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Progressive aortic root dilatation in pediatric heart transplant recipients.

Echocardiography. 2017 Jul;34(7):1035-1039

Authors: Sachdeva S, Zhang L, Simpson P, Frommelt PC

BACKGROUND: To determine prevalence, clinical implication, and risk factors for aortic root dilation (ARD) in pediatric heart transplant recipients.
METHODS: Serial echocardiograms were reviewed in all pediatric heart transplant recipients from 1999 to 2014 to assess maximal systolic diameter at the aortic annulus, aortic sinus, aortic sino-tubular (ST) junction, and ascending aorta. ARD was defined by a sinus/annulus ratio >1.56, ST junction/annulus ratio >1.28, and/or ascending aorta/annulus ratio >1.35.
RESULTS: A total of 147 subjects (53% male) were evaluated; 50% had congenital heart disease (CHD). Of the 74 with CHD, 38 had prior aortic arch reconstruction. The median age at transplant was 3 years (7 days-20.3 years) with a median duration of follow-up of 3.88 years (3 months-15 years). Prevalence of ARD significantly increased in the cohort from 15.6% at the initial echocardiogram to 49.6% at later follow-up (P<.0001). The median duration to development of ARD was 7.6 months. There were no significant differences in prevalence of ARD or days to maximum ratio based on the pretransplant diagnosis. Aortic regurgitation was very rare (7 with ≤mild) and did not correlate with ARD or require any interventions.
CONCLUSION: During intermediate follow-up, ARD commonly develops in children post-heart transplant, and prevalence increases with time after transplant. Within 1 year after transplant, almost 50% had developed abnormalities in aortic root size that were not apparent at the initial posttransplant echocardiogram. Preexisting CHD or need for prior arch reconstruction did not increase the risk of ARD.

PMID: 28646501 [PubMed - indexed for MEDLINE]

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Epidemiology and Outcomes of Cardiac Arrest in Pediatric Cardiac ICUs.

Pediatr Crit Care Med. 2017 Oct;18(10):935-943

Authors: Alten JA, Klugman D, Raymond TT, Cooper DS, Donohue JE, Zhang W, Pasquali SK, Gaies MG

OBJECTIVES: In-hospital cardiac arrest occurs in 2.6-6% of children with cardiac disease and is associated with significant morbidity and mortality. Much remains unknown about cardiac arrest in pediatric cardiac ICUs; therefore, we aimed to describe cardiac arrest epidemiology in a contemporary multicenter cardiac ICU cohort.
DESIGN: Retrospective analysis within the Pediatric Cardiac Critical Care Consortium clinical registry.
SETTING: Cardiac ICUs within 23 North American hospitals.
PATIENTS: All cardiac medical and surgical patients admitted from August 2014 to July 2016.
MEASUREMENTS AND MAIN RESULTS: There were 15,908 cardiac ICU encounters (6,498 medical, 9,410 surgical). 3.1% had cardiac arrest; rate was 4.8 cardiac arrest per 1,000 cardiac ICU days. Medical encounters had 50% higher rate of cardiac arrest compared with surgical encounters. Observed (unadjusted) cardiac ICU cardiac arrest prevalence varied from 1% to 5.5% among the 23 centers; cardiac arrest per 1,000 cardiac ICU days varied from 1.1 to 10.4. Over half cardiac arrest occur within 48 hours of admission. On multivariable analysis, prematurity, neonatal age, any Society of Thoracic Surgeons preoperative risk factor, and Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 4, 5 had strongest association with surgical encounter cardiac arrest. In medical encounters, independent cardiac arrest risk factors were acute heart failure, prematurity, lactic acidosis greater than 3 mmol/dL, and invasive ventilation 1 hour after admission. Median cardiopulmonary resuscitation duration was 10 minutes, return of spontaneous circulation occurred in 64.5%, extracorporeal cardiopulmonary resuscitation in 27.2%. Unadjusted survival was 53.2% in encounters with cardiac arrest versus 98.2% without. Medical encounters had lower survival after cardiac arrest (37.7%) versus surgical encounters (62.5%); Norwood patients had less than half the survival after cardiac arrest (35.6%) compared with all others. Unadjusted survival after cardiac arrest varied greatly among 23 centers.
CONCLUSIONS: We provide contemporary epidemiologic and outcome data for cardiac arrest occurring in the cardiac ICU from a multicenter clinical registry. As detailed above, we highlight high-risk patient cohorts and periods of time that may serve as targets for research and quality improvement initiatives aimed at cardiac arrest prevention.

PMID: 28737598 [PubMed - indexed for MEDLINE]

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Coronary Artery Embolism From a Blood Cyst of the Mitral Valve.

Heart Lung Circ. 2017 Dec;26(12):e118-e120

Authors: Pavsic N, Dolenc-Strazar Z, Cerne Cercek A, Klokocovnik T, Prokselj K

Blood cysts are benign, congenital tumours of the heart endothelium, found most commonly on heart valves. Adult cases are rare, since most spontaneously regress with age. However, reports of symptomatic cases with embolic phenomena and valve dysfunction have been described. We present a case of a previously healthy 44-year-old woman with no cardiovascular risk factors who developed acute myocardial infarction caused by coronary artery embolism from a blood cyst of the anterior mitral valve leaflet.

PMID: 28803841 [PubMed - indexed for MEDLINE]

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Pulmonary arterial hypertension in adult congenital heart disease.

Heart. 2018 May 02;:

Authors: Brida M, Gatzoulis MA

Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Significant progress has been made for patients with Eisenmenger syndrome in pathophysiology, prognostication and disease-targeting therapy (DTT), which needs to be applied to routine patient care. Patients with PAH-CHD and systemic to pulmonary shunting may benefit from late defect closure if pulmonary vascular resistance (PVR) is still normal or near normal. Patients with PAH and coincidental defects, or previous repair of CHD should be managed as those with idiopathic PAH. Patients with a Fontan circulation, despite not strictly fulfilling criteria for PAH, may have elevated PVR; recent evidence suggests that they may also benefit from DTT, but more data are required before general recommendations can be made. CHD-PAH is a lifelong, progressive disease; patients should receive tertiary care and benefit from a proactive DTT approach. Novel biomarkers and genetic advances may identify patients with CHD who should be referred for late defect closure and/or patients at high risk of developing PAH despite early closure in childhood. Ongoing vigilance for PAH and further controlled studies are clearly warranted in CHD.

PMID: 29720395 [PubMed - as supplied by publisher]

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Combined spinal-epidural anesthesia for cesarean delivery in a patient with cor triloculare biventriculare.

BMC Anesthesiol. 2017 Aug 29;17(1):115

Authors: Han Y, Zhang Z, Sun Q, Ding K, Han L, Dong M, Xu Y, Ge L

BACKGROUND: Cor triloculare biventriculare accounts for approximately 0.31% of cases of congenital heart disease (CHD). Moreover, people with cor triloculare biventriculare always have shorter life spans, and cases of gestation are rare. There have been various related reports of cor triloculare biventriculare in recent years.
CASE PRESENTATION: We described an emergency cesarean section of a 30-year-old, 38-weeks-pregnant woman suffering from cardiac insufficiency and fetal distress. Combined spinal-epidural anesthesia was performed safely, and a male baby was smoothly delivered 10 min after the procedure without any adverse outcome on the mother or newborn. After surgery, we advised that the patient submit to an echocardiogram examination, which revealed the congenital heart disease cor triloculare biventriculare.
CONCLUSIONS: Combined spinal-epidural anesthesia was administered to a patient with cor triloculare biventricularethis with good effect, ensuring the patient's safety and meeting the need for emergency surgery.

PMID: 28851292 [PubMed - indexed for MEDLINE]

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Mechanism and prognostic role of qR in V1 in patients with pulmonary arterial hypertension.

J Electrocardiol. 2017 Jul - Aug;50(4):476-483

Authors: Waligóra M, Kopeć G, Jonas K, Tyrka A, Sarnecka A, Miszalski-Jamka T, Urbańczyk-Zawadzka M, Podolec P

BACKGROUND: The presence of qR pattern in lead V1 of the 12-lead surface ECG has been proposed as a risk marker of death in patients with pulmonary arterial hypertension (PAH). We aimed to validate these findings in the modern era of PAH treatment and additionally to assess the relation of qR in V1 to PAH severity. We also investigated the possible mechanisms underlying this ECG sign.
METHODS: Consecutive patients with PAH excluding patients with congenital heart defect were recruited between February 2008 and January 2016. A 12-lead standard ECG was acquired and analyzed for the presence of qR in V1 and other potential prognostic patterns. Cardiac magnetic resonance and echocardiography were used for structural (masses and volumes) and functional (ejection fraction, eccentricity index) characterization of left (LV) and right (RV) ventricles. Standard markers of PAH severity were also assessed.
RESULTS: We enrolled 66 patients (19 males), aged 50.0±15.7years with idiopathic PAH (n=52) and PAH associated with connective tissue disease (n=14). qR in V1 was present in 26(39.4%) patients and was associated with worse functional capacity, hemodynamics and RV function. The main structural determinants of qR in V1 were RV to LV volume ratio (OR: 3.99; 95% CI: 1.47-10.8, p=0.007) and diastolic eccentricity index (OR: 15.0; 95% CI: 1.29-175.5, p=0.03). During observation time of 30.5±19.4months, 20 (30.3%) patient died, 13 (50%) patients with qR and 7 (17.5%) patients without qR pattern. Electrocardiographic determinants of survival were qR (HR: 3.06, 95% CI: 1.21-7.4; p=0.02) and QRS duration (HR: 1.02, 95% CI: 1.01-1.04; p=0.01).
CONCLUSIONS: Presence of qR in V1 reflects RV dilation and diastolic interventricular septum flattening. It is a sign of advanced PAH and predicts the risk of death in this population.

PMID: 28256215 [PubMed - indexed for MEDLINE]

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Significant survival advantage of high pulmonary vein index and the presence of native pulmonary artery in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: results from preoperative computed tomography angiography.

Eur J Cardiothorac Surg. 2017 Aug 01;52(2):225-232

Authors: Jia Q, Cen J, Zhuang J, Zhong X, Liu X, Li J, Liang C, Huang M

OBJECTIVES: The prognosis of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA-VSD-MAPCAs) after surgery shows substantial clinical heterogeneity and predictors for outcomes are lacking. This study aimed to assess the predictive value of preoperative cardiac computed tomography angiography (CTA) for survival in patients with PA-VSD-MAPCAs.
METHODS: We retrospectively analysed PA-VSD-MAPCA patients with preoperative CTA who underwent both right ventricular outflow tract reconstruction and MAPCA unifocalization ( n  = 24) or pulmonary artery rehabilitation ( n  = 28). The end-point was overall survival. Prognostic values of CTA were assessed using Cox univariate and multivariate analyses. The significant threshold of independent parameters was calculated using receiver-operating characteristic (ROC) curves.
RESULTS: During a median follow-up of 1145 days, a total of 13 deaths were observed. Multivariate analysis identified a high pulmonary vein index (PVI) [hazard ratio (HR) = 0.03; 95% confidence interval (CI): 0.03, 0.28; P  <   0.01] and the presence of native pulmonary artery (HR = 0.06; 95% CI: 0.10, 0.35; P  <   0.01) as independent positive predictors of better survival. The area under the ROC curve for PVI was 0.79 ( P  <   0.01), and a cut-off point of 438 mm 2 /m 2 was deemed the significant threshold for survival (sensitivity 92%, specificity 72%).
CONCLUSIONS: Preoperational high PVI and native pulmonary artery presence were significant morphologic predictors of a positive survival advantage in PA-VSD-MAPCA patients. A PVI ≥438 mm 2 /m 2 may be a reliable positive prognosticator that could improve the decision-making strategy for PA-VSD-MAPCA patients.

PMID: 28369397 [PubMed - indexed for MEDLINE]

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Radiological features of azygos and hemiazygos continuation of inferior vena cava: A case report.

Medicine (Baltimore). 2018 Apr;97(17):e0546

Authors: Liu Y, Guo D, Li J, Zhang X, He J, Huang M, Dai J, Cai H

RATIONALE: Azygos and hemiazygos continuation of the inferior vena cava (IVC) is uncommon. It is rare especially when it is not associated with congenital heart disease or deep venous thrombosis.
PATIENT CONCERNS: We report an interesting case of an interstitial lung disease with an interrupted IVC with azygous and hemiazygos continuation. A 67-year-old man suffered from cough and shortness of breath.
DIAGNOSES: Computed tomography revealed absence of the hepatic segment of the IVC with azygos and hemiazygos continuation. The patient did not have congenital anomalies of the remaining thoracoabdominal vasculature and viscera. The diagnosis of azygos and hemiazygos continuation of IVC was made by inferior venacavography.
INTERVENTIONS: There was no significant association between interstitial lung disease and expanded azygos and hemiazygos veins. The patient was treated with corticosteroids.
OUTCOMES: After 6 months of follow-up, the patient was asymptomatic.
LESSONS: It is important to recognize the enlarged azygos vein at the confluence with the superior vena cava and in the retrocrural space to avoid misdiagnosis as a right-sided paratracheal mass or retrocrural adenopathy.

PMID: 29703035 [PubMed - indexed for MEDLINE]

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Clinical and Echocardiographic Prevalence and Detection of Congenital and Acquired Cardiac Abnormalities in Girls and Women with the Turner Syndrome.

Am J Cardiol. 2018 Apr 17;:

Authors: Yetman AT, Starr L, Sanmann J, Wilde M, Murray M, Cramer JW

The prevalence of congenital and acquired heart disease in patients with the Turner syndrome (TS) is based on historic cohorts who underwent imaging before the advent of modern day echocardiography. Recent small studies suggest a higher prevalence of cardiac defects. We reviewed clinical and echocardiographic data on 564 girls and women with TS to assess the prevalence of cardiac defects. Echocardiographic review on a subset of this population was performed to assess for diagnostic limitations of echocardiography in assessing for congenital and acquired defects in this patient cohort. Bicuspid aortic valve was present in 39%, aortic coarctation in 21%, and some forms of structural cardiac anomaly in 56%. Failure to perform a complete congenital echocardiogram with suprasternal and high right or left parasternal windows was associated with failure to identify congenital and acquired cardiac defects. In conclusion, major cardiac defects are present in the majority of patients with TS. Echocardiographic technique can be optimized to avoid missing cardiac lesions of potential hemodynamic significance.

PMID: 29731120 [PubMed - as supplied by publisher]

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Incidence and outcomes of neonatal acute kidney injury (AWAKEN): a multicentre, multinational, observational cohort study.

Lancet Child Adolesc Health. 2017 Nov;1(3):184-194

Authors: Jetton JG, Boohaker LJ, Sethi SK, Wazir S, Rohatgi S, Soranno DE, Chishti AS, Woroniecki R, Mammen C, Swanson JR, Sridhar S, Wong CS, Kupferman JC, Griffin RL, Askenazi DJ

Background: Single-center studies suggest that neonatal acute kidney injury (AKI) is associated with poor outcomes. However, inferences regarding the association between AKI, mortality, and hospital length of stay are limited due to the small sample size of those studies. In order to determine whether neonatal AKI is independently associated with increased mortality and longer hospital stay, we analyzed the Assessment of Worldwide Acute Kidney Epidemiology in Neonates (AWAKEN) database.
Methods: All neonates admitted to 24 participating neonatal intensive care units from four countries (Australia, Canada, India, United States) between January 1 and March 31, 2014, were screened. Of 4273 neonates screened, 2022 (47·3%) met study criteria. Exclusion criteria included: no intravenous fluids ≥48 hours, admission ≥14 days of life, congenital heart disease requiring surgical repair at <7 days of life, lethal chromosomal anomaly, death within 48 hours, inability to determine AKI status or severe congenital kidney abnormalities. AKI was defined using a standardized definition -i.e., serum creatinine rise of ≥0.3 mg/dL (26.5 mcmol/L) or ≥50% from previous lowest value, and/or if urine output was <1 mL/kg/h on postnatal days 2 to 7.
Findings: Incidence of AKI was 605/2022 (29·9%). Rates varied by gestational age groups (i.e., ≥22 to <29 weeks =47·9%; ≥29 to <36 weeks =18·3%; and ≥36 weeks =36·7%). Even after adjusting for multiple potential confounding factors, infants with AKI had higher mortality compared to those without AKI [(59/605 (9·7%) vs. 20/1417 (1·4%); p< 0.001; adjusted OR=4·6 (95% CI=2·5-8·3); p=<0·0001], and longer hospital stay [adjusted parameter estimate 8·8 days (95% CI=6·1-11·5); p<0·0001].
Interpretation: Neonatal AKI is a common and independent risk factor for mortality and longer hospital stay. These data suggest that neonates may be impacted by AKI in a manner similar to pediatric and adult patients.
Funding: US National Institutes of Health, University of Alabama at Birmingham, Cincinnati Children's, University of New Mexico.

PMID: 29732396 [PubMed]