Background: Atrioventricular block (AVB) secondary to transient causes can recover with its correction. However, studies assessing predictors of recovery and long-term recurrence are lacking. Methods: Patients with advanced or complete AVB who had a reversible cause admitted in a single expert center were retrospectively studied. Patients with AVB secondary to acute coronary syndromes were excluded from analysis. Results: In a population of 162 patients, the main factors associated with recovery of rhythm without a permanent pacemaker (PPM) implantation were the presence of chronic kidney disease (CKD) on dialysis (OR 7.6; CI 95% 1.2-47.5 (p = .03)); greater serum potassium levels (OR 2.3; CI 95% 1.28-4.0 (p < .01)), higher dosage of bradycardic drugs (OR 2.2; CI 95% 1.13-4.4 (p = .02)), the association between different bradycardic drugs (OR 9.0; CI 95% 2.02-40.3 (p < .01)) and between drug therapy and hyperkaliemia (OR 5.2; CI 95% 1.8-15.1 (p < .01)). There was an overall high burden of conductions abnormalities which did not correlate with recovery of rhythm (OR 0.5; CI 95% 0.19-1.5 (p = .23)). In 29 patients (17.9%) there was a correction of the AVB. During a maximum follow-up of 130 months, 24 patients (82.8%) had a recurrence which warranted a PPM. In the overall cohort only five patients (3%) had sustained recovery of rhythm. Conclusions: Recovery of AVB was mainly observed with higher doses of drug therapy, higher serum potassium levels or a combination of factors and regardless of baseline conduction abnormalities. The high rate of recurrence during follow-up warrants a close follow-up or PPM implantation at index admission.

Explanation: In the differential diagnosis of the narrow-complex tachycardia with a P:R ratio of 1:2 and QRS 4 alternans between narrow and wide complex one must consider either ventricular bigeminy, atrial bigeminy

Background Pregnancy is associated with both new-onset and exacerbation of pre-existing arrhythmias, particularly supraventricular tachycardia, with increased maternal and fetal risks and with added concerns on the safety of the available drug therapy and catheter ablation techniques. These are often withheld, with worse outcomes, and lead to challenging decisions in the approach to pregnant women with refractory supraventricular arrhythmias. Case presentation We present a case of a 28-year-old 37-weeks pregnant woman with symptomatic, almost incessant, atrial tachycardia causing tachycardia-induced cardiomyopathy, refractory to medical therapy, that evolved in acute cardiac failure and needed emergency cesarian delivery. The patient was afterward submitted to catheter ablation therapy, with an electrical isolation of the ectopic foci on the lower left pulmonary vein with radiofrequency and total suppression of the arrhythmia. The patient and infant were discharged clinically well and during follow-up the patient was asymptomatic, without recurrence of tachycardia and with complete recovery of left ventricle function. Conclusions This case highlights the challenges in the treatment of this special population with a stepwise medical approach that proved ineffective and clinical deterioration requiring termination of pregnancy and catheter ablation in the postpartum period, with a successful maternal and fetal outcome.

Introdução O infarto do miocárdio isolado do ventrículo direito é extremamente raro e é frequentemente silencioso, com apenas 25% dos pacientes desenvolvendo manifestações hemodinâmicas clinicamente evidentes na apresentação. O manejo atual do infarto agudo do miocárdio baseia-se no diagnóstico imediato e na revascularização imediata. Aproximadamente 90% dos pacientes que apresentam infarto do miocárdio com supradesnivelamento do segmento ST apresentam estenose ou oclusão da artéria coronária explicativa. O infarto do miocárdio com artérias coronárias não obstrutivas (MINOCA) deve levar o médico [...]

September 2020 1 Ana Catarina Gomes, MD Luís Rocha Lopes, MD, PhD Inês Cruz, MD Ana Rita Almeida, MD Rita Miranda, MD Sofia Almeida, MD Luís Brandão, MD Hélder Pereira, MD, PhD Sixty four–year-old White overweight (body mass index=26.7 kg/m 2) and smoker male, with no other comorbidities. He had no known family history of cardiac disease, but a personal history of a tachyarrhythmia diagnosed at the age of 40, managed with amiodarone. After 24 years of an uneventful course and with no follow-up or further investigation, he came to medical attention after a 5-minute episode of sudden onset palpitation at rest. During the prehospital transport, a hemodynamically unstable wide-complex tachycardia was detected and successfully cardioverted. At admission in the emergency department, he was asymptomatic in sinus rhythm, with normal vital signs and normal physical examination. Just after the initial approach, he suddenly developed a symptomatic left-bundle-branch-block monomorphic ventricular tachycardia with superior axis, which was electrically cardioverted (Figure 1). The post-cardioversion ECG showed a delta wave in leads V2 to V4 and T wave-inversion in V1 to V3, with no other distinctive findings. The blood tests showed no significant abnormalities, and he was started on IV amiodarone perfusion with no recurrent arrhythmias. The transthoracic echocardiogram at admission showed a dilated right ventricle (RV) with systolic impairment and a mildly dilated nonhypertrophied left ventricle (LV) with mild systolic impairment. In the next days, further investigations were performed. The coronary angiography excluded significant coronary artery disease. In the context of a nonischemic ventricular tachycardia, a delta wave in the first ECG and no cardiac magnetic resonance available in the first days of hospitalization, he was submitted to an electrophysiology study. An accessory pathway was ruled out. After programmed ventricular stimulation, a left-bundle-branch-block monomorphic ventricular tachycardia with superior axis was induced, with degeneration to ventricular fibrillation. A second ECG performed during hospitalization identified an epsilon wave in lead V1. A cardiac magnetic resonance was performed, revealing a severely dilated (181 mL/m2) and impaired RV (RV ejection fraction: 27%) with thin walls and multiple contractile abnormalities, including dyskinesia (multiple micro-aneurisms) in the free and diaphragmatic walls as well as akinesia in the RV outflow tract. The LV was mildly dilated (112 mL/m2) and impaired (LV ejection fraction: 41%) because of hypokinesia of the mid segments of the anterolateral, inferolateral, and inferior walls (all of them with decreased thickness; additional material, Movies I through IV in the Data Supplement). There was a high-intensity signal of fat in the RV-free wall and all LV hypokinetic segments and subepicardial late gadolinium enhancement matching the wall motion abnormalities of both ventricles (Figures 2 through 5). These findings were in keeping with the diagnosis of biventricular arrhythmogenic right ventricular cardiomyopathy (ARVC). © 2020 American Heart Association, Inc. CARDIOVASCULAR IMAGES

HomeCirculation: Cardiovascular ImagingVol. 13, No. 9Arrhythmogenic Right Ventricular Cardiomyopathy Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyRedditDiggEmail Jump toSupplementary MaterialsFree AccessCase ReportPDF/EPUBArrhythmogenic Right Ventricular CardiomyopathyAn Exuberant Case Affecting Both Ventricles Ana Catarina Gomes, MD Luís Rocha Lopes, MD, PhD Inês Cruz, MD Ana Rita Almeida, MD Rita Miranda, MD Sofia Almeida, MD Luís Brandão, MD Hélder PereiraMD, PhD Ana Catarina GomesAna Catarina Gomes Ana Catarina Gomes, MD, Hospital Garcia de Orta, EPE. Av. Prof. Torrado da Silva, 2801-951 Almada, Portugal. Email E-mail Address: [email protected] Hospital Garcia de Orta, Almada, Portugal (A.C.G., L.R.L., I.C., A.R.A., R.M., S.A., L.B., H.P.). , Luís Rocha LopesLuís Rocha Lopes Hospital Garcia de Orta, Almada, Portugal (A.C.G., L.R.L., I.C., A.R.A., R.M., S.A., L.B., H.P.). St Bartholomew's Hospital, Barts Health NHS Trust, London, United Kingdom (L.R.L.). Institute of Cardiovascular Science, UCL, London, United Kingdom (L.R.L.). Centro Cardiovascular da Universidade de Lisboa, Portugal (L.R.L.). , Inês CruzInês Cruz Hospital Garcia de Orta, Almada, Portugal (A.C.G., L.R.L., I.C., A.R.A., R.M., S.A., L.B., H.P.). , Ana Rita AlmeidaAna Rita Almeida Hospital Garcia de Orta, Almada, Portugal (A.C.G., L.R.L., I.C., A.R.A., R.M., S.A., L.B., H.P.). , Rita MirandaRita Miranda Hospital Garcia de Orta, Almada, Portugal (A.C.G., L.R.L., I.C., A.R.A., R.M., S.A., L.B., H.P.). , Sofia AlmeidaSofia Almeida Hospital Garcia de Orta, Almada, Portugal (A.C.G., L.R.L., I.C., A.R.A., R.M., S.A., L.B., H.P.). , Luís BrandãoLuís Brandão Hospital Garcia de Orta, Almada, Portugal (A.C.G., L.R.L., I.C., A.R.A., R.M., S.A., L.B., H.P.). , Hélder PereiraHélder Pereira Hospital Garcia de Orta, Almada, Portugal (A.C.G., L.R.L., I.C., A.R.A., R.M., S.A., L.B., H.P.). Originally published2 Sep 2020https://doi.org/10.1161/CIRCIMAGING.119.010243Circulation: Cardiovascular Imaging. 2020;13:e010243Sixty four–year-old White overweight (body mass index=26.7 kg/m2) and smoker male, with no other comorbidities. He had no known family history of cardiac disease, but a personal history of a tachyarrhythmia diagnosed at the age of 40, managed with amiodarone.After 24 years of an uneventful course and with no follow-up or further investigation, he came to medical attention after a 5-minute episode of sudden onset palpitation at rest. During the prehospital transport, a hemodynamically unstable wide-complex tachycardia was detected and successfully cardioverted. At admission in the emergency department, he was asymptomatic in sinus rhythm, with normal vital signs and normal physical examination.Just after the initial approach, he suddenly developed a symptomatic left-bundle-branch-block monomorphic ventricular tachycardia with superior axis, which was electrically cardioverted (Figure 1). The post-cardioversion ECG showed a delta wave in leads V2 to V4 and T wave-inversion in V1 to V3, with no other distinctive findings. The blood tests showed no significant abnormalities, and he was started on IV amiodarone perfusion with no recurrent arrhythmias.Download figureDownload PowerPointFigure 1. ECG: left-bundle-branch-block-ventricular tachycardia with superior axis.The transthoracic echocardiogram at admission showed a dilated right ventricle (RV) with systolic impairment and a mildly dilated nonhypertrophied left ventricle (LV) with mild systolic impairment.In the next days, further investigations were performed. The coronary angiography excluded significant coronary artery disease. In the context of a nonischemic ventricular tachycardia, a delta wave in the first ECG and no cardiac magnetic resonance available in the first days of hospitalization, he was submitted to an electrophysiology study. An accessory pathway was ruled out. After programmed ventricular stimulation, a left-bundle-branch-block monomorphic ventricular tachycardia with superior axis was induced, with degeneration to ventricular fibrillation. A second ECG performed during hospitalization identified an epsilon wave in lead V1.A cardiac magnetic resonance was performed, revealing a severely dilated (181 mL/m2) and impaired RV (RV ejection fraction: 27%) with thin walls and multiple contractile abnormalities, including dyskinesia (multiple micro-aneurisms) in the free and diaphragmatic walls as well as akinesia in the RV outflow tract. The LV was mildly dilated (112 mL/m2) and impaired (LV ejection fraction: 41%) because of hypokinesia of the mid segments of the anterolateral, inferolateral, and inferior walls (all of them with decreased thickness; additional material, Movies I through IV in the Data Supplement). There was a high-intensity signal of fat in the RV-free wall and all LV hypokinetic segments and subepicardial late gadolinium enhancement matching the wall motion abnormalities of both ventricles (Figures 2 through 5). These findings were in keeping with the diagnosis of biventricular arrhythmogenic right ventricular cardiomyopathy (ARVC).Download figureDownload PowerPointFigure 2. Cardiac magnetic resonance (end-systolic cyne image—4 chamber): severely dilated and impaired wall-thinned right ventricle, with multiple dyskinetic areas at the free wall.A single-chamber implantable cardioverter defibrillator was implanted for secondary prevention of sudden cardiac death.The genetic test found a heterozygous plakophilin-2 variant (PKP2: Ala749Ile), considered in the clinical report as likely pathogenic but not suitable for predictive testing without further evidence of co-segregation. This variant affects a moderately conserved residue in the protein. It had not been previously published or reported in controls including in the Genome Aggregation Database (gnomAD available at https://gnomad.broadinstitute.org/).The patient has remained under follow-up for 3 years at the Inherited Cardiac Disease clinic in New York Heart Association class I with no further events, medicated with sotalol and an angiotensin-converting-enzyme inhibitor. Clinical screening was offered to first-degree relatives, which is still pending.DiscussionARVC is an autosomal dominant inherited cardiomyopathy with incomplete penetrance and variable expression.1 A causal mutation can be found in around 30% of patients. Among desmosomal protein genes, the most commonly involved is PKP2 (plakophylin-2).2Histologically, ARVC is defined by fibro-fatty replacement of the myocardium, affecting mainly the right ventricle.1 It is clinically characterized by life-threatening ventricular arrhythmias or even sudden cardiac death at young age. Later, patients can develop symptoms of heart failure caused by right and/or biventricular dilatation and dysfunction.3The 2010 Task Force Criteria for ARVC diagnosis include anatomic, functional, and electrocardiographic features together with genetic information and family history.1This case is particularly impressive for the exuberant and unusual biventricular dilatation and systolic impairment, LV fat replacement, and the extent of RV regional wall motion abnormalities. Overall, this patient fulfilled 2 major criteria for ARVC: (1) RV wall motion abnormalities associated with dilatation (RV end-diastolic volume ≥110 m/m2) and impairment (RV ejection fraction 27%) and (2) sustained left-bundle-branch-block monomorphic ventricular tachycardia with superior axis.1 Although an epsilon wave was identified, it was only seen in lead V1, therefore, not fulfilling major criteria. Also, the genetic variant did not have enough evidence to be considered as definitely pathogenic.This would represent an advanced stage of RV-dominant/biventricular ARVC, based on the cardiac magnetic resonance findings of biventricular fat and fibrosis, the hallmark of late disease.Despite the extensive anatomic and functional abnormalities, the patient had no clinical signs of overt left or right heart failure, as would be expected. Instead, arrhythmia was the main presenting feature. The arrhythmic presentation in this patient was typical for an ARVC-related tachyarrhythmia, probably correlated with late gadolinium enhancement in the RV wall as the place of ventricular tachycardia origin. The role of electrophysiology study for risk stratification and prediction of adverse events in ARVC is a matter of ongoing debate, although it was necessary in this case because of a possible accessory pathway.The most established predictors of ARVC-related ventricular arrhythmias are prior sustained and nonsustained ventricular tachycardia, RV dilatation and severe impairment, syncope and LV or biventricular involvement. Our patient had presented with a putative previous arrhythmic episode in the past and had unrecognized biventricular and extensive involvement. Unfortunately, however, the patient had no clinical follow-up, medical treatment, and no monitoring for 24 years, precluding a previous assessment of risk or any decision regarding implantation of an implantable cardioverter defibrillator in a primary prevention setting.This index-patient without a known familial history of ARVC or sudden cardiac death had a PKP2 variant detected, which is usually associated with predominantly RV-dominant or biventricular ARVC. The specific variant found was novel with no additional information found both in the published literature or ARVC database (http://www.arvcdatabase.info/).In conclusion, despite harboring a single PKP2 variant, this patient presented with biventricular fibro-fatty infiltration leading to biventricular systolic failure associated with a hemodynamically unstable left-bundle-branch-block monomorphic ventricular tachycardia, which represents an advanced stage of the disease with an adverse arrhythmic prognosis.Download figureDownload PowerPointFigure 3. Cardiac magnetic resonance (late gadolinium enhancement [LGE]—4 chamber): subepicardial fat and late gadolinium enhancement at the left ventricular mid-lateral wall and LGE at the right ventricle free wall.Download figureDownload PowerPointFigure 4. Cardiac magnetic resonance T1-weighted fast spin-echo image without fat suppression: fat in the inferior wall (arrow).Download figureDownload PowerPointFigure 5. Cardiac magnetic resonance T1-weighted fast spin-echo image with fat suppression: fat in the inferior wall (arrow).AcknowledgmentWe received informed consent for case publication.Sources of FundingNone.DisclosuresThere are no conflicts of interest to declare.FootnotesThe Data Supplement is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCIMAGING.119.010243.Ana Catarina Gomes, MD, Hospital Garcia de Orta, EPE. Av. Prof. Torrado da Silva, 2801-951 Almada, Portugal. Email anacatarina.d.[email protected]comReferences1. Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, Calkins H, Corrado D, Cox MG, Daubert JPet al.. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria.Circulation. 2010; 121:1533–1541. doi: 10.1161/CIRCULATIONAHA.108.840827LinkGoogle Scholar2. van Tintelen JP, Entius MM, Bhuiyan ZA, Jongbloed R, Wiesfeld AC, Wilde AA, van der Smagt J, Boven LG, Mannens MM, van Langen IMet al.. Plakophilin-2 mutations are the major determinant of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy.Circulation. 2006; 113:1650–1658. doi: 10.1161/CIRCULATIONAHA.105.609719LinkGoogle Scholar3. Groeneweg JA, Bhonsale A, James CA, te Riele AS, Dooijes D, Tichnell C, Murray B, Wiesfeld AC, Sawant AC, Kassamali Bet al.. Clinical presentation, long-term follow-up, and outcomes of 1001 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients and family members.Circ Cardiovasc Genet. 2015; 8:437–446. doi: 10.1161/CIRCGENETICS.114.001003LinkGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetails September 2020Vol 13, Issue 9Article InformationMetrics Download: 135 © 2020 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.119.010243PMID: 32873069 Originally publishedSeptember 2, 2020 Keywordsimplantable defibrillatorsarrhythmogenic right ventricular cardiomyopathyplakophilin 2ventricular tachycardiamagnetic resonance imagingPDF download SubjectsCatheter Ablation and Implantable Cardioverter-DefibrillatorCardiomyopathy

We describe the case of a 62-year-old female patient with bilateral subclavian vein occlusion, in whom a cardiac resynchronization system was implanted via a femoral vein.

The incidence of left atrial thrombi is higher in patients with mitral valve stenosis. Its presence and location have important implications in deciding on the therapeutic approach, particularly the need for valve repair. We describe the case of a 63-year-old patient, with asymptomatic moderate mitral stenosis, hospitalized due to community-acquired pneumonia, in whom investigation to exclude pulmonary thromboembolism revealed a giant left atrial thrombus, which required urgent surgery.

In this article the authors present a case study of a young man with coarctation of the aorta that underwent surgical correction at 4 months old, for whom the exercise echocardiogram was important in determining the functional importance of recoarctation and the decision of the timing for re-intervention, demonstrating the importance of this technique to discriminate the functional significance of this pathology.

The authors describe the case of a 16-year-old male, who went to the emergency department with a clinical setting characterized by chest pain, electrocardiographic pattern of inferoposterior acute myocardial infarction and elevated markers of myocardial necrosis. Cardiac catheterization revealed no significant coronary lesions and the transthoracic echocardiogram showed no segmental abnormalities. Myocarditis was suspected, and this diagnosis was confirmed by magnetic resonance imaging. This case demonstrates the difficulty of establishing a definitive diagnosis of myocarditis and the increasing importance of cardiac magnetic resonance imaging in this area.

Pregnancy in patients with severe pulmonary arterial hypertension is a high risk situation, which makes pregnancy prevention or termination in the first trimester advisable. For this reason, patients of reproductive age with this pathology are referred for gynecology consultation. Since our unit began operating in 1999, we have had three pregnant patients with severe pulmonary arterial hypertension--one our patient and the other two referred from other centers. In this article we describe these three cases and review the literature on pregnancy and pulmonary arterial hypertension.

A 70-year-old man developed drug refractory acute pulmonary oedema secondary to acute severe mitral regurgitation (MR) immediately after implantation of a dual-chamber pacemaker for complete heart block. Clinical improvement occurred after allowing the patient to resume his native rhythm. A new lead was positioned within the right ventricular outflow tract (RVOT). The echocardiogram during pacing at RVOT showed minimal MR.

The authors report the case of a 64-year-old patient with hypertrophic cardiomyopathy with latent obstruction. The patient complained of fatigue but the left ventricular outflow gradient at rest was not significant. He underwent exercise echocardiography that was stopped after 3 minutes due to exhaustion. The gradient at peak workload was 150 mmHg accompanied by a fall in arterial pressure. After the patient refused surgery, a double chamber pacemaker was implanted. A month later, exercise echocardiography lasted for 12 minutes and the gradient at peak workload was 60 mmHg. The authors highlight the importance of exercise echocardiography in the evaluation of symptomatic patients with hypertrophic cardiomyopathy without significant left ventricular outflow gradient at rest and the possible therapeutic role of pacing in these patients.

In this article the authors present a case study of a young man with coarctation of the aorta that underwent surgical correction at 4 months old, for whom the exercise echocardiogram was important in determining the functional importance of recoarctation and the decision of the timing for re-intervention, demonstrating the importance of this technique to discriminate the functional significance of this pathology.

Left ventricular outflow tract obstruction (LVOTO) has traditionally been associated with hypertrophic obstructive cardiomyopathy, but can occur in other clinical scenarios such as acute myocardial infarction (AMI). In some patients, LVOTO is absent at rest, being detectable only with provocation tests such as stress echocardiography. Timely diagnosis of this phenomenon is very important, as it has therapeutic implications, and relies on clinical suspicion and on recognizing substrates in which LVOTO can occur. We report a case of syncope and AMI associated with LVOTO with systolic anterior motion of the mitral valve and a significant intraventricular gradient.

Treadmill exercise echocardiography with Doppler during effort has been used for several years in our department. This form of echocardiography is used mainly in evaluation of patients with coronary heart disease and it is being increasingly used in valvular heart disease. We report the case of a 49-year-old woman with moderate mitral stenosis, in which stress echocardiography was important in making correct clinical decisions.