Signs Of Right Heart Failure Research Articles

Echocardiographic diagnosis, management and monitoring of pulmonary embolism with right heart thrombus in a patient with myotonic dystrophy: a case report

Acute pulmonary embolism (PE) is a common disease which frequently results in life-threatening right ventricular (RV) failure. High-risk PE, presenting with hypotension, shock, RV dysfunction or right heart thrombus is associated with a high mortality, particularly during the first few hours. Accordingly, it is important to commence effective therapy as soon as possible. In the case described in this report, a 49-year-old woman with myotonic dystrophy type 1 presented with acute respiratory failure and hypotension. Transthoracic echocardiography showed signs of right heart failure and a mobile right heart mass highly suspicious of a thrombus. Based on echocardiographic findings, acute thrombolysis was performed resulting in hemodynamic stabilization of the patient and complete resolution of the right heart thrombus. This case underscores the important role of transthoracic echocardiography for the diagnosis, management and monitoring of PE and underlines the efficacy and safety of thrombolysis in the treatment of PE associated with right heart thrombus.

Long term outcome of mechanical valve prosthesis in the pulmonary position

Objectives Assessment of the long term outcome of mechanical valve prosthesis at pulmonary position in a population of grown-up congenital heart disease patients from a tertiary referral center. Methods From 1977 to 2007, 22 consecutive patients underwent a total of 25 pulmonary valve replacements with mechanical prosthesis. The most frequent underlying cardiac condition was tetralogy of Fallot ( n = 16, 64%) and the mean age at the time of pulmonary valve replacement was 32 ± 11 years (range 14–50 years). Results The postoperative mortality rate was 4% ( n = 1) with no late deaths documented after a mean follow-up of 7.6 ± 7.6 years (range 0.29–24 years). No major bleeding episodes occurred. Three patients presented with valve thrombosis in the setting of long term anticoagulation withdrawal and required valve re-replacement. Two of these patients, both with poor right ventricular function and overt clinical signs of right heart failure at the time of valve re-replacement, experienced further episodes of thrombosis despite correct anticoagulation. All episodes resolved with thrombolysis. After addition of antiplatelet treatment in one case and anticoagulation self-control, in the other, no further thrombosis has been documented. Conclusions Mechanical valve prosthesis may be an alternative to tissue valve prosthesis in patients with congenital heart disease requiring pulmonary valve replacement. Optimal anticoagulation is crucial and additional antiplatelet treatment should be considered. Our data also suggest that patients with severe right ventricular dysfunction and congestive heart failure might be at particular risk for valve thrombosis.

Ebstein’s malformation presenting with tricuspid stenosis: 1-year follow-up after surgical repair

This is a case of a young woman previously completely healthy, with two uneventful pregnancies and deliveries, who presented with bloating, shortness of breath, and signs of right heart failure. A thorough clinical evaluation, along with a work-up including an echocardiogram and a magnetic resonance imaging revealed the diagnosis of Ebstein's anomaly malformation with tricuspid stenosis. A right ventricular outflow tract obstruction due to thrombus formation was thought to exacerbate her symptoms and lead to the diagnosis. Surgery in the form of right ventricular thrombectomy, right atrial reduction, porcine tricuspid valve replacement and bidirectional cavopulmonary anastomosis "bidirectional Glenn", was successfully performed. The patient has been stable clinically more than one year after surgery. Discussion about this rare condition and operative details are provided.

Diagnostik der pulmonalen Hypertonie

Commonly, pulmonary hypertension is clinically suspected because of unexplained exertional dyspnoea or as a chance finding in clinical examination revealing signs of right heart failure. The systematic diagnostic approach and exact classification is based on the Venice classification. Basic investigations include ECG, chest radiograph, lung function studies and echocardiography. Echocardiography is the most important investigation for the diagnosis of pulmonary hypertension. It also serves as non invasive control during treatment and as the main screening test for pulmonary hypertension. Echocardiographic criteria of pulmonary hypertension are a dilated and hypertrophied right ventricle, paradoxic septum movement, a dilated right atrium, and a distended inferior Vena cava. Using Doppler echocardiography, the right ventricular and thus pulmonary arterial systolic pressure can be determined from the tricuspid regurgitant jet velocity. CT of the chest serves to exclude pulmonary embolism and interstitial lung disorders. Cardiac MRI is increasingly being used for analysis of right ventricular morphology, function and haemdynamics. The 6 minute walk test and cardiopulmonary exercise test are used to assess severity and response to treatment and give prognostic information. For the definite diagnosis of pulmonary hypertension, right heart catheterisation is required for the determination of the pulmonary vascular resistance and pharmacological testing of "reversibility".

Pathogenesis of Chronic “Cor pulmonale” in COPD

Although “cor pulmonale” represents a leading cause of death in advanced COPD, there is no clear consensus on its definition. Pulmonary hypertension has been traditionally considered the pathogenetic mechanism leading to right heart dysfunction in COPD, with peripheral oedema usually being attributed to the venous congestion secondary to the increase of right-ventricle filling pressure. The loss in pulmonary capillaries and the vascular remodelling, secondary to chronic hypoxia, cigarette-smoke exposition and systemic inflammation, are well known causes of endothelial dysfunction and pulmonary hypertension in COPD patients. Other mechanisms, such as hypoxic vasoconstriction and alveolar capillary compression in hyperinflated lungs can contribute to the rise of pulmonary arterial pressure during COPD exacerbations, exercise and sleep. In the last decades, however, the role of pulmonary pressure overload in the development of right heart failure has been debated mostly, because of the lack of a clear correlation between pulmonary artery pressure values and the presence of peripheral oedema and/or haemodynamic signs of right heart failure. In addition, the presence of distinct renal and hormonal alterations, suggests that oedema formation in advanced phases of COPD is mostly due to sodium and water retention induced by hypercapnic acidosis and aggravated by severe hypoxemia. Future studies are needed to clarify the possible role of “intermittent hypoxia”, elicited by exercise or sleep, on right heart function and also on sodium and water handling in COPD patients. Keywords: Pulmonary hypertension, vascular remodelling, lung hyperinflation, right heart dysfunction

A 29-year-old male with chest pain and haemoptysis

A 29-yr-old African male was admitted with a 1-week history of chest pain, shortness of breath and haemoptysis. He had returned from Africa by plane 2 weeks previously. The patient denied fever, sweats or weight loss. He had no significant past medical history, was not taking any regular medication, and was a lifelong nonsmoker. He lived in the UK and was in full-time education. Physical examination did not reveal any abnormalities. Laboratory investigations showed elevated inflammatory markers, including an erythrocyte sedimentation rate level of 59 mm·h−1 and a C-reactive protein level of 53 mg·L−1, normal haemoglobin at 139 g·L−1 and leukocytosis of 14.6×109·L−1 with a predominance of neutrophils. Chest radiograph was normal. Computed tomography (CT) pulmonary angiography confirmed the presence of filling defects within branches of the right lower lobe, left lingular and left lower lobe pulmonary arteries, with no radiological evidence of right heart strain. Small nodules of ≤7 mm were identified in both lungs (fig. 1⇓). Several small mediastinal nodes were also identified. The patient was anti-coagulated with warfarin for bilateral pulmonary thromboembolism. Fig. 1— Computed tomography pulmonary angiography. Arrows show small pulmonary nodules. The patient had further episodes of atypical chest pain and haemoptysis over the following month. He was readmitted with signs of right heart failure and normocytic …

Pulmonary Valve Papillary Fibroelastoma

A 67-year-old woman with a past history of hypertension and type 1 diabetes mellitus, complicated by ischaemic heart disease, retinopathy and nephropathy, presented with several months of worsening dyspnoea and clinical signs of right heart failure. Transthoracic echocardiogram (TTE) showed normal left ventricular systolic function with a restrictive pattern of diastolic filling, a mildly dilated right ventricle with mild systolic impairment and a moderately elevated estimated pulmonary arterial pressure of 67 mmHg. Ventilation-perfusion imaging showed extensive, unmatched, non-segmental loss of perfusion bilaterally. This may have indicated multiple pulmonary emboli but due to the non-segmental pattern other aetiologies such as pulmonary hypertension could not be excluded.Subsequently, CT pulmonary angiography found no evidence of thrombo-embolic disease but noted a rounded 10 mm filling defect adjacent to or on one of the leaflets of the pulmonary valve. Repeat TTE delineated a mobile echo-dense mass attached to the pulmonary valve measuring 10 mm2, consistent with the differential diagnosis of a papillary fibroelastoma.© 2008 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000) than in women (13.2/100,000). The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP). The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational) exposures. IPF is typically progressive and leads to significant disability. The median survival is 2 to 5 years from the time of diagnosis. Medical therapy is ineffective in the treatment of IPF. New molecular therapeutic targets have been identified and several clinical trials are investigating the efficacy of novel medication. Meanwhile, pulmonary transplantation remains a viable option for patients with IPF. It is expected that, during the next decade, considerable progress will be made toward the understanding and treatment of this devastating illness.

Iatrogene arteriovenöse Fistel nach Laminektomie: Eine seltene Differentialdiagnose der Herzinsuffizienz

An abdominal murmur was first heard in a now 46-year-old man four years after laminectomy at the age of 21. Signs of right heart failure and, ultimately, of global heart failure developed progressively and increasingly 20 years later. Echocardiography demonstrated enlargement of the right heart cavities, and atrial fibrillation was diagnosed. Cardiac catheterization revealed the typical picture of high output failure (cardiac output 13.9 l/min). Intra-arterial subtraction angiography demonstrated a fistula between the left iliac artery and vein. After operative closure of the fistula the signs of heart failure disappeared. Six months later a residual but insignificant fistula was still present, as well as persistent atrial fibrillation. Medical treatment having failed cardioversion successfully re-established sinus rhythm and the patient became symptom-free. Arteriovenous fistula after laminectomy is a rare cause of heart failure and often diagnosed very late. The prognosis is good once the fistula has been closed.

Association of severe class III angina pectoris and constrictive pericarditis

A 74-year-old man was admitted with dyspnea on exertion and angina pectoris class III. Clinical history was marked by pulmonary tuberculosis treated by antituberculosis and a percutaneous coronary intervention of the left anterior descending artery (LAD). Cardiovascular risk factors were age, sex, tobacco, diabetes mellitus and obesity. Cardiac examination showed a blood pressure at 110/60 mm Hg, respiratory rate at 21 breaths/ minute with signs of right heart failure included lower limb edema, hepatomegaly, jugular vein distension, hepatojugular reflux. Electrocardiogram showed sinus rhythm at 80 c/ mn with normal axis and a T wave inversion in posterior. Chest radiography (figure 1A) revealed pericardial calcifications and moderate effusions of the left pleural cavity. CT (figure 1B) showed a calcified pericardium with a nodule in the left lung. TTE (figure 1C-D) revealed a pericardial thickening, distension of bilateral atrium and the inferior vena cava, posterior residual pericardial effusion, normal global systolic function, increased of the right ventricular end-diastolic pressure and signs of obstruction of the interventricular septum and the posterior wall of the left ventricle. Angiography showed a severe stenosis of LAD. Intraoperative exploration confirmed extensive pericardial calcifications. A Subtotal pericardectomy and coronary artery bypass to LAD-LITA (left internal thoracic artery) were performed without cardiopulmonary bypass. Histological examination of resected specimens revealed tuberculosis inflammation. In postoperative, medical treatment by antituberculosis (isoniazid, rifampin, pyrazinamide and streptomycin) was done. Clinical follow up was un eventful.

Percutaneous mitral valvuloplasty in patients with low cardiac output at high surgical risk

Among 1146 patients undergoing percutaneous mitral valvuloplasty for symptomatic mitral stenosis, 8 (4 men and women) were at high risk for surgery on the basis of the New York Heart Association functional class IV ( n = 8), severe pulmonary hypertension ( n = 5). All these patients aged 30 ±23.6 years had signs of right heart failure, high echocardiographic score (9.6 ± 3.6) and low mitral valve area (0.50 ± 0.19 cm 2). The procedure resulted in an increase in mitral valve area (1.55 ± 0.17 cm 2) with a concomitant reduction in pulmonary artery systolic pressure (58.7 ± 9.9 mm Hg) and decrease in tricuspid regurgitation. At follow-up (mean 14 ± 3 months), one patient with renal failure… presented with a mitral restenosis is scheduled for mitral valve replacement, two patients with severe tricuspid regurgitation required tricuspid annuloplasty. In conclusion, percutaneous mitral valvuloplasty is feasible and safe in patients at high surgical risk and can be considered as an acceptable alternative to surgery.

123 Prevalence of pulmonary arterial hypertension and echocardiographic signs of right heart failure in HIV infected patients

123 Prevalence of pulmonary arterial hypertension and echocardiographic signs of right heart failure in HIV infected patients

Efficacy of Sildenafil as a Rescue Therapy for Patients With Severe Pulmonary Arterial Hypertension and Given Long-term Treatment With Prostanoids: 2-Year Experience

Both prostanoids and sildenafil are effective treatments for severe pulmonary arterial hypertension (PAH). The combined use of these drugs to maximize the clinical benefit is an emerging treatment option. This study describes a 2-year experience with adjunct sildenafil as a rescue therapy for patients with severe PAH treated long term with prostanoids and who showed clinical deterioration or onset of heart failure. Twenty patients (11 men, 9 women; mean age 42 +/- 11 years) with severe PAH, who showed clinical or functional worsening despite ongoing treatment with prostanoids (8 subcutaneous, 7 intravenous, 5 inhaled), were started on adjunct oral sildenafil. New York Heart Association (NYHA) functional class, 6-minute walking test, signs of right ventricular failure and echocardiography were assessed before and after 1 and 2 years of combined therapy. There was a significant improvement of NYHA functional class and signs of right heart failure after 1- and 2-year follow-up. Patients showed a mean increase in 6-minute walking distance of 79 m and 105 m after 1 and 2 years of adjunct sildenafil, respectively. Two patients died during follow-up. The echocardiographic parameters showed a significant reduction of right ventricular end-diastolic diameter and left ventricular diastolic eccentricity index. No serious side effects related to sildenafil were observed. Adjunct sildenafil to long-term prostacyclin therapy in patients with severe PAH provided sustained clinical stabilization and an improved clinical situation, exercise capacity and echocardiographic parameters of right ventricular function. The beneficial effects were strong and lasted >24 months.

Chronic Thromboembolic Pulmonary Hypertension

Since the last World Symposium on Pulmonary Hypertension in 2008, we have witnessed numerous and exciting developments in chronic thromboembolic pulmonary hypertension (CTEPH). Emerging clinical data and advances in technology have led to reinforcing and updated guidance on diagnostic approaches to pulmonary hypertension, guidelines that we hope will lead to better recognition and more timely diagnosis of CTEPH. We have new data on treatment practices across international boundaries as well as long-term outcomes for CTEPH patients treated with or without pulmonary endarterectomy. Furthermore, we have expanded data on alternative treatment options for select CTEPH patients, including data from multiple clinical trials of medical therapy, including 1 recent pivotal trial, and compelling case series of percutaneous pulmonary angioplasty. Lastly, we have garnered more experience, and on a larger international scale, with pulmonary endarterectomy, which is the treatment of choice for operable CTEPH. This report overviews and highlights these important interval developments as deliberated among our task force of CTEPH experts and presented at the 2013 World Symposium on Pulmonary Hypertension in Nice, France.

Right heart failure due to an unroofed coronary sinus in an adult

An unroofed coronary sinus is a rare congenital malformation with a strong association to a persistent left vena cava superior. In most cases it occurs together with complex cardiac anomalies such as left isomerism. We report on a 72-year-old man with clinical signs of right heart failure caused by an isolated unroofed coronary sinus: The diagnosis was made by means of transesophageal echocardiography with contrast injection.

Late pericarditis secondary to pericardial patch implantation 25 years prior

We report a rare case of a 50-year-old man with signs of right heart failure due to localized pericarditis that was secondary to implantation of an artificial pericardial patch 25 years prior following pericardectomy for constrictive pericarditis. The patient's clinical symptoms resolved completely in the 3 years following patch removal.

Abnormalities of Renal Endothelin during Acute Exacerbation in Chronic Obstructive Pulmonary Disease

Circulating and urinary levels of endothelin (ET), an endothelium-derived vasoconstrictive and mitogenic peptide have been reported to increase in patients with chronic obstructive pulmonary disease (COPD), but the mechanisms of these abnormalities are not fully understood. Our study objectives were to evaluate pulmonary and renal ET clearance in COPD patients during an acute exacerbation. Our participants included nine consecutive patients with moderate to severe COPD without signs of right heart failure admitted for acute exacerbation and ten healthy volunteers (HV) as controls. ET was detected by radioimmunoassay in venous and arterial blood as well as in a timed urine specimen. For each subject, arterial/venous immunoreactive ET ratio (ir-ETart/ir-ETven) was evaluated as an index of its pulmonary clearance. Creatinine clearance was employed in each case to obtain a corrected renal ir-ET clearance. Glomerular filtration rate (GFR) was also assessed by dynamic99mTc-diethylenetriamine pentaacetic acid renal scintigraphy in six COPD patients during acute exacerbation and at recovery. The ratio ir-ETart/ir-ETven was comparable in COPD patients (0.75±0.12) and in HV (0.82±0.09). A significant difference was found with respect to 24 h ir-ET urinary excretion between COPD patients during exacerbation as well as at recovery (respectively 142.1±12.8 ng/24 h and 89.0±15.1 ng/24 h) and HV (65.1±10.1 ng/24 h). ET renal clearance was higher in COPD patients than in HV (29.2±5.2 ml min−1in COPD during exacerbation; 17.5±3.9 ml min−1at recovery and 13.6±2.4 ml min−1in HV, P<0.001). GFR was 69.4±10.0 ml min−1in COPD patients during exacerbation and it significantly increased at the recovery (95.5±20.9 ml min−1P<0.001). Corrected renal clearance of the peptide was significantly correlated to GFR values during the exacerbation (r=−0.81, P<0.05). Furthermore change in renal ET production resulted associated with changes in paCO2(r=0.83, P<0.001) and in paO2(r=−0.73 , P<0.05). Acute exacerbation in COPD patients causes an increase in renal ET production which is partially reversible at the recovery, in the absence of significant changes in ET-1 circulating levels. ET might contribute to the renal response to hypoxaemia and hypercapnia in COPD.

Aerosolized Iloprost Therapy Could Not Replace Long-term IV Epoprostenol (Prostacyclin) Administration in Severe Pulmonary Hypertension

To switch patients with severe pulmonary hypertension and previous life-threatening catheter-related complications from long-term IV epoprostenol therapy to aerosolized iloprost therapy. Open, uncontrolled trial. Medical ICU of a university hospital. Two patients with primary pulmonary hypertension and one patient with pulmonary hypertension after surgical closure of atrial septal defect (mean pulmonary artery pressure > or =50 mm Hg). All were classified as New York Heart Association class II under treatment with continuous IV epoprostenol for 4 years. Stepwise reduction of IV epoprostenol (1 ng/kg/min steps every 3 to 10 h) during repeated inhalations of aerosolized iloprost (150 to 300 microg/d with 6 to 18 inhalations/d). Continuous pulmonary and systemic arterial monitoring were performed. Aerosolized iloprost reduced pulmonary artery pressure by 49%, 49%, and 45%, respectively, and increased cardiac output by 70%, 75%, and 41% in the three patients. The effect lasted for 20 min and was similar at different doses of IV epoprostenol. Persistent treatment change to inhaled iloprost could not be achieved because all patients developed signs of right heart failure. After termination of iloprost inhalations, return to standard epoprostenol therapy led to clinical and hemodynamic restoration. Although aerosolized iloprost demonstrated short-term hemodynamic effects, it could not be utilized as alternative chronic vasodilator in patients with severe pulmonary hypertension.

Pericardial disease is often not recognised as a cause of chronic severe ascites

Background/Aims: Severe chronic ascites remains a difficult diagnostic and therapeutic problem. Even in the current era, constrictive pericarditis is an underestimated and sometimes unrecognised cause. Moreover, missing the diagnosis deprives patients of remedial therapy. Methods: Two cases of calcified constrictive pericarditis, complicated with cirrhosis and diagnosed in a late stage, are described. Due to insufficient clinical appreciation and lack of trust in echocardiography results, performed by cardiologists who were insufficiently familiar with the echocardiographic features of constrictive pericarditis, diagnosis was delayed in the two patients. Results: The diagnosis of constrictive pericarditis as a cause of ascites is based upon the clinical signs of right heart failure in a patient with normal systolic left and right ventricular function and a high, serumascitic albumin-content difference. Complementary workup with complete Doppler echocardiography study, right and left heart catheterisation and MRI or cine CT of the heart is necessary to confirm the diagnosis. Conclusion: Careful history taking and clinical examination remain the cornerstone of any diagnostic work-up, even in this era of technological refinement.

Pulmonary artery stenosis 5 years after single lung transplantation in primary pulmonary hypertension

This is a case report about a 56-year-old female patient with primary pulmonary hypertension who underwent single, right lung transplantation. Five years post-operatively she developed signs of right heart failure. History and physical examination suggested pulmonary artery stenosis. Diagnosis was confirmed by pulmonary angiography. Percutaneous placement of a balloon expandable stent normalized pulmonary artery pressure.