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

PulmoCor: National registry for pulmonary hypertension

Contains fulltext : 171308.pdf (publisher's version ) (Open Access

Impact of Interatrial Shunts on Invasive Hemodynamics and Exercise Tolerance in Patients With Heart Failure

Approximately 50% of patients with heart failure have preserved ejection fraction. Although a wide variety of conditions cause or contribute to heart failure with preserved ejection fraction, elevated left ventricular filling pressures, particularly during exercise, are common to all causes. Acute elevation in left-sided filling pressures promotes lung congestion and symptoms of dyspnea, while chronic elevations often lead to pulmonary vascular remodeling, right heart failure, and increased risk of mortality. Pharmacologic therapies, including neurohormonal modulation and drugs that modify the nitric oxide/cyclic GMP-protein kinase G pathway have thus far been limited in reducing symptoms or improving outcomes in patients with heart failure with preserved ejection fraction. Hence, alternative means of reducing the detrimental rise in left-sided heart pressures are being explored. One proposed method of achieving this is to create an interatrial shunt, thus unloading the left heart at rest and during exercise. Currently available studies have shown 3- to 5-mm Hg decreases of pulmonary capillary wedge pressure during exercise despite increased workload. The mechanisms underlying the hemodynamic changes are just starting to be understood. In this review we summarize results of recent studies aimed at elucidating the potential mechanisms of improved hemodynamics during exercise tolerance following interatrial shunt implantation and the current interatrial shunt devices under investigation

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

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

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

FLNC missense variants in familial noncompaction cardiomyopathy

The majority of familial noncompaction cardiomyopathy (NCCM) is explained by pathogenic variants in the same sarcomeric genes that are associated with hypertrophic (HCM) and dilated (DCM) cardiomyopathy. Pathogenic variants in the filamin C gene (FLNC) have been linked to HCM and DCM. We expand the spectrum of FLNC related cardiomyopathies by presenting two families with likely pathogenic FLNC variants showing familial segregation of NCCM and concurrent coarctation of the aorta and/or mitral valve abnormalities