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Infective endocarditis: Refer for expert team care as soon as possible.

Cleve Clin J Med. 2018 May;85(5):365-366

Authors: Pettersson GB, Griffin B, Gordon SM, Blackstone EH

PMID: 29733779 [PubMed - in process]

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Is Fractional Flow Reserve Useful in Assessing Coronary Artery Fistula: A Case-Based Discussion and Review.

Heart Lung Circ. 2018 Apr 19;:

Authors: Hayman S, Goela A, Lavi S

We present two cases where fractional flow reserve (FFR) was utilised to guide management of coronary artery fistula (CAF), an approach advocated in recent case studies. CAF is a coronary anomaly that may present with a variety of syndromes though is frequently asymptomatic. When to exclude the fistula (surgically or percutaneously) is not always clear. A way to quantify if the fistula is physiologically meaningful would be advantageous. Our findings suggest FFR may only be assessing the concomitant epicardial coronary artery disease (CAD) rather than the degree of coronary steal and its routine use in this setting is not supported.

PMID: 29735395 [PubMed - as supplied by publisher]

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Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry.

J Heart Lung Transplant. 2017 Jul;36(7):770-779

Authors: Sithamparanathan S, Nair A, Thirugnanasothy L, Coghlan JG, Condliffe R, Dimopoulos K, Elliot CA, Fisher AJ, Gaine S, Gibbs JSR, Gatzoulis MA, E Handler C, Howard LS, Johnson M, Kiely DG, Lordan JL, Peacock AJ, Pepke-Zaba J, Schreiber BE, Sheares KKK, Wort SJ, Corris PA, National Pulmonary Hypertension Service Research Collaboration of the United Kingdom and Ireland

BACKGROUND: Portopulmonary hypertension (PoPH) is a rare condition associated with poor survival, and the effect of modern therapies that target pulmonary arterial hypertension (PAH) on long-term outcome is unknown. This study investigated the baseline characteristics and survival in the cohort of patients diagnosed with PoPH in the United Kingdom National Pulmonary Hypertension Service.
METHODS: A retrospective review was conducted of all incident treatment-naïve patients with PoPH within the United Kingdom national registry diagnosed between January 2001 and December 2010.
RESULTS: Patients with PoPH (n = 110) had survival rates of 85%, 60%, and 35% at 1, 3, and 5 years. The prevalence of PoPH was 0.85 cases/1 million. Mean age at diagnosis was 53 ± 12 years, with a balanced distribution in gender. Alcohol (n = 57) and hepatitis C (n = 10) were the most common causes of portal hypertension. Phosphodiesterase V inhibitors were the most frequently used targeted therapy, in 63.6% (n = 70) of patients, endothelin receptor antagonists were used in 10% (n = 11) and prostacyclin analogs in 12.7% (n = 14). Univariate and multivariate analysis of baseline characteristics did not demonstrate a significant influence of severity of portal hypertension or liver cirrhosis, World Health Organization Functional Class, cardiopulmonary hemodynamics, or year of diagnosis on survival.
CONCLUSIONS: Survival of patients with PoPH remains poor despite targeted therapy and worse than patients with idiopathic PAH. The benefit of PAH therapies in PoPH on long-term morbidity and mortality outcomes needs further consideration and study.

PMID: 28190786 [PubMed - indexed for MEDLINE]

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Edwards valve-in-valve implantation in tricuspid position.

Cardiol Young. 2017 Oct;27(8):1633-1636

Authors: Álvarez-Fuente M, Haas NA, Del Cerro MJ

We present two cases of percutaneous Sapien XT valve-in-valve implantation in the tricuspid position: a 20-year-old man with severe congenital pulmonary stenosis and percutaneous valvuloplasty, who required surgical implantation of two protheses, pulmonary and tricuspid, and a 12-year-old boy with CHD and a degenerated tricuspid prosthesis. We implanted three Sapien XT valve-in-valves, two in the tricuspid position and one in the pulmonic position. Sapien XT valve-in-valve implantation in the tricuspid position is feasible and can decrease the number of surgeries in CHD patients.

PMID: 28506330 [PubMed - indexed for MEDLINE]

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Delayed recurrent pleuropericardial effusions following atrial septal defect closure with Amplatzer septal occluder device: a case of postpericardiotomy syndrome?

BMJ Case Rep. 2018 May 08;2018:

Authors: Kwok GG, Witts R, Frigiola A

The authors present the case of a 41-year-old woman, who presented with moderate pericardial effusion and bilateral pleural effusion 11 months following a secundum atrial septal defect closure with an Amplatzer septal occluder (ASO) device. The acute presentation responded well to diuretics and a short course of corticosteroid therapy. The patient, however, continued to experience pleuritic chest pain and a recurrence of pericardial effusion a month later. The patient showed significant symptomatic improvement with a 2-month course of down-titrating prednisolone. Six months later, the echocardiogram showed complete remission of pleural and pericardial effusion. The possibility of cardiac erosion following ASO implantation has been excluded. The aetiology of the reoccurrence of steroid-responsive pleuropericarditis remains unknown.

PMID: 29739762 [PubMed - in process]

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Postnatal and Adult Aortic Heart Valves Have Distinctive Transcriptional Profiles Associated With Valve Tissue Growth and Maintenance Respectively.

Front Cardiovasc Med. 2018;5:30

Authors: Nordquist E, LaHaye S, Nagel C, Lincoln J

Heart valves are organized connective tissues of high mechanical demand. They open and close over 100,000 times a day to preserve unidirectional blood flow by maintaining structure-function relationships throughout life. In affected individuals, structural failure compromises function and often leads to regurgitant blood flow and progressive heart failure. This is most common in degenerative valve disease due to age-related wear and tear, or congenital malformations. At present, the only effective treatment of valve disease is surgical repair or replacement and this is often impermanent and requires anti-coagulation therapy throughout life. Therefore, there is a critical need to discover new alternatives. A promising therapeutic area is tissue regeneration and in non-valvular tissues this requires a tightly regulated genetic "growth program" involving cell proliferation. To explore this in heart valves, we performed RNA-seq analysis to compare transcriptional profiles of aortic valve tissue isolated from mice during stages of growth (postnatal day (PND) 2) and adult maintenance (4 months). Data analysis reveals distinct mRNA profiles at each time point and pathway ontology identifies associated changes in biological functions. The PND2 aortic valve is characterized by extensive cell proliferation and expression of mRNAs related to the extracellular matrix (ECM). At 4 months, proliferation is not significant and a differential set of ECM-related genes are expressed. Interestingly there is enrichment of the defense response biological process at this later time point. Together, these data highlight the unique transcriptome of the postnatal valve during stages of growth and maturation, as well as biological functions associated with adult homeostatic valves. These studies create a platform for future work exploring the molecular programs altered in the onset of heart valve disease after birth and provide insights for the development of mechanistic-based therapies.

PMID: 29740591 [PubMed]

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Diagnostic Utility of Three-Dimensional Rotational Angiography in Congenital Cardiac Catheterization.

Pediatr Cardiol. 2016 Oct;37(7):1211-21

Authors: Aldoss O, Fonseca BM, Truong UT, Bracken J, Darst JR, Guo R, Jones TL, Fagan TE

We evaluated the diagnostic utility of the three modalities of three-dimensional rotational angiography (3DRA): rotational angiography (RA), multiplanar reformat (MPR) and three-dimensional angiographic reconstruction (3D-R) in pediatric cardiac catheterization. The 3DRA studies were classified by anatomy of interest based on our injection protocol: pulmonary arteries (PA), aorta, cavopulmonary connection (CPC), and others. Retrospective review of 3DRA images by two reviewers for each modality was conducted with grading as inferior, similar, or superior in comparison with the diagnostic quality of fixed-plane angiography (FPA). The percentages of grades for each modality were averaged. Weighted kappa statistic was used to evaluate inter-rater reliability. In total, 114 3DRA studies were performed on 87 patients between August 2010 and March 2012. Median age was 2.7 years (1 day-48.4 years) and median weight 12.1 kg (3.6-106.5 kg). For RA: 79.4 % of the studies were of diagnostic quality and 52.2 % were superior; 3D-R: 82 % were of diagnostic quality and 65.8 % were superior; and MPR: 83.5 % were of diagnostic quality and 63 % were superior. Overall 3DRA technologies (RA, 3D-R, MPR) were of diagnostic quality or better in 111/114 (97.4 %) studies and 103/114 (90.4 %) were judged superior. Most common reasons for inferior grading were limited opacification and metallic artifact. In pediatric cardiac catheterization, 3DRA imaging was of diagnostic quality and frequently provided additional clinically relevant data when compared to FPA.

PMID: 27278632 [PubMed - indexed for MEDLINE]

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Holodiastolic Flow Reversal at the Descending Aorta on Cardiac Magnetic Resonance is Neither Sensitive Nor Specific for Significant Aortic Regurgitation in Patients with Congenital Heart Disease.

Pediatr Cardiol. 2016 Oct;37(7):1284-9

Authors: Avitabile CM, Whitehead KK, Fogel MA, Kim DW, Kim TS, Rose JD, Keller MS, Fu GL, Harris MA

Holodiastolic flow reversal in the descending aorta on echocardiogram suggests significant aortic regurgitation. The study aim was to determine whether the presence of holodiastolic flow reversal on cardiac magnetic resonance imaging (MRI) correlates with aortic valve regurgitant fraction. We retrospectively reviewed 166 cardiac MRIs (64 % male, age 14.1 ± 9.5 years) from January 2011 to May 2012 where velocity mapping was acquired at both the aortic valve and the descending aorta at the level of the diaphragm. Descending aorta velocity maps were checked for baseline offset using a static reference region. Holodiastolic flow reversal was defined as flow reversal throughout diastole both before and after baseline correction. Significant aortic regurgitation was defined as regurgitant fraction >10 %. Aortic valve regurgitant fraction was <10 % in 144 patients (Group A), 10-20 % inclusive in 7 patients (Group B), and >20 % in 15 patients (Group C). Though the aortic valve regurgitant fraction was significantly higher for patients with holodiastolic flow reversal versus those without (8.5 ± 14.2 vs. 3.8 ± 6.6 %, p = 0.02), holodiastolic flow reversal was present in 32 Group A patients (22 %). In comparison, 4 Group B patients (57 %) and 7 Group C patients (47 %) had holodiastolic flow reversal. The sensitivity (Groups B and C) was 0.5, and the specificity (Group A) was 0.78. Holodiastolic flow reversal in the descending aorta on cardiac MRI was neither sensitive nor specific for predicting significant aortic regurgitation in this study population. Holodiastolic flow reversal in the absence of significant aortic regurgitation may be a relatively common finding in patients with congenital heart disease.

PMID: 27312779 [PubMed - indexed for MEDLINE]

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Predictors of Change in Functional Health Status in Adults with Repaired Tetralogy of Fallot.

Pediatr Cardiol. 2016 Oct;37(7):1334-9

Authors: Lu JC, Yu S, Lowery R, Sagi J, Delong AC, Agarwal PP, Mahani MG, Dorfman AL

Left ventricular (LV) ejection fraction (EF) and right ventricular (RV) ejection fraction by cardiovascular magnetic resonance (CMR) are associated with functional health status in patients with repaired tetralogy of Fallot (TOF) in cross-sectional studies, but few longitudinal data are available. This study aimed to determine predictors of subsequent decrease in functional health status in midterm follow-up. Patients with repaired TOF who had previously completed CMR and assessment with the Short Form 36 version 2 (SF-36) were recruited for repeat CMR, SF-36, and exercise test, if they had not had interval pulmonary valve replacement (PVR). Patients from the same cohort who had undergone PVR were recruited for repeat SF-36. A total of 19 patients (median 33.5 years old, interquartile range [IQR] 26-42 years, 53 % male) had not undergone PVR and were enrolled at a median of 5.0 years (IQR 4.8-5.3) since prior CMR and SF-36. LVEF and RVEF did not change from baseline, while RV end-diastolic volume increased (138 ± 34 vs. 126 ± 31 ml/m(2), p = 0.02). In the overall cohort, SF-36 scores remained stable. However, higher baseline RV end-systolic volume and pulmonary regurgitant fraction correlated with subsequent decreases in SF-36 scores. In 9 patients post-PVR (median 35.9 years old, IQR 24-43), physical functioning increased compared to those without PVR (change in z-score +0.59 ± 0.59 vs. -0.26 ± 0.72, p = 0.005). In adults with repaired TOF who do not undergo PVR, LVEF, RVEF, and functional health status remain stable in midterm follow-up. However, baseline RV end-systolic volume correlates with subsequent change in functional health status, underscoring its importance in prognostication and timing of intervention in this population.

PMID: 27371413 [PubMed - indexed for MEDLINE]

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Noninvasive Assessment of Pulmonary Artery Pressure in Patients with Extracardiac Conduit Total Cavopulmonary Connection.

Pediatr Cardiol. 2016 Oct;37(7):1361-9

Authors: Ajami GH, Mohammadi H, Amirghofran AA, Borzouee M, Amoozgar H, Cheriki S, Edraki MR, Mehdizadegan N, Arabi H, Alvasabi F, Naghshzan A

To assess quantitative measurement of mean pulmonary artery pressure (PAP) in extracardiac total cavopulmonary connection (TCPC) patients by noninvasive echocardiographic inferior vena cava collapsibility index (IVC-CI) and also correlation between the peripheral vein pressure and mean PAP. In 19 TCPC patients with at least 1-year follow-up after completion of TCPC, complete echocardiography including IVC-CI was recorded. All patients underwent cardiac catheterization for mean PAP pressure, peripheral vein pressure (PVP) and contrast study. Different cutoff points of mean PAP were analyzed, and based on the highest cutoff point, patients were categorized into two groups: mean PAP < 17 mmHg (acceptable) and > = 17 mmHg (high) for comparison analysis. Between 2006 and 2015, from 43 patients who had undergone TCPC, nineteen patients with a mean age 12.9 ± 6.6 years and mean follow-up time of 3.4 ± 1.9 years were included in this study. Mean PAP was 14.6 ± 3.97 (range 8-22 mmHg). Thirteen (68 %) patients had PAP < 17 mmHg. No significant statistical difference was detected with respect to age, sex, type of congenital heart disease and fenestration between these two groups. But, right ventricular dominancy was more prevalent in the high PAP group (50 % vs. 7.7 % P value 0.03). IVC-CI had a correlation with mean PAP (r 0.67, P < 0.001). IVC-CI < 21.8 % can predict PAP > = 17 mmHg with 83 % sensitivity and 100 % specificity. Regression analysis proposed an equation for PAP measurement: PAP (mmHg) = 20.2097-0.1796 × (IVC-CI), (r2 = 0.56). Peripheral vein pressure measurement also showed a good correlation with mean PAP and may be used to estimate PAP with the following equation: PAP (mmHg) = 0. 8675 × PVP, (r 0.90, P < 0.0001). In conclusion, IVC-CI as noninvasive and peripheral vein pressure measurement as a minimal invasive method may be useful for quantitative estimation of PAP in patients with extracardiac TCPC.

PMID: 27377526 [PubMed - indexed for MEDLINE]