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.

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.

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.

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.

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.

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.

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.

Risk factors of mortality after surgical correction of ventricular septal defect following myocardial infarction: Retrospective analysis and review of the literature.

BACKGROUND: Rupture of the ventricular septum following acute myocardial infarction (AMI) is an uncommon but serious complication, usually leading to congestive heart failure and cardiogenic shock. Surgical repair is the only definitive treatment for this condition. METHODS: We review our experience of surgical repair of post-infarction ventricular septal defects (VSDs), analyze the associated risk factors and outcomes, and do a complete review of the literature. A retrospective study was performed on 34 consecutive patients who had undergone surgical repair for VSDs following AMI from December 1991 to July 2014. Preoperative, clinical and echocardiographic variables were studied by uni-and multivariate analyses. RESULTS: Mortality was analyzed for the entire group of patients. Mean age was 69 ± 7 years with 44% women. VSDs were anterior in 11 (32%) and posterior in 23 (68%) patients. A majority, 24 (71%) patients were in cardiogenic shock. Median interval from myocardial infarction to VSDs repair was 7 days. The 30 days operative mortality was 65%. Mortality within the posterior VSDs group was 74% and the anterior VSDs group was 46% (P=0.14). Concomitant coronary artery bypass graft (CABG) did not influence early or late survival. Multivariate analysis identified older age (HR=1.11, P=0.0001) and shorter time between AMI and surgery (HR=0.90, P=0.015) as independent predictors of 30-day and long-term mortality. CONCLUSION: In conclusion, surgical repair of post-AMI VSDs carries a high operative mortality. An algorithm of treatment for the management of these patients is suggested.

Usefulness of cardiac resonance imaging in Churg-Strauss syndrome.

: Churg-Strauss syndrome (CSS) is a rare entity that is characterized by widespread vasculitis, which affects both small and medium-sized blood vessels of nearly all organs. More than 50% of these cases have cardiac involvement, which is the major cause of morbidity and mortality. We describe a case of a patient with cardiac biopsy proven CSS, and we discuss the usefulness of cardiovascular MRI for its diagnosis.

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.

What the Cardiologist Should Know About Cardiac Involvement in Behçet Disease.

Behçet disease (BD) is a chronic multisystem inflammatory vasculitis affecting mainly young adults and is characterized by a remitting-relapsing course. In North America, the prevalence is 5.2 per 100,000 population. It is believed that cardiac involvement is one of the most severe complications in patients with BD despite its sporadic occurrence, being greatly correlated with mortality.

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.

When should we consider the diagnosis of giant cell myocarditis? Revisiting "classic" echocardiographic and clinical features of this rare pathology.

OBJECTIVES: Giant cell myocarditis is a rare and often fatal disorder. According to the American Heart Association, the American College of Cardiology Foundation, and the European Society of Cardiology scientific statements, an endomyocardial biopsy should be done to exclude giant cell myocarditis in unexplained new-onset heart failure of 2 weeks to 3 months duration associated with dilated left ventricle and new ventricular arrhythmias, or Mobitz type II second-degree, or third-degree atrioventricular heart block. CASE PRESENTATIONS: Two hundred thirty-five heart transplants were performed since May 1993 at the Institut universitaire de cardiologie et de pneumologie de Quebec, Canada. Giant cell myocarditis was found in the explanted hearts of 5 patients. The preoperative diagnosis of giant cell myocarditis was done by endomyocardial biopsy or at the installation of a left ventricular-assisted device. Patients had symptoms of progressive heart failure of subacute onset. Patients consulted at a mean 32 days after the onset of symptoms. Two patients neither had ventricular arrhythmia nor heart block. Two patients had ventricular arrhythmias and heart block; the other patient had symptomatic heart block. All patients had at least 2 echocardiographies. Two patients had an increase in left ventricular size, enough to reach the criteria of left ventricular dilatation according to the American Society of Echocardiography. During this time, left ventricular ejection fraction showed a rapid decline (mean 37% to 16%). CONCLUSIONS: Ventricular arrhythmia, heart block, and left ventricular dilatation initially can be absent in many patients having giant cell myocarditis with symptoms of progressive heart failure. Endo-myocardial biopsy should be quickly considered in patients with a rapid and dramatic decline of left ventricular ejection fraction, even in the absence of classic clinical and echocardiographic features of giant cell myocarditis to rapidly obtain the diagnosis of this rare but lethal disease.