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Aortic dilatation and outcome in women with Turner syndrome.

Heart. 2018 Oct 27;:

Authors: Duijnhouwer AL, Bons LR, Timmers HJLM, van Kimmenade RRL, Snoeren M, Timmermans J, van den Hoven AT, Kempers M, van Dijk APJ, Fleischer K, Roos-Hesselink JW

BACKGROUND: Women with Turner syndrome (TS) are at increased risk of aortic dissection, which is related to ascending aortic diameter. However, the relation between aortic diameter and outcome is not well determined. This study evaluates the prevalence of aortic dilatation, the growth rate of the aorta and the risk of aortic complications in adults with TS.
METHODS: Single centre, retrospective study of all women with TS followed with a strict protocol in an outpatient TS clinic. Aortic diameters were analysed using advanced imaging. The primary outcome was a combined endpoint of aortic-related mortality, aortic dissection and preventive aortic surgery. The secondary endpoint was aortic growth and prevalence of aortic dilatation, defined as an aortic size index >20 mm/m2 at baseline.
RESULTS: At least one cardiac MR/CT was available in 268 women with TS, having median age of 28.7 (IQR: 21.3-39.7) years. Aortic dilatation was present in 22%. Linear regression identified independent factors associated with larger aortic diameters: age (coefficient=0.23; p<0.001), hypertension (coefficient=2.7; p<0.001), bicuspid aortic valve (coefficient=3.3; p<0.001), 45XO karyotype (coefficient=1.7; p=0.002), weight (coefficient=0.075; p<0.001) and growth hormone treatment (coefficient=1.4; p=0.044). During follow-up (6.8±3.2 years), five women (2%) reached the primary endpoint (two dissections, three aortic surgery). Women withmore than one scan (n=171; 1015 patient-years follow-up), the median aortic growth was 0.20 (IQR: 0.00-0.44) mm/year. In multivariate analysis, aortic growth was not associated with baseline aortic diameter or other variables.
CONCLUSIONS: Aortic dilatation is common and known associations were confirmed in large adult TS cohort However, aortic dissection, related mortality and preventive aortic surgery are rare. Growth hormone treatment in childhood was associated with aortic dimensions.

PMID: 30368486 [PubMed - as supplied by publisher]

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Selection of the Best of 2017 in Congenital Heart Disease.

Rev Esp Cardiol (Engl Ed). 2018 Apr;71(4):302-303

Authors: García-Aranda B, Sarnago F, Velázquez MT, Mendoza A, López-Gude MJ, Alonso-González R

PMID: 29366713 [PubMed - indexed for MEDLINE]

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Left-side pericardial agenesis: Putting the pieces together.

Echocardiography. 2018 03;35(3):420-422

Authors: Ruivo C, Ladeiras-Lopes R, Faria R, Ferreira N, Gama V

A 56-year-old woman complained of atypical chest pain. At initial diagnostic workup, chest radiograph and transthoracic echocardiogram depict specific findings that raised the suspicion of pericardial agenesis. Cardiac computed tomography showed extreme levoposition of the heart and interposition of lung parenchyma between the aorta and the pulmonary artery. Those findings were consistent with the diagnosis of left-side pericardial agenesis, which was subsequently confirmed in cardiac magnetic resonance study. Left-side pericardial agenesis is rare and can result in nonspecific symptoms. An integrated multimodality imaging approach may provide incremental value on diagnosis approach. Its benign prognosis allows a conservative approach.

PMID: 29399902 [PubMed - indexed for MEDLINE]

Hospitalization Trends and Health Resource Use for Adult Congenital Heart Disease-Related Heart Failure.

J Am Heart Assoc. 2018 Aug 07;7(15):e008775

Authors: Burchill LJ, Gao L, Kovacs AH, Opotowsky AR, Maxwell BG, Minnier J, Khan AM, Broberg CS

Background This study assessed trends in heart failure ( HF) hospitalizations and health resource use in patients with adult congenital heart disease ( ACHD ). Methods and Results The Nationwide Inpatient Sample was used to compare ACHD with non- ACHD HF hospitalization and health resource trends. Health resource use was assessed using total hospital charges, hospital length of stay, and procedural burden. A total of 87 175±2676 ACHD -related HF hospitalizations occurred between 1998 and 2011. During this time, ACHD HF hospitalizations increased 91% (4620±438-8809±740, P<0.0001) versus a 21% increase in non- ACHD HF hospitalizations ( P=0.003). ACHD HF hospitalization was associated with longer length of stay ( ACHD HF versus non- ACHD HF, 7.2±0.09 versus 6.8±0.02 days; P<0.0001), greater procedural burden, and higher charges ($81 332±$1650 versus $52 050±$379; P<0.0001). ACHD HF hospitalization charges increased 258% during the study period ($26 533±$1816 in 1998 versus $94 887±$8310 in 2011; P=0.0002), more than double that for non- ACHD HF ( P=0.04). Patients with ACHD HF hospitalized in high-volume ACHD centers versus others were more likely to undergo invasive hemodynamic testing (30.2±0.6% versus 20.7±0.5%; P<0.0001) and to receive cardiac resynchronization/defibrillator devices (4.7±0.3% versus 1.8±0.2%; P<0.0001) and mechanical circulatory support (3.9±0.2% versus 2.4±0.2%; P<0.0001). Conclusions ACHD -related HF hospitalizations have increased dramatically in recent years and are associated with disproportionately higher costs, procedural burden, and health resource use.

PMID: 30371225 [PubMed - in process]

Advanced Imaging to Phenotype Patients With a Systemic Right Ventricle.

J Am Heart Assoc. 2018 Oct 16;7(20):e009185

Authors: Helsen F, Claus P, Van De Bruaene A, Claessen G, La Gerche A, De Meester P, Claeys M, Gabriels C, Petit T, Santens B, Troost E, Voigt JU, Bogaert J, Budts W

Background Reduced ventricular function and decreased exercise capacity are widespread in adults with complete transposition of the great arteries after atrial switch ( TGA -Mustard/Senning) and congenitally corrected TGA (cc TGA ). Advanced imaging techniques may help to better phenotype these patients and evaluate exercise cardiac response. Methods and Results Thirty-three adults with a systemic right ventricle (70% TGA -Mustard/Senning, 37±9 years of age, 24% female, 94% New York Heart Association class I- II ) underwent echocardiogram, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging at rest and during a 4-stage free-breathing bicycle test. They were compared with 12 healthy controls (39±10 years of age, 25% female, all New York Heart Association class I). TGA -Mustard/Senning patients had a higher global circumferential strain (-15.8±3.6 versus -11.2±5.2%, P=0.008) when compared with cc TGA , whereas global longitudinal strain and systemic right ventricle contractility during exercise were similar in both groups. Septal extracellular volume ( ECV ) in cc TGA was significantly higher than in TGA -Mustard/Senning (30.2±2.0 versus 27.1±2.7%, P=0.005). During exercise, TGA -Mustard/Senning had a fall in end-diastolic volume and stroke volume (11% and 8%, respectively; both P≤0.002), whereas cc TGA could increase their stroke volume in the same way as healthy controls. Because of a greater heart rate reserve in TGA -Mustard/Senning ( P for interaction=0.010), cardiac index and peak oxygen uptake were similar between both patient groups. Conclusions Caution should be exercised when evaluating pooled analyses of systemic right ventricle patients, given the differences in myocardial contraction pattern, septal extracellular volume, and the exercise response of TGA -Mustard/Senning versus cc TGA patients. Longitudinal follow-up will determine whether abnormal exercise cardiac response is a marker of earlier failure.

PMID: 30371262 [PubMed - in process]

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Parkes Weber syndrome-Diagnostic and management paradigms: A systematic review.

Phlebology. 2017 Jul;32(6):371-383

Authors: Banzic I, Brankovic M, Maksimović Ž, Davidović L, Marković M, Rančić Z

Objectives Parkes Weber syndrome is a congenital vascular malformation which consists of capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation. Although Parkes Weber syndrome is a clinically distinctive entity with serious complications, it is still frequently misdiagnosed as Klippel-Trenaunay syndrome that consists of the triad capillary malformation, venous malformation, and lymphatic malformation. Methods We performed a systematic review investigating clinical, diagnostic, and treatment modalities of Parkes Weber syndrome (PubMed/MEDLINE, Embase, and Cochrane databases). Thirty-six publications (48 patients) fulfilled the eligibility criteria. Results The median age of patients was 23 years (IQR, 8-32), and 24 (50.0%) were males. Lower extremity was affected in 42 (87.5%) and upper extremity in 6 (12.5%) patients; 15 (31.3%) patients developed high-output heart failure; 12 (25.0%) patients had chronic venous ulcerations, whereas 4 (8.3%) manifested distal arterial ischemia. The spinal arteriovenous malformations were reported in six (12.5%) patients and coexistence of aneurysmatic disease in five (10.4%) patients. The most frequently utilized invasive treatments were embotherapy followed by amputation and surgical arteriovenous malformation resection, and occasionally stent-graft implantation. All modalities showed clinical improvement. However, long follow-up and outcome remained unclear. Conclusion A diagnosis of Parkes Weber syndrome should be made on the presence of capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation (as main defect) in overgrowth extremity. Arteriovenous malformation presents the criterion for distinguishing Parkes Weber syndrome from Klippel-Trenaunay syndrome, which is substantial for treatment strategy. The primary management goal should be patient's quality of life improvement and complication reduction. Embolization alone/combined with surgical resection targeting occlusion or removal of arteriovenous malformation "nidus" reliably leads to clinical improvement.

PMID: 27511883 [PubMed - indexed for MEDLINE]

ESC Working Group Position Paper.

Eur Heart J. 2018 Oct 29;:

Authors: Chessa M, Baumgartner H, Michel-Behnke I, Berger F, Budts W, Eicken A, Søndergaard L, Stein J, Wiztsemburg M, Thomson J

PMID: 30376060 [PubMed - as supplied by publisher]

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Transconduit puncture without per-procedural echocardiography in nonfenestrated extracardiac Fontan using a simplified approach guided by electroanatomic mapping.

Heart Rhythm. 2018 04;15(4):631-632

Authors: Laredo M, Soulat G, Ladouceur M, Zhao A

PMID: 29175487 [PubMed - indexed for MEDLINE]

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Safety and efficacy of atrial antitachycardia pacing in congenital heart disease.

Heart Rhythm. 2018 04;15(4):543-547

Authors: Kramer CC, Maldonado JR, Olson MD, Gingerich JC, Ochoa LA, Law IH

BACKGROUND: Intra-atrial reentrant tachycardia (IART) is a common sequela in the congenital heart disease (CHD) population, and it significantly increases morbidity and mortality. Atrial antitachycardia devices (ATDs) capable of atrial antitachycardia pacing (ATP) therapy have been used to manage IART in the CHD population, but there are limited data on their safety and efficacy.
OBJECTIVES: To determine whether ATD implantation was associated with reduced direct current (DC) cardioversions and to compare ATP success between different CHD diagnoses and ATP programs.
METHODS: A single-center retrospective chart review was performed on CHD patients with ATDs. Demographic data were collected in addition to the number of DC cardioversions required before and after ATD implantation; data on ATP efficacy and the specific ATP program utilized; and adverse events related to ATD implantation or subsequent ATP treatments.
RESULTS: ATD implantation in 91 CHD patients was associated with a significant reduction in DC cardioversions (P < .01). Overall, 72% of IART episodes were successfully terminated by ATP. Patients with levo-transposition of the great arteries experienced lower rates of ATP success than the remainder of the cohort (P < .01). There was no evidence of degeneration to ventricular arrhythmia or death directly attributed to ATP.
CONCLUSION: ATD implantation was associated with reduced DC cardioversion burden. Patients with levo-transposition of the great arteries may experience lower ATP efficacy than patients with other CHDs; however, a larger patient population is required to better determine subgroup efficacy. These results support the safety and efficacy of ATP in the CHD population.

PMID: 29246827 [PubMed - indexed for MEDLINE]

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Cardiac implantable electronic devices in adults with tetralogy of Fallot.

Heart. 2018 Oct 30;:

Authors: Egbe AC, Miranda WR, Madhavan M, Ammash NM, Missula VR, Al-Otaibi M, Fatola A, Kothapalli S, Connolly HM

BACKGROUND: Tachyarrhythmias and bradyarrhythmias affect 20%-50% of adult patients with tetralogy of Fallot (TOF) and some of these patients will require cardiac implantable electronic devices (CIED) such as pacemaker and/or internal cardioverter defibrillator.
METHODS: The Mayo Adult Congenital Heart Disease database was queried for patients with repaired TOF and history of CIED implantation, 1990-2017. The study objectives were: (1) determine the occurrence of device-related complications defined as lead failure, lead recall, device infection and lead thrombus; and (2) determine the occurrence and risk factors for defibrillator shock.
RESULTS: There were 99 patients (age 46±14 years and 66 (66%) men) with CIED, and the CIEDs were 41 (41%) pacemakers and 73 (73%) defibrillators. Indication for defibrillator implantation was for primary prevention in 28 (38%) and secondary prevention in 45 (62%). Device-related complications occurred in 20 (20%) patients (lead failure 17, lead recall 4, device infection 12 and thrombus 3). Twenty-five per cent of all device infections occurred within 30 days from the time of device generator change. Annualised rates of appropriate and inappropriate shocks were 5.7% and 6.2%, respectively. The use of class III antiarrhythmic drug was protective against defibrillator shock.
CONCLUSION: The current study provides useful outcome data to aid patient counselling and clinical decision-making. Further studies are required to explore ways to decrease the risk of postprocedural infection after device generator change, and to determine which patients will benefit from empirical antiarrhythmic therapy as a strategy to decrease incidence of inappropriate defibrillator shock.

PMID: 30377259 [PubMed - as supplied by publisher]