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Loeys Dietz Syndrome and pregnancy: A case report with literature review and a proposed focused management protocol.

Int J Cardiol. 2016 Jul 01;214:491-2

Authors: Cauldwell M, Patel RR, Uebing A, Gatzoulis MA, Swan L

PMID: 27100340 [PubMed - indexed for MEDLINE]

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Pre-Capillary, Combined, and Post-Capillary Pulmonary Hypertension: A Pathophysiological Continuum.

J Am Coll Cardiol. 2016 Jul 26;68(4):368-78

Authors: Opitz CF, Hoeper MM, Gibbs JS, Kaemmerer H, Pepke-Zaba J, Coghlan JG, Scelsi L, D'Alto M, Olsson KM, Ulrich S, Scholtz W, Schulz U, Grünig E, Vizza CD, Staehler G, Bruch L, Huscher D, Pittrow D, Rosenkranz S

BACKGROUND: Pulmonary hypertension (PH) is hemodynamically classified as pre-capillary (as seen in idiopathic pulmonary arterial hypertension [IPAH]) or post-capillary (as seen in heart failure with preserved ejection fraction [HFpEF]). Overlaps between these conditions exist. Some patients present with risk factors for left heart disease but pre-capillary PH, whereas patients with HFpEF may have combined pre- and post-capillary PH.
OBJECTIVES: This study sought to further characterize similarities and differences among patient populations with either PH-HFpEF or IPAH.
METHODS: We used registry data to analyze clinical characteristics, hemodynamics, and treatment responses in patients with typical IPAH (<3 risk factors for left heart disease; n = 421), atypical IPAH (≥3 risk factors for left heart disease; n = 139), and PH-HFpEF (n = 226) receiving PH-targeted therapy.
RESULTS: Compared with typical IPAH, patients with atypical IPAH and PH-HFpEF were older, had a higher body mass index, had more comorbidities, and had a lower 6-min walking distance, whereas mean pulmonary artery pressure (46.9 ± 13.3 mm Hg vs. 43.9 ± 10.7 mm Hg vs. 45.7 ± 9.4 mm Hg, respectively) and cardiac index (2.3 ± 0.8 l/min/m(2) vs. 2.2 ± 0.8 l/min/m(2) vs. 2.2 ± 0.7 l/min/m(2), respectively) were comparable among groups. After initiation of targeted PH therapies, all groups showed improvement in exercise capacity, functional class, and natriuretic peptides from baseline to 12 months, but treatment effects were less pronounced in patients with PH-HFpEF than typical IPAH; with atypical IPAH in between. Survival rates at 1, 3, and 5 years were almost identical for the 3 groups.
CONCLUSIONS: Patients with atypical IPAH share features of both typical IPAH and PH-HFpEF, suggesting that there may be a continuum between these conditions.

PMID: 27443433 [PubMed - indexed for MEDLINE]

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Cardiovascular Magnetic Resonance to Evaluate Aortic Regurgitation After Transcatheter Aortic Valve Replacement.

J Am Coll Cardiol. 2016 Aug 09;68(6):577-85

Authors: Ribeiro HB, Orwat S, Hayek SS, Larose É, Babaliaros V, Dahou A, Le Ven F, Pasian S, Puri R, Abdul-Jawad Altisent O, Campelo-Parada F, Clavel MA, Pibarot P, Lerakis S, Baumgartner H, Rodés-Cabau J

BACKGROUND: Residual aortic regurgitation (AR) following transcatheter aortic valve replacement (TAVR) is associated with greater mortality; yet, determining AR severity post-TAVR using Doppler echocardiography remains challenging. Cardiovascular magnetic resonance (CMR) is purported as a more accurate means of quantifying AR; however, no data exist regarding the prognostic value of AR as assessed by CMR post-TAVR.
OBJECTIVES: This study sought to evaluate the effect of AR assessed with CMR on clinical outcomes post-TAVR.
METHODS: We included 135 patients from 3 centers. AR was quantified using regurgitant fraction (RF) measured by phase-contrast velocity mapping CMR at a median of 40 days post-TAVR, and using Doppler echocardiography at a median of 6 days post-TAVR. Median follow-up was 26 months. Clinical outcomes included mortality and rehospitalization for heart failure.
RESULTS: Moderate-severe AR occurred in 17.1% and 12.8% of patients as measured by echocardiography and CMR, respectively. Higher RF post-TAVR was associated with increased mortality (hazard ratio: 1.18 for each 5% increase in RF [95% confidence interval: 1.08 to 1.30]; p < 0.001) and the combined endpoint of mortality and rehospitalization for heart failure (hazard ratio: 1.19 for each 5% increase in RF; 95% confidence interval: 1.15 to 1.23; p < 0.001). Prediction models yielded significant incremental predictive value; CMR performed a median of 40 days post-TAVR had a greater association with post-TAVR clinical events compared with early echocardiography (p < 0.01). RF ≥30% best predicted poorer clinical outcomes (p < 0.001 for either mortality or the combined endpoint of mortality and heart failure rehospitalization).
CONCLUSIONS: Worse CMR-quantified AR was associated with increased mortality and poorer clinical outcomes following TAVR. Quantifying AR with CMR may identify patients with AR who could benefit from additional treatment measures.

PMID: 27491900 [PubMed - indexed for MEDLINE]

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Exploring Regulatory Mechanisms of Atrial Myocyte Hypertrophy of Mitral Regurgitation through Gene Expression Profiling Analysis: Role of NFAT in Cardiac Hypertrophy.

PLoS One. 2016;11(12):e0166791

Authors: Chang TH, Chen MC, Chang JP, Huang HD, Ho WC, Lin YS, Pan KL, Huang YK, Liu WH, Wu CC

BACKGROUND: Left atrial enlargement in mitral regurgitation (MR) predicts a poor prognosis. The regulatory mechanisms of atrial myocyte hypertrophy of MR patients remain unknown.
METHODS AND RESULTS: This study comprised 14 patients with MR, 7 patients with aortic valve disease (AVD), and 6 purchased samples from normal subjects (NC). We used microarrays, enrichment analysis and quantitative RT-PCR to study the gene expression profiles in the left atria. Microarray results showed that 112 genes were differentially up-regulated and 132 genes were differentially down-regulated in the left atria between MR patients and NC. Enrichment analysis of differentially expressed genes demonstrated that "NFAT in cardiac hypertrophy" pathway was not only one of the significant associated canonical pathways, but also the only one predicted with a non-zero score of 1.34 (i.e. activated) through Ingenuity Pathway Analysis molecule activity predictor. Ingenuity Pathway Analysis Global Molecular Network analysis exhibited that the highest score network also showed high association with cardiac related pathways and functions. Therefore, 5 NFAT associated genes (PPP3R1, PPP3CB, CAMK1, MEF2C, PLCE1) were studies for validation. The mRNA expressions of PPP3CB and MEF2C were significantly up-regulated, and CAMK1 and PPP3R1 were significantly down-regulated in MR patients compared to NC. Moreover, MR patients had significantly increased mRNA levels of PPP3CB, MEF2C and PLCE1 compared to AVD patients. The atrial myocyte size of MR patients significantly exceeded that of the AVD patients and NC.
CONCLUSIONS: Differentially expressed genes in the "NFAT in cardiac hypertrophy" pathway may play a critical role in the atrial myocyte hypertrophy of MR patients.

PMID: 27907007 [PubMed - indexed for MEDLINE]

Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome.

Heart. 2017 Jun 29;:

Authors: Martin-Garcia AC, Arachchillage DR, Kempny A, Alonso-Gonzalez R, Martin-Garcia A, Uebing A, Swan L, Wort SJ, Price LC, McCabe C, Sanchez PL, Dimopoulos K, Gatzoulis MA

OBJECTIVES: Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with Eisenmenger syndrome (ES) is not well studied. Accordingly, we examined the relationship of thrombocytopaenia and mean platelet volume (MPV) to bleeding or thrombotic complications and survival in a contemporary cohort of patients with ES, including patients with Down syndrome.
METHODS: Demographics, laboratory and clinical data were analysed from 226 patients with ES under active follow-up over 11 years.
RESULTS: Age at baseline was 34.6±11.4 years and 34.1% were men. Mean platelet count and MPV were 152.6±73.3×10⁹/L and 9.6±1.2 fL, respectively. A strong inverse correlation was found between platelet count and haemoglobin concentration and MPV. During the study, there were 39 deaths, and 21 thrombotic and 43 bleeding events. On univariate Cox regression analysis, patients with a platelet count <100×10⁹/L had a twofold increased mortality (HR 2.10, 95% CI 1.10 to 4.01, p=0.024). Platelet count was not associated with an increased risk of thrombosis. However, there was a threefold increased thrombotic risk with MPV >9.5 fL (HR 3.50, 95% CI 1.28 to 9.54, p=0.015). Patients with either severe secondary erythrocytosis (>220g/L) or anaemia (<130g/L) were at higher risk of thrombotic events (HR 3.93, 95% CI 1.60 to 9.67, p=0.003; and HR 4.75, 95% CI 1.03 to 21.84, p=0.045, respectively).
CONCLUSIONS: Thrombocytopaenia significantly increased the risk of mortality in ES. Furthermore, raised MPV, severe secondary erythrocytosis and anaemia, but not platelet count, were associated with an increased risk of thrombotic events in our adult cohort.

PMID: 28663364 [PubMed - as supplied by publisher]

Utilizing Hybrid Techniques to Maximize Clinical Outcomes in Congenital Heart Disease.

Curr Cardiol Rep. 2017 Aug;19(8):72

Authors: Bearl DW, Fleming GA

PURPOSE OF REVIEW: The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital cardiology. Recent collaborations between interventional cardiologists and cardiac surgeons have sparked novel innovative hybrid procedures that provide potentially safer, faster, and less invasive alternatives to the management of many diseases. This paper will review the most recent advances in hybrid interventions in the field of pediatric and adult congenital cardiology.
RECENT FINDINGS: The earliest experiences with hybrid interventions included intraoperative stenting of pulmonary arteries and perventricular device closure of ventricular septal defects. Newer hybrid interventions have focused on a hybrid approach to stage 1 palliation for infants with hypoplastic left heart syndrome and hybrid approaches to transcatheter pulmonary valve replacement in patients of all ages. Hybrid approaches to complex congenital heart disease are important in the management of congenital heart disease offering a less invasive approach, and novel hybrid procedures will likely be a focus of future research in this field.

PMID: 28667405 [PubMed - in process]

Outcomes and Costs of Cardiac Surgery in Adults with Congenital Heart Disease.

Pediatr Cardiol. 2017 Jul 01;:

Authors: Nasr VG, Faraoni D, Valente AM, DiNardo JA

Advances in pediatric cardiac surgical and medical care have led to increased survival of patients with congenital heart disease (CHD). Consequently, many CHD patients survive long enough to require cardiac surgery as adults. Using the 2013 Nationwide Inpatient Sample (NIS) database, we compared costs and outcomes for adult patients undergoing surgery for treatment of CHD to a reference population of adults undergoing CABG. Patients were identified using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9 CM) procedure codes. We recorded the demographic characteristics, gender, ethnicity, hospital bed size, hospital length of stay, in-hospital mortality, and comorbidities. Patients with ACHD have higher incidences of in-hospital mortality (2.6 vs. 1.8%), and complication rates including neurologic complications (2.6 vs. 0.9%), thromboembolic complications (3.9 vs. 1.4%), arrhythmias (51.6 vs. 29.8%), hepatic failure (4.44 vs. 2.03%), and sepsis (7.24 vs. 4.61%) (all p < 0.001). In addition, cost is higher in patients with CHD (Coefficient = 0.116, 95% CI, 0.105-0.128; p < 0.001), Elixhauser score ≥ 7 (Coefficient = 0.114, 95% CI, 0.108-0.121; p < 0.001), neurologic complications (Coefficient = 0.169, 95% CI, 0.143-0.196; p < 0.001), thrombotic complications (Coefficient = 0.243, 95% CI, 0.222-0.265; p < 0.001), sepsis (Coefficient = 0.198, 95% CI, 0.185-0.211; p < 0.001), acute kidney injury (Coefficient = 0.056, 95% CI, 0.041-0.063; p < 0.001), elective cases (Coefficient = 0.047, 95% CI, 0.041-0.053; p < 0.001), and length of stay > 6 days (Coefficient = 0.703, 95% CI, 0.697-0.710; p < 0.001). This study shows that ACHD patients undergoing cardiac surgery experience higher hospital costs and poorer outcomes than a reference population of adult CABG patients. Recognition and treatment of comorbidities in ACHD patients undergoing cardiac surgery may provide an opportunity to improve perioperative outcomes in this growing patient population.

PMID: 28669107 [PubMed - as supplied by publisher]

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Spontaneous coronary artery dissection and its association with heritable connective tissue disorders.

Heart. 2016 Jun 01;102(11):876-81

Authors: Henkin S, Negrotto SM, Tweet MS, Kirmani S, Deyle DR, Gulati R, Olson TM, Hayes SN

OBJECTIVE: Spontaneous coronary artery dissection (SCAD) is an under-recognised but important cause of myocardial infarction and sudden cardiac death. We sought to determine the role of medical and molecular genetic screening for connective tissue disorders in patients with SCAD.
METHODS: We performed a single-centre retrospective descriptive analysis of patients with spontaneous coronary artery disease who had undergone medical genetics evaluation 1984-2014 (n=116). The presence or absence of traits suggestive of heritable connective tissue disease was extracted. Genetic testing for connective tissue disorders and/or aortopathies, if performed, is also reported.
RESULTS: Of the 116 patients (mean age 44.2 years, 94.8% women and 41.4% with non-coronary fibromuscular dysplasia (FMD)), 59 patients underwent genetic testing, of whom 3 (5.1%) received a diagnosis of connective tissue disorder: a 50-year-old man with Marfan syndrome; a 43-year-old woman with vascular Ehlers-Danlos syndrome and FMD; and a 45-year-old woman with vascular Ehlers-Danlos syndrome. An additional 12 patients (20.3%) had variants of unknown significance, none of which was thought to be a definite disease-causing mutation based on in silico analyses.
CONCLUSIONS: Only a minority of patients with SCAD who undergo genetic evaluation have a likely pathogenic mutation identified on gene panel testing. Even fewer exhibit clinical features of connective tissue disorder. These findings underscore the need for further studies to elucidate the molecular mechanisms of SCAD.

PMID: 26864667 [PubMed - indexed for MEDLINE]

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[Congenitally corrected transposition of the great arteries in patient with atrial septal defect and acute inferior segment myocardial infarction].

Turk Kardiyol Dern Ars. 2016 Sep;44(6):517-20

Authors: Tosun V, Korucuk N, Karakaş MM, Güntekin Ü

Congenitally corrected transposition of the great arteries is a rare abnormality accounting for approximately 1% of clinically apparent congenital heart disease. Age at time of diagnosis and survival rate vary and depend on associated anomalies, including pulmonary stenosis, ventricular septal defect, atrial septal defect, atrioventricular block, and atrioventricular valve regurgitation. Reported cases of corrected transposition of the great arteries with single coronary ostium anomaly and atrial septal defect are very rare. Described in the present report is the case of a 55-year-old male who presented with acute inferior ST-segment elevation myocardial infarction and coincidental single coronary ostium arising from the right sinus of Valsalva, as observed on coronary angiography. Successful balloon angioplasty and stenting of the circumflex artery were performed. Echocardiography demonstrated the corrected transposition of the great arteries with negative contrast enhancement between the atrial chambers. The patient was discharged with medical therapy on the eighth postoperative day. To our knowledge, the present is the first report to describe corrected transposition of the great arteries, atrial septal defect, single coronary ostium, and acute myocardial infarction as comorbidities.

PMID: 27665336 [PubMed - indexed for MEDLINE]

Illness perceptions in adult congenital heart disease: A multi-center international study.

Int J Cardiol. 2017 Jun 26;:

Authors: Rassart J, Apers S, Kovacs AH, Moons P, Thomet C, Budts W, Enomoto J, Sluman MA, Wang JK, Jackson JL, Khairy P, Cook SC, Subramanyan R, Alday L, Eriksen K, Dellborg M, Berghammer M, Johansson B, Rempel GR, Menahem S, Caruana M, Veldtman G, Soufi A, Fernandes SM, White KS, Callus E, Kutty S, Luyckx K, APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD)

BACKGROUND: Illness perceptions are cognitive frameworks that patients construct to make sense of their illness. Although the importance of these perceptions has been demonstrated in other chronic illness populations, few studies have focused on the illness perceptions of adults with congenital heart disease (CHD). This study examined (1) inter-country variation in illness perceptions, (2) associations between patient characteristics and illness perceptions, and (3) associations between illness perceptions and patient-reported outcomes.
METHODS: Our sample, taken from APPROACH-IS, consisted of 3258 adults with CHD from 15 different countries. Patients completed questionnaires on illness perceptions and patient-reported outcomes (i.e., quality of life, perceived health status, and symptoms of depression and anxiety). Patient characteristics included sex, age, marital status, educational level, employment status, CHD complexity, functional class, and ethnicity. Linear mixed models were applied.
RESULTS: The inter-country variation in illness perceptions was generally small, yet patients from different countries differed in the extent to which they perceived their illness as chronic and worried about their illness. Patient characteristics that were linked to illness perceptions were sex, age, employment status, CHD complexity, functional class, and ethnicity. Higher scores on consequences, identity, and emotional representation, as well as lower scores on illness coherence and personal and treatment control, were associated with poorer patient-reported outcomes.
CONCLUSIONS: This study emphasizes that, in order to gain a deeper understanding of patients' functioning, health-care providers should focus not only on objective indicators of illness severity such as the complexity of the heart defect, but also on subjective illness experiences.

PMID: 28669511 [PubMed - as supplied by publisher]