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Assessment of the precision and reproducibility of ventricular volume, function, and mass measurements with ferumoxytol-enhanced 4D flow MRI.

J Magn Reson Imaging. 2016 Aug;44(2):383-92

Authors: Hanneman K, Kino A, Cheng JY, Alley MT, Vasanawala SS

Abstract
PURPOSE: To compare the precision and interobserver agreement of ventricular volume, function, and mass quantification by 3D time-resolved (4D) flow MRI relative to cine steady-state free precession (SSFP).
MATERIALS AND METHODS: With Institutional Research Board approval, informed consent, and HIPAA compliance, 22 consecutive patients with congenital heart disease (CHD) (10 males, 6.4 ± 4.8 years) referred for 3T ferumoxytol-enhanced cardiac MRI were prospectively recruited. Complete ventricular coverage with standard 2D short-axis cine SSFP and whole chest coverage with axial 4D flow were obtained. Two blinded radiologists independently segmented images for left ventricular (LV) and right ventricular (RV) myocardium at end systole (ES) and end diastole (ED). Statistical analysis included linear regression, analysis of variance (ANOVA), Bland-Altman (BA) analysis, and intraclass correlation (ICC).
RESULTS: Significant positive correlations were found between 4D flow and SSFP for ventricular volumes (r = 0.808-0.972, P < 0.001), ejection fraction (EF) (r = 0.900-928, P < 0.001), and mass (r = 0.884-0.934, P < 0.001). BA relative limits of agreement for both ventricles were between -52% to 34% for volumes, -29% to 27% for EF, and -41% to 48% for mass, with wider limits of agreement for the RV compared to the LV. There was no significant difference between techniques with respect to mean square difference of ED-ES mass for either LV (F = 2.05, P = 0.159) or RV (F = 0.625, P = 0.434). Interobserver agreement was moderate to good with both 4D flow (ICC 0.523-0.993) and SSFP (ICC 0.619-0.982), with overlapping confidence intervals.
CONCLUSION: Quantification of ventricular volume, function, and mass can be accomplished with 4D flow MRI with precision and interobserver agreement comparable to that of cine SSFP. J. Magn. Reson. Imaging 2016;44:383-392.

PMID: 26871420 [PubMed - indexed for MEDLINE]

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Tricuspid annular plane systolic excursion is preserved in young patients with pulmonary hypertension except when associated with repaired congenital heart disease.

Eur Heart J Cardiovasc Imaging. 2017 Apr 01;18(4):459-466

Authors: Hauck A, Guo R, Ivy DD, Younoszai A

Abstract
Aims: Tricuspid annular plane systolic excursion (TAPSE) is a measure of right ventricular (RV) longitudinal function that correlates with functional status and mortality in adults with pulmonary hypertension (PH). The diagnostic and predictive value of TAPSE in children with PH has not been fully examined. We aimed to define TAPSE across aetiologies of paediatric PH and assess the correlation between TAPSE and measures of disease severity.
Methods and results: TAPSE measurements were obtained in 84 children and young adults undergoing treatment for PH and 315 healthy children to establish z-scores at moderate altitude for comparison. The relationships between TAPSE and echocardiographic, biomarker, and functional measures of disease severity between aetiologies were assessed. TAPSE z-scores in PH patients with congenital heart disease (CHD) repaired with open cardiac surgery (n = 20, mean -2.73) were significantly decreased compared with normal children and patients with other aetiologies of PH (P < 0.001) but did not reflect poorer clinical status. TAPSE z-scores in children with idiopathic PH (n = 29, -0.41), unrepaired CHD (n = 11, -0.1), and PH related to systemic disease (n = 14, -0.39) were not different from normal. TAPSE correlated modestly with brain natriuretic peptide, echocardiographic function parameters, and functional class except in patients with repaired CHD.
Conclusion: Children with PH maintain normal TAPSE values early except when associated with repaired CHD. Superior RV adaptation to high afterload in children compared with adults may account for this finding. Reduced TAPSE after repair of CHD does not correlate with functional status and may reflect post-operative changes rather than poor function primarily due to PH.

PMID: 27099276 [PubMed - indexed for MEDLINE]

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Reproductive/Developmental Abnormalities Induced by Epigenetic Aberrations and Possible Environmental Causes.

Nihon Eiseigaku Zasshi. 2016;71(3):195-199

Authors: Kawai T, Hata K

Abstract
Foetal environmental factors, including maternal nutrition, hormonal disturbance, and chemical exposure, affect foetal growth and can cause birth defects. Recent studies have shown the link of poor foetal growth with increased risks of coronary heart disease, type II diabetes, kidney disease, and brain disorders in adulthood. Epigenetic modifications, such as DNA methylation and histone modifications, are involved in tissue- and developmental stage-specific gene expression and silencing, and they can be transmitted stably through mitotic cell division, thereby inducing long-term changes in gene regulation. Developmental programming during the foetal period, therefore, could affect adult health through epigenetic mechanisms. In fact, many studies using animal models have demonstrated that nutrient manipulation during pregnancy induces epigenetic alterations at specific loci or globally in the offspring. In this review, we summarize our findings that elucidate the effects of in utero environments on the human placental epigenome.

PMID: 27725422 [PubMed - indexed for MEDLINE]

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Evolution of right ventricular size over time after tetralogy of Fallot repair: a longitudinal cardiac magnetic resonance study.

Eur Heart J Cardiovasc Imaging. 2017 Mar 01;18(3):364-370

Authors: Rutz T, Ghandour F, Meierhofer C, Naumann S, Martinoff S, Lange R, Ewert P, Stern HC, Fratz S

Abstract
Aims: It is commonly believed that pulmonary regurgitation (PR) after surgical repair of tetralogy of Fallot (TOF) leads to progressive right ventricular (RV) enlargement. However, progressive RV dilatation has never clearly been documented in this patient population. Therefore, we studied the size of the RV over time in patients after surgical TOF repair.
Methods and results: Fifty-one consecutive patients after surgical TOF repair underwent at least two cardiovascular magnetic resonance (CMR) exams using a single CMR scanner. Patients with RV outflow tract obstruction, interventions other than the initial repair and CMR exams with use of sedation were excluded. Three subgroups with different repair techniques were studied: transannular patch repair [n = 22, age 17 ± 10 years], subvalvular patch repair [n = 15, age 22 ± 8 years], or non-patch repair/infundibulectomy (n = 14, age 28 ± 11 years). Right ventricular end-diastolic volume index (RVEDVI) and PR fraction did not change during the 37 ± 21 months follow-up between first and last CMR in the whole group (RVEDVI: 118 ± 23 mL/m2 vs. 119 ± 23 mL/m2, P = 0.720; PR fraction: 33% (23-40%) vs. 32% (24-39%), P = 0.268). RVEDVI remained stable in all subgroups (transannular patch: 120 ± 21 mL/m2 vs. 122 ± 22 mL/m2, subvalvular patch: 112 ± 23 mL/m2 vs. 111 ± 23 mL/m2, non-patch: 123 ± 28 mL/m2 vs. 123 ± 23 mL/m2, P = 0.827). RVEDVI at last CMR did not differ between groups (P = 0.301).
Conclusions: This study shows no progression of RV dilatation in patients after surgical repair of TOF with moderately dilated RVs and significant PR during a 3-year follow-up. RV dilatation in our patient group seems to be independent from surgical repair techniques.

PMID: 28363200 [PubMed - indexed for MEDLINE]

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Recommendations on the echocardiographic assessment of aortic valve stenosis: a focused update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

Eur Heart J Cardiovasc Imaging. 2017 Mar 01;18(3):254-275

Authors: Baumgartner H, Hung J, Bermejo J, Chambers JB, Edvardsen T, Goldstein S, Lancellotti P, LeFevre M, Miller F, Otto CM

Abstract
Echocardiography is the key tool for the diagnosis and evaluation of aortic stenosis. Because clinical decision-making is based on the echocardiographic assessment of its severity, it is essential that standards are adopted to maintain accuracy and consistency across echocardiographic laboratories. Detailed recommendations for the echocardiographic assessment of valve stenosis were published by the European Association of Echocardiography and the American Society of Echocardiography in 2009. In the meantime, numerous new studies on aortic stenosis have been published with particular new insights into the difficult subgroup of low gradient aortic stenosis making an update of recommendations necessary. The document focuses in particular on the optimization of left ventricular outflow tract assessment, low flow, low gradient aortic stenosis with preserved ejection fraction, a new classification of aortic stenosis by gradient, flow and ejection fraction, and a grading algorithm for an integrated and stepwise approach of artic stenosis assessment in clinical practice.

PMID: 28363204 [PubMed - indexed for MEDLINE]

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The use of Macitentan in Fontan circulation: a case report.

BMC Cardiovasc Disord. 2017 May 22;17(1):131

Authors: Demetriades P, Aziz A, Condliffe R, Bowater SE, Clift PF

Abstract
BACKGROUND: The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation.
CASE PRESENTATION: We describe the case of a 50 year old female with tricuspid atresia and transposition of the great arteries. Following complex surgery as a child, she subsequently underwent a fenestrated modified atrial pulmonary Fontan operation which was later converted to a total cavopulmonary anastomosis Fontan circulation. Due to failure of various medications to relieve her worsening symptoms, she was commenced on macitentan in April 2016. Few months later, she demonstrated a significant symptomatic improvement and associated increase in her incremental shuttle walking test distance.
CONCLUSIONS: Macitentan has slower receptor dissociation kinetics compared to other endothelin-receptor antagonists, leading to enhanced pharmacological activity with promising effects in patients with pulmonary arterial hypertension. The patient we report has shown considerable improvement in exercise capacity following introduction of this medication and thus we suggest further randomised trials to establish the role of different endothelin-receptor antagonists in the management of the Fontan circulation.

PMID: 28532389 [PubMed - indexed for MEDLINE]

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High proportion of genetic cases in patients with advanced cardiomyopathy including a novel homozygous Plakophilin 2-gene mutation.

PLoS One. 2017;12(12):e0189489

Authors: Klauke B, Gaertner-Rommel A, Schulz U, Kassner A, Zu Knyphausen E, Laser T, Kececioglu D, Paluszkiewicz L, Blanz U, Sandica E, van den Bogaerdt AJ, van Tintelen JP, Gummert J, Milting H

Abstract
Cardiomyopathies might lead to end-stage heart disease with the requirement of drastic treatments like bridging up to transplant or heart transplantation. A not precisely known proportion of these diseases are genetically determined. We genotyped 43 index-patients (30 DCM, 10 ARVC, 3 RCM) with advanced or end stage cardiomyopathy using a gene panel which covered 46 known cardiomyopathy disease genes. Fifty-three variants with possible impact on disease in 33 patients were identified. Of these 27 (51%) were classified as likely pathogenic or pathogenic in the MYH7, MYL2, MYL3, NEXN, TNNC1, TNNI3, DES, LMNA, PKP2, PLN, RBM20, TTN, and CRYAB genes. Fifty-six percent (n = 24) of index-patients carried a likely pathogenic or pathogenic mutation. Of these 75% (n = 18) were familial and 25% (n = 6) sporadic cases. However, severe cardiomyopathy seemed to be not characterized by a specific mutation profile. Remarkably, we identified a novel homozygous PKP2-missense variant in a large consanguineous family with sudden death in early childhood and several members with heart transplantation in adolescent age.

PMID: 29253866 [PubMed - indexed for MEDLINE]

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Clinical Characteristics of Adult Patients With Congenital Heart Disease Hospitalized for Acute Heart Failure.

Circ J. 2017 Dec 29;:

Authors: Negishi J, Ohuchi H, Miyazaki A, Tsuda E, Shiraishi I, Kurosaki K

Abstract
BACKGROUND: Heart failure (HF) is an important complication in adults with congenital heart disease (CHD), but because only a few studies have focused on acute HF hospitalization in adults with CHD, we study aimed to define the clinical characteristics of such patients and examine the differences in acute HF between adults with CHD and acquired heart disease.Methods and Results:We retrospectively evaluated 50 adults with CHD admitted for treatment of acute HF and compared their data with those from Japanese HF registries. Patient mean age was 37±15 years and 58% were male. In total, 86% of the patients had complex forms of CHD and 62% had undergone corrective surgery, including the Fontan procedure; 66% of patients showed right heart hemodynamic abnormality. In-hospital mortality was 4%, which was comparable to the Japanese HF registries. Survival rate was 93% at 1 year and 75% at 3 years, which was similarly poor to the rates of HF secondary to acquired heart disease.
CONCLUSIONS: We clarified the clinical characteristics of adults with CHD requiring HF hospitalization. Young adults with complex CHD were hospitalized for management of acute right HF. Short-term and mid-term outcomes were similarly poor compared with acute HF secondary to acquired heart disease.

PMID: 29311517 [PubMed - as supplied by publisher]

Lessons Learned From a Critical Analysis of the Fontan Operation Over Three Decades in a Single Institution.

World J Pediatr Congenit Heart Surg. 2017 May;8(3):376-384

Authors: Caneo LF, Turquetto ALR, Neirotti RA, Binotto MA, Miana LA, Tanamati C, Penha JG, Silveira JBD, Alexandre E Silva TM, Jatene FB, Jatene MB

Abstract
BACKGROUND: The Fontan operation (FO) has evolved and many centers have demonstrated improved results relative to those from earlier eras. We report a single-institution experience over three decades, describing the outcomes and evaluating risk factors.
METHODS: Successive patients undergoing primary FO were divided into era I (1984-1994), era II (1995-2004), and era III (2005-2014). Clinical and operative notes were reviewed for demographic, anatomic, and procedure details. End points included early and late mortality and a composite of death, heart transplantation (HTX), or Fontan takedown.
RESULTS: A total of 420 patients underwent 18 atriopulmonary connections, 82 lateral tunnels (LT), and 320 extracardiac conduit (EC) Fontan procedures. Forty-six (11%) patients died; early and late mortality were 7.9% and 3.1%, respectively. Eight (1.9%) patients underwent HTX, 11 (2.6%) underwent Fontan conversion to EC, and 1 (0.2%) takedown of EC to bidirectional Glenn shunt. Prevalence of concomitant valve surgery ( P < .001) and pulmonary artery reconstruction ( P < .001) differed over the eras. Preoperative valve regurgitation was associated with likelihood of early mortality (odds ratio [OR] = 3.5, P = .002). Embolic events (OR = 1.9, P = .047), preoperative valve regurgitation (OR = 2.3, P = .029), diagnosis of unbalanced atrioventricular canal defect (OR = 1.14, P = .03), and concomitant valve replacement (OR = 6.9, P = .001) during the FO were associated with increased risk of the composite end point (death, HTX, or takedown).
CONCLUSION: Technical modifications did not result in improved results across eras, due in part to more liberal indications for surgery in the recent years. Valve regurgitation, unbalanced atrioventricular canal, embolic events, or concomitant valve replacement were associated with FO failure.

PMID: 29318931 [PubMed - in process]

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Interobserver variability in the classification of congenital coronary abnormalities: A substudy of the anomalous connections of the coronary arteries registry.

Congenit Heart Dis. 2017 Dec;12(6):726-732

Authors: Koutsoukis A, Halna du Fretay X, Dupouy P, Ou P, Laissy JP, Juliard JM, Hyafil F, Aubry P, ANOCOR Investigators

Abstract
OBJECTIVE: The diagnosis of anomalous connections of the coronary arteries (ANOCOR) requires an appropriate identification for the management of the patients involved. We studied the observer variability in the description and classification of ANOCOR between a nonexpert group of physicians and a group of expert physicians, using the ANOCOR cohort.
PATIENTS AND DESIGN: Consecutive patients identified by 71 referring cardiologists were included in the ANOCOR cohort. Anomalous connection was diagnosed by invasive and/or computed tomography coronary angiography. Angiographic images were reviewed by an angiographic committee with experience in this field. Both investigators and angiographic committee filled out a questionnaire to classify each anomaly with the type of coronary artery involved, the site of anomalous connection, and the initial course. Observer variability between investigators and angiographic committee was assessed by κ statistics. Anomalous connection with a preaortic course was defined as at-risk.
RESULTS: Among 472 patients of the ANOCOR cohort, 496 abnormalities were identified with a preaortic course present in 31%. The agreement for the type of artery was excellent (κ = 0.92, 95% CI = 0.86-0.98, P < .05), while the agreement for the site of anomalous connection was moderate (κ = 0.50, 95% CI = 0.42-0.58, P < .05), and the agreement for the initial course was only fair (κ = 0.32, 95% CI = 0.28-0.37, P < .05). Observer agreement for the identification of at-risk forms was moderate (κ = 0.497, 95% CI = 0.40-0.59, P < .05).
CONCLUSIONS: Observer variability in the assessment of anomalous connection of the coronary arteries between nonexperienced and experienced physicians can be significant. We found that expert physicians provide a more robust classification in comparison with nonexpert physicians. Therefore, referral to physicians with a relevant experience should be considered, especially if an anomaly at-risk is suspected.

PMID: 28639359 [PubMed - indexed for MEDLINE]

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