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Temporal relationship between instantaneous pressure gradients and peak-to-peak systolic ejection gradient in congenital aortic stenosis.

Congenit Heart Dis. 2017 Dec;12(6):733-739

Authors: Boe BA, Norris MD, Zampi JD, Rocchini AP, Ensing GJ

Abstract
OBJECTIVE: We sought to identify a time during cardiac ejection when the instantaneous pressure gradient (IPG) correlated best, and near unity, with peak-to-peak systolic ejection gradient (PPSG) in patients with congenital aortic stenosis. Noninvasive echocardiographic measurement of IPG has limited correlation with cardiac catheterization measured PPSG across the spectrum of disease severity of congenital aortic stenosis. A major contributor is the observation that these measures are inherently different with a variable relationship dependent on the degree of stenosis.
DESIGN: Hemodynamic data from cardiac catheterizations utilizing simultaneous pressure measurements from the left ventricle (LV) and ascending aorta (AAo) in patients with congenital valvar aortic stenosis was retrospectively reviewed over the past 5 years. The cardiac cycle was standardized for all patients using the percentage of total LV ejection time (ET). Instantaneous gradient at 5% intervals of ET were compared to PPSG using linear regression and Bland-Altman analysis.
RESULTS: A total of 22 patients underwent catheterization at a median age of 13.7 years (interquartile range [IQR] 10.3-18.0) and median weight of 51.1 kg (IQR 34.2-71.6). The PPSG was 46.5 ± 12.6 mm Hg (mean ± SD) and correlated suboptimally with the maximum and mean IPG. The midsystolic IPG (occurring at 50% of ET) had the strongest correlation with the PPSG ( PPSG = 0.97(IPG50%)-1.12, R2  = 0.88), while the IPG at 55% of ET was closest to unity ( PPSG = 0.997(IPG55%)-1.17, R2  = 0.87).
CONCLUSIONS: The commonly measured maximum and mean IPG are suboptimal estimates of the PPSG in congenital aortic stenosis. Using catheter-based data, IPG at 50%-55% of ejection correlates well with PPSG. This may allow for a more accurate estimation of PPSG via noninvasive assessment of IPG.

PMID: 28703367 [PubMed - indexed for MEDLINE]

Related Articles

Changing prevalence of severe congenital heart disease: Results from the National Register for Congenital Heart Defects in Germany.

Congenit Heart Dis. 2017 Dec;12(6):787-793

Authors: Pfitzer C, Helm PC, Ferentzi H, Rosenthal LM, Bauer UMM, Berger F, Schmitt KRL

Abstract
OBJECTIVE: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender.
DESIGN: Cross-sectional registry study.
SETTING: We analyzed data from patients with CHD born between 1996 and 2015.
PATIENTS: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability for earlier identifying minor lesions). At least 15 703 patients with demographic data and detailed medical information were included in the current study.
INTERVENTIONS: None.
OUTCOME MEASURES: Prevalence of CHD in Germany differentiated into gender, severity, and phenotype.
RESULTS: In total, 15 703 patients with CHD (47.1% female) were included in this study. The five most common phenotypes were found to be ventricular septal defect (19.2%), atrial septal defect (13.0%), Tetralogy of Fallot (9.3%), univentricular heart (9.4%), and coractation of the aortae (7.0%). The prevalence of CHD in regard to severity changed over the duration of the observation period. From 1996 to 2007, the number of simple CHD rose steadily (P < .001), whereas the number of severe CHD has grown significantly since 2008/2009 (P < .001). In regard to gender, the prevalence of simple CHD was higher in females, whereas complex lesions were more common in males (P < .001).
CONCLUSIONS: Our study shows a growing number of registered severe CHD in the recent decade in Germany. This development is noteworthy as it implicates a growing demand for first intensive hospital care, expert pediatric cardiologic aftercare, and consequently higher economic impact for this patient population.

PMID: 28719142 [PubMed - indexed for MEDLINE]

Related Articles

Development of quality metrics for ambulatory pediatric cardiology: Infection prevention.

Congenit Heart Dis. 2017 Dec;12(6):756-761

Authors: Johnson JN, Barrett CS, Franklin WH, Graham EM, Halnon NJ, Hattendorf BA, Krawczeski CD, McGovern JJ, O'Connor MJ, Schultz AH, Vinocur JM, Chowdhury D, Anderson JB

Abstract
INTRODUCTION: In 2012, the American College of Cardiology's (ACC) Adult Congenital and Pediatric Cardiology Council established a program to develop quality metrics to guide ambulatory practices for pediatric cardiology. The council chose five areas on which to focus their efforts; chest pain, Kawasaki Disease, tetralogy of Fallot, transposition of the great arteries after arterial switch, and infection prevention. Here, we sought to describe the process, evaluation, and results of the Infection Prevention Committee's metric design process.
METHODS: The infection prevention metrics team consisted of 12 members from 11 institutions in North America. The group agreed to work on specific infection prevention topics including antibiotic prophylaxis for endocarditis, rheumatic fever, and asplenia/hyposplenism; influenza vaccination and respiratory syncytial virus prophylaxis (palivizumab); preoperative methods to reduce intraoperative infections; vaccinations after cardiopulmonary bypass; hand hygiene; and testing to identify splenic function in patients with heterotaxy. An extensive literature review was performed. When available, previously published guidelines were used fully in determining metrics.
RESULTS: The committee chose eight metrics to submit to the ACC Quality Metric Expert Panel for review. Ultimately, metrics regarding hand hygiene and influenza vaccination recommendation for patients did not pass the RAND analysis. Both endocarditis prophylaxis metrics and the RSV/palivizumab metric passed the RAND analysis but fell out during the open comment period. Three metrics passed all analyses, including those for antibiotic prophylaxis in patients with heterotaxy/asplenia, for influenza vaccination compliance in healthcare personnel, and for adherence to recommended regimens of secondary prevention of rheumatic fever.
CONCLUSIONS: The lack of convincing data to guide quality improvement initiatives in pediatric cardiology is widespread, particularly in infection prevention. Despite this, three metrics were able to be developed for use in the ACC's quality efforts for ambulatory practice.

PMID: 28741863 [PubMed - indexed for MEDLINE]

Related Articles

Cardiovascular adaptation to the Fontan circulation.

Congenit Heart Dis. 2017 Dec;12(6):699-710

Authors: Veldtman GR, Opotowsky AR, Wittekind SG, Rychik J, Penny DJ, Fogel M, Marino BS, Gewillig M

Abstract
Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations. The overarching aim is to provide insight into the integrative pathophysiology that governs the Fontan circulation and stimulate thoughtful approaches to advance research.

PMID: 28795517 [PubMed - indexed for MEDLINE]

Related Articles

Bicuspid and unicuspid aortic valves: Different phenotypes of the same disease? Insight from the GenTAC Registry.

Congenit Heart Dis. 2017 Dec;12(6):740-745

Authors: Krepp JM, Roman MJ, Devereux RB, Bruce A, Prakash SK, Morris SA, Milewicz DM, Holmes KW, Ravekes W, Shohet RV, Pyeritz RE, Maslen CL, Kroner BL, Eagle KA, Preiss L, GenTAC Investigators, Asch FM

Abstract
BACKGROUND: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed.
METHODS: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions. Clinical and echocardiographic data from the GenTAC Registry were compared between 17 patients with UAV and 17 matched-controls with BAV.
RESULTS: Baseline characteristics including demographics, clinical findings including family history of BAV and aortic aneurysm/coarctation, and echocardiographic variables were similar between BAV and UAV patients; aortic stenosis was more common and more severe in patients with UAV. This was evidenced by higher mean and peak gradient, smaller aortic valve area, and more advanced valvular degeneration (all P < .05). There were no significant differences in aortic dimensions, with a similar pattern of enlargement of the ascending aorta.
CONCLUSIONS: The similar baseline characteristics with more accelerated aortic valve degeneration and stenosis suggest that UAV represents an extreme in the spectrum of BAV syndromes. Therefore, it is reasonable to consider application of recommendations for the management of patients with BAV to those with the rarer UAV.

PMID: 28805011 [PubMed - indexed for MEDLINE]

Contemporary management and outcomes in congenitally corrected transposition of the great arteries.

Heart. 2018 Jan 11;:

Authors: Kutty S, Danford DA, Diller GP, Tutarel O

Abstract
Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. Some management strategies leave the right ventricle as the systemic arterial pump, but carry the risk of worsening heart failure. More complex 'double switch' repairs establish the left ventricle as the systemic pump, and include an atrial baffle to redirect venous return in combination with either arterial switch or Rastelli operation (if a suitable ventricular septal defect permits). Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and Fontan palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance should be established in an age-appropriate facility with expertise in congenital heart disease care.

PMID: 29326110 [PubMed - as supplied by publisher]

Heart failure in grown-up congenital heart disease.

Minerva Cardioangiol. 2018 Jan 10;:

Authors: Faccini A, Micheletti A, Negura DG, Giugno L, Butera G, Carminati M, Giamberti A, Chessa M

Abstract
The increasing survival in the adulthood of patients with congenital heart disease (CHD) has changed the epidemiology of adult CHD (ACHD) patients and has led to an increment in hospitalization rates due to heart failure (HF). ACHD patients hospitalized for HF have a five-fold higher risk of death HF than those compensated. HF occurs predominantly in patients with tetralogy of Fallot, single ventricles, and after the Mustard operation for transposition of the great arteries. Diagnostic strategies applied in acquired HF patients are usually used to evaluate ACHD patients, but sometimes this can postpone the identification of HF that can become manifest with unusual and peculiar signs or symptoms. In the same way, therapeutic management resembles the acquired HF one, even if no large randomized clinical trials have been conducted in ACHD patients. Therefore, a close monitoring in dedicated units in mandatory in order to identify in time HF manifestations and manage them adequately.

PMID: 29327893 [PubMed - as supplied by publisher]

Executive Function and Internalizing Symptoms in Adolescents and Young Adults With Congenital Heart Disease: The Role of Coping.

J Pediatr Psychol. 2018 Jan 10;:

Authors: Jackson JL, Gerardo GM, Monti JD, Schofield KA, Vannatta K

Abstract
Objective : Executive functioning deficits have been documented among congenital heart disease (CHD) survivors and may contribute to emotional distress. Little research has investigated the role of coping in this association. This study examined the role of coping in accounting for the association between self-reported executive function problems and internalizing symptoms among adolescents and emerging adults (AEAs), as well as young adults (YAs) with CHD. Participants included 74 AEA ( M age  = 19.32  ±  3.47 years, range 15-25 years) and 98 YA CHD survivors ( M age  = 32.00  ±  3.69 years, range 26-39 years), recruited from pediatric and adult outpatient cardiology clinics. Participants completed self-report measures of executive function problems, coping (primary control, secondary control, and disengagement coping), and internalizing symptoms. Lesion severity classification and functional impairment due to symptoms of heart failure were determined from medical chart review. Significant problems in executive function were reported by 5% of AEA and 13% of YA. Coping was not associated with executive function problems or internalizing symptoms for AEA. However, among YA, less use of adaptive coping strategies and more maladaptive coping responses was associated with both more executive function problems and internalizing symptoms. An indirect effect of executive function problems on internalizing symptoms via secondary control coping emerged for YA. Executive function problems may disrupt the ability to use important adaptive coping skills, such as cognitive reappraisal, positive thinking, and acceptance, thereby resulting in greater emotional distress among YA CHD survivors.

PMID: 29329440 [PubMed - as supplied by publisher]

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[Analysis of mortality and hospital stay in cardiac surgery in Mexico 2015: Data from the National Cardiology Institute].

Arch Cardiol Mex. 2018 Jan 09;:

Authors: Rodríguez-Hernández A, García-Torres M, Bucio Reta E, Baranda-Tovar FM

Abstract
OBJECTIVE: To analyse hospital mortality in patients subjected to cardiac surgery in Mexico during the year 2015, and identify the mortality risks factors, and its correlation with days of hospital stay in the cardiovascular intensive care unit.
METHOD: The database of Cardiovascular Intensive Care of the National Institute of Cardiology was examined for this cases and controls study that included only adult patients subjected to cardiac surgery during the year 2015.
RESULTS: A total of 571 patients were subjected to a surgical procedure. The predominant indication was single or multiple valve replacement surgery, followed by coronary revascularisation surgery, and correction of adult congenital heart disease. Overall mortality was 9.2, and 8% died in intensive care. The main risk factors for death were preoperative organ failure or pulmonary hypertension, and prolonged time with extracorporeal circulation. The primary cause of death was secondary to cardiogenic shock. The hospital mortality observed in this population was higher for patients undergoing pulmonary thromboendarterectomy, complex aortic disease surgery, and valvular surgery.
CONCLUSIONS: The mortality of patients undergoing cardiac surgery in Mexico differs slightly from that reported in the world literature, primarily because there were more multivalvular surgeries and mixed complex procedures performed.

PMID: 29329764 [PubMed - as supplied by publisher]

Related Articles

Yield of family screening in patients with isolated bicuspid aortic valve in a general hospital.

Int J Cardiol. 2017 Dec 22;:

Authors: Cozijnsen L, van der Zaag-Loonen HJ, Braam RL, Boo MB, Post JG, Bouma BJ, Mulder BJM

Abstract
AIM: To determine the prevalence of unidentified bicuspid aortic valve (BAV) or aortic dilatation (>40mm) in first degree relatives (FDR) of patients with isolated BAV in a general hospital.
METHODS AND RESULTS: Patients with isolated BAV received information advising cardiac screening of their FDR. Referred and screened were 134 FDR of 54 adult index patients with isolated BAV (median 2 per index patient). FDR's mean age was 49years (range 16-83years) and 41% were male. They comprised 5 parents (3.7%), 52 siblings (39%) and 77 offspring (57%). Among these FDR, the prevalence of BAV was 6.0% (8 patients). In FDR without BAV, 10 (7.5%) had aortic dilatation. 'Familial BAV' was present in 9/54 families (17%).
CONCLUSION: In a general hospital, screening of FDR of patients with isolated BAV resulted in a substantial yield of 13% new cases with BAV or aortic dilatation without BAV.

PMID: 29329770 [PubMed - as supplied by publisher]

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