±13.7). In-hospital, 30-day and one-year mortality were 10.0%, 10.0% and 20.0%, respectively. There were no device- or procedure-related deaths in either group. CONCLUSION: The short- and long-term results of Impella®-supported HR-PCI were comparable to those in the literature. In the CS group, in-hospital and short-term outcomes were poor, with high mortality and non-negligible complication rates.

A Pleasant Surprise in the Face of ST-Segment Elevation.

ST-segment elevation is a dreadful finding in the emergency department because it is often associated with myocardial infarction and demands a prompt and definitive treatment. However, the clinical and echocardiographic assessment of a patient with electrocardiographic changes trumps any electrocardiology expert and should always lead to a clinical decision. (Level of Difficulty: Intermediate.).

Long-term outcomes after radiofrequency catheter ablation of the atrioventricular node: The experience of a Portuguese tertiary center.

INTRODUCTION: In patients with supraventricular arrhythmias and high ventricular rate, unresponsive to rate and rhythm control therapy or catheter ablation, atrioventricular (AV) node ablation may be performed. OBJECTIVES: To assess long-term outcomes after AV node ablation and to analyze predictors of adverse events. METHODS: We performed a detailed retrospective analysis of all patients who underwent AV node ablation between February 1997 and February 2019, in a single Portuguese tertiary center. RESULTS: A total of 123 patients, mean age 69±9 years and 52% male, underwent AV node ablation. Most of them presented atrial fibrillation at baseline (65%). During a median follow-up of 8.5 years (interquartile range 3.8-11.8), patients improved heart failure (HF) functional class (NYHA class III-IV 46% versus 13%, p=0.001), and there were reductions in hospitalizations due to HF (0.98±1.3 versus 0.28±0.8, p=0.001) and emergency department (ED) visits (1.1±1 versus 0.17±0.7, p=0.0001). There were no device-related complications. Despite permanent pacemaker stimulation, left ventricular ejection fraction did not worsen (47±13% vs. 47%±12, p=0.63). Twenty-eight patients died (23%). The number of ED visits due to HF before AV node ablation was an independent predictor of the composite adverse outcome (OR 1.8, 95% CI 1.24-2.61, p=0.002). CONCLUSIONS: Despite pacemaker dependency, the clinical benefit of AV node ablation persisted at long-term follow-up. The number of ED visits due to HF before AV node ablation was an independent predictor of the composite adverse outcome. AV node ablation should probably be considered earlier in the treatment of patients with supraventricular arrhythmias and HF, especially in cases that are unsuitable for selective ablation of the specific arrhythmia.

Análise da construção da autonomia infantil: interações entre crianças e professoras na educação infantil / Analysis of the construction of child autonomy: interactions between children and teachers in early childhood education / Análisis de construcción autonomía de hijo: interacciones entre los niños y los maestros de educación infantil

Este estudo investigou as interações e afetividade entre professoras-criança e criança-criança e suas implicações para o processo da autonomia infantil. Observou-se a rotina diária de uma turma de Maternal 2, com 26 crianças de 3 anos, uma professora e uma monitora em um CREI - Centro de Referência em Educação Infantil, na cidade de João Pessoa-PB. A técnica empregada foi a de videogravação de episódios interativos. Os dados foram tratados por meio da análise microgenética, em que os episódios interativos foram divididos em 3 (três) tipos de díades: criança-criança, professoras-criança e criança-professoras. Os resultados indicam que as crianças resistiam e construíam sua autonomia entre os pares. É oportuno dizer que encontramos um ambiente com interações intercaladas entre afetivas e proporcionadoras da construção da autonomia (criança-criança) e outras disciplinadoras (professora ou monitora-criança).

This study investigated teacher-child and child-child interactions and affectivity and their implications of the process on child autonomy. It was observed the daily routine of a class of Kindergarden 2 with 26 children around 3 year old, a teacher and a monitor in a CREI - Reference Center in Early Childhood Education - in the city of João Pessoa-PB. The technique employed was the video recording of interactive episodes. The data were treated using microgenetic analysis, in which the interactive episodes were divided into 3 (three) types different parallels: child-child, child-teacher and child-teacher. The results indicate that children resisted and built their autonomy among peers. It is worth mentioning that we found an environment with interspersed interactions between affective and providing the construction of autonomy (child-child) and other disciplinarians (teacher or child monitor).

Este estudio investigó las interacciones y la afectividad entre maestras-niño y niño-niño y sus implicaciones para el proceso de autonomía infantil. Se observó la rutina diaria de una clase de Guardería con 26 niños de 3 años, una maestra y una monitora en un Centro de Referencia en Educación Infantil en la ciudad de João Pessoa-PB. La técnica empleada fue la grabación de video de episodios interactivos. Los datos se trataron mediante análisis microgenético, en el que los episodios interactivos se dividieron en 3 (tres) tipos de díadas: niño-niño, niño-maestras y maestras-niños. Los resultados indican que los niños resisten y construyen su autonomía entre sus compañeros. Vale la pena mencionar que encontramos un ambiente con interacciones intercaladas entre afectivas y proporcionadoras de construcción de autonomía (niño-niño) y otras disciplinadoras (maestras o monitora - niños).

Ruptured sinus valsalva aneurysm - an uncommon cause of new onset heart failure onset heart failure / Rotura de aneurisma del seno de valsalva: una causa infrecuente de insuficiencia cardíaca aguda

Abstract: A young man presented to the emergency room with symptoms of recent onset heart failure. On physical examination he showed signs of right heart failure and a continuous murmur. Transesophageal echocardiography an computed tomography confirmed the diagnosis of a ruptured right sinus of Valsalva aneurysm an left to right shunt. After successful surgical repair the patient became asymptomatic, the shunt disappeared and he is well 3 months after surgery.

Resumen: Se presenta el caso clínico de un hombre joven que desarrolla insuficiencia cardíaca de reciente comienzo. Clínicamente lo relevante eran signos de insuficiencia cardíaca derecha y la presencia de un soplo continuo. Por ecocardiografía trans esofágica y Angio TAC, se confirmó la presencia de un aneurisma del seno de Valsalva derecho roto con cortocircuito de izquierda a derecha. Se procedió al cierre del aneurisma, confirmando se buen resultado, acompañado de una evolución asintomática 3 meses después de la intervención.

The effects of transcatheter aortic valve implantation on cardiac electrical properties.

INTRODUCTION: Transcatheter aortic valve implantation (TAVI) is associated with cardiac electrical disturbances. However, beyond the risks of pacemaker implantation, few studies have performed a detailed assessment of the effects of TAVI on several cardiac electrical properties. OBJECTIVES: To assess the frequency and type of electrocardiographic disturbances following TAVI, according to the type of prostheses and to assess predictors of these disturbances. METHODS: We performed a detailed retrospective analysis of all electrocardiograms in patients who underwent TAVI, before and after the procedure, at a tertiary center from August 2007 to October 2016. Patients with permanent pacemakers were excluded. RESULTS: We included 182 patients (78±8 years; 56% female) and self-expanding prostheses (SEP) were implanted in 54%. Most patients (80%) were in sinus rhythm at baseline. After TAVI, 21% of patients developed new-onset atrial fibrillation and there was a significant increase in PR interval at discharge (186±41 ms vs. 176±32; p=0.003), which was not maintained after at six-month follow-up (181±35 ms, p=0.06). There was also a significant increase in QRS duration at discharge (129±28 ms vs. 114±25 at baseline p<0.0001), which persisted at six-months (122±28 ms, p<0.0001). New-onset left-bundle branch block (LBBB) was observed in 25% of patients. The depth of valve implantation was a predictor of new LBBB at discharge after multivariate analysis (OR 37.6, 95% CI 14.6-65.2, p=0.001). CONCLUSIONS: The main electrocardiographic disturbances post TAVI were PR prolongation, increased QRS and new-onset LBBB. These disturbances were more pronounced in patients undergoing SEP implantation and tended to improve at six-month follow-up. The depth of valve implantation was a predictor of conduction disturbances.

Balloon Aortic Valvuloplasty in Patients Admitted for Cardiogenic Shock with Severe Aortic Stenosis: A Retrospective Analysis of 14 Cases.

Introduction Balloon aortic valvuloplasty (BAV), introduced in 1986, quickly lost its wide adoption due to the high incidence of restenosis after the procedure and due to improved skills in transcatheter aortic valve implantation (TAVI). It has seen a re-emergence in the last few years as bailout therapy in critical care patients presenting with cardiogenic shock (CS) and severe aortic stenosis (AS), who are temporarily unable to tolerate such a procedure as TAVI or surgery for valve replacement. Methods We did a retrospective analysis of every BAV performed between January 1, 2008, and November 11, 2018, in our hospital and identified those admitted to the cardiac intensive care unit (CICU) due to cardiogenic shock with severe aortic stenosis, as defined in the European Society of Cardiology Guidelines. Procedures were categorized as emergent (within 24h after the decision to intervene) and urgent (24h after the decision was made but before discharge). Results During this period, of 98 BAV performed, 14 were performed on patients with CS with severe AS, nine of them being emergent. The patients' mean age was 76.2±7.2 years, with 6 (43%) of them being female. On the day of BAV, the mean Euroscore II and sequential organ failure (SOFA) were, respectively, 19±7% and 8.0±2.4 in emergent cases and 11±5% and 4.8±2.9 in urgent cases. In patients deemed emergent, there was a tendency for a decrease in SOFA in the days following the procedure, although not statistically significant (p>0.05). Clinically significant aortic regurgitation did not occur in any patient, neither were there any major post-procedure complications. Thirty-day mortality was 33% in emergent cases and 0% in urgent cases. In emergent cases, four were later submitted to TAVI and one had surgery for aortic valve replacement surgery. Only one patient in the urgent group was regarded as a candidate for TAVI. Discussion and conclusion Emergent cases presented with higher scores of severity and procedure risk and had greater mortality. In this group, a greater proportion of survivors was later deemed fit for definite procedures. This highlights that presenting status does not seem to influence the prognosis of those extremely high-risk patients once the acute event is promptly treated. Nevertheless, the low sample size precludes generalization of the findings. BAV as bailout treatment may be safe in patients presenting with CS and severe AS, allowing patient survival for elective definitive treatment.

Right single coronary artery as an incidental finding in Takotsubo syndrome and acute heart failure: Case report and review of the literature.

INTRODUCTION: Single coronary artery (SCA) with no associated congenital heart disease is a rare congenital anomaly. Most cases are asymptomatic and incidental findings, but SCA can cause ischemia, congestive heart failure, and sudden cardiac death (SCD). CASE REPORT: A 44-year-old woman presented with Takotsubo cardiomyopathy and cardiogenic shock. Selective cannulation of the left coronary artery (LCA) was not possible on coronary angiography (CA); an SCA was revealed arising from the right sinus, continuing distally as the circumflex artery and thereafter as the left anterior descending artery. Coronary computed tomography angiography (CCTA) confirmed left main atresia and no coronary stenosis. Cardiac magnetic resonance imaging (MRI) showed diffuse myocardial edema and no perfusion defects. The patient's clinical course was favorable under conservative management. DISCUSSION: Our paper describes an incidental finding of right SCA. We report a Lipton type R-I, in which a dominant right SCA supplies the entire myocardium. It is the rarest SCA presentation, with an incidence of 0.0008%; only 15 cases have been reported in the literature, all of which were studied by CA. Of these 15, one had SCD, five angina, one ventricular arrhythmia and one complicated acute coronary syndrome. CCTA confirmed the diagnosis in seven patients, MRI in one and transesophageal echocardiography in another. Nine patients had coronary lesions. Two underwent coronary artery bypass grafting, one percutaneous intervention and 11 conservative treatment. CONCLUSION: Right SCA with congenital absence of the LCA is one of the rarest coronary artery anomalies. In a significant percentage of patients it is associated with ischemia and can be life-threatening. CCTA and MRI are the modalities of choice for diagnosis and risk stratification.

Percutaneous treatment of tricuspid regurgitation: A new therapeutic horizon.

Functional tricuspid regurgitation is a prevalent disease, especially among patients with other valve disorders, and is associated with significant morbidity and mortality. Its management is challenging, and many patients deemed at high surgical risk are managed conservatively. Despite optimization of pharmacological treatment, many patients continue to be symptomatic, thus leading to interest in percutaneous interventional techniques. The Mitralign system has recently been used for the treatment of functional tricuspid regurgitation, with favorable clinical and imaging results. We report the first case in Portugal to our knowledge of percutaneous tricuspid regurgitation treatment with the Mitralign system.

Extracorporeal membrane oxygenation in circulatory and respiratory failure - A single-center experience. / Oxigenação por membrana extracorporal na falência circulatória e respiratória ­ experiência de um centro.

INTRODUCTION: Extracorporeal membrane oxygenation (ECMO) provides mechanical pulmonary and circulatory support for patients with shock refractory to conventional medical therapy. In this study we aim to describe the indications, clinical characteristics, complications and mortality associated with use of ECMO in a single tertiary hospital. METHODS: We conducted a retrospective observational cohort study of all patients supported with ECMO in two different intensive care units (general and cardiac), from the first patient cannulated in April 2011 up to October 2016. RESULTS: Overall, 48 patients underwent ECMO: 29 venoarterial ECMO (VA-ECMO) and 19 venovenous ECMO (VV-ECMO). In VA-ECMO, acute myocardial infarction was the main reason for placement. The most frequent complication was lower limb ischemia and the most common organ dysfunction was acute renal failure. In VV-ECMO, acute respiratory distress syndrome after viral infection was the leading reason for device placement. Access site bleeding and hematologic dysfunction were the most prevalent complication and organ dysfunction, respectively. Almost 70% of ECMO episodes were successfully weaned in each group. Survival to discharge was 37.9% for VA-ECMO and 63.2% for VV-ECMO. In VA-ECMO, the number of inotropic agents was a predictor of mortality. CONCLUSION: Patients with respiratory indications for ECMO experienced better survival than cardiac patients. The need for more inotropic drugs was a predictor of mortality in VA-ECMO. This is the first published record of the overall experience with ECMO in a Portuguese tertiary hospital.

Percutaneous closure of periprosthetic paravalvular leaks: A viable alternative to surgery?

INTRODUCTION AND OBJECTIVES: Paravalvular leak (PVL) is a possible complication after prosthetic valve implantation. PVL can cause significant symptoms of congestive heart failure and/or hemolysis. Medical therapy is palliative and reoperation has a high mortality rate. Percutaneous transcatheter closure is a promising alternative for symptomatic patients at high surgical risk. We aim to review the efficacy and safety of percutaneous PVL closure in a consecutive series of patients referred to our center. METHODS: We performed a retrospective analysis of clinical and technical procedural data of patients referred to our center for percutaneous PVL closure between January 2009 and November 2015. RESULTS: Twenty procedures were performed in 18 patients under general anesthesia and under transesophageal echocardiographic and radiographic guidance. Fourteen mitral PVLs were successfully treated in 13 patients and one aortic PVL in one patient. Most (eight) of the PVLs closed were in mitral bioprostheses. Two patients underwent a second intervention, which was technically successful in one. Technical success was achieved in 15 (75%) of the procedures. At discharge, median NYHA functional class decreased by one and hemolytic anemia decreased from seven cases (38.9%) to two (11.1%). Two patients had minor bleeding at the femoral vascular access site. Survival rates at six, 12 and 24 months were 77.8%, 77.8% and 61.1%, respectively. CONCLUSIONS: In our experience, percutaneous PVL closure was overall effective and safe. The procedure is complex and a second intervention may be necessary. Percutaneous PVL closure may be a feasible alternative for selected symptomatic patients at high surgical risk refractory to medical therapy.

Lead Extraction of Cardiac Rhythm Devices: A Report of a Single-Center Experience.

INTRODUCTION AND OBJECTIVES: The rate of implanted cardiac electronic devices is increasing as is the need to manage long-term complications. Lead removal is becoming an effective approach to treat such complications. We present our experience in lead removal using different approaches, analyzing the predictors of the use of mechanical extractors/surgical removal. METHODS: Retrospective analysis of lead extractions in a series of 76 consecutive patients (mean age 70.4 ± 13.8 years, 73.7% men) between January 2009 and November 2015. RESULTS: One hundred thirty-five leads from permanent pacemakers (single chamber 19.7%; dual-chamber 61.8%), implantable cardioverter defibrillators (5.3%), and cardiac resynchronization devices (CRT-P 2.6%; CRT-D 7.9%) were removed, 72.5 ± 73.2 months after implantation. A total of 45.9% were ventricular leads, 40.0% atrial leads, 8.9% defibrillator leads, and 5.2% leads in the coronary sinus; 64.4% had passive fixation. The most common indications for removal were pocket infection (77.8%), infective endocarditis (9.6%), and lead dislodgement (3.7%). A total of 76.3% of the leads were explanted, 20.0% were extracted, and 3.7% were surgically removed. Extraction of the entire lead was achieved in 96.3% of the procedures. After logistic regression (age adjusted), time since implantation was the sole predictor of the need of mechanical extractors/surgical removal. All patients were discharged without major complications. There were no deaths at 30 days. CONCLUSION: Our experience in lead removal was effective and safe. Performing these procedures by experienced electrophysiologists with an adequate cardiothoracic surgery team on standby to cope with any complications is required. Referral of high-risk patients to a high-volume center is recommended to optimize clinical success and minimize procedural complications.

Crianças do Campo: da invisibilidade ao reconhecimento como sujeito de direito / Country Children: from invisibility to recognition as a subject of law / Niños Campesinos: la invisibilidad al reconocimiento de un sujeto de derecho / Enfants Paysans: invisibilité à la reconnaissance en tant que sujet de droit

O objetivo deste artigo é analisar como processos de silenciamento e de invisibilização que caracterizaram historicamente a construção das culturas infantis das crianças do campo, impuseram-lhe formas subalternas de existência, mediante revisão do conceito de socialização. À luz da sociologia da infância e dos estudos pós-coloniais, partimos do pressuposto de que a infância enquanto categoria social é uma construção que implica uma condição de vida não apenas biológica, mas cultural. Com base em abordagens críticas da perspectiva teórica intercultural, examinamos a existência de uma pluralidade de culturas infantis nas quais as crianças do campo constroem-se e são construídas nas relações estabelecidas com os seus pares, os adultos e o mundo rural ao qual pertencem, considerando o tensionamento entre o rural e o urbano. Após análises desenvolvidas a partir das reflexões teóricas efetuadas, concluímos que as tensões e contradições que permeiam a invisibilidade das crianças do campo, ainda que marcados pela subalternização, expressam também a busca por novos caminhos e referenciais para dar visibilidade a este sujeito social.

The aim of this article is to analyze how processes of silencing and invisibilization that historically characterized a construction of the children 's cultures of the rural children, imposed, subaltern forms of existence, by reviewing the concept of socialization. In light of the sociology of childhood and post-colonial studies, we start from the assumption of a childhood as a social category is a construction that implies a condition of life not only biological but cultural. Based on critical approaches from the intercultural theoretical perspective, it examines the existence of a plurality of child cultures in which as rural children are constructed and built in the relationships established with their peers, adults and the rural world to which they belong, considering the tension between rural and urban. After analysis developed from theoretical reflections made, we conclude that as tensions and contradictions that permeate the invisibility of rural children, even if marked by subalternization, also express the search for new ways and referential to the vision of a social subject.

El objetivo de este artículo es analizar como procesos de silenciamiento y de invisibilización que caracterizaron históricamente la construcción de las culturas infantiles de los niños del campo, impusieron formas subalternas de existencia, mediante la revisión del concepto de socialización. A la luz de la sociología de la infancia y de los estudios postcoloniales, partimos del supuesto de que la infancia como categoría social es una construcción que implica una condición de vida no sólo biológica, sino cultural. Sobre la base de enfoques críticos de la perspectiva teórica intercultural, examinamos la existencia de una pluralidad de culturas infantiles en las que los niños del campo se construyen y se construyen en las relaciones establecidas con sus pares, los adultos y el mundo rural al que pertenecen, la tensión entre lo rural y lo urbano. Después de análisis desarrollados a partir de las reflexiones teóricas efectuadas, concluimos que las tensiones y contradicciones que permean la invisibilidad de los niños del campo, aunque marcados por la subalternización, expresan también la búsqueda de nuevos caminos y referencias para dar visibilidad a este sujeto social.

L'objectif de cet article est d'analyser comment les processus de silence et d'invisibilisation qui caractérisent historiquement la construction des cultures infantiles des enfants ruraux lui imposent des formes subalternes d'existence, par la révision de la notion de socialisation. À la lumière de la sociologie de l'enfance et des études post-coloniales, nous partons de l'hypothèse que l'enfance en tant que catégorie sociale est une construction qui implique une condition de vie non seulement biologique mais culturelle. Basé sur des approches critiques du point de vue théorique interculturel, nous examinons l'existence d'une pluralité de cultures infantiles dans lesquelles les enfants ruraux sont construits et construits dans les relations établies avec leurs pairs, les adultes et le monde rural auquel ils appartiennent, compte tenu la tension entre rural et urbain. Après une analyse développée à partir des réflexions théoriques réalisées, nous concluons que les tensions et les contradictions qui imprègnent l'invisibilité des enfants ruraux, même si elles sont marquées par la subalternisation, expriment également la recherche de nouvelles voies et références pour donner une visibilité à cette sujet sociale.

A rare cause of acute coronary syndrome: Kounis syndrome. / Causa rara de síndrome coronária aguda: síndrome de Kounis.

Kounis syndrome is an acute coronary syndrome in the context of a hypersensitivity reaction. The main pathophysiological mechanism appears to be coronary vasospasm. We report the case of a patient with a history of allergy to quinolones, who was given ciprofloxacin before an elective surgical procedure and during drug administration developed symptoms and electrocardiographic changes suggestive of ST-segment elevation acute coronary syndrome. The drug was suspended and coronary angiography excluded epicardial coronary disease. Two hours after withdrawal of the drug the symptoms and ST elevation had resolved completely.

Persistent Type I Endoleak after Endovascular Treatment with Chimney Technique.

Thoracic endovascular aortic repair (TEVAR) is increasingly used in the treatment of acute type B aortic dissection. Type Ia endoleaks are a common complication of the procedure, but its clinical significance and the best treatment strategy remain poorly defined. We present a case of a type Ia endoleak following TEVAR in the treatment of acute type B aortic dissection. Chimney technique approach was used in an attempt to seal the endoleak. Although technical success was suboptimal, the patient remained clinically stable and event free. Data regarding the natural course and management of type Ia endoleaks following TEVAR for aortic dissection are sparse. Future research is required to establish the clinical and technical determinants of the need to treat these endoleaks and the best treatment strategy.

Catecholaminergic polymorphic ventricular tachycardia: a rare cause of recurrent syncope.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia characterized by adrenergically induced polymorphic or bidirectional ventricular tachycardia (VT). Although a rare disease, its recognition is important because of its high mortality rate when left untreated. We report an index case of a 32-year-old woman who presented with recurrent syncope. The diagnosis was confirmed by exercise-induced polymorphic ventricular premature beats and episodes of non-sustained VT, in the absence of structural heart abnormalities. She remained event free with beta-blocker therapy. CPVT is a potentially life-threatening disease and should be considered in the case of recurrent syncope, in young individuals. Diagnosis is based on clinical history and exercise testing, which is the gold standard. Therapy is mandatory in all diagnosed individuals. Exercise testing in first-degree relatives is recommended, even in the case of a mutation-negative index patient.