Elevated Serum Levels of IgG4 in Patients with Heart Failure with Reduced Ejection Fraction: A Prospective Controlled Study.

(1) Background: Immunoglobulin gamma subclass 4 (IgG4) is a serum protein belonging to the immunoglobulin superfamily. It has a central role in certain immune-mediated conditions defined as IgG4-related disease. There is a paucity of data regarding the potential association of IgG4 and cardiovascular diseases. Our aim is to study the serum levels of IgG4 in patients with ischemic and non-ischemic dilated cardiomyopathy (DCM). (2) Methods: patients with ischemic and non-ischemic DCM were included in this study. Non-ischemic DCM was defined as a left ventricular ejection fraction (LVEF) < 40% without coronary artery disease (CAD). Ischemic DCM was defined as a LVEF < 40% and proven CAD. The serum concentrations of IgG4 were measured by turbidimetry. (3) Results: Overall 98 patients with cardiomyopathy had significantly higher levels of IgG4 compared with the control group (77.4 ± 64.0 vs. 50.3 ± 28.8 mg/dL, p < 0.01). Although there was no difference in the total IgG levels in patients with ischemic DCM, the serum concentrations of IgG4 were significantly higher than the corresponding values in the control group (89.8 ± 67.3 vs. 50.3 ± 28.8 mg/dL; interquartile ranges: 40.4−126.5 vs. 31.8−66.8 mg/dL, p < 0.01). This was altered by gender and smoking. (4) Conclusions: The patients with ischemic DCM had increased serum concentrations of IgG4. Future studies are warranted to explore the potential role of an IgG4-mediated process in patients with heart failure with reduced LVEF.

Transthyretin Cardiac Amyloidosis Scintigraphy Using Planar D-SPECT on Dedicated Cardiac CZT Camera.

BACKGROUND: Bone scintigraphy is a main diagnostic tool in suspected ATTR patients. Almost all literature is based on conventional whole body gamma cameras, and there is very sparse data evaluating the use of dedicated cardiac CZT cameras. The aim of this study was to evaluate the utility of bone scintigraphy in suspected transthyretin cardiac amyloidosis (ATTR-CA) patients on a dedicated cardiac CZT camera. METHODS: Seventy-three patients with suspected ATTR-CA underwent planar and SPECT Tc-99 m pyrophosphate scintigraphy using dedicated cardiac CZT camera between May and August 2019. RESULTS: Planar D-SPECT image quality was mostly good. Six patients were identified as ATTR-CA positive. Inter-observer agreement based on both Perugini score and on planar D-SPECT H/CL ratio was excellent. CONCLUSIONS: ATTR-CA scintigraphy using dedicated cardiac CZT camera was feasible, and yielded planar D-SPECT images with excellent inter-observer agreement.

Transthyretin cardiac amyloidosis in patients after TAVR: clinical and echocardiographic findings and long term survival.

AIMS: The aim of this study was to examine the prevalence of amyloid transthyretin (ATTR) cardiac amyloidosis in patients 1-2 years after trans-catheter aortic valve replacement (TAVR) and to assess their clinical and echocardiographic outcome and long-term survival. METHODS AND RESULTS: We enrolled 88 patients, mean age 81 years, 534 (390-711) days after TAVR. Patients underwent a Tc99m-PYP scintigraphy for the diagnosis of ATTR cardiac amyloidosis. Eleven (12.5%) participants were diagnosed with ATTR cardiac amyloidosis. Eighty eight per cent of patients without amyloidosis were in New York Heart Association Classes 1-2 after TAVR, compared with 64% patients with ATTR cardiac amyloidosis (P = 0.022). There were no differences in left ventricular (LV) ejection fraction (P = 0.69) between patients with and without ATTR cardiac amyloidosis at enrolment. The LV mass index and pulmonary artery pressure were significantly higher in patients with ATTR cardiac amyloidosis (P = 0.046 and P = 0.002, respectively). Global longitudinal strain and myocardial work efficiency were significantly lower in patients with ATTR cardiac amyloidosis (P = 0.031 and P = 0.048, respectively). We assessed changes in echocardiographic data, from the time of TAVR to enrolment, and as expected, there was a significant decrease in aortic valve gradient in both groups. There was a significant reduction in LV mass and LV mass index and improvement in basal segment LV strain in the ATTR cardiac amyloidosis negative group (P = 0.045, P = 0.046 and 0.023, respectively). However, in the ATTR cardiac amyloidosis group the change in LV mass and LV mass index and LV basal strain values was not significant (P = 0.24, P = 0.13 and P = 0.35, respectively). The were no significant changes in other echocardiographic parameters in both groups. The patients were followed for 1150 (1086-1221) days after enrolment. Twenty seven patients had at least one cardiac hospitalization during of follow up, of them seven were with ATTR cardiac amyloidosis and 20 patients without amyloidosis (P = 0.017). Eighteen patients (20%) died during follow up; 12 (14%) patients died due to cardiac causes. There was no difference in all-cause and cardiac mortality between patients with and without ATTR cardiac amyloidosis (P = 0.6 and P = 0.53, respectively). CONCLUSIONS: The long-term survival after TAVR is not significantly affected by the presence of ATTR cardiac amyloidosis. However, the clinical course of these patients and the LV hemodynamic improvement is less favourable. This hypothesis-generating study suggests screening for ATTR cardiac amyloidosis in patients who underwent TAVR and have limited clinical or echocardiographic improvement, because they may potentially improve with new therapies for ATTR cardiac amyolidosis.

Wild-type TTR amyloidosis among patients with unexplained heart failure and systolic LV dysfunction.

AIM: Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of heart failure (HF) with preserved left ventricular ejection fraction (LVEF), typically presenting as restrictive cardiomyopathy. The potential co-existence of ATTR-CA with systolic heart failure has not been studied. The aim of this study is to describe the prevalence of ATTR-CA and its clinical characteristics in HF patients with reduced LVEF. METHODS: Patients with an unexplained cause of LV systolic dysfunction were screened for ATTR-CA by a 99mTc-PYP planar scintigraphy. Patients in whom presence of ≥ 2 uptake was confirmed by SPECT imaging were included. Their clinical, laboratory and echocardiographic data were collected. RESULTS: Out of 75 patients (mean age 65±12 years, LVEF 35.8±7.9%) included in this study, 7 (9.3%) patients (mean age 75±6 years, LVEF 32.0±8.3%) had ATTR-CA. Patients with ATTR-CA were more symptomatic at diagnosis (NYHA FC 3-4 (86% vs 35% (p = 0.03)) and had a more severe clinical course evident by recurrent hospitalizations for HF, and a need for intravenous diuretic treatment (p = 0.04 and p<0.01, respectively) at follow-up, compared with patients with no ATTR-CA. Patients with ATTR-CA had similar LVEF but a clear trend for larger LV mass index (157.1±60.6 g/m2 vs. 121.0±39.5 g/m2, p = 0.07) and a larger proportions of ATTR-CA patients had IVS thickness >13 mm (57.1% vs 13.1%, p = 0.02) as compared to HF patients with no ATTR-CA. CONCLUSION: In our study, a meaningful percentage of patients with unexplained LV dysfunction had a co-existing ATTR-CA indicating that the clinical heterogeneity of ATTR-CA is much broader than previously thought.

Residual alterations of cardiac and endothelial function in patients who recovered from Takotsubo cardiomyopathy.

INTRODUCTION: Takotsubo cardiomyopathy (TCM) is characterized by transient left ventricle dysfunction. HYPOTHESIS: A residual cardiac and endothelial dysfunction is present in patients who recovered from TCM. METHODS: In this single-center prospective study, patients with prior TCM were included and followed for 6.4 ± 1.6 years. All underwent comprehensive cardiac function assessment, including tissue Doppler imaging (TDI) and 2-dimensional strain (2DS) echocardiography at their first visit. The number of circulating endothelial progenitor cells and levels of proangiogenic vascular endothelial growth factor (VEGF) and its receptor (VEGF-R) were measured. All measurements were compared with healthy controls. RESULTS: Forty-two women (age 58. ±8.6 years, LVEF 58.1 ± 6.1%) comprised the TCM group. Patients post-TCM had significantly lower early velocities E' (6 (5.0-8.0) vs. 9 (7.0-11.0) cm/s, p = .001) by TDI and higher E/E' ratio (p = .002), lower LV global average longitudinal strain (LGS) (-18.9 ± 3.5% vs. -21.7 ± 2.3%, p = .002) and RV LGS (-20.1 ± 3.9% vs. -23.4 ± 2.8%, p = .003) were evident. There was a trend toward a higher VEGF-R (p = .09) along with decreased VEGF/VEGF-R ratio representing inadequate VEGF production. In-hospital mortality was not reported and only two non-cardiac deaths occurred at long-term follow-up. CONCLUSIONS: Altered TDI and 2DS indices suggest residual biventricular myocardial injury in post-TCM patients with the apparent LV function recovery. Inappropriate production of VEGF and VEGF-R were observed, suggesting a possible underlying endothelial dysfunction in these patients.

Safety and Feasibility of MitraClip Implantation in Patients with Acute Mitral Regurgitation after Recent Myocardial Infarction and Severe Left Ventricle Dysfunction.

Patients with severe mitral regurgitation (MR) after myocardial infarction (MI) have an increased risk of mortality. Transcatheter mitral valve repair may therefore be a suitable therapy. However, data on clinical outcomes of patients in an acute setting are scarce, especially those with reduced left ventricle (LV) dysfunction. We conducted a multinational, collaborative data analysis from 21 centers for patients who were, within 90 days of acute MI, treated with MitraClip due to severe MR. The cohort was divided according to median left ventricle ejection fraction (LVEF)-35%. Included in the study were 105 patients. The mean age was 71 ± 10 years. Patients in the LVEF < 35% group were younger but with comparable Euroscore II, multivessel coronary artery disease, prior MI and coronary artery bypass graft surgery. Procedure time was comparable and acute success rate was high in both groups (94% vs. 90%, p = 0.728). MR grade was significantly reduced in both groups along with an immediate reduction in left atrial V-wave, pulmonary artery pressure and improvement in New York Heart Association (NYHA) class. In-hospital and 1-year mortality rates were not significantly different between the two groups (11% vs. 7%, p = 0.51 and 19% vs. 12%, p = 0.49) and neither was the 3-month re-hospitalization rate. In conclusion, MitraClip intervention in patients with acute severe functional mitral regurgitation (FMR) due to a recent MI in an acute setting is safe and feasible. Even patients with severe LV dysfunction may benefit from transcatheter mitral valve intervention and should not be excluded.

A novel monoclonal antibody targeting aggregated transthyretin facilitates its removal and functional recovery in an experimental model.

AIMS: Cardiac amyloidosis typically manifests as heart failure with preserved left ventricular function due to extracellular plaques comprising aggregated TTR. Despite recent success in halting disease progression with a TTR stabilizer and encouraging preliminary findings with TTR silencers, these agents are not targeting preexisting plaques. Herein, we report the development of a novel monoclonal antibody capable of attenuating experimental cardiac amyloidosis. METHODS AND RESULTS: We generated an IgG1 monoclonal antibody against aggregated TTR that immunoprecipitated the protein in the sera of patients with wild-type ATTR (wtATTR) and robustly stained cardiac plaques from patients. The antibody was shown to facilitate aggregated-TTR uptake by various myeloid cells and to protect cardiomyocytes from TTR-inducible toxicity. In a novel in vivo model of wtATTR amyloidosis, the antibody enhanced the disappearance of the pyrophosphate signals attesting for a rapid amyloid deposit removal and degradation and also exhibited improved echocardiographic measures of cardiac performance. Importantly, a capture ELISA developed based on the antibody exhibited higher levels of aggregated TTR in the sera of wtATTR amyloidosis patients as compared to control patients with heart failure suggesting a potential applicability in diagnosis and pharmacodynamic guidance of dosing. CONCLUSION: We developed a proprietary antibody targeting aggregated TTR that exhibits beneficial effects in a novel experimental wtATTR model and also possesses a potential diagnostic utility. The antibody could potentially be tested as a disease modifying agent in ATTR amyloidosis.

Pregnancy and Marfan syndrome.

Pregnancy in women with Marfan syndrome (MFS) presents challenges to the clinician and the patient due to the increased incidence of maternal complications and involvement of the fetus, and deserves special consideration. The leading cause of morbidity and mortality in MFS is aortic dissection. This article presents an extensive review of available clinical information and provides recommendations for the management of patients with MFS during pregnancy.

Pregnancy and the Risk of Spontaneous Coronary Artery Dissection: An Analysis of 120 Contemporary Cases.

BACKGROUND: Because of the rarity of this condition, information on pregnancy-associated spontaneous coronary artery dissection is limited. We reviewed a large number of contemporary pregnancy-associated spontaneous coronary artery dissection cases in an attempt to define the clinical characteristics and provide management recommendations. METHODS AND RESULTS: A literature search for cases of pregnancy-associated spontaneous coronary artery dissection reported between 2000 and 2015 included 120 cases; 75% presented with ST-segment-elevation myocardial infarction, and 80% had anterior myocardial infarction. Left anterior descending coronary artery was involved in 72% of cases, left main segment in 36%, and 40% had multivessel spontaneous coronary artery dissection. Ejection fraction was reduced to <40% in 44% of cases. Percutaneous coronary intervention was successful in only 50% of cases. Coronary artery bypass surgery was performed in 44 cases because of complex anatomy, hemodynamic instability, or failed percutaneous coronary intervention. Maternal complications included cardiogenic shock (24%), mechanical support (28%), urgent percutaneous coronary intervention (28%), urgent coronary artery bypass surgery (27.5%), maternal mortality (4%), and fetal mortality (2.5%). During follow-up for 305±111 days, there was a high incidence of symptoms because of persistent or new spontaneous coronary artery dissections, and 5 women needed heart transplantation or ventricular assist device implantation. CONCLUSIONS: Pregnancy-associated spontaneous coronary artery dissection is commonly associated with left anterior descending, left main, and multivessel involvement, which leads to a high incidence of reduced ejection fraction, and life-threatening maternal and fetal complications. Percutaneous coronary intervention is associated with low success rate and high likelihood of complications, and coronary artery bypass surgery is often required. Recurrent ischemic events because of persistent or new spontaneous coronary artery dissection are common during long-term follow-up.

Angiogenic Imbalance and Residual Myocardial Injury in Recovered Peripartum Cardiomyopathy Patients.

BACKGROUND: Recent studies suggest that angiogenic imbalance during pregnancy may lead to acute peripartum cardiomyopathy (PPCM). We propose that angiogenic imbalance and residual cardiac dysfunction may exist even after recovery from PPCM. METHODS AND RESULTS: Twenty-nine women at least 12 months after presentation with PPCM, who exhibited recovery of left ventricular (LV) ejection fraction (≥50%), were included in the study (mean age 35±6 years, LV ejection fraction 61.0±3.9%). The number of circulating endothelial progenitor cells (EPCs) and plasma levels of proangiogenic vascular endothelial growth factor and of soluble vascular endothelial growth factor receptor Flt1 (sFlt1) were measured. All patients underwent comprehensive cardiac function assessment, including tissue Doppler imaging and 2-dimensional (2D) strain echocardiography. All measurements were compared with healthy controls. Patients with a history of PPCM have significantly higher sFlt1 concentrations (median [25th-75th percentile]; 149.57, [63.14-177.89] versus 20.29, [15.00-53.89] pg/mL, P<0.001) and significantly decreased vascular endothelial growth factor/sFlt1 ratio (P=0.012) compared with controls, with a trend toward lower concentration of circulating CD34+/KDR+ levels. In addition, patients with PPCM had lower early velocities E' septal (9.9±2.1 versus 11.0±1.5 cm/s, P=0.02), with a significantly lower systolic velocity S' septal (7.6±1.2 versus 8.5±1.2 cm/s, P=0.003) by tissue Doppler imaging. Significantly lower LV global longitudinal (-19.1±3.3 versus -22.7±2.2%, P<0.001) and apical circumferential 2D strain (-16.6±4.9 versus -21.2±7.9, P=0.02) were present in patients with PPCM compared with controls. CONCLUSIONS: Higher concentration of sFlt1 with concomitant decreased circulating endothelial progenitor cell levels along with inappropriate attenuated vascular endothelial growth factor levels may imply an angiogenic imbalance that exists even after recovery and may thus predispose to PPCM. In addition, tissue Doppler imaging and 2D strain were able to identify residual myocardial injury in post-PPCM women with apparent recovery of LV systolic function. Both angiogenic imbalance and residual myocardial injury may play an important role in the recurrence of LV dysfunction during subsequent pregnancies.

Low circulating monocyte count is associated with severe aortic valve stenosis.

BACKGROUND: The pathophysiology of aortic stenosis (AS) involves inflammatory features including infiltration of the aortic valve (AV) by activated macrophages and T cells, deposition of lipids, and heterotopic calcification. OBJECTIVES: To evaluate the correlation between white blood cell (WBC) differential count and the occurrence and progression of AS. METHODS: We identified in our institutional registry 150 patients with AS who underwent two repeated echo studies at least 6 months apart. We evaluated the association between the average of repeated WBC differential counts sampled during the previous 3 years and subsequent echocardiographic AS indices. RESULTS: There was no significant difference in total WBC, lymphocyte or eosinophil count among mild, moderate or severe AS groups. There was a progressive decrease in monocyte count with increasing AS severity (P = 0.046), more prominent when comparing the mild and severe groups. There was a negative correlation between AV peak velocity or peak or mean gradient and monocyte count in the entire group (r = -0.31, -0.24, and -0.25 respectively, all P < 0.01). Similar partial correlations controlling for age, gender, hypertension, smoking, dyslipidemia and ejection fraction remained significant. The median changes over time in peak velocity and peak gradients in AS patients were 0.44 (0-1.3) m/sec/ year and 12 (0-39) mmHg/year, respectively. There was no correlation between any of the WBC differential counts and the change in peak velocity or peak gradient per year. CONCLUSIONS: Severe AS is associated with decreased total monocyte count. These findings may provide further clues to the mechanism underlying the pathogenesis of aortic stenosis.

Appropriateness and complications of the use of spironolactone in patients treated in a heart failure clinic.

OBJECTIVES: The widespread use of spironolactone in patients with congestive heart failure (CHF) has resulted in side effects and complications. We analyzed a cohort of patients treated by a dedicated CHF team, in order to examine the tolerability and safety of spironolactone in clinical practice. METHODS: We retrospectively evaluated data on 157 patients who were followed by the Heart Failure clinic of whom 100 patients on maximal treatment (all on ß blockers, 99% on ACE inhibitors) received spironolactone. The complications following spironolactone use were defined as: hyperkalemia with serum K 5.2 mEq/l; creatinine 2.0 mg/dl; hyponatremia with serum Na 135 mEq/l, hypotension and side effects such as gynecomastia and abdominal pain. RESULTS: At 1 year follow-up 6 patients developed hyperkalemia (range 5.3-5.9), 4 of them had K>5.5 mEq/l. Two patients developed hyponatremia. Six patients stopped spironolactone for: 1-gynecomastia, 2-worsening renal failure and hyperkalemia, 2-hyperkalemia (5.9 mEq/l) and 1 for bradycardia. There was an increase in mean creatinine level at 1 year (1.12±0.35 vs. 1.21±0.38 mg/dl, p=0.02), however, no significant changes were found in GFR (99.9±33.5 vs. 65.7±27.7 ml min(-1)1.73 m(-2), p=ns) and potassium (4.5±0.4 vs. 4.6±0.5 mEq/l, p=ns). We found improvement of GFR by >10% in 19 patients and worsening by >10% in 38 patients. No patient was hospitalized or required urgent treatment for spironolactone-related side effects. CONCLUSIONS: In patients with CHF on optimal therapy with ACE inhibitors and ß blockers appropriate spironolactone use and close follow-up by a dedicated HF team can minimize the risk for adverse events and complications.

Perimyocarditis following streptococcal group A infection: from clinical cases to bioinformatics analysis.

BACKGROUND: Streptococcal infection is known to be associated with non-suppurative complications, including rheumatic fever. A less well recognized complication is perimyocarditis. METHODS: We report 4 cases of myocarditis in young males associated with acute streptoccal infection. Following this clinical observation we employed bioinformatic techniques to identify common epitopes between Streptococcus group A and human muscle proteins. We used Blast to search all the proteome (1697 proteins) of the Streptococcus pyogenes M1 GAS against the human proteome of 34,180 proteins. RESULTS: 4 patients with streptococcal A related myocarditis were treated and made a complete recovery. One cardiac protein, ATP2A2 (NP_733765.1)), a cardiac Ca2+ ATPase, shared an epitope with Streptococcus group A and a high probability of being presented on a MHC Class II molecule. CONCLUSION: Streptococcal myocarditis may be a commoner entity than previously appreciated. Bioinformatic techniques have identified a suspected common epitope between the streptococcal proteins and a cardiac Ca2+ ATPase.

Coronary revascularization alone or with mitral valve repair: outcomes in patients with moderate ischemic mitral regurgitation.

We sought to evaluate retrospectively the outcomes of patients at our hospital who had moderate ischemic mitral regurgitation and who underwent coronary artery bypass grafting (CABG) alone or with concomitant mitral valve repair (CABG+MVr).A total of 83 patients had a reduced left ventricular ejection fraction and moderate mitral regurgitation: 28 patients underwent CABG+MVr, and 55 underwent CABG alone. Changes in mitral regurgitation, functional class, and left ventricular ejection fraction were compared in both groups.The mean follow-up was 5.1 +/- 3.6 years (range, 0.1-15.1 yr). Reduction of 2 mitral-regurgitation grades was found in 85% of CABG+MVr patients versus 14% of CABG-only patients (P < 0.0001) at 1 year, and in 56% versus 14% at 5 years, respectively (P = 0.1), as well as improvements in left ventricular ejection fraction and functional class. One- and 5-year survival rates were similar in the CABG+MVr and CABG-only groups: 96% +/- 3% versus 96% +/- 4%, and 87% +/- 5% versus 81% +/- 8%, respectively (P = NS). Propensity analysis showed similar results. Recurrent (3+ or 4+) mitral regurgitation was found in 22% and 47% at late follow-up, respectively.In patients with moderate ischemic mitral regurgitation, either surgical approach led to an improvement in functional class. Early and intermediate-term mortality rates were low with either CABG or CABG+MVr. However, an increased rate of late recurrent mitral regurgitation in the CABG+MVr group was observed.

An unusual case of allograft neutrophilic myocarditis mimicking acute myocardial infarction in a post-heart transplant patient.

The clinical presentation of myocarditis is variable and can mimic myocardial infarction. The diagnosis of acute myocarditis is frequently empiric, and is made on the basis of the clinical presentation, electrocardiographic changes, elevated cardiac enzymes, and lack of epicardial coronary artery disease and lymphocytic infiltration on myocardial biopsy. We present an unusual case of a young patient with history of heart transplantation who presented with fever and polyarthritis and developed chest pain along with electrocardiographic changes and troponin elevation with no evidence of coronary artery disease. His myocardial biopsy revealed marked neutrophilic infiltration and no evidence of rejection. The clinical picture was compatible with a rare finding of neutrophilic myocarditis in the allograft, possibly related to a systemic inflammatory process.

Reduction in mitral regurgitation in patients undergoing cardiac resynchronization treatment: assessment of predictors by two-dimensional radial strain echocardiography.

BACKGROUND: We utilized the novel approach of 2D radial strain (2-DRS) to evaluate whether left ventricular (LV) mechanical dyssynchrony in mid-LV segments corresponding to papillary muscles insertion sites can predict early mitral regurgitation (MR) reduction post-cardiac resynchronization therapy (CRT). METHODS: We evaluated 32 patients undergoing CRT (mean age 64 +/- 17 years, 54% males) with MR grade > or =3 determined by the MR jet area/left atrial area ratio (JA/LAA). RESULTS: Fifteen (47%) patients responded to CRT (JA/LAA) < 25%). Sixty-seven percent of responders had mild or no residual MR and 33% had mild-to-moderate MR, while 70% of nonresponders had grade 3 or 4 MR (P = 0.0001) post CRT. The percent reduction in LV end-systolic volume was significantly higher in responders (P = 0.03), as was improvement in LVEF (P = 0.007). Significant delay of time-to-peak 2-DRS in the midposterior and inferior segments prior to CRT was found in responders compared with nonresponders (580 +/- 58 vs. 486 +/- 94, P = 0.002 and 596 +/- 79 vs. 478 +/- 127 ms, P = 0.005, respectively). Responders also had higher peak positive systolic 2-DRS in the posterior and inferior segments compared to nonresponders (22 +/- 13 vs. 12 +/- 7%, P = 0.01 and 17 +/- 9 vs. 9 +/- 7%, P = 0.02, respectively). Logistic regression analysis showed that the differences in pre-CRT inferoanterior time-to-peak 2-DRS of >110 ms and MRJA/LAA <40% as well as 2-DRS >18% in the posterior wall were significant predictors of post-CRT improvement in MR. CONCLUSION: The presence of a significant time-to-peak delay on 2-DRS between inferior and anterior LV segments, preserved strain of posterior wall, and MRJA/LAA <40% were found to be associated with significant MR reduction in patients post-CRT.

Intravenous immunoglobulin treatment for acute fulminant inflammatory cardiomyopathy: series of six patients and review of literature.

BACKGROUND: Although an autoimmune mechanism has been postulated for myocarditis and acute-onset inflammatory dilated cardiomyopathy (DCM), immunomodulatory treatment strategies are still under investigation. METHODS AND RESULTS: The clinical data of six patients with acute inflammatory DCM referred for evaluation for possible heart transplantation were reviewed. All patients were admitted with acute congestive heart failure and severely impaired left ventricular (LV) function and were treated with high-dose (2 g/kg) intravenous immunoglobulin (IVIG). The diagnosis of acute inflammatory DCM was based on recent onset of congestive heart failure (New York Heart Association functional class III or IV) with severely depressed LV ejection fraction ([LVEF] 30% or lower) occurring shortly after viral-like illness. All patients had inflammation on endomyocardial biopsy or elevated cardiac enzymes, as well as a normal coronary angiogram. All patients were in New York Heart Association class I or II at the time of hospital discharge. The mean LVEF improved from 21.7+/-7.5% at baseline to 50.3+/-8.6% at discharge (P=0.005). Four patients had complete recovery (LVEF 50% or higher) and two patients had partial LV recovery. Patients were followed for a median 13.2 months (range two to 24 months) and had a mean LVEF of 53+/-6% (P not significant versus LVEF at discharge). CONCLUSIONS: Therapy with intravenous high-dose IVIG may be a potentially useful treatment in selected patients if given early in the course of acute fulminant inflammatory DCM. A randomized, prospective trial is warranted to prove the real benefit of IVIG in this patient population.