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Prenatal to postnatal trajectory of brain growth in complex congenital heart disease.

Neuroimage Clin. 2018;20:913-922

Authors: Ortinau CM, Mangin-Heimos K, Moen J, Alexopoulos D, Inder TE, Gholipour A, Shimony JS, Eghtesady P, Schlaggar BL, Smyser CD

Altered brain development is a common feature of the neurological sequelae of complex congenital heart disease (CHD). These alterations include abnormalities in brain size and growth that begin prenatally and persist postnatally. However, the longitudinal trajectory of changes in brain volume from the prenatal to postnatal environment have not been investigated. We aimed to evaluate the trajectory of brain growth in a cohort of patients with complex CHD (n = 16) and healthy controls (n = 15) to test the hypothesis that patients with complex CHD would have smaller total brain volume (TBV) prenatally, which would become increasingly prominent by three months of age. Participants underwent fetal magnetic resonance imaging (MRI) at a mean of 32 weeks gestation, a preoperative/neonatal MRI shortly after birth, a postoperative MRI (CHD only), and a 3-month MRI to evaluate the trajectory of brain growth. Three-dimensional volumetric analysis was applied to the MRI data to measure TBV, as well as tissue-specific volumes of the cortical gray matter (CGM), white matter (WM), subcortical (deep nuclear) gray matter (SCGM), cerebellum, and cerebrospinal fluid (CSF). A random coefficients model was used to investigate longitudinal changes in TBV and demonstrated an altered trajectory of brain growth in the CHD population. The estimated slope for TBV from fetal to 3-month MRI was 11.5 cm3 per week for CHD infants compared to 16.7 cm3 per week for controls (p = 0.0002). Brain growth followed a similar trajectory for the CGM (p < 0.0001), SCGM (p = 0.002), and cerebellum (p = 0.005). There was no difference in growth of the WM (p = 0.30) or CSF (p = 0.085). Brain injury was associated with reduced TBV at 3-month MRI (p = 0.02). After removing infants with brain injury from the model, an altered trajectory of brain growth persisted in CHD infants (p = 0.006). These findings extend the existing literature by demonstrating longitudinal impairments in brain development in the CHD population and emphasize the global nature of disrupted brain growth from the prenatal environment through early infancy.

PMID: 30308377 [PubMed - indexed for MEDLINE]

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Diagnostic Work-Up of Pulmonary Hypertension - Think About Shunt Flow! A Case Report.

Am J Case Rep. 2019 Feb 05;20:154-158

Authors: Hinrichs L, Horacek M, Neudorf U, Schlosser T, Rassaf T, Totzeck M

BACKGROUND The diagnostic work-up and treatment of pulmonary hypertension can be complex. Pulmonary arterial hypertension (PAH), pulmonary hypertension second to lung or heart diseases and thromboembolic pulmonary hypertension, and other rare causes of pulmonary hypertension such as congenital heart diseases must be considered in the differential diagnostic work-up. CASE REPORT We report on a patient who has been treated for PAH over many years. At the age of 65, progressive symptoms required a complete re-evaluation. Here, a complex shunt vitium with a partial anomalous pulmonary venous return (PAPVR) and a sinus venosus defect (SVD) was diagnosed. CONCLUSIONS PAPVR is a rare congenital heart disease that is often associated with an SVD. It is usually diagnosed during childhood but may also be detected in adult patients who develop pulmonary hypertension and dyspnea as primary symptoms. The initial predominant left-to-right shunting associated with this disease may be undetected for years, with a slow development of right heart failure with right heart volume overload and pulmonary hypertension. Early detection is important, with a subsequent surgical intervention.

PMID: 30718448 [PubMed - in process]

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Reply to Nezic et al.

Eur J Cardiothorac Surg. 2018 09 01;54(3):611-612

Authors: Falk V, Baumgartner H

PMID: 29697765 [PubMed - indexed for MEDLINE]

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Genetic Testing and Pregnancy Outcome Analysis of 362 Fetuses with Congenital Heart Disease Identified by Prenatal Ultrasound.

Arq Bras Cardiol. 2018 Oct;111(4):571-577

Authors: Luo S, Meng D, Li Q, Hu X, Chen Y, He C, Xie B, She S, Li Y, Fu C

BACKGROUND: Congenital heart defects (CHD), as the most common congenital anomaly, have been reported to be associated with chromosomal abnormalities. Currently, patients with CHD are routinely offered karyotyping and chromosomal microarray (CMA) testing, but the genotype-phenotype relationship has not yet been fully established.
OBJECTIVE: To determine the type and frequency of chromosomal abnormalities in fetuses with CHD and to analyze pregnancy outcomes of fetuses with heart abnormalities caused by different genetic factors.
METHODS: A total of 362 cases of CHD were enrolled from 2009 to 2016. Detailed ultrasound and laboratory examinations, including karyotyping and CMA, were performed. Outcome was obtained from discharge summaries.
RESULTS: Of the 362 fetuses, 220 were found with an isolated CHD, and 142 had CHD with extracardiac anomaly. Among these 362 fetuses, 140 were identified with a genetic cause, including 111 cases with aneuploidy, 10 cases with abnormality of chromosomal structure by karyotyping and 19 cases with pathogenic or likely pathogenic copy-number variations (CNVs) by CMA. The detection rate is close to 38.7%. Only one (identified as trisomy 18 syndrome) in 140 positive cases resulted in perinatal death, with the others being induced. The remaining 222 cases had negative results for both genetic testing and of these cases, 56 resulted in induced labor, and 77 had natural childbirth or caesarean births. The pregnancy outcome of the remaining 89 cases was uncertain.
CONCLUSIONS: Karyotyping and CMA are effective and accurate prenatal genetic techniques for identifying fetal chromosomal abnormalities associated with cardiac defects, and this can assist clinical doctors to perform appropriate genetic counselling with regard to the etiology and outcome of CHD.

PMID: 30133550 [PubMed - indexed for MEDLINE]

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Accuracy of risk prediction scores in pregnant women with congenital heart disease.

Congenit Heart Dis. 2019 Feb 06;:

Authors: Kim YY, Goldberg LA, Awh K, Bhamare T, Drajpuch D, Hirshberg A, Partington SL, Rogers R, Ruckdeschel E, Tobin L, Venuti M, Levine LD

OBJECTIVE: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone.
DESIGN: Single-center retrospective study.
SETTING: Tertiary care academic hospital.
PATIENTS: Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014. CARPREG I and ZAHARA risk scores and modified World Health Organization (WHO) criteria were applied to each woman.
OUTCOME MEASURES: The primary outcome was defined by ≥1 of the following: arrhythmia, heart failure/pulmonary edema, transient ischemic attack, stroke, dissection, myocardial infarction, cardiac arrest, death during gestation and up to 6 months postpartum.
RESULTS: Of 178 women, the most common CHD lesions were congenital aortic stenosis (15.2%), ventricular septal defect (13.5%), atrial septal defect (12.9%), and tetralogy of Fallot (12.9%). Thirty-five women (19.7%) sustained 39 cardiac events. Observed vs expected event rates were 9.9% vs 5% (P = .02) for CARPREG I score 0 and 26.1% vs 7.5% (P < .001) for ZAHARA scores 0.51-1.5. ZAHARA outperformed CARPREG I at predicting adverse cardiovascular outcomes (AUC 0.80 vs 0.72, P = .03) but was not significantly better than modified WHO. Clinical predictors of adverse cardiac event were symptoms (P = .002), systemic ventricular dysfunction (P < .001), and subpulmonary ventricular dysfunction (P = .03) with an AUC 0.83 comparable to ZAHARA (P = .66).
CONCLUSIONS: CARPREG I and ZAHARA scores underestimate cardiac risk for lower risk pregnancies in these women. Of the three risk schemes, CARPREG I performed least well in predictive capacity. Clinical factors specific to the population studied are comparable to stratification schemes.

PMID: 30729681 [PubMed - as supplied by publisher]

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Quality of life among patients with congenital heart disease after valve replacement.

Semin Thorac Cardiovasc Surg. 2019 Feb 04;:

Authors: Pragt H, Pieper PG, van Slooten YJ, Freling HG, van Dijk APJ, Sieswerda GTJ, Bouma BJ, Post MC, Jongbloed MRM, Willems TP, Ebels T, van Melle JP

OBJECTIVE: Most studies concerning valve replacement in congenital heart disease (CHD) focus on surgical morbidity and mortality. However, with the increased life expectancy of these patients the focus shifts to quality of life (QOL). The aim of this study was to report and compare the QOL of CHD patients after valve replacement with the general population and to find factors associated with QOL.
METHODS: In a multi-center cross-sectional observational study of adults with CHD, QOL was measured with the RAND-36 questionnaire (a health related QOL questionnaire, with eight domains scoring from 0 to 100, higher scores indicate a better QOL). Functional status was measured with exercise capacity testing. Uni- and multivariable linear regression was used to find associations with QOL.
RESULTS: In total 324 patients with CHD and a prosthetic valve were included in this study. CHD patients with a valve replacement scored significantly lower than the general population on the general health, vitality, and social functioning domains (p<0.05). On the bodily pain domain, they scored significantly higher (less pain) (p<0.001). Higher NYHA class was associated with a lower QOL for all domains, reflecting the importance of functional capacity. Other variables related to aspects of QOL were age, gender, exercise capacity and employment status CONCLUSION: Adult patients with CHD and a prosthetic valve have lower scores on the QOL domains general health, vitality, and social functioning as compared to the general population. NYHA class was negatively associated with al QOL domains. Health care professionals should be aware of these patterns in counseling patients.

PMID: 30731194 [PubMed - as supplied by publisher]

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Factors influencing the use of subcutaneous or transvenous implantable cardioverter-defibrillators: results of the European Heart Rhythm Association prospective survey.

Europace. 2018 05 01;20(5):887-892

Authors: Boveda S, Lenarczyk R, Fumagalli S, Tilz R, Goscinska-Bis K, Kempa M, Defaye P, Marquié C, Capucci A, Ueberham L, Dagres N

The purpose of this European Heart Rhythm Association (EHRA) prospective snapshot survey is to provide an overview of the factors influencing patient selection for the implantation of a particular type of device: subcutaneous implantable cardioverter-defibrillator (S-ICD) or transvenous implantable cardioverter-defibrillator (TV-ICD), across a broad range of tertiary European centres. A specially designed electronic questionnaire was sent via the internet to tertiary reference centres routinely implanting both TV-ICDs and S-ICDs. These centres were asked to prospectively include and fill-in this questionnaire for all consecutive patients implanted with an implantable cardioverter-defibrillator (ICD) (both TV-ICD and S-ICD) during an 8-week period of time. Questions concerned standards of care and policies used for patient management, focusing particularly on the reasons for choosing one or the other type of ICD for each patient. In total 20 centres participated at the survey and entered individual data from a total of 429 consecutive patients (men 76.3%). Indication of implantation was primary prevention for 73% of the patients. Implanted devices were distributed between cardiac resynchronisation therapy (CRT) ones with back-up defibrillators (31.6%), single-chamber TV-ICD (29.5%), S-ICD (19.8%), and dual-chamber TV-ICD (19.1%).The rate of S-ICD shows the current penetration of this treatment in everyday practice. Main reasons favouring the use of an S-ICD were young age (66.7%), anticipated (38.9%) or previous (9.3%) lead-related complications, and elevated risk (18.5%) or previous device infection (7.4%). Importantly, the choice for this device was also based on patient preference (16.7%) or active lifestyle (13%). The three most frequent reasons for the use of a transvenous device were the option of antitachycardia pacing (43.2%), and logically, the current or expected need for CRT (40%) or for permanent pacing (39.6%). This snapshot survey with individual patient data provides a contemporary insight into ICD implantation and management in the European electrophysiology tertiary centres. It also helps to better understand the reasons which condition the choice between a S-ICD and a traditional TV-ICD. Finally, it gives a picture of the distribution of various types of ICD, few years after the introduction of the S-ICD in the Europe.

PMID: 29432525 [PubMed - indexed for MEDLINE]

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[Safety and efficacy of transcatheter closure of ruptured sinus of Valsalva aneurysm].

Zhonghua Xin Xue Guan Bing Za Zhi. 2018 Oct 24;46(10):799-803

Authors: Xiao JW, Niu MN, Wang QG, Zhang DZ, Han XM, Zhang P, Cui CS, Zhu XY

Objective: To investigate the safety and efficacy of transcatheter closure of ruptured sinus of Valsava aneurysm(RSVA). Methods: A total of 33 RSVA patients underwent transcatheter closure from January 2006 to March 2017 in our hospital were included in this retrospective study. The RSVA was diagnosed by echocardiography.Different type of occluders were applied for transcatheter closure based on the aortography results. All the patients were followed up after the procedure. Results: The patients were (37.6±12.1) years old,and the male patients accounted for 78.8%(26 cases).RSVA from right coronary sinus was found in 25 patients,and draining chamber was right atrium in 13 cases, right ventricle in 12 cases. RSVA from noncoronary sinus was diagnosed in 8 patients,and the draining chamber was right atrium. Aortography defined the narrowest diameter at the rupture site was (6.4±1.7)mm. The ratio of Qp/Qs was 2.2±0.5,and the mean pressure of pulmonary artery was 24.0(21.2,33.7)mmHg(1 mmHg=0.133 kPa). One patient developed serious occluder related aortic regurgitation and underwent surgery, transcatheter closure was successfully performed in 32 patients. The success rate of transcatheter closure was 97.0%. Two types of device were used in the study including small-waist double-disk ventricular septal defect(VSD) occluders in 20 cases and patent ductus arteriosus(PDA) occluders in 12 cases. During a median follow-up of 73.5(28.3,89.5) months, there were no infective endocarditis, residual shunt, thrombosis, device displacement,serious aortic regurgitation, serious arrhythmia or death.At the last follow-up, the left atrial diameter((37.4±6.5) mm vs. (41.5±5.3)mm,P<0.01),right atrial diameter((42.4±3.0) mm vs. (48.5±6.0)mm,P<0.01), right ventricular diameter((22.2±3.8) mm vs. (27.7±7.2)mm,P<0.01) and left ventricular end-diastolic diameter((51.3±4.9) mm vs.(55.0±4.3)mm,P<0.01)measured by echocardiography were all smaller than pre-procedural level. Conclusion: Transcatheter closure of RVSA is a safe and effective strategy and associated with a good long-term outcome.

PMID: 30369171 [PubMed - indexed for MEDLINE]

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A case of unguarded tricuspid valve orifice diagnosed accidentally in an adult.

Echocardiography. 2019 Feb 09;:

Authors: Abdelnabi MH, Shehata H, Saleh Y, Elgowelly M, Almaghraby A

Unguarded tricuspid orifice is the most extreme of tricuspid valve dysplasia with a very variable natural history. They can tolerate tricuspid regurgitation well, and they become symptomatic only if significant right ventricular dysfunction or atrial fibrillation occurs. Patients with a mild degree of right ventricular dysfunction can survive to adulthood and even reach old age. Surgical treatment is a difficult option due to variable natural history, and surgical results are not too encouraging.

PMID: 30738004 [PubMed - as supplied by publisher]

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High incidence and clinical characteristics of fibromuscular dysplasia in patients with spontaneous cervical artery dissection: The ARCADIA-POL study.

Vasc Med. 2019 Feb 10;:1358863X18811596

Authors: Talarowska P, Dobrowolski P, Klisiewicz A, Kostera-Pruszczyk A, Członkowska A, Kurkowska-Jastrzębska I, Gąsecki D, Warchoł-Celińska E, Światłowski Ł, Florczak E, Januszewicz M, Michałowska I, Józwik-Plebanek K, Szczudlik P, Błażejewska-Hyżorek B, Protasiewicz M, Odrowąż-Pieniążek P, Tekieli Ł, Michel-Rowicka K, Hanus K, Widecka K, Sołtysiak M, Tykarski A, Stryczyński Ł, Szczerbo-Trojanowska M, Hoffman P, Prejbisz A, Januszewicz A

The association between fibromuscular dysplasia (FMD) and spontaneous cervical artery dissection (SCeAD) has been recognized, but the available evidence on this relationship is scant. Therefore, the main goal of our study was to systematically evaluate FMD frequency, clinical characteristics and vascular bed involvement in patients with SCeAD. Among 230 patients referred to the ARCADIA-POL study, 43 patients (mean age 44.1 ± 8.9 years; 15 men and 28 women) with SCeAD were referred. Also, 135 patients with FMD were compared to patients with and without SCeAD. Patients underwent: ambulatory blood pressure measurements, biochemical evaluation, echocardiographic examination, and whole body computed tomographic angiography. FMD changes were found in 39.5% of patients with SCeAD. There were no differences in clinical characteristics between patients with SCeAD and FMD and those without FMD, except for a tendency towards a higher female ratio in SCeAD patients with FMD. There were no differences in other parameters describing target organ and SCeAD characteristics. Patients with SCeAD and FMD compared to those without SCeAD were characterized by a lower frequency of hypertension and a higher frequency of hyperlipidemia and history of contraceptive hormone use. Our study indicates a high incidence (39.5%) of FMD in subjects with SCeAD. Since there are no distinctive discriminating factors between patients with SCeAD and FMD and those without FMD, FMD should be suspected in all patients with SCeAD.

PMID: 30739593 [PubMed - as supplied by publisher]