Signs Of Right Ventricular Failure Research Articles

Physiologically Guided Thrombolysis in Pulmonary Embolism

We describe the case of a 69-year-old woman who presented with submassive pulmonary embolism without overt shock, but with significant signs of right ventricular failure and dyspnea on minimal exertion. She was managed using a point-of-care ultrasound and pulmonary artery pressure-guided approach in order to minimize total thrombolytic dose while nonetheless achieving significant physiological improvement.

Легочно-сердечная гемодинамика у больных ХОБЛ и муковисцидозом по данным эходопплеркардиографии

Aim: to assess the functionality of the right heart in patients with chronic obstructive pulmonary disease (COPD), mixed cystic fibrosis (CF), and cystic fibrosis lung disease by Doppler echocardiography. Patients and Methods: 30 adults with CF and 82 adults with COPD underwent ultrasonography to evaluate pulmonary and cardiac hemodynamics. All patients were divided into four groups based on the presence/absence of clinical signs of right ventricular failure and pulmonary hypertension. Results: in COPD, diastolic dysfunction of the right heart can be subclinical preceding systolic impairment. Right ventricular hypertrophy and failure in CF occur in relatively low pulmonary artery pressure. Thus, in systolic pulmonary pressure less than 40–50 mm Hg, hypertrophy and dilation were concomitant. As the diastolic function of the right ventricle worsens, right atrial contractility increases while the ratio of left ventricular filling velocities during the early atrial diastole and systole reduces in patients with CF. Conclusion: in severe COPD, significant structural and functional impairments of the right ventricle occur. Compensatory potentialities of the right and left ventricles exhaust, thereby providing the conditions for the progression of heart failure and the development of the chronic pulmonary heart. In CF, diastolic dysfunction of the right heart often precedes systolic dysfunction, as illustrated by changes in the proportion of various filling phases of the right ventricle. In right ventricular diastolic dysfunction, an increase in right atrial contractility occurs that reduces only in severe decompensation of the chronic pulmonary heart. KEYWORDS: chronic obstructive pulmonary disease, cystic fibrosis, echocardiography, Doppler cardiography, pulmonary hypertension, right ventricular failure, diastolic function. FOR CITATION: Titova O.N., Kuzubova N.A., Aleksandrov A.L. et al. Pulmonary and cardiac hemodynamics in COPD and cystic fibrosis by Doppler echocardiography. Russian Medical Inquiry. 2021;5(7):456–461 (in Russ.). DOI: 10.32364/2587-6821-2021-5-7-456-461.

P885 Pulmonary artery stent implantation in an adult with chronic thromboembolic pulmonary stenosis

Abstract Introduction Pulmonary artery stenosis presenting in adults is rare. Chronic thromboembolic pulmonary hypertension (CTEPH) is by far the most common cause of pulmonary artery stenosis. Stenosis in these patients are not caused by an abnormality of the arterial wall itself, but by intraluminal narrowing as a result of the only partially resolved and organized thromboembolism. In contrast to paediatric patients, in adults with pulmonary artery stenosis, pulmonary stenting is not routinely performed. Case report A 51-year male, smoker, diabetic, hypertensive, and with chronic kidney disease. He was diagnosed two years earlier with bilateral multiple pulmonary emboli and was maintained on oral anticoagulation therapy. Recently, he presented with gradually progressive shortness of breath and signs of right ventricular failure. Diagnostic imaging: 1-Transthoracic and transesophageal echocardiography showed normal global systolic left ventricular function with no regional wall motion abnormalities, dilated right ventricle (RV) with moderately impaired function, severe pulmonic valve incompetence, mild tricuspid incompetence and a severely elevated right ventricular systolic pressure (RVSP) of 82 mmHg. In addition, a small rounded mass (6 x 11 mm) was visualized attached to the posterior wall of the RV outflow tract (RVOT) about 15mm proximal to the pulmonary valve annulus, (figure A). 2- Computed tomography pulmonary angiography showed a right main pulmonary artery (RPA) with circumferential narrowing, which was highly suggestive of chronic thrombosis. There was an abrupt tapering noted in the segmental branches of the right lower lobar pulmonary artery, with non-opacification of the distal arteries. No contrast opacification was noted in the right upper lobe pulmonary arteries. The left main pulmonary artery showed thickening of its bifurcation, again suggestive of chronic thrombosis, with narrowing of its left upper lobar branch, (figures B&C). 3-Cardiac magnetic resonance (CMR) showed a non enhancing RVOT mass protruding through the incompetent pulmonary valve during systole with features suggestive of a thrombus. Management In view of the clinical history, CTEPH was considered to be the most likely aetiology of the pulmonary hypertension. The decision was to perform balloon angioplasty and stent implantation in the RPA. Immediately after the procedure, RVSP was reduced from 80 to 50 mmHg. The clinical course after this procedure was uncomplicated and the patient showed significant clinical improvement. Follow up CMR showed patent stent with improvement of RV function ( fig D) Abstract P885 Figure.

P1670Incidence and prognosis of late-onset right ventricular failure following continuous-flow left ventricular assist device implantation as bridge to transplantation

Abstract Early right ventricular failure (RVF) remains a frequent complication and is one of the main factors associated to early mortality following left ventricular assist device (LVAD) implantation. However, late-onset RVF (LoRVF) has emerged as an increasing concern, but little is known about its incidenceand underlying mechanisms. Methods and results We retrospectively analysed the 1-year hemodynamic and clinical data from all patients that, between 2016 and 2018, underwent a right heart catheterization (RHC) after continuous-flow LVAD implantation as bridge to transplantation. Sixty-six patients (84% males, 53±11 years, 60% implanted in Intermacs 1–2, 52% HeartWare LVAD, 48% HM3 LVAD), out of 187 LVAD implants, were studied. LoRVF was defined as central venous pressure>18 mmHg with cardiac index<2.3 L/min/m2 during RHC. LoRVF was present in 17 patients (25.7%) and 12 (71%) of them manifested concomitant clinical signs of RVF. Eleven of the patients who presented LoRVF (65% of the LoRVF) had a pulmonary capillary wedge pressure (PCWP)>15 mmHg. Isolated LoRVF (LoRVF criteria + PCWP≤15 mmhg) was found in 6 patients (35% of the LoRVF) and accounted for 9% of the studied population. Fifty percent of patients who presented isolated LoRVF could be successfully transplanted in high urgent status due to severe chronic RVF and 1 patient died to refractory RVF. We did not find an association between isolated LoRVF and age, renal function, type of LVAD, persistent increased pulmonary vascular resistances or the previous need of temporary right ventricular support due to early acute RVF following LVAD. There was however a significant association between the presence of atrial fibrillation and isolated LoRVF (p<0.05). Incidence and prognosis of LoRVF Conclusion Late-onset RVF is a frequent complication during LVAD support as bridge to transplantation. Most of the cases are associated to a lesser degree of left ventricular unloading. The presence of isolated late-onset RVF with normal PCWP has an impact on the long-term prognosis and the need of urgent heart transplantation and is not related to early RVF following LVAD implantation or persistent increased pulmonary vascular resistance.

ВЗАИМОСВЯЗЬ ПОКАЗАТЕЛЕЙ ФУНКЦИОНАЛЬНОГО СОСТОЯНИЯ ДИАФРАГМЫ И ЛЁГОЧНОЙ ГЕМОДИНАМИКИ У БОЛЬНЫХ ХРОНИЧЕСКОЙ ОБСТРУКТИВНОЙ БОЛЕЗНЬЮ ЛЁГКИХ

The aim of the study was to study the correlation between the indices of the functional state of the diaphragm and pulmonary hemodynamics in COPD patients at different stages of chronic pulmonary heart (CPH). 79 patients with COPD were examined. Patients were divided into 2 groups, taking into account the pulmonary artery pressure and the presence of clinical signs of right ventricular failure. The first group included 40 COPD patients complicated by compensated CPH; the second group included 39 patients with decompensated CPH. A complex examination of intracardiac hemodynamics and functional state of the diaphragm was carried out at the XD-11 XE device PHILIPS (USA). The following results were obtained: in patients with compensated CPH there was an increase in the thickness of the diaphragm, a decrease in the diaphragm mobility during tidal and forced breathing, and an increase in the rate of contraction of the diaphragm during tidal and forced breathing. Patients with decompensated CPH were found to have an increase in the thickness of the diaphragm, a sharp decrease in the diaphragm excursion during tidal and forced breathing, a decrease in the fraction of thickening of the muscular part of the diaphragm. The correlation analysis revealed the most informative ultrasound indices of the impairment of the functional state of the diaphragm in patients with COPD, significantly associated with pulmonary hemodynamic indices: diaphragm excursion during tidal and forced breathing, relaxation rate of the diaphragm muscle, thickness and fraction of thickening of the muscular part of the diaphragm. Determination of the functional state of the diaphragm in patients with COPD allows an early detection of signs of respiratory muscle dysfunction, a timely appointment of corrective therapy, and prevention of the development and progression of pulmonary-cardiac failure.

UP-FRONT TRIPLE COMBINATION THERAPY FOR PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH SYSTEMIC SCLEROSIS: A CASE REPORT

Pulmonary arterial hypertension (PAH) is a progressive and irreversible disease characterized by a steady increase in pulmonary vascular resistance (PVR) and death. Introduction of current PAH-specific drugs has not solved the problem of follow-up of critical patients with PAH associated with systemic sclerosis (SSc). Clinical trials have shown that 1-, 2- and 3-year survival rates are 77, 46, and 33%, respectively. Arguments in favor of starting triple PAH-specific therapy that substantially improves the prognosis of this critical group of patients are being accumulated. The paper describes Russia’s first clinical case of successful up-front triple combination therapy (iloprost, bosentan and sildenafil) in a 40 year old female with SSc and diagnosed functional class (FC) IV PAH. There were clinical, laboratory, and hemodynamic improvements already at 2 weeks after therapy initiation. Thirty-month therapy resulted in a reversal of FC from IV to II, relieved the signs of right ventricular failure, complete right ventricular reverse remodeling and subnormalization of hemodynamic parameters. Hemodynamic improvement including reductions in right atrial pressure to 4 mm Hg, mean pulmonary arterial pressure to 26 mm Hg, and PVR to 2.2 Wood units and an increase in cardiac output up to 7.4 l/min, despite preserved elevated uric acid levels (432 μmol/l). The intriguing feature of this case is critically low values of % predicted/carbon monoxide lung diffusion capacity, which rose from 17% to only 22%, in the presence of positive changes. This clinical case demonstrates the predictive capabilities of up-front triple combination therapy for severe PAH associated with SSc.

Отек легких в ближайшем послеоперационном периоде у больных, перенесших вмешательство на открытом сердце и имеющих признаки правожелудочковой недостаточности

Отек легких в ближайшем послеоперационном периоде у больных, перенесших вмешательство на открытом сердце и имеющих признаки правожелудочковой недостаточности

ACUTE LOWER MYOCARDIAL INFARCTION: CLINICAL AND DIAGNOSTIC FEATURES

The paper displays the need for an extended electrocardiographic diagnosis including right breast leads for all patients with lower myocardial infarction at early stages of medical care. The diagnosis method presented here will allow to evaluate the localization of myocardial damage and to identify patients with signs of right ventricular damage that require clinical evaluation to identify signs of right ventricular failure and special methods for its correction.

Clinical profile and in-hospital outcome of Caucasian patients with takotsubo syndrome and right ventricular involvement

AimTo determine the prevalence, clinical characteristics, in-hospital course and determinants of major adverse events in a cohort of Caucasian patients with Takotsubo syndrome (TTS) and right ventricular involvement (RVi), regardless of left ventricular variant forms. Methods and resultsThe study population consisted of 424 patients (mean age 69.1±11.5years; female 92.2%) with a diagnosis of TTS divided into two groups according to the presence or absence of RVi. RVi patients (n=57; 13.4%) showed a higher prevalence of comorbidities, especially respiratory diseases (p=0.011), and a higher Charlson comorbidity index (CCI; p=0.006) than non-RVi patients. In-hospital major adverse events (acute heart failure, cardiogenic shock and death) occurred more frequently in RVi patients (p<0.001). Heart rate and CCI, along with the echocardiographic parameters of wall motion score index, E/e’ ratio, tricuspid annular plane systolic excursion (TAPSE) and systolic pulmonary artery pressure (sPAP) were associated with adverse in-hospital outcome. At multivariate analysis, CCI (HR: 1.871; 95% CI: 1.202–2.912; p=0.006), sPAP (HR: 1.059; 95% CI: 1.016–1.104; p=0.007) and TAPSE (HR: 0.728; 95% CI: 0.619–0.855; p<0.001) were independent correlates of the composite outcome in patients with RVi. ConclusionPatients with RVi are characterized by distinct clinical profile and should undergo closely clinical and echocardiographic monitoring. The presence of echocardiographic signs of right ventricular failure along with substantial comorbidities burden identify a cohort at higher risk of in-hospital major adverse cardiovascular events.

Predictors of inhospital prognosis in acute pulmonary embolism

The assessment of commonly available demographic, clinical, and easily calculable investigational parameters instead of the conventional complicated indices for prognosis in acute pulmonary embolism may help in triage in a simple and cost-effective way. Clinical, demographic, and investigational parameters were collected and utilized for the assessment of inhospital prognosis of acute pulmonary embolism in 200 consecutive patients admitted to our institute. Overall mortality was 18% and poor outcome at discharge was seen in another 18.5%. In univariate analysis, predominant presenting symptom of fatigue, sudden onset of symptoms, overt right ventricular failure, hypoxemia at admission, low SBP and DBP, coexistent pulmonary or cardiac illness, ECG evidence of right axis deviation, SIQ3T3 pattern, conduction blocks, echocardiographic evidence of right ventricular dysfunction, decreased inspiratory collapse of inferior vena cava, severe pulmonary arterial hypertension, visible thrombus in pulmonary artery, significant tricuspid regurgitation, computed tomographic evidence of total occlusion of major pulmonary arteries, diameter of main pulmonary artery, acute or chronic pulmonary embolism, renal and hepatic dysfunction, hyponatremia, hyperkalemia, troponin elevation, use of fibrin-specific agent, requirement of inotropic support, and mechanical ventilation were the variables found to significantly predict adverse outcome. In multivariate analysis, hypoxemia, no improvement after lysis, deranged liver function test, conduction blocks, and signs of right ventricular failure were the significant variables, while inotropic support requirement had a trend toward significance. Clinical, demographic, and routine investigational parameters help to risk-stratify the patients presenting with acute pulmonary embolism and to prognosticate and manage in a simpler yet effective way.

Urgent Cardiac Surgery During Pregnancy: A Continuous Challenge

Cardiac surgery during pregnancy is reserved for cases of failure of medical treatment due to its detrimental maternal and fetal effects. Between March 2003 and December 2012, 23 pregnant women with severe valve malfunction required open heart surgery. The mean age was 30.3±4.9 years. Gestational age ranged from 14 to 39 weeks. Twenty patients (87%) presented with signs of right ventricular failure. The main presenting lesion was severe aortic stenosis in 3 patients (13%), mitral stenosis in 3 patients (13%), and stuck mechanical mitral valve prosthesis in 17 patients (73.9%). Fourteen patients were operated upon on emergency basis, and 9 patients on urgent basis. Two patients have continued their pregnancy to full term after cardiac surgery. Delivery was done immediately before surgery in 11 patients (47.8%). There were 10 intrauterine fetal deaths, all at gestational age below 28 weeks, 4 were confirmed before induction of anesthesia and 6 after surgery. There were 13 surviving babies, 7 premature babies (30.4%), and 6 full term babies (26.1%). Neonatal complications included respiratory distress syndrome in (38.5%) and prolonged hospital stay (46.2%). Maternal hospital morbidities included oliguria (65.2%), bleeding (30.4%), prolonged intensive care stay (56.5%), and prolonged hospital stay (26.2%). The in-hospital mortality was 8.7%. Urgent and emergency valve replacement in pregnant women can be achieved with good maternal morbidity and mortality; however, high incidence of fetal losses might be expected when surgery is performed at an early gestational age.

Pharmacological management of pulmonary arterial hypertension.

Pharmacological management of pulmonary arterial hypertension.

Experimental right ventricular failure is associated with dysregulation of genes involved in dilated cardiomyopathy

Purpose: We aimed to identify pathways involved in experimental right ventricular failure (RVF) due to chronic pressure overload. Methods: Wistar rats were subjected to pulmonary artery banding (PAB) (1.1mm, n=12) or sham surgery (CON, n=7) and then daily checked for clinical signs of RVF (inactivity, ruffled fur, dyspnea, ascites). At the onset of clinical RVF (necessitating termination) pressure-volume analysis was performed in the rats with RVF and in randomly selected PAB rats without clinical signs of RVF (RVDysfunction, RVD) at the same time points. Gene micro array was performed on RV tissue using GeneChip Rat Gene 1.1 ST (Affymetrics). Quality control and normalization (RMA) were performed according to international standards. False discovery rate was set to 1.3 in RVF vs. RVD. Micro array data were analyzed using gene-set enrichment analysis (GSEA), gene ontology mapping (biological processes) (ErmineJ) and pathway analysis (DAVID). Results: All rats with PAB had RV dysfunction, but clinical RVF ensued in only 5 rats, after a period of 52±5 days. RVF rats had pericardial effusion, liver congestion, and widely dilated right atria, in contrast to RVD rats. RVF rats also had significantly lower cardiac index (0.10±0.04 vs. 0.19±0.04 mL/min/g, RVF vs. RVD, p<0.05). In RVF contractility was preserved (end systolic elastance 269±173 vs. 101±44mmHg/mL, ns), but diastolic function deteriorated (end diastolic elastance 32±21 vs. 11±4mmHg/mL; end diastolic pressure 6±3 vs. 3±1mmHg, RVF vs. RVD, p<0.05). We identified 9 pathways that were differentially regulated in RVF vs. RVD. Among these, the dilated cardiomyopathy (DCM) KEGG-pathway was negatively enriched in RVF compared to RVD (normalized enrichment score= -1.94, p<0.05). Gene ontology mapping supported the dysregulation of the DCM pathway in RVF; 10 ontology categories overlapping with the DCM pathway were strongly decreased in RVF vs. RVD (p<1.00E-10 for all categories). Further pathway analysis and comparison to CON, revealed that within the DCM pathway, the dysregulation involved transforming growth factor beta (TGFβ) 2 (Rank Metric Score: -4.316 RVF vs. RVD), TGFβ 3 (RMS: -3.840) and specific calcium channel-subunits: CACNA2D2 (RMS: -2.429), CACNA2D3 (RMS: -1.754), CACNA1D (RMS: -1.730) and CACNB3 (RMS: -2.429). Conclusions: We identified novel pathways involved in experimental RVF. RVF is associated with dysregulation of specific parts of the dilated cardiomyopathy pathway. New studies should explore whether an imbalance in TGFβ activation or alterations in calcium cycling are central in the pathobiology of RVF.

Early postoperative interventional ASD-closure for severe atrial right to left shunt in a neonate with common arterial trunk

Although closure of an atrial septal defect (ASD II) with an occluding device in the first year of life is not a routine procedure, it is a feasible treatment, even in neonates. Case reports on the off-label use of Amplatzer devices have been repeatedly published, but there are no reports on using the Amplatzer Duct Occluder (ADO) to close an atrial septal defect in a neonate. We report on a successful catheter closure of an ASD II with ADO in a severely cyanotic neonate, seven days after surgical repair of common arterial trunk. Due to progressive cyanosis and clinical signs of right ventricular failure, which developed after common arterial trunk repair, the neonate underwent cardiac catheterization. Diastolic filling impairment of the right ventricle (right ventricle hypertrophy, pulmonary regurgitation, and residual right ventricle outflow tract obstruction) was thought to be the cause of impaired right ventricle diastolic filling, resulting in the right-to-left shunt at the atrial level. Under transesophageal echocardiographic guidance, ADO was delivered through a 5 French sheath into the atrial septal defect. Amplatzer duct occluder closed the defect and proved to be stable in position after disconnection. During the procedure, the child was stable and then transferred to the intensive care unit with significantly improved oxygen saturation. This is the first report on placing a duct occluder in the atrial septal position, which is a novel procedure for-small neonates.

Delayed Myocardial Enhancement by Cardiac Magnetic Resonance Imaging in Pulmonary Arterial Hypertension: A Marker of Severity of Disease

Delayed Myocardial Enhancement by Cardiac Magnetic Resonance Imaging in Pulmonary Arterial Hypertension: A Marker of Severity of Disease

The association between obesity, mortality and filling pressures in pulmonary hypertension patients; the “obesity paradox”

The term "obesity paradox", refers to lower mortality rates in obese patients, and is evident in various chronic cardiovascular disorders. There is however, only scarce data regarding the clinical implication of obesity and pulmonary hypertension (PH). Therefore, in the current study, we evaluated the possible prognostic implications of obesity in PH patients. We assessed 105 consecutive PH patients for clinical and hemodynamic parameters, focusing on the possible association between Body Mass Index (BMI) and mortality. Follow-up period was 19 ± 13 months. Sixty-one patients (58%) had pre-capillary PH and 39 patients (37%) out-of-proportion post-capillary PH. During follow-up period, 30 patients (29%) died. Death was associated with reduced functional-class, inverse-relation with BMI, higher pulmonary artery and right atrial pressures, pulmonary vascular resistance and signs of right ventricular failure. In multivariate analysis, obesity (BMI ≥ 30 kg/m²), was the variable most significantly correlated with improved survival [H.R 0.2, 95% C.I 0.1-0.6; p = 0.004], even after adjustment for baseline characteristics. Obese and very-obese (BMI ≥ 35 kg/m²) patients had significantly less mortality rates during follow-up (12% and 8%, respectively) than non-obese patients (41%), p = 0.01. The tendency of survival benefit for the obese vs. non-obese patients was maintained both in the pre-capillary (10% vs. 46% mortality, p = 0.008) and disproportional post-capillary PH patients (11% vs. 40% mortality, p = 0.04). Obesity was significantly associated with lower mortality in both pre-capillary and disproportional post-capillary PH patients. It seems that in PH, similarly to other chronic clinical cardiovascular disease states, there may be a protective effect of obesity, compatible with the "obesity paradox".

Association between right ventricular two-dimensional strain and exercise capacity in patients with either idiopathic or ischemic dilated cardiomyopathy

To detect right ventricular myocardial function in dilated cardiomyopathy (DCM) using two-dimensional strain echocardiography (2DSE) and to evaluate the relationship between right ventricular dysfunction and response to cardiopulmonary exercise test (CPET). Seventy-five DCM patients (44 idiopathic and 31 ischemic) without clinical signs of right ventricular failure underwent standard echo, 2DSE analysis of right ventricle and bicycle CPET. The two groups were comparable for clinical and standard two-dimensional echocardiographic and Doppler variables, except for right ventricular diameters that were mildly increased in patients with idiopathic DCM. Right ventricular global longitudinal strain (RV GLS) and regional peak myocardial right ventricular strain were significantly impaired in patients with idiopathic DCM compared with ischemic DCM (both P<0.001). A significant correlation was detectable among RV GLS and VO2 peak percentage (r= -0.65, P<0.0001), VE/VCO2 slope (r=0.35, P<0.01), maximum work rate percentage (r= -0.55, P<0.001) and peak circulatory power (r=0.53, P<0.001). These correlations with RV GLS remained significant even in multivariate analysis. 2DSE represents a promising noninvasive technique to assess right ventricular myocardial function in patients with DCM. Reduced right ventricular myocardial deformation is related to decreased ability to perform aerobic exercise and work rate, and to impaired ventilatory response.

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.

Leiomyosarcoma - an Unusual Cause of Right Ventricular Outflow Tract Obstruction

A 76-year-old female patient was admitted with progressive dyspnea on exertion, signs of right ventricular failure, and severe pulmonary stenosis. Intraoperatively an infiltrating right ventricular outflow tract (RVOT) tumor was found and xenograft conduit replacement was performed successfully. Histological examination revealed primary cardiac leiomyosarcoma, the patient was discharged and is in good health condition at 9 months' follow-up. Unusual causes of RVOT obstruction should be considered.

Natural history and risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Management of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is complicated by the incomplete information on the natural history of the disease and by the lack of risk stratification for cardiovascular death. The aim of the study was the identification of risk factors related to long-term prognosis. Data were collected from 130 patients (100 men; age at onset of symptoms, 31.8+/-14.4 years) from a tertiary center between 1977 and 2000 who fulfilled the international standardized diagnostic criteria for ARVD/C. Risk factors for cardiovascular death were determined by a logistic regression model. After a mean follow-up of 8.1+/-7.8 years, 24 deaths were recorded, with a mean age at death of 54+/-19 years (annual mortality rate, 2.3%). There were 21 deaths with a cardiovascular origin (progressive heart failure for 14 patients and sudden death for the remaining 7 patients). All patients who died had a history of ventricular tachycardia. Multivariate analysis showed that after adjustment for sex, history of syncope, chest pain, inaugural ventricular tachycardia, recurrence of ventricular tachycardia, and QRS dispersion, clinical signs of right ventricular failure and left ventricular dysfunction both remained independently associated with cardiovascular mortality. The combined presence of one of these risk factors and ventricular tachycardia identifies high-risk subjects for cardiovascular mortality, whereas patients without ventricular tachycardia displayed the best prognosis. The information on the natural history of patients with ARVD allowed us to identify risks factors for cardiovascular mortality. An analysis of a large international registry is needed to refine these results.