Tatiana Guimarães

Triple-site pacing for cardiac resynchronization in permanent atrial fibrillation: follow-up results from a prospective observational study

Aims Cardiac Resynchronization Therapy (CRT) is associated with a particularly high non-response rate in patients with atrial fibrillation (AF). We aimed to assess the effectiveness of triple-site (Tri-V) pacing CRT in this population. Methods and results Prospective observational study of patients with permanent AF who underwent CRT implantation with an additional right ventricle lead in the outflow tract septal wall. After implantation, programming mode (Tri-V or biventricular pacing) was selected based on cardiac output determination. Patients were classified as responders if NYHA class was reduced by at least one level and echocardiographic ejection fraction (EF) increased ≥ 10%, and as super-responders if in NYHA class I and EF ≥ 50%. Forty patients (93% male, mean age 72 ± 10 years) were included. Thirty-three were programmed in Tri-V. The following results pertain to this subgroup. At baseline, 58% were in NYHA class III and 36% NYHA class II. At 1 year follow-up, Minnesota QoL score was reduced (36 ± 23 vs. 8 ± 6; P = 0.001) and the 6MWT distance improved (384 ± 120 m to 462 ± 87 m, P = 0.003). Mean EF increased (26% ± 8 vs. 39 ± 10; P < 0.001 at 6 months and 41 ± 10; P < 0.001 at 12 months). Responder rate was 59% at 6 months and 79% at 12 months. Super-responder rate was 9% at 6 months and 16% at 12 months. One year survival free from heart failure hospitalization was 87.9%. Conclusion Tri-V CRT yielded higher response and super-response rates than usually reported for CRT in patients with permanent AF using clinical and remodeling criteria.

Progressão da desnervação simpática cardíaca avaliada por cintigrafia com MIBG‐I123 na polineuropatia amiloidótica familiar e o impacto da transplantação hepática

INTRODUCTION Familial amyloid polyneuropathy (FAP) is a rare disease caused by systemic deposition of amyloidogenic variants of the transthyretin (TTR) protein. The TTR-V30M mutation is caused by the substitution of valine by methionine at position 30 and mainly affects the peripheral and autonomic nervous systems. Cardiovascular manifestations are common and are due to autonomic denervation and to amyloid deposition in the heart. Cardiac sympathetic denervation detected by iodine-123 labeled metaiodobenzylguanidine (MIBG) is an important prognostic marker in TTR-V30M FAP. Liver transplantation, widely used to halt neurological involvement, appears to have a varying effect on the progression of amyloid cardiomyopathy. Its effect on the progression of cardiac denervation remains unknown. METHODS In this observational study, patients with the TTR-V30M mutation underwent annual cardiac assessment and serial MIBG imaging with quantification of the late heart-to-mediastinum (H/M) ratio. RESULTS We studied 232 patients (median age 40 years, 54.7% female, 37.9% asymptomatic at the time of inclusion) who were followed for a median of 4.5 years and underwent a total of 558 MIBG scans. During follow-up, 47 patients (20.3%) died. MIBG scintigraphy at inclusion was a strong predictor of prognosis, with the risk of death increasing by 27.8% for each one-tenth reduction in the late H/M ratio. The late H/M ratio decreased with age (0.082/year, p<0.001), but progression of cardiac denervation was so slow that annual repetition of MIBG imaging did not increase its prognostic accuracy. During follow-up, 70 symptomatic patients underwent liver transplantation. The late H/M ratio decreased by 0.19/year until transplantation but no statistically significant differences were detected after the procedure. CONCLUSIONS Cardiac denervation is common during the progression of TTR-V30M FAP and quantification of the late H/M ratio on MIBG scintigraphy is valuable for prognostic stratification of these patients. Liver transplantation stabilizes cardiac denervation, without recovery or further deterioration in cardiac MIBG uptake after the procedure.

Persistent left superior vena cava : a vascular access without limitations

© 2018 Sociedade Portuguesa de Cardiologia. Published by Elsevier Espana, S.L.U. All rights reserved.

Veia cava superior esquerda persistente – Um acesso vascular sem limitações

A veia cava superior esquerda persistente (VCSEP) é a malformação venosa congénita torácica mais frequente e o seu diagnóstico costuma ser incidental. Casos clínicos demonstrativos de implantação de pacemakers de dupla câmara (DDD), cardioversores desfibrilhadores implantáveis e sistemas de ressincronização cardíaca através desse acesso venoso foram já descritos na literatura. Contudo, na grande maioria dos casos apresentados o posicionamento do eletrocater (ECT) ventricular direito (ECT-VD) ficou restrito ao apéx do ventrículo direito (VD). Até ao momento estão descritos na literatura apenas quatro casos de posicionamento do ECT VD no trato de saída do VD através da VCSEP. Os autores apresentam o caso de uma doente do sexo feminino, de 79 anos, com diagnóstico de síncope e bloqueio de ramo esquerdo proposta para implantação de pacemaker DDD. Durante o procedimento obteve-se acesso venoso pela veia cefálica esquerda, verificou-se que o ECT progredia à esquerda da coluna vertebral, sugestivo da presença de VCSEP. Através desse acesso foi possível implantar sequencialmente o ECT-VD no trato de saída do VD e o ECT auricular no apêndice auricular direito, ambos com sistema de fixação ativa (Figura 1).Persistent left superior vena cava (PLSVC) is the most common thoracic congenital malformation and its diagnosis usually occurs by chance. Demonstrative clinical cases of dual-chamber (DDD) pacemaker implantation, implantable cardioverter defibrillators and cardiac resynchronization systems through PLSVC access have already been described in the literature. However, in most of the cases presented, the positioning of the lead in the right ventricle (RV) was restricted to the RV apex. To date, the literature reports only four cases of RV lead positioning in the right ventricular outflow tract (RVOT) via the PLSVC. The authors present the case of a 79-year-old female patient, diagnosed with syncope and left branch bundle block, in whom implantation of a DDD pacemaker was proposed. During the procedure, venous access was obtained via the left cephalic vein, and the lead was found to progress to the left of the spinal column, suggesting the presence of PLSVC. Through this access,

Congenital Submitral Diverticulum

A 36-year-old Guinean woman was referred to our center for a closer examination of systolic murmur. She had no cardiovascular complaints. Echocardiographic examinations depicted a large saccular structure at the left ventricular lateral and infer-lateral walls, in the submitral region (Figure 1). The outpouching structure was connected to the left ventricle (LV) through a large neck. In diastole, blood flowed from the LV into the cavity, and in systole blood flowed from the cavity into the LV. These findings suggested that this cavity itself contracted. No other cardiac abnormalities were found. To better delineate the whole shape of the cavity, a computed tomographic scan was requested. It allowed precise measurement of the outpouching structure (9 x 7 cm), delineation of its morphology and excluded coronary artery disease (Figure 2). To confirm whether the cavity wall consisted of muscle, a cardiac magnetic resonance study was performed; cardiac magnetic resonance cine and late gadolinium enhancement images clearly demonstrated a contractile cavity wall, consisting of muscle with no fibrosis (Figure 3). A diagnosis of large congenital submitral left ventricular diverticulum was made. The patient refused surgery and is followed up at our cardiology clinic. Left ventricle diverticulum is a rare condition and it is important to differentiate it from pseudoaneurysm. While the definitive diagnosis should be based on histopathologic evaluation, a review of the literature showed that there are different clinical and radiologic criteria for distinguishing these lesions. Cardiac magnetic resonance adequately characterizes these outpouchings, allowing accurate diagnosis, a better understanding of their natural history, and can guide proper management decisions. Figure 1.

Unexpected Mass in the Left Atrium

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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.

A Unique Case of Type-1 Facioscapulohumeral Muscular Dystrophy and Sarcomeric Hypertrophic Cardiomyopathy

This report describes a unique case of genetically confirmed overlap of type-1 facioscapulohumeral muscular dystrophy (FSHD1) and obstructive sarcomeric hypertrophic cardiomyopathy (sHCM). We present the case of a 37-year-old woman, diagnosed with FSHD1 and sHCM with substantial left ventricular (LV) hypertrophy and severe symptomatic LV outflow tract obstruction. Molecular analysis confirmed the presence of contraction of the specific 18Kb fragment in the D4Z4 locus of chromosome 4q and the haplotype 4qA, confirming the diagnosis of FSHD1, and the presence of the previously described c.1800.delA (p.Lys600Asnfs*) pathogenic mutation in the MYBPC3 gene, confirming the diagnosis of sHCM. The patient’s father had the FSHD1 phenotype, although molecular analysis was not available. The patient’s mother had the same pathogenic mutation on the MYBPC3 gene, although she had only a mild phenotype. The first symptoms of FSHD1 arose at age 5 years with decreased strength of facial muscles and inability to smile or whistle. In the third decade of life, an asymmetric reduction in upper limb strength developed. Neurological evaluation was remarkable for typical FSHD1 phenotype with bilateral peripheral facial paresis, atrophy of the pectoral muscles and winged scapulae. The electromyogram showed myopathic changes in the orbicularis and trapezius muscles. Creatinine kinase levels were normal. Also in the third decade of life, the patient developed effort dyspnea (New York Heart Association III functional class) and presyncope. N-terminal pro-B-type natriuretic peptide levels were increased (400 pg/mL). Electrocardiography revealed LV hypertrophy (voltage criteria) and T-wave inversion in the inferior leads. Serial echocardiograms showed septal asymmetric hypertrophy (interventricular septum = 25 mm, pulse wave = 13 mm), severe LV outflow tract obstruction (rest peak gradient = 40 mmHg; exercise stress peak gradient = 100 mmHg), anterior systolic motion of the anterior mitral leaflet associated with moderate mitral regurgitation, and grade II diastolic dysfunction. Episodes of nonsustained monomorphic ventricular tachycardia were found on 24-hour Holter monitoring. A single chamber implantable cardioverter-defibrillator was implanted. Titration of beta-blocker therapy was not tolerated (hypotension) and alcohol septal ablation was considered. Coronary angiography was performed, showing the presence of 2 septal arteries that were candidates for alcohol septal ablation. Although the first septal artery irrigated the region of interest, the contrast opacification was suboptimal. The second septal artery irrigated the right portion of the septum, inferior wall, and posteromedial papillary muscle. We considered that the risk-benefit ratio was unfavorable and the patient underwent surgical myectomy. The postoperative period was complicated by complete atrioventricular block, and the monochamber implantable cardioverter-defibrillator

HOW USEFUL IS 99MTC-DPD SCINTIGRAPHY IN DIAGNOSIS OF CARDIAC AMYLOIDOSIS IN TRANSTHYRETIN V30M FAMILIAL AMYLOID POLYNEUROPATHY?

Background: Previous studies suggested that 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scintigraphy may be useful for early diagnosis of hereditary transthyretin (TTR) related cardiac amyloidosis. However its diagnostic value in V30M TTR familial amyloid polyneuropathy (FAP) remains

Active fixation coronary sinus lead extraction – A safe procedure

© 2016 Sociedade Portuguesa de Cardiologia. Published by Elsevier Espana, S.L.U. All rights reserved.