Is Right Ventricular Remodeling in Pulmonary Hypertension Dependent on Etiology? An Echocardiographic Study.

AIMS: Survival in patients (pts) with pulmonary hypertension (PH) differs between subgroups at similar levels of pressure overload. We set to analyze right ventricular (RV) morphology and function in different types of PH using conventional and deformation imaging echocardiography. MATERIAL AND METHODS: Thirty-four pts with PH: 12 pts with idiopathic pulmonary arterial hypertension (IPAH, 42.2 ± 13 years), 11 pts with chronic thromboembolic PH (CTEPH, 50.8 ± 12 years), 11 pts with Eisenmenger syndrome [ES 41.2 ± 15 years, 4 with atrial septal defect (ASD) and 7 with ventricular septal defect (VSD)], and 13 age-matched healthy individuals (38.1 ± 15 years) were evaluated. The following echocardiographic parameters were measured: echo-derived systolic pulmonary pressure (sPAPecho), RV end-diastolic diameter (RVEDD), RV wall thickness (RVWT), TAPSE, RV fractional area change (RVFAC), Tei index, peak systolic velocity of the tricuspid ring (S't), and speckle tracking-derived RV free wall strain. Furthermore, right heart catheterization (RHC) was performed in pts with PH and mean, and systolic pulmonary artery pressure (mPAPcath, sPAPcath), cardiac output (CO), cardiac index (COi), and pulmonary vascular resistance (PVR) were noted. RESULTS: The levels of mPAPcath and sPAPcath were similar between pts with PH (pANOVA = NS). Patients with ES had higher COi compared to other groups (2.94 ± 0.79, 2.28 ± 0.69, and 1.74 ± 0.46 L/min/m(2) for pts with ES, IPAH, and CTEPH respectively, pANOVA = 0.004, P post hoc ES versus all other groups < 0.05). TAPSE, Tei index, and S't were similar between groups and impaired versus controls (pANOVA < 0.001, P post hoc between groups of patients = NS). Patients with ES had better RVFAC (41.1 ± 9, 30.5 ± 10.8, 23.2 ± 9.8%) and RV free wall strain (-20.6 ± 3.5, -16.3 ± 7.5, -10.8 ± 5%), as well as an increased thickness of the RV free wall compared to other groups of patients (9.2 ± 1.5, 7.2 ± 1 and 7.2 ± 1.6 mm for pts with ES, IPAH and CTEPH, respectively) (pANOVA<0.001, P post hoc <0.05 ES versus all other groups). RVFAC and RV free wall strain significantly correlated with COi (r = 0.53, P = 0.006 and r = -0.77, P < 0.001, respectively). CONCLUSION: Patients with ES have a more hypertrophied RV free wall, better RV performance as assessed by RVFAC and RV free wall strain and increased COi compared to other types of PH. Furthermore, RV performance appears to be less dependent on the level of pressure overload. These findings could contribute to the better survival profile of patients with ES.

Right ventricular function predicts clinical response to specific vasodilator therapy in patients with pulmonary hypertension.

INTRODUCTION: We followed patients with pulmonary arterial hypertension (PAH) receiving specific vasodilator therapy and tested for predictors of clinical outcome. METHODS: Thirty-two patients (mean age 39 ± 15 years, 22 women, diagnosed with pulmonary hypertension; PH): 29 with PAH and 3 patients with inoperable chronic thromboembolic PH received therapy with either bosentan, sildenafil, or both and were evaluated with clinical parameters, biomarkers (B-type natriuretic peptide values), and echocardiography before receiving specific medication and every 3 months thereafter. A right heart catheterization was performed at baseline. A composite endpoint of death, worsening of functional class, or the need of a second vasodilator agent was used to define the clinical nonresponders. RESULTS: Patients were followed for 14 months (7.5-21). The endpoint was reached by 15 patients: four patients died (two idiopathic PAH and two PAH in context of Eisenmenger syndrome), seven patients showed 1 functional class worsening, and four patients needed to be switched to combination therapy. Patients who remained clinically stable or improved had at baseline a better cardiac output with a less remodeled right ventricle (RV) and better functioning RV (all P < 0.05). A RV fractional area change (RVFAC) lower than 25.7% and a RV global strain value higher than -13.4% predict with 87% sensitivity and 83% specificity (AUC 87.3%, P = 0.001) and 73% sensitivity and 91% specificity (AUC 84.2%, P = 0.003), respectively, patients who will deteriorate clinically under specific vasodilator therapy. A multivariate model showed RVFAC to be the only independent predictor of the endpoint with a HR of 0.87 (0.8-0.96), P = 0.007. CONCLUSIONS: Over an average period of 1 year, almost half of patients showed signs of clinical deterioration despite specific vasodilator therapy. Parameters of right ventricular morphology and function had prognostic value in these patients.

Screening for Pulmonary Hypertension in Systemic Sclerosis-A Primer for Cardio-Rheumatology Clinics.

Systemic sclerosis (SSc) is a rare disease, with unfavorable clinical course and prognosis, characterized by progressive multisystemic involvement. SSc associated pulmonary hypertension (SSc-PAH) and interstitial lung disease (ILD) are the most important factors for morbi-mortality in these patients, being responsible for more than 60% of total deaths. Though pulmonary arterial hypertension (PAH) is the dominant subtype seen in SSc, PH secondary to ILD, left-heart pathology, and pulmonary veno-occlusive disease (PVOD) are also possible occurrences. Initial evaluation of a SSc case is complex and should be performed with a multidisciplinary approach. Early detection of SSc-PAH is imperative, given the fact that new and effective medications are available and early treatment was shown to improve outcomes. Therefore, screening algorithms must be used adequately and in a cost-effective manner. Sensitivity and negative predictive value (NPV) are the most important performance measures in a screening test. Several algorithms were developed in the last decade (e.g., DETECT and ASIG) and demonstrated higher efficiency when compared to older algorithms. The present manuscript details the risk factors for SSc-PAH and includes a critical description of current detection algorithms, as a primer for clinicians working in the field of cardio-rheumatology.

European Society of Cardiology Working Group on Adult Congenital Heart Disease and Study Group for Adult Congenital Heart Care in Central and South Eastern European Countries consensus paper: current status, provision gaps and investment required.

AIMS: To examine the current status of care and needs of adult congenital heart disease (ACHD) services in the Central and South Eastern European (CESEE) region. METHODS AND RESULTS: We obtained data regarding the national ACHD status for 19 CESEE countries from their ACHD representative based on an extensive survey for 2017 and/or 2018. Thirteen countries reported at least one tertiary ACHD centre with a median year of centre establishment in 2007 (interquartile range 2002-2013). ACHD centres reported a median of 2114 patients under active follow-up with an annual cardiac catheter and surgical intervention volume of 49 and 40, respectively. The majority (90%) of catheter or surgical interventions were funded by government reimbursement schemes. However, all 19 countries had financial caps on a hospital level, leading to patient waiting lists and restrictions in the number of procedures that can be performed. The median number of ACHD specialists per country was 3. The majority of centres (75%) did not have ACHD specialist nurses. The six countries with no dedicated ACHD centres had lower Gross Domestic Product per capita compared to the remainder (P = 0.005). CONCLUSION: The majority of countries in CESEE now have established ACHD services with adequate infrastructure and a patient workload comparable to the rest of Europe, but important gaps still exist. ACHD care is challenged or compromised by limited financial resources, insufficient staffing levels, and reimbursement caps on essential procedures compared to Western Europe. Active advocacy and increased resources are required to address the inequalities of care across the continent.

Fast Progressing His-Purkinje Conduction Disturbances in a Myotonic Dystrophy Pacient.

Myotonic dystrophy leads to multiple systemic complications and the age of death is earlier in myotonic dystrophy patients than in the general population. These patients have a high frequency of sudden death related to respiratory failure, cardiac arrhythmias and in particular to cardiac conduction disturbances. Prophylactic pacemaker implantation should be considered in asymptomatic myotonic dystrophy patients, which in the early stages of disease present minor conduction disturbances in 12-leads ECG. Even if the rate of progression of conduction abnormalities is usually slow, fast progression has been often observed thus making the clinical course of individual patients rather unpredictable.

Different patterns of adaptation of the right ventricle to pressure overload: a comparison between pulmonary hypertension and pulmonary stenosis.

BACKGROUND: The study was designed to compare RV morphological and functional parameters derived from conventional and myocardial deformation echocardiography in two instances of right heart pressure overload: pulmonary arterial hypertension (PAH) and pulmonary stenosis (PS). METHODS: Sixty-two individuals were included: 22 patients with pulmonary arterial hypertension (PAH), 19 patients with PS and 21 healthy individuals who served as a control group. All patients had clinical evaluation with 6-minute walking test, standard and two-dimensional strain echocardiography and B-type natriuretic peptide evaluation. RESULTS: At similar levels of pressure overload (RV systolic pressure, 88.2 ± 31.5 vs 73.4 ± 34.9 mm Hg; P = NS) the right ventricles of patients with PS were less dilated (RV end-diastolic diameter, 31.7 ± 3.7 vs 43.7 ± 10.5 mm; P < .001) and performed significantly better than those of patients with PAH (RV strain, -27.4 ± 5.8% vs 16.2 ± 7.9%; RV fractional area change, 51.1 ± 9.2% vs 29.1 ± 11.3%; P < .001). Although some of the RV functional parameters were comparable with those in healthy individuals, strain rate showed lower values, suggesting subclinical longitudinal dysfunction in patients with PS. Myocardial stress biomarkers were correlated with RV systolic pressure only in patients with PAH (r = 0.64, P = .03), not in those with PS (r = 0.22, P = .50). CONCLUSIONS: At similar levels of pressure overload, the right ventricle is less dilated and performs better in patients with PS compared with those with PAH.

Utility of QRS width and echocardiography parameters in an integrative algorithm for selecting heart failure patients with cardiac dyssynchrony.

BACKGROUND: Cardiac resynchronization therapy (CRT) is an effective treatment in dilated cardiomyopathy (DCM). However, it has been demonstrated that mechanical dyssynchrony is not related to electrical dyssynchrony. We hypothesized that a new QRS width cutoff could be easier to use as a first step in the selection of patients with inter- and intraventricular dyssynchrony. METHODS: We included 58 patients with DCM. Electrocardiographic (PR interval and QRS width) and echocardiographic (left ventricular dimensions, systolic and diastolic function, dyssynchrony parameters) data were evaluated in all patients. RESULTS: According to QRS width, we divided the study population in two groups: Group 1, 25 patients having a narrow QRS (120 ms). Patients in Group 2 had larger left ventricles, with similar systolic function and more severe diastolic dysfunction than patients with narrow QRS. Interventricular dyssynchrony was more frequent in group 2 (54.5% vs 20%, p=0.01), while intraventricular dyssynchrony was highly prevalent in both groups (82.1% vs 72%, p=0.48). A QRS>140 ms best predicted the presence of interventricular dyssynchrony (sensitivity 78.2% and specificity 63.6%), while a QRS>150 ms best predicts intraventricular dyssynchrony (sensitivity 48.6% and specificity 80%). CONCLUSIONS: Intraventricular dyssynchrony has a high prevalence in patients with DCM, irrespective of the QRS width. Using a higher QRS width cutoff (150 ms) might help in patient selection for CRT. Electrocardiography and echocardiography can be combined into a selection algorithm for patients receiving resynchronization therapy.

The bright minds beyond our machines: Henry Coanda and his ideas.

Most cardiologists rarely remember that new ideas dedicated initially to completely different areas, are the technological background of most medical devices. Also, they almost ignore the bright minds beyond these ideas. The Coanda effect, vital in the color-Doppler assessment of valvular regurgitations with eccentric jets--bears the name of a Romanian inventor whose life (1886-1972) was mostly dedicated to aeronautics and who is considered the 'father' of jet engines. His fluid dynamics discovery was patented in 1934, but its importance for medical applications was recognized much later.