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Infective endocarditis in adults with congenital heart disease remains a lethal disease.

Heart. 2017 Jul 28;:

Authors: Tutarel O, Alonso-Gonzalez R, Montanaro C, Schiff R, Uribarri A, Kempny A, Grübler MR, Uebing A, Swan L, Diller GP, Dimopoulos K, Gatzoulis MA

OBJECTIVE: Infective endocarditis (IE) is associated with significant morbidity and mortality. Patients with adult congenital heart disease (ACHD) have an increased risk of developing IE. The aim of this study is to describe the incidence, predictors of outcome and mortality associated with IE in ACHD in a contemporary cohort.
METHODS: All episodes of IE in adults with congenital heart disease referred to our tertiary centre between 1999 and 2013 were included in the study. Patients were identified from the hospital database. The diagnosis of endocarditis was established according to the modified Duke criteria. The primary endpoint of the study was endocarditis-associated mortality.
RESULTS: There were 164 episodes of IE in 144 patients (male 102, 70.8%). Mean age at presentation was 32.3±22.7 years. Out of these, 43% had a simple, 23% a moderate and 32% a complex lesion. It was at least the second bout of IE in 37 episodes (23%). A predisposing event could be identified in only 26.2% of episodes. Surgical intervention during the same admission was performed in 61 episodes (37.2%). During a median follow-up of 6.7 years (IQR 2.9-11.4), 28 (19.4%) patients died. Out of these, 10 deaths were related to IE (IE mortality 6.9%). On unvariate regression analysis, the development of an abscess (OR: 7.23; 95% CI 1.81 to 28.94, p<0.01) and age (OR: 1.05; 95% CI 1.01 to 1.10, p=0.03) were the only predictors of IE-associated mortality. There was no increase in IE cases at our centre during the period of the study.
CONCLUSIONS: IE-associated morbidity and mortality in a contemporary cohort of ACHD patients is still high in the current era.

PMID: 28754810 [PubMed - as supplied by publisher]

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Anomalous Aortic Origin of Coronary Arteries: A Single-Center Experience.

Semin Thorac Cardiovasc Surg. 2016 Winter;28(4):791-800

Authors: Fabozzo A, DiOrio M, Newburger JW, Powell AJ, Liu H, Fynn-Thompson F, Sanders SP, Pigula FA, Del Nido PJ, Nathan M

The aim of this article is to determine the clinical course and outcomes in subjects with anomalous aortic origin of coronary arteries (AAOCA), particularly after surgical repair. A single-center, retrospective review of patients with AAOCA with right or left interarterial or IM (IA or IM) or intraconal course from 1996-2014. Among 155 patients, median age at diagnosis was 8.5 (range: 0.1-50) years, and 65% were male. The AAOCA course was IA or IM in 151 (97%) and intraconal in 4 (3%). Anomalous right coronary artery (CA) was present in 127 (82%), of whom 52 (42%) had repair. Anomalous left CA (ALCA) was present in 28 (18%), of whom 20 (71%) had repair. In the surgical group, 70 (97%) had IA or IM CAs; CA unroofing was performed in 62 (86%). In univariable analysis, surgical management was associated with ALCA (28% vs 10%, P = 0.003), age > 10 years (median 11 vs 6 years, P < 0.001), symptoms (63% vs 13%, P < 0.001), and exercise restriction at the time of diagnosis (47% vs 13%, P < 0.001). In multivariable modeling, surgery was associated with chest pain or syncope (P < 0.001) and older age (P = 0.03). Major perioperative complications occurred in 4 cases (6%) and 1 patient had late aortic valve repair. In the surgical group, no patients died; in the observed group, 2 patients with anomalous right CA (2.3%) died of severe noncardiac comorbidities. In our center, surgery for AAOCA was not associated with mortality, and surgery was recommended in patients with ALCA with IA or IM course. Rare but serious surgical complications highlight the importance of long-term follow up of patients with AAOCA to develop evidence-based management guidelines.

PMID: 28417866 [PubMed - indexed for MEDLINE]

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Hospital-based surveillance of congenital rubella syndrome in Indonesia.

Eur J Pediatr. 2017 Mar;176(3):387-393

Authors: Herini ES, Gunadi, Triono A, Mulyadi AW, Mardin N, Rusipah, Soenarto Y, Reef SE

Congenital rubella syndrome (CRS) has serious consequences, such as miscarriage, stillbirth, and severe birth defects in infants, resulting from rubella virus infection during pregnancy. However, rubella vaccine has not yet been implemented in Indonesia. This study aimed (1) to estimate the incidence of CRS in Indonesia, (2) describe the clinical features of CRS at our referral hospital, and (3) pilot a CRS surveillance system to be extended to other hospitals. We conducted a 4-month prospective surveillance study of infants aged <1 year with suspected CRS in 2013 at an Indonesian hospital. Infants with suspected CRS were examined for rubella-specific IgM antibody or rubella IgG antibody levels. Of 47 suspected cases of CRS, 11/47 (23.4%), 9/47 (19.1%), and 27/47 (57.5%) were diagnosed as laboratory-confirmed, clinically compatible, and discarded CRS, respectively. The most common defects among laboratory-confirmed CRS cases were hearing impairment (100%), congenital cataracts (72.7%), microcephaly (72.7%), and congenital heart defects (45.5%).
CONCLUSION: The number of laboratory-confirmed CRS cases among Indonesian infants is high. Furthermore, hearing impairment is the most common clinical feature of CRS in infants. Our findings indicate the importance of implementation of rubella vaccine in Indonesia. Conducting hospital-based surveillance of CRS in other hospitals in Indonesia may be appropriate. What is Known: •Congenital rubella syndrome (CRS) has serious consequences in infants resulting from rubella virus infection during pregnancy. •The incidence of CRS in most developed countries has greatly decreased since implementation of rubella vaccination. •Rubella vaccine has not yet been implemented in many developing countries. What is New: •The number of laboratory-confirmed CRS cases among Indonesian infants was high. •Implementation of rubella vaccine into immunization programs in Indonesia is important because of the high number of CRS cases. •Our study highlights the need for ongoing prospective surveillance of CRS in Indonesia.

PMID: 28091778 [PubMed - indexed for MEDLINE]

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Cardiac Progenitor Cells Enhance Neonatal Right Ventricular Function After Pulmonary Artery Banding.

Ann Thorac Surg. 2017 Jul 28;:

Authors: Wehman B, Pietris N, Bigham G, Siddiqui O, Mishra R, Li T, Aiello E, Jack G, Wang W, Murthi S, Sharma S, Kaushal S

BACKGROUND: C-kit(+) cardiac progenitor cells (CPCs) have been shown to be safe and effective in large-animal models and in an early-phase clinical trial for adult patients with ischemic heart disease. However, CPCs have not yet been evaluated in a preclinical model of right ventricular (RV) dysfunction, which is a salient feature of many forms of congenital heart disease.
METHODS: Human c-kit(+) CPCs were generated from right atrial appendage biopsy specimens obtained during routine congenital cardiac operations. Immunosuppressed Yorkshire swine (6 to 9 kg) underwent pulmonary artery banding to induce RV dysfunction. Thirty minutes after banding, pigs received intramyocardial injection into the RV free wall with c-kit(+) CPCs (1 million cells, n = 5) or control (phosphate-buffered saline, n = 5). Pigs were euthanized at 30 days postbanding.
RESULTS: Banding was calibrated to a consistent rise in the RV-to-systemic pressure ratio across both groups (postbanding: CPCs = 0.76 ± 0.06, control = 0.75 ± 0.03). At 30 days postbanding, the CPCs group demonstrated less RV dilatation and a significantly greater RV fractional area of change than the control group (p = 0.002). In addition, measures of RV myocardial strain, including global longitudinal strain and strain rate, were significantly greater in the CPCs group at 4 weeks relative to control (p = 0.004 and p = 0.01, respectively). The RV free wall in the CPCs group demonstrated increased arteriole formation (p < 0.0001) and less myocardial fibrosis compared with the control group (p = 0.02).
CONCLUSIONS: Intramyocardial injection of c-kit(+) CPCs results in enhanced RV performance relative to control at 30 days postbanding in neonatal pigs. This model is important for further evaluation of c-kit(+) CPCs, including long-term efficacy.

PMID: 28760475 [PubMed - as supplied by publisher]

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Impact of previous cardiac surgery on long-term outcome of cavotricuspid isthmus-dependent atrial flutter ablation.

Europace. 2016 Jun;18(6):873-80

Authors: Dallaglio PD, Anguera I, Jiménez-Candil J, Peinado R, García-Seara J, Arcocha MF, Macías R, Herreros B, Quesada A, Hernández-Madrid A, Alvarez M, Di Marco A, Filgueiras D, Matía R, Cequier A, Sabaté X

AIMS: The aim of this study was to determine the acute and long-term outcome of radiofrequency catheter ablation (RFCA) for cavotricuspid isthmus-dependent atrial flutter (CTI-AFL) in adults with and without previous cardiac surgery (PCS), and predictors of these outcomes. Structural alterations of the anatomical substrate of the CTI-AFL are observed in post-operative patients, and these may have an impact on the acute success of the ablation and in the long-term.
METHODS AND RESULTS: Clinical records of consecutive adults undergoing RFCA of CTI-AFL were analysed. Two main groups were considered: No PCS and PCS patients, who were further subdivided into acquired heart disease (AHD: ischaemic heart disease and valvular/mixed heart disease) and congenital heart disease [CHD: ostium secundum atrial septal defect (OS-ASD) and complex CHD]. Multivariate analysis identified clinical and procedural factors that predicted acute and long-term outcomes. A total of 666 patients (73% men, age 65 ± 12 years) were included: 307 of them with PCS. Ablation was successful in 647 patients (97%), 96% in the PCS group and 98% in the No PCS group (P = 0.13). Regression analysis showed that surgically corrected complex CHD was related to failure of the procedure [odds ratio 5.6; 95% confidence interval (CI) 1.6-18, P = 0.008]. After a follow-up of 45 ± 15 months, recurrences were observed in 90 patients (14%), more frequently in the PCS group: absolute risk of recurrence 18 vs. 10.5%, relative risk 1.71, 95% CI: 1.2-2.5, P = 0.006. Multivariate analysis indicated that the types of PCS [OS-ASD vs. No PCS: hazard ratio (HR) 2.57; 95% CI: 1.1-6.2, P = 0.03 and complex CHD vs. No PCS: HR 2.75; 95% CI: 1.41-5.48, P = 0.004], female gender (HR 1.55; 95% CI: 1.04-2.4, P = 0.048), and severe LV dysfunction (HR 1.36; 95% CI: 1.06-1.67, P = 0.04) were independent predictors of long-term recurrence.
CONCLUSION: Radiofrequency catheter ablation of CTI-AFL after surgical correction of AHD and CHD is associated with high acute success rates. The severity of the structural alterations of the underlying heart disease and consequently the type of surgical correction correlates with higher risk for recurrence.

PMID: 26506836 [PubMed - indexed for MEDLINE]

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Ratchet syndrome in complex congenital heart disease.

Europace. 2016 Jun;18(6):819

Authors: Shoda M, Takeuchi D, Manaka T

PMID: 27001036 [PubMed - indexed for MEDLINE]

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Beyond Pressure Gradients: The Effects of Intervention on Heart Power in Aortic Coarctation.

PLoS One. 2017;12(1):e0168487

Authors: Fernandes JF, Goubergrits L, Brüning J, Hellmeier F, Nordmeyer S, da Silva TF, Schubert S, Berger F, Kuehne T, Kelm M, CARDIOPROOF Consortium

BACKGROUND: In aortic coarctation, current guidelines recommend reducing pressure gradients that exceed given thresholds. From a physiological standpoint this should ideally improve the energy expenditure of the heart and thus prevent long term organ damage.
OBJECTIVES: The aim was to assess the effects of interventional treatment on external and internal heart power (EHP, IHP) in patients with aortic coarctation and to explore the correlation of these parameters to pressure gradients obtained from heart catheterization.
METHODS: In a collective of 52 patients with aortic coarctation 25 patients received stenting and/or balloon angioplasty, and 20 patients underwent MRI before and after an interventional treatment procedure. EHP and IHP were computed based on catheterization and MRI measurements. Along with the power efficiency these were combined in a cardiac energy profile.
RESULTS: By intervention, the catheter gradient was significantly reduced from 21.8±9.4 to 6.2±6.1mmHg (p<0.001). IHP was significantly reduced after intervention, from 8.03±5.2 to 4.37±2.13W (p < 0.001). EHP was 1.1±0.3 W before and 1.0±0.3W after intervention, p = 0.044. In patients initially presenting with IHP above 5W intervention resulted in a significant reduction in IHP from 10.99±4.74 W to 4.94±2.45W (p<0.001), and a subsequent increase in power efficiency from 14 to 26% (p = 0.005). No significant changes in IHP, EHP or power efficiency were observed in patients initially presenting with IHP < 5W.
CONCLUSION: It was demonstrated that interventional treatment of coarctation resulted in a decrease in IHP. Pressure gradients, as the most widespread clinical parameters in coarctation, did not show any correlation to changes in EHP or IHP. This raises the question of whether they should be the main focus in coarctation interventions. Only patients with high IHP of above 5W showed improvement in IHP and power efficiency after the treatment procedure.

PMID: 28081162 [PubMed - indexed for MEDLINE]

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Pulmonary Hypertension in Infants, Children, and Young Adults.

J Am Coll Cardiol. 2017 May 23;69(20):2551-2569

Authors: Hansmann G

Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary, and systemic diseases contributing to morbidity and mortality. The underlying pulmonary hypertensive vascular disease (PHVD) is characterized by inflammation, pulmonary vascular remodeling, and angio-obliteration leading to elevated pulmonary arterial pressure and resistance, right ventricular dysfunction, left ventricular compression, and subsequent heart failure. Recent advancements in PH-targeted therapies and interventional-surgical procedures have contributed to the improvement in quality of life and survival in PH/PHVD. This paper gives an update on recent developments in the diagnosis and treatment of children and young adults with PH. The focus is on the heterogeneous etiology/pathophysiology of PH in the young, and particularly on PHVD associated with congenital heart disease. Moreover, new pharmacological, surgical, and interventional therapies and their practical application in progressive/severe pulmonary arterial hypertension with inadequate response to conventional pharmacotherapy are discussed.

PMID: 28521893 [PubMed - indexed for MEDLINE]

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Collagen turnover biomarkers and systemic right ventricle remodeling in adults with previous atrial switch procedure for transposition of the great arteries.

PLoS One. 2017;12(8):e0180629

Authors: Lipczyńska M, Szymański P, Kumor M, Klisiewicz A, Hoffman P

BACKGROUND: Myocardial fibrosis is a potential pathophysiological mechanism leading to systemic right ventricular (SRV) deterioration. We hypothesize that circulating levels of collagen deposition markers are elevated in patients with SRV remodeling and this elevation may have a predictive value.
METHODS: We prospectively evaluated 56 patients with D-TGA after the atrial switch procedure (mean age 25.6 ± 4.8, range 18-37 years; 67% males). Serum levels of procollagen type III amino-terminal propeptide (PIIINP), collagen type I carboxy-terminal telopeptide (CITP), procollagen type I N-terminal propeptide (PINP), matrix metalloproteinase (MMP 1, MMP 9) and a tissue inhibitor of matrix metalloproteinase (TIMP 1) and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) were measured and compared with healthy controls. The relationship between these serum markers, echocardiographic and cardiac magnetic resonance parameters and the outcome at a follow-up of 61 months (range, 24-85 months) was determined.
RESULTS: Compared with the healthy control group, the study group had significantly higher levels of TIMP1, PIIINP, CITP, PINP and NT-pro-BNP (p<0.05, each). The levels of PIIINP and CITP were significantly higher among patients with an SRV mass index above the mean value. The level of PIIINP was significantly higher among patients with an SRV EDV index above the mean value. CITP was significantly elevated in SRV late gadolinium enhanced (LGE) positive patients, compared to patients without SRV LGE. MMP9 and TIMP1 predicted an adverse clinical outcome on univariate Cox proportional hazard survival analysis in addition to well proven predictors of outcome (SRV EF and NYHA).
CONCLUSIONS: We demonstrated a pattern of altered collagen turnover adversely related with the indices of SRV remodeling and an adverse clinical outcome in patients with SRV.

PMID: 28767656 [PubMed - in process]

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Exercise performance in Ebstein's anomaly in the course of time - Deterioration in native patients and preserved function after tricuspid valve surgery.

Int J Cardiol. 2016 Sep 01;218:79-82

Authors: Müller J, Kühn A, Tropschuh A, Hager A, Ewert P, Schreiber C, Vogt M

OBJECTIVE: The purpose of this study was to assess the longitudinal follow-up of exercise performance in patients with native Ebstein anomaly and patients who had received previous tricuspid valve surgery.
PATIENTS AND METHODS: We identified 59 patients with Ebstein anomaly who had performed at least two cardiopulmonary exercise tests within a minimum of 6months. 22 patients (15 female, 29.0±13.4years) with native conditions, 37 patients (21 female, 32.1±15.3years) had undergone surgery of their tricuspid valve before baseline testing.
RESULTS: In the non-operated group peak workload (2.58±0.7 to 2.38±0.7W/kg, p=.019) declined during a mean follow-up of 4.6±2.6years, whereas peak workload remained stable in the surgical group (2.02±0.6 to 2.06±0.7W/kg, p=.229) throughout the follow-up of 3.4±2.3years. In the 18 patients who had their surgery less than one year prior to the first CPET the exercise performance even increased during the follow-up. Peak blood pressure as a marker of cardiac adaption to exercise increased in the follow-up examination only in the surgical group (159±23 vs. 171±30mmHg, p=.007). In the non-operated group blood pressure remained unchanged at follow-up (161±26 vs. 166±29, p=.358).
CONCLUSION: Exercise performance deteriorates in non-operated patients with Ebstein anomaly in contrast to patients after tricuspid valve surgery where exercise performance remains stable. These facts promote a surgical intervention latest at the time when exercise performance decreases.

PMID: 27232916 [PubMed - indexed for MEDLINE]