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Evaluation of aortic regurgitation with cardiac magnetic resonance imaging: a systematic review.

Heart. 2017 Aug 19;:

Authors: Lee JC, Branch KR, Hamilton-Craig C, Krieger EV

Abstract
This review summaries the utility, application and data supporting use of cardiac magnetic resonance imaging (CMR) to evaluate and quantitate aortic regurgitation. We systematically searched Medline and PubMed for original research articles published since 2000 that provided data on the quantitation of aortic regurgitation by CMR and identified 11 articles for review. Direct aortic measurements using phase contrast allow quantitation of volumetric flow across the aortic valve and are highly reproducible and accurate compared with echocardiography. However, this technique requires diligence in prescribing the correct imaging planes in the aorta. Volumetric analytic techniques using differences in ventricular volumes are also highly accurate but less than phase contrast techniques and only accurate when concomitant valvular disease is absent. Comparison of both aortic and ventricular data for internal data verification ensures fidelity of aortic regurgitant data. CMR data can be applied to many types of aortic valve regurgitation including combined aortic stenosis with regurgitation, congenital valve diseases and post-transcatheter valve placement. CMR also predicts those patients who progress to surgery with high overall sensitivity and specificity. Future studies of CMR in patients with aortic regurgitation to quantify the incremental benefit over echocardiography as well as prediction of cardiovascular events are warranted.

PMID: 28822982 [PubMed - as supplied by publisher]

Far From the Septum: Transcatheter Cardioform Septal Occluder Device Closure of a Descending Aortic Pseudoaneurysm Late After Interrupted Aortic Arch Repair.

JACC Cardiovasc Interv. 2017 Aug 09;:

Authors: Pater C, Brown NM, Goldstein BH

PMID: 28823776 [PubMed - as supplied by publisher]

Minimally Invasive Approach to Correct Anomalous Inferior Vena Cava Connection to the Left Atrium.

World J Pediatr Congenit Heart Surg. 2017 Jan 01;:2150135117701406

Authors: Pace Napoleone C, Marini D, Baronetto A, Deorsola L, Agnoletti G, Rinaldi M

Abstract
Anomalous drainage of the inferior vena cava into the left atrium is a rare congenital condition. A 20-year-old girl was referred for recurrent transient ischemic attacks. Transthoracic echocardiography revealed a large ostium secundum atrial septal defect, and computed tomography showed anomalous drainage of the inferior vena cava into the left atrium. Through a right mini-thoracotomy, the opening of the inferior vena cava into the atrium was identified under the inferior edge of the interatrial septum, draining into the left atrium, and redirected to the right atrium, using a pericardial patch to reconstruct the atrial septum. Postoperative course was uneventful. Right mini-thoracotomy approach was effective in correcting the anomalous drainage of the inferior vena cava into the left atrium.

PMID: 28825382 [PubMed - as supplied by publisher]

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Pharmacokinetics of Oral and Intravenous Oseltamivir Treatment of Severe Influenza B Virus Infection Requiring Organ Replacement Therapy.

Eur J Drug Metab Pharmacokinet. 2017 Feb;42(1):155-164

Authors: Karsch K, Chen X, Miera O, Peters B, Obermeier P, Francis RC, Amann V, Duwe S, Fraaij P, Heider A, de Zwart M, Berger F, Osterhaus A, Schweiger B, Rath B

Abstract
BACKGROUND AND OBJECTIVES: Patients with severe influenza virus infection, multi-organ failure and organ replacement therapy may absorb and metabolize neuraminidase inhibitors differently. Systematic pharmacokinetic/pharmacodynamic clinical trials are currently lacking in this high-risk group. Inadequate dosing increases the risk of treatment failure and drug resistance, especially in severely ill patients with elevated virus loads. This study aims to explore the impact of organ replacement therapy on oseltamivir drug concentrations.
METHODS: Serial pharmacokinetic/pharmacodynamic measurements and Sieving coefficients were assessed in two patients with severe influenza B infection requiring organ replacement therapy.
RESULTS: Patient #1, a 9-year-old female with severe influenza B virus infection, biventricular assist device, and continuous veno-venous hemodiafiltration, received 75 mg oral oseltamivir twice-daily for 2 days, then intravenous oseltamivir with one-time renoprotective dosing (40 mg), followed by regular intravenous administration of 100 mg twice-daily. Plasma oseltamivir carboxylate concentrations were stable initially, but only regular administration of 100 mg resulted in virus load decline and clinical improvement. Patient #2, a 28-year-old female with influenza B virus infection requiring extracorporeal membrane oxygenation, received 75 mg oral oseltamivir twice-daily, resulting in erratic oseltamivir blood concentrations. In both patients, drug concentrations remained well within safety margins.
CONCLUSIONS: In severe cases with multi-organ failure, administration of 100 mg intravenous oseltamivir twice-daily provided reliable drug concentrations, as opposed to renoprotective and oral dosing, thereby minimizing the risk of treatment failure and drug resistance. Evidence-based pediatric dosing recommendations and effective intravenous antiviral treatment modalities are needed for intensive care patients with life-threatening influenza disease.

PMID: 26994602 [PubMed - indexed for MEDLINE]

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Myocardial cytochrome oxidase activity increases with age and hypoxemia in patients with congenital heart disease.

Perfusion. 2017 May;32(4):306-312

Authors: Onwugbufor M, Levy RJ, Zurakowski D, Jonas RA, Sinha P

Abstract
BACKGROUND: Myocardial tolerance to ischemia is influenced by age and preoperative cyanosis through unknown mechanisms and significantly affects postoperative outcomes. Cytochrome c oxidase (CcOx), the terminal enzyme of the mitochondrial electron transport chain, may play a role in the susceptibility to ischemic-reperfusion (IR) injury. Our study aimed at investigating changes in human myocardial CcOx activity based on age and preoperative oxygen saturation to understand its role in transition from neonatal to mature myocardium and hypoxic conditions.
METHODS: The right atrial appendage from patients undergoing first time surgical repair/palliation of congenital heart defects was analyzed for steady state CcOx activity by oxidation of ferrocytochrome c via spectrophotometry and steady state CcOx subunit I protein content by protein immunoblotting. Student's t-test compared CcOx activity and protein levels between patients with preoperative hypoxia and normoxia. Multiple linear regression analysis was used to assess the effects of age and preoperative arterial oxygen saturations (SaO2) on CcOx protein activity and protein content.
RESULTS: Thirty-two patients with a median (interquartile range) age of 83 days (8-174) and preoperative oxygen saturation 98% (85-100%) were enrolled. Independent of age, preoperative SaO2 ⩽90% was associated with significantly greater CcOx steady state activity (p=0.004). Additionally, older age itself was associated with increased CcOx steady state activity (p=0.022); the combination of preoperative SaO2 and age account for 33% of the variation in CcOx steady state activity (R(2)=0.332). There was no increase in the CcOx subunit I protein content with either age or preoperative hypoxia.
CONCLUSIONS: In patients with congenital heart disease, an increase in CcOx steady state activity is seen with increasing age. Hypoxia leads to upregulation of CcOx steady state activity without an increase in the amount of enzyme protein itself. Higher CcOx activity in older and cyanotic patients may indicate CcOx-dependent reactive oxygen species as the mechanism for IR injury.

PMID: 27913766 [PubMed - indexed for MEDLINE]

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Incidental retroaortic left innominate vein in adult patient.

Radiol Case Rep. 2017 Sep;12(3):475-478

Authors: Semionov A, Kosiuk J

Abstract
Retro-aortic left innominate vein is a rare vascular abnormality, usually associated with congenital heart disease. Here we report a case of isolated retro-aortic left innominate vein in an adult female.

PMID: 28828105 [PubMed]

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Pregnancy in Women with Congenital Heart Disease.

Curr Treat Options Cardiovasc Med. 2017 Aug 22;19(9):73

Authors: Yucel E, DeFaria Yeh D

Abstract
OPINION STATEMENT: Advances in cardiac surgical interventions in infancy and childhood have led to an increased number of women with congenital heart disease of childbearing age. For these women, individualized preconception counseling and pregnancy planning should be a vital component of their medical management, and presentation for obstetric care may even be an opportunity to re-establish cardiovascular care for patients who have been lost to follow-up. These patients have unique cardiovascular anatomy and physiology, which is dependent upon the surgical intervention they may have undergone during childhood or adolescence. These factors are associated with a variety of long-term complications, and the normal hemodynamic changes of pregnancy may unmask cardiac dysfunction and pose significant risk. Among three published risk assessment algorithms, the World Health Organization classification is the most sensitive in predicting maternal cardiovascular events in this population. Women with simple congenital heart defects generally tolerate pregnancy well and can be cared for in the community with careful monitoring. Conversely, women with complex congenital defects, with or without surgical repair and/or residual defects, should be managed in tertiary care centers under a multidisciplinary team of physicians experienced in adult congenital heart disease and high-risk obstetrics, who collaboratively participate in pregnancy planning, management, and care through childbirth and postpartum. Women who are cyanotic with oxygen saturation less than 85%, have significant pulmonary arterial hypertension of any cause, or have systemic ventricular dysfunction should be counseled to avoid pregnancy due to a very high risk of maternal and fetal mortality.

PMID: 28828743 [PubMed]

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[Rendu-Osler-Weber disease : More than just a nosebleed].

Internist (Berl). 2016 Jun;57(6):610-5

Authors: Lücke E, Schreiber J, Zencker M, Braun-Dullaeus R, Herold J

Abstract
A 72-year-old female patient presented with increasing dyspnea of unclear origin classified as New York Heart Association stage III (NYHA III). Using transesophageal echocardiography a patent foramen ovale (PFO) and right heart failure could be diagnosed. Right heart catheterization revealed a large left to right shunt due to an arteriovenous malformation in the liver. Because of additional telangiectasia of the lips the presumptive diagnosis was Rendu-Osler-Weber disease. Typical nosebleeds and other symptoms of the disease were lacking and only two out of four Curaçao criteria were positive; therefore, genetic testing was performed, which verified the clinical diagnosis. Off-label use of the angiogenesis inhibitor bevacizumab was initiated as the therapeutic strategy and led to an improvement in the symptomatic dyspnea.

PMID: 27114237 [PubMed - indexed for MEDLINE]

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Right Ventricular Outflow Tract Obstruction Caused by Isolated Sinus of Valsalva Aneurysm.

Tex Heart Inst J. 2016 Aug;43(4):357-9

Authors: Abu Saleh WK, Lin CH, Reardon MJ, Ramlawi B

Abstract
Isolated sinus of Valsalva aneurysm is a rare occurrence, with an incidence of <1.5% among congenital heart disease repairs in the world. We recount the case of a 64-year-old man who presented with right-sided heart failure symptoms caused by a severely dilated right coronary sinus of Valsalva aneurysm that substantially obstructed the right ventricular outflow tract. Successful surgical repair involved right ventricular outflow tract resection and subcoronary patch repair.

PMID: 27547152 [PubMed - indexed for MEDLINE]

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Hemodynamic consequences of a restrictive ductus arteriosus and foramen ovale in fetal transposition of the great arteries.

J Neonatal Perinatal Med. 2016 Sep 16;9(3):317-20

Authors: Talemal L, Donofrio MT

Abstract
D-transposition of the great arteries (D-TGA) is the most commonly diagnosed cyanotic congenital heart disease presenting in the neonatal period. The survival after an arterial switch operation, with freedom from adverse cardiovascular events, has been reported to be as high as 93% at 25 years. However, despite excellent surgical outcomes, there continues to be significant preoperative morbidity and potential mortality due to compromise in the delivery room from foramen ovale closure requiring urgent balloon atrial septostomy for stabilization in the first minutes of life. The prenatal diagnosis of D-TGA using fetal echocardiography has aided in the perinatal management and delivery planning of these infants, lowering preoperative morbidity and mortality and preventing delivery room compromise. Fetuses with D-TGA have more highly oxygenated blood supplying the pulmonary arteries and ductus arteriosus which likely results in ductal constriction and increased pulmonary blood flow. This may be the cause of foramen ovale restriction or closure in-utero, which then increases the risk for postnatal compromise at delivery. Theories regarding the cause of the abnormal pulmonary vasculature that may be seen in D-TGA, including aorto-pulmonary collateral formation, have been proposed but to our knowledge, observation of the ultrasound findings throughout mid and late gestation describing the progression of the abnormal fetal physiology have not been previously described. We present a case of D-TGA in which serial assessment using fetal echocardiography enabled observation of the in-utero progression of disease, predicting postnatal compromise and facilitating the planning of life-saving specialized delivery room care and intervention.

PMID: 27589547 [PubMed - indexed for MEDLINE]

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