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Unsuccessful transjugular intrahepatic portosystemic shunt for a patient with right heart failure and portal hypertension.

Acta Gastroenterol Belg. 2017 Jan-Mar;80(1):63-66

Authors: Graus L, Verresen L, De Vusser P, Verbrugge FH, Caenepeel P

Abstract
A 60-year-old women with a history of congenital pulmonary valve stenosis developed right heart failure, cardiac cirrhosis and end-stage renal disease requiring renal replacement therapy. Cirrhosis was complicated by portal hypertension, resulting in intractable gastro-intestinal bleedings despite optimal treatment with beta-blockers and endoscopic band ligation. Because of fears for worsening right heart failure, a decision for placement of a transjugular intrahepatic portosystemic shunt (TIPS) was initially turned down. However, as intractable bleeding problems persisted and caused heavy transfusion needs, TIPS was ultimately performed as a rescue procedure. Although TIPS successfully reduced the hepatic venous pressure gradient from 16 mmHg to 4 mmHg, portal pressure remained high at 14 mmHg because of persisting right heart failure with elevated central venous pressure. Hepatic encephalopathy soon developed after TIPS placement and culminated in multi-organ failure after another episode of gastro-intestinal bleeding. At this point, the family of the patient decided to withdraw care and the patient died subsequently. This case illustrates how important it is to diagnose and optimally treat right heart failure before cardiac cirrhosis with its impending complications emerges. Although TIPS may effectively treat complications of portal hypertension in the context of cirrhosis, persisting right heart failure may abrogate its beneficial effects.

PMID: 29364100 [PubMed - indexed for MEDLINE]

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Prognostic Value of Serial N-Terminal Pro-B-Type Natriuretic Peptide Measurements in Adults With Congenital Heart Disease.

J Am Heart Assoc. 2018 Mar 26;7(7):

Authors: Baggen VJM, Baart SJ, van den Bosch AE, Eindhoven JA, Witsenburg M, Cuypers JAAE, Roos-Hesselink JW, Boersma E

Abstract
BACKGROUND: A single NT-proBNP (N-terminal pro-B-type natriuretic peptide) measurement is a strong prognostic factor in adult congenital heart disease. This study investigates NT-proBNP profiles within patients with adult congenital heart disease and relates these to cardiovascular events.
METHODS AND RESULTS: In this prospective cohort, 602 patients with adult congenital heart disease were enrolled at the outpatient clinic (years 2011-2013). NT-proBNP was measured at study inclusion in 595 patients (median age 33 [IQR 25-41] years, 58% male, 90% NYHA I) and at subsequent annual visits. The primary end point was defined as death, heart failure, hospitalization, arrhythmia, thromboembolic event, or cardiac intervention; the secondary end point as death or heart failure. Repeated measurements were analyzed using linear mixed models and joint models. During a median follow-up of 4.4 [IQR 3.8-4.8] years, a total of 2424 repeated measurements were collected. Average NT-proBNP increase was 2.9 pmol/L the year before the primary end point (n=199, 34%) and 18.2 pmol/L before the secondary end point (n=58, 10%), compared with 0.3 pmol/L in patients who remained end point-free (P-value for difference in slope 0.006 and <0.001, respectively). In patients with elevated baseline NT-proBNP (>14 pmol/L, n=315, 53%), repeated measurements were associated with the primary end point (HR per 2-fold higher value 2.08; 95% CI 1.31-3.87; P<0.001) and secondary end point (HR 2.47; 95% CI 1.13-5.70; P=0.017), when adjusted for the baseline measurement.
CONCLUSIONS: NT-proBNP increased before the occurrence of events, especially in patients who died or developed heart failure. Serial NT-proBNP measurements could be of additional prognostic value in the annual follow-up of patients with adult congenitive heart disease with an elevated NT-proBNP.

PMID: 29581225 [PubMed - in process]

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Adult Congenital Heart Disease and Radiation Exposure: The Malignant Price of Cardiac Care.

Circulation. 2018 Mar 27;137(13):1346-1348

Authors: Lang NN, Walker NL

PMID: 29581364 [PubMed - in process]

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Current Knowledge and Recent Advances of Right Ventricular Molecular Biology and Metabolism from Congenital Heart Disease to Chronic Pulmonary Hypertension.

Biomed Res Int. 2018;2018:1981568

Authors: Guimaron S, Guihaire J, Amsallem M, Haddad F, Fadel E, Mercier O

Abstract
Studies about pulmonary hypertension and congenital heart diseases have introduced the concept of right ventricular remodeling leading these pathologies to a similar outcome: right ventricular failure. However right ventricular remodeling is also a physiological process that enables the normal fetal right ventricle to adapt at birth and gain its adult phenotype. The healthy mature right ventricle is exposed to low pulmonary vascular resistances and is compliant. However, in the setting of chronic pressure overload, as in pulmonary hypertension, or volume overload, as in congenital heart diseases, the right ventricle reverts back to a fetal phenotype to sustain its function. Mechanisms include angiogenic changes and concomitant increased metabolic activity to maintain energy production. Eventually, the remodeled right ventricle cannot resist the increased afterload, leading to right ventricular failure. After comparing the fetal and adult healthy right ventricles, we sought to review the main metabolic and cellular changes occurring in the setting of PH and CHD. Their association with RV function and potential impact on clinical practice will also be discussed.

PMID: 29581963 [PubMed - in process]

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Analysis of circulating microRNAs in patients with repaired Tetralogy of Fallot with and without heart failure.

J Transl Med. 2017 Jul 10;15(1):156

Authors: Abu-Halima M, Meese E, Keller A, Abdul-Khaliq H, Rädle-Hurst T

Abstract
BACKGROUND: MicroRNAs (miRNAs) are a class of regulatory RNAs that regulate gene expression post-transcriptionally. Little, however, is known on the expression profile of circulating miRNAs in Tetralogy of Fallot (TOF) patients late after surgical repair. In this study, we aimed to identify the specific patterns of circulating miRNAs in blood of patients with repaired, non-syndromic TOF and to assess whether these specific miRNAs may be useful to differentiate patients with and without heart failure.
METHODS: SurePrint™ 8 × 60 K Human v16 miRNA arrays were used to determine miRNA expression profiles in 15 healthy controls and 37 patients after TOF repair of whom 3 had symptomatic right heart failure. The expression levels of selected miRNAs have been validated by quantitative reverse transcription polymerase chain reaction (RT-qPCR). Enrichment analyses of altered miRNA expression were predicted using bioinformatic tools.
RESULTS: Compared with healthy controls, a total of 49, 58 and 77 miRNAs were found to be significantly altered in TOF patients (TOF-all), TOF patients with (TOF-HF) and without symptomatic right heart failure (TOF-noHF) (>2.0-fold change, adjusted P < 0.05), respectively. Three miRNAs namely miR-181d-5p, miR-206 and miR-625-5p were validated by RT-qPCR in all TOF groups. The area under the receiver operating characteristic curve (AUC) for miR-181d-5p, miR-206 and miR-625-5p were 0.987, 0.993 and 0.769 in TOF-all and 0.990, 0.994 and 0.749 in TOF-noHF, respectively. Moreover, expression levels of miR-625-5p, miR-1233-3p and miR-421 were lower in TOF-HF compared to TOF-noHF (P = 0.012).
CONCLUSIONS: Altered expression levels of circulating miRNAs were found in TOF patients late after surgical repair and are different to those seen in the right ventricular myocardium of infants with TOF. Expression levels of miR-421, miR-1233-3p and miR-625-5p are lower in TOF patients with symptomatic right heart failure and thus may indicate disease progression in these patients.

PMID: 28693530 [PubMed - indexed for MEDLINE]

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Risk comparison for prenatal use of analgesics and selected birth defects, National Birth Defects Prevention Study 1997-2011.

Ann Epidemiol. 2017 Oct;27(10):645-653.e2

Authors: Interrante JD, Ailes EC, Lind JN, Anderka M, Feldkamp ML, Werler MM, Taylor LG, Trinidad J, Gilboa SM, Broussard CS, National Birth Defects Prevention Study

Abstract
PURPOSE: To compare the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and/or opioids to the use of acetaminophen without NSAIDs or opioids with respect to associations with birth defects.
METHODS: We used data from the National Birth Defects Prevention Study (1997-2011). Exposure was self-reported maternal analgesic use from the month before through the third month of pregnancy (periconceptional). Adjusted odds ratios (aORs) were calculated to examine associations with 16 birth defects.
RESULTS: Compared to acetaminophen, mothers reporting NSAIDs were significantly more likely to have offspring with gastroschisis, hypospadias, cleft palate, cleft lip with cleft palate, cleft lip without cleft palate, anencephaly, spina bifida, hypoplastic left heart syndrome, pulmonary valve stenosis, and tetralogy of Fallot (aOR range, 1.2-1.6). Opioids were associated with tetralogy of Fallot, perimembranous ventricular septal defect, and ventricular septal defect with atrial septal defect (aOR range, 1.8-2.3), whereas use of both opioids and NSAIDs was associated with gastroschisis, cleft palate, spina bifida, hypoplastic left heart syndrome, and pulmonary valve stenosis (aOR range, 2.0-2.9).
CONCLUSIONS: Compared to periconceptional use of acetaminophen, selected birth defects occurred more frequently among infants of women using NSAIDs and/or opioids. However, we could not definitely determine whether these risks relate to the drugs or to indications for treatment.

PMID: 28993061 [PubMed - indexed for MEDLINE]

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Diffuse fibrosis is common in the left, but not in the right ventricle in patients with transposition of the great arteries late after atrial switch operation.

Int J Cardiovasc Imaging. 2018 Mar 29;:

Authors: Shehu N, Meierhofer C, Messroghli D, Mkrtchyan N, Martinoff S, Ewert P, Stern H

Abstract
In adult patients with transposition of the great arteries (TGA) late after atrial switch operation (AtSO), each of the ventricles is faced with a profoundly different pressure regimen from the one they are meant to support in normal conditions. The extent of diffuse fibrosis of the right ventricle (RV) and left ventricle (LV) in these patients remains incompletely investigated. Aim of this study was to quantify the degree of fibrosis of the unloaded LV and of the overloaded RV by determining the myocardial extracellular volume (ECV) with non-invasive techniques as T1 mapping. We determined ECV by cardiac magnetic resonance (CMR) in 10 patients (36.8 ± 5.3 years old) with TGA late after AtSO, without relevant pulmonary stenosis, by acquiring T1-maps of the myocardium before and 10 min after injection of Gadolinium-based contrast agent. ECV of the inferior wall (36% (33-41%)) and of the lateral wall (37% (35-39%)) of the LV was significantly increased compared to the ECV of the RV (27% (25-29%)), in both comparisons P < 0.0001. Long-time LV unloading following atrial switch procedures leads to severe myocardial fibrosis of the subpulmonary LV. T1 mapping CMR might be useful for selection of patients with atrial switch operation, in whom reestablishment of the LV as a systemic ventricle by staged arterial switch operation is planned. However larger studies and newer higher resolution methods for T1-mapping are needed to determine the role of ECV in the decision of a surgical intervention in this kind of population.

PMID: 29600485 [PubMed - as supplied by publisher]

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Pulmonary hypertension: Barrier or just a bump in the road in transplanting adults with congenital heart disease.

Congenit Heart Dis. 2018 Mar 30;:

Authors: Menachem JN, Birati EY, Zamani P, Owens AT, Atluri P, Bermudez CA, Drajpuch D, Fuller S, Kim YY, Mascio CE, Palanivel V, Rame JE, Wald J, Acker MA, Mazurek JA

Abstract
BACKGROUND: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Identification of disease progression and timing of referral for advanced therapies is often delayed. However, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience. However, in some cases, PH is a result of elevated systemic filling pressures and low output.
METHODS: We describe the approach used to successfully transplant an ACHD patient with severe pre-HT PH performing HT alone. We review the literature and describe the one patient's journey from primarily palliative, to a combined heart-lung transplant candidate, to successful HT patient.
RESULTS: We discuss the methodology used to successfully transplant a patient, with significantly elevated pulmonary pressures and an initial pulmonary vascular resistance (PVR) > 13 Wood units.
CONCLUSIONS: There are a number of complexities associated with the ACHD population and it is of utmost importance to carefully identify the underlying hemodynamic milieu and inform the appropriate treatment course in order to have successful transplant outcomes.

PMID: 29603626 [PubMed - as supplied by publisher]

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A Translational Model of Incomplete Catch-Up Growth: Early-Life Hypoxia and the Effect of Physical Activity.

Clin Transl Sci. 2018 Mar 30;:

Authors: Radom-Aizik S, Zaldivar FP, Nance DM, Haddad F, Cooper DM, Adams GR

Abstract
Advances in therapies have led to prolonged survival from many previously lethal health threats in children, notably among prematurely born babies and those with congenital heart disease. Evidence for catch-up growth is common in these children, but in many cases the adult phenotype is never achieved. A translational animal model is required in which specific tissues can be studied over a reasonable time interval. We investigated the impact of postnatal hypoxia (HY) (12%O2 (HY12) or 10% O2 (HY10)) on growth in rats relative to animals raised in room air. Subgroups had access to running wheels following the HY period. Growth was fully compensated in adult HY12 rats but not HY10 rats. The results of this study indicate that neonatal hypoxia can be a useful model for the elucidation of mechanisms that mediate successful catch-up growth following neonatal insults and identify the critical factors that prevent successful catch-up growth.

PMID: 29603633 [PubMed - as supplied by publisher]

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Anaesthetic management of breast surgery in a patient with Eisenmenger syndrome.

Rev Esp Anestesiol Reanim. 2017 Jan;64(1):41-45

Authors: Galán Gutiérrez JC, Fernández Suárez FE, Miranda García P, Sopena Zubiria LA

Abstract
Eisenmenger syndrome (ES) is a complex combination of cardiovascular abnormalities defined as pulmonary hypertension with investment or bidirectional flow through an intracardiac or aortopulmonary communication, usually secondary to a congenital heart disease not resolved promptly. It carries a significant risk of perioperative mortality, with an incidence close to 30% for non-cardiac surgery. We report the anaesthetic management in a ES patient undergoing breast surgery, which was successfully performed under general anaesthesia combined with thoracic analgesic blocks. The main pathophysiological implications of this syndrome are discussed, emphasizing the importance of appropriate preoperative evaluation with thorough assessment of associated risks, careful intraoperative management, and postoperative care, which should be initially performed in a critical care unit. The need to individualize and tailor the choice of drugs and anesthetic technique to the hemodynamic condition of the patient and the surgical procedure is highlighted.

PMID: 27554331 [PubMed - indexed for MEDLINE]

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