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Autonomic Reflex Screen Test Abnormalities in Cold-Induced Sweating Syndrome Type 1.

J Clin Neuromuscul Dis. 2017 Dec;19(2):76-79

Authors: El-Dokla AM, Ferdous J, Ali ST, Alam K

Abstract
Cold-induced sweating syndrome (CISS) is a rare autosomal recessive disease due to mutation in the Cytokine receptor-like factor 1 (CRLF1). The characteristic symptom of CISS is the tendency to sweat profusely especially in the upper body and hands when the patient is exposed to cold temperature. We sought to first report the findings of autonomic reflex screen in a case of CISS type 1 with Cytokine receptor-like factor 1 mutation. Valsalva morphology, Valsalva ratio, and heart rate response to deep breathing were normal for the patient's age. Quantitative sudomotor axon reflex test showed nonlength dependent decrease in the sweat volume. Tilt table revealed evidence of reflex (vasovagal) "syncope," however, the patient was asymptomatic without loss of consciousness.

PMID: 29189552 [PubMed - indexed for MEDLINE]

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Massive myocardial infarction due to the complete occlusion of the left anterior descending coronary artery after blunt chest trauma.

Cardiovasc Revasc Med. 2018 Jul 07;:

Authors: Zuin M, Rigatelli G, Fogato L, Faggian G, Zuliani G, Roncon L

PMID: 30006241 [PubMed - as supplied by publisher]

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Short-term cardiopulmonary efficiency improvement after transcatheter baffle leak closure in a Mustard-operated patient.

J Cardiovasc Med (Hagerstown). 2017 06;18(6):447-449

Authors: Mezzani A, Butera G, Chessa M, Micheletti A, Negura D, Calaciura R, Carminati M

PMID: 23314595 [PubMed - indexed for MEDLINE]

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A Rare Cause of Exercise Intolerance and Progressive Dyspnea in an Elderly Woman.

J Cardiothorac Vasc Anesth. 2017 02;31(1):391-394

Authors: Steck DT, Tawil JN, Boettcher BT, Pagel PS

PMID: 27554233 [PubMed - indexed for MEDLINE]

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Percutaneous management of failed bioprosthetic pulmonary valves in patients with congenital heart defects.

J Cardiovasc Med (Hagerstown). 2017 Jun;18(6):430-435

Authors: Tarzia P, Conforti E, Giamberti A, Varrica A, Giugno L, Micheletti A, Negura D, Piazza L, Saracino A, Carminati M, Chessa M

Abstract
AIMS: We reviewed our center experience in the field of transcatheter pulmonary valve-in-valve implantation (TPViV), that is emerging as a treatment option for patients with pulmonary bioprosthetic valve (BPV) dysfunction.
METHODS: Between April 2008 and September 2015, a total of six patients with congenital heart disease (four men) underwent TPViV due to stenosis of preexisting BPV. Four patients received a Melody Medtronic Transcatheter Pulmonary Valve and two an Edward Sapien Valve.
RESULTS: No procedural-related complications occurred. After valve implantation, right ventricular systolic pressure (RVSP, 80.5 ± 25.3-41.2 ± 8.35 mmHg, P < 0.05), right ventricular outflow tract (RVOT) gradient (55.3 ± 23.4-10.6 ± 3.8 mmHg, P < 0.05), and RVSP-to-aortic pressure (0.75 ± 0.21-0.38 ± 0.21, P = 0.01) fell significantly. Echocardiograms at follow-up revealed a significant reduction in estimated RVSP (88.7 ± 22-21.7 ± 4.7 mmHg, P < 0.05), in RVOT (76.2 ± 17.9-25.7 ± 6.1 mmHg, P = 0.005), and in mean RVOT (40.7 ± 9.9-15.5 ± 4.8 mmHg, P < 0.05) gradients. Cardiac magnetic resonance showed no significant change in biventricular dimensions and function. Symptomatic patients reported improvement of symptoms, although cardiopulmonary exercise did not show any significant differences.
CONCLUSION: TPViV is an effective and well tolerated treatment for BPV dysfunction, improving freedom from surgical reintervention. Long-term studies will redefine the management of dysfunctional RVOT, either native or surrogate.

PMID: 27828833 [PubMed - indexed for MEDLINE]

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Large single centre experience with the Cera™ and CeraFlex™ occluders for closure of interatrial communications: usefulness of the flexible rotation feature.

Cardiovasc Interv Ther. 2018 Jan;33(1):70-76

Authors: Apostolopoulou SC, Tsoutsinos A, Laskari C, Kiaffas M, Rammos S

Abstract
We present our experience with the Cera (CO) and the CeraFlex occluder (CFO) in transcatheter closure of interatrial communications (IAC). Between 2013 and 2016, 201 patients (75 males, 16 with patent foramen ovale), aged 27 ± 19 (5-75) years, underwent percutaneous closure of IAC using CO or CFO in our institution. After transoesophageal imaging, the procedure was aborted in 7 young paediatric (6-13 years old) patients (3 multiple holes, 3 too small septum, 1 leak with 38 mm occlusion balloon). The occluder was removed prior to release in 11 patients (5.7%), while occlusion was successful in 183 patients (94.3%) with 44 CO, 136 CFO, and 3 Cera multifenestrated occluders. There were no deaths, embolizations, or major complications. Small residual shunts were demonstrated in 8 patients immediately after implantation, 4 (8.5%) with CO and 4 (2.9%) with CFO, all disappearing after 3 months. Over 1.8 ± 1.7 year follow-up, all patients improved with 2 asymptomatic, transient pericardial effusions and 5 adults with transient supraventricular arrhythmias, treated medically for 6 months. IAC closure with CO and CFO proved safe with favourable success rates and few, nonserious complications. The CFO flexible rotation feature helped in conforming to various septal anatomies, minimising manoeuvres and possibly post-occlusion leaks.

PMID: 27832479 [PubMed - indexed for MEDLINE]

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First Experience With Levosimendan Therapy After Correction of Congenital Heart Disease.

J Cardiothorac Vasc Anesth. 2017 02;31(1):e19-e21

Authors: Giordano R, Cantinotti M, Mannacio VA, Palma G

PMID: 28277244 [PubMed - indexed for MEDLINE]

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Comparison of MR flow quantification in peripheral and main pulmonary arteries in patients after right ventricular outflow tract surgery: A retrospective study.

J Magn Reson Imaging. 2017 Dec;46(6):1839-1845

Authors: Rutz T, Meierhofer C, Naumann S, Martinoff S, Ewert P, Stern HC, Fratz S

Abstract
PURPOSE: To compare the quantification of pulmonary stroke volume (SV) by phase contrast magnetic resonance (PC-MR) in the main pulmonary artery (MPA) to the sum of SVs in both peripheral pulmonary arteries (PPA) in different right ventricular (RV) outflow pathologies.
MATERIALS AND METHODS: Pulmonary SV was determined by PC-MR in the MPA and the PPA in healthy individuals (H, n = 54), patients after correction for tetralogy of Fallot with significant pulmonary regurgitation and without pulmonary or RV outflow tract stenosis (PR, n = 50), and in patients with RV outflow tract or pulmonary valve stenosis (PS, n = 50). Resulting SVs were compared to aortic SV in the ascending aorta.
RESULTS: Mean age was similar between the groups: H 28 ± 17 vs. PR 24 ± 11 vs. PS 22 ± 10 years. Bland-Altman analyses revealed in all groups a relatively small systemic (bias) but large random error (limits of agreement) for pulmonary SV determined in the MPA as compared to summed SVs in the PPA. The largest limits of agreement were present in PS patients: H: MPA 3.9% (-11, + 19) vs. PPA 0.4% (-15, + 15); PR: MPA 5.2% (-25, + 36) vs. PPA 0.6% (-24, + 26); PS: MPA 5% (-36; + 46), PPA -0.03% (-34, + 35).
CONCLUSION: The accuracy of PC-MR in the MPA is reasonable; however, a large random error (precision) is observed that is most pronounced in PS patients. This potential error should be taken into consideration when interpreting MPA flow measurements.
LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2017;46:1839-1845.

PMID: 28301100 [PubMed - indexed for MEDLINE]

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TIMI Risk Index as a Predictor of 30-Day Outcomes in Patients With Acute Pulmonary Embolism.

Heart Lung Circ. 2018 Feb;27(2):190-198

Authors: Zuin M, Conte L, Picariello C, Pastore G, Vassiliev D, Lanza D, Zonzin P, Zuliani G, Rigatelli G, Roncon L

Abstract
BACKGROUND: Available studies have already identified age, heart rate (HR) and systolic blood pressure (SBP) as strong predictors of early mortality in acute pulmonary embolism (PE).
MATERIAL AND METHODS: One-hundred-seventy patients, with acute PE confirmed on computed tomography angiography (CTA) were enrolled. Thrombolysis In Myocardial Infarction (TIMI) risk index (TRI) was calculated using the formula [heart rate (HR) x (AGE/102)/ systolic blood pressure (SBP)]. Study outcomes were 30-day mortality and/or clinical deterioration.
RESULTS: Receiver operating characteristics (ROC) curve revealed that a TRI ≥45 was highly specific for both outcomes (AUC 0.91, 95% CI 0.83-0.98, p<0.0001) with a positive predictive value (PPV) and negative predictive value (NPV) of 8.3 and 96% for 30-day mortality while PPV and NPV for 30-day mortality and/or clinical deterioration were 21.1 and 98.2%, respectively. Multivariate regression analysis showed that TRI ≥45 was an independent predictor of 30-day mortality (O.R. 22.24, 95% CI 2.54-194.10, p=0.005) independently from positive cTnI and RVD (O.R. 9.57, 95% CI 1.88-48.78, p=0.007; OR 24.99, 95% CI 2.84-219.48, p=0.004). Similarly, 30-day mortality and/or clinical deterioration was predicted by TRI ≥45 (O.R. 11.57, 95% CI 2.36-56.63, p=0.003) and thrombolysis (3.83, 95% CI 1.04-14.09, p=0.043), independently from age, RVD and positive cTnI. Cox regression analysis confirmed the role of TRI as independent predictor for both outcomes. Mantel-Cox analysis showed that after 30-day follow-up there was a statistically significant difference in the distribution of survival between patients with and without TRI ≥45 [log rank (Mantel-Cox) chi-square 17.04, p<0.0001].
CONCLUSIONS: Thrombolysis In Myocardial Infarction (TIMI) risk index (TRI) predicted both 30-days mortality (all-causes) and/or clinical deterioration in patients with acute PE.

PMID: 28487060 [PubMed - indexed for MEDLINE]

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Conventional and right-sided screening for subcutaneous ICD in a population with congenital heart disease at high risk of sudden cardiac death.

Ann Noninvasive Electrocardiol. 2017 Nov;22(6):

Authors: Alonso P, Osca J, Rueda J, Cano O, Pimenta P, Andres A, Sancho MJ, Martinez L

Abstract
BACKGROUND: Information regarding suitability for subcutaneous defibrillator (sICD) implantation in tetralogy of Fallot (ToF) and systemic right ventricle is scarce and needs to be further explored. The main objective of our study was to determine the proportion of patients with ToF and systemic right ventricle eligible for sICD with both, standard and right-sided screening methods. Secondary objectives were: (i) to study sICD eligibility specifically in patients at high risk of sudden cardiac death, (ii) to identify independent predictors for sICD eligibility, and (iii) to compare the proportion of eligible patients in a nonselected ICD population.
METHODS: We recruited 102 patients with ToF, 33 with systemic right ventricle, and 40 consecutive nonselected patients. Conventional electrocardiographic screening was performed as usual. Right-sided alternative screening was studied by positioning the left-arm and right-arm electrodes 1 cm right lateral of the xiphoid midline. The Boston Scientific ECG screening tool was utilized.
RESULTS: In high-risk patients with ToF, eligibility was higher with right-sided screening in comparison with standard screening (61% vs. 44%; p = .018). Eligibility in high-risk right ventricle population was identical with both screening methods (77%, p = ns). The only independent predictor for sICD eligibility was QRS duration.
CONCLUSION: In high-risk patients with ToF, right-sided implantation of the sICD could be an alternative to a conventional ICD. In patients with a systemic right ventricle, implantation of a sICD is an alternative to a conventional sICD.

PMID: 28508439 [PubMed - indexed for MEDLINE]

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