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Persistent left superior vena cava: An unusual cause of curable pulmonary hypertension.

Diagn Interv Imaging. 2018 Jan;99(1):47-48

Authors: Demeyere M, Delacour D, Bouchart F, Michelin P, Bauer F, Dubourg B, Dacher JN

PMID: 28919169 [PubMed - indexed for MEDLINE]

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Echogenic intracardiac foci and fetal cardiac anomalies: A review of cases from a tertiary care center in China.

J Clin Ultrasound. 2018 Feb;46(2):103-107

Authors: Guo Y, He Y, Gu X, Zhang Y, Sun L, Liu X, Zhao Y, Han J

PURPOSE: To investigate the relationship between the location of echogenic intracardiac foci (EIFs) and fetal cardiac anomalies in our patient population.
METHODS: We performed a retrospective study of the clinical files of fetuses who underwent an echocardiographic examination at our fetal cardiac medical center from August 2010 to August 2016. Fetuses were grouped by EIF location (left ventricle [LV], right ventricle [RV], or bilateral ventricles [BVs]). The maternal age, reason for referral, and cardiac anomalies in the three groups were analyzed.
RESULTS: We enrolled 2647 fetuses with an EIF. Of these, 2498 (94.4%) were in the LV group, while 45 (1.7%) and 104 (3.9%) were in the RV and BV groups, respectively. Aneuploidy was found in 3 fetuses, and all of these had a left-sided EIF. Cardiac anomalies were found in 93/2498 (3.7%) fetuses with a left-sided EIF, 5/45 (11.1%) of those with a right-sided EIF, and 3/104 (2.9%) of those with bilateral EIFs. The prevalence of congenital heart disease was significantly higher in fetuses with a right-sided EIF than in those with left-sided or bilateral EIFs (P < .05).
CONCLUSIONS: Right-sided EIFs were more frequently associated with fetal cardiac anomalies than were left-sided or bilateral EIFs.

PMID: 28961313 [PubMed - indexed for MEDLINE]

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Histopathological abnormalities in the central arteries and veins of Fontan subjects.

Heart. 2018 02;104(4):324-331

Authors: Hays BS, Baker M, Laib A, Tan W, Udholm S, Goldstein BH, Sanders SP, Opotowsky AR, Veldtman GR

OBJECTIVE: Fontan circulations have obligatory venous hypertension, depressed cardiac output and abnormal arterial elastance. Ventriculovascular coupling is known to be abnormal, but the underlying mechanisms are poorly defined. We aim to describe the histopathological features of vascular remodelling encountered in the central arteries and veins in the Fontan circulation as a possible underlying pathological representation of abnormal ventriculovascular coupling.
METHODS: Postmortemvasculature (inferior vena cava (IVC), superior vena cava (SVC), pulmonary artery (PA), pulmonary vein (PV) and aorta) of 13 patients with a Fontan circulation (mean age 29.9 years, range 9.0-59.8 years) and 2 biventricular controls (ages 17.9 and 30.2 years) was examined.
RESULTS: IVC and SVC: Eccentric and variable intimal fibromuscular proliferation occurred in 11 Fontan subjects. There was variable loss of medial smooth muscle bundles with reciprocal replacement with dense collagenous tissue.PA: Similar intimal fibromuscular proliferation was seen; however, these intimal changes were accompanied by medial thinning rather than expansion, medial myxoid degeneration and elastic alteration.PV: The PVs demonstrated intimal fibroproliferation and disorganisation of the muscular media.Aorta: The aortic lamina intima was thickened, with associated fibromuscular proliferation and elasticisation. There was also moderate lymphocytic inflammation in the aortic wall.
CONCLUSIONS: Vascular architectural remodelling is common in Fontan patients. The central veins demonstrate profound changes of eccentric intimal expansion and smooth muscle replacement with collagen. The pulmonary demonstrated abnormal intimal proliferation, and aortic remodelling was characterised by intima lamina thickening and a moderate degree of aortic wall inflammation.

PMID: 28970278 [PubMed - indexed for MEDLINE]

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Strength training in congenital heart disease: A way to boost respiratory function?

Eur J Prev Cardiol. 2018 Nov 14;:2047487318812505

Authors: Ntelios D, Giannakoulas G, Dimopoulos K

PMID: 30426770 [PubMed - as supplied by publisher]

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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

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

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

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

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

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

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]