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Mind the Brain: Stroke Risk in Young Adults With Coarctation of the Aorta.

J Am Heart Assoc. 2018 06 01;7(11):

Authors: Felling RJ, Ringel RE

PMID: 29858372 [PubMed - indexed for MEDLINE]

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Intravascular assessment of coronary arteries in patients with cyanotic congenital heart disease.

EuroIntervention. 2019 Apr 20;14(17):1744-1750

Authors: Tarp JB, Radu MD, De Backer O, Jensen AS, Celermajer D, Sørensen K, Holstein-Rathlou NH, Engstrøm T, Søndergaard L

Abstract
AIMS: Patients with cyanotic congenital heart disease (CCHD) have been suggested to develop less atherosclerosis than the general population. This study aimed to evaluate the extent of coronary atherosclerosis in patients with CCHD using intravascular ultrasound (IVUS) and near-infrared spectroscopy (NIRS).
METHODS AND RESULTS: Fifteen patients with CCHD (women, 9; median age, 53 years) and 14 acyanotic controls (women, 6; median age, 53 years) were examined with IVUS-NIRS of the right coronary artery (RCA). The patients with CCHD presented with a larger RCA diameter than the controls (external elastic membrane diameter, 6.1 [4.8-6.7] vs. 4.7 [4.1-5.1] mm, respectively; p=0.01). No difference in area stenosis was found between the patients and the controls (15.8% [12.3-19.7] vs. 15.2% [9.5-18.8]; p=0.87). The presence of lipid by NIRS was noted in 43% of patients with CCHD and in 92% of the controls; however, no differences in total or max 4 mm lipid core burden index (LCBI) or in plasma lipid profile were found.
CONCLUSIONS: Patients with CCHD presented with larger coronary arteries than acyanotic controls. No difference in the degree of area stenosis in the coronary arteries was found between the cyanotic and acyanotic patients; however, a lower proportion of patients with CCHD showed a positive LCBI.

PMID: 30451687 [PubMed - indexed for MEDLINE]

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The effect on congenital heart diseases of maternal EPHX1 polymorphisms modified by polycyclic aromatic hydrocarbons exposure.

Medicine (Baltimore). 2019 Jul;98(30):e16556

Authors: Tao J, Li N, Liu Z, Deng Y, Li X, Chen M, Yu J, Zhu J, Yu P, Wang Y

Abstract
Polycyclic aromatic hydrocarbons (PAHs) may be 1 of etiologic factors responsible for congenital heart diseases (CHDs). Variations of the microsomal epoxide hydrolase (EPHX1) gene, as well as their possible interactions with PAHs exposure, may increase susceptibility to CHDs.This case-control study investigated the risk of CHDs in relation to the EPHX1 polymorphisms and assessed the interactions between these polymorphisms and PAHs exposure in 357 mothers of CHDs fetuses and 270 control mothers. Logistic regression models for the risk of CHDs were applied to determine the effect of genetic polymorphisms using additive, recessive, and dominant genetic models, as well as gene-exposure interactions. Multiple testing was adjusted by applying the false discovery rate (FDR).None of the maternal genetic polymorphisms of EPHX1 was associated with CHDs occurrence. Only the single nucleotide polymorphism rs1051740 was associated with an increased risk of right-sided obstructive malformations under the recessive model (adjusted odds ratio [aOR] = 1.852, 95% confidence interval [CI]: 1.065, 3.22) before FDR correction. A possible modifying effect of PAHs exposure on genetic polymorphisms of EPHX1 was found in susceptibility to CHDs, though no multiplicative-scale interactions between maternal exposure to PAHs and polymorphisms of EPHX1 gene were seento affect the risk of CHDs.The role of EPHX1 gene polymorphisms for CHDs need to be further evaluated, in particularly by interacting with PAHs exposure.

PMID: 31348278 [PubMed - indexed for MEDLINE]

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Knowledge of Life-Long Cardiac Care by Adolescents and Young Adults with Congenital Heart Disease.

Pediatr Cardiol. 2019 Jul 31;:

Authors: Fernandes SM, Verstappen A, Clair M, Rummell M, Barber D, Ackerman K, Dummer K, Mares JC, Cannobio MM, Reardon LC, Long J, Crumb S, Bhatt A, Takahashi M, Khairy P, Williams R, Landzberg MJ, Moe T, Pearson D, Adult Congenital Heart Association and the Adult Congenital Cardiac Care Associate Research Group

Abstract
National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13-20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.

PMID: 31367952 [PubMed - as supplied by publisher]

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Ductus arteriosus-associated infective endarteritis: Lessons from the past, future perspective.

Congenit Heart Dis. 2019 Aug 01;:

Authors: Callegari A, Burkhardt B, Relly C, Knirsch W, Christmann M

Abstract
BACKGROUND: Since routine clinical use of antibiotics as well as surgical and catheter-based closure of a patent arterial duct (PDA), PDA-associated infective endarteritis (PDA-IE) is rare but can still occur when the ductus is still open or as it closes. Thus, clinicians should maintain a high index of concern for patients with unexplained fever.
METHODS: We report on a PDA-IE in a young infant shortly after potentially delayed obliteration of a PDA. We discuss this case report by reviewing the literature in regard to the pathogenesis (infection primary or secondary to PDA thrombus formation), clinical (new heart murmur) and diagnostic findings (transthoracic echocardiography, total body MRI, laboratory findings), and clinical outcome during mid-term follow-up after successful antibiotic treatment.
RESULTS: A 7-week-old term infant with Staphylococcus aureus sepsis and a new heart murmur was diagnosed with PDA-IE by transthoracic echocardiography at the pulmonary artery end of an obliterated PDA. Broad diagnostic workup excluded other reasons for sepsis. After 4 weeks of antibiotic treatment the vegetation reduced in size and the infant recovered completely. A review of all cases of PDA-IE (in pediatric and adult patients) previously published was performed.
CONCLUSION: Nowadays, a PDA-IE is an extremely rare, but still life-threating condition that may even affect patients with a nonpatent ductus arteriosus shortly after its obliteration and should be considered as infective complication in preterms, neonates, and small infants. Therefore, in septic neonates with bacteremia, transthoracic echocardiography may be integrated in the diagnostic workup, especially by fever without source and clinical signs of IE such as a new heart murmur.

PMID: 31368237 [PubMed - as supplied by publisher]

Assessing hepatic impairment in Fontan-associated liver disease using the HepQuant SHUNT test.

Congenit Heart Dis. 2019 Aug 01;:

Authors: Lemmer A, VanWagner L, Gasanova Z, Helmke S, Everson GT, Ganger D

Abstract
BACKGROUND & AIMS: Fontan surgery for single ventricle congenital heart disease leads to Fontan-associated liver disease (FALD). Typical laboratory tests, imaging, and histopathology cannot predict clinical severity in FALD. HepQuant SHUNT is a proprietary serum test of hepatic function and physiology that has not yet been evaluated in FALD.
METHODS: Fourteen adult FALD patients at a single urban tertiary care center who underwent a Fontan procedure in childhood received HepQuant SHUNT testing between September 2015 and April 2018. The HepQuant SHUNT disease severity index (DSI) assesses global liver function and physiology from systemic and portal hepatic filtration rates (HFRs, clearances adjusted for body mass) of orally and intravenously administered cholates labeled with deuterium or 13C. The SHUNT parameter of the test measures portal systemic shunting from the ratio of Systemic HFR to Portal HFR. Chart review included laboratory tests, imaging, and clinical findings. Data from FALD patients were compared with data from healthy controls.
RESULTS: The average DSI and SHUNT values for the FALD patients were 17.5% and 36.1%, respectively, compared to 9.2% and 24.1%, respectively, for controls. Twelve (85.7%) FALD patients had a DSI >15 (upper limit of normal). Seven (50.0%) FALD patients had SHUNT values >30% (upper limit of normal), while three FALD patients (21.4%) had SHUNT values >49%. One FALD patient with preoperative SHUNT of 69%, who underwent a combined heart-liver transplant, had confirmed cirrhotic morphology within the liver explant.
CONCLUSIONS: This pilot study demonstrated that most FALD patients had hepatic impairment detected by abnormal DSI, with a smaller number having markedly elevated SHUNT values >49% suggesting intrinsic liver disease. The HepQuant SHUNT test may be useful in detecting and quantifying liver disease severity in FALD patients.

PMID: 31369200 [PubMed - as supplied by publisher]

Giant coronary artery aneurysm in a patient with LEOPARD syndrome.

Eur Heart J Case Rep. 2019 Aug 01;:

Authors: Bourgain M, Iriart X, Thambo JB, Cochet H

PMID: 31369669 [PubMed - as supplied by publisher]

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Right-heart catheterization using antecubital venous access in patients with complex congenital heart defects and Glenn anastomosis.

Turk Kardiyol Dern Ars. 2019 03;47(2):103-110

Authors: Atik Ugan S, Gökalp S, Çınar B, Saltık İL

Abstract
OBJECTIVE: Right-heart catheterization using the antecubital veins has recently regained attention, and studies demonstrating the feasibility and safety of antecubital access in adults have been published. However, no changes have been observed in the preferred entrance sites in right-heart catheterizations in children with congenital heart diseases. This article is a description of the technique and features of the antecubital venous approach in pediatric patients with complex congenital heart defects and a Glenn anastomosis.
METHODS: The data regarding a right cardiac catheterization through the antecubital fossa veins performed in 18 patients with various clinical indications between January 2014 and August 2017 were reviewed retrospectively and the results were assessed.
RESULTS: Ten patients (55%) were male and 8 patients were female. All of the patients but 1 had a complex congenital heart disease with a Glenn anastomosis. One patient had been operated on for a sinus venosus atrial septal defect and an abnormal pulmonary venous return and had a total occlusion of the superior vena cava. A diagnostic catheterization was performed in all cases. Additional procedures consisted of a balloon test occlusion of the pulmonary valve in 2 patients, a superior vena cava-right pulmonary artery anastomosis dilatation in 1, and abnormal veno-venous collateral occlusion with various devices in 2 patients.
CONCLUSION: The antecubital venous approach technique can be performed easily and safely for diagnostic and therapeutic catheterization in patients with complex congenital heart defects. The authors advocate that the antecubital venous approach should be the first site selected for right-heart catheterization, especially in patients with a Glenn anastomosis.

PMID: 30874516 [PubMed - indexed for MEDLINE]

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A single-centre, placebo-controlled, double-blind randomised cross-over study of nebulised iloprost in patients with Eisenmenger syndrome: A pilot study.

Int J Cardiol. 2019 Jul 04;:

Authors: Nashat H, Kempny A, Harries C, Dormand N, Alonso-Gonzalez R, Price LC, Gatzoulis MA, Dimopoulos K, Wort SJ

Abstract
BACKGROUND: Pulmonary arterial hypertension (PAH), is a rare and progressive disease with a high morbidity and mortality. Prostanoid pulmonary vasodilators are the most effective treatment for idiopathic and connective tissue associated PAH. Nonetheless, data examining their safety and efficacy in patients with Eisenmenger syndrome the most severe form of PAH, that is, related to cyanotic congenital heart disease (CHD-PAH) remains limited.
AIM: To evaluate safety and the clinical efficacy of nebulised iloprost in patients with Eisenmenger syndrome who are on maximum background oral PAH therapy.
METHODS: This pilot study was a randomised, double-blind, placebo-controlled, cross-over study. Patients were randomised to receive nebulised placebo or iloprost for 12 weeks and were then crossed over, with a 7-14-day washout. The primary endpoint was a change in 6-minute walk distance (6MWD).
RESULTS: Sixteen patients (11 females, aged 47.3 ± 9.8 year) were recruited, twelve completed the study. All were in WHO-FC III, with a resting oxygen saturation of 84 [81-87] % and a median 6MWD of 290 [260-300] m. There was no significant difference in the primary endpoint between nebulised iloprost (0[-4-9]m) and placebo (10 [-15-51]m), p = 0.58. There were no safety concerns with nebulised iloprost.
CONCLUSIONS: Our pilot study provides preliminary evidence that the addition of nebulised iloprost to maximum oral PAH therapy did not improve the primary endpoint of 6MWD. Nebulised iloprost was well tolerated with no significant safety concerns in CHD-PAH.

PMID: 31371115 [PubMed - as supplied by publisher]

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Changing trends in aortic valve procedures over the past ten years-from mechanical prosthesis via stented bioprosthesis to TAVI procedures-analysis of 50,846 aortic valve cases based on a Polish National Cardiac Surgery Database.

J Thorac Dis. 2019 Jun;11(6):2340-2349

Authors: Bartus K, Sadowski J, Litwinowicz R, Filip G, Jasinski M, Deja M, Kusmierczyk M, Pawlak S, Jemielity M, Jagielak D, Hendzel P, Suwalski P, Tobota Z, Maruszewski B, Kapelak B

Abstract
Background: Aortic valve replacement (AVR) is one of the most common surgeries performed in cardiac surgery operating rooms. We present actual, real life trends from the last 10 years for AVR interventions based on a multicentre National Registry of Cardiac Surgery Database.
Methods: The study population consisted of all 50,846 consecutive adult patients who underwent AVR between January 2006 and August 2016 in all cardiac surgery units in Poland. The main outcome measures were changes in the number of valves, characteristics of valves, operative details, and in-hospital mortality. Analysis consisted of all aortic valve (AV) procedures, including isolated AV surgery, combination procedures and patients who had a prior valve operation.
Results: In last 10 years, the number of procedures increased by 46%. Isolated surgical AVR was performed in 61.2%, AVR + coronary artery bypass grafting (CABG) in 23.9%, AVR + mitral valve replacement (MVR) in 7%, and transcatheter aortic valve implantation (TAVI) in 2.3% of patients. The mean patient age increased significantly from 61.4 years in 2006 to 66.1 years in 2016. Aortic stenosis was diagnosed in 73.4%, severe aortic insufficiency in 15.8% of cases. The most common valve pathology was calcification. Congenital valve dysfunction was diagnosed in 3.7% of cases. There was a 33.7% increase of bioprosthesis, and the most common implanted bioprosthesis was the Hancock II. The most common implanted mechanical prosthesis was the St. Jude Medical Mechanical Valve. In-hospital mortality decreased significantly over the 10-year period in all AV procedures from 10.9% in 2006 to 8.3% in 2016. Mean in-hospital mortality was: 4.0% in isolated surgical AVR, 5.4% in TAVI, 6.8% in AVR + CABG, 9.8% in AVR + MVR, 17.2% in AVR + MVR + CABG.
Conclusions: In the last ten years, the number of AV procedure has doubled. Mortality was significantly lower with all types of AV procedure despite very rapid aging of AVR patients. TAVI procedure rapidly grew in popularity. There is significant increase in the use of bioprosthesis.

PMID: 31372271 [PubMed]

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