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Competing Interests: Conflict of interest: None declared.

Tricuspid regurgitation (TR) is a common condition that is independently associated with high mortality rates in patients with heart failure (HF). Several studies have demonstrated the clinical efficacy of add-on tolvaptan in patients hospitalized for HF. However, the effects of add-on tolvaptan in patients with significant TR are less well understood. Among the patients with moderate-to-severe TR assessed by transthoracic echocardiography during hospitalization for congestive HF, 39 patients who could complete the clinical course after starting add-on tolvaptan were included in the study. Rehospitalization due to HF and cardiac death were defined as adverse cardiac events in this study. We investigated the presence or absence of cardiac events within 2 years following the introduction of tolvaptan and evaluated echocardiographic functional parameters associated with cardiac events. The average patient age was 75 ± 14 years, and 23 patients (59%) experienced adverse cardiac events within 2 years after add-on tolvaptan administration. Serum creatinine (mg/dL) and brain natriuretic peptide (pg/mL) concentrations at discharge were significantly higher in patients with cardiac events than in those without cardiac events {1.48 [1.02-1.58] vs. 1.07 [0.79-1.41], p = 0.03; 526 [414-1044] vs. 185 [104-476], p = 0.01, respectively}. The presence or absence of past hospitalization for HF was also significantly higher in the event-positive group compared to event-free group (78 vs. 44%, p = 0.04). Comparison of echocardiographic parameters revealed that patients with cardiac events had a significantly lower left ventricular ejection fraction (40 ± 16 vs. 49 ± 15%, p = 0.049) and lower right ventricular fractional area change (RVFAC) (35 ± 12 vs. 45 ± 10%, p = 0.008) than those without cardiac events. Multiple logistic regression analysis revealed that RVFAC and past hospitalization for HF were independently associated with cardiac events following the introduction of tolvaptan (odds ratio, 0.934 and 4.992; p = 0.048 and 0.04, respectively). Right ventricular contractility as well as past history of admission for HF, left ventricular ejection fraction, renal function, and brain natriuretic peptide level at discharge may reflect the clinical outcomes after HF hospitalization in patients with significant TR who were treated with tolvaptan., (© 2021. Springer Japan KK, part of Springer Nature.)

Background: Pulmonary hypertension (PHT) is common in β-thalassemia patients due to hemolysis, iron overload and diminished nitric oxide (NO) levels. Biochemical markers can help to understand the pathophysiology and to introduce new therapies for this condition., Aim: This study aimed to evaluate the effectiveness of L-arginine and sildenafil in thalassemia children with PHT at both clinical and biochemical levels., Methods and Results: In a randomized controlled study, 60 β-thalassemia major children with PHT were divided into 3 equal groups; Control group (Conventional thalassemia and PHT management), L-arginine group (Conventional + Oral L-arginine 0.1 mg.kg -1 daily), and sildenafil group (Conventional + Oral sildenafil 0.25 mg.kg -1 two times a day) for 60 days. Tricuspid Regurgitant Jet Velocity (TRJV) with Doppler echocardiography along with serum levels of NO, asymmetric dimethylarginine (ADMA), interleukin 1-beta (IL-1β), E-selectin, and visfatin were followed-up at baseline, 30, and 60 days after treatment. Both drugs reduced the TRJV significantly. NO was significantly higher in both L-arginine and sildenafil groups after 60 days compared to baseline, while visfatin levels were lower. Only L-arginine reduced ADMA levels compared to baseline, while sildenafil did not. E-selectin and IL-1β levels did not change remarkably by both drugs. NO and TRJV showed significant negative correlations in both treatment groups., Conclusion: L-arginine and sildenafil could clinically ameliorate chronic PHT whereas, L-arginine showed superiority to sildenafil on some biochemical markers.

Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010 to 2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy at a median gestational age (GA) of 30.0 weeks (range: 20.9 to 34.9). Most (15/21 = 71%) mothers received NSAIDs, and 12 of 15 (80%) achieved DA constriction after a median of 2.0 days (1.0 to 6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome (live-birth or fetal demise) was 36.1 weeks (30.7 to 39.0) in fetuses with DA constriction versus 33 weeks (23.3 to 37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (p = 0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4 of 9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p = 0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Tricuspid regurgitation is associated with increased rates of heart failure (HF) and mortality. Transcatheter tricuspid valve interventions (TTVI) are promising, but the clinical benefit is unknown., Objectives: The purpose of this study was to investigate the potential benefit of TTVI over medical therapy in a propensity score matched population., Methods: The TriValve (Transcatheter Tricuspid Valve Therapies) registry collected 472 patients from 22 European and North American centers who underwent TTVI from 2016 to 2018. A control cohort formed by 2 large retrospective registries enrolling medically managed patients with ≥ moderate tricuspid regurgitation in Europe and North America (n = 1,179) were propensity score 1:1 matched (distance ± 0.2 SD) using age, EuroSCORE II, and systolic pulmonary artery pressure. Survival was tested with Cox regression analysis. Primary endpoint was 1-year mortality or HF rehospitalization or the composite., Results: After matching, 268 adequately matched pairs of patients were identified. Compared with control subjects, TTVI patients had lower 1-year mortality (23 ± 3% vs. 36 ± 3%; p = 0.001), rehospitalization (26 ± 3% vs. 47 ± 3%; p < 0.0001), and composite endpoint (32 ± 4% vs. 49 ± 3%; p = 0.0003). TTVI was associated with greater survival and freedom from HF rehospitalization (hazard ratio [HR]: 0.60; 95% confidence interval [CI]: 0.46 to 0.79; p = 0.003 unadjusted), which remained significant after adjusting for sex, New York Heart Association functional class, right ventricular dysfunction, and atrial fibrillation (HR: 0.39; 95% CI: 0.26 to 0.59; p < 0.0001) and after further adjustment for mitral regurgitation and pacemaker/defibrillator (HR: 0.35; 95% CI: 0.23 to 0.54; p < 0.0001)., Conclusions: In this propensity-matched case-control study, TTVI is associated with greater survival and reduced HF rehospitalization compared with medical therapy alone. Randomized trials should be performed to confirm these results., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: Reteplase (recombinant plasminogen activator) is a mutant of alteplase. It has a longer half-life than its parent molecule and has shown better vessel patency rates in acute myocardial infarction. In this study, we analyzed the efficacy and safety of reteplase in acute pulmonary embolism (PE)., Methods: This observational study included patients with high- and intermediate-risk acute PE, presenting within 14 days of symptom onset. The patients were treated with reteplase, which was given in two bolus doses of 10 U each, 30 min apart, along with intravenous heparin. Patients with hemodynamic compromise (high-risk or massive PE) and normotensive patients with evidence of right ventricular (RV) dysfunction (intermediate-risk or submassive PE) on echocardiography or computed tomography were included in the study. The efficacy outcomes assessed were in-hospital death and improvement of RV function by echocardiography. The safety outcomes were major bleeding, minor bleeding, and ischemic or hemorrhagic stroke during hospitalization., Results: Of the 40 patients included, 25% were classified as high risk with hemodynamic compromise and 75% were classified as intermediate risk. RV dysfunction was present in all the patients (100%). Concomitant lower extremity deep vein thrombosis was present in 55% of the patients. The mortality rate was 5%. There was significant improvement in RV function and reduction in pulmonary artery systolic pressure and tricuspid regurgitation severity. There was no major bleeding event or stroke, and 7.5% patients had minor extracranial bleeding., Conclusions: Double-bolus reteplase given with heparin is effective in the treatment of high- and intermediate-risk PE, with minimal risk of bleeding., (Copyright © 2019 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

Elevated tricuspid valve regurgitation jet velocity (TRV ≥ 2.5 m/s) is associated with mortality among adults with sickle cell disease (SCD), but correlative biomarkers are not studied according to treatment exposure or genotypes. To investigate the associations between biomarkers and TRV elevation, we examined the relationship between TRV and hemolytic, inflammatory, and cardiac biomarkers, stratified by disease-modifying treatments and SCD genotype. In total, 294 participants with SCD (mean age, 11.0 ± 3.7 years) and 49 hereditary spherocytosis (HS; mean age, 22.9 ± 19.75 years) were included for comparison and enrolled. TRV was elevated in 30.7% of children with SCD overall: 18.8% in HbSC/HbSβ + -thalassemia, 28.9% in untreated HbSS/HbSβ 0 -thalassemia, 34.2% in HbSS/HbSβ 0 -thalassemia hydroxyurea-treated, and 57% in HbSS/HbSβ 0 -thalassemia chronic transfusion treated. TRV was elevated in 10.7% and 27.8% in HS children and adults, respectively. In children with SCD, elevated TRV was correlated with hemoglobin (odds ratio [OR] = 0.78, P = 0.004), lactate dehydrogenase (LDH; OR = 2.52, P = 0.005), and N-terminal pro-brain natriuretic peptide (NT-pro BNP; OR = 1.003, P = 0.004). In multivariable logistic regression, adjusting for genotype, sex, hemolytic index, and treatment, hemoglobin concentration remained the only significant variable associated with elevated TRV in untreated HbSS/HbSβ 0 -thalassemia participants. TRV was not associated with inflammatory markers, other markers of hemolysis, or NT-pro BNP in untreated HbSS/HbSβ 0 -thalassemia. Neither hemoglobin nor LDH was associated with TRV in HbSC/HbSβ + -thalassemia. These results suggest that elevated TRV is influenced by the degree of anemia, possibly reflecting sickling as part of the disease pathophysiology. Prospective studies should monitor hemoglobin concentration as children with SCD age into adulthood, prompting initiation of TRV screening and monitoring., (© 2019 Wiley Periodicals, Inc.)

Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation., Competing Interests: Conflict of Interest Disclosure: The authors have completed and submitted the Methodist DeBakey Cardiovascular Journal Conflict of Interest Statement and none were reported.

Objectives: Our objective was to explore the effects of tolvaptan as a new therapeutic approach in patients with right heart failure with tricuspid insufficiency (TI)., Methods: This prospective, multicenter, non-randomized controlled pilot study enrolled patients (N = 40) with TI from the Shanghai Chest Hospital and Shanghai Tongren Hospital who fulfilled inclusion criteria between March 2015 and June 2016. Participants were assigned to receive either tolvaptan combined with torasemide (n = 20) or torasemide monotherapy (n = 20; control group). The primary endpoints were changes in patient weight and in tricuspid annular plane systolic excursion (TAPSE) after 10 days of treatment. The secondary endpoints included net fluid balance and cardiac functions before and after medication from the first to the tenth day of treatment. Safety was evaluated by monitoring adverse and serious adverse events., Results: TAPSE significantly increased in the tolvaptan group compared with the control group after 10 days of medication (P = 0.029). Daily weight losses in the tolvaptan group significantly increased as the time of treatment increased (time × group, P = 0.022). Recovery to New York Heart Association (NYHA) grade I occurred 4 days earlier in the tolvaptan group. In addition, the net fluid balance and median net fluid balance were significantly higher in the tolvaptan group. Eight adverse events and one serious adverse event were recorded in the tolvaptan group and 15 adverse events were recorded in the control group., Conclusions: Our results indicate that tolvaptan might be a useful and safe drug to improve heart function in patients with right heart failure with TI after left heart valve replacement., Clinical Trial Registration: ClinicalTrials.gov identifier no. NCT02644616.

Objectives: This study sought to define the relationship between functional tricuspid regurgitation (TR) and mortality in patients with heart failure with reduced ejection fraction (HFrEF); and to establish the prognostic value of quantitative measures of TR severity (i.e., effective regurgitant orifice area [EROA] and regurgitant volume)., Background: The significance of TR in chronic heart failure is controversial. Earlier studies have shown an independent impact of TR on mortality, whereas more recent evidence suggests myocardial impairment to be the driving force of mortality rather than TR itself. Earlier studies have used qualitative measures of TR severity, hence the prognostic value of more quantitative measures of TR severity (i.e., EROA and regurgitant volumes) remains unclear., Methods: We enrolled 382 patients with HFrEF on guideline-directed medical therapy and assessed TR EROA and regurgitant volume by Doppler/2-dimensional echocardiography. All-cause mortality was defined as the primary study endpoint., Results: TR severity was associated with the HFrEF phenotype with more symptoms (p = 0.004), higher neurohumoral activation (p < 0.001), progressive right-ventricular dilatation (p < 0.001), and impaired function (p < 0.001). Cox regression showed a strong association between quantitative measures of TR with mortality (all p < 0.001). Quantitative metrics of TR severity were consistently associated with mortality with a hazard ratio of 1.009 (95% confidence interval: 1.004 to 1.013; p < 0.001) per 0.01 cm 2 increase of the EROA and of 1.013 (95% confidence interval: 1.007 to 1.020; p < 0.001) per 1-ml increase in regurgitant volume. Results remained unchanged after bootstrap- or clinical confounder-based adjustment. A spline curve pattern illustrates the association with mortality with thresholds for the EROA ≥0.2 cm 2 , and the regurgitant volume ≥20 ml with sustained excess mortality thereafter., Conclusions: This large-scale outcome study demonstrates the prognostic value of quantitative Doppler-echocardiographic measures of TR severity in HFrEF. The thresholds for EROA and TR regurgitant volume associated with mortality in our study fall within current ranges defining nonsevere TR. This may potentially impact therapeutic decision making, particularly timing of intervention., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Competing interests: None declared.

The vast majority of tricuspid valve regurgitations are of low degree without prognostic relevance in healthy individuals; however, morbidity and mortality increase with the degree of regurgitation, which can be secondary to either primary (structural) or secondary (functional) alterations of the valve. Due to the frequent lack of symptoms, echocardiographic examinations should be annually performed in patients with higher degree (at least moderate) tricuspid valve regurgitation, in particular in the presence of risk factors. Individual therapeutic management strategies should consider the etiology of the tricuspid valve regurgitation, the degree of regurgitation, the valve pathology and the risk-to-benefit ratio of the envisaged therapeutic procedure. Medicinal treatment options for tricuspid valve regurgitation are limited and generalized recommendations cannot be provided due to the lack of conclusive clinical trials. Symptomatic therapeutic measures encompass especially (loop) diuretics for the reduction of preload and afterload of the right ventricle. Pharmaceutical reduction of the heart rate should be avoided in patients with right heart insufficiency. While symptomatic therapeutic measures are often associated with only moderate effects, the most effective therapy of tricuspid valve regurgitation consists in the treatment of underlying illnesses, in most cases pulmonary hypertension due to pulmonary arterial hypertension (PAH), left heart disease or acute pulmonary embolism. Based on a number of published clinical studies and licensing of new drugs, treatment options for patients with PAH and heart failure with reduced ejection fraction (HFrEF) have substantially improved during the past years allowing for a differentiated, individualized management.

Pericardiocentesis is performed to treat cardiac tamponade or diagnose the cause of pericardial effusion. Cardiogenic shock with right ventricular (RV) dysfunction is a rare complication after pericardiocentesis. We report a case of an 82-year-old man who suddenly suffered cardiopulmonary arrest 12 h after pericardiocentesis. A transthoracic echocardiogram showed remarkable RV dysfunction and tricuspid valve dysfunction. Tricuspid valve closure was severely impaired, and the tricuspid regurgitation signal showed laminar flow with an early peak. However, after treatment with high-dose inotropic drugs, hemodynamic parameters gradually recovered. A transthoracic echocardiogram performed 24 h later showed improved motion of the RV and the tricuspid valve, resulting in a reduction in tricuspid regurgitation. RV and tricuspid valve dysfunction after pericardiocentesis needs to be recognized as a critical complication. Physicians also need to pay attention to not only the amount of drainage but also underlying RV dysfunction.

Sildenafil, a phosphodiesterase-5 inhibitor, is a controversial treatment option for pulmonary arterial hypertension (PAH), a significant complication of bronchopulmonary dysplasia (BPD). The objective of this study was to evaluate the use of sildenafil in infants with PAH secondary to BPD. This was a retrospective review of medical records of all premature infants with PAH associated with BPD treated with sildenafil between January 2009 and May 2013 in a level 3 neonatal intensive care unit. The primary outcomes were clinical response (20 % decreases in respiratory support score or oxygen requirements) and echocardiographic response (20 % decrease in tricuspid regurgitation gradient or change of at least 1° of septal flattening). Twenty-three infants were included in the study. Significant echocardiographic and clinical responses were, respectively, observed in 71 and 35 % of cases. Most clinical responses were observed in the first 48 h of treatment, and the median time to an echocardiographic response was of 19 days. The median dose of sildenafil used was 4.4 mg/kg/day, with a median time to reach the maximum dose of 9 days. Transient hypotension was the primary reported side effect, and it was observed in 44 % of our study population. Sildenafil treatment in patients with PAH secondary to BPD was associated with an echocardiographic improvement in the majority of patients, whereas clinical improvement was observed in a minority of patients. Many infants presented with transient hypotension during the course of the treatment. Further prospective studies are required to better assess safety and efficacy of this treatment in this population.

Tricuspid infective endocarditis is rare and represents five to 10% of all cases of infective endocarditis. It occurs predominantly in intravenous drug users, and patients with central venous catheters or intracardiac probes. We report on the case of subacute tricuspid infective endocarditis in a girl of 17 years. She had no particular cardiovascular history. She was admitted for a persistent fever with cachexy, cough and thoracic pains, and right heart failure that appeared one month after a clandestine abortion. Transthoracic echocardiography found several vegetations on the tricuspid valve with massive tricuspid regurgitation. The chest X-ray showed bilateral excavated lung abscesses and condensation areas. Blood culture was not done and broad-spectrum antibiotic therapy was given. She was apyretic after 10 days. However, the massive tricuspid regurgitation with right heart failure persisted. She was discharged from hospital after 40 days of treatment. Although rare, infective endocarditis is one of the more serious complications of gynaecological procedures, particularly clandestine abortion. Therefore any young girl with persistent fever must be suspected of clandestine abortion.

Aims: Imatinib mesylate, as add-on therapy in patients with pulmonary arterial hypertension (PAH) who remain inadequately treated despite receiving at least two PAH-specific drugs, improves exercise capacity and haemodynamics. We evaluated whether 24 weeks of add-on therapy with imatinib compared with placebo also improves right ventricular (RV) function assessed by echocardiography., Methods and Results: Echocardiograms were obtained at baseline, 12 weeks, and 24 weeks in 74 patients randomized to imatinib or placebo in the Imatinib in Pulmonary arterial hypertension, a Randomized Efficacy Study (IMPRES) trial. Right ventricular function was assessed by tissue Doppler tricuspid annular peak systolic velocity (TA S'), tricuspid annular plane systolic excursion (TAPSE), RV Tei index, and RV fractional area change. Between-treatment-group differences in the changes from baseline to week-24 were assessed using an ANCOVA with the last observation carried forward. At week-24 patients randomized to imatinib demonstrated greater improvements in TA S' (1.6 ± 2.3 imatinib vs. 0.5 ± 2.4 cm/s placebo, P = 0.007) and RV Tei index (-0.11 ± 0.18 imatinib vs. 0.05 ± 0.18 placebo, P = 0.005) compared with placebo, but not in TAPSE (0.07 ± 0.44 imatinib vs. 0.03 ± 0.32 cm placebo, P = 0.08). Imatinib therapy was also associated with significant reduction in peak tricuspid regurgitation velocity, increase in LV size, and improvement in LV early diastolic relaxation velocity., Conclusions: Among patients with advanced PAH who remain symptomatic on at least two PAH-specific drugs, treatment with imatinib compared with placebo is associated with significant improvements in echocardiographic measures of RV function, in addition to LV size and LV early diastolic relaxation., Clinical Trial Registration: NCT00902174 (Clinicaltrials.gov)., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.)

We report three cases of tricuspid valve infective endocarditis associated with intravenous nyoape use. Nyoape is a variable drug combination of an antiretroviral (efavirenz or ritonavir), heroin, metamphetamines and cannabis. Its use is becoming increasingly common among poor communities in South Africa. All our patients were young HIV-positive men from disadvantaged backgrounds. They all presented with tricuspid regurgitation and septic pulmonary emboli. They were treated with prolonged intravenous antibiotic courses, and one required referral for surgery.

Pulmonary arteries aneurysms are rare and the rarely described bilateral aneurysms. A 45-year-old patient carrier of mitral stenosis was admitted for dyspnoea class III of the NYHA, chest pain and a not infectious cough. The clinical examination found semiology of mitral stenosis, tricuspid incapacity and pulmonary arterial hypertension. The electrocardiogram showed atrial fibrillation and right ventricle hypertrophy. Chest X-ray found a cardiomegaly, an aspect of double outline of the inferior right bow, a prominent aspect of the left average bow reminding an aneurysm of the left pulmonary artery. The echocardiography Doppler found a pure tight mitral stenosis (mitral surface=0.6 cm(2)), a dilation of the trunk of the pulmonary artery (diameter=74 mm) and of its branches (diameter of the right pulmonary artery=28 mm, diameter of the left pulmonary artery=36 mm) seat of a spontaneous contrast. The left atrium and right cardiac cavities were also dilated. There was an important tricuspid incapacity with a major pulmonary hypertension (systolic pulmonary arterial=109 mmHg). The thoracic angioscan showed a pseudoaneurysm of the trunk of pulmonary artery and its branches to their distal parts. Under diuretic, anticoagulating and analgesic treatment the clinical signs improved however the spontaneous contrast persisted. The patient was rejected by the surgery for exceeded clinical board. She is at present followed in our service for 5 months., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

A 78-year-old man admitted with complaints of breathlessness of 1 year and typical chest pain of 2 months duration. He had a blunt chest trauma 7 years back. Transthoracic echocardiography revealed severe tricuspid regurgitation due to a flail anterior leaflet of the tricuspid valve. It also revealed global left ventricular dysfunction. Flail tricuspid valve causing severe regurgitation is usually due to mechanical trauma. Since it is well tolerated for years, the diagnosis may be delayed or missed entirely. Echocardiography has allowed easier diagnosis of this condition resulting in earlier and, hence, more effective treatment.

Introduction: Systemic sclerosis (SSc) is an autoimmune disease characterized by multisystem involvement. Heart involvement occurs in 80-100% of cases and represents one of the more common risk factors of death. Myocardial fibrosis and vascular microangiopathy lead to arrhythmias and impaired contractility with secondary left and right heart failure. Pulmonary arterial hypertension in some patients with systemic sclerosis results in right heart failure and low output syndrome during the end-stage of the disease. The aim of the present study was to analyze four cases of systemic sclerosis with severe cardiovascular complications and a fatal outcome., Case Descriptions: Case 1: A 68-year-old female who probably suffered from limited SSc (lcSSc) and pulmonary arterial hypertension for more than 10 years was unsuccessfully treated with vasodilatators (sildenafil, iloprost). Case 2: A 52-year-old female with a 5-year history of diffuse cutaneous SSc (dcSSc), interstitial lung disease, and pulmonary arterial hypertension underwent aggressive therapy with immunosuppressive and antiproliferative (treprostinil) agents but without effect on the progression of the disease. Case 3: A 50-year-old male with dcSSc and coexistent cardiomyopathy with dominating symptoms of right heart failure was placed on high doses of immunosuppressants (i.v. cyclophosphamide) and had a cardioverter-defibrillator implanted as part of primary prevention. The patient died with symptoms of severe, therapy-resistant, two-sided heart failure. Case 4: A 58-year-old female with a 6-year history of dcSSc, interstitial lung disease, and heart involvement with severe ventricular arrhythmias and significant mitral and tricuspid insufficiency received intensive immunosuppressive therapy (i.v. cyclophosphamide) and underwent arrhythmia ablation. Sudden cardiac arrest in this patient was attributed to arrhythmia due to cardiomyopathy associated with systemic sclerosis., Conclusions: Lung and heart involvement is the most common reason for poor prognosis in systemic sclerosis. Arrhythmias, usually latent clinically, and right heart failure associated with cardiomyopathy or pulmonary hypertension are the main reasons of cardiac death in SSc patients. Severe and fatal cardiovascular complications occur more often in dcSSc, particularly during the first few years after diagnosis. Early detection of cardiovascular manifestations should be a priority in systemic sclerosis.

History and Admission Findings: A 70-year-old female patient was admitted with progressive dyspnea and peripheral edema. The patient had a medical history of myocardial hypertrophy, diastolic dysfunction and concomitant pulmonary hypertension (PH)., Investigations: The physical exam was suggestive of cardiac decompensation. Echocardiography showed myocardial hypertrophy, an enlarged left atrium as well as enlarged right-sided heart chambers. A prominent tricuspid regurgitation jet was present, and the estimated systolic right ventricular pressure was 65 mmHg. Invasive hemodynamic measurements showed a marked pressure elevation in the pulmonary circulation (mean PAP 51 mmHg), combined with an elevated left ventricular end-diastolic pressure (LVEDP) of 30 mmHg and a profound increase in the transpulmonary gradient (TPG, 21 mmHg)., Treatment and Course: The synopsis of these findings led to the diagnosis of postcapillary PH with a prominent precapillary involvement and cardiac decompensation. Due to signs of volume overload, an adequate diuretic therapy was initiated. The patient was recompensated and lost 7 kg of weight, which was associated with substantial clinical improvement. At invasive follow-up hemodynamic measurement, the patient's PAP was substantially decreased and almost reached normal values. The previously diagnosed precapillary involvement had disappeared., Conclusion: PH is a frequent phenomenon in patients with systolic and diastolic heart failure, and might initially appear as a combination of pre- and postcapillary involvement. The patients' volume status has a major influence on pulmonary hemodynamics. An adequate therapy of the underlying heart failure, especially an adequate diuresis, may have marked beneficial effects on pulmonary hemodynamics. Hemodynamic measurements should always be performed in compensated status., (© Georg Thieme Verlag KG Stuttgart · New York.)

Burkholderia cepacia complex organisms are environmental Gram-negative bacteria which rarely cause disease in otherwise immunocompetent individuals. We describe a case of tricuspid valve endocarditis secondary to B. cepacia complex in an injecting intravenous drug user in Auckland, New Zealand.

In adults with sickle cell disease (SCD), an increased tricuspid regurgitation velocity (TRV) by Doppler echocardiography is associated with increased morbidity and mortality. Although sildenafil has been shown to improve exercise capacity in patients with pulmonary arterial hypertension, it has not been evaluated in SCD. We therefore sought to determine whether sildenafil could improve exercise capacity in SCD patients with increased TRV and a low exercise capacity. A TRV ≥ 2.7 m/s and a 6-minute walk distance (6MWD) between 150 and 500 m were required for enrollment in this 16-week, double-blind, placebo-controlled sildenafil trial. After 74 of the screened subjects were randomized, the study was stopped early due to a higher percentage of subjects experiencing serious adverse events in the sildenafil arm (45% of sildenafil, 22% of placebo, P = .022). Subject hospitalization for pain was the predominant cause for this difference: 35% with sildenafil compared with 14% with placebo (P = .029). There was no evidence of a treatment effect on 6MWD (placebo-corrected effect -9 m; 95% confidence interval [95% CI] -56-38; P = .703), TRV (P = .503), or N-terminal pro-brain natriuretic peptide (P = .410). Sildenafil appeared to increase hospitalization rates for pain in patients with SCD. This study is registered at www.clinicaltrials.gov as NCT00492531.

Cabergoline, an ergot-derived dopamine receptor agonist, is used widely in the treatment of Parkinson's disease (PD) and hyperprolactinemia, but may cause heart valve fibrosis, retraction, and clinically significant regurgitation in PD patients. While cabergoline has been used at much lower doses in patients with hyperprolactinemia, controversy persists as to whether it may cause heart valve disease in this situation. Cabergoline is also used in acromegaly at doses similar to those used in hyperprolactinemia. The case is reported of a female patient with acromegaly who had been taking low-dose (0.5 mg/day) cabergoline for one year, and presented with signs and symptoms of right-sided heart failure. Echocardiography revealed a thickened and retracted tricuspid valve associated with severe tricuspid regurgitation and enlargement of the right-heart chambers. The morphology of the tricuspid valve was typical for cabergoline-related valvulopathy. Cabergoline may not be totally safe even at lower doses, and close echocardiographic monitoring is recommended in patients receiving cabergoline treatment, regardless of the dose level employed.

The right-sided heart valves are affected in about 10% of patients with infective endocarditis. However, the tricuspid valve is the most frequently involved valve in intravenous drug users with infective endocarditis. When treated with antibiotics, the prognosis is considered favorable. Reported here is the case of a drug-addicted patient with polymicrobial (Staphylococcus aureus and Streptococcus pneumoniae) infective endocarditis of the tricuspid valve and a lethal outcome due to multiple organ failure. The indications and options to perform cardiac surgery in patients with infective endocarditis of the tricuspid valve are discussed.

Disseminated gonococcal infection (DGI) occurs in 1-3% of all gonococcal infections; endocarditis is a complication in 1-2% of patients with DGI. We present the fourth reported case of gonococcal tricuspid valve endocarditis, this one occurring in a 53-year-old male with a 2-month history of shortness of breath., (Copyright © 2009 International Society for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

Right-sided endocarditis is relatively rare and can occasionally be complicated by vertebral osteomyelitis (VO). We describe successful treatment, including valve repair for tricuspid endocarditis associated with VO. A 77-year-old man presented with back pain and high fever. Magnetic resonance imaging demonstrated VO. Despite 2 months of intravenous antibiotics, the infectious signs persisted and both legs became edematous. Enterococcus faecalis was isolated from blood cultures, and echocardiography showed severe tricuspid regurgitation with large vegetations attached to the anterior leaflet (AL). A series of echocardiographic assessments revealed that the antibiotic therapy did not affect the tricuspid lesions. In surgery, the infection was extended to some chordae and over half of the AL was resected. The AL was repaired with autologous pericardium and artificial chordae. Antibiotic therapy was continued for 2 months after surgery, and the infections did not reoccur. Follow-up echocardiography showed mild regurgitation of the tricuspid valve. The patient remains free from endocarditis at 2 years after surgery.

Hydatid Cyst disease involves the heart in 0.02-2% of the cases. It can appear with symptoms very similar to coronary artery disease, cardiac valvular disease and pericarditis. We present a case of hydatid cyst that was located on the posterior tricuspid leaflet and that caused tricuspid regurgitation in 37 year old female patient who has gone through hydatid cyst excision from the bilateral lungs with median sternotomy 2 years ago. In addition to the right atrial and ventricular dilatation, second degree tricuspid regurgitation and significant pulmonary hypertension was found. The 2 x 2 cm smooth surfaced mass was resected from the posterior leaflet of the tricuspid valve and the defect was closed with suture with the aid of cardiopulmonary bypass. The patient followed with long term albendazole treatment. Cardiac echinococcosis should be kept in mind in some patients throughout their life with a history of previous hydatid cyst disease. Surgical excision without rupture is the treatment of choice for cardiac hydatid cyst, with following medical therapy in order to prevent recurrence.

Hydroxyurea and higher hemoglobin F improve the clinical course and survival in sickle cell disease, but their roles in protecting from pulmonary hypertension are not clear. We studied 399 children and adolescents with sickle cell disease at steady state; 38% were being treated with hydroxyurea. Patients on hydroxyurea had higher hemoglobin concentration and lower values for a hemolytic component derived from 4 markers of hemolysis (P < or = .002) but no difference in tricuspid regurgitation velocity compared with those not receiving hydroxyurea; they also had higher hemoglobin F (P < .001) and erythropoietin (P = .012) levels. Hemoglobin F correlated positively with erythropoietin even after adjustment for hemoglobin concentration (P < .001). Greater hemoglobin F and erythropoietin each independently predicted higher regurgitation velocity in addition to the hemolytic component (P < or = .023). In conclusion, increase in hemoglobin F in sickle cell disease may be associated with relatively lower tissue oxygen delivery as reflected in higher erythropoietin concentration. Greater levels of erythropoietin or hemoglobin F were independently associated with higher tricuspid regurgitation velocity after adjustment for degree of hemolysis, suggesting an independent relationship of hypoxia with higher systolic pulmonary artery pressure. The hemolysis-lowering and hemoglobin F-augmenting effects of hydroxyurea may exert countervailing influences on pulmonary blood pressure in sickle cell disease.

Echocardiographically documented tricuspid valve regurgitation appeared immediately after surgical treatment of cor triatriatum dexter in a two-month-old rottweiler. Medical treatment was instituted with benazepril, spironolactone and furosemide. Pimobendan was added after five months, and all treatment was discontinued two months later when clinical signs of ascites and hepatomegaly had resolved and tricuspid valve regurgitation was markedly reduced on echocardiography. To the authors' knowledge, this is the first report describing the development and spontaneous improvement of haemodynamically significant tricuspid valve regurgitation following surgical treatment of cor triatriatum dexter in a dog. It is hypothesised that the increase in right atrial volume and pressure following cor triatriatum dexter repair and transient ischaemia of papillary muscles led to dilatation of the right atrioventricular annulus and subsequent severe tricuspid valve regurgitation in the face of an anatomically normal valve. Time and pharmacological preload reduction as well as normalisation of right atrial inflow and subsequent cardiac remodelling substantially reduced tricuspid valve regurgitation and eliminated clinical signs of heart failure. It is also possible that heart recovery has been spontaneous.

Cardiovascular manifestations of hyperthyroidism are frequent and sometimes are relevant in the clinical picture. Usually an hyperdynamic circulatory state hallmarks the disease with low peripheral resistance, increased intravascular volume and cardiac output. However, right chamber dilatation with tricuspid valve regurgitation and cardiac failure are unusual. We present the case of a young woman with Graves-Basedow disease without cardiovascular history who complained about palpitations, peripheral edemas, weight loss and fever. The clinical findings were tachycardia with irregular pulse, right heart failure and regurgitant tricuspid murmur that increased with inspiration. The chest radiograph and the echocardiogram showed right ventricular dilatation and severe tricuspid regurgitation without pulmonary hypertension. The treatment with propranolol, corticosteroids and diuretics was successful. The patient was asymptomatic with sinus rhythm. We discuss the effects of thyroidal hormones on the cardiovascular system and postulate pathophysiologic mechanisms of heart failure in hyperthyroidism.

Because the pulmonary vascular resistance is very elevated at birth, severe tricuspid regurgitation is poorly tolerated and even life-threatening in the newborn. The etiology may be tricuspid valve papillary muscle rupture or the more ominous Ebstein's anomaly, with its associated dysfunctional right ventricle. After the diagnosis is established and the patient is supported with prostaglandin infusion and nitric oxide, definitive surgical management is undertaken with the expectation of excellent outcomes for isolated tricuspid valve regurgitation. For neonates with Ebstein's anomaly, therapy is tailored to the severity of the malformation and the degree of right ventricular outflow tract obstruction, assessed in the context of declining pulmonary vascular resistance. The surgical approach may involve ligation of a patent ductus arteriosus, placement of a systemic to pulmonary shunt, establishment of functional tricuspid atresia, or tricuspid valve repair. With the application of these various approaches, the outlook for neonatal Ebstein's anomaly has improved remarkably.

Hyperthyroidism may present with signs and symptoms related to dysfunction of a variety of organs. Cardiovascular pathology in hyperthyroidism is common. A few case reports describe isolated right heart failure, tricuspid regurgitation, and pulmonary hypertension as the prominent cardiovascular manifestations of hyperthyroidism. Although most textbooks do not mention hyperthyroidism as a cause of pulmonary hypertension and isolated right heart failure, the literature suggests that some hyperthyroid patients may develop reversible pulmonary hypertension and isolated right heart failure. We report a case of hyperthyroidism presenting with signs and symptoms of isolated right heart failure, tricuspid regurgitation, and pulmonary hypertension, which resolved with treatment of hyperthyroidism.

Carcinoid heart disease is a frequent complication of the carcinoid syndrome, usually presenting as right-sided valvular heart disease. Although the diagnosis is most often made with cardiac ultrasound, MRI and CT are valuable techniques for diagnosing carcinoid heart disease.

Atrial fibrillation (AF) and tricuspid regurgitation (TR) may induce congestive heart failure (CHD). Using electrocardiography and echocardiography, we examined the clinical characteristics and haemodynamic findings in 100 patients with AF + TR + CHF, AF + TR, AF or TR. The fractional shortening in all groups with AF was significantly decreased compared with the TR group. The ejection fraction in patients with AF + TR + CHF was significantly lower than in the TR group. Twenty-four of the 72 patients with AF and TR (with or without CHF) were treated, and 13 were monitored for heart rate and severity of TR. Eight months after start of treatment the heart rate and typical symptoms and signs of heart failure had improved significantly in nine patients, but the severity of TR did not change. TR worsened in the remaining four patients but they did not develop CHF. Our results suggest that increased heart rate due to the combination of AF and TR could be responsible for CHF.

Objective: To study the fetal response to prenatal therapy in non-hydropic fetuses with supraventricular tachycardia (SVT) as a function of fetal haemodynamic status at presentation., Study Design: Retrospective study., Material and Methods: Between 1990 and 2000, 40 non-hydropic fetuses presented with SVT. Twenty-eight had reciprocating SVT and 12 had atrial flutter. Ten fetuses had significant tricuspid valve regurgitation. All fetuses were treated prenatally. The main outcome measurement was fetal response to therapy as assessed by the rate of prenatal SVT reduction and by the mean time interval to sinus rhythm restoration., Results: The mean gestational age at presentation was 29 +/- 4.9 weeks. Overall, there were 39 live births and 1 intrauterine death. Reduction of SVT was achieved prenatally in 32 cases (80%). Among the 30 cases without tricuspid regurgitation, prenatal conversion to sinus rhythm was achieved in 27 cases (90%) with a mean time interval of 7 days. Among the 10 fetuses presenting with tricuspid regurgitation, the rate of prenatal conversion was significantly lower (5/10) and the mean time interval to conversion was significantly longer (24 days; p = 0.04, Mann-Whitney test). In the subgroup treated by digoxin as first-line therapy (n = 32), the interval to sinus rhythm restoration was also significantly higher in the presence of tricuspid regurgitation, with a slightly but not significantly lower reduction rate., Conclusion: The response to prenatal therapy may be poorer in cases presenting with tricuspid regurgitation., (Copyright 2002 S. Karger AG, Basel)

A case of right-sided endocarditis due to Salmonella typhi is described involving a native tricuspid valve in a child who was human immunodeficiency virus negative with no evidence of intravenous drug addiction. The patient had classic features of typhoid and tricuspid regurgitation without clinical evidence of bacterial endocarditis. Transthoracic echocardiography confirmed the tricuspid regurgitation. However, transesophageal echocardiography was necessary to demonstrate the vegetations affecting the tricuspid valve leaflets that made possible the diagnosis of endocarditis. The infection was cured with intravenous ceftriaxone and oral amoxicillin.