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Increased incidence of infective endocarditis in patients with ventricular septal defect.

Congenit Heart Dis. 2018 Sep 27;:

Authors: Lee PT, Uy FM, Foo JS, Tan JL

BACKGROUND: Ventricular septal defect (VSD) is one of the most common congenital heart anomalies in childhood and there is an increasing prevalence of VSDs in the adult population. The long-term risk of infective endocarditis (IE) is of concern. The aim of this study was to clarify and compare the incidence of IE in adults with repaired and unrepaired VSDs.
METHODS: Patients with VSDs were identified using the Adult Congenital Heart Disease registry at the National Heart Centre Singapore. Patients were divided into Group 1 (repaired VSD) and Group 2 (unrepaired VSDs). The electronic medical records were searched for hospitalization due to IE during a 10-year period (October 2, 2007-October 1, 2017).
RESULTS: Four hundred seventy-nine patients (53% male) were identified, with a mean age of 35.0 ± 13.7 years. There were 164 patients (34.2%) in Group 1 and 315 patients (65.8%) in Group 2. In total, there were eight episodes of IE from six patients (3 male, mean age of 42.2 ± 20.7 years). Two patients had recurrent IE. The overall incidence of IE was 1.67/1000 y, and this is 11-15-fold higher compared to general adult population. The incidence of IE in Group 2 was 1.90/1000 y. There were no IE cases in Group 1.
CONCLUSION: Patients with VSDs, especially if unrepaired, carry a substantially increased risk of IE compared to the general population.

PMID: 30259666 [PubMed - as supplied by publisher]

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Contributors to disease-specific health knowledge in adults with congenital heart disease: A correlational study.

Congenit Heart Dis. 2018 Sep 27;:

Authors: Saef J, Sodhi S, Tecson KM, Al Rashida V, Mi Ko J, White KS, Ludbrook PA, Cedars AM

OBJECTIVE: Growth in the adults with congenital heart disease (ACHD) population represents a challenge to the health care infrastructure. As patients with chronic disease are increasingly held accountable for their own care, contributors to disease-specific health knowledge, which are known to correlate with patients' participation in care, merit investigation to design patient-focused interventions.
DESIGN: We conducted a single-site, cross-sectional study of ACHD patients. Investigators retrospectively gathered clinical data as well as psychometric and health status assessments completed at the time of enrollment.
OUTCOME MEASURES: We investigated the impact of clinical and psychological variables on Leuven Knowledge Questionnaire for Congenital Heart Diseases health knowledge composite scores (HKCS). Variables with significant associations were considered in a stepwise multivariable regression model to determine which combination of variables jointly explained variability in HKCS.
RESULTS: Overall HKCS was associated with the number of prior cardiac surgeries (r = 0.273; 95% CI: 0.050-0.467; P = .016), perceived stress (r = 0.260; 95% CI: 0.033-0.458; P = .024), SF-36 emotional well-being (r = -0.251; 95% CI: -0.451, -0.024; P = .030), history of noncardiac surgery (P = .037), cirrhosis (P = .048), and presence of implantable cardioverter-defibrillator (P = .028). On multivariable modeling, only the number of cardiac surgeries was found to correlate with HKCS.
CONCLUSIONS: While univariate correlations were found between HCKS and several other clinical and psychological variables, only number of prior cardiac surgeries independently correlated with disease-specific health knowledge in ACHD patients. These results suggest that clinical and psychological variables are not impediments to disease-specific health knowledge.

PMID: 30259669 [PubMed - as supplied by publisher]

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Age-related cardiovascular risk in adult patients with congenital heart disease.

Int J Cardiol. 2018 Sep 12;:

Authors: Häcker AL, Oberhoffer R, Hager A, Ewert P, Müller J

AIMS: Since the number of adults with congenital heart disease (ACHD) is increasing, age-related cardiovascular diseases become a relevant risk for ACHD. While previous studies investigated isolated risk factors only, this study examines the cardiovascular risk of ACHD based on the PROCAM scores.
METHODS AND RESULTS: From January 2017 to April 2018, 551 ACHD aged 30 years or older (43.9 ± 9.9 years, 48.3% female) were analyzed for their risk factors of major cardiovascular events within the next ten years using the PROCAM quick check and PROCAM health check. Compared to their individual reference, ACHD had a significantly lower absolute cardiovascular event risk in PROCAM quick check (ACHD: 2.5 ± 4.9%, reference: 3.8 ± 5.2%, p < .001) and PROCAM health check (ACHD: 1.8 ± 3.5%, reference: 3.9 ± 5.3%, p < .001). The relative risk of ACHD was 37% lower than in the general population calculated with the PROCAM quick test, and 57% lower with the PROCAM health check. Only 3.4% of the ACHD had a LDL cholesterol higher than 190 mg/dl, 8.3% had a HDL cholesterol lower than 40 mg/dl, and 26.0% had triglyceride higher than 150 mg/dl. Diabetes mellitus was prevalent in 4.0% of the ACHD and 10.9% were current smokers.
CONCLUSION: According to the PROCAM risk score, ACHD have a lower 10-year risk for major cardiovascular events compared to a healthy reference population. Whether this lower rate of the established risk factors leads to a lower rate of acquired cardiovascular disease has to be clarified in this particular population.

PMID: 30262228 [PubMed - as supplied by publisher]

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Aortic dissection and prophylactic surgery in congenital heart disease.

Int J Cardiol. 2018 Sep 12;:

Authors: Kuijpers JM, Koolbergen DR, Groenink M, Matthijs Boekholdt S, Meijboom FJ, Jongbloed MRM, Hoendermis ES, Duijnhouwer AL, Mulder BJM, Bouma BJ

BACKGROUND: Proximal aortic dilatation in certain congenital heart diseases (CHD) prompts concerns about dissection and consideration of prophylactic surgery. To evaluate contemporary prophylactic practice, we determined incidence of aortic dissection and prophylactic surgery in aortopathy-associated CHD, compared to Marfan syndrom (MFS) and controls.
METHODS AND RESULTS: We followed patients from the CONCOR adult CHD registry (2002-2015), with a native proximal aorta and aortopathy-associated CHD, comprising bicuspid aortic valve/aortic stenosis ('BAV/AS'; n = 2239) and aortic coarctation/conotruncal defects/univentricular heart/ventricular septal defect ('At-risk CHD'; n = 5439). As reference, we selected MFS (n = 356) and 'Control' (atrial septal defect, pulmonary stenosis; n = 2940) patients. Cumulative incidences of dissection and prophylactic proximal aortic replacement - considered competing events - were determined, and compared corrected for age and sex. Median follow-up was 6.7 years. Ten-year dissection-incidence was 0.3% (95%CI: 0.0-0.7) in BAV/AS and 0.2% (0.0-0.3) in At-risk CHD, both significantly lower than in MFS (4.1%; 1.8-6.4) and similar to Controls (0.1%; 0.0-0.3). Ten-year prophylactic-surgery incidence was 9.3% (7.6-11.0) in BAV/AS and 0.7% (0.5-1.0) in At-risk CHD, both significantly lower than in MFS (21.3%; 16.3-26.3) and higher than in Controls (0.1%; 0.0-0.3).
CONCLUSIONS: In contemporary practice, aortic-dissection incidence is low in adults with aortopathy-associated CHDs, while prophylactic-surgery incidence is high in BAV/AS. To reduce surgical burden, BAV/AS patients could benefit from more individualised prophylactic-surgery algorithms.

PMID: 30266350 [PubMed - as supplied by publisher]

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Management of Heart Failure in Adult Congenital Heart Disease.

Heart Fail Clin. 2018 Oct;14(4):569-577

Authors: Sabanayagam A, Cavus O, Williams J, Bradley E

There are more than 1 million adults with congenital heart disease (ACHD) in the United States. Heart failure (HF) is the most common late cardiovascular complication. These patients are challenging to manage given their diverse presentation, anatomy, and complex hemodynamics. Examination of underlying anatomy is crucial because many require late transcatheter and surgical interventions after developing HF. Management of arrhythmia is equally important because this can modify HF symptoms. A multidisciplinary team with expertise in the care of ACHD-HF is critical.

PMID: 30266365 [PubMed - in process]

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Brain Abscess in an Adult Patient With Congenital Heart Disease.

JAMA Cardiol. 2018 Sep 26;:

Authors: Frenchu KK, Ahmed MB

PMID: 30267087 [PubMed - as supplied by publisher]

What are the mechanisms for FALD and how can we prevent the progression?

Int J Cardiol. 2018 Sep 22;:

Authors: Ohuchi H

PMID: 30268385 [PubMed - as supplied by publisher]

Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease.

Heart. 2018 Sep 29;:

Authors: Ntiloudi D, Apostolopoulou S, Vasiliadis K, Frogoudaki A, Tzifa A, Ntellos C, Brili S, Manginas A, Pitsis A, Kolios M, Karvounis H, Tsioufis C, Goudevenos J, Rammos S, Giannakoulas G, CHALLENGE investigators

OBJECTIVE: Despite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population.
METHODS: Data were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018.
RESULTS: Overall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1-6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class.
CONCLUSIONS: Long-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population.

PMID: 30269081 [PubMed - as supplied by publisher]

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Late presentation of ALCAPA syndrome in an elderly Asian lady.

Med J Malaysia. 2016 08;71(4):217-219

Authors: Yew KL, Kang Z, Anum A

Coronary artery anomalies are often discovered incidentally during cardiac catheterization or computed tomography coronary angiography and may involve the affected coronary artery origin and its course. Coronary artery anomalies are associated with congenital heart disease. The affected coronary arteries may have an unusual high take off origin, origin from contralateral or non-coronary sinus, origin from the pulmonary artery, single coronary system or coronary artery fistula.

PMID: 27770127 [PubMed - indexed for MEDLINE]

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Electronic spatiotemporal image correlation improves four-dimensional fetal echocardiography.

Ultrasound Obstet Gynecol. 2018 Mar;51(3):357-360

Authors: Guasina F, Bellussi F, Morganelli G, Salsi G, Pilu G, Simonazzi G

OBJECTIVES: To compare the efficiency of electronic spatiotemporal image correlation (eSTIC) with that of conventional STIC to acquire four-dimensional (4D) fetal cardiac volumes of diagnostic quality.
METHODS: This was a randomized controlled trial of 100 patients in mid-gestation with normal sonograms. In half of the cases, STIC volumes of the fetal heart were obtained with a conventional mechanical 4D probe and in the remaining cases eSTIC volumes were obtained with an electronic 4D probe. Examinations were kept within the timeframe allotted for a standard examination of fetal anatomy, and a maximum of two attempts were made at obtaining a 4D cardiac volume. Datasets were stored on a computer and subsequently analyzed and categorized as being of optimal, satisfactory or inadequate quality, depending on whether or not it was possible to perform an extended basic cardiac examination, including obtaining a three vessels and trachea view, as well as a clear reconstruction of both the aortic and ductal arches in the sagittal plane.
RESULTS: The eSTIC volume datasets were more frequently of optimal or satisfactory diagnostic quality compared with conventional STIC (94% vs 76%, P < 0.0001). Failure to obtain an eSTIC volume of adequate quality was in all cases the consequence of an unfavorable position of the fetus.
CONCLUSIONS: Compared with a standard mechanical probe, the electronic 4D probe facilitates acquisition of sonographic cardiac volumes in mid-trimester fetuses. In our hands, eSTIC volumes of optimal or satisfactory diagnostic quality, allowing a detailed offline evaluation of the fetal heart, were obtained in more than 90% of cases within the time frame of a standard examination of fetal anatomy. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

PMID: 28337810 [PubMed - indexed for MEDLINE]