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Temporary balloon occlusion of atrial septal defects in suspected or documented left ventricular diastolic dysfunction: Hemodynamic and clinical findings.

Catheter Cardiovasc Interv. 2019 Feb 12;:

Authors: Miranda WR, Hagler DJ, Reeder GS, Warnes CA, Connolly HM, Egbe AC, Taggart NW

Abstract
OBJECTIVE: To review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction.
BACKGROUND: ASD closure in patients with LV diastolic dysfunction may precipitate LV failure. Temporary ASD occlusion has been used in this scenario but data are limited.
METHODS: Retrospective review of 27 patients age ≥ 50 years undergoing temporary ASD balloon occlusion between 2000 and 2018 for suspected LV diastolic dysfunction or elevated LV end-diastolic pressure (LVEDP).
RESULTS: Median age was 72 years (IQR 66.7; 75.2). Atrial fibrillation was seen in 58% of patients, hypertension in 58%, and coronary artery disease in 26%; 52% were females. Median ASD size was 13 mm (10; 18) and Qp/Qs 1.8 (1.6; 2.2). Median LVEDP was 14 mmHg (12; 22); pulmonary artery wedge pressure (PAWP) 12 mmHg (9; 16.5) and left atrial pressure (LAP) 13.5 mmHg (8; 16.3). After a median of 5 min (3; 10) of balloon occlusion, patients with baseline LVEDP ≥15 mmHg had more significant increases in LVEDP (9 [6; 12] vs. 2 mmHg [0.5; 5]; p = 0.03) and LAP/PAWP (10.5 [8.3; 16.3] vs. 1.5 mmHg [-1.5; 3]; p = 0.0003) than those with baseline LVEDP <15 mmHg. None of those with a baseline LVEDP <15 mmHg had a LAP/PAWP >15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg.
CONCLUSION: LVEDP might be used to predict LAP post-ASD closure. Comorbidities typically associated with LV diastolic dysfunction are common in these patients and should be considered in their management.

PMID: 30747481 [PubMed - as supplied by publisher]

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Tell me once, tell me soon: parents' preferences for clinical genetics services for congenital heart disease.

Genet Med. 2018 11;20(11):1387-1395

Authors: Kasparian NA, De Abreu Lourenco R, Winlaw DS, Sholler GF, Viney R, Kirk EPE

Abstract
PURPOSE: As the molecular basis of congenital heart disease (CHD) comes into sharper focus, cardiac genetics services are likely to play an increasingly important role. This study aimed to identify parents' preferences for, and willingness to participate in, clinical genetics services for CHD.
METHODS: A discrete choice experiment was developed to assess parents' preferences for pediatric cardiogenetics services based on four attributes: appointment format, health professionals involved, waiting time, and information format. Data were analyzed using a mixed logit model.
RESULTS: One hundred parents with a living child diagnosed with CHD requiring surgical intervention between 2000 and 2009 completed the discrete choice experiment. Parents expressed a clear preference for cardiac genetics services featuring (i) a single appointment, (ii) the presence of a clinical geneticist and a genetic counselor, (iii) both verbal (oral) and Web-based information about CHD and genetics, and (iv) availability of an appointment within 2 weeks. If offered such conditions, 93% of respondents indicated that they would attend. The choice of service was most strongly influenced by the presence of both a clinical geneticist and a genetic counselor.
CONCLUSION: Parents of children with CHD favor a single, timely genetics appointment with both a geneticist and a genetic counselor present. If appointments offered match these preferences, uptake is likely to be high.

PMID: 29493584 [PubMed - indexed for MEDLINE]

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Not So Incidental: Pheochromocytoma in an Adult with Unrepaired Cyanotic Heart Disease.

Eur J Case Rep Intern Med. 2018;5(10):000959

Authors: Adunuri N, Mrkobrada M

Abstract
Pheochromocytoma, a rare neuroendocrine tumour, is often encountered in the general internal medicine clinic as an adrenal incidentaloma. Even rarer is its presence in adult cyanotic heart disease, although there are a few documented reports of this association in various paediatric populations, with chronic hypoxia being the likely driving force. Here we present the case of a 38-year-old adult with unrepaired complex cyanotic congenital heart disease with biochemically proven pheochromocytoma presenting as an adrenal incidentaloma to a general internal medicine clinic.
LEARNING POINTS: There is an association between pheochromocytoma and congenital cyanotic heart disease.It is important to maintain clinical vigilance for laboratory testing for pheochromocytoma in patients with cyanotic heart disease who are seen at general internal medicine clinics.

PMID: 30755984 [PubMed]

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Cardiac Arrhythmias in Adults with Congenital Heart Disease.

Card Electrophysiol Clin. 2017 06;9(2):xv-xvi

Authors: Balaji S, Mandapati R, Shivkumar K

PMID: 28457248 [PubMed - indexed for MEDLINE]

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Pregnancy, Marfan syndrome, and type-B aortic dissection.

BJOG. 2018 03;125(4):494

Authors: Johnson MR, Roos Hesselink JW

PMID: 28600893 [PubMed - indexed for MEDLINE]

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Heart beat but not respiration is the main driving force of the systemic venous return in the Fontan circulation.

Sci Rep. 2019 Feb 14;9(1):2034

Authors: Gabbert DD, Hart C, Jerosch-Herold M, Wegner P, Ravesh MS, Voges I, Kristo I, Bulushi AAL, Scheewe J, Kheradvar A, Kramer HH, Rickers C

Abstract
The Fontan procedure provides relief from cyanosis in patients with univentricular hearts. A major clinical unmet need is to understand whether the venous flow patterns of the Fontan circulation lead to the development of congestive hepatopathy and other life-threatening complications. Currently, there is no consensus on whether heart beat or respiration is the main driving force of venous return and which one affects the periodic flow changes for the most (i. e., pulsatility). The present study, for the first time, quantified respiratory and cardiac components of the venous flow in the inferior vena cava (IVC) of 14 Fontan patients and 11 normal controls using a novel approach ("physio-matrix"). We found that in contrast to the normal controls, respiration in Fontan patients had a significant effect on venous flow pulsatility, and the ratio of respiration-dependent to the cardiac-dependent pulsatility was positively associated with the retrograde flow. Nevertheless, the main driving force of net IVC flow was the heart beat and not respiration. The separate analysis of the effects of respiration and heart beat provides new insights into the abnormal venous return patterns that may be responsible for adverse effects on liver and bowel of the patients with Fontan circulation.

PMID: 30765829 [PubMed - in process]

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Pulmonary arterial hypertension in adults with systemic right ventricles referred for cardiac transplantation.

Clin Transplant. 2019 Feb 15;:e13496

Authors: Kim YY, Awh K, Acker M, Atluri P, Bermudez C, Crespo M, Diamond JM, Drajpuch D, Forde-Mclean R, Fuller S, Goldberg L, Mazurek J, Mascio C, Menachem JN, Rame E, Ruckdeschel E, Tobin L, Wald J

Abstract
BACKGROUND: Systemic right ventricular [RV] failure may progress necessitating referral for orthotropic heart transplantation [OHT]. Pulmonary hypertension [PH] frequently coexists in adult congenital heart disease and can complicate the assessment for OHT.
METHODS: Single-center case series of six patients [median age 34.9 years (IQR, 31.9-42.4)] with systemic RV physiology with PH referred for OHT evaluation from 2008-2017.
RESULTS: One-third (n=6) of 18 patients with systemic RV physiology referred for OHT evaluation had pulmonary arterial hypertension [PAH] defined as mean pulmonary artery pressure [mPAP] > 25 mmHg and pulmonary vascular resistance [PVR] > 3 Wood Units. Two of the six patients were considered OHT-ineligible due to PH and comorbidities. Of the remaining four, two had pre-capillary PH and underwent heart-lung transplant [HLTx]. The other two demonstrated reversibility of PVR with vasodilator testing and underwent OHT alone, one of whom died post-transplant from PH crisis.
CONCLUSIONS: PAH is common in systemic RV patients referred for OHT. Systemic RV dysfunction places these patients at risk for post-capillary PH but pre-capillary PH can exist. Despite management with selective pulmonary vasodilators and afterload reduction, criteria for listing patients for HLTx versus OHT are not known and need further elucidation. This article is protected by copyright. All rights reserved.

PMID: 30770573 [PubMed - as supplied by publisher]

Trends in surgical and catheter interventions for isolated congenital shunt lesions in the UK and Ireland.

Heart. 2019 Feb 16;:

Authors: Farooqi M, Stickley J, Dhillon R, Barron DJ, Stumper O, Jones TJ, Clift PF, Brawn WJ, Drury NE

Abstract
OBJECTIVE: To evaluate time trends in the use of catheter and surgical procedures, and associated survival in isolated congenital shunt lesions.
METHODS: Nationwide, retrospective observational study of the UK National Congenital Heart Disease Audit database from 2000 to 2016. Patients undergoing surgical or catheter procedures for atrial septal defect (including sinus venosus defect), patent foramen ovale, ventricular septal defect and patent arterial duct were included. Temporal changes in the frequency of procedures, and survival at 30 days and 1 year were determined.
RESULTS: 40 911 procedures were performed, 16 604 surgical operations and 24 307 catheter-based interventions. Transcatheter procedures increased over time, overtaking surgical repair in 2003-2004, while the number of operations remained stable. Trends in interventions differed according to defect type and patient age. Catheter closure of atrial septal defects is now more common in children and adults, although surgical interventions have also increased. Patent foramen ovale closure in adults peaked in 2009-2010 before falling significantly since. Surgery remains the mainstay for ventricular septal defect in infants and children. Duct ligation is most common in neonates and infants, while transcatheter intervention is predominant in older children. Excluding duct ligation, survival following surgery was 99.4% and ≈98.7%, and following catheter interventions was 99.7% and ≈99.2%, at 30 days and 1 year, respectively.
CONCLUSIONS: Trends in catheter and surgical techniques for isolated congenital shunt lesions plot the evolution of the specialty over the last 16 years, reflecting changes in clinical guidelines, technology, expertise and reimbursement, with distinct patterns according to lesion and patient age.

PMID: 30772822 [PubMed - as supplied by publisher]

Characterization of the Mechanism and Substrate of Atrial Tachycardia Using Ultra-High-Density Mapping in Adults With Congenital Heart Disease: Impact on Clinical Outcomes.

J Am Heart Assoc. 2019 Feb 19;8(4):e010535

Authors: Mantziari L, Butcher C, Shi R, Kontogeorgis A, Opel A, Chen Z, Haldar S, Panikker S, Hussain W, Jones DG, Gatzoulis MA, Markides V, Ernst S, Wong T

Abstract
Background Atrial tachycardia ( AT ) is common in patients with adult congenital heart disease and is challenging to map and ablate. We used ultra-high-density mapping to characterize the AT mechanism and investigate whether substrate characteristics are related to ablation outcomes. Methods and Results A total of 50 AT s were mapped with ultra-high-density mapping in 23 procedures. Patients were followed up for up to 12 months. Procedures were classified to group A if there was 1 single AT induced (n=12) and group B if there were ≥2 AT s induced (n=11 procedures). AT mechanism per procedure was macro re-entry (n=10) and localized re-entry (n=2) in group A and multiple focal (n=6) or multiple macro re-entry (n=5) in group B. Procedure duration, low voltage area (0.05-0.5 mV), and low voltage area indexed for volume were higher in group B (159 [147-180] versus 412 [352-420] minutes, P<0.001, 22.6 [12.2-29.8] versus 54.2 [51.1-61.6] cm2, P=0.014 and 0.17 [0.12-0.21] versus 0.26 [0.23-0.27] cm2/mL, P=0.024 accordingly). Dense scar (<0.05 mV) and atrial volume were similar between groups. Acute success and freedom from arrhythmia recurrence were worse in group B (100% versus 77% P=0.009 and 11.3, CI 9.8-12.7 versus 4.9, CI 2.2-7.6 months, log rank P=0.004). Indexed low voltage area ≥0.24 cm2/mL could predict recurrence with 100% sensitivity and 77% specificity (area under the curve 0.923, P=0.007). Conclusions Larger low voltage area but not dense scar is associated with the induction of multiple focal or re-entry AT s, which are subsequently associated with longer procedure duration and worse acute and midterm clinical outcomes.

PMID: 30773973 [PubMed - in process]

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The Real-World Utility of the LINQ Implantable Loop Recorder in Pediatric and Adult Congenital Heart Patients.

JACC Clin Electrophysiol. 2019 Feb;5(2):245-251

Authors: Bezzerides VJ, Walsh A, Martuscello M, Escudero CA, Gauvreau K, Lam G, Abrams DJ, Triedman JK, Alexander ME, Bevilacqua L, Mah DY

Abstract
OBJECTIVES: This study sought to determine the practical use of the recently introduced LINQ implantable loop recorder (LINQ-ILR) in a cohort of pediatric and adult congenital arrhythmia patients.
BACKGROUND: Correlating symptoms to a causative arrhythmia is a key aspect of diagnosis and management in clinical electrophysiology.
METHODS: Retrospective review of clinical data, implantation indications, findings, and therapeutic decisions in patients who underwent LINQ-ILR implantation from April 1st, 2014 to January 30th, 2017 at Boston Children's Hospital.
RESULTS: A total of 133 patients were included, of which 76 (57%) were male. The mean age at implantation was 15.7 ± 9.1 years with a duration of follow-up of 11.8 months. Congenital heart disease was present in 34 patients (26%), a confirmed genetic diagnosis in 50 (38%), and cardiomyopathy in 22 (26%), and the remainder were without a previous diagnosis. Syncope was the most common indication for LINQ-ILR implantation, occurring in 59 patients (44%). The median time to diagnosis was 4.5 months, occurring in 78 patients (59%). Cardiac device placement occurred in 17 patients (22%), a medication change in 9 (12%), electrophysiology study/ablation in 5 (6%), or LINQ-ILR explantation in 42 (54%). Infection or erosion occurred in 5 patients. Syncope was correlated with a diagnostic transmission (54% vs. 31%, p = 0.01).
CONCLUSIONS: The LINQ-ILR is an important diagnostic tool, providing useful data in more than one-half of patients in <6 months. Adverse events are low. Patient selection is critical and undiagnosed syncope represents an important presenting indication for which a LINQ-ILR implant should be considered.

PMID: 30784698 [PubMed - in process]

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