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Post-operative Outcomes in Children Undergoing Fontan Palliation in a Regionalized Surgical System.

Pediatr Cardiol. 2017 Dec;38(8):1654-1662

Authors: Martin BJ, Ross DB, Aklabi MA, Harder J, Dyck JD, Rebeyka IM

Evidence suggests that outcomes in pediatric cardiac surgery are improved by consolidating care into centers of excellence. Our objective was to determine if outcomes are equivalent in patients across a large regional referral base, or if patients from centers without on-site surgery are at a disadvantage. Since 1996, all pediatric cardiac surgery has been offered at one of two centers within the region assessed, with the majority being performed at Stollery Children's Hospital. All patients who underwent a Fontan between 1996 and 2016 were included. Follow-up data including length of stay (LOS), repeat surgical interventions, and transplant-free survival were acquired for each patient. The association between post-operative outcomes and home center was assessed using Kaplan-Meier survival analysis and Cox proportional Hazards models. 320 children (median age 3.3 years, IQR 2.8-4.0) were included; 120 (37.5%) had the surgical center as their home center. Cardiac anatomy was hypoplastic left heart syndrome in 107 (33.4%) subjects. Median LOS was 11 days (IQR, 8-17), and there were 8 in-hospital deaths. There were 17 deaths and 11 transplants over the course of follow-up. Five-year transplant-free survival was 92.5%. There was no difference in hospital re-intervention, late re-intervention, or survival by referral center (all p > 0.05). In multivariable analysis, home center was not predictive of either LOS (R 2 = -0.40, p = 0.87) or transplant-free survival (1.52, 95%CI 0.66, 3.54). In children with complex congenital heart disease, a regionalized surgical care model achieves good outcomes, which do not differ according to a patient's home base.

PMID: 28831564 [PubMed - indexed for MEDLINE]

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Electrocardiographic Markers of Appropriate Implantable Cardioverter-Defibrillator Therapy in Young People with Congenital Heart Diseases.

Pediatr Cardiol. 2017 Dec;38(8):1663-1671

Authors: Benítez Ramos DB, Cabrera Ortega M, Castro Hevia J, Dorantes Sánchez M, Alemán Fernández AA, Castañeda Chirino O, Cruz Cardentey M, Martínez López F, Falcón Rodríguez R

Implantable cardioverter-defibrillators (ICDs) are increasingly utilized in patients with congenital heart disease (CHD). Prediction of the occurrence of shocks is important if improved patient selection is desired. The electrocardiogram (ECG) has been the first-line tool predicting the risk of sudden death, but data in CHD patients are lacking. We aim to evaluate the predictive value of electrocardiographic markers of appropriate therapy of ICD in young people with CHD. We conducted a prospective, longitudinal study, in twenty-six CHD patients (mean age 24.7 ± 5.3 years) who underwent first ICD implantation. Forty-two age- and diagnosis-matched controls were recruited. Twelve-lead ECG and 24 h Holter analysis were performed during a mean follow-up of 38.9 months. Data included heart rate, heart rate variability, QRS duration (QRSd), QTc interval and its dispersion, Tpeak-Tend (Tp-Te) interval and its dispersion, presence of fragmented QRS (fQRS), T wave alternans, atrial arrhythmias, and non-sustained ventricular tachycardia. Implant indication was primary prevention in ten cases (38.5%) and secondary prevention in 16 (61.5%). Overall, 17 subjects (65.3%) received at least one appropriate and effective ICD discharge. fQRS was present in 64.7% of cases with ICD therapy compared with patients without events or controls (p < 0.0001). Tp-e and Tp-e dispersion were significantly prolonged in patients with recurrences (113.5 and 37.2 ms) versus patients without ICD discharge (89.6 and 24.1 ms) or controls (72.4 and 19.3 ms) (p < 0.0001 and p < 0.0001, respectively). On univariate Cox regression analysis QRSd (hazard ratio: 1.19 per ms, p = 0.003), QTc dispersion (hazard ratio: 1.57 per ms, p = 0.002), fQRS (hazard ratio: 3.58 p < 0.0001), Tp-e (hazard ratio: 2.27 per ms, p < 0.0001), and Tp-e dispersion (hazard ratio: 4.15 per ms, p < 0.0001), emerged as strong predictors of outcome. On multivariate Cox analysis fQRS, Tp-e and Tp-e dispersion remained in the model. The presence of fQRS, and both Tp-e and Tp-e dispersion are useful ECG tools in daily clinical practice to identify CHD patients at risk for appropriate ICD therapy.

PMID: 28871362 [PubMed - indexed for MEDLINE]

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Successful simultaneous transcatheter treatment for a secundum atrial septal defect complicated by valvular pulmonary stenosis in an infant.

Cardiol Young. 2018 Jul 11;:1-3

Authors: Asada D, Tomita H, Fujii T

Only few reports have described successful simultaneous transcatheter intervention for CHD in infants. We present an infant with secundum atrial septal defect complicated by valvular pulmonary stenosis. Percutaneous transcatheter pulmonary valvuloplasty was performed first, followed by transcatheter closure of the secundum atrial septal defect uneventfully. Simultaneous transcatheter correction is an effective therapeutic option even in infants.

PMID: 29991370 [PubMed - as supplied by publisher]

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Copy Number Variants and Exome Sequencing Analysis in Six Pairs of Chinese Monozygotic Twins Discordant for Congenital Heart Disease.

Twin Res Hum Genet. 2017 Dec;20(6):521-532

Authors: Xu Y, Li T, Pu T, Cao R, Long F, Chen S, Sun K, Xu R

Congenital heart disease (CHD) is one of the most common birth defects. More than 200 susceptibility loci have been identified for CHDs, yet a large part of the genetic risk factors remain unexplained. Monozygotic (MZ) twins are thought to be completely genetically identical; however, discordant phenotypes have been found in MZ twins. Recent studies have demonstrated genetic differences between MZ twins. We aimed to test whether copy number variants (CNVs) and/or genetic mutation differences play a role in the etiology of CHDs by using single nucleotide polymorphism (SNP) genotyping arrays and whole exome sequencing of twin pairs discordant for CHDs. Our goal was to identify mutations present only in the affected twins, which could identify novel candidates for CHD susceptibility loci. We present a comprehensive analysis for the CNVs and genetic mutation results of the selected individuals but detected no consistent differences within the twin pairs. Our study confirms that chromosomal structure or genetic mutation differences do not seem to play a role in the MZ twins discordant for CHD.

PMID: 29192580 [PubMed - indexed for MEDLINE]

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A predominance of hypertensive heart disease among patients with cardiac disease in Buea, a semi-urban setting, South West Region of Cameroon.

BMC Res Notes. 2017 Dec 04;10(1):684

Authors: Nkoke C, Makoge C, Dzudie A, Mfeukeu LK, Luchuo EB, Menanga A, Kingue S

OBJECTIVE: The pattern of heart disease is diverse within and among world regions. The little data on the spectrum of heart disease in Cameroon has been so far limited to major cities. We sought to describe the pattern of heart disease in Buea, the South West Region of Cameroon, a semi-urban setting. This was a descriptive cross-sectional study. Between June 2016 and April 2017 the echocardiography register of the Buea Regional Hospital was surveyed. We extracted data on the age, sex and echocardiographic diagnosis.
RESULTS: Out of 529 patients who underwent echocardiography, 239 (45.2%) had a definite heart disease. There were 137 (57.3%) females. The mean age was 58 years (range 3-94 years). The most common echocardiographic diagnoses were hypertensive heart disease (43.2%), dilated cardiomyopathies (17.6%), ischemic heart diseases (9.6%), and cor pulmonale (8.8%). Rheumatic heart disease affected 6.7% of the patients. The most common rheumatic heart disease was mitral stenosis followed by mitral regurgitation. Congenital heart disease represented 2.1% and 5 patients (2.1%) had pulmonary hypertension. Hypertensive heart disease is the most common cardiac disease in this semi-urban region in Cameroon. Rheumatic heart disease still affects a sizable proportion of patients. Prevention of cardiac disease in our setting should focus on mass screening, the treatment and control of hypertension.

PMID: 29202813 [PubMed - indexed for MEDLINE]

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Use of cardiovascular magnetic resonance in the evaluation of a giant right atrial appendage aneurysm: a case report and review of the literature.

BMC Res Notes. 2017 Dec 04;10(1):681

Authors: Sivakumaran L, Sayegh K, Mehanna E, Sanchez FW, Fields J, Cury R

BACKGROUND: Right atrial appendage aneurysms are rare entities that may have significant clinical consequences. When co-existing with atrial fibrillation, patients are at risk of developing pulmonary or paradoxical systemic emboli.
CASE PRESENTATION: An elderly patient presented to medical attention with symptoms of acute diverticulitis. On abdominal computed tomography, a massively enlarged right atrial appendage aneurysm was discovered incidentally. The aneurysm caused marked compression of the right ventricle and contained an area of hypoenhancement concerning for an intraluminal thrombus. Gadolinium-enhanced cardiovascular magnetic resonance was performed and first-pass perfusion images demonstrated that the area of hypoenhancement was in fact poorly mixing blood. The patient was therefore managed medically.
CONCLUSION: Right atrial appendage aneurysms are infrequently encountered cardiac abnormalities. In the literature, surgery has been offered to patients who are young, symptomatic, or have evidence of thrombotic disease, although whether this practice pattern is associated with superior clinical outcomes is unclear. In the present case, gadolinium-enhanced cardiovascular magnetic resonance imaging was used to exclude the presence of intraluminal thrombus in an elderly patient, which helped orient the patient's treating team towards medical-rather than surgical-therapy.

PMID: 29202817 [PubMed - indexed for MEDLINE]

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Near to One's Heart: The Intimate Relationship Between the Placenta and Fetal Heart.

Front Physiol. 2018;9:629

Authors: Camm EJ, Botting KJ, Sferruzzi-Perri AN

The development of the fetal heart is exquisitely controlled by a multitude of factors, ranging from humoral to mechanical forces. The gatekeeper regulating many of these factors is the placenta, an external fetal organ. As such, resistance within the placental vascular bed has a direct influence on the fetal circulation and therefore, the developing heart. In addition, the placenta serves as the interface between the mother and fetus, controlling substrate exchange and release of hormones into both circulations. The intricate relationship between the placenta and fetal heart is appreciated in instances of clinical placental pathology. Abnormal umbilical cord insertion is associated with congenital heart defects. Likewise, twin-to-twin transfusion syndrome, where monochorionic twins have unequal sharing of their placenta due to inter-twin vascular anastomoses, can result in cardiac remodeling and dysfunction in both fetuses. Moreover, epidemiological studies have suggested a link between placental phenotypic traits and increased risk of cardiovascular disease in adult life. To date, the mechanistic basis of the relationships between the placenta, fetal heart development and later risk of cardiac dysfunction have not been fully elucidated. However, studies using environmental exposures and gene manipulations in experimental animals are providing insights into the pathways involved. Likewise, surgical instrumentation of the maternal and fetal circulations in large animal species has enabled the manipulation of specific humoral and mechanical factors to investigate their roles in fetal cardiac development. This review will focus on such studies and what is known to date about the link between the placenta and heart development.

PMID: 29997513 [PubMed]

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Clinical and Parental Status of Patients with Congenital Heart Disease Associated Pulmonary Arterial Hypertension.

Isr Med Assoc J. 2017 08;19(8):489-493

Authors: Nir A, Berkman N

BACKGROUND: Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity is associated with increased morbidity and mortality.
OBJECTIVES: To evaluate the clinical and demographic characteristics of adults with congenital heart diseases (ADCHD) and PAH at a single center.
METHODS: A prospective registry of all patients with PAH was conducted between 2009 and 2015.
RESULTS: Thirty-two patients were identified. The mean age at the last visit was 44 years (range 19-77 years). The prevalence of PAH among all ADCHD patients was 6% (95% confidence interval 4.3%-8.4%). A much higher prevalence (53%) was found in patients with Down syndrome. Most patients with PAH had moderate or severe disease. Fifteen patients (47%) were treated with pulmonary vasodilators and 6 (19%) with combination therapy. The average World Health Organization functional class was 2.6. Morbidity included cerebral vascular accident or transient ischemic attack in 22% (mostly in patients with right-to-left shunt) and arrhythmia in 37% of the patients. During a median follow-up of 3.5 years, 5 patients (15.6%) died. Of 13 women with no mental retardation, 11 were or had been married and all had children (between 1 and 13, mean 3.3).
CONCLUSIONS: Patients with congenital heart disease and PAH have significant morbidity and mortality. PAH is more prevalent in patients with Down syndrome. While pulmonary pressure during the reproductive years was not always known, 27% of women with PAH at the time of the study were multiparous.

PMID: 28825767 [PubMed - indexed for MEDLINE]

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Early outcomes of percutaneous pulmonary valve implantation using the Edwards SAPIEN XT transcatheter heart valve system.

Int J Cardiol. 2018 Jan 01;250:86-91

Authors: Haas NA, Carere RG, Kretschmar O, Horlick E, Rodés-Cabau J, de Wolf D, Gewillig M, Mullen M, Lehner A, Deutsch C, Bramlage P, Ewert P

BACKGROUND: Patients with congenital or acquired heart defects affecting the pulmonary valve and right ventricular outflow tract (RVOT) commonly require multiple surgical interventions, resulting in significant morbidity. A less invasive alternative is percutaneous pulmonary valve implantation (PPVI). Though studies have previously reported the safety and efficacy of the early generation transcatheter heart valves (THVs), data on more recent devices are severely lacking.
METHODS AND RESULTS: We performed a multinational, multicentre, retrospective, observational registry analysis of patients who underwent PPVI using the Edwards SAPIEN XT THV. Of the 46 patients that were enrolled, the majority had tetralogy of Fallot as the underlying diagnosis (58.7%), and stentless xenograft as the most common RVOT anatomy (34.8%). Procedural success rate was high (93.5%), with a low frequency of periprocedural complications and adverse events (6.5% and 10.9%, respectively). At 30days post-procedure, NYHA class had improved significantly (90.6% were at NYHA I or II). The rate of moderate/severe pulmonary regurgitation had decreased from 76.1% at baseline to 5.0% at 30days, and the calculated peak systolic gradient had decreased from 45.2 (SD±21.3) mmHg to 16.4 (SD±8.0) mmHg, with these values remaining low up to 2years.
CONCLUSIONS: The data suggest the efficacy and safety of the SAPIEN XT THV in PPVI in common anatomies in patients with conduits, as well as those with native pulmonary valves or transannular patches. Continued data collection is necessary to verify long-term findings. CLINICALTRIALS.

PMID: 29017776 [PubMed - indexed for MEDLINE]

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Quality of life after surgical treatment of coarctation in long-term follow-up (CoAFU): Predictive value of clinical variables.

Int J Cardiol. 2018 Jan 01;250:116-119

Authors: Bambul Heck P, Pabst von Ohain J, Kaemmerer H, Ewert P, Hager A

BACKGROUND: We sought to analyze the quality of life and the predictive value of clinical variables from previous follow-up study in patients late after surgical treatment of aortic coarctation on the quality of life.
METHODS: All patients, who have participated in the prospective cross-sectional COALA Study in 2000 with a structural clinical investigation including blood pressure measurement and symptom-limited exercise test were contacted for the health-related quality of life questionnaire SF-36 from January 2013 through December 2014.
RESULTS: From 273 eligible patients, we received data from 135 patients, 9 of them died during the follow-up time at the median age of 46years (range 30-64years). Seventy-four patients did not participate in the study, other 64 patients moved to remote or unknown areas and could not be contacted. Quality of life was good in the fields of physical role and pain. However, patients reported a significant impairment in general health and in health transition, depending on the age. Arterial hypertension and variables from echocardiography or exercise testing from the COALA study were not predictive on functional health status.
CONCLUSION: Quality of life in patients late after aortic coarctation repair is fairly good compared with healthy controls. Impairments in general health and health transition depend mainly on age, can be explained due to numerous comorbidities and reinterventions in long-term. The predictive value of the commonly assessed clinical variables on quality of life is limited.

PMID: 29042092 [PubMed - indexed for MEDLINE]