Estimation Of Pulmonary Pressure Research Articles

Echocardiographic estimation of pulmonary pressure in patients with severe tricuspid regurgitation

ObjectivesThe estimation of systolic pulmonary artery pressure (sPAP) by transthoracic echocardiography (TTE) is challenging in patients with severe tricuspid regurgitation (TR). The study aimed to determine the reliability of the...

Genetics of Pulmonary Pressure and Right Ventricle Stress Identify Diabetes as a Causal Risk Factor.

Background Epidemiologic studies have identified risk factors associated with pulmonary hypertension and right heart failure, but causative drivers of pulmonary hypertension and right heart adaptation are not well known. We sought to leverage unbiased genetic approaches to determine clinical conditions that share genetic architecture with pulmonary pressure and right ventricular dysfunction. Methods and Results We leveraged Vanderbilt University's deidentified electronic health records and DNA biobank to identify 14 861 subjects of European ancestry who underwent at least 1 echocardiogram with available estimates of pulmonary pressure and right ventricular function. Analyses of the study were performed between 2020 and 2022. The final analytical sample included 14 861 participants (mean [SD] age, 63 [15] years and mean [SD] body mass index, 29 [7] kg/m2). An unbiased phenome-wide association study identified diabetes as the most statistically significant clinical International Classifications of Diseases, Ninth Revision (ICD-9) code associated with polygenic risk for increased pulmonary pressure. We validated this finding further by finding significant associations between genetic risk for diabetes and a related condition, obesity, with pulmonary pressure estimate. We then used 2-sample univariable Mendelian randomization and multivariable Mendelian randomization to show that diabetes, but not obesity, was independently associated with genetic risk for increased pulmonary pressure and decreased right ventricle load stress. Conclusions Our findings show that genetic risk for diabetes is the only significant independent causative driver of genetic risk for increased pulmonary pressure and decreased right ventricle load stress. These findings suggest that therapies targeting genetic risk for diabetes may also potentially be beneficial in treating pulmonary hypertension and right heart dysfunction.

P391 THE DIAGNOSTIC CHALLENGE BETWEEN CHRONIC PULMONARY EMBOLISM AND PULMONARY ARTERY SARCOMA: A CASE REPORT

Abstract We report the case of a 63–year–old obese and hypertensive woman, admitted in hospital with worsening dyspnea for 2 weeks and syncope. A suspected chronic thromboembolism was diagnosed nine months before for a similar clinical picture; hematologic screening excluded thrombophilia and NOACs were prescribed. Two–dimensional transthoracic echocardiography showed enlargement of right chambers, the interventricular septum shifted to the left ventricle, showing “D” sign. Color Doppler flow imaging showed moderate regurgitation in tricuspid valve, with indirect pulmonary pressure estimation 90 mmHg. Left ventricle was normal for dimensions and function. ECG evidenced sinus tachycardia and no specific ST–T alterations. Blood exams evidenced anemia (Hb 9 mg/dl), negative neoplastic markers and absence of occult blood in feces, mildly elevated inflammatory markers, T–Troponin and D–dimer. Naso–pharyngeal swab for SARSCoV2 infection was negative. Chest computed tomography with contrast revealed a large filling defect within bilateral main pulmonary arteries. No lymph nodes or pulmonary pneumonia were detected. Lower extremity venous ultrasound was positive for right popliteal deep vein thrombosis. The global clinical picture suggested an acute pulmonary embolism recurrence, with a severe right ventricle impairment and a concomitant deep vein thrombosis. Some factors were not completely clear: chronic anemia, only mildly increased D–dimer, no specific increase of inflammatory markers. The patient was referred to Cardiac Surgery Unit of IRCCS Foundation Policlinico San Matteo in Pavia, for pulmonary endarterectomy. Unexpectedly the surgical finding was a bilateral pulmonary artery sarcoma, confirmed by histological exam. We thus clarified some uncertain clinical aspects, explainable in the context of a severe neoplastic picture. There were no immediate complications, and a chemotherapy was initiated after a period of cardio–pulmonary rehabilitation. Pulmonary artery intimal sarcoma is a very rare disease, its prevalence is about 0.001–0.003%, it can originate from the left and right pulmonary arteries and intimal layer of pulmonary arteries, forming a tumor growing in the nodular cavity or spreading along the intimal surface. PAS is often misdiagnosed as acute or chronic pulmonary thromboembolism due to its clinical presentation and radiological findings. Thus, early diagnosis is very crucial and may improve patient outcome.

Pulmonary Vascular Remodeling and Prognosis in Patients Evaluated for Heart Transplantation: Insights from the OCTOPUS-CHF Study.

In patients with advanced heart failure, the intravascular optical coherence tomography (OCT) of subsegmental pulmonary artery measurements is correlated with right heart catheterization parameters. Our aim was to study the prognostic value of pulmonary OCT, right heart catheterization data, and the echocardiographic estimation of pulmonary pressure in patients studied for elective heart transplants. This research is an observational, prospective, multicenter study involving 90 adults with a one-year follow-up. A total of 10 patients (11.1%) died due to worsening heart failure before heart transplantation, 50 underwent a heart transplant (55.6%), and 9 died in the first year after the transplant. The patients with and without events (mortality or heart failure-induced hospitalization) had similar data regarding echocardiography, right heart catheterization, and pulmonary OCT (with a median estimated pulmonary artery systolic pressure of 42.0 mmHg, interquartile range (IQR) of 30.3-50.0 vs. 47.0 mmHg, IQR 34.6-59.5 and p = 0.79, median pulmonary vascular resistance of 2.2 Wood units, IQR 1.3-3.7 vs. 2.0 Wood units, IQR 1.4-3.2 and p = 0.99, and a median pulmonary artery wall thickness of 0.2 ± 0.5 mm vs. 0.2 ± 0.6 mm and p = 0.87). Pulmonary vascular remodeling (evaluated with echocardiography, right heart catheterization, and pulmonary OCT) was not associated with prognosis in a selected sample of adults evaluated for elective heart transplants. Pulmonary OCT is safe and feasible for the evaluation of these patients.

Cardiac Dysfunction in Neonatal HIE Is Associated with Increased Mortality and Brain Injury by MRI.

Describe the association between cardiac dysfunction and death or moderate-to-severe abnormalities on brain magnetic resonance imaging (MRI) in neonates undergoing therapeutic hypothermia for hypoxic ischemic encephalopathy (HIE). Retrospective study in neonates with moderate or severe HIE undergoing therapeutic hypothermia between 2008 and 2017. Primary outcome was death or moderate-to-severe brain injury using the Barkovich score. Conventional and speckle-tracking echocardiography measures were extracted from available echocardiograms to quantify right (RV) and left (LV) ventricular functions. A total of 166 newborns underwent therapeutic hypothermia of which 53 (36.5%) had echocardiography performed. Ten (19%) died prior to hospital discharge, and 11 (26%) had moderate-to-severe brain injury. There was no difference in chronologic age at echocardiography between the normal and adverse outcome groups (22 [±19] vs. 28 [±21] hours, p = 0.35). Cardiac findings in newborns with abnormal outcome included lower systolic and diastolic blood pressure (BP) at echocardiography (p = 0.004) and decreased tricuspid annular plane systolic excursion (a marker of RV systolic function; p = 0.01), while the ratio of systolic pulmonary artery (PA) pressure to systolic BP indicated isosystemic pressures (>2/3 systemic) in both groups. A multilogistic regression analysis, adjusting for weight and seizure status, indicated an association between abnormal outcome and LV function by longitudinal strain, as well as by ejection fraction. Newborns who died or had moderate-to-severe brain injury had a higher incidence of cardiac dysfunction but similar PA pressures when compared with those who survived with mild or no MRI abnormalities. · Newborns with HIE with functional LV/RV dysfunction are at risk for death or brain injury.. · All neonates with HIE had elevated pulmonary pressure, but neonates with poor outcome had RV dysfunction.. · When evaluating newborns with HIE by echocardiography, beyond estimation of pulmonary pressure, it is important to assess biventricular function..

Transthoracic echocardiogram by protocol apical-subcostal in prone position during invasive mechanical ventilation

Funding AcknowledgementsType of funding sources: None.IntroductionDuring COVID-19 pandemic reports increased of transthoracic echocardiography (TTE) in prone and invasive mechanical ventilation (IMV), mostly describing four and five chamber apical views, and inferior vena cava (IVC) by lateral IVC window (from right side of the patient).PurposeEvaluate quality of images obtained with protocol of apical-subcostal TTE in patients with IMV and prone position.MethodsProspective study, between August and December 2020, in adults who required prone position during IMV. After placing the patient in the prone position, left arm was extended overhead, and a pillow was placed only under the left hemithorax to elevate and facilitate the apical window and space below the patient for subcostal window. Operator stands on the left side of the patient and takes images with the transducer in your right hand, starting with apical window and subsequently subcostal window. Apical views were apical four, two and three-chambers, to obtain function parameter of right and left ventricle (LV), evaluation of aortic valve, mitral and tricuspid valve. Subcostal views were four cardiac chambers and IVC, to obtain qualitative ventricle function, presence of pericardial effusion, and volume status and estimation of pulmonary pressure (together to peak systolic tricuspid pressure gradient from apical views). The images were acquired by cardiologist, then were saved, and finally evaluated by two echocardiography cardiologist experts.Results16 ETTs were performed. Male gender and obesity predominate. Positive end-expiratory pressure average was 10.8 cm of water. One patient cannot be assessed by absence of acoustic window and the rest (n = 15) were analyzed with experts. It was achieved a four-chamber apical view in 100% (n = 15), a two-chambers apical view in 60% (n = 9) and a three-chambers apical view in 100% (n = 15). It was possible to assess global function of the LV in 100% (n = 15), LV segmental function in 53% (n = 8), LV outflow tract velocity time integral in 100% (n = 15) and tricuspid annular plane systolic excursion in 100% (n = 15). Pulsed wave doppler of mitral valve in 100% (n = 15) and tissue doppler of lateral mitral valve annulus 100% (n = 15). Continuous wave doppler of aortic valve in 100% (n = 15) and tricuspid valve in 93% (n = 14). Subcostal four-chamber 80% (n = 12), presence pericardial effusion 100% (n = 15) and IVC 93% (n = 14). Non-complications associated with obtaining the position.ConclusionsETT in the prone position during IMV was possible and interpretable images were achieved. The position described allows assessment by apical and subcostal views at the same time and position of the operator and the patient. In addition, it was a safe technique, and the position was easy to be incorporated by the health team. Limitations were obtaining the two-chamber apical view and evaluation of segmental alterations of the LV. Better validation requires a larger sample.

Correlation between Doppler echocardiography and right heart catheterization assessment of systolic pulmonary artery pressure in patients with severe aortic stenosis

The aim of this study was to investigate reliability and accuracy of noninvasive measurement method by echocardiography compared to invasive measurement of systolic pulmonary artery pressure (SPAP) in a large cohort of aortic stenosis (AS) patients. Pulmonary hypertension (PH) is common in patients with cardiac disease, especially in left heart disease like severe AS. Invasive measurement by right heart catheterization (RHC) is the gold standard to assess pulmonary pressures. Nevertheless, echocardiography is widely used in everyday practice for estimation of pulmonary pressures and diagnosing PH. A total of 1400 patients with AS and full invasive hemodynamic assessment by RHC and noninvasive measurements by Doppler echocardiography were included. Mean patient age was 81.5±6.8years, and 46.3% were males. SPAP was 44.7±15.1mmHg by echocardiography and 45.3±15.2mmHg by RHC. Pearson's correlation coefficient was r=.820 (P<.0001). Bland-Altman analysis showed a bias of -0.56mmHg (95% limits of agreement -18.38 to+17.26mmHg) and 80.6% measurement accuracy. Pulmonary hypertension defined by RHC as a mean PAP ≥25mmHg was reliably diagnosed via an echocardiographically measured SPAP of >40mmHg (82.2% sensitivity, 80.2% specificity, 83.1% positive predictive value, 79.2% negative predictive value). In a large cohort of patients with severe aortic stenosis, we could demonstrate a very good correlation of SPAP between Doppler echocardiography and invasive RHC measurement. Pulmonary hypertension could be diagnosed by echocardiography with high sensitivity and specificity.

For a better estimation of pulmonary pressures by echocardiography: Need for quality improvement

Echocardiography provides controversial accuracy in estimations of systolic pulmonary pressures (sPAP). The objective of this study was to determine the factor influencing the accuracy using echocardiography in patients with history of heart failure with preserved ejection fraction or pulmonary arterial hypertension. Between January 2014 and January 2018, 184 patients with suspected pulmonary hypertension, underwent right heart catheterization (RHC) and transthoracic echocardiography within 5 days in our referral centre. Right ventricular systolic pressure was estimated according to tricuspid regurgitation (TR) signal and compared to hemodynamic sPAP. Twelve parameters were studied to determine the reasons of lack of accuracy in the echocardiographic sPAP result, namely: the cause of the pulmonary hypertension, the heart rate, the presence of atrial fibrillation, the right atrial size, the operator's experience (young or experienced), the severity of the TR, the TR signal quality, the number of incidences recorded of TR images, the inferior vena cava size, the difference between echo and hemodynamic right atrial pressure measurement, the cardiac index and the mean PAP. A difference of 10 mmHg between echo and hemodynamic sPAP measurement was considered as significant. Seventy-nine patients (50%) had a very good TR signal quality. Twenty-seven patients (15%) had no signal or very poor TR signal quality. Seventy-eight patients (50%) had a difference > 10 mmHg between echo and Hemodynamic sPAP estimation. The parameters associated with a difference of 10 mmHg or more between both measurements were a higher mean PAP (43 ± 10 vs. 39 ± 8 mmHg; P = 0.001), the experience of the operator ( P = 0.008), and the number of acquisition of the TR signal ≥ 3 ( P = 0.017). Echocardiography reliably estimates sPAP when attention is given to quality metrics, the experience of the reader, and the mean PAP.

Estimación de la presión arterial pulmonar mediante ecocardiografía

Los avances tecnologicos en ecocardiografia durante los ultimos 30 anos han aumentado la precision en la evaluacion canatomica y funcional de las cavidades cardiacas derechas, asi como en la estimacion de la presion arterial pulmonar y en la evaluacion hemodinamica pulmonar. Las conclusiones derivadas de un informe ecocardiografico deben sugerir la probabilidad de hipertension pulmonar (HTP) en funcion de los elementos que permiten estimar la presion sistolica, diastolica o media en la arteria pulmonar mas la presencia de elementos indirectos de HTP. Analizamos la evidencia que sustenta la utilidad de los diferentes metodos ecocardiograficos para la estimacion de la presion pulmonar. Es de suma importancia que el cardiologo tenga presente los diferentes metodos –directos e indirectos– que permiten evaluar la HTP.

FLOW-CORRECTED ESTIMATES OF PULMONARY PRESSURE FOR THE ASSESSMENT OF DIASTOLIC DYSFUNCTION IN PATIENTS WITH UNEXPLAINED DYSPNEA

Objective: An echocardiographic diastolic stress test (DST) may be useful in subjects with unexplained exertional breathlessness. The E/e′ ratio and tricuspid regurgitant jet velocity (TRV) are used to differentiate normal vs abnormal responses. However, absence of flow-corrected estimates of pulmonary pressure may reduce the diagnostic performance of the test. We sought to compare flow-corrected and flow-uncorrected estimates of pulmonary pressure during exercise in consecutive patients undergoing a DST, using as gold-standard reference exercise right heart catheterization (RHC). Design and method: Retrospective analysis of patients undergoing a DST. Echo estimates of mean pulmonary artery pressure (PAP) and cardiac output (Q) were used to generate PAP/Q relationship during exercise. The slope of the latter and the ratio between mean PAP and Q at peak exercise (TPR) were used as flow-corrected estimates of abnormal response of the pulmonary circulation. In a subset of patients, P/Q slope and TPR obtained at RHC were compared with echocardiographic results. Results: Out of 38 patients with tricuspid regurgitation during exercise, all had TRV > 2.8 m/s so that none could be classified to have normal diastolic function at exercise; 92% had “indeterminate” diastolic function. When a flow-corrected estimate of PAP was used, the DST could be defined “normal” in 6 cases (17%, with both TPR < 3 and P/Q slope <3). P/Q slope and TPR were both >3 in 18 subjects, nine of whom underwent also exercise RHC, allowing confirmation of HFpEF in 78% of them. When comparing the results of the 15 patients undergoing both DST and exercise RHC, we found a correlation between invasive measurements and non-invasive estimate of P/Q slope (R = 0.70) and TPR (R = 0.66). Echocardiographic estimates of P/Q slope and TPR were accurate as compared with RHC measurements, but showed large confidence intervals (figure 1).Conclusions: Our preliminary data suggest that flow-corrected estimates of pulmonary pressure might provide additional pathophysiological information during a noninvasive DST, but suffer of large limits of agreement as compared with RHC.

Diminished right ventricular function at diagnosis of pulmonary hypertension is associated with mortality in bronchopulmonary dysplasia

Pulmonary vascular disease and resultant pulmonary hypertension (PH) have been increasingly recognized in the preterm population, particularly among patients with bronchopulmonary dysplasia (BPD). Limited data exist on the impact of PH severity and right ventricular (RV) dysfunction at PH diagnosis on outcome. The purpose of this study was to evaluate if echocardiography measures of cardiac dysfunction and PH severity in BPD-PH were associated with mortality. The study is a retrospective analysis of the echocardiography at three months or less from time of PH diagnosis. Survival analysis using a univariate Cox proportional hazard model is presented and expressed using hazard ratios (HR). We included 52 patients with BPD and PH of which 16 (31%) died at follow-up. Average gestational age at birth was 26.3 ± 2.3 weeks. Echocardiography was performed at a median of 43.3 weeks (IQR: 39.0–54.7). The median time between PH diagnosis and death was 117 days (range: 49–262 days). Multiple measures of PH severity and RV performance were associated with mortality (sPAP/sBP: HR 1.02, eccentricity index: HR 2.02, tricuspid annular plane systolic excursion Z-score: HR 0.65, fractional area change: HR 0.88, peak longitudinal strain: HR 1.22). Hence, PH severity and underlying RV dysfunction at PH diagnosis were associated with mortality in BPD-PH patients. While absolute estimation of pulmonary pressures is not feasible in every screening echocardiography, thorough evaluation of RV function and other markers of PH may allow to discriminate the most at-risk population and should be considered as standard add-ons to the current screening at 36 weeks.

What influences echocardiography reliability in estimation of pulmonary pressure?

If echocardiography provides reliable estimations of pulmonary pressures remain controversial. The objective of this study was to determine the accuracy of estimating right ventricular systolic pressure (RVSP) and the factor influencing this accuracy using echocardiography in patients with history of heart failure with preserved ejection fraction (HFpEF) or pulmonary arterial hypertension. Between January 2014 and December 2015, 118 patients with suspected pulmonary hypertension (PH), underwent right heart catheterization (RHC) and transthoracic echocardiography within 5 days in our referral center. The correlation in estimating RVSP according to tricuspid regurgitation (TR) signal quality and reader expertise were retrospectively determined. TR signal quality using continuous-wave Doppler were classified subjectively in three groups: good (type 1), moderate (type 2) and poor (type 3). We compared measurements from the echocardiography laboratory clinical report assessed by level 3–trained cardiologists certified in echocardiography, with offline reassessment by a level 1–young cardiologist blinded to RHC values. In total, 86 patients (72%) had a good or moderate TR signal quality. The comparison between clinical reports and young cardiologist reassessment gave a linear regression line having the equation y = −0, 1549 + 1, 0286 x. Correlation coefficient between RVSP estimation from RHC and echocardiography were respectively 0,69 (P < 0,001) from the clinical report and 0,62 (P < 0,001) from level 1 reader. When TR signal quality was good and moderate, correlation coefficient was respectively 0,75 (P < 0,001) from the clinical report and 0,72 (P < 0,001) from the level 1 reader. In 50 patients with type 1 TR quality signal, the correlation coefficient were respectively 0,83 (P < 0,001) and 0,78 (P < 001). Echocardiography reliably estimates RVSP when attention is given to quality metrics and the level of the reader.

Inaccuracy of Right Atrial Pressure Estimates Through Inferior Vena Cava Indices

The precision of echocardiography in estimating pulmonary pressures has been debated. A value of right atrial pressure (RAP) is needed for pulmonary pressure estimation, and it could be partly responsible for the estimation error. Several schemes based on the inferior vena cava (IVC) are commonly used in clinical practice and in experimental studies for RAP estimation. However, the majority lack proper validation, and thus far, no study has compared them all. In this prospective, blinded study, a comprehensive transthoracic echocardiography was performed on 200 patients referred for right heart catheterization. The IVC was measured in different views and RAP was estimated according to 6 different schemes. One hundred ninety patients were suitable for analysis. IVC measurements were significantly but poorly associated with invasive RAP. All RAP schemes showed poor accuracy compared with invasive RAP (average accuracy 34%). None of the schemes showed a clear superiority over the others. No echocardiographic or clinical variables showed a relevant impact on the estimation error. In conclusion, RAP estimation based on the IVC is highly inaccurate irrespective of the method used and should be avoided whenever possible. Whether adding estimated RAP values affects the estimation of pulmonary pressures is yet to be determined.

Contribution of Speckle Tracking to Estimation of Pulmonary Hypertension by Standard Doppler Echocardiography in Patients with Systemic Sclerosis and MCTD

Objectives: Development of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) represents one of the most serious complications. The study aimed at assessing right ventricular (RV) global longitudinal strain and its relation to pulmonary artery pressure. Methods: Echocardiography was performed in 74 patients of whom 60 had SSc and 14 had mixed connective tissue disease (MCTD). Besides routine left and right ventricle assessment and pulmonary pressure estimation by Doppler echocardiography, RV fractional area change (FAC) and 2D strain of the RV free wall were evaluated. At the same time, levels of N-terminal pro-brain natriuretic peptide (NT-proBNP), troponin T and auto-antibodies were measured and all patients had diffusing capacity of the lung for carbon monoxide (DLCO) determined. All patients with probable PAH underwent right heart catheterization. Results: Pulmonary hypertension was found to be unlikely in 59 patients (79.7%), possible in 10 patients (13.5%) and probable in 5 patients (6.7%). Significant associations were found between pulmonary artery systolic pressure (PASP) and RV free wall global longitudinal strain (r=0.292; p=0.023), between PASP and NT-proBNP (r=0.436; p=0.001) and between PASP and FAC (r=0.320; p=0.005). Pulmonary artery systolic pressure did not correlate with left ventricular systolic or diastolic function parameters in this cohort. Conclusion: Speckle tracking of the RV represents a useful additional tool in RV assessment in relation to PH in SSc and MCTD patients; right heart catheterization remains an essential method for PAH confirmation.

Echocardiography and Pulmonary Arterial Hypertension

Pulmonary Arterial Hypertension (PAH) is an heterogeneous condition brought on by a wide range of causes. It is characterized by structural changes in small pulmonary arteries, that produce a progressive increase in pulmonary artery pressure and pulmonary vascular resistance, ultimately leading to right ventricle failure and death. Given the non-specific nature of its early symptoms and signs, PAH is often diagnosed in its advanced stages. Along with a careful clinical assessment and an accurate electrocardiogram/Chest X-ray interpretation, echocardiography is an essential test in the evaluation of patient with PAH. In fact it not only provides an accurate estimate of pulmonary pressure at rest and during exercise, but may also help to exclude any secondary causes, predict the prognosis, monitor the efficacy of specific therapeutic interventions and detect the preclinical stage of the disease.

The utility of transoesophageal echocardiography for estimating right ventricular systolic pressure.

With the reduction in use of the pulmonary artery catheter, alternative methods of pulmonary pressure estimation are required. The use of echocardiographically-derived right ventricular systolic pressure has recently been questioned, but this technique has not been validated in anaesthetised surgical patients with transoesophageal echocardiography. One hundred measurements of right ventricular systolic pressure with transoesophageal echocardiography were compared with the pulmonary artery systolic pressure obtained simultaneously from a pulmonary artery catheter in patients undergoing cardiac surgery. Simultaneous right ventricular systolic pressure and pulmonary artery systolic pressure measurements were possible in all patients, and these measurements were strongly correlated (r = 0.98, p<0.001), with minimal bias and narrow limits of agreement (approximately -5 to +5mmHg), across a broad range of pulmonary pressures. Measurement of right ventricular systolic pressure using tranoesophageal echocardiography is readily achievable and closely correlates with pulmonary artery systolic pressure, with minimal bias, in cardiac surgical patients undergoing general anaesthesia and positive pressure mechanical ventilation of the lungs.

Estimation of Pulmonary Pressures and Diagnosis of Pulmonary Hypertension by Doppler Echocardiography: A Retrospective Comparison of Routine Echocardiography and Invasive Hemodynamics

To date, Doppler echocardiography is the most widespread and well-recognized technique for the noninvasive evaluation of systolic pulmonary artery pressure (sPAP). However, recent studies have reported reservations about the relevance of Doppler echocardiography or the tool's reliability in the diagnosis and follow-up of patients with pulmonary hypertension (PH). Thus, the aim of this dedicated retrospective study was to address the questions of Doppler echocardiography's relevance and accuracy for PH diagnosis in the routine activity of a conventional echocardiography department. Institutional databases were used to extract and analyze the records of 310 patients who underwent both hemodynamic and echocardiographic investigations within a single hospitalization period. Despite an underestimation of absolute Doppler sPAP values compared with measurements on right heart catheterization, data analysis revealed a strong correlation (r= 0.80, P < .00001, n= 310). Targeting a mean pulmonary pressure on right heart catheterization of 25 mm Hg for the definition of PH, receiver operating characteristic curve analysis demonstrated a strong association between sPAP and PH diagnosis (area under the curve, 0.82; n= 155). The cutoff obtained for sPAP was 38 mm Hg, and when applied on a second-test subgroup population (n= 155), sensitivity, specificity, and accuracy were 88%, 83%, and 86%, respectively. When patients with examination intervals of <2 days were selected (n= 115), sensitivity and specificity reached 89% and 89%, respectively. No combination of parameters produced an improvement on the initial results. In the real-world practice of a conventional echocardiography department, Doppler echocardiography is associated with high accuracy, sensitivity, and specificity for PH evaluation, thus confirming its major position as a primary noninvasive tool.

Late diagnosis of incomplete Cantrell's syndrome on CT scan

MOTS CLES Syndrome de Cantrell ; Cardiopathie congenitale ; Scanner cardiaque A 45-year-old man with chronic myeloid leukaemia was referred for cardiac evaluation before allogeneic bone-marrow transplantation. His medical history included isolated right ventricle (RV) dilatation discovered 10 years earlier, with episodes of right heart failure induced by severe postchemotherapy anaemia. The electrocardiogram showed first-degree atrioventricular block, complete right bundle branch block and left atrial enlargement (Fig. 1). Chest radiographs showed cardiomegaly and large bowel images in the area anterior to the heart (Fig. 2). Transthoracic echocardiography showed isolated RV enlargement (53mm) with normal ejection fraction. Left ventricle (LV) dimension and ejection fraction were normal. The RV/LV diameter ratio was 1.2. No atrial or ventricular septal defects or pericardial abnormality was seen. Systolic pulmonary pressure estimation was 45mmHg. Pulmonary-to-systemic blood flow ratio (Qp/Qs) was 2.1 with no visible left-to-right shunt. Right heart catheterization showed normal pulmonary artery occlusion pressure (5mmHg), normal mean pulmonary pressure (18mmHg) and no sign of restrictive cardiomyopathy. Oximetric study demonstrated an oxygen saturation of 66% in the superior and inferior vena cava, 84% in the right atrium, 80% in the RV and 77% in the pulmonary artery. Qp/Qs was 1.6. A computed tomography scan showed the association of transdiaphragmatic hernia, partial apical agenesis of the pericardium, and sinus venosus atrial septal defect with abnormal pulmonary venous return (Fig. 3).

Comparison Between Two Drugs on the Hemodynamic Evaluation of Pulmonary Hypertension Prior to Heart Transplantation

Introduction Invasive estimation of pulmonary pressure is part of the usual protocol prior to heart transplantation. The aim of this study was to compare the results of 2 different vasodilators, nitric oxide (NO) and prostacyclin, in an acute vasodilator test (AVT) for patients with pulmonary venous hypertension. Materials and Methods From January 2000 to December 2006, 94 right-sided heart catheterizations were performed in our center within pretransplantation evaluations. AVT was performed if the mean pulmonary artery pressure (mPAP) >35 mm Hg or if the pulmonary vascular resistance (PVR) was >4 Wood units (WU). Epoprostenol was administered to 40 patients, NO to 6 patients, and both agents to 8 patients. Results A significant decrease in both mPAP and PVR was shown with maximum doses of epoprostenol, with an average variation of 8.96 mm Hg in mPAP ( P < .001) and 3.26 WU in PVR ( P < .001). An increased cardiac output (CO) was observed with epoprostenol, with a mean difference of 1.9 L/min ( P < .001) at maximum compared with baseline doses. A tendency for the mPAP and PVR to decrease was also observed with maximum NO doses, with mean decreases of mPAP and PVR of 5.62 mm Hg and 1.14 WU, respectively. A tendency for CO to decrease was observed with NO (0.75 L/min; P = .039). Conclusions In our experience, NO is the best drug for AVT due to its pulmonary tree selectivity. A study with epoprostenol was complementary; both drugs can be used in these patients prior to heart transplantation.

Exercise echocardiography in pulmonary hypertension

Doppler echocardiography plays a key role in the detection of pulmonary hypertension, the leading cause of right ventricular (RV) dysfunction. Reliable estimates of pulmonary pressures and cardiac output (CO) can be obtained at rest, allowing for screening and diagnosis of pulmonary arterial hypertension (PAH), a group of diseases characterized by increased pulmonary vascular resistance progressing to RV failure and death. However, these resting indices poorly correlate with symptoms, exercise response, and have modest prognosis significance. This is partially explained by the limited information obtained from pressure measurements on the severity of RV dysfunction, the major determinant of exercise response and prognosis in PAH. Exercise echocardiography (Ex-echo) may provide significant additional information in the understanding and the management of the disease. In asymptomatic subjects at risk of PAH, it may uncover abnormal increases in pulmonary pressure and CO during exercise, which may be reflecting early stage disease. In addition, the technique may be useful to better understand the physiology of exercise limitation, a typical manifestation of PAH. Several limitations need to be considered when using Ex-echo in the management of PAH, including the absence of a clear consistent definition of normal values, an unknown threshold for abnormal response and the limited quantitative information on RV function provided by simple pressure analysis. Newer echocardiographic techniques such as Tissue-Doppler imaging, and strain and strain rate analysis may provide further insights in the understanding of RV dysfunction. However, technical limitations currently prevent their application to Ex-echo. Clinical studies are underway to better explore the potential role of Ex-echo in the management of PAH.