Journal Watch

Response to the letter, "The cardiovascular burden of congenital heart disease - not only in times of COVID-19".

Int J Cardiol. 2020 May 28;:

Authors: Tan W, Aboulhosn J

PMID: 32473922 [PubMed - as supplied by publisher]

Children's heart and COVID-19: Up-to-date evidence in the form of a systematic review.

Eur J Pediatr. 2020 May 30;:

Authors: Sanna G, Serrau G, Bassareo PP, Neroni P, Fanos V, Marcialis MA

The new coronavirus disease outbreak in 2019 (COVID-19) represents a dramatic challenge for healthcare systems worldwide. As to viral tropism, lungs are not the only COVID-19 target but also the heart may be involved in a not negligible percentage of the infected patients. Myocarditis-related cardiac dysfunction and potentially life-threatening arrhythmias are the main aftermaths. A few studies showed that myocardial injury in adult patients is often linked with a fatal outcome. Conversely, scientific evidence in children is sparse, although several reports were published with the description of a cardiac involvement in COVID-19 paediatric patients. In these young subjects, a background of surgically treated congenital heart disease seems to be a predisposing factor.Conclusion: This systematic review is aimed at summarizing all COVID-19 cases with a cardiac involvement published in paediatric age and trying to explain the underlying mechanisms responsible for COVID-19-related myocardial damage.What is Known:• Coronaviruses proved to be able to jump from animals to humans.• The outbreak of COVID-19 started from China (Dec 2019) and became pandemic.What is New:• Even in childhood, COVID-19 is not without the risk of cardiac involvement.• Myocarditis, heart failure, and arrhythmias are among the possible manifestations.

PMID: 32474800 [PubMed - as supplied by publisher]

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

Heart. 2019 07;105(14):1103-1108

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 - indexed for MEDLINE]

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Intracardiac mass in a 66-year-old woman.

Heart. 2019 07;105(14):1062-1128

Authors: Zhang W, Pei F, Wan J

PMID: 30923174 [PubMed - indexed for MEDLINE]

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Comorbidities and Survival in Patients With Lipodystrophy: An International Chart Review Study.

J Clin Endocrinol Metab. 2019 11 01;104(11):5120-5135

Authors: Akinci B, Oral EA, Neidert A, Rus D, Cheng WY, Thompson-Leduc P, Cheung HC, Bradt P, Foss de Freitas MC, Montenegro RM, Fernandes VO, Cochran E, Brown RJ

CONTEXT: Limited natural history data are available in patients with non-HIV-related lipodystrophy syndromes who never received disease-specific therapies, making interpretation of benefits of therapies in lipodystrophy syndromes challenging.
OBJECTIVE: We assessed the natural history of non-HIV-related generalized lipodystrophy (GL) and partial lipodystrophy (PL) in patients who have never received leptin or other lipodystrophy-specific therapies.
DESIGN/SETTING/PATIENTS: We conducted an international chart review of 230 patients with confirmed GL or PL at five treatment centers who never received leptin or other lipodystrophy-specific therapies. Patients were observed from birth to loss to follow-up, death, or date of chart abstraction.
OUTCOME MEASURES: Lifetime prevalence of diabetes/insulin resistance and select organ abnormalities, time to diabetes/insulin resistance, first organ abnormality, disease progression, and mortality were described.
RESULTS: Diabetes/insulin resistance was identified in 58.3% of patients. Liver abnormalities were the most common organ abnormality (71.7%), followed by kidney (40.4%), heart (30.4%), and pancreatitis (13.0%). Kaplan-Meier estimates of mean (SE) time to first organ abnormality were 7.7 years (0.9) in GL and 16.1 years (1.5) in PL (P < 0.001). Mean time to diabetes/insulin resistance was 12.7 years (1.2) in GL and 19.1 years (1.7) in PL (P = 0.131). Mean time to disease progression was 7.6 years (0.8) and comparable between GL and PL subgroups (P = 0.393). Mean time to death was 51.2 years (3.5) in GL and 66.6 years (1.0) in PL (P < 0.001).
CONCLUSIONS: This large-scale study provides comprehensive, long-term data across multiple countries on the natural history of non-HIV-related lipodystrophy.

PMID: 31314093 [PubMed - indexed for MEDLINE]

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Trends in the use of echocardiography in patients with Staphylococcus aureus bacteremia: an analysis using the Nationwide Inpatient Sample data.

Echocardiography. 2019 09;36(9):1625-1632

Authors: Urja P, Walters RW, Vivekanandan R, Kumar M, Abdulghani S, Hari Belbase R, Zook N, Mahesh Alla V

BACKGROUND: Infective endocarditis occurs in approximately 10%-30% of patients with Staphylococcus aureus bacteremia (SaB). Guidelines recommend echocardiography in patients with SaB and risk factors for infective endocarditis in the absence of any obvious source of infection. Herein, we explored the trends in the use of echocardiography in patients with SaB and its relationship to outcomes using a large national database.
METHOD: All patients with a principal discharge diagnosis of SaB were identified using the National Inpatient Sample database from 2001 to 2014. Procedure code 88.72 was used to identify echocardiography. Logistic regression models were estimated to identify the year-over-year trends in echocardiogram, predictors of use, and association with mortality.
RESULTS: From 2001 to 2014, there were 668 423 hospitalizations with SaB diagnosis and 86 387 (12.9%) had echocardiogram. The rate of echocardiography increased from 10.7% in 2001 to 15.2% in 2014 (ptrend  < 0.001). Major predictors of echocardiogram usage were younger age, male gender, presence of sepsis, valvular or congenital heart disease, prosthetic heart valve (PHV), cardiac implantable electronic device (CIED), hemodialysis, and drug abuse. The adjusted rates of echocardiography increased from approximately 10% to 15% in hospitalizations without risk factors for IE while for high-risk groups like PHV and CIED it remained constant at 30% and 19%, respectively. Echocardiography was associated with 31% lower odds of in-hospital mortality.
CONCLUSION: The increase in echocardiography rate was largely attributable to increased use in those without risk factors while usage in those with PHV and CIED remained much lower than expected. Echocardiography use was associated with lower risk-adjusted mortality. These findings require further study and confirmation.

PMID: 31471983 [PubMed - indexed for MEDLINE]

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Absence of the fetal right pulmonary artery complicated with coarctation of the aorta: Prenatal and postnatal diagnosis.

Echocardiography. 2019 09;36(9):1787-1789

Authors: Wang Y, Zhang J, Feng W, Wu Y, Yang L, Yin J

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiovascular malformation that can present as an isolated lesion or may be associated with other congenital heart malformations. Several studies have reported UAPA after birth. To our knowledge, the absence of the right pulmonary artery in the fetus has not been reported. Here, we report a rare case of fetal right pulmonary artery absence with aortic coarctation, which was confirmed by postpartum ultrasound and computed tomography angiography (CTA). Our case demonstrates that fetal echocardiography, especially the three-vessel view, is beneficial for the prenatal diagnosis of pulmonary artery malformations.

PMID: 31487058 [PubMed - indexed for MEDLINE]

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Long-term performance of bicuspid and quadricuspid aortic valves: Similarities and differences.

Echocardiography. 2019 09;36(9):1701-1705

Authors: Sharif D, Azzam E, Sharif-Rasslan A

While bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, quadricuspid aortic valve (QAV) is rare. The usual three-leaflet aortic valve is characterized by engineering advantages with superior long-term performance, and thus, degenerative changes and significant functional deterioration appear at advanced age.
AIM: Evaluation of long-term performance, similarities, and differences between QAV and BAV.
METHODS: Screening of 19 000 consecutive echocardiographic studies was performed.
RESULTS: BAV was reported in 131 subjects with a prevalence of 0.7%, while QAV was seen in 11 with a prevalence of 0.06%, P < .00001. Age of BAV patients was younger, 45 ± 20 years vs 62 ± 17 years in QAV, P < .05, with higher proportion of females in those with QAV, 40% vs 30%. Chamber diameters were similar in both groups. Higher atrial contraction-A-wave mitral peak velocities and longer E-wave deceleration times were found in subjects with QAV, P < .05 for both. Dilated ascending aorta was found in 25% of patients with BAV and in 18% of those with QAV, P = .2. Moderate and severe aortic valve stenosis were found in 21% of patients with BAV and in 27% of those with QAV, P = ns. More than moderate aortic regurgitation was found in 15.5% of BAV patients and in 9% of QAV, P = ns. Aortic valve infective endocarditis was found in 1.5% of BAV patients and in 9% of those with QAV.
CONCLUSIONS: BAV is a common congenital anomaly, while QAV is rare. Similar prevalence of significant valve disease and aortopathy was found in both anomalies, though at younger age in BAV patients.

PMID: 31490580 [PubMed - indexed for MEDLINE]

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Histological examination of explanted tissue-engineered bovine pericardium following heart valve repair.

Interact Cardiovasc Thorac Surg. 2020 01 01;30(1):64-73

Authors: Deutsch O, Bruehl F, Cleuziou J, Prinzing A, Schlitter AM, Krane M, Lange R

OBJECTIVES: Our goal was to present histopathological findings of human explants of a tissue-engineered bovine pericardium CardioCel (Admedus Regen Pty Ltd, Malaga, WA, Australia) used for heart valve repair in patients with congenital and acquired heart valve disease.
METHODS: Sixty patients underwent heart valve repair from May 2014 to November 2018 using CardioCel as a substitute for valve tissue. We identified 9 cases in which the CardioCel patch was explanted following valve repair and available for histomorphological analyses. CardioCel explants were evaluated histologically using haematoxylin and oeosin, Elastica van Gieson and immunohistochemical stains.
RESULTS: The indications for explantation were related to the CardioCel patch in 6 patients. Median time between the implantation and explantation was 242 (range 3-1247) days. We demonstrated a characteristic remodelling pattern with superficial coating of the tissue-engineered bovine pericardium by granulation tissue composed of histiocytes, few lymphocytes and fibrin. We had 2 cases with a multifocal nodular disruption, fragmentation and sclerosis of the decellularized collagen matrix with focal calcification after 795 and 1247 days in situ.
CONCLUSIONS: Our data suggest that the tissue-engineered CardioCel patch is initially tolerated in the valvular position in the majority of patients. However, we also experienced graft failures that showed degeneration with fragmentation of the collagen matrix and even 2 cases with focal calcification evident from the histopathological analysis. Further analyses of mid- and long-term performance are mandatory.

PMID: 31605480 [PubMed - indexed for MEDLINE]

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A novel mutation of ABHD5 gene in a Chanarin Dorfman patient with unusual dermatological findings.

Lipids Health Dis. 2019 Dec 28;18(1):232

Authors: Eskiocak AH, Missaglia S, Moro L, Durdu M, Tavian D

BACKGROUND: Chanarin Dorfman Syndrome (CDS) is a rare autosomal recessive disorder characterized by the multisytemic accumulation of neutral lipids inside the cytoplasmic lipid droplets. This condition is caused by mutations in the abhydrolase domain containing 5 gene (ABHD5). In CDS the skin involvement is the prevalent and always observed clinical feature, consisting of a non-bullous congenital ichthyosiform erythroderma (NCIE). Moreover, a variable involvement of the liver and neuromuscular system can be also observed. In this report, we aimed to perform the clinical and genetic characterization of a patient affected by CDS with atypical dermatological findings, considering this rare inborn error of neutral lipid metabolism.
METHODS: Genomic DNA samples obtained from patient and his parents were used to perform the sequencing of the ABHD5 exons and their intron/exon boundaries. Bioinformatic analyses were performed to investigate the possible effect of the identified mutation on protein structure.
RESULTS: Here we present the case of a 29-year-old male patient with CDS, who, for long time, has been misdiagnosed as pityriasis rubra pilaris (PRP). He has a history of increasing hyperlipidemia; hepatomegaly associated with hepatosteatosis was also detected. ABHD5 molecular analysis revealed a novel missense mutation, the c.811G > A (p.G271R). Bioinformatic investigations showed that the variant has a deleterious effect on ABHD5 function, probably causing an incorrect folding of the mutant protein.
CONCLUSIONS: These results highlihts the importance of genetic testing for ABHD5 in unresolved cases of patients presenting unusual skin lesions, that resemble PRP, associated with a history of hyperlipidemia and nonalcoholic fatty liver.

PMID: 31883530 [PubMed - indexed for MEDLINE]