Results Of Right Heart Catheterization Research Articles

Clinical Characteristics and Outcomes Associated With Distinct Hemodynamic Patterns in End-stage Liver Disease: A Retrospective Cohort Analysis

IntroductionDespite advances in the diagnosis and therapeutics strategies for pulmonary hypertension (PH) in patients with end-stage liver disease (ESLD), the impact of hemodynamic patterns among ESLD patients identified through right heart catheterization (RHC) on clinical outcomes remains poorly understood. MethodsThis single-center retrospective cohort study identified patients diagnosed with ESLD who underwent RHC from August 2018 to June 2023. Demographic and clinical data, including comorbidities, transthoracic echocardiography, and RHC findings, were obtained. Our outcomes of interest were all-cause mortality and the chance of receiving orthotopic liver transplantation (OLT) within a year after RHC. Kaplan-Meier with log-rank test was employed to generate survival curves. ResultsWe identified 415 ESLD patients with the RHC results. The median (IQR) age was 59 years (52-66), and 62% were male. Caucasians accounted for 43%, followed by African Americans (30%). Up to 89% had a diagnosis of portal hypertension. Median MELD-Na score was 30 (19-36). The etiology of ESLD was mainly from alcohol use (55%). Patients were classified based on RHC results as pre-capillary PH (19%), post-capillary PH (28%), and non-PH (53%) groups. Overall, one-year mortality post-RHC was 22%, with no significant difference in mortality regardless of hemodynamic group. However, the pre-capillary PH group was less likely to receive OLT compared to other groups (p<0.001). ConclusionWe observed no difference in all-cause mortality among hemodynamic groups. However, pre-capillary PH group were less likely to undergo OLT compared to others. Further investigations are necessary to determine how this should be addressed in clinical practice.

A Twins with Congenital Heart Disease: A Case Report

Background: Twin pregnancies have a higher risk of congenital heart defects compared to singleton pregnancies. The most commonly found congenital heart defect in both monochorionic and dichorionic twin gestations is ventricular septal defect (VSD). Approximately 10% of individuals with congenital heart defects will develop pulmonary arterial hypertension-congenital heart disease (PH-CHD).  Case Presentation: We diagnosed an 11-year-old twin girl with ventricular septal defect. First Patient had a severe PH with mean Pulmonary Artery Pressure (mPAP) 68 and a defect size of 9-12 mm, while second patient had a mild PH with mPAP 30 and a defect size of 6-9 mm. The patient presented with failure to thrive, exercise intolerance during daily activities, and a history of recurrent respiratory tract infections. Echocardiography and right heart catheterization (RHC) results showed that the first patient with VSD Left to Right Shunt with low flow high resistance, non-reactive oxygen test and pulmonary hypertension. The second patient with VSD Let to Right shunt. Both of the patients were diagnosed with ventricular septal defect (VSD) and pulmonary hypertension (PH). However, they received different treatments based on the RHC results.  Conclusion: Promptly addressing the long-term implications of CHD is essential to prevent pulmonary hypertension (PH) and systemic disorders. Advanced PH treatments are safe and can result in improved hemodynamic, exercise capacity, quality of life, growth and development and potentially survival.

Incidence of pulmonary hypertension in COPD and its relation to inflammatory marker interleukin-1

BackgroundMany COPD patients present with severe PH defined by a pulmonary vascular resistance (PVR) > 5 WU as measured by right heart catheterization (RHC), and inflammation is thought to be contributing strongly to pulmonary vascular remodeling in COPD besides hypoxia. Interleukin-1 (IL-1) is thought to be a major cytokine that may be involved in development of PH in these patients.ObjectiveThis study aimed to identify the incidence of PH in COPD and its relation to inflammatory marker IL-1.Patients and methodsOne-hundred COPD patients underwent echocardiography and serum IL-1 analysis, and patients with high probability of PH underwent pulmonary artery catheterization using Swan-Ganz catheter.ResultsThe mean serum IL-1 level was 82 pg/ml ± 22 pg/ml (the normal IL-1 serum level is known to be 0:5 pg/mL), 51% of the participants were categorized as having a low probability of PH, 35% was intermediate, and 14% was high. RHC results were as follows: mild combined pre- and postcapillary PH was diagnosed in 14.3% of the patients. Mild precapillary PH was found in 42.9%, making it the most common type. Severe combined pre- and postcapillary PH was noted in 21.4% of the patients. Severe precapillary PH was present in 14.3%. A significant positive correlations were observed between serum IL-1 and tricuspid regurgitation velocity (TRV) (r = 0.409, P < 0.001), estimated systolic pulmonary artery pressure (ESPAP) (r = 0.508, P < 0.001), and mean pulmonary artery pressure (mPAP) (r = 0.410, P = 0.140).ConclusionSerum IL-1 is a potent predictor of a high probability of PH in COPD patients, and there was significant positive correlation between serum IL-1 and echocardiographic findings and PH probability and RHC findings in COPD patients.

Non-invasive evaluation of pulmonary capillary wedge pressure using the left atrial expansion index in mitral valve stenosis, prosthesis and repair.

Pulmonary capillary wedge pressure (PCWP) non-invasive evaluation is limited in patients with mitral valve (MV) stenosis, prosthesis, and surgical repair. This study aimed to assess the left atrial expansion index (LAEI) measured through transthoracic echocardiography (TTE) as a novel parameter for estimating PCWP in these challenging cardiac conditions. We performed a retrospective, cross-sectional study, including chronic cardiac patients receiving within 24h a clinically indicated right heart catheterization (RHC) and transthoracic echocardiographic (TTE) exam. PCWP measured during RHC was used as the reference. TTE measurements were performed offline, blinded to RHC results. LAEI was calculated as LAEI = [(LAmaxVolume-LAminVolume)/LAminVolume] × 100. We included 167 patients (age = 73 ± 11.5years; PCWP = 18 ± 7.7mmHg) with rheumatic mitral valve (MV) stenosis (16.2%), degenerative MV stenosis (51.2%), MV prosthesis (18.0%), and MV surgical repair (13.8%). LAEI correlated logarithmically with PCWP, and the log-transformed LAEI (lnLAEI) showed a good linear association with PCWP (r = -0.616; p < 0.001). lnLAEI was an independent PCWP determinant, providing added predictive value over conventional clinical (age, atrial fibrillation, heart rate, MV subgroups) and echocardiographic variables (LVEF, MV effective orifice area, MV mean gradient, net atrioventricular compliance, and pulmonary arterial systolic pressure). lnLAEI identified PCWP > 12mmHg with AUC = 0.870, p < 0.001; and PCWP > 15mmHg with AUC = 0.797, p < 0.001, with an optimal cut-off of lnLAEI < 3.69. The derived equation PCWP = 36.8-5.5xlnLAEI estimated the invasively measured PCWP ± 6.1mmHg. In this cohort of patients with MV stenosis, prosthesis, and surgical repair, lnLAEI resulted in a helpful echocardiographic parameter for PCWP estimation.

The accuracy and influencing factors of Doppler echocardiography in estimating pulmonary artery systolic pressure: comparison with right heart catheterization: a retrospective cross-sectional study

BackgroundNoninvasive assessment of pulmonary artery systolic pressure by Doppler echocardiography (sPAPECHO) has been widely adopted to screen for pulmonary hypertension (PH), but there is still a high proportion of overestimation or underestimation of sPAPECHO. We therefore aimed to explore the accuracy and influencing factors of sPAPECHO with right heart catheterization (RHC) as a reference.MethodsA total of 218 highly suspected PH patients who underwent RHC and echocardiography within 7 days were included. The correlation and consistency between tricuspid regurgitation (TR)-related methods and RHC results were tested by Pearson and Bland–Altman methods. TR-related methods included peak velocity of TR (TR Vmax), TR pressure gradient (TR-PG), TR mean pressure gradient (TR-mPG), estimated mean pulmonary artery pressure (mPAPECHO), and sPAPECHO. With mPAP ≥ 25 mm Hg measured by RHC as the standard diagnostic criterion of PH, the ROC curve was used to compare the diagnostic efficacy of sPAPECHO with other TR-derived parameters. The ratio (sPAPECHO–sPAPRHC)/sPAPRHC was calculated and divided into three groups as follows: patients with an estimation error between − 10% and + 10% were defined as the accurate group; patients with an estimated difference greater than + 10% were classified as the overestimated group; and patients with an estimation error greater than − 10% were classified as the underestimated group. The influencing factors of sPAPECHO were analyzed by ordinal regression analysis.ResultssPAPECHO had the highest correlation coefficient (r = 0.781, P < 0.001), best diagnostic efficiency (AUC = 0.98), and lowest bias (mean bias = 0.07 mm Hg; 95% limits of agreement, − 32.08 to + 32.22 mm Hg) compared with other TR-related methods. Ordinal regression analysis showed that TR signal quality, sPAPRHC level, and pulmonary artery wedge pressure (PAWP) affected the accuracy of sPAPECHO (P < 0.05). Relative to the good signal quality, the OR values of medium and poor signal quality were 0.26 (95% CI: 0.14, 0.48) and 0.23 (95% CI: 0.07, 0.73), respectively. Compared with high sPAPRHC level, the OR values of low and medium sPAPRHC levels were 21.56 (95% CI: 9.57, 48.55) and 5.13 (95% CI: 2.55, 10.32), respectively. The OR value of PAWP was 0.94 (95% CI: 0.89, 0.99). TR severity and right ventricular systolic function had no significant effect on the accuracy of sPAPECHO.ConclusionsIn this study, we found that all TR-related methods, including sPAPECHO, had comparable and good efficiency in PH screening. To make the assessment of sPAPECHO more accurate, attention should be paid to TR signal quality, sPAPRHC level, and PAWP.

Pulmonary vascular remodeling and right ventricular adaptation in precapillary pulmonary hypertension

Background: In precapillary pulmonary hypertension (PH) as pulmonary arterial hypertension (PAH), chronic thromboembolic PH (CTPEH) and PH due to hypoxia and lung disease (HPH) the different pathomechanisms of vascular remodeling result in different right ventricular (RV) adaptation. Routine clinical factors which could predict vascular remodeling are lacking. Purpose: Our aim was to investigate the differences in parameters of pulmonary vascular remodeling and RV adaptation in subgroups of precapillary PH and to correlate the severity of PH with these parameters. Methods: Fifty-one patients (age 57±15 years) with precapillary PH (PAH N=18, CTEPH N=15, HPH N=18;) underwent right heart catheterization (RHC) and results of RHC, echocardiography and laboratory tests were analyzed retrospectively. Results: The majority of parameters did not show difference among PH groups, but diastolic PAP was higher in PAH than in CTEPH and HPH (p&lt;0.05) and RV outflow tract velocity time integral (RVOT VTI) and RV stroke work index were increased in PAH compared to HPH (p&lt;0.05). Pulmonary vascular resistance (PVR) showed a significant relation to pulmonary arterial compliance (PAC) in the total cohort (p&lt;0.05, r=–0.661) and in all subgroups (PAH p&lt;0.05, r=–0.70; CTEPH p&lt;0.05, r=–0.525; HPH p&lt;0.05, r=–0.793). In PAH, all parameters including RVOT VTI and acceleration time (AT), tricuspid annular plane systolic excursion, RV diameter and right atrial area showed correlations with PVR. In the HPH group, only RVOT AT had a correlation with PVR, but no parameters in the CTEPH group showed a relationship to other factors. Conclusion: In precapillary PH the different degree of RV adaptation in the subgroups is shown by RVSWI as an invasive parameter. The severity of PH is related to PAC in precapillary PH. Some parameters can better characterize the severity of vascular and cardiac changes in PAH than in CTEPH or HPH.

455 Haemodynamic prediction of primary graft dysfunction in lung transplant recipients

Abstract Aims Primary graft dysfunction (PGD) is a form of acute lung injury, that occurs after lung transplantation (LTx), characterized by pulmonary oedema and diffuse alveolar damage. Pulmonary hypertension is a well-known risk factor for PGD and some invasive and non-invasive studies showed an association between PGD and altered left heart filling pressure. Despite the cardiopulmonary haemodynamic seems to be mainly involved in the pathogenesis of PGD, no reliable predictive parameter has been demonstrated. The aim of our study is to test whether pulmonary arterial pressure and left diastolic function may be considered in the risk PGD stratification. Methods and results we retrospectively analyzed the results of right heart catheterization (RHC) performed in occasion of the assessment for the LTx eligibility. All patients have been assessed at the Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico of Milan (Italy) from 2006 to 2018. We included all form of respiratory diseases except for cystic fibrosis. PGD was defined as PaO2/FIO2 &amp;lt; 300, with infiltrates at 72 h after reperfusion. We grouped patients in two groups according to the PGD development or absence (PGD+ and PGD−). Ninety patients were analyzed (mean age 55 ± 10; 53 male). Bilateral LTx was performed in 57 cases (63%). The most frequent indications for LTx were Interstitial Lung Disease (38%), Idiopathic Pulmonary Fibrosis (32%) an COPD (16%). Mean pulmonary arterial pressure (mPAP 29.4 ± 11.5 mmHg vs. 24.2 ± 9.7 mmHg, P = 0.016) and PAWP (12.9 ± 4.3 mmHg vs. 10.4 ±.,8 mmHg, P = 0.012) values were significantly higher in the PGD+ than in the PGD− group as well as PAWP values. At the multivariate analysis, both mPAP and PAWP were independent risk factors for PGD development even adjusted for BMI, age, or indication for LTx (mPAP non-adjusted OR: 1.05, 95% CI: 1.01–1.10, P = 0.027; mPAP adjusted OR: 1.06, 95% CI: 1.00–1.12, P = 0.046; PAWP non-adjusted OR: 1.13, 95% CI: 1.02–1.25, P = 0.016; PAWP adjusted OR: 1.14, 95% CI: 1.01–1.29, P = 0.036). No difference was observed between pulmonary vascular resistance (PVR) values. After a sub analysis of the patients with PAWP ≥15 mmHg, we observed that the ratio between PVR and PAWP was significantly higher in the PGD + group (0.18 ± 0.11 vs. 0.09 ± 0.05, P = 0.036). Conclusion s our data confirmed that pulmonary circulation plays a crucial role in the prediction of PGD and elevated mPAP is the one of the main risk factors. Of note, despite in both group PAWP in was within normal values (&amp;lt;15 mmHg), it was determinant in the risk stratification for PGD development. We suppose that the increased PVR due to pulmonary parenchymal diseases may “mask” and “underestimate” the role of the left ventricular diastolic dysfunction creating a sort of vascular barrage witch is overcome after lung transplantation hesitating in pulmonary oedema. This hypothesis is corroborated by the significative difference of PVR/PAWP ratio that can select patients at risk for PGD.

Internal jugular vein ultrasound in patients with chronic congestive heart failure

Abstract Background Bedside assessment of intravascular volume in patients with chronic congestive heart failure (CHF) is often difficult. Under- and over-diuresis are common causes of morbidity and readmissions in these patients. Purpose We hypothesized that ultrasound assessment of the internal jugular vein would be easier and more reproducible than clinically assessing jugular venous pressure (JVP). Our goal was to create a bedside test that would be simpler to learn than inferior vena cava (IVC) assessment and easier to perform in obese patients. Methods Adults with HF (n=53, 52% men, mean age 65 years, mean BMI 29.6 kg/m2, mean LVEF 44%) scheduled for right heart catheterization (RHC) had an ultrasound of their right internal jugular (RIJ) vein performed immediately prior. Cross-sectional area of RIJ was measured during normal breathing with patients at 90 and 45 degrees recumbency and was indexed by height (RIJI). JVP was also assessed clinically. Results were compared to right atrial pressure (RAP) measured by RHC. Operators were blinded to RHC results and vice versa. Results JVP was correctly assessed clinically in only 43%. RIJI at 90 and 45 degrees were significantly larger in patients with elevated RAP compared to euvolemic patients (Table). At 90 degrees, RIJI of &amp;gt;15 predicted a RAP of &amp;gt;10 mmHg with 68% sensitivity and 72% specificity. At 45 degrees, RIJI of &amp;gt;10 predicted a RAP of &amp;gt;10 mmHg with 94% sensitivity and a negative predictive value of 80% (Table). Simply being able to see the RIJ at 90 degrees (n=34) had an 82.4% positive predictive value for elevated RAP. IVC data could not be obtained on 23% of patients due to body habitus or inability to lay flat. Conclusion Ultrasonographic RIJI is more accurate than clinical assessment in patients with CHF and can be accurately performed even in obese patients. It requires only a basic linear ultrasound probe and was easily performed by clinicians at various stages of training with reproducible results. With the increased availability of bedside ultrasound in clinical practice, it is a feasible method of evaluating chronic CHF patients. Funding Acknowledgement Type of funding sources: None.

Left Atrial Expansion Index for Noninvasive Estimation of Pulmonary Capillary Wedge Pressure: A Cardiac Catheterization Validation Study

Pulmonary capillary wedge pressure (PCWP) plays a pivotal role in cardiac disease diagnosis and management. Right heart catheterization (RHC) invasively provides accurate PCWP measurement, but it is impractical for widespread use in all patients. The left atrial expansion index (LAEI), measured on transthoracic echocardiography, describes the relative left atrial volume increase during the left atrial reservoir phase. The aim of this study was to validate LAEI as a noninvasive parameter for PCWP estimation. A total of 649 chronic cardiac patients (mean age, 66±14years; mean PCWP, 14±7.6mm Hg; mean left ventricular ejection fraction, 50±15%) who underwent both clinically indicated RHC and transthoracic echocardiography within 24hours were retrospectively enrolled. Patients were randomly divided into derivation (n=509) and validation (n=140) cohorts. PCWP was measured during RHC and defined as elevated when >12mm Hg. Transthoracic echocardiographic parameters and LAEI were measured offline, blinded to RHC results. In the derivation cohort, LAEI correlated logarithmically with PCWP, and the log-transformed LAEI (lnLAEI) correlated linearly with PCWP (r=-0.73, P<.001). lnLAEI showed an independent and additive predictive role for PCWP estimation over clinical and diastolic dysfunction (DD) parameters. The diagnostic accuracy of lnLAEI for elevated PCWP identification (area under the curve=0.875, P<.001; optimal lnLAEI cutoff<4.02) was higher than either the single DD parameters or their combination. In the validation cohort, lnLAEI cutoff < 4.02 showed higher accuracy than the 2016 DD algorithm (88% vs 74%) for elevated PCWP identification. Finally, the equation PCWP=38.3 - 6.2×lnLAEI, obtained from the derivation cohort, predicted invasively measured PCWP in the validation cohort. In a cohort of patients with various chronic cardiac diseases, lnLAEI performed better than DD parameters and the 2016 DD algorithm for PCWP estimation. lnLAEI might be a useful echocardiographic parameter for noninvasive PCWP estimation.

Correlation between right ventricular-pulmonary artery coupling and the prognosis of patients with pulmonary arterial hypertension.

This study aimed to analyze the correlation between the efficiency coefficient of right ventricular–pulmonary artery coupling (ηvv) and the prognosis of patients with pulmonary arterial hypertension (PAH).A total of 64 patients who underwent right heart catheterization (RHC) were enrolled and divided into PAH and control groups depending on the RHC results. Pressure and volumetric methods were adopted to analyze the results of RHC and cardiac magnetic resonance imaging examination. The ηvv of patients in 2 groups were calculated, and the relationship between ηvv calculated by the 2 methods and the 2-year prognosis of patients with PAH was evaluated.The hemodynamic index and right ventricular–pulmonary artery coupling parameter of patients with PAH were significantly higher than those in the control group (P < .05). The right ventricular volume parameter in the PAH group was significantly different from that in the control group (P < .05). For patients with PAH, the end-systolic elastance/effective arterial elastance (Ees/Ea) calculated by the volumetric method was significantly related to the prognosis of patients (odds ratio = 0.192, 95% confidence interval: 0.042–0.868, P = .032). When Ees/Ea <0.67 was calculated by the volumetric method, the adverse prognosis of patients with PAH increased significantly (P < .05).The Ees/Ea calculated by the volumetric method may be better an independent factor for the prognosis of patients with PAH.

The impact of the new World Symposium on Pulmonary Hypertension definition of pulmonary hypertension on the prevalence of pre-capillary pulmonary hypertension.

Since the first World Symposium on Pulmonary Hypertension (WSPH; Geneva, 1973), pulmonary hypertension (PH) has been defined as a mean pulmonary artery pressure (mPAP) ≥25 mm Hg measured at right heart catheterization (RHC) while at rest in the supine position. At the 6th WSPH congress (Nice, 2018), a new proposal was presented defining pre-capillary PH as mPAP >20 mm Hg, with pulmonary arterial wedge pressure (PAWP) <15 mm Hg, and pulmonary vascular resistance (PVR) >3 WU. The aim of this study was to investigate the impact of the new definition of PH on the number of pre-capillary PH patients. The results of RHC performed with various clinical indications between 2017 and 2018 were analyzed. The 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) and the 6th WSPH congress PH definitions were used to identify PH patients. Fifty-eight RHC procedures were performed in our hospital in a 1-year period. Most were performed with a suspicion of PH (n=52). The remainder (n=6) were performed with indications of valvular heart disease or left heart disease. There were 40 females (69%) and 18 males (31%). The mean age was 53.3±16.6 years. The RHC results revealed a mean PAP of 36.4±16.4 mm Hg, PAWP of 12.6±3.9 mm Hg, and PVR of 4.9±4.4 WU. Forty-three of 58 patients (74.1%) were classified as pre-capillary PH according to the ESC/ERS PH guideline, whereas 50 of 58 patients (86.2%) had pre-capillary PH according to the new WSPH definition. The results of this study indicated that the impact of the new definition of PH on the number of pre-capillary PH patients identified was greater than the predicted <10%.

A case difficult to diagnose in adults: High sinus venous atrial septal defect

Sinus venous atrial septal defect (SVD) is highly difficult to diagnose because of its location. Below, we report a case of SVD which is misdiagnosed as pulmonary hypertension and anomalous pulmonary venous return. A 57-year-old female patient was referred to congenital disease outpatient clinic of a tertiary center. She was admitted to the hospital with complaints of fatigue and exercise dyspnea which had started a year ago. She had transthoracic echocardiography (TTE) examination done in another hospital which showed dilated right heart chambers and pulmonary hypertension. She underwent transesophageal echocardiography (TEE) examination with the suspicion of atrial septal defect (ASD), but no defect was seen. As her symptoms persisted, we repeated the TTE and TEE examination in our center. TEE revealed 0.6 cm ASD on the upper side of the interatrial septum. All four pulmonary veins were draining into the left atrium. Right heart catheterization (RHC) confirmed the diagnosis. A left-to-right shunt was detected and localized by a significant step-up in blood oxygen saturation found between mid and upper segments of the right atrium. According to our TEE and RHC results, we planned the surgical closure of the defect. Sinus venous ASD is deficiency of the superior portion of atrial septum adjacent to superior vena cava. Diagnosis of SVD is often more difficult than other forms of ASD and may require special imaging such as TEE, magnetic resonance imaging, or computed tomographic scanning. In conclusion, cardiologists must be aware about the possibility of SVD patients who have unexplained exertional dyspnea and fatigue, dilated right atrium and ventricle, pulmonary hypertension, paradoxical embolism, or atrial arrhythmias in their respective populations.

Deeper S Wave in Lead V5 and Broader Extent of T Wave Inversions in the Precordial Leads are Clinically Useful Electrocardiographic Parameters for Predicting Pulmonary Hypertension.

Electrocardiography (ECG) is used to screen for pulmonary hypertension (PH). However, it is unclear which parameters of ECG are the most useful for screening.ECG parameters related to right ventricular hypertrophy criteria were examined in 145 ECGs of subjects who were suspected to have PH and underwent right heart catheterization (RHC) (age 58.4 ± 17.5 years, 112 women, mean pulmonary arterial pressure [MPAP] 35.4 ± 13.3 mmHg). Based on the results of RHC, 108 subjects had PH (56 pulmonary arterial hypertension [PAH] and 52 chronic thromboembolic pulmonary hypertension [CTEPH]).Fourteen of 17 ECG parameters in the present study were significantly associated with PH on univariate analysis. On multivariable logistic regression analysis, S wave depth in lead V5 (odds ratio [OR] 1.25, 95% confidence interval [CI] 1.10-1.47) and depth of T wave inversion in lead V4 (OR 1.21, 95% CI 1.03-1.46) were independent predictors of MPAP ≥ 25 mmHg, and the cut-off values determined by receiver operating characteristic curve analyses were 0.42 mV and -0.28 mV, respectively.In conclusion, a deeper S wave in lead V5 and the presence of a wider extent of negative T waves in the precordial leads may be clinically simple and useful ECG parameters for screening for PH.

Cardiopulmonary exercise test predicts right heart catheterization.

Right heart catheterization (RHC) is usually required to confirm the diagnosis of pulmonary artery hypertension (PAH). As an invasive test, RHC may be associated with possible complications, so noninvasive parameters able to predict PAH at RHC would be extremely useful. To ascertain possible correlations between cardiopulmonary exercise testing (CPET) and hemodynamic parameters at RHC indicative of pulmonary hypertension (PH). Thirty-six consecutive outpatients with suspect of PAH underwent CPET and RHC; the intercept of ventilation (VEint) on the VE vs carbon dioxide production (VE/VCO2 ) and VE/VCO2 slope at CPET and diastolic pressure gradient (DPG), trans-pulmonary pressure gradient (TPG), mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) at RHC were assessed and compared. Ventilation VCO2 slope was directly related to DPG (r: .41, P: .019), TPG (r: .45, P: .01), mean pulmonary arterial pressure (mPAP, r: .36, P: .031), PVR (r: .41, P: .029), VEint and VE/VCO2 slope inversely related to DPG (r: -.63, P < .001), TPG (r: -.67, P < .001), mPAP (r: -.68, P < .001) and PVR (r: -.5, P < .001). In patients with suspected PAH, VEint during exercise and the VE/VCO2 slope might provide useful information to predict results of RHC. Their correlations with PVR and with DPG may be helpful in discriminating patients with isolated postcapillary PH from those with combined postcapillary and precapillary.

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Introduction: Right ventricular systolic pressure (RVSP) derived non-invasively from transthoracic echocardiogram (TTE) is routinely used to estimate pulmonary arterial systolic pressure (PASP) derived invasively from right heart catheterization (RHC). Studies have shown a wide range of correlation between the two values, but little evidence exists regarding the degree of correlation in patients with or without typical cardiovascular risk factors. Methods: In this retrospective cohort study, we reviewed the results of RHC and TTE performed between 2010 and 2012. Patients who had RHC within 6 months of TTE irrespective of history of pulmonary hypertension were included. A total of 161 patients met study criteria. We further characterized the patients in terms of presence or absence of comorbidities and risk factors such as gender, age, hypertension, diabetes mellitus, and smoking. Correlations were determined using Pearson product-moment correlations. Results: The correlation of RVSP and PASP was stronger in females (n=83, r=0.567, p<0.001) vs males (n=78, r=0.459, p<0.001), in non-hypertensives (n=44, r=0.675, p<0.001) vs hypertensives (n=114, r=0.463, p<0.001), in non-smokers (n=95, r=0.596, p<0.001) vs smokers (n=66, r=0.422, p<0.001), in non-diabetics (n=101, r=0.567, p<0.001) vs diabetics (n=60, r=0.439, p<0.001), and in patients aged > 70 years (n=79, r=0.554, p<0.001) vs < 70 years (n=82, r=0.477, p<0.001). Conclusions: In addition to differences in gender and age, the presence of cardiovascular comorbidities appears to decrease the ability of RVSP to identify pulmonary hypertension. We suggest that, in our population, findings of elevated RVSP on TTE in a female patient older than 70 years with no cardiovascular risk factors should lead to further evaluation for pulmonary hypertension.

Absence of acute vasoreactivity response in patients with pulmonary arterial hypertension associated with toxic oil syndrome

Purpose: Acute vasodilator testing (AVT) is important in patients with pulmonary arterial hypertension (PAH). Positive test predicts a better survival and response to calcium channel blockers therapy. The last guidelines recommend performing this test in patients with PAH, but its usefulness in different forms than idiopathic PAH is unclear. The aim of this study is to analyze the results in AVT in patients with PAH associated with toxic oil syndrome (TOS). Results: From 1993 to 2011 have been diagnosed in our hospital 32 late cases patients with PAH associated with TOS and have done an acute vasodilator test in 24 of them, in all cases the result was negative. The causes were analyzed as hemodynamic severity of disease at diagnosis, mutations in the BMPR2 gene or different histological changes. When comparing the results of right heart catheterization at the time of diagnosis among our population and a group of 52 patients with idiopathic PAH diagnosed in the same period, found no statistically significant differences but a tendency to greater severity in terms of mean RA pressure (9.8±5.9 vs 8.5±5.7 mmHg, p = 0.356), CO (3.8±1.2 vs 4.2±1, 4 lpm, P = 0.178) and PVR (17.2±9.4 vs 13.5±5.6 WU, p = 0.249). BMPR2 mutations in this population were not found vs 8 (15.4%) in iPAH, p= 0,048. A micromorphometric study of pulmonary vessels, in 4 TOS patients, showed that there was a marked venular component with intimal fibrosis, presence of double elastic membrane and lumen obliteration, in addition to plexiform arteriopathy (Fig 1). ![Figure][1] Figure 1. Study of pulmonary venules Conclusions: A negative AVT in patients with PAH associated with TOS may be due to more severe hemodynamic disease with additional venules involvement. Additional studies are needed to correlate the absence of BMPR2 mutation and lack of vasoreactivity. [1]: pending:yes

Cardiac magnetic resonance in pulmonary arterial hypertension: a step in the right direction

Pulmonary arterial hypertension (PAH) is clinically a highly malignant disease. Although pathological changes are limited to the vascular wall, uncontrolled proliferation is mostly directed towards vascular lumen. Pulmonary input impedance usually increases at a rate which cannot be effectively matched by the right ventricular (RV) adaptive mechanisms. A series of consequences including RV dilatation, functional tricuspid regurgitation, decreased pulmonary flow, compromised LV filling, and low systemic cardiac output contribute to a fatal vicious circle. The expected median survival of 2.8 years in conventionally treated idiopathic PAH is indeed similar to that of advanced lung or breast cancer.1 In the last decade, important progress has been made in the understanding of the physiopathology and therapy of this disease. Several drugs interfering with important physiopathological pathways of PAH progression were approved, based on the results of right heart catheterization (RCT).2 None of those therapies permit a cure. Nevertheless, in many patients, it is now possible to slow, stop, and sometimes partly revert the otherwise progressive pulmonary vascular obliteration. This gives the RV chance to adapt to increased afterload. Sustained clinical improvement can sometimes be achieved already with a first-line oral drug. However, the individual long-term response to any form of medical therapy may vary and cannot be predicted. Monitoring of each patient, whether treated with oral calcium channel blockers (CCB), endothelin antagonists, PDE5i, or parenteral prostacycline analogues, is therefore very important. Wasting time for less effective therapy might result in irreversible progression of pulmonary vascular lesions with all clinical consequences. Current therapeutic algorithm suggests haemodynamic test for pulmonary vasoreactivity and assessment of the WHO functional class (FC) as sufficient for selecting initial therapy. Decisions whether and when to change the oral drug, switch to combination and/or parenteral therapy, perform interventions such as atrial septostomy or list for lung transplantation require … Corresponding author. Tel: +48 22 4312114; fax: +48 22 4312414. E-mail address : a.torbicki{at}igichp.edu.pl

Anomalies pulmonaires au cours des maladies hépatiques

Respiratory manifestations are common in cirrhotic patients, with dyspnea as the most frequent. In most cases, these manifestations are related to non specific disorders including bacterial infections or lung base atelectasia in case of large ascites. Less frequently, cirrhotic patients develop specific respiratory disorders: hepatic hydrothorax, hepatopulmonary syndrome and portopulmonary hypertension. Hepatic hydrothorax results from the passage of ascites into the pleural cavity. It can be observed even in the absence of large ascites. Hepatic hydrothorax may be responsible for dyspnea and hypoxemia. Despite diuretics, pleural effusion can restore rapidly after thoracentesis. Iterative thoracentesis should be avoided because of a high incidence of complications. Liver transplantation represents the best therapeutic option. TIPS placement or chemical pleurodesis are acceptable alternative therapies when liver transplantation is not possible, but their efficacy is inconstant. Hepatopulmonary syndrome is characterized by an intrapulmonary shunt resulting from massive microvascular dilatation. It occurs in about 5 % of cirrhotic patients and the main manifestation is hypoxemia. Hepatopulmonary syndrome significantly reduces life expectancy in cirrhotic patients. Oxygen therapy can improve manifestations but liver transplantation remains the only proven therapy based upon the total resolution of hypoxemia in almost all patients. Portopulmonary hypertension results from pulmonary arterial vasoconstriction and concentric intimal fibrosis leading to precapillary pulmonary hypertension. It occurs to 1 to 2% of cirrhotic patients and remains latent until mean pulmonary artery pressure exceeds 40 mmHg. Hypoxemia is absent or moderate. In the absence of any specific treatment, the prognosis is poor, and death is due in most cases to cardiac arrest or to end-stage cardiac insufficiency. Echocardiography is helpful for detecting portopulmonary hypertension but definitive diagnosis should be based on the results of right heart catheterization. Prostacyclin analogues may sometimes decrease durably pulmonary artery pressure. Liver transplantation can be performed when pulmonary artery pressure is only mildly elevated but post transplantation resolution seems inconstant. Severe portopulmonary hypertension represents a contraindication to transplantation because of a high rate of perioperative mortality.

Echocardiographic characterization of the improvement in right ventricular function in patients with severe pulmonary hypertension after single-lung transplantation

Objectives. This study was designed to characterize immediate, early and long-term changes in right ventricular structure and function, as defined by two-dimensional and Doppler echocardiography, after single-lung transplantation in patients with severe pulmonary hypertension.Bacground. Single-lung transplantation has recently been shown to dramatically improve hemodynamics in patients with primary pulmonary hypertension who had unsuccessful medical therapy.Method. Fourteen patients with severe pulmonary hypertension who underwent single-lung transplantation were studied with transthoracic and transesophageal two-dimensional and Doppler echocardiography. Right ventricular dimensions were measured in the apical four-chamber view. Right ventricular ejection and acceleration times and peak velocity of tricuspid regurgitation were measured by Doppler study. Results of right heart catheterization were available early (<3 months) after transplantation in 10 of 13 patients and late after transplantation (6 month to 2 years) in 11 patients.Results. In the early posttransplantation studies, right ventricular dimensions decreased and fractional area change and ejection fraction increased in all patients, but right ventricular wall thickness did not change significantly. Tricuspid regurgitation lessened markedly in all patients. Long-term decreases in right ventricular dimension and improvement in systolic function were sustained. Right ventricular wall thickness significantly decreased compared with the early postoperative value (0.76 ± 0.1 cm compared with 0.63 ± 0.14 cm, p < 0.02).Conclusions. Two-dimensional echocardiography demonstrates sustained improvement in right ventricular function after single-lung transplantation for severe pulmonary hypertension despite severe preoperative dysfunction.

MR Velocity Measurement in the Pulmonary Arteries: A Comparative Study

The quantitative estimation of pulmonary hemodynamics is of clinical interest in various benign and malignant diseases of the lungs, especially in cases of preoperative planning of parenchymal resections and/or pulmonary arterial hypertension. In a comparative clinical study with 30 patients MR velocity measurements in the pulmonary arteries (main, right and left) were performed by the RACE-technique at 1.5 T. MR data were correlated (a) with the results of perfusion scintigraphy, (b) with pw-US doppler echo-cardiography, and (c) with right heart catheterization. MR-RACE showed good results for quantification of the relative right to left pulmonary arterial flow but was not able to indicate the correct absolute flow related to the results of right heart catheterization. Most probably this is due to interferences by overlapping vessels which are unavoidable in a projection technique. Better correlation with right heart catheterization can be expected using a two dimensional MR technique (e.g. „PHASE-MAPPING” or „PHASE-CONTRAST”) in patients without cardiac arrhythmias.