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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

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

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

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

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

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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Surveillance Testing and Preventive Care After Fontan Operation: A Multi-Institutional Survey.

Pediatr Cardiol. 2019 Jan;40(1):110-115

Authors: Di Maria MV, Brown DW, Cetta F, Ginde S, Goldberg D, Menon SC, Phelps HM, Rychik J, Schumacher KR, Thrush P, Veldtman G, Wright G, Younoszai AK

More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500-2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3-4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.

PMID: 30159585 [PubMed - indexed for MEDLINE]

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VGLL4 plays a critical role in heart valve development and homeostasis.

PLoS Genet. 2019 Feb 21;15(2):e1007977

Authors: Yu W, Ma X, Xu J, Heumüller AW, Fei Z, Feng X, Wang X, Liu K, Li J, Cui G, Peng G, Ji H, Li J, Jing N, Song H, Lin Z, Zhao Y, Wang Z, Zhou B, Zhang L

Heart valve disease is a major clinical problem worldwide. Cardiac valve development and homeostasis need to be precisely controlled. Hippo signaling is essential for organ development and tissue homeostasis, while its role in valve formation and morphology maintenance remains unknown. VGLL4 is a transcription cofactor in vertebrates and we found it was mainly expressed in valve interstitial cells at the post-EMT stage and was maintained till the adult stage. Tissue specific knockout of VGLL4 in different cell lineages revealed that only loss of VGLL4 in endothelial cell lineage led to valve malformation with expanded expression of YAP targets. We further semi-knockout YAP in VGLL4 ablated hearts, and found hyper proliferation of arterial valve interstitial cells was significantly constrained. These findings suggest that VGLL4 is important for valve development and manipulation of Hippo components would be a potential therapy for preventing the progression of congenital valve disease.

PMID: 30789911 [PubMed - as supplied by publisher]

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Trends in Utilization, Mortality, Major Complications and Cost after Total Artificial Heart Implantation in the United States (2009-2015).

Hellenic J Cardiol. 2019 Feb 18;:

Authors: Briasoulis A, Akintoye E, Mohsen A, Inampudi C, Briasouli A, Asleh R, Alvarez P

BACKGROUND: Total Artificial Heart (TAH) is a viable bridge to transplant (BTT) strategy for patients with severe biventricular failure, or complex congenital heart disease. These patients have higher mortality and morbidity compared with patients undergoing left ventricular assist device (LVAD) implantation.
OBJECTIVE: To assess national trends in in-hospital mortality, major complications, cost, length of stay and disposition of patients undergoing TAH implantation.
METHODS: Data from the National Inpatient Sample, the largest all-payer inpatient data set in the United States, and the US Census Bureau, for the years 2009 to 2015 were analyzed. Participants included all adult patients who received TAH from 2009 to 2015. End points included in-hospital mortality, in-hospital complications, heart transplantation (HT) in the same admission, length of stay, cost, and disposition at the time of discharge.
RESULTS: We identified a total of 143 (weighted = 703) TAH implantations. The number of TAH implants increased during the study period (average annual change +5.8%, p=0.03). Rates of in-hospital mortality and major complications including ischemic stroke, major bleeding, post-operative infections, acute kidney injury requiring dialysis and HT did not change significantly over the study period. Although the length of stay and disposition patterns did not change over time, we found a significant increase in cost of hospitalization (average annual change +44,362, p = 0.01). The number of HT during the same hospital stay decreased significantly (average annual change -8.1%, p=0.02).
CONCLUSION: In-hospital mortality and complication rates associated with TAH implantation remain increased and did not change in the era of continuous flow LVADs.

PMID: 30790715 [PubMed - as supplied by publisher]

Related Articles

Prosthetic pulmonary valve and conduit endocarditis in congenital heart disease.

Asian Cardiovasc Thorac Ann. 2019 Feb 21;:218492319832769

Authors: Rebollal-Leal F, Felipe-Abella R, Gutierrez-García F, A Mestres C, Bautista-Hernandez V

BACKGROUND: Pulmonary valve replacement is one of the most common procedures in patients with congenital heart disease. Little is known about prosthetic valve endocarditis in this population.
OBJECTIVES: To review management and outcomes of pediatric and adult patients with a prosthetic pulmonary valve or right ventricle-to-pulmonary artery conduit infective endocarditis.
METHODS AND RESULTS: A multiinstitutional cohort of 10 patients is reported. Median age at endocarditis was 24 years (range 8-41 years). The most common causative organisms were Staphylococcus ( n = 4) and Streptococcus species ( n = 2). In addition to medical treatment, 5 patients required surgery. Majors complications such as renal damage, life-threatening hemoptysis, and septic shock were observed. No patient died in hospital or during follow-up.
CONCLUSIONS: Pulmonary prosthetic valve endocarditis is a rare condition associated with significant morbidity and a high risk of requiring operative intervention. Larger studies are required to optimize the management.

PMID: 30791694 [PubMed - as supplied by publisher]

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Robotic Repair of a Congenital Isolated Cleft of Anterior Tricuspid Valve Leaflet.

J Investig Med High Impact Case Rep. 2019 Jan-Dec;7:2324709618823809

Authors: Hamandi M, Al-Azizi K, Crawford A, Fan J, DiMaio JM, Smith RL

Congenital isolated tricuspid valve (TV) cleft in the anterior leaflet is a rare occurrence, while clefts of the mitral valve leaflets are more common and are usually associated with other congenital heart diseases. In this article, we report a case of TV regurgitation in a young adult female due to an isolated congenital cleft in the anterior TV leaflet, which was surgically repaired using a minimally invasive robotic approach.

PMID: 30791722 [PubMed - in process]