Gustavo Lima da Silva

Triple-site pacing for cardiac resynchronization in permanent atrial fibrillation – Acute phase results from a prospective observational study

INTRODUCTION AND AIM Multi-site pacing is emerging as a new method for improving response to cardiac resynchronization therapy (CRT), but has been little studied, especially in patients with atrial fibrillation. We aimed to assess the effects of triple-site (Tri-V) vs. biventricular (Bi-V) pacing on hemodynamics and QRS duration. METHODS This was a prospective observational study of patients with permanent atrial fibrillation and ejection fraction <40% undergoing CRT implantation (n=40). One right ventricular (RV) lead was implanted in the apex and another in the right ventricular outflow tract (RVOT) septal wall. A left ventricular (LV) lead was implanted in a conventional venous epicardial position. Cardiac output (using the FloTrac™ Vigileo™ system), mean QRS and ejection fraction were calculated. RESULTS Mean cardiac output was 4.81±0.97 l/min with Tri-V, 4.68±0.94 l/min with RVOT septal and LV pacing, and 4.68±0.94 l/min with RV apical and LV pacing (p<0.001 for Tri-V vs. both BiV). Mean pre-implantation QRS was 170±25 ms, 123±18 ms with Tri-V, 141±25 ms with RVOT septal pacing and LV pacing and 145±19 with RV apical and LV pacing (p<0.001 for Tri-V vs. both BiV and pre-implantation). Mean ejection fraction was significantly higher with Tri-V (30±11%) vs. Bi-V pacing (28±12% with RVOT septal and LV pacing and 28±11 with RV apical and LV pacing) and pre-implantation (25±8%). CONCLUSION Tri-V pacing produced higher cardiac output and shorter QRS duration than Bi-V pacing. This may have a significant impact on the future of CRT.

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.

TCT-87 Transcatheter treatment of severe tricuspid regurgitation using the MitraClip® system: 30-day clinical results in 13 consecutive patients.

The aim of this study was to investigate the procedural feasibility and 30-day results of transcatheter tricuspid valve repair using the MitraClip® system in selected, highly symptomatic patients with severe tricuspid regurgitation. Thirteen consecutive patients were treated for severe symptomatic

Leukaemic myocardial infiltration presenting as acute heart failure

A 34-year-old man was admitted to our haematology department for M5b acute monocytic leukaemia associated with hyperleukocytosis (81.3 × 109/L). He underwent induction chemotherapy with 7+3 IDAC (cytarabin plus idarubicin) protocol. On the first day of therapy the patient developed acute heart failure (AHF). Transthoracic echocardiogram revealed severe concentric left ventricular (LV) hypertrophy with hyperechogenic ventricular walls, …

Taquicardia mediada por via Mahaim

We present the case of a previously healthy 42-year-old man who attended the emergency department due to a sudden onset of rapid and regular palpitations. The ECG showed 190 bpm, wide QRS with left bundle branch block tachycardia. He was started on amiodarone with progression to 230 bpm, wide QRS tachycardia with multiple morphologies, followed by spontaneous conversion to sinus rhythm, normal PR interval and rS pattern in LIII. The echocardiogram was negative for structural heart disease. The electrophysiological study demonstrated the presence of an accessory pathway with anterograde decremental conduction and no retrograde conduction. Both episodes of clinical tachycardia were induced. A diagnosis of Mahaim fiber-mediated antidromic atrioventricular reentrant tachycardia and pre-excited atrial fibrillation was made. Mapping was performed with detection of an M potential (His-like) at the lateral region of the tricuspid ring followed by radiofrequency ablation with immediate success criteria. Post-ablation there was a change to a qR pattern in LIII. At 12-months follow-up there was no recurrence of the tachycardia.

Surgical Epicardial CRT-D Implantation in a Patient with Complete Obstruction of the Superior Vena Cava

Current guidelines clearly define the subset of heart failure patients who benefit from device implantation.1 Although first-time trans-venous device implantation has a high success rate, some patients present complex and challenging technical problems.2

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

Synthesis of Spironucleosides: Past and Future Perspectives

Spironucleosides are a type of conformationally restricted nucleoside analogs in which the anomeric carbon belongs simultaneously to the sugar moiety and to the base unit. This locks the nucleic base in a specific orientation around the N-glycosidic bond, imposing restrictions on the flexibility of the sugar moiety. Anomeric spiro-functionalized nucleosides have gained considerable importance with the discovery of hydantocidin, a natural spironucleoside isolated from fermentation broths of Streptomyces hygroscopicus which exhibits potent herbicidal activity. The biological activity of hydantocidin has prompted considerable synthetic interest in this nucleoside and also in a variety of analogues, since important pharmaceutical leads can be found among modified nucleoside analogues. We present here an overview of the most important advances in the synthesis of spironucleosides.

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