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Eisenmenger syndrome: diagnosis, prognosis and clinical management.

Heart. 2020 Jul 20;:

Authors: Arvanitaki A, Giannakoulas G, Baumgartner H, Lammers AE

Abstract
Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. Despite early detection of CHD and major advances in paediatric cardiac surgery, ES is still prevalent and requires a multidisciplinary approach by adult CHD experts in tertiary centres. Central cyanosis is the primary clinical manifestation leading to secondary erythrocytosis and various multiorgan complications that increase morbidity and affect quality of life. Close follow-up is needed to early diagnose and timely manage these complications. The primary goal of care is to maintain patients' fragile stability. Although the recent use of advanced PAH therapies has substantially improved functional capacity and increased life expectancy, long-term survival remains poor. Progressive heart failure, infectious diseases and sudden cardiac death comprise the main causes of death in patients with ES. Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. Endothelin receptor antagonists are used as first-line treatment in symptomatic patients, while phosphodiesterase-5 inhibitors may be added. Due to the lack of evidence, current guidelines do not provide a clear therapeutic strategy regarding treatment escalation. Additional well-designed trials are required to assess the comparative efficacy of various PAH agents and the benefit of combination therapy. Finally, the development of a risk score is of utmost importance to guide clinical therapy.

PMID: 32690623 [PubMed - as supplied by publisher]

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Congenital Rubella Syndrome Surveillance in South Africa Using a Sentinel Site Approach: A Cross-sectional Study.

Clin Infect Dis. 2019 05 02;68(10):1658-1664

Authors: Motaze NV, Manamela J, Smit S, Rabie H, Harper K, duPlessis N, Reubenson G, Coetzee M, Ballot D, Moore D, Nuttall J, Linley L, Tooke L, Kriel J, Hallbauer U, Sutton C, Moodley P, Hardie D, Mazanderani AH, Goosen F, Kyaw T, Leroux D, Hussain A, Singh R, Kelly C, Ducasse G, Muller M, Blaauw M, Hamese M, Leeuw T, Mekgoe O, Rakgole P, Dungwa N, Maphosa T, Sanyane K, Preiser W, Cohen C, Suchard M

Abstract
BACKGROUND: Congenital rubella syndrome (CRS) includes disorders associated with intrauterine rubella infection. Incidence of CRS is higher in countries with no rubella-containing vaccines (RCV) in their immunization schedules. In the World Health Organization African region, RCVs are being introduced as part of the 2012-2020 global measles and rubella strategic plan. This study aimed to describe the epidemiology of confirmed CRS in South Africa prior to introduction of RCVs in the immunization schedule.
METHODS: This was a descriptive study with 28 sentinel sites reporting laboratory-confirmed CRS cases in all 9 provinces of South Africa. In the retrospective phase (2010 to 2014), CRS cases were retrieved from medical records, and in the prospective phase (2015 to 2017) clinicians at study sites reported CRS cases monthly.
RESULTS: There were 42 confirmed CRS cases in the retrospective phase and 53 confirmed CRS cases in the prospective phase. Most frequently reported birth defects were congenital heart disease and cataracts. The median age of mothers of CRS cases was 21 years in the retrospective phase (range: 11 to 38 years) and 22 years in the prospective phase (range: 15 to 38 years).
CONCLUSION: Baseline data on laboratory-confirmed CRS will enable planning and monitoring of RCV implementation in the South African Expanded Programme on Immunization program. Ninety-eight percent of mothers of infants with CRS were young women 14-30 years old, indicating a potential immunity gap in this age group for consideration during introduction of RCV.

PMID: 30203002 [PubMed - indexed for MEDLINE]

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An unusual case of redo tricuspid valve replacement and repair of a previously unidentified anomalous pulmonary venous return in a patient with congenitally corrected transposition of the great arteries.

Clin Case Rep. 2020 Jul;8(7):1241-1246

Authors: Sadeghi AH, Van de Woestijne P, Taverne YJHJ, Van Dijk APJ, Bogers AJJC

Abstract
Associated cardiovascular malformations in congenitally corrected transposition of the great arteries (CCTGA) should not be missed when a patient requires surgical correction. We present a case of an adult CCTGA patient who required redo surgery for recurrent tricuspid (left atrioventricular) valve regurgitation and previously unidentified partial anomalous pulmonary venous return.

PMID: 32695367 [PubMed]

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A Matched Case-Control Study on the Association Between Colds, Depressive Symptoms during Pregnancy and Congenital Heart Disease in Northwestern China.

Sci Rep. 2019 01 24;9(1):589

Authors: Guo L, Zhao D, Zhang R, Li S, Liu R, Wang H, Dang S, Yan H

Abstract
The purpose of this study was to explore the association between colds, depressive symptoms during pregnancy and offspring congenital heart disease (CHD). A 1:2 matching case-control study was conducted in Northwest China. Information was gathered by a structured questionnaire and was reviewed by investigators on the spot. Multivariate logistic regressions and nonlinear mixed effect model were performed. 614 cases and 1228 controls were available in this study. After adjusting for potential confounders, the colds during the entire pregnancy were associated with increased risk of offspring CHD (OR = 1.44(1.12-1.85)). Similarly, there was a higher depression score in CHD group than the control group (OR = 1.89(1.48-2.41)). In addition, the women with both colds and higher depression scores had a higher risk of offspring CHD (OR = 2.72(1.87-3.93)) than their counterparts with only colds (OR = 1.48(1.04-2.09)) or with only higher depression scores (OR = 1.94(1.37-2.74)). The combined effects were significant in the multiplication model (OR = 2.04(1.47-2.83)) but not in the additive model (S = 1.40(0.70-2.81), AP = 0.19(-0.15-0.53) and RERI = 0.55(-0.54-1.64)). In conclusion, the colds and depressive symptoms during pregnancy were found associated with increased risk of offspring CHD and we found for the first time that there existed a statistically multiplying interaction effect of colds and depression on increasing risk of offspring CHD.

PMID: 30679633 [PubMed - indexed for MEDLINE]

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Thoracic Aortic Aneurysm Growth in Bicuspid Aortic Valve Patients: Role of Aortic Stiffness and Pulsatile Hemodynamics.

J Am Heart Assoc. 2019 04 16;8(8):e010885

Authors: Rooprai J, Boodhwani M, Beauchesne L, Chan KL, Dennie C, Nagpal S, Messika-Zeitoun D, Coutinho T

Abstract
Background Bicuspid aortic valve ( BAV ) is the most common congenital cardiac abnormality. A thoracic aortic aneurysm ( TAA ) is present in ≈50% of BAV patients, who also have an 8-fold higher risk of aortic dissection than the general population. Because the health of the aorta is directly reflected in its stiffness and pulsatile hemodynamics, we hypothesized that measures of aortic stiffness and arterial load would be associated with TAA growth in BAV . Methods and Results Twenty-nine unoperated participants with TAA due to BAV who had serial imaging were recruited. Aortic stiffness and steady and pulsatile arterial load were evaluated with validated methods that integrate arterial tonometry with echocardiography. TAA growth was assessed retrospectively based on available imaging, blinded to hemodynamic status. Multivariable linear regression assessed associations of aortic stiffness and hemodynamic variables with TAA growth, adjusting for potential confounders. Overall, 66% of participants were men. Mean±SD for age, baseline aneurysm size, growth rate, and follow-up time were 57.2±8.3 years, 46.9±3.6 mm, 0.75±0.81 mm/y, and 2.9±3.3 years, respectively. We found that greater aortic stiffness (β± SE for carotid-femoral pulse wave velocity: 0.30±0.13. P=0.03) and aortic characteristic impedance (β± SE : 0.46±0.18, P=0.02), as well as lower total arterial and proximal aortic compliance (β± SE : -0.44±0.21, P=0.05, and -0.63±0.16, P=0.001, respectively) were independently associated with faster aneurysm growth. Conclusions In patients with TAA due to BAV , measures of greater aortic stiffness and pulsatile arterial load indicate an association with accelerated aneurysm expansion. Assessing arterial hemodynamics may be useful for risk stratification and disease monitoring in TAA patients with BAV .

PMID: 30966855 [PubMed - indexed for MEDLINE]

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Reconstruction of right ventricular outflow tract stenosis and right ventricular failure after Ross procedure - comprehensive assessment of adult congenital heart disease with four-dimensional imaging: a case report.

J Med Case Rep. 2020 Jul 23;14(1):113

Authors: Takigami M, Itatani K, Nakanishi N, Morichi H, Nishino T, Miyazaki S, Nakaji K, Yamano M, Kajiyama Y, Maeda Y, Matoba S, Yaku H, Yamagishi M

Abstract
BACKGROUND: Re-intervention after Ross procedure into the right ventricular outflow tract might be needed in patients in the long term. However, right ventricular outflow tract re-intervention indications are still unclear. Comprehensive assessment of total hemodynamics is needed. A 42-year-old Japanese woman was referred to our hospital for moderately severe pulmonary regurgitation and severe tricuspid regurgitation after a Ross-Konno procedure. Thirteen years after surgery, she developed atrial fibrillation and atrial flutter and complained of dyspnea. Electrophysiological studies showed re-entry circuit around the low voltage area of the lateral wall on the right atrium. Four-dimensional flow magnetic resonance imaging revealed moderate pulmonary regurgitation, severe tricuspid regurgitation, and a dilated right ventricle. Flow energy loss in right ventricle calculated from four-dimensional flow magnetic resonance imaging was five times higher than in normal controls, suggesting an overload of the right-sided heart system. Her left ventricular ejection fraction was almost preserved. Moreover, the total left interventricular pressure difference, which shows diastolic function, revealed that her sucking force in left ventricle was preserved. After the comprehensive assessments, we performed right ventricular outflow tract reconstruction, tricuspid valve annuloplasty, and right-side Maze procedure. A permanent pacemaker with a single atrial lead was implanted 14 days postoperatively. She was discharged 27 days postoperatively. Echocardiography performed 3 months later showed that the size of the dilated right ventricle had significantly reduced.
DISCUSSION: A four-dimensional imaging tool can be useful in the decision of re-operation in patients with complex adult congenital heart disease. The optimal timing of surgery should be considered comprehensively.

PMID: 32698864 [PubMed - in process]

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The case of the missing pulmonary vein: A focused update on anomalous pulmonary venous connection in congenital cardiovascular disease.

Echocardiography. 2019 10;36(10):1930-1935

Authors: Han F, Kiparizoska S, Campbell W, Richards C, Kogon B, Holloway M, Watson C, Kerut EK, McMullan M

Abstract
Partial anomalous pulmonary venous connection is defined by one or more of the pulmonary veins draining to the heart into a location other than the left atrium. Depending on the location of the anomalous venous connection, they can be categorized as supracardiac, infracardiac, cardiac, and mixed types. In some cases, there is no hemodynamic consequence; in others, it can result in tricuspid regurgitation, right heart dilation, and pulmonary hypertension. Frequently, the reason for referral can be asymptomatic right heart dilation of unknown significance. Diagnosis is often difficult by transthoracic echocardiogram unless there is a high index of suspicion, and the appropriate views are obtained. Cardiac CT (computed tomography) or cardiac MRI (magnetic resonance imaging) can provide more precise anatomic detail as needed. The current article reviews the etiology and pathophysiology of partial anomalous pulmonary venous connection, and also reviews the current knowledge on their treatment.

PMID: 31573698 [PubMed - indexed for MEDLINE]

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X-ray fused with MRI guidance of pre-selected transcatheter congenital heart disease interventions.

Catheter Cardiovasc Interv. 2019 Sep 01;94(3):399-408

Authors: Grant EK, Kanter JP, Olivieri LJ, Cross RR, Campbell-Washburn A, Faranesh AZ, Cronin I, Hamann KS, O'Byrne ML, Slack MC, Lederman RJ, Ratnayaka K

Abstract
OBJECTIVES: To determine whether X-ray fused with MRI (XFM) is beneficial for select transcatheter congenital heart disease interventions.
BACKGROUND: Complex transcatheter interventions often require three-dimensional (3D) soft tissue imaging guidance. Fusion imaging with live X-ray fluoroscopy can potentially improve and simplify procedures.
METHODS: Patients referred for select congenital heart disease interventions were prospectively enrolled. Cardiac MRI data was overlaid on live fluoroscopy for procedural guidance. Likert scale operator assessments of value were recorded. Fluoroscopy time, radiation exposure, contrast dose, and procedure time were compared to matched cases from our institutional experience.
RESULTS: Forty-six patients were enrolled. Pre-catheterization, same day cardiac MRI findings indicated intervention should be deferred in nine patients. XFM-guided cardiac catheterization was performed in 37 (median age 8.7 years [0.5-63 years]; median weight 28 kg [5.6-110 kg]) with the following prespecified indications: pulmonary artery (PA) stenosis (n = 13), aortic coarctation (n = 12), conduit stenosis/insufficiency (n = 9), and ventricular septal defect (n = 3). Diagnostic catheterization showed intervention was not indicated in 12 additional cases. XFM-guided intervention was performed in the remaining 25. Fluoroscopy time was shorter for XFM-guided intervention cases compared to matched controls. There was no significant difference in radiation dose area product, contrast volume, or procedure time. Operator Likert scores indicated XFM provided useful soft tissue guidance in all cases and was never misleading.
CONCLUSIONS: XFM provides operators with meaningful three-dimensional soft tissue data and reduces fluoroscopy time in select congenital heart disease interventions.

PMID: 31062506 [PubMed - indexed for MEDLINE]

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Coronary Fistula Between Left Anterior Descending Artery (LAD) and Pulmonary Artery (PA) Leading to Sudden Cardiac Death: Case Report with Literature Review.

Curr Cardiol Rev. 2020;16(2):98-102

Authors: Battisha A, Madoukh B, Sheikh O, Altibi A, Sheikh S, Al-Sadawi M

Abstract
Coronary Artery Fistulas (CAF) are inappropriate connections between a coronary artery and a major vessel or a cardiac chamber. These fistulas may or may not present with symptoms, but they need to be detected as early as possible in order to decide the most appropriate treatment methodology (i.e. surgery vs. conservative management). We report the case of a 67-year-old female with no modifiable cardiovascular risk factors who had an unwitnessed sudden death at home during her ongoing evaluation of a fistula detected incidentally between the Left Anterior Descending Artery (LAD) and the Pulmonary Artery (PA). This case highlights that early diagnosis and treatment of symptomatic CAF are crucial to minimize the risk of sudden cardiac death. Although the symptomatic fistula of LAD to PA has been reported in the literature multiple times, it has been rarely reported that this fistula can result in sudden cardiac death.

PMID: 31593531 [PubMed - indexed for MEDLINE]

Related Articles

Should We Judge Stroke Risk by Static or Dynamic Risk Scores? A Focus on the Dynamic Nature of Stroke and Bleeding Risks in Patients With Atrial Fibrillation.

J Cardiovasc Pharmacol. 2019 12;74(6):491-498

Authors: Domek M, Gumprecht J, Mazurek M, Chao TF, Lip GYH

Abstract
Atrial fibrillation (AF) is the most prevalent cardiac arrhythmia and a major risk factor for stroke. The number of patients with AF is predicted to increase in the next few decades. AF has also negative impact on quality of life as well as it significantly increases the risk of cardiovascular disease and overall mortality. Because the stroke is a pivotal outcome of AF, its prevention with the use of anticoagulation therapy constitutes an important component of AF management. The decision on oral anticoagulants' prescription should be based on appropriate risk stratification to allow for comprehensive assessment of benefit/hazard ratio of stroke and bleeding along with patients' preference. Several risk scores for stroke and bleeding as well as for stroke and systemic embolism have been developed, mainly in patients on vitamin K antagonists. AF guidelines stress the need for repetitive evaluation of thromboembolic and bleeding risks to tailor optimal AF management. Indeed, risk is not a static "one off" process and it should be adjusted for dynamic nature of risk factors. However, most risk scores are calculated according to baseline characteristics of patients, but the older the patients get, the more comorbidities they acquire, which influences stroke risk significantly. Hence, the default management of every patient with AF should include a regular reassessment of stroke and bleeding risk factors.

PMID: 31599783 [PubMed - indexed for MEDLINE]

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