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Utility of Fluid Assessment Based on the Intrathoracic Impedance Monitoring in a Peripartum Woman with Heart Disease.

Int Heart J. 2018 Mar 30;59(2):435-438

Authors: Daimon A, Kamiya CA, Sawada M, Ueda Y, Horiuchi C, Miyoshi T, Tsuritani M, Iwanaga N, Neki R, Okamura H, Kusano S, Yoshimatsu J

Recently, implantable cardioverter-defibrillators (ICD) have become capable of monitoring intrathoracic impedance to detect an increased fluid volume and heart failure. Pregnancy is a well-known cause of an increased body fluid volume; however, it is not clear whether the measurement of intrathoracic impedance by ICD is clinically useful for precisely detecting heart failure in pregnant women. We herein report the case of a 39-year-old woman with an ICD that had been implanted after an event of ventricular fibrillation due to severe aortic regurgitation with a bicuspid aortic valve. Elevated right ventricular pressure and brain natriuretic peptide levels were detected at 37 weeks of gestation and postpartum. At the same time, the ICD's stored fluid index gradually increased and exceeded the threshold on the 10th day after delivery. She was treated with diuretics and recovered from postpartum heart failure. The physiological volume changed in the perinatal period, but we were still able to detect heart failure by ICD. Intrathoracic impedance monitoring is effective in the perinatal field.

PMID: 29445057 [PubMed - indexed for MEDLINE]

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"How long will I continue to be normal?" Adults with a Fontan circulation's greatest concerns.

Int J Cardiol. 2018 Jun 01;260:54-59

Authors: du Plessis K, Peters R, King I, Robertson K, Mackley J, Maree R, Stanley T, Pickford L, Rose B, Orchard M, Stewart H, d'Udekem Y

BACKGROUND: Little is known about adults living with a Fontan circulation's concerns outside the scope of their clinical outcomes. We examined adults with a Fontan circulations' greatest concerns, as well as their concerns around anti-coagulation, pregnancy and finances.
METHODS: Adults with a Fontan circulation in the Australian and New Zealand Fontan Registry were invited to complete an anonymous online survey, of which 57 participated. A qualitative method approach using thematic analyses was used.
RESULTS: The greatest concerns for adults living with a Fontan circulation were fear of death/uncertainty around life expectancy which for many individuals colored their concerns around physical health, pregnancy and having children, quality of life and finances. Improving information about outcomes to patients with a Fontan circulation might alleviate uncertainties about their future.
CONCLUSIONS: Fear of death is the primary concern of adults with a Fontan circulation. It may require improved communication and targeted psychological interventions. Physical exercise incorporated as part of their lifestyle should be encouraged to alleviate physical concerns and also improve psychological well-being.

PMID: 29622455 [PubMed - in process]

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Bridging the psychological issues of living with the Fontan circulation.

Int J Cardiol. 2018 Jun 01;260:72-73

Authors: Brida M, Grbcic S, Holbein C, Veldtman GR

PMID: 29622457 [PubMed - in process]

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Spontaneous Closure of a Secundum Atrial Septal Defect.

Methodist Debakey Cardiovasc J. 2018 Jan-Mar;14(1):60-62

Authors: Wang SY, Welch TD, Elfenbein A, Kaplan AV

Spontaneous closure of an atrial septal defect (ASD) is well described in pediatric cardiology but may be less familiar to adult internists and cardiologists. We report a moderately sized 6-mm ASD that closed spontaneously without intervention. A literature review found that a smaller defect size and an early age of diagnosis are the most important predictors of closure. Possible mechanisms of a spontaneous ASD closure include adaptive endothelial migration, limited myocardial proliferation, and fibroblast migration with extracellular matrix deposition.

PMID: 29623173 [PubMed - in process]

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Abdominal imaging findings in adult patients with Fontan circulation.

Insights Imaging. 2018 Apr 05;:

Authors: Kim TH, Yang HK, Jang HJ, Yoo SJ, Khalili K, Kim TK

The Fontan procedures, designed to treat paediatric patients with functional single ventricles, have markedly improved the patient's survival into adulthood. The physiology of the Fontan circuit inevitably increases systemic venous pressure, which may lead to multi-system organ failure in the long-term follow-up. Fontan-associated liver disease (FALD) can progress to liver cirrhosis with signs of portal hypertension. Focal nodular hyperplasia-like nodules commonly develop in FALD. Imaging surveillance is often performed to monitor the progression of FALD and to detect hepatocellular carcinoma, which infrequently develops in FALD. Other abdominal abnormalities in post-Fontan patients include protein losing enteropathy and pheochromocytoma/paraganglioma. Given that these abdominal abnormalities are critical for patient management, it is important for radiologists to become familiar with the abdominal abnormalities that are common in post-Fontan patients on cross-sectional imaging.
TEACHING POINTS: • Fontan procedure for functional single ventricle has improved patient survival into adulthood. • Radiologists should be familiar with unique imaging findings of Fontan-associated liver disease. • Focal nodular hyperplasia-like nodules commonly develop in Fontan-associated liver disease. • Hepatocellular carcinoma, protein-losing enteropathy, pheochromocytoma/paraganglioma may develop.

PMID: 29623675 [PubMed - as supplied by publisher]

Adult Congenital Heart Disease with Pregnancy.

Korean Circ J. 2018 Apr;48(4):251-276

Authors: Niwa K

The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk. Most frequent complications during pregnancy and delivery are heart failure, arrhythmias, bleeding or thrombosis, and rarely maternal death. Complications of fetus are prematurity, low birth weight, abortion, and stillbirth. Risk stratification of pregnancy and delivery relates to functional status of the patient and is lesion specific. Medication during pregnancy and post-delivery (breast feeding) is a big concern. Especially prescribing medication with teratogenicity should be avoidable. Adequate care during pregnancy, delivery, and the postpartum period requires a multidisciplinary team approach with cardiologists, obstetricians, anesthesiologists, neonatologists, nurses and other related disciplines. Caring for a baby is an important issue due to temporarily pregnancy-induced cardiac dysfunction, and therefore familial support is mandatory especially during peripartum and after delivery. Timely pre-pregnancy counseling should be offered to all women with CHD to prevent avoidable pregnancy-related risks. Successful pregnancy is feasible for most women with CHD at relatively low risk when appropriate counseling and optimal care are provided.

PMID: 29625509 [PubMed]

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Long-Term Risk of Hemorrhagic Stroke in Young Patients With Congenital Heart Disease.

Stroke. 2018 Apr 06;:

Authors: Giang KW, Mandalenakis Z, Dellborg M, Lappas G, Eriksson P, Hansson PO, Rosengren A

BACKGROUND AND PURPOSE: The risk of ischemic stroke is increased in patients with congenital heart disease (CHD); however, data on the risk of hemorrhagic stroke, including intracerebral hemorrhage (ICH) and subarachnoid hemorrhage (SAH), are lacking.
METHODS: The Swedish Patient Register was used to identify all patients who were born with a diagnosis of CHD between 1970 and 1993. Each patient was compared with 10 randomly selected controls from the general population, matched for age, sex, and county. Follow-up data were collected until December 2011 for both cases and controls.
RESULTS: Of 21 982 patients with CHD, 70 developed ICH and 57 developed SAH up to the age of 42 years. CHD patients had more than an 8× higher risk (incidence rate ratio, 8.23; 95% confidence interval, 6-11.2) of developing ICH and almost an 8× higher risk of developing SAH (incidence rate ratio, 7.64; 95% confidence interval, 5.41-10.7) compared with controls. The absolute risk of ICH and SAH was low, with incidence rates of 1.18 and 0.96 cases per 10 000 person-years, respectively. Patients with severe nonconotruncal defects (incidence rate ratio, 16.5; 95% confidence interval, 5.63-51.2) or coarctation of the aorta (incidence rate ratio, 17.3; 95% confidence interval, 6.63-51.8) had the highest relative risk of developing hemorrhagic stroke, with incidence rates of 3.22 and 2.79 cases per 10 000 person-years, respectively.
CONCLUSIONS: The relative risk of hemorrhagic stroke among children and young adults with CHD was almost 8× higher than that of matched controls from the general population, although the absolute risk was low. The highest risk of ICH and SAH occurred in patients with severe nonconotruncal defects and coarctation of the aorta.

PMID: 29626133 [PubMed - as supplied by publisher]

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Understanding the experiences of fathers of children with congenital heart disease: An interpretative phenomenological analysis.

J Health Psychol. 2017 Sep;22(11):1447-1457

Authors: Gower C, Higgins A, Doherty N, McCormack D

Little is known about the experiences of fathers of children with congenital heart disease. Using interpretative phenomenological analysis, the lived experiences of six fathers were explored. Two master themes emerged: 'relinquishing and reclaiming control' and 'living in the shadow of illness'. Subordinate themes included playing the support act, a pragmatic/emotional dichotomy, and becoming the child's protector. Other themes included living in limbo and redefining 'normality' to encompass the condition. Theoretical links are made between these findings and existing literature while clinical implications include the need to involve fathers in decision-making and to respect their need to maintain control.

PMID: 26908585 [PubMed - indexed for MEDLINE]

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Imaging Adults With Congenital Heart Disease Part II: Advanced CMR Techniques.

J Thorac Imaging. 2017 Jul;32(4):245-257

Authors: Sahu A, Slesnick TC

Because of great strides in medical care, survival into adulthood has become a common expectation in patients suffering from nearly all forms of congenital heart disease. As this aging population expands, the utilization of cardiac magnetic resonance imaging in their care continues to grow. Magnetic resonance technology has developed exponentially over the last 2 decades, and several advanced techniques for imaging adults with congenital heart disease have moved from the purely research arena into routine clinical care. In particular, 4-dimensional phase-contrast imaging allows comprehensive hemodynamic assessment, myocardial characterization quantifies fibrosis, stress magnetic resonance imaging detects vulnerable myocardium and hemodynamic reserve, and 3-dimensional modeling enables preinterventional planning, all of which are now widespread in their applications.

PMID: 28632653 [PubMed - indexed for MEDLINE]

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Down syndrome and Moyamoya disease: unusual cause of stroke.

BMJ Case Rep. 2017 Jun 24;2017:

Authors: Tavares Bello C, Barreiros C, Gil I, Vasconcelos C

Down syndrome is a frequent clinical entity, being considered one of the most frequent chromosomal aberrations. It is characterised by a typical clinical phenotype and is associated with a heterogeneous group of organ and system-specific abnormalities. The cardiovascular system is commonly affected and if so, it may be associated with an increased morbidity and mortality. Cerebrovascular events in patients with Down syndrome are multifactorial, being possibly related to congenital heart disease, vascular malformations and traditional cardiovascular risk factors. Moyamoya disease is a rare chronic occlusive vascular disease causing stenosis of the distal portion of the internal carotid artery, which has been associated with Down syndrome. The authors report the case of a 26-year-old woman with Down syndrome who presented with an acute stroke secondary to Moyamoya disease. The case is noteworthy for the rarity of this clinicopathological entity, and serves as a reminder for the possible association between these two conditions.

PMID: 28647711 [PubMed - indexed for MEDLINE]