Background: The ratios of tricuspid annular plane systolic excursion (TAPSE)/echocardiographically measured systolic pulmonary artery pressure (PASP), fractional area change/invasively measured mean pulmonary artery pressure, right ventricular (RV) area change/end-systolic area, TAPSE/pulmonary artery acceleration time, and stroke volume/end-systolic area have been proposed as surrogates of RV-arterial coupling. The relationship of these surrogates with the gold standard measure of RV-arterial coupling (invasive pressure-volume loop-derived end-systolic/arterial elastance [Ees/Ea] ratio) and RV diastolic stiffness (end-diastolic elastance) in pulmonary hypertension remains incompletely understood. We evaluated the relationship of these surrogates with invasive pressure-volume loop-derived Ees/Ea and end-diastolic elastance in pulmonary hypertension. Methods: We performed right heart echocardiography and cardiac magnetic resonance imaging 1 day before invasive measurement of pulmonary hemodynamics and single-beat RV pressure-volume loops in 52 patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. The relationships of the proposed surrogates with Ees/Ea and end-diastolic elastance were evaluated by Spearman correlation, multivariate logistic regression, and receiver operating characteristic analyses. Associations with prognosis were evaluated by Kaplan-Meier analysis. Results: TAPSE/PASP, fractional area change/mean pulmonary artery pressure, RV area change/end-systolic area, and stroke volume/end-systolic area but not TAPSE/pulmonary artery acceleration time were correlated with Ees/Ea and end-diastolic elastance. Of the surrogates, only TAPSE/PASP emerged as an independent predictor of Ees/Ea (multivariate odds ratio: 18.6; 95% CI, 0.8–96.1; P =0.08). In receiver operating characteristic analysis, a TAPSE/PASP cutoff of 0.31 mm/mm Hg (sensitivity: 87.5% and specificity: 75.9%) discriminated RV-arterial uncoupling (Ees/Ea <0.805). Patients with TAPSE/PASP <0.31 mm/mm Hg had a significantly worse prognosis than those with higher TAPSE/PASP. Conclusions: Echocardiographically determined TAPSE/PASP is a straightforward noninvasive measure of RV-arterial coupling and is affected by RV diastolic stiffness in severe pulmonary hypertension. Clinical Trial Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT03403868.

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ABSTRACT

Objective: To assess long term changes in cardiac morphology and function in survivors of severe twin to twin transfusion syndrome (TTTS) after intrauterine laser coagulation of placental anastomoses. Design: Prospective follow up of fetuses with severe TTTS treated by laser coagulation of intrauterine placental anastomoses. Fetal echocardiography and Doppler studies of feto-placental haemodynamic function were performed at the time of laser coagulation (median gestational age of 21.7 weeks). Postnatal cardiac follow up included a detailed echocardiographic study of systolic and diastolic cardiac function at a median age of 21.1 months. Setting: Paediatric cardiology unit. Patients: 89 survivors from 73 consecutive pregnancies with severe TTTS. Results: Before laser treatment, 28 of 51 (54.9%) recipient twins had typical signs of cardiac dysfunction due to volume overload and 9 of 38 (23.7%) donors had absent or reversed end diastolic flow in the umbilical artery. Echocardiography was normal in 87.6% of the survivors (34 of 38 donors, 44 of 51 recipients). The prevalence of congenital heart disease and particularly of pulmonary stenosis, which was recorded only in recipients, was increased in comparison with the general population (congenital heart disease, 10 of 89 (11.2%) v 0.3%; pulmonary stenosis, 4 of 51 (7.8%) v 0.03%). Findings before laser treatment were not correlated with the development of structural heart disease. Conclusions: Despite the high rate and severity of prenatal cardiac overload in recipients, the majority of cases of TTTS are normalised after laser treatment. However, given the increased prevalence of congenital heart disease and in particular pulmonary stenosis, intrauterine and postnatal follow up is warranted.

Patients with SS have a high overall survival. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. Surgical treatment of SS is beneficial in reducing symptoms, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic evaluation and to the patient's age.