Platypnea orthodeoxia syndrome after recent stroke:A case report of a sandwiched right atrium

Background: Platypnea orthodeoxia syndrome (POS) is a condition characterized by onset or worsening of dyspnoea and desaturation in upright position that is relieved by returning to a supine position. This case report illustrates a sudden onset of severe platypnea caused by compression of the right atrium (RA) due to aortic dilatation and unilateral diaphragmatic paralysis after a recent stroke. Case summary: A 71-year-male patient with a medical history of recent stroke of the left hemisphere was referred to emergency department with acute dyspnoea. During observation in the emergency department, desaturation was noted in upright position. A contrast computed tomography excluded pulmonary embolism but revealed a dilated aortic root and an elevated right hemidiaphragm. The RA was compressed between these two structures (sandwiched). Given the clinical suspicion of a POS, a transoesophageal echocardiography was performed which confirmed the presence of a persistent foramen ovale (PFO) in supine position. In upright position, there was a torrential increase in right-to-left shunting. The PFO was closed using an Occlutech™ device. Directly after the procedure, the patient was symptom free. Discussion: A rise in RA pressure or difference in flow pattern in the RA can make a PFO become symptomatic. Elevated RA pressure was ruled out. Most anatomical pathologies influencing the flow pattern develop slowly over time. This case shows a presentation of POS after a recent stroke possible due to change in anatomy because of right hemidiaphragm paralysis in combination with the aortic dilatation

Epicardial Adipose Tissue and Invasive Hemodynamics in Heart Failure With Preserved Ejection Fraction

Objectives: This study examined associations between epicardial adipose tissue (EAT), invasive hemodynamics, and exercise capacity in patients with heart failure with preserved ejection fraction (HFpEF). Background: EAT is increased in patients with HFpEF and may play a role in the pathophysiology of this disorder. Methods: Patients with heart failure and a left ventricular ejection fraction >45% who underwent right and left heart catheterization with simultaneous echocardiography were included. Pulmonary capillary wedge pressure (PCWP), left ventricular end-diastolic pressure (LVEDP), right ventricular end-diastolic pressure (RVEDP), and pulmonary vascular resistance (PVR) were invasively measured. Obesity was defined as body mass index (BMI) ≥30 kg/m2. EAT thickness alongside the right ventricle was measured on echocardiographic long- and short-axis views. Cardiopulmonary exercise testing was performed to obtain maximal oxygen uptake (VO2-max). Results: This study examined 75 patients, mean age 74 ± 9 years; 68% were women, mean BMI was 29 ± 6 kg/m2, and 36% were obese. Higher BMI was strongly associated with increased EAT (r = 0.74; p < 0.001). Increased EAT was associated with higher RVEDP, independent of PVR (odds ratio [OR]: 1.16; 95% confidence interval [CI]: 1.02 to 1.34; p = 0.03), but not independent of obesity (p = 0.10). Increased EAT and higher RVEDP were both associated with lower VO2-max (r = −0.43; p < 0.001 and r = −0.43; p = 0.001, respectively). Increased EAT remained associated with lower VO2-max after adjustment for PVR (OR: 0.64; 95% CI: 0.49 to 0.84; p = 0.002) and obesity (OR: 0.69; 95% CI: 0.53 to 0.92; p = 0.01). EAT thickness was not associated with left-sided filling pressures (i.e., PCWP and LVEDP). Conclusions: In HFpEF, obesity and increased EAT were associated with higher right-sided filling pressures and with reduced exercise capacity

Right Heart Dysfunction in Heart Failure with Preserved Ejection Fraction:the Impact of Atrial Fibrillation

BACKGROUND: Right ventricular (RV) dysfunction and atrial fibrillation (AF) frequently coexist in heart failure with preserved ejection fraction (HFpEF). The mechanisms underlying the association between AF and RV dysfunction are incompletely understood. METHODS AND RESULTS: 102 patients were identified. RV function was assessed using multiple echocardiographic parameters and dysfunction was present if ≥2 parameters were below the recommended cutoff. RV function, right atrial (RA) reservoir strain and RA emptying fraction, were compared between AF and sinus rhythm. 91 patients with sufficient echocardiographic quality were included: 45 (50%) had no history of AF; 14 (15%) had prior AF while in sinus rhythm; 32 (35%) had current AF. The prevalence of RV dysfunction varied across subgroups never AF, prior AF and current AF (20%, 43% and 63%, respectively, p=0.001). AF was associated with RV dysfunction (OR 4.70 [1.82-12.1], p=0.001) - independent of pulmonary pressures. In patients in sinus rhythm with prior AF, RA emptying fraction was lower compared to patients without AF history (41 vs. 60%, p=0.002). Prior AF was also associated with reduced RA reservoir strain (OR 4.57 [1.05-19.9], p=0.04) - independent of RV end-diastolic pressure. CONCLUSIONS: Atrial fibrillation is strongly related to reduced RV and RA function in HFpEF, independent of pulmonary pressures

Six-Minute Walk Test in Patients With Down Syndrome:Validity and Reproducibility

Contains fulltext : 81543.pdf (publisher's version ) (Closed access)OBJECTIVES: To examine the validity of the six-minute walk test (6MWT) as a tool to evaluate functional exercise performance in patients with Down syndrome (DS). DESIGN: Comparison of the six-minute walk distance (6MWD) in 2 distinct groups of DS patients: with and without severe cardiac disease. To test reproducibility, a group of patients with DS performed the 6MWT twice. SETTING: Tertiary referral centers for patients with congenital heart defects and outpatient clinics for people with intellectual disabilities. PARTICIPANTS: Adult patients with DS with (n=29) and without (n=52) severe cardiac disease categorized by cardiac echocardiography. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE: Distance walked on the 6MWT. RESULTS: The mean 6MWD in the group with severe cardiac disease was 289+/-104 m and in the group without severe cardiac disease 280+/-104 m (P=.70). Older age, female sex, and severe level of intellectual disability were all found to be independently and significantly correlated with a lower 6MWD (r=.67, P<.001). The paired 6MWD was not significantly different (310+/-88 m vs 317+/-85 m; P=.40) in patients who performed the 6MWT twice. The coefficient of variation was 11%. CONCLUSIONS: The 6MWD between the 2 groups was not significantly different. However, the walking distance inversely correlated with the level of intellectual disability. Therefore, the 6MWT is not a valid test to examine cardiac restriction in adult patients with DS

Right ventricular recovery after bilateral lung transplantation for pulmonary arterial hypertension

OBJECTIVES: Pulmonary arterial hypertension (PAH) is a progressive and often fatal disease characterized by increased pulmonary vascular resistance (PVR) and right ventricular (RV) failure. End-stage PAH is often an indication for a lung transplant (LTX). Our goal was to study ventricular recovery using cardiac magnetic resonance imaging late after LTX. METHODS: We studied 10 patients with PAH who underwent isolated bilateral LTX. RV and left ventricular (LV) volumes, function and mass were measured. In addition, the RV stroke volume/end-systolic ratio (SV/ESV), the LV eccentricity index, the RV/LV volume ratio, the area of the tricuspid valve annulus and the severity of tricuspid regurgitation (TR) were calculated. RESULTS: The median age was 44 [30-54] years and the mean PVR was 1020 ± 435 dynes·s·cm â ' 5. Six patients had ≥ moderate TR. After LTX, the RV ejection fraction increased from 32 to 64% (P < 0.001) and both RV volume (from 118 to 51 ml/m 2, P < 0.001) and RV mass (from 69 to 33 g/m 2, P < 0.001) decreased. The mean SV/ESV ratio increased from 0.5 to 1.9 (P < 0.001) and the LV mass increased from 55 to 61 g/m 2 (P = 0.005). There was a decrease in both the LV eccentricity index (from 2.8 to 1.1, P < 0.001) and the RV/LV volume ratio (from 2.3 to 0.8, P < 0.001). The area of the tricuspid valve annulus also decreased (from 9.8 to 4.6 cm 2 /m 2, P < 0.001); no patient had ≥ mild TR post-LTX. CONCLUSIONS: Cardiac magnetic resonance imaging confirms ventricular recovery after isolated bilateral LTX for end-stage PAH

The role of cystatin C as a biomarker for prognosis in pulmonary arterial hypertension due to congenital heart disease

AbstractBackgroundAdults with pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) have a poor prognosis. Identifying patients with a high risk for clinical events and death is important because their prognosis can be improved by intensifying their treatment. Cystatin C, a novel cardiac biomarker, correlates with right ventricular dimensions in patients with idiopathic PAH, giving it potential to determine prognosis in PAH-CHD patients. We investigated the predictive value of cystatin C for long-term mortality and clinical events.MethodsFifty-nine PAH-CHD patients (mean age 42 SD 13 years, 42% male) were included in this prospective observational study, with cystatin C measurements between 2005 and 2015 on the outpatient clinic. Patients were evaluated with a standardized evaluation protocol including laboratory, functional and echocardiographic variables. Clinical events comprised worsening functional classification, worsening heart failure, symptomatic hyperviscosity, haemoptysis and arrhythmia. We used Cox regression to determine predictors for mortality and clinical events.ResultsMean follow-up was 4.4years, during which 12 (20%) patients died. Cystatin C (HR 1.3, p<0.001), creatinine (HR 1.2, p<0.001), NT-pro-BNP (HR 2.0, p=0.012), hs-troponin T (HR 1.9, p=0.005), 6-MWD (HR 0.8, p=0.044) and TAPSE (HR 0.8, p<0.001) predicted mortality. Similar results were found for the prediction of clinical events. When adjusted for NT-pro-BNP or glomerular filtration rate in multivariate analysis, cystatin C remained predictive for mortality.ConclusionsCystatin C, a novel cardiac biomarker, predicts long-term mortality and clinical events in patients with PAH-CHD. Consequently, cystatin C may attribute to clinical decision making regarding treatment intensity

Right ventricular-vascular coupling in heart failure with preserved ejection fraction and pre- vs. post-capillary pulmonary hypertension

Aims Many patients with heart failure with preserved ejection fraction (HFpEF) develop post-capillary pulmonary hypertension (PH) due to increased left-sided filling pressures. However, a subset of patients develops combined postand pre-capillary PH. We studied the value of echocardiographic right-sided characterization for the discrimination between pre- vs. post-capillary PH in HFpEF, using invasive haemodynamics as gold standard. Methods and results 102 consecutive HFpEF patients with simultaneous right heart catheterization and echocardiography were identified. Patients were divided into: 'no PH', 'isolated post-capillary PH', and 'post-and pre-capillary PH'. Systolic pulmonary arterial pressure (SPAP), tricuspid valve annular plane systolic excursion (TAPSE), right ventricular-vascular coupling (TAPSE/SPAP), and VO2-max were assessed. Primary endpoint was all-cause mortality. A total of 97 patients were included: 22% no PH, 47% isolated post-capillary PH, and 31% post- and pre-capillary PH. Patients with post-and pre-capillary PH had more often diabetes mellitus (47 vs. 24%, P = 0.04), had more heart failure hospitalizations (57 vs. 26%, P = 0.007) and lower VO2-max (10 vs. 13 mL/min/kg, P = 0.008), compared with those with isolated post-capillary PH. Patients with post-and pre-capillary PH also had more reduced TAPSE (17 vs. 21 mm, P = 0.001) and TAPSE/SPAP (0.3 vs. 0.5, P <0.001). TAPSE/SPAP ratio <0.36 had a good accuracy to identify patients with additional pre-capillary PH (C-statistic 0.86, sensitivity 86% and specificity 79%). TAPSE/SPAP ratio was associated with increased mortality (HR 2.51 [95% CI 1.25-5.01], P = 0.009). Conclusion Abnormal right ventricular-vascular coupling identifies patients with HFpEF and additional pre-capillary PH, and predicts poor outcome in HFpEF

Diabetes Mellitus and Right Ventricular Dysfunction in Heart Failure With Preserved Ejection Fraction

Diabetes mellitus is associated with left-sided myocardial remodeling in heart failure with preserved ejection fraction (HFpEF). Little is known about the impact of diabetes mellitus on right ventricular (RV) function in HFpEF. We therefore studied the relation between diabetes mellitus and RV dysfunction in HFpEF. We have examined patients with HFpEF who underwent simultaneous right-sided cardiac catheterization and echocardiography. RV systolic function was assessed using multiple established echocardiographic parameters, and systolic dysfunction was present if parameters were outside the normal range. RV diastolic function was assessed using the peak diastolic tissue velocity of the lateral tricuspid annulus (RV e') and was present if = 7.0 mmol/L, a positive glucose intolerance test result, or a glycated hemoglobin level of >= 6.5%. A total of 91 patients were studied (mean age 74 +/- 9 years, 69% women). A total of 37% had RV systolic dysfunction and 23% RV diastolic dysfunction. Thirty-seven percent of the patients had type 2 diabetes mellitus. These patients had higher pulmonary artery pressure (34 mm Hg vs 29 mm Hg, p = 0.004), more RV systolic dysfunction (57% vs 29%, p = 0.009), more RV diastolic dysfunction (46% vs 12%, p = 0.001), and lower RV e' (8.7 cm/s vs 11.5 cm/s, p = 0.006). The presence of diabetes mellitus was independently associated with RV systolic dysfunction (odds ratio 2.84, 95% confidence interval 1.09 to 7.40, p = 0.03) and with RV diastolic dysfunction (odds ratio 4.33, 95% confidence interval 1.25 to 15.07, p = 0.02), after adjustment for age, gender, and pulmonary pressures. In conclusion, diabetes mellitus is strongly associated with RV systolic and diastolic dysfunctions in patients with HFpEF, independent of RV afterload. (C) 2017 Elsevier Inc. All rights reserved

Hypertensive response to exercise in adult patients with repaired aortic coarctation

OBJECTIVE: The clinical and prognostic implications of a hypertensive response to exercise after repair of coarctation of the aorta (CoA) remain controversial. We aimed to determine the prevalence of a hypertensive response to exercise, identify factors associated with peak exercise systolic blood pressure (SBP) and explore the association of peak exercise SBP with resting blood pressure and cardiovascular events during follow-up. METHODS: From the Dutch national CONgenital CORvitia (CONCOR) registry, adults with repaired CoA who underwent exercise stress testing were included. A hypertensive response to exercise was defined as a peak exercise SBP ≥210 mm Hg in men and ≥190 mm Hg in women. Cardiovascular events consisted of coronary artery disease, stroke, aortic complications and cardiovascular death. RESULTS: Of the original cohort of 920 adults with repaired CoA, 675 patients (median age 24 years (range 16-72 years)) underwent exercise stress testing. Of these, 299 patients (44%) had a hypertensive response to exercise. Mean follow-up duration was 10.1 years. Male sex, absence of a bicuspid aortic valve and elevated resting SBP were independently associated with increased peak exercise SBP. Peak exercise SBP was positively predictive of office SBP (β=0.11, p<0.001) and 24-hour SBP (β=0.05, p=0.03) at follow-up, despite correction for baseline SBP. During follow-up, 100 patients (15%) developed at least 1 cardiovascular event. Peak exercise SBP was not significantly associated with the occurrence of cardiovascular events (HR 0.994 (95% CI 0.987 to 1.001), p=0.11). CONCLUSIONS: A hypertensive response to exercise was present in nearly half of the patients in this large, prospective cohort of adults with repaired CoA. Risk factors for increased peak exercise SBP were male sex, absence of a bicuspid aortic valve and elevated resting SBP. Increased peak exercise SBP independently predicted hypertension at follow-up. These results support close follow-up of patients with a hypertensive response to exercise to ensure timely diagnosis and treatment of future hypertension