Clinical and echocardiographic presentation of postmyocardial infarction papillary muscle rupture.

BACKGROUND: Severe mitral regurgitation (MR) can occur following myocardial infarction (MI) with either partial or complete papillary muscle rupture (pPMR or cPMR). Although the incidence of this complication has significantly decreased, it is still associated with significant mortality. We sought to evaluate the different echocardiographic and clinical presentations of pPMR and cPMR. METHODS AND RESULTS: A review of all the urgent procedures for ischemic MR between January 2000 and June 2016 was performed to identify patients who underwent surgery for PMR. Surgical protocols and echocardiographic studies were used to identify patients with cPMR and pPMR. A total of 37 patients had cardiac surgery for PMR (18 cPMR, 19 pPMR). All patients with cPMR were in cardiogenic shock at the time of diagnosis, as opposed to only 53% of patients with pPMR (P = 0.0008). Between the time of diagnosis and surgery, 7 patients with pPMR developed cardiogenic shock. Transthoracic echocardiography (TTE) led to the diagnosis in 72% of cPMR and 32% of pPMR (P = 0.02). TEE had a yield of 100% for both cPMR and pPMR. Six pathologic varieties of post-MI PMR were recognized on echocardiography and during surgery. Early postoperative, 1 (72% vs 84%), 3 (67% vs 84%), and 5 years (67% vs 74%) survival rates were similar for cPMR and pPMR (P = 0.26). CONCLUSIONS: Partial PMR is associated with a different clinical and echocardiographic presentation than cPMR. Still, most pPMR patients progress toward cardiogenic shock. Prompt diagnosis and referral for surgery are critical and could potentially decrease mortality.

Early Use of Cardiac Resynchronization Therapy to Accelerate Symptomatic Relief and Complete Left Ventricular Function Recovery in Peripartum Cardiomyopathy.

Peripartum cardiomyopathy (PPCM) is a rare cause of heart failure that develops during the last month of pregnancy or within first months of delivery. We report the case of a 40-year-old woman diagnosed with severely symptomatic PPCM characterized by left ventricular ejection fraction (LVEF) of 10% and significant dyssynchrony secondary to a left bundle branch block (LBBB). Early cardiac resynchronization therapy (CRT) was used to achieve remarkable functional and LVEF recovery. This case suggests that early CRT must be considered for patients suffering from severely symptomatic PPCM despite optimal medical therapy for whom advanced heart failure therapies are proposed.

Prevalence and Impact of Prosthesis-Patient Mismatch Following Surgical Aortic Valve Replacement for Pure Aortic Regurgitation.

BACKGROUND: Prosthesis-patient mismatch (PPM) is highly prevalent among patients undergoing aortic valve replacement (AVR) to treat aortic stenosis. Data regarding the prevalence and impact of PPM on left ventricular remodeling and outcomes in patients who have undergone surgical AVR to treat pure severe aortic regurgitation (AR) are, however, scarce. METHODS: A retrospective analysis was conducted of clinical and echocardiographic data acquired from 50 consecutive patients with pure severe AR, without evidence of significant coronary artery disease, who underwent AVR between 2004 and 2010 at the authors' institution. PPM was defined as a projected in vivo effective orifice area (EOA) 0.85 cm2/m2. RESULTS: The incidence of PPM was 16%, but no severe mismatch occurred. At a mean follow up of 52 ± 39 months, event-free survival (a composite of all-cause mortality and hospitalization for cardiovascular causes) was similar between patients with and without PPM (p = 0.73). Within seven days after surgery, mean reductions in indexed left ventricular end-diastolic diameter (LVEDD) and indexed left ventricular end-systolic diameter (LVESD) were similar between patients with and without PPM [4.4 mm/m2 versus 5.0 mm/m2; p = 0.67 and 1.6 mm/m2 versus 2.2 mm/m2; p = 0.35, respectively]. At follow up, no difference was observed for mean reductions in indexed LVEDD and indexed LVESD [6.9 mm/m2 versus 7.1 mm/m2; p = 0.91 and 4.1 mm/m2 versus 5.1 mm/m2; p = 0.57, respectively], and mean improvement in left ventricular ejection fraction (4.4% versus 5.1%; p = 0.87). CONCLUSIONS: PPM occurs less frequently in patients undergoing AVR for pure severe AR than for aortic stenosis, and seems to have a less significant impact on ventricular remodeling and outcomes.

Clinical Presentation and Value of Echocardiography in the Diagnosis of Freestyle Aortic Bioprosthesis Leaflet Tears: A Retrospective Study.

BACKGROUND: The unique design of the Freestyle stentless aortic bioprosthesis has led to different mechanisms of failure, particularly leaflet tearing. The aim of this retrospective study was to review the clinical presentation and echocardiographic data of symptomatic patients with leaflet tears and significant aortic regurgitation (AR) following implantation of the Freestyle bioprosthesis. METHODS: Between January 1993 and May 2011, a total of 430 consecutive patients was identified at the authors' institution who had undergone primary aortic valve replacement with a Freestyle stentless aortic bioprosthesis. Clinical and echocardiographic data were collected prospectively for all patients. Structural valve deterioration was the major cause of bioprosthetic valve failure. RESULTS: Twenty symptomatic patients presented with significant AR due to leaflet tears in the absence of more than mild valvular calcification. At presentation, all patients complained of dyspnea. Some 50% of patients (n = 10) presented with acute pulmonary edema, and 10% (n = 2) with cardiogenic shock. A leaflet tear was initially diagnosed using transthoracic echocardiography in five cases (25%), using transesophageal echocardiography (TEE) in eight cases (40%), or at surgery in seven cases (35%). An appropriate diagnosis of leaflet tearing was recognized at surgery in more than one-third of patients. Consequently, clinicians must be aware of the variety of clinical presentations and should have a high degree of suspicion regarding leaflet tears in patients who have received a Freestyle stentless aortic bioprosthesis and present with moderate to severe AR. CONCLUSIONS: For the optimal management of patients with Freestyle stentless aortic bioprosthesis and new moderate to severe AR, TEE should be considered in all patients.

Outcomes following surgical correction of pure aortic regurgitation in presence or absence of significant functional mitral regurgitation.

BACKGROUND: Functional mitral regurgitation (MR) can occur secondary to severe aortic regurgitation (AR). However, data on the overall impact of mitral surgical intervention after aortic valve replacement (AVR) are scarce. We sought to study the left ventricular (LV) remodeling process and determine predictors of clinical outcomes of patients with pure severe AR in presence or absence of significant functional MR. METHODS: Patients were categorized into AR-MR group (≤ mild MR; n = 51, 76%) and AR + MR group (≥ moderate MR; n = 16, 24%). All patients in the AR + MR group underwent AVR and MR correction. Serial echocardiographic measurements and clinical follow-up up to 5 years were obtained in all patients. RESULTS: Significant reverse LV remodeling occurred in both groups compared with baseline. No 30-day deaths occurred. Mortality and heart failure-related hospitalization rates, at follow-up, were significantly higher in the AR + MR group (19% vs. 2%, P = 0.04 and 38% vs. 12% P = 0.03, respectively), but a similar proportion of patients from both groups was in New York Heart Association class I or II (87% vs. 92%, P = 0.62). Preoperative indexed stroke volume (SV) <50 mL/m2 was the only independent predictor of death and/or rehospitalization after surgery (odds ratio: 61.1, [95% CI, 12.6­425.2]; P < 0.0001). CONCLUSION: Despite being a higher risk population, patients with moderate-to-severe functional MR secondary to severe AR experience similar postoperative mortality at the expense of a moderately higher 5-year overall mortality, rate of hospitalization for congestive heart failure, and medication use. Preoperative indexed SV < 50 mL/m2 may be helpful in predicting long-term outcomes.

Concomitant symptomatic cardiac sarcoidosis and systemic sclerosis with cardiac involvement: a case report.

Sarcoidosis and systemic sclerosis are two inflammatory multisystemic disorders of unknown etiology that may be life-threatening especially when there is cardiac involvement. Both diseases may coexist, however, there are very few case reports of patients with both cardiac sarcoidosis and systemic sclerosis in the literature. We report the case of a 72-year-old female who was initially referred for dyspnea. A chest computed tomography scan showed multiple hilar and mediastinal adenopathy with a non-specific opacity in the middle pulmonary lobe. FDG-PET-scan showed increased FDG uptake in the adenopathy, the middle lobe and the right ventricular free wall. Sarcoidosis was confirmed with a lung biopsy. Both electrocardiogram and echocardiogram were normal. Four months later, the patient developed a high-grade atrioventricular block deemed secondary to her cardiac sarcoidosis. Two years later, the patient was referred to a rheumatologist for severe Raynaud's symptoms, sclerodactyly and acrocyanosis. After thorough investigations, a diagnosis of limited cutaneous systemic sclerosis with systemic and cardiac sarcoidosis was made. This case demonstrates that both cardiac sarcoidosis and systemic sclerosis may coexist. In the literature, either disease may come first. In cases where cardiac symptoms appear after the diagnosis of concomitant sarcoidosis and systemic sclerosis, it might be difficult for clinicians to confirm which disease is responsible for the heart involvement. This is important since early cardiac sarcoidosis treatment should be done to prevent major complications and may well differ from systemic sclerosis treatment. In this review, we discuss the main clinical manifestations and imaging findings seen with cardiac disease secondary to sarcoidosis and systemic sclerosis.

Relation of mitral valve morphology and motion to mitral regurgitation severity in patients with mitral valve prolapse.

BACKGROUND: Mitral valve thickness is used as a criterion to distinguish the classical from the non-classical form of mitral valve prolapse (MVP). Classical form of MVP has been associated with higher risk of mitral regurgitation (MR) and concomitant complications. We sought to determine the relation of mitral valve morphology and motion to mitral regurgitation severity in patients with MVP. METHODS: We prospectively analyzed transthoracic echocardiograms of 38 consecutive patients with MVP and various degrees of MR. In the parasternal long-axis view, leaflets length, diastolic leaflet thickness, prolapsing depth, billowing area and non-coaptation distance between both leaflets were measured. RESULTS: Twenty patients (53%) and 18 patients (47%) were identified as having moderate to severe and mild MR respectively (ERO = 45 ± 27 mm² vs. 5 ± 7 mm², p < 0.001). Diastolic leaflet thickness was similar in both groups (5.5 ± 0.9 mm vs. 5.3 ± 1 mm, p = 0.57). On multivariate analysis, the non-coaptation distance (OR 7.9 per 1 mm increase; 95% CI 1.72-37.2) was associated with significant MR. Thick mitral valve leaflet as traditionally reported (≥ 5 mm) was not associated with significant MR (OR 0.9; 95% CI 0.2-3.4). CONCLUSIONS: In patients with MVP, thick mitral leaflet is not associated with significant MR. Leaflet thickness is probably not as important in risk stratification as previously reported in patients with MVP. Other anatomical and geometrical features of the mitral valve apparatus area appear to be much more closely related to MR severity.

Usefulness and limitation of dobutamine stress echocardiography to predict acute response to cardiac resynchronization therapy.

BACKGROUND: It has been hypothesized that a long-term response to cardiac resynchronization therapy (CRT) could correlate with myocardial viability in patients with left ventricular (LV) dysfunction. Contractile reserve and viability in the region of the pacing lead have not been investigated in regard to acute response after CRT. METHODS: Fifty-one consecutive patients with advanced heart failure, LV ejection fraction 120 ms, and intraventricular asynchronism >or= 50 ms were prospectively included. The week before CRT implantation, the presence of viability was evaluated using dobutamine stress echocardiography. Acute responders were defined as a >or=15% increase in LV stroke volume. RESULTS: The average of viable segments was 5.8 +/- 1.9 in responders and 3.9 +/- 3 in nonresponders (P = 0.03). Viability in the region of the pacing lead had an excellent sensitivity (96%), but a low specificity (56%) to predict acute response to CRT. Mitral regurgitation (MR) was reduced in 21 patients (84%) with acute response. The presence of MR was a poor predictor of response (sensibility 93% and specificity 17%). However, combining the presence of MR and viability in the region of the pacing lead yields a sensibility (89%) and a specificity (70%) to predict acute response to CRT. CONCLUSION: Myocardial viability is an important factor influencing acute hemodynamic response to CRT. In acute responders, significant MR reduction is frequent. The combined presence of MR and viability in the region of the pacing lead predicts acute response to CRT with the best accuracy.

Contractile reserve assessed using dobutamine echocardiography predicts left ventricular reverse remodeling after cardiac resynchronization therapy: prospective validation in patients with left ventricular dyssynchrony.

BACKGROUND: The presence of viable myocardium may predict response to cardiac resynchronization therapy (CRT). The aim of this study is to evaluate in patients with left ventricular (LV) dyssynchrony whether response to CRT is related to myocardial viability in the region of the pacing lead. METHODS: Forty-nine consecutive patients with advanced heart failure, LV ejection fraction < 35%, QRS duration > 120 ms and intraventricular asynchronism > or = 50 ms were included. Dobutamine stress echocardiography was performed within the week before CRT implantation. Resting echocardiography was performed 6 months after CRT implantation. Viability in the region of LV pacing lead was defined as the presence of viability in two contiguous segments. Response to CRT was defined by evidence of reverse LV remodeling (> or =15% reduction in LV end-systolic volume). RESULTS: Thirty-one patients (63%) were identified as responders at follow-up. The average of viable segments was 5.9 +/- 2 in responders and 3.2 +/- 3 in nonresponders (P = 0.0003). Viability in the region of the pacing lead had a sensitivity of 94%, a specificity of 67%, a positive predictive value of 83%, and a negative predictive value of 86% for the prediction of response to CRT. CONCLUSIONS: In patients with LV dyssynchrony, reverse remodeling after CRT requires viability in the region of the pacing lead. This simple method using echocardiography dobutamine for the evaluation of local viability (i.e., viability in two contiguous segments) may be useful to the clinician in choosing the best LV lead positioning.

Complex drug interactions in an HIV-seropositive heart transplant recipient.

Highly active antiretroviral therapy has led to greater life expectancy for human immun-deficiency virus (HIV)-positive patients. This was a report of 11 years of follow-up of an HIV-seropositive patient who underwent heart transplantation in 2006, with emphasis on the management challenges of complex drug interactions over time.

Is heart transplantation a valuable option in patients with diffuse systemic sclerosis and primary cardiac involvement?

Systemic sclerosis patients with primary cardiac involvement can be reliably diagnosed by cardiac magnetic resonance imaging and are associated with a poor prognosis. This case report highlights the importance of considering heart transplantation in those patients as a lifesaving procedure.

Usefulness of Left Ventricular Assist Device in the Recovery of Severe Amphetamine-Associated Dilated Cardiomyopathy.

Exposure to synthetic drugs such as amphetamines may lead to significant consequences on the cardiovascular system. The prognosis of young adults with amphetamine-induced cardiomyopathy remains poor. We present 2 young patients (aged <40 years) who developed severe dilated cardiomyopathy after chronic amphetamine use. Thorough psychological evaluations demonstrated favorable features with patients being reliable and committed to their health problems. A left ventricular assist device (HeartMate II; Abbott, Chicago, IL) was implanted in the patients shortly after admission to optimize hemodynamic support and evaluate the potential for recovery. Within less than 1 year, significant improvement was achieved and successful explantation of left ventricular assist device was performed in both patients.

Usefulness of NT-pro BNP monitoring to identify echocardiographic responders following cardiac resynchronization therapy.

BACKGROUND: Cardiac resynchronization therapy (CRT) improves left ventricular (LV) volumes, mitral regurgitation (MR) severity and symptoms of patients with heart failure (HF). However, >or= 30% of patients have no significant clinical or echocardiographic improvement following CRT. Reverse remodeling after CRT correlates with improved clinical outcomes. We hypothesized that in NT-pro BNP monitoring is accurate to identify responders following CRT. METHODS: 42 consecutive patients (mean age 66 +/- 12 years, male 68%) with HF undergoing CRT were prospectively enrolled. Responders at follow-up were defined by echocardiography (decrease in LV end systolic volume >or= 15%). Echocardiography and NT-pro BNP measurement were performed at baseline and repeated 3 to 6 month after CRT. RESULTS: There was no significant difference between responders (n = 29, 69%) and non-responders (n = 13, 31%) regarding baseline NT-pro BNP level. Responders had significantly higher decrease in NT-pro BNP levels during follow-up than non-responders (absolute: -1428 +/- 1333 pg x ml-1 vs. -61 +/- 959 pg x ml-1, p = 0.002; relative: -45 +/- 28% vs. 2 +/- 28%, p < 0.0001). A decrease of >or= 15% in NT-pro BNP 3-6 months after CRT identifies echocardiographic responders with a sensitivity of 90% and a specificity of 77%. CONCLUSION: NT-pro BNP monitoring can accurately identify echocardiographic responders after CRT.

What the Cardiologist Should Know About Mitochondrial Cardiomyopathy?

Mitochondrial diseases are a heterogeneous group of rare hereditary disorders that may manifest with single organ involvement or as multisystemic disease. The pathophysiology of mitochondrial disease is complex and related to mutations of genes encoding mitochondrial proteins that are crucial to the cellular respiratory chain. Given its almost exclusive aerobic metabolism, the heart is particularly susceptible to mitochondrial dysfunction and commonly involved in mitochondrial disorders. Various clinical presentations are described, making clinical recognition challenging. Some patients may evolve towards the early need for heart transplantation, which emphasizes the importance of appropriate diagnosis and referral to a specialized centre.

Atypical Presentation of Aspergillus Mediastinitis Infection in a Heart Transplant Patient: the Importance of Combined Medical and Surgical Treatment.

Aspergillus fumigatus is an opportunistic fungus that mainly affects immunocompromised patients. Due to significant immunosuppressive therapy, patients who undergo orthotopic heart transplant have an increased risk of infection. Aspergillosis is the most common fungal infection in orthotopic heart transplant recipients (70%) and usually presents as invasive aspergillosis, which has a rapidly progressive course and is highly fatal. In heart transplant patients with invasive aspergillosis, overall mortality may range from 53% to 78%. Aspergillus mediastinitis infection is somewhat rare in orthotopic heart transplant recipients, with only 6 reported cases. Treatment may require early surgical drainage and antifungal therapy. We present the case of a 50-year-old man who developed Aspergillus mediastinitis 1 year after heart transplant surgery. This case illustrates the diagnostic challenge of an atypical presentation of Aspergillus mediastinitis and the importance of multiple drainage procedures in refractory disease, combined with long-term antifungal therapy.

The dosage of chromatin proteins affects transcriptional silencing and DNA repair in Saccharomyces cerevisiae.

Alterations in protein composition or dosage within chromatin may trigger changes in processes such as gene expression and DNA repair. Through transposon mutagenesis and targeted gene deletions in haploids and diploids of Saccharomyces cerevisiae, we identified mutations that affect telomeric silencing in genes encoding telomere-associated Sir4p and Yku80p and chromatin remodeling ATPases Ies2p and Rsc1p. We found that sir4/SIR4 heterozygous diploids efficiently silence the mating type locus HMR but not telomeres, and diploids heterozygous for yku80 and ies2 mutations are inefficient at DNA repair. In contrast, strains heterozygous for most chromatin remodeling ATPase mutations retain wild-type silencing and DNA repair levels. Thus, in diploids, chromatin structures required for DNA repair and telomeric silencing are sensitive to dosage changes.

Dynamic left ventricular dyssynchrony and severe mitral regurgitation caused by exercise: should we go beyond the guidelines?

Guidelines for cardiac resynchronization therapy (CRT) have been established, but there may be a subgroup of patients not identified in these guidelines who may benefit from this therapy. We report a patient with a dynamic left ventricular dyssynchrony and severe mitral regurgitation caused by exercise successfully treated with CRT. Exercise testing should be considered in patients with left ventricular ejection fraction <35% and QRS <130 ms with severe heart failure symptoms that are unexplained by rest echocardiography evaluation in order to rule out ischemia and/or dynamic left ventricular dyssynchrony. In the presence of exercise-induced left ventricular bundle branch block, the implantation of CRT should be contemplated.

Direction of persistent ischemic mitral jet after restrictive valve annuloplasty: implications for interpretation of perioperative echocardiography.

BACKGROUND: Ischemic mitral regurgitation (MR) often persists after restrictive mitral valve annuloplasty (MVA) and is associated with a poor prognosis. It was hypothesized that the anterior displacement of the posterior aspect of the annulus caused by annuloplasty could induce a change in the direction of MR jet. METHODS: The echocardiograms of 21 patients who underwent restrictive MVA for ischemic MR and who had detectable postoperative MR were analyzed before and early after surgery to evaluate the direction of MR jet. RESULTS: The MR jet direction was posterior in 15 patients (72%) and central or anterior in six patients (28%) before the operation, compared with four patients (20%) and 17 patients (80%), respectively, after MVA (P<0.001). Overall, the jet direction was modified in 16 of 21 patients (76%) following MVA. Among the subset of 11 patients with clinically significant persistent MR (vena contracta width greater than 3 mm), the MR jet direction changed in nine patients (82%) compared with their preoperative evaluation. Importantly, the initial clinical interpretation, based on a subjective evaluation, had classified MR severity as nonsignificant in six of 11 patients (55%), likely due to the eccentricity of the jet and its change in direction. CONCLUSION: The direction of the persistent MR jet early after annuloplasty is often different from that of preoperative MR jet and may lead to significant misinterpretation of the postoperative echocardiogram.

Very Late Continued Reverse Remodelling After Cardiac Resynchronization Therapy in Patients With Extreme Left Ventricular Dilatation.

Response to cardiac resynchronization therapy (CRT) varies greatly among patients. We present 2 patients with severe heart failure symptoms (New York Heart Association class IV) and extreme initial left ventricular (LV) dilatation (LV end-diastolic diameter of 92 mm and 80 mm, respectively) and severe functional mitral regurgitation who underwent CRT device implantation. Long-term follow-up showed late (≥ 4 years) normalization of LV ejection fraction (LVEF), LV dimensions, and functional status. In a subgroup of patients with nonischemic dilated cardiomyopathy and complete left bundle branch block, late continued LV reverse remodelling may lead to normalization of LV volumes and LVEF and significant improvement in functional class.

Management of Loeffler's Endocarditis With Bivalvular Involvement and a FIP1L1/PDGFRA-Negative Mutation.

Hypereosinophilic syndromes (HESs) are a group of disorders characterized by end-organ damage caused by eosinophilic infiltration. We present a patient with idiopathic HES with severe tricuspid and mitral regurgitation secondary to Loeffler's endocarditis. In addition to prednisone, imatinib therapy initially helped control the eosinophil count. However, successful long-term remission was achieved with hydroxyurea therapy.