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Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein's anomaly: comparison with CMR-derived ejection fraction in 49 patients.

Eur Heart J Cardiovasc Imaging. 2016 Aug;17(8):930-5

Authors: Kühn A, Meierhofer C, Rutz T, Rondak IC, Röhlig C, Schreiber C, Fratz S, Ewert P, Vogt M

Abstract
AIMS: Ebstein's anomaly (EA) is often associated with right ventricular (RV) dysfunction. Data on echocardiographic quantification of RV function are, however, rare. The aim of this study was to determine how non-volumetric echocardiographic indices and qualitative assessment of global systolic RV function correlate with cardiovascular magnetic resonance (CMR)-derived RV ejection fraction (EF).
METHODS AND RESULTS: We compared six echocardiographic indices and qualitative assessment of RV function with the gold standard CMR. A total of 49 unoperated patients with EA and a mean age of 32 ± 18 years were examined. Tricuspid annular plane systolic excursion, tissue Doppler myocardial velocities (peak S and IVA) and 2D strain and strain rate measures for the RV were compared with CMR-derived EF. Only 2D global longitudinal strain (2D-GLS), out of the six parameters investigated, showed a weak, although statistically significant correlation with CMR-derived RVEF (R = -0.4, P = 0.01). Using a cut-off value of -20.15, 2D-GLS sensitivity (77%) and specificity (46%) in detecting patients with a CMR-derived EF of <50% were comparable with qualitative assessment (sensitivity 77%, specificity 45%).
CONCLUSION: Overall echocardiographic parameters of RV function correlate poorly with CMR-derived EF in patients with EA. Only 2D global longitudinal RV strain correlated weakly with CMR-derived RVEF. However, the sensitivity and specificity for detecting RV dysfunction using 2D strain imaging were comparable with qualitative RV functional assessment.

PMID: 26453545 [PubMed - indexed for MEDLINE]

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Pregnancy-associated spontaneous coronary artery dissection: insights from a case series of 13 patients.

Eur Heart J Cardiovasc Imaging. 2017 Jan;18(1):54-61

Authors: Cade JR, Szarf G, de Siqueira ME, Chaves Á, Andréa JC, Figueira HR, Gomes MM, Freitas BP, Filgueiras Medeiros J, Dos Santos MR, Fiorotto WB, Daige A, Gonçalves R, Cantarelli M, Alves CM, Echenique L, de Brito FS, Perin MA, Born D, Hecht H, Caixeta A

Abstract
AIMS: We sought to present a series of 13 pregnancy-associated spontaneous coronary artery dissection (P-SCAD), their angiographic and multimodal imaging findings, acute phase treatment, and outcomes.
METHODS AND RESULTS: Between 2005 and 2015, 13 cases of P-SCAD were collected from a database of 11 tertiary hospitals. The mean age was 33.8 ± 3.7 years; most patients had no risk factors for coronary artery disease, and the majority were multiparous. P-SCAD occurred during the puerperium in 12 patients with a median time of 10 days. Only one patient presented with P-SCAD in the 37th week of pregnancy, and she was the only patient who died in this series. Six patients (46%) presented with ST-segment elevation acute myocardial infarction (STEMI), six (46%) presented with non-STEMI, and one presented with unstable angina; one-third of women had cardiogenic shock. In 12 patients, the dissection involved the left anterior descending or circumflex artery, and it extended to the left main coronary artery in 6 patients. Intravascular ultrasound or optical coherence tomography helped to confirm diagnosis and guide treatment in 46% of cases. Seven women were managed clinically; percutaneous coronary intervention was performed in five cases, and coronary artery bypass grafting was performed in one patient.
CONCLUSION: In these 13 cases of P-SCAD, clinical presentation commonly included acute myocardial infarction and cardiogenic shock. Multivessel dissections and involvement of the left coronary artery and left main coronary artery were highly prevalent. Clinicians must be aware of angiographic appearances of P-SCAD for prompt diagnosis and management in these high-risk patients.

PMID: 26928981 [PubMed - indexed for MEDLINE]

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Multiple congenital arteriovenous fistulas draining into the right internal mammary vein with a large internal mammary varicose vein.

Eur Heart J Cardiovasc Imaging. 2016 Sep;17(9):1067

Authors: Rybicka J, Kowalski M, Demkow M, Dobrowolski P, Hoffman P

PMID: 27225805 [PubMed - indexed for MEDLINE]

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Computer-generated real-time digital holography: first time use in clinical medical imaging.

Eur Heart J Cardiovasc Imaging. 2016 Aug;17(8):845-9

Authors: Bruckheimer E, Rotschild C, Dagan T, Amir G, Kaufman A, Gelman S, Birk E

Abstract
AIMS: Assessment of the feasibility of creating real-time interactive 3D digital holograms in a standard catheterization laboratory. 3D medical images are typically displayed and interacted with on 2D screens limiting their usefulness. A digital computer-generated real-time holographic display of patient's 3D data could provide a spatially accurate image with all the depth cues and afford interaction within the image.
METHODS AND RESULTS: We performed a feasibility study of creating real-time interactive 3D digital holograms with a purpose-built prototype using intraprocedural data from 3D rotational angiography and live 3D transesophageal echocardiography. The primary objective was to demonstrate that all the anatomical landmarks identified on standard imaging can be similarly identified using dynamic and static holographic images. The secondary objective was to demonstrate the usability of interactions with the image. Parameters were assessed by a rating scale. Eight patients were enrolled of whom five underwent transcatheter ASD closure using 3DTEE and three patients were evaluated by 3D rotational angiography. In all cases dynamic real-time and static 3D holograms were created in standard cath lab conditions. Four individual observers identified all anatomical landmarks on the holographic display independently from the 2D display. Interactions with the hologram including marking, cropping and rotation were performed. There were no adverse events.
CONCLUSIONS: This study demonstrates, for the first time, the feasibility of generating high quality, clinically relevant, 3D real-time colour dynamic holograms in a standard clinical setting with real patient volumetric data. The impact of computer-generated holography needs to be evaluated in controlled clinical trials.

PMID: 27283456 [PubMed - indexed for MEDLINE]

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Comparison of Intraoperative Aminophylline Versus Furosemide in Treatment of Oliguria During Pediatric Cardiac Surgery.

Pediatr Crit Care Med. 2016 Aug;17(8):753-63

Authors: Onder AM, Rosen D, Mullett C, Cottrell L, Kanosky S, Grossman OK, Iqbal HI, Seachrist E, Samsell L, Gustafson K, Rhodes L, Gustafson R

Abstract
OBJECTIVES: To determine if intraoperative aminophylline was superior to furosemide to prevent or attenuate postoperative cardiac surgery-associated acute kidney injury.
DESIGN: Single-center, historical control, retrospective cohort study.
SETTING: PICU, university-affiliated children's hospital.
PATIENTS: Children with congenital heart disease in PICU who received furosemide or aminophylline to treat intraoperative oliguria.
INTERVENTIONS: Intraoperative oliguria was treated either with furosemide (September 2007 to February 2012) or with aminophylline (February 2012 to June 2013). The postoperative 48 hours renal outcomes of the aminophylline group were compared with the furosemide group. The primary outcomes were acute kidney injury and renal replacement therapy use at 48 hours postoperatively. Surgical complexity was accounted for by the use of Risk Adjustment for Congenital Heart Surgery-1 score.
MEASUREMENTS AND MAIN RESULTS: The study involves 69 months of observation. There were 200 cases younger than 21 years old reviewed for this study. Eighty-five cases (42.5%) developed acute kidney injury. The aminophylline group patients produced significantly more urine (mL/kg/hr) during the first 8 hours postoperatively than furosemide patients (5.1 vs 3.4 mL/kg/hr; p = 0.01). The urine output at 48 hours postoperatively was similar between the two groups. There was no difference in acute kidney injury incidence at 48 hours between the aminophylline and furosemide groups (38% vs 47%, respectively; p = 0.29). Fewer aminophylline group subjects required renal replacement therapy compared to the furosemide group subjects (n = 1 vs 7, respectively; p = 0.03). In the multi-variant predictive model, intraoperative aminophylline infusion was noted as a negative predictive factor for renal replacement therapy, but not for cardiac surgery-associated acute kidney injury.
CONCLUSION: The intraoperative use of aminophylline was more effective than furosemide in reversal of oliguria in the early postoperative period. There were less renal replacement therapy-requiring acute kidney injury in children in the aminophylline group. Future prospective studies of intraoperative aminophylline to prevent cardiac surgery-associated acute kidney injury may be warranted.

PMID: 27355823 [PubMed - indexed for MEDLINE]

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Early pulmonary arterial hypertension immediately after closure of a ventricular or complete atrioventricular septal defect beyond 6months of age.

Int J Cardiol. 2017 Feb 01;228:313-318

Authors: Bambul Heck P, Eicken A, Kasnar-Samprec J, Ewert P, Hager A

Abstract
BACKGROUND: Pulmonary arterial hypertension (PAH) is virtually absent after closure of ventricular septal defect (VSD) in the first six months of life. However the prevalence of PAH in patients, who underwent VSD closure later, is not clear. The aim of this study was to analyse the prevalence of PAH after a successful VSD closure after the age of 6months and whether there are risk factors for developing PAH.
METHODS: Echocardiographic and right heart catheter data of patients with VSD or complete atrioventricular septal defect, who underwent VSD closure after the age of 6months in our institution between 01/2005 and 06/2014, were retrospectively analyzed. PAH was defined as mean pulmonary arterial pressure (mPAP) of ≥25mmHg or tricuspid regurgitation jet velocity of ≥3.5m/s.
RESULTS: In 228 patients (median age at shunt closure 4.0years, range 0.5-69) and 174 complete follow-up data (median follow-up 3.7years, range 0.5-39.4), 9 patients needed pulmonary vasodilator therapy after shunt closure, 4 of them temporarily for up to 79months. Three patients are still on vasodilator treatment 1, 2.6 and 6years after surgery, other two were lost to follow-up. Another 6 patients with preoperatively borderline hemodynamics due to elevated mPAP and pulmonary vascular resistance, recovered well without signs of postoperative PAH.
CONCLUSION: With the current practice for safe late VSD closure, PAH is very rare at least in the first years of follow-up. In most patients with perioperative PAH, this condition appears to be transient and shows good response on pulmonary vasodilator treatment.

PMID: 27866021 [PubMed - indexed for MEDLINE]

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Survival and cardiovascular events after coarctation-repair in long-term follow-up (COAFU): Predictive value of clinical variables.

Int J Cardiol. 2017 Feb 01;228:347-351

Authors: Bambul Heck P, Pabst von Ohain J, Kaemmerer H, Ewert P, Hager A

Abstract
OBJECTIVE: Long-term sequelae and events after coarctation repair are well described. However, the predictive value of variables from clinical follow-up investigation for late events and survival has rarely been investigated.
METHODS: All patients who participated in the prospective cross-sectional COALA Study in 2000 with a structural clinical investigation including blood pressure measurement and symptom-limited exercise test were contacted for reevaluation of survival, current clinical status and major cardiovascular events.
RESULTS: Of 273 eligible patients, 209 were available for follow-up. Nine patients had died at a median age of 46years (range 30-64years), five of them due to cardiovascular complications. Late mortality after surgical intervention was 5.7% with a median age of 41years (range 16-64years). Twenty-five patients had a major cardiovascular event: 12 had procedures at the aortic valve or aortic arch, 8 had procedures for restenosis, 2 had endocarditis, 2 had a cerebrovascular insult and 1 an aortic dissection. The presence of bicuspid aortic valve (p=0.009), brachial-ankle blood pressure gradient >20mmHg (p<0.001) and reduced left ventricular function (p=0.002) correlated with major cardiovascular events.
CONCLUSION: Surgical correction of coarctation of the aorta shows fairly low mortality in the long-term follow-up. Late morbidities include recoarctation, but also the consequences of the hemodynamics produced by a congenital bicuspid aortic valve, presence of which is predictive for aortic valve procedures: however the predictive value of clinical variables is limited.

PMID: 27866026 [PubMed - indexed for MEDLINE]

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Transcatheter closure of calcified patent ductus arteriosus in older adult patients: Immediate and 12-month follow-up results.

Congenit Heart Dis. 2017 May;12(3):289-293

Authors: Gu X, Zhang Q, Sun H, Fei J, Zhang X, Kutryk MJ

Abstract
OBJECTIVE: To present our experience in transcatheter closure of calcified patent ductus arteriosus (PDA) in older adult patients, which has rarely been reported.
PATIENTS: From 2009 to 2014, a total of 16 patients (median age 58 years) with calcified PDA underwent transcatheter closure in our center. All patients were symptomatic with major symptoms being exertional dyspnea (in 12), palpitations (in 8), and fatigue (in 5). A continuous murmur was heard in all patients. The median ductus diameter was 4 mm (range 3-7 mm). The median Qp/Qs was 1.6 (range 1.4-2.9).
INTERVENTIONS: Transcatheter closure was performed for all patients. The size of the occluder selected was 2-3 mm greater than the narrowest portion of PDA. We experienced difficulties in advancing the multipurpose catheter through the calcified duct in about one third of patients (5/16). Considering that calcified tissue has a greater tendency to rupture, hence, to close PDA in these patients, they adopted the retrograde wire-assisted technique and modified the procedure to reduce the shear stress of sheath and avoid any sheath kinking. For the remaining 11 patients, the advancement of the multipurpose catheter through the calcified duct was smooth and the conventional antegrade approach was applied.
OUTCOME MEASURES: Clinical examination, standard 12-lead electrocardiography, chest x-ray, and transthoracic echocardiography were performed before hospital discharge, at 1-, 3-, 6-, and 12-months follow-ups.
RESULTS: All PDAs were successfully closed. There were no deaths. Three patients had a trivial residual shunt, with one also having intravascular hemolysis. Following pharmacological treatment, hemolysis signs vanished at 7 days postprocedure. The trivial residual shunt disappeared in all three patients at 3-month follow-up. No new-onset residual shunt, device embolization, device dislocation, infective endocarditis, or embolism was observed at all follow-up time points.
CONCLUSION: Successful closure of calcified PDA with few complications in older adult patients was achieved using the duct occluder.

PMID: 27874259 [PubMed - indexed for MEDLINE]

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A first population-based long-term outcome study in adults with repaired tetralogy of Fallot in Malta.

Congenit Heart Dis. 2017 May;12(3):301-308

Authors: Caruana M, Grech V

Abstract
OBJECTIVES: To determine overall and reintervention-free survival for repaired Maltese tetralogy of Fallot patients and to investigate the potential impact of gender, age at repair, genetic syndromes, previous palliation, and type of repair on these outcomes.
DESIGN: All 130 tetralogy of Fallot patients born before the end of 1997 included in the local database were extracted. Surgical repair type, age at repair and operative survival were analyzed among the 103/130 repaired patients. Kaplan-Meier survival analyses were performed on the 75 repair survivors with complete follow-up data (mean follow-up 26.37 ± 9.27 (range 9.95-51.21) years).
RESULTS: Patients born after 1985 were operated at a younger age (median 1.28 years) compared with patients born before 1985 (median 9.64 years) (P < .001). Transannular patch repair was the commonest operation among patients born after 1985 (43.90%), while repair without transannular patch use prevailed among those born before 1985 (66.13%). 90.24% of patients born after 1985 survived reparative surgery compared with 70.37% of those born before 1985. Of the 75 repair survivors, 7 (9.33%) died of cardiac causes and 22 (29.33%) needed reintervention during follow-up. Overall estimated mean survival was 45.56 years (95% CI 41.67-49.24) with estimated survival rate of 77.5% at 40 years from repair. Estimated mean reintervention-free survival was 37.71 years (95% CI 33.75-41.66) with estimated reintervention-free survival rate of 59.2% at 40 years. Patients with genetic syndromes had significantly lower overall survival after repair. Transannular patch repair was associated with significantly lower reintervention-free survival (median 32.37 years (95% CI 12.75-51.99)) compared with repair without transannular patch [median 44.21 years (95% CI 43.06-45.35); P = .03].
CONCLUSIONS: Although survival after tetralogy of Fallot repair in contemporary patients is very good, cardiac death can occur at any stage and structural reintervention is common. Regular follow-up with imaging and rhythm monitoring remains of utmost importance in all patients.

PMID: 27893189 [PubMed - indexed for MEDLINE]

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The prevalence of sexual dysfunction and its association with quality of life in adults with congenital heart disease.

Int J Cardiol. 2017 Feb 01;228:953-957

Authors: Neiman A, Ginde S, Earing MG, Bartz PJ, Cohen S

Abstract
BACKGROUND: The prevalence of sexual dysfunction (SD) and its impact on quality of life (QOL) in adults with congenital heart disease (CHD) is not well known. The aims of this study were to: determine the prevalence of SD, evaluate the risk factors associated with SD, and determine the association between SD and QOL in adults with CHD.
METHODS: This was a cross-sectional study of adults (≥18years) with CHD presenting for routine follow-up at our institution. Subjects completed the CDC HRQOL-14 "Health Days Measure" to assess mental and physical health, and either the Sexual Health Inventory for Men or the Female Sexual Function Index to assess sexual function. Baseline characteristics were obtained via chart review.
RESULTS: 105 subjects were enrolled. The mean age was 31.9±11.7years, 53 (51%) were men, 81% were NYHA Functional Class 1, and 76% had moderate or complex CHD. The rates of SD were 28% overall in the cohort, 30% in men and 25% in women. Men with SD were more likely to be taking spironolactone (p<0.001) and digoxin (p=0.002). Men with SD reported a greater number of days of poor mental health (p=0.004), feeling anxious, worried or tense (p=0.003), needing assistance (p=0.042), and inhibited activity of daily living (p=0.009). Women with SD were more likely to have atrial arrhythmias (p=0.002) and to report fewer days feeling healthy and energetic (p=0.031).
CONCLUSIONS: SD is highly prevalent and associated with several indicators of worse health-related QOL in this young and well-functioning population.

PMID: 27912205 [PubMed - indexed for MEDLINE]

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