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Etiology and Morphogenesis of Congenital Heart Disease: From Gene Function and Cellular Interaction to Morphology

Book. 2016

Authors: Nakanishi T, Markwald RR, Baldwin HS, Keller BB, Srivastava D, Yamagishi H

Abstract
A c-Kit (CD117) is a well-known cell surface marker for adult somatic stem cells. We harvested c-Kit-positive cardiac stem cells (CSCs) from adult rat hearts by performing magnetic-activated cell sorting (MACS) and subjected them to long-term bulk culture more than 40 times. We made 11 attempts to obtain c-Kit-positive cells from adult (6–8-month-old) rats. Our initial expectation was of obtaining cells with homogenous cardiac phenotypes. However, each CSC bulk culture expressed varying degrees of the genes and cell surface markers belonging to cardiac and other mesenchymal lineages. The results suggested that these CSCs retained multiple developmental potential to some extent. Consequently, we investigated these CSCs in detail, hoping to establish the regeneration method by using c-Kit-positive cardiac cells [1–12].


PMID: 29787151

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Percutaneous closure of a giant coronary artery fistula after surgical pericardiectomy. Review of the literature.

Cardiovasc Revasc Med. 2017 Jul - Aug;18(5):384-389

Authors: Unzué L, García E, Díaz-Antón B, Fernández-Portales J, Teijeiro R, Rodríguez-Del-Río M

Abstract
Coronary artery fistulae (CAF) are uncommon heart defects defined as a communication between a coronary artery and a cardiac chamber or vascular structure. They are frequently asymptomatic; nevertheless, they can produce angina, dyspnea or cardiac failure. CAF are believed to be congenital; however, isolated cases of CAF have been described as rare complications of cardiac surgery. We report the percutaneous closure of a giant CAF in an adult patient with angina and previous pericardiectomy.

PMID: 28320605 [PubMed - indexed for MEDLINE]

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Number of thoracotomies predicts impairment in lung function and exercise capacity in patients with congenital heart disease.

J Cardiol. 2018 Jan;71(1):88-92

Authors: Müller J, Ewert P, Hager A

Abstract
OBJECTIVE: Many patients with congenital heart disease (CHD) require surgery to ensure survival into adulthood. But history of previous thoracotomies is associated with respiratory muscle weakness, impairments in chest wall compliance, and moderately to severely impaired lung function. This study evaluated the impact of thoracotomies on functional outcome in patients with CHD.
PATIENTS AND METHODS: In total 1372 adolescents and adults with CHD (32.4±11.5 years, 624 female), who underwent spirometry and cardiopulmonary exercise testing in our institution from January 2010 to August 2015, were analyzed.
RESULTS: After adjusting for confounding variables, with every thoracotomy the prevalence for a restrictive ventilatory pattern increased by 1.8-fold (CI: 1.606-2.050; p<0.001). The number of thoracotomies had no direct influence on an impaired exercise capacity in a multivariate model, but with every percentage point increase in forced vital capacity probability of impaired exercise capacity diminished (OR: 0.944, CI: 0.933-0.955, p<0.001). There was a moderate correlation of forced vital capacity and peak oxygen uptake (r=0.464, p<0.001). After a follow-up of 2.1±1.6 years 21 patients had died. Survival was only related to age (p<0.001) and peak oxygen uptake (p<0.001) after considering together with thoracotomies, oxygen saturation at rest and forced vital capacity in a multivariate model.
CONCLUSIONS: Independent of CHD complexity and other risk factors, multiple thoracotomies lead to restrictive lung pattern. It could be suggested that those limitations in forced vital capacity contribute to impairments in exercise capacity, which turned out to be the strongest predictor for survival.

PMID: 28687271 [PubMed - indexed for MEDLINE]

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Relationships Among Conduit Type, Pre-Stenting, and Outcomes in Patients Undergoing Transcatheter Pulmonary Valve Replacement in the Prospective North American and European Melody Valve Trials.

JACC Cardiovasc Interv. 2017 Sep 11;10(17):1746-1759

Authors: Cabalka AK, Hellenbrand WE, Eicken A, Kreutzer J, Gray RG, Bergersen L, Berger F, Armstrong AK, Cheatham JP, Zahn EM, McElhinney DB

Abstract
OBJECTIVES: This study sought to evaluate the incidence of and risk factors for conduit and stent-related outcomes following transcatheter pulmonary valve replacement (TPVR).
BACKGROUND: Stent fracture (SF) and right ventricular outflow tract (RVOT) reintervention are among the most important adverse outcomes after TPVR using the Melody valve (Medtronic, Minneapolis, Minnesota). The conduit environment and conduit preparation practices vary among patients who undergo TPVR.
METHODS: Data from 3 prospective Melody valve multicenter studies were pooled and analyzed. All patients who had successful implant of a Melody valve that was present at hospital discharge comprised the study cohort; patients who had TPVR into a stentless conduit comprised the analysis cohort. SF was diagnosed using protocol-specified or clinical fluoroscopy or radiography, and classified as major or minor.
RESULTS: Of 358 patients who underwent catheterization with intent to perform TPVR, 309 were discharged with the Melody valve in place (study cohort) of which 251 patients had TPVR into a stentless conduit (analysis cohort). Median follow-up was 5 years. New pre-stents were placed in 68% of patients with a stentless conduit, and 22% received multiple pre-stents. At 3 years, freedom from any SF and major SF was 74 ± 3% and 85 ± 2%, respectively, and freedom from RVOT reintervention was 85 ± 2%. New pre-stents were associated with longer freedom from SF and RVOT reintervention than was no pre-stent.
CONCLUSIONS: Risks of SF and reintervention after TPVR with a Melody valve were reduced by implantation of pre-stents, which has become standard practice. This study supports pre-stenting as an important component of TPVR therapy.

PMID: 28823778 [PubMed - indexed for MEDLINE]

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Acute Success of Balloon Aortic Valvuloplasty in the Current Era: A National Cardiovascular Data Registry Study.

JACC Cardiovasc Interv. 2017 Sep 11;10(17):1717-1726

Authors: Boe BA, Zampi JD, Kennedy KF, Jayaram N, Porras D, Foerster SR, Armstrong AK

Abstract
OBJECTIVES: The aim of this study was to evaluate practice patterns and outcomes of a contemporary group of patients undergoing balloon aortic valvuloplasty (BAV) for congenital aortic stenosis (AS).
BACKGROUND: BAV is the most common treatment for isolated congenital AS.
METHODS: Within the IMPACT (Improving Pediatric and Adult Congenital Treatments) Registry, all BAV procedures performed between January 2011 and March 2015 were identified. Procedures were separated into those performed for critical versus noncritical AS. Outcomes were stratified into optimal, adequate, and inadequate, with optimal and adequate outcomes defining "successful" procedures. Multivariate logistic regression was used to identify patient and procedural characteristics associated with unsuccessful BAV. Mortality and adverse events rates were compared across patient cohorts.
RESULTS: Of the 1,026 isolated BAV procedures captured in IMPACT, 718 (70%) were "successful." Success rates were 70.9% for noncritical AS (n = 916) and 62.7% for critical AS (n = 110). Multivariate analysis revealed that prior cardiac catheterization, mixed valve disease, baseline aortic valve gradient >60 mm Hg, baseline aortic insufficiency greater than mild, presence of a trainee, and multiple balloon inflations were associated with unsuccessful BAV in the noncritical AS cohort. There were no factors associated with unsuccessful procedures in the critical AS group. No procedural deaths occurred, but 2.4% of patients did not survive to hospital discharge. Adverse events occurred in 15.8% of all cases and were more frequent in procedures performed for critical AS (30.0% vs. 14.1%; p < 0.001).
CONCLUSIONS: BAV is an effective treatment for congenital AS with low rates of mortality and adverse events. Patients with critical AS have a higher risk for procedure-related adverse events.

PMID: 28882282 [PubMed - indexed for MEDLINE]

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Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis.

Eur Respir J. 2016 Oct;48(4):1081-1095

Authors: Goutaki M, Meier AB, Halbeisen FS, Lucas JS, Dell SD, Maurer E, Casaulta C, Jurca M, Spycher BD, Kuehni CE

Abstract
Few original studies have described the prevalence and severity of clinical symptoms of primary ciliary dyskinesia (PCD). This systematic review and meta-analysis aimed to identify all published studies on clinical manifestations of PCD patients, and to describe their prevalence and severity stratified by age and sex.We searched PubMed, Embase and Scopus for studies describing clinical symptoms of ≥10 patients with PCD. We performed meta-analyses and meta-regression to explain heterogeneity.We included 52 studies describing a total of 1970 patients (range 10-168 per study). We found a prevalence of 5% for congenital heart disease. For the rest of reported characteristics, we found considerable heterogeneity (I2 range 68-93.8%) when calculating the weighted mean prevalence. Even after taking into account the explanatory factors, the largest part of the between-studies variance in symptom prevalence remained unexplained for all symptoms. Sensitivity analysis including only studies with test-proven diagnosis showed similar results in prevalence and heterogeneity.Large differences in study design, selection of study populations and definition of symptoms could explain the heterogeneity in symptom prevalence. To better characterise the disease, we need larger, multicentre, multidisciplinary, prospective studies that include all age groups, use uniform diagnostics and report on all symptoms.

PMID: 27492829 [PubMed - indexed for MEDLINE]

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Pulmonary arterial hypertension in congenital heart disease: translational opportunities to study the reversibility of pulmonary vascular disease.

Eur Heart J. 2017 Jul 07;38(26):2034-2041

Authors: van der Feen DE, Bartelds B, de Boer RA, Berger RMF

Abstract
Pulmonary arterial hypertension (PAH) is a progressive and lethal pulmonary vascular disease (PVD). Although in recent years outcome has improved by new treatments that delay disease progression, a cure has not yet been achieved. In PAH associated with congenital heart disease (CHD), remodeling of the pulmonary vasculature reaches an irreversible phenotype similar to all forms of end-stage PAH. In PAH-CHD, however, also an early stage is recognised, which can be completely reversible. This reversible phase has never been recognised in other forms of PAH, most likely because these patients are only diagnosed once advanced disease has developed. We propose that the clinical model of PAH-CHD, with an early reversible and advanced irreversible stage, offers unique opportunities to study pathophysiological and molecular mechanisms that orchestrate the transition from reversible medial hypertrophy into irreversible plexiform lesions. Comprehension of these mechanisms is not only pivotal in clinical assessment of disease progression and operability of patients with PAH-CHD; specific targeting of these mechanisms may also lead to pharmacological interventions that transform 'irreversible' plexiform lesions into a reversible PVD: one that is amenable for a cure. In recent years, significant steps have been made in the strive to 'reverse the irreversible'. This review provides an overview of current clinical and experimental knowledge on the reversibility of PAH, focussing on flow-associated mechanisms, and the near-future potential to advance this field.

PMID: 28369399 [PubMed - indexed for MEDLINE]

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Cardiovascular profile score as a predictor of acute intrapartum non-reassuring fetal status in infants with congenital heart defects.

J Matern Fetal Neonatal Med. 2017 Dec;30(23):2831-2837

Authors: Miyoshi T, Katsuragi S, Neki R, Kurosaki KI, Shiraishi I, Nakai M, Nishimura K, Yoshimatsu J, Ikeda T

Abstract
OBJECTIVES: To investigate the predictive factors of urgent cesarean delivery (CD) due to acute intrapartum non-reassuring fetal status (NRFS) in infants with congenital heart defects (CHDs).
STUDY DESIGN: This was a retrospective review of 199 singletons prenatally diagnosed with a CHD and for whom vaginal delivery was attempted in our institution between 2007 and 2014. A cardiovascular profile (CVP) score was used to assess fetal heart failure.
RESULTS: The number of urgent CDs due to NRFS was 37 (18.6%). Fetuses with a CVP score ≤7 were significantly more likely to require urgent CD due to NRFS than those with a CVP score ≥8 (p < 0.001). Infants with right heart defects or biventricular cyanotic heart defects had a significantly higher frequency of urgent CD due to NRFS than those with other types of CHD (p = 0.017). Multivariate analysis showed that a CVP score ≤7, a birth weight <2500 g, and primipara status were significant predictors of urgent CD due to NRFS.
CONCLUSIONS: Fetal heart failure, low birth weight, and primipara status were revealed to be independent predictors of urgent CD due to acute intrapartum NRFS in CHD infants. The CVP score may be a useful echocardiographic marker in perinatal management planning.

PMID: 27892791 [PubMed - indexed for MEDLINE]

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Discrimination between QRS and T Waves Using a Right Parasternal Lead for S-ICD in a Patient with a Single Ventricle.

Pacing Clin Electrophysiol. 2017 Jul;40(7):904-907

Authors: Nishiyama T, Kimura T, Nishiyama N, Aizawa Y, Fukuda K, Takatsuki S

Abstract
The subcutaneous implantable cardioverter-defibrillator (S-ICD) is a useful option for patients with a single ventricle (SV) in which transvenous leads are contraindicated because of intracardiac shunts. We report a case in which a right parasternal lead placement was indicated for an S-ICD in a resuscitated patient with an SV. There were significant changes in the magnitude of R to T waves ratio in the right compared to the left parasternal lead position. Screening in the right parasternal position is effective for selecting appropriate patients with congenital heart disease for S-ICD implantations.

PMID: 28185283 [PubMed - indexed for MEDLINE]

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Pulmonary hypertension in congenital heart disease.

Future Cardiol. 2018 May 24;:

Authors: Pascall E, Tulloh RM

Abstract
Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg. We focus on its relevance in congenital heart disease, reviewing pathophysiology, diagnosis and management. Pulmonary hypertension is a relatively common complication of congenital heart disease, with adult prevalence between 5 and 10%. A multifactorial cause is recognized, relating to the size and nature of cardiac defect as well as environmental and genetic factors. More complex disease is increasingly recognized rather than pure Eisenmenger complex. Remodeling of the pulmonary vascular bed causes increased pulmonary vascular resistance diagnosed by a collection of investigations including echocardiography, exercise testing, cardiac catheterization, MRI and CT scanning. Management employs disease-modifying medications which are now used with increasing benefit.

PMID: 29792339 [PubMed - as supplied by publisher]

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