Detection of a rare cause of pulmonary hypertension by multimodality imaging: Left ventricular endomyocardial fibrosis

The Clinical Trials Update highlights new and ongoing research trials that are evaluating therapies for PAH. In this issue, Fernando Torres, MD, examines a study on patients with pulmonary hypertension associated with left ventricular systolic dysfunction.

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Prognostic relevance of sleep apnea syndrome in patients with pulmonary arterial hypertension

European Heart Journal ◽

10.1093/ehjci/ehaa946.2243 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

J Precek ◽

K Vykoupil ◽

F Kovacik ◽

M Hutyra

Keyword(s):

Pulmonary Hypertension ◽

Sleep Apnea ◽

Pulmonary Arterial Hypertension ◽

General Population ◽

Arterial Hypertension ◽

Left Ventricular ◽

Sleep Study ◽

Prognostic Relevance ◽

Obstructive Sleep ◽

Pulmonary Arterial

Abstract Introduction Sleep disordered breathing (SDB) is a group of ventilatory disorders during sleep which includes obstructive sleep apnea (OSA), central sleep apnea (CSA), and sleep related hypoventilation. In patients with SDB, the prevalence of pulmonary hypertension (PH) ranges from 17% to 52%. While SDB is prevalent in the general population with recent estimates of 20% to 30%, in those with cardiovascular disease, particularly left ventricular failure, there is a higher reported prevalence of 47%. Aims The aims of this study were to determine the prevalence and prognostic relevance of sleep apnea in a cohort of patients with newly diagnosed pulmonary arterial hypertension (ESC/WHO Group 1 pulmonary hypertension). Methods We evaluated prospectively 76 patients with the pulmonary arterial hypertension (mean age 54±16 years; 45% male). All patients underwent right heart catheterisation, clinical assessments, sleep study, standard laboratory testing and evaluation of subjective sleepiness by the Epworth Sleepiness Scale. Sleep test was provided with an ApneaLink Plus, consisting of nasal pressure sensor, respiratory effort band, and pulse oximeter worn on the finger. Subjects previously treated for or diagnosed with SDB were excluded from the study. Results Sleep apnea (SA) – defined as apnea-hypopnea index (AHI) ≥5/h – was found in 59 (77.6%) of the pulmonary arterial hypertension (PAH) patients. Mean AHI in the cohort of PAH patients with SA was 26.1±16.6/h. Mean follow-up was 24 months, during which 15 (19.7%) patients died. Characteristics of parameters related to SA in groups of survivors and deceased are in table 1. From the sleep apnea-related parameters, only time with O2Sat <90% – T90 was significantly associated with mortality (AUC 0.856; 95% CI 0.693 – 1.019; p<0.001). Conclusions The presence of sleep apnea in pulmonary arterial hypertension patients is high. The prevalence of sleep apnea is higher in PAH patients than in the general population. The presence of sleep apnea in patients with PAH was not associated with worse prognosis, but noctural hypoxemia (time with O2Sat <90%) was related to poor prognosis. Sleep apnea in patients with PAH should be screened for systematically. Funding Acknowledgement Type of funding source: None

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Challenges in the diagnosis of peripartum cardiomyopathy: a case series

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytab001 ◽

2021 ◽

Vol 5 (2) ◽

Author(s):

Fabio Chirillo ◽

Anna Baritussio ◽

Umberto Cucchini ◽

Ermanno Toniolli ◽

Angela Polo ◽

...

Keyword(s):

Heart Failure ◽

Multimodality Imaging ◽

Systolic Dysfunction ◽

Case Series ◽

Left Ventricular ◽

Peripartum Cardiomyopathy ◽

Lv Function ◽

Prognostic Information ◽

Clinical Scenarios ◽

Anterolateral Wall

Abstract Background Peripartum cardiomyopathy (PPCM) is usually characterized by overt heart failure, but other clinical scenarios are possible, sometimes making the diagnosis challenging. Case summary We report a case series of four patients with PPCM. The first patient presented with acute heart failure due to left ventricular (LV) systolic dysfunction. Following medical treatment, LV function recovered completely at 1 month. The second patient had systemic and pulmonary thromboembolism, secondary to severe biventricular dysfunction with biventricular thrombi. The third patient presented with myocardial infarction with non-obstructed coronary arteries and evidence of an aneurysm of the mid-anterolateral LV wall. The fourth patient, diagnosed with PPCM 11 years earlier, presented with sustained ventricular tachycardia. A repeat cardiac magnetic resonance, compared to the previous one performed 11 years earlier, showed an enlarged LV aneurysm in the mid-LV anterolateral wall with worsened global LV function. Discussion Peripartum cardiomyopathy may have different clinical presentations. Attentive clinical evaluation and multimodality imaging can provide precise diagnostic and prognostic information.

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Echocardiographic Prevalence and Risk Predictors of Ventricular Dysfunction in Connective Tissue Disorders: Tertiary Care Hospital-Based Prospective Case-Control Study

Indian Journal of Clinical Cardiology ◽

10.1177/2632463620966143 ◽

2020 ◽

Vol 1 (3-4) ◽

pp. 132-141

Author(s):

Saru Thakur ◽

Geeta Ram Tegta ◽

Prakash Chand Negi ◽

Kunal Mahajan ◽

Ghanshyam Verma ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Connective Tissue ◽

Lupus Erythematosus ◽

Systolic Dysfunction ◽

Tertiary Care ◽

Left Ventricular ◽

Care Hospital ◽

Connective Tissue Disorders ◽

Cardiac Symptom ◽

Recorded Data

Background: There is a paucity of contemporary Indian data about the prevalence of cardiac abnormalities in patients of connective tissue disorders (CTD) and their risk determinants. Methods: We prospectively recorded data from 35 consecutive CTD patients who presented to our out-patient department and had no significant cardiovascular risk factors at baseline. We also recorded data from their age- and sex-matched controls. All cases and controls were subjected to 12 lead electrocardiogram and echocardiography after routine investigations. Results: The CTD group comprised 19 (54.3%) patients of systemic lupus erythematosus, 12 (34.3%) patients of systemic sclerosis, 2 (5.7%) patients of mixed CTD, and 1 (2.9%) patient each of overlap syndrome and dermatomyositis. Cardiovascular involvement on echocardiography was documented in 71.4% of CTD patients despite majority of them having no cardiac symptom. Overt left ventricular (LV) systolic dysfunction was observed in 3 (8.6%) CTD patients, while subclinical LV systolic dysfunction was recorded in 13 (37.1%) patients. LV diastolic dysfunction was observed in 11.4% (n = 4) patients. RV systolic dysfunction was prevalent in 20% (n = 7) patients. Pulmonary hypertension was observed in 40% (n = 14) of CTD patients. Conclusion: The present study evaluated subclinical LV systolic dysfunction and pulmonary hypertension in about one third of CTD patients. It is imperative to screen for these abnormalities in CTD to ensure timely diagnosis and treatment.

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