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Morphology and mechanisms of a novel absorbable polymeric conduit in the pulmonary circulation of sheep.

Cardiovasc Pathol. 2018 Oct 25;38:31-38

Authors: Brugmans M, Serrero A, Cox M, Svanidze O, Schoen FJ

Abstract
BACKGROUND: Right ventricular outflow tract (RVOT) conduits used in children with congenital heart disease often degenerate rapidly or develop other complications, and they do not grow with the patient. This leads to multiple surgeries until adult-sized conduits can be implanted. We report experimental in vivo experience with an entirely synthetic absorbable graft, designed to be replaced by tissue in-vivo by host cells, in a process termed Endogenous Tissue Restoration (ETR), and to grow commensurate with somatic growth.
METHODS: We characterized the structure, mechanical properties, biocompatibility, and in vivo remodelling of a bioabsorbable polyester based on the self-complementary ureido-pyrimidinone (UPy) quadruple hydrogen-bonding motif. Electrospinning was used to process the polymer into a tubular graft with a highly porous wall structure, which was implanted as a pulmonary artery interposition graft in 9 adult sheep with a maximum follow-up of 1 year, followed by pathologic and mechanical analysis.
RESULTS: All grafts were patent by transthoracic echocardiography. Eight were intact at post-mortem examination. One graft had aneurysmal dilation. Graft polymer resorption in vivo was consistent among specimens. Histologic examination revealed progressive tissue replacement of graft polymer, ongoing at one year, with remodeling to a structure that had some key features of native vascular wall. Burst pressures for all explants at 8 weeks and beyond were higher than those of native pulmonary artery (PA) and largely determined by newly formed tissue.
CONCLUSIONS: Preclinical studies of a new, absorbable polymeric graft for PA replacement showed remodelling by endogenous cells up to one-year follow-up. Our results show that ETR leads to progressive and substantial replacement of an off-the-shelf synthetic bioabsorbable conduit by functional host tissue to one year in sheep. Thus, further development of this novel concept is warranted.

PMID: 30428421 [PubMed - as supplied by publisher]

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In-frame Variants in FLNA Proximal Rod 1 Domain Associate With a Predominant Cardiac Valvular Phenotype.

Rev Esp Cardiol (Engl Ed). 2018 Jul;71(7):545-552

Authors: Fernández L, Tenorio J, Polo-Vaquero C, Vallespín E, Palomares-Bralo M, García-Miñaúr S, Santos-Simarro F, Arias P, Carnicer H, Giannivelli S, Medina J, Pérez-Piaya R, Solís J, Rodríguez M, Villagrá A, Rodríguez L, Nevado J, Martínez-Glez V, Heath KE, Lapunzina P

Abstract
INTRODUCTION AND OBJECTIVES: X-linked cardiac valvular dysplasia is a rare form of male-specific congenital heart defect mainly characterized by myxomatous degeneration of the atrioventricular valves with variable hemodynamic consequences. It is caused by genetic defects in FLNA-encoded filamin A, a widely expressed actin-binding protein that regulates cytoskeleton organization. Filamin A loss of function has also been associated with often concurring neurologic and connective tissue manifestations, with mutations in the first half of the Rod 1 domain apparently expressing the full cardiac phenotype. We contribute to previous genotype-phenotype correlations with a multidisciplinary approach in a newly-described family.
METHODS: Cardiologic, dysmorphologic, and genetic evaluation of available members were complemented with transcriptional and X-chromosome inactivation studies.
RESULTS: A novel FLNA mutation c.1066-3C>G cosegregated with a male-expressed, apparently isolated, cardiac phenotype with no skewed X-inactivation pattern in female carriers. This variant was shown to result in an in-frame deletion of 8 amino acid residues near the N-terminal region of the protein.
CONCLUSIONS: A nonimprinted, partial loss of function of filamin A proximal Rod 1 domain seems to be the pathogenetic mechanism of cardiac valvular dysplasia, with some cases occasionally expressing associated extracardiac manifestations.

PMID: 29146485 [PubMed - indexed for MEDLINE]

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Does statin therapy impact the proximal aortopathy in aortic valve disease?

QJM. 2018 Sep 01;111(9):623-628

Authors: Sequeira Gross T, Naito S, Neumann N, Petersen J, Kuntze T, Reichenspurner H, von Kodolitsch Y, Girdauskas E

Abstract
Background: Studies have demonstrated that statin therapy decreases the growth rate of abdominal aneurysms. However, the effect of statin therapy on the proximal aortic disease has not been sufficiently elucidated.
Aim: We aimed to analyse the association between statin treatment and the severity of proximal aortopathy in patients with aortic valve disease.
Design: Cross-sectional study.
Methods: We prospectively identified 458 patients who were referred for aortic valve surgery from 2008 to 2014. Pre-operative measurement of the proximal aorta was performed by TEE, CT or MRT scan. Data of dyslipidemia treatment was obtained by questionnaire.
Results: The mean ascending aortic diameter in the whole study population was comparable in patients with vs. without statin therapy (i.e. 42.7 mm vs. 43.6 mm, P = 0.46). Logistic regression analysis showed no significant association between statin therapy and proximal aortopathy ≥ 40 mm in the whole study group (OR = 0.69, P = 0.10). For the BAV sub-group, HDL (OR = 0.54, P = 0.038) and cholesterol levels (OR = 2.00, P = 0.038) were found significantly associated with the proximal aortic disease. In the BAV cohort, the statin users with target HDL levels presented a significantly smaller proximal aortic diameter (40.1 mm vs. 46 mm, P = 0.02).
Conclusion: Pre-operative statin therapy demonstrated no significant association with the expression of proximal aortopathy. However, more than 40% of the statin users presented uncontrolled lipid levels at the time of the study. In the BAV sub-group, the statins users with target HDL levels showed a significantly smaller ascending aorta diameter. Target HDL and cholesterol levels were strongly associated with proximal aortic dilation in BAV patients.

PMID: 29917097 [PubMed - indexed for MEDLINE]

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Rare cause for a common presentation: isolated pulmonary valve endocarditis yet another mimicker.

BMJ Case Rep. 2018 Jul 18;2018:

Authors: Reza AS, Anand D, Cheng SH, Anand D

Abstract
Isolated pulmonary valve endocarditis (PVE) is a rare condition. Known risk factors in previous case reports were intravenous drug abuse and congenital heart disease. Epidemiology of PVE has been changing. It is now being reported specially following invasive healthcare-related procedures even in patients with structurally normal heart. Vast majority of patients present with respiratory symptoms and diagnosis of endocarditis may be challenging unless there is high index of suspicion. Various microorganisms had been isolated as aetiological agents; however, Enterococcus faecalis is being increasingly isolated. PVE mostly managed conservatively with intravenous antibiotics with option for surgical intervention in specific situations. We present a patient with isolated PVE due to E. faecalis following colonoscopy and polypectomy with predisposing risk factor of alcohol excess, mimicking clinically as pneumonia and radiologically as a neoplastic lesion.

PMID: 30021734 [PubMed - indexed for MEDLINE]

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Effects of Hepatitis C Virus Antibody-Positivity on Cardiac Function and Long-Term Prognosis in Patients With Adult Congenital Heart Disease.

Am J Cardiol. 2018 Dec 01;122(11):1965-1971

Authors: Konno R, Tatebe S, Sugimura K, Satoh K, Aoki T, Miura M, Yamamoto S, Sato H, Kozu K, Terui Y, Miyata S, Adachi O, Kimura M, Saiki Y, Shimokawa H

Abstract
It was reported that hepatitis C virus (HCV) antibody-positivity adversely affects cardiac function. As the screening for HCV began in 1992, we hypothesized that HCV antibody-positive rate would be high in adult congenital heart disease (ACHD) patients who underwent heart surgery before 1992 and adversely affected cardiac function and long-term prognosis. We retrospectively enrolled 243 ACHD patients (mean age 25.9 years) who underwent cardiac surgery before 1992 and visited our hospital from 1995 to 2015. We compared clinical characteristics including cardiac function and long-term prognosis between HCV antibody-positive (n = 48) and antibody-negative (n = 195) patients. The composite end point (CEP) included cardiac death, heart failure hospitalization, lethal ventricular arrhythmias, and cardiac reoperation. The prevalence of reduced systemic ventricular ejection fraction <50% was significantly higher in the HCV antibody-positive group compared with the HCV antibody-negative group (17 vs 5.4%, p = 0.014). During a mean follow-up period of 10.1 years (interquartile range 6 to 14 years), the CEP was noted in 51 patients. Kaplan-Meier analysis showed the HCV antibody-positive group had significantly poor event-free survival than the HCV antibody-negative group (log-rank, p = 0.002). In contrast, HCV ribonucleic acid-positivity was not a significant predictor of the CEP in the HCV antibody-positive group (log-rank, p = 0.442). Furthermore, the HCV antibody-positivity was significantly associated with the CEP in both univariable and multivariable Cox regression models (hazard ratio 2.37, 95% confident interval 1.32 to 4.15, p = 0.005 and 1.96, 1.06 to 3.63, p = 0.032, respectively). In conclusion, these results suggest that more attention should be paid to HCV antibody-positivity in the management of ACHD patients.

PMID: 30442226 [PubMed - in process]

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Echocardiography in Congenital Heart Disease.

Prog Cardiovasc Dis. 2018 Nov 13;:

Authors: Mcleod G, Shum K, Gupta T, Chakravorty S, Kachur S, Bienvenu L, White M, Shah SB

Abstract
As the prevalence of adult congenital heart disease continues to grow secondary to advances in surgical and diagnostic techniques, it is important for a physician to supplement their examinations with non-invasive imaging techniques to assess their patients. Although a number of these patients have regular cardiology followup, some may be new patients that do not even know their cardiac history. Echocardiography has proven to be a useful tool for this purpose and its utility has expanded drastically with the development of better technology and newer techniques. In this article, we highlight some of these advancements including 2D echocardiography, agitated saline, contrast echocardiography, stress, and 4D, in addition to how each modality can help assess key aspects of the structure and function of a congenital heart defect.

PMID: 30445162 [PubMed - as supplied by publisher]

Low Gradient Aortic Stenosis: Who Benefits from Intervention?

JACC Cardiovasc Imaging. 2018 Nov 08;:

Authors: Baumgartner H

PMID: 30448121 [PubMed - as supplied by publisher]

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Letter regarding Groves et al. "Feasibility of low radiation dose retrospectively-gated cardiac CT for functional analysis in adult congenital heart disease".

Int J Cardiol. 2019 Jan 01;274:402

Authors: Sun C, Yu B, Li R, Shi Y, Wang W, Leng T, Lv J, Wang X

PMID: 30449336 [PubMed - in process]

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Heart Failure in Women with Congenital Heart Disease.

Heart Fail Clin. 2019 Jan;15(1):87-96

Authors: Bradley EA, Saraf A, Book W

Abstract
Heart failure remains the most common cause of morbidity and mortality in adults with congenital heart disease (CHD). Although gender-specific outcomes are not robust, it seems that women with CHD may be more affected by late heart failure (HF) than men. A specialized and experienced adult CHD team is required to care for these women as they age, including assessment for reversible causes of HF and in the management of pregnancy, labor, and delivery.

PMID: 30449383 [PubMed - in process]

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Ventricular Septal Defect Closure Devices, Techniques, and Outcomes.

Interv Cardiol Clin. 2019 Jan;8(1):1-10

Authors: Morray BH

Abstract
Transcatheter closure of ventricular septal defects (VSDs) was first documented in 1988. The early studies of VSD closure were successful but there were high complication rates, particularly early and late-onset complete heart block. However, the development and use of new vascular occlusion devices in an off-label fashion has improved the range of patients who can be treated and reduced the complication rates. In particular, the rate of complete heart block documented in contemporary studies of VSD closure has fallen to levels at or below those documented in the surgical VSD closure literature.

PMID: 30449417 [PubMed - in process]

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